Poster Presentation Australian & New Zealand Obesity Society 2015 Annual Scientific Meeting

A PWS clinic – is it worth the effort? (#255)

Janet Franklin 1 , Elisia Manson 1 , Georgina Loughnan 1 , Tegan Picone 1 , Tania Markovic 1 2
  1. Metabolism and Obesity Services, RPAH, Sydney, NSW, Australia
  2. The Boden Institute of Obesity, Nutrition, Exercise & Eating Disorders, University of Sydney, Sydney, NSW, Australia

Prader-Willi Syndrome (PWS) is a genetic syndrome associated with severe obesity and a historic life expectancy of late 20s. PWS obesity results from an energy imbalance influenced by hypothalamic dysfunction. People with PWS have cognitive limitations and display emotional immaturity often with behavioural outbursts. Maintaining health in this population is difficult and often requires unpopular strategies. Medical presentations can differ markedly from the general population, thus complicating or delaying management. Given the complexities of this syndrome, we sought to identify a successful model of care for people with PWS.

Evaluation of the PWS clinic at RPAH was undertaken to determine if a specific clinic achieves meaningful outcomes for clients with PWS and their families/carers and to determine what methods were most effective in the treatment of their obesity. Data collected over 24 years was used to determine weight and health outcomes.

Eighty six patients have attended the clinic, 46 of whom have attended regularly. The mean age was 30 yrs (range 11 to 58) with 5 patients >50yrs (to July 2015). 20% of patients are currently in the healthy or overweight range. The mean maximum BMI of the patients was 47kg/m2 and the current mean BMI of the clinic population is 40 kg/m2 (range 22 – 76). Of the cohort 16 have died, four of whom resided in appropriate residential care. 81% of deaths were from obesity related complications.
Residential care was the most important marker for successful treatment. Restrictive practices, consistent management and home-based exercise equipment contributed to superior results. Preliminary carer feedback indicated specialised healthcare, training and advocacy were the most beneficial components of the clinic.

A specialised PWS clinic effectively addresses the complex needs of this population and also provides support and education for their families, caregivers and health professionals contributing to greater longevity and improved health.