Thursday, September 18, 2014

Risk behaviors in a rural community with a known point-source exposure to chronic wasting disease

Chronic Wasting Disease Human-Exposure Study

 

While public health agencies continue to point out that there has been no known incident of chronic wasting disease developing in humans, a group of people known to have been exposed to venison from a CWD-infected deer is under study by a team of researchers from the Laboratory of Biomedical Anthropology and Neuroscience at the State University of New York at Binghamton.

 

The Oneida County (New York) Chronic Wasting Disease Surveillance Project was launched in 2005 in response to exposure to an infected deer at a March 2005 sportsmen's feast in Upstate New York. Eighty-one exposed individuals participated in the 2005 baseline data collection, and were sent follow-up questionnaires following each deer hunting season between 2005 and 2011.

 

According to researchers, over that six year period participants reported a reduction in overall venison consumption. However, they reported no significant changes in health conditions other than those normally associated with aging, such as vision loss, heart disease, type 2 diabetes, weight changes, hypertension and arthritis.

 


 

 

>>>over that six year period participants reported a reduction in overall venison consumption. However, they reported no significant changes in health conditions other than those normally associated with aging, such as vision loss, heart disease, type 2 diabetes, weight changes, hypertension and arthritis. <<<

 

 

PLEASE NOTE ;

 

 

At a hearing in Parliament last Wednesday, the Science and Technology Committee was told that vCJD continued to pose a “significant” risk to UK public health and that more than one in every 2000 people could be silent carriers of the disease. *** vCJD can have an incubation period of over 30 years. ...tss

 

*** In some cases, the incubation period may be as long as 50 years

 


 

 

Research Open Access

 

Risk behaviors in a rural community with a known point-source exposure to chronic wasting disease

 

Ralph M Garruto*1,2, Chris Reiber2, Marta P Alfonso2, Heidi Gastrich2, Kelsey Needham2, Sarah Sunderman2, Sarah Walker2, Jennifer Weeks2,3, Nicholas DeRosa4, Eric Faisst3,4, John Dunn4, Kenneth Fanelli4 and Kenneth Shilkret4

 

Address: 1Laboratory of Biomedical Anthropology and Neurosciences, State University of New York at Binghamton, PO Box 6000, Binghamton, New York, 13902-6000, USA, 2Graduate Program in Biomedical Anthropology, State University of New York at Binghamton, PO Box 6000, Binghamton, New York, 13902-6000, USA, 3Madison County Health Department, Wampsville, New York, 13163, USA and 4Oneida County Health Department, Utica, New York, 13501, USA

 

Email: Ralph M Garruto* - rgarruto@binghamton.edu; Chris Reiber - creiber@binghamton.edu; Marta P Alfonso - madurruty@yahoo.com; Heidi Gastrich - hj.gastrich@gmail.com; Kelsey Needham - kneedha1@binghamton.edu; Sarah Sunderman - ssunder2@binghamton.edu; Sarah Walker - swalker4@binghamton.edu; Jennifer Weeks - jennifer.weeks@co.madison.ny.us; Nicholas DeRosa - nderosa@ocgov.net; Eric Faisst - eric.faisst@co.madison.ny.us; John Dunn - jdunn@ocgov.net; Kenneth Fanelli - kfanelli@ocgov.net; Kenneth Shilkret - kshilkret@ocgov.net * Corresponding author

 

Abstract

 

Background: The emergence and continuing spread of Chronic Wasting Disease (CWD) in cervids has now reached 14 U.S. states, two Canadian provinces, and South Korea, producing a potential for transmission of CWD prions to humans and other animals globally. In 2005, CWD spread for the first time from the Midwest to more densely populated regions of the East Coast. As a result, a large cohort of individuals attending a wild game feast in upstate New York were exposed to a deer that was subsequently confirmed positive for CWD.

 

Methods: Eighty-one participants who ingested or otherwise were exposed to a deer with chronic wasting disease at a local New York State sportsman's feast were recruited for this study. Participants were administered an exposure questionnaire and agreed to follow-up health evaluations longitudinally over the next six years.

 

Results: Our results indicate two types of risks for those who attended the feast, a Feast Risk and a General Risk. The larger the number of risk factors, the greater the risk to human health if CWD is transmissible to humans. Long-term surveillance of feast participants exposed to CWD is ongoing.

 

Conclusion: The risk data from this study provide a relative scale for cumulative exposure to CWD-infected tissues and surfaces, and those in the upper tiers of cumulative risk may be most at risk if CWD is transmissible to humans.

 

Background

 

Chronic Wasting Disease (CWD) was first observed in the United States in the late 1960s, though its origins are still unclear [1,2]. The term "chronic wasting disease" was first used in 1967 to describe clinical symptoms in captive Colorado mule deer [1,2]. In 1978, the disease was diagnosed as a spongiform encephalopathy [1,3]. By 1980, CWD had been described in captive elk and mule deer herds in both Colorado and Wyoming [1,2]. Subsequently in 1985, CWD was found in free-ranging elk in Wyoming, and recognized in free-ranging mule deer and whitetail deer in both states by 1990 [3]. CWD has since been described in 14 states across the US and in 2 provinces in Canada (Figure 1).

 

In April of 2002, the New York State Department of Environmental Conservation (NYSDEC) began a statewide surveillance program for CWD in whitetail deer [4]. The first positive samples (five in total) were confirmed in April 2005 from animals on two domestic deer farms in Oneida County, NY. The second deer farm, located very near to the first, received two deer that tested positive from the first deer farm. In response, the NYSDEC implemented an intensive mandatory surveillance of CWD, primarily in Oneida and Madison counties [4]. In order to monitor the wild deer herd, a CWD containment area was created encompassing approximately a 10 mile radius around the location of the CWD positive domestic deer farms in Oneida and Madison counties (Figure 1) [4]. During the initial phase of intensive monitoring (through April 30, 2005), 317 wild deer were collected and tested from this containment area as well as from the Town of Arietta in Hamilton County. Wild deer (two in total), in close proximity to the domestic deer farms, were found to be positive for CWD. During the second phase of the intensive monitoring program, all deer that died or were killed within this area were subject to mandatory testing (Figure 2). Additionally, the NYSDEC expanded its testing program statewide [4], and now the annual testing of deer for CWD includes 800–1000 animals within the containment area (Figure 1) and approximately 5000 animals statewide [4]. No additional positive deer, wild or domestic, have been found since April 2005. Details of the distribution of the deer tested across New York State for CWD can be found on the DEC website [4].

 

Traditions of hunting deer, elk, moose, and other cervids, and management of domestic cervid preserves bring humans into contact with animals that could have CWD. Currently, it is unclear whether CWD prions can be naturally transmitted from infected cervids to humans or to non-cervids. The question of cross-species transmission of CWD has been raised in the past [5-13]. In 2002 an unusual cluster of Creutzfeldt-Jakob Disease (CJD) – a human prion disease – developed in individuals who were avid lifelong Idaho deer hunters [14]; however, this and other reports investigating clusters of CJD have failed to establish a link with exposure to cervids through hunting or consuming the animals, or through other associated behaviors [12,14-18]. Although still unclear for CWD, epidemiological and laboratory findings have established an unequivocal link between Bovine Spongiform Encephalopathy (BSE) and vCJD [11,19-26].

