CLAIMS DATABASE ANALYSIS TO ASSESS THE COMPLEXITY AND SEVERITY OF PATIENT CASES, DISEASE BURDEN, AND HEALTH CARE RESOURCE UTILIZATION (HCRU) IN US BARTH SYNDROME PATIENTS
Author(s)
Mary Kay Koenig, MD1, Melissa Huang, PhD2.
1Professor & Associate Vice-Chair for Clinical Research, UTHealth Houston, Houston, TX, USA, 2Barth Syndrome Foundation, Edison, NJ, USA.
1Professor & Associate Vice-Chair for Clinical Research, UTHealth Houston, Houston, TX, USA, 2Barth Syndrome Foundation, Edison, NJ, USA.
Presentation Documents
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.
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.
Conference/Value in Health Info
2026-05, ISPOR 2026, Philadelphia, PA, USA
Value in Health, Volume 29, Issue S6
Code
EE278
Topic
Economic Evaluation
Topic Subcategory
Cost/Cost of Illness/Resource Use Studies
Disease
SDC: Cardiovascular Disorders (including MI, Stroke, Circulatory), SDC: Gastrointestinal Disorders, SDC: Musculoskeletal Disorders (Arthritis, Bone Disorders, Osteoporosis, Other Musculoskeletal), SDC: Neurological Disorders, SDC: Rare & Orphan Diseases