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Thursday, October 11, 2012

First Case of Chronic Wasting Disease Found in Pennsylvania Deer

First Case of Chronic Wasting Disease Found in Pennsylvania Deer

HARRISBURG, Pa., Oct. 11, 2012 /PRNewswire-USNewswire/ -- The Pennsylvania Department of Agriculture today confirmed the first positive case of Chronic Wasting Disease (CWD) in the state on a deer farm in Adams County.

The disease is fatal in deer, elk and moose, but there is no evidence that CWD can be transmitted to humans, according to the Centers for Disease Control and Prevention and The World Health Organization.

The positive sample was taken from a white-tailed deer at 1491 New Chester Rd., New Oxford, and tested as part of Pennsylvania's intensive CWD monitoring efforts. The sample tissue was tested at the Pennsylvania Veterinary Laboratory in Harrisburg and verified at the National Veterinary Services Laboratory in Ames, Iowa.

In addition to the Adams County location, the department has quarantined two farms directly associated with the positive deer at 6464 Jacks Hollow Rd., Williamsport, Lycoming County, and 61 Pickett Rd., Dover, York County. The quarantine prevents movement of animals on and off the premises.

"Pennsylvania has an aggressive Chronic Wasting Disease surveillance program and a strong response plan," said Agriculture Secretary George Greig. "Steps are being taken to prevent further spread of this disease to the state's captive and wild deer populations."

An interagency CWD task force is in place to address the threat of the disease to Pennsylvania's captive and wild deer, elk and moose populations. The task force includes representatives of the departments of Agriculture, Environmental Protection and Health, the Pennsylvania Game Commission and the U.S. Department of Agriculture.

The task force will carry out the response plan, which includes education and outreach with public meetings and minimizing risk factors through continued surveillance, testing and management.

"To date CWD has not been found in Pennsylvania's wild deer population," said Pennsylvania Game Commission Executive Director Carl G. Roe. "Concerns over CWD should not prevent anyone from enjoying deer hunting and consuming meat from healthy animals."

Roe said that hunters should shoot only healthy-appearing animals, and take precautions like wearing rubber gloves when field-dressing their deer and wash thoroughly when finished.

"Though no human disease has been associated with CWD, the Centers for Disease Control and Prevention recommends people or other animals do not eat any part of an animal diagnosed with or showing signs of CWD," said Acting Health Secretary Michael Wolf.

CWD attacks the brains of infected deer, elk and moose, producing small lesions that eventually result in death. It is transmitted by direct animal-to-animal contact through saliva, feces and urine.

Signs of the disease include weight loss, excessive salivation, increased drinking and urination, and abnormal behavior like stumbling, trembling and depression. Infected deer and elk may also allow unusually close approach by humans or natural predators. The disease is fatal and there is no known treatment or vaccine.

CWD was first discovered in Colorado captive mule deer in 1967, and has since been detected in 22 states and Canadian provinces, including Pennsylvania's neighboring states of New York, West Virginia and Maryland. Pennsylvania is the 23rd state to find CWD in either a captive or wild population of deer and the 13th state to have it only in a captive deer herd.

Surveillance for CWD has been ongoing in Pennsylvania since 1998. The agriculture department coordinates a mandatory CWD monitoring program for more than 23,000 captive deer on 1,100 breeding farms, hobby farms and shooting preserves.

In addition, the Game Commission collects samples from hunter-harvested deer and elk and those that appear sick or behave abnormally. Since 1998, the commission has tested more than 38,000 free-ranging deer and elk for CWD and all have tested negative.

For more information from the departments of Agriculture and Health and the Pennsylvania Game Commission, visit: (click on the "Chronic Wasting Disease Information" button on the homepage), (click on "CWD Info"), and (click on "Diseases and Conditions")


Lou said...

I live in Potter and have seen several deer which were ill in the past few years. One young buck had swollen ankles and was thin and weak. He would lie down right next to my house. Also, his posture was hunched over.

This year a young buck had one swollen ankle and was thin. Haven't seen him in over a month.

Wonder what they have? Couldn't find it when I researched it.

