cystic fibrosis foundation patient registry 2016 annual data report, bethesda, maryland

What is the Cystic Fibrosis Foundation patient registry?

Registries such as the CFFPR are important tools for research, clinical care, and tracking incidence, mortality and population trends. The Cystic Fibrosis Foundation Patient Registry. Design and Methods of a National Observational Disease Registry Ann Am Thorac Soc. 2016 Jul;13(7):1173-9.doi: 10.1513/AnnalsATS.201511-781OC.

How many people with cystic fibrosis are diagnosed in 2020?

Symptoms Reported at CF Diagnosis All Individuals (%) Diagnosed in 2020 (%) Diagnosed in 2020 Age < 1 (%) Diagnosed in 2020 Age ≥ 1 (%) Number of Individuals (n) 31,411 708 522 186 Asymptomatic

How has the incidence of cystic fibrosis changed with birth cohorts?

40Cystic Fibrosis Foundation Patient RegistryAnnual Data Report 2020 For individuals born between 1991 and 2005, we observed improved weight and height percentiles with successive birth cohorts. Beginning with birth cohorts in 2006, there have been fewer observed increases compared to subsequent birth cohorts.

What is the history of cystic fibrosis (CF)?

The cystic fibrosis transmembrane conductance regulator (CFTR) gene and the most prevalent CF-causing variant (F508del) were both discovered in 1989. Since then, genotyping has become a key component of the diagnostic work-up.

How is CFFPR used?

The CFFPR contains data on almost 50,000 unique patients and has been used for research reported in over 120 peer-reviewed manuscripts, in addition to numerous quality improvement and benchmarking initiatives. The CFFPR captures a substantial portion of the U.S. patient population with CF and has robust and high-quality data in key variables of interest, such as lung function, nutritional status and hospitalizations. Data in the registry have been used for many years to compare center-level variation in care and outcomes. As CF registries are implemented in other countries, the CFFPR has also been used to compare treatment and outcomes between the United States and other countries ( 35 ). International comparisons leverage variation in availability and physician preference of therapies and can be facilitated with standardization of data collection across registries.

What does CF stand for in medical terms?

Definition of abbreviations: CF = cystic fibrosis; MRSA = methicillin-resistant Staphylococcus aureus; MSSA = methicillin-sensitive Staphylococcus aureus. An audit of Cystic Fibrosis Foundation Patient Registry (CFFPR) data suggests high accuracy and low missingness of most variables compared with the medical record.

How many patients were seen at CF Foundation in 2012?

As reported by the care centers during the annual reaccreditation process, there were 1,875 patients who were seen at accredited care centers in 2012 who did not provide consent to participate in the registry. This represents 6.3% of patients seen at CF Foundation–accredited care centers in 2012.

What is electronic data capture system?

The electronic data capture system contains basic data validations to reduce the entry of implausible values. For some data fields, previous values are highlighted to alert data entry personnel to potential errors. Key fields are required so that a minimum amount of information is collected for a given form. Users at care centers are able to edit only records that were entered by their care center. Data are reviewed to flag what appear to be erroneous values from measures such as height, weight, body mass index, and laboratory tests. The CF Foundation verifies death and transplant dates as well as suspicious dates of birth and other important variables as needed.

How many people in the US have CF in 2012?

On the basis of the two methods of estimating the number of persons with CF in the United States using national birth and death data, we derived estimates of 33,292 and 34,327 individuals with CF in the United States in 2012, respectively. In 2012, the CFFPR contained 27,804 individuals. Thus, approximately 81–84% of persons with CF were captured in the CFFPR in 2012, the most recent year for which national birth and mortality data were available.

What is demographic form?

The demographic form captures date of birth, sex, race, Hispanic ethnicity, state of birth, and other information needed to verify that the record is for a unique patient. Information on the date and cause of death are also captured on this form.

How is CFFPR data collected?

The CFFPR data are collected through a web-based portal, PortCF, which contains five electronic data capture forms: demographic, diagnosis, encounter, care episode, and annual review forms. All data are entered by staff at the care center programs from the data available in the medical record or in forms completed by patients or families. CFFPR questionnaires are available in the annual reports ( 2 ).