June 1, 2002 Rocky Mountain News (Denver, CO) by Lou KilzerShortly after mad cow disease was first detected in the British countryside in 1986, officials stampeded to the microphones to assure the public that there was no risk to human health.
Ten years later, they admitted they had been wrong.
With the emergence of a similar disease in Colorado elk and deer, official response, based on available science, echoes the early years of mad cow: Chronic wasting disease poses little risk to humans.
As recently as April, Mike Miller, the veterinarian spearheading Colorado's effort to contain the disease, lamented that the public's concern about CWD as a human health threat was "misguided." He said there is no evidence that chronic wasting disease in deer and elk will infect humans. "It just seems that it is not at all likely" that humans could get the disease, he said.
But new federally sponsored research and two small clusters of a disease rarely seen in young people have other scientists concerned that Miller might be overly optimistic.
Response to mad cow in Great Britain and the United States was flawed. Consider:
After disease detectives in Great Britain determined that mad cow, or bovine spongiform encephalopathy (BSE), was spread by feeding cattle infected meal, British officials banned the practice - in their own country. But they didn't ban the export of the feed around the globe, spreading BSE and causing economic devastation in continental Europe and Japan.
It took the United States 10 years to follow Britain's lead and ban the feeding of cattle-derived meat and bone meal (MBM) back to cattle. But the U.S. allows - indeed, actively promotes - the export of MBM to other countries. The Food and Drug Administration (FDA) makes no requirement that warning labels against feeding it to cattle be placed on the product.
One country that exports over a million cattle a year to the United States - Mexico - is just now adopting the ban on MBM as cattle feed. And the enforcement of that ban is suspect.
The FDA has allowed hundreds of meat-processing facilities to fudge on its MBM rules, handing out a handful of warning letters and taking little action to force compliance.
The FDA asked pharmaceutical companies to stop using bovine serum in children's vaccines. For seven years it allowed the companies to ignore its request.
RESPONSE TO CWD
No one can say if the record of dealing with chronic wasting disease will prove equally inadequate, but it, too, has been marked by caution and denial.
A decade ago, Beth Williams, the scientist who discovered CWD, warned that the growing practice of raising elk on private ranches could spread CWD not only within those facilities but into the wild. State regulators paid little heed, allowing elk ranching to expand dramatically.
Williams reported in the same scientific paper that "a few surplus deer and elk" from CWD-infected state-run pens near Fort Collins had been released back into the wild. How many and in what years remains unclear. State officials say they can find few records.
Until recent years, as public pressure has grown more intense following mad cow disease, the state's main approach to CWD was to monitor its growth.
It is now known that chronic wasting disease can infect human cells in test tube experiments, although what happens in nature is often far different from what happens in a laboratory. Scientists have also discovered that at least one form of these diseases, which collectively are called transmissible spongiform encephalopathies (TSEs), can be injected in one set of test animals where it is undetectable and then passed to another set where it is uniformly fatal.
Two small clusters in the U.S. of a rare and related disease have appeared in young people. Federal scientists stress that there is no link to CWD. Others are concerned.
Patrick Bosque, a leading TSE researcher now with Denver Health who has worked closely with Nobel laureate Stanley Prusiner, said the most recent cluster "was the strangest thing I've ever seen." He said it was improbable that the two cases do not have a common cause, but not impossible.
THE ORIGINS OF MAD COW
The British cases started on Paul Lysley's farm in August 1983.
Dairy cows numbers 221 and 117 began behaving strangely - stumbling about, losing weight, becoming irritable.
When veterinarian Ray Williams arrived at the farm in Wiltshire, England, he was stumped. He could find no virus, bacteria or fungus to explain the condition.
This was something different. Something strange.
Then came cows 226 and 5, all on Lysley's farm and all getting sick and dying. Williams was now becoming alarmed.
He sent samples to another lab. Again, nothing was found.
Next cows 36 and 141 got sick and died.
