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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.
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 :
At 3 and 6 months after hospitalization :
Evaluate the impact of the intervention on :
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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Interventional group | Experimental | Patients will receive the usual care, with the intervention of the multidisciplinary team which defines with the patient the objectives of the stay and the inter-stay period, without "specific therapeutic education" on adapted physical activity, nutrition and eating behaviour. |
|
| Standard care group | No Intervention | Patients will benefit from the activities already proposed as part of the usual practice and in addition a multidisciplinary staff will take place in order to interpret the assessments of the patients included and to direct the patients according to their phenotypic profile towards the different programmes of adapted physical activity. |
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Program combing APA, Nutrition, and Therapeutic Education | Other | Patients will benefit from the activities already proposed as part of the usual practice and in addition a multidisciplinary staff will take place in order to interpret the assessments of the patients included and to direct the patients according to their phenotypic profile towards the different programmes of adapted physical activity. |
| Measure | Description | Time Frame |
|---|---|---|
| Change in Body Mass Index (BMI) | week 4 | |
| Change in Body Mass Index (BMI) | week 7 |
| Measure | Description | Time Frame |
|---|---|---|
| six minute walking test (6MWT) | week 4 | |
| Forced Vital Capacity | baseline and week 4 | |
| Maximum Volume Expired in the 1st Second |
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Inclusion Criteria:
Exclusion Criteria:
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| Name | Affiliation | Role |
|---|---|---|
| Hélène PASTRE | Assistance Publique - Hôpitaux de Paris | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Service diététique Hôpital Marin d'Hendaye- AP-HP | Hendaye | 64701 | France |
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|
| baseline and week 4 |
| Prospective assessment of clinical features before and after Adapted Physical Activity Program | Weight in kilograms, Height in meters to calculate Body Mass Index (BMI) in kg/m^2 | baseline and week 4 |
| Difference of CRP dosage between before and after Adapted Physical Activity Program | CRP measured before and after Adapted Physical Activity Program : difference between these two dosages | baseline and week 4 |
| Difference of Glycated hemoglobin (Hba1C) between before and after Adapted Physical Activity Program | Glycated hemoglobin (Hba1C) measured before and after Adapted Physical Activity Program : difference between these two dosages | baseline and week 4 |
| Difference of fasting blood glucose between before and after Adapted Physical Activity Program | fasting blood glucose level measured before and after Adapted Physical Activity Program : difference between these two dosages | baseline and week 4 |
| Difference of cholesterol between before and after Adapted Physical Activity Program | Cholesterol measured before and after Adapted Physical Activity Program : difference between these two dosages | baseline and week 4 |
| Difference of Glycemy between before and after Adapted Physical Activity Program | Glycemy measured before and after Adapted Physical Activity Program : difference between these two dosages | baseline and week 4 |
| Difference of transaminases between before and after Adapted Physical Activity Program | Transaminases measured before and after Adapted Physical Activity Program : difference between these two dosages | baseline and week 4 |
| Difference of Gamma GT between before and after Adapted Physical Activity Program | Gamma GT measured before and after Adapted Physical Activity Program : difference between these two dosages | baseline and week 4 |
| Difference of Alkaline phosphatase between before and after Adapted Physical Activity Program | Alkaline phosphatase measured before and after Adapted Physical Activity Program : difference between these two dosages | baseline and week 4 |
| Difference of urea between before and after Adapted Physical Activity Program | Urea measured before and after Adapted Physical Activity Program : difference between these two dosages | baseline and week 4 |
| Difference of creatinine between before and after Adapted Physical Activity Program | creatinine measured before and after Adapted Physical Activity Program : difference between these two dosages | baseline and week 4 |
| Difference of albumin between before and after Adapted Physical Activity Program | albumin measured before and after Adapted Physical Activity Program : difference between these two dosages | baseline and week 4 |
| RELIME : REal LIfe Meal Evaluation score | This score is devided in 3 under-scores which are:
| baseline and week 4 |
| Adhesion Grid Scores | 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. | baseline and week 4 |
| Ricci & Gagnon self-questionnaire | 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" | baseline, Month 4, Month 7 |
| Hyperphagia Questionnaire | This score is devided in 3 under-scores which are :
| baseline, Month 4, Month 7 |
| Goal Attainment Scaling | 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:
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 | baseline, Month 4, Month 7 |
| Weight | Month 2, Month 3, Month 4, Month 5, Month 6, Month 7 |
| ID | Term |
|---|---|
| D011218 | Prader-Willi Syndrome |
| D009765 | Obesity |
| ID | Term |
|---|---|
| D008607 | Intellectual Disability |
| D019954 | Neurobehavioral Manifestations |
| D009461 | Neurologic Manifestations |
| D009422 | Nervous System Diseases |
| D000015 | Abnormalities, Multiple |
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D025063 | Chromosome Disorders |
| D030342 | Genetic Diseases, Inborn |
| D000096803 | Imprinting Disorders |
| D050177 | Overweight |
| D044343 | Overnutrition |
| D009748 | Nutrition Disorders |
| D009750 | Nutritional and Metabolic Diseases |
| D001835 | Body Weight |
| D012816 | Signs and Symptoms |
| D013568 | Pathological Conditions, Signs and Symptoms |
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| ID | Term |
|---|---|
| D009752 | Nutritional Status |
| ID | Term |
|---|---|
| D009747 | Nutritional Physiological Phenomena |
| D000066888 | Diet, Food, and Nutrition |
| D010829 | Physiological Phenomena |
| D006304 | Health Status |
| D003710 | Demography |
| D011154 | Population Characteristics |
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