Abstract Details

Title State of the Health-related Quality of Life Evidence in Amyotrophic Lateral Sclerosis (ALS): A Systematic Literature Review

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) P1 - Poster Session 1 (12:00 PM-1:00 PM)

Poster/Presentation
Number 1-012

Objective Conduct a systematic literature review (SLR) to identify studies estimating the health-related quality of life (HRQoL) impact of ALS on patients, healthcare systems, and caregivers.

Background ALS is a rare neurological disease caused by the degeneration of motor neurons in the brain and spinal cord leading to progressive weakness and death over 2-5 years. Although potentially substantial, the HRQoL impact for people living with ALS (PALS) and caregivers has yet to be comprehensively analyzed.

Design/Methods Articles were searched in MEDLINE, EMBASE, EconLit, and the National Health Service Economic Evaluation Database through April 2019. HRQoL studies were grouped according to three objectives: changes over patient lifetime, changes by disease severity, and impact on caregivers.

Results The HRQoL review identified 203 publications, of which 57 studies addressed the research objective. Not all identified studies found a decline in HRQoL over time due to ALS. When reported, a decline was influenced by functional and behavioral changes instead of cognitive changes. In studies not reporting a relationship between PALS HRQoL and time, there may have been a response shift where prioritization of areas contributing to PALS HRQoL changed over time. Trends demonstrated a decrease in HRQoL as disease severity increases with greater HRQoL impacts in physical over emotional domains. Studies showed that caregivers reported poorer HRQoL than the general population. Increased physical and behavioral impairments in PALS were associated with a greater impact on caregiver HRQoL. Social support was identified as a potential protective factor of caregiver burden.

Conclusions The findings of this SLR tend to demonstrate the substantial impact of ALS on both PALS and caregivers. The results highlight that HRQoL is a multifaceted concept where patient care and caregiver support needs are highly individual. The impact of ALS is substantial to both patients and caregivers; however, new therapies have the potential to provide significant HRQoL benefits.

Authors/Disclosures
PRESENTER	No disclosure on file
	No disclosure on file
Stephen Apple	Stephen Apple has received personal compensation for serving as an employee of Mitsubishi Tanabe Pharma America, Inc.
Terry D. Heiman-Patterson, MD (Temple University Lewis Katz School of Medicine)	Dr. Heiman-Patterson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for MTPA. Dr. Heiman-Patterson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Amylyx. Dr. Heiman-Patterson has received personal compensation in the range of $0-$499 for serving as a Consultant for novartis. Dr. Heiman-Patterson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for biogen. The institution of Dr. Heiman-Patterson has received research support from MTPA. The institution of Dr. Heiman-Patterson has received research support from State of Pennsylvania . The institution of Dr. Heiman-Patterson has received research support from ALS Association. The institution of Dr. Heiman-Patterson has received research support from ALS United. The institution of Dr. Heiman-Patterson has received research support from ALS Hope Foundation.
Tomas H. Holmlund, MD, FAAN (Dent Neurologic Institute)	No disclosure on file
	No disclosure on file
Lauren M. Webb, MD	Dr. Webb has nothing to disclose.
	No disclosure on file