2013 Cystic Fibrosis Foundation Patient Registry Annual Data Report

8/9/2019 2013 Cystic Fibrosis Foundation Patient Registry Annual Data Report

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PatientRegistry

2013Annual Data Report


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To the CF Community and Friends,

We are pleased to present the Cystic Fibrosis Foundation’s 2013 Patient RegistryAnnual Data Report.

For close to 50 years, the Foundation has collected information on the health of peoplewith cystic brosis who receive care at Foundation-accredited care centers. We then sharethe information with the wider CF community, highlighting trends in key health outcomesto help people with CF and their families, clinicians and researchers work together to raisethe quality of care.

Thanks to steady progress in CF care and treatment, the face of the disease has changeddramatically over the last few decades. We are now on the cusp of an important milestone,when more than half of those with CF in the United States will be 18 years and older, andwe anticipate continued growth in the adult CF population in the years to come.

Positive trends shown in this year’s report include:

• Continued improvements in pulmonary function and nutritional status

• Increase in the number of new CF diagnoses through newborn screening

• Decrease in lung infections from Pseudomonas aeruginosa and MRSA

The data also show that much work remains in order for all people with CF to be betterable to lead healthy and fullling lives. Many people with CF still require hospitalization fortreatment of exacerbations and, as the CF population ages, many now face other healthproblems like CF-related diabetes and depression, which add to the daily demands ofliving with this disease. We remain committed to addressing these challenges.

This year, we have used more graphics to summarize the data and convey more fully theimpact that CF has on those living with the disease. We hope this report encouragespeople with CF to partner with their care center teams and take an active role in shapingan individualized care plan to stay healthy and thrive.

We are deeply grateful to all who have contributed to this report, especially people withCF and their families who so generously agree to share their information.

Thank you for your continued commitment to our mission to cure CF.

Sincerely,

Preston W. Campbell, III, MDExecutive Vice PresidentMedical Affairs

Bruce C. Marshall, MDSenior Vice PresidentClinical Affairs

Preston W.Campbell, III,

Bruce C.Marshall, MD

Robert J. Beall, PhDPresident and CEO

The Mission of the Cystic Fibrosis FoundationThe mission of the Cystic Fibrosis Foundation is to cure cystic brosis and toprovide all people with the disease the opportunity to lead full, productive lives byfunding research and drug development, promoting individualized treatment, andensuring access to high-quality, specialized care.

Since its inception in 1966, the Cystic FibrosisFoundation’s Patient Registry has been central to ourefforts to ensure all people with CF receive the highest-quality, specialized care and most effective treatments.As the Registry has expanded to capture and analyze abroader range of data, it has become an indispensabletool in many important initiatives – from qualityimprovement in Foundation-accredited care centers tothe design of clinical trials studying a wide array of CFtherapies.

The steady gains in the health of people with CFhighlighted in this year’s Registry report reect the hardwork and dedication of many, especially those livingwith CF and their families. I thank our community for its

contributions to this crucial endeavor and look forwardto working with you to achieve our shared mission ofending this disease.


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Highlights of CF Foundation Patient Registry Data

From the analysis of the CF Foundation Patient Registry data in 2013

More than

28,000 people with CF were seen at aCF Foundation-accredited care

center and consented to have theirdata or their child’s data entered into

the Registry. Almost 50 percent ofthose followed in the Registry were

age 18 years or older.

2,697 people with CF

were 40 years or older.

Sweat testing is an extremely valuable diagnostic

test, yet fewer people currently havea sweat test recorded in the Registry

than in the past. This is especiallycommon among those with twocopies of the F508del mutation.

66%of new CF diagnoses

were made in therst year of life.

Pseudomonas prevalence is declining and the

prevalence of MRSA has stabilized.Nontuberculous mycobacteria (NTM)

infections are of concern.

Of adults with CF,

46%are working full time

or part time and

22%were students.

The median predicted age ofsurvival has increased from

33.4 years in 2003 to

40.7 years in 2013.

