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I recently lost a good friend

I recently lost a good friend to some kind of wasting disease. He was in his 50s and in relatively good health. Then over a period of about two years he became confused, then incoherent, then child-like then DEAD!

His sister tried, while he was alive, to have him tested for Bovine Spongiform Encephalopathy (BSE), or Mad Cow Disease. None of the doctors would test him! After he died he was of course autopsied by the state medical examiner and they would not test him for BSE.

Finally his sister was able to have a tissue sample sent to a lab (I won’t say who or where) that would test for BSE. They would not tell her the results of the testing but they did say that if the test was positive for BSE that they would forward the sample to the US government’s primary BSE research university but if the sample were negative for BSE the tissue would be returned to the state medical examiner.

I am being obtuse on purpose because I don’t know who is on what side in this and I certainly don’t want to blow anyone’s cover or grants or credibility.

After testing, my friend’s brain tissue was sent to the government facility!

Now my friend was a beef nut. He absolutely ate hamburger in some form EVERY DAY! Our friendship was based on BBQ and age and Viet Nam and the love of a certain baseball team. We shared a lot of experiences in baseball and BBQ. Sorry for the digression, but he was a really nice guy and we all miss him.

About the time this happened my daughter, who is one of those “do gooder”, “tree hugging”, “save the whales” types (wonder where he got that?) came up with the theory that if the beef population were infected at a greater rate than the USDA/FDA reported, and if the accumulation of BSE prions in the human body were a slow process, then there is the potential for many, many people to become infected.

With these ideas in mind (and my penchant for numbers) I have come up with the following facts. My sources are documented and there is additional information on the subjects following my exercise.

Excuse me now; I’m trying to perfect my TVP chili recipe. Oh, you might want to grab your favorite triple cheeseburger while you enjoy this info.

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The US butchers 37 million cows per year

The United States Department of Agriculture (USDA) testing rate is 268,000 cows per 18 month period or 178,667 cows per year (less than ½ of a percent).
A cow infected with Bovine Spongiform Encephalopathy (BSE), often called Mad Cow Disease, turned up in Washington from Canada in 2003. Since rapid testing, which confirms results within hours rather than weeks, became available in 2004, a U.S.-born cow in Texas was confirmed to have BSE in 2005 and another cow in Alabama was confirmed BSE infested in 2006. These incidents are cited in a 2006 Congressional Research Service Report to Congress.
That means in 2003, 2004, and 2005, one cow per 178,667 was found to have BSE, so if all of the cattle were tested we could have potentially found 207 cows with BSE each year

Now the average 1,150 pound steer yields a 714 pound carcass. Approximately 146 pounds of fat and bone are trimmed off leaving about 568 pounds of retail beef cuts.

Each year, 207 BSE infected cows would produce 117,576 pounds of infected meat. If we ignore the mixing of large quantities of meat from many different cows for the production of hamburger and assume that all the beef is sold in neat, cow specific, 1/4 pound servings then our BSE cows would yield 470,304 individual servings of contaminated beef.

Not adjusting for those who “super size”; we have a potential infection rate of 470,304 Americans per year!

