Epidemiology

About 10,900 people in the UK, 32,000 people in the US, and >100,000 people worldwide have CF.[3][4]​​​​ The incidence among white people is estimated to be between 1 in 3000 and 1 in 6000; the incidence is lower among people of African, Hispanic, and Asian descent.[5][6]​​ CF is most common among people of European descent.[5]​

CF incidence has decreased since the advent of newborn screening (and may be associated with population-based carrier screening).[7][8]​​​​[9][10]​​​ The Cystic Fibrosis Foundation Patient Registry reports that 1017 new cases of CF were diagnosed in the US in 2006, compared with 779 new cases in 2021 (of which 21.2% and 64.4%, respectively, were detected by the newborn screening programme).[4]

Traditionally thought of as a childhood disease, advances in the management of CF mean that life expectancy is increasing. There are now more adults than children with CF in developed countries.[8] In the US, the median age at death was 33.9 years in 2021, and it is anticipated that patients born between 2018 and 2022 will live to 56 years.[4][11]​​ Primary lung disease and issues related to lung transplantation are the most common causes of death.[4]​ However, post-transplant survival remains stable, with 85.3%, 67%, and 54.3% surviving to 1, 3, and 5 years, respectively.[12]

Pseudomonas aeruginosa is a common respiratory tract infection that affects more than 60% of adults. Its overall prevalence is 29.8% and 49.6% among patients with CF in Europe and the US, respectively.[13][14]​ Despite high prevalence, aggressive treatment has been associated with a slight decrease over recent years.[15][16]

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