Registry data showscontinuous

improvement in

nutrition and

pulmonary health outcomes inchildren and adults.

97% of people with CF have had

their mutationsidentied through genetic

testing.

About the CF Foundation Care Model 4

• Care Center Network 4

• CF Foundatio n Clinical Care Practice Guidelines 4

• Quality Improvement Initiative 4

• About the CF Foundation Patient Registr y 5

• CF Foundatio n Resources and Assistance Programs 5

Overview of the Health of People with CFand the Standards of Care in theCF Foundation Care Center Network 6

• Demographics 6

• Diagn osis of CF 8

• Sweat Test Data 9

• CF Genetics 10

• CF Clinical Care Practice Guidelines: Care, Screening and Prevention 12

• Microbiology 13

• Growth and Nutrition 14

• Lung Health 16

• Complications 19

• Transplantation 21

• Survival 22

Resources 23

Table of Contents


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CF Foundation Clinical Care Practice Guidelines

The CF Foundation provides accredited care centers with clinical care practiceguidelines, which are updated regularly based on the latest research, care andtreatments. The Foundation brings together committees of subject-matter experts,including physicians, nurses, respiratory therapists and dietitians, along with adultswith CF and CF parents, to develop care recommendations on each topic.

Quality Improvement Initiative

Through its quality improvement initiative, the CF Foundation works closely withcare centers to ensure all people with CF receive the highest quality of care. Thequality improvement initiative is aimed at identifying best practices for CF care andtreatment, and providing training and tools to implement improvements across thecare center network. People with CF and their families are important partners in thisprocess.

About the CF Foundation Care Model

Care Center Network

The CF Foundation accredits and funds anationwide network of more than 120 care centers.Multidisciplinary teams of health care professionalsat the care centers work together to provide expert,age-appropriate care tailored to meet the uniqueneeds of individuals living with CF.

Each center undergoes an assessment by theCF Foundation’s Care Center Committee before it

receives accreditation and funding. Accredited centersare reevaluated annually to ensure that people withCF receive effective and consistent levels of care andstate-of-the-art treatments.

The CF Foundation’s care center network has beenwidely recognized as a national model for care of achronic disease and for driving improvements in care.

nurse physician respiratorytherapist

Required Team Members Recommended Team Members

dietitian

socialworker

programcoordinator

physicaltherapist

researchcoordinator

psychologist pharmacist

The Multidisciplinary Team

RT ψ

About the Cystic Fibrosis Foundation Patient Registry

Each year, information on the health status of children and adults with CF whoreceive care at CF Foundation-accredited care centers is entered into the Registry.This information provides critical data to help care teams and researchers identify newhealth trends, recognize the most effective treatments, design CF clinical trials anddevelop clinical care practice guidelines.

Uses of theCystic Fibrosis Foundation Patient Registry

DISEASESURVEILLANCE

FRAMEWORK FORCLINICAL TRIALS

POST-MARKETINGSURVEILLANCE

STUDIES

QUALITYIMPROVEMENT

COMPARATIVEEFFECTIVENE

RESEARCH

Track progressin curing CF and

the impact oftreatments

Test promisingnew therapies

Ensure safetyand effectiveness

of approvedproducts

Provideall patients

withhigh-quality care

Promoteevidence-based

clinicaldecision making

In 2013,

25% of people

with CFparticipated in a

patient assistanceprogram through

CFF or anothersource.

CF Foundation Resources and Assistance Programs

The CF Foundation offers a variety of resources and programs to helppeople with CF obtain essential CF care and treatments. Please referto the appendix in the back of this report for information on patientassistance resources.


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Overview of the Health of People with CF and the Standards of Care in the CF Foundation Care Center Network

Demographics Records of 28,103 people with CF were included in the Registry in 2013.

People with CF are living longer and healthier lives than ever before. Today, nearlyhalf of all people with CF in the United States are adults. We project that survival willcontinue to improve over the next decade and beyond.

