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UKHCDO. UKHCDO annual report 2019 including bleeding disorder statistics for 2018/2019. http://www.ukhcdo.org/wp-content/uploads/2019/11/UKHCDO-Annual-Report-2019.pdf (accessed May 2022)
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An update for general dental practitioners on a new medication for the management of patients with haemophilia A

From Volume 49, Issue 6, June 2022 | Pages 477-480

Authors

Stephanie Fenesan

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

Articles by Stephanie Fenesan

Email Stephanie Fenesan

Bella Madan

Consultant Haematologist, Centre for Haemostasis and Thrombosis, St Thomas' Hospital, Guy's and St Thomas' NHS Foundation Trust

Articles by Bella Madan

Najla Nizarali

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

Articles by Najla Nizarali

Abstract

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.

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