Clinical trial NCT06457451: 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
Official title: 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
Study type: INTERVENTIONAL
Overall status: RECRUITING
Phase: NA
Conditions: Cystic Fibrosis
Keywords: Elexacaftor-Tezacaftor-Ivacaftor
Cystic Fibrosis
Adapted Physical Activity
Body composition
Dietetic
Lead sponsor: University Hospital, Tours
Sponsor class: OTHER
Collaborators: name: Fondation Ildys
First posted: 2024-06-13
Enrollment: 100
Sex: ALL
Minimum age (years): 18
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Allocation: RANDOMIZED
Intervention model: PARALLEL
Primary purpose: SUPPORTIVE_CARE

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.

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

Primary outcomes:
measure: Evolution of percentage of patients lean mass as a percentage of body mass
timeFrame: From randomization, up to 12 months
Secondary outcomes:
measure: Evolution of percentage of patients fat mass as a percentage of body mass
timeFrame: From randomization, up to 12 months
measure: Weight evolution
timeFrame: From randomization, up to 12 months
measure: Body Mass Index (BMI) evolution
timeFrame: From randomization, up to 12 months
measure: Cardio-respiratory endurance
timeFrame: From randomization, up to 12 months
measure: Bilateral Handgrip strength
timeFrame: From randomization, up to 12 months
measure: Lower limb muscle power
timeFrame: From randomization, up to 12 months
measure: Upper limb muscle power
timeFrame: From randomization, up to 12 months
measure: Static trunck extensors muscle endurance
timeFrame: From randomization, up to 12 months
measure: Static trunck flexors muscle endurance
timeFrame: From randomization, up to 12 months
measure: Balance between static muscular endurance of extensors and flexors
timeFrame: From randomization, up to 12 months
measure: Posterior chain flexibility (Hamstring, hips and lower back)
timeFrame: From randomization, up to 12 months
measure: Upper limb flexibility
timeFrame: From randomization, up to 12 months
measure: Forced Expiratory Volume in 1 second (FEV1)
timeFrame: From randomization, up to 12 months
measure: Physical activity volume and sedentary time
timeFrame: From randomization, up to 12 months
measure: Evolution of quality of life
timeFrame: From randomization, up to 12 months
measure: Program feedback questionnaire
timeFrame: 12 months after randomization
Other outcomes:
Locations:
city: Angers
country: France
facility: Cystic Fibrosis Resource and Competence Centre, University Hospital, Angers
city: Roscoff
country: France
facility: Cystic Fibrosis Resource and Competence Centre, Fondation Ildys, Roscoff
city: Tours
country: France
facility: Cystic Fibrosis Resource and Competence Centre, University Hospital, Tours
city: Vannes
country: France
facility: Cystic Fibrosis Resource and Competence Centre, Hospital, Tours
Study officials:
affiliation: University Hospital, Tours
name: Julie MANKIKIAN, MD
role: PRINCIPAL_INVESTIGATOR
References: