Perioperative Management of Hemophilia Patients

Abstract

Purpose of ReviewHemophilia is a rare, typically inherited, condition where a specific clotting factor is reduced or even near absent. Patients with hemophilia who present for an invasive procedure, whether elective or urgent, are at increased risk of bleeding intraoperatively and postoperatively.Recent FindingsTen years ago, most patients with hemophilia with moderate or severe disease were treated with standard half-life factor replacement therapy, either prophylactic or on-demand. Now, patients may present on extended half-life factor therapy, or on a non-factor hemostatic therapy, or as a recipient of gene therapy. Further complicating the challenge of caring for these patients is that most of these new modalities will typically require supplementation with traditional factor therapy when surgery is required.SummaryAn in-depth understanding of all the potential treatment options for hemophilia is essential when anesthesiologists care for a patient with hemophilia. And there are several perioperative arenas (neuraxial procedures, point-of-care coagulation tests such as ROTEM or TEG, and cardiopulmonary bypass) where hemophilia creates unique considerations.