Clinical trial NCT05249998: Improving Care of Prader-Willi Syndrome : Evaluation of a New Care Program Combining Adapted Physical Activity, Nutrition and Therapeutic Education
Official title: Improving Care of Prader-Willi Syndrome : Evaluation of a New Care Program Combining Adapted Physical Activity, Nutrition and Therapeutic Education
Study type: INTERVENTIONAL
Overall status: ACTIVE_NOT_RECRUITING
Phase: NA
Conditions: Prader-Willi Syndrome
Obesity
Keywords: Prader Willi Syndrome
Therapeutic Education
Adapted Physical Activity
Nutrition
Lead sponsor: Assistance Publique - Hôpitaux de Paris
Sponsor class: OTHER
First posted: 2022-02-22
Enrollment: 128
Sex: ALL
Minimum age (years): 18
Maximum age (years): 60
Standard ages: ADULT
Accepts healthy volunteers: no
Allocation: RANDOMIZED
Intervention model: PARALLEL
Primary purpose: PREVENTION

Brief summary: Prader-Willi syndrome (PWS) is a rare and complex genetic disease characterized by hypothalamic-pituitary axis dysfunction combining eating disorders associated with hyperphagia and satiety deficiency, mild intellectual deficit and behavioral disorders. This disease requires continuous management through specific therapeutic education to prevent metabolic and cardiorespiratory complications related to obesity. Physical activity must therefore be regular, adapted to the disability, taking into account cognitive deficits and behavioral disorders.

Prader-Willi syndrome (PWS) is a rare and complex genetic disease characterized by hypothalamic-pituitary axis dysfunction combining eating disorders associated with hyperphagia and satiety deficiency, mild intellectual deficit and behavioral disorders. This disease requires continuous management through specific therapeutic education to prevent metabolic and cardiorespiratory complications related to obesity. Physical activity must therefore be regular, adapted to the disability, taking into account cognitive deficits and behavioral disorders.

The Unit of Care and Rehabilitation, of the Hôpital Marin d'Hendaye, receives patients with PWS or other obesity from rare causes, at a rate of 1 to 2 annual stays of 1 to 2 months, which allow the regulation of somatic disorders and the initiation of weight loss. However, the difficulty is to keep a stable weight curve between stays. Only 20% of patients over the last 2 years managed to stabilize or decrease their BMI during the 6 months following their hospitalization.

The proposed study aims to evaluate an innovative and individualized care program combining Physical activity, Nutrition and therapeutic education for adults with PWS who will be admitted to the hospital for 5 weeks.

We hypothesize that this program will allow to stabilize or decrease the BMI of patients at 6 months after hospitalization by inducing a behavioral change in terms of physical activity and eating behavior.

We will conduct a randomized controlled trial on 128 patients who will participate in this program, or will benefit from standard care.

Detailed description: Main objective :

Evaluate the effectiveness at 6 months of an intensified and individualised care programme combining theoretical and practical therapeutic education workshops on adapted physical activity, nutrition and eating behaviour (i.e. intervention) during hospitalisation, compared to usual care (i.e. control).

The assessment will be done by comparing the proportion of patients stabilizing or decreasing their BMI at 6 months after the end of the stay (M7) in the 2 groups (intervention/control).

Secondary objectives:

During hospitalization:

To evaluate, compared to the usual care, the impact of the intervention on :

1. The evolution of physical capacities,
2. The evolution of metabolic criteria and metabolic and cardio-respiratory risk factors,
3. The evolution of eating behaviour,
4. Adherence to the program during hospitalization.

At 3 and 6 months after hospitalization :

Evaluate the impact of the intervention on :

1. The evolution of weekly physical activity at home,
2. The evolution of eating behaviour at home,
3. Reaching the personalized objective set for the inter-stay.

At 6 months after hospitalization:

Evaluate, in comparison with the usual management, the impact of the intervention on the evolution of metabolic criteria and metabolic and cardio-respiratory risk factors during a consultation at the hospital in their reference center.

Eligibility criteria: Inclusion Criteria:

* Patient between 18 and 60 years old Patient with genetically confirmed PWS
* Admitted for a 5-week stay in the Hendaye rehabilitation care unit
* Patient or legal guardian (tutor or curator) who has given written informed consent to participate in the study
* With the presence of an external relay available to coordinate the patient's journey during the protocol: family or educational referent (home)
* Affiliated with French social security except patient under AME (State medical aid)

Exclusion Criteria:

* Decompensation of a cardiorespiratory or psychiatric pathology in the 3 months prior to inclusion that required hospitalisation
* Patients whose condition may interfere with their optimal participation in the study (e.g., severe behavioural problems preventing participation in group workshops)

Primary outcomes:
measure: Change in Body Mass Index (BMI)
timeFrame: week 4
measure: Change in Body Mass Index (BMI)
timeFrame: week 7
Secondary outcomes:
measure: six minute walking test (6MWT)
timeFrame: week 4
measure: Forced Vital Capacity
timeFrame: baseline and week 4
measure: Maximum Volume Expired in the 1st Second
timeFrame: baseline and week 4
measure: Prospective assessment of clinical features before and after Adapted Physical Activity Program
timeFrame: baseline and week 4
measure: Difference of CRP dosage between before and after Adapted Physical Activity Program
timeFrame: baseline and week 4
measure: Difference of Glycated hemoglobin (Hba1C) between before and after Adapted Physical Activity Program
timeFrame: baseline and week 4
measure: Difference of fasting blood glucose between before and after Adapted Physical Activity Program
timeFrame: baseline and week 4
measure: Difference of cholesterol between before and after Adapted Physical Activity Program
timeFrame: baseline and week 4
measure: Difference of Glycemy between before and after Adapted Physical Activity Program
timeFrame: baseline and week 4
measure: Difference of transaminases between before and after Adapted Physical Activity Program
timeFrame: baseline and week 4
measure: Difference of Gamma GT between before and after Adapted Physical Activity Program
timeFrame: baseline and week 4
measure: Difference of Alkaline phosphatase between before and after Adapted Physical Activity Program
timeFrame: baseline and week 4
measure: Difference of urea between before and after Adapted Physical Activity Program
timeFrame: baseline and week 4
measure: Difference of creatinine between before and after Adapted Physical Activity Program
timeFrame: baseline and week 4
measure: Difference of albumin between before and after Adapted Physical Activity Program
timeFrame: baseline and week 4
measure: RELIME : REal LIfe Meal Evaluation score
timeFrame: baseline and week 4
measure: Adhesion Grid Scores
timeFrame: baseline and week 4
measure: Ricci & Gagnon self-questionnaire
timeFrame: baseline, Month 4, Month 7
measure: Hyperphagia Questionnaire
timeFrame: baseline, Month 4, Month 7
measure: Goal Attainment Scaling
timeFrame: baseline, Month 4, Month 7
measure: Weight
timeFrame: Month 2, Month 3, Month 4, Month 5, Month 6, Month 7
Other outcomes:
Locations:
city: Hendaye
country: France
facility: Service diététique Hôpital Marin d'Hendaye- AP-HP
Study officials:
affiliation: Assistance Publique - Hôpitaux de Paris
name: Hélène PASTRE
role: PRINCIPAL_INVESTIGATOR
References: