Perception and participation in sport and exercise in cystic fibrosis: The impact of CFTR modulators.

BACKGROUND

People with cystic fibrosis (pwCF) have barriers to physical activity including exercise intolerance and fatigue. The advent of small molecule cystic fibrosis transmembrane conductance regulator (CFTR) modulators have shown great clinical improvements in pwCF; however, the effect of CFTR modulators on exercise perception and participation is unknown. The purpose of this study was to investigate whether the administration of CFTR modulators changed the perception and participation in sport and exercise in pwCF.

METHODS

A survey-based, retrospective cohort study was conducted including individuals aged 18 and above.

RESULTS

Eighty-three participants were recruited with a mean age of 30.0 ± 10.5 years. The majority (82 %) of participants were taking CFTR modulators. Participants in the modulator group rated enjoyment of exercise and importance of exercise higher than the non-modulator group and were more likely to exercise as a means to socialize. Participants in the modulator group reported lack of time as the most frequent barrier to sport and exercise whereas fatigue was most reported in the non-modulator group. Twenty-eight percent of the modulator group participated in team/structured sports, whereas no participant engaged in team and structured sports in the non-modulator group.

CONCLUSIONS

Adults with CF who used CFTR modulators rated exercise more favorably and engaged in more team sport activities compared to pwCF who did not use CFTR modulators. The most frequent barrier to exercise in pwCF who used modulators might no longer be CF-related. More research is needed to confirm whether CFTR modulator administration resulted in increased overall physical activity.