After a Creutzfeldt-Jakob exposure, should patients be told?

Recently we have learned that 15 patients in New England were exposed to a rare infection called Creutzfeldt-Jakob disease (CJD) when neurosurgical instruments contaminated with the infection were used in their care. Each had undergone a brain or spinal surgery in early 2013, and now their future was uncertain.

Creutzfeldt-Jakob disease is caused by an infectious agent called a prion — as is “mad cow disease.” Originally discovered in the brain-eating Fore cannibals of Papua New Guineaprions are misshapen proteins that are the vampires of the protein world. Prions cozy up to normal human proteins and convert them into misshapen proteins themselves. These new prion converts can then turn additional human proteins into misshapen proteins, and a chain reaction sets up. Eventually the accumulation of misshapen proteins crowds out the normal contents of brain cells, spongy deposits of protein litter the tissues of the brain, and victims of prion diseases develop what is called spongiform encephalopathy.

Since prions are resistant to standard sterilization techniques, neurosurgeons can transmit CJD from patient to patient. For instance, doctors might harvest tissue from the nervous system of an organ donor not recognized to have CJD and then the graft recipient will succumb to the infection years later. Early cases of CJD were diagnosed in recipients of human growth hormone derived from ground up pituitary glands. Others developed CJD from contaminated brain electrodes. In all of history, there have been only four reported cases of CJD in patients whose sole risk was to undergo a neurosurgical procedure with instruments contaminated with the infection. However, the discovery that surgical instruments contaminated with CJD still cause an uproar: at Emory Hospital in 2004, fully 98 patients were exposed to contaminated surgical instruments and the group is still being monitored for signs of disease.

Creutzfeldt-Jakob disease takes years to develop. Patients exposed to the infection feel fine for years but then they become moodier and forgetful and over the course of months subtle cognitive defects progress to severe dementia. Patients with CJD forget the names and faces of loved ones, they lose the ability to walk, speak, or swallow, and they lapse into a coma that has proven fatal one hundred percent of the time.

To avoid neurosurgical prion transmission, the instruments used in a patient suspected to have CJD should be destroyed or specially decontaminated. With such measures in practice, it has been thirty years since a case of CJD was attributed to contaminated surgical instruments. At Catholic Medical Center in Manchester, New Hampshire, though, the instruments used to biopsy the patient later diagnosed with CJD were sterilized via standard techniques and put back into use.

When Creutzfeldt-Jakob disease contamination of surgical instruments is discovered, doctors at Catholic Medical Center and other hospitals wonder if it is right to tell patients. In most cases the disease will never occur and there is no effective means of prevention. Even for the unlucky patients who do develop the disease — if any of them do — there is no effective therapy so advance warning gives no extra measure of hope. Worse still, there is no diagnostic test to predict who will escape infection or die, so the only thing patients can do once notified is wait. That means the only real world impact of disclosure, regardless of the eventual outcome, is patient distress. So in this case is knowledge power, or is knowledge just knowledge?

Beyond the desire not to worry patients unnecessarily, Catholic Medical Center doctors were likely tempted to hide the error in sterilization technique. Yet doctors value transparency and trusting relationships with patients, and most patients want to be notified if physicians make an error or discover an abnormal finding incidentally.

Like the 98 patients at Emory, eight patients at Catholic Medical Center in New Hampshire who were exposed to Creutzfeldt-Jakob disease were notified last week, and counseled that they will have to wait years to find out if they contracted the infection. At Cape Cod Hospital in Massachusetts and the West Haven Veterans Affairs Hospital in Connecticut, which rented the same neurosurgical instruments from Catholic Medical Center, seven additional patients will join in the wait. Fortunately, whether or not patient notification was a good idea, surveys show that patients do not suffer serious long-term distress after learning they were exposed to surgical instruments contaminated by prions. Few of us can maintain a state of high alert for very long in response to a risk that is both minuscule and abstract.

Dean Foster and colleagues at the University of Pennsylvania devised a free online calculator that estimates life expectancy based on demographics, habits and family history. Punch in the facts and out pops an estimate. Not surprisingly, smokers and drinkers are predicted to live shorter lives, whereas people in low stress jobs who get a lot of exercise live longer. Or at least that is what the calculator says.

But no online calculator can predict lightning, terrorists, and other random calamities. None asks, after assessing alcohol consumption and seat belt use, “Do you expect to be notified your neurosurgical procedure was contaminated by a rare prion disease?” That means that by their calculation, beyond eating our broccoli and squeezing in a run from time to time, we really cannot know the day of our demise.

