Management of obesity in a patient with Prader-Willi syndrome (PWS) requires a tailored, multidisciplinary approach that addresses the unique challenges of this genetic condition alongside general obesity management principles. Firstly, early and ongoing assessment of weight status and comorbidities is essential, using BMI and waist-to-height ratio to classify obesity severity and associated health risks. This helps identify complications such as type 2 diabetes, hypertension, and cardiovascular disease, which are common in obesity and may be exacerbated in PWS NICE NG246.
Behavioural interventions should be adapted to the cognitive and developmental profile of the patient with PWS, focusing on structured environments to control food access and promote healthy eating habits. Given the hyperphagia and impaired satiety characteristic of PWS, behavioural strategies must include family and carers to maintain strict supervision and support sustained behavioural changes NICE NG246 Passone et al. 2018.
Physical activity should be encouraged within the patient’s capabilities to improve energy expenditure and overall health, considering any physical or developmental limitations. Tailored exercise programs can help manage weight and improve wellbeing NICE NG246.
Referral to specialist obesity management services is recommended for patients with PWS, especially when weight-related comorbidities are present or when behavioural interventions alone are insufficient. Specialist input can provide multidisciplinary support including dietitians, endocrinologists, and psychologists experienced in PWS NICE NG246.
Pharmacological and surgical interventions for obesity in PWS should be considered cautiously and typically reserved for specialist settings due to the complex nature of the syndrome and potential risks. Current UK guidelines emphasise behavioural and supportive care first, with specialist referral for advanced therapies NICE NG246. Recent literature highlights the importance of a comprehensive approach that integrates genetic, metabolic, and behavioural aspects unique to PWS Passone et al. 2018.
Throughout management, clinicians should engage in shared decision-making with the patient and their family or carers, considering psychosocial factors, developmental stage, and readiness to engage with interventions. This holistic approach aligns with NICE principles for obesity care and the specific needs of PWS patients NICE NG246.