2012 CFF Patient Registry

8/9/2019 2012 CFF Patient Registry

1/32

PatientRegistry

2012Annual Data Report


2/32

If you have any questions aboutCF care, please talk with your CF carecenter or contact the Foundation at1-800 FIGHT CF or [email protected] .

THE CF FOUNDATION WISHES TO THANKthe people with CF and their families whocontributed their photos to this report. PHOTOGRAPHY BY Cade Martin Photography

SOURCE OF DATACystic brosis patients under care at CF Foundation-accredited care centers in the United States, whoconsented to have their data entered in 2012.

SUGGESTED CITATIONCystic Fibrosis Foundation Patient Registry2012 Annual Data ReportBethesda, Maryland©2013 Cystic Fibrosis Foundation


3/32

1

To the CF Community and Friends,

Each year, the Cystic Fibrosis Foundation collects information on the health of the morethan 27,000 people with CF who receive care at Foundation-accredited centers nationwide.

Te Foundation shares this information with the wider community through the PatientRegistry Annual Data Report , which highlights progress made in cystic brosis (CF) careand areas where more work is needed.

Te data in this report, collected through 2012, shows that the outlook for people with CF

continues to improve. Here are some highlights:• Te median predicted age of survival continues to rise.

• Adults and children with CF are experiencing better overall lung function andimproved nutrition and growth.

• Tanks to nationwide newborn screening, more CF diagnoses are now made in therst year of life.

• Nearly half of all people with CF are now ages 18 and older.

Tese steady gains reect the vital partnerships among all members of the CF care

team — people with CF, their families and care center staff. We hope the informationin this report helps strengthen these partnerships by serving as a tool for shared learningand 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 toshare their data. We look forward to working together in the year ahead.

Sincerely,

Bruce C. Marshall, M.D. Leslie Hazle, M.S., R.N.Senior Vice President of Clinical Affairs Director of Patient Resources


4/32

HOW DO I USE THE REGISTRY REPORT?

For people with cystic brosis (CF) and their families, this report can help you talk withthe care center about your or your child’s 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 thehealth and quality of life of people with CF in the United States.

KEY WORDS IN THE REGISTRY DATA

o help you understand what the Patient Registry data means, here are denitions of somekey words used in this report.

• Median: Te median is the middle. Tat means half the people with CF in thisgroup are above this number and half are below.

• Percent: A percent is one part in a hundred. For example, 60 percent means thatthere are 60 out of 100. If 95 percent of people with CF have their u shot thatmeans 95 people out of 100 got their u shot. Tis is sometimes called a percentage.

• BMI: Body mass index or BMI is based on a person’s weight and height. It is used toscreen 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 person’s height inmeters squared (m2).

• BMI percentile: BMI percentile matches one child’s BMI to the BMI of otherchildren in the United States who are the same age and gender. A BMI of the 50thpercentile means the child is in the middle. Half of the children of the same age andgender are larger and half are smaller.

• FEV 1 percent predicted: FEV 1 is one measure of lung function. It measuresthe forced exhaled volume of air in the rst second. It is shown as a percentpredicted, based on the FEV 1 of healthy, non-smoking people of the same age,height and gender.


5/323

ABOUT CYSTIC FIBROSIS AND THE CF FOUNDATION 4What Is Cystic Fibrosis? 4What Is the Cystic Fibrosis Foundation? 4

WHAT IS THE CYSTIC FIBROSIS FOUNDATION’S PATIENT REGISTRY? 5

OVERVIEW OF THE HEALTH OF PEOPLE WITH CF 6Median Predicted Survival Age 6

Age at Diagnosis 7

Number of Clinic Visits in the First Year of Life 8

Adults With CF 8Number of Children and Adults with CF 9

Characteristics of Adults with CF 9

CF and Fertility 10

CF Genetics 10Most Common CF Gene Mutations 11

MEETING THE GOALS OF CF CARE 12

Goal 1: Full Members of the CF Care Team 12CF Care Guidelines 12

Sample Patient Summary Report 13

Care, Screening and Prevention Guidelines 14

Goal 2: Normal Growth and Nutrition 14

Nutrition for Children and Teens 14Median BMI Percentile 15

Nutrition for Adults 15Median BMI 15

Nutrition and Lung Function 16FEV1 Percent Predicted vs. BMI Percentile for Children 6 to 19 Years 16