 

On March 13, 2005, a local fire company in Oneida County, New York, hosted approximately 200–250 individuals at their annual Sportsmen's Feast, during which local wild game, including venison (deer meat), was prepared, cooked in various ways, served, and consumed by individuals primarily from Oneida and neighboring counties. Shortly thereafter, laboratory tests indicated that one of the deer served was positive for CWD [27]. Since 2002, the New York State Department of Environmental Conservation has required the mandatory testing of deer for CWD harvested from a domestic deer farm. However, there is no requirement that the meat not be consumed before the results are made available and thus reaction to the finding that the deer tested positive ranged from unconcerned to anger. Feast attendees were exposed to the contaminated meat through a variety of activities, including butchering and processing, cooking, consumption of venison, and/or through contact with contaminated surfaces. This incident represents the only known large scale point-source exposure of humans to an animal with confirmed CWD.

 

In response to this known point-source exposure, the Oneida County Health Department and the State University of New York at Binghamton (SUNY) proposed and launched the Oneida County Chronic Wasting Disease Surveillance Project, a cohort study designed to examine a natural experimental model of human exposure to CWD. The objective of the study are to determine the potential human health risks associated with exposure to CWDcontaminated cervids by following the events and health outcomes of attendees at the Sportsmen's Feast. Study participants are being followed for a minimum period of six years from the time of exposure. This is based on the minimum incubation period and earliest age at onset for known human prion diseases including vCJD [11] and kuru [28-31]. The current report describes the initial results of a risk analysis based on behaviors associated with the wild game feast in upstate New York.

 

snip...

 

General Risk

 

Results regarding General Risk factors are presented in Figure 5. In relation to deer hunting practices, of the 81 participants, 69.1% hunted deer, and of those who hunted, 80.4% harvested a deer between the years 2000–2005. Of those who hunted, 25.0% reported hunting only in Oneida County. Furthermore, a total of 32.1% of the participants indicated that they hunted in the Oneida County CWD containment area, although not exclusively. Of those who hunted, 96.4% field dressed, 75.5% removed antlers and 70.9% butchered harvested deer. However, only 26.4% of them wore gloves while butchering deer. Of those who hunted, 92.7% consumed the deer they killed, and 96.3% of all feast participants reported eating venison outside of the Sportsmen's Feast. Of the 81 participants, 24.6% also reported eating venison from other states. In addition to hunting, the 81 participants reported general contact with animals, including deer (39.5%), cattle (13.6%), sheep (1.2%), alpacas (1.2%), and other animals (9.9%).

 

Discussion

 

The purpose of this report is to describe the events surrounding the exposure of a large cohort of individuals to a CWD-infected animal, and present information on risk behaviors for activities at the feast and general hunting behaviors outside the feast. This study focuses on two levels of risk, that specific to activities at the feast, Feast Risk, and activities outside the feast, General Risk. Because CWD has not been definitively linked to the development of a prion disease in humans, the risk behaviors evaluated reflect those associated with known prion disease transmission routes [11,19-26,28-33].

 

Studies have shown that CWD can be experimentally transmitted to voles [13], non-human primates [7], cattle [5,8], and sheep [9]. Since transmissible spongiform encephalopathies have the ability to cross species barriers and are resistant to degradation [5-10,13,16,18,28,29,32- 34], food chain transmission of prion diseases is a growing human and animal health concern. Infected prions are found in the blood, skeletal muscle, and saliva as well as the central nervous system of infected animals [5,10,33,35]. Contact with these tissues is a likely mode of transmission of CWD from animal to animal, and could potentially present a risk to humans. It is currently considered unlikely that CWD can cross the species barrier to humans [12,14,18]. However, if it can, those with multiple risk factors may be most vulnerable. The information presented above provides a relative scale for cumulative exposures to CWD-infected tissues and surfaces.

 

Since CWD has spread from regions of the Midwest with generally low human population densities to high-density regions of the Eastern U.S. (New York and West Virginia), direct contact between infected cervids and humans has increased. Additionally, the potential for cross-species transmission of CWD from deer to cattle to humans may be increased as a result of increasing contact between large herds of potentially infected cervids and cattle in pastures on smaller Eastern U.S. farms.

 

Conclusion

 

The Oneida County CWD Surveillance Project introduced here, and the data reported in this paper, provides the first step in developing a natural experimental model of possible transmission of CWD prions from deer to humans [36,37] with a known point-source exposure. Surveillance of the cohort will continue for a minimum of six years (and likely extended) through annual follow-up questionnaires including self-report health information. Since human prion diseases are reportable in New York State, the Oneida County Health Department and health departments in other neighboring counties will be especially vigilant in this surveillance effort. This prospective cohort study will provide new information previously unavailable from retrospective studies where the CWD status of cervids was unknown and hunting behaviors only evaluated retrospectively many years after onset of cases of CJD.

 


 

February 21, 2003 / 52(07);125-127

 

Fatal Degenerative Neurologic Illnesses in Men Who Participated in Wild Game Feasts --- Wisconsin, 2002

 

Creutzfeldt-Jakob disease (CJD) is a fatal neurologic disorder in humans. CJD is one of a group of conditions known as transmissible spongiform encephalopathies (TSEs), or prion diseases, that are believed to be caused by abnormally configured, host-encoded prion proteins that accumulate in the central nervous tissue (1). CJD has an annual incidence of approximately 1 case per million population in the United States (1) and occurs in three forms: sporadic, genetically determined, and acquired by infection. In the latter form, the incubation period is measured typically in years. Recent evidence that prion infection can cross the species barrier between humans and cattle has raised increasing public health concerns about the possible transmission to humans of a TSE among deer and elk known as chronic wasting disease (CWD) (2). During 1993--1999, three men who participated in wild game feasts in northern Wisconsin died of degenerative neurologic illnesses. This report documents the investigation of these deaths, which was initiated in August 2002 and which confirmed the death of only one person from CJD. Although no association between CWD and CJD was found, continued surveillance of both diseases remains important to assess the possible risk for CWD transmission to humans.

 

Case Reports

 

Case 1. In December 1992, a Wisconsin man aged 66 years with a history of seizures since 1969 sought treatment for recurring seizures, increasing forgetfulness, and worsening hand tremors. Electroencephalographic (EEG) examination demonstrated focal epileptiform activity and nonspecific diffuse abnormalities, but no specific diagnosis was made. In February 1993, he was hospitalized for increasing confusion, ataxia, and movement tremors of his extremities. A magnetic resonance image (MRI) demonstrated mild, nonspecific enhancement along the inferior parasagittal occipital lobe. A repeat EEG showed bifrontal intermittent, short-interval, periodic sharp waves, suggesting a progressive encephalopathy; a diagnosis of CJD was suspected. The man died later that month; neuropathologic examination of brain tissue during autopsy indicated subacute spongiform encephalopathy, compatible with CJD.

 

The man was a lifelong hunter who ate venison frequently. He hunted primarily in northern Wisconsin but also at least once in Montana. He hosted wild game feasts at his cabin in northern Wisconsin from 1976 until shortly before his death. Fixed brain tissue obtained during the autopsy was sent for analysis to the National Prion Disease Pathology Surveillance Center (NPDPSC) and reexamined at the institution where the autopsy was conducted. Histopathologic examination did not substantiate the diagnosis of prion disease. In addition, 27 brain tissue sections were negative for prions by immunostaining despite positive antibody reactions against other proteins (controls), which indicated that other epitopes in the tissue samples were preserved.

 

Case 2. In May 1999, a Minnesota man aged 55 years with no previous history of a neurologic disease sought evaluation and treatment following a 3-month history of progressive difficulty in writing and unsteadiness of gait. A computerized tomography (CT) scan and MRI examination of his head did not indicate any abnormality. In June 1999, he was hospitalized following onset of dementia, speech abnormalities, and myoclonic jerking. An EEG indicated left-hemispheric periodic sharp waves and moderate generalized background slowing; CJD was diagnosed clinically. In July 1999, following worsening symptoms and development of right upper extremity dystonia, the patient died. Neuropathologic evaluation of brain tissue during autopsy demonstrated widespread subcortical spongiform lesions, consistent with CJD.

 

The man was not a hunter but had a history of eating venison. He made an estimated 12 visits to the cabin where the wild game feasts were held, but he participated in only one feast during the mid-1980s. Sections of fixed and frozen brain tissue obtained during autopsy were analyzed at NPDPSC, and prion disease was confirmed by immunohistochemical and Western blot testing. The Western blot characteristics and prion disease phenotype in this patient were consistent with the most common form of sporadic CJD, classified as M/M (M/V) 1 (3). Subsequent genetic typing confirmed the presence of methionine homozygosity (M/M) at codon 129 of the patient's prion protein gene.

 

Case 3. In June 1992, a Wisconsin man aged 65 years sought treatment for progressive slowing of speech, worsening memory, and personality changes. By January 1993, his speech was reduced to one-word utterances. Neurologic examination showed a flat affect, decreased reflexes, and apraxia. A CT head scan showed mild atrophy, and an EEG was normal. Pick's disease was diagnosed. By May, he was unable to perform any daily living activities; he died in August 1993. Neuropathologic evaluation of brain tissue during autopsy showed symmetrical frontal lobe cerebral cortical atrophy and mild temporal lobe atrophy. No Pick's bodies or spongiform lesions were observed.

 

The man had a history of eating venison and participated regularly in wild game feasts held at the cabin owned by patient 1. He was a lifelong hunter and hunted mostly in Wisconsin but also in Wyoming and British Columbia. No game was brought to the wild game feasts from his hunting trips outside of Wisconsin. Examination of fixed brain tissue sent to NPDPSC demonstrated no lesions indicative of CJD, and immunohistochemical testing with antibody to the prion protein did not demonstrate the granular deposits seen in prion diseases.

 

Epidemiologic Investigation

 

Wild game feasts consisting of elk, deer, antelope, and other game that occurred at a cabin in northern Wisconsin owned by patient 1 began in 1976 and continued through 2002. These feasts typically involved 10--15 participants and usually occurred on weekends before or during hunting seasons in the fall and occasionally in the spring. Wild game brought to these feasts usually were harvested in Wisconsin, but three men who attended these feasts reported hunting in the western United States and bringing game back to Wisconsin. These activities took place in Colorado (near the towns of Cortez, Trinidad, Collbran, Durango, and Meeker), Wyoming (near the towns of Gilette and Cody), and Montana (near the town of Malta). CWD was not known to be endemic in these areas at the time that these hunting activities took place.

 

Information was obtained for 45 (85%) of 53 persons who were identified as possibly participating in the wild game feasts; all were male. Information was obtained by direct interview or from family members of decedents. Of the 45 persons, for whom information was obtained, 34 were reported to have attended wild game feasts. Seven of the 34 feast attendees were deceased, including the three patients. None of the four other decedents had a cause of death attributed to or associated with a degenerative neurologic disorder. None of the living participants had any signs or symptoms consistent with a degenerative neurologic disorder.

 

Reported by: JP Davis, MD, J Kazmierczak, DVM, M Wegner, MD, R Wierzba, Div of Public Health, State of Wisconsin Dept of Health and Family Svcs. P Gambetti, National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, Ohio. L Schonberger, MD, R Maddox, MPH, E Belay, MD, Div of Viral and Rickettsial Diseases, National Center for Infectious Diseases; V Hsu, MD, EIS Officer, CDC.

 

Editorial Note:

 

CWD was first described in the United States in the 1960s and classified as a TSE in 1978. Previously localized to a contiguous endemic area in northeastern Colorado and southeast Wyoming, since 2000, CWD has been found in free-ranging deer or elk in Illinois, Nebraska, New Mexico, South Dakota, Wisconsin, and outside the previously known endemic areas of Colorado and Wyoming. CWD has been identified also in captive deer or elk in Colorado, Kansas, Minnesota, Montana, Nebraska, Oklahoma, South Dakota, and Wisconsin (4). Because a variant form of CJD, with specific neuropathologic and molecular characteristics that distinguish it from sporadic CJD, has been associated with eating cattle products infected with a prion that causes bovine spongiform encephalopathy (5), concern has been raised about the possibility that the prion associated with CWD might be transmitted to humans in a similar way.

 

In this investigation, because only one of the three cases in Wisconsin had neuropathologic confirmation of a prion disease, no association could be made between case participation in the wild game feasts and the development of CJD. Although patient 2 had confirmed CJD, he was unlikely to have eaten CWD-infected venison at these feasts because venison and other game from outside Wisconsin that was served at these feasts did not originate from known CWD-endemic areas, and the man participated in the feasts only once. In addition, the prion disease in this case was consistent with the most common form of sporadic CJD, without apparent unusual neuropathologic or molecular characteristics that might occur if the prion related to CWD had been responsible for the disease.

 

The findings in this report are subject to at least two limitations. First, not all members participating in wild game feasts could be identified, and not all persons listed as participating could be contacted for interviews. Second, interviews that were conducted required recall of events that occurred up to 25 years ago, limiting the detail or accuracy of events. However, the similar responses obtained from different sources support the accuracy of the investigation findings.

 

A previous investigation of unusually young CJD patients in whom the transmission of CWD was suspected also did not provide convincing evidence for a causal relationship between CWD and CJD (2). However, limited epidemiologic investigations cannot rule out the possibility that CWD might play a role in causing human illness. Ongoing surveillance of CJD, particularly in states with CWD, is important to assess the risk, if any, for CWD transmission to humans. Because the confirmation of CJD and the detection of a new prion disease require neuropathologic study of brain tissue, physicians are encouraged to contact NPDPSC (http://www.cjdsurveillance.com; telephone, 216-368-0587) to confirm diagnoses of CJD and to distinguish its various subtypes. Because of the known severity of TSEs in humans and the possibility that the CWD prion can affect humans, animals with evidence of CWD should be excluded from the human food or animal feed chains. Hunters and wild venison consumers should follow precautionary guidelines available from the Wisconsin Department of Agriculture, Trade, and Consumer Protection (http://datcp.state.wi.us/core/consumerinfo) to prevent potential exposures to the CWD agent.

 

References

 


 

 

PLEASE NOTE ;

 

*** In some cases, the incubation period may be as long as 50 years

 


 

At a hearing in Parliament last Wednesday, the Science and Technology Committee was told that vCJD continued to pose a “significant” risk to UK public health and that more than one in every 2000 people could be silent carriers of the disease. *** vCJD can have an incubation period of over 30 years.

 

Monday, February 03, 2014

 

CREUTZFELDT-JAKOB DISEASE T.S.E. PRION U.K. UPDATE As at 3rd February 2014

 


 

*** Because typical clinical signs of BSE cannot always be observed in nonambulatory disabled cattle, and because evidence has indicated these cattle are more likely to have BSE than apparently healthy cattle, FDA is designating material from nonambulatory disabled cattle as prohibited cattle materials.

 


 


 


 


 

***In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures.

 


 

Hence, the data presented here are important for a risk- based SRM definition.

 

Competing interests

 

The authors declare that they have no competing interests.

 


 

see much more here ;

 

Saturday, December 21, 2013

 

Complementary studies detecting classical bovine spongiform encephalopathy infectivity in jejunum, ileum and ileocaecal junction in incubating cattle

 


 

From: TSS

 

Subject: CWD--THE FEAST--Investigators find no common source in hunters' deaths $$$ (or did they???)

