The 'Mad' Matter 

• Linda Faillace with her controversial sheep

Vicki Soukup saw her mother-in-law metamorphose over a matter of weeks from a proper, British-born woman of taste to a bewildered invalid who no longer recognized her own family. The disease struck like Alzheimer’s in fast-forward. Her mother-in-law grew confused and agitated and then quickly declined. Over one weekend, the young-looking and active 75-year-old “lost the ability to toilet herself, bathe herself. She lost all orientation with reality,” Vicki Soukup recalled. “Her mind just got fried.”

Joyce Soukup died on November 18, 1998, about five months after she became ill. Her death was a blessing of sorts, since it ended her horrible suffering. Now Vicki Soukup, who lives near Cleveland, Ohio, is left with many unanswered questions. How did her mother-in-law get sick? Was it “mad cow” disease, contracted by eating infected meat when she lived in and later returned to visit England, where the mysterious brain-wasting sickness originated? Soukup questions whether U.S. health officials have overlooked cases of the disease in this country. And she asks if government agencies are vigilant enough to stop its spread in the United States.

“We need to do more research, get it identified, get more people to understand it’s out there,” she said.

For years, the number of mad cow cases held more or less steady. But recently, the disease is on the loose again, stampeding through the United Kingdom and across the European continent, decimating the beef industry in several countries and dashing hopes that the epidemic first seen in the 1980s had peaked. With this and a recent outbreak of hoof-and-mouth disease — and, in Vermont, Johne’s disease — there couldn’t be a worse time to be a cattle farmer.

American health officials have taken strong measures to make sure mad cow in particular does not cross the Atlantic. Most experts agree that the risk for U.S. cattle and consumers remains extremely low. Yet some critics feel the precautions in place may not be enough. The disease is going global, spread by international trade and the bizarre practice of turning cattle into carnivores by feeding rendered animal protein back to bovines. And the U.S. for years allowed a host of questionable food safety practices that could ulimately facilitate its spread.

Sheldon Rampton, a Madison, Wisconsin, researcher and co-author of the book Mad Cow USA: Can the Nightmare Happen Here? has followed mad cow disease for more than a decade, long before the bizarre illness dominated headlines in Europe and at home. He said government regulations designed to prevent the spread of the disease are not being followed.

“If they continue the lax policies with regard to feeding rendered animal products to other animals, they are just leaving the door wide open to an outbreak in this country, as they have seen in Europe,” he said.

In the past few months, infected animals were found in Austria, in Spain and at an Italian slaughterhouse that supplies the McDonald’s fast-food chain. Belgium has acknowledged 21 cases. And Germany, long proud of its safety measures to avoid the contagion, was shocked by its first case in November and has since uncovered more than a dozen infected animals. As the beef industry evaporates and consumers shun sausage and steak tartare, some restaurants on the continent post signs announcing that only U.S. beef is served.

Halting the outbreak will be costly, not just for meat packers and farmers, but for European taxpayers as well. Beef sales have dropped 27 percent across Europe, while the cost of containing the disease and compensating farmers could reach $20 billion, according to a recent BusinessWeek estimate.

If the disease took hold in the United States, the financial — and potential public health — impact would be even greater. Bovine products are used for food, but also for pharmaceuticals, cosmetics, lubricants and other products. The beef industry is the largest sector of the U.S. agricultural economy and a pillar of American trade. Any hint that mad cow had surfaced here could send the industry into a tailspin.

Big money is at stake — and so are human lives. An advisory committee to the U.S. Food and Drug Administration just over a month ago responded to the outbreak in Europe and recommended banning blood donations from people who had lived 10 years or more in France, Ireland or Portugal. People who lived in Britain for six months or more from 1980 to 1996 are already banned from giving blood. No one knows if the human version of mad cow disease could be spread by transfusions, but animal studies suggest it might. The disease can strike decades after exposure, and the government and nonprofit blood centers are taking no chances.

It’s better to limit blood donations now “than to look back five years from now and say this is what we should have done,” said Carol Dembeck, a spokeswoman for the Red Cross blood center in Burlington. The recommendation to protect the domestic blood supply is one of many preventive steps taken by U.S. officials — measures that have included the planned seizure of two flocks of Vermont sheep suspected of harboring the disease. The owners, Houghton Freeman of Stowe and Linda and Larry Faillace of Warren, have fought a government seizure order in court, and the appeals have played out in the Vermont media for months. U.S. District Judge J. Garvan Murtha ruled recently that they must surrender their animals for slaughter.

