Self-reported quality of life in amyotrophic lateral sclerosis

Objectives: To continuously assess overall quality of life (QOL) and disease progression in patients with amyotrophic lateral sclerosis (ALS) at different stages of the disease and compare the results between these two variables. Design/subjects: Twenty-six patients with ALS were interviewed with a questionnaire to assess their QOL from 0 to 10, where 10 is the highest QOL and questions concerning physical function, psychological status, and civil status. Their disease progression was estimated by ALS Functioning Rating Scale (ALS FRS). Nine patients were interviewed only once and 17 patients were interviewed 2-7 times. The interviews were repeated every second visit (range, 4-7 months). All values were ranked and linear regression was used to calculate the slope of QOL and ALS FRS. Results: The mean QOL value for all 26 patients was 5.8 (0-10-point scale). For the 17 patients interviewed 2-7 times, which correspond to a follow-up period of 5-28 months, there was no significant change in QOL-value (p = 0.247) among the interviews despite a significant disease progression (p = 0.0001). Conclusion: It can be concluded that ALS does not necessarily result in a low overall QOL and that despite disease progression overall QOL changes only slightly over time.