Is the Beef We Eat Safe? A Critical Assessment of Mad Cow Disease
by Ben Dobson

Bovine Spongiform Encephalopathy, or BSE, simply sounds dreadful. What is does is catastrophic. This particular strain can change large land-moving mammals into drooling, wobbling, belligerent beasts. Different variations of BSE, which part of a family of deadly neurological disorders called transmissible spongiform encephalopathies (TSEs), can infect sheep, deer, and elk. This disease is also known as Mad Cow Disease and has sparked fears of eating beef in Britain, where most of the confirmed cases have arisen.

However, there is a part to this story which has received enormous attention in Europe and Canada, but has scarcely been reported in the United States media; it's a disease called variant Creutzfeldt-Jakob Disease (vCJD). It is nothing short of the human equivalent, a mutation of BSE, that can strike humans and cause the same debilitating symptoms which invariably lead to death that are seen in our bovine counterparts.

The Ottawa Citizen reported the story of Zoe Jeffries. An otherwise health, athletic and popular young woman, she "first turned nasty. Then she became a crying, screaming child afraid of shadows. Finally Zoe was unable to walk, talk, feed herself or control her bowels." It turns out, after an autopsy, that Zoe's brain has become "sponge-like, riddled with tiny holes." Authorities attributed her condition to the British beef she had eaten her whole life.

The disease vCJD is believed to be caused by "abnormal mutation of protein molecules, known as prions, found in cells." Normal prions exist throughout the human body, and in particular, can be found in the brain and spinal cord. But when even a single protein is folded into an abnormal shape, it becomes a "rogue prion which is replicated throughout the body. It eventually attacks the brain, leading to neurological breakdown and death."

Aside from the description of these prions that cause vCJD, there is another startling fact: the immune system is not capable of combating the rogue prions. Scientists have tried "freezing, boiling, burning, soaking in chemicals, and exposing them to devices that normally kill other infectious agents." Unfortunately, prions survive. Professor Brian Heap, who chaired a working group on a report by the Royal Society and Academy of Medical Sciences in Britain, told the London Times that "according to one research report, some prions ... may still be active even after heading to 1112 degrees Fahrenheit." Right now, there are no tests available to confirm vCJD aside from doing a post-mortem autopsy. As such, anyone who has vCJD in their bloodstream may contaminate the blood supply. Surgical instruments which normally would be disinfected of all other agents, could still have active prions on them.

Finally, the most terrifying part of the disease, second only to its incurable consequences, is its stealth-like qualities. European microbiologist Dr. Stephen Dealler told the Belfast News Letter in May that, "Prion diseases develop over a quite different time-scale [than normal infectious agents] and we cannot rule out an epidemic that evolves over decades." Professor John Collinge, at the Imperial College School of Medicine, has done research on the incubation period of vCJD. He told the Bloomberg news Service that "the incubation period for the human form of mad cow disease may average 30 years, with some patients not showing symptoms for as long as 50 years..."

A conference was held beginning on June 11, 2001 which was sponsored by the United Nations to get all the data on the table about the potential for the human spread of vCJD. The conference was told that the human form of mad cow disease has joined AIDS as a major public health challenge facing the world. Dr. David Heymann from the World Health Organization told the conference that there are many "striking similarities between mad cow disease and AIDS, which exploded onto the state 20 years ago as a frightening disease that baffled scientists." He noted that "both have a very long incubation period, one which permitted the disease to be widely distributed before it was even recognized."

With that in mind, the channels for mad cow disease to be transmitted is much wider than anyone in this country cares to believe. First, the Ottawa Citizen reported that cattle had been fed the protein-rich remains of other cattle. This is a common practice in the West, including the Untied States. As such, "part of the rendered product was turned into a dried powder called meat and bone meal, that could be added to animal feed. It improved the milk output of dairy cattle and added to their muscle mass. The danger, however, was that the pooled products could quickly spread the disease. This practice has since been banned, but there is no way to tell what, if any, infected beef may have gotten into our food supply by this manner.

Plus, there are other ways that vCJD may be spread that eludes the best regulations to-date over how cows are raised, and beef is served. The Ottawa Citizen found evidence that cosmetic creams and vaccines that used British beef ingredients during processing could be transmitters, plus some dietary supplements which are largely unregulated in the U.S. and Canada may include powdered cow brains and glands. Unclean surgical instruments and donated blood and organs from those unknowingly incubating vCJD could be transmitting the disease.

There is an assumption that BSE does not exist anywhere in the United States, and that it is limited to Britain. However, cases have already sprung up in France, Ireland and Hong Kong. While each of those instances have been traced to eating British beef, it demonstrates how this potential epidemic could so easily cross international borders. And if the incubation period is so long, people could have been harboring this disease long before any safeguards were put into place.

There are still many unanswered questions regarding this disease. Even the question of whether the disease will become an epidemic is in dispute, although the consensus seems to be that many more people will succumb to the disease. The division is whether the death roll will be in the hundreds, or in the millions.

The Ottawa Citizen laid out several unknown variables that could have a significant effect on how big a deal vCJD becomes. First, there are still lingering questions about how long the incubation period is for the disease. If it is ten to fifteen years, then perhaps we've already seen the peak. If it is twenty to thirty years, as has been strongly suggested by European scientists, then this may be the metaphoric tip of the iceberg.

