A Qualitative Approach to Health Related Quality-of-Life in Congenital Muscular Dystrophy

J Neuromuscul Dis. 2018;5(2):251-255. doi: 10.3233/JND-170252.

Abstract

Congenital muscular dystrophies (CMD) cause progressive muscle weakness resulting in severe motor disabilities. Previous studies focused on the effects of motor disability. Here, we explore other factors affecting health related quality-of-life (HRQOL) in CMD. Qualitative interviews were conducted with participant-parent dyads to identify symptoms having the greatest impact on HRQOL. Symptoms were classified into themes and domains representing physical, mental, social health, and disease specific issues. Social role limitations and specific activity impairment were frequently mentioned. A greater understanding of symptoms impacting HRQOL will provide a framework for improved clinical care and patient centered outcomes as new therapies are developed.

Keywords: Muscular dystrophy; bethlem myopathy; quality of life; ullrich congenital muscular dystrophy.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Contracture / physiopathology*
  • Contracture / psychology
  • Female
  • Health Status*
  • Humans
  • Male
  • Mental Health*
  • Middle Aged
  • Muscular Dystrophies / congenital*
  • Muscular Dystrophies / physiopathology
  • Muscular Dystrophies / psychology
  • Parents
  • Qualitative Research
  • Quality of Life*
  • Role
  • Sclerosis / physiopathology*
  • Sclerosis / psychology
  • Social Participation*
  • Young Adult

Supplementary concepts

  • Bethlem myopathy
  • Muscular dystrophy congenital, merosin negative
  • Scleroatonic muscular dystrophy