Health-related quality of life across disease stages in patients with amyotrophic lateral sclerosis: results from a real-world survey

Background Amyotrophic lateral sclerosis (ALS) is characterized by a rapid disease course, with disease severity being associated with declining health-related quality of life (HRQoL) in persons living with ALS (pALS).

The main objective of this study was to assess the impact of disease progression on HRQoL across King’s, Milano-Torino Staging (MiToS), and physician-judgement clinical staging.

Additionally, we evaluated the impact of the disease on the HRQoL of care partners (cALS).

Methods Data were sourced from the Adelphi ALS Disease Specific Programme (DSP)™, a cross-sectional survey of neurologists, pALS and cALS presenting in a real-world clinical setting between July 2020 and March 2021 in Europe and the United States.

Results Neurologists ( n  = 142) provided data for 880 pALS.

There were significant negative correlations between all three clinical staging systems and EuroQol (European Quality of Life) Five Dimension Five Level Scale (EQ-5D-5L) utility scores and visual analogue scale (VAS) ratings.

Although not all differences were significant, 5-item Amyotrophic Lateral Sclerosis Assessment Questionnaire (ALSAQ-5) scores showed a stepwise increase in HRQoL impairment at each stage of the disease regardless of the staging system.

At later stages, high levels of fatigue and substantial activity impairment were reported.

As pALS disease states progressed, cALS also experienced a decline in HRQoL and increased burden.

Conclusions Across outcomes, pALS and cALS generally reported worse outcomes at later stages of the disease, highlighting an unmet need in this population for strategies to maximise QoL despite disease progression.

Recognition and treatment of symptoms such as pain and fatigue may lead to improved outcomes for pALS and cALS.