Background: Amyotrophic lateral sclerosis (ALS) is a multisystemic neurodegenerative disease, clinically presenting with motor and non-motor symptoms. Patient symptomatology contributes to greater difficulties in performing activities of daily living (ADLs) and need for caregiver assistance as disease progresses.
Objective: Assess the impact of ALS symptoms on the ability to perform ADLs independent of a caregiver and describe the impact of low independence on other patient outcomes.
Approach: Data were drawn from the Adelphi ALS Disease Specific Programme™, a point-in-time survey of neurologists and their ALS patients in the USA. Data were collected from July–November 2020. Neurologists recorded patient demographics, symptoms, hospitalizations, ability to perform ADLs, emotional well-being, and number of patient-reported falls. Logistic regression was used to assess the association between number of ALS symptoms and the likelihood of ADL-dependence, defined as patients being “completely care-dependent” in ≥1 ADL. Odds ratios (OR) were calculated for the number of motor/bulbar, cognitive, behavioral, and respiratory symptoms. Comparisons of outcomes between ADL-dependence groups were made using Fisher’s exact and t-tests.
Results: Twenty-one neurologists reported data for 166 ALS patients of whom 54% were ADL-dependent for ≥1 ADL. A significant association was observed between the number of respiratory symptoms and likelihood of ADL-dependence (OR 3.41; 95% confidence interval 2.17–5.36; P<.001). ADL-dependent vs ADL-independent patients experienced a greater number of hospitalizations and falls in the last 12 months. Additionally, a larger proportion of ADL-dependent patients had poorer emotional well-being (all P<.001) and had expressed suicidal thoughts (P=.045) vs ADL-independent patients.
Conclusions: Our findings suggest that compared with other symptoms, respiratory symptoms had a greater impact on patients’ ability to independently perform ADLs, and ADL-dependent patients had more hospitalizations, falls, and poorer emotional health. Treatments that delay or minimize respiratory symptoms could allow ALS patients to be independent for longer.