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
NCT Number: NCT06457451
Acronym: DIAPASOM
Status: NOT_YET_RECRUITING
Study Type: INTERVENTIONAL
Phases: NA
Conditions: Cystic Fibrosis
Interventions: OTHER: DIAPASOM program
Sponsor: University Hospital, Tours
Collaborators: Fondation Ildys
Sex: ALL
Age: ADULT, OLDER_ADULT
Enrollment: 100
Study Design: Allocation: RANDOMIZED|Intervention Model: PARALLEL|Masking: NONE|Primary Purpose: SUPPORTIVE_CARE
Start Date: 2024-10
Completion Date: 2027-10
Locations: Cystic Fibrosis Resource and Competence Centre, University Hospital, Angers, Angers, 49033, France|Cystic Fibrosis Resource and Competence Centre, Fondation Ildys, Roscoff, Roscoff, 29684, France|Cystic Fibrosis Resource and Competence Centre, University Hospital, Tours, Tours, 37044, France|Cystic Fibrosis Resource and Competence Centre, Hospital, Tours, Vannes, 56017, France

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.

Primary Outcome Measures: Evolution of percentage of patients lean mass as a percentage of body mass, Impedancemetry, From randomization, up to 12 months
Secondary Outcome Measures: Evolution of percentage of patients fat mass as a percentage of body mass, impedancemetry, From randomization, up to 12 months|Weight evolution, Weight measurement, From randomization, up to 12 months|Body Mass Index (BMI) evolution, Weight and height measurement, From randomization, up to 12 months|Cardio-respiratory endurance, 6-Minute Walk Test (6MWT), From randomization, up to 12 months|Bilateral Handgrip strength, Handgrip Test, From randomization, up to 12 months|Lower limb muscle power, 30-second Sit-to-Stand test, From randomization, up to 12 months|Upper limb muscle power, Pump test, From randomization, up to 12 months|Static trunck extensors muscle endurance, "Superman" test, From randomization, up to 12 months|Static trunck flexors muscle endurance, Shirado-Ito test, From randomization, up to 12 months|Balance between static muscular endurance of extensors and flexors, Calculated using the Shirado-Ito/"Superman" ratio, From randomization, up to 12 months|Posterior chain flexibility (Hamstring, hips and lower back), Front trunk flexion test, From randomization, up to 12 months|Upper limb flexibility, scapulohumeral mobility test, From randomization, up to 12 months|Forced Expiratory Volume in 1 second (FEV1), Spirometry, From randomization, up to 12 months|Physical activity volume and sedentary time, Physical activity and sedentary behavior self-questionnaire (ONAPS-PAQ), From randomization, up to 12 months|Evolution of quality of life, Cystic Fibrosis Questionnaire-Revised (CFQR-14), From randomization, up to 12 months|Program feedback questionnaire, Self-questionnaire about how patients in the experimental group feel about the program, 12 months after randomization