OBJECTIVES : ALS is a debilitating disease in which approximately 80% of patients die within 5 years of diagnosis. In 2019, approximately 17,000 individuals were diagnosed with ALS in the US. We sought to comprehensively review relevant evidence regarding QOL in ALS patients in order to further understand the humanistic burden and to encourage future research.

METHODS : A systematic literature review (SLR) was conducted following Cochrane Collaboration’s guidelines for systematic reviews for quality of life studies published between 2009-2019 in the following databases: Embase, Ovid Medline and Cochrane. Relevant congresses were also searched.

RESULTS : A total of 2,800 records were identified and underwent subsequent screening. After applying exclusion criteria, 101 records were selected. The most frequently reported ALS symptoms included fatigue (up to 90%), pain (up to 78%) and depression (up to 68%) with fatigue as the most prevalent, bothersome and undertreated. Other symptoms included progressive muscle weakness, loss of voluntary movement, difficulty breathing, speaking and swallowing. The average delay between symptom onset and diagnosis is 13 months. ALS patients had significantly worse QOL compared to the general population as demonstrated with the SF-36, largely driven by decrements in both physical and mental function. Moreover, approximately half of ALS caregivers experience high levels of burden (48% reporting a ZBI score ≥17). Over the course of the disease, patients experience functional and QOL deterioration with limited treatment options. Utility captured with EQ-5D and standard gamble methods was separated into four ALS health states across studies (primarily derived from King’s staging), mild, moderate, severe and terminal. Utilities ranged from 0.79-0.65, 0.67-0.53, 0.71-0.35, and 0.50-0.12, respectively, demonstrating severe health decline.

CONCLUSIONS : Published evidence demonstrates a need for novel treatment strategies to alleviate a heavy humanistic burden as ALS is associated with numerous symptoms, significant reductions in QOL and increases in caregiver burden.