The quandary of Creutzfeldt-Jakob disease

• Published in: Canadian Medical Association journal (CMAJ) 1998-10, Vol.159 (7), p.789-792
• Main Author: Larke, B
• Format: Article
• Language: English
• Subjects: Bioethics; Blood Component Transfusion - adverse effects; Blood donation & transfusion; Brain Diseases; Canada; Creutzfeld-Jakob disease; Creutzfeldt-Jakob Syndrome - transmission; Disclosure; Disease Notification; Duty to Recontact; Health Policy; Humans; Physician patient relationships; Risk Assessment; Risk Factors; Surveys and Questionnaires; Truth Disclosure; Uncertainty
• ISSN: 0820-3946; 1488-2329

Second, there is no direct evidence of CJD transmission through blood transfusion. Readers of CMAJ have been well-served by several excellent articles on the transmissible spongiform encephalopathies, which include CJD, "mad cow disease" in Europe and its recently recognized human counterpart, new variant CJD (nvCJD).(2,5-7) There is conclusive evidence that many infectious agents -- bacteria, protozoa and viruses -- can be transmitted from human to human through transfusion of cellular blood components, plasma and fractionated blood products.(f.8) However, reviews of the world's scientific literature have provided no direct evidence that CJD or nvCJD, caused by prions, has been transmitted through the blood supply. The risk, at present, is appropriately regarded as theoretical, although debate continues.(f.1,7.9-14) There is no question that the emergence of nvCJD in the United Kingdom, first reported in 1996, has turned up the heat on this debate. The infectious agent associated with nvCJD has crossed species barriers, has been recovered from human tonsillar tissue and has tropism for B lymphocytes. But nvCJD has not been detected in Canada or the United States, and the priori associated with CJD does not have the more ominous biological characteristics of nvCJD. The experience of these 2 Toronto hospitals, as well as other presentations at the conference, left those in attendance with the impression that most patients favoured being notified about possible exposure to CJD. But what about the other recipients, approximately 20% of those questioned? Among patients or their parents who have been included in CJD look-back programs, opinion is sharply divided on the merits of being notified, as Barbara Sibbald describes on page 829 of this issue. For some, it has been a very negative experience and, in the absence of evidence of transmission of CJD through blood, is seen as an injustice. However, even for the recipients who are most insistent on and supportive of patient notification, there may be a harmful outcome. Sibbald relates the consternation of one Calgary physician, who was invited to a meeting of the Canadian CJD Society, a local support group that includes a number of recipients identified in the 1995 recall of blood products. According to her article, some members of the society seriously believe that several of the recipients are already showing preliminary clinical manifestations of CJD. These people almost certainly do not have early CJD but r