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      Cost analysis of acute care resource utilization among individuals with sickle cell disease in a middle-income country

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          Abstract

          Background

          The 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.

          Methods

          The 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.

          Results

          In total, 1144 patients, median age 17 years (range 0–70), 903 (78.9%) with HbSS/HbSβ 0-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/HbSβ 0-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/HbSβ 0-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.

          Discussion

          In 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/HbSβ 0-thalassemia but is dependent on adherence level.

          Supplementary Information

          The online version contains supplementary material available at 10.1186/s12913-021-07461-6.

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          Most cited references22

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          Unbiased Recursive Partitioning: A Conditional Inference Framework

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            Sickle cell disease

            Sickle cell disease (SCD) is a group of inherited disorders caused by mutations in HBB, which encodes haemoglobin subunit β. The incidence is estimated to be between 300,000 and 400,000 neonates globally each year, the majority in sub-Saharan Africa. Haemoglobin molecules that include mutant sickle β-globin subunits can polymerize; erythrocytes that contain mostly haemoglobin polymers assume a sickled form and are prone to haemolysis. Other pathophysiological mechanisms that contribute to the SCD phenotype are vaso-occlusion and activation of the immune system. SCD is characterized by a remarkable phenotypic complexity. Common acute complications are acute pain events, acute chest syndrome and stroke; chronic complications (including chronic kidney disease) can damage all organs. Hydroxycarbamide, blood transfusions and haematopoietic stem cell transplantation can reduce the severity of the disease. Early diagnosis is crucial to improve survival, and universal newborn screening programmes have been implemented in some countries but are challenging in low-income, high-burden settings.
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              Crizanlizumab for the Prevention of Pain Crises in Sickle Cell Disease.

              The up-regulation of P-selectin in endothelial cells and platelets contributes to the cell-cell interactions that are involved in the pathogenesis of vaso-occlusion and sickle cell-related pain crises. The safety and efficacy of crizanlizumab, an antibody against the adhesion molecule P-selectin, were evaluated in patients with sickle cell disease.
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                Author and article information

                Contributors
                jane.hankins@stjude.org
                Journal
                BMC Health Serv Res
                BMC Health Serv Res
                BMC Health Services Research
                BioMed Central (London )
                1472-6963
                8 January 2022
                8 January 2022
                2022
                : 22
                : 42
                Affiliations
                [1 ]GRID grid.488951.9, ISNI 0000 0004 0644 020X, Instituto Estadual de Hematologia do Rio de Janeiro HEMORIO, ; Rio de Janeiro, RJ Brazil
                [2 ]GRID grid.440558.8, ISNI 0000 0004 0552 4014, Fundação Centro Universitário Estadual da Zona Oeste UEZO, ; Rio de Janeiro, Brazil
                [3 ]GRID grid.240871.8, ISNI 0000 0001 0224 711X, Department of Global Pediatric Medicine, , St Jude Children’s Research Hospital, ; 262 Danny Thomas Place, TN 38105 Memphis, USA
                [4 ]GRID grid.240871.8, ISNI 0000 0001 0224 711X, Department of Hematology, , St. Jude Children’s Research Hospital, ; TN 38105 Memphis, USA
                Article
                7461
                10.1186/s12913-021-07461-6
                8742916
                34998394
                51470e31-72ac-45a3-a4b7-2d2733eb0005
                © The Author(s) 2022

                Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

                History
                : 12 August 2021
                : 28 December 2021
                Categories
                Research
                Custom metadata
                © The Author(s) 2022

                Health & Social care
                sickle cell anemia,pain,hospitalization,admission,financial analysis,emergency room,emergency department,micro-costing,acute complications,hydroxycarbamide,medication possession ratio

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