Rare Creutzfeld-Jacob Outbreak in New England?

Health officials recently informed 15 patients across New England that they may be infected with Creutzfeldt-Jacob disease (CJD). Unfortunately, there is no screening mechanism for the disease, so maybe they will know for sure in five to ten years. CJD, which is caused by a misshapen protein called a prion, is a rare disease accounting for only 200 cases in the United States every year. However, our inability to screen for prion diseases, as well as their relation to Mad Cow disease and Kuru (the cannibal’s disease) cast an ominous and media-hyped light on CJD cases. Prions convert our brains’ proteins into other prions, creating a cascade of neurogenerative disease. But we don’t really know how this happens.

Once symptoms like cognitive defects and dementia arise, there is little that can be done to stop CJD. Like all prion diseases, there is no cure for CJD.

In this outbreak, eight patients at Catholic Medical Center in New Hampshire, five at Cape Cod Hospital in Massachusetts, and two at the Veterans Affair Hospital in Connecticut were exposed to CJD through contaminated surgical equipment shared between the facilities. Typically, surgical equipment used on a CJD patient is either destroyed or sterilized with the most stringent chemical processes possible because prions can survive typical sterilization techniques. In this case, a patient was found to likely have died from CJD during their autopsy, and the equipment used in their surgery had already been reused.

It is believed the original patient suffered sporadic CJD—the most common cause of CJD ahead of direct transmission and mother-to-child transmission. In sporadic CJD—which accounts for 85 percent of all cases—the prion appears suddenly in the brain, perhaps a mutation of existing proteins.    

Because CJD is so rare, it is impractical to always use extreme sterilization procedures in the hopes of preempting an exposure like this. However, in 2004, 98 patients of Emory University Hospital in Georgia experienced a similar notification: equipment used in your surgery was contaminated by a previous patient with CJD; you may be infected; we will monitor you over the next several years.

To date, none of the 98 patients from Emory University Hospital or the 15 patients in New England has been diagnosed with CJD. None have developed symptoms. For them, this outbreak is now a waiting game.

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