My book Brain Trust argues that that this is definitely an American problem. Not only do we have evidence that mad cow disease has been in the United States for over 20 years, but we also have an epidemic of deer and elk disease that has spread like wildfire through nearly 20 states. The Europeans have only to deal with mad cow disease. We have both mad cow and deer/elk disease. With eleven million hunters beginning hunting season as we speak, it is a matter of some urgency that they be warned about possible dangers of field dressing deer/elk or eating venison. Cooking mad cow or mad deer meat does not make it safer to eat. You have to carbonize meat at 600C to make it safe. By the time it is safe it is a black lump of charcoal.
Mad cow disease is caused by an infectious protein called a prion. A prion is not a virus and it is not a bacterium. It is simply a different shape of a protein. Normal prions play a helpful role in the cell, but when they change shape, they can become lethal. They then kill brain cells by the billions.
Prion diseases kill humans (CJD), cows (BSE aka mad cow disease), sheep (scrapie), deer/elk (Chronic Wasting Disease: CWD) and an assortment of other animal species including mink, squirrels, cats, ostriches etc. More worryingly, prions can jump species. This lead to questions for hunters like: can prions jump from deer/elk to humans? Or can prions jump from deer/elk to cattle?
In the United Kingdom in the 1980s and 1990s, hundreds of thousands of cows died from mad cow disease. For years, health authorities issued press releases telling the anxious British public that eating beef was perfectly safe. It was routine for officials to go on television to assure the public there was nothing to worry about. And then the unthinkable happened. Beginning in the 1990s, scores of teenagers and young people in their twenties began to die from eating tainted beef.
Can the same thing happen in the United States?
We will not know until it is too late unless the United States Department of Agriculture (USDA) actually begins to test the beef being sold to consumers. Currently, there are 35 million cattle slaughtered in the United States, and under pressure, the USDA has agreed to begin testing about 200,000 animals per year for mad cow disease. The huge majority of cattle that reach our dinner tables are never tested for mad cow disease. In Japan and in Ireland, every cow that is eaten gets tested and in Europe over 30% are tested. In the US, even with “expanded” testing, less than 1% of animals are tested. What’s wrong with that picture?
Consumer groups have long criticized the USDA for looking out for big cattle interests rather than looking out for the consumer. Their obstinate refusal to test more cattle in the US for mad cow disease is simply because the cattle industry has deemed it unnecessary. USDA scientists argue that more testing is “unscientific”, but Professor Stanley Prusiner, who won the Nobel Prize in 1997 for his discovery of prions, recently stated that the level of testing for mad cow disease in the USA is grossly inadequate. Should we believe a Nobel Prize winning scientist, or should we believe a USDA spokesperson? And when Creekstone Farms in Arkansas decided to test all the cattle in their plants for mad cow disease, the USDA stepped in and, invoking an obscure 1913 law, prohibited the company from testing their cattle!! It is this surreal situation that led to charges by consumers that the USDA does not have the interests of American consumers in mind.
Prions diseases, whether in humans (CJD) in cows (BSE) or in deer/elk (CWD) are 100% fatal. And prions are almost indestructible. They contaminate surgical instruments, even ones that are sterilized. They remain lethally infectious after two years in a pasture. Most viruses or bacteria die within days. Not prions.
And new evidence shows that prions are passed via blood transfusion. Both blood donors and acceptors have died in the UK and in France. A looming question for the health authorities in the United States is: how safe is the American blood supply?
So what is the connection between the human prion disease (CJD) and Alzheimer’s Disease (AD)? Firstly, the astounding recent increase in AD in the United States has not been sufficiently highlighted by the media. In 1979, only 653 people died of AD. In 2002, that number had increased to reach 50,000 deaths. A 9000% increase in deaths for any disease in a mere 25 years should be cause for a national emergency. Instead, old people now are almost expected to die of AD. This was not the case a few generations ago. AD was a rare disease in the 1960s.
AD is quite difficult to diagnose. Mood swings, psychiatric problems, sleep problems, eye problems, memory problems are all loosely associated with dementia. There are some overlaps between symptoms of CJD and AD making them difficult to distinguish during the early stages. CJD kills within a few months of diagnosis, while AD victims take years to die. In both cases, the victims die a horrible death. The gold standard for diagnosing CJD is via autopsy followed by pathology of the brain.
Two shocking studies, one from Yale University and the second from University of Pittsburgh really opened my eyes when I first saw them. When researchers studied the brains of dead Alzheimer’s disease patients, they found that between 5-13% had actually been wrongly diagnosed. These people had really died of CJD. Now, what does this say regarding the supposed rarity of CJD in the USA?
We are routinely told that CJD is so rare, only 1 in a million people get it. But with 4-5 million Alzheimer’s Disease patients putting an enormous strain on the US healthcare system, if even a small percentage of these people actually have CJD, not AD, then CJD is much more common than we have been told. These figures imply thousands of CJD cases in the US that are going undetected because of a lack of autopsies. Recent reports indicate that most pathologists do not want to conduct autopsies on CJD patients for fear of contaminating their facilities with the indestructible prions. And to make matters even worse, CJD is not even a mandatory reportable disease in about half the states in the USA. That means authorities have no real idea of how many CJD cases actually exist.
Recently (October 2004), a suspected cluster of CJD was spotted in Ulster County New York. Several months ago (March 2004), another cluster of CJD was noticed in New Jersey. Clusters are worrying because they may point to an infectious entity. In both New York and New Jersey clusters, the health authorities assured the public that these were “sporadic CJD” and hence there was nothing to worry about. Sporadic CJD has no known cause, the health authorities tell us. Both USDA and CDC tell us that sporadic CJD arises randomly and has nothing to do with eating tainted beef. According to health officials, only variant CJD or vCJD, is caused by eating tainted beef. We are also told that there has never been a case of human mad cow disease in the US.
But is that really true scientifically?
New research from Professor John Collinge in London suggests that some cases of sporadic CJD may also be caused by BSE. This research was conducted in transgenic mice with human prions, so critics have argued maybe the same does not apply to humans. But, if Professor Collinge’s data are indeed true, then it is much more likely that people in the US have died of CJD from eating tainted beef (or venison). We will never know until we (a) dramatically increase the number of autopsies of people dying from “dementia”, AD and CJD and (b) dramatically increase testing for mad cow disease in the US.
If we look at the current low level of testing for mad cow disease in the US and we combine it with the current epidemic of deer/elk disease in this country and with the lack of autopsies to determine how many people in the US are actually dying of CJD, we may be facing a grim reality. When the Europeans, who have just gone through ten years of devastating disease and have lost billions of dollars, look across at the United States, they shake their heads in disbelief.
Surely, we can learn from the mistakes made in Europe?
Brain Trust was published on October 19, 2004 and is currently in bookstores nationwide. For more information, click on: http://www.colmkelleher.com
The Author, Colm A Kelleher PhD, is a research scientist. Currently in the biotechnology sector, Dr. Kelleher is a biochemist with a fifteen-year research career in cell and molecular biology. Following his Ph.D. in biochemistry from the University of Dublin, Trinity College in 1983, Dr. Kelleher worked at the Ontario Cancer Institute, the Terry Fox Cancer Research Laboratory, and the National Jewish Center for Immunology and Respiratory Medicine. Before moving to the biotechnology sector as a senior research scientist, Dr Kelleher worked as project manager and team leader at a private research institute, from 1996-2004, using forensic science methodology to unravel scientific anomalies.