 

Date: November 22, 2002 at 6:22 am PST

 

Investigators find no common source in hunters' deaths 2 of 3 show absence of prions

 

By JOHN FAUBER and LEE BERGQUIST jfauber@journalsentinel.com Last Updated: Nov. 21, 2002

 

Three hunters who ate wild game together and later died of rare brain disorders did not contract their diseases from a common source such as venison, a four-month-long investigation concluded Thursday. [19335] Chronic Wasting Disease For complete archived coverage of chronic wasting disease in Wisconsin, go to our SPECIAL SECTION Quotable The idea here is that there is no fear now. These cases are one of the things that stopped many spouses, or hunters from hunting, because it sounded so plausible - and now it is completely debunked. - Fred Bannister, Chetek physician who attended many game dinners with the late Wayne Waterhouse Related Coverage Deer hunting: Season starts saturday Section: Outdoors Section: Chronic wasting disease

 

A report by federal and state health investigators found that one of the men apparently had been misdiagnosed with Creutzfeldt-Jakob disease, a neurological disorder caused by prions. Prions are the same unusual infectious agents that cause chronic wasting disease in deer and elk.

 

Pathologists were able to run new tests of brain tissue from Wayne Waterhouse of Chetek, who died in 1993, and determined that there was no evidence of a prion-related illness, said Jeffrey Davis, the state epidemiologist for communicable diseases.

 

Health officials said they did not know what brain disorder killed Waterhouse, an avid hunter and outdoorsman.

 

In September, health officials said new test results from brain tissue samples of another man, Roger Marten of Mondovi, also showed no evidence of prions or Creutzfeldt-Jakob disease. A third man, James Botts of Minneapolis, did die of the disease, the new analysis of his tissue confirmed.

 

"These results are important because if all three men had developed a rare disease like CJD (Creutzfeldt-Jakob disease), such a cluster would suggest a common source of exposure," Davis said. "Thanks to a new testing process not available at the time of the initial diagnosis of CJD in these patients, we were able to demonstrate the absence of prions in brain tissues of two of the patients."

 

The new analysis, part of a joint investigation by the Wisconsin Division of Health and the U.S. Centers for Disease Control and Prevention, contradicts a 1993 diagnosis made at the Mayo Clinic, where Waterhouse died.

 

The new report could buoy the confidence of deer hunters. The report was issued two days before the start of the 2002 gun deer season.

 

The hunt is considered the most important in decades because of a plan by the state Department of Natural Resources to eradicate 25,000 deer in a 411-square-mile region of Dane, Iowa and Sauk counties to control the spread of chronic wasting disease.

 

The disease has prompted questions about the safety of venison and has helped drive down the number of deer hunters buying licenses this year.

 

"From a hunters' standpoint, (the report) may give hunters a little more confidence about consuming venison," said Darrell Bazzell, secretary of the DNR.

 

Sales of deer-hunting licenses have picked up in the past few weeks, but as of Wednesday sales lagged 16% behind the same time last year, according to the DNR. So far this year, there have been 118,441 fewer licenses sold.

 

Bazzell said the new findings could give an additional last-minute boost to license sales.

 

Thursday's report contradicts the findings of a Mayo Clinic doctor, who in a May 17, 1993, letter to Waterhouse's family said a postmortem exam of Waterhouse confirmed that he died of Creutzfeldt-Jakob disease.

 

In fact, when reached at home on Thursday night, Joseph Parisi, a Mayo pathologist, said the new tests on Waterhouse were "inconclusive" and could not confirm that he died of the disease.

 

That differs from a statement issued Thursday by Davis' office, which says that Waterhouse did not have Creutzfeldt-Jakob disease. Davis also said the latest analysis was confirmed by pathologists at the National Prion Disease Pathology Surveillance Center in Cleveland.

 

The center receives federal funding to monitor prion diseases, including the human version of mad cow disease, and is considered one of the top prion labs in the country.

 

Davis said it was his understanding that pathologists at the lab concurred with pathologists at Mayo and also were in agreement on the new analysis of Waterhouse's tissue.

 

The case of the three outdoorsmen was first reported in the Journal Sentinel in July and has since attracted widespread attention and heightened concerns about the safety of venison.

 

Waterhouse, Marten and Botts, a former Chetek resident who later moved to Minneapolis, all had eaten wild game at the Waterhouse family cabin on the Brule River in northern Wisconsin.

 

Chetek physician Fred Bannister, who attended many of the game dinners with Waterhouse, emphasized that no deer from the dinners came from the 411-square-mile eradication zone.

 

Bannister, also a deer hunter, said he had been waiting for scientific corroboration that his friend had not died of Creutzfeld-Jacob disease.

 

"How can you have good news about someone who died?" Bannister said. "But the idea here is that there is no fear now. These cases are one of the things that stopped many spouses, or hunters from hunting, because it sounded so plausible - and now it is completely debunked."

 

However, Judy Botts, wife of James Botts, said the new findings had not changed her mind.

 

Botts still suspects that her husband's consumption of venison played a role in his death, "but it can't be proven," she said. "I knew it all along."

 

Botts died in 1999. New analysis of his tissue confirmed his diagnosis of Creutzfeldt-Jakob disease, which occurs at the annual rate of about one per million people.

 

Marten died in 1993. He initially was diagnosed with Pick's disease, another rare brain disease. A new analysis of his brain tissue found that although he did have Pick's, he did not have a prion disease.

 

About 75 men were known to attend the wild game feasts, Davis said. Investigators have been able to contact about 45 of them. No other case of rare brain disease has been found.

 

Davis said the new findings were consistent with earlier statements by health organizations that chronic wasting disease prion "has not been shown to cause human illness."

 

Some neurologists and prion researchers have cautioned that the question of whether chronic wasting disease can jump to people remains unanswered.

 

Some have said that until proved otherwise, it is reasonable to assume that the disease may jump to people in a manner similar to mad cow disease. Mad cow disease is believed to have caused at least 130 cases of variant-Creutzfeldt-Jakob disease, an always fatal disorder, in about 130 people, mainly in Great Britain.

 

The new information could entice some wavering hunters to head into the woods, said David Ladd, chairman of the Big Game Committee of the Conservation Congress, a citizens group that advises the DNR.

 

But Ladd said the discovery of chronic wasting disease had altered the psyche of the 2002 hunt.

 

"A lot of hunters are probably not going to make a decision until they pull the trigger," he said.

 

A version of this story appeared in the Milwaukee Journal Sentinel on Nov. 22, 2002.

 


 

HMMM???????

 

SNIP...

 

Thursday's report contradicts the findings of a Mayo Clinic doctor, who in a May 17, 1993, letter to Waterhouse's family said a postmortem exam of Waterhouse confirmed that he died of Creutzfeldt-Jakob disease.

 

In fact, when reached at home on Thursday night, Joseph Parisi, a Mayo pathologist, said the new tests on Waterhouse were "inconclusive" and could not confirm that he died of the disease.

 

That differs from a statement issued Thursday by Davis' office, which says that Waterhouse did not have Creutzfeldt-Jakob disease. Davis also said the latest analysis was confirmed by pathologists at the National Prion Disease Pathology Surveillance Center in Cleveland.

 

The center receives federal funding to monitor prion diseases, including the human version of mad cow disease, and is considered one of the top prion labs in the country.

 

SNIP...

 

$$$

 

TSS

 

11/21/02, LAB FINDINGS RELEASED ON FATAL CASES OF NEUROLOGIC DISEASES IN OUTDOORSMEN

 

Contact: Jeffrey P. Davis, M.D. (608) 267-9003 James Kazmierczak, DVM (608) 266-2154 CDC Press Office (404) 639-3286

 

FOR IMMEDIATE RELEASE

 

(MADISON ? November 21, 2002) -- The Wisconsin Division of Public Health today released completed test results related to the investigation into the fatal cases of degenerative neurological illnesses in three men who consumed wild game served during a series of feasts.

