Prions, Kuru, Cannibals and Sanguinarians

Author: Sarasvati

Prion diseases are scary. In the bluntest terms, they are fatal within a year of symptoms developing. In humans, the symptoms are depression, memory loss, loss of coordination and fine motor control, eventually leading to inability to move and death. The thing that causes the disease is called a “Prion”. It is a little protein in the brain, all brains. The part that causes the problem is a mutant form of this little protein. The mutant form, when it encounters a healthy form, mutates the prion. Eventually, the mutant prions work their way through the brain, causing little fatty spots and holes in the brain, destroying brain function.

The human forms of Prion diseases include Crutzfield-Jakob disease (CJD), new variant Crutzfield-Jakob disease (nvCJD), and Kuru. CJD is a genetic form, originally discovered through the Jewish community where it appeared to be most prevalent. CJD’s origins are still not completely clear. It is possible that the genetic change in the prion was a genetic mutation of the DNA at some point, but that is speculation. CJD typically does not begin to show the symptoms until an elderly age. Kuru is a similar disease that was originally found in 1955 during an outbreak in a native New Guinea tribe. The disease in the tribe was traced back to the practice of cannibalizing their dead in ritual. Kuru was most predominant in the women and children of the tribe. This fact lead to the connection that the infectious agent (prion) probably resided in the brain as the women and children usually were given the brain to eat (as they were considered lower in status then the males) and the males consumed the heart, or other flesh parts. When they stopped this practice in 1960, the outbreak stopped. It is not yet known where this outbreak began either.

Now, for the one you hear the most about, nvCJD, or human Mad Cow Disease (Bovine Spongioform Encephalopothy or BSE). This disease was first recognized in 1985, when young people in Great Britain began to shown signs and symptoms of CJD. After death, scans of the brain cells showed a CJD like disease, but there were enough differences that the medical community could not call it CJD, so it was called nvCJD. At the same time, Mad Cow disease was breaking out across Great Britain. Mad Cow Disease was characterized by similar symptoms. The cow would have difficulty standing and coordination would fail. The cow would stumble about the pen, and be very excitable. Also about this time, there was a change in feeding practices. It was found that ground byproducts from other animals (mostly sheep and cows) increased the protein the animal put on, and thus increased the amount of meat on the animal. Byproducts are “leftovers” from slaughter, including the brain and spinal cord. Since Cows had never shown such a disease before, people began to wonder where it came from. Sheep seemed the likely culprit, since sheep byproducts were also in the new feed. Sheep have a similar disease called Scrapie (named due to the scratching of sheep with the disease because they think they itch). Scrapie had been known for over 250 years, but had never been shown to cross species from sheep to people. It is thought that the sheep scrapie crossed species into the cows through the byproduct feed, and then into the human population through contaminated meat. These byproducts were also put into some pet foods, resulting in BSE-like disease in cats, but not in dogs, even though the same byproducts would have been used in both types of pet food. Feeding of animal byproducts to any mammal was banned in 1988. The cow herds of Britain and the USA are now closely monitored for any signs of BSE. Recent cases of BSE in cattle in the USA and Canada have prompted further research.

Chronic Wasting Disease in Elk and Deer has also been sited, however there has never been any evidence to link CWD animals to human disease.

So, how does all of this relate to Sanguinarians? Well, it has been said we are cannibals. First, no, thats not quite right, cannibalism involves the death of the one being cannibalized, either from natural or unnatural causes. Sanguins do not kill. Secondly, the prion protiens can be found in the blood of infected animals, but in very very low concentrations. The disease is contagous in low amounts, but that is even more rare then the disease itself. Typically, high concentrations must be consumed, such as are found only in neurological tissue (brain and spinal cords). So how did those with nvCJD catch it? It is thought that sloppy slaughter practices allowed pieces of brain or spinal cord to contact the meat that was sold, and thus pass on to the people. Do sanguins have to be careful of butcher’s blood? You bet. It IS a very real, very nasty disease. The good side, is that blood of animals raised and slaughtered in the USA have almost no prion risk. Better yet if pig blood is used if needed, since there has never been a prion disease diagnosed in pigs in any country. Sheep, Goat, and Cow should be avoided.

This disease has most recently been the most often mentioned reason for Sanguins not to drink blood. Well, if I didn’t have to, I wouldn’t. Not having that choice, learn as much as you can about the risks present, and nvCJD is one of those risks. But it does not result from cannibalism. Simply being cannabalistic does not cause the mutant prion to spontaneously appear, it has to come from somewhere, be that beef or brain. This applies to blood- drinking too, simply drinking blood does not cause nvCJD, it has to be present for ingestion. This disease is new enough that rumors are flying, and as with rumors, most aren’t true.

Prion Diseases and species affected:
Bovine Spongioform Encephalopothy (BSE or Mad-Cow Disease): Cows
Scrapie: Sheep
Kuru: Humans
Crutzfield-Jakob Disease: Human, familial.
new variant Crutzfield-Jakob Disease: Human (from BSE)
Chronic Wasting Disease: Elk, Mountain Goats, Deer, and other wild cud-chewing animals
Feline Spongioform Encephalopothy: Cats
Transmissable Mink Encephalopathy: Mink and Ferrets
Gerstmann-Straussler-Scheinker syndrome: Humans, familial and sporadic.
Fatal Familial Insomnia: Humans (causes thalmic atrophy).
Alpers Syndrome: Prion disease in Infants. All show similar symptoms of reduced or impaired brain function.

~Sarasvati, May 5, 2006

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