What was at first curious was turning downright ominous.
All the researchers in Williams' group "believed we were dealing with an unusual condition for which we had no real diagnosis," the veterinarian later told an investigating committee.
In late 1986 came an answer. English cows were dying from a disease never seen before in cattle - bovine spongiform encephalopathy.
Soon, the world would know it by another name - mad cow disease.
The fight against BSE focused on one question: How were cows becoming infected?
The man tasked with finding the answer was epidemiologist John William Wilesmith of the British Central Veterinary Laboratory.
Wilesmith did what disease detectives do around the world - he tried to find out what these cows had in common, with his first guess being that perhaps the disease was being caused by a toxic chemical.
He looked into vaccines the cows may have had in common, their genetic backgrounds, and the way they were disinfected.
All the while, more and more cows were coming down with the disease. Within a year, Wilesmith had his theory down pat.
He had detected changes in the late 1970s and early 1980s in the amount of meat and bone meal (MBM) being used as a protein supplement in cattle feed, and in the temperatures used to cook the meal.
English sheep had been afflicted with a TSE called scrapie for more than 200 years. Wilesmith believed that a sheep with scrapie was rendered into meat and bone meal and ingested by a cow that caught the disease.
Sick cattle were rendered into still more meat and bone meal, setting off an explosive chain reaction.
By the time farmer Lysley's cows started getting sick, cattle throughout the country had already contracted the infectious agent - a simple mutant protein called a prion.
The government immediately banned the feeding of rendered remains of sheep and cattle to sheep and cattle, an action credited with eventually curbing the spread of the disease.
But it didn't ban the export of MBM or other meat products overseas. Will Hueston, head of the Center for Animal and Food Safety at the University of Minnesota, believes that mad cow could infect 10 to 15 other countries where it is yet to be detected. In May, mad cow was diagnosed in Poland, the most recent country to host the disease.
At the height of the BSE epidemic, the United Kingdom exported 500,000 tons of beef byproducts around the world, including 168,000 metric tons of MBM between 1990 and 1996. It also exported 3.2 million cattle to 36 countries.
A Harvard study said that the exact amount sent to the U.S. was unknown, but it noted that at least 69 tons of "mammalian meal and flour" and 334 cattle were shipped here during the period.
Even in the U.K., hope that the feed ban would stop the BSE epidemic in domestic herds was soon dashed.
Cattle born after the feed ban began getting sick with the disease. Government officials passed around dozens of confidential memos concerning the post-feed-ban cases, and police and others launched quiet investigations.
The conclusion: There was rampant fraud among feed producers, causing prohibited beef products to continue to find their way into the food chain. One confidential memo to the secretary of the forestry commissioners concluded that "the practice of providing falsified documents was a widespread and long term one" involving eight of the nation's nine major slaughterhouses.
An October 1995 government document concluded that infected animals "may have been getting into animal feed because of poor controls at slaughterhouses."
Still, officials presented the issue as a public relations problem with no implications for public health.
BSE, after all, could never infect humans.
That mantra was repeated again and again by official after official. Those expressing doubt were shrugged off as fringe elements.
Wilesmith's epidemiology had proved that scrapie had crossed the species barrier to become mad cow in cattle. But popular opinion remained that it couldn't again cross a species barrier into humans.
The British government did make one concession to public concern. Any animals that looked sick - that is, showing clinical signs of mad cow - would be put to "slaughter and destruction." This, an official report said, would suffice "to protect (humans) against the remote possibility of transmission."
All the feel-good rhetoric came to a sudden end on March 20, 1996, when British officials said they had been wrong. At least 10 young people were dead or dying of new variant Creutzfeldt-Jakob disease, or vCJD, which is the name given the disease that mad cow causes in humans.
Even then there were some skeptics. Ten days after the British announcement, Miller, the Colorado Division of Wildlife veterinarian and head of the state's CWD program, told the Rocky Mountain News that he doubted BSE had really jumped to humans. The species barrier was just too strong.
