[Identification of psychosocial practices and interventions that support the adaptation and rehabilitation of adults with Prader-Willi Syndrome]

Beaumier I, St-Jacques S, Ndjepel J, Lehoux MC
Record ID 32018001697
English, French
Original Title: Identification des pratiques et des interventions psychosociales favorisant l'adaptation et la réadaptation des personnes adultes présentant le syndrome de Prader-Willi
Authors' objectives: To identify psychosocial practices and interventions that support adaptation and rehabilitation in adults with PWS.
Authors' results and conclusions: Of the 10 evaluated interventions listed in the scientific literature, the majority use a cognitive-behavioural approach. The approaches evaluated are different and the components worked on are multiple. Programs and interventions must be adapted to the needs and particularities of each individual with PWS: the application of a single program or intervention does not seem possible for this population, since individuals differ greatly from each other.Interventions should aim to prevent and avoid behavioural disorders. It is important to analyze and understand behaviours and their context. Food has a highly reinforcing effect and is a source of stress and anxiety. The acquisition of full autonomy by a person with PWS does not seem possible, regardless of his profile.
Authors' recommendations: PSYCHOSOCIAL INTERVENTIONS AND PRACTICES - the multimodal analysis and intervention approach for intervention strategies and crisis prevention (MMAI) and behaviour management in crisis situations (Therapeutic Intervention in Aggressive Behaviour (TIAB), OMEGA-plus) are to be preferred for adults with PWS. - In order to prevent and avoid behavioural problems, it is necessary to analyze and try to understand the residents’ behaviours. - The use of a non-food reward system to motivate and maintain good behaviour should be promoted and maintained. -Although the results from the literature are not generalizable and that there is not a single program, some of the interventions and strategies identified could be attempted with consultation with stakeholders, taking into account the characteristics, needs and capacities of each individual with PWS. CLINICAL, ORGANIZATIONAL AND ARCHITECTURAL REQUIREMENTS - The members of the working team should be highly qualified in the specific issue of PWS. An intellectual disability training would not be sufficient. - All staff (clinical, management and support) should receive initial training in PWS before working with clients, as well as ongoing training..
Authors' methods: A systematic literature review was conducted in seven bibliographic databases. In order to to identify relevant documents in the grey literature, 32 Websites were consulted and the Google search engine was used.
Project Status: Completed
Year Published: 2019
Requestor: Health professional involved with adults with Prader-Willi syndrome
English language abstract: An English language summary is available
Publication Type: Full HTA
Country: Canada
MeSH Terms
  • Prader-Willi Syndrome
  • Rehabilitation
  • Psychosocial Intervention
  • Psychosocial Support Systems
  • Cognitive Behavioral Therapy
  • Prader-Willi syndrome
  • rehabilitation
  • cognitive-behavioural approach
Organisation Name: Centre intégré universitaire de santé et de services sociaux de la Capitale-Nationale
Contact Address: 525, boulevard Wilfrid-Hamel, bureau A-122
Contact Name: Sylvie St-Jacques
Contact Email: uetmisss.deau.ciussscn@ssss.gouv.qc.ca
This is a bibliographic record of a published health technology assessment from a member of INAHTA or other HTA producer. No evaluation of the quality of this assessment has been made for the HTA database.