Patient

Registry
Annual Data Report

2012

The CF Foundation wishes to thank

the people with CF and their families who
contributed their photos to this report.
Photography by
Cade Martin Photography
SOURCE OF DATA
Cystic fibrosis patients under care at CF Foundationaccredited care centers in the United States, who
consented to have their data entered in 2012.
SUGGESTED CITATION
Cystic Fibrosis Foundation Patient Registry
2012 Annual Data Report
Bethesda, Maryland
2013 Cystic Fibrosis Foundation

If you have any questions about

CF care, please talk with your CF care
center or contact the Foundation at
1-800 FIGHT CF or info@cff.org.

To the CF Community and Friends,

Each year, the Cystic Fibrosis Foundation collects information on the health of the more
than 27,000 people with CF who receive care at Foundation-accredited centers nationwide.
The Foundation shares this information with the wider community through the Patient
Registry Annual Data Report, which highlights progress made in cystic fibrosis (CF) care
and areas where more work is needed.
The data in this report, collected through 2012, shows that the outlook for people with CF
continues to improve. Here are some highlights:
The median predicted age of survival continues to rise.
Adults and children with CF are experiencing better overall lung function and
improved nutrition and growth.
Thanks to nationwide newborn screening, more CF diagnoses are now made in the
first year of life.
Nearly half of all people with CF are now ages 18 and older.
These steady gains reflect the vital partnerships among all members of the CF care
team people with CF, their families and care center staff. We hope the information
in this report helps strengthen these partnerships by serving as a tool for shared learning
and open communication throughout the CF community.
We encourage you to use this report to start a conversation with your CF care center.
Your input is key to continued advances in CF care and improved health for those living
with the disease.
We are deeply grateful to the people with CF and their families who generously agree to
share their data. We look forward to working together in the year ahead.
Sincerely,

Bruce C. Marshall, M.D.

Senior Vice President of Clinical Affairs

Leslie Hazle, M.S., R.N.

Director of Patient Resources

HOW DO I USE THE REGISTRY REPORT?

For people with cystic fibrosis (CF) and their families, this report can help you talk with
the care center about your or your childs health. Some questions you may want to ask are:
What does this report mean to me?
Am I getting the recommended CF care, such as the right tests or medications?
How does my health compare with the health of others with CF?
What can I do to improve my health?
How can I get even better care?
What would ideal CF care look like?
What works really well at my care center?
What is my center doing to improve CF care?
How can I help?
For volunteers and donors, this report shows how your support is helping improve the
health and quality of life of people with CF in the United States.

KEY WORDS IN THE REGISTRY DATA

To help you understand what the Patient Registry data means, here are definitions of some
key words used in this report.
Median: The median is the middle. That means half the people with CF in this
group are above this number and half are below.
Percent: A percent is one part in a hundred. For example, 60 percent means that
there are 60 out of 100. If 95 percent of people with CF have their flu shot that
means 95 people out of 100 got their flu shot. This is sometimes called a percentage.
BMI: Body mass index or BMI is based on a persons weight and height. It is used to
screen for a weight that may be too low or too high and may lead to health problems.
BMI is calculated by dividing body weight in kilograms (kg) by the persons height in
meters squared (m2).
BMI percentile: BMI percentile matches one childs BMI to the BMI of other
children in the United States who are the same age and gender. A BMI of the 50th
percentile means the child is in the middle. Half of the children of the same age and
gender are larger and half are smaller.
F
 EV1 percent predicted: FEV1 is one measure of lung function. It measures
the forced exhaled volume of air in the first second. It is shown as a percent
predicted, based on the FEV1 of healthy, non-smoking people of the same age,
height and gender.

Table of Contents
ABOUT Cystic Fibrosis AND THE CF Foundation
What Is Cystic Fibrosis?
What Is the Cystic Fibrosis Foundation?

4
4
4

What Is the Cystic Fibrosis FoundationS Patient Registry?

Overview of the Health of People with CF

Median Predicted Survival Age
Age at Diagnosis
Number of Clinic Visits in the First Year of Life
Adults With CF
Number of Children and Adults with CF
Characteristics of Adults with CF
CF and Fertility
CF Genetics
Most Common CF Gene Mutations

Meeting the Goals of CF Care

Goal 1: Full Members of the CF Care Team
CF Care Guidelines
Sample Patient Summary Report
Care, Screening and Prevention Guidelines
Goal 2: Normal Growth and Nutrition
Nutrition for Children and Teens
Median BMI Percentile
Nutrition for Adults
Median BMI
Nutrition and Lung Function
FEV1 Percent Predicted vs. BMI Percentile for Children 6 to 19 Years
FEV1 Percent Predicted vs. BMI for Adults 20 to 40 Years
Goal 3: Maintain Lung Health
Median FEV1 Percent Predicted by Age
People with CF with Normal/Mild Lung Disease
Lung Function in 18 Year Olds
Lung Health Research
Therapies for Lung Health
People with CF Who Were Prescribed CF Medications
Primary Airway Clearance by Age
Keeping Lungs Healthy
Tobacco Smoke Exposure by Age
Goal 4: Reduce Lung Germs
Germs Found in the Lungs by Age
Goal 5: Complications Related to CF
Common Complications by Age
Other Problems Related to CF
Goal 6: Transplantation and End-of-Life Care
Number of People with CF Who Had a Lung Transplant by Year
Goal 7: Access to Care
People with CF by Type of Health Insurance Coverage

Summary TABLES
Summary of the Data
Number of People with CF by State

6
6
7
8
8
9
9
10
10
11
12
12
12
13
14
14
14
15
15
15
16
16
16
17
17
17
18
18
19
19
20
20
21
22
22
23
24
24
25
25
26
26
27
27
28

About Cystic Fibrosis and the CF Foundation

What Is Cystic Fibrosis?

Cystic fibrosis (CF) is a lifelong genetic disease that causes mucus to build up and clog some
of the bodys organs. In people with the disease, two mutations in the CF gene lead to a
protein, called CFTR, that does not work right. This defect in the protein causes mucus to be
thick and sticky. The buildup of mucus leads to problems in the lungs and digestive system.
People with CF often get lung infections and have inflammation or swelling in the lungs.
Over time, these problems lead to lung damage. CF can also make it hard for the body to
get nutrients from food. It is hard for some people with CF to grow and have a healthy
body weight.
CF is found in people of all racial and ethnic groups. Each year, about one in 3,500 babies
born in the United States has CF. It is more common in white people (Caucasians) than any
other race. About 30,000 people in the United States have CF, and about 70,000 worldwide.

What Is the Cystic Fibrosis Foundation?

