Which dietary modifications are used in the treatment of Prader-Willi syndrome (PWS)?

Updated: Oct 10, 2018
  • Author: Ann Scheimann, MD, MBA; Chief Editor: Luis O Rohena, MD, FAAP, FACMG  more...
  • Print
Answer

Patients with Prader-Willi syndrome have hyperphagia (onset in children aged 1-6 y) and diminished basal metabolic rate. Various treatment modalities for weight control, ranging from behavioral modification to anorexic agents, have been largely unsuccessful in curbing hyperphagia. However, these modalities may yield some success when used at group home settings. [7, 57]

  • Significant dietary restrictions are not implemented during early childhood to ensure optimal myelination.

  • Institution of a balanced hypocaloric diet (1000 calories with supplementation of vitamins and calcium) is generally implemented at early school age with careful monitoring by a dietitian.

  • As children with Prader-Willi syndrome become ambulatory, limitation of access to foods is essential for modulation of weight. Placement of locks on cupboards and refrigerators, use of smaller dishes, and restriction of access to food in the school environment help deter excessive weight gain.

  • In patients with morbid obesity, a protein-sparing modified fast with careful medical and nutritional supervision over several weeks may facilitate short-term weight loss. [58]

  • Based on the feeding behaviors of patients with Prader-Willi syndrome, one study assessed laboratory changes, metabolic changes, and growth changes and identified a total of 7 different nutritional phases, with 5 main phases and subphases in phases 1 (poor feeding and failure to thrive in infancy) and 2 (hyperphagia leading to obesity in later childhood). The study shows that progression of the nutritional phases is much more complex than the previously recognized 2 nutritional stages; awareness of these phases may help develop therapies and prevent or delay the early onset of obesity. [59]


Did this answer your question?
Additional feedback? (Optional)
Thank you for your feedback!