(378) Use of Health Services by People Living with Alexander's Disease

Background: : Alexander disease (AxD) is a rare genetic leukodystrophy affecting people of all ages. The prevalence is suspected to be 1 in one million people in the United States. The economic burden of the condition on the health care system has not be described. We provide that information here using a recently implemented ICD-10 code.

Objective: : Our objective in this study was to characterize the economic burden of medical care, in terms of dollars expended and frequency of health service utilization for people living with AxD.

Methods: : A retrospective cohort study was conducted using a nationally representative claims data set from Komodo Health. AxD patients were identified by the presence of at least two insurance claims with an ICD-10-CM code G31.86 at least 30 days apart between January 01, 2023, and October 31, 2024. Paid charges from 2023 were adjusted to 2024 using the Consumer Price Index for Medical Care and 2024 charges were extrapolated to 12 months. Health care utilization is described for inpatient, emergency room visits, and outpatient services. We also provide some demographic description. We report charges for 2023 and 2024. Utilization is reported only for 2023 due to completeness of data.

Results: : 55 patients living with AxD were identified in 2023 and 76 in 2024. Drawing demographic and clinical factors from 2024, the average age was 28; 45% were < 18, and 20% were >=55. 58% had claims indicating seizures, 58% respiratory conditions, 38% movement conditions, and 30% scoliosis.

Mean paid charges for all services were $107,261 in 2023 and $63,483 in 2024. In 2023, 43.6% of people with AxD were hospitalized for at least one day and 66% made at least one visit to the ER during the year. Of those admitted for inpatient services, 1/3 were admitted from the ER. Of those with an inpatient visit, 48% included a stay in the ICU. The mean length of stay in the hospital was 11 days, the median was 5 days. Home health services were used by 56.2% of people with AxD, and nearly 100% used outpatient and pharmacy services.

Conclusions: : This is the first report of health care utilization for people living with Alexander's Disease. While rare, people living with AxD are heavy users of health services, particularly inpatient, ICU and ER care. At an annual mean of $63,000 to $107,000, the cost represents a burden of care higher than other severe chronic diseases. However, even this represents a fraction of the total burden of AxD to families and society.

Sponsorship:: Ionis Pharmaceuticals