New cases of CWD, chronic wasting disease, have been detected in deer in Missouri and Arkansas. This is concerning because CWD is very similar to "mad cow disease."
Undoubtedly, our food supply is much safer today. The outbreak was
due to farmers chopping up unwanted cow parts and feeding them to other
cows, spreading the infectious agent from cow to cow and eventually to
humans. In 1997, the FDA largely banned this practice. Therefore, an outbreak of mad cow disease from beef should not happen again.
But there are no such safety measures in place for people who consume
deer sausage, venison, or any other meat that comes from cervids (i.e.,
deer, elk, and moose). Concernedly, CWD is spreading among these
animals all across the United States.
Chronic Wasting Disease (CWD) in Deer, Elk, and Moose
Like mad cow disease, CWD is caused by an infectious agent known as a
prion. Prions are normal proteins found in the brain and nervous tissue
that go rogue. For proteins to function properly, they need to be
folded in very precise ways. Prions are misfolded. Worse, instead of
being destroyed and recycled by the cell -- the typical fate of
misfolded proteins -- prions resist degradation and cause other normal
prion proteins to misfold. A slow chain reaction occurs in the brain
over the course of many years, leading to neurodegenerative disease and
death.
Why does the prion protein exist at all? That's an open question.
Prion exists in the brains of all types of animals, including mammals,
birds, and reptiles, but its function is not well understood. Mice that
don't have prion have some health problems and act a little strangely,
but goats and cattle that are missing prion appear just fine*.
Symptoms of CWD in cervids include postural and behavioral changes,
teeth-grinding, tremors, loss of muscle control, and eventually weight
loss, a paradoxical outcome given that the animal also shows signs of an
increased appetite. The prion that causes CWD is thought to spread
through saliva, urine, and feces, and a 2015 paper published in Annual Review of Animal Biosciences shows that the disease has been detected in at least 23 states (after Arkansas is added).
Is CWD in Deer a Threat to Humans?
So far, there have been no known cases of CWD being transmitted to
humans. That doesn't mean it can't or hasn't already happened. The
incubation period for these diseases in humans is measured in years or
even decades. Furthermore, scientists have successfully infected monkeys using CWD-tainted deer and elk meat. It is indeed quite likely that CWD someday will appear in humans.
Having said that, the likelihood of acquiring CWD is quite low.
Consider all the millions of people who potentially were exposed to
contaminated beef from the UK. Only about 200 people died, which means
that it is not particularly easy for prion disease to spread. Perhaps
those who succumb to the disease have a unique genetic susceptibility.
To avoid being one of the unlucky few who may develop CWD one day, there are safety tips to follow.
Most importantly, hunters should not consume any cervids that look
unhealthy or behave strangely. Additionally, people should not consume
meat that contains nervous tissue, not just from cervids but from any
animal.
i cant donate blood here in Australia ,because i lived in the UK in the 1980s,
more info on CJD
Creutzfeldt-Jakob disease (CJD) fact sheet
Creutzfeldt-Jakob disease (CJD) is a rare brain disease that is caused by abnormal brain proteins called prions. Classical CJD usually occurs by chance. Variant CJD, which is linked to consumption of contaminated meat products, does not occur in Australia.
Last updated: 07 September 2016
What is Creutzfeldt-Jakob disease?
Creutzfeldt-Jakob disease, also known as CJD, is a rare degenerative disease of the brain that is fatal. It is one of a group of diseases known as the transmissible spongiform encephalopathies.
In CJD, the structure of a normal brain protein changes slightly forming prions. The build up of prions damages brain cells and causes the neurological symptoms of CJD. Unlike bacteria or viruses, prions resist normal methods of heat and chemical sterilization and, very rarely, prions can be transmitted to others.
There are two different types of Creutzfeldt-Jakob disease:
Classical CJD occurs in Australia and about one in one million people per year develops the disease. There are three types of classical CJD. About 90% of classical CJD cases occur by chance (sporadic CJD) and 10% of cases are hereditary (familial or inherited CJD). The disease has very rarely been transferred between patients following medical procedures such as brain surgery or the use of dura mater grafts or contaminated human pituitary hormones (iatrogenic CJD).
Variant CJD, is a disease that emerged in the UK in the 1990s. Variant CJD is linked to the consumption of meat products from cattle infected with bovine spongiform encephalopathy (BSE, or "mad cow disease"). Variant CJD is a separate disease to classical CJD, although some of the symptoms are similar. No cases of variant CJD have been identified in Australia to date. Australian cattle remain free of BSE.
What are the symptoms?
People with classical CJD have progressive neurological symptoms that may include behavioural changes, blindness, weakness, loss of balance and incoordination, difficulty walking or speaking and muscle spasm. Confusion in the early stages usually progresses to dementia. The disease is fatal, usually weeks to months after onset of symptoms.