The CF Foundation was started in 1955 by parents of children with CF. The mission of
the Foundation is to find a cure for CF and to improve the quality of life for people with
the disease.
The Foundation accredits a network of more than 110 CF care centers across the United
States to meet the health care needs of people with CF. The Foundation provides these care
centers with:
Patient Registry data to track the health of people with CF.
CF care guidelines based on research published in medical journals.
Education and advocacy resources for people with CF and their families.
Updates on CF care and research at the annual North American CF Conference.
Training in quality improvement.
Grants to give some funding for care and research.
The Foundation also supports the work of researchers to discover and develop new therapies
to improve the length and quality of life for those with the disease. Developing new CF
drugs takes time and a lot of money. To help speed development of potential new therapies,
the Foundation funds promising research to target the disease from all angles. A research
priority of the Foundation is to find drugs that target the underlying cause of CF, the CFTR
protein that does not work right.
To learn more about different CF drugs in development, visit the Foundations website
at www.cff.org/research/DrugDevelopmentPipeline. You can also learn more about cystic
fibrosis, the Foundation, its accredited care centers, guidelines and research at www.cff.org.

What Is the Cystic Fibrosis Foundations

Patient Registry?
The Cystic Fibrosis Foundations Patient Registry tracks the health and treatments of people
with CF across the United States. Information is collected every year on close to 28,000 people
who get care at CF Foundation-accredited care centers (adult, pediatric and affiliate programs)
and who agree to take part in the Registry. The data include state of residence, height, weight,
gender, CF mutations, lung (pulmonary) function test (PFT) results, medication use and
problems (complications) related to CF.
The Patient Registry gives health care professionals and researchers information they need to:
Improve the delivery of care.
Study the effects of treatments on people with CF.
Develop care guidelines.
Design clinical trials to test new drugs and therapies.
The Registry also gives people with CF and their families a look at the overall health of those
living with CF. It is vital for everyone with CF to agree to have their data entered into the
Registry. The participation of all people with CF means data are more complete and helps
create a better picture of the current health of people with CF. These data can also be used to
find areas where improvements can be made.
The CF Foundation has set seven goals to help improve the health of people with CF. This
report highlights these goals and the work being done to meet them. The goals are:
Goal 1:  People with CF and their families will be full members of the care team.

Communication will be open so everyone can be involved in care decisions. Care

will be respectful of the person with CFs needs, preferences and values.
Goal 2:  Children, adolescents and adults with CF will have normal growth and nutrition.
Goal 3:  People with CF will receive appropriate therapies for maintaining lung function.

Pulmonary exacerbations will be detected early and treated aggressively to return

people with CF to their previous levels of lung function.
Goal 4:  People with CF, their families and CF care center staff will be well-informed and

active partners in reducing the spread of germs, including Pseudomonas aeruginosa

(Pseudomonas) and Burkholderia cepacia (B. cepacia) complex.
Goal 5:  People with CF will be screened and managed aggressively for complications of the

disease, particularly CF-related diabetes (CFRD).

Goal 6:  People with CF and their families will be supported by their CF care center when

facing decisions about transplantation and end-of-life care.

Goal 7:  People with CF and their families will have access to appropriate therapies,

treatments and support regardless of race, age, education or ability to pay.

OverVIEW OF THE Health of People with CF

The Patient Registry is a tool used to measure improvements in the health and survival of
people with CF in the United States, from diagnosis through adulthood. For an overview about
people with CF in the Patient Registry, including race, gender and age range, see the Summary
of the Data on page 27. To learn how many people with CF are in each state, see page 28.
The median predicted age of survival for people with CF has risen steadily over the last 25
years. Since 2002, the median predicted survival age has increased by almost 10 years
from age 31.3 in 2002 to age 41.1 in 2012.
The chart below shows the rise in median predicted survival age since 1988 in 5-year bands
or groups. For 2008-2012, the median predicted survival was 37.8 years.
Median predicted survival is calculated using a method called life table analysis. This is best
known for its use in the life insurance industry. Using this calculation, half of the people in
the Patient Registry are expected to live beyond the median predicted survival age, and half are
expected to live less than the median predicted survival age.
Year-to-year differences are normal when measuring health outcomes for any disease or medical
condition. What is important is that, over time, survival is going up.
Median Predicted Survival Age, 1988-2012

In 5-Year Bands

Median Predicted Survival (Age in Years)

40

36

32

28

24
1988-1992

1993-1997

1998-2002
Years

2003-2007

2008-2012

The graph below shows all people in the Registry and their age at the time of diagnosis with
cystic fibrosis. Most people with CF are diagnosed before the age of 2 years. However, CF
can be diagnosed at any age. Today, many people with CF are diagnosed shortly after birth
because of newborn screening. But, each year some children and adults are still diagnosed
because of symptoms.
In 2012, 52 people over 40 years old were diagnosed with CF. The oldest person diagnosed with
CF in 2012 was 76 years old.
You can learn more about the testing for CF at www.cff.org/AboutCF/Testing.
Age at Diagnosis of All People with CF, 2012
8,000

Number of People with CF

7,000
6,000
5,000
4,000
3,000
2,000
1,000
0

Pre- 01 13 46 711 1
2
3
4
5
6
7
8
9 10 11 12 13 14 15 16 21 31 over
natal _________________ ________________________________________________________________________________________
20 30 40 40
Months
Years

Age

Early diagnosis may play an important role in improving survival. Since 2010, all 50
states and the District of Columbia screen all newborns for CF. Research shows that
people with CF who are diagnosed because of newborn screening have better weight
and healthier lungs later in life than those diagnosed because of symptoms.
More than 61 percent of people with CF who were diagnosed in 2012 were found
because of an abnormal newborn screen. The earlier CF is diagnosed, the sooner
treatment can begin.

The CF Foundation has

information to help parents
and families of newborns at
www.cff.org/AboutCF/
Testing/NewbornScreening.

To help babies with CF grow well, the Foundations CF infant care guidelines state
that infants with CF should go to the CF care center every month during the first 6 months of
life, and every one to two months in the second 6 months. The graph on the next page shows the
number of times infants with CF go to CF clinic in the first 12 months of life.
R
 ead the Foundations infant care guidelines at www.cff.org/treatments/CFCareGuidelines/
AgeSpecificCare.

Number of CF Clinic Visits in the First Year of Life for Babies Born with CF in 2011 and
Diagnosed through Newborn Screening
90
Number of People with CF

80
70
60
50
40
30
20
10
0
0

10

11

12

13

14

15

16

17

18

19

20

21

Number of Clinic Visits

The Centers for Disease

Control and Prevention
growth charts at www.cdc.
gov/growthcharts
show that infants should
increase their weight to
three times their birth
weight by 1 year of age.

