Psychosocial issues in families affected by maple syrup urine disease

Wendy Packman, Shelly L Henderson, Indira Mehta, Rama Ronen, Dean Danner, Beth Chesterman, Seymour Packman

Research output: Contribution to journalArticle

21 Scopus citations

Abstract

The primary aim of this study was to ascertain the psychosocial issues faced by families affected by maple syrup urine disease (MSUD). The psychosocial adjustment and quality of life of children with MSUD were also described. Participants included 55 families and their children (ages 5 to 18 years) and teachers. Measures included a MSUD Family Survey, the Behavior Assessment System for Children (BASC) and the Pediatric Quality of Life Inventory (PedsQL). Parents reported via the MSUD Family Survey that the greatest sources of stress were financial and emotional. Many parents reported difficulty interacting with the medical staff and with schools. On the BASC, half of the children fell within the average range in psychosocial adjustment, although there were elevations in scales measuring attention, hyperactivity, and learning problems. On the PedsQL, the mean quality of life scores were closer to children with cancer than to a healthy sample. Despite the emotional and financial burden, parents reported that MSUD has also had a positive influence on their lives, leading to a world-view that is more compassionate and caring.

Original languageEnglish (US)
Pages (from-to)799-809
Number of pages11
JournalJournal of Genetic Counseling
Volume16
Issue number6
DOIs
StatePublished - Dec 2007
Externally publishedYes

Keywords

  • Families
  • MSUD
  • Psychosocial
  • Support

ASJC Scopus subject areas

  • Genetics(clinical)

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