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Monitoring progress in a multi disciplinary team prader willi syndrome (PWS) clinic – all is not always what it seems
  1. C Smith1,
  2. S Kanumakala2,
  3. A Livesey3
  1. 1Nutrition and Dietetics, Brighton and Sussex University Hospitals NHS Trust, Brighton, UK
  2. 2Paediatric Medicine, Brighton and Sussex University Hospitals NHS Trust, Brighton, UK
  3. 3Community Child Health, Southdowns NHS Trust, Brighton, UK

Abstract

Aims Children with Prader Willi Syndrome (PWS) are often reviewed in general clinics where knowledge of PWS is limited and advice fragmented. We describe the benefits of a multi disciplinary PWS clinic and additional monitoring tools, illustrated by a case study.

Methods The clinic combines expertise in paediatric endocrinology, dietetics and community child health. Advice is given on guidance on eating & other behaviours, and meal composition. Use of 3 day food diaries is combined with measuring body composition using bioelectrical impedence and international PWS growth charts.

Results Prospective guidance on eating behaviour and expectations from an early age has been associated with more controlled growth patterns in PWS children. Advice & interventions for the range of growth, behavioural, medical & educational needs have been facilitated through this joint approach. Parent feedback is positive. X is a 6 year old boy with PWS. Over the last 3 years weight on UK90 growth charts has shown accelerated weight velocity increasingly out of proportion to height. Equivalent plots on American PWS specific charts showed growth velocity and centile positions (both for weight and height) appropriate for age. Equivalent plots on European PWS charts also showed centile position more appropriate for age. 3 day food diaries over the 3 year period indicated caloric intakes below the Reference Nutrient Intake for age. The balance of nutrients remained steady and no significant micronutrient deficiency was identified as a result of following a reduced calorie diet. Serial measurements of body composition using impedance showed fluctuations in %fat but some level of stability in the face of steadily rising body mass index (19 in year 1 increasing to 24 in year 3).

Conclusion The management of PWS children can ideally be delivered by a local multidisciplinary team who develop experience in the condition and can access appropriate monitoring equipment. Dietary analysis, bioelectrical impedance measurements and use of international reference data is quick, repeatable and cheap. As highlighted in our case study these provide invaluable feedback to families where positive aspects of management would have gone unrecognised without specific additional monitoring.

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