Health-related quality of life and participation of adult patients with spinal muscular atrophy and patients with amyotrophic lateral disorder

Abstract

The rehabilitation medical management of patients with SMA and ALS focuses on maintaining participation and quality of life (QOL). The objective of this dissertation was to gain a better understanding of these important themes in order to increase the quality of rehabilitation counseling for this group. Patients with SMA who are relatively mildly affected and those who experience feelings of anxiety and depression are at risk for a lower QOL. Compared with patients with other diagnoses (e.g., spinal cord injury), SMA patients appeared to be equally satisfied with their participation. Lower motor skills, feelings of depression, and fatigue were found to be associated with participation in daily life. Studies among ALS patients showed that Acceptance and Perceived benefits were related to QOL, ten months after diagnosis. Increased Feeling of Helplessness was independently related to lower QOL at study start and increased Feeling of Helplessness measured at study start was an independent predictor of lower with follow-up. Lung capacity, functional mobility, fatigue, and feelings of helplessness were independently associated with participation limitations. Patients with relatively mild physical symptoms also experienced participation limitations (sexuality, social activities in their home environment) and loss of autonomy in important activities. Prioritizing a patient's participation in social and meaningful activities is one of the hallmarks of person-centered care and improves quality of life.