Prion diseases; is the lid about to come off?

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Eye from Albany
May 2004

Prion diseases; is the lid about to come off?
by Paul M. Bray

One of government’s most valuable functions is public health including the prevention, monitoring and response planning for diseases like interspecies lyme disease and West Nile Virus and health threats like bio-terrorism. Yet, for decades policy and political attention has been monopolized on modes and costs of patient treatment. This could, in part, be the effect of the conservative notion that government can do no good and everything should be left to the market. Whatever the cause, we had better wake up to make sure our public health assets are fully engaged to address the growing health threats ranging from obesity to instantaneous threats like smallpox. Let me share a public health issue that may be raising its ugly presence.

Last fall a colleague of mine from the middle of the State died. When I mentioned his death to another colleague, a non-alarmist type, who lived near the deceased, he replied, “Yes, it is a very tragic death, he died of mad cow”. I was speechless, as the thought that anyone in this nation was vulnerable to mad cow just didn’t seem possible.

Shortly after that episode, I read about prions, a type of protein, that is the cause of a number of brain related diseases, most notably Creutzfeldt-Jacob disease (CJD), bovine spongiform encephalopathy (human mad cow disease), scrapie in sheep and chronic wasting disease in deer. CJD is generally considered to be caused by genetic mutation, proteins losing their shape over time, while mad cow is caused by infection of prions from outside sources. CJD is reported to cause about 250 to 300 hundred relatively swift deaths each year in the USA.

Complicating matters is the lack of rapid testing capacity. Case Western Reserve has the only facility that can test for human mad cow disease. The decreasing number of autopsies performed also limits the ability of health authorities to track cases of CJD or other prion related diseases.

In addition, Alzheimer’s disease has many common symptoms that doctor who are not specialists may not distinguish from CJD and human mad cow disease. Italian researchers have recently found a second strain of mad cow disease resembling CJD.

A feature of prion biology is that a single protein is able to generate different infectious strains each with its own specific characteristics. Prion pathology is an emerging science and scientists are just finding that prions have the ability to jump the species barrier and infect other mammals.

While the science is emerging, there is a question whether we also have an emerging public health crisis affecting our food supply.

So far, the signs of impending crisis are small, but growing in attention. You may have noticed an article, “The Case of the Cherry Hill Cluster by D.T. Max in the NY Times Magazine on March 28th of this year, about how a cluster of CJD deaths in New Jersey may really have been deaths caused by mad cow. The writer notes, “Many public health advocates and some scientists say they suspect that mad cow does in fact exist in America”.

A gadfly, Janet Skarbek, has picked up on the New Jersey cluster and has been peppering the NJ Department of Health and Centers for Disease Control and Prevention with her theory contaminated meat caused the cluster of deaths and that “There has to be a strain of mad cow disease in America that is rare enough or different enough to have avoided detection”.

Skarbek “sees herself unraveling a conspiracy of government and business interests (of food suppliers) to hide an epidemic”.

On the west coast, the Seattle Post-Intelligencer reported “Now largely because of that made cow case (the diseased cow found in Yakima, Washington), more people are wondering whether their elderly relative might have suffered from CJD-a degenerative disease of the nervous system-or its variant, the human form
of mad cow disease”.

The fear and debate surrounding these circumstances present a serious challenge to our public health authorities. Can surveillance and testing develop rapidly enough to contain a real mad cow “epidemic” and/or the hysteria over the unknown and mysteries surrounding CJD and other prion related, interspecies brain diseases? Today, for example, not enough autopsies are being performed to adequately monitor prion pathology.

In New York State, we are fortunate to have the world class State Wadsworth public health labs in Albany and other research facilities like one in Staten Island where a fast test for chronic wasting disease was recently developed. This may be a time and an issue when the premium will be on trust in the capacity of our public health authorities to protect us. Yet, one can only wonder if we have invested enough in public health resources to adequately have them there to meet new and increasing complex threats to our health.

Paul M. Bray is President of P.M.Bray LLC, a planning and environmental law firm in Albany, New York. His e-mail is