FEV1 Percent Predicted vs. BMI for Adults 20 to 40 Years 16

Goal 3: Maintain Lung Health 17Median FEV 1 Percent Predicted by Age 17

People with CF with Normal/Mild Lung Disease 17 Lung Function in 18 Year Olds 18

Lung Health Research 18

Therapies for Lung Health 19People with CF Who Were Prescribed CF Medications 19

Primary Airway Clearance by Age 20

Keeping Lungs Healthy 20Tobacco Smoke Exposure by Age 21

Goal 4: Reduce Lung Germs 22Germs Found in the Lungs by Age 22

Goal 5: Complications Related to CF 23Common Complications by Age 24 Other Problems Related to CF 24

Goal 6: Transplantation and End-of-Life Care 25Number of People with CF Who Had a Lung Transplant by Year 25

Goal 7: Access to Care 26People with CF by Type of Health Insurance Coverage 26

SUMMARY TABLES 27Summary of the Data 27

Number of People with CF by State 28

TABLE OF CONTENTS


6/32

4

ABOUT CYSTIC FIBROSIS AND THE CF FOUNDATION

WHAT IS CYSTIC FIBROSIS?

Cystic brosis (CF) is a lifelong genetic disease that causes mucus to build up and clog someof the body’s organs. In people with the disease, two mutations in the CF gene lead to aprotein, called CF R, that does not work right. Tis defect in the protein causes mucus to bethick and sticky. Te buildup of mucus leads to problems in the lungs and digestive system.

People with CF often get lung infections and have inammation or swelling in the lungs.Over time, these problems lead to lung damage. CF can also make it hard for the body toget nutrients from food. It is hard for some people with CF to grow and have a healthybody weight.

CF is found in people of all racial and ethnic groups. Each year, about one in 3,500 babiesborn in the United States has CF. It is more common in white people (Caucasians) than anyother race. About 30,000 people in the United States have CF, and about 70,000 worldwide.

WHAT IS THE CYSTIC FIBROSIS FOUNDATION?

Te CF Foundation was started in 1955 by parents of children with CF. Te mission ofthe Foundation is to nd a cure for CF and to improve the quality of life for people withthe disease.

Te Foundation accredits a network of more than 110 CF care centers across the UnitedStates to meet the health care needs of people with CF. Te Foundation provides these carecenters 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.

• raining in quality improvement.

• Grants to give some funding for care and research.

Te Foundation also supports the work of researchers to discover and develop new therapiesto improve the length and quality of life for those with the disease. Developing new CFdrugs takes time and a lot of money. o help speed development of potential new therapies,the Foundation funds promising research to target the disease from all angles. A researchpriority of the Foundation is to nd drugs that target the underlying cause of CF, the CF Rprotein that does not work right.

o learn more about different CF drugs in development, visit the Foundation’s websiteat www.cff.org/research/DrugDevelopmentPipeline. You can also learn more about cysticbrosis, the Foundation, its accredited care centers, guidelines and research at www.cff.org .


7/32

5

WHAT IS THE CYSTIC FIBROSIS FOUNDATION’SPATIENT REGISTRY?

Te Cystic Fibrosis Foundation’s 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. Te data include state of residence, height, weight,gender, CF mutations, lung (pulmonary) function test (PF ) results, medication use andproblems (complications) related to CF.

Te 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.

Te Registry also gives people with CF and their families a look at the overall health of thoseliving with CF. It is vital for everyone with CF to agree to have their data entered into theRegistry. Te participation of all people with CF means data are more complete and helpscreate a better picture of the current health of people with CF. Tese data can also be used tond areas where improvements can be made.

Te CF Foundation has set seven goals to help improve the health of people with CF. Tisreport highlights these goals and the work being done to meet them. Te 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 CF’s 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 returnpeople 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 andactive partners in reducing the spread of germs, includingPseudomonas aeruginosa (Pseudomonas) and Burkholderia cepacia (B. cepacia) complex.

Goal 5: People with CF will be screened and managed aggressively for complications of thedisease, particularly CF-related diabetes (CFRD).

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


8/32

6

OVERVIEW OF THE HEALTH OF PEOPLE WITH CF

Te Patient Registry is a tool used to measure improvements in the health and survival ofpeople with CF in the United States, from diagnosis through adulthood. For an overview aboutpeople with CF in the Patient Registry, including race, gender and age range, see the “Summaryof the Data” on page 27. o learn how many people with CF are in each state, see page 28.

