Related Experiment Video

Updated: May 11, 2026

Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study
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Managing an ageing cystic fibrosis population: challenges and priorities

Freddy J Frost1,2, Daniel G Peckham3, Imogen C Felton4

  • 1Adult Cystic Fibrosis Centre Liverpool Heart and Chest Hospital NHS Foundation Trust, Liverpool, UK ffrost@liverpool.ac.uk.

European Respiratory Review : an Official Journal of the European Respiratory Society
|May 14, 2025
PubMed

Insights

People with cystic fibrosis (pwCF) are living longer due to medical advances. Future care must address the unique health challenges of aging pwCF, focusing on quality of life.

Area of Science:

  • Pulmonology
  • Gerontology
  • Genetics

Background:

  • Increasing life expectancy in people with cystic fibrosis (pwCF) necessitates a shift in care towards managing age-related complexities.
  • Advancements in early diagnosis, multidisciplinary care, and CFTR modulator therapies have significantly improved outcomes for pwCF.
  • The aging CF population presents unique health challenges requiring focused research and adapted clinical strategies.

Purpose of the Study:

  • To review the key challenges and research priorities for the health needs of an aging population with cystic fibrosis.
  • To highlight the evolving landscape of care for pwCF, considering the impact of increased longevity and therapeutic advancements.
  • To identify emerging issues and propose multidisciplinary research approaches for holistic wellbeing in aging pwCF.

Main Methods:

  • Literature review synthesizing current evidence on aging in cystic fibrosis.
  • Analysis of research gaps and future priorities in managing age-related conditions in pwCF.
  • Integration of clinical, patient, and community perspectives for comprehensive care strategies.

Main Results:

  • Aging pwCF face heightened risks of cancers, cardiovascular diseases, and altered metabolic profiles.
  • CFTR modulators show promise but long-term effects on inflammation, immunity, and disease trajectories require further study.
  • Nutritional management, obesity, accelerated immune/pulmonary aging, menopause, antimicrobial resistance, and chronic inflammation are critical considerations.

Conclusions:

  • Effective management of aging pwCF requires multidisciplinary research integrating diverse perspectives.
  • Leveraging CF registries, clinical trial networks, and aging research collaborations is crucial.
  • The goal is to ensure aging pwCF not only live longer but also achieve improved quality of life and holistic wellbeing.

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