An Introduction to Cystic Fibrosis: For Patients and Their Families

An Introduction to Cystic Fibrosis: For Patients and Their Families
An Introduction to Cystic Fibrosis
For Patients and Their Families
SIXTH EDITION
James C. Cunningham, M.D.
Lynn M. Taussig, M.D.
An Introduction to Cystic Fibrosis
for Patients and Their Families
SIXTH EDITION
James C. Cunningham, M.D.
Fort Worth Cystic Fibrosis Center
Cook Children’s Medical Center
Fort Worth, Texas
Lynn M. Taussig, M.D.
National Jewish Medical and Research Center
Denver, Colorado
Editors
Leslie Hazle, M.S., R.N., C.P.N.
Judy Marciel, M.S.N., R.N., P.C.N.S., C.P.N.P.
Michelle Quirk
This information meets the CF Foundation
guidelines and standards for education
materials as reviewed by the Cystic Fibrosis
Foundation Education Committee.
Copyright 1989, 1991, 1994, 1999, 2003, 2013 Cystic Fibrosis Foundation
This publication was made possible by a generous unrestricted grant from Aptalis Pharma Inc.
3
DEDICATION
This book is dedicated, with love
and respect, to people with CF and
their families, who teach us daily
about life and CF.
5
Table of Contents
PREFACE
How to Use This Book................................................................................... 7
PART I
DEFINITION AND DIAGNOSIS
Chapter 1
Chapter 2
What Is Cystic Fibrosis?........................................................... 11
How CF Is Diagnosed.............................................................. 23
PART II
HOW CF AFFECTS THE BODY
Chapter 3
Chapter 4
Chapter 5
Chapter 6
Sweat Glands............................................................................ 35
Respiratory System................................................................... 41
Gastrointestinal System........................................................... 61
Reproductive System............................................................... 73
PART III
LIVING WITH CYSTIC FIBROSIS
Chapter 7
Chapter 8
Chapter 9
Chapter 10
Chapter 11
Chapter 12
Chapter 13
Chapter 14
Chapter 15
Overview: Living with CF........................................................ 85
Staying Healthy: What You Can Do ....................................... 87
Staying Healthy: Avoiding Germs..........................................101
Raising a Child with CF........................................................... 115
Adolescence and CF............................................................... 125
Adult Life with CF.................................................................... 131
The CF Care Team: Who They Are and What They Do....... 135
The Cystic Fibrosis Foundation.............................................. 143
The Future and CF Research.................................................. 151
PART IV
RESOURCES
Appendix A
Appendix B
Appendix C
Appendix D
Appendix E Medical Words to Know.......................................................... 159
Resource List ........................................................................... 183
Postural Drainage and Percussion......................................... 187
Daily Record of Diet, Treatments and Symptoms.......…......195
CF Foundation–Accredited Care Centers.............................199
7
PREFACE
How to Use This Book
If you are the parent or relative of someone who has just been
diagnosed with cystic fibrosis (CF) or an adult who has just learned
that you have CF, you may be worried, confused or scared. You
might have questions about CF and its treatment. This is normal
and expected. This book will help you learn more about CF. It will
answer questions, clear up confusion and tell you how you can
learn more and where to find support.
This book has 15 chapters. Many chapters end with “Review
Questions” so you can check your learning. As you read, it may
be helpful to write down your own questions in the space given.
Cover one chapter at a time. Bring this book and your questions
to meetings with your CF care team at your CF care center to get
answers. We are always learning more about CF and how to treat
it. This book cannot answer all of your questions, but it will help
you better understand CF and its treatment.
All of the words that are bolded and italicized at their first mention
in this book are in “Appendix A: Medical Words to Know” at the
back of the book. The “Resource List” in Appendix B can direct
you to additional information about many of the topics covered
in this book. Appendix C can help you learn more about postural
drainage and percussion, an airway clearance technique. Appendix
D has examples of blank forms you can copy and use to help you
organize your or your child’s CF care. Cystic Fibrosis Foundation–
accredited care centers are listed in Appendix E.
You may have been given a DVD to go with this book. It will
introduce you to CF and the CF care team. This book will cover the
same topics, but in more detail. Keep both the book and DVD for
future use, or to share with relatives and friends who want to learn
more about CF.
PART I
Definition and Diagnosis
WHAT IS CYSTIC FIBROSIS? • 11
Chapter 1: What Is Cystic Fibrosis?
Cystic fibrosis (usually called CF) is an inherited* disease. It
causes certain glands in the body to not work properly. These
glands are called the exocrine (outward-secreting) glands.
Exocrine glands normally make thin, slippery secretions
including sweat, mucus, tears, saliva and digestive juices. These
secretions move through ducts (small tubes) to the surface of
the body or into hollow organs, such as intestines or airways.
Exocrine glands and their secretions help the body function
normally.
Lung Cells
Thin, Slippery Mucus
Mucous Duct
Non-CF
In a mucous gland,
secretions easily move
through the duct.
Thick, Sticky Mucus
Mucous Duct
CF
CF causes the mucus
to be thick and sticky.
In CF, exocrine glands (except sweat glands) make mucus
that is too thick and sticky. This mucus plugs ducts and other
passageways. Mucous plugs are most often in the lungs and
intestines and can cause problems with breathing and digestion.
CF also affects sweat glands. The levels of salt (sodium and
chloride) and potassium in the sweat are too high. This may
cause problems during times of increased sweating.
CF does not affect endocrine glands. Endocrine glands and
exocrine glands are different. Endocrine (inward-secreting)
glands make hormones that pass into the blood.
*Bold and italicized words are
defined in Appendix A, “Words to
Know,” page 159.
12 • WHAT IS CYSTIC FIBROSIS?
What CF Is Not
Many people are confused about CF. Below are some common
topics that may cause confusion:
CF Is Not Contagious.
Because CF causes coughing, some people think you can “catch”
it. CF is genetic. You are born with it. No one can “catch” CF.
You cannot give it to anyone else. We will talk more about how a
person gets CF later.
CF Is Not Caused by Anything the Mother or Father Did, or
Did Not Do, Before or During Pregnancy.
Parents feel responsible for what happens to their children.
When their children have CF, some parents feel guilty. Nothing
parents did before or during pregnancy caused CF!
CF Does Not Affect the Brain.
Some people confuse CF with cerebral palsy or CP. CF does not
affect the brain, nervous system or the capacity to learn.
CF Has No Cure at This Time.
But with treatment, most people with CF grow up and lead active,
full lives. A lot of time, energy and money are being spent to find
new, better ways to treat CF.
CF does not affect the
brain. Children with CF
have the same ability to
learn as children without
the disease.
Is CF a New Disease?
About 30,000 people in
the United States have CF.
CF is not a new disease. As early as 1595, writings suggested
that there were children who likely had CF. “Cystic fibrosis of
the pancreas” was first described in 1936 by Dr. Guido Fanconi
of Switzerland. Dr. Dorothy Andersen at Babies and Children’s
Hospital in New York City wrote the first full report of CF as its
own disease in 1938. We have learned much more about CF
since then.
WHAT IS CYSTIC FIBROSIS? • 13
How Common Is CF?
CF is one of the most common genetic (inherited, hereditary)
disorders in white people. In the United States, CF occurs in one
of every 3,200 live white births. It occurs in one of every 15,000
live African American births. CF is uncommon in Asians and most
Native American tribes. It is being seen more often in Hispanics.
It occurs equally in males and females.
In the United States, about 800 to 900 persons are diagnosed
with CF yearly. There are approximately 30,000 people with CF
in the United States today. That number is rising as more people
are diagnosed, treated sooner and living longer.
Genes, Chromosones and How a Person Gets CF
In
the United States, one person in every 31 carries a mutation
of the CF gene. That means more than 10 million Americans
have one copy of a CF gene mutation. People who carry one CF
gene mutation do not have CF. But not all gene carriers can be
identified. Research to develop a test for all carriers is ongoing.
Every person with CF was born with CF. It is a genetic disease
that starts at conception. The age when symptoms start, type of
symptoms and the severity of the disease vary. In most people,
the lungs are the most affected part of the body; in others, the
digestive system may be affected the most.
Conception occurs when the mother’s egg joins the father’s
sperm. Both the egg and the sperm have thousands of genes.
Genes are the main units of heredity. They decide body traits
including eye and hair color, height, facial features and many
health conditions. All people probably have seven or eight
genes that could be connected with serious health problems
such as CF.
One in every 31 Americans
is a symptomless carrier of
one copy of the gene that
causes CF.
Genes are found on chromosomes. These are threadlike structures
found in the nucleus of all cells. Each chromosome has thousands
of different genes. Genes come in pairs. Each parent gives one
gene from his or her pair to make up a new pair for the child.
Genes pass on family traits from one generation to the next.
CF
Mutations
Genes (the dark lines on the
chromosome) are located on
chromosomes. They determine
things such as eye color, hair
color, height and CF.
Nucleus with Chromosomes
Chromosome
14 • WHAT IS CYSTIC FIBROSIS?
An Autosomal-Recessive Disease
CF is an autosomal-recessive disease. Autosomal means that
the CF gene is not on the sex chromosome. In other words, both
males and females can get CF. If the CF gene mutation is paired
with a normal gene, the normal gene will do all the work and the
CF gene mutation will be recessive. This person will not have
CF but will be a carrier. A carrier is someone with one CF gene
mutation and one normal gene. A carrier has no symptoms and
no disease.
Both parents of a child with CF are carriers of at least one CF
gene mutation. They inherited this gene from one of their
parents, who inherited the gene from one of their parents.
Every child with CF got genes for this disease from both the
mother and father. So a CF gene mutation is present on both
sides of the family.
When both parents are carriers and each gives the child a CF
gene mutation, there is no gene that works right. The child will
have CF.
More about the CF Gene
The gene that causes CF was identified in 1989. Genes are the
main units of heredity. Genes have small building blocks called
base pairs. The gene that causes CF has a mutation or change in
the genetic material, causing an exchange or loss of a building
block. Since 1989, more than 1,500 different mutations of the
CF gene have been found, and new ones are still being found.
Scientists are studying the effects of the different CF gene
mutations.
Finding the CF gene has helped us learn how and why this gene
causes CF. That information has led to research on new ways to
treat CF. Learn more about “The Future and CF Research” in
Chapter 15.
Can CF Carriers Be Identified?
If both parents are carriers of the CF
gene mutations, there are tests that
can be done prenatally to see if the
fetus has CF.
Because a carrier has no CF symptoms, we often do not know
if the person is a carrier until he or she becomes the parent of
a child with CF. Carriers of a CF gene mutation often can be
identified if a relative has CF. First, the blood or cells brushed
from the inside of the cheek of the person with CF are tested. If
the mutations in the CF gene are identified, then relatives can be
tested for those same mutations to see if they are carriers.
Finding CF carriers in the general public is harder. Known gene
mutations can be looked for, but we cannot know if the person
is a carrier of an unknown mutation. Also, most laboratories only
WHAT IS CYSTIC FIBROSIS? • 15
test for the most common mutations. Screening for all mutations
is difficult. So screening the general public for carriers of the CF
gene mutation is not possible.
What about the Risk of CF in Future Pregnancies?
When both parents carry a CF gene mutation, their children will
not always have CF. The CF gene is carried by half of the father’s
sperm and half of the mother’s eggs.
Inheriting CF
The father’s sperm with a CF gene
mutation may join with the mother’s
egg that has a CF gene mutation.
With two CF gene mutations, the
child has CF.
A sperm with a normal CF gene may
join with an egg that has a CF gene
mutation. The child will not have CF
but will be a carrier.
A sperm with the CF gene mutation
may join with an egg with a normal
CF gene. The child will not have CF
but will be a carrier.
How a Person Gets CF
To have CF, you must get one copy of the CF gene from each parent.
That means that each parent must be a carrier of the CF gene.
When two people who
are carriers have a child,
there is a 25 percent chance of
having a child with CF.
Carrier
Carrier
When one parent has CF
and one parent is a carrier,
there is a 50 percent chance
having a child with CF.
Has CF
Carrier
A sperm with a normal CF gene may
join with an egg with a normal CF
gene. The child will not be a carrier
and will not have CF.
The chart on this page shows what
might happen when the sperm and
egg of parents who carry a CF gene
mutation join.
Has CF
Carrier
Carrier
Not a
Carrier
When both parents carry a CF gene mutation, each baby has a:
• One in four (25 percent) chance of not being a carrier and not having CF
• Two in four (50 percent) chance of being a carrier but not having CF
• One in four (25 percent) chance of having CF
Each pregnancy has these odds, even if the parents already have a
child with CF.
Has CF
Has CF
Carrier
Carrier
16 • WHAT IS CYSTIC FIBROSIS?
How Do Altered Genes
Cause CF?
Outside Cell
Chloride Ion
CF is genetic. The healthy version
of the gene directs the making of
a protein called the Cystic Fibrosis
Transmembrane conductance
Regulator (CFTR). This protein forms
a channel so that salt (chloride and
sodium) and water can move in and out
of cells (called ion transport). The CF
gene mutation changes CFTR so that
CFTR Protein Channel
the protein does not work properly.
Chloride Ion
As a result, salt and water transport
Inside Cell
breaks down (abnormal ion transport).
The biggest changes are in the sweat
Chloride Ions move from inside to
and mucous glands. Knowing how CFTR works gives clues to
outside a cell through the CFTR
researchers about new potential treatments.
protein channel.
Sweat Glands
The CF gene mutation causes a chain of events that leads to
abnormal secretions. In CF, the sweat glands make sweat that
is more salty than in someone who does not have CF. Research
shows that abnormal salt transport in cells occurs in all of the
exocrine glands in CF. This problem is called the basic defect
in CF.
Mucous Glands
Normal mucus is thin and slippery. In the airways, it helps remove
dust and germs. It also lubricates the ducts and passageways of
other organs, like oil in a car.
In CF, the changes in salt transport cause changes in water
transport. The mucus loses some of its water and becomes
thick and sticky. This thick mucus blocks tubes and organ
passageways.
Mucous blockages cause many CF symptoms. The most serious
are:
Chronic Lung Disease
Lung airways clogged by thick mucus can get infected and
inflamed. Infections and inflammation cause chronic lung disease.
At some point, all people with CF get chronic lung disease.
WHAT IS CYSTIC FIBROSIS? • 17
Impaired Digestion
Digestion problems occur in 90 percent of people with CF. Thick
mucus keeps digestive enzymes from the pancreas from getting
to the intestines where they are needed to help digest food.
CF also affects other body parts including the liver and
reproductive system. For most people with CF, these problems
are not as bad as the effects on breathing and digestion. Later
chapters will look at the effects on the lungs, intestines and other
body parts.
We do not know everything about CF, but we have made great
strides. Scientists still study CF gene mutations, the CF gene’s
protein product and the basic defect to help treat the cause of
CF, not just treat the symptoms.
Sinuses
Sweat Gland
Lungs
Liver
Pancreas
Intestines
Reproductive
System
CF causes changes in many
parts of the body, including the
lungs, pancreas, liver, intestines,
reproductive system and sweat
glands.
18 • WHAT IS CYSTIC FIBROSIS?
Review Questions
Answers follow the questions. More than one answer may be
correct.
1. CF is a disease of the:
a.Blood
b.Brain
c.Exocrine glands
d.Endocrine glands
2. A person gets CF by:
a.Being near someone with CF who coughs
b.Touching a dirty tissue
c.Inheriting two CF gene mutations
d.Touching a person who has CF
3. Exocrine glands produce these:
a.Mucus
b.Sweat
c.Tears
d.Saliva
e.Hormones
f. Digestive juices
4. CF affects these parts of the body:
a.Liver
b.Brain
c.Lungs
d.Pancreas
e.Intestines
5. Which of these is a symptom of carriers of a
CF gene mutation?
a.Cough
b.Wheezing
c.Pneumonia
d.Abnormal stools
e.Difficulty gaining weight
f.All of the above
g.None of the above
6. When parents already have a child with CF, what are the odds that their next child will have CF?
a.The same odds apply to every pregnancy.
b.There is no chance the next child will have CF.
c.The parents will be less likely to have another child with CF.
d.The parents will be more likely to have another child with CF.
7. In CF, mucus and secretions become too:
a.Thin and dry
b.Thin and watery
c.Thick and sticky
8. Which statement about CF is true?
a.Smoking during pregnancy increases the
chance of having a child with CF.
b.CF is not caused by anything the parents did or did not do before or during the pregnancy.
c.Eating the right foods
during pregnancy reduces the chance of having a child with CF.
d.The chance of having a
child with CF is decreased by not drinking alcohol during pregnancy.
WHAT IS CYSTIC FIBROSIS? • 19
9. When does CF begin in a person?
a.At conception
b.At about 2 years of age
c.At different times for different people
d.When the person first begins to feel sick
10.Which of these statements about CF genes is true?
a.One in every 31 people in the United States carries a CF gene mutation.
b.Both parents of a child with CF carry a CF gene mutation.
c.If the mother or father carries a CF gene mutation, the child will have CF.
d.A child must inherit one CF gene mutation from each parent to have CF.
11.Which of the following statements is true?
a.Anyone can be tested to see if they carry a CF gene mutation with 100 percent accuracy.
b.A family member of a person with CF can be tested for carrier status with a high level of accuracy.
20 • WHAT IS CYSTIC FIBROSIS?
Answers
To learn more, turn to the page number shown after the answer.
1. C
CF is a disease of the exocrine glands. Page 11.
2. C
A person gets CF by inheriting two CF gene mutations. Page 15.
3. A, B, C, D, F
Exocrine glands produce mucus, tears, sweat, saliva and
digestive juices produced in the pancreas. Hormones are made
by the endocrine glands. Page 11.
4. A, C, D, E
CF affects the liver, lungs, pancreas and intestines. CF does not
affect the brain. Page 12, 17.
5. G
None of the above is a symptom of carriers of a CF gene
mutation. Carriers of CF have no symptoms of CF and do not
have CF. Page 14.
6. A
The same odds apply to every pregnancy. Because they already
have a child with CF, both parents are carriers of one CF gene
mutation. Therefore, they have a one-in-four chance of having a
child with CF from each and every pregnancy. Page 15.
7. C
In CF, mucus and secretions become too thick and sticky. Page 11.
8. B
It is true that CF is not caused by anything the parents did or
did not do before or during the pregnancy. Page 12.
9. A
CF is inherited and begins at the moment of conception. The
age when symptoms first appear varies; they can appear at birth
or anytime during a person’s life. Page 13.
10. A, B, D
It is true that one in every 31 people in the United States carries
a CF gene mutation, that both parents of a child with CF carry
a CF gene mutation and that a child must inherit one CF gene
from each parent to have CF. It is not true that if the mother or
father carries a CF gene mutation, the baby will automatically
have CF. Each parent gives one gene to make up the child’s gene
pairs. Both parents must be carriers and each give the child a CF
gene mutation for their child to have CF. Page 14-15.
WHAT IS CYSTIC FIBROSIS? • 21
11. B
It is true that a family member of a person with CF can be tested
for carrier status. In all likelihood, the CF gene mutation will be
the same for all members of the family. Because there are more
than 1,500 CF gene mutations, a person cannot be tested with
100 percent accuracy. Page 14.
Notes and Questions
Chapter 2: How CF Is Diagnosed
Diagnosing CF
Cystic fibrosis (CF) is diagnosed by using a medical history,
physical examination and laboratory tests.
Medical History
The doctor will get the person’s medical history, which describes
the person’s problems and symptoms. A family history, which is a
medical history of close relatives, is also helpful. The history plays
a big part in pointing the doctor to CF because CF is a genetic
disease.
Physical Examination
The doctor will do a full physical examination to look for CF signs
or other problems.
Laboratory Tests
Laboratory tests are needed to confirm CF and to find out how
CF has affected parts of the body. The sweat test is the standard
test for CF, and a genetic test can diagnose CF as well. Details
about CF diagnostic tests are found later in this chapter.
When Does CF Start?
CF starts at conception (before birth).
However, CF symptoms may not show
up for a while. The start of symptoms
varies from person to person. For
most people with CF the disease is
diagnosed or symptoms begin to show
up in the first 2 years of life.
About 10 percent to 15 percent of
people with CF have symptoms at
birth. They have a blocked intestine,
which is called meconium ileus. When
testing newborns, CF can usually be
diagnosed even before symptoms
show up.
People are born with CF, but some
will not show any symptoms of the
disease until later in their lives.
Most children are diagnosed within
the first 2 years of life. However,
some people are not diagnosed
until the teen years and even
adulthood.
24 • HOW CF IS DIAGNOSED
CF Symptoms
The type and severity of CF symptoms varies from person to
person. Many other health problems have symptoms like those
of CF. This can make CF hard to identify, or diagnose. CF may be
mislabeled or undiagnosed for years.
Common CF symptoms:
Salty-tasting skin
Slow weight gain, even with good appetite
• Abnormal bowel movements (chronic diarrhea; large, frequent, greasy, foul-smelling stools)
• Wheezing
• Cough and increased lung mucus
• Pneumonia
• Nasal polyps (small fleshy growths in the nose)
• Clubbing (enlargement) of the fingertips and toes
• Rectal prolapse (the rectum sticks out through the anus)
•
•
These symptoms are not seen only in people with CF. They may
be seen in other illnesses. Someone with CF may not have all of
these symptoms. When many of these symptoms are present,
however, the doctor may want to do a test for CF. The standard
test to diagnose CF is the sweat test.
Later chapters will cover how CF affects the body. They also
cover more about CF symptoms, why they occur and how they
are treated.
A sweat test is the standard way to diagnose CF. The test should be
done at a Cystic Fibrosis Foundation–accredited care center. These
care centers specialize in the diagnosis and treatment of CF.
HOW CF IS DIAGNOSED • 25
Sweat Test
CF is usually diagnosed by a sweat test. The sweat test measures
the amount of salt in a person’s sweat. CF affects exocrine
glands. Sweat glands are one kind of exocrine gland. The CF
gene mutation changes how the body handles salt. More salt is
lost in sweat. The amount of sweat is normal, but the salt in it is
high. This is the basis for the test used to diagnose CF.
The sweat test is an easy, accurate way to diagnose CF. The
sweat glands are made to produce sweat with a mild chemical
called pilocarpine and a little electricity (pilocarpine iontophoresis).
A gauze pad or piece of filter paper is put on the skin to collect
the sweat. Then, the area is wrapped in plastic for 30 minutes.
A small plastic strap that looks like a wristwatch also may be
used to collect the sweat. It collects the sweat into a small tube.
If the person has CF, the sweat will be high in salt (sodium and
chloride). If the sweat test shows increased salt, the doctor will
do a second sweat test to be sure.
The sweat test is easy, painless, accurate and inexpensive. But it
must be done and analyzed correctly. Sweat tests must be done
by trained, experienced people at a CF Foundation–accredited
care center. Laboratories at CF Foundation–accredited care
centers do many sweat tests each year, and have their sweat
tests reviewed by a CF center director.
Other Facts about Sweat Tests
Sweat tests can only diagnose CF. They cannot predict the
severity of CF. There is no connection between sweat salt level
and how severe the disease is.
Sweat tests cannot identify CF carriers. Carriers do not have CF,
so the salt in their sweat is normal.
What about Testing Relatives?
Because CF is genetic, siblings of a person with CF should be
tested. Siblings should be tested whether or not they show
symptoms of CF. The age when symptoms start varies from
person to person. Finding CF early means that treatment can
begin early. Early treatment can mean better health overall.
Other relatives (such as first cousins and half-siblings) should
be sweat tested if they have any CF symptoms or if the family is
concerned that they may have CF. This can be done with a sweat
test. It may also be done with a gene test.
26 • HOW CF IS DIAGNOSED
Gene Tests
About 90 percent of the time, gene tests can diagnose CF and
tell which gene mutations are present. This can be done with
a blood sample or cells brushed from the inside of the cheek
(buccal smear). Half the people with CF have two copies of the
F508del (sometimes written Delta F508 or ∆F508 mutation).
You may hear this called homozygous for F508del or
F508del/F508del. Most others with CF have one F508del
mutation and one other CF gene mutation. A person who
has one F508del and one of the hundreds of other CF gene
mutations is heterozygous for F508del.
The genes are found by tests done in laboratories that specialize
in genetic testing. There is some relation between the gene
mutation and CF severity, but it is not simple to predict. CF
varies even in people with the same gene mutations. This may
be caused by other genetic and non-genetic factors. Knowing
which CF gene mutations a person has does not give information
about how healthy the person will be, how well they will do in life
or how long they will live.
Recent research has shown that some treatments work for
people with certain gene mutations. Therefore, knowing
someone’s specific CF gene mutations may be important in
choosing therapies for that person.
Newborn Screening and Diagnosing Newborns
with CF
Newborn Screening for CF
Newborn screening (NBS) is a nationwide program to
identify babies who may have certain health conditions.
CF screening tests newborns to identify babies who are
at high risk of having the disease. If a baby has a positive
NBS for CF, the baby will need to have a sweat test to see
if he or she actually has CF.
By the end of 2009, all states included CF in their NBS
tests. NBS is done so that the diagnosis of CF can be
made early. Starting treatment early is important to keep
the child as healthy as possible. Early treatment can
lead to improved growth, keeping lungs healthy, fewer
hospital stays and a longer life.
The NBS test is done during the first 2 to 3 days after
birth, usually by the health care provider in the hospital.
Most states check for a chemical made by the pancreas,
an organ that helps with digestion. This chemical is
HOW CF IS DIAGNOSED • 27
called immunoreactive trypsinogen, or IRT. IRT is normally found
in small amounts in the body. When the pancreas is stressed
before a baby is born, more IRT is released into the baby’s blood.
IRT can be high if a baby is premature or had a stressful delivery,
or for other reasons. IRT also tends to be high in people who
have CF.
Some states use an IRT/IRT, or “IRT-only” method when they
screen newborns for CF. If the first IRT is high, it is considered
“positive,” or abnormal. Babies with a high-level IRT from the
blood collected in the hospital need to have a second IRT test at
the doctor’s office.
Most babies with a high IRT do not have CF. A positive or
abnormal IRT result simply means the baby could have CF. If the
second IRT test remains high, the parents are asked to bring the
baby to a CF Foundation–accredited care center for a sweat test.
The sweat test is used to diagnose, or to see, if the baby has CF.
Most states now use an IRT/DNA method for NBS tests. If the
IRT is high from the blood sample taken in the hospital, a second
test is done on the same blood sample to check for some of the
changes in the gene that causes CF. This is called a DNA test.
When an IRT or IRT/DNA test is positive, the sweat test is used
to see if the baby has CF.
Some babies that have a positive NBS test for CF do not have
CF. This is called a false-positive. Some babies with a negative
NBS test for CF do have CF. This is called a false-negative.
Therefore, anyone, at any age, who has symptoms of CF should
have a sweat test to see if they have CF.
Diagnosing Newborns with CF
When babies have a positive CF NBS test, the sweat test is used
to make the diagnosis, or to see if the baby actually has CF. The
sweat test should be done at a CF Foundation–accredited care
center. The sweat test can often be done on an infant as young
as 2 weeks old. Sometimes babies do not make enough sweat
to get an accurate sweat test. In those cases, the sweat test has
to be done when the baby is older and enough sweat can be
collected for the test.
Can CF Be Diagnosed Before Birth?
Some genetic conditions can be found before birth, or
prenatally. CF can sometimes be diagnosed before birth. This
can be done by amniocentesis or chorionic villus sampling
(CVS). In amniocentesis, a small amount of fluid around the
fetus is tested. In CVS, a small piece of placenta is tested. The
chromosomes in cells from an amniocentesis or CVS are studied
for known CF gene mutations. Also, CF can be diagnosed by
looking at proteins in amniotic fluid.
28 • HOW CF IS DIAGNOSED
Couples who are carriers of the CF gene who do not want a child
with CF can consider in vitro fertilization. The embryo can be
tested for CF before it is implanted in the mother.
These prenatal tests are not 100 percent accurate. Standard
prenatal genetic screening tests look for only the most common
CF gene mutations. There are more than 1,500 CF gene
mutations. The parent or parents could carry a gene mutation
that was not included in the prenatal testing. Because of this,
infants should still have a CF NBS test done.
Remember
False-positive and false-negative results do occur with CF NBS.
All infants with a positive NBS test should have a sweat test.
Although CF symptoms may start at any age, anyone with
symptoms of CF should have a sweat test done for diagnosis,
even if they have had a negative NBS.
In some cases, a genetic test to diagnose CF can be done
instead of a sweat test. A person has to have two abnormal CF
genes or CF mutations to be diagnosed with CF.
Because CF is inherited from the parents, siblings of a person
with CF should be tested. Because both parents inherited CF
gene mutations from their parents, siblings of the parents may
also carry the gene. First cousins of the person with CF should be
tested if they have CF symptoms.
Early diagnosis is important so that medical and nutritional
treatments can be started as soon as possible. Early treatments
lead to improved long-term health.
HOW CF IS DIAGNOSED • 29
Review Questions
Answers follow the questions. More than one answer may be
correct.
1. A person who has symptoms of CF should have a sweat test to see if he or she has CF.
a. True
b. False
2. Which of these symptoms are seen only in CF and not in any other disease?
a. Cough
b. Wheezing
c. Reoccurring lung infections
d. Difficulty gaining weight
e. All of the above
f. None of the above
3. Which kind of test is used to diagnose CF?
a. A test to check mental skills
b.A test to check muscle coordination
c. A test to check for too much salt in the sweat
d. A test to check for
congestion in the nose and lungs
4. Which of these are true about the sweat test for CF?
a.The test is simple, painless and reliable.
b.The test cannot identify carriers of the CF gene.
c.The test can tell if the person has a mild or severe case of CF.
d.The test should be
done by a medical lab that does many of these tests every year, such as a CF Foundation–
accredited care center.
5. When a person is found to have CF, who else also should be tested?
a.The parents of the person
b.Brothers and sisters of
the person
c.Anyone who has had close contact with the person
6.Which of the following tests may be used to diagnose CF?
a.Sweat test
b.Gene test using a blood sample
c.Gene test using cells from the inside the cheek
d.All of the above
30 • HOW CF IS DIAGNOSED
Answers
To learn more, turn to the page number shown after the answer.
1. A
It is true that a person who has symptoms of CF should have a
sweat test to see if he or she has CF. Page 24.
2. F
None of the above symptoms are seen only in CF. These
symptoms are seen in other diseases as well as CF. Page 24.
3. C
A test to check for too much salt in the sweat is used to
diagnose CF. Mental skills and muscle coordination are not
affected in CF. Congestion in the nose and lungs occurs in many
people who do not have CF. Page 25.
4. A, B, D
It is true that the test is simple, painless and reliable; it cannot
identify carriers of the CF gene and it should be done by a
medical lab that does many of these tests every year, such as a
CF Foundation–accredited care center. The sweat test can only
tell if a person has CF. The amount of salt in the sweat does not
tell you if the person has a mild or severe case of CF.
Page 25.
5. B
When a person is diagnosed with CF, the brothers and sisters
of the person also should be tested. The symptoms of CF
begin at different ages for different people. Brothers and sisters
could have CF and not know it. Because CF is inherited and not
contagious, there is no reason to test anyone simply because
they have had close contact with a person with CF. Page 23.
6. D
All of the above tests can diagnosis CF. The sweat test is used
most often. A positive sweat test is always repeated to confirm
the diagnosis. Gene testing also can be done or may be used if
the sweat result is unclear. Gene testing can be done on either
blood or cheek cells. Pages 25-26.
Notes and Questions
PART II
How CF Affects the Body
Chapter 3: Sweat Glands
Cystic fibrosis (CF) affects the exocrine glands. It causes these
glands to make abnormal secretions. The secretions made by
most exocrine glands in a person with CF are thick and sticky.
The thick and sticky secretions block organ passageways. How
CF affects the sweat glands is different than how CF affects other
exocrine glands.
How CF Affects the Sweat Glands
Skin
Salt
Sweat glands are exocrine glands, but the sweat in CF is not
thick and sticky. The sweat in CF is normal except for being too
salty. This is because of how the sweat glands work in someone
with CF. There is two to five times more salt in the sweat of a
person with CF. This high salt level is the reason a person is
tested for CF with the sweat test.
The Symptoms
The high amount of salt in a person’s sweat is a classic sign of CF.
The family is often the first to notice this sign. When they kiss the
child, the child’s skin may taste salty. People with CF lose more
salt than normal in their sweat. For people with CF, it is rarely a
problem because there is usually enough salt in food to replace
the salt they lose in their sweat. But, when they sweat more, they
lose more salt, and that can be a problem.
People sweat more:
Duct
Sweat Gland
In hot weather
• When they exercise
• With a fever
•
The symptoms of losing too much salt are:
Tiredness
• Weakness
• Vomiting
• Fever
• Muscle cramps
• Stomachache
•
Losing too much salt can lead to:
Dehydration
• Heat stroke
•
Sweat produced in a person with
CF has a very high level of salt.
36 • SWEAT GLANDS
Treatment
Losing too much salt is an easy problem to treat. People with CF
should eat salty foods and add salt to other foods. People with
CF should use salt freely. They should have their own salt shaker.
Having unlimited salt helps avoid problems.
When sweating more (in hot weather, during exercise or with a
fever), someone with CF should drink more and eat more salty
food. If symptoms of salt loss are present, the doctor may give
extra salt.
People with CF may need to avoid some times of heavy sweating
but should not limit their activity. No one should have too much
sun or heat. It is important to use common sense.
Remember
Everyone with CF loses more salt in their sweat than normal.
When sweating more (in hot weather, during exercise or with a
fever), people with CF should drink more and take more salt.
Learn the symptoms of salt loss in this chapter. If you see these
symptoms, call the doctor. Very few people with CF have to
watch how much salt they eat.
SWEAT GLANDS • 37
Review Questions
Answers follow the questions. More than one answer may be
correct.
1.CF affects sweat glands by:
a.Increasing the amount a person sweats
b.Increasing the amount of salt in sweat
c.Making the sweat thick and sticky
2.Which of these is a symptom of losing too much salt?
a.Fever
b.Muscle cramps
c.Tiredness and weakness
d. Stomachache and
vomiting
e. Heat stroke and dehydration
f. All of the above
3.Which statement about CF
and hot weather is not true?
a.A person with CF should eat salty snacks.
b.People with CF should never play in hot weather.
c.Most people with CF do a good job of adjusting their salt intake.
d.Extra fluids and salt may be needed when a
person with CF is sweating more.
38 • SWEAT GLANDS
Answers
To learn more, turn to the page number shown after the answer.
1. B
CF affects the sweat glands by increasing the amount of salt in
sweat. The amount of sweat in CF is normal and it is watery like
the sweat of someone without CF. Page 35.
2. F
All of the above are symptoms of losing too much salt. Page 35.
3. B
It is not true that people with CF should never play in hot
weather. People with CF do well in hot weather as long as they
drink more, eat salty snacks and do not overdo it. Page 36.
Notes and Questions
Chapter 4: Respiratory System
Breathing problems (also called respiratory or pulmonary
problems, which affect the lungs and the airways) are the most
serious problems associated with cystic fibrosis (CF). When
breathing problems are first noticed is different for each person
with CF. How severe these problems are will also differ for each
person. For most people with CF, lung disease affects how well
they do and how long they live. Many treatments are available to
keep the lungs as healthy as possible.
How the Respiratory System Works
The respiratory system has two main parts:
1. Upper respiratory tract — the nose and sinuses
The upper respiratory tract filters out particles such as dust and germs in the air we breathe. It also warms and moistens the air.
2. Lower respiratory tract — the airways and lungs
The lower respiratory tract starts at the trachea (the main windpipe).
How the Respiratory System Works
Air is filtered of dust and dirt, warmed and moistened in the nasal cavity and sinuses.
•
Sinuses
The air then passes down the trachea and into the bronchus of each lung.
•
Nasal Cavity
Lungs
Bronchioles
The air then moves into the bronchioles or smaller airways until it reaches the alveoli, or air sacs. This is where oxygen is taken from the air into the body and carbon dioxide is removed from the body.
•
Trachea
Bronchus
Bronchioles
Alveoli or
Air Sacs
42 • RESPIRATORY SYSTEM
The trachea branches into one bronchus (breathing tube or bronchial tube) for each lung. The bronchus then branches
into bronchial tubes that spread through the lungs like tree
branches. The smaller branches are called bronchioles. The
smallest branches end in tiny air sacs called alveoli. These air
sacs inflate and deflate like tiny balloons with each breath. It is
here that gas exchange occurs. Gas exchange is getting oxygen
into the body and waste gases out of the body.
The upper airways filter out most of the particles in the air we
breathe. But some do get through and into the lower respiratory
tract. If allowed to stay, they can block the tiny air tubes. This can
allow germs to grow and cause lung infections.
Healthy Airways
Germs, Dust
Thin, Slippery Mucus
Cilia
Healthy airways have a thin
layer of mucus, which can easily
be moved to the throat by the
cilia and then coughed out or
swallowed.
The Problem
Germs, Dust
Thick, Sticky Mucus
Cilia
In CF, the airway mucus is thick
and sticky, which stops the cilia
from moving the mucus to the
throat to be coughed out or
swallowed.
Exocrine glands in the airways help remove things such as dust
and germs that get into the lower respiratory tract. These glands
make thin, slippery mucus that lines the inside of the airways,
trapping particles and germs. Under the mucus, the airways
have cells with special cleaning tools called cilia. The cilia are like
small hairs. They move together to push the mucus to the throat.
The particles and germs we breathe in are trapped in the mucus
and then carried to the throat where they can be coughed out or
swallowed.
The main purpose of the respiratory system is gas exchange. Gas
exchange has two parts:
1. Oxygen exchange — the respiratory system takes oxygen from the air we breathe and transfers it to the blood. Oxygen is then carried to all body parts.
2. Waste gas exchange — the respiratory system transfers the waste gas (carbon dioxide) from the blood to the air we breathe out.
The Problem
CF affects both the upper and lower respiratory tracts. The most
important effects of CF are to the lower respiratory tract.
Lower Respiratory Tract
In CF, the airway mucus is too thick and sticky. It traps particles,
but the mucus is so thick and sticky that the cilia cannot easily
move the mucus to the throat. So, instead of cleaning the
airways, the mucus clogs them. When mucus blocks an airway, it
is called a mucous plug.