This week a federal appeals court in New York denied the owners’ last-minute request for a stay of execution, but have set a date for yet another appeal to be heard.

The sheep case is an example of the government acting with extreme caution in the face of unknown risk. Although sheep have never been infected with so-called mad cow disease outside of laboratory experiments, the USDA says the animals or their ancestors may have been exposed to tainted feed before they were imported from Europe. Tests on four of the animals from Freeman’s farm in Greensboro showed signs that the sheep were infected with some version of transmissible spongiform encephalopathy (TSE), the family of illnesses that includes mad cow disease and a far more common sheep version.

A scientist testifying for the USDA could not say last summer which TSE the animals had. But the flock owners say the tests were inconclusive and lacked proper scientific controls, and that none of the sheep — or their ancestors in Europe — have shown signs of infection. Congress appropriated $2.4 million last fall to purchase the flocks, totaling 276 sheep, at a per-animal cost of $8700. The Vermont shepherds turned down the offer, saying the amount did not come close to the value of their investment.

Agriculture and health officials have banned European meat and bone meal from entering this country, barred imports of beef from the United Kingdom and other countries, and have tested U.S. cattle that show signs of illness. But the blockade erected to prevent mad cow disease from crossing the Atlantic may not be working.

If the disease is here, the conditions that fostered its spread in Europe existed in this country until recently as well, according to a report issued last summer by a scientific advisory commission to the European Union.

Mad cow disease swept through the British cattle herd — and later, experts believe, infected European livestock — through the practice of feeding dead animals to bovines. Turning cows into cannibals solved a nasty waste-disposal problem for the meatpacking industry. Organs and other offal left after an animal is slaughtered can be converted into high-protein pellets and used to supplement plant-based feeds.

Scientists believe that mad cow disease can occur spontaneously through natural mutations in about one in 1 million animals. A British government commission concluded this fall that the disease spread to tens of thousands of animals in England when sick cattle were recycled into feed in the early 1980s. Another theory blames the outbreak on diseased sheep that were turned into cattle feed.

In 1997, the FDA outlawed the practice here of feeding ruminants — such as cattle, sheep, goats and deer — to other ruminants. But before that time, U.S. feed mills had few controls to prevent potentially contaminated products from entering the mills and being distributed throughout the country, the EU report said.

The report’s authors concluded that a mad cow disease outbreak in the United States was “unlikely,” but nonetheless possible. The report could not rule out that U.S. “cattle are clinically or pre-clinically infected” with the disease-causing agent. (“Pre-clinical” refers to an animal being infected before it shows signs of illness that could lead to a diagnosis.) And the feed ban — designed to prevent infected cattle or sheep from infecting other animals — has been poorly enforced. FDA officials, who are responsible for ensuring the safety of animal feed, admitted in January that nearly a quarter of the 180 large companies that render cattle and sheep were not properly labeling their products.

In addition, these companies did not have a system to prevent commingling of ruminant-derived feed with other products. Some 6000 to 8000 feed mills are so small they do not require FDA licenses. They are nonetheless subject to the regulations, and of 1593 small feed producers that handle ruminant material and have been inspected, 40 percent were not using approved labels and 25 percent had no system in place to prevent commingling, officials said.

Meanwhile, potentially tainted animal products have continued to reach U.S. shores, despite a 1989 import ban on most British beef products. The EU scientific advisory commission disclosed last summer that 37 tons of meat and offal were sent from the United Kingdom to this country in 1997, after the shipments were supposedly outlawed. In December, the USDA imposed a new ban on “rendered products” from Europe or from any country where mad cow disease is present.

But loopholes remain. Manufacturers of health supplements are still allowed to use imported glandular material, and are not required to list country of origin on their labels. Other imported beef products that can enter this country include blood, fat, gelatin, bone mineral extracts, tallow, collagen and amniotic fluid.