People with variant CJD tend to be younger, have a slower rate of deterioration and tend to have more psychiatric symptoms or personality changes than patients with classical CJD.
How is it spread?
Most cases of CJD occur because of mutations with a person's brain and are not spread from other people.
Some medical procedures carried out on people with CJD have very rarely resulted in the disease being transmitted to other people. For example:
Human pituitary hormones derived from people who died with CJD resulted in transmission in the past and five of these cases occurred in Australia. Therapeutic pituitary hormones are no longer obtained from this source and no high-risk treatments occurred in Australia after 1985;
Neurosurgical instruments contaminated following an operation on someone with CJD has resulted in five cases worldwide, and none since the 1970s;
Dura mater grafts taken from donors who had CJD and used to patch holes in the lining outside the brain; corneal grafts taken from donors who had CJD and used in others have resulted in three cases of transmission worldwide and none in Australia.
Screening procedures and improvements to standards of infection control have made transmission of CJD in the modern health care setting extremely unlikely.
Variant CJD may be spread more easily from person-to-person than classical CJD. In contrast to classical CJD, variant CJD may also be transmitted to humans after eating contaminated meat and meat products from cattle with BSE, via transfusion of blood and blood products, and some other operations if the instruments are not processed properly.
Who is at risk?
Each year, about one in every million Australians develops sporadic CJD and most have no risk factors for the disease. The average age of onset is about 65 years.
Familial or inherited CJD includes familial CJD, Gerstmann-Sträussler-Scheinker (GSS) syndrome and fatal familial insomnia (FFI) and is carried from one generation of a family to the next by abnormal genes.
The risk of transmission in the health care setting is extremely low.
How is it prevented?
Special infection control precautions are used for patients thought to at risk of CJD. Products or instruments potentially contaminated with prions are removed from use.
In Australia, there is a very low risk of variant CJD; to safeguard the blood supply, people are excluded from donating blood if they have lived in the United Kingdom for more than 6 months between 1980 and 1996.
How is it diagnosed?
A definite diagnosis of Creutzfeldt-Jakob disease can only be made by special tests of the brain tissue; this almost always occurs after the patient has died. Other specialized tests for people with typical signs and symptoms can help to make a diagnosis, but do not confirm the diagnosis.
To confirm the diagnosis after death an autopsy is usually recommended.
There is no screening test for CJD.
How is it treated?
There is no specific treatment for Creutzfeldt-Jakob disease.
What is the public health response?
Hospitals and laboratories are required to notify patients with Creutzfeldt-Jakob disease to the local public health unit (under the NSW Public Health Act 2010). The Australian National Creutzfeldt-Jakob Disease Registry coordinates surveillance and testing for CJD cases in Australia. The Registry works with NSW Health and local public health units to identify if cases have had high-risk procedures and to provide specialist advice for clinicians.
Further information
12 Monkeys............... 1995 ‧ Science fiction film/Thriller ‧ 2h 11m a must for AFT
Research involving a former brain-eating tribe from Papua New Guinea is helping scientists better understand so-called prion conditions such as Parkinson's disease and dementia.
People of the Fore tribe, studied by scientists from Britain and PNG, have developed genetic resistance to a mad cow-like disease called kuru (a prion condition), which was spread mostly by the now abandoned ritual of eating relatives' brains at funerals.
Experts say the cannibalistic practice led to a major epidemic of kuru prion disease among the Fore people, which at its height in the late 1950s caused the death of up to 2 per cent of the population each year.
In findings published in the scientific journal Nature, the researchers said they had identified the specific prion resistance gene — and found that it also protects against all other forms of Creutzfeldt-Jakob disease (CJD).
"This is a striking example of Darwinian evolution in humans, the epidemic of prion disease selecting a single genetic change that provided complete protection against an invariably fatal dementia," said John Collinge of the Institute of Neurology's prion unit at University College London, which co-led the work.
Prions are infectious agents that cause often fatal brain diseases such as CJD in humans, scrapie in sheep and BSE (bovine spongiform encephalopathy, or mad cow disease) in cattle.
They are also a rare but important cause of dementia, and scientists say it is now recognised that the process involved in these diseases — in which prion proteins change shape and stick together to form polymers that damage the brain — is also what happens in common dementias such as Alzheimer's, and in Parkinson's and other neurodegenerative diseases.
Mr Collinge said his team was now conducting more studies to understand the molecular basis of this effect, hoping to find clues on the seeds of other misshapen proteins that develop in the brain and cause the common forms of dementia.
Worldwide, about 47.5 million people have dementia and there are 7.7 million new cases every year, according to the World Health Organisation.
The total number of cases is projected to reach 75.6 million in 2030 and to almost triple by 2050 to 135.5 million.