1 8

2013

49.7%

People with CF age18 years or older

or older

1986

29.2%

0 – 99

100 – 199

200 – 499

500 – 999

≥1,000

618

407

2,332

402

189

180

125

138

373

1,744

552

54

65

16

261

366

246

113

74

319

261

717

392

592

649

1,047 6781,523

1,063

1,659

262

570

667

217 444 782

1,434

381

883

667

1,743

236

38

68538385

28499

914183

118

Number of people with CF

Number of People with CF Included in the Registry in Each State

Number of Children and Adults with CF, 1986–2013

0

5,000

10,000

15,000

20,000

25,000

30,000

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 13

N u m

b e r o

f P a

t i e n

t s

Year

Adults 18 Years and OlderChildren Under 18 Years

Of adults with CF,

46%work

full time orpart time

and

22% are students.

As people with CF are living longer and pursuing moreopportunities, there are more college graduates in theCF population. There are also more adults who areworking full time or part time. Since the late 1990s, thenumber of people with CF with college degrees hasmore than doubled.

Characteristics of Adults 18 Years and Older with CF in 2013

Marital Status

Widowed0.2%

Separated/Divorced

4.9%

Married/ Living

Together 40.9%

Single54.0%

Unemployed8.2%

Homemaker4.3%

EmploymentRetir1.6

Disabled17.6%

Student22.0%

Full T34.3

PartTime

12.0%

Education

Less ThanHigh School

6.8%

Masters/Doctoral-Level

Degree6.4%

High SchoolDiploma23.8%

SomeCollege33.7%

CollegeGraduate

29.2%

Distribution of Race/Ethnicity among People with CF

African American4.3%

Hispanic 7.9%

Other 1%

White86.8%


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Diagnosis of CF Nationwide newborn screening for CF has been in place since 2010. As a result, morepeople with CF are now diagnosed in infancy, often before symptoms of the diseaseappear. While a growing proportion of people with CF are diagnosed by newbornscreening, some are not diagnosed until adolescence or adulthood. E arly diagnosisallows for earlier treatment, which may lead to better lung function and nutritionaloutcomes later in life.

While newborn screening provides opportunities for early intervention, it alsoincreases the risk of misdiagnosing symptomless infants. In these cases, infantsmay receive unneeded CF treatments that can be potentially harmful. Visiting a CFFoundation-accredited care center to receive a complete diagnostic evaluation isextremely important for people who are newly diagnosed. A complete diagnosticevaluation includes a sweat test, genetic test and clinical evaluation.

In 2013, 60% of newdiagnoses were detected

by newborn screening.

Age at Diagnosis for all People with CF in the Registry, 2013

Prenatal, 2.3%

Under 1 Month, 29.0%

1 to 3 Months, 13.2%



1 Year Old, 6.6%

2-15 Years Old, 20.8%

16 Years and Older, 6.8%

Under1 Year,65.7%

Percent of New Diagnoses Detected by Newborn Screening, 1990-2013

Sweat Test Data The CF Foundation guidelines for diagnosis of cystic brosis recommend thata sweat chloride test be part of the diagnostic evaluation for CF. Des pite thisrecommendation, there is a decrease in the number of individuals with a sweatchloride value reported in the Registry among individuals who are newly diagnosedand especially among those with two copies of the F508del mutation. This decreasein reported sweat tests may be due to an increased reliance on genetic testing todetermine a denitive diagnosis of CF.

97 82 75Percent of

newly diagnosedpeople with CF

with sweat valuesrecorded.

Percent of Patients with Sweat Chloride Values Reported by Year of Diagnosis, 1986-2013

1993 2003 2013

60

70

80

90

100

1988 1993 1998 2003 2008 2013

P e r c e n

t o

f P

a t i e

n t

s

Year

F508del Homozygotes Other

Percent of Patients with a Sweat Chloride Reported by Year of Diagnosis, 1988–2013

0

20

40

60

80

100

90 91 92 93 94 95 96 97 98 99 00 01 02 03 04 05 06 07 08 09 10 11 12 13

P e r c e n

t

Year


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Homozygotes (two copies) - 46.5%Heterozygotes (one copy) - 39.9%

97%of peoplewith CFhad theirmutationsidentiedthroughgenetictesting.

