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

Cystic Fibrosis Clinical Trial

— DIAPASOM  

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

Clinical Trial Details — Status: Not yet recruiting

Administrative data

• NCT number: NCT06457451
• Other study ID #: 2023-A02715-40
• Secondary ID: DR220270
• Status: Not yet recruiting
• Phase: N/A
• Start date: September 2024
• Est. completion date: September 2027

Study information

• Verified date: June 2024
• Source: University Hospital, Tours
• Contact: Amelie GIBORY
• Phone: 0247474747
• Email: a.gibory[@]chu-tours.fr
• Is FDA regulated: No
• Study type: Interventional

Clinical Trial 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.

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.

Recruitment information / eligibility

• Status: Not yet recruiting
• Enrollment: 100
• Est. completion date: September 2027
• Est. primary completion date: September 2027
• Accepts healthy volunteers: No
• Gender: All
• Age group: 18 Years and older

Eligibility

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

Related Conditions & MeSH terms

• Cystic Fibrosis
• Fibrosis

Intervention

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.

Locations

Country	Name	City	State
France	Cystic Fibrosis Resource and Competence Centre, University Hospital, Angers	Angers
France	Cystic Fibrosis Resource and Competence Centre, Fondation Ildys, Roscoff	Roscoff
France	Cystic Fibrosis Resource and Competence Centre, University Hospital, Tours	Tours
France	Cystic Fibrosis Resource and Competence Centre, Hospital, Tours	Vannes

Sponsors (2)

Lead Sponsor	Collaborator
University Hospital, Tours	Fondation Ildys

Country where clinical trial is conducted

France, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Evolution of percentage of patients lean mass as a percentage of body mass	Impedancemetry	From randomization, up to 12 months
Secondary	Evolution of percentage of patients fat mass as a percentage of body mass	impedancemetry	From randomization, up to 12 months
Secondary	Weight evolution	Weight measurement	From randomization, up to 12 months
Secondary	Body Mass Index (BMI) evolution	Weight and height measurement	From randomization, up to 12 months
Secondary	Cardio-respiratory endurance	6-Minute Walk Test (6MWT)	From randomization, up to 12 months
Secondary	Bilateral Handgrip strength	Handgrip Test	From randomization, up to 12 months
Secondary	Lower limb muscle power	30-second Sit-to-Stand test	From randomization, up to 12 months
Secondary	Upper limb muscle power	30-second Arm Curl Test	From randomization, up to 12 months
Secondary	Static trunck extensors muscle endurance	"Superman" test	From randomization, up to 12 months
Secondary	Static trunck flexors muscle endurance	Shirado-Ito test	From randomization, up to 12 months
Secondary	Balance between static muscular endurance of extensors and flexors	Calculated using the Shirado-Ito/"Superman" ratio	From randomization, up to 12 months
Secondary	Posterior chain flexibility (Hamstring, hips and lower back)	Front trunk flexion test	From randomization, up to 12 months
Secondary	Upper limb flexibility	scapulohumeral mobility test	From randomization, up to 12 months
Secondary	Forced Expiratory Volume in 1 second (FEV1)	Spirometry	From randomization, up to 12 months
Secondary	Physical activity volume and sedentary time	Physical activity and sedentary behavior self-questionnaire (ONAPS-PAQ)	From randomization, up to 12 months
Secondary	Evolution of quality of life	Cystic Fibrosis Questionnaire-Revised (CFQR-14)	From randomization, up to 12 months
Secondary	Program feedback questionnaire	Self-questionnaire about how patients in the experimental group feel about the program	12 months after randomization