Anderson JA, Brewer A, Creagh D Guidance on the dental management of patients with haemophilia and congenital bleeding disorders. Br Dent J. 2013; 215:497-504 https://doi.org/10.1038/sj.bdj.2013.1097
Escobar MA, Brewer A, Caviglia H Recommendations on multidisciplinary management of elective surgery in people with haemophilia. Haemophilia. 2018; 24:693-702 https://doi.org/10.1111/hae.13549
Collins PW, Liesner R, Makris M Treatment of bleeding episodes in haemophilia A complicated by a factor VIII inhibitor in patients receiving emicizumab. Interim guidance from UKHCDO Inhibitor Working Party and Executive Committee. Haemophilia. 2018; 24:344-347 https://doi.org/10.1111/hae.13495
Knight T, Callaghan MU. The role of emicizumab, a bispecific factor IXa- and factor X-directed antibody, for the prevention of bleeding episodes in patients with hemophilia A. Ther Adv Hematol. 2018; 9:319-334 https://doi.org/10.1177/2040620718799997
Mahdi AJ, Obaji SG, Collins PW. Role of enhanced half-life factor VIII and IX in the treatment of haemophilia. Br J Haematol. 2015; 169:768-776 https://doi.org/10.1111/bjh.13360
Oldenburg J, Mahlangu JN, Kim B emicizumab prophylaxis in hemophilia A with inhibitors. N Engl J Med. 2017; 377:809-818 https://doi.org/10.1056/NEJMoa1703068
Muto A, Yoshihashi K, Takeda M Anti-factor IXa/X bispecific antibody ACE910 prevents joint bleeds in a long-term primate model of acquired hemophilia A. Blood. 2014; 124:3165-3171 https://doi.org/10.1182/blood-2014-07-585737
Kruse-Jarres R, Callaghan MU, Croteau SE Surgical experience in two multicenter, open-label phase 3 studies of emicizumab in persons with hemophilia A with inhibitors (HAVEN 1 and HAVEN 2). Blood. 2017; 130 https://doi.org/10.1182/blood.V130.Suppl_1.89.89
An update for general dental practitioners on a new medication for the management of patients with haemophilia A Stephanie Fenesan Bella Madan Najla Nizarali Dental Update 2024 49:6, 707-709.
Authors
StephanieFenesan
BDS, BSc(Hons), AKC, MSc, MSCD RCS Ed
Specialty Trainee in Special Care Dentistry, Department of Sedation and Special Care Dentistry, Guy's Dental Institute, Guys and St Thomas' NHS Foundation Trust
Specialist in Sedation and Special Care Dentistry, Department of Sedation and Special Care Dentistry, Floor 26 Tower Wing, Guy's Hospital, London Bridge, London SE1 9RT, UK
Emicizumab is a new medication used in the treatment of people with haemophilia A and there is little in the current literature providing guidance for dental treatment. It is important to risk assess dental procedures appropriately according to their bleeding risk and to liaise closely with the patient's haematology team. This article provides an overview of emicizumab, its mechanism of action and implications for dental treatment, as well as examples of surgical management plans provided by the haemophilia team.
CPD/Clinical Relevance: It is important to liaise closely with the patient's haemophilia team to ensure provision of safe dental treatment.
Article
Emicizumab (Hemlibra) is a relatively new prescription medication administered to people with haemophilia A and used for routine prophylaxis to prevent or reduce the frequency of bleeding episodes. Haemophilia is a congenital bleeding disorder in which there is a deficiency of clotting factors. Haemophilia A is a sex-linked recessive condition that results in a deficiency of clotting factor VIII and accounts for approximately 85% of haemophilia cases, affecting approximately 1:10,000, with over 8400 people registered with haemophilia A in the UK.1 People with haemophilia A can present on a spectrum of severity as mild, moderate or severe, depending on the level of clotting factor VIII present (Table 1).
Conventional treatment for haemophilia A depends on the severity of haemophilia and the factor VIII percentage present, and aims to increase the levels of factor VIII. This can be carried out prophylactically if known surgical intervention is planned, and adjusted according to the procedure required, or in response to bleeding caused by trauma. This could include the use of desmopressin (desamino-8-D-arginine vasopressin or DDVAP), which acts to release bound factor VIII and increase plasma levels. However, this is only suitable for patients with mild or moderate haemophilia A and not suitable for patients with severe haemophilia A because they will not have bound factor VIII available to be released.
Register now to continue reading
Thank you for visiting Dental Update and reading some of our resources. To read more, please register today. You’ll enjoy the following great benefits: