Anticipatory guidance for parents of Prader-Willi children

Pediatr Nurs. 2003 Nov-Dec;29(6):427-30, 451.


Prader-Willi syndrome (PWS) is a complex genetic disorder. It is characterized by hypotonia, short stature, hypogonadism, mental retardation, behavioral problems, and hyperphagia, which result in excessive obesity (Lindgren et al., 2000). The abnormal body composition resembles children seen with growth hormone deficiency (Carrel & Allen, 2001) . The dysmorphic features characteristic of PWS include a narrow forehead, a broad nasal bridge, slightly up slanting almond-shaped palpebral fissures, a down turned mouth with a thin upper lip, and narrow hands and feet (Martin et al., 1998). Management of children with PWS requires an ongoing multidisciplinary approach. The delivery of care includes assistance from geneticists, nutritionists, internists, endocrinologists, physical therapists, and psychologists to meet the medical, developmental, behavioral, and social needs. The focus of the nurse practitioner should include assisting the family in the management of these complex patients throughout their childhood.

Publication types

  • Review

MeSH terms

  • Adaptation, Psychological
  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Diet
  • Disease Progression
  • Growth Hormone / therapeutic use
  • Health Education*
  • Humans
  • Infant
  • Infant, Newborn
  • Parents*
  • Prader-Willi Syndrome / diagnosis
  • Prader-Willi Syndrome / nursing*
  • Prader-Willi Syndrome / therapy


  • Growth Hormone