Lyman said a mad cow outbreak on U.S. turf would make AIDS seem like the common cold. His candor inspired Oprah to utter: "It has just stopped me from eating another burger!" It also inspired Engler & Cactus Feeders cattle company to sue Winfrey, Lyman and Harpo Productions for 12 million dollars each. But U.S. District Judge Mary Lou Robinson threw out the Texas cattlemen's libel angle. Today, Lyman may sport an I-told-you-so smirk. With almost 100 Europeans dead and cow chaos on the rise, the present question is not what if mad cow disease lands on U.S. shores-but how long before it does?

In 1986, the United Kingdom's first bad bovine led to the 15-year slaughter of 3.7 million cows. Currently, bovine spongiform encephalopathy (BSE) rages across Europe with World War III ferocity. In February, the German government authorized the massacre of 400,000 cattle. The Irish plan to terminate 300,000 by June. With sick cows in France, Denmark, Poland, Spain, Austria, Belgium and Italy, the European Union intends to kill 2 million more animals.

In the meantime, British veterinarians armed with insecticides and bullets hope to avert hoof-and-mouth disease, a non-fatal, transmutable virus (with human symptoms similar to a bad cold) found in cloven-hoofed animals such as cows, pigs, goats and sheep. Though Dutch authorities intend to vaccinate herds, Britain's Ministry of Agriculture so far plans to wipe out up to half of Britain's 63 million livestock. Under international terms, countries that inoculate livestock forfeit their disease-free status and cannot export their livestock on world markets.

Amid such ominous forecasts as "food scarcity" and "catastrophic profit loss" authorities impulsively kill and reluctantly vaccinate. Yet no one has addressed the factory farm environment, a literal breeding ground for contagious infections. Every year 10 billion animals are confined without enough room to stand or move comfortably. Artificially fattened animals, filled with antibiotics, hormones and steroids, have depressed immune systems. Most are castrated, de-beaked, de-clawed, and de-tailed until too mutilated to struggle. On the killing floor, harried workers frequently fail to stun animals and often dismember and skin them alive.

Almost a decade after Britain banned ground meat and bone (MBM) as feed for cows, the U.S. Food and Drug Administration imposed a similar restriction. The 1997 USDA ruling, which let producers mix bovine proteins in poultry and pig feed, was a sketchy precaution at best. No one knows if mad cow disease can cross over from cow-to pig, sheep or chicken-and back again.

Last December, the USDA barred all European animal protein products. Thus, our fail-safe barricade against mad cow or hoof-and-mouth disease boasts an operating time of 5 months. InJanuary the FDA reported that 20% of U.S. feed plants ignore preventative guidelines and many fail to adequately label goods.

In our international economy, it is nearly impossible to track the conduits of disease. Professor Steve Best describes how an animal bred in Britain may be "fattened in France, slaughtered in Spain and eaten in Ecuador." To stem the spread of hoof-and-mouth, USDA inspectors spray British air travelers with a bleach-water brew and search their bags for contraband meat. Forget drugs. Smuggled meat now detains passengers and carries $100 (or more) in fines.

American consumers are assured that cows are safe for dinner. But what about venison or lamb? To curtail widespread panic, variations on the mad-cow theme get scant publicity. BSE falls within the disease group "transmissible spongiform encephalopathies," or TSEs. Brain-ravaging TSEs can attack sheep, deer, elk, cows, mink, cats, squirrels, monkeys, humans and other species. An affiliated TSE disease, chronic wasting disease (CWD), now afflicts 4 to 8% of deer and elk herds between Colorado and Wyoming.

In 1999, CWD emerged on a commercial elk farm near Philipsburg, Montana. Though health authorities swiftly killed 81 elk, CWD's robust disease catalyst, a prion protein, can withstand standard decontamination methods. Can CWD pass from deer and elk to humans? The species-barrier theory applied to mad cow disease in Britain proved false.

Last year, Doug McEwan of Utah displayed motor control loss, mood swings and confusion. The 28-year-old's brain biopsy verified Creutzfeld-Jakob disease. McEwan was one of 3 young hunters profiled in a Boston Globe article. A Centers for Disease Control statistician informed reporter Terry Allen that another under-30 CJD victim "might tip the balance" in America.

Last summer, an Indiana man with a taste for beef-brain sandwiches succumbed to CJD. Forensic pathologist John Hiedingsfelder told the Evansville Courier he'd observed 3 CJDcases in one year. To date, mad cow disease is not linked to the Indiana victims. The cattleman's association allegedly warned staff writer Roberta Heiman to lay off the sandwich stories.

Scrapie, the sheep variation, has shown-up in herds around the country. In March, federal agents snatched the second of two suspect dairy flocks in Vermont, prompting protesters outside one family farm to give them the Nazi salute. Though the sheep's owners cited faulty tests, U.S. officials packed 126 animals on death-trucks bound for Iowa.

Despite every knee-jerk effort, mad cow and hoof-and-mouth diseases endure. By the time this column is published, new outbreaks will headline the news. Most reports will overlook the obvious way to avoid tainted meat: the transition to a healthy, ecologically sustainable plant-based diet.

Unlike true carnivores, humans don't salivate over living animals. We purchase their cellophane-wrapped remains in the supermarket. According to cardiologist Stephen Lenhoff, the human body is designed to be vegetarian. "If you look at herbivorous animals, they have longer guts. The longer guts are to absorb nutrients. The human small intestine is 26 feet long. Carnivorous animals have short guts."

Humans clog their long and winding intestines to fulfill a conditioned craving for meat's smell, look and taste. A "beautiful cut of meat" symbolizes prosperity. Meat is the centerpiece of Sunday barbecues, family reunions, and other nurturing acts of love. Children learn to separate the flesh and bone on their plates from the gentle creatures who provided it.

But our meat-addicted society has a few glitches. Up to 1 billion people go to sleep hungry, while affluent countries feed 16 pounds of grain to cattle to produce just 1 pound of beef. Livestock eat enough grains and soybeans to feed 1.3 billion people and they guzzle 50% of the water supply. Chicken factories alone can use up to 100,000 gallons a day.

The meat industry also leaves the stench of 68,000 pounds of manure per second. A report compiled by the U.S. Senate Committee on Agriculture, Nutrition and Forestry revealed that livestock produce 130 times as much fecal matter as the entire human population.

We are a bandaid-culture, committed to quick fixes rather than long-term solutions. We can arbitrarily poison, shoot and burn millions of animals, until their carcasses litter the earth and their souls ascend in a memory of rising smoke. For what? To ensure a "few seconds of pleasure in one of the 5 nerve endings of the human tongue," writes humanitarian author Michael Tobias.

The meat-disease epidemic is merely another indicator that modern agribusiness is nonsustainable. The need to overhaul intensive confinement farming transcends animal rights, Lyman concludes. "It has to do with common sense-with making the world sustainable for yourself, your children and your grandchildren."

by Brenda Shoss, whose website is KinshipCircle.org
To subscribe to Kinship Circle Letters for Animals, email: subscribe@kinshipcircle.org

Related Links

• Fact Sheet: Downed Animals
  http://www.kinshipcircle.org/fact_sheets/DownedOnTheFarmPHOTOS.pdf
• Horton Hears A Cow!
  http://www.kinshipcircle.org/fact_sheets/HortonHearsACow.pdf
  
• Mad Cowboy
  http://www.kinshipcircle.org/fact_sheets/MadCowboy.pdf

The research paves the way for a simple blood test "to eliminate infected animals from the human food chain, even before the onset of clinical signs," said the report, published this month in the Oxford Journal Nucleic Acids Research.

Eating meat from animals infected with BSE (bovine spongiform encephalopathy) has been linked with a new form of the fatal, brain-wasting Creutzfeldt-Jakob disease, which has killed at least 200 people worldwide.

Currently the only way to accurately test for BSE, and for similar wasting diseases that affect sheep, elk and other relatives of cattle, is to dissect each animal's brain after slaughter, said principal investigator Christoph Sensen of the University of Calgary.