 

The test results announced today indicate that Wayne Waterhouse, a northern Wisconsin resident who died in 1993, did not have Creutzfeldt-Jakob disease (CJD) or any other evidence of prions or prion-related illness. Results already made public indicated that another of the patients, Roger Marten, a northern Wisconsin resident who died in 1993, also did not have CJD or any other evidence of prions or prion-related illness. Only one of the three men, James Botts, a Minnesota resident who died in 1999, had a confirmed diagnosis of CJD.

 

"These results are important because if all three men had developed a rare disease like CJD, such a cluster would suggest a common source of exposure," said Dr. Jeffrey Davis, Wisconsin State Epidemiologist for Communicable Diseases, in reporting the findings. "Thanks to a new testing process not available at the time of the initial diagnosis of CJD in these patients, we are able to demonstrate the absence of prions in the brain tissues of two of the patients. Therefore, these three cases cannot be attributed to a common source of illness."

 

The current investigation, conducted by the Division of Public Health and the U.S. Centers for Disease Control and Prevention, was initiated after reports surfaced that rare degenerative neurological diseases had occurred in three acquaintances who shared meals of wild game in northern Wisconsin. The reports generated considerable public interest due to the concern that these illnesses might somehow be linked to chronic wasting disease (CWD) of deer and elk.

 

"These findings are consistent with earlier statements by the CDC and the World Health Organization that the CWD prion has not been shown to cause human illness," Dr. Davis added. "They also illustrate the ongoing need to apply the most current scientific techniques to the important issue of understanding CWD and other prion-related conditions."

 

Specimens of brain tissue from each of the three men had been collected during the individuals? autopsies. The tissues were recently forwarded to the National Prion Disease Pathology Surveillance Center in Cleveland, Ohio, where pathologists examined the specimens for evidence of CJD and the presence of abnormal prion proteins which cause the illness. This pathology center was established during 1996-1997 by CDC in collaboration with the American Association of Neuropathologists to enable state-of-the-art laboratory investigation of physician-diagnosed and suspected cases of prion disease in the United States.

 

Creutzfeldt-Jakob disease is a fatal degenerative brain condition of humans believed to be caused by an abnormally-shaped protein called a prion. It occurs at a rate of about one case per million people per year throughout much of the world, and was first described in the 1920s. Chronic wasting disease in deer and elk is also believed to be caused by a prion and produces lesions in the brain, but the deer CWD prion has not been shown to affect humans.

 

Dr. Davis reiterated that while these new results are reassuring, it is still impossible to say with absolute certainty that the CWD prion will never cause human illness. As a precaution, he continues to advise that hunters process their venison in a safe manner and not ingest tissues where the CWD prion is known to concentrate. These tissues include brain, spinal cord, eyes, lymph nodes and spleen. The Wisconsin Department of Agriculture, Trade, and Consumer Protection has issued recommendations on processing deer. These can be found on their website at http://datcp.state.wi.us/ah/agriculture/animals/disease/chronic/pdf/venison_safety_2side.pdf .

 

-30-

 

Last Revised: November 21, 2002

 


 

Date: Fri, 22 Nov 2002 14:14:12 –0600

 

Reply-To: Bovine Spongiform Encephalopathy Sender: Bovine Spongiform Encephalopathy

 

From: "Terry S. Singeltary Sr."

 

Subject: Re: Investigators find no common source in hunters' deaths $$$ (or did they???)

 

######## Bovine Spongiform Encephalopathy #########

 

greetings list members,

 

"jimmeny cricket" !!!

 

how about a statement from Gambetti et al, instead of a bunch of alleged second hand statements.

 

does state epidemiologist or even mayo really have access to a specialized panel of _proven_ antibodies and know how with prion ihc??? probably not.

 

only gambetti and prusiner have the necessary reference collections if i am not mistaken $$$

 

who validated it, false positive, false negatives?

 

what's going on here???

 

just in time too to avert a public panic and at the opening weekend of deer hunting season at that, how convenient and excellent timing$$$

 

unpublished, unpeer-reviewed = unreliable

 

and just more BSeee.

 

SNIP...

 

THE FEAST WISCONSIS

 

Wednesday, November 16, 2011

 

Wisconsin Creutzfeldt Jakob Disease, CWD, TSE, PRION REPORTING 2011

 


 

Q93. If Chronic Wasting Disease was found in an area where you deer hunt in Maryland and regulations were implemented to prohibit the removal of whole deer carcasses from the area, do you agree or disagree that you would stop deer hunting in that area?

 

Strongly agree

 

20 26 33 32

 

Percent who strongly or moderately agree they would stop deer hunting given the following conditions.

 

30 39 49 44

 

Q93. If Chronic Wasting Disease was found in an area where you deer hunt in Maryland and regulations were implemented to prohibit the removal of whole deer carcasses from the area

 


 

Hunters’ Attitudes Toward CWD and Management Efforts in Hampshire County 65 Note: Graph shows results obtained from seven questions. Each item was asked about individually.

 

14

 

28

 

28

 

27

 

27

 

27

 

19

 

0 20 40 60 80 100

 

Q87. The presence of Chronic Wasting Disease in Hampshire County

 

Q91. The ban on baiting and feeding deer in Hampshire County

 

Q88. Because you are concerned the deer you harvest or the meat you consume might be infected with Chronic Wasting Disease

 

Q92. The carcass transportation restrictions for Hampshire County

 

Q89. Because you feel you cannot be sure the deer you harvest in Hampshire County is not infected with Chronic Wasting Disease

 

Q90. Because you are concerned you might get Chronic Wasting Disease from deer in Hampshire County

 

Q93. The presence of the West Virginia Division of Natural Resources at checking stations in Hampshire County Percent (n=259)

 

Percent who indicated that the following strongly or moderately influenced their decision to deer hunt less or stop deer hunting in Hampshire County since 2004. (Among those whose deer hunting participation in Hampshire County since 2004 decreased or stopped.)

 


 

THESIS HUNTERS’ RESPONSE TO CHRONIC WASTING DISEASE IN FOUR STATES

 

Submitted by Katie M. Lyon Department of Human Dimensions of Natural Resources In partial fulfillment of the requirements For the Degree of Master of Science Colorado State University Fort Collins, Colorado Spring 2011

 

snip...

 

Results

 

Bivariate analysis

 

Across the entire sample, 27% of respondents indicated that they would stop hunting because of CWD (Table I). All five independent variables were statistically significant predictors of stopping hunting in the state and thus provide evidence to support the first hypothesis. The greater the prevalence of CWD in the state the more likely hunters were to quit. At the lowest hypothetical prevalence level, 13% indicated that they would no longer hunt in the state. When prevalence reached 50% statewide, 52% said that they would stop hunting. The difference in these distributions was statistically significant (χ2 = 3,338.46, p < .001, r = .37).

 

If CWD were to cause human death, respondents were significantly more likely to stop hunting in the state (χ2 = 1,187.99, p < .001, r = .25). Forty-three percent indicated that they would quit hunting in the hypothetical scenarios where a hunter had died due to CWD; only 19% said they would stop in the “no human death” scenarios. When hunters’ perceived extreme risks associated with CWD, 46% would stop hunting in the state. By comparison, 19% would quit hunting when they perceived no CWD related risks (χ2 = 600.27, p < .001, r = .17).

