Search results for ‘Subject term:"prader-willi syndrome"’ Sort:
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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.
Values in the care for young persons with Prader-Willi syndrome: creating a meaningful life together
- Authors:
- van HOOREN R.H., et al
- Journal article citation:
- Child: Care, Health and Development, 31(3), May 2005, pp.309-319.
- Publisher:
- Wiley
Caregivers sometimes feel frustrated by present emphasis on self-determination in the care for individuals with an intellectual disability. They are confronted with situations in which there is a conflict between providing high quality care and respecting the client's autonomy. Such conflicts are prominent in the care for people with PraderWilli syndrome (PWS). This condition is characterized by overeating behaviour which frequently leads to serious obesity. This study investigates how persons involved, parents and professional caregivers, deal with the problems of the prevention of obesity in young individuals with PWS in everyday life. In-depth qualitative interviews were conducted with 23 parents and 14 professional caregivers of 18 persons with PWS. The interview data were analysed using the constant comparative method as described in grounded theory. From analysis of the interviews four values emerge that parents and professional caregivers indicate as relevant and directive to their acting towards the child or the client: physical health, well-being, freedom and responsibility, and a liveable life. Caregivers also mentioned several enabling and limiting conditions that influence these values. And they named several interventions they used to realize the values. Of importance is the question how to create life in such a way that it is acceptable and meaningful for everybody involved. By building up a relationship to which acceptance and trust are central, a certain amount of independence and responsibility can be promoted. By creating trust it is possible to deal with rules in a less rigid way and to look where there is room for choices.