Mucous plugs keep air from getting into or out of some alveoli
(air sacs). Oxygen cannot get into the blood and carbon dioxide
cannot get out. The thick, sticky mucus in the small airways:
Gets in the way of air getting into and out of alveoli, and
• Makes it hard to remove particles and germs from the airways.
•
RESPIRATORY SYSTEM • 43
If not removed, mucous plugs can lead to lung infections and
lung damage. Lung infections cause problems for people with
CF because:
Infections cause inflammation, which can damage the cilia and airways, making them even less able to clear mucus, particles and germs from the lungs.
•
Infections often cause more mucus to be made. This can lead to more mucous plugs and worse infections.
•
Inflammation
Infection
More Mucus
The infection–inflammation
cycle causes lung damage
in CF.
This infection–inflammation
cycle causes lung damage.
Mucus plugs airways and
germs grow, causing
infection. The lungs react by
making more mucus to clean
the airways, but the mucus
plugs more airways. The
infection grows more and the
cycle repeats.
People with CF are at risk for
certain lung infections. There
are many bacteria or germs
that cause CF lung infections.
Two are:
•
Staphylococcus aureus (or simply staph, pronounced “staff”)
Pseudomonas aeruginosa (or simply Pseudomonas,
pronounced “soo-doe-mow-nas”)
•
Treatment of CF in the lower airways focuses on clearing the
airways and treating the bacteria in the lungs. (See the Treatment
section later in this chapter on page 46.)
Upper Respiratory Tract
CF also affects the upper respiratory tract — the nose and
sinuses. Although CF does not often cause big problems there,
two conditions may occur:
Sinusitis
Sinusitis can occur in CF. Sinus inflammation is caused by thick,
sticky mucus blocking the sinuses. This blockage may lead to
sinus infections. Sinusitis may be treated with antihistamines and
decongestants to open up the sinuses, and with antibiotics to
control infections. Sinus irrigation (flushing) or other treatment
may be needed. Sometimes, surgery may be needed to open up
and drain the sinuses.
44 • RESPIRATORY SYSTEM
Nasal Polyps
Nasal polyps are small, fleshy growths in the nose. These are less
common than sinusitis. We do not know what causes polyps.
They may be caused by problems with mucous glands in the
nose. Nasal polyps do not always need treatment. If they block
the nose or cause problems, then medicines or surgery may be
needed.
Symptoms
Most of the respiratory symptoms of CF are caused by thick
mucus blocking airways. When respiratory symptoms of CF
are first noticed, and how severe they are, is different for each
person. The symptoms increase over time as CF lung disease
gets worse. From time to time, the lungs of a person with CF get
worse. This is called a pulmonary exacerbation or flare-up. Lung
infections cause exacerbations. A person with CF will need extra
treatments during an exacerbation.
It is important to know what symptoms are normal for a person
with CF, and then to notice when the symptoms are worse. If
CF respiratory symptoms become worse, the person with CF
usually needs treatment. New or worsened symptoms should
be reported to the CF care provider early, so that treatment can
begin early.
Some symptoms are:
•
Coughing
•
Coughing up mucus (sputum, phlegm)
•
Wheezing
•
Repeated lung infections (bronchitis or pneumonia)
Shortness of breath or labored breathing (cannot “catch” your breath)
•
•
Difficulty exercising, getting tired or needing to be less active
Tests
Your CF care team learns a lot by hearing your report of
symptoms and how the person with CF is feeling and acting. The
physical exam gives them more information. Your CF doctor may
want to do some tests to learn still more. Tests are done on a
routine basis. They are also done when a person may be having
more symptoms. There are four kinds of tests that help your CF
doctor know how your lungs and airways are doing.
RESPIRATORY SYSTEM • 45
Chest X-Ray
Chest X-rays let doctors look inside the lungs. X-rays show
how CF is affecting the lungs. A chest X-ray will be done about
every 1 to 2 years, and when a person is having more breathing
problems.
Pulmonary Function Tests
Pulmonary function tests (PFTs) or breathing tests
measure lung function, or how well the lungs are
working. PFTs give information about airways that are
blocked by mucus. They measure how fast and how
much air can get in and out of the lungs. PFTs help the
doctor see lung changes over time and help guide
treatment. PFTs can also measure how the airways
respond to inhaled medications.
Routine breathing tests may not start until a child is 5
or 6 years old, though some children may be able to
do PFTs at an earlier age. It is hard to do these tests
on infants and young children. However, some CF care
centers may have the special equipment to be able to
do these tests in younger children and infants.
Sputum Cultures (Phlegm or Mucus Cultures)
Cultures check sputum, or mucus from the lungs, for germs that
cause lung infections. Some mucus is put in a dish with a culture
medium that helps bacteria such as staph and Pseudomonas
grow. Later, the dish is checked for these and other bacteria
that cause lung infections. Tests can then be done to see which
antibiotics will work to treat that bacteria and which ones the
bacteria would be able to resist. Antibiotics are medicines that
fight the bacteria.
When a person is older, he or she can usually cough up some
sputum. Infants and younger children cannot “expectorate,” or
cough up mucus. If a person cannot cough up some sputum or
phlegm, an oropharyngeal (OP) swab or culture is done. Some
centers call this a throat culture, or a “gagged” sputum culture
and some may call it a “deep throat culture.” To get a good
sample, the throat culture swab should make the person gag so
some sputum comes up in the throat from the lungs.
Blood Tests
Blood tests are done to help check the health of a person with
CF. A small sample of blood is usually taken with a syringe and
sent to a laboratory for testing.
Pulmonary function tests help
CF doctors to see how well the
lungs are working or if there are
problems.
46 • RESPIRATORY SYSTEM
Blood tests can be done to check:
•
Nutrition status
•
Vitamin levels
•
For liver disease
•
For diabetes
•
For infections
•
The body’s response to infection
•
To make sure medicines are at the right level in the body
•
Possible side effects of medicine
Treatment
CF lung disease begins early and often happens in the first few
months of life. Lung disease begins even before any symptoms
are noticed. Many treatments are started even before symptoms
are noticed. These chronic or long-term treatments are aimed at
keeping the lungs as healthy as possible. The goals of treatment
are to clear blocked airways and to treat or prevent infections.
Because CF affects people differently, treatment is specific to
each person. Your doctor and CF care team will work with you to
develop the best treatment plan.
When you perform the daily, chronic treatments, you are working
to prevent exacerbations or flare-ups and the damage they can
cause to the lungs and airways. If an exacerbation happens,
treating it as early as possible also helps keep the lungs healthier.
Know what your or your child’s normal symptoms are, and contact
your CF care center if you notice an increase or change in these
symptoms.
Chronic Treatments
Chronic treatments are essential to keep the lungs as healthy as
possible. Some chronic treatments will be described here. Talk
to your CF care provider and team to see what the best chronic
treatments are for you or your child.
Airway Clearance Techniques
Airway clearance techniques (ACTs) are treatments that help
people with CF stay healthy and breathe easier. ACTs are
recommended for all people with CF. ACTs loosen thick, sticky
lung mucus so it can be cleared by coughing or huffing. Clearing
the airways reduces lung infections and improves lung function.
There are many ACTs. Most are easy to do. For infants and
toddlers, ACTs must be done by the parent. Older kids and
adults can do their own ACTs. ACTs are often used with other
RESPIRATORY SYSTEM • 47
treatments, such as inhaled bronchodilators and antibiotics.
Bronchodilators should be taken before or with ACTs to open
airways. Inhaled antibiotics should be taken after ACTs to treat
opened airways. Your CF care team will help you choose the
best ACT and other treatments. Each year, review and update
your routine with your CF care team. Different types of ACTs are
described next.
Huff Coughing
Huff coughing is the most basic ACT. Huff coughs are the most
effective coughs. Huff coughing involves taking a breath in and
actively exhaling. It is like “huffing” onto a mirror or window to
steam it up. It is not as forceful as a cough but can work better
and be less tiring. Good strong huff coughs help get rid of mucus.
People with CF should cough. Coughing is helpful and should be
encouraged. People with CF should never try to suppress coughs
or keep themselves from coughing. People with CF should not
use cough suppressants. Coughing is a healthy, natural way for
the lungs to get rid of mucus. Always cough into a tissue, throw
it away in a trashcan and then clean your hands.
Chest Physical Therapy or Postural Drainage and Percussion
Chest physical therapy (CPT or Chest PT) or postural drainage
and percussion (also called PD & P) is an ACT. With postural
drainage, the person gets in several different positions (postures)
that help drain mucus from different lung parts. Gravity pulls
mucus from small to large airways where it can be coughed out.
With chest percussion, the chest is clapped and vibrated to
dislodge and move mucus. This is done in a variety positions to
drain all lung parts. To learn more, go to Appendix C , Page 187,
and read “An Introduction to Postural Drainage and Percussion.”
High-Frequency Chest Wall Oscillation
High-frequency chest wall oscillation also is called a “vest” or
“oscillator.” An inflatable vest is attached to a machine that vibrates
it at high frequency. The vest vibrates the chest to loosen and
thin mucus. Every 5 minutes the person stops the machine and
huff coughs.
Positive Expiratory Pressure Therapy
Positive expiratory pressure (PEP) therapy gets air into the lungs
and behind the mucus in the (collateral) airways. PEP holds airways
open, keeping them from closing. A PEP system includes a mask
or mouthpiece attached to a resistor set by your CF care team.
The person breathes in normally and breathes out a little harder
against the resistance.
Many people with CF use a vest
that helps them cough the thick,
sticky mucus out of their lungs.
48 • RESPIRATORY SYSTEM
Oscillating Positive Expiratory Pressure
Oscillating positive expiratory pressure (oscillating PEP) is an
ACT where the person blows all the way out many times through
a device. Types of oscillating PEP devices include the Flutter,™
Acapella,™ Cornet™ and intrapulmonary percussive ventilation
(IPV). Breathing with these devices vibrates the air in large and
small airways. This vibration dislodges and moves mucus. After
blowing through the device many times, the person huff coughs.
This cycle is repeated many times.
Oscillating PEP
Active Cycle of Breathing Technique
Active cycle of breathing technique (ACBT) involves a set of
breathing techniques. It can be changed to meet each person’s
needs. It gets air behind mucus and clears mucus. ACBT
includes:
Breathing control — normal, gentle breathing with the lower chest while relaxing the upper chest and shoulders.
•
Chest expansion exercises — deep breaths in. Some use a 3-second hold of breath to get more air behind the mucus. This may be done with chest clapping or vibrating, followed by breathing control.
•
• Forced expiration technique — huffs of varied lengths with breathing control.
Autogenic Drainage
Autogenic drainage (AD) means “self-drainage.” It uses different
speeds of breathing to move mucus. This moves mucus from
small to large airways. AD has three parts:
1. Dislodging mucus
2. Collecting mucus
3. Clearing mucus
The person inhales to different levels and then adjusts how
he or she breathes out to change how fast the air flows out of
the lungs and moves mucus. At first, AD takes hard work and
practice. It is best for people who are older than 8 years of age.
The best ACT method is the one that works best for the person
with CF. Talk to your CF care provider, respiratory therapist or
physical therapist. They can help you choose what is best for you
and learn to do the technique accurately. If you have questions,
please contact your local CF care center.
The CF Foundation also has information about airway clearance
on its website at www.cff.org or you can call 1-800-FIGHT CF.
RESPIRATORY SYSTEM • 49
Exercise
Everybody needs exercise. People of all ages with CF should
exercise and be active. Most people with CF can do any exercise
and play sports and games (except scuba diving) that they want.
Exercise can:
•
Loosen mucus in the lungs so it can be coughed up easier
•
Cause coughing, which helps clear the lungs
•
Strengthen breathing muscles
•
Strengthen the heart
Good Nutrition
Good nutrition is very important for people with CF of all ages.
There is a connection between good nutrition and good lung
function. A balanced high-calorie diet with fat and protein gives
the body what it needs to grow normally.
Normal gains in weight and height help:
•
Build strong lungs
•
Keep lungs as healthy as possible
•
Build and keep a strong immune system to fight infections
Medications
Bronchodilators
Bronchodilators open the airways to help clear mucus and make
breathing easier. Bronchodilators are given by breathing in a
medication. Albuterol is a bronchodilator.
Mucolytics
Mucolytics are medications that thin mucus, making it easier to
cough out. Pulmozyme® (DNase) is a mucus-thinning medication
that is taken by inhaling, usually once a day.
Hypertonic Saline
Hypertonic saline is extra-salty water that is sterile, so there are
no germs in it. CF airways do not have enough salt and water in
them. Hypertonic saline is inhaled in the lungs as a mist, which
helps to clear the thick mucus from the lungs.
Azithromycin
Azithromycin, also known as Zithromax,® is a commonly used
antibiotic. It is a pill that is is taken by mouth 3 times a week.
Azithromycin has been shown to improve lung function when
taken regularly. Many people without CF have taken this drug to
treat pneumonia, sore throats or ear infections.
50 • RESPIRATORY SYSTEM
Ibuprofen
Ibuprofen is an anti-inflammatory and non-steroidal agent (does
not affect hormonal balance). It is taken as a pill. Ibuprofen has
been used in some people with CF and has been shown to slow
the rate of lung disease.
Ibuprofen is not right for everyone with CF. There are several
factors to consider before taking this drug long term. When
ibuprofen is taken for CF, the doctor needs to prescribe the right
amount. The doses needed are quite high and are based on a
blood test.
Do not start taking ibuprofen from the drug store for CF without
talking to your CF doctor. Too low a dose means that you or your
child will miss the benefit and still risk side effects. There is an
even greater risk if the dose is too high. Talk to your CF doctor
to find out if ibuprofen is right for you or your child.
Many CF medications are aerosol
treatments and inhaled into the
lungs.
Ivacaftor
In CF, the gene mutation changes the CFTR protein so it
doesn’t work properly. Kalydeco™ (ivacaftor) helps the CFTR
protein work in people with at least one mutation called G551D.
Kalydeco is a pill taken by mouth twice a day for the treatment of
CF in people with this mutation ages 6 years and older. It helps
improve lung function, lower sweat chloride levels and increase
weight gain. Research is being done to find out what other CF
mutations Kalydeco might help work.
More research is being done to find more medicines that will
help the CFTR protein work for people with CF with other
mutations, like those with at least one F508del (sometimes called
Delta F508). You can learn more about this research and different
mutations of CF on the CF Foundation’s website at www.cff.org.
Inhaled Antibiotics
Inhaled antibiotics are used to fight or control infections. The CF
care provider uses the results of the sputum culture to see if an
inhaled antibiotic should be taken.
Inhaled antibiotics go right to where they are needed — the
lungs. The antibiotics especially made for people to inhale
are tobramycin inhalation solution (such as TOBI®), Cayston®
(aztreonam for inhalation solution) and TOBI® Podhaler™
(tobramycin inhalation powder). These antibiotics are used to
improve respiratory symptoms in people with CF who have
Pseudomonas aeruginosa.
How to Give Inhaled Medications
Inhaled medicines are given by aerosol, dry powder inhaler
(DPI) or metered dose inhaler (MDI).
RESPIRATORY SYSTEM • 51
Aerosols are mist treatments made from liquid medicines. The
drug is put in a cup (called a nebulizer) and attached to a small
air compressor. Some of these medicines are mixed with saline
(salt water) in the nebulizer. The compressor blows air through
the nebulizer cup making a mist. The mist is inhaled through
a mouthpiece or mask for several minutes. Some inhaled
medicines may be used with ACTs to help clear the mucus from
the lungs.
Some inhaled medicines are given through a DPI. This medicine
is a powder that is inhaled using a small device. Medicine given
by an MDI use a small device also. An MDI gives one dose of
medicine at a time as a spray. When using an MDI, always use a
spacer or holding chamber.
There are several different types of nebulizer and compressor
devices. Certain aerosol medications require that specific devices
are used. Similarly, different DPI and MDI devices use different
techniques in order to properly get the medicine in the lungs.
Your CF care team can teach you how to use these devices
correctly.
How to Refill Prescribed Medications
Many people with CF use medications
every day. This means that you will need
to know when and how to get your
medicine refilled. You should order your
refills about 8 to 10 days before you
run out of medicine. This allows your
pharmacy to get the medicine or, if you
are using a mail-order pharmacy, for it
to be mailed to you. The information
about how to refill your medicine is on
the label of the medicine bottle or box.
Steps to refill prescriptions:
ABC Pharmacy
Anytown, USA 00001
Medication
Name and
Dose
Your Prescription
Your Directions on
How to Take the Medicine
Prescription
Number
Order refills 8 to 10 days before you run out.
Your Name
•
Qty: 30
RX # C100060
Prescriber: John Smith
Have the label handy when you are ordering refills.
•
On the label, find either the telephone number or Web address you can use to order the refill.
•
•
Give the prescription number when you order.
If you are using a mail-order pharmacy, make sure the pharmacy has your right mailing address.
•
3 refill(s) before 1/1/14
Store Phone: (555) 555-XXXX
Phone
Number
to Call
for Refills
52 • RESPIRATORY SYSTEM
Treatment for Exacerbations
Sometimes CF symptoms may get worse or you may notice new
symptoms. This could be a pulmonary exacerbation or flare up,
which may need extra treatment. Call your CF care center if you
think you or your child are having a pulmonary exacerbation so
that treatment can start right away and lung damage can be less.
Symptoms of a pulmonary exacerbation may include any of the
following:
•
More coughing and/or wheezing
•
Chest congestion
•
More mucus (sputum, phlegm)
•
Change in sputum color (to dark yellow or green)
•
Tiredness, less energy
•
Being less able to exercise
•
Weight loss and/or poor appetite
• Sometimes blood-streaked mucus (always let your CF care provider know about blood in the mucus)
•
Sometimes fever
Treatment for a pulmonary exacerbation includes antibiotics,
more airway clearance and good nutrition. Antibiotics may be
given by mouth, also called oral, or intravenous, also called IV.
Oral antibiotics are liquids, tablets or capsules that are
swallowed. Oral antibiotics are used to treat mild pulmonary
exacerbations. They work against many bacteria, but do not
always work against Pseudomonas.
IV antibiotics are often necessary for CF pulmonary exacerbations.
IV antibiotics are solutions with antibiotics that are put into the
blood through a small tube called an IV catheter. They are needed
if oral antibiotics do not work, or if the infection is caused by
bacteria that are only sensitive to an IV antibiotic. People with
pulmonary exacerbations who need IV antibiotics are usually
admitted to the hospital to receive the care they need. Sometimes,
if they are able to get the extra airway clearance and nutrition at
home, they can complete the IV antibiotics at home.
Possible Respiratory Complications of CF
There are other possible respiratory complications of CF, which are
discussed here.
RESPIRATORY SYSTEM • 53
Bronchiectasis
In most people with CF, the airways get inflamed and damaged.
This causes the airways to stretch and get floppy. This is called
bronchiectasis. It makes it harder to clear the airways of mucus
and causes a change in breathing. Airway clearance techniques
with huffing and coughing can help clear floppy airways of
mucus.
Pneumothorax
Pneumothorax is a break in lung tissue or an
airway that lets air escape from the lung. This
air gets trapped between the lung and the
chest wall. As more air escapes, it may cause
the lung to collapse, making it harder to
breathe. Call the CF doctor if there is sudden
shortness of breath or chest pain!
Pneumothorax occurs in less than 4 percent of
people with CF. With a mild pneumothorax,
the air may re-enter the lung over time and
you will not need medical help, or only
need oxygen. A few people that have a
pneumothorax may need more medical help
to remove the trapped air so the lung can
expand. Some people may need surgery to
keep a pneumothorax from happening again.
Bronchus
Lungs
Pneumothorax
Chest Wall
A pneumothorax is air trapped
between the lung and the chest wall.
Clubbing
Clubbing is when the tips of the fingers and toes get bigger
and rounder. This is common in CF. The cause is not known, but
it seems to occur with lung disease. Clubbing can change over
time. It is not a sign of lung disease severity. Clubbing also occurs
in people with heart disease and other types of lung disease.
Hemoptysis
Hemoptysis means to cough up blood. In CF, it may look like
small blood streaks in the sputum. This may be a sign of an
exacerbation that needs treatment. It is caused by bleeding from
the lining of an airway. This lining may become inflamed and
easily damaged. The thick, sticky mucus can then scrape or tear
the lining, and cause bleeding.
Less often, hemoptysis may occur when an artery breaks and
bleeds into an airway. This is more serious and may need
immediate medical treatment or surgery. If the sputum has
blood, call your CF care center.
54 • RESPIRATORY SYSTEM
Cor Pulmonale
In some people, after years of living with CF, a lot of lung
damage may cause the right side of the heart to grow thicker
and larger. This is called cor pulmonale. The lung damage limits
gas exchange, so less oxygen gets into the blood. To get the
oxygen the body needs, the heart works harder to pump more
blood through the lungs.
Also, lung damage may increase the blood pressure of the
arteries going from the heart to the lungs. This makes the heart
work harder to pump blood to the lungs. The strain on the heart
makes the right side of the heart grow larger. Then the heart may
tire and not pump as well. This may cause swelling, especially of
the feet and ankles.
Cor pulmonale is treated by improving lung function and health.
Extra oxygen and diuretics may help. Diuretics are medicines
that get rid of extra fluid in the body.
Lung Transplantation
When someone with CF develops severe lung disease, the
CF care team may discuss the option of lung transplantation
with the person. The CF doctor can refer the person to a lung
transplant center for evaluation. The transplant center evaluates
the person’s health to determine if a lung transplant is right for
that person, and if it is the right time for it to be done.
Tests examine how well the lungs, heart and kidneys function,
and the types of bacteria in the lungs. Because of the serious
health care implications of transplantation, the person’s
psychological well-being is also evaluated. The transplant
center will evaluate the person’s social support system including
family, friends and professional support. Most parts of the
evaluation are standard, but each center can have some specific
requirements. The decision whether to accept a person for a
transplant is specific to that center.
As many as 90 percent of people with CF are alive 1 year after
transplantation, and more than half are alive after 5 years.
Following surgery, a person may be discharged from the hospital
in a few days or a few months, depending on the person’s
health and complications. The average time in the hospital after
transplant is 2 weeks.
The immune system protects the body from foreign material,
which is anything not belonging in the body, such as germs or
transplanted organs. Therefore, after the transplant, the immune
system naturally reacts against — or rejects — the new organs.
Drugs to stop the immune system from rejecting the organ,
called immunosuppressive drugs, must be taken daily for life.
RESPIRATORY SYSTEM • 55
These drugs may cause side effects such as diabetes, kidney
problems, cancer and osteoporosis (i.e., thinning of the bones).
Research on immunosuppressive drugs shows promise in helping
people live longer with lung transplants.
Transplanted lungs come from people who do not have CF, so
the new lungs do not have CF. However, after the transplant, the
recipient still has CF in the sinuses, pancreas, intestines, sweat
glands and reproductive tract. The new lungs do not “get” CF,
but immunosuppressive drugs may decrease the body’s ability
to fight germs that cause respiratory tract infections and other
types of infections.
Lung transplantation was first done in the United States in
the early 1980s. Over time, survival after a lung transplant has
improved. The CF Foundation supports research on CF and
transplantation and policies that help people with CF get
lung transplants. It is hoped that the CF Foundation’s efforts
to develop new drugs for CF will reduce the need for lung
transplants.
Making the decision to have a lung transplant is a very difficult
and personal one. People with CF should talk to their CF care
team members to get more information about lung transplant.
Visit the CF Foundation website (www.cff.org) for more
information.
Staying Healthy and Avoiding Germs
It is always better to prevent a health problem than to try
to fix one after it has started. For instance, vaccinations
(immunizations) are vital for prevention. Everyone should get
the recommended vaccinations, and a yearly flu shot. Avoiding
germs as much as possible is always best. Washing hands or
hand hygiene should be done often. It is very important for
people with CF to avoid exposure to tobacco smoke.
Read more about “Staying Healthy: What You Can Do” in
Chapter 8, and “Staying Healthy: Avoiding Germs” in Chapter 9.
Remember
The effects of CF on the respiratory system require a variety
of daily treatments including ACTs and inhaled medicines.
Good nutrition and exercise are also important for respiratory
health. Staying on track with the daily schedule of treatments
— and talking to the CF health care team about any changes in
symptoms — will help a person with CF keep his or her airways
as strong and healthy as possible.
56 • RESPIRATORY SYSTEM
Review Questions
Answers follow the questions. More than one answer may be
correct.
1. Which of these statements is true about lung disease in CF?
a. CF affects the lungs.
b. Breathing problems are first noticed at different times for each person.
c. Many treatments are available to keep the lungs as healthy as
possible.
2. The function of mucus in the lower airways is to:
a. Moisten the air we
breathe
b. Seal leaks that may occur in the smallest air passages
c. Plug up bad or damaged airways and direct air to only the healthy parts of the lung
d.Trap particles and germs
so they can be carried toward the throat and
coughed up or swallowed
3. Which of these statements about the mucous plugs in CF is true:
a.Mucous plugs are
uncommon in people with CF.
b. Mucous plugs block
the flow of air in the lower airways.
c. If not removed, mucous plugs can lead to lung infections.
d. Mucous plugs make it hard to remove particles and germs from the airways.
4. Which of these symptoms
occur when there is a pulmonary exacerbation?
a. Tiredness
b. Weight loss and/or poor appetite
c. More coughing and/or wheezing
d. More mucus (sputum/
phlegm)
e. Any of the above.
5. The purpose of airway clearance techniques in CF is to:
a. Clear the airways
b. Prevent spread of CF to others
c. Loosen the mucus so it can be coughed out of the lungs
d. Wash out the lungs and cure CF
RESPIRATORY SYSTEM • 57
6. Which of these statements about exercise is true for people with CF?
a. Exercise helps to loosen mucus in the lungs.
b. Exercise helps
strengthen breathing muscles.
c. Exercise may cause coughing and help clear the airways.
d. Exercise should be avoided because it may make the lung disease worse.
7. In CF, antibiotics are used to:
a. Treat chronic lung
infections, or control infections
b. Treat acute pulmonary exacerbations, or fight infections
c. Treat both chronic lung infections and acute pulmonary exacerbations
8. In people with CF, coughing is:
a. Not a common problem
b. How the disease is
spread to others
c. Hard on the lungs, and should be treated with cough medicines
d. One way the body
removes mucus from
the lungs, and should be encouraged
58 • RESPIRATORY SYSTEM
Answers
To learn more, turn to the page number shown after the answer.
1. A, B, C
All of the statements about lung disease in CF are true. CF
affects the lungs. When breathing problems are first noticed is
different for each person. Page 41.
2. D
The function of mucus in the lower airways is to trap inhaled
particles and germs so they can be carried toward the throat
and coughed up or swallowed. The problem in CF is that the
mucus is so thick and sticky that it cannot be easily carried to the
throat, so the normal cleaning function of the mucus is impaired.
Page 42.
3. B, C, D
It is true that mucous plugs block the flow of air, if not removed
can lead to lung infections and make it hard to remove particles
and germs from the airways. Page 42.
4. E
Any of the above (tiredness, weight loss and/or poor appetite,
more coughing and/or wheezing, more mucus) may be
symptoms of a pulmonary exacerbation. Page 44.
5. A, C
The purpose of airway clearance techniques (ACTs) in CF is to
clear the airways and loosen the mucus so it can be coughed
out of the lungs. ACTs help people with CF stay healthy. ACTs do
not cure CF. Page 46-48.
6. A, B, C
For people with CF, it is true that exercise helps to loosen mucus
in the lungs, strengthen breathing muscles and may cause
coughing and help clear the airways. Exercise should NOT be
avoided. Exercise is good for people with CF. Page 49.
7. C
In CF, antibiotics are used to treat both chronic lung infections
and acute pulmonary exacerbations. Page 50.
8. D
In people with CF, coughing is one way the body helps clear the
lungs, and should be encouraged. Page 46.
Notes and Questions
Chapter 5: Gastrointestinal System
Poor digestion is a common problem in cystic fibrosis (CF). The
good news is that proper diet and enzyme supplements with
meals and snacks can help solve this problem.
How the Gastrointestinal (GI) System Works
Digestion and food absorption are complex. When food is
chewed and swallowed, it goes through the esophagus and into
the stomach. Food in the stomach causes exocrine glands in the
pancreas to make digestive enzymes. The pancreas is a gland
in the body connected to the small intestine. Food goes from
the stomach into the small intestine. The small intestine is where
most food digestion and absorption occurs. Digestive enzymes
from the pancreas enter the small intestine through a small duct,
or passageway, called the pancreatic duct.
Other exocrine glands in the walls of the intestines make mucus
to lubricate the digestive tract. This makes it easier for food and
waste material to pass through the intestines.
How the Gastrointestinal System
Works
Mouth
Esophagus
After food is swallowed, it goes down the esophagus and into the stomach. Food is churned in the stomach. Then, it moves into the small intestine.
•
The pancreas makes enzymes to help
digest fat and protein in foods. These enzymes enter the small intestine to digest food.
•
Liver
Stomach
Pancreas
Small
Intestines
Large
Intestines
What remains moves through the large intestine, where water is absorbed. Waste then moves out of the body.
•
62 • GASTROINTESTINAL SYSTEM
The Problem
For most people with CF, the exocrine glands in the pancreas
make such thick secretions that the digestive enzymes are not
able to get through the pancreatic ducts
and do not reach the small intestine.
Mucous Plug
Stomach
Pancreas
Pancreatic
Duct
Small Intestine
Digestive enzymes from the pancreas are blocked and do
not make it into the small intestine.
With no enzymes to break down
food, the food is poorly digested and
absorbed. Much of the proteins, fats
and carbohydrates in food are not
absorbed for use in the body. This is
called malabsorption.
Malabsorption of proteins and fats can
lead to poor growth and malnutrition.
Proteins are needed for growth and
body tissue repair or healing. Fats
are calorie-rich food sources and give
the energy needed for growth and
development and to stay healthy. Fat
also is needed for absorption of some
vitamins.
For some people with CF, the mucus that lubricates the
intestines is so thick and sticky that it may block them. A
blocked intestine needs special treatment.
Symptoms of Malabsorption
Symptoms of malabsorption include:
•
Poor weight gain
•
Frequent, large, greasy, foul-smelling stools
•
Stomachache
•
Excessive gas
Poor Weight Gain
Poor weight gain may occur even though the person with
CF is hungry and eating a lot. It is caused by malabsorption
of protein and fat. Lung disease may make weight gain even
harder, because more calories are used for breathing and lung
repair or healing.
Frequent, Large, Greasy, Foul-Smelling Stools
This is caused by the fat in the stools, because the fat is not
absorbed for use by the body.
Stomachache or Abdominal Pain
People with malabsorption often complain of a stomachache,
usually from poorly digested food in the intestine. This should
GASTROINTESTINAL SYSTEM • 63
stop with treatment of malabsorption. At diagnosis, many
parents of young children with CF report fussiness with feedings.
This is likely because of discomfort from fat malabsorption. After
a person with CF starts pancreatic enzyme replacements, this
discomfort should go away. Other causes of stomachache are
sore muscles from coughing or blocked intestines.
Excessive Gas
This also is caused by malabsorption.
People with CF who are not taking enzymes may have any or all
of these symptoms. Once enzymes are started, the symptoms
should improve. As children grow and gain weight, their dosage
of enzymes will need to be increased. If children or adults who
are taking enzymes develop symptoms of malabsorption, they
may need an adjustment in the dosage of enzymes. Call your CF
care provider to report symptoms of malabsorption. Remember
not to change your dosage of enzymes without instruction from
your CF care provider.
Treatment
For most people, the digestive, or gastrointestinal, problems of
CF are easy to treat. There are three common treatments:
•
Diet and nutrition
•
Pancreatic enzymes
•
Vitamins
Diet and Nutrition
A good diet is important for normal growth and development.
It helps the body fight infection. It helps lungs grow and heal.
Good nutrition helps keep breathing muscles strong. Good
nutrition has been shown to be closely related to good lung
health in people with CF.
People with CF need the same good foods that everyone needs.
In addition, they need more calories because of malabsorption.
They also use more calories for breathing and to keep their lungs
healthy.
People with CF may need up to twice the amount of calories per
day compared with other people their age and weight.
For people with CF, fats are good! Fats are rich in calories and a
good source of fuel for growth, development and energy. People
with CF should eat a high-calorie, high-protein, high-fat, high-salt
diet. Fats should not be restricted!
Most people with CF do well eating a healthy diet and taking
pancreatic enzymes. If children are not gaining weight well, or
adults are not maintaining a healthy weight, ask the CF doctor
64 • GASTROINTESTINAL SYSTEM
or dietitian about ways to add calories. Nutritional supplements
and ways to boost calories may help with weight gain. Besides
high-calorie meals, snacks and nutritional supplements, some
people with CF may need tube feedings to keep a healthy
weight.
Talk to the CF doctor and dietitian about how a person with CF
can use good nutrition to stay healthy.
Pancreatic Enzyme Replacements or “Enzymes”
About 90 percent of people with CF need pancreatic enzymes
to replace the natural enzymes that are not able to get through
the blocked pancreatic duct. Enzyme replacements come in
capsules and are taken by mouth. Inside each capsule are small
beads that contain digestive enzymes. Each bead has a coating
called an “enteric coating” that allows it to dissolve in the small
intestine. The enzymes are then released in the small intestine to
help digest food so the body can absorb it.
The amount (or dosage) of enzymes needed will vary with the
person’s age and weight, what he or she eats and how much CF
has affected the pancreas. Enough enzymes should be taken
to control symptoms of malabsorption. With enough enzymes,
children should grow well, and adults should maintain a healthy
weight.
Stomach acid may make pancreatic enzymes not work well.
Sometimes acid blockers or antacids are prescribed to neutralize
stomach acid.
Pancreatic enzymes come in
capsules and are taken by mouth.
They pass through the stomach
and into the intestines, where they
help to break down food so it can
be absorbed by the body.
Very high doses of enzymes can damage the large intestine. The
CF doctor and dietitian will keep track of enzyme doses. If signs
of malabsorption are present, call the CF care team. DO NOT
give more enzymes on your own. Talk with the CF dietitian or
doctor before giving more or less enzymes.
Pancreatic enzymes:
• Help digest fats, proteins and carbohydrates (the three parts of food that give us calories).
• Promote good nutrition and normal weight gain in children, and a healthy weight in adults.
•
Help the body to absorb many essential nutrients.
How Enzymes Are Given
Enzymes should be taken at the beginning of each meal and
snack. For infants and small children, the capsules may be
opened. The beads in each capsule may be mixed in a small
amount of soft acidic food, such as applesauce, and given from
a spoon. For babies, it may help to give breast milk or formula
after the beads are given, so that the beads are swallowed.
Older children and adults can swallow the capsules.
GASTROINTESTINAL SYSTEM • 65
More About Enzymes
If you notice that you are not getting the brand of enzymes your
CF doctor ordered or that you usually take, contact your CF care
center. They will help you to get the right enzymes.
• Enzymes should be taken at the beginning of each meal, snack or feeding.
Take enzymes with all meals, snacks, milk, formula and nutritional supplements, including tube feedings that contain fat or protein.
•
• Do not skip taking enzymes. Keep some enzymes with you for meals or snacks away from home.
Enzymes work for about an hour after taking them.
•
• Always take the right dosage, as prescribed by your CF care provider.
• Slightly higher dosages of enzymes may be needed with high-fat foods such as “fast foods,” fried foods and pizza. Tell your CF dietitian or care provider if high-fat foods cause signs of malabsorption.
• Some foods and drinks do not require enzymes, because they contain only “simple carbohydrates” (or sugars) that digest easily. Examples of foods that do not require enzymes are fruits, juice, soft drinks, sports drinks, tea and coffee (without cream), hard candy (such as lollipops), fruit snacks, jelly beans, gum, popsicles or flavored ice.
• If the enzyme capsules are opened, do not crush or chew the beads. If beads are crushed or chewed, they will not work as well and could cause mouth sores.
• For infants and small children who need the capsules opened, mix the beads with a soft, acidic food, such as applesauce. Do not mix enzymes with milk-based foods such as yogurt or pudding because they will not work as well. Do not mix enzymes into foods ahead of time.
• Keep enzymes at room temperature (59° F to 86° F). Keep enzymes away from heat and cold. Do not store them in places that get hot or cold such as the top of a toaster oven, inside a hot car or in the refrigerator.
Check the expiration date on each enzyme bottle to make sure they are “fresh.”
•
Ask the pharmacist if you can get the enzymes in the original, sealed, unopened bottle from the manufacturer.
•
•
Always keep the enzyme bottle lid tightly sealed.
Most people with CF take enzymes
before eating.
66 • GASTROINTESTINAL SYSTEM
• Some babies, children, teens and even adults refuse to take their enzymes at times. Enzymes are essential in the care of CF. Talk with your CF care provider or dietitian if taking enzymes becomes a problem.
Vitamins
Because people with CF have trouble absorbing fats, certain
vitamins are not absorbed from food. Vitamins A, D, E and
K are “fat-soluble” vitamins. These vitamins need fat to be
absorbed. Taking specially formulated water-soluble vitamins
recommended by the CF care provider should help prevent
vitamin deficiencies. These specially formulated vitamins should
be taken once or twice daily, as prescribed by your CF care
provider. The vitamins come in liquid, chewable tablet or capsule
form. Chewable tablets should be chewed before they are
swallowed.
Possible Gastrointestinal (GI) Complications
Meconium Ileus
For a few infants, the first symptom of CF is a blocked intestine
at birth called meconium ileus. Meconium (intestinal secretions)
is normally passed by infants in their first 24 hours. About 1
baby in 5 with CF has meconium that is too thick. It blocks the
intestines and has to be removed with enemas or surgery.
Distal Intestinal Obstructive Syndrome
Distal intestinal obstructive syndrome (DIOS) used to be called
meconium ileus equivalent. It is like meconium ileus, but occurs
after infancy. It is caused by abnormal mucus in the intestine
and poorly digested food. This results in stool that cannot move
through the intestines normally. Partial or complete intestinal
blockage may occur.
Causes of DIOS may include dehydration, not enough
pancreatic enzymes and diet changes. The symptoms of DIOS
are stomachache and fewer bowel movements than normal. If a
person with CF has no bowel movements for 24 hours and has a
stomachache, the CF care center should be called.
DIOS is often treated with:
• Good diet and enzyme replacement; sometimes the person will need to have nothing to eat for a period of time.
•
More fluids.
