Post on 01-Jun-2018
8/9/2019 2013 Cystic Fibrosis Foundation Patient Registry Annual Data Report
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PatientRegistry
2013Annual Data Report
8/9/2019 2013 Cystic Fibrosis Foundation Patient Registry Annual 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%
4 to 6 Months, 12.2%
7 to 11 Months, 9.0%
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 info@cff.org
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
8/9/2019 2013 Cystic Fibrosis Foundation Patient Registry Annual Data Report
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CYSTIC FIBROSIS FOUNDATION6931 Arlington RoadBethesda, MD 208141.800.FIGHT.CFwww.cff.orginfo@cff.org