Impact of CFTR modulators on exercise capacity in adolescents with cystic fibrosis

Abstract

Background Exercise capacity is an independent predictor of clinical worsening in cystic fibrosis (CF). There is limited evidence of the impact of cystic fibrosis transmembrane conductance regulator (CFTR) modulators on exercise capacity in children with CF. The aim of the present study was to assess the impact of CFTR modulators on exercise capacity in a cohort of adolescents with CF. Methods A prospective single-centre cohort study was carried out. Cardiopulmonary exercise testing (CPET) was performed at baseline, prior to starting Symkevi or Kaftrio and between 4 and 8 months after starting treatment. Results 19 adolescents with CF had CPET performed prior to and after CFTR modulator treatment, between December 2019 and March 2022. Breathing reserve improved in the whole cohort, with greater improvement in the modulator-naïve patients after starting treatment with Kaftrio. There was no improvement in peak oxygen uptake and anaerobic threshold after 4 to 8 months of treatment with CFTR modulators. Conclusion Exercise testing with CPET can be used as an additional tool to monitor response to CFTR modulators. Breathing reserve on CPET may provide a surrogate marker to monitor the improvement in CF lung disease with CFTR modulator treatment.