Prevalence of the 25 Most Common CFTR Mutations in 2013

MUTATION NUMBER OF PEOPLEPERCENT OF PEOPLE WITH

ONE OR MORE COPY OFTHE MUTATION

F508del 23,478 86.4

G542X 1,252 4.6

G551D 1,182 4.4

R117H 767 2.8

N1303K 672 2.5

W1282X 625 2.3

R553X 493 1.8

621+1G->T 437 1.6

1717-1G->A 425 1.6

3849+10kbC->T 411 1.5

2789+5G->A 369 1.4

3120+1G->A 267 1.0

I507del 220 0.8

D1152H 196 0.7

R1162X 193 0.7

3659delC 189 0.7

1898+1G->A 187 0.7

G85E 178 0.7

R560T 165 0.6

R347P 158 0.6

2184insA 151 0.6

R334W 145 0.5

A455E 142 0.5

Q493X 129 0.5

2184delA 116 0.4

CF Genetics In people with CF, mutations in both copies of the cystic brosis transmembraneconductance regulator (CFTR) gene disrupt normal production of the CFTR protein.Different mutations cause CFTR to malfunction in different ways. In some people with CF,little to no CFTR is produced. In others, the defective protein is produced, but cannotmove to the surface of the cell where it is needed to regulate the transfer of chlorideand water in and out of cells. In others, CFTR is produced and moves to the surfaceof the cell but the gate that controls chloride movement does not open properly. Themalfunctioning CFTR leads to an accumulation of unusually thick and sticky mucus in thelungs, pancreas and other organs.

Today, as new therapies are developed to target problems caused by specicCF mutations, it is extremely important for each person with CF to know his orher mutations. There are more than 1,500 known CFTR mutations, many of whichresearchers have categorized into different groups. People with two mutations inclasses I, II and III typically exhibit more severe pulmonary disease and pancreatic

insufciency as compared to people with at least one mutation in classes IV and V.

CFTR Mutation Classes

Adapted from: http://www.umd.be/CFTR/W_CFTR/gene.html

Cl -Cl-

Cl -

Cl -

Cl - Cl-

XX

X X Cl -Cl

-

X

Cl -Cl-

Class I

NofunctionalCFTR

created.

G542XW1282XR553X

Class II

CFTR proteinis created,but misfolded,keeping it from

reaching thecell surface.

F508delN1303K I507del

Class III

CFTR proteinis created andreaches cellsurface, but

does notfunction properly.

G551DS549N V520F

Class IV

The openingin the CFTRprotein ion

channelis faulty.

R117HD1152HR347P

Class V

CFTRis createdin insufcient

quantities.

3849+10kbC->T2789+5G->A

A455E

Normal

CFTR is created,reaches cellsurface and

functions properly,allowing transfer

of chloride and water.

The chart below shows just one of the ways that researchers classify CFTR mutations. Individuals with CFcan consult with a genetic counselor or other member of their care team to learn more about their specicmutations. To nd more information on CFTR and mutation classes, visit www.cftr2.org.

E X A M P L E S

D E S C R I P T I O N


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CF Clinical Care Practice Guidelines: Care, Screening and Prevention

CF clinical care practice guidelines are developed by expert multidisciplinary committees, basedon published evidence and clinical experience. Guidelines are intended to inform care centersabout CF care and treatment best practices and to be adapted by care center teams to theneeds, preferences and values of the individual with CF and his or her family.

Current CF Foundation clinical care practice guidelines recommend that individuals ages 6 andolder visit their care center at least four times, receive four microbiological cultures and performtwo pulmonary function tests (PFTs) per year. There are other annual guidelines for CF care,some of which are listed below.

