Mad Cows and Prions: Legal, Ethical and Operational Challenges in Responding to Creutzfeldt-Jakob Disease (CJD) and Variant CJD
The University of Sydney Law School
DISPUTES & DILEMMAS IN HEALTH LAW, Ian Freckelton & Kerry Petersen, eds., Federation Press, 2006
Sydney Law School Research Paper No. 07/13
This paper provides an introduction to some of the legal, ethical and operational challenges associated with public health responses to Creutzfeldt-Jakob disease (CJD) and variant CJD. Variant CJD is the human equivalent of Bovine Spongiform Encephalopathy (or BSE, popularly known as mad cow disease). vCJD was first identified in 1996, initially in the United Kingdom, in people who had eaten BSE-infected beef and beef products. CJD and variant CJD belong to a class of diseases known as transmissible spongiform encephalopathies (TSEs) that affect both humans and animals. TSEs are rare, fatal neurodegenerative diseases that cause rapidly progressive dementia. TSEs are known as prion diseases because they are caused by a unique disease agent: a rogue form of a naturally occurring cellular protein: the prion protein.
For several reasons, TSEs (including both CJD and vCJD) pose unusually complex challenges for public health authorities. TSEs are contagious: prion infectivity affects a range of central nervous system tissue and (in the case of vCJD) also lymphoreticular tissue. There is also evidence that variant CJD is transmissible through blood. A minority of TSEs are hereditary, raising the difficult psychosocial issues commonly associated with other fatal, late-onset, neurodegenerative diseases. For the most part, however, there is currently no effective screening test to determine the presence of pre-clinical infection. This creates risks of secondary transmission, particularly during certain forms of surgery and (for vCJD) through blood donation. These risks are further complicated by the fact that prion is uniquely resistant to deactivation by conventional sterilisation methods: subsequent use of surgical instruments that came in contact with infected tissues can expose downstream patients to a risk of infection. This creates difficult questions about disclosure of risk and look-backs. While the period of presymptomatic infection can span several decades, TSEs are invariably fatal and there are no proven treatments or vaccines.
This paper reviews the risks posed by, and some institutional responses to, both CJD and vCJD in Australia. It explores the policy challenges raised by efforts to secure the safety of blood and blood products in the face of vCJD risk, particularly in Australia and the United Kingdom. It explores issues raised by look-backs and patient notifications carried out in response to exposure to classical CJD in healthcare organisations, taking one incident that occurred at a Melbourne hospital as a case study. Finally, it identifies some of the key issues for consideration if a screening test for vCJD becomes available. The risks of TSE transmission are prompting significant investment in the development of prion reduction filters and screening tests, and may also lead to the development of a standard universal precautions approach to certain kinds of surgery. In the meantime, the paper advocates a precautionary, yet cross-disciplinary approach to assessing and responding to TSE risks.
Number of Pages in PDF File: 35
Keywords: Creutzfeldt-Jakob disease, CJD, transmissible spongiform encephalopathies (TSEs), mad cow disease, public health, communicable diseases, risk, screening, blood, surgery, hospitals, infection, patients, disclosure, look-backs
JEL Classification: I18, K3
Date posted: April 22, 2006