But the evidence would soon be clear. The disease was affecting unusually young people, mostly under 30, versus the median age of 68 in the classic CJD. Victims with vCJD live for an average of 14 months, versus four months with CJD. And the pathology in the brain is different - victims of vCJD develop brain plaques that have a daisy-like floral pattern. Not so with CJD. And the 10 had something else in common - they ate beef that had potentially been infected.
U.S. REACTION IS SLIPSHOD
American reaction to BSE was marked by some quick action coupled with official blind spots.
In 1989, the U.S. banned live cattle and most cattle products from countries that had active BSE - but not from countries that had imported British cattle and MBM.
And because of that latter omission, the U.S. cannot be certain it has remained BSE-free, according to the General Accounting Office (GAO), the watchdog of Congress.
The United States imported 1,000 cattle, 23 million pounds of inedible meat byproducts, 101 million pounds of beef and 24 millions pounds of prepared beef products during the past 20 years from countries that now have BSE-infected livestock, according to the GAO.
The U.S. also allowed the continued feeding of meat and bone meal to American cattle even after Wilesmith's warnings that MBM was inherently dangerous.
After all, sheep scrapie is endemic in America just as it is in Great Britain.
Dr. Stephen Sundlof, the FDA's director of the Center for Veterinary Medicine, said the FDA proposed banning the rendering of sheep to be fed to cattle in 1994, but backed down after objections from the sheep and rendering industry.
However, "everything changed" when it was announced in March 1996 that BSE had been transmitted to humans. "It was not only an animal disease anymore," Sundlof said.
It took almost another year and a half for the FDA to put its slippered foot down, banning the feeding of most mammalian protein back to cattle and other ruminants.
The pressure behind the FDA's directive has been anything but heavy. As happened in the U.K., some meat processors in the U.S. frequently did not heed the FDA's restrictions. And the FDA didn't seem all that interested in enforcing them, according to sharply critical reports by the GAO.
In a year 2000 report, the GAO said that 1,700 of 9,100 firms involved in some facet of MBM manufacturing and distribution were still unaware of the regulations.
Twenty-eight percent of firms that were required to put warning labels on their feed prohibiting its use for cattle weren't doing so, and some 20 percent of firms did not have a system to prevent comingling of prohibited and non-prohibited feed.
Those violations resulted in just two warning letters from the FDA.
The GAO revisited the issue this year and was again highly critical of the FDA. The GAO said the FDA had failed to enforce the feed ban, issuing warning letters to a small percentage of firms not in compliance and shutting no one down.
The FDA explained to the GAO that it moved slowly because it believed industry needed time to become educated concerning the feed ban. It said it is now beefing up enforcement. However slowly it acted, the FDA notes that its ban on beef entering the U.S. from active BSE countries has stopped mad cow from infecting American cattle.
Following in the first footsteps of the British a decade earlier, the FDA has not imposed the same restrictions on exported MBM. In fact, since the American ban went into effect, annual U.S. exports of MBM have jumped from 291,000 tons to 467,000 tons, a 60 percent increase.
American renderers aren't required to warn their foreign customers about feeding ruminant protein - that rendered from sheep or cattle - to cattle.
However, three large renderers contacted by the News say they label their products that way regardless of the lack of regulations.
Denver's National By-Products said it ships its MBM to China and Indonesia in large shipping containers, not in individually marked bags. But it stamps on its bills of lading a warning against feeding the product to ruminants.
The stamp is in English.
Once American meat and bone meal arrives in the purchasing country, the manufacturer has no further control over how it is labeled, said National By-Products district manager Ken Kage.
A spokesman for the USDA and officials with the National Renderers Association say that foreign trade in U.S. MBM is not a problem because there have been no cases of mad cow disease in this country.
U.S. MEAT AND BONE MEAL IN MEXICO
Some countries importing MBM have had few if any rules concerning its use as cattle feed.
