Longitudinal changes in habitual physical activity in adult people with cystic fibrosis in the presence or absence of treatment with elexacaftor/tezacaftor/ivacaftor

• DOI: 10.3389/fspor.2024.1284878
• Publication Date: 2024-02-23T12:31:47Z
• Authors: Wolfgang Gruber; Florian Stehling; Christopher Blosch; Stefanie Dillenho...; Margarete Olivier; Folke Brinkmann; Cordula Koerner-R...; Sivagurunathan Su...; Uwe Mellies; Christian Taube; Matthias Welsner
• Keywords: Ivacaftor; Longitudinal Study

Background Habitual physical activity (PA) and exercise training are accepted as important aspects of care for people with cystic fibrosis (pwCF) to improve health-related measures fitness, which in turn have a positive impact on quality life prognosis. In the last decade, effective CFTR modulator therapies become promising treatment pwCF by targeting underlying cause CF. This highly therapy improves clinical outcomes specific mutations. Little is known about longitudinal pattern PA or Elexacaftor/Tezacaftor/Ivacaftor (ETI) adult pwCF. study assessed course device-based measurement evaluated effects ETI habitual those who were eligible ETI. Methods Data from (aged ≥18 years) analysed at baseline follow-up, using identical assessments both time points. Outcome parameters steps/day intensity PA. The group that received was treated an average 33 weeks not entire duration period. data collected between 2021 2022, following removal absolute pandemic restrictions/lockdowns. Results Follow-up 5.6 years (ETI group, n = 21) 6.5 without (non-ETI 6). From had significant increase (+25%, p 0.019) non-significant moderate-to-vigorous (+5.6%, 0.352). Conversely, individuals non-ETI showed decrease −3.2%, 0.893) (−25%, 0.207). percent predicted forced expiratory volume 1 s (ppFEV ) FEV z-score before start treatment, improved significantly after initiation. Body weight body mass index also use. Conclusions These suggest has effect behavior studied.