Overcoming Hurdles in Hemophilia Care: The Impact of World Federation of Hemophilia Humanitarian Aid on Arthroplasties and Orthopedic Surgeries in Low-Middle-Income Setting

Abstract

Introduction: Hemophilia is a hereditary bleeding disorder characterized by the absence or deficiency of clotting factors, leading to prolonged bleeding and joint damage. In lower-middle-income countries (LMICs) like India, access to standard care for hemophilia has been limited, resulting in many children not receiving prophylaxis and developing severe joint disease in young adulthood. This significantly impacts their quality of life and productivity. Arthroplasty has offered hope for hemophiliacs with established joint disease, but its accessibility in LMICs faces numerous hurdles, including lack of laboratory diagnostics and monitoring facilities, shortage of hematologists, absence of advanced orthopedic surgical teams, limited insurance coverage, and inadequate access to clotting factor concentrates (CFCs) to support surgeries. Comprehensive care centers can address some challenges, but the availability of CFCs remains crucial for effective corrective arthroplasties and other orthopedic surgeries. Aim: This retrospective analysis aims to assess the impact of the World Federation of Hemophilia (WFH) humanitarian aid in facilitating effective corrective arthroplasties and other orthopedic surgeries at a comprehensive care center in a lower-middle-income country. Methods: The study was conducted at a comprehensive care center in India that previously lacked access to CFCs until the initiation of WFH humanitarian aid. Clinical data of patients with hemophilia who underwent major orthopedic surgeries and received CFC support from WFH humanitarian aid between November 2017 and June 2023 were reviewed. Patients with inhibitor-positive status were excluded. Patients were assessed in a comprehensive clinic prior to the elective arthroplasty. The analysis evaluated the utilization of CFCs in orthopedic surgeries, immediate surgical outcomes, and the effect of regulating CFC dose guided by lab monitoring. Additionally, the study audited the impact on CFC utilization when performing two surgeries in one sitting. Data analysis was performed using SPSS version 20.Continuous variables are depicted as mean±SD or median with range. Percentages represent categorical variables. Results: The study included 21 patients with Hemophilia {19 Hemophilia A(90%) and 2 Hemophilia B(10%)} who underwent orthopedic surgeries with CFC support from WFH humanitarian aid.There were 18(85%) patients with severe Hemophilia.Elective arthroplasty was done in 17 patients (81%) and 4 patients(19%) underwent emergency internal fixation. The median age of patients who underwent surgery was 31(range 18-75) years. Elective unilateral knee arthroplasty was done in 7(33%)patients, hip arthroplasty in 2(10%)patients and elbow arthroplasty in 1(5%)patient.Bilateral knee arthroplasty was done in a single surgical setting for efficient CFC utilization in 7 (33%) patients. Extended Half-life (EHL)CFC was used in 10 patients (48%), Standard half-life (SHL) CFC in 2 patients (10%), while the others received a combination of both. Mean use of CFC was 410(Range 270-767) units/kg in Hemophilia A and 650(range 500-800)units/kg in Hemophilia B. The mean EHL FVIII utilization in Bilateral TKR was 400 (Range 322-536) units/kg. None of the patients experienced major intraoperative bleeding or post-operative thromboembolism.No patient developed inhibitor positivity when tested post operatively. Notably, CFC utilization in all patients was found to be less than the empirically recommended levels in WFH guidelines for resource-constrained settings. Table 1 demonstrates the demographics, Type of surgery, CFCs used and immediate outcomes of orthopaedic surgeries done using WFH Humanitarian Aid. Conclusion: The findings of this retrospective analysis demonstrate the transformative impact of WFH humanitarian aid in a comprehensive care center for hemophiliacs in a lower-middle-income country. With access to CFCs, effective corrective arthroplasties and orthopedic surgeries were made possible, providing hope and improved quality of life for patients with established joint disease. The study underscores the vital role of WFH humanitarian aid in enhancing care outcomes for hemophiliacs in resource-limited settings and emphasizes the need for continued support and collaboration to advance hemophilia care globally.