Anonymous said...

Hunters, Please, please realize this is a “PRESS RELEASE” designed to put your mind at ease.

Do your research, research what a Prion is, CWD, CJD, Scrapie, BSE.

The CDC says there is a LOW possibility of transmission to humans, It Does not say there is No possibility.

There HAVE been humans with CWD. Read this link, then read it again, then google search these terms and make your choice. I am telling you now there is link between these and human Alzheimer’s Disease.

“However, the transmission of BSE to humans and the resulting vCJD indicate that, provided sufficient exposure, the species barrier may not completely protect humans from animal prion diseases. Because CWD has occurred in a limited geographic area for decades, an adequate number of people may not have been exposed to the CWD agent to result in a clinically recognizable human disease.”
Not the word MAY rather than DOES not. It also says WE, the CDC may not have enough people to “Clinically” call it a disease. It does not say not does not exist

“Such observations have given rise to the concept of a “species barrier,” which would need to be overcome before an infecting prion strain caused disease in a recipient host”
The “Species Barrier” is only a concept. Do you want to risk your life to a “concept”

Anonymous said...

In 2001, the case of a 25-year-old man who reportedly died of a prion disease after an illness lasting ≈22 months was investigated (Table 2). Although this man had hunted deer only rarely, his grandfather hunted deer and elk throughout much of the 1980s and 1990s and regularly shared the venison with the case-patient’s family. The grandfather primarily hunted in southeastern Wyoming, around the known CWD-endemic area. The case-patient’s illness began with a seizure and progressed to fatigue, poor concentration, and depression. Memory loss, ataxia, speech abnormalities, combative behavior, and recurrent seizures also developed. Histopathologic, immunohistochemical, and Western blot testing of brain autopsy samples confirmed a prion disease diagnosis. Analysis of the prion protein gene indicated a P102L mutation coupled with valine at the polymorphic codon 129 in the mutant allele, confirming a diagnosis of Gerstmann-Sträussler-Scheinker syndrome (GSS). This case-patient was unusually young even for a person with a GSS P102L mutation. It remains unknown whether the possible exposure of the case-patient to CWD-infected venison potentially contributed to the early onset of his prion disease.
In 2001, two additional CJD patients 26 and 28 years of age were reported from a single state (Table 2) (34). The patients grew up in adjacent counties and had illness onset within several months of each other. As a result of this fact and their unusually young age, a possible environmental source of infection, including exposure to CWD-infected venison, was considered. One of the patients died after an illness lasting 5–6 months that was characterized by progressive aphasia, memory loss, social withdrawal, vision disturbances, and seizure activity leading to status epilepticus and induced coma. Histopathologic, immunohistochemical, and Western blot testing of brain biopsy and autopsy samples confirmed a CJD diagnosis. The patient’s disease phenotype corresponded to the MM2 sporadic CJD subtype reported by Parchi et al. (35). This patient did not hunt, and family members provided no history of regularly eating venison. The patient may have occasionally eaten venison originating from the Upper Peninsula of Michigan while away from home during his college years. However, ongoing surveillance has not detected CWD in Michigan deer (36).
The second patient died from an illness lasting ≈16 months. The patient’s illness began with behavioral changes, including unusual outbursts of anger and depression. Confusion, memory loss, gait disturbances, incontinence, headaches, and photophobia also developed. Western blot analysis of frozen brain biopsy tissue confirmed a prion disease diagnosis. Immunohistochemical analysis of brain tissue obtained after the patient’s death showed prion deposition consistent with GSS. A prion protein gene analysis could not be performed because appropriate samples were lacking. However, prion protein gene analysis of a blood sample from one of the patient’s parents indicated a GSS P102L mutation. The patient did not hunt but may have eaten venison from Michigan once when he was 1–2 years old. The GSS diagnosis greatly reduced the likelihood that the two patients reported from adjacent counties had disease with a common origin.

Anonymous said...

