title: Multidisciplinary management of patients with haemophilia with inhibitors undergoing surgery in the United States: perspectives and best practices derived from experienced treatment centres.
abstract: Since the 1980s, major surgical interventions in patients with congenital haemophilia with inhibitors have been performed utilizing bypassing agents for haemostatic coverage. While reports have focused on perioperative management and haemostasis, the US currently lacks consensus guidelines for the management of patients with inhibitors during the surgical procedure, and pre- and postoperatively. Many haemophilia treatment centres (HTCs) have experience with surgery in haemophilia patients, including those with inhibitors, with approximately 50% of these HTCs having performed orthopaedic procedures. The aim of this study was to present currently considered best practices for multidisciplinary care of inhibitor patients undergoing surgery in US HTCs. Comprehensive haemophilia care in the US is provided by ~130 federally designated HTCs staffed by multidisciplinary teams of healthcare professionals. Best practices were derived from a meeting of experts from leading HTCs examining the full care spectrum for inhibitor patients ranging from identification of the need for surgery through postoperative rehabilitation. HTCs face challenges in the care of inhibitor patients requiring surgery due to the limited number of surgeons willing to operate on this complex population. US centres of excellence have developed their own best practices around an extended comprehensive care model that includes preoperative planning, perioperative haemostasis and postoperative rehabilitation. Best practices will benefit patients with inhibitors and allow improvement in the overall care of these patients when undergoing surgical procedures. In addition, opportunities for further education and outcomes assessment in the care of this patient population have been identified.
authors: Gulf States Hemophilia and Thrombophilia Center, Houston, TX 77030, USA. Miguel.escobar@uth.tmc.edu
authors: M
authors: M
authors: Escobar
authors: J
authors: J
authors: Maahs
authors: E
authors: E
authors: Hellman
authors: J
authors: J
authors: Donkin
authors: A
authors: A
authors: Forsyth
authors: N
authors: N
authors: Hroma
authors: G
authors: G
authors: Young
authors: L A
authors: LA
authors: Valentino
authors: R
authors: R
authors: Tachdjian
authors: D L
authors: DL
authors: Cooper
authors: A D
authors: AD
authors: Shapiro
mesh_terms: D000933:Antifibrinolytic Agents / Q000627:therapeutic use; D025861:Blood Coagulation Disorders, Inherited / Q000150:complications* / Q000534:rehabilitation; D019774:Blood Coagulation Factor Inhibitors / Q000097:blood*; D003191:Comprehensive Health Care; D015942:Factor VIIa / Q000627:therapeutic use; D006470:Hemorrhage / Q000517:prevention & control; D006801:Humans; D007592:Joint Diseases / Q000150:complications / Q000601:surgery*; D011182:Postoperative Care; D011300:Preoperative Care; D011785:Quality Assurance, Health Care; D011994:Recombinant Proteins / Q000627:therapeutic use; D014481:United States; D054556:Venous Thromboembolism / Q000209:etiology
chemical_list: D000933:Antifibrinolytic Agents; D019774:Blood Coagulation Factor Inhibitors; D011994:Recombinant Proteins; C103587:recombinant FVIIa; D015942:Factor VIIa