Quantifying Health Status and Function in Marfan Syndrome

J Surg Orthop Adv. 2016 Spring;25(1):34-40.

Abstract

Two hundred thirty patients were prospectively enrolled in this study and completed various portions of the Short Form 36 and a study-specific questionnaire (visual analog scale 1 to 10, comprising three separate questionnaires) to evaluate quality of life and function in patients with Marfan syndrome. The greatest health concern was cardiac problems (high in 70% of patients), followed by spine issues and generalized fatigue (both high, in 53%). The most severe reported pain involved the back: 105 patients (46%) rated pain as 6 to 10 on the visual analog scale. Among the 72 patients who responded to work life questions, work hours were reduced because of treatment in 59 (82%) or directly because of Marfan syndrome in 29 (40%). Across all Short Form 36 domains, patients scored significantly lower than United States population norms (p<.05); physical health scores were considerably lower than mental health scores.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Cohort Studies
  • Employment
  • Fatigue / etiology
  • Fatigue / physiopathology*
  • Fatigue / psychology
  • Female
  • Health Status*
  • Humans
  • Male
  • Marfan Syndrome / complications
  • Marfan Syndrome / physiopathology*
  • Marfan Syndrome / psychology
  • Mental Health*
  • Middle Aged
  • Pain / etiology
  • Pain / physiopathology*
  • Pain / psychology
  • Prospective Studies
  • Quality of Life*
  • Surveys and Questionnaires
  • Work*
  • Young Adult