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Cystic fibrosis (CF) is the most common life-limiting genetic disease. The treatment landscape of CF lung disease is changing dramatically with widespread availability of CF transmembrane conductance regulator (CFTR) modulator treatments targeting the primary protein defect. The latest generation of CFTR modulators (elexacaftor–tezacaftor–ivacaftor) has shown remarkable improvements in lung function, sweat chloride concentration, exacerbation rates and health-related quality of life of people with CF (pwCF),1 2 which in turn impacts individuals’ daily airway clearance therapy (ACT) and physical activity routine. In light of the improvements in the overall health status of pwCF, investigating the role of physical activity and exercise is timely and desired by the CF community.3 Two papers in this issue of Thorax focus on physical activity and exercise in pwCF, respectively, either as a substitute for traditional ACT or as an intervention to promote physical activity.4 5
Saynor et al4 addressed 1 of the top 10 research questions in CF ‘Can exercise replace chest physiotherapy’3 to support removal and prevent accumulation of secretions. The research group conducted a UK-based e-Delphi survey among physicians, physiotherapists, caregivers of pwCF and pwCF aiming to establish consensus on the role of exercise as ACT as well as on the type, intensity and duration of exercise to be tested against traditional ACTs in a prospective feasibility trial. The work by Saynor et al4 is of great interest to the CF community as a substantial proportion of pwCF (44%–48%) already substitute traditional ACTs with physical activity and exercise,6 7 although the current evidence on the long-term effects supporting this practice …
Funding The author has not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests TR is Coordinator of the Exercise Working Group of the European Cystic Fibrosis Society; he has previously collaborated with several professionals listed as authors of both studies.
Provenance and peer review Commissioned; externally peer reviewed.