• Special liquids to drink that move fluid into the intestine and stool — these liquids may be given through a nasogastric (NG) tube that is placed in the nose going into the stomach.
•
Enemas.
GASTROINTESTINAL SYSTEM • 67
DIOS must be treated promptly to prevent worse problems. It
often can be treated with medicines, enemas and diet. Surgery is
rarely needed to fix the obstruction.
Rectal Prolapse
Rectal prolapse is when the rectum sticks out through the
anus. It is usually caused by hard-to-pass, abnormal stools, plus
increased coughing and poor weight gain. It usually improves
when food absorption and lung health improve. Surgery is rarely
needed.
Other GI Problems
Cirrhosis of the Liver
The liver has ducts (or tubes) that drain bile secretions. These
are called bile ducts. In CF, thick secretions may obstruct these
ducts. This may cause cirrhosis of the liver. To watch for liver
problems, CF doctors get a blood sample to check liver function
yearly, and more often if needed. People with abnormal results
on their liver function tests may be treated with medicines such
as ursodeoxycholic acid (URSO). Serious liver problems are not
common in CF. About 10 percent of people with CF have CFrelated liver problems.
Cystic Fibrosis–Related Diabetes
Cystic fibrosis–related diabetes (CFRD) is a form of diabetes that
can occur in people with CF. Diabetes is a problem in which a
person’s blood glucose (a type of sugar) level is too high. CFRD
is not the same as diabetes in people without CF. The diagnosis
and treatment are not exactly the same. If you or your child has
been diagnosed with CFRD, you should be able to do all the
things you want to do, and even eat all the foods you like. CFRD
is common in people with CF especially as they get older. CFRD
is found in about 35 percent of adults with CF ages 20 to 29 years
and in about 43 percent of those ages 30 years and older.
Causes of CFRD
In people who do not have CF, the most common types of
diabetes are type 1 and type 2 diabetes. Type 1 diabetes used to
be called insulin-dependent or juvenile-onset diabetes. It occurs
most often in childhood. People with type 1 diabetes can’t make
any insulin, so they must take insulin to stay alive.
Type 2 diabetes used to be called non–insulin-dependent or
adult-onset diabetes. It is caused by the lack of a normal response
to insulin (sometimes called insulin-resistance) in addition to the
body not making enough insulin. This type of diabetes occurs
most often in people who are overweight.
68 • GASTROINTESTINAL SYSTEM
People with type 2 diabetes do not always need insulin to
manage their diabetes. Some take pills. Some use insulin. Some
manage type 2 diabetes through diet, weight loss and exercise.
CFRD is different from type 1 or type 2 diabetes, but has some
features of both. People with CFRD do not make enough insulin.
This is probably because the thick mucus in the pancreas causes
scarring. The scarring results in the pancreas not making enough
insulin. Like people with type 2 diabetes, people with CFRD also
have some insulin resistance. People with CFRD do not have
diabetes because of being overweight. Therefore, unlike people
with type 1 or 2 diabetes, people with CFRD should not treat
their diabetes by limiting calories or losing weight.
Symptoms of CFRD
Having to urinate often (polyuria) and needing to drink often
(polydipsia) are classic symptoms of diabetes. These symptoms
are caused by high blood sugar levels. It is not easy to notice
these symptoms in CF. People with CF often drink more (and
then use the bathroom more). Other symptoms of CFRD include
feeling very tired, losing or not gaining weight and unexplained
decline in lung function. However, many people with CFRD do
not have any symptoms at all.
CFRD Screening and Diagnosis
Because many people with CFRD do not know they have it until
they are tested, your CF care provider will be testing you or your
child for diabetes every year. Beginning at age 10 years, you or
your child will have an oral glucose tolerance test (OGTT). An
OGTT is done after a person has had nothing to eat or drink for
8 to 12 hours. Blood samples are taken before and up to 2 hours
after drinking a set amount of glucose. The OGTT may be done
sooner than 10 years of age if a person is having any symptoms
of diabetes.
Treatment of CFRD
The treatment for CFRD is insulin. Insulin helps sugars and
proteins to move from the blood into the body’s cells. This is
needed to provide energy and to build muscle. Insulin is given
by injection. Some people with CFRD wish they could take a pill,
like some people with type 2 diabetes do. Right now, only insulin
is used to treat CFRD.
It is also important for people with CFRD to get enough calories.
To ensure good health, it is important to maintain a healthy body
weight. With type 1 or type 2 diabetes, people are often advised
to eat a low-fat, low-salt and sometimes low-calorie diet. People
with CF have different nutrition needs, though. When people
have CFRD, they still need to eat their normal high-calorie,
GASTROINTESTINAL SYSTEM • 69
high-protein, high-fat, high-salt diet to help get and maintain a
healthy body weight. CFRD is not treated by limiting calories or
losing weight.
Keeping blood glucose levels at a normal or near-normal level
helps people with CFRD gain weight, feel better and have more
energy. It also lowers the risk of problems caused by diabetes.
If you or your child is diagnosed with CFRD, your CF care
provider will help you learn more about CFRD. He or she will
also refer you to an endocrinologist who is a doctor with special
training in the diagnosis and treatment of diabetes. Besides
routine visits to the CF Foundation–accredited care center,
people with CFRD should also be seen by a diabetes care team
every 3 to 4 months. These visits are very important to help
manage CFRD. You can also learn more about CFRD from the CF
Foundation website at www.cff.org.
Gallstones
Gallstones occur in about 12 percent of people with CF. We do
not know why. It may be related to how the body handles fats
and bile acids. Gallstones do not often cause symptoms or need
treatment. Rarely, surgery is needed to remove the gallbladder.
Remember
Most of the gastrointestinal problems with CF are caused by
blocked pancreatic ducts. The blockage keeps enzymes from
reaching the small intestine. This causes digestive problems
and malabsorption. But good, effective treatment is available. It
involves:
• Pancreatic enzyme replacements — capsules/beads taken with food and drinks (such as milk or formula)
Good nutrition — with foods high in calories, fats, proteins and salt
•
•
Extra vitamins — specially formulated water-soluble vitamins
•
Good fluid intake
70 • GASTROINTESTINAL SYSTEM
Review Questions
Answers follow the questions. More than one answer may be
correct.
1. The most common gastrointestinal problem in
CF is:
a.Bile duct blockage that
prevents bile from draining out of the liver
b.Blockage in the bowel
caused by thick secretions and abnormal stools
c.Blockage in the pancreatic duct that prevents digestive enzymes from reaching the small intestine
2. The blockage of enzymes in the pancreas can cause:
a. Ulcers in the bowel
b. Poor digestion of protein and fat
c. Excessive weight gain
3. Symptoms of malabsorption
in CF include:
a.Excessive gas
b.Poor weight gain
c.Stomachache
d.Frequent, large, greasy stools
e.All of the above symptoms
f. None of the above symptoms
4. These are all common treatments for malabsorption except:
a.Vitamins
b A well-balanced diet
c.A low-fat diet
d.Pancreatic enzymes
5. Which of the following statements is true about the use of pancreatic enzymes?
a.Enzymes should be taken with meals.
b.Slightly higher doses of enzymes may be needed with high-fat foods.
c.If the capsules are opened up, do not crush
or chew the beads because they will not work as well.
GASTROINTESTINAL SYSTEM • 71
Answers
To learn more, turn to the page number shown after the answer.
1. C
The most common gastrointestinal problem in CF is blockage
in the pancreatic duct that prevents digestive enzymes from
reaching the small intestine. Page 62.
2. B
The blockage of enzymes in the pancreas can cause poor
digestion of protein and fat. This condition is called
malabsorption. Page 62.
3. E
Symptoms of malabsorption in CF include all of the above
symptoms. Excessive gas, poor weight gain, stomachache and
frequent, large, greasy stools are all symptoms of malabsorption
in CF. Page 62.
4. C
These are all common treatments of malabsorption except a
low-fat diet. The poor digestion in many people with CF causes
malabsorption of proteins, fats and carbohydrates. Pancreatic
enzymes help digestion to occur more normally. A well-balanced
diet high in calories, fats and proteins helps provide good
nutrition for healthy growth and development. Pages 63-66.
5. A, B, C
All of these statements are true. Correct use of enzymes is
essential to ensure that people with CF get all possible nutrients
from the food they eat.
Pages 64-65.
Notes and Questions
Chapter 6: Reproductive System
Sexuality and reproduction are important concerns for teens and
adults with and without cystic fibrosis (CF). If you are the parent
of a young child, you may have questions about what the future
holds for your child in terms of sexual development, sexual
function and reproduction. We will describe the effects of CF on
the reproductive system (the sex organs) after a brief review of
normal function.
Vas Deferens
Sperm
Mucous
Plug
Bladder
Male Reproductive System
Sperm are made in the testes. The sperm travel through a tube
called the vas deferens to the prostate gland. The sperm and
semen mix and travel through the urethra to the penis where
they are ejaculated.
The Problem
In most males with CF, the vas deferens
may not have developed normally
or thick mucus may block it. Sperm
cannot pass. Even though the testes
make sperm and intercourse is normal,
the semen ejaculated has no sperm
so it cannot cause pregnancy. Men
with CF are not sterile, although most
are infertile. The sex hormones and
glands are not affected by CF. Sexual
development is normal. Sexual desire
and performance are normal.
Absence of the vas deferens or blockage
often occurs before birth. There is no
way to fix this right now. Because of this,
about 98 percent of males with CF are
infertile. But men with CF should not
assume they are infertile. In a few men
with CF, the vas deferens is there and
is not blocked. These men have some
sperm in their semen and may be able
to have children through intercourse.
Vas Deferens
Pubic Bone
Pubic
Bone
Large
Intestine
Bladder
Seminal
Vesicle
Urethra
Prostate Gland
Penis
Epididymis
Scrotum
Testis
A test, called a semen analysis (or sperm count), can be done
when the male with CF matures. This test shows if sperm are in
the semen.
Anus
74 • REPRODUCTIVE SYSTEM
Infertility Treatments for Men with CF
New infertility treatments mean that some men with CF can
biologically father children. Sometimes, sperm can be taken
from the testes, combined with an egg taken from the woman,
and then the fertilized egg is put back into the woman. There
are different procedures used to take sperm from males. These
procedures are called:
•
Microepididymal sperm aspiration (MESA)
•
Percutaneous epididymal sperm aspiration (PESA)
•
Testicular sperm extraction (TESE)
These procedures take sperm directly from the epididymis (fine
tubules behind the testes). The procedure to collect the sperm
involves an operation, usually done under local anesthesia.
After the sperm are collected, they can be used for in vitro
fertilization, with or without intracytoplasmic sperm injection
(ICSI). In vitro fertilization is a process where the egg cell is
fertilized by the sperm outside the body. When ICSI is used, a
single sperm is injected directly into the egg. The fertilized egg
is then put into the woman’s uterus.
Female Reproductive System
Ovary
Fallopian
Tube
Cervix
Uterus
Large
Intestine
Bladder
Pubic
Bone
Anus
Urethra
Vagina
Eggs are made in the ovaries and go down
the fallopian tubes to the uterus, where the
baby grows. Sperm, after being deposited
in the vagina, travel through the cervix
and into the uterus to fertilize the egg. The
vagina has exocrine glands that secrete
mucus to lubricate the vagina and aid the
passage of sperm. Women with CF may
be less fertile than other women. However,
conception is possible and many women
with CF have had children.
The sex hormones and glands in women with
CF are not affected. Sexual development is
normal. Sexual desire and performance are
normal.
The Problem
In women with CF, the vaginal and cervical
mucus may be so thick and sticky that it
makes it more difficult for the sperm to
travel and reach the egg for fertilization to take place. Women
with CF may ovulate (release eggs from the ovaries) less often
and may have irregular periods, especially when having lung
and/or nutrition problems.
REPRODUCTIVE SYSTEM • 75
Infertility Treatments for Women with CF
If a woman with CF makes the decision to have a child (see
Making the Decision to Have a Child and Pregnancy, pages
76-77), she may be able to conceive without any infertility
treatments. If infertility treatments are necessary, a fertility expert
can perform a variety of tests to determine the exact causes of
infertility. Some of the following treatments may be appropriate
for a woman with CF who is trying to get pregnant.
Insemination
Insemination is a procedure when sperm are inserted into the
cervix or directly into the uterus. Ovarian stimulation to produce
more eggs may or may not be used with insemination.
In Vitro Fertilization
In vitro fertilization involves four steps:
1. Stimulation of the ovaries
2. Egg collection
3. Fertilization
4. Embryo transfer
Stimulation of the ovaries involves taking medication (hormones)
to produce more eggs. Maturity of the eggs is determined by
ultrasound and blood tests. Once the egg cells are mature, they
are collected.
The mature eggs are collected under ultrasound guidance,
with local anesthesia. The male partner is asked to produce a
sperm sample on the same day. Fertilization takes place in the
laboratory. The procedure is intended to produce a fertilized
egg, called an embryo.
Embryos are usually transferred to the woman’s uterus 48 hours
after egg collection. Once the embryos are transferred to the
uterus, medication is usually taken for 15 days to increase the
chances of the embryo implanting in the uterus. A pregnancy
test is then performed.
Safe Sex and Contraception
Most men with CF are infertile. Some women with CF are less
likely to get pregnant. However, having CF is not birth control. All
sexually active men and women with CF should practice safe sex.
Protection from sexually transmitted diseases (STDs) is essential
for everyone. Birth control should be used until a decision is
made to have a child.
76 • REPRODUCTIVE SYSTEM
For people with CF, issues of contraception are similar for
people who do not have CF, but there are some differences.
Some forms of contraception are discussed in the next
paragraphs. People with CF should talk to their CF care team
and primary care physician or gynecologist about safe sex and
which form of contraception is right for them.
The Condom
The condom provides contraception and protection from STDs.
Birth Control Pills
Birth control pills are often a good choice of contraception for
women with CF. The pill does not provide any protection against
STDs. Birth control pills may be less effective when certain
antibiotics are taken. Also, women with liver disease or CFRD
(diabetes) should talk to their gynecologist and CF doctor before
starting birth control pills.
The Patch
The patch is a once-a-week birth control method that is as
effective and works on the same principle as the pill. It does not
give protection against STDs. When certain antibiotics are taken,
the patch may be less effective. Some women like the patch
better than the pill because it only has to be replaced once a
week.
The IUD
An IUD is an intrauterine device made of plastic and/or copper
that is inserted into the womb (uterus) by way of the vagina.
There are different types of IUDs. Certain IUDs may be a good
choice of contraception for a woman with CF. It does not give
protection against STDs.
Contraceptive Injections
Contraceptive injections, such as Depo-Provera,™ are given
by an injection every 3 months. It does not give protection
against STDs. Contraception injections are NOT recommended
for women with CF. People with CF are already susceptible
to osteoporosis, a condition that results in bone loss. Using
contraceptive injections further increases the risk of developing
osteoporosis.
Making the Decision to Have a Child
For both men and women with CF, the question is not “Can I
have child?” but “Is having a child right for me?” Like people
who do not have CF, it is important for people with CF to make
having a child a decision. Having a child is a life-changing,
REPRODUCTIVE SYSTEM • 77
long-term responsibility for all parents. People with CF who are
making the decision about having a child should talk very openly
to their CF care providers and obstetrician.
People with CF who are thinking about having a child should
have genetic counseling. CF carrier screening should be offered
to their partners.
Will a Parent with CF Have Children with CF?
Parents with CF will pass on one CF gene to all of their children.
Because the parent with CF has two CF gene mutations, every
sperm or egg will carry one CF gene mutation. So the genetic
makeup of the partner will determine whether any children have
CF.
How a Person Gets CF
To have CF, you must get one copy
of the CF gene from each parent.
If one parent has CF, and the other
parent is not a CF carrier all the
children will be carriers.
If one parent has CF and the other does not have CF and is not
a carrier of a CF gene mutation, then all children will be carriers.
The children will not have CF.
If one parent has CF and the other is a CF carrier, there is a 50
percent chance that any child born will have CF. If the child does
not have CF, the child will be a carrier of CF.
Has CF
Not a
Carrier
If both parents have CF, then any child they have together will
have CF. Chapter 1 (pages 14-15) has more about how CF is
inherited and the odds of having a child with CF.
Pregnancy
Women with CF should have good lung health and weight
before getting pregnant. Having a healthy baby means making
sure that the mother is healthy too. Many things affect how
well a woman does with her pregnancy. CF affects pregnancy.
Pregnancy affects CF.
Carrier
Carrier
Carrier
Carrier
When one parent has CF
and one parent is a carrier,
there is a 50 percent chance
of having a child with CF.
Women with CF can get pregnant. In the United States,
hundreds of women with CF have had babies. How the mother
and baby do during pregnancy depends primarily on the
mother’s health when she gets pregnant.
Any woman with CF who wants to get pregnant should know
the possible risks. Also, the added demands of raising a child
and how it affects her health should be considered. Because
problems caused by CF vary, the decision is personal and should
be discussed with her partner and a doctor who knows about CF
and the woman’s health.
During pregnancy, women with CF should be very closely
monitored by their CF care provider and high-risk obstetrician.
They should be watched closely and treated early for pulmonary
exacerbations. They should be monitored for diabetes. Careful
attention should be paid to being well nourished and having
good weight gain during pregnancy.
Carrier
Has CF
Has CF
Has CF
Carrier
Carrier
78 • REPRODUCTIVE SYSTEM
Lung Function
The woman’s lung health and lung function are the most
important factors that affect her health and the baby’s health.
Women should talk to their CF care providers and obstetrician
about how healthy their lungs are, and how a pregnancy is likely
to affect them.
Nutritional Status
Women with CF need to be well nourished. The woman will
need to be able to take in enough calories for herself and the
baby to grow while pregnant.
Folic Acid
One of the most important things a woman can do to help
prevent serious birth defects in her baby is to get enough folic
acid every day, especially before conception and during early
pregnancy.
After the Baby Is Born
After the baby is born, any parent with CF will need to continue
to set aside time to take care of their own health. They will need
to plan with their spouse and family, before the baby is born, so
that they have help in place to take care of the baby and do their
CF treatments.
Remember
For both men and women with CF, the sex hormones and glands
are not affected. Sexual desire and performance are normal. Safe
sex should always be practiced by everyone. Most males with
CF are infertile. But men with CF should NOT assume they are
infertile. Women with CF are sometimes less fertile than women
without CF. Until making the decision to have a child, all sexually
active men and women with CF should use contraception.
For more information, talk to your CF care provider. Your care
center or the CF Foundation also has more resources.
REPRODUCTIVE SYSTEM • 79
Review Questions
Answers follow the questions. More than one answer may be
correct.
1. Which of the following statements about CF and the male reproductive system is true?
a. Men with CF are usually infertile because of blockage or absence of sperm passageways, but men should NOT assume they are infertile.
b. CF does affect the male reproductive system.
c. Men with CF are not
sterile.
d.All of the above.
2. Which of the following statements about CF and the female reproductive system is true?
a. Women with CF cannot become pregnant.
b. CF does not affect the female reproductive system.
c. Women with CF are usually sterile because of low hormone levels.
d. Thick, sticky mucus makes it difficult for sperm to reach the egg.
3. Which of the following statements is true about birth control pills?
a.Women with liver disease or CFRD should discuss the use of the pill carefully with their gynecologist and CF physician.
b.They may cause problems
for women with CF.
c. Certain antibiotics may cause birth control pills to be less effective.
d. Birth control pills are
the only acceptable method of birth control for women with CF.
4. Which of the following statements about women with CF are true?
a.They may be less fertile.
b.They can become pregnant.
c.They cannot have children safely.
d.All children born to a
woman with CF will carry at least one CF gene mutation.
80 • REPRODUCTIVE SYSTEM
Answers
To learn more, turn to the page number shown after the answer.
1. D
All of the above. All of the statements are true. Men with CF
are usually infertile because of blockage or absence of sperm
passageways, but men should NOT assume they are infertile.
CF does affect the male reproductive system. Men with CF are
not sterile. Page 73.
2. D
It is true that thick, sticky mucus makes it more difficult for sperm
to reach the egg. Page 74.
3. A, C
The following statements are true: Women with liver disease
or CFRD should discuss the use of the pill carefully with their
gynecologist and CF doctor and certain antibiotics may cause
birth control pills to be less effective. A gynecologist who knows
CF should be consulted before use of birth control pills. An
alternative form of birth control should be used when the woman
is on certain antibiotics. Page 76.
4. A, B, D
It is true that women with CF may be less fertile, they can
become pregnant and that all children born to a woman with
CF will carry at least one CF gene mutation. Women with CF
can have a safe pregnancy and healthy children. How the mother
with CF and baby do during pregnancy depends on the mother’s
health when she gets pregnant. A woman with CF should talk
with her CF care team and partner before becoming pregnant.
Pages 74-78.
Notes and Questions
PART III
Living with Cystic Fibrosis
Chapter 7: Overview: Living with CF
Life with cystic fibrosis (CF) may
seem filled with a lot of schedules,
treatments and diet rules. People with
CF often face four main issues:
1. Staying fit and healthy
Food Diary
Date:________________________
BREAKFAST: Start time________
End time________
Food and drinks (type/amount):
Number of enzymes:
Comments:
Time taken:
2. Sticking to daily treatments
3. Managing respiratory problems
4. Managing nutrition and digestive problems
Parents manage these issues for small
children. They also teach their child to
manage his or her own CF care as soon
as the child is able. Start promoting
independence early, so that by the
time children are ages 12 to 14 years
they do most of their own care. Teens
should spend the first part of their clinic
visits talking with the CF care team by
themselves. The parent can then join
them toward the end of the visit.
MORNING SNACK: Start time________
End time________
Food and drinks (type/amount):
Number of enzymes:
Comments:
Time taken:
LUNCH: Start time________
End time________
Food and drinks (type/amount):
Number of enzymes:
Comments:
Time taken:
AFTERNOON SNACK: Start time________
End time________
Food and drinks (type/amount):
Number of enzymes:
Comments:
Time taken:
DINNER: Start time________
End time________
Food and drinks (type/amount):
EVENING SNACK: Start time________
Some people find it helpful to make a
Food and drinks (type/amount):
daily calendar of CF care. This will help
them keep track of what needs to be
done and when. It may also be helpful
to keep a diary of daily fluid and food
intake, medicines, enzymes and bowel
habits. Letting the CF doctor see it
at the next checkup can help show, for example, if the enzyme
dose is correct. Some people keep computer records of their
medicines. This helps them keep track of prescription refills.
Before going to the clinic, they can print updated lists to show
the care team.
See Appendix D, page 195, for examples of daily record
forms that can be used to keep a food diary, a treatment diary
and a symptom diary. Your CF care center staff can also offer
suggestions for sources for forms and programs to help you
keep such records.
Number of enzymes:
Comments:
Time taken:
End time________
Number of enzymes:
Time taken:
Comments:
Notes and Questions
Chapter 8: Staying Healthy: What You Can Do
We have described cystic fibrosis (CF), how CF affects the body
and treatments for CF. With routine therapies and regular visits
to a Cystic Fibrosis Foundation–accredited care center, most
people with CF can lead active lives. This chapter describes
other things to do to stay healthy.
Knowing Yourself
Staying healthy begins with knowing
yourself, your body and how you feel.
Of course, for infants and children,
parents need to observe their behavior
and health symptoms.
Everyone, with or without CF, needs to
“listen to their body.” For example, our
bodies tell us when we're hungry and
need to eat, and when we're tired and
need to rest. Listening to our bodies
helps us know how to take care of
ourselves. People with CF also need to
know how CF affects their body, which
will help them understand what their
body is telling them.
For example, some people with CF
have a slight cough that they may notice only in the morning
when they wake up. For those people, when they notice a
cough off and on during the day, they know that their body is
telling them that something is going on — that their CF may be
worsening a little. This is the time to call their CF care provider.
It might be time to go in for a CF clinic visit, get some oral
antibiotics and increase airway clearance from 2 times per day to
3 times per day for a week or two.
Another example of “knowing your CF” is for people with CF to
know how energetic they feel when they are feeling their best.
Sometimes people with CF just don't feel as energetic as they
normally do. Sometimes having less of an appetite goes along
with having less energy. These may be signs of an early lung
infection or pulmonary exacerbation (even without having a
cough!). This would be the time to call or see the CF care team.
Exercise strengthens muscles and
increases appetite. Like everyone
else, people with CF should
exercise regularly.
88 • STAYING HEALTHY: WHAT YOU CAN DO
Noticing changes in symptoms early and getting treatment early
can help keep you or your child from having worse problems that
are harder to treat.
Knowing how you or your child feels when feeling really well is
also called knowing your or your child’s “best baseline.” Knowing
their best baseline helps people with CF recognize when they
are not feeling their best — that's the time to call or visit the CF
care center.
When a person is feeling his or her best and healthy, he or she
is energetic, active and interested in interacting with the world
around them. All people should be able to have interests and
hobbies and goals and dreams for what they want to do with
their lives.
Based on your or your child’s best baseline and goals, you
and the rest of your CF care team will make a plan for you or
your child to stay healthy, which we call the CF treatment plan.
Treatment plans typically include a high-calorie, high-fat diet;
airway clearance therapies to loosen the clogged mucus from
the airways; aerosolized medications for the lungs; enzymes and
vitamins for nutrition; and a plan to be active and exercise.
Sticking to Your Treatment Plan
Sticking to your treatment plan can be thought of in two ways:
1. Sticking to your or your child’s plan of doing the prescribed treatments every day.
2. Sticking to a plan of making visits to your CF care center to see your team.
Sticking to the daily treatment plan helps to keep a person with
CF active and healthy. Even though the person with CF may not
actually “feel” it, these daily treatments are keeping his or her
lungs healthy. It's important to find a way to make daily CF care a
part of your routine daily activities.
Making regular visits to your CF care center is a very important
part of the CF treatment plan. People with CF need to visit their
CF care center at least four times per year for regular visits and
all of the recommended tests. Some people have treatment
plans that include regular visits more often than every 3 months.
More visits are necessary when a person with CF is not feeling his
or her best.
Why are CF care center visits important, even when a person with
CF is feeling “okay”? Your CF care provider and team need to see
people with CF regularly to see how they are doing and monitor
their health on a regular basis, just as people that do not have CF
need to see their primary care provider (PCP) on a regular basis.
STAYING HEALTHY: WHAT YOU CAN DO • 89
For people who do not have CF, the PCP may find that they have
blood pressure that is higher than normal or, on a routine blood
test, a cholesterol level that is too high. High blood pressure and
cholesterol level can be treated early and may prevent a stroke
or heart attack! Yet when your blood pressure or cholesterol level
start going up, you can't “feel” that! But your PCP can determine
that there is a problem.
For people with CF, regular visits to the CF center include tests
such as pulmonary function tests (PFTs), sputum or throat cultures
and blood tests. People with CF cannot feel small decreases
in lung function or a germ growing in the lungs. They only feel
symptoms when these issues get worse and start causing bigger
problems.
By the time a person with CF notices symptoms, he or she may
have big decreases in lung function, more symptoms and even
some permanent lung damage. Your CF care provider can
detect these things early with tests and can treat these problems
early. For example, early treatments of a first-time growth of
Pseudomonas may be able to get rid of the germs from your
lungs. If the Pseudomonas is not caught and treated early
enough, a person with CF might have to deal with Pseudomonas
for the rest of his or her life.
CF clinic visits also give you the opportunity to talk to your CF
team about how you or your child is doing. Ongoing, open
and honest communication with your CF care team will make it
possible to continue adjusting the treatment plan to be the best
for you or your child.
Daily care for CF takes a lot of time and commitment. We live
in a busy world, where none of us seems to have enough time.
Family and close friends can be a great support to you. And
remember, you are part of a team, and team members help
each other. Talk to your CF team members! Your CF team social
worker and psychologist can help you, for example, with time
management and coping skills. Sometimes it takes finding ways
to stick to your treatment plan. Sometimes it takes adjusting your
treatment plan. Your team is experienced in helping people with
CF and their families with these issues, so don’t hesitate to ask
them for advice.
CF treatment plans change over time. Talk to your CF care team
about how you or your child feels, and how you feel about the
treatment plan. Sticking to your CF treatment plan may seem
difficult at times. But when you take the time to eat well, exercise
and be active; do your daily CF treatments and medications;
and visit your CF care center regularly, you will find that you have
more energy to do the things you want to do.
90 • STAYING HEALTHY: WHAT YOU CAN DO
Exercising and Staying Active
Exercise and staying active should be a part of everyone’s daily
routine. People of all ages with CF, including those with lung
disease, can and should exercise. People who have CF can
benefit in many important ways from being active and exercising.
Remember that exercise does not have to be something formal
or done in a gym. It can be playing soccer with friends in the park.
There are so many benefits of exercise and staying active! Some
are listed here:
•
Exercise helps keep lungs healthy.
• Exercise loosens mucus in the lungs so the mucus can be coughed up more easily.
Exercise makes breathing deeper, and gets air around mucus in the airways.
•
Exercise (such as lifting weights and doing push-ups) helps build stronger muscles.
•
• Stretching exercises can improve flexibility and posture. Good posture can make it easier for a person with CF to take deep breaths.
• Weight-bearing exercise (such as walking, jumping rope, playing basketball and running) helps strengthen bones and can prevent osteoporosis.
•
Exercise can help improve a person’s energy level.
• Exercise increases appetite. A person with a good appetite eats more food, is more likely to have better nutrition and is more likely to have more energy.
People with CF should be
physically active. Most people with
CF have no limitations when it
comes to exercising. In fact, it has
many benefits for those with CF.
• Better fitness leads to longer and healthier lives for people with CF and without CF.
Regular exercise promotes a feeling of well-being, decreases stress and improves mood.
•
People with CF should talk to their CF care provider or physical
therapist about an exercise and activity program that is right for
them. Remember that people with CF need extra fluids and salt
with increased sweating or in hot weather.
Good Nutrition
Good nutrition is very important for people with CF of all ages.
There is a connection between good lung health and good
nutrition. A balanced, high-calorie diet with fat and protein
gives the body what it needs to grow normally and live well.
Remember: people with CF need extra calories to meet the
needs of their bodies.
STAYING HEALTHY: WHAT YOU CAN DO • 91
Good nutrition
• Develops strong lungs.
•
Keeps lungs as healthy as possible.
•
Builds and keeps a strong immune system to fight infections.
Getting Children to Eat
Getting children to eat can often be a challenge. For parents of
a child with CF, this can be a real source of concern. Here are
some things that parents can do to help children eat well to stay
healthy.
Plan Ahead
• Have three meals and three scheduled snacks every day, at about the same time every day.
• Before starting a meal or snack, do your best to have all the food ready.
•
Reduce distractions (no TV, toys, games or books at the table).
Make Mealtimes Structured
• Let your child know that it’s time to eat. (Say, “It’s breakfast time,” rather than asking,“Would you like some breakfast now?”)
Do not coax and beg your children to eat or punish them for not eating.
•
Sit at the table with your children. Relax and enjoy being together.
•
•
•
Praise your children when they are eating well.
Limit the length of meals to about 20 minutes.
• Reward your child for good mealtime behavior with a nonfood treat.
Give Choices
• Do not try to “trick” your child by hiding new or disliked foods in food they like.
• Let young children choose whether to open their enzyme capsules or have you do it for them.
Allow your child to choose between two high-energy/high-
calorie additives to foods (for example, extra cheese or extra butter or both).
•
• Even if your child chooses not to eat a new food, keep offering it. It often takes 10 to 15 “exposures” to new foods before children are willing to try them.
92 • STAYING HEALTHY: WHAT YOU CAN DO
Dealing With Emotions and Attitudes
• Try not to worry if a meal ends and your child has not eaten much. He or she will eventually get hungry.
Have a relaxed discussion with your child about the importance of healthy eating, at a time other than meals or in CF clinic.
•
Give your child an opportunity to talk about his or her feelings about having to eat a lot and about having CF.
•
Talk frequently with the dietitian, nurse, social worker and/or psychologist in your center for support and suggestions.
•
• Ask the dietitian, nurse, social worker and/or psychologist
to connect you with another parent of a child with CF who has found things that helped improve his or her child’s eating.
For more information about getting children to eat, you may want to read Nutrition: How to Encourage Healthy Eating, which is available from your CF care center or the CF Foundation's website at www.cff.org.
•
Adolescence, Puberty and Nutrition
Adolescence, or the teenage years, is the time of transition from
being a child to becoming an adult. Puberty, the period of time
when a person's body changes and becomes sexually mature,
takes place during adolescence. There is no other time
in life, except during infancy, when there is such intense
growth and development. Puberty is a process that takes
3 to 5 years.
At this time, for a young person with CF, getting enough
calories is very important to support all of the physical
growth and changes he or she is going through. Good
nutrition and enough body fat will help an adolescent
with CF start puberty and grow like their peers without CF.
Adults
For adults with CF, your busy work or school schedules
can get in the way of preparing high-calorie meals and
snacks. You can ask your CF dietitian about how you can
make some quick-to-grab snacks and plan for meals. For
helpful suggestions, visit the “Staying Healthy” section of
the CF Foundation’s website at www.cff.org.
STAYING HEALTHY: WHAT YOU CAN DO • 93
Cystic Fibrosis–Related Diabetes (CFRD)
When people have cystic fibrosis-related diabetes (CFRD), it is
important to keep it under control. Keeping the glucose at a
normal or near-normal level and getting enough calories helps
people with CFRD:
• Gain weight (for children) and maintain a healthy weight (for adults).
•
Feel better and have more energy.
•
Have better lung function and fewer pulmonary exacerbations.
• Lower their risk of long-term problems that diabetes can cause (including blindness and kidney disease).
Bone Health
As people get older, bones get weaker and can break more
easily. Two common bone diseases are osteoporosis and
osteopenia.
Minerals such as calcium, phosphorus, magnesium and fluoride
build bones. With osteopenia, bones do not have enough of
these minerals. This is called low mineral density, or low bone
density. Normal bones look like a “honeycomb” and have holes
in them. With osteoporosis, because of low bone density, the
holes get too big and make the bones weak.
People who do not have CF are at risk for osteoporosis and
osteopenia, but this is usually a problem that begins when
people are in their 50s or older. People with CF are at risk for
osteoporosis and osteopenia at a much younger age, even in
their teens.
Things that help keep bones healthy include:
• Good nutrition: getting enough calories and nutrients, including calcium and vitamin D
•
Taking enzymes and fat-soluble vitamins
• Weight-bearing and resistance exercise such as walking, jogging or weight lifting
• Getting regular CF care and getting tests to check nutrition (including vitamin levels) and special bone X-rays as your CF care provider and dietitian recommend
• Avoiding things that lower bone density, including alcohol, caffeine, carbonated drinks and smoking
For more information on bone health, you may want to read
“Nutrition: Bone Health and Cystic Fibrosis,” available from your
CF care center or the CF Foundation at www.cff.org.
94 • STAYING HEALTHY: WHAT YOU CAN DO
Ways to Increase Calories
People with CF need more calories than people without CF.
This is true for children who are growing, and for adults who are
trying to maintain a healthy weight while managing busy lives.
Sometimes people with CF may need up to twice the calories
that a person without CF their same age, sex, height and weight
would need. Taking in this many calories can be hard to do. Even
when people with CF eat often and well, they still may not be
getting enough calories.
Some ways to add calories to the diet are:
•
Drinking milkshakes and smoothies or nutrition drinks.
Trying some fast and easy snacks and meals such as frozen breakfasts, sandwiches or frozen meals to microwave, cheese and cracker snacks, granola or protein bars, pudding snacks, nuts or sunflower seeds.
•
Talk to your CF dietitian for more ideas based on your lifestyle
and what you like to eat!
Avoid Smoking and Secondhand Smoke
Breathing tobacco smoke is harmful to the lungs of all people —
especially for those with CF. This includes secondhand smoke.
Even the particles left behind on a smoker’s clothes, skin, hair
and breath can irritate the airways of people with CF.
Caregivers
Parents, relatives and caregivers of children with CF should not
smoke. If parents smoke, their children are more likely to smoke.
One of the best things you can do to help yourself or your child
stay healthy is to have a smoke-free environment. If you smoke,
there is help for quitting. Call the American Lung Association at
(800) LUNG-USA or go to www.lungusa.org.
While you are working to quit smoking, here are some simple
things you can do to help keep your child healthy:
•
Smoke outside away from your child.
•
Do not smoke in your home or car.
Do not let others smoke around your child or in your home
or car.
•
Secondhand Smoke and Lung Irritants
Secondhand smoke in enclosed areas, such as cars, homes,
apartments and restaurants remains in enclosed areas for hours,
days or longer. If a relative or friend who smokes in his or her
home invites you over with the promise that they won’t smoke
while you are there, the secondhand smoke is still present in the
air that you breathe.
STAYING HEALTHY: WHAT YOU CAN DO • 95
People with CF also should not work in an environment where
they are exposed to secondhand smoke, such as bars in cities
or states that do not have laws against smoking in public places.
They should also not work in places that expose them to other
lung irritants, such as dust, paint fumes, volatile chemicals and
vehicle exhaust.
Smoking by People with CF
For people with CF, smoking is disastrous. Quite simply, the
effects of smoking damage your lungs’ ability to breathe. For
young people, smoking harms the lungs’ ability to grow and
reach their full potential. Exposure to tobacco smoke also makes
people more likely to get respiratory tract illnesses, and for those
illnesses to be more severe.
Tobacco smoke — even secondhand — irritates the lungs and
sinuses and causes inflammation and swelling, which damages
the tissue. People with CF already have a hard time eating
enough and gaining weight. Exposure to tobacco smoke can
decrease the sense of smell and taste, thus decreasing their
appetites. Being a smoker may even disqualify a person from
being eligible for a lung transplant.
Tobacco smoke also shuts down the cilia, the tiny hairlike cells on
the lining of the airways that help remove mucus, dirt and germs
from the lungs and sinuses. Compared with normal lungs, lungs
with CF have mucus that is thicker, and the cilia have less fluid to
help them move. When the cilia are weakened by smoke, they
are even less able to keep the lungs clean.
When you have CF, you must constantly work to keep your
lungs in good shape so you can live a long, healthy life. To put it
bluntly, smoking and even exposure to other people’s smoke will
damage your lungs and make you sicker, and, ultimately, shorten
your life.