Reuters
12 Monkeys............... 1995 ‧ Science fiction film/Thriller ‧ 2h 11m a must for AFT
Every time hubby and I watch a "Survivors" or "Post apocalypse" type film and the cannibals come out, I comment on the prion risk of cannibalism. Evil AND stupid!
I didn't deer hunt this year. First time since I was old enough to shoot! My wife's an RN and after the reports of CWD in our area she refuses to eat venison! With the kids grown, the two of us were wasting a lot of meat every year to start with and there's no way I can eat an entire deer on the few days she's gone!I'm not a very good wild turkey hunter and rabbit and furry tailed tree rat hunting isn't as much fun! Though I do love squirrel stew! I've started hunting feral hogs but it's amazing how intelligent, sensitive and aware they are! I grew up in cattle country so i'd have to be starving to eat goat or mutton! I think States should outlaw baiting deer because so much of the chicken feed being used as bait is mixed (contaminated) with butchered bovine spinal/lymph/organ waste! Besides, baiting and using trail cams aren't really hunting anyway!
Those animals who naturally eat meat as part of their diet have better defenses against prion disease. Evolution saw to that for us. A carnivore or omnivore who was succeptable to prion disease stood very little chance of successfully passing on its genes. There are always exceptions, but that was the norm.
Herbivores, throughout previous ages and epochs, had no such natural selection. They are defenseless. Feeding them anything containing animal-source proteins is begging for trouble. It is like composting with ground elder. (For the non botanist: ground elder spreads like wildfire, chokes out all other herbs and will flourish on a compost heap.)
Thanks, Techno, I've been yelling about CWD for years! Whitetail deer in the US like to graze around dairy cattle, so I expect the prion to jump species eventually.
I was working/living in the UK during the Mad Cow cull, so I cannot donate blood as well. I ate plenty of British beef, and was not concerned as the cull had been initiated.
However, if I start shaking violently, my wife will be very upset with me.
I think you are probably OK, Chuck. It seems that our recent hominid omnivore history has protected most of us. Human BSE (Bovine Spongiform Encephalitis) appears quite rare and, athough the disease was very slow to present itself, the top of the bell curve seems now to have passed.
There was far less fuss made over scrapie in sheep, but I suspect that was one of the causes of altzheimers.
Again, as Carbon posted, parkinsons may be a disease of similar source, although I do not know from precisely which animal.
All this is one of the reasons I will not eat farmed rabbit. They are too close a relative, so we have lots of prions in common and, despite being an obligate herbivore, they often love flesh products. (I had a pet one who would only eat cat food.) Humans (being stupid) insist on messing about with animal feeds. Modern intensie farming to get higher egg yields uses so much fish meal in layers pellets that the latest chicken breeds HAVE to have animal products in their feed for good health. If you want to grow your own chicken food you need to pick an older breed.
Anyway, rant over, I do believe we are begging for big troubble on this one. The CWD prion seems to be transmissable by touch (I hope that is unique to this prion, or at least very rare.) and actually fulfills all the criteria for a species wiper (fatal, slow to manifest symptims, persistent after death and transmissible by other species who can act as a host reservoir). NASTY! NASTY! NASTY!
After reading this I am almost glad my boyfriend did not get a deer when he went hunting this year, lol. Although I was really wanting the deer hyde and jerky.
Can't give blood in the US either - I tried again a few months back with no luck. My wife lived in the UK long enough to be exempt as well, and we couldn't even donate my son's cord blood.
Prion diseases are nasty and probably not going away anytime soon, but from a scientific standpoint they are absolutely fascinating. The idea that a protein can change shape and thereby go under the body's radar to build up into lethal plaques is remarkable in itself, but the way they're believed to change normal proteins merely by contact is mindblowing.
I agree - interspecies transmission how we ended up with BSE in the first place, and it'll undoubtedly happen again.
"Buy it cheap. Stack it deep" "Any community that fails to prepare, with the expectation that the federal government will come to the rescue, will be tragically wrong." Michael Leavitt, HHS Secretary.
CWD, a deadly prion disease with no known treatment, was found to potentially be transmissible to humans. Monkeys were fed with contaminated meat and the results were alarming, with three out of five testing positive after three years. ( USFWS Mountain-Prairie | Wikimedia Commons )
Chronic Wasting Disease was first observed among Colorado deer in 1967. Since then, the neurological disease has spread to 24 U.S. states and Canada.
There have been no reports of human contamination so far, but a recent Canadian study has once again sparked worries that the disease could be contracted by humans.
In a long-term study at the University of Calgary, 18 macaques were exposed to the disease in different ways, including injecting infected material straight to the brain; feeding infected meat; skin contact; and intravenously.
CWD Found To Infect Macaque Monkeys
For the experiment, researchers selected macaques, a species of monkey from Asia, because their genetic makeup is closer to that of human beings' in comparison to other animals.
