Assessment of Quadriceps Muscle Electrostimulation Used in Patients Suffering From Cystic Fibrosis

Cystic fibrosis is an autosomal recessive genetic disease due to a mutation of the CFTR protein gene. The CFTR protein transports chloride ions (Cl-) across cell membranes in the lungs, pancreas, digestive tract, reproductive tract, and skin. CFTR mutation mainly leads to a dysfunction of the pulmonary system and pancreas exocrine function. Several studies showed that cystic fibrosis commonly induces a reduction of effort tolerance, peripheral muscular strength and work capacity. Patients suffering from cystic fibrosis with a high endurance capacity have a lower risk of poor prognosis. Those with a severe dyspnea have a higher benefit with a force training or a combined force and endurance training than with endurance training alone. We propose to study the effect of a quadriceps electrostimulation program, performed prior to an endurance retraining program using a cycloergometer, in patients with cystic fibrosis associated with severe pulmonary dysfunction, to enhance their muscular performance and increase their adherence to the cycloergometer retraining program. This is a randomized trial with two groups: Group A: experimental group, twenty patients. Six weeks with electrostimulation program followed by six weeks under a cycloergometer program. Group B: control group, twenty patients. Six weeks with their usual physical activity followed by six weeks with a cycloergometer program. The Randomization is stratified on expiratory volume per second. The size of randomization blocks is random because of the open design.