Health-related quality of life, pain, and fatigue in myotonic dystrophy type 2: a 13-year follow-up study.

Abstract

To examine the long-term progression of health-related quality of life (HRQoL), pain, and fatigue in myotonic dystrophy type 2 (DM2) compared to adult-onset myotonic dystrophy type 1 (DM1). Data on HRQoL (Short Form 36 Health Survey (SF-36)), pain (McGill Pain Questionnaire), and fatigue (Checklist Individual Strength) were assessed in DM2 patients and age- and sex-matched DM1 patients, at baseline and after 13 years. Twenty-nine DM2 and 29 DM1 patients participated at baseline. Data of 18 DM2 and 16 DM1 patients were recollected at follow-up. Ten DM2 and 13 DM1 patients had passed away, and one DM2 patient did not consent participation. In DM2, mental health on SF-36 subscales (social functioning, mental health) decreased during follow-up without significant increase in pain or fatigue. In contrast, in DM1, both physical and mental health decreased, and pain and fatigue increased. HRQoL decreased over 13 years in DM2. Pain was present early in the DM2 disease course and did not increase significantly. Healthcare professionals should be aware of the long-term impact of myotonic dystrophy (DM) on pain, fatigue, physical and mental functioning, including social functioning. Symptomatic treatment of these aspects may decrease disease impact, because DM cannot be cured.