16 hours ago This Annual Data Report contains data from individuals diagnosed with CF who (a) have consented to participate in the Registry, and (b) were seen in a CF Care Center during the 2020 calendar year, including those who were born, diagnosed, or died in the year. >> Go To The Portal
The Patient Registry is an invaluable tool for researchers conducting observational studies about people with CF in the U.S. About 50,000 individuals have been followed in the Registry since its inception in 1986; many of them have been included for over 20 years. 4 min read
ABOUT THIS REPORT The Annual Data Report is based on data entered in the CF Foundation Patient Registry through our online portal, PortCF©. Data are entered by teams of dedicated health professionals in our nationwide network of more than 120 CF Foundation-accredited Care Centers. Inclusion Criteria
The CF Foundation Patient Registry (CFFPR) is composed of data collected via the CF Foundation Care Center Network, including participant demographic characteristics, routine clinical measurements, therapeutic history, hospitalizations, transplant, and vital status.
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
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.
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.
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.
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.
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.
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.
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 ).