OBJECTIVES : Amyotrophic lateral sclerosis (ALS) is a debilitating neurological disease resulting in progressive loss of voluntary motor activity with 5yr mortality of 29%. As the disease progresses, it impacts patients’ activities of daily living and they require greater caregiver assistance. This study critically assesses existing evidence on medical and non-medical costs including caregiver burden over the course of the disease.

METHODS : Systematic literature review was conducted following Cochrane Collaboration’s guidelines for studies published between 2009-2019.

RESULTS : Of the 2,230 records screened, 37 publications were selected. ALS results in significant economic burden on patients and their caregivers. Lifetime total cost per patient over a 10-year period in US was estimated to be $1,433,992. The cost drivers were home care (47%), ventilation (15%), and hospitalization (8%). Medical costs also increased with disease progression; in UK mean costs tripled from King’s clinical stage 1 to stage 4.

As disease progresses, patients need more emotional and physical support from their caregivers. Zarit Burden Interview (ZBI) and Caregiver Burden Inventory are used to measure caregiver burden. About 50% of caregivers experienced high burden and psychological distress on ZBI. However, none of the questionnaires quantify time spent by the caregiver to assist patients. In US, the cost of paid caregiving represented 25% of the total annual non-medical costs ($18,000). Caregivers of patients requiring 16-24 hours of daily care earned $21,600 less annually than caregivers of patients requiring less substantial care.

CONCLUSIONS : Medical costs increase with ALS progression. However, economic impact of premature death and caregiver burden is not systematically captured by clinical stage of disease. Depending on severity of disease, family members may spend majority of the day providing care, resulting in substantial humanistic and economic burden of care. Therefore, a robust approach to measure economic impact of ALS on caregiver and to society as a whole is warranted.