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 O2extraction 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.