U.S. not immune from prion diseases

April 2, 2001 Minneapolis Star Tribune by Deborah S. Rogers

I woke up sensing something other than my husband in bed with me. The snuffling turned out to be a young deer mouse looking for a warm place to nest. I chased it out of my bed and turned over uneasily. For years, my family and I have lived with this deadly threat. Deer mice carry hantavirus, a disease that is fatal to about a third of those who contract it.

A man just up the road died of hantavirus a few years back after going through a mousey box of eight-track tapes. We have a ranch homestead with numerous decrepit barns and outbuildings: a veritable Mecca for field mice. We consider the possibility of death every time we sweep out the garage or open a box of old photos.

Over the past several years, we have come to understand an impending threat of much greater proportions: the prion diseases, including, most famously, mad cow disease -- bovine spongiform encephalopathy, or BSE -- and its human counterpart, Creutzfeldt Jakob Disease (CJD).

Our neighbors, mostly ranchers, don't like to talk about it. They stand to lose their livelihoods to the ensuing panic, long before anyone loses a life. With low cattle prices, and suburban development driving up property taxes, even the rumor of mad cow disease could result in loss of the family ranch.

The main thing we all want to know, of course, is whether mad cow disease could strike in the United States. We are assured repeatedly that it isn't here, and that all necessary precautions are being taken to keep it out. But that is the wrong question. Mad cow disease is just one of many strains of prion disease, so the real question is: Which strains are here and what are their risks?

The answers are not reassuring. One strain of BSE in cattle was documented in 1985, when farmed mink in Wisconsin developed a prion disease after eating "downer" cattle. The symptoms of this American form of BSE are somewhat different from those of British BSE: Instead of going "mad," cattle simply drop over. Brain autopsies also show differences from the British variety of mad cow disease. With around 300,000 downer cattle keeling over every year in American pastures, we need an extensive study to determine whether a form of BSE is already well established, as suggested by the late prion disease expert, Richard Marsh.

Another prion epidemic is already underway. Chronic wasting disease (CWD) is present in herds of wild and domestic elk and deer in Colorado, Wyoming and South Dakota. The disease appears to be spreading from a Colorado research facility that once specialized in studying scrapie, a prion disease of sheep. Scrapie can spread when animals nibble at placental tissues (afterbirth) in pastures. Hunters in this region have been asked to send in heads from their kills, and have been warned about the risks of butchering or eating their quarry. At least two hunters potentially exposed to CWD have died of CJD, although there is no way to prove a connection. And that leads us to the biggest question of all: How many people are dying of CJD in the United States? CJD, like BSE, has several strains. Death certificates over the years indicate that about one person per million has CJD. But are the death certificates accurate?

A study at the University of Pittsburgh determined through autopsies that around 5 percent of the people who were assumed to have died with dementia (such as Alzheimer's) actually had CJD. If 5 percent is a realistic figure, perhaps as many as 200,000 of the supposed Alzheimer's patients in this country could have CJD right now.

As with BSE, the American strain of CJD may have different symptoms from those of the new British variant of CJD. Immediate and extensive studies are needed to determine the true extent of CJD.

What should be our reaction to the risks of this poorly understood, rapidly evolving group of diseases? Panic and mass-eradication of animals won't protect us. But there are several laws we need to implement immediately, and those are the laws of nature.

Herbivores must not be fed ground-up animal parts, nor should omnivores be fed diseased meat. The Food and Drug Administration banned feeding ruminants back to ruminants (cattle, sheep and goats), but enforcement has been lax. Animals such as pigs, poultry and even farmed fish must be protected from animal-protein feed supplements as well; new studies suggest they, too, can contract prion disease. Given the potential for the spread of prions, all livestock should be fed a strictly vegetarian feed ration. Don't get me wrong. I've got no secret vegetarian agenda. At the dinner table, people often suggest I might want to kill my steak before I eat it. But CJD is nothing to mess around with. Like it or not, we're in a well-known evolutionary game called disease-host co-evolution, and prions play for keeps.

Meanwhile, we had two downer cows in the pasture we lease to neighbors. A local elk hunter and friend of my family recently died of CJD. I threw out our venison salami, but we still have mice in the farmhouse. I've set about 20 traps. If that doesn't work, I'll bulldoze the place and build a mouse-tight cabin for my weekend visits. Did I mention I've moved to town?

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