The impact of tailored exercise interventions on exercise capacity, lung function and physical activity adherence in people with cystic fibrosis: a pilot study

Abstract

Objective: The advent of highly effective cystic fibrosis transmembrane regulator protein (CFTR) modulator therapies (HEMTs) has significantly impacted the clinical management of cystic fibrosis (CF) care. Exercise Physiology within CF care is limited and not as established alongside the standardised therapy treatment methods. This study assesses the role alongside standard CF care, particularly focusing on the impact of tailored exercise plans and adherence to lifestyle changes. Method(s): This was a retrospective single-centre cohort study spanning October 2023 to December 2024. The intervention was tailored exercise plans. 58 adults, 31 males, age range 17-57, and 27 females, age range 19-64, were evaluated. There were 30 participants (19 male and 11 female) in the intervention group and 28 (13 male and 15 female) within the control group. Participants underwent spirometry (n=58), 6-minute walk test (6MWT) (n=44), A-Step (n=4), and Chester Step (n=5). Physical Activity was measured via the validated GODIN leisure-time exercise questionnaire (GODIN, 2011). The impact of HEMTs was also analysed. SPSS version 30.0.0 was used for statistical analysis. Result(s): No significant change (p >.005) in FEV1 % pred between both groups, regardless of exercise intervention or HEMT. The 6MWT distance observed increases in both the control (546+/-44m vs.478+/-40m) and intervention group (460+/-140m vs.384+/-127m, p >.005). However, the intervention group did report increased activity levels compared to baseline (p<.004) via GODIN. Conclusion(s): Although exercise interventions did not impact pulmonary function, it did demonstrate a positive impact on lifestyle changes. This impact is in addition to the known benefits of HEMTs. Therefore, it is imperative to explore the impact of tailored exercise interventions on pulmonary exacerbations and non-pulmonary measures of health, such as weight and quality of life measures contributing to suboptimal physical activity and exercise intolerance. Copyright © 2025