Mad Deer Disease?

Mad Deer Disease?

May 19, 2001 National Review by James A. Swan
Some friends recently sent me a Reuters press release out of Winnipeg, Manitoba, proclaiming "Canada Finds First 'Mad Deer' Disease In The Wild." With haunting thoughts of stacks of cattle and sheep burning in England, I checked with Dr. Valerius Geist, emeritus professor of wildlife biology at the University of Calgary. A sigh of relief came, sort of, when I learned that a single wild mule deer with "chronic wasting disease" (C.W.D.) had been found. Animal diseases become human diseases when they impact public health and economics. To appreciate the nature of the incident in Canada, a short review of recent animal diseases in Europe is useful.

C.W.D. is a form of transmissible spongiform encephalopathy (T.S.E.), a type of disease that attacks the brain of an infected animal, and is fatal. It is related to, but not the same thing as, "mad cow disease," or bovine spongiform encephalopathy (B.S.E.). Understanding the difference between C.W.D. and B.S.E. is important in these times. Biological diseases can also infect the human mind with fear. That is bad enough, but fear is also the weapon of terrorists.

B.S.E., first discovered in Great Britain in l986, manifests as abnormally small folded proteins called "prions" that form clusters and eat holes in the brain. B.S.E is fatal to cattle, spreads slowly, and is difficult to detect. By the time the Brits realized the extent of the problem, more than half of Britain's dairy herd had B.S.E. The U.K. has killed more than five million head of livestock to prevent its spread.

In addition to B.S.E. killing cattle, U.K. scientists also believe that some people who ate B.S.E.-infected beef may have contracted a new variant of a rare, fatal brain disorder with symptoms like B.S.E. - Creutzfeldt-Jakob Disease. This is very significant as often there is a "species barrier" in the transmission of T.S.E.s.

Creutzfeldt-Jakob Disease (C.J.D.) is found worldwide in approximately one case per million people per year. The symptoms are similar to Alzheimer's and dementia. C.J.D. is almost always fatal according to the Creutzfeldt-Jakob Foundation . Its cause is not well understood. It appears to be infectious, but not transmissible like the flu or the plague. Sometimes it appears to be inherited.

Typically C.J.D. appears in men or women of all races between 50-75. It does occur in the U.S. - about 300 cases per year - but there never has been a case linking it to eating meat. B.S.E. has never been found in the U.S., but the chilling image of being poisoned by a steak or a cheeseburger has caught the media's attention, prompting many news stories that stir up emotions, such as the infamous Oprah episode that led to litigation.

The numbers of cases of new variant C.J.D. in the U.K. are not large. According to the Creutzfeldt-Jakob Surveillance Unit at the University of Edinburgh in Scotland, there were 33 definite or probable C.J.D. deaths in the U.K. in 1990. By 1998, the number of deaths was 89. In 2000 there were 75. As of April 2, there have been 18 this year. The number of cases of new variant C.J.D. to date is 56 in Britain, 2 in France, and 1 in Ireland. C.J.D. diagnosis requires an autopsy. The rise in the number of cases could be associated with better reporting and diagnosis as much as any new outbreak.

For perspective: The Center for Disease Control reports that every year in the U.S. 76 million people contract food poisoning, and foodborne illnesses lead to 300,000 hospitalizations and 5,000 deaths.

Numbers of new variant C.J.D. in the U.K. are starting to decrease, but the incubation time can be a decade or more, so the number of those now infected is uncertain. Estimates range from hundreds to thousands. There are many stories here, but let's return to wildlife because this is an "Outdoors" column.

Chronic Wasting Disease, which is found in deer and elk, is somewhat similar to "scrapie," a T.S.E. found in sheep and goats and first discovered in the l700s. C.W.D. is thought to be spread through contact of bodily fluids and tissues, like AIDS, but according to Dr. Dave Samuel, former chair of wildlife biology at the University of West Virginia, "the method of transmission is unknown." Normal cooking won't kill a T.S.E. One theory of the origin of B.S.E. in Britain is that it came from cattle feed contaminated with offal of scrapie-infected sheep. There never has been a documented case of a human becoming infected with a T.S.E. originating from scrapie. Dr. Samuel points out that C.W.D. seems to have originated in game farms, so it is possible that scrapie-infected feed is the original cause, but this is still under study.