Research shows that some infants with CF may not grow well even with early
diagnosis and treatment. The Foundation is supporting research to find out why.
For example, the Baby Observational and Nutrition Study (BONUS) is looking
at what might get in the way of good growth for a baby with CF.
Learn more about this and other research for infants with CF at
www.cff.org/research/ClinicalResearch/Find; click on Advanced Search
and type in keyword infant.
Watch CF Infant Care: First Year of Life on the Foundations web site at
www.cff.org/LivingWithCF/StayingHealthy/CFInfantCare.

ADULTS WITH CF

The graph at the top of the next page shows how fast the number of adults with CF in the
Patient Registry has grown in relation to the number of children with CF in 1986-2012. The
blue line shows the number of adults by year. The green line shows the number of children.
In 1986, 29.2 percent of people with CF were 18 years of age and older. In 2012, over 49
percent were adults. The growing number of adults with CF means that people with CF are
healthier and living longer than ever before.
It is likely that there will soon be more adults with CF in the United States than children with
the disease. Many people with CF are living well into adulthood. In 2002, the oldest person
with CF was 75 years old. In 2012, the oldest person living with CF was 82 years old.
The Foundation continues to support the care of adults with CF and is helping more doctors
learn about adult CF care.
Find Foundation-accredited CF care centers that care for adults at www.cff.org.
Read the guidelines on adult CF care at www.cff.org/treatments/CFCareGuidelines/
AgeSpecificCare.
8

Number of Children and Adults with CF, 1986-2012

Number of People with CF

15,000

49.1%

12,000

9,000

6,000

3,000

Children Under 18 Years

Adults 18 Years and Older

29.2%

0
86 87 88 89 90 91 92 93 94 95 96 97 98 99 00 01 02 03 04 05 06 07 08 09 10 11 12
Year

The charts below show that many adults with CF are leading active lives going to college,
getting married and working. To learn more about how adults with CF are living active lives,
visit the For Adults with CF pages at www.cff.org/Adults.

t Characteristics of Adults with CF 18 Years of Age and Older, 2012

Education

Employment
Part-time
11.8%

Some College
33.7%
Marital Status
High School
Diploma
24.4%

Less than
High School
7.1%

College
Graduate
29.1%

Masters/Doctoral
Level Degree
5.7%

Full-time
33.6%

Married/Living
Together
39.8%
Single
54.9%

Retired
1.3%

Disabled
17.0%

Student
23.5%

Homemaker
4.3%
Unemployed
8.4%

Separated/Divorced 5.0%
Widowed 0.3%

CF Foundation-accredited care centers can help teach children, teens and adults how to manage
their daily CF care. It is important for teens with CF to learn how to take charge of their own
health as they move into adulthood.
Learn more about adult CF care and moving from pediatric to adult care in the Partnering
for Care webcast series at www.cff.org/LivingWithCF/Webcasts/ArchivedWebcasts/
PartneringCare.
9

CF and FERTILITY

Many adults with CF wish to have children. In the 1980s, it was thought to be impossible
for a man with CF to physically father a child because of infertility and too risky for a
woman with CF to get pregnant. With advances in fertility medicine, men with CF now
have the option to father children. Also, thanks to better nutrition and lung function, many
women with CF are able to have a healthy pregnancy. In 2012, the Patient Registry reported
that 249 women with CF were pregnant. In 1997, only 137 women with CF were pregnant.
Talk with your CF care center about what people with CF should think about before starting
a family.
Learn more about male and female fertility issues in CF at
www.cff.org/LivingWithCF/Webcasts/ArchivedWebcasts/AgeSpecific.

CF GENETICS
Learn more:
CFTR protein:
www.cff.org/
LivingWithCF/Webcasts/
ArchivedWebcasts/
Genetics/
CF clinical trials:
www.cff.org/research/
ClinicalResearch/
Participate
Clinical trial alerts:
www.cff.org/research/
ClinicalResearch/Find/
ClinicalTrialAlerts
Ivacaftor (Kalydeco):
www.cff.org/treatments/
Therapies/Kalydeco
Drug Development
Pipeline: www.
cff.org/research/
DrugDevelopment
Pipeline

10

There are more than 1,800 known mutations of the CF gene. It takes two
mutations for a person to have CF. Mutations in the CF gene lead to a CFTR
protein that does not work right. This leads to thick, sticky mucus in the lungs
and digestive system. The Foundation is working with pharmaceutical (drug)
companies to find drugs that can help the CFTR protein work better in people
with CF. Much of this research is looking at how to treat specific mutations of CF.
In January 2012, ivacaftor (Kalydeco) was approved for people with CF ages
6 years and older who have at least one G551D CF gene mutation. Research
showed that people with the G551D mutation who took the drug had major
improvements in lung function and weight.
The Foundation is supporting research to find out if ivacaftor can help people
with other CF mutations. Also, new drugs are being tested that may help
people with the most common CF mutation, F508del (Delta F508), and other
mutations. Find out more about this research (called CFTR Modulation) and
more on the CF Foundations drug development pipeline.
To test these potential new drugs, people with CF are needed to take part in CF
clinical trials. Talk with your CF care center to find out what research you or
your child might be able to join.
People with CF should know what CF gene mutations they have. The CF
Foundations Cystic Fibrosis Mutation Analysis Program (MAP) provides free
genetic testing for people with CF to find out which mutations they have
(www.cff.org/LivingWithCF/AssistanceResources/MAP). Your CF doctor or
nurse can sign you up for the program if you or your child have not been tested.

The table below lists the most common CF gene mutations in the United States. According
to the Patient Registry, almost 47 percent of people with CF have two F508del (Delta F508)
mutations. More than 39 percent of people with CF have one F508del gene mutation.
The Foundation supports ongoing research to find out more about the symptoms of CF
mutations and which mutations cause CF. You can look up a CF mutation and learn more
about it at www.cff.org/AboutCF/Testing/Genetics.
Most Common CF Gene Mutations, 2012
Mutation

Number of People with CF*

Percent of People with CF*

F508del

23,053

86.7

G542X

1,217

4.6

G551D

1,149

4.3

R117H

729

2.7

N1303K

659

2.5

W1282X

616

2.3

R553X

495

1.9

621+1G->T

453

1.7

1717-1G->A

431

1.6

3849+10kbC->T

412

1.5

2789+5G->A

344

1.3

3120+1G->A

281

1.1

I507del

218

0.8

R1162X

206

0.8

1898+1G->A

190

0.7

3659delC

183

0.7

G85E

167

0.6

D1152H

167

0.6

R560T

164

0.6

R347P

154

0.6

2184insA

142

0.5

A455E

140

0.5

R334W

134

0.5

Q493X

124

0.5

2184delA

122

0.5

*Includes people with one or two copies of the mutation.