Te median predicted age of survival for people with CF has risen steadily over the last 25years. 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.

Te chart below shows the rise in median predicted survival age since 1988 in 5-year bandsor groups. For 2008-2012, the median predicted survival was 37.8 years.

Median predicted survival is calculated using a method called life table analysis. Tis is bestknown for its use in the life insurance industry. Using this calculation, half of the people inthe Patient Registry are expected to live beyond the median predicted survival age, and half areexpected to live less than the median predicted survival age.

Year-to-year differences are normal when measuring health outcomes for any disease or medicalcondition. What is important is that, over time, survival is going up.

24

28

32

36

40

1988-1992 1993-1997 1998-2002 2003-2007 2008-2012

M e

d i a n

P r e

d i c t e d S u r v

i v a l

( A g e

i n Y e a r s )

Years

Median Predicted Survival Age, 1988-2012 In 5-Year Bands


9/327

0

1,000

2,000

3,000

4,000

5,000

6,000

7,000

8,000

N u m

b e r o

f P e o p l e

w i t h C F

Age

Age at Diagnosis of All People with CF, 2012

Early diagnosis may play an important role in improving survival. Since 2010, all 50states and the District of Columbia screen all newborns for CF. Research shows thatpeople with CF who are diagnosed because of newborn screening have better weightand 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 foundbecause of an abnormal newborn screen. Te earlier CF is diagnosed, the soonertreatment can begin.

o help babies with CF grow well, the Foundation’s CF infant care guidelines statethat infants with CF should go to the CF care center every month during the rst 6 months oflife, and every one to two months in the second 6 months. Te graph on the next page shows thenumber of times infants with CF go to CF clinic in the rst 12 months of life.

• Read the Foundation’s infant care guidelines at www.cff.org/treatments/CFCareGuidelines/ AgeSpecicCare.

The CF Foundation hasinformation to help parentsand families of newborns atwww.cff.org/AboutCF/Testing/NewbornScreening .

Te graph below shows all people in the Registry and their age at the time of diagnosis withcystic brosis. Most people with CF are diagnosed before the age of 2 years. However, CFcan be diagnosed at any age. oday, many people with CF are diagnosed shortly after birthbecause of newborn screening. But, each year some children and adults are still diagnosedbecause of symptoms.

In 2012, 52 people over 40 years old were diagnosed with CF. Te 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/ esting .

Pre-natal

0–1 1–3 4–6 7–11 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16–20

21–30

31–40

over40_________________

Months________________________________________________________________________________________

Years


10/328

Research shows that some infants with CF may not grow well even with earlydiagnosis and treatment. Te Foundation is supporting research to nd out why.For example, the Baby Observational and Nutrition Study (BONUS) is lookingat 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 Foundation’s web site at www.cff.org/LivingWithCF/StayingHealthy/CFInfantCare.

ADULTS WITH CF

Te graph at the top of the next page shows how fast the number of adults with CF in thePatient Registry has grown in relation to the number of children with CF in 1986-2012. Teblue line shows the number of adults by year. Te 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 49percent were adults. Te growing number of adults with CF means that people with CF arehealthier and living longer than ever before.

It is likely that there will soon be more adults with CF in the United States than children withthe 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.

Te Foundation continues to support the care of adults with CF and is helping more doctorslearn 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/ AgeSpecicCare.

The Centers for DiseaseControl and Preventiongrowth charts at www.cdc.gov/growthchartsshow that infants shouldincrease their weight tothree times their birthweight by 1 year of age.

0

10

20

30

40

50

60

70

80

90

0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 ≥ 21

N u m

b e r o

f P e o p

l e w

i t h C F

Number of Clinic Visits

Number of CF Clinic Visits in the First Year of Life for Babies Born with CF in 2011 andDiagnosed through Newborn Screening


11/329

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

CF Foundation-accredited care centers can help teach children, teens and adults how to managetheir daily CF care. It is important for teens with CF to learn how to take charge of their ownhealth as they move into adulthood.

• Learn more about adult CF care and moving from pediatric to adult care in the “Partneringfor Care” webcast series at www.cff.org/LivingWithCF/Webcasts/ArchivedWebcasts/PartneringCare.

