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2020 Updated Guidelines on the use of prophylactic factor replacement for haemophilia
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People with haemophilia A or B may be given coagulation factor replacement either in response to a bleed or regularly to prevent bleeding.

In the UK, prophylactic factor replacement is being started at an increasingly young age and some adults, who did not have prophylaxis as a child, now begin prophylaxis later in life to preserve musculoskeletal function.1
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The recent guideline compiled by the writing group of UKHCDO addresses the optimum use of prophylaxis in children and adults with haemophilia A and B and provides evidence-based recommendations where appropriate.1

UKHCDO, United Kingdom Haemophilia Centre Doctors’ Organisation
Key recommendations include:*1
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the importance of prophylaxis in maintaining good joint health
Whilst the primary goal of prophylaxis is to prevent bleeding, the ISTH have defined their aims of prophylaxis in relation to joint health.
Prophylaxis should be offered to any person with haemophilia (PWH) who has sustained one or more spontaneous joint bleeds.
Prophylaxis should be offered to a PWH who has established joint damage due to haemarthroses who experiences ongoing bleeding.
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extending prophylaxis to moderate patients
Primary prophylaxis should be considered for all children with baseline factor levels of 1–3 iu/dl.
In a person with severe haemophilia, or moderate haemophilia with a baseline level of 1-3 IU/dl, primary prophylaxis should be started before or immediately after the first joint bleed.
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individualisation of prophylaxis to be tailored to patient’s lifestyle requirements
The prophylaxis regimen should not be based on target peak and trough levels but should be tailored to prevent bleeding for an individual within his usual daily activity schedule. A trough of >1 iu/dl or even >3 iu/dl may be required in many cases to achieve this.
The prophylaxis regimen should be individualised, determined jointly with the patient and based on PK data, patient activity and patient preferences.
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treatment choices should involve the patient
The choice of factor replacement product must involve shared decisions making with the person with haemophilia and/or their patent/legal guardian.
Counselling should be provided before changing treatment and consideration given to individual lifestyles, particularly with regard to high impact activity.
*Please note this is an abstract of the guidelines. For full details of the guidelines please refer to Rayment R, Chalmers E, Forsyth K, et al. Guidelines on the use of prophylactic factor replacement for children and adults with Haemophilia A and B. doi:10.1111/bjh.16704. First published 10 May 2020
you can also find out more about the guidelines by speaking to a Sobi representative:
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Sobi: improving the lives of patients with haemophilia
At Sobi we are commited to bringing innovative therapies and services to improve the lives of patients with haemophilia
To find out more about our haemophilia products visit:
www.sobi-uk.co.uk
Reference
1. Rayment R et al. Guidelines on the use of prophylactic factor replacement for children and adults with Haemophilia A and B. BJH. https://doi.org/10.1111/bjh.16704 [Published online May 2020]
Job code: PP-8462
Date of preparation: July 2020
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