Now I love beef and I don’t wish the cattle industry any ill will, in fact my father-in-law was a life-long cattle rancher, but these numbers give me and each member of my family a 1.5 chance in a thousand of contracting the human version of BSE, variant Creutzfeldt-Jakob Disease (vCJD)1, every time we eat beef, and it’s just not worth it to me!
1. CJD belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs). Spongiform refers to the characteristic appearance of infected brains, which become filled with holes until they resemble sponges under a microscope. CJD is the most common of the known human TSEs. Other human TSEs include kuru, fatal familial insomnia (FFI), and Gerstmann-Straussler-Scheinker disease (GSS). Kuru was identified in people of an isolated tribe in Papua New Guinea and has now almost disappeared. FFI and GSS are extremely rare hereditary diseases, found in just a few families around the world. Other TSEs are found in specific kinds of animals. These include bovine spongiform encephalopathy (BSE), which is found in cows and is often referred to as “mad cow” disease; scrapie, which affects sheep and goats; mink encephalopathy; and feline encephalopathy. Similar diseases have occurred in elk, deer, and exotic zoo animals.
From an Italian Website, http://www.cremonini.it/en/osservatorio/ricette.asp?IdRicetta=4&IdLingua=en
HOW IS THE CONSUMER PROTECTED?
The most important measure in a position to guarantee total safety of bovine meat is the elimination of all organs and tissue potentially at risk from the moment slaughtering begins. This measure has been adopted on all bovines slaughtered in our country since 1st of October 2000 but was already in force since 1998 for all animals that originated in countries that were at risk.
In comparison to animals originating in the United Kingdom, similar measures have been adopted and have been in force since 1990 up until the moment a total embargo was placed on the importation of animals in 1996.
DOES THE CONSUMPTION OF MEAT REPRESENT A RISK?
The agents responsible for BSE have never been discovered in the muscle of the bovine and no scientific evidence exists that permits any health risk in the case where these products are consumed. Experimental attempts made by transmitting BSE through the muscle of the infected bovine have never given positive results and no risk was ever assumed relating to the consumption of bovine muscle, namely minute steak, entrecote, meat rounds, fillet, sirloin etc.
WHAT MEASURES ASSURE PROTECTION FOR THE CONSUMER?
At present, it is not always possible to individualize bovines with the disease while in the BSE incubation stage. Therefore, as a precaution, all organs and tissue potentially at risk are systematically removed and destroyed by a process of incineration and thanks to this elimination, tissue recognized as a potential risk will never arrive on the table of the consumer.
WHAT SPECIFIC MATERIALS ARE AT RISK?
Materials at risk are: the intestine of all bovines, the cranium including brain and eyes, the spinal marrow, the tonsils and the spinal column of bovines older than 12 months which are systematically removed from the slaughtering industry and destined for the incinerator.
ARE THE ANTI-BSE TESTS CARRIED OUT ON ALL ANIMALS OLDER THAT 24 MONTHS?
From the 1st of January 2001, a grand scale-monitoring program was started by utilizing the anti-BSE tests with the aim of the public level having a better knowledge about the state of the disease in the bovine estate.
PRINCIPLES OF THE TEST
During the incubation of BSE, the healthy prion protein, naturally produced by the central nervous system of the bovine, is transformed into abnormal prion protein. This then accumulates progressively in the brain of the bovine. The rapid tests at disposal are able to evidence the presence of abnormal prion protein in the brain in less than 12 hours and thanks to these tests, BSE can be established in the bovine without having shown any previous symptoms. Therefore it is possible to trace BSE in bovines with the disease while in the incubation phase.
DOES THE TEST PERMIT THE ISOLATION OF THE DISEASE ON ALL BOVINES?
The test does not permit the isolation of all bovines carrying the disease since BSE is only exposed in the final stage of development; that is, the moment it affects the brain. Due to this reason, the systematic test does not replace the destruction of the organs at risk, which are in any case carried out on all slaughtered animals regardless of the test result.
THE ORIGINS OF BSE AND THE ADAPTED MEASURES FOR ELIMINATION
Diet, has been shown and recognised as the principle way in which BSE is transmitted.
HOW WAS BSE ABLE TO GROW INSIDE THE BOVINES ESTATE?
In the 80's, the disease encephalopathy spongiform hit 180.000 English bovines, a disease similar to Scrapie. Encephalopathy spongiform is typical in ovines and has been around for the last 200 years.
Studies have shown that the disease in ovines was provoked by utilizing proteinic flour, obtained from carcasses of affected sheep, in the diet of bovines.
Furthermore, also in the 80's in the United Kingdom, the procedure of producing animal flour derived from recycling carcasses of animals between those that could have been infected, was modified: a shorter cooking time at a less elevated temperature reduced the capacity to deactivate the infective agents of BSE. The re-utilization therefore provoked the epidemic in which the evolution is summarized in the graphic. It is therefore clear that not only did the animal flour as such provoke the disease, but the flour originating from affected animals in countries in which the disease was present in a significant way.
THE BOVINE EPIDEMIC IN THE UNITED KINGDOM
(Source: International epizootic office)