Recently, rare neurologic disorders resulting in the deaths of three men who participated in “wild game feasts” in a cabin owned by one of the decedents created concern about the possible relationship of their illnesses with CWD (Table 2) (37). Two of the patients reportedly died of CJD, and the third died from Pick’s disease. More than 50 persons were identified as possibly participating in these feasts; the three patients were the only participants reported to have died of a degenerative neurologic disorder. Reanalysis of autopsy brain tissues from the three patients at the National Prion Disease Pathology Surveillance Center indicated that two of them had no evidence of a prion disease by immunohistochemical analysis. CJD was confirmed in the third patient, who had clinicopathologic, codon 129, and prion characteristics similar to the most common sporadic CJD subtype (MM1/MV1) (35). This patient participated in the feasts only once, perhaps in the mid-1980s. In addition, the investigation found no evidence that the deer and elk meat served during the feasts originated from the known CWD-endemic areas of Colorado and Wyoming.
In 2003, CJD in two deer and elk hunters (54 and 66 years of age) was reported (38). The report implied that the patients had striking neuropathologic similarities and that their illness may represent a new entity in the spectrum of prion diseases. A third patient (63 years of age), who was also purported to have been a big game hunter, was subsequently reported from the same area. However, none of the three patients were reported to have eaten venison from the CWD-endemic areas of the western United States. The 66-year-old patient hunted most of his life in Washington State. Although information about the 54-year-old patient was limited, there was no evidence that he hunted in CWD-endemic areas. The third patient was not a hunter but ate venison harvested from Pennsylvania and Washington. The neuropathologic changes, Western blot profile, and genotype at codon 129 of the three patients each fit the MM1, VV1, or VV2 sporadic CJD subtype, indicating absence of phenotypic similarity among the cases or atypical neuropathologic features (35).
To date, only two nonfamilial CJD cases with a positive history of exposure to venison obtained from the known CWD-endemic areas have been reported. One of the patients was a 61-year-old woman who grew up in an area where this disease is known to be endemic, and she ate venison harvested locally. She died in 2000, and analysis of autopsy brain specimens confirmed that the patient’s CJD phenotype fit the MM1 subtype, with no atypical neuropathologic features. The second patient was a 66-year-old man who was reported to have eaten venison from two deer harvested in a CWD-endemic area. Both deer tested negative for CWD, and the patient’s illness was consistent with the MM1 CJD phenotype.

Anonymous said...

Scientist: Wasting disease's potential to infect humans underestimated

A new paper from a Denver neurologist suggests the possibility of humans contracting chronic wasting disease is underplayed and that the "most reasonable assumption" is CWD can be transmitted to some people.
Patrick Bosque, an assistant professor at the University of Colorado School of Medicine, also wrote that scrapie - a CWD-like disease in sheep - can likely be transmitted to humans. Evidence to the contrary is "unconvincing," he said. Like many other researchers, Bosque said there is no known instance of a human infected by CWD, which fatally damages the brains of deer and elk via a rogue protein called a prion. But since prion diseases of any kind are so rare in humans, scientists might not be able to identify a CWD case were it to occur, he said.

"It would be obvious by now if humans were highly susceptible to CWD," Bosque wrote, noting the disease has been present in wild deer and elk in northeastern Colorado for two decades or more without any reported cluster of a human version of the disease.

Bosque's paper, published in Current Neurology and Neuroscience Reports, sounds a cautionary tone throughout and concludes that "practical measures to limit human exposure to animal prions, particularly CWD-infected deer and elk, should be improved."

But, he said, the relative risk involved appears extremely small. As an example, he compared England's annual rate of mad cow disease in humans - a disease in the same family as CWD - with the rate of U.S. deaths to far more common food-borne illnesses, such as E. coli. The former is lower than one case in 2 million. The latter, about one in 50,000.

Bosque, who earlier worked under Nobel Prize-winning prion researcher Stanley Prusiner, said that should CWD infect humans, the susceptibility rate would probably be similar to that of mad cow disease - that is, very low. So far, scientists believe 138 people have contracted the human version of mad cow even though millions were likely exposed to beef that might have carried the infectious prions. ['So far' being the key phrase here. The incubation period may be decades. Current estimates suggest that thousands more people may dieBSE coordinator]

Medical experts had an advantage in recognizing mad cow in humans, Bosque said: Its victims were far younger than those who typically contract a rare, sporadic human version of the fatal prion illness called Creutzfeldt-Jakob disease, or CJD. In addition, the disease left a distinctive pattern in the brains of its victims.