A report states that to date, three out of the five macaques fed with 5 kilograms (11 lbs.) of infected deer meat over a period of three years tested positive for CWD. In humans, such diet is equivalent to eating a 7-ounce steak each month.
What's even more alarming is that two of the three monkeys fed with deer meat exhibited symptoms of the disease such as anxiety, ataxia, and tremors.
One macaque shed one-third of its body weight over a six-month period, while two animals that had infected matter injected into their brains also developed CWD.
"No one should consume animal products with a known prion disease," said Stefanie Czub, a prion researcher at the Canadian Food Inspection Agency, who presented the partial findings of the study in Edinburgh, Scotland last May 2017.
2004 CDC Study Relates Similar Results
In 2004, a study conducted by a team at the Centers for Disease Control and Prevention concludes that humans consuming infected meat are not safe from potential exposure.
Through an in vitro cell-free experiment, the team observed that CWD-associated prions can convert human prion protein, strongly proving that it is transmissible to humans. Despite this apparent observation, the lack of evidence still puts the risk of human transmission to a low level.
Moreover, the study claims it is possible for CWD to develop new strains targeting human beings due to the migration of infected deer.
What Is Chronic Wasting Disease?
The CDC classifies CWD as a prion disease that affects, deer, elk, reindeer, and moose. An infected animal could take more than a year before exhibiting symptoms, such as drastic weight loss or wasting and stumbling.
It can be contracted by animals of any age and is fatal to their health. Currently, there are no known treatments or vaccines for the disease.
If I may digress here to clarify a few points before I continue:
PRIONS Proteins are large molecules comprising several amino acids joined togethar in a specific way. The molecules are usually folded into a particular shape. Prions are the exact same protein but jumbled up (Screwed up into a ball instead of folded into a precise origami shape, would be a great analogy.) so they can not perform their proper function. When a prion touches another protein, nothing happens: UNLESS it is the same protein. If the proteins match, the healthy (origami) protein screws up like the prion. The precise protein is encoded (described in the cookbook and origami instructions) in an animal's DNA.
DNA The closer the biological relationship between two animals, the more DNA they have in common. That means they have more proteins (and potentially prions) in common too.
RELATIONSHIPS In Biological Taxonomy (naming) there is a ranking system which attempts to describe the closeness of relationship between species. The higher up the tree they meet, the closer the relationship.
The highest taxonomic relationship is Domain, followed by Kingdom, Phylum, Class, Order, Family, Genus and finally Species. Refrence: https://en.wikipedia.org/wiki/Taxonomic_rank
At this point it is not surprising that the prion has jumped species to other deer as they are all in the same family. The macaque is more surprising as it has no common link to deer until the sub: Class theria (All species with a womb are theria - it is a broad class.) and it splits off into a sub, sub bit of that eutheria. Refrence: https://www.itis.gov/servlet/SingleRpt/SingleRpt?search_topic=TSN&search_value=180098#null.
'AND THAT LEAP FROM DEER TO MONKEYS IS A HUGE JUMP FOR A PRION TO MAKE! THE JUMP ON TO US WOULD BE NO LARGER! IF IT WERE TO JUMP TO MONKEYS IN CHINA, INDIA OR PARTS OF EUROPE, THEN THE LEAP TO US WOULD BE A VERY SHORT HOP.
NEITHER SCRAPIE, CJD OR MAD COW DISEASE APPEARED TO BE TRANSMISSABLE BY TOUCH. THIS PRION IS. THIS MUST BE STOPPED NOW OR IT COULD CONQUER THE WORLD.
This is actually getting scary. As I mentioned before, all the conditions of a species wiper were already met before these developments. This is terrible news for the white tailed deer. It could be so for us as well.
The incubation time of spongiform encephalopathies (ie. new variant CJD, "mad cow" in humans) is so long that we may have infected folks wandering around, and likely do.
Local sports news in Chicagoland: my guess is that the deer culled in this season were mostly eaten.
There were 15 northern Illinois counties open to the Special CWD Season in 2017-18, while 14 counties were open for the 2016-17 seasons. The Special CWD season is used to assist in slowing the spread of chronic wasting disease in the Illinois deer herd.
Some hunters in Colorado had the brains of their kills tested. But I have been out of hunting scene for a long time so I do not know if they are still doing this or how accurate the test is. I can tell you I do not eat deer, elk or moose. I have not eaten it for over 30 years.
CWD is serious and people are not paying attention.
Hubby and I have been discussing this. We are about to drop all mammalian products from our diets. That includes: pig/pork/bacon/ham, cow/beef, sheep/lamb/mutton, deer/venison, rabbit, hare and milk, cheese and butter. We think fish and fowl are probably safe as the proteomics are so different.
I'm with you Flumom. Damn! I love a bit of venison.
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Technophobe wrote:
