How to Avoid Contracting variant Creutzfeldt‐Jakob Disease

Variant Creutzfeldt-Jakob Disease (vCJD) or more colloquially, "Mad Cow Disease" in humans, is a fatal, almost untreatable neurological disorder in humans caused by misfolded prions which are ingested by eating infected tissue from animals that have a similar neurological disorder. Prions cause rare progressive neurodegenerative disorders that affect both humans and animals. They are distinguished by long incubation periods, characteristic spongiform or brain cell structure changes associated with neuronal loss, and a failure to induce an inflammatory response[1]. Prion diseases like vCJD impair brain function and memory. This includes personality changes, a decline in intellectual function (dementia), and neurological problems that worsen over time. In the U.S., human infections are extremely rare, resulting in an infection of approximately one in one million people. Once infected, there is no cure and death may occur within months of disease onset.


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    Learn critical facts about prions.
    • Prions are misfolded proteins that can cross the blood brain barrier and are nearly impossible to treat. The afflicted human has no choice, ultimately, but to suffer with the symptoms until it's their time to die.
    • Prion transmission from animal to human occurs when humans consume infected tissue from other animals that have had this disease. Other animals are primarily ruminants like cattle, sheep, deer, bison, and elk. In cattle and bison, the disease is known as Bovine Spongiform Encephalopathy (BSE) in deer Chronic Wasting Disease (CWD), and in sheep and goats, Scrapie. Generally when encompassing all ruminants, this disorder is known as Transmissible Spongiform Encephalopathy (TSE). The tissue that is most likely to retain the prions of this disease include the skull, brain, trigeminal ganglia (nerves attached to the brain), spinal cord, eyes, tonsils, dorsal root ganglia (nerves attached to the spinal cord), and the distal ileum (part of the small intestine)[2]. Although this may seem a non-point for the average meat-consumer, contamination may occur during meat processing especially if the processing is not hygienic or slaughtering/butchering requirements are not followed by law.
    • Unlike bacteria or viruses, prions cannot be removed from meat by sanitation or heat.
    • The incubation period of TSE is 8 to 10 years. Since most livestock are slaughtered well before that age, the risk of contracting vCJD may be significant, if and only if neither the producer nor the meat packers are careful to extract and eliminate any SRM (Specified Risk Material) that will contaminate supply. Just because a prion is incubating does not mean it is harmless, thus any SRM is removed on the processing floor before the meat is chilled and butchered for the supermarket shelves. In Canada and the EU, SRMs are not produced as livestock feed, unlike in the United States. Producers also follow mandatory feed regulation guidelines held by the federal government which mean feeding absolutely no animal by-products to livestock. This ban is mandatory in Canada, whereas the U.S. is a little less strict as it allows chicken waste, which contains remnants of animal by-product in it (chicken manure isn't just the manure, it also contains some feed dropped by the birds), to be fed to cattle. Chicken waste is prohibited as ruminant supplemental feed in Canada and the European Union due to risks of BSE.
    • Visit various sites that have more scientific facts on vCJD, BSE, TSE and Scrapie.[3] is a good place to start, as well as information from the CFIA on BSE[4]. Both sites are very informative on everything to do with BSE or Mad Cow Disease.
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    Watch for meat recalls. In the United States, the U.S. Food and Drug Administration (FDA) is responsible for food recall decisions and public notification. In other countries like in Canada, the CFIA (Canadian Food Inspection Agency) is also responsible for such actions. Both the FDA and CFIA are responsible for inspecting packing plants for any flaws in the system, and to ensure the meat being produced is safe to eat. The FDA and CFIA are also where producers can report reportable diseases like BSE, CWD, Scrapie, and others.
    • Watching for meat recalls is easier said than done. The FDA reports on recalled food consistently. This information can be found at[5] Rather than wasting your time weeding through the lengthy list, it is better to subscribe to public health alerts through your email address.
    • Historically, prion diseases were big news in the media in the U.S. Unfortunately, this is no longer the case, as shown by a lack of coverage on a confirmed case of BSE in 2012 after a dairy cow in California became supposedly “lame."[6] You now have to actively seek out that information yourself instead of relying on the media to let you know about it. If you are in Canada, the CFIA also has a site and a means to keep you updated on reportable diseases that are found[7]. So far, in 2014, no BSE cases were found, but 4 CWD[8] and 7 Scrapie[9] cases have been confirmed.
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    Only buy meat from local farmers. Often, that the pound of ground beef you picked up at the local grocery store came from multiple cattle [10]. Ground meat is continually produced and is composed of different cuts of meat from different cattle. This increases the likelihood of contamination. Contamination is less likely if the meat you are getting is not ground, but in specified cuts for roasts and steaks. On the other hand, buying from local farms in bulk provides a premium (meat is more expensive, but you pay more for a better product), allows you to know where your beef comes from and how it was raised and fed, and is less likely to be contaminated.
    • The larger the packer facility, the more product is produced resulting in increased chances of oversight and errors in processing occurring, thus the increased risk of contamination.
    • Of course, get to know your local farmer and their history. If your local farmer has had no meat recalls in the past, has their animals primarily on pasture and/or fodder, and does not feed the animals any animal by-products like chicken litter, then the health of the animals is likely very good. It's often considered that local meat is safer than meat that is mass-produced.
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    Cut back on meat consumption. Change your source of meat (fish and poultry) or become vegetarian. Becoming vegetarian is probably the best way to prevent vCJD; obviously if you don’t eat contaminated meat then, in theory, you can’t get infected with any food-borne diseases. However, even the spinach you buy may have risk of being contaminated with E.coli. That said, many of our foods come contaminated, in much the same way as they have throughout history––through error, oversight and sloppy work practices. It’s best to know where your food comes from and to pay a little extra for foodstuffs that are high risk, such as meat and fresh produce.
    • Local foods are less likely to become contaminated. This is also true for processing; the less exposure our food has before being consumed, the less likely it is to be contaminated by any infectious rare agent.


  • Keep the risk in perspective. In the U.S. the risk of acquiring a prion disease is one in one million, whereas E. coli infections are 24,000 per one million. In the UK, to October 2009, 166 people had died from variant Creuztfeldt-Jakob disease.[11]
  • For some more perspective on the American meat packing industry during the 20th century, check out a classic, The Jungle by Upton Sinclair.


  • If you think you may have vCJD, please visit your general physician before jumping to conclusions about what you may or may not have.

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Categories: Neurological Disorders