Some common vaccines used to inoculate millions of people also pose a theoretical risk because they contain European bovine products. The FDA recently warned that several widely used vaccines were produced using bovine-derived material from countries that could harbor mad cow disease. The agency did not order a recall, but recommended that manufacturers phase out use of the suspect ingredients.

The global trade in food and animal products has hastened the spread of mad cow disease to Europe and possibly beyond. The United Nations recently estimated that at the height of the epidemic in the United Kingdom, British companies exported at least 500,000 tons of untraceable bovine byproducts to Western Europe and other nations, including the United States. “The potentially contaminated material could be repackaged and re-exported from these countries,” Maura Ricketts, of the World Health Organiz-ation’s animal- and food-related risk unit, told the Knight-Ridder news service.

There is also an issue of spontaneous cases of mad cow disease in this country and questions about whether those sick animals could get into the U.S. feed supply or be served up as steak. The question is not whether the disease is here, according to some experts. The numerical odds almost guarantee that it is. Since TSEs occur spontaneously in one in a million animals, and 36 million cattle are slaughtered each year, 36 animals are probably infected annually, and some of them could have entered the food system, despite stringent safety regulations.

“Will [mad cow disease] ever come to America?” asks Pulitzer Prize-winning author Richard Rhodes in Deadly Feasts, his 1997 book that chronicles mad cow disease research. “The answer seems to be: It’s already here, in native form, a low-level infection that industrial cannibalism could amplify to epidemic scale.”

Vicki Soukup has seen first-hand the suffering that would take place if the disease spread in this country. In her mother-in-law’s case, doctors first suspected Alzheimer’s, but she was ultimately diagnosed with Creutzfeld-Jakob disease (CJD), a rare human malady closely related to mad cow disease.

Joyce Soukup’s decline occurred in a matter of weeks, but even as her brain deteriorated, she had moments of lucidity. She loved to play crossword puzzles. One day, as her husband sat in her room completing a game, he tossed out the clues to her. Something clicked and her mind responded. “She didn’t have a clue who he was, but she could fire back the answers,” Vicki Soukup said.

The disease changed her mother-in-law’s personality. “She didn’t know who she was. She just panicked. One time she picked up her treasured antiques and began throwing them around,” Vicki Soukup recalled.

Mad cow disease is a member of the always-fatal TSE family of illnesses. The sheep form of TSE is called scrapie, while deer and elk are susceptible to a version called chronic wasting disease, or CWD. Cattle get a version called bovine spongiform encephalopathy, or BSE. The strain that has crossed the species barrier from cows to people is known as new variant Creutzfeld-Jakob disease, or vCJD.

TSEs are particularly frightening because of their potentially long incubation period. People exposed to the human version, for example, can be healthy for 30 or more years before it strikes. Eighty-eight people in the United Kingdom and in Europe have died or are dying from vCJD.

Two types of TSEs are endemic in the United States. Scrapie has infected sheep here since 1947 and has been found in 45 states. Chronic wasting disease has struck elk and deer in Colorado, Wyoming, South Dakota and Oklahoma. In Canada, authorities destroyed 1700 elk last year after they were exposed to the disease. As Seven Days reported two years ago, scientists are trying to determine whether chronic wasting disease could spread from elk or deer to humans.

Three Americans under the age of 30 who had Creutzfeld-Jakob disease ate deer and elk meat when they were young, according to the Centers for Disease Control and Prevention (CDCP) in Atlanta. The deaths were unusual, since CJD rarely strikes young people — although in England most of the victims of vCJD were in fact young.

The three U.S. cases “suggest a possible relationship with CWD,” but investigators found “no strong evidence of a causal link” with the patients’ illnesses, CDCP’S Dr. Ermias Belay told an FDA advisory panel in January.

To make matters even more eerie, the family of diseases that killed Joyce Soukup and decimated the British beef industry are spread by an infectious agent that is not “alive” and thus cannot be “killed.”

Researchers believe that TSEs cripple and kill their victims when a protein normally found in the brain folds into an abnormal shape and then triggers other proteins to do the same. As these abnormally shaped proteins — dubbed prions — grow, they leave dead spaces inside the brain tissue, giving it a sponge-like appearance. Prions can multiply out of control, yet they lack the RNA or DNA that are considered the building blocks of all life. Prions can remain lethal despite conventional sterilization techniques, heat up to 600 degrees Celsius and blasts of radiation.

