Deer, Elk, get Mad Cow Disease

The disease, which is one of several belonging to a family of disorders called transmissible spongiform encephalopathies (TSE) that literally turns the brain into a sponge. The human form, Creutzfeldt-Jakob Disease (CJD), occurs in about one/million people over the age of 50 and about one/billion at the age of 30.

TSE is thought to be caused by a mysterious particle, a renegade protein called a prion, which, abnormally for proteins, is apparently indestructible – it cannot be destroyed by boiling or burning. When medical instruments contaminated with prions are boiled at high temperatures for 30 minutes, the prions remain infectious — When infected materials are incinerated, the ash contains prions, which remain infectious. Funeral homes are refusing to embalm… if an autopsy, which would expose brain tissue, is performed. (My experience from microbiology is that protein will denature at 108 degrees, a relatively low temperature, so that cooking meat well done normally gives protection from protein based diseases. It is truly surprising to me that a protein would survive high temperatures.)

People are worried about a version of mad cow disease that is endemic in deer and elk in parts of Colorado and Wyoming, raising the possibility that hunters who eat infected animals might similarly develop a human form of the disease. Health and Wildlife officials are quick to point out that there is no proof that mad deer or elk disease has “jumped” to humans. (No proof could also mean there is no research.)

 

No one knows what it will look like…

Dr. Thomas Pringle, a scientific consultant for the Sperling Foundation, a charitable public health organization, points out that no one knows what TSE in wildlife would look like in a human and tests to determine the nature of such a disease have not been done.

So far the disease in the wild has not spread beyond Colorado and Wyoming.

(This is not a scientific statement. It would be more proper to state the disease has not been found beyond Colorado and Wyoming which then begs the question: Is anyone looking?)

Hunters in several states are required to turn in deer and elk heads so that the brain tissue can be tested… (Which states? Why not Oregon!)

According to wildlife officials the disease (called “chronic wasting“) affects about 5 percent of the deer and 1 percent of the elk found in a small area in northeastern Colorado and southern Wyoming. (The disease is more widespread in captive deer and elk herds.)

 

High Rate of Infection

“The fact that we have a disease in elk and deer at levels equivalent to mad cow disease at the height of the British epidemic is truly alarming.” said John Stauber, director of the Center for Media and Democracy and the author of “Mad Cow, USA” a book critical of American health officials.

 

A Suspicious Case

According to the Oregonian, dated March 7, 1999, Doug McEwen, a 30-year-old hunter who ate game meat, lies dying at his home near Salt Lake City, while consumer advocates and government regulators are squabbling over a terrifying question that cannot be answered to anyone’s satisfaction:

Is it possible to contract a version of mad cow disease from eating deer or elk?

Moreover, McEwen was a frequent donor of blood plasma, and his plasma was pooled with many blood products and shipped to 21 countries…

American public health officials say that TSE in deer and elk and McEwen’s plasma donations pose no threat to people… “No increase in brain disease is seen,” said Dr. Lawrence Schonberger, a physician and epidemiologist who coordinates surveillance activities for Creutzfeldt Jakob disease in the center for Disease Control and Prevention in Atlanta.

Classic CJD can have an incubation period of thirty years according to the FDA… (that means the increase in brain disease will occur about the year 2020.)

Alarmed by McEwen’s age and the speed of his illness, state health officials sent a brain tissue sample to Pierluigi Gambetti, an expert on prion diseases at Case Western Reserve University in Cleveland. Gambetti ruled out most known causes of CJD. McEwen never received transplanted tissues that might have been infected with abnormal prions. His disease was not inherited, and it definitely did not come from cattle. The diagnosis: Sporadic CJD, meaning no known cause. (Let’s rephrase this diagnosis… Sporadic means we don’t know where it comes from.)

Meanwhile, federal officials are caught in a classic regulatory bind of weighing risks and benefits. The risks of spreading CJD contracted from deer and elk seem miniuscule compared with threatening jobs, frightening hunters needlessly, and shaking confidence in the nation’s blood supply. Mike Miller, a veterinarian with the Colorado Department of Wildlife who is familiar with the problem, said that thousands of people have consumed deer and elk meat and field dressed hundreds of infected animals in the Fort Collins area over the last 25 years. “We make no secret of where the disease occurs, and leave it up to hunters to decide the risks for themselves,” Mike said, “although hunters are advised to wear gloves when cutting up animals and avoid brain and spinal cord tissue.” (T-bone steaks contain spinal cord tissue!)