Vaccinations and Avoiding Germs
It is always better to prevent a health problem than to try
to fix one after it has started. For instance, vaccinations
(immunizations) are vital for prevention. Everyone should get
the recommended vaccinations, and a yearly flu shot. Avoiding
germs as much as possible is always best. Washing hands or
hand hygiene should be done routinely. Read more about
avoiding germs in Chapter 9, Staying Healthy: Avoiding Germs.
96 • STAYING HEALTHY: WHAT YOU CAN DO
Remember
There is a lot that you can do to help keep yourself or your child
with CF healthy and prevent difficult problems from occurring.
Know your or your child’s usual health status so that when
something changes, you will be ready to tackle it early, before it
develops into something worse.
Sticking with the daily CF treatments may seem tiresome at
times, but it is extremely important in order to keep a person
with CF as healthy as possible. Good nutrition, including a
high-calorie diet, is vital for a person with CF. Staying away from
tobacco smoke, including secondhand smoke, will prevent lung
damage. Although no one can avoid exposure to all germs,
doing good hand hygiene and getting the recommended
vaccinations will help a great deal. Your CF care team can help
you find ways to fit all of this into your life.
STAYING HEALTHY: WHAT YOU CAN DO • 97
Review Questions
Answers follow the questions. More than one answer may be
correct.
1.Which of the following statements about tobacco smoke are true?
a. Exposure to tobacco smoke makes people more likely to have respiratory tract illnesses.
b.If parents smoke, their children are more likely to smoke.
c. Secondhand smoke can linger in an enclosed area for hours, days or longer.
d. Being a smoker may disqualify a person from being eligible for a lung transplant.
e. All of the above
2. Which of the following are ways to get some exercise?
a. Playing soccer with friends on the weekend
b. Being on the high school football team and participating in all practices and games
c. Working out at the gym 3 days a week during lunch time
d. Going to a family picnic and running around and
playing games
e. All of the above
98 • STAYING HEALTHY: WHAT YOU CAN DO
Answers
To learn more, turn to the page number shown after the answer.
1. E
All of the above are true. Exposure to tobacco smoke makes
people more likely to have respiratory tract illnesses. If parents
smoke, their child is more likely to copy them and smoke.
Secondhand smoke can remain in an enclosed area for hours,
days or longer. Being a smoker may disqualify a person from
being eligible for a lung transplant. Pages 94-95.
2. E
All of the above. There are many ways to get exercise. Exercise
can be a formal schedule of walking, running or working out;
participation in sports; and informal activities. Page 90.
Notes and Questions
Chapter 9: Staying Healthy: Avoiding Germs
We live in a germ-filled world. People with cystic fibrosis (CF) get
more lung infections, so some things should be done to cut their
risk. For the person with CF and his or her family, this means a
balance between ignoring germs and worrying about them. It is
best to help the body fight germs and avoid germs when possible.
Germs and CF
Germs are tiny organisms that can cause infection. Germs
are all around us. For people with CF, some germs can
cause serious lung problems.
There are three major groups of germs:
1. Bacteria
2. Viruses
3. Fungi and molds
Many CF germs are spread by contact (or touch) and
droplets from coughing, sneezing and nose blowing.
Some germs, such as the viruses that cause colds, the
flu and respiratory syncytial virus (RSV) affect people
with and without CF. But when people with CF get a
respiratory virus, they may get sicker because of their
lung disease. Other germs — such as the bacteria
Pseudomonas aeruginosa (Pseudomonas) and
Burkholderia cepacia complex (B. cepacia) — cause
more harm in people with CF than in people who do
not have CF.
Remember that the gene mutation that causes CF leads to a
problem in how salt moves in and out of the cells in the lungs.
This causes thick, sticky mucus. Germs stick, stay and grow
well in this mucus in the lungs. The airways swell because of
inflammation and make more mucus. More germs grow, and the
cycle continues.
How Do Germs Spread?
Germs, such as bacteria and viruses that cause lung infection,
can spread between people in many ways. These are known as
“routes of transmission.”
To prevent the spread of germs,
use a tissue when coughing and
clean your hands afterward.
102 • STAYING HEALTHY: AVOIDING GERMS
The three main ways for germs to spread are:
1. By contact
2. In a droplet
3. Through tiny remains of droplets floating in air (airborne)
The most common way germs spread is by contact. This is
also called “direct contact transmission” or “indirect contact
transmission.” Viruses that cause common colds, RSV and CFspecific germs such as Pseudomonas and B. cepacia are spread
this way.
Direct contact is when germs spread by bodies touching, such as
through shaking hands, hugging or kissing.
Indirect contact involves touching something with germs on it,
such as touching a doorknob or sharing a cup. Germs spread to
you when you touch something with germs on it and then touch
your eyes, nose or mouth.
Routine Scheduled
Vaccinations for Children:
•
Diphtheria
•
Haemophilus B (Hib)
•
Hepatitis A
•
Hepatitis B
Human papillomavirus
(HPV)
•
•
Measles
•
Meningococcal
•
Mumps
•
Pertussis
•
Pneumococcal
•
Polio
•
Rotavirus
•
Rubella
•
Tetanus
•
Varicella (chickenpox)
When a person talks, sings, coughs, sneezes or laughs, droplets
are made. These tiny drops of liquid may have germs inside. The
droplets with germs can land in the eyes, noses or mouths of
others. This is how germs can be spread by droplet transmission.
These drops can travel 6 feet and more through the air before
they fall to the ground. The flu (influenza) and whooping cough
(pertussis) are spread this way.
Some germs travel through the air on specks of dust or particles
made when a person talks, sings, sneezes, coughs or laughs.
These germs can float in the air for a long time. They can be
carried a long way by air currents. Illness occurs when people
breathe in the germs floating in air. Tuberculosis (TB), measles
and chickenpox are some of the germs spread by airborne
transmission.
Vaccinations
One of the best ways to help the body fight germs is for
people to get their vaccinations. Vaccinations are also known
as immunizations, or “shots.” There are 2 different groups of
vaccinations: routine scheduled vaccinations, and seasonal or
yearly/annual vaccinations.
Routine Scheduled Vaccinations
EVERYONE, children and adults, should get the vaccinations
or shots recommended by the Centers for Disease Control and
Prevention (CDC). These vaccines help prevent illness. Getting
vaccinated, or immunized, is easy, low cost and saves lives!
All people with CF should get all their routine immunizations.
Household members, close relatives and people in close contact
with people with CF should also be immunized.
STAYING HEALTHY: AVOIDING GERMS • 103
Children should get their first immunizations before they are
2 months old. Older children and adults need to get routine
scheduled vaccinations throughout their lives. People should get
their vaccinations from their primary care providers. They may
also be able to give you information about special programs that
provide free immunizations.
Routine Scheduled
Vaccinations for Adults:
•
Diphtheria
•
Hepatitis A
If a person is behind on routine immunizations, he or she should
check with his or her primary care provider to schedule the
vaccinations needed to get caught up.
•
Hepatitis B
Seasonal Vaccinations
•
Measles
Influenza (Flu)
Influenza (the flu) is an illness caused by a virus that can make
people very sick. Every year in the United States, flu epidemics
occur during the winter months. Influenza is very contagious.
It can spread from person to person in droplets created by
coughing and sneezing. It can also be spread when people
cough or sneeze onto their hands and then touch things.
•
Meningococcal
•
Mumps
•
Pertussis
•
Pneumococcal
•
Rubella
•
Tetanus
•
Varicella (chickenpox)
•
Zoster
Anyone can get the flu, including people with CF. However, when
people with CF get the flu, they can get much sicker than people
who do not have CF. In people with CF, the flu can lead to a lung
infection.
Flu outbreaks in the United States usually occur between
December and March. Flu shots are often available as early as
August. People with CF should get the flu shot as soon as it is
available.
People with CF who are 6 months and older, and who are not
allergic to eggs, should get a flu shot every year in the fall.
Household members, relatives, day care and health care staff
and close friends of those with CF also should get flu vaccines
to prevent the flu. The flu vaccine comes as a shot or nasal spray.
People with CF should NOT get the nasal spray vaccine.
The flu vaccine does not give you the flu. Viruses in the flu shot
are killed (inactivated). They are weakened (attenuated) in the
nose spray. The risk of the flu vaccine causing serious harm is
extremely small. However, like any vaccine, mild side effects are
possible. Side effects may be a low-grade fever or some mild
aches that may begin shortly after the vaccination and usually last
1 or 2 days. The shot may cause soreness, redness or swelling
where it was given.
People who are allergic to eggs should talk to their primary care
provider or CF care provider about other ways to prevent the flu.
Human papillomavirus
(HPV)
•
104 • STAYING HEALTHY: AVOIDING GERMS
Symptoms of the flu include:
•
Body aches and headache
•
Fatigue
•
Fever and chills
•
Increased cough
•
Sore throat
If you or your child gets the flu, be sure to call your CF care
center, even if you did get the flu vaccine. If you or your child did
NOT get a flu vaccine and are exposed to someone with the flu,
call your CF care center. Your CF care provider may recommend
starting a medicine that may help you or your child have milder
symptoms and a shorter illness if you do get the flu.
Pandemic Viruses
Every year some people get the flu in the fall and winter. That
is why it is called “seasonal” flu. However, sometimes new
influenza viruses appear. These situations are called pandemic,
or worldwide infections. During pandemics of new flu viruses,
people can get the flu at any time during the year.
In 2009, for example, there was a pandemic flu caused by the
H1N1 flu virus. It was also called “swine flu.” The virus was first
detected in the United States in April 2009, and it was not limited
to a specific season. Now, when you get your flu shot, you are
also protected from the H1N1 flu virus.
Pandemic flu occurs from time to time. During these times, be
sure to find out if you should receive another flu vaccine.
Respiratory Syncytial Virus (RSV)
Respiratory Syncytial Virus (RSV) is another germ that anyone
can get. RSV can make babies, young children and the elderly
very sick. There is a special shot to decrease how severe RSV
is in young children. The child must receive the shot every
month during RSV season, which is the winter months. The CF
Foundation recommends that the RSV shot is considered for
children with CF younger than 2 years old. Good hand cleaning
reduces the risk of getting RSV or other germs for people of all
ages.
Getting immunized is a very important way of avoiding germs.
Everyone should get all their routine vaccinations and yearly flu
vaccines and maybe vaccines for other viruses. Everyone should
make sure to get all vaccinations recommended by the CDC at
the proper time, and to keep records of all immunizations. For
more and current recommended vaccinations, refer to the CDC.
Call toll free 1-800-CDC-INFO or 1-800-232-4636, or visit
www.cdc.gov/vaccines.
STAYING HEALTHY: AVOIDING GERMS • 105
Handwashing (Hand Hygiene)
One of the best ways to keep
from catching or spreading
germs is handwashing, or
hand hygiene. Clean your
hands often. (See “When to
Clean Your Hands” in the
next section.) Clean your
hands after touching things
that have contacted mucous
membranes, sputum or
phlegm.
You can reduce the risk of germs by
cleaning your hands regularly.
There are two ways to clean
your hands. The first is to
clean your hands with soap
and water, the second is
to clean your hands with
alcohol-based hand gel (see
“Cleaning with Soap and
Water” and “Cleaning with
Hand Gel.”)
If you see dirt on your hands, wash with soap and water. If you do
not see dirt, you can wash with soap and water, or use alcoholbased hand gel. Hand gel or sanitizer should contain at least 60
percent alcohol to clean hands. Some people carry hand gel with
them to use before eating and after nose blowing, coughing,
sneezing or shaking hands.
People with CF, parents and siblings can reduce some of the
risks of illness by regular handwashing or hand hygiene. Good
handwashing or hand hygiene should be practiced everywhere
— at home, school and work, during play, on vacation, at the
clinic or in the hospital. Be sure to ask everyone around you,
including health care workers, to wash their hands, too!
When to Clean Your Hands
Whenever your hands look dirty, use soap and water, not just
hand gel.
You should clean your hands often, especially at the times listed
here:
•
After coughing or sneezing or blowing your nose
•
Before eating
•
Before and after preparing food
•
Before giving and taking medicine
•
Before and after breathing treatments
Cleaning with Soap and
Water
1.Wet hands with warm water.
2.Apply soap. Liquid, antibacterial soap is best.
3.Rub hands together to lather.
4.Scrub hands well,back and front, up to wrist and between fingers. Clean under nails. This should take 20 seconds to complete. (This is
about the time it takes
to hum or sing the “Happy Birthday” song from beginning to end, twice.)
5.Rinse in warm, running water.
6.Use a clean paper towel to dry your hands completely.
7.Turn off the faucet by using the paper towel.
8.Throw paper towel away.
106 • STAYING HEALTHY: AVOIDING GERMS
•
Before and after airway clearance
•
Before touching your face or eyes or putting in contact lenses
•
After being around someone who is sick
•
After going to the bathroom
After changing diapers or cleaning up a child who has used the toilet
Cleaning with Hand Gel
1. Be sure the hand
sanitizer is alcohol-
based (at least 60
percent alcohol).
2. Apply the sanitizer to the palm of one hand.
3. Rub hands together.
4. Rub the sanitizer over all surfaces of hands and fingers.
5. Keep rubbing until hands are dry.
6. Rubbing should take about 15 seconds.
7. Remember, if you see that your hands are dirty, wash with soap and water.
•
•
Before and after clinic visits
•
After touching surfaces in the hospital room
•
After touching garbage
•
After touching an animal or cleaning up after your pet
Cough Hygiene — Cover Your Cough!
Many serious respiratory illnesses, including the flu, are spread
by coughing or sneezing. To help stop the spread of germs,
cover your cough!
Cover your mouth and nose with a tissue when you cough or
sneeze.
Put your used tissue in the waste basket.
If you don’t have a tissue, cough or sneeze into your upper
sleeve or elbow, not your hands.
Remember to clean your hands by washing them with soap and
warm water or using an alcohol-based hand rub.
Cleaning Respiratory Equipment
Germs can get on respiratory equipment and cause lung
infections. Clean and disinfect all equipment used for inhaled
medicines or airway clearance by using the guidelines described
here and in the manufacturer’s instructions. It is vital to clean and
disinfect equipment such as nebulizers that have been in contact
with mucous membranes, sputum or phlegm. To clean and
disinfect your nebulizer, follow these steps:
1.Clean your hands.
2.Clean the nebulizer parts.
The nebulizer parts must be cleaned before they can be disinfected. With a new paper towel, wash the inside and outside of the nebulizer parts with clear liquid dish soap and hot water. Be careful not to damage any of the parts. Throw the paper towel away, then rinse the nebulizer parts with water. Clean the nebulizer right after it is used to keep the medicine and debris from drying. Once debris dries, STAYING HEALTHY: AVOIDING GERMS • 107
it is difficult to wash off. You also can clean the nebulizer
parts in an automatic dishwasher if the nebulizer’s manufacturer’s instructions allow.
3. Disinfect the nebulizer parts.
DO NOT USE VINEGAR. Vinegar is not strong enough to kill the germs a person with CF might get.
Instead, ask your CF care team what is the best way to disinfect the nebulizer parts. Some options are:
• Using an electronic steam sterilizer (e.g., used for baby bottles)
• Boiling (in water) for 5 minutes
•
Microwaving (in water) for 5 minutes
Washing in dishwasher, if the water is hotter than 158° F, for 30 minutes
•
•
Soaking in 70 percent isopropyl alcohol for 5 minutes
•
Soaking in 3 percent hydrogen peroxide for 30 minutes
Read the manufacturer’s instructions to learn about cleaning and disinfecting your nebulizer. Do not use a nebulizer that cannot be disinfected.
4. Rinse the nebulizer parts.
If you disinfect with isopropyl alcohol or hydrogen peroxide, rinse all parts well with sterile water.
DO NOT USE WATER FROM THE FAUCET, BOTTLED WATER OR DISTILLED WATER. You can make water sterile by boiling it for 5 minutes. Use this water once, then throw it out. If you disinfect by other methods, you do not need to rinse the nebulizer.
5.Air-dry the nebulizer parts.
After the final rinse, drain the parts on a clean surface covered with new paper towels. Replace wet paper towels with dry ones and fully air-dry all parts. Germs will grow on anything that stays wet. Store the dry nebulizer in a clean, dry bag in a clean, dry place.
Some respiratory equipment may need to be cleaned but
not disinfected. These items can be cleaned often with liquid
soap and hot water. Ask the respiratory therapist, the nurse
or physician at your CF care center, how often to clean your
equipment and the best way to do so.
108 • STAYING HEALTHY: AVOIDING GERMS
When People With CF Live Together
People with CF who live together can get germs
from each other. To decrease the spread of germs,
they should limit contact with each other’s mucous
membranes, sputum or phlegm.
To decrease the risk of getting germs from each other,
people with CF should NOT share:
•
Respiratory equipment
•
Airway clearance devices
•
Toothbrushes
•
Eating utensils or drinking cups
Anything that has been in contact with mucous membranes, sputum or phlegm
•
When a person coughs, germs can spread 6 feet or
more. Doing airway clearance at different times and in
different rooms can help decrease the spread of germs.
People with CF who live together
should do airway clearance in
separate rooms and not share
respiratory equipment.
Being With Other People With CF
People with CF may benefit from knowing others with CF. These
benefits include support, friendship, advice and having a positive
role model. Many also benefit from feeling that they are not
alone in their fight against CF.
However, people with CF can pass germs to each other. Some
germs are worse than others. There are many different types of
germs out there. For example, Pseudomonas and B. cepacia
complex are both types of bacteria that can be passed between
people with CF.
People with CF who do not live together should avoid faceto-face activities and those that spread germs, such as handshaking, hugging or kissing. Keeping your hands clean helps
prevent the spread of germs.
At School
If your child has CF, consider letting the school nurse know.
Work with your CF care team to decide what information to tell
the school. Members of the CF care team can help you talk to
the school about CF. More than one child with CF may attend
the same school. They should not be in the same classroom. To
lower exposure to germs, ask the school to schedule common
activities, such as lunch, at different times. Again, hand hygiene
can help prevent the spread of germs that cause infections.
STAYING HEALTHY: AVOIDING GERMS • 109
Camps
CF germs have been passed between people with CF at CFspecific camps. People with CF should not attend CF-specific
camps. The risks of getting CF germs outweigh the benefits.
Because it is vital to exercise, make friends and build support
systems, people with CF are encouraged to join in camps, social
groups and sports activities that are not specifically for people
with CF.
CF Foundation’s Infection Prevention and Control
Policy
The CF Foundation’s Infection Prevention and Control Policy is
focused on protecting and maintaining the health of people with
CF at all Foundation events, meetings and offices.
Medical evidence shows that all people with CF could have
germs in their lungs and sinuses that might be spread to others
with CF. The infection prevention and control policy includes
practices and activities to promote safety and reduce the risk of
cross-infection among people with CF.
Besides cleaning your hands and covering your cough, the key
elements are:
At any CF Foundation-sponsored indoor event, meeting or office only one person with CF may be present. This person will be designated by the Foundation.
•
At CF Foundation-sponsored outdoor events or gatherings, people with CF need to maintain a distance of at least 6 feet from each other.
•
Under no circumstances shall individuals with CF who have ever had a confirmed positive sputum culture for Burkholderia cepacia (B. cepacia) complex attend any CF Foundation events, meetings or offices.
•
You can read the full policy on the CF Foundation’s website:
www.cff.org.
Germs in Public
Everyone should avoid unnecessary contact with people who
have a cold, the flu or appear ill. This does not mean that
children with CF should be kept from attending school or
activities because parents are afraid the child will get sick or
“catch” something. Nor does it mean that people with CF
cannot go to college, have jobs and enjoy active full and lives.
110 • STAYING HEALTHY: AVOIDING GERMS
Along with doing good hand hygiene and getting vaccinations,
parents of children who have CF can help their children avoid
germs by:
• Keeping children from play times or a sleepover with a child who is sick.
• Cleaning toys passed from another child to yours. Soak toys for 10 minutes in a solution of one quarter cup chlorine bleach and one gallon of water and rinse in plain water. Soft toys should be put through the washing machine regularly.
• If children are in day care, find out what the day care center’s policies are on children with illnesses who attend. Ask how often the toys are cleaned.
Beware of indoor play areas at malls, theme parks and other places where a lot of children pass through, such as a ball pit. The cleaning may be less than ideal.
•
People with CF need to:
• Avoid touching their eyes, nose and mouth, because germs spread this way.
Stay away from others if you are ill, and stay away from people who are ill. This helps prevent the spread of germs.
•
Avoid spas and hot tubs. Pseudomonas has been found in spas and hot tubs.
•
• Avoid pools without enough chlorine to kill Pseudomonas. Your city or county public health department can tell you
what chlorine levels kill germs and meet public health standards.
Consider your work environment:
- Work involving toddlers and school-age children — for example, work in day care centers and elementary schools — increases your exposure to respiratory viruses and may increase the frequency of CF lung infections.
- Work in a health care setting may expose you to germs that are potentially harmful to your lungs.
•
Infection Prevention and Control in the CF Care
Center Clinic and Hospital
“Infection control” or “infection prevention and control” is
a term used in the hospital or clinic setting. It refers to the
policies and procedures used to minimize the risk of spreading
infections. All CF Foundation–accredited care centers have
infection control policies and procedures in place to protect
people with CF.
STAYING HEALTHY: AVOIDING GERMS • 111
In addition, guidelines called “standard precautions” are
recommended by the CDC for reducing the risk of spreading of
germs from blood and other sources. Standard precautions apply
to all people receiving care in the clinic or hospital regardless
of their diagnosis or whether they have an infection. Standard
precautions apply to blood and all body fluids except sweat. This
means that health care workers should use gloves and gowns
and sometimes eyewear when they come into contact with blood
and body fluids.
You can read the guidelines for infection control on the CF
Foundation’s website: www.cff.org
Talk to your CF care team if you have any questions or concerns
about your CF clinic’s infection control policies.
Remember
Knowing how to avoid germs can help people with CF stay
healthier. Everyone should get all recommended vaccinations;
this includes the flu shot. All people with CF should avoid
unnecessary contact with people who have an illness, such as a
cold or the flu. Good hand hygiene helps prevent the spread of
germs. But no one can prevent exposure to all germs. Do not
panic when you or your child gets a cold. You cannot avoid all
germs without avoiding life. Children and adults with CF should
live life to the fullest. Mental and emotional health depends on a
normal, healthy contact with the world.
The following resources have helpful information about avoiding
germs:
• The CF Foundation (www.cff.org):
What You Should Know About Germs
Germs and Infection Control (webcasts)
• The Centers for Disease Control and Prevention
(www.cdc.gov):
Handwashing: www.cdc.gov/handwashing
Vaccines: www.cdc.gov/vaccines
112 • STAYING HEALTHY: AVOIDING GERMS
Review Questions
Answers follow the questions. More than one answer may be
correct.
1. All people with CF who are 6 months and older and who are not allergic to eggs should get the influenza vaccine every year before flu season starts.
a. True
b. False
2. Which of the following statements are true?
a. Children with CF should not be in group day care.
b. Hand hygiene is an
important part of preventing illness.
c. People with CF cannot pass germs to others with CF.
d. Alcohol-based hand gels is one way to clean hands.
STAYING HEALTHY: AVOIDING GERMS • 113
Answers
To learn more, turn to the page number shown after the answer.
1. A
It is true. ALL people with CF, who are 6 months and older and
are not allergic to eggs should get the flu shot every year before
flu season starts. In fact, household members, relatives and close
friends of people with CF; day care staff and health care staff
should get the flu shot as well! Page 103.
2. B, D
It is true that hand hygiene is an important part of preventing
illness and alcohol-based hand gels is one way to clean hands.
Children should live a normal life. They do not need to stay
home for fear of getting sick. People with CF can pass germs to
each other. Pages 105-106.
Notes and Questions
Chapter 10: Raising a Child with CF
Like any chronic condition, cystic fibrosis (CF) can cause social,
emotional and psychological challenges. Understanding these
issues can help you deal with them and raise a happy, healthy
child.
Emotional Impact of CF
The diagnosis of CF can cause many emotions.
This is normal. It is important to recognize them
so you can work through them. Talking about your
feelings with your family, friends and CF care team
can help. It also may help to talk with a counselor
or psychologist. Ask your CF social worker about
resources and counseling options.
Some of the emotions you may feel include:
•
Concern — for your child’s health
•
Worry — about the future
• Guilt — for having a child who inherited the disease from you
• Fear — of the unknown (How will I cope? How sick will my child be? How long will my child live?)
•
Anger — that your child has CF
• Resentment — for the time and attention that the child with CF needs
Keeping Life as Normal as Possible
Most people with CF live a normal daily life, with the challenge
of incorporating daily medications, airway clearance techniques
and other treatments and medications. Children with CF grow
up, go to school, have friends, have hobbies and can exercise
and play sports. Many go to college. Many marry and have
families. The people with CF who do best are often those who
were raised with the same expectations and rules as other
children.
The diagnosis of CF leads
to a whole host of feelings.
Understanding the impact of these
feelings is the first step in learning
how to deal with them and raising a
happy, healthy child.
116 • RAISING A CHILD WITH CF
In guiding your child’s emotional and psychosocial development,
here are some basic rules:
•
Treat your child as a normal child who happens to have CF.
•
Do not be overprotective or neglectful.
•
Foster your child’s independence as he or she grows up.
All parents want to protect their children. It is vital for children to
go through normal life events if they are to grow up and be well
adjusted. Mental and physical health are closely related. Try to
avoid being overly protective.
One way to encourage independence is to
help children learn about nature, science, art,
other cultures and the world. Such children
are less apt to be self-centered and selfpitying. If they can see that they are a part of
a world that is bigger than their family and
community, they will see that the world does
not revolve around them. They also will know
the joy of living and learning.
Foster a love of books by reading to your
child. Reading takes a child to other places,
far from the world of CF. It also is something
to do during airway clearance and breathing
treatments.
Let the child learn about nature,
science, art, other cultures and the
world.
Teach your child empathy for others and for their challenges.
Encourage your child to help others. Help your child see and
appreciate all that is good in life.
Parenting at Major Transitions
Certain issues can be expected as your child grows:
Age 0–3: Playing in playgroups or play centers, and going to
babysitters and/or day care centers are common at this age.
Besides your child’s normal developmental tasks, there are things
to teach other parents, babysitters and day care staff about
CF. This may include checking the cleanliness of the facility or
showing the staff how to give enzymes or other medicines to
your child.
Age 3–5: Children at this age are entering preschool and being
cared for by babysitters. Consider a program where the child
attends school all day for a few days a week so they can learn
about eating and taking enzymes away from home and family.
Problems with mealtime behavior are common in preschool
and school-age children. In children with CF, these may include
refusing or avoiding food, disruptive behavior and refusing
enzymes. Many parents feel pressure to get their child to eat.
RAISING A CHILD WITH CF • 117
Scolding, pleading, lecturing and forcing do not work and may
make things worse. Your CF dietitian can give you some tips on
how to have positive mealtimes and make good food choices.
Good nutrition is a team effort with the family and CF care team.
Let them know when you are stressed or need help with feedings
or mealtimes.
Age 5–6: At this age, children enter kindergarten. With teachers,
as with doctors, the goal is teamwork. Explain that you are glad
to answer questions or offer help, but you want your child to be
as independent as possible in taking enzymes before lunch and
snacks. Ask the teacher to help you promote independence and
self-care. Ask the teacher for suggestions. Mention that rarely are
there classroom emergencies with CF.
Some of the issues to discuss with your child’s teachers each year
are:
Coughing
• Frequent bathroom visits
• More water fountain visits (or the ability to have a water bottle)
• Taking enzymes at the beginning of all meals and snacks containing protein or fat
• Having extra snacks and extra portions of meals
• Medicines
• Absences when ill
It may help to send a letter to the teacher from you or the CF
doctor. Ask your CF care team for information to give to the
teacher. The CF Foundation has A Teacher’s Guide to CF and a
letter that may be helpful. For a free copy, go to www.cff.org or
call 1-800-FIGHT CF.
•
School-age children can often take their own enzymes and
medicines. Parents should become comfortable with other
people supervising their child taking enzymes.
Plan how to handle schooling and homework when a child is
hospitalized. Many children with CF are in the hospital for a week
or two, each year or so, for treatment of an exacerbation. Plan
ahead so it does not interfere too much with school. Talk to the
principal and teachers about how to handle schoolwork during
absences and hospital stays.
Call your CF social worker for help in planning. There may be
services such as tutoring or in-hospital schools that can help
your child stay on top of schoolwork. Consider giving the school
a written copy of this plan. The best way to help your child is to
stay involved.
118 • RAISING A CHILD WITH CF
Sometimes, students with CF and their families choose to
prepare an Individualized Education Plan (IEP), under the
Individuals with Disabilities Education Act, or a 504 Plan, under
Section 504 of the Rehabilitation Act of 1973. These laws help
people with CF get services they need to secure a free and
appropriate public education while maintaining their health.
For example, IEPs can help establish ways that students can
get special tutoring if they are absent because of illness or
hospitalization. Contact your child’s school at the start of each
school year about getting an IEP. Your CF care center has a
sample letter that your CF doctor can use to write to the school
to explain how CF affects your child and to identify some things
that may help.
To learn more about CF and school issues, talk to your care
center team. The CF Foundation pamphlet “School and CF” is
also available by visiting www.cff.org or by calling 1-800-FIGHT CF.
How to Talk to Your Child About CF
A child’s first questions about CF often come after starting
school. He or she sees that other children do not take enzymes
or do daily airway clearance. They may be asked questions about
CF by other children, adults or teachers.
The goal of telling your child about CF is to help your child know
what CF means and why eating right, taking enzymes and other
medicines, and doing airway clearance is important. Another
goal is to get your child ready to do his or her own care.
The hardest question for everyone is about death. Other children
may have heard that CF is a fatal disease of childhood, which
it once was. One mother explained it this way: “We told him,
‘There are a lot of ways a person can die. We do not know if you
will die from CF or not. Right now, you have medicines that help
keep you well. If you take your medicines and do what you are
supposed to do, you will probably be okay, but CF is something
you will have for the rest of your life.’”
Telling your child that he or she
has CF may seem hard. Yet,
understanding CF and why the
treatments and medicines are so
important helps your child prepare
for the future. Your CF care team
can help you explain CF to your
child.
Children should be taught about CF as soon as they can
understand. What you say to your child depends on his or her
age, personality and ability to understand. If you have questions,
ask your CF care team for help.
Parents should teach their children how to talk about CF. The
goal is for the child to be able to talk about it in a matter-of-fact
way. For example, “I take these enzymes to help my body digest
food”; “I do airway clearance to clear my lungs”; or, “The reason
I cough is to help my lungs.” Your CF care team can help you
teach your child about CF and how to answer questions.
RAISING A CHILD WITH CF • 119
What About Discipline?
Many parents of children with CF are concerned about
discipline. Many are hesitant or feel guilty about disciplining
their child. Discipline is vital for any child to be well adjusted and
psychologically healthy.
It is important that you apply the same discipline and standards
to all children in the family. If you have other children who do not
have CF, they may resent what they see as special treatment for
the child with CF.
It helps to teach and model social manners. You want to avoid
having a child with a chronic illness who acts “bratty.” Good
manners help any child to be accepted.
Sometimes, your child may refuse treatments. Explain how
important the treatments are for his or her health. Make the
treatments pleasant and fun by playing music, playing games,
reading or watching TV during treatments. The best way for your
child to learn how vital treatments are is for you to be consistent
in giving them. If they still refuse, you may need to withhold
“privileges” such as TV or video games to motivate cooperation.
If you need help, talk with your child’s CF doctor, social worker or
psychologist about a referral to a behavior specialist. Do not give
up: It is important for your child’s health!
Siblings (Brothers and Sisters)
Although siblings of a child with CF may feel ignored
or left out at times, some siblings also learn to care
about others. Children with CF get extra attention
because of the treatments. Teach siblings about CF
to help them understand why the child with CF may
get extra attention. Books about CF, like this one,
are good for siblings and relatives. To help siblings
feel important, spend some time with each one
alone every day. Allowing the siblings to attend clinic
visits or help with treatments will also help them feel
involved in caring for the child with CF.
School Friends and School Issues
When children start school, they make new friends and develop
a social life. Now you are dealing with classmates, playmates
and their families. It is hard to decide how much to tell others
about CF. Most families find a matter-of-fact approach is best.
School friends and their families may need to know that CF is
not contagious and that your child’s cough is not a symptom of
something that their child can get.
Siblings of a child with CF may
feel left out because of the extra
attention being given to the child
with CF. Your CF care team can
help you explain CF to all family
members.
120 • RAISING A CHILD WITH CF
If sleepovers, long visits or meals together
are planned, make sure your child’s
enzymes, other medicines and treatments
are in the plans. However, once in a while
your child might miss an airway clearance
treatment or a dose of medicine so that
he or she can be involved in activities with
a friend. It may be better for your child’s
psychological and social well-being to go to
a friend’s house than to stay home and not
miss a treatment. But make sure your child
does not skip treatments routinely.
It is important to speak to your child’s
teacher about CF and about the
importance of infection control for your
child’s health.
Classmates and friends join a child
with CF at a CF Foundation Great
Strides event. When questions are
asked about CF, encourage your
child to give a brief explanation
and let people know that they
cannot get CF from someone else.
Please keep in mind that the germs often carried by children who
do NOT have CF can be harmful to those with CF. Therefore, a
child with a cold or flu should be encouraged to stay home. If a
child with an illness is in the classroom, he or she should do hand
hygiene frequently and use tissues when sneezing, coughing or
blowing his or her nose.
CF-specific germs, such as Pseudomonas aeruginosa and
Burkholderia cepacia complex, usually are not harmful to people
who do not have CF. Therefore, a child with CF who has a lung
infection with these germs cannot pass it on to a child who does
not have CF. However, these germs can be harmful to others with
CF. If there is more than one person with CF in the school, such
as another student or a teacher, special attention to infection
control guidelines should be followed. (See Chapter 9 for more
information about avoiding germs.)
How to Handle Unwanted Questions
Just because your child has CF does not mean you have to open
your life up to everyone. Teach others about CF, but remember
that you and your family have a right to privacy. You may have to
deal with unwanted questions from concerned or nosy people
you meet in public places. They may ask questions or make
comments about your child’s cough. There are many ways to
deal with unwanted questions or comments:
•
Ignore them.
•
Smile and shrug.
•
Say, “My child has a lung problem that causes coughing.”
•
Say that your child has CF and explain a little about it.
RAISING A CHILD WITH CF • 121
Building a Relationship Between Your Child and the
CF Doctor
Doctor and clinic visits can be scary for young children. Reading
children’s books about doctor visits can help, as can a toy
doctor’s bag for play doctor visits. As your child gets older, go
over their questions before each doctor visit. Encourage your
child to ask questions. Role play to practice the visit. During the
visit, try to keep your child involved in the conversation. Let the
child answer the doctor’s questions. You can add the details later.
This helps teach children independence in their care.
It is important to help your child build a good relationship with the CF doctor.
Hospital Stays
When a child is in the hospital, parents should talk to the staff
about their level of involvement in their child’s care. Some care
has to be done by the staff; some can be done by parents or
other family members. Talk to the CF care team and hospital staff
about what is best for your child. Remember, you are part of the
team working to help your child get better.
During hospital stays, bring things from home to make you
and your child more comfortable (toys, videos, favorite stuffed
animal, pillow, favorite blanket or books). Check with the nurse
to see what you can bring and to get other ideas to make your
child’s hospital visit better.
122 • RAISING A CHILD WITH CF
Review Questions
Answers follow the questions. More than one answer may be
correct.
1. Who can help you find ways to work through the emotional impact of having a child diagnosed with CF?
a.Family
b.Friends
c. CF care team
d. Counselor or
psychologist
e. All of the above
2. Which of the following are true statements about
raising a child with CF?
a. Treat your child as a normal child who
happens to have CF.
b. Teach your child about CF as he or she grows.
c. During a hospital stay, parents are a part of the team working to help the child.
d. All of the above
RAISING A CHILD WITH CF • 123
Answers
To learn more, turn to the page number shown after the answer.
1. E
All of the above are true. Other people can help you work
through the emotional impact of a CF diagnosis. The diagnosis
can cause feelings of concern, worry, guilt, fear, anger and
resentment. These feelings are normal. Page 115.
2. D
All of the above are true. Your child is a normal child who
happens to have CF. Teach your child about CF as he or she
grows. During a hospital stay, parents are a part of the team
working to help the child. Pages 115, 118, 121.
Notes and Questions
Chapter 11: Adolescence and CF
Adolescence (the teen years) is the time of transition from being
a child to being an adult. As you grow and your body changes,
and you gain more freedom, you also take on more and more
responsibility.This means that many of the CF-related tasks that
parents once took care of will become the teen’s job. The CF
care center team is there to help with this transition.
Staying Healthy
Dating, learning how to drive, working
your first job and choosing a path for
higher education or job training beyond
high school are just a few of the exciting
milestones of adolescence. You should
also plan to reach for your goals. It is more
important than ever that you stick to your
CF treatment plans.
You want to enjoy your life now and have
fun with your friends, and it may seem as if
CF treatments are getting in the way of your
fun. But, remember, if you get sick, you will
have even less time to do fun things. Staying
healthy and avoiding CF complications by
sticking to your treatments will help you
enjoy life more. If you feel stressed out
about the amount of time needed each day
for CF treatments, or if you are wondering why you need to do a
particular treatment, talk about it with your CF doctor or nurse.
Perhaps you can work together to find a way to make treatments
less time-consuming.
Young people with CF go through puberty and develop into men
and women just as other people without CF do. When puberty
starts may depend on the overall health of a person. Teens with
CF with good nutrition will usually have a normal growth spurt.
Having enough body fat helps puberty start. Getting enough
calories is very important to support all of the physical growth
and changes you are going through.
Doing CF treatments every day will
help keep you healthy and help you
reach your goals.
126 • ADOLESCENCE AND CF
No one should smoke — especially not people with CF. Smoking
harms just about every part of your body, but it is devastating
for your lungs, which already have enough to deal with from CF.
Some adolescents with CF, if under peer pressure to smoke,
find it works well to use their CF as an excuse to say no. You
also need to stay away from secondhand smoke, or breathing in
smoke from other people’s burning tobacco. If you smoke, ask a
member of your CF care center team to help you find ways to stop.