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

Education

Some College33.7%

CollegeGraduate

29.1%

Less thanHigh School

7.1%

Masters/DoctoralLevel Degree

5.7%

High SchoolDiploma24.4% Married/Living

Together39.8%

Single54.9%

Separated/Divorced 5.0%

Widowed 0.3%

Marital StatusFull-time

33.6%Student23.5%

Retired1.3% Unemployed

8.4%

Homemaker4.3%

Part-time11.8%

Disabled17.0%

Employment

0

3,000

6,000

9,000

12,000

15,000

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

N u m

b e r o

f P e o p

l e w

i t h C F

Year

Children Under 18 Years

Adults 18 Years and Older

Number of Children and Adults with CF, 1986-2012

29.2%

49.1%


12/3210

CF AND FERTILITY

Many adults with CF wish to have children. In the 1980s, it was thought to be impossiblefor 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 nowhave 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.alk 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/AgeSpecic.

CF GENETICS

Tere are more than 1,800 known mutations of the CF gene. It takes twomutations for a person to have CF. Mutations in the CF gene lead to a CF Rprotein that does not work right. Tis leads to thick, sticky mucus in the lungsand digestive system. Te Foundation is working with pharmaceutical (drug)companies to nd drugs that can help the CF R protein work better in people

with CF. Much of this research is looking at how to treat specic mutations of CF.

In January 2012, ivacaftor (Kalydeco™) was approved for people with CF ages6 years and older who have at least one G551D CF gene mutation. Researchshowed that people with the G551D mutation who took the drug had majorimprovements in lung function and weight.

Te Foundation is supporting research to nd out if ivacaftor can help people

with other CF mutations. Also, new drugs are being tested that may helppeople with the most common CF mutation, F508del (Delta F508), and othermutations. Find out more about this research (called “CF R Modulation”) andmore on the CF Foundation’s drug development pipeline.

o test these potential new drugs, people with CF are needed to take part in CFclinical trials. alk with your CF care center to nd out what research you oryour child might be able to join.

People with CF should know what CF gene mutations they have. Te CFFoundation’s Cystic Fibrosis Mutation Analysis Program (MAP) provides freegenetic testing for people with CF to nd out which mutations they have

( www.cff.org/LivingWithCF/AssistanceResources/MAP). Your CF doctor ornurse can sign you up for the program if you or your child have not been tested.

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 DevelopmentPipeline: www.cff.org/research/DrugDevelopment

Pipeline


13/3211

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

Te table below lists the most common CF gene mutations in the United States. Accordingto 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.

Te Foundation supports ongoing research to nd out more about the symptoms of CFmutations and which mutations cause CF. You can look up a CF mutation and learn moreabout it at www.cff.org/AboutCF/ esting/Genetics.

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


14/3212

MEETING THE GOALS OF CF CARE

Te Foundation works to improve CF care based on seven goals of care (see page 5). Te datain the Registry show how the work being done in CF Foundation-accredited care centershelps 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. Communicationwill be open so everyone can be involved in care decisions. Care will be respectful ofthe person with CF’s needs, preferences and values.

Te CF Foundation’s 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.

Tese reports help all members of the CF team nd new ways to improve care. Te samplePatient Summary Report on page 13 is a snapshot of a person’s lung health, weight and otherinformation from the Registry. People with CF and their families should ask their CF carecenter for a copy of their Patient Summary Report at clinic visits.

When looking at your or your child’sPatient Summary Report , think about asking your CFcare 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?

o learn more about becoming a full member of your CF care team:• Watch the webcasts on:

Partnering for care: www.cff.org/LivingWithCF/Webcasts/ArchivedWebcasts/PartneringCare

Quality improvement: www.cff.org/LivingWithCF/Webcasts/ArchivedWebcasts/PartneringCare/#Partnering_for_Improvement

• Read how others have worked to improve care: www.cff.org/LivingWithCF/QualityImprovement.

CF Care Guidelines Care or practice guidelines are used to improve the health care for manydiseases and conditions. Tese guidelines recommend treatments for patients with specic diseases or conditions based on published medical research.

o develop a CF care guideline, the Foundation gathers CF health experts, aperson with CF and parent of a child with CF to review the medical literatureand data from the Patient Registry.

o help people with CF stay as healthy as possible, CF care guidelines lists thenumber of clinic visits, throat or sputum cultures and lab tests that those with

CF Care Guidelines Visit www.cff.org/treatments/CFCareGuidelines to readcare guidelines on: • Diagnosis • Nutrition/GI • Respiratory • Infection Control • Age-Specic Care


15/3213

Sample Patient Summary Report


16/3214

Care, Screening and Prevention Guidelines for People with CF

People with CF who:2007 2012

ALL Under18 Years18 Years

and Older ALLUnder

18 Years18 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 inuenza vaccine (u shot)

if 6 months of age or older89.4 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.