The question then is whether there is something, anything, to be gained from knowing that somewhere deep in the brain a rampaging misshapen protein with an appetite for global domination is quietly, mercilessly, taking apart the very substance of who you are. Or not. Personally I would rather live without that kind of shadow hanging over me.

Timothy Lahey is an infectious disease physician and medical ethicist who blogs at [M U R M U R S].  This article originally appeared in The Atlantic.

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  • Ron Smith

    I had a family that had Huntington’s chorea and I remember when someone (in the larger family as I recall) was requesting testing on a child around age 15 (as I recall) who was my patient. It was not done.

    Testing is very uncommonly done on those individuals as a routine and the ethical stance is to not know either. In this case there is more specificity about the diagnosis, disease time frame, and the progression of the illness.

    I agree with Timothy on this. I can see how some knowledge borders on cruelty, and rather than empowering patients to live a quality life, it could cripple them instead.

    Ron Smith, MD
    www (adot) ronsmithmd (adot) com

    • Judgeforyourself37

      However, it is in the best interest of the parents to have the child tested. If he tests positive, they will be able to be prepared for what is to come, and later, they must share this information with their child when he is of age or before. If he tests negative, they can breath as sigh of relief, otherwise they will always wonder regarding a condition that they may need to understand

      • Ron Smith

        You might think that, but that is actually not the way most Huntington’s disease families see it. Having been through this with the child and carefully thinking through and understanding the logic of those doing genetic counseling, I do not recommend that children be told either. The adult who was asking about it was not one of the members of the family who actually had a chance of having the disease. You and I live our lives as though we are never going to die…we will, but it doesn’t pervade our daily waking thoughts. For individuals with Huntington’s in their families just knowing that they might have inherited it is enough. The firm knowledge that they are affected becomes cruel. We all know we can get killed driving but we still drive. If you knew what day it was that you were absolutely going to die in a car crash…then you would likely never drive at all. That’s a waste of life.

        Ron Smith, MD
        www (adot) ronsmithmd (adot) com

  • GT

    Why don’t you just show them the Australian SBS video on Kuru, and then ask them whether or not they should be “allowed” to find out whether your negligence has exposed them to this disease?

    I’m sure all of them will say, “Oh, no, no. Doctor knows best. If you think it’s not good for me to find out whether I might die a shuddering drooling mindless mess of an incontinent protoplasm because of your incompetence, I’ll go along with that.” And then you can both go have a cup of tea, whistling “Que Sera, Sera” in unison.

  • KJK

    Im not sure why there is a question about telling the patients. It is there right to know that they are now going to mentally degrade from this, It is there right to know what will happen to them and how. How terrifying to go in for a surgery that will save your life, and then leave with a disease that will slowly kill you from the inside out…

  • http://www.myheartsisters.org/ Carolyn Thomas

    This is a subject that, except for physicians educated during the ‘don’t worry your pretty little head’ days of medical paternalism, is hardly worthy of debate in 2013. Our bodies, our health, our future – not yours. Thus you don’t get to play God, and your personal opinion of revealing the truth about this preventable medical error to affected patients and their families is irrelevant.

    Here’s a surprising twist about nurses who discover cases of preventable medical error compared to docs who do. A 2004 Australian study on disclosure of preventable medical error found that nurses report incidents more habitually than doctors do “…due to a culture which provided directives, protocols and the notion of security, whereas the medical culture was less transparent and favoured dealing with incidents ‘in-house’.” Researchers also cautioned medical institutions to assume that media coverage of large-scale adverse medical events is inevitable, so all responses should “demonstrate the institution’s commitment to honesty and transparency to build public trust.”

    And for a patient to accidentally find out after the fact – which they most certainly WILL – because of physicians’ reluctance to “worry” affected patients is far more devastating a fate than being honest and upfront.

  • Jeri Burtchell

    I read this blog as a patient who is interested in medicine, but without a medical background. The topics always fascinate me.

    It struck me that the dilemma over telling the patient they have been exposed assumes that they are powerless to do anything with that knowledge. But how many amazing discoveries that changed the world were made by people whose lives depended on it?

    You shouldn’t treat them as only “patients” who are powerless to effect any real change, but partners in their own health care. To be armed with knowledge provides them with the tools they need to help find the answers.

    By withholding the facts you are possibly condemning the patient to a horrific death, while preventing them from using whatever resources they might possess (and are unknown to you) to make strides toward conquering this disease.

    Certainly if history has taught us anything it should be that things we perceive to be a death sentence today are often cured tomorrow. Never underestimate the power of serendipitous connections. Things happen for a reason.