4

clinic visits 4

cultures

2

PFTs

CF Foundation Annual Clinical Care Practice Guidelines

Care, Screening and Prevention Guidelines for People with CF

PERCENT OF PEOPLE WITH CF WHO HAD2013

ELIGIBILITY CRITERIAFOR RECOMMENDATIONUNDER

18 YEARS18 YEARS

AND OLDER ALL

4 or more clinic visits 74 56 65 All

4 or more sputum/throat cultures 63 40 52 All

2 or more lung function tests (PFTs) 91 87 89 If 6 years of age or older and physically able

An inuenza vaccine (u shot) 96 93 95 If 6 months of age or olderFat-soluble vitamin blood levels measured(Vitamins A, D & E) 90 80 85 All

An oral glucose tolerance test (OGTT) 50 29 37 If 10 years of age or older

A blood test to measure liver enzymes 84 76 80 All

Microbiology Pulmonary infections are a serious and chronic problem for many living with CF. Peoplewith CF are at greater risk of getting lung infections because the thick and sticky mucusthat accumulates in their lungs allows germs to thrive and multiply. The prevalence ofthe bacteria, Pseudomonas aeruginosa or Pseudomonas has been gradually decreasingover time in people with CF. Prevalence of nontuberculous my cobacteria (NTM)infections is increasing in the general population. This is of concern because peoplewith CF are at a higher risk of developing NTM infections which require long periods oftreatment with multiple antibiotics.

To help reduce the spread of germs, the infection prevention and control guidelinesfor CF were created. These CF Foundation guidelines provide recommendations forpeople with CF, their families and CF health care professionals to help reduce thespread of germs in the clinic and hospital setting, and at home, school or work.

S. aureus

P. aerugino

MRSA

MDR-PA

H. inuenz

S. maltoph

A. xylosox

B.cepaciacomplex

Prevalence of Respiratory Microorganisms in People with CF, 1988–2013

The majority of people with CF followed in the Registry receive care as recommended bythe Guidelines; however, adults with CF do not receive guideline-recommended care to thesame extent as children. There are various possible reasons for this difference. Teens andadults may be busy with school or jobs, and some may have milder disease.

Change in percent of people with CF with a positiveculture of Pseudomonas over the last 10 years

Percent ofpeople with CFtested between2010 and 2013who hadNTM infections

down

8%

0

20

40

60

80

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 13

P e r c e n

t o

f P

a t i e

n t

s

Year

.

.

. I

.

.

Prevalence of Respiratory Microorganisms, 1988–2013

16%


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1993

20%2013

6%

Percentage of people with CFwith a BMI percentile less than 10.

1993

20%2013

7%

Percentage of people with CFwith a BMI less than 18.

Median BMI Value by Age, In Adults 20 years and Older, 1988-2013

19

20

21

22

23

1988 1993 1998 2003 2008 2013

B M I

Years

20 Year Olds 25 Year Olds 30 Year Olds 35 Year Olds

l ,

Better nutrition is associated with better lung function in people with CF. Thisassociation suggests that maintaining a healthy weight is important not only tonutrition and growth, but to lung function as well. This association is especiallyimportant for infants whose length and weight can be measured, but who cannotperform lung function tests.

Growth and Nutrition

Children and teens with CF need adequate nutrition to grow andprosper, and it’s important for adults to maintain proper nutritionto stay healthy. Because of the thick secretions that build up in thepancreas and prevent the release of digestive enzymes, people withCF have difculty absorbing vital vitamins and nutrients from food,which leads to poor growth and malnutrition. To address this, mostpeople with CF take pancreatic enzyme supplements.

In recent decades, nutritional outcomes have improved markedly forboth children and adults. Body mass index (BMI) and BMI percentile are two important measures for assessing growth, nutrition andgastrointestinal function in people with CF.

Despite the gains in BMI, height and weight percentiles amongpeople with CF remain below the 50th percentile, suggesting roomfor improvement.

BMI Percentile

BMI percentilematches a child’s BMI to otherchildren in the United Statesof the same age and gender.A BMI of the 50th percentilemeans half of the children of thesame age and gender are largerand half are smaller.

