Today, the CF Foundation announced that the population of people with cystic fibrosis has increased over the past decade, according to a new estimate. Close to 40,000 children and adults are living with cystic fibrosis in the United States and a total estimated 105,000 people have been diagnosed with CF across 94 countries. The CF population was last estimated in 2012 to be more than 30,000 people in the U.S. and 70,000 globally.
The updated estimate of the total population of people with CF was derived from the Cystic Fibrosis Foundation Patient Registry and the Vital Statistics of the United States. The estimated total number of global CF diagnoses is based on data collected from established registries and scientific reports across 94 countries and subsequently published in 20221.
“The improvements in research and care in CF in the past 20 years have been nothing short of extraordinary, and today’s announcement of the increase in population of people living with CF is a testament to that,” said Bruce Marshall, MD, executive vice president and chief medical officer of the Cystic Fibrosis Foundation.
Behind the Numbers
In the years following the initial description of the disease in 1938, children with CF rarely made it to adulthood, but in the past few decades, evolutions in the CF care model have helped change the face of cystic fibrosis. Advancements in multidisciplinary care, treatments, nutrition, and universal newborn screening have extended lives2. For a child born between 2017 and 2021, the median predicted age of survival is 53 years old — up from 38 years a decade prior3, 4, resulting in the expanded population.
There are now more adults living with CF than children. People with CF are achieving milestones never before thought possible — attending college, building careers, getting married, and starting families of their own. As people with CF get older, they often experience more complications and a more complex course of this progressive disease.
Marshall added, “We must learn what challenges adults with CF face when living longer with a chronic disease. This generation of adults with CF will help us better understand what it means to live a long life with CF. Through evolving our care model, clinical care guidelines, and adding clinical expertise, such as pharmacists, gastroenterologists, and endocrinologists, we are working to ensure that adults with CF get the care they need.”
About the Cystic Fibrosis Foundation Patient Registry
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. Fifteen percent of participants in the Patient Registry are identified as racially or ethnically diverse.
Information from the Patient Registry is used to inform CF care guidelines, assist care teams in providing care to individuals with CF, and guide quality improvement initiatives. Researchers also use the Patient Registry to study CF treatments and outcomes and to design CF clinical trials. Established in 1966, CF Patient Registry today is a best-in-class resource that has driven progress in CF care and research and has become a model for other disease registries. The Patient Registry releases an annual data report to share trends in the care and health of people with cystic fibrosis.
1Guo J, Garratt A, Hill A. Worldwide rates of diagnosis and effective treatment for cystic fibrosis. Journal of Cystic Fibrosis. 2022;21(3):456-462. doi:10.1016/j.jcf.2022.01.009
2McBennett K, Davis P, Konstan M. Increasing life expectancy in cystic fibrosis: Advances and challenges. Pediatr Pulmonol. 2021;57(S1). doi:10.1002/ppul.25733
32021 Cystic Fibrosis Foundation Patient Registry Highlights. Bethesda, Maryland. ©2022 Cystic Fibrosis Foundation
4Cystic Fibrosis Foundation Patient Registry 2020 Annual Data Report. Bethesda, Maryland ©2021 Cystic Fibrosis Foundation