“Was nothing learned from the British experience?” asked Dr. Thomas Pringle, a scientific consultant for the Sperling Foundation, a charitable public health organization. (The British government ordered the destruction of more than 4 million animals.) The Canadian government officially prohibits blood donations from anyone who has spent more than six months in Great Britain from 1980 through 1999. In the US the FDA will hold public hearings on similar guidelines for blood donations.

 

Reported in the Oregonian June 1, 1999

Officials find no proof of brain disease epidemic.

Judith Willig, 45, died on May 8 of CJD… Lane county has had two confirmed CJD deaths so far in the year: a woman in her 60s from Cottage Grove and Willig from Leaburg. Willig loved to eat wild meat and often dined on elk and deer. She was an avid gardener and spread “sacks and sacks of bone meal on the ground without wearing a mask” according to her partner Kathryn Albere. Willig worked as an anthropologist and spent long days in the field digging in the earth for artifacts and bones. She underwent knee surgery several years ago.

The numbers predict an expected 2.5 deaths in Oregon annually from the disease. A study of the years 1990 through 1995 yielded evidence that five people in the state died of the disease in each of those years. (Twice the statistically expected number!)

 

Estimates distrusted

Those interested in the disease say they distrust goverment estimates, citing among other things, published reports estimating that 6 to 8 percent of people who are told they have Alzheimers’ disease may actually have a form of CJD. The symptoms are identical and without studying the brain tissue CJD is difficult to diagnose.

 

Illness under-reported

“Hospitals don’t want the nursing homes where they send these people to get alarmed about a possible infectious disease, so they call it Alzheimer’s.” Dr. Tom Pringle said. “Then it goes down on the death certificate as Alzheimer’s.”

According to the state’s Center for Health Statistics, Oregon has the third highest Alzheimer’s disease death rate in the nation, with more than 500 people dying from the disease annually. (Who is higher? It may be significant particularly if they are states that are high in hunter populations.)

(It’s time for more careful scrutiny…)

 

Reported in the Oregonian December 19, 1999

In an article by Bill Monroe, the wildlife editor, who quotes an ominous warning from the Oregon Department of Fish and Wildlife written to 250 Oregon taxidermists, dated September 21, 1999.

“We are writing … about a potential wildlife health risk and disposal of deer and elk parts from specific areas in Colorado or Wyoming. If you do not work with deer or elk harvested in thses states, the following probably does not apply directly to you.”

The memo advised the wearing of dust mask, goggles and rubber gloves when working with skulls and brain tissue.

Wildlife biologists throughout the west are warily erecting what barriers they can to the spread of a wildlife disease known as chronic wasting. The incurably fatal, stubbornly resistant and little-understood brain disorder first appeared in the 1960s and ’70s in state research herds and private elk ranches in Rocky Mountain and Midwest states and Saskatchewan, Canada.

Bill Monroe reports that chronic wasting has spread to a nine county area of southeast Wyoming and north central Colorado, but not beyond… (emphasis mine… again not a scientific statement… it’s more proper to state that it hasn’t been found outside the stated area… and then we can ask if anyone has been looking.)

… Even there, it has been found in less than 10 percent of wild deer and elk, and mostly in a single county. (The British outbreak occurred in less than 5 percent of their cattle herd!)

…Oregon and Washington officials said neither chronic wasting nor venison-related Creutzfeldt-Jakob have been reported in the Northwest. (Technically this may be correct but there certainly have been some suspicious deaths!)

… Hunters or their guides almost always skin deer and elk in the field, which includes carefully removing the scalp, called a “cape”, and discarding the skull and bones. (Hunters wishing to save the “horns” saw into the brain case to retain enough skull to hold the antlers together… and the State of Oregon requires hunters to retain proof of sex.. (the horns) together with the eyes of the animal… tell me how anyone does that without retaining a portion of the skull!)

 

Reported in the Oregonian Tuesday, December 21, 1999

A study suggests a new brain disease has the same cause as the cattle illness.

by Paul Recer, The Associated Press.

A laboratory experiment give powerful new evidence that an infectious protein that causes mad cow disease also causes a new type of fatal human brain disease that has killed 51 people in Europe…

experts suggested mad cow disease possibly could be linked to the brain-wasting human disease… Because the new form of Creutzfeldt-Jakob is thought to have an incubation period of at least 10 years, experts say it is impossible to know how many people have been infected.

 

Announced by the Red Cross and reported on television February 2, 2000

The Red Cross will not accept blood from donors who have visited Britain during the period of years mentioned above….