Improving Care of Prader-Willi Syndrome : Evaluation of a New Care Program Combining Adapted Physical Activity, Nutrition and Therapeutic Education

Not Applicable

Completed

• Conditions: Prader-Willi Syndrome; Obesity

• Registration Number: NCT05249998
• Lead Sponsor: Assistance Publique - Hôpitaux de Paris

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.

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.

Study Timeline

• Study First Submitted: 2021-10-15
• Study Start: 2021-11-06
• Study First Submitted QC: 2022-02-21
• Study First Posted: 2022-02-22
• Primary Completion: 2024-05-04
• Study Completion: 2024-11-15
• Status Verified: 2025-09
• Last Update Submitted: 2025-09-30
• Last Update Posted: 2025-10-03

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 128

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)

Study & Design

• Study Type: INTERVENTIONAL
• Study Design: PARALLEL

Primary Outcome Measures

Name	Time	Method
Change in Body Mass Index (BMI)	week 7

Secondary Outcome Measures

Name	Time	Method
six minute walking test (6MWT)	week 4
Forced Vital Capacity	baseline and week 4
Maximum Volume Expired in the 1st Second	baseline and week 4
Prospective assessment of clinical features before and after Adapted Physical Activity Program	baseline and week 4	Weight in kilograms, Height in meters to calculate Body Mass Index (BMI) in kg/m\^2
Difference of CRP dosage between before and after Adapted Physical Activity Program	baseline and week 4	CRP measured before and after Adapted Physical Activity Program : difference between these two dosages
Difference of Glycated hemoglobin (Hba1C) between before and after Adapted Physical Activity Program	baseline and week 4	Glycated hemoglobin (Hba1C) measured before and after Adapted Physical Activity Program : difference between these two dosages
Difference of fasting blood glucose between before and after Adapted Physical Activity Program	baseline and week 4	fasting blood glucose level measured before and after Adapted Physical Activity Program : difference between these two dosages
Difference of cholesterol between before and after Adapted Physical Activity Program	baseline and week 4	Cholesterol measured before and after Adapted Physical Activity Program : difference between these two dosages
Difference of Glycemy between before and after Adapted Physical Activity Program	baseline and week 4	Glycemy measured before and after Adapted Physical Activity Program : difference between these two dosages
Difference of transaminases between before and after Adapted Physical Activity Program	baseline and week 4	Transaminases measured before and after Adapted Physical Activity Program : difference between these two dosages
Difference of Gamma GT between before and after Adapted Physical Activity Program	baseline and week 4	Gamma GT measured before and after Adapted Physical Activity Program : difference between these two dosages
Difference of Alkaline phosphatase between before and after Adapted Physical Activity Program	baseline and week 4	Alkaline phosphatase measured before and after Adapted Physical Activity Program : difference between these two dosages
Difference of urea between before and after Adapted Physical Activity Program	baseline and week 4	Urea measured before and after Adapted Physical Activity Program : difference between these two dosages
Difference of creatinine between before and after Adapted Physical Activity Program	baseline and week 4	creatinine measured before and after Adapted Physical Activity Program : difference between these two dosages
Difference of albumin between before and after Adapted Physical Activity Program	baseline and week 4	albumin measured before and after Adapted Physical Activity Program : difference between these two dosages
RELIME : REal LIfe Meal Evaluation score	baseline and week 4	This score is devided in 3 under-scores which are: * "eating behavior score" scale of 0 to 30; * "general behavior score" scale of 0 to 20; * "social behavior score" scale of 0 to 16 The final score is on a scale of 0 to 66. 0 is the minimum and the best score. 66 is the maximum and worst score.
Adhesion Grid Scores	baseline and week 4	This score is devided in 2 scores which are : "adhesion" on a scale of 0 to 2 "motivation" on a scale of 0 to 2; The final score is the addition of them on a scale of 0 to 4. The minimum and worst score is 0. The maximum and best score is 4.
Ricci & Gagnon self-questionnaire	baseline, Month 4, Month 7	This score is on a scale of 0 to 45, 0 is the minimum and worst score and 45 the maximum and worst score of physical activity.; Score under 18 means "inactive" Score between 18 and 35 means "active" Score superior to 35 means "very active"
Hyperphagia Questionnaire	baseline, Month 4, Month 7	This score is devided in 3 under-scores which are : * "hyperphagic behavior" score on a scale of 5 to 25; * "hyperphagic drive" score on a scale of 4 to 20; * "hyperphagic severity" score on a scale of 2 to 10 The final score is on a scale of 11 to 55. 11 is the minimum and best score. 55 is the maximum and worst score.
Goal Attainment Scaling	baseline, Month 4, Month 7	Goal Attainment Scaling (GAS) is a method for writting personalized evaluation scales in order to quantify progress towards defined goals.; The GAS methodology consists of: * defining an rehabilitation goal with the patient; * choosing an observable behavior that reflects the degree of goal attainment; * assessing the patient's initial level (pre- intervention) with respect to the goal; * defining five goal attainment levels , ranging from "no change" (-2) to "a much better than expected outcome" (+2); * Evaluating the patient after the set time frame and determine the GAS score between -2 and +2; A five point scale is used : " -2 " is the baseline ; " -1 " represents progression to the goal without goal attainment ; " 0 " is the expected level after intervention ; " +1 " is represent a better outcome than expected ; " +2 " is the best possible outcometha t could have been expected to the goal; Scores between " 0 " and " +2 " indicate that the patient has achieved the objective
Weight	Month 2, Month 3, Month 4, Month 5, Month 6, Month 7

Trial Locations

Locations (1)

Service diététique Hôpital Marin d'Hendaye- AP-HP; 🇫🇷 Hendaye, France