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Journal of Genetic Counseling

, Volume 16, Issue 6, pp 799–809 | Cite as

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
Original Research

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.

Keywords

MSUD Psychosocial Families Support 

Notes

Acknowledgements

This project was supported in part by funding from the MSUD Support Group. No personnel from the Support Group participated in the design, data analyses, or interpretation of the results of this study. The authors thank Julie Cederbaum, B.A. for granting us permission to adapt the Family Survey for MSUD families. We thank Carol Ohnstad Zlatunich, RD, CSP, Kara Weisiger, MS, and Cynthia Morgan, MS, for their contributions to this work. Most importantly, the authors thank all of the families who participated in this research.

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Copyright information

© National Society of Genetic Counselors, Inc. 2007

Authors and Affiliations

  • Wendy Packman
    • 1
  • Shelly L. Henderson
    • 1
  • Indira Mehta
    • 2
  • Rama Ronen
    • 1
  • Dean Danner
    • 3
  • Beth Chesterman
    • 4
  • Seymour Packman
    • 2
  1. 1.Pacific Graduate School of PsychologyPalo AltoUSA
  2. 2.University of CaliforniaSan FranciscoUSA
  3. 3.Emory University School of MedicineAtlantaUSA
  4. 4.California State UniversityEast BayUSA

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