Abstract
BackgroundThe costs associated with the treatment of sickle cell disease (SCD) are understudied in low and middle-income countries (LMIC). We evaluated the cost of treating SCD-related acute complications and the potential cost-savings of hydroxyurea in a specialized hematology center in Brazil.MethodsThe costs (US dollars) of emergency department (ED) and hospitalizations from SCD-related complications between 01.01.2018 and 06.30.2018 were ascertained using absorption and micro-costing approaches. The reasons for acute hospital visits were grouped as: 1) vaso-occlusive (VOC) pain, 2) infection, 3) anemia exacerbation, and 4) chronic organ damage complications. Hydroxyurea adherence was estimated by medication possession ratio (MPR) during the study period.ResultsIn total, 1144 patients, median age 17 years (range 0–70), 903 (78.9%) with HbSS/HbSβ0-thalassemia, 441 (38.5%) prescribed hydroxyurea, visited the ED, of whom 381 (33%) were admitted. VOC accounted for 64% of all ED visits and 60% of all admissions. Anemia exacerbation was the most expensive reason for ED visit ($321.87/visit), while chronic organ damage carried the highest admission cost ($2176.40/visit). Compared with other genotypes, individuals with HbSS/HbSβ0-thalassemia were admitted more often (79% versus 21%, p < 0.0001), and their admission costs were higher ($1677.18 versus $1224.47/visit, p = 0.0001). Antibiotics and analgesics accounted for 43% and 42% of the total ED costs, respectively, while housing accounted for 46% of the total admission costs. Costs of ED visits not resulting in admissions were lower among HbSS/HbSβ0-thalassemia individuals with hydroxyurea MPR ≥65% compared with visits by patients with MPR <65% ($98.16/visit versus $182.46/visit, p = 0.0007). No difference in admission costs were observed relative to hydroxyurea use.DiscussionIn a LMIC hematology-specialized center, VOCs accounted for most acute visits from patients with SCD, but costs were highest due to anemia exacerbation. Analgesics, antibiotics, and housing drove most expenses. Hydroxyurea may reduce ED costs among individuals with HbSS/HbSβ0-thalassemia but is dependent on adherence level.
Highlights
Sickle cell disease (SCD) is a pleiotropic genetic disorder of the hemoglobin with a chronic course of progressive end-organ dysfunction superimposed by recurrentLobo et al BMC Health Services Research (2022) 22:42 episodes of acute vaso-occlusion (VOC) leading to frequent acute care resource utilization [i.e., visits to the emergency department (ED) and hospitalizations]
Due to the loss of splenic function early in life, individuals with sickle cell disease (SCD) are at high risk of infections from encapsulated organisms, prompting additional acute hospital visits in the event of fever, which is considered an emergency in SCD [1]
All patients admitted to the inpatient setting at HEMORIO must be seen and treated in the ED first, all hospital admissions are a subset of the ED visit encounters
Summary
Sickle cell disease (SCD) is a pleiotropic genetic disorder of the hemoglobin with a chronic course of progressive end-organ dysfunction superimposed by recurrent. In high-income countries (HIC), greater than 95% of children with SCD survive to age 18 years [2]. In low-and middle- income countries (LMIC), such as Brazil, improvements in survival have been observed [3, 4]. SCD has become a chronic medical disease in Brazil, characterized by a pattern of recurrent episodes of acute events and progressive organ dysfunction as seen in HIC, [6] collectively leading to increased acute care utilization and financial burden on the health care system [7]. The costs associated with the treatment of sickle cell disease (SCD) are understudied in low and middle-income countries (LMIC).
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