33 hours ago The CF Foundation Patient Registry collects information on the health status of people with cystic fibrosis who receive care in CF Foundation-accredited care centers and agree to … >> Go To The Portal
The CF Foundation Patient Registry collects information on the health status of people with cystic fibrosis who receive care in CF Foundation-accredited care centers and agree to participate in the Registry.
Annual Data Report 2020 Cystic Fibrosis Foundation Patient Registry 13 In 2020, the median age of people with CF in the Registry was 20.3 years. The range is from birth to 89.7 years.
Age (Years) 62Cystic Fibrosis Foundation Patient RegistryAnnual Data Report 2020 Complications of CF, 2020 Age < 18 (%) Age ≥ 18 (%) All (%) Number of Individuals (n) 13,425 16,251 29,676
As the CF population continues to age, adult CF care providers should continue to screen individuals for these complications, as recommended by the CF Foundation clinical care guidelines for CFRD.39 Annual Data Report 2020 Cystic Fibrosis Foundation Patient Registry 69
Cite This ItemChicago citation style: Cystic Fibrosis Foundation. Cystic Fibrosis Foundation . United States, 2002. ... APA citation style: Cystic Fibrosis Foundation. (2002) Cystic Fibrosis Foundation . United States. ... MLA citation style: Cystic Fibrosis Foundation. Cystic Fibrosis Foundation . United States, 2002.
The CF Foundation Patient Registry collects information on the health status of people with cystic fibrosis who receive care in CF Foundation-accredited care centers and agree to participate in the Registry.
The Foundation and community held more than 950 virtual events and raised more than $73 million for the fight against CF, demonstrating the CF community's talents and commitment to connecting with one another and fueling our shared mission as they weathered the pandemic.
About the Cystic Fibrosis Foundation The Cystic Fibrosis Foundation is the world's leader in the search for a cure for cystic fibrosis. The CF Foundation funds more cystic fibrosis research than any other organization, and nearly every CF drug available today was made possible because of CF Foundation support.
According to the Cystic Fibrosis Foundation Patient Registry, in the United States: There are close to 40,000 children and adults living with cystic fibrosis in the United States (and an estimated 105,000 people have been diagnosed with CF across 94 countries).
Worldwide, about 70,000 to 100,000 people have cystic fibrosis.
Michael Boyle, MDSummary. Michael Boyle, MD, is president and chief executive officer of the Cystic Fibrosis Foundation. Dr. Boyle is internationally known for his clinical research and for his work to improve care for adults with CF.
Cystic Fibrosis FoundationFoundedDecember 16, 1955Expenses (2016)$299,650,531Employees (2016)731Volunteers (2016)250,000Websitewww.cff.org11 more rows
The CF Foundation spent a total of $218.1 million on research and development as well as the CF Foundation Therapeutics Lab in 2020.
“CF is no longer a death sentence, and the research we are doing will continue to help our patients live longer and better lives.” CF, a genetic disease, affects a person's organs, primarily the lungs, and creates a thick, sticky mucus in the body that can lead to blockages, damage or infections to the affected organs.
Cystic fibrosis tends to get worse over time and can be fatal if it leads to a serious infection or the lungs stop working properly. But people with cystic fibrosis are now living for longer because of advancements in treatment. Currently, about half of people with cystic fibrosis will live past the age of 40.
Lung disease eventually worsens to the point where the person is disabled. Today, the average life span for people with CF who live to adulthood is about 44 years. Death is most often caused by lung complications.
The mission of the Cystic Fibrosis Foundation is to cure cystic fibrosis and to provide all people with CF the opportunity to lead long, fulfilling lives by funding research and drug development, partnering with the CF community, and advancing high-quality, specialized care.
The Cystic Fibrosis Foundation (CFF) is a voluntary, non-profit organization. Its mission is to assure the development of the means to cure and control cystic fibrosis (CF) and to improve the quality of life for those with the disease.
FRIDAY, Feb. 18, 2022 (HealthDay News) -- Hundreds of new proteins that may be linked to cystic fibrosis have been identified by researchers and could point the way to better treatments for people with the genetic disease. There is no cure for cystic fibrosis, which affects more than 90,000 people worldwide.
The Cystic Fibrosis Foundation is the world's leader in the search for a cure for CF and supports a broad range of research initiatives to tackle the disease from all angles.
The majority of patients who receive a diagnosis of CF are less than 2 years of age ( 1 ). However, with improved genetic testing, CF is being diagnosed in an increasing number of older patients ( 2 ).
Related. Cystic fibrosis (CF) has long been thought of as a disease of childhood. However, with innovative new therapies, patients are living longer and healthier lives, and CF is slowly being more widely recognized as a disease of adults as well. Despite advances in treatment, the median life expectancy for a patient with CF is still in ...