 

Whether or not CWD had been detected in the state and the respondents’ state of residency were also significant predictors of hunters’ behavioral intentions. Individuals who had hunted in states that did not have CWD were slightly more likely (30%) to stop hunting than those who had hunted in a CWD state (25%). Nonresidents (29%) were slightly more likely to quit than residents (24%). These relationships, however, were not strong for either the presence of CWD in a state or residency (r = -.05 in both cases).

 

snip...

 

The significant 3-way interaction quit hunting * perceived risk * resident, for example, indicated that nonresidents of the state who perceived greater risk were more likely to quit hunting deer in the state. In the 4-way interaction, stopping hunting increased: (a) when prevalence increased, (b) a human death attributable to CWD had occurred, and (c) if CWD had been detected in the state. Under the worst case scenario (i.e., 50% prevalence statewide, human death, a non-CWD state), 64% of the respondents would stop hunting in the state (Table 3.3). If the prevalence of CWD was 50% statewide, a human death had occurred, and the disease had been detected in the state, 60% would quit hunting. Consistent with past research, if CWD is concentrated in a single area at relatively low prevalence levels, few hunters would quit the activity.

 

snip...

 

Interactions among the predictors were hypothesized to increase the potential for stopping hunting in the state. Multivariate analysis confirmed that the decision to stop hunting interacted with all five predictors and suggested that combinations of these predictors increase the probability of quitting. The 4-way interaction, for example, revealed that 60% or more of our respondents would stop hunting if CWD prevalence ever reached 50% statewide and a human death attributable to CWD had occurred. These findings support our second hypothesis and have implications for management, theory, and research.

 


 

Human Dimensions of Wildlife, 9:211–231, 2004 Copyright © Taylor & Francis Inc. ISSN: 1087–1209 print / 1533-158X online DOI: 10.1080/10871200490479990

 

Hunters’ Behavior and Acceptance of Management Actions Related to Chronic Wasting Disease in Eight States

 

MARK D. NEEDHAM JERRY J. VASKE MICHAEL J. MANFREDO Department of Natural Resource Recreation and Tourism Human Dimensions in Natural Resources Unit Colorado State University Fort Collins, Colorado, USA

 

The impacts of chronic wasting disease (CWD) on hunters’ behavior and beliefs about acceptable management actions are not clearly understood. This article presents findings from an initial phase of a multi-stage, multi-state effort to address these knowledge gaps. Data were obtained from mail surveys (n = 659) of resident and nonresident deer hunters in eight states and elk hunters in three states. Hunters were presented with hypothetical situations of increasing:

 

(1) CWD prevalence (all eight states), and

 

(2) human health risks (two states).

 

Logistic regression equations estimated that at current prevalence levels in some states, 3% (residents) to 5% (nonresidents) of hunters would stop hunting deer/elk in their state.

 

If 50% of the deer or elk across the state were infected, approximately 42% (residents) and 54% (nonresidents) would stop hunting deer/elk in their state.

 

In hypothetical situations where a hunter died from CWD at this prevalence level, the percentage was 68%.

 

Potential for conflict indices (PCI) showed that as prevalence and human health risks increased, acceptability of testing and lethal management increased and acceptability of allowing CWD to take its natural course decreased.

 

snip...

 

Hunters’ Responses to CWD in Eight States 219

 

Results

 

Descriptive and Bivariate Findings

 

In total, 5% of the hunters reported that they would stop hunting deer/elk in the state if 10% of the deer or elk in zone A and 0% in the rest of the state (zones B and C) were infected with CWD (Table 1). This prevalence level is consistent with current conditions in parts of some states (e.g., Colorado, Wyoming). The percentage of respondents that would stop hunting deer/elk in the state increased as prevalence and distribution increased. For example, if CWD prevalence was 50% in zone A, 30% in zone B, and 10% in zone C, 32% of hunters would stop hunting deer/elk in the state. If 50% of the deer or elk across the entire state were infected, 49% of hunters reported that they would stop hunting deer/elk in the state.2 Across all eight states, a similar proportion of respondents hunted most often in zone A (30%), B (33%), or C (37%) in 2002.

 

snip...

 

Results, however, suggest more serious potential ramifications of CWD. Research has shown that although it is unlikely to occur, CWD can reach higher prevalence levels in deer and elk populations (Gross & Miller, 2001; Miller et al., 2000; Williams & Young, 1980) and the potential for human susceptibility to CWD may exist (Belay et al., 2004; Raymond et al., 2000). If CWD prevalence among deer or elk ever increases to 50% across a state, 49% of hunters will stop hunting deer/elk in the state. Based on the findings from South Dakota and Wisconsin, 60% to 68% of hunters will stop hunting deer/elk in their state if this Hunters’ Responses to CWD in Eight States 227 prevalence level exists and CWD is shown to be transmissible to humans or cause human death. Even at current prevalence levels (e.g., 10%) in parts of some states (e.g., Colorado, Wyoming), 16% to 20% of hunters will stop hunting deer/ elk in their state if CWD affects humans or causes human death.

 

These findings suggest that if CWD prevalence increases dramatically, deer and/or elk hunting participation will substantially decrease in several states. If high levels of prevalence are combined with threats to human health, the decline could be even greater. This could have compounding and catastrophic effects on revenues for wildlife agencies, financial and logistical support for wildlife programs, management and control of deer and elk populations, public support for wildlife agencies and their ability to manage wildlife resources, the preservation of cultural and family traditions, and the economic viability of rural communities that are dependent on hunting revenues. Findings also suggested that nonresident hunters are more likely than residents to stop hunting deer/elk in the state as CWD conditions worsen. Declining numbers of nonresidents could significantly reduce agency revenue from license sales because they often pay much higher fees for hunting licenses. Taken together, these consequences of a decline in hunting participation due to CWD suggest the need for agencies and other stakeholders to engage in long-term and proactive management planning efforts for addressing the disease.

 

Although most of the CWD conditions manipulated in this study (i.e., high CWD prevalence, human health risks) are extremely unlikely, increased testing of harvested deer and elk (i.e., postmortem samples), advancements in lymphoid and tonsillar biopsy techniques for testing live animals (i.e., antemortem sampling), and in-vitro laboratory experiments of CWD in human cells may provide a more realistic assessment of current and future CWD prevalence levels and possible risks to human health associated with the disease (Raymond et al., 2000; Sigurdson et al., 1999; Wild, Spraker, Sigurdson, O’Rourke, & Miller, 2002; Wolfe et al., 2002).

 

snip...

 

Keywords chronic wasting disease, hunting, risk behavior, wildlife management, potential for conflict index

 

This article is based on a project of the Human Dimensions Committee of the Western Association of Fish and Wildlife Agencies (WAFWA). The authors thank Chris Burkett (Wyoming Game and Fish Department), Dana Dolsen (Utah Division of Wildlife Resources), Jacquie Ermer (North Dakota Game and Fish Department), Larry Gigliotti (South Dakota Department of Game, Fish and Parks), Ty Gray (Arizona Game and Fish Department), Larry Kruckenberg (Wyoming Game and Fish Department), Bruce Morrison (Nebraska Game and Parks Commission), Peter Newman (Colorado State University), Jordan Petchenik (Wisconsin Department of Natural Resources), Duane Shroufe (Arizona Game and Fish Department), Linda Sikorowski (Colorado Division of Wildlife), and Tara Teel (Colorado State University) for their assistance.

 

Address correspondence to Mark D. Needham, Department of Natural Resource Recreation and Tourism, Human Dimensions in Natural Resources Unit, Colorado State University, Fort Collins, Colorado 80523-1480, USA. E-mail: mneedham@cnr.colostate.edu

 


 

CWD STRAINS TO HUMANS ???