Mexico, for example, implemented labeling rules only this year, according to Alberto Celis, the National Renderers Association regional director for Latin America.
That was news to many agricultural business people attending an animal feed trade show in Guadalajara in March. Representatives from three animal feed bag manufacturers said they had heard of no such regulations and that their bags remain warning free.
Mexico exports over a million live cattle a year to the United States. Mexican cattlemen said these "feeder" cows are not typically fed animal protein, though there is little evidence that the government has an adequate inspection program to make certain.
Mexican government officials responded that MBM rules were promulgated last summer, and that they will be vigorously enforced. They said Mexico stopped importing MBM from countries with a BSE problem in 1991 and that there are no known cases of BSE in the country.
The World Health Organization says Mexico's experience with American MBM is reflected throughout the world.
The United Nations agency was "concerned that some countries which received (MBM) materials do not have surveillance systems to detect the disease in animals or the human population," said WHO's Dr. Maura Ricketts at a news conference in December 2000.
She said once the MBM leaves one country, it begins a "murky movement" that is almost impossible to track.
Taking heed of such warnings, the European Union (EU) decided that the risk to public health was too great even if an importing country insisted that it would use MBM only as poultry feed - which, along with pet food, is its major use in the U.S.
The EU adopted the ban of all exports of MBM in 2000.
Instead of adopting a similar policy, the USDA saw the ban as a golden opportunity.
"Importing countries of EU MBM may be forced to seek alternative suppliers of animal protein meals, such as the United States," said a December 2000 report by the USDA. "The United States should be well positioned to take advantage of that situation to increase its own exports of MBM."
And it has.
Render, the magazine of the National Renderers Association, noted in its April issue that exports of many products were under competitive pressure from vegetable oils.
But it noted "a bright spot is meat and bone meal exports that continue to increase." The chief foreign markets for American MBM, in order of sales amounts, were Indonesia, Mexico, Egypt, China, Canada, Thailand, Bangladesh, the Philippines and Venezuela.
In 1998, Egypt imported 96,000 metric tons of MBM from the EU, and only 3,100 metric tons from the U.S. By 2001, the U.S dominated the Egyptian market, selling over 73,000 metric tons.
Jorge Gutierrez Moreno, general manager of Apelsa, one of Mexico's largest renderers, complains that American competition has become particularly cutthroat.
Americans have cut their MBM prices from $500 a ton to as low as $230, he said. Already one large Mexican cattle operation has shifted to the U.S. product, he said.
"They don't care how they do it," Moreno said of the American MBM sellers. "When the product comes from America, the companies don't care what happens."
Tom Cook, president of the U.S. National Renderers Association, said it was untrue that American firms were dumping their product in Mexico or elsewhere.
"We're putting the product in there at the prevailing market price," Cook said. American meal "has gone down considerably" in price, he acknowledged. But he said that's because it's a "cyclical" product that fluctuates in price against competing products, such as soy protein.
Richard Patton, a Ph.D. nutritionist and independent consultant to the American dairy industry, said U.S. MBM in Mexico should be watched as carefully as "you would watch a hand grenade with the pin pulled."
Patton, who was at the Guadalajara animal feed expo, said that while most large operations were likely aware of the dangers of MBM, he doubted smaller ones would be. "Let's be realistic," he said.
Surveillance for BSE in Mexico will be tough for the lack of inexpensive ways to detect it. "If you can't measure it, you can't manage it," he said.
But Celis of the National Renderers Association said he didn't anticipate a problem. "We will minimize the risk as much as possible," he said. "But there is no such thing as zero risk. ... The Mexican government is concerned about the issue and is implementing the regulations."
This in March 2002 - five years after the U.S. banned feeding meat and bone meal to cattle, and 15 years after Great Britain took the action.