C.W.D. was first identified in l967 at the Foothills Wildlife Research Center in Ft. Collins, Colo. It has been subsequently identified in game farms in several states and in Canada. But, and this is a big but, until its recent discovery in that wild mule deer in Saskatchewan, C.W.D. was only known to exist in the wild in a herd of about 62,000 deer and elk that live between Fort Collins, Colo., and Cheyenne, Wyo. Between 4 and 8 percent of that herd have it for certain, but some biologists believe that the actual proportion infected may be as high as 15 percent. Like other T.S.E.s, the disease lies dormant for a long time (2-7 years) and cannot be confirmed without performing an autopsy.

Everyone's fear is that C.W.D. could be transferred to humans like B.S.E., but the World Health Organization, the Center for Disease Control, and an Advisory Panel to USFDA agree that there is no evidence to support the idea that C.W.D. can be transferred to humans, nor that it can be transferred to cattle or sheep. Also, as Dr. Geist points out, hunters do not normally eat brains, blood, and lymph glands, all of which are commonly eaten in the U.K. and Europe.

If you'd like to find out more about more about C.W.D., check out the website for the Colorado Division of Wildlife and the Rocky Mountain Elk Foundation. RMEF is one of the major reasons why there are ten times as many elk today (about 1.5 million) as there were a hundred years ago.

Saskatchewan, incidentally, has already implemented a program of reducing herds of elk and deer by hunting in the area where that one case of C.W.D. was reported. Hunters are encouraged to turn in game heads for analysis. And they have killed off 3000 elk on game farms. As yet there is no vaccine for C.W.D.

Sadly, some animal-rights activists have openly cheered the recent outbreaks of animal disease, saying, basically, "It serves the meat eaters right." This only serves to heighten existing tensions, and to expose the dark underbelly of the animal-rights movement.

On April 18, Sen. Larry Craig (R., Idaho) and Rep. Michael Simpson (R., Idaho) issued a statement of concern that animal-rights activists might actually try to bring animal diseases to the U.S. Their statement was prompted by a comment to reporters by Ingrid Newkirk, President of People for the Ethical Treatment of Animals (PETA). Newkirk said, "I openly hope that it [hoof and mouth disease] comes here. It will bring economic harm only for those who profit from giving people heart attacks and giving animals a concentration camp-like existence."

In addition to B.S.E., the U.K. has also experienced a recent outbreak of hoof and mouth (H.M.D.), which is a viral infection that causes lesions, blisters, fevers, weight loss, and reduced milk production in animals. Infected animals become more susceptible to other illnesses and the overall vitality of the herd declines, but H.M.D. is not fatal to most adult animals. Nor is it harmful to humans.

In contrast to B.S.E. or C.W.D., H.M.D. is extremely contagious and can be spread in the wind. It would not be difficult to bring such a biological agent into the U.S. and disseminate it. And there have been reports of a missing vial of H.M.D. culture from a British laboratory. Sen. Craig and Rep. Simpson deserve credit for speaking out. Animal diseases could be spread by eco-terrorists. The damage unleashed would be psychological, as well as economic and biological. Panic is a disease. H.M.D. control illustrates this point.

H.M.D. is common in areas of Africa and South America. In these countries, in which economies are weak and food is scarce, vaccination is the most common strategy to combat the disease. The U.K. and other European countries have taken the hard-line approach, killing 2.6 million head of livestock and burning the carcasses. Some wonder if the European strategy of slaughtering all livestock, healthy or no, within a mile or two of any sign of H.M.D., is wise or necessary. This is extremely important to consider as USDA officials formulate policy about responding to hoof and mouth on U.S. soil, regardless of its origins, because here we have the additional issue of wildlife populations.

In Europe, wildlife populations are not that large, thus not at huge risk to spread the disease. Nonetheless, in the U.K., hunting has been sharply cut back or banned for the present. In North America, where deer, elk, and antelope are plentiful, wild game could spread H.M.D., as well as C.W.D. Choosing the European control strategy could subject wild herds to massive slaughter. But would it be necessary or even effective? Many wildlife biologists believe that we should be examining other strategies to control and eradicate animal diseases without resorting to massive slaughter, which could have massive implications for economics and ecology, and possibly be relatively ineffective for disease control in free-ranging animals in the long run. As always, an ounce of prevention is worth a pound of cure.

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