11

MEETING THE GOALS OF CF CARE

The Foundation works to improve CF care based on seven goals of care (see page 5). The data
in the Registry show how the work being done in CF Foundation-accredited care centers
helps CF care teams reach these goals.
Goal 1: FULL MEMBERS OF THE CF CARE TEAM
People with CF and their families will be full members of the care team. Communication
will be open so everyone can be involved in care decisions. Care will be respectful of
the person with CFs needs, preferences and values.

The CF Foundations Patient Registry includes reports on the health of:

Each person with CF.
All people seen at a CF Foundation-accredited care center.
Everyone with CF in the United States.
These reports help all members of the CF team find new ways to improve care. The sample
Patient Summary Report on page 13 is a snapshot of a persons lung health, weight and other
information from the Registry. People with CF and their families should ask their CF care
center for a copy of their Patient Summary Report at clinic visits.
When looking at your or your childs Patient Summary Report, think about asking your CF
care center the following questions:
What does the data mean?
How can we work together for the best possible health for me or my child with CF?
What can we do as a team to improve care at the CF center?
To learn more about becoming a full member of your CF care team:
Watch the webcasts on:
 artnering for care: www.cff.org/LivingWithCF/Webcasts/ArchivedWebcasts/
P
PartneringCare
 uality improvement: www.cff.org/LivingWithCF/Webcasts/ArchivedWebcasts/
Q
PartneringCare/#Partnering_for_Improvement
Read how others have worked to improve care: www.cff.org/LivingWithCF/
QualityImprovement.
CF Care Guidelines

CF Care Guidelines

Visit www.cff.org/treatments/
CFCareGuidelines to read
care guidelines on:

Care or practice guidelines are used to improve the health care for many
diseases and conditions. These guidelines recommend treatments for patients
with specific diseases or conditions based on published medical research.

Diagnosis
Nutrition/GI
Respiratory
Infection Control
Age-Specific Care

12

To develop a CF care guideline, the Foundation gathers CF health experts, a

person with CF and parent of a child with CF to review the medical literature
and data from the Patient Registry.
To help people with CF stay as healthy as possible, CF care guidelines lists the
number of clinic visits, throat or sputum cultures and lab tests that those with

Sample Patient Summary Report

13

CF should have every year. The table below lists these and shows the percentage of all people
with CF in the Patient Registry who met the guidelines in 2007 and 2012.
All people with CF should have the tests in the table below done every year during one of
their four CF clinic visits, but not all patients get these tests. If you or your child have not
had the tests in the table, talk with your CF center.
Care, Screening and Prevention Guidelines for People with CF
2007
People with CF who:

2012

ALL

Under
18 Years

18 Years
and Older

ALL

Under
18 Years

18 Years
and Older

Had 4 or more clinic visits

59.3

68.6

47.7

64.7

73.4

55.6

Had 4 or more sputum/throat cultures

41.0

49.4

29.9

50.9

61.8

38.4

Had 2 or more lung function tests (PFTs)

if 6 years of age or older and physically able

88.8

91.2

86.5

89.4

91.9

87.4

Had an influenza vaccine (flu shot)

if 6 months of age or older

89.4

92.1

85.9

94.5

95.7

93.0

Had fat-soluble vitamin blood levels measured

(Vitamins A, D & E)

78.5

84.5

71.2

84.4

89.0

79.6

Had an oral glucose tolerance test (OGTT)

if 10 years of age or older

14.9

16.1

14.0

32.6

43.5

25.5

Had a blood test to measure liver enzymes

80.1

81.8

77.9

79.6

83.1

75.9

Goal 2: NORMAL GROWTH AND NUTRITION

Children, teens and adults with CF will have normal growth and nutrition.
BMI Formula
weight in kg
----- = BMI
(height in m)2
Calculate your BMI at:
www.cdc.gov/
healthyweight/
assessing/bmi

Food gives you the energy and nutrients, such as proteins, fats and vitamins, that
you need to be healthy. By eating enough of these nutrients, you can have good
nutrition. Children and teens need good nutrition to grow. Adults need good
nutrition to be healthy. Body mass index (BMI) is one way to know if you have
good nutrition.
BMI is based on a persons weight and height. BMI is calculated by dividing body
weight in kilograms (kg) by the persons height in meters squared (m2).
The CF nutrition care guidelines set goals for BMI for children and adults with CF
(www.cff.org/treatments/CFCareGuidelines/Nutrition.)

Nutrition for Children and Teens

For children and teens, BMI is stated as a percentile. BMI percentile shows how the BMI of
a child with CF matches up with the BMI of children in the United States of the same age
and gender who do not have CF. The goal is for children with CF to grow and develop at the
same rate as children without CF.
The graph at the top of the next page shows how much the BMI percentile of children and
teens with CF has improved in the last 10 years. But there is more work to be done, so
nutrition for children and teens with CF can continue to improve.
14

Median BMI Percentile for Children and Teens by Age - 1992, 2002 and 2012*
70
60
Goal: 50th Percentile

Percentile

50
40
30
20
10
1992

0
2

2002
5

2012
7

10

11

12

13

14

15

16

17

18

19

20

Age (Years)
*This graph starts at age 2 because the growth of younger children is measured as weight-for-length on a growth chart. The goal for
children under 2 years is to grow at the 50th percentile of weight-for-length. Growth charts for children of different ages are available at
www.cdc.gov/growthcharts/clinical_charts.htm. Ask your care center about your childs growth chart.

Nutrition for Adults

For adults with CF, the BMI goals are 23 for males and 22 for females. The overall goal for
all adults with CF is an average BMI of 22.5. The graph below shows how much the BMI of
adults with CF has improved. It also shows that more work can be done to improve the BMI
of young adults with CF. You can work closely with your CF care center to help improve or
maintain your growth and nutrition.
Learn more about CF and nutrition:
www.cff.org/LivingWithCF/StayingHealthy
www.cff.org/LivingWithCF/Webcasts/ArchivedWebcasts/Nutrition
Median BMI for Adults by Age - 1992, 2002 and 2012
24
1992

2002

2012

23
Goal: 22.5

BMI

22

21

20

19
20

21

22

23

24

25

26

27

28

29

30

31

32

33

34

35

36

37

38

39

40

Age (Years)

15

Nutrition and Lung Function

Normal nutrition for people with CF is important. Patient Registry data show a link between a
higher BMI and better lung function in both children and adults with CF. Lung function tests
(PFTs) help find out how well the lungs work. In CF, lung damage is caused by inflammation
(swelling) and infections that injure the lungs and airways. This damage may make the lungs
not work as well and lower PFTs. With every lung infection, a little more damage may happen.
FEV1 percent predicted is one measure of lung function. It measures the forced exhaled
volume in the first second. It is shown as a percent predicted, based on the FEV1 of healthy,
non-smoking people of the same age, race, height and gender. The following graphs show
that good nutrition and lung health seem to go hand in hand.
For people with CF ages 6 to 19 years, the first graph below shows the link between higher
BMI percentile and better lung function. The bar in the middle is the 50th percentile BMI
goal for children with CF.
The graph at the bottom of the page shows the link between higher BMI and better lung
function for adults with CF.
FEV1 Percent Predicted vs. BMI Percentile for Children 6 to 19 Years, 2012