Food gives you the energy and nutrients, such as proteins, fats and vitamins, thatyou need to be healthy. By eating enough of these nutrients, you can have goodnutrition. Children and teens need good nutrition to grow. Adults need goodnutrition to be healthy. Body mass index (BMI) is one way to know if you havegood nutrition.

BMI is based on a person’s weight and height. BMI is calculated by dividing body weight in kilograms (kg) by the person’s height in meters squared (m2).

Te 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 ofa child with CF matches up with the BMI of children in the United States of the same ageand gender who do not have CF. Te goal is for children with CF to grow and develop at thesame rate as children without CF.

Te graph at the top of the next page shows how much the BMI percentile of children andteens with CF has improved in the last 10 years. But there is more work to be done, sonutrition for children and teens with CF can continue to improve.

CF should have every year. Te 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 oftheir four CF clinic visits, but not all patients get these tests. If you or your child have nothad the tests in the table, talk with your CF center.

BMI Formula

weight in kg————-----— = BMI(height in m) 2

Calculate your BMI at:

www.cdc.gov/healthyweight/assessing/bmi


17/32

15

Nutrition for Adults

For adults with CF, the BMI goals are 23 for males and 22 for females. Te overall goal forall adults with CF is an average BMI of 22.5. Te graph below shows how much the BMI ofadults with CF has improved. It also shows that more work can be done to improve the BMIof young adults with CF. You can work closely with your CF care center to help improve ormaintain your growth and nutrition.

Learn more about CF and nutrition:

• www.cff.org/LivingWithCF/StayingHealthy

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

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

0

10

20

30

40

50

60

70

2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20

P e r c e n

t i l

e

Age (Years)

1992 2002 2012

Median BMI Percentile for Children and Teens by Age - 1992, 2002 and 2012*

Goal: 50th Percentile

19

20

21

22

23

24

20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40

B M I

Age (Years)

1992 2002 2012

Median BMI for Adults by Age - 1992, 2002 and 2012

Goal: 22.5


18/32

16

60

70

80

90

100


19/3217

40

50

60

70

80

90

100

6 8 10 12 14 16 18 20 22 24 26 28 30

Age (Years)

1992 2002 2012

Median FEV 1 Percent Predicted by Age - 1992, 2002 and 2012

Research shows that people of all ages with CF, including infants, have some lungdamage — even when their FEV 1 percent predicted is normal. Over time, FEV 1 gets lowerbecause of damage from CF lung disease and exacerbations. Te lower a person’s FEV 1 percent predicted, the more severe the lung disease. Here is a guide to FEV 1 percentpredicted and what it may mean about lung health in CF:

• FEV 1 of 90 percent predicted or higher is thought of as normal. However, even withnormal 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.

Te table below shows how, today, more people with CF have normal or mild lung diseasethan 10 and 20 years ago.

Goal 3: MAINTAIN LUNG HEALTH

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

People with CF often have pulmonary exacerbations. Tis is when lung symptomsget worse and more treatments are needed. Exacerbations can be caused by

infection and inammation. It is important to call your CF care center right awayto nd out if you or your child has an exacerbation and to start treatment early.

Te graph below shows that the lung health of people with CF has improved overthe last 10 years. Since 2002, FEV 1 is usually in a normal range (90 to 100 percentpredicted) when rst measured at around 6 years of age.

Percentage (%) of People with CF with Normal/Mild Lung Disease (FEV 1 >– 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

Signs of an exacerbation:• Coughing more

• More sputum

• More shortness of breath

• A loss of appetite or

• A loss of energy

F E V

1

P e r c e n

t P r e

d i c t e d


20/3218

Te graph below shows the growing number of 18 year-olds with CF who have normal lungfunction or mild lung disease (green bars). Tis increase means more people with CF arehealthier today than ever before.

Lung Health Research

Te CF Foundation supports research to nd new drugs to keep the lungs of people with CFas healthy as possible. Tese drugs may improve FEV 1 or help the CF R protein work better.Learn more about research to keep lungs healthy on the CF Foundation’s 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 makesure it is safe and works. People with CF play a key role in nding new drugs to treat CF.

