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

NCT06457451 | Recruiting

• 2 other identifiers: 2023-A02715-40DR220270
• Study Type: interventional
• Target: 100
• Locations: 1 country
• Sites: 4

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.

Conditions

Cystic Fibrosis

Keywords

Elexacaftor-Tezacaftor-Ivacaftor; Cystic Fibrosis; Adapted Physical Activity; Body composition; Dietetic

Outcome Measures

Primary Outcomes (1)

• Evolution of percentage of patients lean mass as a percentage of body mass

  Impedancemetry

  From randomization, up to 12 months

Secondary Outcomes (16)

• Evolution of percentage of patients fat mass as a percentage of body mass

  From randomization, up to 12 months

• Weight evolution

  From randomization, up to 12 months

• Body Mass Index (BMI) evolution

  From randomization, up to 12 months

• Cardio-respiratory endurance

  From randomization, up to 12 months

• Bilateral Handgrip strength

  From randomization, up to 12 months

Study Arms (2)

DIAPASOM program

EXPERIMENTAL

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

Management as usual

NO INTERVENTION

Management of nutrition and physical activity as usual

Interventions

DIAPASOM program OTHER

A program of adapted physical activity carried out remotely by a specialist instructor, combined with personalized dietetic care by a dietician for one year.

DIAPASOM program

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)

You may qualify if:

• 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.

You may not qualify if:

• 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

Sponsors & Collaborators

• University Hospital, Tours: lead
• Fondation Ildys: collaborator

Study Sites (4)

Cystic Fibrosis Resource and Competence Centre, University Hospital, Angers

Angers, 49033, France

RECRUITING

Cystic Fibrosis Resource and Competence Centre, Fondation Ildys, Roscoff

Roscoff, 29684, France

RECRUITING

Cystic Fibrosis Resource and Competence Centre, University Hospital, Tours

Tours, 37044, France

RECRUITING

Cystic Fibrosis Resource and Competence Centre, Hospital, Tours

Vannes, 56017, France

RECRUITING

MeSH Terms

Conditions

Cystic Fibrosis

Condition Hierarchy (Ancestors)

Pancreatic Diseases; Digestive System Diseases; Lung Diseases; Respiratory Tract Diseases; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Infant, Newborn, Diseases

Study Officials

• Julie MANKIKIAN, MD

  University Hospital, Tours

  PRINCIPAL INVESTIGATOR

Central Study Contacts

Amelie GIBORY

CONTACT

0247474747; a.gibory@chu-tours.fr

Arnaud DE LUCA, MD

CONTACT

a.deluca@chu-tours.fr

Study Design

• Study Type: interventional
• Phase: not applicable
• Allocation: RANDOMIZED
• Masking: NONE
• Purpose: SUPPORTIVE CARE
• Intervention Model: PARALLEL
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: June 7, 2024
• First Posted: June 13, 2024
• Study Start: November 25, 2024
• Primary Completion (Estimated): November 1, 2027
• Study Completion (Estimated): November 1, 2027
• Last Updated: December 1, 2025

Record last verified: 2025-11

Locations

FR (4)