More tests are needed, Sensen told AFP in a telephone interview, but he predicted that within a few years slaughterhouses will conduct routine tests on all animals to rule out the disease.

Each two-dollar test could potentially cover 10 animals and take about two hours, he said.

"It's a fairly simple thing ... it could be done by veterinarians in slaughterhouses as animals go in, as part of the pipeline that processes beef.
It's a very promising breakthrough because it tests blood instead of brains, and live animals instead of dead animals," he said.

The scientists infected elk and cattle by feeding them several grams of infected brain material. The rate of both infection and fatality was 100%, noted Sensen. "It's very dangerous, deadly stuff," he said.

As the elk infected with Chronic Wasting Disease took about two years to sicken and die, scientists drew blood from each animal monthly and tested circulating nucleic acids, said Sensen.

Months before the infected animals showed the first visible symptoms of disease, he said that scientists detected patterns in the blood - genetic material unique to diseased animals, and not found in healthy animals.
The genetic changes were caused by the creature's responses at a molecular level to the stress of the infection. “We are measuring what genes are being turned on, and when," he said.

“Full testing of the cattle infected with BSE will take longer than tests on elk, partly because cattle take longer to die,” said Sensen, adding that the researchers are also developing tests for bison, sheep and other breeds of cattle.

In the future, said Sensen, the livestock tests may help human doctors diagnose Creutzfeldt-Jakob disease, adding that it could "also be used in other chronic diseases, like Alzheimer's and Parkinson's."

The research team included scientists from the University of Calgary, Alberta and Canadian government agencies, Germany's Federal Research Institute for Animal Health and the University of Göttingen, and Chronix Biomedical of the United States.

Wasting diseases are an economic as well as a human health threat. Infections have been periodically detected in cattle throughout much of Europe and North America, leading to global import bans from affected countries and mass slaughters, including about 4.4 million animals killed during a 1990's outbreak in Britain.

The bad news came with the death of an elderly patient in Britain two years ago. While seemingly unremarkable, this was automatically the subject of an autopsy because the patient had a blood transfusion in 1999 from a donor who had died later from the human form of mad cow disease: variant Creutzfeldt-Jakob disease (vCJD).

In Britain, paranoia is rife about human-to-human vCJD transmission via blood. This disease is the newest type of prion disease in a group that includes bovine spongiform encephalopathy (BSE). This automatic look-back policy on British blood product recipients proved a grim genetic revelation, as well as the first solid indication of what experts had feared: that the BSE epidemic that has wiped out millions of cows was entering a new phase - secondary transmission through humans.

Reported in 2004, elderly patients were the second probable case of blood-borne vCJD transmitted between humans. The first was identified months earlier, in December 2003, in a 62-year-old man who died of vCJD six years after a blood transfusion. This man's blood donor had been an apparently healthy young man who died of vCJD three years after the donation.

A third blood transfusion-related case was identified in March this year. All three were in Britain, the epicentre of the declining but continuing two-decades-old BSE epidemic in which more than 184,000 cows have been diagnosed with BSE and millions more have been slaughtered as a precaution.

Although the patient died of unrelated causes, the spleen in the second case was "loaded" with abnormal prion protein, according to one expert. Prion protein is a Jekyll-and-Hyde protein. In normal brains it helps create nerve cells. In diseased brains it changes shape, misfolds and causes terminal damage.

In vCJD, unlike other types of CJD, it starts in the lymphoreticular tissue. This includes the spleen, appendix, lymph nodes and tonsils, and takes years to reach the brain where the abnormal prion creates tiny "spongy" holes.

The second case marked a turning point in the history of vCJD, which burst into the annals of disease just a decade ago (March 20, 1996) with the announcement in the House of Commons that a new disease had been identified in 10 young British people and was linked to the BSE epidemic.