Most teenagers experience intense emotions at times, including
sadness, anxiety and confusion. This may sometimes be even
more true for teenagers who have with CF. If you are feeling
overwhelmed by your emotions, talk to someone you trust, such
as a family member, a friend, a teacher, your school counselor
or a member of your CF care team. The social worker or
psychologist at your CF care center can be helpful in times like
these.
Sports and Hobbies
Most people with CF have normal experiences with school,
sports and hobbies. The more exercise and activity you do,
the better for your health. Exercise helps keep lungs healthy,
improves your appetite, reduces stress and makes you feel good.
Remember, when exercising or playing sports or when you are
out in the heat, people with CF lose more salt in their sweat than
others. You should carry water bottles when
exercising or when in the heat, and stop for a
big drink of water at least every half hour. You
should also eat salty foods before and after
exercise.
Talk to your CF doctor or physical therapist
before starting an exercise program about what
type of exercise is appropriate for you, how
long it is safe to exercise and whether there are
any precautions. If participating in highly active
sports is not possible for you, look for other
activities and clubs you can join to share your
interests and skills with your peers at school
and in your community.
People with CF should do activities
they enjoy.
Your Identity and CF
Yes, you have CF. But CF does not define who you are. During
adolescence, you are developing your identity — a continuing
process of learning, exploring your interests, developing your
strengths and interacting with others to figure out who you are.
It is a very exciting time, but it also can be a time of great anxiety
for any adolescent.
ADOLESCENCE AND CF • 127
Adolescents with CF may have unique worries. They may be
self-conscious about their cough. They may be smaller than
their friends. They may tire more easily. They may have to take
medicines or treatments at school. They may worry that no one
will want to date them because they have CF.
Your discomfort may be eased by learning as much as you
can about what CF is, the challenges it can bring and why the
treatments and medicines are needed. It will help if you can
explain to friends that:
•
The cough is not contagious and helps you clear your lungs.
Medicines help digest food, fight respiratory infection or provide vitamins.
•
•
CF is what you have, not who you are.
Most adolescents find that being open about having CF makes
life easier, especially with close friends and people they date.
Letting friends know about CF can prevent some awkward
moments, such as having to explain why you go to the bathroom
more or need to take so many pills with meals. Many adolescents
who have been open with friends say it does not change their
friendships. Removing the mystery helps build acceptance and
understanding. Role playing questions and answers with parents
or siblings may also be helpful.
Becoming Independent
Adolescents and young adults want independence. They may
rebel against schedules, treatments, medicines and limits. They
may refuse to do regular airway clearance. It is normal for you to
want independence. Learning independence and responsibility
is a vital part of growing up. However, refusing to do your CF
therapies can cause serious — even life-threatening — harm to
your health.
Work with your parents and the CF care team to gradually
shift the bulk of responsibility for CF care from your parents to
yourself. Some of the things you must start to do include:
Learn about your treatments, including the names of your medications, what dosage to take, what time to take and how often to take.
•
Learn how to take care of your airway clearance equipment, including cleaning it correctly.
•
•
Call the CF clinic yourself to ask questions.
Write down questions for your CF care team before your clinic appointments.
•
128 • ADOLESCENCE AND CF
• See the doctor first alone during CF care center visits, then with your parent or guardian.
•
Get to know the ins and outs of your health insurance plan.
Transferring to the Adult CF Clinic
Toward the later part of adolescence, most people with CF
transfer from a CF clinic that specializes in treating children
to one that specializes in treating adults with CF. It may be a
little sad and scary to leave the clinic and the people who you
know so well, but adults with CF are different from children with
CF. The staff at the adult CF clinic are experts in caring for the
special health care needs of adults with CF. The transition should
be a planned process over time, not an abrupt change.
Planning for the Future
Adolescence is the time to start planning for the future. Even
though you have CF, you should be thinking about education
and jobs just like your friends without CF are. Start thinking
about your future education or job training at the start of high
school, not at graduation. There are scholarships available
specifically for people with CF. There also are programs to help
people with CF cope with the stress of high school and college
or to find job training. Your CF social worker can help you find
these programs.
Colleges, universities, vocational training centers and other
places of higher education that get federal funds cannot refuse
to admit a qualified student solely because the student has a
disability such as CF. Most colleges and universities have an
office for students with disabilities that can help students get the
accommodations they need. You do not have to tell the school
that you have CF unless you request accommodations because
of CF.
ADOLESCENCE AND CF • 129
After you graduate from higher education, make sure that the
employer you choose offers a health insurance plan and other
benefits, such as sick leave, that fit your needs. The social worker
at your CF care center can help you with this. You can also find
more information about what to look for in a health insurance
plan on the CF Foundation website, www.cff.org.
For more information about school and adulthood with CF, see
the resource list in Appendix B, page 183.
Remember
The teen years are a time of incredible growth for your body and
mind. You will take on more and more responsibility for CF care
and other tasks as you get closer to adulthood. Keeping yourself
healthy by sticking to your CF treatments will help you make the
most of this exciting time and prepare for the future. Base your
developing identity on your interests and strengths, not your
CF. Talk to your CF care center team about how to find a healthy
balance between CF care and the other parts of your life.
Notes and Questions
Chapter 12: Adult Life with CF
People with CF are living longer and healthier
lives. As those with CF move into adulthood,
new issues develop concerning independent
living, marriage, family planning, career and
finances. It can be hard to address these issues
at the same time that a person is managing a
chronic condition. People with CF should be
encouraged to settle into an adult lifestyle,
mixing good health care with independence,
marriage, family and career. Your CF care team
can help you with these issues.
Transition Issues
Most people with CF transition from pediatric to
adult care between the ages of 17 and 21. This
means changing from a pediatric to an adult CF
care setting. This transition should not happen
abruptly, but instead should be a planned process over time.
During this transition, the responsibility for CF care moves from
parents to the person with CF. Adult clinic staff usually talk with
parents only after getting the adult with CF’s permission. They
also deal with adult issues, including marriage, parenthood and
careers.
Independence
Most adults with CF live on their own and do their own CF care.
Some airway clearance techniques offer more independence
with treatments. Ask your CF respiratory therapist which
technique is best for you.
During illness, adults with CF may need help with their care. The
CF care team, family or friends may be able to arrange for help
and support during these times.
Family Issues
Young adults with CF may deal with marriage and family issues.
Even though CF does not affect a person’s ability to participate
in sexual activity, CF can make it harder to get pregnant. Women
with CF may be less fertile than women without CF. Ninetyeight percent of men with CF are infertile. This can make family
People with CF are now living
longer and healthier lives and they
are facing new challenges.
132 • ADULT LIFE WITH CF
planning stressful. Your CF care team can arrange a fertility
evaluation so you can find out your fertility status and determine
whether any medical help will be needed.
For a woman with CF to have a successful pregnancy, it is
important that she has good lung function and nutritional status.
If you are thinking about getting pregnant, talk to your CF doctor
about what you need to do to make sure your health is strong
enough. Also, if you get pregnant, it is important to have some
help with child care in place before the baby is born so that you
are able to also care for your own health and the baby.
Because a person with CF has two copies of the CF gene
mutation, any biological child he or she produces will inherit one
copy of the gene mutation. (It takes two copies for a person to
have CF.) Therefore, if the other person in the couple does not
have CF, and it is not known whether he or she carries a CF gene
mutation, some couples may decide to have that person tested
so that they know how likely it is that they will have a child with
CF. However, the test cannot detect every CF gene mutation.
Your CF care center can help arrange genetic testing and
genetic counseling. Some couples decide that adoption is the
best choice for them.
It is important to keep in mind that if people with CF do not
wish to become pregnant, they still should use contraception.
People with CF also need to use barrier protection, such as a
condom, to protect against sexually transmitted diseases, just
like anybody else would.
See Chapter 6 in this book for more detail about the reproductive
system in people with CF.
People with CF should plan for the
future like everyone else.
ADULT LIFE WITH CF • 133
Career
Just like other adults, those with CF should look for a satisfying
job or career that uses their special skills and interests. However,
adults with CF must manage the effects of chronic illness on their
careers. Treatment schedules and medicine routines must fit into
work schedules. Doctor visits and illness may cause missed work
days. Some adults with CF seek employers who offer flexibility or
choose to be self-employed. Access to quality health insurance is
another important consideration. The CF care team can help with
treatment and work schedules and can help educate employers
about CF.
People with CF should use common sense with regard to their
work environment. For example, people with CF should select
work that avoids exposure to lung irritants, such as smoke, dust
and chemicals. Work with young children, such as in a day care
center, and certain health care settings could expose a person
with CF to germs that may cause respiratory infections.
Talk to your CF social worker and care team about what to look
for and what to avoid in the workplace, as well as your legal
rights, and whether or when to share your CF diagnosis with your
employer. For more information about your legal rights, see the
resources in Appendix B in the back of this book.
Remember
As they grow up, people with CF should plan ahead to have
the same experiences as adults without CF, including living
independently, a career, marriage and children. Choose your own
unique path, and work with your CF care team to figure out what
you need to do to fulfill your dreams in a way that also maintains
your health.
Notes and Questions
Chapter 13: The CF Care Team: Who They Are and What They Do
People with cystic fibrosis (CF) need a team approach for all
aspects of their care. At Cystic Fibrosis Foundation–accredited
care centers, the team includes the person with CF, the family
and several members from different medical disciplines and
specialties. With so many people on the CF care team, it is easy
to get confused. This chapter describes the members of the CF
care team.
Who Is on the CF Care Team?
The most important members of the CF care
team are the person with CF and his or her
family members. The clinic part of the team is
headed by the physician who sometimes is the
CF care center director. The CF Foundation
requires that the following medical people are
part of every CF Foundation–accredited care
center:
•
Physician/CF care center director
•
Program/clinic coordinator
•
Nurse
•
Dietitian
•
Social worker
•
Respiratory or physical therapist
Many CF care centers also have other specialists
on the care team. These members may include:
•
Other physician specialists
•
Doctors in training
•
Nurse practitioners
•
Physician assistants
•
Clinical nurse specialists
•
Pharmacists
•
Psychologists
•
Child life specialists
•
Chaplains
•
Genetic counselors
Since CF is a complex disease, it
requires a team approach. The CF
care team consists of the person
with CF, the family and several
health care professionals.
136 • THE CF CARE TEAM: WHO THEY ARE AND WHAT THEY DO
Role of the People on the CF Care Team
People with CF, their families and CF health care professionals
make up the CF care team. Every member of the CF care team
has a vital role in helping provide the best personal care for the
person with CF.
The Person with CF and His or Her Family
People with CF and their families are full partners of the CF
care team in managing this chronic condition. Information
and communication must flow to and from the person with
CF and family members and other team members in an open
and trusting environment. Every person with CF and his or her
family members are able to be involved in care at the level he
or she desires. Care is respectful of the persons’ and families’
preferences, needs and values.
Physician and CF Care Center Director
The CF care center director is the doctor who
heads up the CF care center and health care
team. Not all doctors at a CF center are the
center director, but they are experts in how to
care for people with CF.
The CF doctor works with the person with CF
and his or her family, and the medical care team
members, to make a treatment plan. He or she
also teaches other doctors, health care workers
and the public about CF. They may also do CF
research. Your CF doctor will communicate and
work with your primary care provider to provide
the best care.
The team approach to CF care may
seem confusing at first, but it gives
you access to experts in CF care.
Program/Clinic Coordinator
The program or clinic coordinator is a health care professional
who coordinates the CF health care team. He or she works with
the director to be sure that the CF program runs smoothly. Much
of what the coordinator does is “behind the scenes.” If you have
a question about how the clinic runs, you may want to ask the
coordinator.
Nurses
CF nurses specialize in CF care. CF nurses fill a variety of nursing
roles in CF care centers. Nurses are the primary contact for people
with CF and their families. CF nurses work in the CF clinic setting,
provide bedside care for people needing hospitalization and
provide phone contact. Nurses have the primary responsibility for
providing CF education at initial diagnosis and throughout life.
THE CF CARE TEAM: WHO THEY ARE AND WHAT THEY DO • 137
CF nurses:
•
Help coordinate and carry out health care plans.
Aid communication between the CF care team and the person with CF and family.
•
Alert team members to psychological, social and financial concerns.
•
• Educate the person with CF, the family, the public and other health care workers.
•
Support the person with CF and his or her family.
Dietitians
A dietitian (RD) is trained to look at a person’s diet, growth and
overall nutrition. A dietitian can teach people with CF and their
families how to adjust their diets so that the person with CF can
get the calories and nutrients he or she needs to be healthy.
Dietitians help people with CF and their families keep the person
with CF well nourished. They advise about:
•
Baby formulas
•
High-calorie, high-protein diets for people of all ages
•
Supplemental vitamins
•
Pancreatic enzyme supplements
•
Dietary supplements
Social Workers
Social workers (SWs) help people with CF and their families with
the social, emotional and psychological impact of dealing with
CF. The social worker helps families deal with situations and
issues that may interfere with their ability to handle health
problems. He or she aids people to figure out how to get help
with health care insurance, career choices and school issues. The
social worker helps people with CF and their families by:
Providing assessments and counseling regarding the psychosocial aspects of CF.
•
• Aiding communication between the family and other members of the care team.
•
Teaching ways to cope with the stress of a chronic condition.
• Providing help to people with CF and families as they negotiate
multiple systems, including insurance and employment.
•
Finding financial assistance for families.
•
Preparing teens and young adults for independence.
•
Identifying family stress that may need professional help.
•
Supporting family-centered care to provide optimal CF care.
138 • THE CF CARE TEAM: WHO THEY ARE AND WHAT THEY DO
Respiratory Therapists
Respiratory therapists (RTs) plan, teach and carry out respiratory
care programs. In the hospital, RTs care for people with CF,
giving airway clearance and aerosol treatments. RTs teach
people with CF and their families how to do these treatments
at home. They are also responsible for the care and use of
equipment, including nebulizers, air compressors and oxygen
systems. The RT does pulmonary function tests (breathing tests).
Physical Therapists
Physical therapists (PTs) design and direct exercise and activity
programs for people with CF. A PT is trained to help a person
make an activity and exercise program to match his or her
age, health and interests. The PT helps people regain strength
and endurance through various techniques and improve their
mobility and lung function. In some CF centers, the PT will help
with airway clearance.
Other Physician Specialists
Physician specialists are also referred to as subspecialists.
Physician specialists have extensive training and practice in a
particular field of medicine or surgery.
Pulmonologist
A pulmonologist is a doctor who has special training in the
diagnosis and treatment of diseases of the lungs. Some
specialize in the care of children and teens, and others specialize
in the care of adults.
Gastroenterologist
A gastroenterologist is a doctor who has special training in the
diagnosis and treatment of diseases of the digestive system. This
includes problems with the esophagus, stomach, intestines and
liver.
Endocrinologist
An endocrinologist is a doctor with special training in the
diagnosis and treatment of diabetes and other hormonal
diseases, including problems with thyroid hormone and growth
hormone.
Health Care Professionals in Training
If the person with CF is cared for at a CF Foundation–accredited
care center that has training programs, he or she may see other
people who are learning about CF. These may include other
doctors, medical students and health care personnel who are
learning about CF and how best to treat people with CF.
THE CF CARE TEAM: WHO THEY ARE AND WHAT THEY DO • 139
Fellows
A fellow is a doctor who has finished medical school and
residency and is getting specialty training. The primary or
“attending” CF doctor supervises fellows. At CF care centers,
they are often pediatricians or internal medicine doctors who
are specializing in CF and lung diseases. After years of special
training, the fellow often goes to another CF care center to be a
CF doctor.
Residents
A resident is a doctor who has finished medical school and
received a medical degree. He or she is getting more training,
often in pediatrics or internal medicine.
Medical Students
A medical student is working to become a doctor. Medical
students are learning about diagnosing illnesses, caring for
people and evaluating and reporting a person’s condition and
progress to the attending physician.
Nurse Practitioners
A nurse practitioner (NP) is a nurse who has an advanced
degree with more education than other nurses. An NP is trained
and licensed to do physical examinations and to prescribe
medications and treatments. A pediatric nurse practitioner
(PNP) is an NP who cares for children. An FNP is a family nurse
practitioner. An ANP is an adult nurse practitioner.
NPs work closely with the CF doctor to plan and carry out a
health care plan for each person and family. They also help the
other nurses coordinate care for and provide education for
people with CF and their families.
Physician Assistants
A physician assistant (PA) is trained and licensed to practice
medicine under the supervision of a physician. A PA can do
physical examinations and prescribe medications. PAs work
closely with the CF doctor to plan and carry out a health care
plan for each person and family.
Clinical Nurse Specialists
Clinical nurse specialists (CNSs) are nurses who have an
advanced degree with more education than other nurses. CNSs
have special training with groups of people with special health
care needs, often people with chronic conditions. CNSs work
with other CF team members to coordinate and manage care
and provide education for people with CF and their families.
140 • THE CF CARE TEAM: WHO THEY ARE AND WHAT THEY DO
Pharmacists
A pharmacist helps manage the use
of medicines in the hospital and
home. A pharmacist looks to make
sure medicines being prescribed do
not react with each other and checks
the dosages to help avoid errors. A
pharmacist can show people how to
take medicines and tell them what
possible side effects to watch for.
Psychologists
A psychologist is a health care
professional with expertise in
assessing and treating problems
with behavior, learning, emotions
and group/family interactions. A psychologist helps with such
problems as depression, learning disabilities or behavior issues.
A CF team psychologist may also focus on the problems people
might have managing CF. These include balancing treatments
and other aspects of life, helping those who are having trouble
developing self-management skills and helping children and
teens manage peer issues related to CF. Psychology services may
be brief consultations during regular CF clinic visits or during CF
hospitalizations, or may require referral for ongoing psychology
services. Psychologists help families and people with CF by
providing:
• Adjustment to diagnosis (directed toward parents of newborns, and children and adults who are diagnosed later)
• Behavior management with young children (both general and CF-related issues)
Assessment of cognitive, academic and emotional functioning
•
Assistance with issues related to emotions, stress and being able to stick to the CF treatment plan
•
Genetic Counselors
A genetic counselor is a medical professional trained in genetics.
A discussion with a genetic counselor can help a person
understand inherited conditions and how an inherited disease is
passed on to one’s children. For example, families with a history
of CF may learn their risk of having a child with CF through a
genetic counselor.
THE CF CARE TEAM: WHO THEY ARE AND WHAT THEY DO • 141
Child Life Specialists
Child life specialists are trained to help children and their families
cope with medical conditions and the tests and treatments
required in the clinic and hospital and at home. A child life
specialist helps children understand what is happening in a
simple way according to their age and abilities. This helps
children deal with their fears about illness and medical tests and
treatments. Child life specialists:
Plan activities and entertainment during clinic visits and hospitalizations to provide ways to distract children from the stress of the medical treatments and the clinic or hospital environment.
•
•
Teach skills for dealing with the stress of a chronic illness.
• Teach and direct medical play therapy to help children understand treatments and procedures.
• Provide a space in the clinic and hospital that is free from medical procedures, to keep a space for children that is “normal” and “safe.”
• Work with other team members to teach children about CF, its effects and its treatment.
Chaplains
Many CF care centers have chaplains on the CF care team. They
assist the person with CF and his or her family with spiritual issues.
Remember
The multidisciplinary team approach to CF health care may be
confusing at first because so many people are involved. But,
remember, everyone is working together with you to provide the
best care for you or your child. And the best way the team can
succeed is through open communication with you — the person
with CF and/or the family members. If you are not sure who is the
correct person to answer a particular question, start with your CF
nurse, who can direct you to the right team member.
Notes and Questions
Chapter 14: The Cystic Fibrosis Foundation
The Cystic Fibrosis Foundation, a nonprofit, donor-supported
organization, is the world’s leader in the search for a cure for
cystic fibrosis (CF). The CF Foundation’s mission is to find a cure
for CF and to improve the quality of life for people living with
the disease. It accomplishes this by working to provide access to
specialized medical care and effective treatments for people with
CF and by supporting research to help develop new CF drugs
and therapies.
The CF Foundation also works on behalf of people with CF
through its public policy and advocacy program and provides
educational resources to people with CF and their families, the
public and medical professionals to increase knowledge of CF.
Medical Care
The CF Foundation funds and
accredits more than 110 CF care
centers, with affiliate programs
and child and adult CF care
programs throughout the
country. At these clinics, people
with CF have access to state-ofthe-art CF care delivered by a
team of CF experts from multiple
specialties. This approach helps
CF Foundation–supported
ensure that people with CF have
researchers are developing new
the best health possible. Each
therapies to treat CF.
center is thoroughly reviewed
by the CF Foundation’s Center Committee before it receives
accreditation and funding. The review for accreditation occurs
every year.
More than 40 years ago, the CF Foundation started collecting
patient data, such as height, weight and gender, to track health
trends and find better treatments. More than 27,000 people with
CF get care at CF care centers across the United States. Patient
data are kept in the confidential Patient Registry database. The
registry provides the opportunity for wider insight into the disease
and helps identify the best treatment methods and improve the
quality of care.
144 • THE CYSTIC FIBROSIS FOUNDATION
Every year the CF Foundation publishes a Patient Registry
Annual Data Report, which provides information on the health
of people with CF and how much it has improved over time. In
2001, the Patient Registry was used to look at the overall health
of people with CF by care center. The information showed that
there were differences between care centers. Because of this,
the CF Foundation began its quality improvement (QI) work. This
effort is focused on speeding up the rate of improvement in care
throughout the CF care center network. The work includes
training care center staff in QI and helping make the partnership
between people with CF, families and care center staff stronger.
To improve CF care, the CF Foundation and care centers work to
reach the following goals:
1. People with CF and their families will be full members of the care team.
2. People with CF will have normal growth and nutrition.
3. People with CF will get the right therapies to keep lungs healthy and have fewer respiratory infections.
4. People with CF, their families and CF health care professionals will work together to decrease the spread of germs among people with CF.
5. People with CF will be followed closely to find and treat complications early.
6. People with CF and their families are supported when facing decisions about transplantation and end-of-life care.
7. People with CF will get care no matter their race, age, education or insurance coverage.
An important part of the QI work is to create up-to-date CF care
guidelines to be used in all CF care centers. Care or practice
guidelines recommend treatment based on published reports
of clinical trials that look at safety, effectiveness and potential
benefit. The guidelines include CF-related topics about nutrition/
gastrointestinal health, respiratory health, infection control and
more. The CF Foundation brings together experts on specific
subjects, including doctors, nurses, respiratory and physical
therapists, dietitians, adults with CF and parents of a child with
CF to write guidelines on each topic.
The CF Foundation also has a Quality Improvement Toolkit.
This toolkit helps people with CF, their families and care centers
become stronger partners in improving the quality of CF care.
For more information, visit the CF Foundation’s website
(www.cff.org.)
THE CYSTIC FIBROSIS FOUNDATION • 145
Patient Assistance Resource Center
The Cystic Fibrosis Patient Assistance
Foundation (CFPAF), a subsidiary of the CF
Foundation, is a nonprofit organization that
helps patients and their families living in the
United States afford medications and devices
they need to manage CF.
For eligible enrollees, the CFPAF provides the
following types of financial assistance:
• Payment of co-payments for approved prescription drugs and devices
Payment of co-insurance for approved prescription drugs and devices
•
• Payment assistance with deductibles for approved prescription drugs and devices
The CFPAF also serves patients and families through:
•
Insurance benefit verification and insurance counseling
•
Reimbursement support for pre-authorizations and appeals
•
Referrals to other patient assistance drug programs
Referrals to alternative support programs, including rental
assistance, local/regional assistance and other nonprofit organizations
•
•
Patient advocacy and case management services
• An insurance access program to determine state and federal insurance eligibility, as well as Supplemental Security Income (SSI) and Social Security Disability Insurance (SSDI).
To learn more about the CFPAF, visit www.CFPAF.org or call
1-888-315-4154.
Your care center social worker and the CF Foundation have more
information available about private health insurance coverage and
public assistance programs. To learn more, call 1-800-FIGHT CF
or 301-951-4422 or go to www.cff.org and look for the “Living
with CF” section. More resources also can be found in Appendix B
in the back of this book.
The CF Foundation also has the Patient Assistance Resources
Library, an online database that provides up-to-date material to
help people with CF, their families and CF health care providers
work together on health insurance issues. Learn more at
www.cff.org/LivingWithCF/AssistanceResources/Library.
146 • THE CYSTIC FIBROSIS FOUNDATION
The CF Legal Information Hotline provides free information about
the laws that protect the rights of people with CF. Supported by
the CF Foundation, the hotline is an important resource for people
with CF who are facing challenges with health care coverage or
reimbursement issues. You can contact the hotline at 1-800-622-0385
or CFLegal@cff.org.
For information about all of the Patient Assistance Resource
Center programs offered by the CF Foundation, visit
www.cff.org/AssistanceResources.
CF Services Pharmacy
The CF Foundation is affiliated with Cystic Fibrosis Services Inc., a
Walgreens alliance partner. CF Services is the nation’s leading provider
of specialty CF drugs, case management services and reimbursement
support for people with CF. For more information, visit
www.cfservicespharmacy.com.
Research
The CF Foundation drives CF research forward. One way it does so
is with its support of the CF research center network. These centers
are located at universities and medical schools around the country.
They bring together researchers who are experts in different areas of
medicine. Scientists at these centers share ideas and information to
increase what we know about CF. As knowledge about CF and how it
affects the body has grown, researchers are finding new ways to treat
the disease.
CF Drug Development Pipeline
The CF Foundation has a drug development pipeline of potential
CF drugs that address all aspects of the disease. The CF Foundation
started the Therapeutics Development Program in the late 1990s. This
program gives scientists the financial support they need to participate
in CF drug development and also creates opportunities for biotech
companies to get involved in CF drug development.
Potential new drugs discovered through this work have shown
encouraging results in laboratories and in clinical trials of people with
CF. These results are very exciting, because some of these potential
drugs aim to help correct the underlying cause of CF, instead of
treating just the symptoms.
Clinical Trials
For CF treatments to be approved by the FDA, they have to be tested
for safety and effectiveness in people with CF. The CF Foundation has
an active network to help these studies be done more quickly. The
Therapeutics Development Network (TDN) is made up of CF care
centers that have training in doing clinical trials. The CF Foundation
THE CYSTIC FIBROSIS FOUNDATION • 147
has many resources to help people with CF learn more about
volunteering for a clinical trial, helping develop new treatments
and playing a role in the search for a cure.
To learn more about CF research, talk to your CF care center
team, visit www.cff.org/research or call 1-800-FIGHT CF.
Public Policy and Advocacy
The CF Foundation advocates on behalf of people
with CF at all levels of government. It urges
support for critical research, access to quality care
and increased awareness of CF among state and
federal lawmakers.
Bringing together a vast network across all 50
states, the CF Foundation connects people with
CF, families, friends and other volunteers with their
elected officials. The CF Foundation also supplies
volunteers with the tools they need to advocate on
behalf of people with CF.
The policy and advocacy goals of the CF
Foundation include:
• Moving Research Forward: Quickly move new treatments from research and development into the hands of people with CF by urging support for the national agencies engaged in drug development, such as the National Institutes of Health
(NIH) and the FDA.
• Improving Access: Secure and protect the right of people with CF to benefit from high-quality private and public health care programs, such as Medicaid, and work to decrease the financial burden of the disease.
Increasing Awareness: Educate elected officials about CF and the needs of the CF community. This educational outreach is done though the Congressional Cystic Fibrosis Caucus. You can learn more about the caucus at
www.cff.org/GetInvolved/Advocate/CFCaucus.
•
To help achieve these goals, the CF Foundation:
• Connects CF advocates with elected officials in Washington, D.C., and their home states.
Educates key members of Congress on issues important to the CF community.
•
• Supports an advocacy network of 90,000 people with CF and their families and friends to work with their elected officials on important issues.
Many people with CF and their
families work directly with their
elected officials on behalf of
people with CF.
148 • THE CYSTIC FIBROSIS FOUNDATION
• Helps CF care centers work with federal and state officials to support laws that benefit the CF community.
Works to help people with CF and their families get and keep insurance coverage.
•
Visit www.cff.org/GetInvolved/Advocate or call 1-800-FIGHT CF
to learn more.
CF Education
The CF Foundation works to increase knowledge about CF
for people with CF, their families, the public and medical
professionals. The CF Foundation has information for people
with CF, families and the general CF community, including an
e-newsletter, Connections.
These resources are available on the CF Foundation website at
www.cff.org and at CF Foundation-accredited CF care centers.
The CF Foundation’s educational webcasts bring the CF
community together in a virtual “CF Education Day” forum to
learn from the experts about living with CF and the latest in CF
research. The webcasts are on the CF Foundation website for
viewing at any time at www.cff.org/LivingWithCF/Webcasts.
The CF Foundation sponsors the annual North American
Cystic Fibrosis Conference (NACFC), an international medical
conference where scientists, researchers, doctors, nurses,
dietitians, respiratory and physical therapists, social workers
and other health care professionals gather to learn the latest in
research and practice. The CF Foundation also supports training
of health care professionals at CF care centers.
The CF Foundation also helps increase awareness and knowledge
of CF through social media channels such as Facebook, Twitter and
YouTube. Members of the CF Foundation’s online community
can connect with thousands of others who have CF or are
related to someone with CF, and can also learn more about CF
Foundation resources, programs and advocacy activities. Visit
www.cff.org to learn more.
See Appendix B for a listing of many educational resources, as
well as more information about the CF Foundation’s medical
care, research and public policy programs.
THE CYSTIC FIBROSIS FOUNDATION • 149
Local CF Foundation Chapters
The CF Foundation has about 75 chapters and branch offices
around the country, staffed by professionals who help raise funds
to support CF research and care. Each chapter has a group of
volunteers who help make the fundraising events, such as golf
tournaments, black-tie dinners and the annual national walk,
Great Strides, so successful. Chapters also offer opportunities for
volunteers to help spread awareness of CF and meet others in
the CF community. Parent volunteers often make friends and find
support with other parents who are dealing with CF.
Visit www.cff.org or call 1-800-FIGHT CF to find a chapter near
you.
Notes and Questions
Chapter 15: The Future and CF Research
The Cystic Fibrosis Foundation was formed in 1955 by a group
of parents who had children with cystic fibrosis (CF). At that time,
little was known about the disease and no effective treatments
were available. Since then, the CF Foundation has raised
hundreds of millions of dollars to support research to develop
new CF therapies. This research has led to great progress in
understanding and treating CF, which has made a huge
difference in the lives of those living with CF.
Today, many people with CF are living into adulthood, and
research to find a cure has never been more promising.
The CF Foundation and Research
The mission of the CF Foundation is to find a cure for CF and
improve the quality of life for people living with the disease.
Nearly every CF drug available today was made possible
because of the CF Foundation’s support and its ongoing work
with researchers to find a cure for CF.
The discovery of the CF gene in 1989 — the single most
important discovery in CF research — was the result of an
international research collaboration. With the CF gene in hand,
CF Foundation researchers for the first time could look at the
root cause of CF in their research efforts to find a potential cure.
In the 1990s, the CF Foundation created the Therapeutics
Development Network (TDN). The TDN was created to bridge
the gap between what has been learned in the laboratory and
the development of new therapies. The TDN is a nationwide
network of nearly 80 CF clinical research centers. These centers
specialize in conducting clinical trials to evaluate the safety and
effectiveness of new CF therapies.
CF Drug Development Pipeline
The CF Foundation created a way to measure the progress of
developing therapies, called a “drug development pipeline.”
To make it more likely that effective new therapies will become
available to people with CF, the CF Foundation makes sure that
several types of potential drugs are in development at the same time.
CF is a complex disease, and therapies must target problems
in different areas, such as the airways and the digestive system.
Different therapies also work on different issues, such as
infection, inflammation and thick mucus.
New treatments developed with
the support of the CF Foundation
have helped improve the health and
quality of life of people with CF.
152 • THE FUTURE AND CF RESEARCH
Some therapies focus on fixing the cause of CF, and some
therapies focus on better treatments for the problems CF causes.
The following are several areas of CF research currently in the CF
drug development pipeline.
CFTR Modulation
One way to fix the problems caused by the CF gene mutation
is to focus on the gene’s protein product, called the CF
Transmembrane conductance Regulator (or CFTR). People
without CF make healthy CFTR protein. This protein’s job in
certain cells is to make a tiny channel for salt and water to move
in and out of the cells (See “How Do Altered Genes Cause CF?,”
page 16). When this channel is defective, the balance of salt and
water is lost and the body makes thick, sticky mucus.
Researchers have been looking for and testing treatments that
will help the CFTR protein work in people with CF. This is called
CFTR “modulation” (meaning to adjust or change the CFTR
protein). Restoring the health of the CFTR protein targets the
underlying cause of CF and works at the most basic level of the
body.
In 2012, the U.S. Food and Drug Administration (FDA) approved
the CFTR modulator Kalydeco™ — the first drug to target the
underlying cause of CF — in people ages 6 and older with a
specific mutation of CF that is found in 4 percent of people with
CF in the United States. Research is in progress to discover and
develop additional drugs that will work in people with other
mutations of the CF gene.
Restore Airway Surface Liquid
In CF, changes in the balance of salt causes mucus to be
thick and sticky. Some research targets ways other than CFTR
modulation to improve the movement of salt in and out of
cells. This approach will help the mucus to become thinner and,
therefore, cleared more easily.
Mucus Alteration
Mucus alteration studies evaluate drugs for their effectiveness in
thinning and clearing thick mucus from the airways. For example,
Pulmozyme® is an inhaled medication that thins and loosens
mucus in the airways of people with CF. Approved in 1993,
Pulmozyme was the first new drug specifically developed for CF
in 30 years. Other methods to alter mucus are being studied.
Anti-inflammatory
Part of the damage to the lungs in people with CF is caused by
inflammation. Anti-inflammatory drugs are being studied to help
reduce inflammation in CF lungs, and thereby slow lung damage.
THE FUTURE AND CF RESEARCH • 153
Anti-infective
Anti-infectives are used to fight or control infections. They can
be used to fight chronic infections as well as exacerbations.
Researchers are searching for and testing anti-infectives that are
particularly useful against the types of bacteria that affect people
with CF, such as Pseudomonas aeruginosa. Researchers have
developed anti-infectives especially for people with CF that are
inhaled directly into the lungs. This way, they go straight to the
place they are needed to fight germs.
Transplantation
Lung transplants are serious and difficult procedures. But the
growing success rate has made them a treatment option for
some with severe lung disease. One area of research includes
ways to improve how well people with CF do after receiving a
lung transplant.
Nutrition
Good nutrition means better health for people with CF. Research
focusing on nutrition looks at better ways for people with CF to
absorb fats and proteins and, therefore, get more calories for
growth and energy. Different enzymes and specially formulated
nutritional supplements and vitamins are being researched.
For a current “snapshot” of therapies being studied, see
www.cff.org and click on Drug Development Pipeline in the
Quick Links or ask your CF care center.
CF Clinical Trials
A clinical trial is a health-related research study in
human beings. Clinical trials follow a very specific
study plan, which is called a “protocol.” Protocols are
carefully designed to protect the health of the person
in the research study, while answering a specific
research question. The research question is usually
to find out if a new treatment for people with CF
improves their health.
A protocol describes who can take part in the clinical
trial; the schedule of tests, procedures, medications
and dosages; and the length of the study. People
who participate in the clinical trial are seen regularly
by the research staff to monitor their health and
to determine the safety and effectiveness of their
treatment.
The clinical trial team includes doctors and nurses, and may
include other health care professionals such as respiratory
therapists. The research staff at CF care centers may be people
Ask your CF doctor or research
coordinator about a clinical trial you
or your child might join.
154 • THE FUTURE AND CF RESEARCH
that you have already met who are on the CF care team. They may
also be new to you if they work only in the research part of the center.
If you are thinking about joining a clinical trial, always think carefully
about what the benefits and risks of participating in a specific clinical
trial may be. Each specific clinical trial will have very specific risks and
benefits.
In general, benefits for the person participating in the study may
include:
•
Playing an active role in one’s own health care.
• Possibly gaining access to new research treatments before they are widely available. (Read about “placebos” later in this section.)
• Obtaining expert medical care at leading health care facilities during the trial.
•
Helping others by contributing to medical research.
There are always possible risks involved in clinical trials that people
must consider:
• There may be unpleasant, serious or even life-threatening side effects from the experimental treatment.
The experimental treatment may not be effective for the participant.
•
The protocol may require more of their time and attention than would a non-protocol treatment, including trips to the study site, more treatments, hospital stays or complex dosage requirements.
•
A placebo is an inactive pill, liquid or powder that has no medical
effect. In CF clinical trials, the new experimental treatments are
often compared with placebos. This is so the effectiveness of a new
treatment can be compared with not receiving the treatment. During
the study, the participant does not know whether he or she is receiving
the new treatment or placebo.
Before participating in a clinical trial, people should know as much
about the clinical trial as possible. The following questions might be
helpful for the person thinking about being in a study to discuss with
the health care team.
•
What is the purpose of the study?
•
Who is going to be in the study?
• Why do researchers believe the experimental treatment being tested may be effective?
•
Has it been tested before?
•
What kinds of tests and experimental treatments are involved?
How do the possible risks, side effects and benefits in the study compare with those of my current treatment?
•
THE FUTURE AND CF RESEARCH • 155
•
How might this trial affect my daily life?
•
How long will the trial last?
•
Will hospitalization be required?
•
Who will pay for the experimental treatment?
•
Will I be reimbursed for other expenses?
•
What type of long-term follow-up care is part of this study?
•
How will I know that the experimental treatment is working?
•
Will results of the trials be provided to me?
•
Who will be in charge of my care?
There are many opportunities to help develop new drugs for CF.
More potential therapies to treat CF are in development today
than ever before. That gives all of us great cause for hope, but it
also means more people with CF than ever before are needed to
participate in CF clinical trials.
If you are interested in learning more about clinical trials, check
with your CF care center. You may also learn more by going to
www.cff.org/research/ClinicalResearch/ and to
www.clinicaltrials.gov.
Remember
As the understanding of the science of CF increases, there is also
an exciting growth in the number of opportunities to discover
and develop new potentially lifesaving therapies. Today, there are
many trials underway studying an increasing number of potential
new treatments for CF. With the CF Foundation’s specialized
network of CF research centers that have the expertise to do
clinical trials, and the dedicated people with CF who want to
participate in clinical trials, the future for people living with this
disease has never been brighter.
