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Dietary Management in PWS

Hyperphagia is just the medical term for excessive appetite and overeating.
Failure to Thrive is common during the first stage of the syndrome. Feeding problems are common: newborns often have very poor suck; they may fail to awake for feeding sessions and when woken may appear listless and disinterested. Often this leads to inadequate intake of energy and nutrients and results in poor weight gain. Sometime after the age of one year this failure to thrive gives way to the second stage of PWS - a seemingly insatiable, indiscriminate appetite.
Although the two extremes of the feeding continuum - failure to thrive (Phase 1) and hyperphagia (Phase 2) - are exhibited in PWS, both can be managed effectively.

Most babies have an inability to suck - tube feeding is necessary. Bottle feeding can be assisted by special bottles eg Haberman, Mead Johnson soft, or Rosti; a different teat can help eg Haberman, NUK or Lamb's teats. Some infants progress better with the Ameda cup, or Avent or Mag-Mag mugs with soft spouts. Solids can be introduced from around four months of age, following normal good weaning practices. Sucking, chewing and swallowing are hard work for the infant with PWS; reduced saliva secretion is a contributory factor to delayed chewing. Signs of poor feeding are subtle; care is needed to ensure an adequate energy intake for growth.

Throughout the second phase, the diet (for those with PWS) can follow general principles of "Healthy Eating" with a controlled energy intake (calories). Weight control is difficult, but not impossible. An abnormal body composition - a high fat to lean mass ratio - is the principle reason for a lowered energy requirement. Normal children grow on 11 - 14 kcal/cm height; PWS children need only 1011 kcal/cm ht. For weight loss, around 8 - 10 kcal/cm (occasionally 7kcal/cm) suffices. Vitamin and mineral requirements are for other age-equivalent children and adults.

An adequate calcium intake alone will not prevent osteoporosis, but may contribute to prevention role. Milk (or milk products) are excellent sources of calcium; semi-skimmed milk can be used from the age of two.

When using Artificial Sweeteners, Acceptable Daily Limits should not be exceeded.

While acknowledging some with PWS can control their food intake, many have obsessions around food or food related topics, and/or food-seeking or food-stealing tendencies. Preventative measures are preferable (e.g. limit access to money, shops); environmental controls (e.g. locks on doors, fridges, freezers) can help.

Children can be taught:

  • about "better" foods
  • which (because they are energy dense) are "best avoided"
  • what a normal serving is
and
  • acceptable behaviour eg eating slowly, putting their knife and fork down between each mouthful.
Regular exercise is essential for many reasons and should be part of the normal daily routine. A further essential routine is regular weighing. Weight gain can be extremely rapid, weight loss not so. Realistic targets should be set for weight loss (0.5 - 1.0 kg per week). If using BMI in adults, 30 should be regarded as an acceptable level.

Two PWSA(UK) publications with more information are:

  1. Weight Control and Suggestions to Assist with Weight Management
  2. Prader-Willi Syndrome: Food and Health

 

© Margaret Gellatly SRD)