CFTR Modulator Therapy and Aerobic Fitness Changes in Aerobic Fitness Following Initiation of CFTR Modulator Therapy: A Systematic Review and Meta-Analysis.
Pediatr PulmonolSep 1, 2026
Ingrid Silveira de Almeida, Fernanda Maria Vendrusculo, Mariana Severo da Costa et al.
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In 30 seconds
This systematic review and meta-analysis evaluated the impact of CFTR modulator therapy on aerobic fitness in individuals with cystic fibrosis (n=578). The analysis found no significant improvement in peak oxygen uptake (peak VO2) after therapy (MD=-0.22; 95% CI: -2.10 to 1.65; p=0.82), although breathing reserve and workload increased significantly.
Key findings
- No significant change in peak VO2 after CFTR modulator therapy (MD=-0.22; p=0.82).
- VO2 at the first ventilatory threshold significantly decreased post-intervention (MD=-1.85; p<0.00001).
- Breathing reserve increased significantly (MD=7.94; p=0.007).
- Workload also increased significantly (MD=15.39; p=0.02).
Why it matters
Understanding the effects of CFTR modulator therapy on aerobic fitness is crucial for managing cystic fibrosis, as it informs clinicians about potential changes in exercise capacity and respiratory function in their patients.