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EDITORIALS: Kumanan Wilson and Maura N Ricketts A new human genotype prone to variant Creutzfeldt-Jakob disease BMJ 2006; 332: 1164-1165 [Full text]

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Danger – Linked industry and livelihoods to support.

Alfred P J Lake   (14 June 2006)

Danger – Linked industry and livelihoods to support. 14 June 2006
Alfred P J Lake, Consultant in Anaesthesia and Pain Management Glan Clwyd Hospital, LL18 5UJ. Send response to journal: Re: Danger – Linked industry and livelihoods to support.	I was surprised by the total absence of posted responses to either this editorial or the associated original research paper (1) when I first looked and still there are none. If it really is an important topic, are readers not interested, apathetic or perhaps beyond commenting as the juggernaut seems to roll on under its own momentum? The financial and social burden consequent upon Bovine Spongiform Encephalopathy (BSE) and New variant Creutzfeldt-Jakob disease (vCJD) , already huge, will continue to increase significantly if, as a society, we persist in basing our overall management on what may be a false premise and incur both direct and, perhaps more importantly, indirect extra costs. The false premise in question is that the condition vCJD is simply caused by a transmissable infectious agent to support which there was (2) and, I would contend, still remains, a very poor standard of evidence on the basis of which the precautionary actions being taken are perhaps, rather, excessive and out of proportion (3). Dire predictions for the future were, and continue to be, based upon this supposed risk to humans. A language of fear is used, all of it predicated upon there being a transmissable infectious agent; makes you worry just to read…..tramsmit, asymptomatic carriers, epidemic, iatrogenic spread, …new evidence ‘may rekindle fears of a larger epidemic....an ongoing threat. Designed to create a climate of acceptance, much like the ‘war on terror’. We should, in fact be very cautious in interpreting the results of the study which simply demonstrated 3 out of 12674 samples as containing prion protein; full stop! No need to suggest that the true prevalence should be higher or overinterpret the data or put the frighteners on with ‘secondary spread’ from surgery or blood transfusion (1) or that single cases should have major implications for future estimates of vCJD in the UK (4) 161 cases overall is not a major problem; why are the findings of the new study so worrisome? With so few cases, perhaps part of the problem, shocking as it may be, is that (bad for the industry) there in fact is no future for the vCJD ‘epidemic’ (5) and other possible causes are not considered. We do need to continue surveillance, investigate and diagnose both clinical and pre-clinical forms of the disease (4) but not while wearing blinkers. Prion proteins exist but we still need to find out why, exactly and quite specifically, in the 1980s BSE hit the UK. Something happened then which probably isn’t applicable now. Perhaps it’s simply down to a susceptibility or priming based upon genetic (and other) factors and animals (and humans) become AFFECTED rather than INFECTED, a certain ‘load’ of prion protein affecting susceptible patients at risk. Carcinogens are not (with a few exceptions) infectious agents but produce the disease in susceptible individuals after very variable rates or degrees of exposure. Society is becoming more risk averse, risk elimination rather than proper management the goal, and encouraged to be so by issues such as this and the health and safety industry in general. We are being made fearful of each other and living. The need remains to challenge the current orthodoxy and overcome the vested interest and status quo surrounding vCJD. Risk aversion is the illness and hyper-caution the epidemic (6). 1. Ironside JW, Bishop MT, Connolly K, Hegazy D, Lowrie S, Le Grice M et al. Variant Creutzfeldt-Jakob disease: prion protein genotype analysis of positive appendix tissue samples from a retrospective prevalence study. BMJ 2006; 332: 1186-8. 2. Venters GA. New variant Creutzfeldt-Jakob disease: the epidemic that never was. BMJ 2001; 323: 858-861. 3. Wilson K, Ricketts MN. Transfusion transmission of vCJD: a crisis avoided? Lancet 2004; 364: 477-9. 4. Peden AH, Head MW, Ritchie DL, Bell JE, Ironside JW. Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient. Lancet 2004; 364: 527-9. 5. Aguzzi A, Glatzel M. vCJD tissue distribution and transmission by tranfusion – a worst-case scenario come true? Lancet 2004; 363: 411-2. 6. Jenkins S. Don’t panic – or our culture of caution will be the death of us. The Sunday Times June 11, 2006; 1:18. Competing interests: None declared