BOVINE SERUMS IN VACCINES
MBM isn't the only hole that mad cow disease could slip through. Vaccines using bovine blood serum, cosmetics and even food supplements have all worried scientists. Vaccines are of particular concern because they are given to the most vulnerable - our children.
The FDA asked manufacturers of vaccines to stop using bovine blood serum and other materials from British cattle in their products. But this was not an order, simply a recommendation.
Some drug companies ignored the recommendation for seven years, complains Peter Lurie, a doctor and deputy director of the consumer group Public Citizen's Health Research Group.
"My main beef about vaccines was the failure of the FDA to regulate the industry and the consequent thumbing of the nose by the industry to the FDA," Lurie said.
Lurie said he doubted any vaccine with infectious prions had been administered to American kids, but he wondered why the government would take any chance at all.
In 1998, the United States imported from Great Britain 162,000 kilograms of vaccines and 2,765 kilograms of fetal bovine serum.
The FDA says the danger of not using the vaccines is far greater than the risk of using them because the risk of actually having prions in the vaccines is "remote and theoretical."
Lurie also complains that there is little regulation of nutritional supplements, many of them containing matter from cow brains. The industry says it is self-regulated, but critics say there is no way to know for sure what is and what is not in a supplement - or, just as important, where the material originated.
FAILURE TO CONTAIN CWD
Deer started dying in 1967 at an outdoor research pen west of Fort Collins.
Involved in an experiment to see how they could be better fed during rough Colorado winters, the penned deer began withering away, stumbling and acting strange. Ten years later, CWD was proved a form of transmissible spongiform encephalopathy.
After British scientist Wilesmith described how sheep scrapie prions had infected cows that later infected humans, several of those working at or near the original 1960s Fort Collins experiment recalled something else about the advent of chronic wasting disease.
Sheep had been confined in the same pen as the deer. Researchers recall that some of the sheep had scrapie, though no papers survive to prove the point.
The issue didn't become urgent until 1996 when it was announced that mad cow had jumped species once again, this time to humans.
Could CWD behave similarly?
The story of CWD disease in Colorado deer and elk has eerie echoes from the early days of BSE in England.
Officials continually say there is no proof that chronic wasting disease can jump from deer to humans.
One of the state's chief goals has been to dampen any panic and preserve the hunting industry, while reporting what is known about the disease.
What seems clear from the record is that Colorado state officials and members of the media have not been overly concerned about CWD until quite recently.
The first mention of the disease in the Rocky Mountain News was in 1995, 18 years after scientists knew there was a TSE spreading in elk and deer.
The newspaper said officials considered CWD "a minor problem in localized populations of deer and elk."
There was a brief eight-story flurry of interest in 1996 - four in the News and four in The Denver Post - after the March 20 announcement that humans could get mad cow disease.
That same year big-game managers actually tried to increase the deer herds along the Front Range by restricting hunting permits - a policy that could only spread the disease, and one that has since been reversed. State wildlife officials, however, advised hunters not to eat obviously sick animals - a step once taken as an adequate measure for protecting humans in Great Britain.
The problem with this otherwise sound advice is that a deer can last for two to three years having infectious CWD prions without showing outward signs of the disease, according to CWD researcher Williams.
By 1998, officials had extended their advice to include a recommendation that hunters should not eat brain and spinal cord materials from any deer or elk.
From 1996 through 1998, the Colorado Division of Wildlife made it mandatory for hunters in about a dozen hunting units in the endemic area to bring in the heads of their animals for testing.
In February 1999, state wildlife officials said CWD was not spreading, and reiterated that there was no risk to humans, something they repeated almost every time the disease was mentioned.
In 2000, it was decided that the number of hunters voluntarily bringing in heads was high enough that the requirement was lifted in all but three hunting units where there were special, late hunts.
Even after the disease was detected in elk ranches, no real alarm went off. "We don't think the problem is a big deal," veterinarian Miller told The New York Times in October 2000.
