Earlier this year, a number of media outlets reported that a North Carolina hospital was attempting to contact 18 neurosurgical patients who might have been exposed to Creutzfeldt-Jakob Disease (CJD). The potential source was a patient who had symptoms of CJD and had a neurosurgical procedure.


Standard surgical instrument disinfection techniques are ineffective against prions (the agent that transmits CJD). Guidelines for infection control involve the sequestering, incinerating, or high-intensity sterilizing of neurosurgical instruments if transmissible CJD is suspected. Apparently none of these extra precautions were employed for instruments subsequently used in the 18 neurosurgical patients. The hospital said it was attempting to notify these patients.


First, a little discussion about CJD, since almost none of those reading this post have ever seen a case.


It’s rare. Estimates of risk range from 1 in 100,000 to 1 in 1 million. The incubation period for surgical exposure ranges from 6 months to 20 years. Iatrogenic transmission is estimated to account for under 1% of CJD cases. There are no good tests for exposure. There is no treatment. Once symptoms appear, patients die within a year.


The two most common sources of iatrogenic transmission of CJD over the decades were (a) human growth hormone; and (b) cadaveric dura for patches. Years ago, human growth hormone was concentrated from cadaver pituitaries. Today, it’s safely engineered genetically via recombinant techniques. And cadaveric dura is quite resistant to destruction by 0.1 N sodium hydroxide which can neutralize prions. This allows dura to be treated and effectively sterilized before implantation. Further, there are many substitutes that can be used in place of cadaveric dura. So, human growth hormone and cadaveric dura do not pose the risks for CJD they once did.


Back to surgical instruments. Why not just sterilize all neurosurgical instruments using enhanced techniques? The likely response is: (a) it’s expensive; (b) the enhanced techniques probably increase the risk that fragile instruments will wear down or break sooner; and (c) it’s not entirely clear the risk is completely eliminated.


Now the sticky wicket of disclosure. It’s difficult to know whether any exposed patient will even develop CJD until decades later. And fear and concern associated with disclosure might create an even “greater harm” given the low likelihood of transmission and absence of diagnostic methods and treatments.


Strong arguments can be made for disclosure. A patient has a right to be informed they were exposed to a deadly disease – even if it takes decades to manifest. To such patients, the perceived harm may be increased if the patient later learns the hospital paternalistically withheld that information. Also, diagnostic testing and treatment might very well become available in the future. Knowledgeable patients might be able to take advantage of such options.


On the other hand, what if YOU were the exposed patient? Wouldn’t you want to be in the driver’s seat? Wouldn’t you want to control whether you knew about the exposure, given the low risk of transmission. Or would you want the information shoved down your throat? (perhaps that’s a bit harsh.) Every patient is different. Some would want to know. Some wouldn’t.


The genetic diagnostic company, 23andme.com, offers a battery of tests to inform consumers about their risk for various diseases / conditions. The list includes whether you carry the genes for blue eyes, lactose intolerance, and baldness. (Of course, sometimes it’s just easier to look in the mirror to get these answers). The panel also includes tests for homozygous apo e4 alleles which confers significant risk for late onset Alzheimer’s disease. While most of 23andme’s results are displayed on a dashboard, the apo e4 results require an additional consent process. Those results are locked unless and until you click on a consent button to unlock the answer. 23andme believes, appropriately, that not all patients will want to know the answer, and it should be an informed, deliberate decision.


Issues related to disclosure pop all in all types of human interaction dilemmas. A husband (or wife) might have had an affair many years ago. On the deathbed, the “cheating” spouse may be tempted to clear his or her conscience by disclosing the infidelity. What’s the right thing to do? Perhaps it makes sense to address the hypothetical well before the actual event, so that the living spouse isn’t burdened by an unwanted disclosure. You have two parties here, and each has interests and concerns about disclosure.


The VA system has tackled this difficult subject. It released a document which included “MATRIX TO AID IN ADVERSE EVENT DISCLOSURE DECISIONS FOR LARGE SCALE EVENTS”. For events that are clinically severe, but low probability, the policy states there is no requirement to disclose “however disclosure or notification may be warranted on the basis of ethical, clinical, or other considerations.” The VA policy directive notes that low probability means less than one patient in 10,000 patients subject to the event or exposure are expected to have any short-term or long-term health effect that would require any treatment or cause serious illness if untreated. It appears exposure to instruments used in CJD case at VA facility would be considered a low probability event. There would be no mandatory disclosure as a matter of pre-defined policy.

So, some patients will want to know. Some won’t. What if patients locked in their preference in advance of any potential exposure? Imagine a hospital questionnaire which asked in its standard surgical consent form:

“In rare events, a patient might be exposed to surgical instruments which were inadequately sterilized, which can cause a debilitating disease ending in death decades down the road. The risk of developing this disease even if exposed is 1/100,000 to 1/1 million. There are no tests of treatment for such conditions. If exposed, would YOU want to know?”

Now, add to that question.

“For those who choose to be informed about such exposure, the hospital will pay you $250,000 to compensate for the increased worry. With that in mind, if exposed, would YOU want to know?