Presentation (CTI)

OBJECTIVES: Claims database analysis assessed the complexity of patient cases, disease burden, and health care resource utilization (HCRU) for US BTHS patients.
METHODS: Claims containing the ICD-10 diagnostic code for BTHS (E78.71) were identified using Healthcare Cost and Utilization Project data from the National (Nationwide) Inpatient Sample (NIS) (2020-2022 data [all ages]) and the Kids’ Inpatient Database (KID) (2019 and 2022 data [<21 years of age]). The inventory of primary and secondary diagnosis codes where E78.71 was present was assessed. Primary diagnosis codes stratified by age range (0-4, 5-12, and 13-20 years, inclusive) were assessed from the KID 2019 and 2022 data. Medicare Severity Diagnosis-Related Groups (MS-DRGs) were analyzed for highest volume MS-DRGs for BTHS in KID 2022 data.
RESULTS: Databases revealed 68 claims for BTHS, with >300 diagnostic codes reported. Frequently reported secondary diagnoses associated with claims for BTHS included dilated cardiomyopathy, dehydration, and neutropenia. For the 0-4 years age group, the most common primary diagnosis codes were single live-born infant, delivered vaginally, BTHS, dehydration, and cyclic neutropenia. The 5-12 year age group included influenza virus, neutropenia, and sepsis. Age 13-20 age group included soft tissue disorders, acute upper respiratory infection, and BTHS. Dehydration and neutropenia were identified in each age group. MS-DRGs 809 (major hematological and immunological diagnoses) and 641 (miscellaneous disorders of nutrition, metabolism, fluids and electrolytes) were the highest volume MS-DRGs for BTHS. Dilated cardiomyopathy and dehydration were common. Dilated cardiomyopathy average charge was $15,838-$52,249 and dehydration was $19,266-$42,249 for MS-DRGs 809 and 641, respectively.
CONCLUSIONS: This analysis demonstrated that patients with diagnostic codes linked to BTHS are highly complex with frequent comorbidities. This high-degree of complex BTHS can become significant, necessitating a high level of HCRU for various diagnosis codes. Elamipretide recently received FDA accelerated approval for BTHS, which may help to alleviate disease-related HCRU.