By volunteering to take part in clinical trials and research, you or your child helpresearchers learn more about the disease and nd new drugs and therapies.

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/Clinical rialAlerts.

Learn more about whatthe FDA does at:

www.fda.gov

0

25

50

75

100

1986 1990 1995 2000 2005 2010 2012

Lung Function in 18 Year Olds, 1986-2012

n FEV 1 Normal/Mild ( >–70%) n FEV 1 Moderate (40% to 69%) n FEV 1 Severe (


21/3219

Therapies for Lung Health

Te following table shows the percentage of people with CF who are taking CF drugsfor 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 .

Not all drugs are right for everyone with CF. Tere are criteria, or reasons, that a drug isprescribed only for some people. For example, the criteria for prescribing the CF medicationivacaftor (Kalydeco™) are:

• Age 6 years and older

• A G551D CF gene mutation

o nd out if you or your child might be helped by one of these drugs, talk with your CFcare center.

Medications alone cannot keep CF lungs healthy. Getting the thick mucus out of the lungsis key. Airway clearance techniques (AC s) help move mucus out of the lungs. Te body’s

natural AC is a cough, which is a reex that clears mucus with high-speed airow. However,for people with CF, mucus cannot be cleared by coughing alone.

Te care guidelines for AC state that people with CF should do airway clearance every dayto keep lungs healthy, even when they are feeling well. When sick, airway clearance should bedone more often.

People with CF often use one or more types of AC . Te graph on the next page shows, byage, the type of AC used most often by people with CF.

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

Azithromycin (such as Zithromax ®) 70.8 70.6

• At least 6 years old• P. aeruginosa in cultures• Weight over 25 kg (55 lbs)• FEV 1 over 30% predicted

High-dose ibuprofen(e.g., 25-30 mg/kg) 3.2 3.3

• 6– 12 years old• FEV 1 over 60% predicted

Ivacaftor (Kalydeco™) -* 81.4 • At least 6 years old• A G551D gene mutation

*Ivacaftor was not available in 2011.


22/32

20

0

10

20

30

40

50

60

70

80

90

< 2 2 - 5 6 - 17 18+

P e r c e n

t o

f P e o p

l e w

i t h C F

Age (Years)

Primary Airway Clearance by Age, 2012

Learn more about:

• CF medications and airway clearance: www.cff.org/treatments/Terapies

• CF respiratory care guidelines: www.cff.org/treatments/CFCareGuidelines/Respiratory

• Watch the CF Education webcasts on:

Respiratory/lungs: www.cff.org/LivingWithCF/Webcasts/ArchivedWebcasts/Respiratory

Partnering for care: www.cff.org/LivingWithCF/Webcasts/ArchivedWebcasts/

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.

• ake medications as prescribed by your CF care center.

• Clean and disinfect nebulizers after every treatment.

• Get a u 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 damagethan CF does alone. Ask your doctor or CF care center staff to help you quit. People with CFshould not smoke. Also, they should avoid secondhand smoke to keep their lungs as healthy aspossible. Secondhand smoke is smoke exhaled by a smoker or from burning tobacco.

** 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 ).

n HFCWO (vest)

n None

n Postural Drainage**

n PEP/Osc PEP

n Exercise*

n Forced Expiration


23/32

21

0

10

20

30

40

50

2006 2007 2008 2009 2010 2011 2012

P e r c e n

t o

f P e o p

l e w

i t h C F

Year

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

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

In 2012, the Registry showed 12.5 percent of people with CF lived withsomeone who smoked. Also, over 330 adults with CF reported that theysmoked. If you smoke, think about quitting. If you have CF and live withsomeone who smokes, ask them to quit. Until that person can quit, ask them toonly smoke outside and away from you. Keeping the home and car smoke-free

can also help the person quit smoking. Te only way to protect yourself or yourchild from secondhand smoke is to be in a 100 percent smoke-free environment.

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

• Learn more about “Secondhand Smoke and CF”: www.cff.org/LivingWithCF/Webcasts/ArchivedWebcasts/Respiratory .

• Te Centers for Disease Control and Prevention has information

about smoking and tobacco use and how to quit at www.cdc.gov/tobacco/quit_smoking .

To make a smoke-free

environment:

• Do not let anyone smokein your home or car.

• Ask people around younot to smoke, even whenoutside.

• Stay away from peoplewhen they are smoking

and teach children to dothe same.

• Ask your employer fora smoke-free work andeating space.

