Background:
ROHHAD (rapid-onset obesity with hypothalamic dysregulation , hypoventilation , and autonomic dysregulation) is a rare, life threatening syndrome affecting approximately seventy-five people worldwide, two of which are patients being treated at The Children’s Hospital at Westmead (CHW). Patients with ROHHAD are challenging to manage as the obesity does not respond to standard dietary restriction and weight management advice.
Methods:
A Protein Sparing Modified Fast (PSMF) diet has been used at SickKids in Toronto, Canada and shown to be effective. The diet consisted of 2-2.5g/kg of protein per day calculated as protein equivalent exchanges. Carbohydrate intake was limited to 20-30g carbohydrate per day from vegetable sources. Vitamin and mineral supplementation was included to ensure adequate intake. Fluid intake was set at 2-3L per day.
Results:
Both patients (aged 9 and 6) at CHW were placed on a PSMF diet, commencing on stage 2. After 6 months of adherence to the diet both patients had a reduction in weight of 10kg.This was the first-time weight loss had occurred since diagnosis. Parents reported an improvement in the child’s hyperphagia and an improvement in overall behaviour and concentration.
Both patients saw a rapid increase in their weight gain whenever the diet was relaxed and the carbohydrate content increased to over 50g per day. Bimonthly appointments, family wide changes and specifying appropriate food products available were shown to improve dietary adherence.
Conclusion:
PSMF diets were effective in two paediatric patients being treated at the CHW for ROHHAD. Reduced adherence to the diet resulted in weight gain indicating the need to stay on the diet long term in order for weight to be controlled. Regular appointments, support and guidance (recipe ideas and meal plans) are necessary to ensure adherence and dietary variety.