title: Clinical implications of emerging pathogens in haemophilia: the variant Creutzfeldt-Jakob disease experience.
abstract: The impact of variant Creutzfeldt-Jakob disease (vCJD) on the clinical practice of haemophilia in the UK is coloured by the haemophilia community's experience of hepatitis C virus and human immunodeficiency virus (HIV) transmission via plasma-derived therapies in the 1980s, when the delay in recognizing and acting on the potential risks cost many patients their lives and left others to manage another chronic disease. This crisis prompted organisations such as the United Kingdom Haemophilia Centre Doctors' Organisation to advocate for the introduction of haemophilia therapies that would not be susceptible to contamination with blood-borne pathogens. After the identification of vCJD in 1996, a number of public health measures were taken in response to a government-sponsored vCJD risk assessment, and following reports of transfusion-transmission of vCJD, additional guidelines have been developed to prevent person-to-person transmission, some of which may impact the quality and availability of medical and surgical care. Variant CJD has had a significant negative effect on the UK haemophilia community, shaking patient confidence in the therapies they have received over the last 21 years, affecting the quality of care and creating the risk of stigmatizing the community as it was in the 1980s. As with HIV and vCJD, emerging blood-borne infectious agents will likely affect blood and blood-derived therapies well before we become aware of its presence. As a result, only therapies with the lowest level of risk should be used for care of patients with haemophilia.
authors.affiliation: Department of Haematology, University Hospital, Queen's Medical Centre, Nottingham, UK. gerry.dolan@nottingham.ac.uk
authors.forename: G
authors.initials: G
authors.lastname: Dolan
mesh_terms: D000818:Animals; D016913:Blood Component Transfusion / Q000009:adverse effects; D017848:Blood-Borne Pathogens; D002417:Cattle; D021821:Communicable Diseases, Emerging / Q000635:transmission*; D007562:Creutzfeldt-Jakob Syndrome / Q000635:transmission*; D018562:Disease Transmission, Infectious; D006467:Hemophilia A / Q000150:complications* / Q000628:therapy*; D006801:Humans; D018570:Risk Assessment; D065227:Transfusion Reaction; D006113:United Kingdom; D015047:Zoonoses