Open discussion of TSE ethical problems

Question: Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive, fatal, neurodegenerative disorder caused by a transmissible protein called a prion. CJD has been transmitted through human pituitary growth hormone extracts, dura mater grafts, corneal transplants and deep brain EEG electrodes. The incubation period can be years or decades.

There are no documented cases of the transmission of CJD by blood. Highly transfused patient groups such as hemophiliacs have yet to experience a case of CJD. The risk of transmission of CJD by blood is thought to be theoretical, or close to zero. The American Red Cross (ARC) and the Food and Drug Administration have no policy on notifying recipients of CJD blood other than to suggest that blood banks consult their institutional review board or ethics committee.

A hospital blood bank director was notified by the ARC that two years ago at his hospital three patients were transfused blood products from a donor who subsequently died of CJD. He has the names of the recipients and must decide whether to inform them that they received blood from a CJD donor. Ordinarily, he would inform them if they were at risk, but in this case, as far as can be known, there is no risk. There is no diagnostic test and no treatment for CJD.

He feels that informing these patients and explaining the zero or minuscule risk will frighten them unnecessarily. How would you advise the blood bank director?

Response: The three patients should be told what has happened. There is no way of knowing that there is no risk. It is slightly misleading to say, "As far as can be known, there is no risk." It would be more precise to say, "It is not known for certain whether any risk exists." The fact that hemophiliacs have to date shown no increased incidence of CJD is reassuring but not at all conclusive.

The disease has been transmitted from exposure to several kinds of infected tissue and it is entirely plausible that infected blood could also be an agent of transmission. All that can be said to these patients is that their risk of contracting CJD, with the disease's often decades-long incubation period, may be minuscule but there is no way of knowing whether it is zero.

Suppose it turns out that there is a slight risk of contracting the disease through transfusion and that one or more of these three patients' contracts CJD in another 20 years. Suppose further that, the meantime, a drug has been discovered that can prevent or delay the disease's appearance if, and only if, it is given in the early years of the prion's incubation period.

Can you imagine saying in some years to a now fatally ill CJD patient, "I'm really sorry we didn't tell you what we knew about your situation, but we didn't want to worry you too much."

In addition to the possible prevention of harm to these three patients, there is another strong and important argument for informing them. Whenever one chooses a course of action in a morally problematic situation, one is in effect declaring that the same choice can justifiably be made in all morally equivalent situations.

If one chooses not to tell in this situation, one would generalize as follows: "I believe that in any situation when patients are at a very small risk of death, even though that death might be preventable, doctors may conceal that risk from the patients in order not to worry patients overmuch." The effect of patients knowing that doctors followed such a rule would be to undermine significantly the trust that patients have in what doctors tell them.

For at any given time, patients would be justified in thinking, "I wonder if there is some slight risk of death that my doctor is holding back from me because she doesn't want me to worry?"

The way in which a patient is told of a risk can be as morally significant as whether the risk is disclosed at all. It would be appropriate to tell these patients the facts of their situations in an appropriately minimizing fashion. One might start by saying, "There is some-thing I want to discuss with you about one of the transfusions you obtained several years ago.

There is just the slightest risk that you were exposed to a serious infection that could cause you trouble some day, although from what we know, there is next to no chance that will hap-pen. But our policy here is to be totally honest with patients about any risks and that's why I'm mentioning this." This might cause anxiety in the three patients, but an ethically adequate disclosure of risks always may have that effect.

And the anxiety would be balanced (though not ameliorated) by the patients' being reassured that their physicians were per-sons to be trusted not to conceal possibly significant knowledge out of paternalistic motives.