Despite no symptoms of vCJD, the second patient was carrying the infectious rogue prion and was a danger to anyone who received that person's blood, organs or tissue, or whose surgery followed any procedure performed in which the same metal instruments were used. Most worrying was the genetic make-up in this case on one tiny point of a gene all humans and other mammals carry - the prion protein gene (PRNP) on chromosome 20.

To date, all 191 victims of vCJD have shared a genetic trait. They are MM at codon 129 of the PRNP gene. Forty per cent of caucasians have the MM genotype. It had been hoped this was the only susceptible population group.

But the second case, sadly, had the most common genetic type at the codon 129 position of the PRNP gene. It is known as MV - a trait shared by half of all caucasians.

BEFORE vCJD, there was another orally transmitted prion disease. Kuru (the "shivering" disease, named after one symptom) probably originated with a single case of CJD around 1900, in the Fore tribe of the eastern highlands of Papua New Guinea. The disease nearly wiped out the Fore, who ate the brains of dead relatives in a mourning ritual.

However, cannibalism was prohibited in the 1950s and gradually the epidemic died. But with the odd case still emerging, the longest incubation period for this disease is now more than 40 years and serves as a model for what might happen with vCJD.

Recent genetic analysis of the scrupulously preserved records and blood samples from the kuru epidemic show victims with the MM genetic variation were younger when symptoms began (many were children), probably had a shorter incubation period and a shorter illness. Another study revealed most MV or VV individuals survived the epidemic or developed kuru when older. The VV genotype - reassuringly for those who might die of something else first - had the longest incubation period, according to a 2001 study published in the Journal of Infectious Diseases.

LAST month two papers provided additional foundation for the kuru findings - that the MM genotype may affect merely the shorter incubation victims of the human mad cow epidemic and that MV or VV genotypes will live longer, but not necessarily escape the same fate.

In the first, published in The Lancet Neurology, mice whose PRNP gene had been replaced with the human counterpart did not develop BSE when deliberately injected with it. However, when vCJD was injected into MM and MV mice they were "equally susceptible" to infection, although most of the MV mice did not develop symptoms before the end of the 700-day experiment - their natural life span.

The transmission rate of vCJD in the VV transgenic mice was much less but indicated a rather grim scenario: that all three genotypes on codon 129 showed subclinical or symptomless infection. What differed was the incubation periods until symptoms either appeared or the mice died. The mice were useful in showing that once BSE has passed into humans in the form of vCJD, it was altered, which also raised "concerns relevant to the possibility of secondary transmission of vCJD through blood transfusion, fractionated blood products, or contaminated surgical instruments", the authors warned.

And if the news could get worse, it did. A retrospective study published in the British Medical Journal analysed the genotypes of the three positive samples of infected tissue found among 11,109 appendixes and 1565 tonsils removed in operations in Britain between 1995 and 2000 from anonymous patients aged between 20 and 29. With enough DNA left in only two of the three positive appendixes, the researchers found that is was "perhaps surprising" to find they were both VV. This bad news is the first time that the VV population in humans - the smallest population of the three genotypes at codon 129 on the PRNP gene - has been found to be potentially susceptible to vCJD infection.

Reaffirming earlier findings, the researchers from the national CJD Surveillance Unit at the University of Edinburgh stated: "Genetic studies of kuru, another orally transmitted human prion disease, found that PRNP codon 129 MV and VV genotypes were associated with longer incubation periods than the MM genotype."

And the ramifications? With such a long incubation period projected for VV genotypes, a potential risk for horizontal transmission of vCJD infection by "blood transfusion, blood products or contaminated surgical instruments" arises, underscoring the vital need for continued surveillance of cases well into the future, they warned.

Dr Andrew Hill, a molecular genetics expert who heads a laboratory studying prion diseases at the Bio21 Institute at the University of Melbourne, was one of the first to reveal through mouse experiments reported in 2000 that there was a silent reservoir of infection in symptomless prion disease carriers. The latest mice revelations "confirm concerns we raised in our study", he says. "These studies underlie the urgent need for a rapid, sensitive test for infectious prions so we can identify potential asymptomatic carriers.