Background Cystic fibrosis (CF) affects >70,000 people worldwide, yet the microbiologic trigger for pulmonary exacerbations (PExs) remains unknown. The objective of this study was to identify changes in bacterial metabolic pathways associated with clinical status. Methods Respiratory samples were collected at hospital admission for PEx, end of intravenous (IV) antibiotic treatment, and follow-up from 27 hospitalized children with CF. Bacterial DNA was extracted and shotgun DNA sequencing was performed. MetaPhlAn2 and HUMAnN2 were used to evaluate bacterial taxonomic and pathway relative abundance, while DESeq2 was used to evaluate differential abundance based on clinical status. Results The mean age of study participants was 10 years; 85% received combination IV antibiotic therapy (beta-lactam plus a second agent). Long-chain fatty acid (LCFA) biosynthesis pathways were upregulated in follow-up samples compared to end of treatment: gondoate ( p = 0.012), oleate ( p = 0.048), palmitoleate ( p = 0.043), and pathways of fatty acid elongation ( p = 0.012). Achromobacter xylosoxidans and Escherichia sp. were also more prevalent in follow-up compared to PEx ( p < 0.001). Conclusions LCFAs may be associated with persistent infection of opportunistic pathogens. Future studies should more closely investigate the role of LCFA production by lung bacteria in the transition from baseline wellness to PEx in persons with CF. Impact Increased levels of LCFAs are found after IV antibiotic treatment in persons with CF. LCFAs have previously been associated with increased lung inflammation in asthma. This is the first report of LCFAs in the airway of persons with CF. This research provides support that bacterial production of LCFAs may be a contributor to inflammation in persons with CF. Future studies should evaluate LCFAs as predictors of future PExs.
The US Cystic Fibrosis Foundation (CFF) began in 1955 with a mission to support the development of new drugs to fight the disease, improve the quality of life for those with cystic fibrosis (CF), and ultimately to find a cure for this disease.1 The CFF does this by supporting basic science and clinical research in CF, supporting the care of CF patients through accredited CF centres nationwide and advocating for CF patients at the state and national level. Recognising the critical role of data collection and measurement of outcomes to better understand the natural history of CF, the CFF created a patient registry in 1966, the CFF Patient Registry (CFFPR).2 The CFFPR has evolved over the years from a few demographic variables including vital status to a comprehensive database that gives healthcare providers, researchers, policy makers and change agents data to support epidemiological and clinical research as well as efforts to improve quality of care. The specific purpose of this commentary is to describe the CFFPR and primarily to focus on how the CFFPR and its associated tools are being used for quality improvement (QI) activities, with the hope that it may help CF healthcare teams in the USA who are not familiar with the registry's capabilities, CF providers outside the USA with registries at various stages of development, and others interested in how a patient registry has been used to improve care. The CFFPR contains detailed demographic and diagnostic data dating back to 1986 with current annual and encounter-based data on over 300 unique variables including outcomes (eg, microbiology, lung function and nutritional metrics, CF complications) and care processes (eg, hospitalisations, medications, surveillance measures) for each of its more than 27 000 participants in 2012; in all, there are over 46 000 unique individuals’ data in the registry.3 …
Background Specialized clinical care for cystic fibrosis (CF) in Cyprus, a small island country, has been implemented since the 1990s. However, only recently, a national CF patient registry has been established for the systematic recording of patients’ data. In this study, we aim to present data on the epidemiological, genotypic and phenotypic features of CF patients in the country from the most recent data collection in 2019, with particular emphasis on notable rare or unique cases. Results Overall, data from 52 patients are presented, 5 of whom have deceased and 13 have been lost to follow-up in previous years. The mean age at diagnosis was 7.2 ± 12.3 years, and the mean age of 34 alive patients by the end of 2019 was 22.6 ± 13.2 years. Patients most commonly presented at diagnosis with acute or persistent respiratory symptoms (46.2%), failure to thrive or malnutrition (40.4%), and dehydration or electrolyte imbalance (32.7%). Sweat chloride levels were diagnostic (above 60 mmol/L) in 81.8% of examined patients. The most common identified mutation was p.Phe508del (F508del) (45.2%), followed by p.Leu346Pro (L346P) (6.7%), a mutation detected solely in individuals of Cypriot descent. The mean BMI and FEV1 z-scores were 0.2 ± 1.3 and − 2.1 ± 1.7 across all age groups, respectively, whereas chronic Pseudomonas aeruginosa colonization was noted in 26.9% of patients. The majority of patients (74.5%) were eligible to receive at least one of the available CFTR modulator therapies. In 25% of patients we recovered rare or unique genotypic profiles, including the endemic p.Leu346Pro (L346P), the rare CFTR-dup2, the co-segregated c.4200_4201delTG/c.489 + 3A > G, and the polymorphism p.Ser877Ala. Conclusions CF patient registries are particularly important in small or isolated populations, such as in Cyprus, with rare or unique disease cases. Their operation is necessary for the optimization of clinical care provided to CF patients, enabling their majority to benefit from evolving advances in precision medicine.