CFDB - Cystic Fibrosis DataBase

primary studies published RCT

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

Study design (if review, criteria of inclusion for studies)

Randomized controlled pilot study

Participants

People with cystic fibrosis (PwCF) on highly effective modulator therapy (HEMT) (stable elexacaftor/tezacaftor/ivacaftor)

Interventions

Participants were randomized to a 12-week coached home-based exercise intervention or standard care. Intervention participants received weekly coaching and progressed to 180 min/wk of moderate-intensity exercise.

Outcome measures

Primary outcome was change in cardiorespiratory fitness measured by the Incremental Shuttle Walk Test. Secondary outcomes included adherence, changes in lung function (FEV(1) % 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.

Main results

Twenty-one PwCF were enrolled, with 14 participants completing the study (intervention: n = 8; control: n = 6). The intervention group demonstrated a significant improvement in Incremental Shuttle Walk Test distance compared with the control group (76.3 ± 98.4 m vs -66.7 ± 93.3 m, respectively). The between-group difference favored the intervention by 14.3 m (95% CI, 2.96-25.63; P = .03). No significant changes were observed in FEV(1) % predicted, Cystic Fibrosis Questionnaire-Revised total score, barrier scores, or ventilation defect percent.

Authors' conclusions

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.

Keywords: exercise; Home; Home Care Services; non pharmacological intervention - devices OR physiotherapy; non pharmacological intervention - psyco-soc-edu-org; Ventilators; Organization;