Medical studies show that, like mutant TSEs in cattle, CJD occurs spontaneously in about one person out of 1 million. But Vicki Soukup thinks many cases are missed, as her mother-in-law’s nearly was.

Doctors are not required to report cases of Creutzfeld-Jakob to federal health officials. And Soukup said elderly victims may be misdiagnosed as suffering from senility or Alzheimer’s. “I believe it’s undercounted,” she said. “How many cases are treated as Alzheimer’s?”

Research supports Soukup’s concern. A 1989 study at the University of Pittsburgh found that when patients diagnosed with Alzheimer’s or some other dementia were autopsied, 5.5 percent in fact had died of CJD. A similar study at Yale turned up CJD in 13 percent of the cases that were initially classified as Alzheimer’s. Michael Hansen, a researcher at Consumers Union, a nonprofit organization that publishes Consumer Reports magazine, agrees that many CJD cases are probably overlooked.

“Since there are over 2 million cases of Alzheimer’s disease currently in the United States, if even a small percentage of them turned out to be CJD, there could be a hidden CJD epidemic,” he said in testimony before the FDA.

Joyce Soukup lived in England for several years in the 1970s, near a village where epidemiologists have found a cluster of vCJD victims who apparently contracted the illness from eating local beef products. She returned to visit the area frequently in the 1980s. It’s “right where she was,” said Vicki Soukup.

But because Joyce Soukup was not autopsied, no one will ever know for sure if she was the first U.S. victim of mad cow disease. A conclusive diagnosis of vCJD can be made only by examining brain tissue.

Dr. William Pendlebury, a University of Vermont pathologist, has his doubts. A specialist in diagnosing Alzheimer’s disease and other neurological disorders, he doesn’t buy the idea that many CJD cases are misdiagnosed as Alzheimer’s. The two illnesses have quite different characteristics, he said. CJD causes a much faster decline — those afflicted die within months, while Alzheimer’s patients can live for a decade or more. CJD can be identified by measuring the electronic brain waves of its victims, he said. And scientists have recently developed a test that analyzes a spinal fluid protein that is considered a marker for CJD. “Given the tools that we have to look at clinical diagnosis, it’s highly unlikely to mistake the two,” Pendlebury said.

Doctors can also distinguish the variant vCJD — the strain believed to be caused by eating infected cows — from regular CJD, according to Pendlebury. Patients with vCJD lack the characteristic brain-wave pattern seen in those with the more common strain; they often live longer, and the disease seems to strike younger people, he said.

British researchers were interested in Joyce Soukup’s case because of her proximity to the village where the cluster of vCJD has been found. The doctors discounted the possibility that she had that disease, however, because of her age. But then a week later, vCJD was confirmed in a 79-year-old patient.

Pendlebury noted that vCJD has killed fewer than 100 people in the United Kingdom and Europe, and that Britain has eliminated the feed practices that fostered the spread of mad cow disease. He believes the number of cattle cases will dwindle, and that its human analogue will also decline.

“I think it’s premature to predict this is going to become a significant human health problem,” he said. “I know there are some experts who are convinced that it is. …But my guess is that the new variant form will die out, as mad cow disease in England and the United Kingdom is dying out, and that it won’t amount to a major epidemic.”

That doesn’t help the Faillaces or the Freemans, though, who feel their healthy animals are being used literally as sacrificial lambs in the government’s zeal to protect the domestic beef industry. U.S. officials are concerned that overseas markets for American meat will evaporate if the Vermont sheep are allowed to live, according to internal memos.

Officials noted that Japan and Korea suspended trade with Canada after one cow was found to be infected in 1999. “Japanese officials are monitoring the Vermont sheep case. Japan is the largest export market for U.S. beef, and imports approximately $1.4 billion annually,” one USDA memo says.

For now, all ag eyes are on Vermont. The death-row sheep threaten the reputation of the U.S. beef industry, which so far has escaped the blight and market panic that followed the discovery of more mad cow cases in Europe, said Leon Graves, Vermont’s commissioner of agriculture. “I have a sickening feeling the genie is out of the bottle in the United Kingdom,” said Graves. “The USDA has worked very hard to manage this to make sure we don’t have a situation like that.”