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 ID: NCT06457451

Last Updated: 2025-12-01

Basic Information

• Recruitment Status: RECRUITING
• Clinical Phase: NA
• Total Enrollment: 100 participants
• Study Classification: INTERVENTIONAL
• Study Start Date: 2024-11-25
• Study Completion Date: 2027-11-30

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.

Conditions

Cystic Fibrosis

Study Design

• Allocation Method: RANDOMIZED
• Intervention Model: PARALLEL
• Primary Study Purpose: SUPPORTIVE_CARE
• Blinding Strategy: NONE

Study Groups

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.

Group Type: EXPERIMENTAL

DIAPASOM program

Intervention Type: 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.

Management as usual

Management of nutrition and physical activity as usual

Group Type: NO_INTERVENTION

No interventions assigned to this group

Interventions

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.

Intervention Type: OTHER

Eligibility Criteria

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

• Minimum Eligible Age: 18 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

Fondation Ildys

OTHER

Sponsor Role: collaborator

University Hospital, Tours

OTHER

Sponsor Role: lead

Responsible Party

Responsibility Role: SPONSOR

Principal Investigators

Julie MANKIKIAN, MD

Role: PRINCIPAL_INVESTIGATOR

University Hospital, Tours

Locations

Cystic Fibrosis Resource and Competence Centre, University Hospital, Angers

Angers, , France

Site Status: RECRUITING

Cystic Fibrosis Resource and Competence Centre, Fondation Ildys, Roscoff

Roscoff, , France

Site Status: RECRUITING

Cystic Fibrosis Resource and Competence Centre, University Hospital, Tours

Tours, , France

Site Status: RECRUITING

Cystic Fibrosis Resource and Competence Centre, Hospital, Tours

Vannes, , France

Site Status: RECRUITING

Countries

France

Central Contacts

Amelie GIBORY

Role: CONTACT

a.gibory@chu-tours.fr

0247474747 ext. +33

Arnaud DE LUCA, MD

Role: CONTACT

a.deluca@chu-tours.fr

Facility Contacts

Pascaline PRIOU, MD

Role: primary

Sophie RAMEL, MD

Role: primary

Julie MANKIKIAN, MD

Role: primary

Baptiste ARNOUAT, MD

Role: primary

Other Identifiers

DR220270

Identifier Type: OTHER

Identifier Source: secondary_id

2023-A02715-40

Identifier Type: -

Identifier Source: org_study_id