Angelman Syndroom en Prader-Willi Syndroom:  een overzicht, gecombineerd met praktische kennis voor zorgverleners: De zorgbehoeften onderzocht en besproken in de context van het AAIDD-Model.

Aim: The aim of this thesis is to contribute to the orthopedagogical knowledge on Angelman Syndrome (AS) and Prader-Willi Syndrome (PWS), to create a useful framework for identifying support needs and to obtain more structure and uniformity in existing knowledge. This is done on the basis of two research questions: 1) What are the characteristics (genotype and phenotype) of the Angelman Syndrome and the Prader-Willi Syndrome? 2) Is it known from literature whether people with AS or PWS have an intensive need for care? Is a distinction noticeable between the group from the literature and the group of clients with AS, who all have an intensive care demand, in the SCORE research? Method: For the first research question, extensive literature research at meta level was performed. In addition, the literature has been analyzed on the basis of the domains of the AAIDD-model. A multiple case study was conducted for the second research question. Of the 225 clients, 2 were clients with AS and...Show moreAim: The aim of this thesis is to contribute to the orthopedagogical knowledge on Angelman Syndrome (AS) and Prader-Willi Syndrome (PWS), to create a useful framework for identifying support needs and to obtain more structure and uniformity in existing knowledge. This is done on the basis of two research questions: 1) What are the characteristics (genotype and phenotype) of the Angelman Syndrome and the Prader-Willi Syndrome? 2) Is it known from literature whether people with AS or PWS have an intensive need for care? Is a distinction noticeable between the group from the literature and the group of clients with AS, who all have an intensive care demand, in the SCORE research? Method: For the first research question, extensive literature research at meta level was performed. In addition, the literature has been analyzed on the basis of the domains of the AAIDD-model. A multiple case study was conducted for the second research question. Of the 225 clients, 2 were clients with AS and there were no clients with PWS. The results of the BSID-III, CI, VABS, DBC-A and the SMS have been analyzed. Results: People with AS have a profound intellectual disability. There is a severe disorder in every domain of the VABS. There is little to no participation in society. People with AS often have sleep disturbances, seizures, ataxia, and motor development delays. The living environment differs for each client. People with PWS have a mild intellectual disability with an average IQ between 60-70 but in addition also have severe medical and physical challenges. Conclusion: People with AS have an intensive need for care due to their low functioning level. More research is needed on the health care needs of people with PWS to conclude whether they have intensive care needs.Show less

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Angelman Syndroom en Prader-Willi Syndroom: een overzicht, gecombineerd met praktische kennis voor zorgverleners: De zorgbehoeften onderzocht en besproken in de context van het AAIDD-Model.