Exercise in People With Cystic Fibrosis on CFTR Modulator Therapy

Changes in peripheral muscle strength,Changes in inspiratory/expiratory muscle strength (MIP/MEP) (cmH2O),Changes in functional capacity: lower limbs power capacity,Changes in functional capacity: walking capacity,Change in Cardiorespiratory fitness: maximal oxygen consumption,Change in Cardiorespiratory fitness: Ventilatory threshold VT1

Recently, the treatment of Cystic Fibrosis (CF) incorporated new modulators/enhancers of the
 Cystic Fibrosis Transmembrane Conductance Regulator (CFTR). It is thus increasingly important
 to study the side effects of these drugs, their extrapulmonary effects and possible
 interaction with other drugs and with exercise. For this purpose, a randomized controlled
 trial is proposed to determine the effects of a telematic exercise intervention on muscle
 health, in a group of 48 children and adolescents with CF treated with these new generation
 CFTR modulators. They will be randomly assigned to two groups (exercise and control group).
 The effect of the intervention will be analysed measuring the variables of muscle health,
 cardiorespiratory fitness, lung function, body composition, inflammatory biomarkers and
 miRNAs. After completion of the intervention program, adherence to exercise and clinical
 evolution after one year will be analysed.