Median Nutritional Outcome Percentiles for Children and Adolescents Ages 2 to 19 years, 1986-2013

0

10

20

30

40

50

60

86 89 92 95 98 01 04 07 10 13

P e r c e n

t i l

e

Year

BMI Weight Height

BMI Body mass index (BMI)is based on a person’sweight and height. BMI is

calculated by dividing body weightin kilograms (kg) by the person’sheight in meters squared (m 2).

87% of people with CF takepancreatic enzymesupplements.


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37.342.1

51.056.8

66.671.9

38.634.5

34.8

33.7

27.8 21.0

24.1 23.414.2

9.6 5.5 7.2

0

25

50

75

100

1988 1993 1998 2003 2008 2013

P e r c e n

t o

f P

a t i e

n t

s

l

37.3

38.6

24.1

42.1

34.5

23.4

51.0

34.8

14.2

56.8

33.7

9.6

66.6

27.8

5.5

71.9

21.0

7.2. .

..

..

..

..

. .. .

. .. .

FEV1 Severe (


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Complications

CF is often associated with complications other than lung disease and impairednutritional status. These complications can be a direct result of the disease or aresult of treatments for CF.

Detecting complications early and managing them properly is crucial for the healthand well-being of those with CF. Complications of CF include cystic brosis-relateddiabetes (CFRD), liver disease, bone disease, distal intestinal obstructive syndrome(DIOS), gastroesophageal reux disease (GERD) and depression.

35% of peoplewith CF age18 years andolder have

CFRD.

Prevalence of Common Complications by Age in 2013

0

10

20

30

40

50


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4,657

people with CFused insulin

in 2013.

Cystic Fibrosis-Related Diabetes (CFRD)

CFRD is a form of diabetes that is unique to people with CF. Peoplewith CFRD have to undergo more treatments than those without thecomplication and most commonly use insulin. It is one of the mostwidespread complications of the disease, especially among adultswith CF. As the number of adults with CF has increased, so has theprevalence of CFRD. CFRD is also more common in people withmutations in classes I – III.

Research shows that early diagnosis and treatment of CFRD leads tobetter nutrition and pulmonary function. The CF care guidelines forCFRD recommend that people with CF ages 10 and older should betested annually for CFRD via the oral glucose tolerance test (OGTT).

Prevalence of CFRD, 1988–2013

Transplantation Lung transplantation remains an option for some people with CF whohave severe lung disease. However, lung transplantation has its ownrisks and requires life-long, post-transplant care.

The Registry also collects data on people with CF who have had othertypes of transplants, and there were 135 people with CF followed whoreceived a kidney, heart or liver transplant in 2013 or in a prior year.

Number of Patients Receiving a Lung Transplant, 1990–2013

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 13

N u m

b e r o

f P a

t i e n

t s

Year

In 2013,

245people with CFreceived alung transplant.

The medianage ofrecipientswas year

Complications

31

0

5

10

15

20

25

30

35

40

1988 1993 1998 2003 2008 2013

P e r c e n

t o

f P a

t i e n

t s w

i t h C F R D

Ye a r

Patients 10 to 17 Years Patients 18 Years and Older


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Survival

People with CF are living longer than ever before with the median predictedsurvival age continuing to increase.

40.7years

Medianpredicted

survival agein 2013.

Median Predicted Survival Age, 1989–2013 (in 5 year bands)

MedianPredictedSurvival Age

The median predictedsurvival age is the age towhich half of the currentRegistry populationwould be expected tosurvive, given their agesin 2013 and assuming thatmortality rates do notchange. Median predictedsurvival age is calculatedusing a method called life

table analysis.

The CF Foundation is committed to promoting individualized treatmentand ensuring access to high-quality, specialized care for people with CF.The ndings from 2013 Registry data provide much insight into the currenthealth of the CF population and also provide clinicians with a road map forimproving care. As new treatments and therapies emerge, the Registry willremain a rich resource for researchers, clinicians, people with CF and familiesas they partner to improve care, treatment and research.

