TY - JOUR
T1 - A creative approach to managing a case of haemophilia A with inhibitors in a low-resource country
T2 - case report
AU - Bhatia, Simran
AU - Archana, M. V.
AU - Bhat, Vasudeva
AU - Balthillya, Ganesh M.
AU - Badagabettu, Sulochana
N1 - Publisher Copyright:
© 2023 Simran Bhatia et al., published by Sciendo.
PY - 2023/1/1
Y1 - 2023/1/1
N2 - The development of antibodies to therapeutic factor VIII, known as inhibitors, is a leading cause of morbidity and mortality in people with haemophilia. This is particularly challenging in areas where access to novel treatment options is limited. This case report presents a clinical scenario in South Western India involving a 16-year-old male with severe haemophilia A with high titre inhibitors, who sustained a tibia-fibula shaft fracture necessitating emergency surgical intervention. The successful management of this patient required a multidisciplinary approach, encompassing haemostasis optimisation, innovative factor replacement strategies to work around financial constraints, and a comprehensive rehabilitation plan. The patient's history of multiple factor VIII concentrate transfusions contributed to inhibitor development. Limited funds for bypassing agents prompted the utilisation of fresh frozen plasma to achieve haemostasis before access to FEIBA and recombinant factor VII. Emicizumab, a subcutaneously administered bispecific antibody, was used to assist perioperative haemostasis. A comprehensive rehabilitation plan with regular physiotherapy was followed. Emicizumab prophylaxis was initiated and the patient now shows improvement. This case emphasises the importance of novel treatment options such as FEIBA and emicizumab in dealing with complications in haemophilia such as inhibitors. In resource-limited settings, there is still a need to improve the availability of these life-saving interventions to optimise surgical outcomes in such cases.
AB - The development of antibodies to therapeutic factor VIII, known as inhibitors, is a leading cause of morbidity and mortality in people with haemophilia. This is particularly challenging in areas where access to novel treatment options is limited. This case report presents a clinical scenario in South Western India involving a 16-year-old male with severe haemophilia A with high titre inhibitors, who sustained a tibia-fibula shaft fracture necessitating emergency surgical intervention. The successful management of this patient required a multidisciplinary approach, encompassing haemostasis optimisation, innovative factor replacement strategies to work around financial constraints, and a comprehensive rehabilitation plan. The patient's history of multiple factor VIII concentrate transfusions contributed to inhibitor development. Limited funds for bypassing agents prompted the utilisation of fresh frozen plasma to achieve haemostasis before access to FEIBA and recombinant factor VII. Emicizumab, a subcutaneously administered bispecific antibody, was used to assist perioperative haemostasis. A comprehensive rehabilitation plan with regular physiotherapy was followed. Emicizumab prophylaxis was initiated and the patient now shows improvement. This case emphasises the importance of novel treatment options such as FEIBA and emicizumab in dealing with complications in haemophilia such as inhibitors. In resource-limited settings, there is still a need to improve the availability of these life-saving interventions to optimise surgical outcomes in such cases.
UR - https://www.scopus.com/pages/publications/86000379526
UR - https://www.scopus.com/inward/citedby.url?scp=86000379526&partnerID=8YFLogxK
U2 - 10.2478/jhp-2023-0014
DO - 10.2478/jhp-2023-0014
M3 - Article
AN - SCOPUS:86000379526
SN - 2055-3390
VL - 10
SP - 82
EP - 89
JO - Journal of Haemophilia Practice
JF - Journal of Haemophilia Practice
IS - 1
ER -