FEV1 Percent Predicted

100

90

80
Male
Female

Goal: 50th percentile

70

60
<5

10

20

30

40

50

60

70

80

90

BMI Percentile

FEV1 Percent Predicted vs. BMI for Adults 20 to 40 Years, 2012

90

FEV1 Percent Predicted

80
70
60

Male
Female

50

BMI Goals:
22 for Females
23 for Males

40
30
15

16

17

18

19

20

21

22
BMI

16

23

24

25

26

27

28

29

30

Goal 3: MAINTAIN LUNG HEALTH

All people with CF will receive appropriate therapies for maintaining lung function and
reducing acute episodes of infection. Pulmonary exacerbations will be detected early
and treated aggressively to return lung function to previous levels.

People with CF often have pulmonary exacerbations. This is when lung symptoms
get worse and more treatments are needed. Exacerbations can be caused by
infection and inflammation. It is important to call your CF care center right away
to find out if you or your child has an exacerbation and to start treatment early.

Signs of an exacerbation:
Coughing more
More sputum
More shortness of breath

The graph below shows that the lung health of people with CF has improved over
the last 10 years. Since 2002, FEV1 is usually in a normal range (90 to 100 percent
predicted) when first measured at around 6 years of age.

A loss of appetite or
A loss of energy

Median FEV1 Percent Predicted by Age - 1992, 2002 and 2012

100

FEV1 Percent Predicted

90
80
70
60
50
1992

40
6

10

2002
12

2012
14

16

18
20
Age (Years)

22

24

26

28

30

Research shows that people of all ages with CF, including infants, have some lung
damage even when their FEV1 percent predicted is normal. Over time, FEV1 gets lower
because of damage from CF lung disease and exacerbations. The lower a persons FEV1
percent predicted, the more severe the lung disease. Here is a guide to FEV1 percent
predicted and what it may mean about lung health in CF:
FEV1 of 90 percent predicted or higher is thought of as normal. However, even with
normal lung function, some lung disease may be present.
70 to 89 percent predicted shows mild lung disease.
40 to 69 percent predicted shows moderate lung disease.
Less than 40 percent predicted is a sign of severe lung disease.
The table below shows how, today, more people with CF have normal or mild lung disease
than 10 and 20 years ago.
Percentage (%) of People with CF with Normal/Mild Lung Disease (FEV1 >
70%)
1992

2002

2012

6-17 Years

65.9

78.7

87.8

18-29 Years

27.7

40.5

52.8

30 Years and Older

15.9

22.7

34.3

17

The graph below shows the growing number of 18 year-olds with CF who have normal lung
function or mild lung disease (green bars). This increase means more people with CF are
healthier today than ever before.
Lung Function in 18 Year Olds, 1986-2012
100

Percent of People with CF

n FEV1 Normal/Mild (>

70%)

n FEV1 Moderate (40% to 69%)

n FEV1 Severe (<40%)

75

50

25

0
1986

1990

1995

2000
Year

2005

2010

2012

Lung Health Research

The CF Foundation supports research to find new drugs to keep the lungs of people with CF
as healthy as possible. These drugs may improve FEV1 or help the CFTR protein work better.
Learn more about research to keep lungs healthy on the CF Foundations drug development
pipeline at www.cff.org/research/DrugDevelopmentPipeline.
Before a drug can be prescribed, it must be tested in people during clinical trials to make
sure it is safe and works. People with CF play a key role in finding new drugs to treat CF.
By volunteering to take part in clinical trials and research, you or your child help
Learn more about what
researchers learn more about the disease and find new drugs and therapies.
the FDA does at:
www.fda.gov

Results from clinical trials are shown to the U.S. Food and Drug Administration
(FDA). Once the FDA approves a drug, it can be prescribed to a patient.
Learn more about how you can help with CF research at
www.cff.org/research/ClinicalResearch.
Watch webcasts about CF research at
www.cff.org/LivingWithCF/Webcasts/ArchivedWebcasts/Research.
Find CF clinical trials at www.cff.org/research/ClinicalResearch/Find.
Get CF clinical trial email alerts at
www.cff.org/research/ClinicalResearch/Find/ClinicalTrialAlerts.

18

Therapies for Lung Health

The following table shows the percentage of people with CF who are taking CF drugs
for lung health that are listed in the 2013 Cystic Fibrosis Pulmonary Guidelines: Chronic
Medications for Maintenance of Lung Health.
Learn more about the guidelines for these medications at
www.cff.org/treatments/CFCareGuidelines/Respiratory.
Percentage (%) of People with CF Who Met the Criteria and Were Prescribed the Medication
Recommended Chronic Medications for Lung Health

2011

2012

Criteria for Prescribing Medication

Dornase alfa (Pulmozyme)

82.6

83.7

At least 6 years old

Hypertonic saline

55.6

60.4

At least 6 years old

Tobramycin for inhalation solution

(such as TOBI)

66.2

65.7

At least 6 years old

P. aeruginosa in cultures

Aztreonam for inhalation solution

(such as Cayston)

35.9

39.0

At least 6 years old

P. aeruginosa in cultures

70.8

70.6

At least 6 years old

P. aeruginosa in cultures
Weight over 25 kg (55 lbs)
FEV1 over 30% predicted

High-dose ibuprofen
(e.g., 25-30 mg/kg)

3.2

3.3

612 years old

FEV1 over 60% predicted

Ivacaftor (Kalydeco)

-*

81.4

At least 6 years old

A G551D gene mutation

Azithromycin (such as Zithromax)

*Ivacaftor was not available in 2011.

Not all drugs are right for everyone with CF. There are criteria, or reasons, that a drug is
prescribed only for some people. For example, the criteria for prescribing the CF medication
ivacaftor (Kalydeco) are:
Age 6 years and older
A G551D CF gene mutation
To find out if you or your child might be helped by one of these drugs, talk with your CF
care center.
Medications alone cannot keep CF lungs healthy. Getting the thick mucus out of the lungs
is key. Airway clearance techniques (ACTs) help move mucus out of the lungs. The bodys
natural ACT is a cough, which is a reflex that clears mucus with high-speed airflow. However,
for people with CF, mucus cannot be cleared by coughing alone.
The care guidelines for ACT state that people with CF should do airway clearance every day
to keep lungs healthy, even when they are feeling well. When sick, airway clearance should be
done more often.
People with CF often use one or more types of ACT. The graph on the next page shows, by
age, the type of ACT used most often by people with CF.

