Evidence map›Paper›PMID 40368426›Full record

ReviewEuropean respiratory review : an official journal of the European Respiratory Society2025

Managing an ageing cystic fibrosis population: challenges and priorities.

Freddy J Frost, Daniel G Peckham, Imogen C Felton, Joanna E Snowball, Robert D Gray, Andrew M Jones, Nicholas J Simmonds, Robert W Lord, Gregory Y H Lip, Hannah Chandler and 9 more

Abstract readReview
In one paragraph

Review in European respiratory review : an official journal of the European Respiratory Society, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.

0numbers the graph read from it
0cells of the map it votes in
5citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

5 citing papers in PubMed.

  1. Article
  2. Review
  3. Review
  4. Review
  5. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

19 authors.

Freddy J FrostAdult Cystic Fibrosis Centre Liverpool Heart and Chest Hospital NHS Foundation Trust, Liverpool, UK ffrost@liverpool.ac.uk.ORCID https://orcid.org/0000-0002-3902-6502
Daniel G PeckhamLeeds Institute of Medical Research, University of Leeds, Leeds, UK.
Imogen C FeltonAdult Cystic Fibrosis Centre, Royal Brompton and Harefield Hospitals, part of Guy's and St Thomas' NHS Foundation Trust, London, UK.
Joanna E SnowballOxford Adult CF Centre, Oxford University Hospitals NHS Foundation Trust, Oxford, UK.
Robert D GraySchool of Infection and Immunity, University of Glasgow, Glasgow, UK.ORCID https://orcid.org/0000-0002-2203-2703
Andrew M JonesManchester Adult Cystic Fibrosis Centre, Manchester University NHS Foundation Trust, Manchester, UK.
Nicholas J SimmondsAdult Cystic Fibrosis Centre, Royal Brompton and Harefield Hospitals, part of Guy's and St Thomas' NHS Foundation Trust, London, UK.
Robert W LordManchester Adult Cystic Fibrosis Centre, Manchester University NHS Foundation Trust, Manchester, UK.ORCID https://orcid.org/0000-0001-9008-2502
Gregory Y H LipLiverpool Centre for Cardiovascular Sciences at University of Liverpool, Liverpool John Moores University and Liverpool Heart and Chest Hospital NHS Foundation Trust, Liverpool, UK.
Hannah ChandlerCardiff University Brain Research Imaging Centre, Cardiff University, Cardiff, UK.
Kevin MurphyCardiff University Brain Research Imaging Centre, Cardiff University, Cardiff, UK.
Damian G DowneyWellcome-Wolfson Institute for Experimental Medicine, Queen's University, Belfast, UK.
David N SheppardSchool of Physiology, Pharmacology and Neuroscience, University of Bristol, Bristol, UK.
Jane C DaviesNational Heart and Lung Institute, Imperial College, London, UK.
Jane BullCystic Fibrosis Trust, London, UK.
Paula SommerCystic Fibrosis Trust, London, UK.
Belinda CupidCystic Fibrosis Trust, London, UK.
Lucy AllenCystic Fibrosis Trust, London, UK.
Jamie DuckersAll Wales Adult Cystic Fibrosis Centre, University Hospital Llandough, Penarth, UK.ORCID https://orcid.org/0000-0003-3004-279X

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

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.

Indexed as

AgingCystic FibrosisHealth PrioritiesLungAge FactorsCystic Fibrosis Transmembrane Conductance RegulatorFemaleHumansLife ExpectancyNutritional StatusQuality of LifeRisk FactorsTreatment OutcomeCFTR protein, humanCystic Fibrosis Transmembrane Conductance Regulator

Identifiers

PMID40368426
PMCPMC12076158

What Socratic holds

Textmetadata
LicenceCC BY
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.