But should CWD affect humans, it might not leave the same clues as the human version of mad cow disease did, Bosque writes.

Bosque also questions the oft-cited notion that a so-called species barrier will prevent the transmission of CWD to humans. He calls the term misleading, saying the impregnability of such a barrier appears to depend on prion "strains," each of which exhibit different characteristics, and on differences in the prion's amino acid sequences.

Bosque's paper comes at the height of hunting season in Colorado. Early indications suggest that most hunters aren't concerned about the disease, as only about one in five appears to be seeking to test their animals for CWD.

Anonymous said...

Can chronic wasting disease affect humans? We don't know

"But do nine years of looking and failing to link CWD to human illnesses mean we're out of the woods?

Not necessarily, according to Dr. Andrew Badley, an infectious disease specialist at Mayo Clinic who also is a deer hunter. Speaking for himself, not for Mayo Clinic, he said the current situation hits him very close to home, as he owns hunting land in the CWD surveillance area north of Rochester.

"To date, there is no definitive evidence in the wild that CWD can cross the species barrier, but there could be a wide number of reasons for that," he said. "In order to get an infection, you need a certain amount of infectious dose, and you need a certain incubation period, and then the disease will manifest. So the reason we haven't seen it crossing species in the wild yet might be too low an infectious dose, or it might be we haven't waited long enough."

"Deer hunters were spooked, wondering if the meat they'd been feeding their families was safe. The medical community kept saying, "There's no evidence that people can be infected with CWD," but at the same time hunters were told to wear gloves while field-dressing their deer and to avoid all contact with brains or spinal cord material. To reassure non-hunting customers, some butcher shops made very public declarations that they would no longer let any venison come into their buildings."

Anonymous said...

prions are nasty. they can survive about anything... not something you can get rid of it it's in the meat (or anything else, fluids, linens, whatever)

some of them can lurk for many many years in your body before striking.

scary stuff

Anonymous said...

Quit your fear mongering!!!

Anonymous said...

The symptons may not show up in a deers behavior for 2 years.

Some of the above information is from the CDC, Center for Disease Control, it is researched based.

You owe it to yourself and your family to research this and not just believe what you are told from the people who have the greatest interest in preserving the industry and their jobs.

The risk may be small or maybe not so small. They will not tell you 100% it is safe. They cant, because thay do not know.The CDC says there is a low possibility of transmission.

Do you ask your self why they say to where gloves and protective gear if its alright to eat? You cant kill a prion by cooking it.

Reread the posts, then have you wife and children read, research then decide. Then think about how the people in charge spin things for their agenda.

Don't let your hunting pride or ranger rick make a decision for you that could end you being pushed around in a wheelchair wearing a diaper eating through a tube

questionasker said...

"It was recognized as a spongiform encephalopathy in 1978. To date, no strong evidence of CWD transmission to humans has been reported."

The above quote is from the CDC's own web site. Note the phrase "no strong evidence"

It begs the questions
Was there some evidence?
Was there a small amount of evidence?
Was there a moderate amount of evidence?
Was there evidence which was not reported?

The only thing that quote says is that there is "no strong evidence" nothing more nothing less.

Why did they not say there is no evidence?
or absolutly no evidence?

It would seem to indicate, there is evidence, reported or not, but just not quite strong enough to get excited about, yet.

Anonymous said...

Are there any obvious signs to looks for in the deer we harvest besides the falling down and stuff? Any open sores or anything like that? I ask because I shot a doe last week that had a mean wound on her face. Looked like a hole. But it wasnt infected or anything and the meat smelled fine. I brushed it off as a thorn or something that she had been digging. Just wondering

Anonymous said...

Keep your eye on the prion... That's what I always say.

Anonymous said...

Holy Sh*&! why don't you guys just post your articals instead of entering a blog encyclopedia thesis!