19

Primary Airway Clearance by Age, 2012

90

n HFCWO (vest)

Percent of People with CF

80

n None

70

n Postural Drainage**

60

n PEP/Osc PEP

50

n Exercise*

40

n Forced Expiration

30
20
10
0
<2

2-5

Age (Years)

6 - 17

18+

** Postural drainage is also called postural drainage & percussion (PD&P) and sometimes chest physiotherapy (CPT).
*The Cystic Fibrosis Pulmonary Guidelines: Airway Clearance Therapies states that while exercise is important, it does not replace ACT
(www.cff.org/treatments/CFCareGuidelines/Respiratory).

Learn more about:

CF medications and airway clearance: www.cff.org/treatments/Therapies
CF respiratory care guidelines: www.cff.org/treatments/CFCareGuidelines/Respiratory
W
 atch the CF Education webcasts on:
 espiratory/lungs: www.cff.org/LivingWithCF/Webcasts/ArchivedWebcasts/
R
Respiratory
 artnering for care: www.cff.org/LivingWithCF/Webcasts/ArchivedWebcasts/
P
PartneringCare
Keeping Lungs Healthy

Each time a person with CF has an exacerbation or an infection, lung damage may occur.
Here are some things you or your child can do to be healthy and reduce the risk of getting sick:
Do airway clearance every day to keep lungs as clear as possible.
Take medications as prescribed by your CF care center.
Clean and disinfect nebulizers after every treatment.
Get a flu shot every fall for everyone in the house 6 months of age and older.
Reduce the risk of getting germs; see page 22 to learn more.
Exercise regularly to keep muscles strong.
Avoid tobacco smoke.
If you have CF and smoke, you are making your lung disease worse and causing more damage
than CF does alone. Ask your doctor or CF care center staff to help you quit. People with CF
should not smoke. Also, they should avoid secondhand smoke to keep their lungs as healthy as
possible. Secondhand smoke is smoke exhaled by a smoker or from burning tobacco.
20

Tobacco Smoke Exposure in People with CF by Age, 2006-2012

Percent of People with CF

50

40

30

20

10

-------- <18 years

-------- 18 to 24 years

-------- 25 to 34 years

-------- 35+ years

0
2006

2007

2008

2009
Year

2010

Secondhand smoke is harmful for people with CF. The graph above shows,
by age group, the percentage of people with CF who smoke or breathe in
secondhand tobacco smoke. The good news is that, in 2012, people with CF
have less exposure to tobacco smoke than they did in 2006. Staying away from
tobacco smoke will help you keep your lungs strong.

2011

2012

To make a smoke-free
environment:
Do not let anyone smoke
in your home or car.

In 2012, the Registry showed 12.5 percent of people with CF lived with
someone who smoked. Also, over 330 adults with CF reported that they
smoked. If you smoke, think about quitting. If you have CF and live with
someone who smokes, ask them to quit. Until that person can quit, ask them to
only smoke outside and away from you. Keeping the home and car smoke-free
can also help the person quit smoking. The only way to protect yourself or your
child from secondhand smoke is to be in a 100 percent smoke-free environment.

Ask people around you

not to smoke, even when
outside.

For adult non-smokers (who do not have CF), secondhand smoke can cause
lung cancer and heart disease. Research has shown that children without CF
who breathe in secondhand smoke have more lung infections. The U.S. Surgeon
General states that breathing even a little secondhand smoke can be harmful to
your health.

Ask your employer for

a smoke-free work and
eating space.

Learn more about Secondhand Smoke and CF:

www.cff.org/LivingWithCF/Webcasts/ArchivedWebcasts/Respiratory.
The Centers for Disease Control and Prevention has information
about smoking and tobacco use and how to quit at
www.cdc.gov/tobacco/quit_smoking.

Stay away from people

when they are smoking
and teach children to do
the same.

Make sure that your day

care center or school is
smoke-free.
Choose restaurants and
other businesses that are
smoke-free.
Let owners of businesses
that are not smoke-free
know that secondhand
smoke is harmful to you
or your familys health and
that it will stop you from
going to their business.

21

Goal 4: REDUCE LUNG GERMS

People with CF, their families and CF care center staff will be well-informed and
active partners in reducing the spread of germs, including Pseudomonas aeruginosa
(Pseudomonas) and Burkholderia cepacia (B. cepacia) complex.

Everyone can help reduce the spread of germs. The best way to avoid sharing or getting
germs is to:
Clean hands often with soap and water or 60 percent alcohol-based hand gels.
Use a tissue when coughing or sneezing, and then clean your hands.
Get all immunizations (shots), including a yearly influenza vaccine (flu shot).
Limit how much you touch your eyes, nose or mouth.
Stay away from people who are sick.
If you are ill, stay away from others.
Learn more about:
Infection prevention
and control
MRSA
ABPA
NTM
www.cff.org/
LivingWithCF/Webcasts/
ArchivedWebcasts/Germs

Avoiding germs is important for people with CF. Repeated lung infections and
exacerbations from germs can cause damage. When the lungs are damaged,
infections and exacerbations happen more often and CF lung disease gets worse.
Research shows that germs like Pseudomonas and B. cepacia complex can spread or
be shared between people with CF. The infection prevention and control guidelines
recommend that people with CF limit their face-to-face contact with others with
CF to reduce the risk of a lung infection. While not the same as being face-to-face,
todays technology makes it easier for people with CF to connect with each other,
such as by text messages or on the Internet, without increasing the risk of infection.
The graph below shows some of the germs that are found in the lungs of people
with CF. Talk with your CF care center and learn what you can do to stay healthy.

Germs Found in the Lungs of People with CF by Age, 2012

80

Percent of People with CF

70
60
50
40
30
20
10
0
<2

2 to 5

6 to 10

11 to 17
18 to 24
Age (Years)

35 to 44

P.
*P.aeruginosa*
aeruginosa

H.
H. influenzae
influenzae

B.cepacia
B.
cepaciacomplex
complex

S.
aureus
S.
aureus

MRSA
MRSA

Achromobacter
Achromobacter

S. maltophilia

MDR-PA**
**MDR-PA

*P. aeruginosa includes people with MDR-PA.

**MDR-PA is multi-drug resistant Pseudomonas aeruginosa (P. aeruginosa).

S. aureus includes people with MRSA.

MRSA is methicillin-resistant Staphylococcus aureus (S. aureus).

22

25 to 34

45+

You can also:

Read the infection prevention and control care guidelines at
www.cff.org/treatments/CFCareGuidelines/InfectionControl.
Learn more about how clean hands save lives at www.cdc.gov/handwashing.
Find out who should get what immunizations (shots) at
www.cdc.gov/vaccines.
The Foundation supports research to learn more about the germs found in the
lungs of people with CF. For example, researchers are studying the best way to
treat methicillin-resistant Staphylococcus aureus (MRSA) in people with CF.
The Foundation also supports the Burkholderia cepacia Research Laboratory
and Repository at the University of Michigan, Ann Arbor.

