Impact of a Coordinated Dietetic-adapted Physical Activity Program on the Percentage of Lean Body Mass in Adults With Cystic Fibrosis Treated With Elexacaftor-Tezacaftor-Ivacaftor: Multicentre Randomised Controlled Trial
- Conditions
- Cystic Fibrosis
- Interventions
- Other: DIAPASOM program
- Registration Number
- NCT06457451
- Lead Sponsor
- University Hospital, Tours
- Brief Summary
Cystic fibrosis is an autosomal recessive inherited disease linked to various mutations in the gene coding for the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) protein, with respiratory and digestive disorders conditioning the prognosis.
Digestive damage may be responsible for malnutrition of multifactorial origin (insufficient energy intake, increased energy losses, increased basal metabolic rate), and studies show a correlation between reduced lean body mass and respiratory function.
In 2019, the French National Authority for Health (HAS) redefined undernutrition by including "quantified reduction in muscle mass and/or function" as a phenotypic diagnostic criterion.
Elexacaftor-Tezacaftor-Ivacaftor, an innovative therapy (authorization in 2021) for this population, aims to restore the function of CFTR protein. Significant improvements in lung function and weight gain were observed from the first weeks of treatment. These improvements have also led to the emergence of lesser-known nutritional problems in these patients, such as overweight and the development of metabolic complications. Nonetheless, new management options in terms of dietary adjustments and adapted physical activity for these patients are possible, given the development of their abilities.
Adapted Physical Activity (APA) helps to improve general muscular function by strengthening respiratory and skeletal muscles, improving aerobic capacity, and aiding bronchial drainage through muscle strengthening and endurance work. Maintaining or even increasing muscle mass depends not only on appropriate food intake and optimal dietary management, but also on regular physical activity, as recommended by the HAS.
Our hypothesis is therefore that a structured dietetic/adapted physical activity program (DIAPASOM program) can increase the percentage of lean body mass at 12 months in adult cystic fibrosis patients treated with Elexacaftor-Tezacaftor-Ivacaftor.
- Detailed Description
Cystic fibrosis is an autosomal recessive inherited disease linked to various mutations in the gene coding for the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) protein, with respiratory and digestive disorders conditioning the prognosis.
Digestive damage may be responsible for malnutrition of multifactorial origin (insufficient energy intake, increased energy losses, increased basal metabolic rate), and studies show a correlation between reduced lean body mass and respiratory function.
In 2019, the French National Authority for Health (HAS) redefined undernutrition by including "quantified reduction in muscle mass and/or function" as a phenotypic diagnostic criterion.
Elexacaftor-Tezacaftor-Ivacaftor, an innovative therapy (authorization in 2021) for this population, aims to restore the function of CFTR protein. Significant improvements in lung function and weight gain were observed from the first weeks of treatment. These improvements have also led to the emergence of lesser-known nutritional problems in these patients, such as overweight and the development of metabolic complications. Nonetheless, new management options in terms of dietary adjustments and adapted physical activity for these patients are possible, given the development of their abilities.
Adapted Physical Activity (APA) helps to improve general muscular function by strengthening respiratory and skeletal muscles, improving aerobic capacity, and aiding bronchial drainage through muscle strengthening and endurance work. Maintaining or even increasing muscle mass depends not only on appropriate food intake and optimal dietary management, but also on regular physical activity, as recommended by the HAS.
Our hypothesis is therefore that a structured dietetic/adapted physical activity program (DIAPASOM program) can increase the percentage of lean body mass at 12 months in adult cystic fibrosis patients treated with Elexacaftor-Tezacaftor-Ivacaftor.
Recruitment & Eligibility
- Status
- RECRUITING
- Sex
- All
- Target Recruitment
- 100
- Subject aged 18 or over
- Suffering from cystic fibrosis
- Treated with Elexacaftor-Tezacaftor-Ivacaftor for at least 6 months
- Affiliated to a social security scheme
- with a signed Informed Consent form.
- Pregnant and breast-feeding women
- Subject under legal protection, guardianship or curatorship
- Subject whose physical activity is not medically authorised or whose physical and motor capacities do not allow them to take part in physical activity.
- Subject who is unable to comply with the requirements of the DIAPASOM program
- Difficulty in understanding the self-questionnaires
- Wearing a pacemaker or metal prosthesis
- Fluid retention
Study & Design
- Study Type
- INTERVENTIONAL
- Study Design
- PARALLEL
- Arm && Interventions
Group Intervention Description DIAPASOM program DIAPASOM program Program of adapted physical activity carried out remotely by a specialized instructor for one year: * Phase 1 (3 months): 2 supervised weekly sessions. * Phase 2 (3months): 1 supervised weekly session and 1 independent weekly session. * Phase 3 (6months): 2 independent weekly sessions with monthly telephone follow-up. Combined with personalized dietetic care by a dietician for one year too: In person dietetic consultation every 3 months to monitor personalized objectives, with telephone follow-up between each face-to-face consultation to improve compliance.
- Primary Outcome Measures
Name Time Method Evolution of percentage of patients lean mass as a percentage of body mass From randomization, up to 12 months Impedancemetry
- Secondary Outcome Measures
Name Time Method Evolution of percentage of patients fat mass as a percentage of body mass From randomization, up to 12 months impedancemetry
Weight evolution From randomization, up to 12 months Weight measurement
Body Mass Index (BMI) evolution From randomization, up to 12 months Weight and height measurement
Cardio-respiratory endurance From randomization, up to 12 months 6-Minute Walk Test (6MWT)
Bilateral Handgrip strength From randomization, up to 12 months Handgrip Test
Lower limb muscle power From randomization, up to 12 months 30-second Sit-to-Stand test
Upper limb muscle power From randomization, up to 12 months Pump test
Static trunck extensors muscle endurance From randomization, up to 12 months "Superman" test
Static trunck flexors muscle endurance From randomization, up to 12 months Shirado-Ito test
Balance between static muscular endurance of extensors and flexors From randomization, up to 12 months Calculated using the Shirado-Ito/"Superman" ratio
Posterior chain flexibility (Hamstring, hips and lower back) From randomization, up to 12 months Front trunk flexion test
Upper limb flexibility From randomization, up to 12 months scapulohumeral mobility test
Forced Expiratory Volume in 1 second (FEV1) From randomization, up to 12 months Spirometry
Physical activity volume and sedentary time From randomization, up to 12 months Physical activity and sedentary behavior self-questionnaire (ONAPS-PAQ)
Evolution of quality of life From randomization, up to 12 months Cystic Fibrosis Questionnaire-Revised (CFQR-14)
Program feedback questionnaire 12 months after randomization Self-questionnaire about how patients in the experimental group feel about the program
Trial Locations
- Locations (4)
Cystic Fibrosis Resource and Competence Centre, University Hospital, Angers
🇫🇷Angers, France
Cystic Fibrosis Resource and Competence Centre, Fondation Ildys, Roscoff
🇫🇷Roscoff, France
Cystic Fibrosis Resource and Competence Centre, University Hospital, Tours
🇫🇷Tours, France
Cystic Fibrosis Resource and Competence Centre, Hospital, Tours
🇫🇷Vannes, France