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.

Prophylaxis of Venous Thromboembolism in Knee Replacement Surgery

“…The synthesis of recommendation for VTE prophylaxis in knee replacement has been provided by guidelines produced in the recent past years by international scientific societies [66][67]. The American college of Chest Physicians antithrombotic and prevention of thrombosis guidelines [69] suggests that in patients undergoing TKA the administration of LMWH, fondaparinux , apixaban , dabigatran , rivaroxaban , LDUH , adjusted dose warfarin of finally aspirin has grade 1 B recommendation, while intermittent compression devices (ICPD) has a grade 1c recommendation.…”

“…Peculiar conditions in which a VTE prophylaxis may be not indicated is the presence of inhibitors, antibodies against recombinant factors developed by some patients, able to limit the efficacy of clotting factors [66][67]. In such patients, the high risk of bleeding is more consistent than the risk of VTE.…”

Surgery in patients with inherited bleeding disorders

“…Until full adoption of 7 days a week working practice in the NHS, elective procedures should be scheduled to take place at the beginning (Monday or Tuesday) rather than at the end of the working week [2]. In most hospitals, this ensures full availability of staff in the various departments involved in the care of the patient.…”

“…The exact factor level required for haemostasis and the duration of clotting factor replacement after surgery is not known [2,3], but the following guidelines are generally accepted in the UK and other Western countries.…”

“…In the vast majority of cases, the designated haemophilia centre will plan and execute all factor administration, both pre-and postoperatively. It is highly recommended that all, but the most minor procedures, should be carried out in a Haemophilia Comprehensive Care Centre [2], with access to full coagulation laboratory facilities, specialist physiotherapy support, and outpatient specialist haemophilia nurse support. It is also important that surgeons, anaesthetists, physiotherapists and haematologists involved in patients' care are all experienced in managing such patients [3].…”