Feasibility and Impact of a Coached Home-Based Exercise Program on Cardiorespiratory Fitness in Adults With Cystic Fibrosis on Highly Effective Modulator Therapy

Background: In the era of highly effective modulator therapy (HEMT), people with cystic fibrosis (PwCF) are living longer and experiencing a shift in disease burden, with exercise emerging as an essential strategy to support cardiorespiratory and metabolic health. Despite known benefits, adherence to exercise remains low, and evidence guiding implementation of home-based interventions is limited. This study assessed the feasibility and clinical impact of a coached home-based exercise program in PwCF on HEMT. Research Question: Does a personalized, coached home-based exercise program improve cardiorespiratory fitness in PwCF who are on stable HEMT? Study Design and Methods: % predicted), health-related quality of life (Cystic Fibrosis Questionnaire-Revised), perceived barriers to physical activity, and ventilation defect percent measured by hyperpolarized xenon-129 MRI scan. Results: % predicted, Cystic Fibrosis Questionnaire-Revised total score, barrier scores, or ventilation defect percent. Interpretation: Our results show that a personalized, coached home-based exercise program significantly improved cardiorespiratory fitness in PwCF on HEMT but had no measurable impact on lung function or quality of life. These findings support the feasibility of a home-based exercise coaching program. Clinical Trial Registration: ClinicalTrials.gov; No.: NCT05239611; URL: www.clinicaltrials.gov.