• Make sure that your daycare center or school issmoke-free.

• Choose restaurants andother businesses that are

smoke-free.• Let owners of businesses

that are not smoke-freeknow that secondhandsmoke is harmful to youor your family’s health andthat it will stop you fromgoing to their business.

--------


24/3222

0

10

20

30

40

50

60

70

80


25/3223

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.

Te Foundation supports research to learn more about the germs found in thelungs of people with CF. For example, researchers are studying the best way totreat methicillin-resistantStaphylococcus aureus (MRSA) in people with CF.

Te Foundation also supports the Burkholderia cepacia Research Laboratoryand Repository at the University of Michigan, Ann Arbor.

Even if you are careful and do all you can to avoid germs, you may still get a lung infection.It’s hard to avoid germs completely. If you or your child starts to feel ill — are coughingmore, have more sputum or have a loss of energy or appetite — call your CF care center sotreatment 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 thedisease, particularly CF-related diabetes.

A complication is a disease or injury that happens because of another diseaseor treatment. For people with CF, one of the most common complicationsof CF is cystic brosis-related diabetes (CFRD). In 2012, 19.7 percent ofall people with CF had CFRD. Among those ages 18 years and older, 33.8percent had CFRD.

CFRD is different from diabetes. Research shows that early diagnosis and

treatment of CFRD leads to better nutrition and health. o keep people withCF as healthy as possible, everyone ages 10 years and older should be testedevery year for CFRD. Te test is called an oral glucose tolerance test (OG ).

In 2012, 32.6 percent of people with CF ages 10 and older who did nothave CFRD were tested for CFRD with an OG . (See the Care, Screeningand Guidelines table on page 14). People who have CFRD that is not treatedhave more exacerbations and worse nutrition than those who work with theirdoctors and dietitians to keep their blood sugar under control.

Other problems (or complications) of CF are bone disease and joint problems, such asarthritis 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 orosteoporosis). Preventing bone disease begins early when bones are growing.

A healthy weight, regular exercise and a diet with enough calories, vitamin D, calcium andother vitamins and minerals can help prevent bone disease. o learn more about keepingyour or your child’s bones and joints healthy, talk with your CF care center.

Depression is another problem people with CF may face. For adults with CF, the Registrydata show that 22.2 percent have signs of depression. People living with other chronicdiseases also have signs of depression. If you or your child are feeling hopeless or helpless, youmight be depressed.

Read about:

• MRSA research:www.cff.org/research/DrugDevelopmentPipeline/AdditionalClinicalInitiatives

• Burkholderia cepacia complex:www.cff.org/LivingWithCF/StayingHealthy/Germs/Bcepacia

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


26/3224

It is important to be diagnosed and treated for depression as soonas possible. alk with your CF care center if you think you or yourchild might be depressed. People often respond well to treatment fordepression, by both working with a therapist and taking medicine.

Other complications of CF are listed in the table below. For example, theRegistry data show that 29.7 percent of people with CF reported havingsinus disease in 2012. Tese symptoms include, but are not limited to,headaches, dental pain and feeling facial congestion or fullness. If you oryour child are having these symptoms, talk with your CF care center.

People with CF may also have problems such as gastroesophageal reux

(GERD), distal intestinal obstructive syndrome (DIOS) and liver disease.Te Foundation continues to work to nd ways to help people with CFand these complications.

Other Problems Related to CF, 2012

Complication Percentage (%) of PeopleWho Reported This Problem

Cystic brosis-related diabetes (CFRD) 19.7

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 reux (GERD) 30.5

Distal intestinal obstructive syndrome (DIOS) 5.1

Asthma 25.2

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 andwww.Medlineplus.gov

• Partnering for care:www.cff.org/LivingWithCF/Webcasts/ArchivedWebcasts/PartneringCare

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

• GERD, DIOS and liver disease:

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

0

10

20

30

40

50


27/32

25

Goal 6: TRANSPLANTATION AND END-OF-LIFE CARE

People with CF and their families will be supported by their CF care center when facingdecisions 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 transplantationhas 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 benet from a lung transplant.Te graph below shows the number of people with CF who have had a lung transplant since1990, by year.

