Prader-Willi syndrome: clinical picture, psychosocial support and current management

Authors:
WIGREN M., HANSEN S.
Journal article citation:
Child: Care, Health and Development, 29(6), November 2003, pp.449-456.
Publisher:
Wiley

Maps the need for psychosocial support and current management of children and adolescents with Prader–Willi syndrome (PWS), a rare, genetically based disorder occurring in about 1 in 15,000 live-born children whose cardinal symptoms include low muscle tone at birth, overeating, motor and speech impairments, delayed pubertal development, cognitive dysfunctions and psychiatric symptoms. To raise a child with PWS is challenging for parents and requires support from multiprofessional habilitation services. Parents of 58 children with PWS aged 5–18 who received their diagnosis at a mean age of 2.5 completed questionnaires covering clinical, diagnostic and psychosocial issues. Growth hormone treatment was given to 72% and 63% were not overweight. Early neuropsychiatric symptoms were common, some related to obesity. Most parents wanted information on availability of external resources and future child needs. Few needed family-directed support. The overall impression is that the disorder is managed relatively well. Even so, PWS symptoms typically exacerbate over time and consequently parents need continuous support throughout childhood and adolescence. Greater attention should be paid to idiosyncrasies in cognitive functioning and to clinical markers of neuropsychiatric problems.

Subject terms:
learning disabilities, mental health problems, parents, Prader-Willi syndrome, children;
Content type:
research
Link:
Journal home page
ISSN online:
1365-2214
ISSN print:
0305-1862

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