Miller said recently he takes the problem very seriously - as an animal disease. He said the The Times took his comment out of context.
In November 2000, though, the state began examining plans to try to control the disease by lowering the population of deer in the endemic area.
The disease was spreading faster and wider than anything officials had anticipated.
Michael Hansen, a Consumers Union scientist, says leaving the problem up to the Division of Wildlife might have contributed to the spread, not because of any malicious motive but because of the agency's function and tradition. The division exists to manage wildlife, and it is funded by hunting and fishing licenses.
But the division is obviously fully engaged these days.
Russell George, who took over the Division of Wildlife in late 2000, told a reporter last year: "I think CWD is as important as anything we are doing."
He began holding regular meetings about the disease and for the first time started a program of killing animals in the infected areas instead of depending on hunters to do so.
"It's watching what happened in Great Britain that says to us we must not let that happen here," George said.
"We're not going to ever be in a position as a government that we knew something and didn't act on it, or that we knew something and didn't tell about it."
George's new aggressiveness, coupled with news that CWD was spreading to other states, caused a major expansion of interest in the once backwater field. Denver's dailies published 116 stories on CWD last year, and 153 as of mid-May 2002.
CAN CWD SPREAD TO HUMANS?
The stakes are high.
At least in penned deer and to a lesser extent in the wild, CWD is highly contagious.
And there is no proof, but some evidence, that humans are susceptible to the disease.
In test tubes, CWD prions can convert human prion protein into the deadly form, albeit inefficiently. They do this at nearly the identical rate that BSE prions convert human prions to mutants, according to research at the Montana labs.
No one can be certain what CWD might look like if it should pass to humans. And no primate research is currently being conducted to try to answer that question, something the FDA says should change.
There are no proven cases of humans being infected, but there is a statistical anomaly that has caught scientists' eyes.
After mad cow disease had migrated to humans in 1996, researchers began looking for any increase in TSE-like disease in young people, which is almost unheard of. In a 17-year period ending in 1996, there were only four sporadic CJD cases among those 30 or younger in the United States. Then, in short order, there were four more cases.
Researchers found that the four new cases, appearing from 1996 to May 31, 2000, had no known risk factors for CJD, such as taking human growth hormones or travel to Europe.
But they were concerned that three of the four were venison eaters. The Centers for Disease Control launched an investigation to determine if their cases were connected to CWD.
The CDC found that none was known to have hunted in the endemic areas of Colorado or Wyoming, though two did eat meat from animals killed in non-endemic areas in Wyoming and the third had his meat processed at a plant that also butchered Colorado elk.
In microscopic examinations of brain tissue after the three died, the disease looked more like traditional CJD than it did tissue from British vCJD victims.
This led scientists to conclude that there was "no strong evidence for a causal link" between CWD and the three young venison eaters.
Ermias Belay of the U.S. Centers for Disease Control in Atlanta and the scientist who wrote those words, told the News: "I can't say I'm absolutely convinced" that the venison eaters didn't contract their disease from eating venison.
"All I can say is that all the evidence we have to date does not indicate that chronic wasting disease has caused illness in the three patients. I have to be cautious because we can only go by what we have."
Asked if it was possible for CWD to infect humans, he said: "That's possible. Definitely possible. But do we have evidence of it? No."
Bosque, the Colorado prion scientist, said the cases of the three venison eaters constitute "a bit of evidence that the disease might be transmissible to humans."
"I think that if humans were very susceptible to CWD, we would know by now, since hunters have probably been consuming infected deer for some time," he said.
"But if CWD is transmitted at a low rate - say 1 in 1,000 or one in 10,000 - we might not be able to easily see this."
Wildlife veterinarian Miller is less equivocal. "CDC made of it that there's no connection to chronic wasting disease. I would make of it that there's no connection. There simply is or is not a relationship and in this case there's not."
Hansen of the Consumers Union said he believes Belay has made an assumption that human brains infected with CWD would look like vCJD found in British sufferers.