WHAT MEASURES HAVE BEEN ADAPTED TO COMBAT THE DISEASE IN BOVINES?
The principal instrument adapted in keeping the disease in bovines under control is surveillance action on the bovine estate and the restrictions relative to the diet of the ruminatia. The surveillance on the bovine estate is based on the obligation on the part of the veterinary service to report any symptom that may be apparent in live animals referable to BSE (passive surveillance).
Furthermore, from the 1st January 2001, after an experimental period, systematic tests are executed on all animals slaughtered older than 30 months extended to 24 months from the 12th September 2001 and on all animals considered to be at risk (animals are urgently put down in the case of any incidents or death in the cattleshed).
In the first 10 months of 2001, animals that were tested in Italy exceeded 310,000 in which 34 cases tested positive, one of the lowest incidence rates in Europe. Furthermore, since 1994 the utilization of animal flour is prohibited in the diet of the ruminatia (bovine, ovine).
THE MEASURES RELEVANT TO THE DIET OF THE ANIMALS HAVE BEEN REINFORCED EVERY YEAR SINCE 1988
1988
British animal flour is forbidden in the diets of the ruminatia (bovine, ovine)
1990
All flour for the ruminatia is prohibited in the diet of the bovines.
1994
Mammalian animal flour is forbidden for all ruminatia. Permitted only for swine's, poultry and farmed ichthyic products.
1996
Compulsory treatment on animal flour according to the recommended procedure against infective agents of BSE (20 minutes at 133°C and pressure at 3 bars).
2000
Compulsory to utilize inferior products suitable for human consumption in the diets of the animals. New rules based on the prohibition of utilizing animal flour on all farmed animals.
THE CONTROL NETWORK FOR BSE
BSE is a disease that has the obligation of declaration, this means that the breeder has the legal obligation to report any abnormal symptoms the moment in which they are present in the bovine to the veterinary. In the case that the veterinary confirms the suspect of BSE to the management of the veterinary service, necessary measures are taken according to the sanitary norms relating to BSE:
- Official declaration of the suspect of BSE, farm placed under control, putting down of animals, withdrawal from brain of animal to send to Experimental Zooprophylactic Institute that will determine the diagnosis.
- In the case where BSE is confirmed, all animals, or those that have shared the same risk factor, are put down and the carcasses destroyed through an incinerator. The breeder is then completely compensated for the sudden loss.
- In the case where BSE is very evident, the National Sanitary Institute will carry out an inquiry on the supplier to establish the origin of the contamination.
IN SPITE OF THE ADAPTED SYSTEM OF PRECAUTION, WHY HAS ITALY NOT HAD POSITIVE RESULTS IN THE TESTS?
The average incubation period of BSE has an duration of 5 years, the effectiveness of the adapted measures to eliminate contamination may be only verified after this period; BSE cases verified in 2001 revealed that contamination in the food was only verified before the regulations relating to the diets of the animals were reinforced.

IS IT NECESSARY TO ELIMINATE A WHOLE HERD IF A CASE OF BSE HAS BEEN FOUND?
After numerous experiments, the Scientific Governing Board concluded that the measure of putting down all animals in the cattle shed where a case of BSE was verified was not necessary, it may be substituted by selective putting down only on animals where the identical risk factors have been exposed.
Today, thank you together with the measures in force relating to the BSE phenomenon and;
- The utilization of animal flour prohibited on all animals,
- Control of the bovine estate and obligatory declaration in cases where BSE is followed by the putting down of an entire herd, or animals at risk in the same herd,
- Reinforcement of the control on bovines upon their arrival at the slaughterhouse,
- Systematic withdrawal and incineration of all organs and tissue potentially at risk,
the system in force in our country guarantees effective consumer protection.
WHAT IS THE LINK BETWEEN BSE AND THE HUMAN DISEASE CREUTZFELD-JACOB?
The greater part of the cases of the human disease Creutzfeld-Jacob, which has already been around since the 1920's, is not linked to BSE or bovine spongiform encephalopathy, better known as "Mad Cow Disease".
At the time of its identification, which took place in 1986 in a bovine estate situated in the United Kingdom, it did not seem that BSE had anything to do with mankind. In fact, BSE which belongs to a group of diseases among which ovine scrapie and the human disease Creutzfeld-Jacob, two infections that have been dated back since the XVIII century after the 1920's have not demonstrated any link until now.
In March 1996, experts in the British Public Health Department announced that the transmission to man of BSE was possible. As a result, together with scientific results which have reinforced this hypothesis and that today one thinks that BSE in some way, which is still to be clarified, is linked to cases of the new variant of the Creutzfeld-Jacob disease which has appeared in France and United Kingdom in the young generation.
It must be underlined that the new variant of the Creutzfeld-Jacob disease, identified in 1996 and the classic Creutzfeld-Jacob disease, identified in the 1920's, are two different affections and are provoked by diverse characteristics. In Italy, no cases have ever been identified with regard to the new variant of Creutzfeld-Jacob in spite of the fact that there is an obligation to make a differential diagnosis between the two forms every time suspicious symptoms are present in mankind.

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