Without support and good management of diet and nutritional behaviours, PWS will lead to morbid obesity. This is due to the combination of the overriding desire to eat, coupled with low muscle tone. In order to manage the person with PWS’s weight, it is important to have a daily schedule for the person to follow. This would include daily activities or schoolwork, meal and snack times and exercise or leisure routines.
Establishing these behaviours from a young age including what your child eats, when they eat and how much they eat, will enable them to manage a healthy weight over time.
When establishing a plan of what and how much your child eats, it is advised to consider the following:
· low in fat,
· low in energy/kilojoules (kJ),
· tailored to the child’s needs to enable growth in height,
· healthy – to ensure nutrient requirements are met,
· sustainable in the long term,
· manageable for the whole family and reviewed regularly as the child grows.
It is important to ensure your child is eating the correct types and amounts of food and also to restrict your child’s access to food to ensure they are not consuming extra energy.
Work with your dietitian to determine your child’s allowances from each of the core food groups to allow for satisfactory growth. These allowances need to be reviewed and adjusted at least once a year as your child grows.
All family members can benefit from low fat cooking techniques. Other family members will need larger serving sizes and extra snacks, while other children in the family may receive food-related treats separately. However, it is important for siblings, friends and other family members to understand the rules and routines at home that are important to manage the energy intake of their sibling with PWS. This is all easier said than done, but very important for success with weight control.
Some low fat cooking tips include:
· choose lean cuts of meat and remove all visible fat, remove skin from chicken,
· avoid creamy sauces and high fat gravies,
· use low fat dairy products instead of full fat,
· limit takeaway foods,
· avoid rich cakes and pastries and use low fat products, try rice cakes and honey or jam without margarine,
· choose low fat cooking methods such as grilling, baking and steaming.
*this content is extracted with permission from ‘Need to know nutrition for children with Prader Willi Syndrome’ by Professor Peter SW Davies, The Children’s Nutrition Research Centre, The University of Queensland and Helen d’Emden, Mater Health Services, Co-author Brigid Knight, Mater Health Services.