title: Comprehensive care of the patient with haemophilia and inhibitors undergoing surgery: practical aspects.
abstract: Congenital haemophilia is a rare and complex condition for which dedicated specialized and comprehensive care has produced measurable improvements in clinical outcomes and advances in patient management. Among these advances is the ability to safely perform surgery in patients with inhibitor antibodies to factors VIII and IX, in whom all but the most necessary of surgeries were once avoided due to the risk for uncontrollable bleeding due to ineffectiveness of replacement therapy. Nevertheless, surgery continues to pose a major challenge in this relatively rare group of patients because of significantly higher costs than in patients without inhibitors, as well as a high risk for bleeding and other complications. Because of the concentration of expertise and experience, it is recommended that any surgery in patients with haemophilia and inhibitors be planned in conjunction with a haemophilia treatment centre (HTC) and performed in a hospital that incorporates a HTC. Coordinated, standard pre-, intra- and postoperative assessments and planning are intended to optimize surgical outcome and utilization of resources, including costly factor concentrates and other haemostatic agents, while minimizing the risk for bleeding and other adverse consequences both during and after surgery. This article will review the special considerations for patients with inhibitors as they prepare for and move through surgery and recovery, with an emphasis on the roles and responsibilities of individual members of the multidisciplinary team in facilitating this process.
authors.affiliation: Division of Pediatric and Adolescent Hematology/Oncology, Department of Pediatrics and Human Development, Michigan State University College of Human Medicine, East Lansing, MI 48824, USA. roshni.kulkarni@hc.msu.edu
authors.forename: R
authors.initials: R
authors.lastname: Kulkarni
mesh_terms: D019774:Blood Coagulation Factor Inhibitors / Q000097:blood; D003029:Coagulants / Q000037:antagonists & inhibitors / Q000627:therapeutic use; D003191:Comprehensive Health Care / Q000458:organization & administration*; D005164:Factor IX / Q000037:antagonists & inhibitors / Q000627:therapeutic use; D005169:Factor VIII / Q000037:antagonists & inhibitors / Q000627:therapeutic use; D006467:Hemophilia A / Q000628:therapy*; D002836:Hemophilia B / Q000628:therapy*; D006470:Hemorrhage / Q000517:prevention & control; D006801:Humans; D033183:Interdisciplinary Communication; D019990:Perioperative Care / Q000379:methods*; D013514:Surgical Procedures, Operative
chemical_list: D019774:Blood Coagulation Factor Inhibitors; D003029:Coagulants; D005169:Factor VIII; D005164:Factor IX