January 17, 2004 British Medical Journal editorial 2004;328:118-119

The issues faced are more parlous than for HIV, against which the blood supply is protected by HIV testing and surgical instruments by autoclaving.4-6 As yet we have no blood test for vCJD and no cure, and surgical instruments used on patients with vCJD have to be destroyed. People who have received blood or blood products that are highly at risk for vCJD will now need to be managed as if vCJD had been diagnosed. This means surgical instruments (including dental) used on these patients cannot be reused. They would also be ineligible for occupations that entail procedures that may expose others to risk of vCJD.4 7

These recipients will also have concerns about the unquantified potential for possible maternal or sexual transmission, if any, of vCJD, as will the children or consorts of patients who have developed vCJD.7 The risk of vCJD transmission may vary considerably by blood, surgical instruments, maternally, occupationally, or sexually and according to age group,2 incubation period, route of exposure, inoculum, and genetics of donor or recipient. All those who have received at vCJD risk blood or blood products, or are caught up in the "surgical web" spun out from them, or anyone who has suffered percutaneous injury involving blood at risk must refrain from blood or tissue donation and may have to accept restrictions on what they can do.

The responsibilities of recipients include to avoid transmission to others of vCJD and to contribute key data7 so that uncertainty for them and others about the size of risks is resolved as quickly as possible. But we also need to take steps to protect their rights and the rights of the public (box 1).

Box 1: Steps to protect the rights of recipients and of the public

* Develop a national protocol for vCJD counselling
* Develop a national standard for categorising vCJD risk
* Define actions to minimise vCJD transmission and say which of them are mandatory-these would depend on the category of the individual's risk4
* Clarify criminal justice liabilities for reckless and culpable transmission of vCJD6
* Make a national provision of the insurance and mortgage terms for which individuals would have been eligible had they not been caught up with at vCJD risk blood or blood products
* Provide information about clinical or public health research studies for which they are eligible or to which it is their responsibility to contribute in the national public health interest
* Provide meaningful redress against intrusion by the press
* Express national gratitude that they take up such exceptional burdens to protect others from a condition that they may not even have but may be at risk of having

To prevent further human to human transmission, the steps that need to be taken for every patient who is suspected of having any type of CJD are listed in box 2.

This case has implications for the national prospective collection of tonsils to be tested anonymously for detectable PrPSC, which is to begin in 2004.8 9 We do not know for certain that a child whose tonsillar tissue tests positive for abnormal PrPSC will develop vCJD. However, the unknown child is at risk of vCJD, and the transmission of vCJD through blood or blood products may now mean that any test positive specimen will have to be identifiable, so that the person can be managed in accordance with rights and responsibilities that fall to recipients of blood that is at risk of vCJD Would parents permit testing of tonsillar tissue if it were not to remain anonymous? Can the United Kingdom's chief medical officers and ministers of health risk the responsibility of allowing the destruction of operative tissue, when testing that material8 9 could otherwise prevent possible transfusion, surgical, and dental risks to other citizens? Similar testing could be done in postmortems.

Box 2: Steps to prevent further human to human transmission

* Every patient suspected of having any form of CJD needs to be immediately notified to the national CJD surveillance unit and cross checked against the list of identified recipients of blood potentially contaminated with vCJD-to check for human to human transmission
* Blood transfusion services must stop immediately the use of any blood or tissue that a patient suspected of having CJD may have donated-to safeguard blood supply
* Recipients of potentially vCJD contaminated blood transfusion or products should be alerted immediately without waiting for a definitive diagnosis to be made-to prevent surgical and other transmission
* We need to identify recipients of potentially vCJD contaminated blood who have subsequently undergone surgery-to check the extended network of those at risk of vCJD
* Counsel individuals in the risk network to alert them about the categorisation of their own risk, the mandatory actions they need to take to reduce risk to others, and the data requested from them for better calibration of risk

Until more is known about bloodborne vCJD transmission we must hope for the best and protect against the worst. Thus we need to collate key data about other recipients of blood and blood products derived from the vCJD donors, which are necessary to calibrate risk (see box on bmj.com). The network of those at risk extends further-to immediate family7 and, if recipients undergo surgery, possibly to patients on whom their instrument set was next used. We also need to seek agreement from recipients in life for various investigations to be performed after death, especially on their tonsils and brains, so that key data are not lost. Many are likely to be caught up in this web spun out from the recipients of at vCJD risk blood and blood products. Public, press, and professions must have much respect for individuals who have been unwillingly (and so far unknowingly) caught up in this web, the danger of which is now real but poorly quantified. These individuals shoulder immense responsibility for the good of us all.

Sheila M Bird, senior statistician

MRC Biostatistics Unit, Cambridge CB2 2SR