Managing an ageing cystic fibrosis population: challenges and priorities
File(s)Eur Respir Rev-2025-Frost-240261.pdf (640.13 KB)
Published version
Author(s)
Type
Journal Article
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.
Date Issued
2025-04-01
Date Acceptance
2025-02-23
Citation
European Respiratory Review, 2025, 34 (176)
ISSN
0905-9180
Publisher
European Respiratory Society (ERS)
Start Page
240261
End Page
240261
Journal / Book Title
European Respiratory Review
Volume
34
Issue
176
Copyright Statement
©The authors 2025 This version is distributed under the terms of the Creative Commons Attribution Licence 4.0 (https://creativecommons.org/licenses/by/4.0/)
License URL
Identifier
10.1183/16000617.0261-2024].
Subjects
Lung
Humans
Cystic Fibrosis
Cystic Fibrosis Transmembrane Conductance Regulator
Treatment Outcome
Life Expectancy
Risk Factors
Age Factors
Nutritional Status
Aging
Quality of Life
Health Priorities
Female
Publication Status
Published
Article Number
ARTN 240261
Date Publish Online
2025-05-14