Appendix of Resources

About the CF Foundation Care Model Care Center Network

Care Center Networkwww.cff.org/treatments/CareCenterNetwork/

Partnering for Care: CF Experts Talk About Managing Lifewith CF

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

CF Foundation Clinical Care Practice Guidelines

CF Care Guidelineswww.cff.org/treatments/CFCareGuidelines

Quality Improvement Initiative

Improve Your CF Carewww.cff.org/LivingWithCF/QualityImprovement/

ImproveYourCare/Quality Improvement

www.cff.org/LivingWithCF/Webcasts/ArchivedWebcasts/PartneringCare/#Partnering_for_Improvement

www.cff.org/LivingWithCF/QualityImprovement

Overview of the Health of People with CF andthe Standards of Care in the CF FoundationCare Center Network Demographics

CF Healthcare Coverage and Advocacy and Building LifeSkills to Manage CF

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

Cystic Fibrosis Patient Assistance Foundationwww.cfpaf.org

Information for Adults with CFwww.cff.org/Adults

Patient Assistance Resource Centerwww.cff.org/LivingWithCF/AssistanceResources

Scholarships and Financial Aid for People with CFwww.cff.org/LivingWithCF/Scholarships/

Diagnosis of CF

CF Care Guidelines – Age Specic Carewww.cff.org/treatments/CFCareGuidelines/

AgeSpecicCare

CF Infant Care: First Year of Lifewww.cff.org/LivingWithCF/StayingHealthy/CFInfantCare

Testing for Cystic Fibrosiswww.cff.org/AboutCF/Testing

Webcast – CF Infant Care: F irst Year of Lifewww.cff.org/LivingWithCF/Webcasts/ArchivedWebcasts/

AgeSpecic/#Infant_Care

Sweat Test Data

The Sweat Testwww.cff.org/aboutcf/testing/sweattest/

CF Genetics

About CF Geneticswww.cff.org/AboutCF/Testing/Genetics

CF Mutation Analysis Programwww.cff.org/LivingWithCF/AssistanceResources/MAP

Clinical and Functional Translation of CFTR (CFTR2)www.cftr2.org/

Genetics-Related CF Webcastswww.cff.org/LivingWithCF/Webcasts/ArchivedWebcast

Genetics/

Types of CF Mutationswww.cff.org/AboutCF/Testing/Genetics/CFMutations/

Microbiology

Additional Clinical Initiatives: Burkholderia cepacia,Methicillin-resistant Staphylococcus aureus (MRSA) andNontuberculous Mycobacteria (NTM)

www.cff.org/research/DrugDevelopmentPipeline/AdditionalClinicalInitiatives

Centers for Disease Control and Prevention (CDC)Information on Handwashing

www.cdc.gov/handwashing

Centers for Disease Control and Preventions (CDC)Information on Vaccines

www.cdc.gov/vaccines

CF Care Guidelines – Infection Prevention and Controlwww.cff.org/treatments/CFCareGuidelines/

InfectionControl

Get Germ Smartwww.cff.org/LivingWithCF/StayingHealthy/GermSmart

Information about Burkholderia cepaciawww.cff.org/LivingWithCF/StayingHealthy/Germs/

Bcepacia

Information about Allergic BronchopulmonaryAspergillosis

www.cff.org/LivingWithCF/StayingHealthy/Germs/ABP

Methicillin-resistant Staphylococcus aureus (MRSA) and

Cystic Fibrosiswww.cff.org/LivingWithCF/StayingHealthy/Germs/MR

Webcasts on Germs and Infection Controlwww.cff.org/LivingWithCF/Webcasts/ArchivedWebcast

Germs

24

28

32

36

40

1989 - 1993 1994 - 1998 1999 - 2003 2004 - 2008 2009 - 2013

M e

d i a n

P r e

d i c t e d S u r v

i v a l

( Y e a r s

)