 

*** These results would seem to suggest that CWD does indeed have zoonotic potential, at least as judged by the compatibility of CWD prions and their human PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests that if zoonotic CWD occurred, it would most likely effect those of the PRNP codon 129-MM genotype and that the PrPres type would be similar to that found in the most common subtype of sCJD (MM1).

 


 

as I said, what if ?

 

*** our results raise the possibility that CJD cases classified as VV1 may include cases caused by iatrogenic transmission of sCJD-MM1 prions or food-borne infection by type 1 prions from animals, e.g., chronic wasting disease prions in cervid. In fact, two CJD-VV1 patients who hunted deer or consumed venison have been reported (40, 41). The results of the present study emphasize the need for traceback studies and careful re-examination of the biochemical properties of sCJD-VV1 prions. ***

 


 

===========================================

 

Thursday, January 2, 2014

 

*** CWD TSE Prion in cervids to hTGmice, Heidenhain Variant Creutzfeldt-Jacob Disease MM1 genotype, and iatrogenic CJD ??? ***

 

WHAT IF ?

 


 

Saturday, April 19, 2014

 

Exploring the zoonotic potential of animal prion diseases: In vivo and in vitro approaches

 


 

*** PPo3-7: Prion Transmission from Cervids to Humans is Strain-dependent

 

*** Here we report that a human prion strain that had adopted the cervid prion protein (PrP) sequence through passage in cervidized transgenic mice efficiently infected transgenic mice expressing human PrP,

 

*** indicating that the species barrier from cervid to humans is prion strain-dependent and humans can be vulnerable to novel cervid prion strains.

 

PPo2-27:

 

Generation of a Novel form of Human PrPSc by Inter-species Transmission of Cervid Prions

 

*** Our findings suggest that CWD prions have the capability to infect humans, and that this ability depends on CWD strain adaptation, implying that the risk for human health progressively increases with the spread of CWD among cervids.

 

PPo2-7:

 

Biochemical and Biophysical Characterization of Different CWD Isolates

 

*** The data presented here substantiate and expand previous reports on the existence of different CWD strains.

 


 

Envt.07:

 

Pathological Prion Protein (PrPTSE) in Skeletal Muscles of Farmed and Free Ranging White-Tailed Deer Infected with Chronic Wasting Disease

 

***The presence and seeding activity of PrPTSE in skeletal muscle from CWD-infected cervids suggests prevention of such tissue in the human diet as a precautionary measure for food safety, pending on further clarification of whether CWD may be transmissible to humans.

 


 

>>>CHRONIC WASTING DISEASE , THERE WAS NO ABSOLUTE BARRIER TO CONVERSION OF THE HUMAN PRION PROTEIN<<<

 

*** PRICE OF CWD TSE PRION POKER GOES UP 2014 ***

 

Transmissible Spongiform Encephalopathy TSE PRION update January 2, 2014

 

Wednesday, January 01, 2014

 

Molecular Barriers to Zoonotic Transmission of Prions

 

*** chronic wasting disease, there was no absolute barrier to conversion of the human prion protein.

 

*** Furthermore, the form of human PrPres produced in this in vitro assay when seeded with CWD, resembles that found in the most common human prion disease, namely sCJD of the MM1 subtype.

 


 


 

PRION2013 CONGRESSIONAL ABSTRACTS CWD

 

Sunday, August 25, 2013

 

HD.13: CWD infection in the spleen of humanized transgenic mice

 

***These results indicate that the CWD prion may have the potential to infect human peripheral lymphoid tissues.

 

Oral.15: Molecular barriers to zoonotic prion transmission: Comparison of the ability of sheep, cattle and deer prion disease isolates to convert normal human prion protein to its pathological isoform in a cell-free system ***However, they also show that there is no absolute barrier ro conversion of human prion protein in the case of chronic wasting disease.

 

PRION2013 CONGRESSIONAL ABSTRACTS CWD

 

Sunday, August 25, 2013

 

***Chronic Wasting Disease CWD risk factors, humans, domestic cats, blood, and mother to offspring transmission

 


 

Friday, November 09, 2012

 

*** Chronic Wasting Disease CWD in cervidae and transmission to other species

 


 

there is in fact evidence that the potential for cwd transmission to humans can NOT be ruled out.

 

I thought your readers and hunters and those that consume the venison, should have all the scientific facts, personally, I don’t care what you eat, but if it effects me and my family down the road, it should then concern everyone, and the potential of iatrogenic transmission of the TSE prion is real i.e. ‘friendly fire’, medical, surgical, dental, blood, tissue, and or products there from...like deer antler velvet and TSE prions and nutritional supplements there from, all a potential risk factor that should not be ignored or silenced. ...

 

the prion gods at the cdc state that there is ;

 

''no strong evidence''

 

but let's see exactly what the authors of this cwd to human at the cdc state ;

 

now, let’s see what the authors said about this casual link, personal communications years ago. see where it is stated NO STRONG evidence. so, does this mean there IS casual evidence ????

 

“Our conclusion stating that we found no strong evidence of CWD transmission to humans”

 

From: TSS (216-119-163-189.ipset45.wt.net)

 

Subject: CWD aka MAD DEER/ELK TO HUMANS ???

 

Date: September 30, 2002 at 7:06 am PST

 

From: "Belay, Ermias"

 

To:

 

Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias"

 

Sent: Monday, September 30, 2002 9:22 AM

 

Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS

 

Dear Sir/Madam,

 

In the Archives of Neurology you quoted (the abstract of which was attached to your email), we did not say CWD in humans will present like variant CJD.

 

That assumption would be wrong. I encourage you to read the whole article and call me if you have questions or need more clarification (phone: 404-639-3091). Also, we do not claim that "no-one has ever been infected with prion disease from eating venison." Our conclusion stating that we found no strong evidence of CWD transmission to humans in the article you quoted or in any other forum is limited to the patients we investigated.

 

Ermias Belay, M.D. Centers for Disease Control and Prevention

 

-----Original Message-----

 

From:

 

Sent: Sunday, September 29, 2002 10:15 AM

 

To: rr26k@nih.gov; rrace@niaid.nih.gov; ebb8@CDC.GOV

 

Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS

 

Sunday, November 10, 2002 6:26 PM ......snip........end..............TSS

 

Thursday, April 03, 2008

 

A prion disease of cervids: Chronic wasting disease

 

2008 1: Vet Res. 2008 Apr 3;39(4):41

 

A prion disease of cervids: Chronic wasting disease

 

Sigurdson CJ.

 

snip...

 

*** twenty-seven CJD patients who regularly consumed venison were reported to the Surveillance Center***,

 

snip...

 

full text ;

 


 


 


 

***********CJD REPORT 1994 increased risk for consumption of veal and venison and lamb***********

 

CREUTZFELDT JAKOB DISEASE SURVEILLANCE IN THE UNITED KINGDOM THIRD ANNUAL REPORT AUGUST 1994

 

Consumption of venison and veal was much less widespread among both cases and controls. For both of these meats there was evidence of a trend with increasing frequency of consumption being associated with increasing risk of CJD. (not nvCJD, but sporadic CJD...tss)

 

These associations were largely unchanged when attention was restricted to pairs with data obtained from relatives. ...

 

Table 9 presents the results of an analysis of these data.

 

There is STRONG evidence of an association between ‘’regular’’ veal eating and risk of CJD (p = .0.01).

 

Individuals reported to eat veal on average at least once a year appear to be at 13 TIMES THE RISK of individuals who have never eaten veal.

 

There is, however, a very wide confidence interval around this estimate. There is no strong evidence that eating veal less than once per year is associated with increased risk of CJD (p = 0.51).

 

The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).

