title: The Cystic Fibrosis Foundation Patient Registry. Design and Methods of a National Observational Disease Registry.
abstract: The Cystic Fibrosis Foundation Patient Registry (CFFPR) is an ongoing patient registry study that collects longitudinal demographic, clinical, and treatment information about persons with cystic fibrosis (CF) in the United States. CF is a life-shortening genetic disorder that occurs in approximately 1 in 3,500 births in the United States. High-quality observational data is important for clinical research, quality improvement, and clinical management.
  
  
  
  To describe the data collection, patient population, and key limitations of the CFFPR.
  
  
  
  Inclusion criteria for the CFFPR include diagnosis with CF or a CFTR-associated disorder, care at an accredited care center program, and provision of informed consent. Data from clinic visits and hospitalizations are collected through a secure website. Loss to follow-up and generalizability were examined using several methods. The accuracy of CFFPR data was evaluated with an audit of 2012 CFFPR data compared to the medical record.
  
  
  
  Since 1986, the CFFPR contains the records of 48,463 individuals with CF. Participation among individuals seen at accredited care centers is high, and loss to follow-up is low. An audit of 2012 CFFPR data suggests that the CFFPR contains 95% of clinic visits and 90% of hospitalizations found in the medical record for these patients, and nearly all of the audited fields were highly accurate.
  
  
  
  Registries such as the CFFPR are important tools for research, clinical care, and tracking incidence, mortality and population trends.
authors.affiliation: 1 Cystic Fibrosis Foundation, Bethesda, Maryland.
authors.forename: Emily A
authors.identifier: 0000-0003-3953-9874
authors.initials: EA
authors.lastname: Knapp
authors.affiliation: 1 Cystic Fibrosis Foundation, Bethesda, Maryland.
authors.forename: Aliza K
authors.initials: AK
authors.lastname: Fink
authors.affiliation: 2 Department of Medicine, University of Washington, Seattle, Washington; and.
authors.forename: Christopher H
authors.initials: CH
authors.lastname: Goss
authors.affiliation: 3 Sewall, Inc., Bethesda, Maryland.
authors.forename: Ase
authors.initials: A
authors.lastname: Sewall
authors.affiliation: 1 Cystic Fibrosis Foundation, Bethesda, Maryland.
authors.forename: Josh
authors.initials: J
authors.lastname: Ostrenga
authors.affiliation: 1 Cystic Fibrosis Foundation, Bethesda, Maryland.
authors.forename: Christopher
authors.initials: C
authors.lastname: Dowd
authors.affiliation: 1 Cystic Fibrosis Foundation, Bethesda, Maryland.
authors.forename: Alexander
authors.initials: A
authors.lastname: Elbert
authors.affiliation: 1 Cystic Fibrosis Foundation, Bethesda, Maryland.
authors.forename: Kristofer M
authors.initials: KM
authors.lastname: Petren
authors.affiliation: 1 Cystic Fibrosis Foundation, Bethesda, Maryland.
authors.forename: Bruce C
authors.initials: BC
authors.lastname: Marshall
mesh_terms: D000293:Adolescent; D000328:Adult; D000553:Ambulatory Care / Q000706:statistics & numerical data; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000453:epidemiology*; D000068598:Data Accuracy; D005260:Female; D005582:Foundations; D006760:Hospitalization / Q000706:statistics & numerical data; D006801:Humans; D015994:Incidence; D059012:Lost to Follow-Up; D008297:Male; D012042:Registries; D014481:United States / Q000453:epidemiology; D055815:Young Adult
keywords: cystic fibrosis; registries