Another question is whether the disease strikes down everyone, or if there is a particular genetic make-up that might make people more susceptible. So far, "all of the vCJD victims have shared a similar genetic make-up, one that occurs in about 40 percent of the Caucasian population. If only they can get vCJD, this will limit the toll." Still, scientists caution that this particular susceptibility may simply allow the symptoms to show up sooner than in most people.

Finally, no one knows the "threshold" for getting the disease. For example, "how much BSE- infected beef has to be eaten before a human gets vCJD -- one hamburger or hundreds of meals over several months or years?" Further, where the meat comes from on the cow may make a difference; burgers, sausages and meat pies are ground-up and may "contain fibers from the nerves, where the BSE prion is clustered." Filet mignon, on the other hand, would be considered less risky.

Finally, there is the question of whether BSE may be present in the United States. Testing has been extraordinarily limited, and some experts believe that not enough has been done to have an adequate sample of cows tested to ensure that there is truly no chance of BSE in the beef Americans eat. Right now, according to the Ottawa Citizen, "Canada and the U.S. use ‘surveillance' systems to identify a cow with BSE. Any animal showing symptoms must be killed and the brain tested." Consider that in Canada, there are 14.9 million cows. In a seven-year span betwen 1992 and 1999, only 4,512 cows were tested for BSE. One tested positive. Consider that in the first two months of this year alone, "the European Union tested 900,000 cows for BSE." In Britain one expert told the Ottawa Citizen that as far as Canada goes, "They won't test because they don't want to find it. They won't know what to do. They would panic."

Panic is a major fear that governments face, and may be a reason why more is not being done. When vCJD first became a problem in England in the 1980's, the head of the Ministry of Agriculture, Fisheries, and Food's central veterinary laboratory's pathology department, Ray Bradley, wrote an internal memo: "If the disease turned out to be bovine scrapie it would have severe repercussions to the export, trade, and possibly also for humans if, for example, it was discovered that humans with spongiform encephalopathies had close association with cattle. It is for these reasons that I have classified this document confidential. At present, I would recommend playing it low key."

To date, there is no evidence of a similar cover-up in the United States. However, it is worth asking whether the U.S. government might try to conceal a major health threat like this, protect the beef industry which is enormously powerful, and keep a very low profile on this matter. It is also worth wondering whether the absence of these news items from the mainstream U.S. press (save for a Newsweek cover story which reassured readers that the U.S. supply of beef is likely safe) has anything to do with what happened after Oprah announced she would no longer eat beef because of Mad Cow concerns. The meat industry sued her. Media companies realize that a significant portion of advertising revenues come not just from the beef industry itself, (Beef! It's what's for dinner!) but by a wide variety of fast food companies that make their money off of selling these processed meat products.

Right now, there are no cures for vCJD, nor are there any treatments. The only way to test for it is to actually examine the brain after death occurs. For the present, this is an invariably fatal disease. However, science has concluded that there is a sufficient threat for an epidemic that money could be made with new diagnostic tools and treatments and cures. First, blood tests may soon be able to pick up the vCJD in humans. Right now, the prions are too small to be detected, but a new technique developed by Claudio Soto and his colleagues at the Serono Pharmaceutical Research Institute in Geneva have found a way to "amplify the offending prion" for detection. In the meantime, a team at Oxford University, led by pathologist William James, is using artificial strands of RNA to stick to molecules of a given shape. In this case, they would bond to the prions making them easier to detect. This team is working with VITEX corporation out of Watertown, Massachusetts to produce a regular blood-testing kit that could screen blood supplies and provide a quick medical diagnostic tool for the primary care physician.

Talk of a cure follows similar lines. The VITEX company also plans to remove prions from blood by pasing it through huge columns containing aptamers -- that system would "mop up any infectiou molecules on the way through." That might provide a form of treatment for humans, not unlike a regular kidney dialysis appointment.

Yet another study, published in the NewsRX online newsletter, works on this premise, "that the most likely route of infection of BSE and vCJD has been through the food chain -- eating products contaminated with abnormal prion protein. This infectious prion then replicates in lymph tissues before moving through the nerves to the spinal cord or brain stem. Thus, anything that could impair replication of abnormal prions in the lymph nodes would delay the onset of clinical signs of this type of disease." Researchers in Switzerland and Scotland believe they have discovered that using cobra venom could have a dramatic effect on depleting the types of prions that lead to vCJD.

It is also true that there are no publically documented cases of BSE in the United States beef population. However, the best solutions to pursue require plenty. First, there must be stepped-up screenings in this country. With the trend towards globalization, no nation can be immune from this threat. Second, money should be spent to research the disease, find out which genetic composition may be most susceptible to attacks on the neurological system by prions. Public education is another area that should be assessed, and the government should not be afraid to work to ensure that our food supply is completely 100% BSE-free. Further, media companies should not avoid the topic. Instead, meat companies should welcome the scrutiny, since they have said all along that there is no threat to humans and that our meats are the safest in the world. Through these steps, a potentially explosive situation may be reasonably managed.