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 (DIAPASOM)

Impact d'un Programme coordonné diététique-activité Physique adaptée Sur le Pourcentage de Masse Maigre d'Adultes Atteints de Mucoviscidose traités Par Elexacaftor-Tezacaftor-Ivacaftor : Essai contrôlé randomisé Multicentrique

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.

Study Overview

• Status: Recruiting
• Conditions: Cystic Fibrosis
• Intervention / Treatment: Other: DIAPASOM program

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.

• Study Type: Interventional
• Enrollment (Estimated): 100
• Phase: Not Applicable

Contacts and Locations

• Study Contact: Name: Amelie GIBORY; Phone Number: +33 0247474747; Email: a.gibory@chu-tours.fr
• Study Contact Backup: Name: Arnaud DE LUCA, MD; Email: a.deluca@chu-tours.fr

Study Locations

• France
  • Angers, France, 49033
    • Recruiting
    • Cystic Fibrosis Resource and Competence Centre, University Hospital, Angers
    • Principal Investigator: Pascaline PRIOU, MD
    • Contact: Pascaline PRIOU, MD
  • Roscoff, France, 29684
    • Recruiting
    • Cystic Fibrosis Resource and Competence Centre, Fondation Ildys, Roscoff
    • Principal Investigator: Sophie RAMEL, MD
    • Contact: Sophie RAMEL, MD
  • Tours, France, 37044
    • Recruiting
    • Cystic Fibrosis Resource and Competence Centre, University Hospital, Tours
    • Principal Investigator: Julie Mankikian, MD
    • Contact: Julie MANKIKIAN, MD
  • Vannes, France, 56017
    • Recruiting
    • Cystic Fibrosis Resource and Competence Centre, Hospital, Tours
    • Contact: Baptiste ARNOUAT, MD
    • Principal Investigator: Baptiste ARNOUAT, MD

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Adult; Older Adult
• Accepts Healthy Volunteers: No

Description

Inclusion Criteria:

• 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.

Exclusion Criteria:

• 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 Plan

How is the study designed?

Design Details

• Primary Purpose: Supportive Care
• Allocation: Randomized
• Interventional Model: Parallel Assignment
• Masking: None (Open Label)

Number of Arms

2

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
Experimental: 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.	Other: DIAPASOM program; A program of adapted physical activity carried out remotely by a specialist instructor, combined with personalized dietetic care by a dietician for one year.
No Intervention: Management as usual; Management of nutrition and physical activity as usual

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Evolution of percentage of patients lean mass as a percentage of body mass	Impedancemetry	From randomization, up to 12 months

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
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
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
Upper limb muscle power	Pump test	From randomization, up to 12 months

Collaborators and Investigators

• Sponsor: University Hospital, Tours
• Collaborators: Fondation Ildys
• Investigators: Principal Investigator: Julie MANKIKIAN, MD, University Hospital, Tours

Study record dates

Study Major Dates

• Study Start (Actual): November 25, 2024
• Primary Completion (Estimated): November 1, 2027
• Study Completion (Estimated): November 1, 2027

Study Registration Dates

• First Submitted: June 7, 2024
• First Submitted That Met QC Criteria: June 7, 2024
• First Posted (Actual): June 13, 2024

Study Record Updates

• Last Update Posted (Actual): December 1, 2025
• Last Update Submitted That Met QC Criteria: November 27, 2025
• Last Verified: November 1, 2025

More Information

Terms related to this study

• Keywords: Cystic Fibrosis; Body composition; Adapted Physical Activity; Dietetic; Elexacaftor-Tezacaftor-Ivacaftor
• Additional Relevant MeSH Terms: Genetic Diseases, Inborn; Respiratory Tract Diseases; Digestive System Diseases; Lung Diseases; Infant, Newborn, Diseases; Pancreatic Diseases; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Cystic Fibrosis
• Other Study ID Numbers: 2023-A02715-40; DR220270 (Other Identifier: Sponsor)

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No