Self concept in people with Williams syndrome and Prader-Willi syndrome

This study explored self concepts in matched groups of adolescents and adults with Williams syndrome (WS) and Prader-Willi syndrome (PWS), using Damon and Hart's semi-structured interview. The main findings were that the WS participants were more productive in their responses to the interview, providing more self characteristics. The WS group also used more social and psychological categories in describing their self concept, and their responses were in general at a higher level than the responses for the PWS group. There were significant age-group differences, with the adolescents offering more self descriptions that were coded as physical and active, whereas the adults gave more social and psychological responses. Different themes were emphasized in the interviews from the two groups. These themes reflected the distinct phenotypes associated with these disorders, the participants life experiences, and their overall attitudes toward their syndrome.