Cystic fibrosis is a genetic disorder affecting the entire body and associated with respiratory exacerbations, impaired quality of life and reduced life expectancy. The therapeutic management of cystic fibrosis has been profoundly changed by the recent arrival of a combination of highly effective cystic fibrosis transmembrane conductance regulator (CFTR) modulators, Elexacaftor-Tezacaftor-Ivacaftor (ETI), which improve quality of life, respiratory function and reducing the number of exacerbations. The impact of these treatments on exercise adaptation has not been clearly identified.
The main objective is to estimate the prevalence of ventilatory reserve amputation during submaximal exercise testing assessed by the 6-minute walk test (6MWT) in patients with cystic fibrosis treated with ETIs.
Male or female
Adult aged 18 or over
Suffering from cystic fibrosis
Treated at the CRCM in Lille and Créteil
Medical contraindication or inability to perform a stress test according to ERS recommendations
Absolute contraindications
Exacerbation of the condition in the 4 weeks preceding the V1 visit (27).
Pregnant or breastfeeding women
Single group
Measurement of resistance through forced oscillations and continuous measurement of ventilation and inspiratory capacity during TM6
DeviceMeasurement of resistance through forced oscillations and continuous measurement of ventilation and inspiratory capacity during TM6
Estimate the prevalence of ventilatory limitation measured by a portable spirometer during a submaximal exercise test (6-minute walk test) in patients with cystic fibrosis undergoing ETI.
The primary endpoint is ventilatory limitation defined as the difference between the estimated theoretical maximun minute ventilatory volume (VMM in L/min) estimated by multiplying FEV1 (L) x 35 and the measurement of external ventilation during exercise (VE, L/min) using a portable spirometer divided by the theoretical VM x100 less than 15%. Ventilatory limitation = (MMV (L/min) - VE (L/min) /MMV (L/min))\*100 \< 15%
Estimate the prevalence of ventilatory limitation measured using a portable spirometer during a maximal incremental step test (A-STEP) with progressive steps in patients with cystic fibrosis undergoing ETI.
The assessment criterion is the difference between the estimated theoretical maximun minute ventilatory volume (VMM in L/min) estimated by multiplying FEV1 (L) x 35 and the measurement of external ventilation (VE in L/min) using a portable spirometer during exercise divided by the theoretical VM x100 less than 15%.
Assessment of the prevalence of dynamic distension during a submaximal (TM6) or maximal (incremental step test according to the A-Step protocol) exercise test by spirometry
The criterion for judgement is a decrease in inspiratory capacity (L) of more than 150 mL within 30 seconds after the end of the effort compared to the pre-effort inspiratory capacity (L) measured by spirometry.
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University Hospital, Lille
Lead sponsor
Vaincre la Mucoviscidose
Collaborator