"The blood bans [banning the donation of blood from any Australian who has spent a cumulative six months or more in Britain between 1980 and 1996 or who has ever had a blood transfusion] still apply and people might question why."

While Australia remains free of BSE "you can't ignore this just because it's happening overseas", he says.

Given Australia's historic migratory and tourism ties with Britain in particular, Australian prion disease experts think it inevitable that vCJD will emerge in Australia from someone infected in Britain.

"I suppose it all really underscores the need for continuing CJD surveillance in Australia," Professor James Ironside, from Edinburgh's CJD Surveillance Centre, told the Herald. More silent carriers as well as symptomatic vCJD cases in the 25 BSE-affected countries affected to date also loom.

Any continuing pattern of change to the genotypes of vCJD victims could mark the beginning of the next phase of the human epidemic.

THE TRAIL

Prion diseases

Author: Donald Macintyre
© 2006 Independent News and Media Limited

TSEs are a group of diseases that affect the central nervous system. These diseases are fatal and are characterized by spongy degeneration of the brain. Bovine spongiform encephalopathy (BSE), which affects cattle and is commonly referred to as "mad cow disease", belongs to the TSE group of diseases. Scrapie is a TSE that affects sheep and goats. Chronic wasting disease (CWD) is a TSE that affects cervids such as white-tailed deer, mule deer, black-tailed deer, moose and elk..

Creutzfeldt-Jakob Disease (CJD) is a rare and fatal form of TSE that affects humans worldwide. A form of CJD called variant Creutzfeldt-Jakob disease (vCJD) has been diagnosed since 1996 and is thought to be linked to the consumption of meat products derived from BSE-infected cattle.

There are no treatments and no vaccines for these diseases.

How did these diseases come into existence?

The cause of these diseases is unknown, but infected individuals and animals produce abnormal proteins which are laboratory markers used in the detection of infection.

The true origin of BSE is still unknown. There is international consensus that BSE is spread by feeding infected ruminant (cattle, sheep, goats, deer, elk , bison) protein products to cattle. The protein that is linked to BSE is resistant to normal inactivation procedures such as heat.

Scrapie has been in existence for several hundred years. The disease is thought to spread most commonly from ewe to offspring and to other lambs in the same group through contact with the placenta and placental fluids.

CWD was first identified in 1967 in deer at research facilities in Colorado. Scientists believe that deer and elk transmit the disease through contact with feed and water contaminated by saliva or feces from infected animals.

Do these diseases exist in Canada?

In 1993 BSE was found in a beef cow that had been imported from Britain in 1987. The animal was destroyed and additional measures were taken immediately by the federal government to deal with any risk that Canadian cattle might have been affected.

Canada's first case of BSE in a domestic animal was found in May 2003. This case and all of the limited number of subsequent cases have been thoroughly investigated.

Scrapie occurs in countries all over the world. It occurs in Canada at a very low level and has been the target of a stringent control program since 1945.

CWD has been reported in the United States, and in the Canadian provinces of Saskatchewan and Alberta. It was first diagnosed in Canada in farmed elk in Saskatchewan in 1996. The disease is thought to have been introduced by elk imported from the United States in the late 1980s.

Can BSE, scrapie or CWD be transmitted to humans and other animals?

Variant Creutzfeldt-Jakob disease (vCJD), was first reported in March 1996 in the UK and is thought to be linked to the consumption of meat products derived from BSE-infected cattle.

There is currently no scientific evidence that scrapie or CWD can be transmitted to humans or cattle.

What is Canada doing to prevent the spread of TSEs?

The CFIA is responsible for implementing animal health and disease control programs. The key elements of these programs include:

• Veterinary services
  
• Disease surveillance
  
• Laboratory-based diagnostic testing
  
• Domestic and import controls
  
• Food safety controls
  
• Livestock feed program
  
• Voluntary Scrapie Flock Certification Program
  
• CWD Voluntary Herd Certification Program

The CFIA has the legislative authority to impose quarantine measures, and to order the humane destruction and disposal of animals or animal products suspected of disease infection. There is also a program to compensate owners for animals and animal products ordered destroyed.

