27 hours ago Abstract. Rationale: 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. >> Go To The Portal
The oldest person with cystic fibrosis was 82 years of age, and an American citizen. He or she was still alive in 2014 at the time of a published article in which they are mentioned. Though it’s not likely that they are still alive seven years later, this person is the oldest known cystic fibrosis patient to have achieved the average person’s life expectancy (and then some)!
Cystic fibrosis is a serious, life-threatening disease that significantly shortens a person’s lifespan. Fortunately, with advances in treatment, many people with CF are now living into their 40s and 50s, and babies born with CF today can expect to live into their 50s and 60s.
These statistics reflect current life expectancy rates in developed countries, such as the U.S. and the United Kingdom. However, Cystic Fibrosis Worldwide suggest that in developing countries, such as El Salvador and India, life expectancy for people with CF is under 15 years of age.
Cystic fibrosis is most common among people of Northern European descent. However, it’s known to occur in all ethnic groups. People who have a family history of cystic fibrosis are also at an increased risk because it’s an inherited disorder.
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 Cystic Fibrosis Foundation Patient Registry was established in the 1960s and has continually evolved to keep pace with changes in technology and regulations, as well as improvements in the treatment of cystic fibrosis (CF).
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.
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 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 ).
All individuals diagnosed with CF and associated disorders (CFTR-related metabolic syndrome and CFTR-related disorders) who are seen at CF Foundation–accredited care center programs and provide informed consent are eligible to participate in the CFFPR. The CF Foundation has developed and sustains a network of 121 accredited CF care centers (comprised of 121 pediatric care programs and 105 adult care programs) and 51 affiliate programs across the United States ( see additional information available in the online supplement). All accredited care programs are required to participate in the CFFPR. A portion of care center funding is based on the number of patients enrolled in the CFFPR and the completeness of their records. Each program obtains institutional review board approval and written informed consent and assent, as appropriate, from participants and/or their legal guardians. The CF Foundation provides online user manuals, data entry guidelines, training sessions, and user support for care center staff who enter data into the CFFPR. The CF Foundation serves as the coordinating center for data collection and data analysis.
The CFFPR was established in the 1960s to collect information on patient demographics and survival ( 3 ). Regular updates have been made to comply with changes in regulations, improvements in data collection, and advances in new technology ( Figure 1) ( 4 ).