Weight management | food security | Diet | Nutrition | Hyperphagia
The services of a dietician should be sought early. Due to low muscle tone at birth, infants with Prader-Willi Syndrome require assistance with feeding and ensuring they are receiving adequate nutrition.
Due to their ‘failure to thrive’, most infants with Prader-Willi Syndrome will leave hospital with a nasal-gastric tube. A speech Therapist and Dietician should oversee the nasal-gastric tube weaning process. Following on from assistance with nasal-gastric tube weaning a dietician will continue to work on weight management and adequate nutrition with diet.
The International Prader-Willi Syndrome Organisation (IPSOW)provides information on their website to help us understand the hunger drive in people with PWS. Their website states the following:
“To understand the importance of this hunger drive, try to look at Prader-Willi Syndrome as a ‘starvation’ syndrome rather than an over-eating one. Because of the dysfunction in the hypothalamus, there is no on/off mechanism that tells the brain, “I’ve eaten enough”. What happens instead is that the brain keeps telling the stomach, “you’re starving, you need food”, and the drive to find food overrides everything else”
Nutrition Therapy or consultation with a dietitian can assist you in developing and adjusting a feeding plan for your child. A dietitian experienced in Prader-Willi Syndrome can help you ensure your child is receiving the vitamins and nutrients needed for optimal development, while also considering your child’s specific energy (kJ) needs. Assistance from a dietician will continue well beyond infancy.
The diet of someone with Prader-Willi Syndrome can be very different to the general population who are trying to maintain their weight.
As we are learning more and more everyday about Prader-Willi Syndrome, It is important that your dietician has an understanding of the complexities of PWS and is abreast of all the latest research in their respective field of expertise, as foods that were once thought to beneficial in a PWS diet may not be today.
Further information with regards to Optimal Nutrition for People with PWS can be found on the Foundation for Prader-Willi Research website.
A person with Prader-Willi Syndrome will need to have a well-balanced, low-calorie diet and maintain regular exercise for the rest of their life. Height, weight, and BMI should be monitored every 6 months during the first 10 years of life after infancy and regular monitoring of weight for the rest of the person’s life to make sure he or she is maintaining a healthy weight.