Effect of a Coordinated Diet and Physical Activity Program on Lean Body Mass in Adults with Cystic Fibrosis Taking Elexacaftor-Tezacaftor-Ivacaftor

What this Trial Is About

Cystic fibrosis is a genetic disease caused by mutations in the gene for the CFTR protein, leading to respiratory and digestive problems that affect health outcomes. Digestive issues can cause malnutrition with reduced muscle mass, which is linked to poorer lung function. Recently, undernutrition has been redefined to include loss of muscle mass or function. The therapy Elexacaftor-Tezacaftor-Ivacaftor, approved in 2021, aims to improve CFTR protein function and has shown early benefits in lung function and weight gain, but also new nutritional challenges like overweight and metabolic problems. This study explores how diet and exercise can help these patients. The study compares two groups of adults with cystic fibrosis treated with Elexacaftor-Tezacaftor-Ivacaftor. One group participates in the DIAPASOM program, which includes a year-long remote adapted physical activity program led by a specialist instructor, with phases of supervised and independent sessions, combined with personalized dietetic care involving consultations every three months and phone follow-ups. The other group receives usual care for nutrition and physical activity. Participants will be followed for 12 months to assess changes in lean body mass as a percentage of total body mass. Researchers will also measure fat mass, weight, BMI, muscle strength and endurance, flexibility, lung function, physical activity levels, sedentary time, and quality of life. The programs feedback questionnaire will be collected at 12 months. This thorough evaluation aims to understand how coordinated diet and adapted physical activity affect muscle mass and overall health in cystic fibrosis patients.

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