Elexacaftor-Tezacaftor-Ivacaftor improves exercise capacity in
adolescents with cystic fibrosis
Abstract
This study investigated whether Elexacaftor-Tezacaftor-Ivacaftor
(Kaftrio ®), a cystic fibrosis (CF) transmembrane
conductance regulator (CFTR) modulator, could improve exercise capacity
in adolescents with CF. After six weeks treatment, Kaftrio
® improved both maximal and submaximal indices of
aerobic fitness. Improvements were independent of changes in ventilatory
function during exercise and physical activity. Interestingly, pulmonary
oxygen uptake per unit of power output was higher in two, out of three,
cases who presented with more severe CF lung disease. These findings
suggest improved O 2 extraction and/or consumption in
the exercising muscle and demonstrate, for the first time, that
short-term treatment with Kaftrio ® might improve
aerobic fitness in people with CF, especially in those with more severe
lung disease and deconditioning.