"If chronic wasting disease is moving into the human population, we wouldn't know what it looks like," he said.
But Hansen agrees that there is no proof that CWD has broken the species barrier.
As long ago as 1986, a research paper by Nobel Prize winner Carleton
Gajdusek found a significant statistical link between CJD and human exposure to deer and animal organs.
Denver mathematician Dr. George Bardwell called the data - which looked at 26 CJD cases against 40 controls - "highly significant."
In one troubling scenario, researchers at the Hamilton NIH lab said TSE disease could exist silently in a sleeper species before jumping into another. In a research report last November, the team wrote, "A similar situation could occur with CWD. CWD transmission to other cervids or livestock could change its characteristics, including its potential for transmission to people."
Wildlife director George is frustrated as a layman dealing with scientific issues.
"I want those people to tell me, is there a risk. What should we do? I cannot predict our future."
A MYSTERY IN MICHIGAN, WISCONSIN
Scientists have turned their attention to two small clusters of patients who appear to have contracted Creutzfeldt-Jakob disease, but at an abnormally young age.
The story began last August when two men, 26 and 28 years old, entered the University of Michigan Hospital in Ann Arbor suffering dementia.
No one thought of CJD at the time because it was assumed they were simply too young to get it. Only one case in 270 million occurs annually in persons age 30 or under in the United States, according to the CDC's Belay.
The chance of two young people coming down with the disease at the same place and time are much more remote.
"While it is certainly improbable," said Denver prion researcher Bosque. "It might still be that the two cases were just a chance association."
The young men didn't have telltale brain scans that most CJD patients have. One didn't have a diagnostic protein in spinal fluid that is characteristic of CJD. The other man's spinal fluid was not tested.
CDC and Michigan officials launched an investigation. They found no common risk factor, including the eating of elk or deer.
Dr. Norman Foster, a University of Michigan neurologist, understands the odds. He believes there must be far more cases of young people developing TSE disease than previously thought. Perhaps, he theorizes, many young people diagnosed with viral encephalitis actually have CJD.
The CDC's Belay holds the theory that this is a coincidence. But Hansen of the Consumers Union isn't buying that. He does not believe in coincidence, at least not on that scale.
He said the CDC can't be sure the two men didn't eat venison, a delicacy in that part of the Upper Midwest.
The Michigan cluster isn't the only one raising eyebrows.
In Wisconsin, Sue Kanchana, a neurologist, ponders the case of a 42-year-old woman who has a neurodegenerative disease that apparently started when she was 37. The woman has been moved to a hospice and is expected to die within weeks. As with the young men, doctors didn't think it was CJD - the woman was just too young to get a rare disease that strikes people at the mean age of 68.
But the number of alternative diagnoses has dwindled. And the woman's spinal fluid is positive for 14-3-3, the marker protein associated with CJD.
"From the way we see this, there is nothing else this can be," said Kanchana. "It cannot be anything else."
That is disconcerting because the woman would be the fourth CJD case in the past three years at the hospital where Kanchana works. Before it was practically unheard of.
"CJD was one of those things that you used to laugh at and say, 'Right!' " she said. "But now we're seeing them."
Kanchana's other patient is about 50 years old and she is unaware of the ages of the other two. They are not her patients.
"We see these and these are supposed to be things we aren't supposed to be seeing," she said.
She thinks something is changing, but she has no theory why.
Tracie McEwen, the widow of the first young venison eater whose case was investigated by the Centers for Disease Control, does have a theory.
"I'm not a scientist, but I don't understand why that's so hard for people to make that leap, when you had them in Britain saying, oh, the beef is safe, the beef is safe."
None of this in convincing to Mike Miller.
As far as CWD's connection to CJD, he said, "There's no connection in any way, shape or form." To suggest otherwise, he said, would involve "inference and innuendo.
"There is no evidence that CWD is infective to humans."