Managing an ageing cystic fibrosis population: challenges and priorities

Frost, Freddy J, Peckham, Daniel G, Felton, Imogen C, Snowball, Joanna E, Gray, Robert D, Jones, Andrew M, Simmonds, Nicholas J, Lord, Robert W, Lip, Gregory Y H, Chandler, Hannah, Murphy, Kevin, Downey, Damian G, Sheppard, David N, Davies, Jane C, Bull, Jane, Sommer, Paula, Cupid, Belinda, Allen, Lucy and Duckers, Jamie 2025. Managing an ageing cystic fibrosis population: challenges and priorities. European Respiratory Review 34 (176) , 240261. 10.1183/16000617.0261-2024

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Abstract

The increasing life expectancy of people with cystic fibrosis (pwCF), largely driven by advancements in early diagnosis, multidisciplinary care and the recent introduction of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies, is likely to herald a shift in the focus of care toward managing the complexities of ageing. This review highlights key challenges and research priorities for addressing the health needs of an ageing CF population. A growing body of evidence underscores the heightened risks of cancers, cardiovascular diseases and changing nutritional and metabolic profiles as pwCF age. CFTR modulators have improved clinical outcomes, but their effects on inflammation, immunity and long-term disease trajectories remain incompletely understood. Nutritional management, particularly the implications of obesity and body composition, poses new challenges, as does the potential accelerated ageing of immune and pulmonary systems in CF. Emerging issues such as menopause in females with CF, lifetime antimicrobial resistance and the interplay between chronic inflammation and ageing further complicate the care landscape. The review emphasises the urgent need for multidisciplinary research programmes that integrate clinical, patient and community perspectives. Leveraging established CF registries, clinical trial networks and collaborations with ageing research frameworks is critical to addressing these challenges. Ultimately, the goal is to ensure that pwCF not only live longer but also experience improved quality of life and holistic wellbeing as they realise the full benefits of therapeutic advances.

Item Type:	Article
Date Type:	Published Online
Status:	Published
Schools:	Schools > Psychology Research Institutes & Centres > Cardiff University Brain Research Imaging Centre (CUBRIC)
Additional Information:	License information from Publisher: LICENSE 1: Title: cc by, Type: cc by
Publisher:	European Respiratory Society
ISSN:	0905-9180
Date of First Compliant Deposit:	9 June 2025
Date of Acceptance:	23 February 2025
Last Modified:	09 Jun 2025 11:00
URI:	https://orca.cardiff.ac.uk/id/eprint/178903

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