Notes and Questions
PART IV
Resources
Appendix A: Medical Words to Know
A
Absorb/Absorption
The passage or uptake of substances into or across tissues, such
as the uptake of digested food and water from the intestines into
the bloodstream. Because of a lack of digestive enzymes in CF,
some foods eaten by people with CF may not be well absorbed
and used by the body. (Also see Digestive System, Enzyme,
Intestine, Malabsorption, Pancreas.)
Active Cycle of Breathing Technique (ACBT)
An airway clearance technique that involves using a set of
different breathing techniques to loosen and clear mucus. (Also
see Airway Clearance Techniques.)
ACTs
See Airway Clearance Techniques.
Aerosol
An inhaled medicine mist that treats lung problems. The aerosol
or mist is created by a nebulizer attached to an air compressor.
(Also see Air Compressor, Nebulizer.)
Air Compressor
A machine that blows out a high flow of air through a tube. (Also
see Aerosol, Nebulizer.)
Airway Clearance Techniques (ACTs)
Different methods to loosen thick, sticky mucus in the lungs
so it can be coughed or huffed out. (Also see Active Cycle
of Breathing Technique, Autogenic Drainage, Chest Physical
Therapy, Cough, High-Frequency Chest Wall Oscillation, Huffing,
Intrapulmonary Percussive Ventilation, Oscillating Positive
Expiratory Pressure, Percussion, Positive Expiratory Pressure
Therapy, Postural Drainage.)
Airways
Tubes that conduct outside air into the lungs. The lungs have
many airways of varied sizes. The largest, or central, airway is the
trachea. This branches into smaller airways called the bronchi.
These divide into even smaller branches called bronchioles that
end in alveoli. (Also see Alveoli, Bronchioles, Bronchus, Lower
Respiratory Tract, Respiratory System, Trachea, Upper Respiratory
Tract.)
160 • MEDICAL WORDS TO KNOW
Alveoli
The millions of tiny air sacs in the lungs at the ends of
bronchioles where oxygen is exchanged for carbon dioxide from
the blood. In CF, mucus clogs alveoli and interferes with oxygen–
carbon dioxide exchange. (Also see Airways, Lower Respiratory
Tract, Mucus, Respiratory System.)
Amniocentesis
A test used to find or “screen” for genetic defects in the fetus.
A hollow needle is put through the mother’s abdomen into the
uterus. A small amount of amniotic fluid around the fetus is taken
and tested. (Also see Amniotic Fluid, Chorionic Villus Sampling,
Fetus, Gene.)
Amniotic Fluid
The fluid around the fetus in the uterus. (Also see Amniocentesis,
Fetus.)
Antibiotic
A drug that can kill or slow the growth of bacteria, often used in
CF to treat lung infections. (Also see Bacteria.)
Antihistamine
A drug used to treat allergies that blocks histamine.
Anti-inflammatory
Reduces inflammation or swelling of body tissues. (Also see
Inflammation.)
Anus
The lower opening of the digestive system where stool leaves
the body. (Also see Digestive System, Stool.)
Autogenic Drainage (AD)
An airway clearance technique that involves using various airflows
to move mucus. (Also see Airway Clearance Techniques.)
Autosomal-Recessive
A genetic trait or disorder that appears only when a person
inherits a pair of chromosomes, each with the gene for the trait.
One chromosome of the pair comes from the father and the
other from the mother. Autosomal-recessive disorders occur only
if each parent either is a carrier of the trait or has the trait. CF is
autosomal-recessive. (Also see Base Pairs, Carrier, Chromosome,
Gene, Genetic, Inherited, Hereditary.)
MEDICAL WORDS TO KNOW • 161
B
Bacteria
Tiny one-celled organisms that are often the cause of infections.
People with CF are prone to bacterial lung infections (often
caused by Staphylococcus aureus or Pseudomonas aeruginosa).
However, some bacteria normally found in the body are helpful.
For example, Escherichia coli lives in the intestines and helps
with digestion. (Also see Antibiotic, Pseudomonas aeruginosa,
Intestine, Staphylococcus aureus.)
Base Pairs
Small building blocks that make up genes. (Also see AutosomalRecessive, Carrier, Chromosome, Gene, Genetic, Heredity,
Inherited.)
Basic Defect
In CF, the CFTR gene mutation makes faulty CFTR protein. (Also
see Cystic Fibrosis Transmembrane Conductance Regulator.)
Bile
A liver secretion that helps digestion. (Also see Digestion,
Secretion.)
Biotech
Short for biotechnology. Applying biological science to
technology, including drug development. (Also see Research.)
Blocked Intestine
The inability of food to move from the stomach through the
intestine and out of the body.
Blood Pressure
Force exerted by the heart pumping blood.
Bronchiectasis
Chronic condition when the bronchi of the lungs are stretched or
dilated beyond their normal dimensions. This leads to abnormal
breathing and a productive cough. This can occur in CF. (Also see
Bronchus, Chronic, Cough, Lower Respiratory Tract, Respiratory
System.)
Bronchioles
The smallest airways of the lungs leading from the bronchi to the
alveoli. (Also see Airways, Alveoli, Bronchus, Lower Respiratory
Tract, Respiratory System, Trachea.)
162 • MEDICAL WORDS TO KNOW
Bronchitis
An inflammation of the bronchi caused by infection or exposure
to cold or irritants. Symptoms include fever and cough. Many
people with CF often have bronchitis. (Also see Bronchus,
Symptom.)
Bronchodilator
Medicine that opens bronchial tubes for easier breathing and
to clear mucus, often given to relieve bronchospasm. (Also see
Bronchioles, Bronchus, Mucus.)
Bronchus/Bronchi
The large airways that move air from the trachea to the lungs.
The bronchi branch into smaller airways called bronchioles.
These lead to the alveoli. In CF, mucus clogs the bronchi
and interferes with breathing. (Also see Airways, Alveoli,
Bronchiectasis, Bronchioles, Lower Respiratory Tract, Mucus,
Respiratory System, Trachea.)
Buccal Smear
Cells brushed from the inside of the cheek. (Also see Cell.)
Burkholderia Cepacia Complex
A group of bacteria called B. cepacia for short. In CF, B. cepacia
can cause lung infection. B. cepacia may be spread from person
to person with CF.
C
Carrier
A person having a single gene for a genetic trait or disorder
such as CF. Carriers show no signs of the disease. In CF, each
parent of a child with CF either has CF or is a CF carrier. (Also see
Autosomal-Recessive, Gene, Genetic, Heredity, Inherited.)
Cell
The basic unit of living organisms.
Cervical
Having to do with the cervix. (Also see Cervix, Reproductive
System.)
Cervix
The opening of the uterus. (Also see Uterus, Reproductive
System.)
CFRD
See Cystic Fibrosis–Related Diabetes.
MEDICAL WORDS TO KNOW • 163
CFTR
See Cystic Fibrosis Transmembrane Conductance Regulator.
Chest Physical Therapy (Chest PT or CPT)
An airway clearance technique that often includes both postural
drainage and percussion. Helps to loosen mucus in the lungs.
(Also see Airway Clearance Techniques, Percussion, Postural
Drainage.)
Chorionic Villus Sampling
A test to find gene defects in the fetus. A small piece of placenta
is taken and tested early in pregnancy. A thin tube is put through
the mother’s vagina and cervix, or a thin needle is inserted
through the abdomen into the uterus. (Also see Amniocentesis,
Fetus, Placenta.)
Chromosome
The threadlike material that carries genes, the units of heredity.
Chromosomes are in the nucleus of every living cell. Every
person should have 23 pairs of chromosomes in each cell. (Also
see Cell, Gene, Genetic, Inherited, Heredity.)
Chronic
A disease or condition that lasts and is persistent — not acute,
which is characterized by a sudden onset, sharp rise and short
course. CF is a chronic disease.
Cilia
Hairlike structures found on the surface of many cells in the
body. In the respiratory system, cilia line the airways. They move
together to push mucus to the trachea (windpipe) where it
can be coughed up or huffed out or swallowed. Thick mucus,
infection, cigarette smoke and other irritants can slow cilia and
hinder this natural cleaning mechanism. (Also see Airways,
Cough, Huffing, Mucus, Respiratory System, Tissue, Trachea.)
Cirrhosis
Scarring of the liver; this can be caused by many diseases.
Occurs in about 1 percent of people with CF. This is caused
by thick secretions blocking the bile ducts. (Also see Duct,
Secretion.)
Clubbing
Rounded, enlarged tips of the fingers and toes. Clubbing often
represents a chronic shortage of oxygen in the blood. Occurs
in CF, congenital heart disease and other heart, lung and
gastrointestinal diseases. (Also see Chronic, Sign.)
164 • MEDICAL WORDS TO KNOW
Compressor
See Air Compressor.
Conception
When a man’s sperm joins with a woman’s egg to form an
embryo. (Also see Embryo, Fertilization, Fetus.)
Contagious
Able to be spread from person to person, like an illness. CF is
not contagious!
Contraception
Methods used to prevent pregnancy.
Cor Pulmonale
Enlargement of the right side of the heart. This happens when the
heart has to work harder to get blood through the lungs. It can
lead to right-sided heart failure. Cor pulmonale can occur in CF.
Cough/Coughing
A normal way for the body to clear the respiratory system of
irritating and harmful things such as smoke, gases, dust and
increased mucus. (Also see Respiratory System, Mucus.)
Culture Medium
What sputum is put on for germs to grow in the laboratory, so
the germ can be identified. (Also see Sputum.)
Cystic Fibrosis–Related Diabetes (CFRD)
A form of diabetes that can occur in CF. Diabetes is a problem in
which a person’s blood glucose (a type of sugar) level is too high.
(Also see Diabetes, Glucose.)
Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)
A protein that makes the channel in the cell where chloride
moves in and out.
D
Debris
Remains of something. For inhaled medicine, debris could be
medication or sputum.
Dehydrate/Dehydration
Loss of too much water.
MEDICAL WORDS TO KNOW • 165
Diabetes (Diabetes Mellitus)
A pancreatic problem causing abnormal insulin production.
Diabetes affects how the body uses sugar and other foods. It is
treated by diet changes (less sugar intake) and insulin. (Also see
Pancreas, Cystic Fibrosis–Related Diabetes.)
Diagnose
To find the cause of health problems.
Diaphragm
The main breathing muscle — a dome-shaped muscle between
the chest and abdomen. People with CF may learn to use the
diaphragm to cough better or make breathing easier.
Diarrhea
Frequent, loose stools. (Also see Stool.)
Digestion/Digest
The process of breaking down the food we eat and absorbing
its nutrients into the body for energy. (Also see Absorption,
Digestive System.)
Digestive System
The organs that take in, digest and get rid of food. Includes the
mouth, salivary glands, throat (pharynx), esophagus, stomach,
intestines, liver, pancreas, colon, rectum and anus. In CF, thick
mucus blocks some passages in the digestive system, including
the passage between the pancreas and intestines. (Also see
Anus, Digestion, Duct, Esophagus, Intestine, Mucus, Pancreas,
Rectum.)
DIOS
See Distal Intestinal Obstructive Syndrome.
Direct Contact Transmission
Spreading germs by touching someone’s body.
Disinfect
To kill most of the germs.
Distal Intestinal Obstructive Syndrome (DIOS)
Partially or completely blocked intestine by abnormal stool.
Occurs in older infants, children and adults with CF. (Also see
Intestine, Meconium, Meconium Ileus Equivalent.)
Diuretics
Medicines that help remove excess fluid from the body.
166 • MEDICAL WORDS TO KNOW
DNA
Deoxyribonucleic acid. The chemical coding for a gene.
DNA decides the “genetic message” in each cell, organ and
organism. (Also see Cell, Chromosome, Gene, Genetic, Inherited,
Heredity.)
Droplet
A tiny drop of liquid from the mouth, nose or lungs.
Dry Powder Inhaler (DPI)
A device that delivers a very fine powder of medicine that a
person breathes in.
Duct
A tube or passageway for secretions. Ducts are found in organs,
organ systems and exocrine glands. In CF, thick mucus can
block these ducts. (Also see Exocrine Glands, Mucus, Pancreas,
Secretion.)
E
Egg
A cell from the woman containing half of the chromosomes
needed to form an embryo. When it unites with a sperm from the
man, which also contains half of the chromosomes, an embryo
is created. (Also see Cell, Chromosome, Embryo, Reproductive
System.)
Embryo
An unborn child, from conception to 3 months after conception.
(Also see Conception, Fertilize, Fetus, In Vitro Fertilization.)
Endocrine Glands
Ductless glands that make hormones that pass into the blood.
Includes pituitary, adrenal and thyroid glands. These are not
affected in CF. (Also see Duct, Hormone.)
Endocrinologist
A doctor who specializes in treating the endocrine system or
endocrine glands. (Also see Endocrine Glands.)
Enzymes
Proteins that help make and increase certain chemical processes
in the body, such as the breaking down of foods in digestion.
Because people with CF have mucus that often blocks the
passageways through which digestive enzymes from the
pancreas flow, they may need enzyme replacements to digest
food. (Also see Absorption, Digestive System, Mucus, Pancreas.)
MEDICAL WORDS TO KNOW • 167
Epididymis
Part of the male reproductive system — a tube that stores and
carries sperm from the testes to the vas deferens. (Also see
Microsurgical Epididymal Sperm Aspiration, Percutaneous
Epididymal Sperm Aspiration, Reproductive System, Testes, Vas
Deferens.)
Esophagus
The tube that leads from the throat (pharynx) to the stomach.
(Also see Digestive System.)
Evidence-Based
The information is based on strong and reliable data from
research.
Exacerbation
Signs and symptoms that show a need for treatment. (Also see
Sign, Symptom.)
Exhaling
Breathing out; the flow of air out of the lungs with each breath.
Exocrine Glands
Glands that normally make thin, slippery secretions including
sweat, mucus, tears, saliva and enzymes. These secretions move
through ducts (small tubes) to the surface of the body or into
hollow organs, such as intestines or airways. CF affects these
glands. Their ducts can be blocked by mucus. (Also see Airways,
Duct, Intestine, Mucus, Secretion.)
F
Fallopian Tubes
The tubes that carry the egg from the ovaries to the uterus. (Also
see Egg, Ovaries, Reproductive System, Uterus.)
False-Negative
Test result that incorrectly shows the absence of a disease or
condition. A false-negative sweat test would show that the
person does not have CF when they actually do have CF. (Also
see Sweat Test.)
False-Positive
Test result that incorrectly shows the presence of a disease or
condition. A false-positive sweat test would show that the person
has CF when they do not have CF. (Also see Sweat Test.)
168 • MEDICAL WORDS TO KNOW
Fertile/Fertility
The ability to conceive and have children. (Also see Conception,
Fertilize.)
Fertilize/Fertilization
When a man’s sperm unites with a woman’s egg to form
an embryo. (Also see Conception, Embryo, Fetus, In Vitro
Fertilization.)
Fetus
An unborn child, from 3 months after conception to birth. (Also
see Conception, Embryo.)
G
Gallbladder
The gallbladder is a small organ attached to the liver and is part
of the gastrointestinal tract. The gallbladder stores extra bile the
liver produces. Bile fluid helps digest food in the intestines.
Gallstone
A stonelike mass in the gallbladder or bile duct. (Also see Duct.)
Gastroenterologist
A doctor who specializes in treating the digestive system. (Also
see Digestive System, Gastrointestinal.)
Gastrointestinal (GI)
Pertaining to the stomach and intestine, or digestive system.
(Also see Digestive System, Intestine.)
Gene
The main unit of heredity. Each chromosome carries hundreds
of genes. Genes decide body traits such as eye and hair color,
height and facial features and also many health problems.
CF is caused by a mutation of a gene. A child inherits CF at
conception, when two CF genes are received, one from each
parent. (Also see Autosomal-Recessive, Base Pairs, Carrier,
Chromosome, Genetic, Hereditary, Inherited, Mutation.)
Genetic
Hereditary or inherited. (Also see Autosomal-Recessive, Base
Pairs, Carrier, Chromosome, Gene, Hereditary, Inherited.)
Germs
Organisms that can cause infection. This includes bacteria and
viruses. (Also see Bacteria, Virus.)
MEDICAL WORDS TO KNOW • 169
GI
See Gastrointestinal.
Glucose
A type of sugar that is found in many foods, and an important
nutrient used by all cells for energy. Blood glucose levels are
high if a person has diabetes. (Also see Cystic Fibrosis–Related
Diabetes, Diabetes.)
Gynecologist
A doctor who specializes in treating the female reproductive
system. (Also see Reproductive System.)
H
Hand Hygiene
A general term for cleaning hands; this includes the use of soap
and water or an alcohol-based hand gel.
Heat Stroke
A health problem that occurs when the body gets too hot. The
signs are weakness, nausea, dizziness and profuse sweating. It
can occur when it is hot outside or in an extra-warm room.
Hemoptysis
Coughing up blood, often with sputum, because of broken small
blood vessels in the lungs. Can occur in CF. (Also see Sputum.)
Hereditary/Heredity
Traits or conditions that are genetically passed from parents
to their children. (Also see Autosomal-Recessive, Base Pairs,
Carrier, Chromosome, Gene, Genetic, Inherited.)
Heterozygous
Someone who has inherited two different genes for a trait or
disease. A person who is a heterozygote for CF has two different
mutations in the genes for CF. (Also see Autosomal-Recessive,
Base Pairs, Carrier, Chromosome, Gene, Genetic, Hereditary,
Inherited, Mutation.)
High-Frequency Chest Wall Oscillation
An airway clearance technique that involves wearing an inflatable
vest attached to a machine that vibrates rapidly to loosen mucus
in the lungs. (Also see Airway Clearance Technique, Mucus.)
170 • MEDICAL WORDS TO KNOW
Homozygous
Someone who has inherited two of the same genes for a certain
trait or disease. Someone with CF who is homozygous for CF has
two of the same mutation of the CF gene. (Also see AutosomalRecessive, Base Pairs, Carrier, Chromosome, Gene, Genetic,
Hereditary, Inherited, Mutation.)
Hormone
Secretion from the endocrine glands. Hormones regulate body
functions including growth, maturation and heart rate. (Also see
Endocrine Glands, Secretion.)
Huffing/Huff
An airway clearance technique using a gentle type of cough.
Huffing is done by tightening the stomach muscles while
forcefully pushing air out with the mouth open. It is like what we
do when we “huff” onto a mirror or window to fog it up. (Also
see Airway Clearance Techniques, Cough.)
I
ICSI
See Intracytoplasmic Sperm Injection.
IDEA
See Individuals with Disabilities Education Act.
IEP
See Individualized Education Plan.
Immunization
Vaccination or “shot” to help the body build a defense against
an illness. (Also see Vaccination.)
Immunoreactive Trypsinogen (IRT) Test
A blood test used 2 or 3 days after birth that may be done with
DNA tests to find out if a baby has CF. Immunoreactive
trypsinogen, or IRT, is a chemical normally found in small amounts
in the body. People with CF tend to have high levels of IRT.
Indirect Contact Transmission
Spreading germs by touching something that another person
has touched (such as a doorknob or cup) with any part of the
body.
MEDICAL WORDS TO KNOW • 171
Individualized Education Plan (IEP)
A plan between a child’s family and a school to help meet the
child’s learning needs. The plan tells how the school will manage
things such as absences or medical treatments at school, such as
taking enzymes.
Individuals with Disabilities Education Act (IDEA)
A federal law that makes public elementary and high schools
give free and appropriate education to children with disabilities.
Infertile/Infertility
Unable to get pregnant (female), or to cause pregnancy (male).
Inflammation/Inflammatory
The swelling of body tissues because of irritation or injury.
Inflammation occurs with an infection.
Inhalation
Breathing in; the flow of air into the lungs.
Inherit/Inherited
Traits or conditions that are genetically passed from parents to
their children. (Also see Autosomal-Recessive, Base Pairs, Carrier,
Chromosome, Gene, Genetic, Heredity.)
Insulin
A hormone, made by the pancreas, that helps sugar move from
the blood into the cells. Sugar is fuel for the body to grow and
fight infection.
Intestinal
Having to do with the intestine. (Also see Digestive System,
Gastrointestinal, Intestine.)
Intestine
Tube in the digestive system that connects the stomach to the
anus. The long, narrow, upper part is the small intestine. The
short, wide, lower part is the large intestine. (Also see Anus,
Digestive System.)
Intracytoplasmic Sperm Injection (ICIS)
Fertilization of an egg by injecting a sperm into it during the
process of in vitro fertilization. (Also see Egg, Fertilization, In Vitro
Fertilization, Sperm.)
Intrapulmonary Percussive Ventilation (IPV)
An airway clearance technique that involves breathing through a
device that creates a rapid vibration to loosen the mucus in the
lungs. (Also see Airway Clearance Technique, Mucus.)
172 • MEDICAL WORDS TO KNOW
Intravenous (IV)
Putting a medicine right into a blood vessel, usually a vein, using
a thin needle and a tube.
In Vitro Fertilization
Mixing sperm with an egg outside the woman’s body. The
fertilized egg is then put into the woman’s uterus. (Also see
Conception, Egg, Embryo, Fertilization, Sperm, Uterus.)
Ion Transport
The movement of sodium and chloride in and out of the cell is
one type of ion transport.
IRT
See Immunoreactive Trypsinogen Test.
IV
See Intravenous.
L
Legislative
Having to do with making laws.
Lower Respiratory Tract
The airways and the lungs (trachea, bronchi, bronchioles and
alveoli). (Also see Alveoli, Bronchioles, Bronchus, Respiratory
System, Trachea.)
M
Malabsorption
Poor uptake of nutrients from food for use by the body. In
CF, mucus may plug ducts of digestive organs and block the
secretion of enzymes and hormones. This makes many nutrients
unavailable for use in body maintenance and growth. (Also
see Absorption, Digestion, Digestive System, Duct, Enzyme,
Hormone, Mucus, Pancreas, Secretion.)
MDI
See Metered Dose Inhaler.
Meconium
The first newborn stool, often passed within a few hours
after birth. It contains mucus and other secretions. (Also see
Meconium Ileus, Mucus, Secretion.)
MEDICAL WORDS TO KNOW • 173
Meconium Ileus
Blockage of the intestines of a newborn with very thick
meconium. It can be the earliest symptom of CF, and occurs in
7 percent to 10 percent of people with CF. (Also see Intestine,
Meconium, Symptom.)
Meconium Ileus Equivalent (Distal Intestinal Obstructive Syndrome [DIOS])
Partially or completely blocked intestine by abnormal stool. It
occurs in older infants, children and adults with CF. (Also see
Distal Intestinal Obstructive Syndrome, Intestine, Meconium,
Meconium Ileus.)
MESA
See Microepididymal Sperm Aspiration.
Metered Dose Inhaler (MDI)
A device that helps a person to measure and inhale a certain
amount of medicine.
Microepididymal Sperm Aspiration (MESA)
An outpatient surgical technique in which sperm are taken
directly from a man’s epididymis. For men with CF who are
infertile, this method may help them conceive a biological
child when combined with in vitro fertilization (and usually
intracytoplasmic sperm injection). (Also see Epididymis,
Intracytoplasmic Sperm Injection, In Vitro Fertilization,
Percutaneous Epididymal Sperm Aspiration, Testicular Sperm
Extraction.)
Mucolytics
Medicines that thin mucus, making it easier to cough out. (Also
see Mucus.)
Mucous Membrane
Tissue that contains mucus-making glands. Mucous membranes
are found in the nose, mouth, lungs, esophagus, stomach and
intestines. (Also see Esophagus, Intestine, Mucus, Tissue.)
Mucous Plugs
Thick mucus in a duct or airway that can block the flow of
secretions or air. (Also see Airway, Duct, Mucus, Pancreas,
Secretion.)
Mucus
A fluid made by mucous membranes and glands. Mucus is
normally thin and slippery. In CF, the mucus is often thick and
sticky. (Also see Mucous Membrane, Phlegm, Sputum.)
174 • MEDICAL WORDS TO KNOW
Mutation(s)
A change in a gene that lasts forever. (Also see AutosomalRecessive, Base Pairs, Carrier, Chromosome, Gene, Genetic,
Hereditary, Inherited.)
N
Nasal Polyps
Small growths of swollen mucous membrane that project into
the nasal passages. They are common in people with CF, and
are often multiple or recurrent. Nasal polyps can be surgically
removed. (Also see Mucous Membrane.)
Nebulizer
A device that makes and delivers a medicine mist when attached
to an air compressor. (Also see Aerosol, Air Compressor,
Antibiotics, Mucolytics.)
Nervous System
The part of the body that includes the brain, spinal cord and
nerves.
Nucleus
The center or “brain” of the cell, containing the chromosomes.
(Also see Cell, Chromosome.)
O
Oral Glucose Tolerance Test (OGTT)
A test to diagnose diabetes or impaired glucose tolerance. It
is usually done after a person has had nothing to eat or drink
for 12 hours. Blood samples are taken before and up to 2 hours
after drinking a set amount of glucose. (Also see Cystic Fibrosis–
Related Diabetes, Diabetes, Glucose)
Oscillating Positive Expiratory Pressure (Oscillating PEP)
An airway clearance technique that involves breathing out
through a device many times in a row to loosen mucus in the
lungs. (Also see Airway Clearance Technique, Mucus.)
Osteopenia
When bones have fewer minerals and are weak.
Osteoporosis
When bones are less thick or dense and are weak. People with
osteoporosis have a higher risk for bone breaks (fractures).
MEDICAL WORDS TO KNOW • 175
Ovaries
The female organs where hormones and eggs are made. (Also
see Hormone, Egg, Reproductive System.)
Ovulate
To release an egg into the fallopian tube. (Also see Egg,
Fallopian Tube, Ovaries, Reproductive System.)
P
Pancreas
A long organ with glands found behind the stomach. The
pancreas secretes enzymes through ducts into the intestine to
break down food. In CF, mucus may obstruct the pancreatic
ducts, preventing digestion. Another part of the pancreas has
endocrine tissue, which makes the hormone insulin. Insulin
controls storage and oxidation of sugar. (Also see Cystic Fibrosis–
Related Diabetes, Digestive System, Digestion, Duct, Endocrine
Glands, Enzyme, Exocrine Glands, Hormone, Intestine, Mucus,
Tissue.)
Pancreatic
Having to do with the pancreas. (Also see Pancreas.)
Penis
The male organ through which urine and semen leave the body.
(Also see Reproductive System, Semen.)
PEP
See Positive Expiratory Pressure.
Percussion
An airway clearance technique that involves clapping, with a
cupped hand, and vibrating the chest to loosen mucus in the
lungs. (Also see Airway Clearance Technique, Chest Physical
Therapy, Mucus.)
Percutaneous Epididymal Sperm Aspiration (PESA)
An outpatient surgical technique in which sperm are taken
directly from a man’s epididymis. For men with CF who are
infertile, this method may help them conceive a biological
child when combined with in vitro fertilization (and usually
intracytoplasmic sperm injection). (Also see Epididymis,
Intracytoplasmic Sperm Injection, In Vitro Fertilization,
Microepididymal Sperm Aspiration, Testicular Sperm Extraction.)
PESA
See Percutaneous Epididymal Sperm Aspiration.
176 • MEDICAL WORDS TO KNOW
PFTs
See Pulmonary Function Tests.
Phlegm
Mucus made from glands in the lungs and airways. (Also see
Airways, Mucus, Secretion, Sputum.)
Pilocarpine Iontophoresis
A test that causes sweating by putting the chemical pilocarpine
on a small area of the skin, which then receives a small electrical
current. Quantitative pilocarpine iontophoresis is the usual way
to do a sweat test to diagnose CF. (Also see Sweat Test.)
Placenta
What an unborn child gets oxygen and nutrition from through
the mother.
Pneumonia
An inflammation of the lungs often caused by a bacterial or viral
infection. Pneumonia is a problem in people with CF. (Also see
Bacteria, Virus.)
Pneumothorax
A sudden, partial or complete lung collapse caused by a break
in lung tissue or an airway, which lets air escape from the lung
and get trapped between the lung and chest wall. It can occur in
people with CF.
Positive Expiratory Pressure (PEP) Therapy
An airway clearance technique that involves breathing through
a mouthpiece or mask connected to a prescribed resistor. (Also
see Airway Clearance Technique.)
Postural Drainage (PD or Bronchial Drainage)
An airway clearance technique that involves lying in various
positions to drain mucus from the lungs. (Also see Airway
Clearance Technique, Chest Physical Therapy, Mucus, Percussion.)
Prenatal/Prenatally
Refers to the time between conception and birth. (Also see
Conception.)
Primary Care Provider
Also referred to as a PCP. Usually a primary care doctor (a family
doctor or pediatrician) who sees patients on a regular basis for
routine care, such as immunizations and well-child visits; common
illnesses or problems, such as ear infections and rashes; and
sports or school physicals. A PCP may also be a physician assistant
or nurse practitioner. A PCP arranges referrals to specialists.
MEDICAL WORDS TO KNOW • 177
Prostate Gland
A gland in the male body that makes semen. (Also see
Reproductive System, Semen.)
Pseudomonas aeruginosa
A type of bacteria that often lives in the lungs of people with CF
and causes lung infections. (Also see Antibiotic, Bacteria.)
Pulmonary
Relating to the lungs.
Pulmonary Function Tests (PFTs)
Tests to check lung function. Along with patient history and
examination, PFTs help doctors diagnose, plan therapy and
determine response to therapy. They can be used with children
aged 5 years and older. PFTs measure air flow and lung volumes.
Pulmonologist
A doctor who specializes in treating the respiratory system. (Also
see Respiratory System.)
R
Rectal Prolapse
When the inner lining of the rectum comes out (prolapses)
through the anus. This may occur in children with CF because
of digestion problems. CF is the most common cause of rectal
prolapse in infants and children in the United States. (Also see
Anus, Digestion, Rectum.)
Rectum
The last part of the large intestine joining the colon to the anus.
(Also see Anus, Digestive System, Intestine.)
Reproductive System
In the male, includes the testes, sperm, vas deferens, prostate
gland, semen, urethra, scrotum and penis. In the female, includes
the eggs, ovaries, fallopian tubes, uterus, cervix and vagina. (Also
see Cervix, Egg, Fallopian Tubes, Ovaries, Penis, Prostate Gland,
Semen, Sperm, Testes, Uterus, Vagina, Vas Deferens.)
Research
• Applied Research: Studies that apply basic research findings to problems including diseases and symptoms. Creating new respiratory equipment or studying cell defects in the sweat glands of people with CF are applied research. (Also see Cell, Symptom.)
178 • MEDICAL WORDS TO KNOW
• Basic Science Research: Studies that increase knowledge of basic life processes. To learn more about CF, scientists do basic science studies such as gene studies and research on how cells work. (Also see Cell, Gene, Genetic.)
Clinical Research: Studies that improve diagnosis and treatment. Studies on medicines, lung function testing, nutrition and sweat testing methods are examples. (Also see Diagnose, Pulmonary Function Tests, Sweat Test.)
•
Respiratory Syncytial Virus (RSV)
A virus that can cause severe respiratory infections, especially in
young children and the elderly.
Respiratory System
The part of the body that includes all structures that air moves
through while breathing. Also includes the pleura, ribs and
intercostal muscles that support these structures. The upper
respiratory tract includes the nose, sinuses, throat (pharynx
and larynx). The lower respiratory tract includes the trachea,
bronchus, bronchioles and alveoli. In CF, thick mucus clogs
parts of the respiratory system. (Also see Trachea, Bronchus,
Bronchioles, Alveoli, Sinuses.)
S
Saline
A mixture of salt and water similar to the body’s normal tissue
fluids. (Also see Tissue.)
Secretion
A product of a gland, such as sweat or saliva. (Also see Endocrine
Gland, Exocrine Gland.)
Section 504 of the Rehabilitation Act of 1973
A federal law that prohibits discrimination against a person
because of a disability by any group that gets federal funds. This
law applies to day care and the public and private schools that
get federal funds.
Semen
A sticky, white fluid of the male reproductive system that contains
the sperm. (Also see Prostate Gland, Reproductive System,
Sperm.)
Sexually Transmitted Diseases (STDs)
Diseases that can be caught through sexual contact.
Sibling(s)
Brothers and sisters.
MEDICAL WORDS TO KNOW • 179
Sign
The clues of an illness or problem that you can see or measure,
such as fingertip clubbing, nasal polyps or rectal prolapse. (Also
see Clubbing, Nasal Polyps, Rectal Prolapse, Symptom.)
Sinuses
The air spaces in the bones of the skull, mostly connected with
the nose. (Also see Respiratory System, Sinusitis.)
Sinusitis
An inflammation of the lining of the sinuses that causes fluid to
drain into the nasal cavity. (Also see Sinuses, Respiratory System.)
Sperm
A cell from the male containing half of the chromosomes needed
to form an embryo. When it unites with an egg from the woman,
which also contains half of the chromosomes, an embryo is
created. (Also see Cell, Chromosome, Embryo, Reproductive
System.)
Sputum
Mucus or phlegm coughed up from lungs. (Also see Mucus,
Phlegm.)
Sputum Culture
A test to see what germs are growing in the sputum.
Staphylococcus aureus (Staph)
A type of bacteria that can cause infections. In CF, staph often
causes lung infections. It is treated with antibiotics. (Also see
Antibiotic, Bacteria.)
Sterile/Sterility
The absence of sperm, the male sex cell. Men with CF are usually
not sterile, but are very often infertile. (Also see Infertile/Infertility,
Sperm.)
Stool
Another word for bowel movement, poop or feces.
Sweat Test
The test to diagnose CF. Measures the salt (sodium and chloride)
in sweat. (Also see Diagnose, Pilocarpine Iontophoresis.)
Symptom
A sign of a person’s disease or condition. Something felt by
the person. CF symptoms include persistent cough; wheeze;
shortness of breath; bulky, very foul-smelling stools; and
stomachache. (Also see Cough, Sign, Wheeze.)
180 • MEDICAL WORDS TO KNOW
T
TESE
See Testicular Sperm Extraction.
Testes
The round organs in the scrotum where hormones and sperm
are made. Sometimes called testicles. (Also see Hormone,
Reproductive System, Sperm.)
Testicular Sperm Extraction (TESE)
An outpatient surgical technique in which sperm are taken
directly from a man’s testes. For men with CF who are infertile,
this method may help them conceive a biological child when
combined with in vitro fertilization (and usually intracytoplasmic
sperm injection). (Also see Intracytoplasmic Sperm Injection,
In Vitro Fertilization, Microepididymal Sperm Aspiration,
Percutaneous Epididymal Sperm Aspiration, Testes.)
Tissue
A group of cells of a similar type and function. (Also see Cell.)
Trachea
The windpipe, or largest central airway. Connects the upper
respiratory tract to the lower respiratory tract. (Also see Airways,
Lower Respiratory Tract, Respiratory System, Upper Respiratory
Tract.)
U
Upper Respiratory Tract
The nose, sinuses, throat (pharynx and larynx). (Also see
Respiratory System, Sinuses.)
Uterus
A muscle sac (organ), also called the womb, where the baby
grows until birth. (Also see Reproductive System.)
V
Vaccination
Immunization or “shot” to help the body build a defense against
an illness. (Also see Immunization.)
Vagina
In women, the canal that leads from the uterus to the outside of
the body. (Also see Reproductive System, Uterus.)
MEDICAL WORDS TO KNOW • 181
Vas Deferens
A duct in the male reproductive system, which carries sperm from
the testes to the prostate gland. In males with CF, this duct is
often blocked, leading to sterility. (Also see Duct, Prostate Gland,
Reproductive System, Sperm, Testes.)
Virus
An organism, smaller than bacteria, which causes infections
such as influenza, viral pneumonia, colds and hepatitis. (Also see
Bacteria, Pneumonia.)
Vitamins
Substances in foods needed for the body to function normally.
When digestive enzymes are blocked in CF, vitamins from foods
may be poorly absorbed by the body, so vitamin supplements
are needed. (Also see Absorption, Digestive System,
Malabsorption, Pancreas.)
W
Wheeze/Wheezing
To breathe hard and with a whistling sound.
Appendix B: Resource List
In addition to the resources listed here, your cystic fibrosis (CF)
care center has many resources available to share with you.
Also, the Cystic Fibrosis Foundation’s website, www.cff.org, has
a wealth of fact sheets, answers to frequently asked questions,
research updates and much more on a wide variety of CF-related
topics and the latest breaking CF-related news.
If you choose to surf the Web for information related to CF,
keep in mind that anyone can put information on the Internet.
Although there is some good information online, there is also
a great deal of information that is not correct. For tips on using
the Internet to research health information, read the “Guide to
‘Healthy’ Web Surfing: Ways to Evaluate the Quality of Health
Information on Web Sites” available from your CF care center or
at www.cff.org.
Cystic Fibrosis Foundation:
www.cff.org
info@cff.org
1-800-FIGHT CF or 301-951-4422
6931 Arlington Road
Bethesda, MD 20814
The CF Foundation’s website has information for adults and
children with CF as well as parents and families related to CF
care, CF care centers, the Patient Registry Report, advocacy,
insurance and more.
Book:
Orenstein DM. Cystic Fibrosis: A Guide for Patient and Family,
4th ed., New York, NY: Lippincott Williams & Wilkins; 2012.
Support Groups:
Contact your local CF care center. (See Appendix E)
184 • RESOURCE LIST
Cystic Fibrosis Family Education Program Publications:
The following resources are available from your local CF care
center.
• Beginning
CF Care
• Managing
Nutrition & Digestive Problems
• Managing
Lung & Other Respiratory Problems
• Becoming
a CF Manager
• Working
With Your Child
• Lisa and Jason: Family, Friends, and Everyday Life: For Young Children With Cystic Fibrosis and Their Families (stories and activities for young children)
CF Roundtable:
www.cfroundtable.com
248-349-4553
cfroundtable@usacfa.org
USACFA
PO Box 1618
Gresham, OR 97030
Newsletter for adults with CF from the United States Adult Cystic
Fibrosis Association Inc. (USACFA).
MedlinePlus:
www.medlineplus.gov
MedlinePlus is the National Institutes of Health's website for
patients and their families and friends. Produced by the National
Library of Medicine, it provides information about diseases,
conditions and wellness issues. MedlinePlus offers free, reliable,
up-to-date health information.
National Institutes of Health:
www.clinicaltrials.gov
Website to find clinical trials.
Centers for Disease Control and Prevention (CDC):
www.cdc.gov
The CDC works to create the information and tools that people
and communities need to protect their health — through health
promotion; prevention of disease, injury and disability; and more.
American Lung Association:
www.lungusa.org
1-800-548-8252
RESOURCE LIST • 185
Medicare:
www.medicare.gov
1-800-MEDICARE
Medicare provides health care coverage for people with longterm disabilities (and most Americans ages 65 years and older).
Social Security Disability Insurance (SSDI):
www.ssa.gov/disability
1-800-772-1213
The SSDI program provides income to people who are no longer
able to work because of a disability; benefits are based on a
person’s work history. People who receive SSDI are eligible for
Medicare after 2 years.
State Children’s Health Insurance Program (SCHIP):
www.insurekidsnow.gov
1-877-KIDS-NOW (1-877-543-7669)
SCHIP provides health care coverage to uninsured children in
families with incomes that are too high to qualify for Medicaid.
Supplemental Security Income (SSI):
www.ssa.gov/pubs/11000.html
1-800-772-1213
The SSI program provides a monthly stipend to people with
disabilities (and low-income Americans ages 65 years and older)
to help meet basic needs for food, shelter and clothing. People
who receive SSI are often eligible for Medicaid.
CF Services Pharmacy Inc.:
www.cfservicespharmacy.com
1-800-541-4959
Cystic Fibrosis Services Inc. is a Walgreens alliance partner
and is affiliated with the CF Foundation. A national mailorder pharmacy, CF Services is the nation’s leading provider
of specialty CF drugs, case management services and
reimbursement support for people with CF.
CF Patient Assistance Foundation:
www.cfpaf.org
1-888-315-4154
A nonprofit subsidiary of the CF Foundation, offers financial
assistance for CF medications and devices by providing copayment and co-insurance assistance for eligible medications
and devices, insurance benefit verification, reimbursement
support, case management, patient advocacy and referrals to
alternative assistance programs.
186 • RESOURCE LIST
CF Legal Information Hotline:
www.cff.org
CFLegal@cff.org
1-800-622-0385
The Hotline provides free information about the laws that protect
the rights of people with CF. It serves as a resource for CF care
centers, people with CF and their families.
Health care and insurance information:
www.healthcare.gov
From the U.S. Department of Health and Human Services.
State Insurance Commissioners:
www.naic.org
1-816-783-8500
For information on health insurance or filing a complaint, visit the
National Association of Insurance Commissioners.
Organ Donation and Transplantation:
www.organdonor.gov
U.S. Department of Health and Human Services.
College Scholarships:
• The Cystic Fibrosis Scholarship Foundation:
www.cfscholarship.org
• CFCareForward Scholarship:
www.CFCareForwardScholarship.com
• The Elizabeth Nash Foundation Scholarship Program:
www.elizabethnashfoundation.org
Federal Student Aid Information Center, U.S. Department of Education, ”The Student Guide":
www.studentaid.ed.gov
1-800-4-FED-AID (1-800-433-3243)
PO Box 84
Washington, DC 20044-0084
•
Appendix C: An Introduction to Postural Drainage and Percussion
Postural drainage and percussion (PD & P), also known as chest
physical therapy (CPT), is a way to help people with cystic fibrosis
(CF) breathe with less difficulty and stay healthy. PD & P uses
gravity and percussion to loosen the thick, sticky mucus in the
lungs so it can be removed by coughing. Unclogging the airways
is key to keeping lungs healthy.
PD & P is easy to do using the techniques described here. For
the person with CF, PD & P can be done by physical therapists
(PTs), respiratory therapists (RTs), nurses, parents, family and even
friends.
People with CF sometimes use other types of treatments, such
as inhaled bronchodilators and antibiotics, to keep their lungs
healthy. If ordered, bronchodilators should be taken before
PD & P to open the airways, and inhaled antibiotics should be
taken after PD & P so that the medicine gets to the infection
better. Your care center doctor or therapist will help you figure
out a routine that will work best for you or your child.
Know Your Lungs
Learning more about the respiratory system and its relationship
to other organs in the body can help you to understand why
PD & P treatments are effective.
Getting Rid of Mucus
The goal of PD & P is to clear mucus from each of the five lobes
of the lungs by moving mucus into the larger airways so that it can
be coughed out. The right lung is composed of three lobes: the
upper lobe, the middle lobe and the lower lobe. The left lung is
made up of only two lobes: the upper lobe and the lower lobe.
FRONT
Apical
Figure 1: Anatomy of the Lungs
BACK
Apical
Apical
Apical
Posterior
Anterior
Anterior
Superior
Lingula
Anterior
Basal
Middle
Lobe
RIGHT
HEART
Anterior
Basal
Lateral
Basal
LEFT
Superior
Posterior
Basal
Posterior
Basal
LEFT
Lateral
Basal
RIGHT
188 • POSTURAL DRAINAGE AND PERCUSSION
The lobes are divided into smaller sections called segments. The
upper lobes on the left and right sides are each made up of three
segments: top (apical), back (posterior) and front (anterior).
The lungs are made up of a network of air tubes, air sacs and
blood vessels. These sacs allow for the exchange of oxygen and
carbon dioxide between the blood and air. It is these segments
that are being drained. Note the position of each lung segment
in Figure 1 on the previous page.
Performing PD & P
The performance of PD & P involves a combination of techniques
including multiple positions to drain the lungs, percussion,
vibration, deep breathing and coughing.
Once the person is in one of the positions, the caregiver does
percussion on the chest wall. This is usually given for a period
of 3 to 5 minutes and sometimes followed by vibration over
the same area for approximately 15 seconds (or during 5
exhalations). The person is then encouraged to cough or huff
forcefully to get the mucus out of the lungs.
Description of PD & P Techniques
Postural drainage uses gravity to help move mucus from the
lungs up to the throat. The person lies or sits in various positions
so that the part of the lung to be drained is as high as possible.
The part of the lung is then drained using percussion, vibration
and gravity. For a complete description of these positions, see
the diagrams later in this appendix. Your CF care team may tailor
these positions to your or your child’s needs.
Percussion or clapping by the caregiver on the chest wall over
the part of the lung to be drained helps move the mucus into the
larger airways. The hand is cupped as if to hold water but with
the palm facing down as in Figure 2. The cupped hand curves to
the chest wall and traps a cushion of air to soften the clapping.
Figure 2: Cupped Hand
Percussion is done forcefully and with a steady beat. It should
not be painful or sting if the hand is cupped properly. Each
percussion also should have a hollow sound. Most of the
movement is in the wrist with the arm relaxed, making percussion
less tiring to do.
POSTURAL DRAINAGE AND PERCUSSION • 189
Special attention must be taken to not clap over the spine,
breastbone, stomach and lower ribs or back to prevent injury to
the spleen on the left, the liver on the right and the kidneys in
the lower back.
Different devices may be used instead of the traditional cupped
palm method for percussion. Ask your doctor or physical or
respiratory therapist for advice.
Vibration gently shakes the mucus into the larger airways. The
caregiver places a hand firmly on the chest wall over the part of
the lung being drained and tenses the muscles of the arm and
shoulder to create a fine shaking motion. Then, the caregiver
applies a light pressure over the area being vibrated. (The
caregiver also may place one hand over the other, then press
the top and bottom hand into each other to vibrate.) Vibration is
done with the flattened hand, not the cupped hand, as in Figure
3. Exhalation should be as slow and as complete as possible.
Figure 3: Flat Hand
Deep breathing moves the loosened mucus and may lead to
coughing. Breathing with the diaphragm, belly breathing or
lower chest breathing is used to help the person take deeper
breaths and get the air into the lower lungs. The belly moves
outward when the person breathes in and sinks in when he or she
breathes out.
Coughing is key to clear the airways of mucus. A forced but not
strained exhalation, following a deep inhalation, may help a
person cough. The mucus can then be coughed out.
Huffing
Huffing is a type of cough. It also involves taking a breath in and
actively exhaling. It is more like “huffing” onto a mirror or window
to steam it up. It is not as forceful as a cough but may work
better and be less tiring.
Timing of PD & P
Generally, each treatment session can last for 20 to 40 minutes.
PD & P is best done before meals or 1½ to 2 hours after eating
to decrease the chance of vomiting. Early morning and bedtimes
usually are recommended. The length of PD & P and the number
of times of day it is done may need to be increased if the person
is more congested or getting sick. Your CF doctor or therapist will
help you know what positions, how often and how long PD & P
should be done.
190 • POSTURAL DRAINAGE AND PERCUSSION
Enhancing PD & P for the Person With CF and the Caregiver
Both the person with CF and the caregiver should be comfortable
during PD & P. Before starting, the person with CF should remove
tight clothing, jewelry, buttons and zippers around the neck, chest
and waist. Light, soft clothing, such as a T-shirt, may be worn. Do
not do PD & P on bare skin. The caregiver should remove rings
and other bulky jewelry such as watches or bracelets. A supply of
tissues or a place to cough out the mucus should be nearby.
Doing PD & P Comfortably and Carefully
The caregiver should not lean forward when doing percussion,
but should remain in an upright position to protect his or her
back. The table on which the person with CF lies should be at a
comfortable height for the caregiver.
Pillows, sofa cushions, bundles of newspapers under pillows for
support, cribs with adjustable mattress heights/tilts, foam wedges
and bean bag chairs work for many. Infants can be positioned with
or without pillows in the caregiver’s lap.
Purchasing Equipment
Equipment such as drainage tables, electrical and nonelectrical
palm percussors and vibrators may be helpful. These can be
purchased from medical equipment stores. Older children and
adults may find percussors useful when doing their own PD & P.
Talk to your doctor or therapist at your CF care center about
equipment for PD & P.
Making PD & P More Enjoyable
To enhance the quality of the time you spend doing PD & P, do
one of the following:
•
Schedule PD & P around a favorite TV show.
•
Play favorite music or recorded stories.
•
Spend time talking or singing before, during and after PD & P.
• For kids, encourage blowing or coughing games during
PD & P, such as blowing pinwheels or coughing the deepest cough.
• Ask willing and capable relatives, friends, brothers and sisters to do PD & P. This can provide a welcome break from the daily routine.
•
Minimize interruptions.
Finding ways that make PD & P more enjoyable can help you
keep a regular routine and get maximum health benefits.
POSTURAL DRAINAGE AND PERCUSSION • 191
Instructions for Postural Drainage Positions
The following diagrams show and describe the positions for PD & P. The shaded areas show
where the chest should be percussed or clapped.
Pillows may be used for added comfort. If the person tires easily, the order of the positions
can be varied, but all areas of the chest should be percussed or clapped.
Please remember to percuss and vibrate only over the ribs. Avoid percussing and vibrating
over the spine, breastbone, stomach and lower ribs or back to prevent injury. Do not percuss
or vibrate on bare skin.
Self-Percussion–Upper Lobes
The person with CF should sit upright and
reach across his or her chest to clap on front
of chest over the muscular area between the
collarbone and the top of the shoulder blade.
Repeat on the opposite side.
Upper Front Chest–Upper Lobes
Have the person with CF sit upright. Clap
on both sides of upper front chest over the
muscular area between the collarbone and
the top of the shoulder blade.
* The people with CF are shown without shirts to better demonstrate the PD & P technique in illustrations. Images are from the CF Family Education Program and used with permission from Baylor College of Medicine.
192 • POSTURAL DRAINAGE AND PERCUSSION
Upper Back Chest–Upper Lobes
Have the person with CF sit up and lean
forward on a pillow over the back of a
sofa or soft chair at a 30 degree angle.
Stand or sit behind him or her and clap
both sides of the upper back. Take care
not to clap on the backbone.
Upper Front Chest–Upper Lobes
Have the person with CF lie on his
or her back with arms to sides. Stand
behind his or her head. Clap both sides
of the chest between the collarbone
and nipple.
Left Side Front Chest
Have the person with CF lie with left
side up and raise his or her left arm over
head. Clap over the ribs just below the
nipple area on front side of left chest.
Do not clap on the stomach.
* The people with CF are shown without shirts to better demonstrate the PD & P technique in illustrations. Images are from the CF Family Education Program and used with permission from Baylor College of Medicine.
POSTURAL DRAINAGE AND PERCUSSION • 193
Right Side Front Chest
Have the person with CF lie with right
side up and raise right arm over head.
Clap over the chest just below the
nipple area on front side of right chest.
Do not clap lower ribcage.
Lower Back Chest–Lower Lobes
Have the person with CF lie on his or
her stomach. Clap both sides at the
bottom of chest just above the bottom
edge of the ribcage. Do not clap lower
ribcage or over the backbone.
Left Lower Side Back Chest–Lower
Lobe
Have the person with CF lie with left
side up and roll toward the caregiver
a quarter turn so he or she can reach
the back of the person with CF. Clap on
lower left side of chest just above the
bottom edge of the ribcage.
* The people with CF are shown without shirts to better demonstrate the PD & P technique in illustrations. Images are from the CF Family Education Program and used with permission from Baylor College of Medicine.
194 • POSTURAL DRAINAGE AND PERCUSSION
Right Lower Side Back–Lower Lobe
Have the person with CF lie right side
up and roll toward the caregiver a
quarter turn so he or she can reach the
back of the person with CF. Clap on
lower right side of the chest just above
the bottom edge of the ribcage.
* The people with CF are shown without shirts to better demonstrate the PD & P technique in illustrations. Images are from the CF Family Education Program and used with permission from Baylor College of Medicine.
Appendix D: Daily Record of Diet, Treatments
and Symptoms
Many people with CF and parents of children with CF find it
helpful to keep daily records. The records on the following pages
can help you keep track of any changes in health. You can copy
these pages if you wish to keep such records, and bring them to
clinic visits.
196 • DAILY RECORD OF DIET, TREATMENTS AND SYMPTOMS
Food Diary
Date:________________________
BREAKFAST: Start time________
End time________
Food and drinks (type/amount):
Number of enzymes:
Time taken:
Comments:
MORNING SNACK: Start time________
End time________
Food and drinks (type/amount):
Number of enzymes:
Time taken:
Comments:
LUNCH: Start time________
End time________
Food and drinks (type/amount):
Number of enzymes:
Time taken:
Comments:
AFTERNOON SNACK: Start time________
End time________
Food and drinks (type/amount):
Number of enzymes:
Time taken:
Comments:
DINNER: Start time________
End time________
Food and drinks (type/amount):
Number of enzymes:
Time taken:
Comments:
EVENING SNACK: Start time________
End time________
Food and drinks (type/amount):
Number of enzymes:
Time taken:
Comments:
DAILY RECORD OF DIET, TREATMENTS AND SYMPTOMS • 197
Treatment Diary
Date:________________________
AIRWAY CLEARANCE
Type(s):
How long?
Time(s):
Comments:
INHALED MEDICATIONS
Name(s) of medication: Time(s) taken:
Dosage(s):
Comments:
ENZYMES
Name of enzyme:
Times taken:
Dosages:
Comments:
VITAMINS
Name of vitamin:
Time(s) taken:
Dosage(s):
Comments:
OTHER MEDICATIONS
Name of medication(s):
Time(s) taken:
Dosage(s):
Comments:
198 • DAILY RECORD OF DIET, TREATMENTS AND SYMPTOMS
Symptom DiaryDate:________________________
COUGH AND BREATHING
When does How does the Amount of
coughing
cough sound? sputum coughed
occur?
(e.g., Does it up, color and
sound wet?) thickness
Any wheezing,
shortness of
breath and/or
trouble
breathing?
Other comments
about cough or
breathing
Gas? (occasional, frequent or
excessive)
Other (describe)
BOWEL MOVEMENTS (BM)
How many a
day?
Appearance Bad smell?
(e.g., hard, soft, floats, greasy, loose)
OTHER DIGESTIVE SYMPTOMS
Abdominal pain Reflux
Appetite different Rectal prolapse
from normal
OTHER COMMENTS
Other (describe)
Appendix E: CF Foundation–Accredited Care Centers
Some people with cystic fibrosis (CF) live near only one CF
Foundation–accredited care center. Others have a choice of
several centers. Here are some questions to ask yourself about
your CF care center.
• How well do you or your family work with the doctor and other center team members? Do you trust the CF care center team? Sometimes the fit may be better with one team than with another.
How far away is the center? Will the location work out for you and your family if you or your child has to stay at the hospital for treatment?
•
•
•
•
•
Is there a program for adults with CF?
Does the center and hospital accept your insurance?
Where is the hospital that people with CF are admitted?
What services are available at the hospital?
• Are parents allowed to stay with their child? Is there a playroom? Is a teacher available at the hospital to help with schoolwork? Is there a child life specialist who can help your child cope with staying at the hospital?
• For adults with CF, can family members stay? Is there Internet access so you can continue to work or go to school?
To inspire action and strengthen partnerships, the CF Foundation
provides care center data for key health measures for each of its
accredited care centers at www.cff.org. This information is a tool
for people with CF and their families to use to partner with their
care center to improve CF care.
It may be helpful to visit each of the CF care centers near you
to find out which will best fit your needs or the needs of your
child and family. Remember, CF care teams at CF Foundation–
accredited CF care centers have the most current information
about treatments to keep you or your child in the best health
possible.
The CF Foundation is available at 1-800-FIGHT CF and
info@cff.org to answer questions about your local CF care
centers. Also see the additional care center–related resources
in Appendix B.
200 • CF FOUNDATION–ACCREDITED CARE CENTERS
Alabama
Arkansas
The Children’s Hospital/UAB
(Pediatric)
1600 7th Ave. South
Birmingham, AL 35233
(205) 638-9583
Hector H. Gutierrez, M.D.
Wynton Hoover, M.D.
Arkansas Children’s Hospital
(Pediatric)
800 Marshall St.
Little Rock, AR 72202
(501) 364-4000
John Carroll, M.D.
Gulnar Com, M.D.
University of Alabama at
Birmingham (Adult)
The Kirklin Clinic
2000 6th Ave. South
Birmingham, AL 35205
(205) 975-3258
Veena Antony, M.D.
Kevin Leon, M.D.
University of Arkansas for
Medical Sciences (Adult)
4301 W. Markham St., #555
Little Rock, AR 72205
(501) 603-1400
Paula Anderson, M.D.
Alaska
Marin (Outreach CF Clinic)
1100 Larkspur Landing Circle,
Suite 150
Larkspur, CA 94939
(415) 461-3498
Patty Sylvester
Providence Alaska Medical
Center (Pediatric and Adult)
Pediatric Subspecialty Clinic
3300 Providence Dr., Suite 314
Anchorage, AK 99508
(907) 212-4824
Dion Roberts, M.D.
Elizabeth Galloway, M.D.
Arizona
Phoenix Children’s Hospital
(Pediatric and Adult)
1919 East Thomas Rd.
Phoenix, AZ 85016
(602) 933-0985
Pediatric: Peggy Radford, M.D.
Adult: Gerald D. Gong, M.D.
Tucson CF Center (Pediatric and
Adult)
535 N. Wilmot Rd., Ste. 100
Tucson, AZ 85711
(520) 694-9988
Wayne J. Morgan, M.D., C.M.
Cori Daines, M.D.
California
Miller Children’s Hospital
(Pediatric)
2801 Atlantic Ave.
Long Beach, CA 90806
(562) 933-8749
Eliezer Nussbaum, M.D.
Terry Chin, M.D., Ph.D.
Long Beach Memorial Medical
Center (Adult)
2801 Atlantic Ave.
Ground Floor
Long Beach, CA 90806
(562) 933-2820
Jeffrey Riker, M.D.
Inderpal Randhawn, M.D.
Children’s Hospital of
Los Angeles (Pediatric)
4650 Sunset Blvd. MS# 83
Los Angeles, CA 90027
(323) 361-4545
Thomas Keens, M.D.
CF FOUNDATION–ACCREDITED CARE CENTERS • 201
USC Keck School of Medicine
(Adult)
1500 San Pablo St.
Los Angeles, CA 90033
(323) 442-8330
Adudpa Pursh Rao, M.D.
Kaiser Foundation Los Angeles
Medical Center (Pediatric and
Adult)
4700 Sunset Blvd., Module 3C
Los Angeles, CA 90027
(800) 954-8000
Muhammad Saeed, M.D.
Tina Chou, M.D.
Children’s Hospital Central
California (Pediatric)
9300 Valley Childrens Place
Madera, CA 93636
(559) 353-5550
Reddy Sudhakar, M.D., Ph.D.
Children’s Hospital of Orange
County (Pediatric)
Pulmonary Clinic
455 South Main St.
Orange, CA 92868
(714) 532-7983
Bruce Nickerson, M.D.
Stanford University Medical
Center (Pediatric)
Mary Johnson Ambulatory
Care Center
730 Welch Rd.
Palo Alto, CA 94304
(650) 497-8841
Carlos E. Milla, M.D.
Stanford University (Adult)
300 Pasteur Dr.
Stanford, CA 94305
(650) 498-6840
Paul Mobabir, M.D.
Children’s Hospital at Oakland
(Pediatric)
747 52nd St., OPC-5409
Oakland, CA 94609
(510) 428-3314
Karen A. Hardy, M.D.
Pleasanton (Outreach CF Clinic)
5820 Stoneridge Mall Rd.
Suite 210
Pleasanton, CA 95488
(925) 463-8970
Patty Sylvester
California Pacific Medical Center
(Adult)
2351 Clay St., #501
San Francisco, CA 94115
(415) 923-3421
Ryan Dougherty, M.D.
Kaiser Foundation Roseville
(Pediatric)
Department of Pediatric
Pulmonology
1600 Eureka Rd. - MOB II
Roseville, CA 95661
(916) 474-2267
Gregory F. Shay, M.D.
Kaiser Foundation Oakland
(Pediatric)
12th Floor, Pediatric Subspecialty
3505 Broadway
Oakland, CA 94619
(510) 752-6906
Gregory F. Shay, M.D.
Kaiser Foundation Oakland
(Adult)
280 West MacArthur
Oakland, CA 94611
(510) 752-6906
Bryon Quick, M.D.
Sutter Medical Center (Pediatric)
1625 Stockton Blvd, Suite 112
Sacramento, CA 95816
(916) 262-9114
Bradley E. Chipps, M.D.
University of California at Davis
Medical Center (Pediatric)
Glasrock Clinic Building
2521 Stockton Blvd
Sacramento, CA 95817
(916) 734-3112
Ruth McDonald, M.D.
This listing is current as of June
2012. For the most up-to-date
list of care centers, visit the
CF Foundation’s website
(www.cff.org) or call
1-800-FIGHT CF.
202 • CF FOUNDATION–ACCREDITED CARE CENTERS
University of California at Davis
Medical Center (Adult)
2825 J St., Suite 400
Sacramento, CA 95816
(916) 734-3738
Brian Morrissey, M.D.
Carroll Cross, M.D.
Kaiser Foundation South
Sacramento (Adult)
MOB 2 Pulmonary Dept. 322
6600 Bruceville Rd.
Sacramento, CA 95823
(916) 688-2200
Bryon Quick, M.D.
Loma Linda University Medical
Center (Pediatric)
2195 Club Center Dr., Suite G
San Bernardino, CA 92408
(909) 835-1808
Yvonne Fanous, M.D.
Henry Opsimos, M.D.
Rady Children’s Hospital and
Health Center at the University
of California San Diego
(Pediatric)
3020 Children’s Way
San Diego, CA 92123
(858) 966-5846
Mark S. Pian, M.D.
The Regents of the University
of California at San Francisco
(Pediatric)
UCSF Pediatric Specialties Clinic
400 Parnassus Ave., 2nd Floor
San Francisco, CA 94143
(415) 353-7337
Dennis W. Nielson, M.D., Ph.D.
University of California at
San Francisco (Adult)
UCSF Faculty Chest Practice
UCSF Ambulatory Care Center
400 Parnassus Ave., 5th Floor
San Francisco, CA 94143
(415) 353-2961
Mary Ellen Kleinhenz, M.D.
California Pacific Medical Center
(Outreach CF Clinic)
3700 California St., B554
San Francisco, CA 94115
(510) 428-3305
Patty Sylvester
Kaiser Permanente Foundation
(Pediatric)
Pediatrics, MOB 190
710 Lawrence Expressway
Santa Clara, CA 95051
(510) 752-7098
Gregory F. Shay, M.D.
University of California San
Diego Medical Center-Thornton
(Adult)
9300 Campus Point Dr., MC 7381
La Jolla, CA 92037
(858) 657-7073
Douglas J. Conrad, M.D.
Kaiser Foundation Santa Clara
(Adult)
Pulmonary Dept 282
710 Lawrence Expressway
Santa Clara, CA 95051
(408) 851-1161
Bryon Quick, M.D.
Naval Medical Center San
Diego–FOR MILITARY
PERSONNEL ONLY (Pediatric
and Adult)
34800 Bob Wilson Dr.
Department of Pediatrics
San Diego, CA 92134
(619) 532-6896
Henry Wojtczak, M.D.
Tahoe Forest Multi-Specialty
Clinic (Outreach CF Clinic)
10956 Donner Pass Rd., Suite 210
Truckee, CA 96161
(530) 587-3523
Estella Iniguez
Pediatric Diagnostic Center
(Pediatric and Adult)
3160 Loma Vista Rd.
Ventura, CA 93003
(805) 652-6255
Chris Landon, M.D.
CF FOUNDATION–ACCREDITED CARE CENTERS • 203
Walnut Creek (Outreach CF
Clinic)
2401 Shadelands Dr.
Walnut Creek, CA 94598
(925) 979-4000
Patty Sylvester
Colorado
Central Connecticut Cystic
Fibrosis Center (Adult)
80 Seymour St.
Pulmonary Lab: Jefferson
Building
Hartford, CT 06102
(860) 545-4644
James Pope, M.D.
The Children’s Hospital
Colorado (Pediatric)
Pediatric Pulmonary Medicine
Cystic Fibrosis Program
Children’s Hospital, Box B395
13123 East 16th Ave.
Aurora, CO 80045
(720) 777-6181
Scott Sagel, M.D., Ph.D.
Yale University School of
Medicine (Pediatric)
Yale New Haven Children’s
Hospital
2nd Floor Subspecialty Center
20 York St.
New Haven, CT 06520
(203) 785-4081
Marie E. Egan, M.D.
National Jewish Medical and
Research (Adult)
1400 Jackson St.
Denver, CO 80206
(303) 398-1178
Jerry A. Nick, M.D., B.S.
Yale University School of
Medicine (Adult)
Winchester Clinic
20 York St., 2nd Floor
New Haven, CT 06520
(203) 785-4198
Jonathan L. Koff, M.D.
Children’s Hospital Colorado
Outpatient Specialty Care
(Outreach CF Clinic)
4125 Briargate Parkway
Colorado Springs, CO 80920
(720) 777-5869
Melissa Burkey
Pediatric Partners of the
Southwest
575 Rivergate Lane, Suite 109
Durango, CO 81301
720-777-5869
Melissa Burkey
Connecticut
Central Connecticut Cystic
Fibrosis Center (Pediatric)
Pediatric Pulmonology
Connecticut Children’s Medical
Center
282 Washington St.
Hartford, CT 06106
(860) 545-9440
Craig D. Lapin, M.D.
Delaware
Alfred I. duPont Hospital for
Children (Pediatric and Adult)
1600 Rockland Rd.
WIlmington, DE 19803
(302) 651-6400
Raj Padman, M.D.
Aaron Chidekel, M.D.
District of Columbia
Children’s National Medical
Center (Pediatric and Adult)
111 Michigan Ave., NW
Suite 1030
Washington, DC 20010
(202) 476-2128
Pediatric: Anastassios
Koumbourlis, M.D., M.P.H.
Adult: Peter Levit, M.D.
This listing is current as of June
2012. For the most up-to-date
list of care centers, visit the
CF Foundation’s website
(www.cff.org) or call
1-800-FIGHT CF.
204 • CF FOUNDATION–ACCREDITED CARE CENTERS
Florida
Children’s Hospital of Southwest
Florida/Children’s Specialists
Pediatric Pulmonology & Sleep
Medicine (Pediatric)
7970 Summerlin Lakes Drive
Fort Myers, FL 33905
(239) 437-5500
Oscar A. Alea, M.D.
University of Florida (Pediatric)
Medical Plaza Pediatric
Specialties Clinics
2000 SouthWest Archer Rd.
Gainesville, FL 32608
(352) 273-8381
Pamela M. Schuler, M.D.
University of Florida (Adult)
1600 SW Archer Rd., Suite 1901
POB 100383
Gainesville, FL 32610
(352) 273-8735
Mark Brantly, M.D. (Interim)
Joe DiMaggio Children’s
Hospital (Pediatric and Adult)
3341 Johnson St.
Hollywood, FL 33021
(954) 265-6333
Juan Martinez, M.D.
Nemours Children’s Clinic–
Jacksonville (Pediatric)
807 Children’s Way
Jacksonville, FL 32207
(904) 697-3788
Bonnie B. Hudak, M.D.
David Schaeffer, M.D.
Pulmonary and Critical Care
Associates of Jacksonville
(Adult)
425 North Lee St.
Suite 202
Jacksonville, FL 32204
(904) 366-3738
Harish Bhaskar, M.D.
University of Miami (Pediatric
and Adult)
Batchelor Children’s Research
Institute
1580 NW 10th Ave., 1st Floor
Miami, FL 33136
Pediatric: (305) 243-6162
Annabelle Quizon, M.D.
Andrew R.A. Colin, M.D.
Adult: (305) 243-6388
Matthias Salathe, M.D.
Miami Children’s Hospital
(Pediatric)
3200 SW 60 Court
Medical Arts Building, Suite 203
Miami, FL 33155
(305) 669-5864
Maria E. Franco, M.D.
Arnold Palmer Hospital Specialty
Practice (Pediatric)
83 West Columbia St.
Orlando, FL 32806
(321) 841-6317
Mark Weatherly, M.D.
Nemours Children’s Clinic–
Orlando (Pediatric)
1717 South Orange Ave.
Orlando, FL 32806
(407) 650-7270
Floyd Livingston, M.D.
Central Florida Pulmonary
Group (Adult)
326 North Mills Ave.
Orlando, FL 32803
(407) 841-1100
Francisco Calimano, M.D.
Daniel Layish, M.D.
Nemours Children’s Clinic Pensacola (Pediatric and Adult)
5153 N. 9th Ave.
Pensacola, FL 32504
(850) 505-4785
Barbara Stewart, M.D.
CF FOUNDATION–ACCREDITED CARE CENTERS • 205
All Children’s Specialty Care
Clinic of Sarasota (Outreach CF
Clinic)
5811 Rand Blvd.
Sarasota, FL 34238
(727) 767-3995
Stasia Lehmann, RN, BSN
All Children’s Hospital (Pediatric)
601 5th St. South, Suite 708
St. Petersburg, FL 33701
(727) 767-3995
Magdalen Gondor, M.D.
Tampa General Hospital (Adult)
5 Tampa General Circle, Suite 300
Tampa, FL 33606
(813) 844-4634
Mark Rolfe, M.D.
University of South Florida
(Pediatric)
13101 Bruce B. Downs Blvd.
Tampa, FL 33612
(813) 259-8767
Bruce M. Schnapf, D.O.
Georgia
Georgia Regents University
(Pediatric)
1120 15th St.
Augusta, GA 30912
(706) 721-2635
Katie McKie, M.D.
Georgia Regents University
(Adult)
1120 15th St.
Augusta, GA 30912
(706) 721-4658
Caralee Forseen, M.D.
Emory University (Pediatric)
2015 Uppergate Dr.
Atlanta, GA 30322
(404) 727-5728
Michael Schechter, M.D.
Emory University (Adult)
1365 Clifton Rd. N.E.
4th Floor, Bldg A #4325
Atlanta, GA 30322
(404) 778-7928
Seth Walker, M.D.
Children’s Healthcare of Atlanta
at Scottish Rite (Pediatric)
1100 Lake Hearn Dr.
Suite 450
Atlanta, GA 30342
(404) 785-2898
Kevin Kirchner, M.D.
Hawaii
Tripler Army Medical Center–
FOR MILITARY PERSONNEL
ONLY (Pediatric and Adult)
1 Jarrett White Rd.
Honolulu, HI 96859
(808) 433-5907
Jane E. Gross, M.D., Ph.D.
Idaho
Saint Luke’s Cystic Fibrosis
Center of Idaho (Pediatric and
Adult)
100 E. Idaho St.
Suite 200
Boise, ID 83712
(208) 381-7092
Henry R. Thompson, M.D.
Illinois
Ann & Robert H. Lurie Children’s
Hospital of Chicago (Pediatric)
225 E. Chicago Ave.
Chicago, IL 60611
(312) 227-6730
Susanna A. McColley, M.D.
Adrienne L. Prestridge, M.D.
Northwestern University (Adult)
Suite 14-002B
676 N. St. Clair
Chicago, IL 60611
(773) 880-4382
Manu Jain, M.D.
This listing is current as of June
2012. For the most up-to-date
list of care centers, visit the
CF Foundation’s website
(www.cff.org) or call
1-800-FIGHT CF.
206 • CF FOUNDATION–ACCREDITED CARE CENTERS
Rush University Medical Center
(Pediatric)
Pediatric Subspeciality Clinic
1725 W. Harrison St., POB 710
Chicago, IL 60612
(312) 942-3034
Girish Sharma, M.D.
John Lloyd-Still, M.D.
Rush University Medical Center
(Adult)
1725 W. Harrison, POB 054
Chicago, IL 60612
(312) 942-6744
Robert A. Balk, M.D.
University of Chicago (Pediatric)
5721 S. Maryland Ave.
Comer 2, 4th Floor
Chicago, IL 60637
(773) 702-6178
Lucille A. Lester, M.D.
University of Chicago (Adult)
5841 S. Maryland Ave.
DCAM, 4th Floor
Chicago, IL 60637
(773) 702-9660
Edward Naureckas, M.D.
Loyola University Medical
Center (Pediatric and Adult)
2160 S. First Ave.
Maywood, IL 60153
(708) 327-9134
Sean Forsythe, M.D.
Advocate Lutheran General
Children’s Hospital CF Center
(Pediatric)
1675 Dempster St.
2nd Floor
Park Ridge, IL 60068
(847) 318-9330
Gabriel Aljadeff, M.D.
Lutheran General Hospital
(Adult)
North Suburban Pulmonary
Specialists
8780 W. Golf Rd., Suite 102
Niles, IL 60714
(847) 759-4770
Arvey M. Stone, M.D.
Advocate Hope Children’s
Hospital (Pediatric)
4440 West 95th St.
Oak Lawn, IL 60453
(708) 684-5810
Javeed Akhter, M.D.
Saint Francis Medical Center
(Pediatric and Adult)
320 East Armstrong Ave.
Peoria, IL 61603
(309) 624-6565
Pediatric: Jalayne M. Lapke, M.D.,
F.A.A.P.
Adult: W. Anthony Sauder, M.D.
Southern Illinois University
School of Medicine (Pediatric
and Adult)
751 North Rutledge St.
Room 0300
Springfield, IL 62702
(217) 545-5864
Mark Johnson, M.D.
Joseph Henkle, M.D.
Indiana
Deaconess Hospital (Outreach
CF Clinic)
600 Mary St.
Evansville, IN 47710
(812) 858-3131
Sara King
Lutheran Children’s Hospital
(Pediatric and Adult)
7950 W. Jefferson Blvd.
Primary Life Services
Fort Wayne, IN 46804
(260) 435-7123
Pushpom Z. James, M.D.
Riley Hospital for Children
Indiana University Medical
Center (Pediatric)
Riley Hospital for Children
702 Barnhill Drive, ROC 2nd Floor
Indianapolis, IN 46202
(317) 274-7208
Michelle S. Howenstine, M.D.
CF FOUNDATION–ACCREDITED CARE CENTERS • 207
Indiana University (Adult)
550 N. University Blvd.
Indianapolis, IN 46202
(317) 948-8660
Michael Ober, M.D.
Saint Joseph Regional Medical
Center (Pediatric and Adult)
Pediatric Specialty Clinics
611 East Douglas Rd.
Suite 405
Mishawaka, IN 46545-1468
574-335-6240
James B. Harris, III, M.D.
Iowa
Mary Greeley Hospital–
McFarland Clinic (Pediatric and
Adult)
1215 Duff Ave.
Ames, IA 50010
(515) 239-4482
Edward. Nassif, M.D.
Blank Children’s Health Center
(Pediatric)
1212 Pleasant St. Ste. 204
Des Moines, IA 50309
(515) 241-6548
Ricardo Flores, M.D.
University of Iowa (Pediatric)
Pediatric Dept., UIHC
200 Hawkins Drive
Iowa City, IA 52242
(319) 356-2229
Miles Weinberger, M.D.
Richard Ahrens, M.D.
University of Iowa (Adult)
Medical Subspecialty Clinics UIHC
Department of Internal Medicine
200 Hawkins Drive
Iowa City, IA 52242
(319) 356-8133
Douglas Hornick, M.D.
Kansas
University of Kansas Medical
Center (Pediatric)
3901 Rainbow Blvd
MS 4004
Kansas City, KS 66160
(913) 588-6364
Mitzi Scotten, M.D.
University of Kansas Medical
Center (Adult)
Suite A Bell Hospital
Mailstop 3007
3901 Rainbow Blvd.
Kansas City, KS 66160
(913) 588-1227
Joel Mermis, M.D.
Steven Stites, M.D.
Via Christi–Saint Francis Campus
(Pediatric and Adult)
707 N. Emporia
Wichita, KS 67214
(316) 858-3463
Pediatric: Natalie Sollo, M.D.
Adult: Janel Harting, M.D.
Kentucky
University of Kentucky
(Pediatric)
Kentucky Clinic - 2nd Floor - Wing C
740 South Limestone St.
Lexington, KY 40536
(859) 323-6211
Jamshed F. Kanga, M.D.
University of Kentucky (Adult)
Kentucky Clinic - 5th Floor - Wing D
740 South Limestone St.
Lexington, KY 40536
(859) 323-9555
Michael I. Anstead, M.D.
University of Louisville
(Pediatric)
234 E. Gray St.
Suite 270
Louisville, KY 40202
(502) 629-8830
Nemr S. Eid, M.D.
This listing is current as of June
2012. For the most up-to-date
list of care centers, visit the
CF Foundation’s website
(www.cff.org) or call
1-800-FIGHT CF.
208 • CF FOUNDATION–ACCREDITED CARE CENTERS
University of Louisville (Adult)
401 E. Chestnut St., Suite 310
Louisville, KY 40202
(502) 813-6500
Rodney J. Folz, M.D., Ph.D.
Louisiana
Tulane University (Pediatric and
Adult)
1415 Tulane Ave.
New Orleans, LA 70112
(504) 988-5800
Pediatric: Scott H. Davis, M.D.
Adult: Dean B. Ellithorpe, M.D.
Louisiana State University Health
Sciences Center (Pediatric and
Adult)
Department of Pediatric
Pulmonary
1501 Kings Highway
Shreveport, LA 71103
(318) 675-6094
Kimberly L. Jones, M.D.
Maine
Eastern Maine Medical Center
(Pediatric and Adult)
417 State St., Suite 305
Bangor, ME 04401
(207) 973-4051
Thomas Lever, M.D.
Maine Medical Partners
Pediatric Specialty Care
(Pediatric)
887 Congress St., Suite 320
Portland, ME 04102
(207) 662-5522
Anne Marie Cairns, D.O.
Maine Medical Center (Adult)
Chest Medicine Associates
100 Foden Rd.
South Portland, ME 04106
(207) 828-1122
Jonathan Zuckerman, M.D.
Maryland
Johns Hopkins University
(Pediatric)
David Rubenstein Child Health
Building
200 North Wolfe St., Lower Level
Baltimore, MD 21287
(410) 955-2795
Peter J. Mogayzel, Jr., M.D., Ph.D.
Pamela L. Zeitlin, M.D., Ph.D.
Johns Hopkins University (Adult)
601 N. Caroline St.
Baltimore, MD 21287
(410) 502-7044
Michael P. Boyle, M.D., F.C.C.P.
National Institutes of Health
(Adult)
NIH Clinical Center
ACRF 9
10 Center Drive
Bethesda, MD 20892
(301) 496-6821
Milica S. Chernick, M.D.
James E. Balow, M.D.
National Naval Medical Center–
FOR MILITARY PERSONNEL
ONLY (Pediatric and Adult)
Pulmonology Clinic
8901 Rockville Pike
Bethesda, MD 20889
(301) 295-4959
Andrew J. Lipton, M.D., M.P.H.,
T.M.
Massachusetts
Children’s Hospital Boston
(Pediatric)
300 Longwood Ave.
Mailstop 208
Boston, MA 02115
(617) 355-1900
Henry L. Dorkin, M.D.
CF FOUNDATION–ACCREDITED CARE CENTERS • 209
Bringham and Women’s Hospital
(Adult)
74 Francis St.
Center for Chest Diseases
Boston, MA 02115
(617) 355-1900
Ahmet Uluer, D.O.
Massachusetts General Hospital
(Pediatric)
Joey O’Donnell Cystic Fibrosis
Center
275 Cambridge St., Suite 530
Boston, MA 02114
(617) 726-8707
Samuel Moskowitz, M.D.
Massachusetts General Hospital
(Adult)
55 Fruit St. Cox 2
Boston, MA 02114
(617) 724-0520
Leonard Sicilian, M.D.
Tufts Medical Center
(Pediatric and Adult)
Floating Hospital for Children
2nd Floor
800 Washington St.
Boston, MA 02111
(617) 636-7917
William F. Yee, M.D.
Baystate Medical Center
(Pediatric and Adult)
3300 Main St., Suite 4B
Springfield, MA 01199
(413) 794-0815
Robert Gerstle, M.D.
UMass Memorial Health Care–
Pediatric Pulmonary, Asthma
and CF Center (Pediatric)
Dept. of Pediatrics, S5-860
55 Lake Ave.
Worcester, MA 01655
(508) 856-4155
Brian P. O’Sullivan, M.D.
UMass Memorial Health Care
(Adult)
Benedict Building, University
Campus
55 Lake Ave., North
Worcester, MA 01655
(508) 856-4155
Oren Schaeffer, M.D.
Michigan
University of Michigan Health
System (Pediatric and Adult)
1500 E. Medical Center Dr.
Taubman Center, Floor 1
Reception D
Ann Arbor, MI 48109
Pediatric: (734) 764-4123
Samya Nasr, M.D.
Adult: (734) 647-9342
Richard H. Simon, M.D.
Children’s Hospital of Michigan
(Pediatric)
3901 Beaubien Blvd.
Detroit, MI 48201
(313) 745-5541
Ibrahim Abdulhamid, M.D.
Wayne State University Harper
University Hospital (Adult)
4201 St. Antoine, Suite 4C
Detroit, MI 48201
(313) 745-9151
Dana G. Kissner, M.D.
Ayman Soubani, M.D.
Hurley Children’s Clinic at
Mott Children’s Health Center
Outreach Program (Outreach CF
Clinic)
One Hurley Plaza
Flint, MI 48503
(810) 257-9344
Cem Demirci, M.D.
Helen DeVos Women and
Children’s Center (Pediatric)
330 Barclay Avenue, NE
Suite 200
Grand Rapids, MI 49503
(616) 267-2200
John Schuen, M.D.
This listing is current as of June
2012. For the most up-to-date
list of care centers, visit the
CF Foundation’s website
(www.cff.org) or call
1-800-FIGHT CF.
210 • CF FOUNDATION–ACCREDITED CARE CENTERS
Helen DeVos Women and
Children’s Center (Adult)
Spectrum Health, Butterworth
Hospital
100 Michigan St.
Grand Rapids, MI 49503
(616) 267-8244
Stephen Fitch, M.D.
Mississippi
Western Michigan University
School of Medicine Clinics
(Pediatric and Adult)
1000 Oakland Drive
Kalamazoo, MI 49008
(269) 337-6433
Douglas Homnick, M.D., M.P.H.
Adult: (601) 815-1145
Nauman Chaudary, M.D.
Michigan State University CF
Center – Lansing (Pediatric and
Adult)
1200 E. Michigan Ave., Suite 145
Lansing, MI 48912
(517) 364-5440
Myrtha Gregoire-Bottex, M.D.
Minnesota
Children’s Hospitals and Clinics
(Pediatric)
2530 Chicago Ave. South
Suite 400
Minneapolis, MN 55404
(612) 813-3330
John McNamara, M.D.
University of Minnesota
(Pediatric)
Pediatric Specialty Clinic
516 Delaware St. SE, 4th Floor
Minneapolis, MN 55455
(612) 625-5995
Warren Regelmann, M.D.
University of Minnesota (Adult)
516 Delaware St. SE, 2nd Floor
Minneapolis, MN 55455
(612) 625-5995
Jordan Dunitz, M.D.
University of Mississippi Medical
Center (Pediatric and Adult)
2500 North State St.
Jackson, MS 39216
Pediatric: (601) 984-5205
Joseph Marc Majure, M.D.
Missouri
Children’s Hospital University of
Missouri Health Sciences Center
(Pediatric and Adult)
1101 Hospital Dr.
Columbia, MO 65212
Pediatric: (573) 882-6978
James Acton, M.D.
Adult: (573) 882-6978
Melissa Kouba, M.D.
The Children’s Mercy Hospital–
University of Missouri at Kansas
City (Pediatric and Adult)
Pulmonology and Cystic Fibrosis
Clinics
Room 1740.00, 1st Floor
Out-Patient Care Center
2401 Gillham Rd.
Kansas City, MO 64108
(816) 983-6490
Philip Black, M.D.
Freemont Medical Building
(Outreach CF Clinic)
1965 S. Freemont St., Suite 220
Freemont Medical Building
Springfield, MO 65807
(573) 882-6978
James Acton, M.D.
Cox South Medical Center
(Outreach CF Clinic)
3443 S. National Ave.
Springfield, MO 65807
(417) 269-4856
John Carlile
CF FOUNDATION–ACCREDITED CARE CENTERS • 211
St. Louis Children’s Hospital
Washington University School of
Medicine (Pediatric)
One Children’s Place
Ambulatory Clinic, Suite C
St. Louis, MO 63110
(314) 454-2694
Peter Michelson, M.D., M.S.
Washington University School of
Medicine (Adult)
4921 Parkview Place, Suite 8B
St. Louis, MO 63110
(314) 454-8640
Daniel Rosenbluth, M.D.
Cardinal Glennon Children’s
Medical Center St. Louis
University (Pediatric)
1465 S. Grand Blvd.
St. Louis, MO 63104
(314) 268-4107
Blakeslee E. Noyes, B.A., M.D.
Gary Albers, M.D.
Saint Louis University Medical
Center (Adult)
3660 Vista Ave., Suite 200
St. Louis, MO 63110
(314) 977-6190
Ravi P. Nayak, M.D.
Montana
Billings Clinic (Pediatric and
Adult)
2800 Tenth Ave. North
Billings, MT 59101
(406) 238-5137
Jerimiah Lysinger, M.D.
Nebraska
Lincoln–Outreach Clinic
(Outreach CF Clinic)
445 South 86th St.
Lincoln, NE 68526
(402) 559-6275
Carla Pospisal
North Platte CF Clinic
(Outreach CF Clinic)
210 McNeil Lane
North Platte, NE 69101
(402) 559-6275
Carla Pospisal
Children’s Hospital and Medical
Center
8200 Dodge St.
Omaha, NE 68114
(402) 559-6275
John L. Colombo, M.D.
University of Nebraska Medical
Center (Adult)
42nd & Emile
Omaha, NE 68198
(402) 559-9101
Peter J. Murphy, M.D.
Nevada
University of Nevada School
of Medicine Children’s Lung
Specialists (Pediatric)
Cystic Fibrosis Center of
Southern Nevada
University Medical Center of
Southern Nevada
2231 West Charleston Blvd.
Las Vegas, NV 89102
(702) 598-4411
Craig T. Nakamura, M.D.
Adult Cystic Fibrosis Center
(Adult)
University Medical Center
Total Life Care
2231 West Charleston Blvd.
Las Vegas, NV 89107
(702) 598-4411
Angelica Honsberg, M.D.
Renown Regional Medical
Center Children’s Hospital
(Pediatric and Adult)
1155 Mill St.
Reno, NV 89502
(775) 982-5123
Sonia Budhecha, M.D.
This listing is current as of June
2012. For the most up-to-date
list of care centers, visit the
CF Foundation’s website
(www.cff.org) or call
1-800-FIGHT CF.
212 • CF FOUNDATION–ACCREDITED CARE CENTERS
New Hampshire
Dartmouth Hitchcock Medical
Center (Pediatric)
Children’s Hospital at Dartmouth
One Medical Center Drive
Lebanon, NH 03756
(603) 653-9884
Pamela Hofley, M.D.
Margaret Guill, M.D.
Dartmouth Hitchcock Medical
Center (Adult)
Adult CF Clinic–Desk 5C
One Medical Center Drive
Lebanon, NH 03756
(603) 653-9884
H. Worth Parker, M.D.
New Jersey
Saint Barnabas Medical Center
(Pediatric and Adult)
200 South Orange Ave.
Suite 225, Pediatric Specialties
Livingston, NJ 07039
(973) 322-7600
Dorothy S. Bisberg, M.D.
Monmouth Medical Center
(Pediatric)
279 Third Ave., Suite 604
Long Branch, NJ 07740
(732) 222-4474
Robert L. Zanni, M.D.
Monmouth Medical Center
(Adult)
279 Third Ave., Suite 303
Long Branch, NJ 07740
(732) 222-4474
Doantrang Du, M.D.
Goryeb Children’s Hospital
of Atlantic Health System
(Pediatric)
100 Madison Ave.
Morristown, NJ 07960
(973) 971-4142
Arthur B. Atlas, M.D.
Morristown Memorial Hospital
(Adult)
101 Madison Ave.
Morristown, NJ 07960
(973) 971-4103
Stanley B. Fiel, M.D.
Frederic Scoopo, M.D.
Bristol-Myers Squibb Children’s
Hospital at Robert Wood
Johnson University Hospital
(Pediatric)
Department of Pediatrics
Pulmonary Medicine and Cystic
Fibrosis Center
CHINJ, RM 2300
89 French St.
New Brunswick, NJ 08901
(732) 235-5210
Thomas F. Scanlin, M.D.
Bristol-Myers Squibb Children’s
Hospital at Robert Wood
Johnson University Hospital
(Adult)
Adult Pulmonary
125 Paterson St.
New Brunswick, NJ 08901
(732) 235-5210
Sabina Hussain, M.D.
St. Joseph’s Children’s Hospital
(Pediatric and Adult)
DePaul Ambulatory Center
11 Getty Ave., Second Floor
Paterson, NJ 07503
(973) 754-2550
Roberto V. Nachajon, M.D.
New Mexico
University of New Mexico
School of Medicine (Pediatric
and Adult)
University Hospital
2211 Lomas Blvd. NE, ACC 3
Albuquerque, NM 87106
Pediatric: (505) 272-6633
Lea Davies, M.D.
Adult: (505) 272-4751
Theresa Heyne Kamp, M.D.
CF FOUNDATION–ACCREDITED CARE CENTERS • 213
New York
Albany Medical College
(Pediatric)
Pediatric Pulmonary & Cystic
Fibrosis Center
47 New Scotland Ave.
Albany, NY 12208
(518) 262-6880
Paul G. Comber, M.D., Ph.D.
Albany Medical College (Adult)
Pulmonary & Critical Care
Medicine
47 New Scotland Ave.
Albany, NY 12208
(518) 262-5196
Jonathan M. Rosen, M.D.
Good Samaritan Hospital
(Pediatric)
655 Deer Park Ave.
Babylon, NY 11702
(631) 321-2100
Louis Guida, Jr., M.D.
Women and Children’s Hospital
of Buffalo (Pediatric)
218 Bryant St.
Buffalo, NY 14222
(716) 878-7524
Drucy Borowitz, M.D.
Women and Children’s Hospital
of Buffalo (Adult)
219 Bryant St.
Buffalo, NY 14222
(716) 878-7524
Carla Frederick, M.D.
University Medical Center at
Stony Brook (Pediatric)
37 Research Way
East Setauket, NY 11733
(631) 444-5437
Catherine Kier, M.D.
The Steven & Alexandra Cohen
Children’s Medical Center of
New York (Pediatric)
Pediatric Cystic Fibrosis Center
865 Northern Blvd., Suite 103
Great Neck, NY 11021
(516) 622-5280
Joan K. DeCelie-Germana, M.D.
The Children’s Hospital at
Westchester Medical Center/
New York Medical College
(Pediatric and Adult)
19 Bradhurst Ave., Suite 1400
Hawthorne, NY 10532
(914) 493-7585
Pediatric: Allen J. Dozor, M.D.
Adult: Caren Behar, M.D.
Beth Israel Medical Center
Cystic Fibrosis Center (Pediatric
and Adult)
1st Ave. & 16th St.
Bernstein Building, 7th Floor
New York, NY 10003
(212) 420-4100
Pediatric: Maria N. Berdella, M.D.
Adult: Patricia A. Walker, M.D.
Children’s Hospital of New York
Columbia University (Pediatric)
Children’s Lung and Cystic
Fibrosis Center
3959 Broadway, 7 Central
New York, NY 10032
(212) 305-5122
Meyer Kattan, M.D.
Columbia University Medical
Center (Adult)
Gunnar Esiason Adult Cystic
Fibrosis and Lung Program
622 West 168th St., PH 859
New York, NY 10032
(212) 305-0290
Emily DiMango, M.D.
This listing is current as of June
2012. For the most up-to-date
list of care centers, visit the
CF Foundation’s website
(www.cff.org) or call
1-800-FIGHT CF.
214 • CF FOUNDATION–ACCREDITED CARE CENTERS
Mount Sinai School of Medicine
(Pediatric)
Pediatric Pulmonary Division
Box 1202B
One Gustave L. Levy Place
New York, NY 10029
(212) 241-7788
Richard. J. Bonforte, M.D.
Andrew Ting, M.D.
New York University Medical
Center (Pediatric and Adult)
NYU Fink Ambulatory Care
Center
160 East 32nd Street
L-3 Medical
New York, NY 10016
(212) 263-5940
Robert Giusti, M.D.
University of Rochester Medical
Center Strong Memorial
Hospital (Pediatric)
601 Elmwood Ave., Box 667
Rochester, NY 14642
(585) 275-2464
Karen Z. Voter, M.D.
Clement L. Ren, M.D.
University of Rochester Medical
Center Strong Memorial
Hospital (Adult)
913 Culver Rd.
Rochester, NY 14609
(585) 654-5432
Robert Horowitz, M.D.
SUNY Upstate Medical
University (Pediatric and Adult)
750 E. Adams St.
Syracuse, NY 13210
(315) 464-6323
Pediatric: Ran D. Anbar, M.D.
North Carolina
Mission Children’s Clinic
(Pediatric)
11 Vanderbilt Park Drive
Asheville, NC 28803
(828) 213-1740
Bruce K. Bacot, M.D.
Asthma & Allergy Specialists, PA
(Pediatric)
8045 Providence Rd., Suite 300
Charlotte, NC 27277
(704) 341-9600
William Ashe, M.D.
Hugh R. Black, M.D.
University of North Carolina at
Chapel Hill (Pediatric)
NC Children’s Hospital
UNC Chapel Hill
101 Manning Drive
Chapel Hill, NC 27514
(919) 966-1401
Margaret W. Leigh, M.D.
George Retsch-Bogart, M.D.
University of North Carolina at
Chapel Hill (Adult)
Adult Cystic Fibrosis Clinic
UNC Pulmonary Clinic
3400 Ambulatory Care Clinic
Mason Farm Rd., CB#7705
Chapel Hill, NC 27599
(919) 966-6838
Michael R. Knowles, M.D.
James R. Yankaskas, M.D.
Adult: James Sexton, M.D.
Duke University Medical Center
(Pediatric)
2301 Erwin Rd.
Durham, NC 27710
(919) 684-3364
Judith Voynow, M.D.
Thomas Miles Murphy, M.D.
Samaritan Medical Center
(Pediatric and Adult)
830 Washington St.
Watertown, NY 13601
(315) 786-0254
Melynne Youngblood, M.D.
Duke University Medical Center
(Adult)
3116 North Duke St.
Durham, NC 27704
(919) 668-7360
Peter S. Kussin, M.D.
CF FOUNDATION–ACCREDITED CARE CENTERS • 215
Wake Forest University Baptist
Medical Center (Pediatric and
Adult)
Medical Center Blvd.
Winston Salem, NC 27157
Pediatric: (336) 713-4500
Karl H. Karlson, Jr., M.D.
University of Cincinnati Medical
Center (Adult)
University Hospital
234 Goodman Ave.
Cincinnati, OH 45219
(513) 475-8523
Patricia M. Joseph, M.D.
Adult: (336) 716-4843
Victor Ortega, M.D.
University Hospitals Case
Medical Center (Pediatric and
Adult)
Rainbow Babies and Children’s
Hospital
11100 Euclid Ave.
Cleveland, OH 44106
(216) 844-7700
Pediatric: Michael W. Konstan,
M.D.
North Dakota
St. Alexis Heart and Lung Clinic
(Pediatric and Adult)
310 N. 10th St.
Bismarck, ND 58501
(701) 530-7500
Pediatric: James A. Hughes, M.D.
Adult: Carla Zacher, M.D., F.A.A.P.
MeritCare Medical Center
(Pediatric)
801 North Broadway
Fargo, ND 58122
(701) 234-6600
Stephen Tinguely, M.D.
Ohio
Children’s Hospital Medical
Center of Akron (Pediatric and
Adult)
Lewis H. Walker Cystic Fibrosis
Center
1 Perkins Square
Akron, OH 44308
(330) 543-3249
Pediatric: Nathan C. Kraynack,
M.D.
Adult: Steven D. Strausbaugh,
M.D. F.C.C.P.
Nationwide Children’s Hospital
(Pediatric and Adult)
700 Children’s Drive
Columbus, OH 43205
(614) 722-4766
Pediatric: Karen S. McCoy, M.D.
Adult: John Heintz, M.D.,
Alpa Patel, M.D.
The Children’s Medical Center of
Dayton/Wright State University
School of Medicine (Pediatric
and Adult)
Children’s Medical Center
One Children’s Plaza
Dayton, OH 45404
(937) 641-3440
Pediatric: Robert J. Fink, M.D.
Adult: Titus Sheers, M.D.
Adult: Gary M. Onady, M.D., Ph.D.
Cincinnati Children’s Hospital
Medical Center (Pediatric)
3333 Burnet Ave.
Cincinnati, OH 45229
(513) 636-6627
Gary McPhail, M.D.
Northwest Ohio Cystic Fibrosis
Center (Pediatric and Adult)
CF Care Center
2121 Hughes Drive, Suite 640
Toledo, OH 43606
Pediatric: (419) 291-2207
Pierre A. Vauthy, M.D.
Adult: (419) 291-4626
Jeffrey Lewis, M.D.
This listing is current as of June
2012. For the most up-to-date
list of care centers, visit the
CF Foundation’s website
(www.cff.org) or call
1-800-FIGHT CF.
216 • CF FOUNDATION–ACCREDITED CARE CENTERS
Oklahoma
Pennsylvania
Oklahoma Cystic Fibrosis Center
(Pediatric and Adult)
Pediatric Pulmonary and Cystic
Fibrosis Clinic
1200 Phillips Ave., OUCPB
Suite 14101
Oklahoma City, OK 73104
(405) 271-6390
Pediatric: James Royall, M.D.
Lehigh Valley Hospital & Health
Adult: Kellie Jones, M.D.
Oklahoma Cystic Fibrosis
Center–Tulsa (Pediatric and
Adult)
OU Physicians - Pediatrics
444 E. 41st St., 2nd floor
Tulsa, OK 74135
(918) 502-2000
Joseph Walter, M.D.
Oregon
Oregon Health Sciences
University (Pediatric)
OHSU Pediatric Cystic Fibrosis
Center
Dept. of Pediatrics
707 SW Gaines, L106
Portland, OR, 97239
(503) 494-8023
Michael Wall, M.D.
Oregon Health Sciences
University (Adult)
Physicians Pavilion
3181 SW Sam Jackson Park Road
UHN 67
Adult Cystic Fibrosis Clinic
Suite 320
Portland, OR 97239
(503) 494-1620
Gopal Allada, M.D.
Kaiser Permanente Northwest
Region (Pediatric and Adult)
3550 N. Interstate Ave.
Portland, OR 97227
(503) 813-2000
Richard. C. Cohen, M.D.
Network (Pediatric and Adult)
Pediatric Specialty Center
2545 Schoenersville Rd.
3rd Floor
Bethlehem, PA 18017
(610) 402-3844
Robert W. Miller, M.D.
Dharmesh Suratwala, M.D.,
M.B.B.A.
Saint Luke’s Hospital (Outreach
CF Clinic)
Route 512 and Broadhead
Leigh Valley Industrial Park
Bethlehem, PA 18017
(610) 954-4975
Laurie Varlotta, M.D.
Geisinger Medical Center
(Pediatric)
100 N. Academy Ave.
Danville, PA 17822
(888) 675-5437
Carlos Perez, M.D.
Hershey Medical Center
Pennsylvania State University
(Pediatric and Adult)
500 University Drive
Hershey, PA 17033
Pediatric: (717) 531-5412
Gavin R. Graff, M.D.
Adult: (717) 531-6525
Robert L. Vender, M.D.
Children’s Hospital of
Philadelphia University of
Pennsylvania (Pediatric)
3615 Civic Center Blvd.
Philadelphia, PA, 19104
(215) 590-3749
Ronald Rubenstein, M.D., Ph.D.
CF FOUNDATION–ACCREDITED CARE CENTERS • 217
University of Pennsylvania
Hospital (Adult)
Perelman Center for Advanced
Medicine
3400 Civic Center Blvd.
Philadelphia, PA, 19104
(215) 662-3202
Denis Hadjiliadis, M.D., M.H.S.
Drexel University College of
Medicine, St. Christopher’s
Hospital for Children (Pediatric)
Erie at Front St., Suite 2215
Philadelphia, PA 19134
(215) 427-5183
Laurie Varlotta, M.D.
Drexel University of Medicine,
Hahnemann University Hospital
(Adult)
Drexel Adult CF Center
219 N. Broad. St., 9th Floor
Philadelphia, PA 19107
(215) 762-2688
Jeffrey Hoag, M.D., M.S., FCCP
Children’s Hospital of Pittsburgh
of UPMC–University of
Pittsburgh (Pediatric)
4401 Penn Ave.
3rd Floor, Main Building
Pittsburgh, PA 15224
(412) 692-5630
David M. Orenstein, M.D.
Daniel Weiner, M.D.
University of Pittsburgh School
of Medicine (Adult)
4th Floor, Falk Medical Building
Comprehensive Lung Center
3601 Fifth Ave.
Pittsburgh, PA 15213
(412) 648-6161
Joel H. Weinberg, M.D.
Joseph M. Pilewski, M.D.
Rhode Island
Brown University Medical School
Rhode Island Hospital Cystic
Fibrosis Center (Pediatric and
Adult)
Hasbro Children’s Hospital
Respiratory and Immunology
Center
593 Eddy St., Hasbro Lower Level
Providence, RI 02903
(401) 444-6540
Pediatric: Karen Daigle, M.D.
Adult: Walter Donat, M.D.
South Carolina
Medical University of South
Carolina (Pediatric)
MUSC Pediatric Pulmonology
135 Rutledge Ave., Suite 279
Charleston, SC 29425
(843) 876-0444
Isabel Virella-Lowell, M.D.
Medical University of South
Carolina (Adult)
Pulmonology and Critical Care
Medicine
96 Jonathan Lucas St., Suite 812
CSB
Charleston, SC 29425
(843) 792-0729
Patrick A. Flume, M.D.
University of South Carolina
(Pediatric)
9 Medical Park, Suite 505
Columbia, SC 29203
(803) 434-2505
Daniel C. Brown, M.D.
Greenville Hospital System
University Medical Group
(Pediatric)
200 Patewood Dr.
Suite A300
GHS UMG Pediatric Pulmonology
Greenville, SC 29615
(864) 454-5530
Jane Vance Gwinn, M.D.
This listing is current as of June
2012. For the most up-to-date
list of care centers, visit the
CF Foundation’s website
(www.cff.org) or call
1-800-FIGHT CF.
218 • CF FOUNDATION–ACCREDITED CARE CENTERS
South Dakota
Sanford USD Medical Center
(Pediatric)
Sanford Children’s Specialty
Clinic
1600 W. 22nd St.
Sioux Falls, SD 57117
(605) 312-1000
James Wallace, M.D.
Sanford USD Medical Center
(Adult)
1100 South Euclid Ave.
Sioux Falls, SD 57117
(605) 312-1000
Susan Rohr, M.D.
David Thomas, M.D.
Tennessee
T.C. Thompson Children’s
Hospital (Pediatric)
910 Blackford St.
Chattanooga, TN 37403
(423) 778-6501
Joel C. Ledbetter, M.D.
East Tennessee Children’s
Hospital (Pediatric)
2100 W. Clinch Ave.
Medical Office Building
Suite 310
Knoxville, TN 37916 (865) 541-8830
John Rogers, M.D.
Eduardo Riff, M.D.
University of Tennessee Medical
Center (Adult)
University Pulmonary and Critical
Care
1940 Alcoa Hwy, Ste. E-210
Knoxville, TN 37920
(865) 305-5888
Bruce Ludwig, M.D.
University of Tennessee Medical
Center (Pediatric)
Pediatric Pulmonary Medicine
Physician’s Office Building
777 Washington Ave. Suite P110
Memphis, TN 38103
(901) 287-5222
Dennis Stokes, M.D.
University of Tennessee Adult
CF Program (Adult)
880 Madison
5th Floor, Suite A&B
Memphis, TN 38104
(865) 305-5888
Luis Murillo, M.D.
Vanderbilt Children’s Hospital
Vanderbilt University Medical
Center (Pediatric)
2200 Children’s Way
11215 Doctors’ Office Tower
Nashville, TN 37232
(615) 343-7617
Rebekah Brown, M.D.
Vanderbilt University Medical
Center (Adult)
1301 Medical Center Drive
Suite B-817, TVC
Nashville, TN 37232
(615) 322-2386
Bonnie S. Slovis, M.D.
Texas
Dell Children’s Medical Center
of Central Texas (Pediatric)
4900 Mueller Blvd.
Austin, TX 78723
(512) 324-0137
Bennie McWilliams, M.D.
Allan Frank, M.D.
Dell Children’s Medical Center
of Central Texas/UTSW Austin
(Adult)
3305 Northland Dr., Suite 512
(512) 324-0137
Jason Fullmer, M.D.
CF FOUNDATION–ACCREDITED CARE CENTERS • 219
Children’s Medical Center
of Dallas/University of Texas
Southwestern (Pediatric and
Adult)
1935 Medical District Drive
Dallas, TX 75235
(214) 456-2361
Pediatric: Carolyn Cannon, M.D.,
Ph.D.
Adult: Raksha Jain, M.D.
Tri-Services Military Cystic
Fibrosis Center–FOR MILITARY
PERSONNEL ONLY
(Pediatric and Adult)
3851 Roger Brooke Drive
Fort Sam Houston, TX 78234
(210) 916-4927
Pediatric: John M. Palmer, M.D.
Adult: Catherine Shoff, M.D.
Cook Children’s Medical Center
(Pediatric and Adult)
901 Seventh Ave., Suite 420
Fort Worth TX 76104
(682) 885-6299
Pediatric: James C. Cunningham,
M.D.
Nancy L. Dambro, M.D.
Adult: John Burk, M.D.
Steven Q. Davis, M.D., M.S.,
F.C.C.P.
Baylor College of Medicine/
Texas Children’s Hospital
(Pediatric)
Pulmonary Medicine Service
6701 Fannin St., Ste. 1040
Houston, TX 77030
(832) 822-2778
Christopher M. Oermann, M.D.
Peter Hiatt, M.D.
Baylor College of Medicine/
Baylor Clinic/The Methodist
Hospital (Adult)
6620 Main, Ste. 11B.15
Houston, TX 77030
(713) 798-2400
Marcia Katz, M.D.
Texas Tech University Health
Sciences Center (Pediatric and
Adult)
Texas Tech Physicians Pediatrics
3601 4th St., Mail Stop 9903
Lubbock, TX 79430
806-743-7336
Adaobi Kanu, M.D.
Christus Santa Rosa Children’s
Hospital (Pediatric and Adult)
David Goldsbury Center for
Children and Families
Cystic Fibrosis Clinic
Second Floor
333 North Santa Rosa
San Antonio, TX 78207
Pediatric: (210) 704-2338
Donna Beth Willey-Courand,
M.D.
Adult: (210) 223-3010
Pedro Sepulveda, M.D.
Children’s Hospital at Scott &
White (Pediatric and Adult)
2401 South 31st St., Building 27
Temple, TX 76508
(254) 724-5504
Alma Chavez, M.D.
University of Texas Health
Center at Tyler (Pediatric and
Adult)
11937 US Highway 271
Tyler, TX 75708
(903) 877-5270
Rudolfo Amaro, M.D.
Utah
Intermountain Cystic Fibrosis
Center University of Utah Health
Sciences Center (Adult)
University Hospital and Clinics
50 North Medical Drive - Clinic 3
Salt Lake City, UT 84132
(801) 585-2804
Ted G. Liou, M.D.
Holly Carveth, M.D.
This listing is current as of June
2012. For the most up-to-date
list of care centers, visit the
CF Foundation’s website
(www.cff.org) or call
1-800-FIGHT CF.
220 • CF FOUNDATION–ACCREDITED CARE CENTERS
Intermountain Cystic Fibrosis
Center University of Utah Health
Sciences Center (Pediatric)
Pulmonology Division
100 N. Mario Capecchi Drive
Salt Lake City, UT 84113
(801) 662-1765
Barbara Chatfield, M.D.
Vermont
Vermont Children’s Hospital
(Pediatric and Adult)
Children’s Specialty Center
Fletcher Allen Health Care
111 Colchester Ave.
ACC 4th Floor, East Pavillion
Burlington, VT 05401
Pediatric: (802) 847-8600
Thomas Lahiri, M.D.
Adult: (802) 847-1158
Laurie A. Leclair, M.D.
Virginia
University of Virginia (Pediatric)
2270 Ivy Rd.
Charlottesville, VA 22903
(434) 924-2250
Deborah K. Froh, M.D.
University of Virginia (Adult)
1220 Lee St.
Charlottesville, VA 22908
(434) 924-5219
Cynthia Brown, M.D.
Veronica Indihar, M.D.
Pediatric Lung Center (Pediatric
and Adult)
2730A Prosperity Ave.
Fairfax, VA 22031
(703) 289-1410
John Osborn, M.D.
Children’s Hospital of the King’s
Daughters Eastern Virginia
Medical School (Pediatric and
Adult)
601 Children’s Ln.
Norfolk, Va. 23507
(757) 668-7137
Pediatric: Cynthia A. Epstein,
M.D.
Adult: Ignacio Ripoll, M.D.
Naval Medical Center,
Portsmouth–FOR MILITARY
PERSONNEL ONLY (Pediatric
and Adult)
Cystic Fibrosis Center
Department of Pediatrics
620 John Paul Jones Circle
Portsmouth, VA 23708
(757) 953-2955
Lori Vanscoy, M.D.
Nam Ly, M.D.
Virginia Commonwealth
University (Pediatric and Adult)
Nelson Clinic 1st Floor
403 N. 11th St.
Richmond, VA 23298
(804) 828-2982
H. Joel Schmidt, M.D.
Washington
Seattle Children’s Hospital
(Pediatric)
4800 Sand Point Way NE
M/SA-5921
Seattle, WA 98105
(206) 987-2024
Ronald L. Gibson, M.D., Ph.D.
University of Washington
Medical Center (Adult)
Medicine Subspecialties Clinic
1959 NE Pacific St.
Seattle, WA 98195
(206) 598-4215
Moira L. Aitken, M.D.
CF FOUNDATION–ACCREDITED CARE CENTERS • 221
Pediatric Pulmonary and CF
Clinic (Pediatric and Adult)
105 W 8th Ave., Suite 660E
Spokane, WA 99204
(509) 474-6960
Michael M. McCarthy, M.D.
Gundersen Lutheran Medical
Center (Pediatric and Adult)
1900 South Ave.
La Crosse, WI 54601
(608) 775-4143
Todd Mahr, M.D.
Mary Bridge Children’s Health
Center (Pediatric and Adult)
311 So. L St., M/S 311-01-0C
Tacoma, WA 98415
(253) 403-3131
Lawrence A. Larson, D.O.
David H. Ricker, M.D.
University of Wisconsin
(Pediatric)
American Family Children’s
Hospital
1675 Highland Ave.
Madison, WI 53792
(608) 263-6420
Michael J. Rock, M.D.
Madigan Army Medical Center–
FOR MILITARY PERSONNEL
ONLY (Pediatric and Adult)
9040 A Fitzsimmons Drive
Tacoma, WA 98431
(253) 968-1878
Donald Moffitt, M.D.
West Virginia
West Virginia University
Charleston Division (Pediatric
and Adult)
830 Pennsylvania Ave., Suite 104
Charleston, WV 25302
(304) 388-1552
Raheel Khan, M.D., F.A.A.P.
Kevin Mupin, M.D.
West Virginia University CF
Center (Pediatric)
1 Stadium Dr., POC
Morgantown, WV 26507
(304) 598-4835
Kathryn S. Moffett, M.D.
Wisconsin
Saint Vincent’s Hospital
(Pediatric and Adult)
835 S. Van Buren St.
Green Bay, WI 54301
(920) 433-8508
Peter Holzwarth, M.D.
University of Wisconsin (Adult)
600 Highland Ave.
Madison, WI 53792
(608) 263-7203
Guillermo A. doPico, M.D.
Keith Meyer, M.D.
Marshfield Clinic (Pediatric and
Adult)
1000 North Oak Ave.
Marshfield, WI 54449
(715) 387-5251
Keith Pulvermacher, M.D.
Children’s Hospital of Wisconsin
Froedtert & Medical College of
Wisconsin (Pediatric)
9000 W. Wisconsin Ave.
PO Box 1997, MS B620
Milwaukee, WI 53201
(414) 266-6730
Diana Quintero, M.D.
Froedtert & Medical College of
Wisconsin (Adult)
9200 W. Wisconsin Ave.
2PV- Pulmonary Clinic
Milwaukee, WI, 53226
(414) 805-9400
Julie A. Biller, M.D.
This listing is current as of June
2012. For the most up-to-date
list of care centers, visit the
CF Foundation’s website
(www.cff.org) or call
1-800-FIGHT CF.
ACKNOWLEDGMENTS
The Cystic Fibrosis Foundation wishes to thank the people with
CF and their families and the CF care centers who contributed
photos to this book.
Project Managers:
Leslie Hazle, M.S., R.N., Director of Patient Resources, CF Foundation
Melissa Chin, B.A., Clinical Programs Specialist, CF Foundation
CF Foundation Editorial Support:
Ashling Knight
Marybeth McMahon, Ph.D.
Leslie Rigby
Design:
Joan Christie Design
Medical Illustrator:
Litwak Illustration Studio
Photography:
Cade Martin Photography
Dakota Fine
Doug Gritzmacher
Paul Nordmann
This information meets the CF Foundation guidelines and standards for
education materials as reviewed by the Cystic Fibrosis Foundation
Education Committee.
2012 CF Foundation Education Committee Members:
Lauren Ahrens, B.S.N., R.N.; Nancy Brager, M.D., F.R.C.P.C.;
Cindy Capen, M.S.N., R.N.; Abigail Dwyer; Laura Grande, M.S., R.D., L.D.;
Karen Boren Harville, L.C.S.W., C.C.L.S.; Crystal Holdeman;
Kathleen Lemanek, Ph.D.; Paula Lomas, R.N., M.A.S., C.C.R.P.;
Susan Butler McNamara, M.M.Sc, P.T., C.C.S.; Lisa Monchil, R.RT-NPS, C.C.R.C.,
C.C.M., A.E-C., C.P.F.T.; Sarah Phillips, M.S., R.D., L.D., C.D.E.;
Adrienne Prestridge, M.D.; Mary Sachs R.N., C.N.P., M.S.N.;
Veronica Wetmore, R.N., M.S.
This publication was made possible by a generous
unrestricted grant from Aptalis Pharma Inc.
Cystic Fibrosis Foundation
6931 Arlington Road,
Suite 200
Bethesda, Maryland 20814
1.800.FIGHT.CF
www.cff.org
This publication was made
possible by a generous
unrestricted grant from
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