13

56 55

93104

148140

152

129 129

152 147138

146

171

154

204

180

161

206

190

232

205

0

50

100

150

200

250

90 91 92 93 94 95 96 97 98 99 00 01 02 03 04 05 06 07 08 09 10 11 12

N u m

b e r o

f L u n g

T r a n s p

l a n

t s

Year

Number of People with CF Who Had a Lung Transplant by Year

Learn more about lung transplantation:

• Read the frequently asked questions at www.cff.org/treatments/Lung ransplantation.

• Watch the CF webcast series at www.cff.org/LivingWithCF/Webcasts/ArchivedWebcasts/Respiratory.

• Read about living with a transplant athttp://transplantliving.org .

• Find data at http://optn.transplant.hrsa.gov/data .

• Search for research at www.clinicaltrials.gov/ct2/search, using the search term “lungtransplant.”

• Find out how to be an organ donor, visit www.OrganDonor.gov .


28/3226

*Data are not mutually exclusive, except “no health insurance,” as people with CF may have more than onetype of insurance.

**“No health insurance” shows the percentage who said they did not have any health insurance in 2012.

Percentage (%) of People with CF by Type of Health InsuranceCoverage, 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

Goal 7: ACCESS TO CARE

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

Research suggests that people with CF who live in households with lower incomes are morelikely to have lower lung function and lower body mass index (BMI) or BMI percentile. Tispattern of health in lower-income households is also common in other chronic diseases, such

as diabetes.Data from the Patient Registry show that drugs such as Pulmozyme®, OBI®,Cayston® and Kalydeco™ are available to people with CF, regardless of incomelevel. o learn more about medication assistance and how others manageCF, watch the webcast series “CF Healthcare Coverage and Advocacy” and“Building Life Skills to Manage CF” at www.cff.org/LivingWithCF/Webcasts/

ArchivedWebcasts/PartneringCare.

Tere are a number of programs available to help people with CF get the careand medications they need. o nd more assistance programs for people

with CF, visit the Foundation’s Patient Assistance Resource Center at www.cff.org/LivingWithCF/AssistanceResources.

Te Foundation supports changes to health care that help people with CF. Tehealth 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 CFages 18 through 25 years stayed on their parents’ health insurance.

Te table below shows the percentage of people with CF and the types of health insurancecoverage they had in 2012.

A local CF care center is the best source for up-to-date information on health care coverageprograms in your area and state. Call your CF care center for help.

The Cystic FibrosisPatient AssistanceFoundation (CFPAF), anonprot subsidiary ofthe CF Foundation, helpspeople with CF in theUnited States get theirprescribed FDA-approved

drugs and devices.• Visit CFPAF’s website

www.cfpaf.org

• Call 1-888-315-4154


29/3227

Summary of the Data: People in the CF Foundation’s Patient Registry

Characteristic 1992 2002 2012

People with CF (number) 18,497 23,064 27,804

Newly diagnosed (number) ‡‡ 1,011 1,040 920

Age at diagnosis (median) 6 months 6 months 4 months

Age range (years) 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

Predicted median survival (years) – 95% condence interval 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 AmericanHispanic

96.13.34.1

95.33.95.7

94.14.57.7

Males (%) 53.9 52.7 51.7Nutrition 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

99.936.120.0

--

95.042.221.3

8.631.7

87.352.022.2

11.443.0

Respiratory/Lungs FEV 1 % predicted (mean) 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-

57.84.53.1

55.99.2

49.69.52.6

69.026.5

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., 25–30 mg/kg) Ivacaftor (i.e., Kalydeco™)

-------

64.7-

70.8-

11.66.8

-

83.760.465.739.070.63.3

81.4

Transplants (number) Lung (any type) Liver Kidney

5512-

138141

2051911

Clinical research participation (number) 966 5,872 5,439

Pregnancies (number) 116 182 249

Live birth rates (per 100 women ages14–45 years) 1.5 1.9 2.0

‡‡ Due to data adjustments, some people diagnosed with CF in 2012 will be entered into the Registry in 2013.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 goalfor children with CF ages 2-19 years is 50th BMI percentile. For adults with CF, the national goal for weight is aBMI 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.

SUMMARY TABLES


30/3228

Number of People with CF by State*

State Number Percent State Number Percent

Alabama 451 1.62 Nebraska 242 0.87

Alaska 101 0.36 Nevada 186 0.67

Arizona 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 1 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 4 0.01Montana 123 0.44 Foreign 7 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.


31/32


32/32

CYSTIC FIBROSIS FOUNDATION

6931 Arlington RoadBethesda, MD [email protected]

Documents

2012 CFF Patient Registry