Medical, psychological and social features in a large cohort of adults with Prader-Willi syndrome: experience from a dedicated centre in France.
Adults with Prader-Willi syndrome usually carry several medical and psychiatric diagnoses plus fragile social lives, so BCBAs should build broad, lifelong support plans.
01Research in Context
What this study did
Worsham et al. (2015) looked at 154 adults with Prader-Willi syndrome who attend a special clinic in France. The team listed every medical, mood, and social problem each person had. They also noted which genetic subtype each adult carried.
What they found
Almost every adult had several health problems at once. Many also had psychiatric diagnoses such as mood swings or psychosis. Most lived with parents or in locked units; very few held jobs or had friends outside the home.
How this fits with other research
The picture matches earlier, smaller reports. Schneider et al. (2006) saw the same heavy medical load in 56 Australian adults. Fyfe et al. (2007) first warned that adults with the maternal UPD subtype get more mood episodes; the French data repeat that warning. Farrant et al. (1998) counted 17% affective psychosis in the UK; the French clinic sees the same pattern, just in a bigger group. Together the papers say: expect multiple comorbidities, and check genotype for mood-risk.
Why it matters
If you serve adults with PWS, plan for a full medical-psychiatric work-up every visit. Screen mood carefully in clients with maternal UPD. Start housing and job planning early; most adults will need supervised living and structured day activity. Use the French list as a quick comorbidity checklist so nothing gets missed.
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02At a glance
03Original abstract
BACKGROUND: Prader-Willi syndrome (PWS) is a developmental genetic disorder characterised by a variable expression of medical, cognitive and behavioural symptoms. In adulthood, the prevalence and severity of these symptoms determine the quality of life of the affected persons. Because of their rare disease condition, data on health and social problems in adults with PWS are scarce. In this research, we present medical, psychological and social features of a large cohort of adults admitted to a specialised PWS centre in France and analyse the differences according to genotype, gender and age. METHODS: Data from 154 patients (68 men/86 women), with a median age of 27 years (range 16-54), were collected during their stay in our centre. Clinical histories were completed using information from parents or main caregivers, and the same medical team performed the diagnosis of different clinical conditions. Statistical analyses were performed to determine the influence of factors such as genotype, age or gender. RESULTS: Paternal deletion genotype was the most frequent (65%) at all ages. Most patients had mild or moderate intellectual disability (87%). Only 30% had studied beyond primary school and 70% were in some special educational or working programme. Most of them lived in the family home (57%). The most prevalent somatic comorbidities were scoliosis (78%), respiratory problems (75%), dermatological lesions (50%), hyperlipidaemia (35%), hypothyroidism (26%), Type 2 diabetes mellitus (25%) and lymph oedema (22%). Some form of psychotropic treatment was prescribed in 58% of subjects, and sex hormones in 43%. Patients with deletion had a higher body mass index (44 vs. 38.9 kg/m(2)) and displayed higher frequency of sleep apnoeas. Non-deletion patients received insulin treatment (19% vs. 4%) and antipsychotic treatment (54.8% vs. 32.7%) more frequently. No difference was observed in the prevalence of Type 2 diabetes between the two genotype groups. Patients >27 years of age had a higher rate of comorbidities (Type 2 diabetes, hypertension, respiratory problems and lymph oedema). Gender differences were minor. CONCLUSIONS: Adult patients with PWS showed high prevalence of comorbid health problems that need to be monitored for early treatment. Some of them are influenced by genotype and age. Another salient problem concerns the lack of adapted structures for better social integration. Further data about the real life and health conditions of adults with PWS are necessary to further our knowledge of the natural history of the disease and to design appropriate care strategies.
Journal of intellectual disability research : JIDR, 2015 · doi:10.1111/jir.12140