People with PWS have a high risk of becoming obese due to the high fat to muscle ratio causing reduced energy expenditure and the need for reduced energy intake, whilst the altered appetite regulation and food reward signalling can make a person obsessive about food and likely to be feeling hungrier than others. Imposing a strict diet on someone with hyperphagia can be difficult, but without effective dietary management, people with PWS will rapidly become morbidly obese and suffer serious health issues that will impact the quality and length of their life.
Understanding nutritional phases in PWS
In the past, PWS was described as a 2 stage syndrome – failure to thrive followed by hyperphagia. It is now known that the changes in appetite and weight gain are more gradual and complex. Parents of children diagnosed in infancy have the opportunity to instil diet modifications and healthy eating habits well before the child’s appetite or interest in food increases. As a result, when phase 3 begins, it is often less severe in families who have implemented early intervention measures.
Phase Ages Characteristics
1a 0 – 9 months Hypotonia with difficulty feeding and decreased appetite
1b 9 – 25 months Improved feeding and appetite; growing appropriately
2a 2.1 – 4.5 years Weight increasing without appetite increase or excess calories
2b 4.5 – 8 years Increased appetite and interest in food, but can feel full
3 8 years – adulthood Hyperphagic (abnormally increased appetite); rarely feels full
4 adulthood Appetite is no longer insatiable (only very few adults)
WHAT IS THE BEST DIET FOR PWS?
- A reduced-energy intake, well-balanced diet improves weight control in children with Prader-Willi syndrome – J. L. Miller, C. H. Lynn, J. Shuster, & D. J. Driscoll, 2012.
- Exercise and Physical Activity for children with Prader Willi Syndrome: A Guide for Parents and Carers – Kirsty Reid and Peter SW Davies, Children’s Nutrition Research Centre, University of Queensland
- New Concepts in Nutrition: PWS Nutrition Revised – Linda M. Gourash, MD, Pittsburgh Partnership, 2017
- Optimal Nutrition in Prader-Willi Syndrome – Slides by Melanie Silverman MS, RD, IBCLC.
- Nutrition Tips for PWS – VIDEO of Hannah Stahmer, MS, RDN, LD, presenting at The Mac Pact PWS Conference, 2017
- Overview of Diets explored in the TREND Community PWS Diet Initiative (also includes ‘The White Paper’ review, 2016, and diet resource sheets by TREND and The Charlie Foundation.)
- Prader-Willi Syndrome: The Behavioral Challenge – A Brief Summary for Professionals (FOOD SECURITY) – Drs Gourash and Forster, Pittsburgh Partnership, 2016
- Food Security The TRAIN Model – VIDEO of Dr J Forster presenting at the 3rd Asia-Pacific PWS Conference, 2015
- Food Security Checklists – Janice L. Forster, MD & Linda M. Gourash, MD, Pittsburgh Partnership
- What’s Wrong with Food Rewards in PWS? – Drs Gourash and Forster, Pittsburgh Partnership, 2015
- Food is Never OK in the Classroom – from The Gathered View (ISSN 1077-9965), PWSA(USA), 2006
- Increase Success in the Workplace: Limit Access to Food – by Lisa Graziano, Prader-Willi California Foundation
- Beyond The Diet – For the Dietitian – by Janalee Heinemann, from The Gathered View (ISSN 1077-9965), PWSA(USA), 2005
- Meals and Snack Ideas for PWS – by Melanie Silverman MS, RD, IBCLC.
- PWS Recipe Exchange – A Facebook group for parents and carers to share tips and recipes.