Year

l -

1989–1993 1994–1998 1999–2003 2004–2008 2009–2013


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Growth and Nutrition

Centers for Disease Control and Prevention (CDC) ClinicalGrowth Charts

www.cdc.gov/growthcharts/clinical_charts.htm

CF Care Guidelines – Nutrition/Gastrointestinal (GI)www.cff.org/treatments/CFCareGuidelines/Nutrition

Food and Recipes for People with CFwww.cff.org/LivingWithCF/StayingHealthy/Diet/

FoodIdeasRecipes/

Information about Body Mass Indexwww.cdc.gov/healthyweight/assessing/bmi

Nutrition/Gastrointestinal (GI) Webcastswww.cff.org/LivingWithCF/Webcasts/ArchivedWebcasts/

Nutrition

Staying Healthy with CFwww.cff.org/LivingWithCF/StayingHealthy

World Health Organization (WHO) Growth Standards andWHO Growth Charts

www.cdc.gov/growthcharts/who_charts.htm

Lung Health

About Clinical Trialswww.cff.org/research/ClinicalResearch

Centers for Disease Control and Prevention (CDC)Information about How to Quit Smoking

www.cdc.gov/tobacco/quit_smoking/index.htm

CF Care Guidelines - Respiratorywww.cff.org/treatments/CFCareGuidelines/Respiratory

Drug Development Pipelinewww.cff.org/research/DrugDevelopmentPipeline

Find a Clinical Trialwww.cff.org/research/ClinicalResearch/Find

Global Lung Initiative (GLI) Reference Equations forSpirometry

lungfunction.org/les/GLI-2012_Reference_values.pdf

Information about CF and Exercisewww.cff.org/Adults/DailyLife/Exercise

Risks of Smoking for People with CFwww.cff.org/LivingWithCF/StayingHealthy/LungHealth/

Smoking/

Sign up for Clinical Trial Alertswww.cff.org/research/ClinicalResearch/Find/

ClinicalTrialAlerts

Therapies for Cystic Fibrosiswww.cff.org/treatments/Therapies

Webcasts on CF Researchwww.cff.org/LivingWithCF/Webcasts/ArchivedWebcasts/

Research

Webcasts on Respiratory/Lungswww.cff.org/LivingWithCF/Webcasts/ArchivedWebcasts/

Respiratory

Complications

CF and Depression and Anxietywww.cff.org/Adults/Other/DepressionAnxiety

Cystic Fibrosis-Related Diabetes (CFRD)www.cff.org/LivingWithCF/StayingHealthy/Diet/

Diabetes/

Nutrition and Bone Healthwww.cff.org/UploadedFiles/treatments/Therapies/

Nutrition/BoneHealth/Nutrition-Bone-Health-and-Cystic-Fibrosis.pdf

Transplantation

Clinical Trials Relevant to Lung Transplantationwww.clinicaltrials.gov/ct2/search using the term “lung

transplant”

Information on Lung Transplantationwww.cff.org/treatments/LungTransplantationtransplantliving.orgoptn.transplant.hrsa.gov/data

Organ Donationwww.organdonor.gov

Webcasts on Lung Transplantationwww.cff.org/LivingWithCF/Webcasts/ArchivedWebcasts/

Respiratory

Survival

CF Life-Expectancywww.cff.org/AboutCF/Faqs/#CF-life-expectancy

If you have any questions about CF care, pleasetalk with your CF care center or contact theFoundation at 1-800 FIGHT CF or [email protected]

SOURCE OF DATACystic brosis patients under care at CF Foundation-accreditedcare centers in the United States, who consented to have theirdata entered in 2013.

SUGGESTED CITATIONCystic Fibrosis Foundation Patient Registry2013 Annual Data ReportBethesda, Maryland©2014 Cystic Fibrosis Foundation

COVER PHOTO BY Cade Martin Photography


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CYSTIC FIBROSIS FOUNDATION6931 Arlington RoadBethesda, MD [email protected]

2013 Cystic Fibrosis Foundation Patient Registry Annual Data Report

Documents

Transcript of 2013 Cystic Fibrosis Foundation Patient Registry Annual Data Report