For more information on Canada’s approach to controlling and eradicating TSEs, please see the individual fact sheets on BSE, scrapie, and CWD.

Where can I get more information on TSEs?

Suggested websites include:

• Chronic Wasting Disease Alliance
  
• Scrapie Canada
  
• World Health Organization (WHO)
  
• World Organisation for Animal Health (OIE)

Information on this page is from the Canadian Food Inspection Agency

Canada is continually assessing international scientific information as it becomes available and modifying policies as required, based on new information.

Chronic wasting disease (CWD) is a progressive, fatal nervous system disease known to naturally infect white-tailed deer, mule deer, black-tailed deer, moose and elk.

CWD belongs to the family of diseases known as transmissible spongiform encephalopathies (TSEs). Though it shares features with other TSEs, such as bovine spongiform encephalopathy (BSE) in cattle and scrapie in sheep, it is a distinct disease known at this time to only affect members of the deer (cervid) family.

Is CWD a risk to human health?

At this time there is no scientific evidence to suggest that CWD in deer and elk can be transmitted to humans. However, it is recommended that any tissue which may have come from a CWD-infected animal not be used or consumed by humans.

Measures have been taken at both the federal and provincial levels to reduce human exposure to products potentially contaminated by CWD by preventing infected animals from entering the food chain and, in areas where CWD is known to exist in wild cervids, providing warnings and precautions to hunters.

What are the clinical signs of CWD?

Animals with CWD may show a number of different signs as the disease slowly damages their brain. They may include:

Signs can last for weeks to months before the animal dies; however, some animals may not show clinical signs except for acute pneumonia. Animals are usually three to four years old before clinical signs appear, but signs have been seen in animals as young as 18 months or as old as 13 years.

Where is CWD found?

First recognized as a "wasting syndrome" in a research facility in Colorado in 1967, CWD has only been found in captive and wild cervids in North America and in captive cervids in South Korea.

CWD was first detected in Canada on a Saskatchewan elk farm in 1996. The disease has been routinely detected in Saskatchewan, with a few cases in Alberta.

How is CWD transmitted and spread?

Both direct (animal-to-animal) and indirect environmental (animal-to-premises-to-animal) transmission occurs in cervids. It is believed that direct transmission occurs via shedding of the infectious agent in saliva and feces. The incubation period can last from 16 to 36 months.

There is no evidence that CWD-affected deer and elk can transmit the disease to other species.

How is CWD diagnosed?

CWD is tentatively diagnosed based on clinical signs, but is usually confirmed by testing of tissue from the affected animal after it is dead.

How is CWD treated?

No treatment is available for animals affected with CWD. No vaccine is available to prevent CWD infection.

What is done to protect Canadian livestock from CWD?

A CWD disease control and eradication policy was implemented by the Canadian Food Inspection Agency (CFIA) in October 2000. Ongoing provincial surveillance, which has detected the recent cases, varies with each particular province’s perceived threat and infection status. Testing is mandatory in Manitoba, Saskatchewan, Alberta and the Yukon; it is voluntary elsewhere.

How would the CFIA respond to an outbreak of CWD in Canada?

CWD is a "reportable disease" under the Health of Animals Act. This means that all suspected cases must be reported to the CFIA for immediate investigation by inspectors.

The CFIA quarantines all herds in which an animal tests positive for CWD and tracks the movements of animals onto and off of the affected premises. Exposed animals are usually destroyed.

Owners whose animals are ordered destroyed may be eligible for compensation.
For more information:

Contact your CFIA Area office:

Atlantic Area: 506-851-7651

Quebec Area: 514-283-8888

Ontario Area: 519-837-9400

Western Area: 403-292-4301

You can find your local CFIA District office on the CFIA Web site or by consulting the blue pages of your local phone directory.

For more information on CWD, you can visit the CFIA’s CWD page. Further information is also available from the CWD Alliance.

Information on this page is from the Canadian Food Inspection Agency

Canada is continually assessing international scientific information as it becomes available and modifying policies as required, based on new information.