If you see a deer that doesn't appear healthy or seems to be doing the huckelbuck in your yard., DONT SHOOT & EAT IT! Unless it has a nice rack!

Lou said...

Folks, if you post something, please put a name to it so we can tell who is saying what. You don't have to use your real name. You can use the name of your grandma or your first dog...just something to make it easier to respond.

By the way, a beautiful eight point was in my front yard last night, looked VERY healthy:

Anonymous said...

All you long winded posters sound like you are members of PETA! I smell a dirty rat... Nice try!!! I'll stop harvesting critters and eating them when I die of old age. The lengths that some organizations will go through. How dare you!!!

Kill'er, Skinn'er, Eat'er....

Anonymous said...

Mad cow for the deer population?

Well, venison was never all that good. Well... not THAT good anyways.

The meat won't smell or look any different.

Do some research into what mad cow is, how it works and maybe you will understand the gravity of the situation.

Want to 'git r done' ? You go right ahead.
We need some ignorant test bunnies willing to eat whatever the hell they can cook or we'll never know if it is dangerous or not.

Me? I'll lean towards being cautious when it comes to my family's health.

It's not fear mongering when you live in a society so well supplied with food I can pick a dozen other meat sources that are as close to guaranteed safe as possible to choose from.
I can't see why people would take the risk.

Anonymous said...

Chuck Norris sprinkles CWD on every steak before he eats it. Just saying

Anonymous said...


The only ignorant person here is you! You should go back to watching your sea shepherd reruns. Might as well start slathering everything you eat with hand sanitizer as well... How's that working for you. Your family sick all the time?

Kill'er, skinn'er, and eat'er...

Anonymous said...

6:49, you are correct. it is a member of the TSE family (transmissible spongiform encephalopathy). You sound like you have read alot about it and have considered it wisely. I am doing the same. Too many unknowns and not worth the risk and the "Species Barrier" they talk about is just a theory, it is not proven.

It is a complex subject that none of us are familiar with. We just cant afford to rely on spin and sugar coating when we are talking about something that can kill you.

Mad Cow can jump species & I bet this can too.

Congratulations 12:17 you are the last person in the country who trusts your government with your life & believe they have your best interest in mind. Info from the CDC Center for Disease Control not peta

Here is a good article

Symptons -
Animals infected with CWD may not show any symptoms in the early stage; however, as the disease progresses, the animals begin to lose bodily functions and stagger or stand with poor posture. Animals may carry the head and ears lowered. Infected animals become emaciated (thus wasting disease) and will appear in very poor body condition. Animals with CWD also often will stand near water and consume large amounts of it. Drooling or excessive salivation may be apparent. Although these symptoms may also indicate other diseases, you should note the animal’s location and immediately contact the nearest Game Commission Regional Office if you see a deer or elk displaying these symptoms. Do not attempt to disturb, kill, or remove the animal. (In Pennsylvania, you can find the office nearest you on the Game Commission’s Web site.)

Anonymous said...


Can you provide some facts and findings from your research into CWD & TSE's that contradict 6:49's opinion & choice?

I'm sure it feels good to defend your hunting choices with all the redneck bravado you can muster, but most people consider this a serious choice hunters have to make and it should not sink
to ad hominem attacks.

Anonymous said...

Oh wait... That's right. The government is behind all of this. Now I get it.. Ok, I'll stop hunting and eating critters that the good lord put on this earth for me to make good use of. I don't think so! Not only does this sound like it is a PETA rouse now you are starting to sound like an anti-firearm koo koo as well. While you guys are our running around in the woods frolicking with flowers in your hair and trying to give a black squirrel a hug and gnawing on your protein bar I'll be sitting at my table eating an original organic meal striaght from the forest. Good luck with that and please stay away from my hunting blind..... You all smell likehand sanitizer....

Anonymous said...

Hahahahaha if you see an infected deer contact the gamesters....And I am sure they will find it right away. I think killing it and leaving it for the coyotes would be a better choice

Anonymous said...

Upgrade to a Smart phone, it may be your only hope.