Read about:
MRSA research:
www.cff.org/research/
DrugDevelopmentPipeline/
AdditionalClinicalInitiatives
Burkholderia cepacia
complex:
www.cff.org/LivingWithCF/
StayingHealthy/Germs/
Bcepacia

Even if you are careful and do all you can to avoid germs, you may still get a lung infection.
Its hard to avoid germs completely. If you or your child starts to feel ill are coughing
more, have more sputum or have a loss of energy or appetite call your CF care center so
treatment can be started as soon as possible.
Goal 5: COMPLICATIONS RELATED TO CF
People with CF will be screened and managed aggressively for complications of the
disease, particularly CF-related diabetes.

A complication is a disease or injury that happens because of another disease

or treatment. For people with CF, one of the most common complications
of CF is cystic fibrosis-related diabetes (CFRD). In 2012, 19.7 percent of
all people with CF had CFRD. Among those ages 18 years and older, 33.8
percent had CFRD.
CFRD is different from diabetes. Research shows that early diagnosis and
treatment of CFRD leads to better nutrition and health. To keep people with
CF as healthy as possible, everyone ages 10 years and older should be tested
every year for CFRD. The test is called an oral glucose tolerance test (OGTT).
In 2012, 32.6 percent of people with CF ages 10 and older who did not
have CFRD were tested for CFRD with an OGTT. (See the Care, Screening
and Guidelines table on page 14). People who have CFRD that is not treated
have more exacerbations and worse nutrition than those who work with their
doctors and dietitians to keep their blood sugar under control.

Learn more about CFRD:

Watch the webcast series:
www.cff.org/
LivingWithCF/Webcasts/
ArchivedWebcasts/
Nutrition
Read the CFRD care
guidelines:
www.cff.org/treatments/
CFCareGuidelines/
Nutrition

Other problems (or complications) of CF are bone disease and joint problems, such as
arthritis or arthropathy. Data from the Patient Registry show that over 36 percent of
people with CF ages 35 and older reported bone disease (such as fracture, osteopenia or
osteoporosis). Preventing bone disease begins early when bones are growing.
A healthy weight, regular exercise and a diet with enough calories, vitamin D, calcium and
other vitamins and minerals can help prevent bone disease. To learn more about keeping
your or your childs bones and joints healthy, talk with your CF care center.
Depression is another problem people with CF may face. For adults with CF, the Registry
data show that 22.2 percent have signs of depression. People living with other chronic
diseases also have signs of depression. If you or your child are feeling hopeless or helpless, you
might be depressed.
23

Common Complications by Age, 2012

50
Percent of People with CF

Bone Disease
Depression

Diabetes (CFRD)
Joint Problems

40
30
20
10
0
<6

6 to 10

11 to 17

18 to 24

25 to 34

35 to 44

45+

Age (Years)

Learn more:
Vitamins and minerals:
www.cff.org/LivingWithCF/
StayingHealthy/Diet/Vitamins
Exercise:
www.cff.org/Adults/DailyLife/
Exercise
Depression and anxiety:
www.cff.org/Adults/Other and
www.Medlineplus.gov
Partnering for care:
www.cff.org/LivingWithCF/
Webcasts/ArchivedWebcasts/
PartneringCare

It is important to be diagnosed and treated for depression as soon

as possible. Talk with your CF care center if you think you or your
child might be depressed. People often respond well to treatment for
depression, by both working with a therapist and taking medicine.
Other complications of CF are listed in the table below. For example, the
Registry data show that 29.7 percent of people with CF reported having
sinus disease in 2012. These symptoms include, but are not limited to,
headaches, dental pain and feeling facial congestion or fullness. If you or
your child are having these symptoms, talk with your CF care center.
People with CF may also have problems such as gastroesophageal reflux
(GERD), distal intestinal obstructive syndrome (DIOS) and liver disease.
The Foundation continues to work to find ways to help people with CF
and these complications.

Sinus disease:
www.cff.org/LivingWithCF/
Webcasts/ArchivedWebcasts/
Respiratory

Other Problems Related to CF, 2012

Cystic fibrosis-related diabetes (CFRD)

19.7

GERD, DIOS and liver disease:

www.cff.org/LivingWithCF/
Webcasts/ArchivedWebcasts/
Nutrition

Bone disease (fracture, osteopenia or osteoporosis)

14.8

Joint problems (arthritis/arthropathy)

2.9

Depression

12.0

Sinus disease

29.7

Nasal polyps (requiring surgery)

4.5

Liver disease

10.1

Gastroesophageal reflux (GERD)

30.5

Distal intestinal obstructive syndrome (DIOS)

5.1

Asthma

25.2

24

Complication

Percentage (%) of People

Who Reported This Problem

Goal 6: TRANSPLANTATION AND END-OF-LIFE CARE

People with CF and their families will be supported by their CF care center when facing
decisions about transplantation and end-of-life care.

People with CF who have severe lung disease often think about having a lung transplant.
However, the supply of donor lungs for transplant is limited. Also, organ transplantation
has its own set of risks and long-term medical care. It is important to talk with your CF care
center to learn if you or your child might benefit from a lung transplant.
The graph below shows the number of people with CF who have had a lung transplant since
1990, by year.
Number of People with CF Who Had a Lung Transplant by Year
250

Number of Lung Transplants

232
200

148
100
93
50

56

55

91

92

140

152

152 147
129 129

138

161

154

146

205
190

180

171

150

206

204

104

13
90

93

94

95

96

97

98

99

00

01 02
Year

03

04

05

06

07

08

09

10

11

12

Learn more about lung transplantation:

Read the frequently asked questions at www.cff.org/treatments/LungTransplantation.
Watch the CF webcast series at
www.cff.org/LivingWithCF/Webcasts/ArchivedWebcasts/Respiratory.
Read about living with a transplant at http://transplantliving.org.
Find data at http://optn.transplant.hrsa.gov/data.
Search for research at www.clinicaltrials.gov/ct2/search, using the search term lung
transplant.
Find out how to be an organ donor, visit www.OrganDonor.gov.

25

Goal 7: ACCESS TO CARE

People with CF and their families will have access to appropriate therapies, treatments
and support regardless of race, age, education or ability to pay.

Research suggests that people with CF who live in households with lower incomes are more
likely to have lower lung function and lower body mass index (BMI) or BMI percentile. This
pattern of health in lower-income households is also common in other chronic diseases, such
as diabetes.
The Cystic Fibrosis
Patient Assistance
Foundation (CFPAF), a
nonprofit subsidiary of
the CF Foundation, helps
people with CF in the
United States get their
prescribed FDA-approved
drugs and devices.
Visit CFPAFs website
www.cfpaf.org
Call 1-888-315-4154

Data from the Patient Registry show that drugs such as Pulmozyme, TOBI,
Cayston and Kalydeco are available to people with CF, regardless of income
level. To learn more about medication assistance and how others manage
CF, watch the webcast series CF Healthcare Coverage and Advocacy and
Building Life Skills to Manage CF at www.cff.org/LivingWithCF/Webcasts/
ArchivedWebcasts/PartneringCare.
There are a number of programs available to help people with CF get the care
and medications they need. To find more assistance programs for people
with CF, visit the Foundations Patient Assistance Resource Center at
www.cff.org/LivingWithCF/AssistanceResources.
The Foundation supports changes to health care that help people with CF. The
health care reform law, passed in 2010, allows children to stay on their parents
health insurance until the age of 26. In 2012, 53.7 percent of adults with CF
ages 18 through 25 years stayed on their parents health insurance.

The table below shows the percentage of people with CF and the types of health insurance
coverage they had in 2012.
A local CF care center is the best source for up-to-date information on health care coverage
programs in your area and state. Call your CF care center for help.
Percentage (%) of People with CF by Type of Health Insurance
Coverage, 2012*
Type of Insurance

17 Years and Younger

18 Years and Older

Health Insurance Policy (e.g., Private Insurance)

55.6

64.9

Medicare/Indian Health Services

0.6

17.8

Medicaid/State Programs

53.0

34.2

TriCare or Other Military Health Plan

2.9

2.1

Other

4.0

4.3

No Health Insurance**

0.4

1.7

*Data are not mutually exclusive, except no health insurance, as people with CF may have more than one
type of insurance.
**No health insurance shows the percentage who said they did not have any health insurance in 2012.

26

SUMMARY TABLES
Summary of the Data: People in the CF Foundations Patient Registry
Characteristic

1992

2002

2012

People with CF (number)

18,497

23,064

27,804

Newly diagnosed (number)

1,011

1,040

920

6 months

6 months

4 months

0-71.0

0-75.8

0-82.7

Total deaths (number)

362

426

425

Predicted median survival (years)

29.4

31.3

41.1

28.2-30.8

29.7-33.1

37.4-43.1

Adults 18 years and older (%)

32.8

40.1

49.1

Race/Ethnicity (%)
Caucasian
African American
Hispanic

96.1
3.3
4.1

95.3
3.9
5.7

94.1
4.5
7.7

Males (%)

53.9

52.7

51.7

99.9
36.1
20.0

95.0
42.2
21.3

87.3
52.0
22.2

8.6
31.7

11.4
43.0

67.4

73.4

77.3

People with CF with respiratory cultures positive for (%)

Pseudomonas aeruginosa (P. aeruginosa)
Multi-drug resistant P. aeruginosa (MDR-PA)
Burkholderia cepacia complex (B. cepacia)
Staphylococcus aureus (S. aureus)**
Methicillin-resistant S. aureus (MRSA)

58.0
2.8
29.1
-

57.8
4.5
3.1
55.9
9.2

49.6
9.5
2.6
69.0
26.5

Therapies(%)***
Dornase alfa (i.e., Pulmozyme)
Hypertonic Saline
Tobramycin solution for inhalation (e.g., TOBI)
Aztreonam for inhalation solution (e.g., Cayston)
Azithromycin (e.g., chronic oral macrolide antibiotic)
High-dose ibuprofen (e.g., 2530 mg/kg)
Ivacaftor (i.e., Kalydeco)

64.7
70.8
11.6
6.8
-

83.7
60.4
65.7
39.0
70.6
3.3
81.4

Transplants (number)
Lung (any type)
Liver
Kidney

55
12
-

138
14
1

205
19
11

Clinical research participation (number)

966

5,872

5,439

Pregnancies (number)

116

182

249

Live birth rates (per 100 women ages1445 years)

1.5

1.9

2.0

Age at diagnosis (median)

Age range (years)

Predicted median survival (years) 95% confidence interval

Nutrition
Pancreatic enzyme supplements use (%)
BMI percentile ages 2 to 19 years (median)*
BMI ages 20 to 40 years (median)*
Supplemental feeding (%)
By tube
By mouth only
Respiratory/Lungs
FEV1 % predicted (mean)

 ue to data adjustments, some people diagnosed with CF in 2012 will be entered into the Registry in 2013.
D
Next year the number of people who were diagnosed in 2012 is expected to be higher.
*The Centers for Disease Control and Prevention provide body mass index (BMI) calculators. The national goal
for children with CF ages 2-19 years is 50th BMI percentile. For adults with CF, the national goal for weight is a
BMI of 23 for males and 22 for females. For more information, see www.cdc.gov/healthyweight/assessing/bmi.
- Data was not available in that year.

Includes people with CF with MDR-PA.

**Includes people with CF with MRSA.
***This is the percentage of people who were eligible for a therapy and had it prescribed at least once in 2012.

27

Number of People with CF by State*

State

Number

Percent

Alabama

451

1.62

Alaska

101

Arizona

State

Number

Percent

Nebraska

242

0.87

0.36

Nevada

186

0.67

397

1.43

New Hampshire

192

0.69

Arkansas

265

0.95

New Jersey

679

2.44

California

2,297

8.26

New Mexico

134

0.48

Colorado

530

1.91

New York

1,643

5.91

Connecticut

313

1.13

North Carolina

863

3.10

Delaware

64

0.23

North Dakota

77

0.28

District of Columbia

30

0.11

Ohio

1,511

5.43

Florida

1,376

4.95

Oklahoma

226

0.81

Georgia

746

2.68

Oregon

371

1.33

Hawaii

21

0.08

Pennsylvania

1,461

5.25

Idaho

184

0.66

Rhode Island

98

0.35

Illinois

1,062

3.82

South Carolina

372

1.34

Indiana

667

2.40

South Dakota

103

0.37

Iowa

380

1.37

Tennessee

662

2.38

Kansas

343

1.23

Texas

1,778

6.39

Kentucky

548

1.97

Utah

374

1.35

Louisiana

334

1.20

Vermont

156

0.56

Maine

248

0.89

Virgin Islands

0.00

Maryland

529

1.90

Virginia

654

2.35

Massachusetts

857

3.08

Washington

606

2.18

Michigan

1,060

3.81

West Virginia

250

0.90

Minnesota

585

2.10

Wisconsin

621

2.23

Mississippi

242

0.87

Wyoming

57

0.21

Missouri

723

2.60

Unknown

0.01

Montana

123

0.44

Foreign

0.03

*These are only people with CF seen at a CF Foundation-accredited care center who agree to have their data entered in the Patient Registry.

28

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