A long-term population-based clinical and morbidity review of Prader-Willi syndrome in Western Australia.
Adults with Prader-Willi syndrome carry heavy medical and psychiatric loads and will soon need more sheltered housing and specialized care.
01Research in Context
What this study did
Schneider et al. (2006) counted every person with Prader-Willi syndrome in Western Australia. They listed each person’s health problems, medicines, and where the person lived.
The team wanted a full picture of medical and housing needs across the lifespan.
What they found
Most adults with PWS still lived with aging parents. Many already had diabetes, sleep apnea, or scoliosis.
The authors warned that sheltered housing and medical services will be needed as these adults grow older.
How this fits with other research
Worsham et al. (2015) later studied 154 French adults and saw the same pattern: heavy medical load and few social outlets. Their larger sample added genotype details, extending the Australian snapshot.
Fyfe et al. (2007) followed the same population forward and found repeated mood episodes, especially in the maternal-upd subtype. This longitudinal view extends the 2006 cross-sectional picture.
Farrant et al. (1998) had earlier flagged a 17% rate of affective psychosis in PWS. The 2006 study’s broader count lines up with that warning and shows the medical system must plan for both mental and physical care.
Why it matters
If you serve adults with PWS, expect a wave of dual needs: medical complications plus mood or psychotic episodes. Start housing referrals before parents burn out. Build psychiatric screening into annual checks. Use the French and Australian data when you ask funders for expanded medical and residential slots.
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02At a glance
03Original abstract
BACKGROUND: An investigation of the clinical morbidity and genetic profiles of individuals with Prader-Willi syndrome (PWS) in Western Australia (WA) was undertaken as part of a wider study into the effects of intellectual disability (ID) on the life course of individuals. METHODS: All persons with a diagnosis of PWS were identified from the records of the Disability Services Commission of WA (DSC). The DSC client files formed the main data source, and were supplemented by information from other state health data sets. The analysis was retrospective and quantitative in nature. RESULTS: A total of 56 individuals were identified, 10 of whom exhibited normal methylation patterns and so were analysed separately (PWS-like). The ages of the PWS group ranged from 0.9 to 48.3 years, with six persons deceased. Most people with PWS (76%) had mild or moderate ID, and 70% lived in their family home. The birth prevalence of the disorder was 1 in 29 500 births. Respiratory disorders, dentistry and gastrointestinal disorders were common reasons for hospital admission, with epilepsy or convulsions also reported at moderate frequency. The PWS-like group shared many clinical features in common with PWS patients, the principal exceptions being hypotonia and feeding difficulties in infancy. CONCLUSIONS: The estimated birth prevalence of PWS was lower than expected; however, the case ascertainment method may have excluded some individuals. Older people with PWS were generally living in sheltered accommodation. As the cohort ages, demand for places in similar accommodation will increase, adding to the existing burden on service providers. Substantial future increases in the use of medical services and hospital-based care also are predicted with the onset of age-associated disorders.
Journal of intellectual disability research : JIDR, 2006 · doi:10.1111/j.1365-2788.2005.00770.x