Disparities in Spinal Muscular Atrophy-Related Mortality in the United States, 2018-2023

Area of Science:

• Neurology
• Epidemiology
• Public Health

Background:

• Previous studies indicate increased mortality in individuals with Spinal Muscular Atrophy (SMA).
• Limited data exists on demographic and geographic disparities in SMA-related mortality.
• This study investigates SMA mortality trends in the United States during the post-treatment era (2018-2023).

Purpose of the Study:

• To examine disparities in Spinal Muscular Atrophy (SMA)-related mortality.
• To identify specific demographic groups and regions with higher SMA mortality rates.
• To inform strategies aimed at mitigating the impact of SMA.

Main Methods:

• Population-based study utilizing the CDC WONDER database.
• International Classification of Disease (ICD) codes (G12.0, G12.1, G12.8, G12.9) identified SMA cases.
• Data stratified by sex, race/ethnicity, and Census regions; age-adjusted mortality rates (AAMR) and rate ratios (RR) calculated.

Main Results:

• 821 SMA-related deaths occurred; males exhibited higher AAMR than females (RR=1.189).
• Non-Hispanic White individuals had the highest SMA-AAMR, followed by Non-Hispanic Black and Asian individuals.
• The West region showed the highest AAMR; infants (<1 year) had the highest AAMR across all age groups.

Conclusions:

• SMA-related mortality is highest among infants, Non-Hispanic White individuals, males, and in the West region.
• These findings highlight significant demographic and geographic disparities in SMA mortality.
• Data can guide future public health initiatives to reduce the burden of Spinal Muscular Atrophy.