 

There is some evidence that risk of CJD INCREASES WITH INCREASING FREQUENCY OF LAMB EATING (p = 0.02).

 

The evidence for such an association between beef eating and CJD is weaker (p = 0.14). When only controls for whom a relative was interviewed are included, this evidence becomes a little STRONGER (p = 0.08).

 

snip...

 

It was found that when veal was included in the model with another exposure, the association between veal and CJD remained statistically significant (p = < 0.05 for all exposures), while the other exposures ceased to be statistically significant (p = > 0.05).

 

snip...

 

In conclusion, an analysis of dietary histories revealed statistical associations between various meats/animal products and INCREASED RISK OF CJD. When some account was taken of possible confounding, the association between VEAL EATING AND RISK OF CJD EMERGED AS THE STRONGEST OF THESE ASSOCIATIONS STATISTICALLY. ...

 

snip...

 

In the study in the USA, a range of foodstuffs were associated with an increased risk of CJD, including liver consumption which was associated with an apparent SIX-FOLD INCREASE IN THE RISK OF CJD. By comparing the data from 3 studies in relation to this particular dietary factor, the risk of liver consumption became non-significant with an odds ratio of 1.2 (PERSONAL COMMUNICATION, PROFESSOR A. HOFMAN. ERASMUS UNIVERSITY, ROTTERDAM). (???...TSS)

 

snip...see full report ;

 


 

Thursday, October 10, 2013

 

*************CJD REPORT 1994 increased risk for consumption of veal and venison and lamb**************

 


 

CJD9/10022

 

October 1994

 

Mr R.N. Elmhirst Chairman British Deer Farmers Association Holly Lodge Spencers Lane BerksWell Coventry CV7 7BZ

 

Dear Mr Elmhirst,

 

CREUTZFELDT-JAKOB DISEASE (CJD) SURVEILLANCE UNIT REPORT

 

Thank you for your recent letter concerning the publication of the third annual report from the CJD Surveillance Unit. I am sorry that you are dissatisfied with the way in which this report was published.

 

The Surveillance Unit is a completely independant outside body and the Department of Health is committed to publishing their reports as soon as they become available. In the circumstances it is not the practice to circulate the report for comment since the findings of the report would not be amended. In future we can ensure that the British Deer Farmers Association receives a copy of the report in advance of publication.

 

The Chief Medical Officer has undertaken to keep the public fully informed of the results of any research in respect of CJD. This report was entirely the work of the unit and was produced completely independantly of the the Department.

 

The statistical results reqarding the consumption of venison was put into perspective in the body of the report and was not mentioned at all in the press release. Media attention regarding this report was low key but gave a realistic presentation of the statistical findings of the Unit. This approach to publication was successful in that consumption of venison was highlighted only once by the media ie. in the News at one television proqramme.

 

I believe that a further statement about the report, or indeed statistical links between CJD and consumption of venison, would increase, and quite possibly give damaging credence, to the whole issue. From the low key media reports of which I am aware it seems unlikely that venison consumption will suffer adversely, if at all.

 


 

*** The potential impact of prion diseases on human health was greatly magnified by the recognition that interspecies transfer of BSE to humans by beef ingestion resulted in vCJD. While changes in animal feed constituents and slaughter practices appear to have curtailed vCJD, there is concern that CWD of free-ranging deer and elk in the U.S. might also cross the species barrier. Thus, consuming venison could be a source of human prion disease. Whether BSE and CWD represent interspecies scrapie transfer or are newly arisen prion diseases is unknown. Therefore, the possibility of transmission of prion disease through other food animals cannot be ruled out. There is evidence that vCJD can be transmitted through blood transfusion. There is likely a pool of unknown size of asymptomatic individuals infected with vCJD, and there may be asymptomatic individuals infected with the CWD equivalent. These circumstances represent a potential threat to blood, blood products, and plasma supplies.

 


 

Wednesday, September 17, 2014

 

*** Cost benefit analysis of the development and use of ante-mortem tests for transmissible spongiform encephalopathies ***

 

BOTTOM LINE $$$

 

IF YOU TEST, YOU FIND, and the more you test, the more you will find.

 

HUMANS ARE EXPENDABLE WITH A SLOW, LONG INCUBATING, 100% FATAL DISEASE, ONCE CLINICAL DISEASE...tss

 


 

Wednesday, September 17, 2014

 

Cervid Health Business Plan Fiscal Years 2014 to 2018 Animal and Plant Health Inspection Service Veterinary Services

 


 

Monday, May 05, 2014

 

*** Member Country details for listing OIE CWD 2013 against the criteria of Article 1.2.2., the Code Commission recommends consideration for listing ***

 


 

*** We conclude that TSE infectivity is likely to survive burial for long time periods with minimal loss of infectivity and limited movement from the original burial site. However PMCA results have shown that there is the potential for rainwater to elute TSE related material from soil which could lead to the contamination of a wider area. These experiments reinforce the importance of risk assessment when disposing of TSE risk materials.

 

*** The results show that even highly diluted PrPSc can bind efficiently to polypropylene, stainless steel, glass, wood and stone and propagate the conversion of normal prion protein. For in vivo experiments, hamsters were ic injected with implants incubated in 1% 263K-infected brain homogenate. Hamsters, inoculated with 263K-contaminated implants of all groups, developed typical signs of prion disease, whereas control animals inoculated with non-contaminated materials did not.

 

PRION 2014 CONFERENCE

 

CHRONIC WASTING DISEASE CWD

 

A FEW FINDINGS ;

 

Conclusions. To our knowledge, this is the first established experimental model of CWD in TgSB3985. We found evidence for co-existence or divergence of two CWD strains adapted to Tga20 mice and their replication in TgSB3985 mice. Finally, we observed phenotypic differences between cervid-derived CWD and CWD/Tg20 strains upon propagation in TgSB3985 mice. Further studies are underway to characterize these strains.

 

We conclude that TSE infectivity is likely to survive burial for long time periods with minimal loss of infectivity and limited movement from the original burial site. However PMCA results have shown that there is the potential for rainwater to elute TSE related material from soil which could lead to the contamination of a wider area. These experiments reinforce the importance of risk assessment when disposing of TSE risk materials.

 

The results show that even highly diluted PrPSc can bind efficiently to polypropylene, stainless steel, glass, wood and stone and propagate the conversion of normal prion protein. For in vivo experiments, hamsters were ic injected with implants incubated in 1% 263K-infected brain homogenate. Hamsters, inoculated with 263K-contaminated implants of all groups, developed typical signs of prion disease, whereas control animals inoculated with non-contaminated materials did not.

 

Our data establish that meadow voles are permissive to CWD via peripheral exposure route, suggesting they could serve as an environmental reservoir for CWD. Additionally, our data are consistent with the hypothesis that at least two strains of CWD circulate in naturally-infected cervid populations and provide evidence that meadow voles are a useful tool for CWD strain typing.

 

Conclusion. CWD prions are shed in saliva and urine of infected deer as early as 3 months post infection and throughout the subsequent >1.5 year course of infection. In current work we are examining the relationship of prionemia to excretion and the impact of excreted prion binding to surfaces and particulates in the environment.

 

Conclusion. CWD prions (as inferred by prion seeding activity by RT-QuIC) are shed in urine of infected deer as early as 6 months post inoculation and throughout the subsequent disease course. Further studies are in progress refining the real-time urinary prion assay sensitivity and we are examining more closely the excretion time frame, magnitude, and sample variables in relationship to inoculation route and prionemia in naturally and experimentally CWD-infected cervids.

 

Conclusions. Our results suggested that the odds of infection for CWD is likely controlled by areas that congregate deer thus increasing direct transmission (deer-to-deer interactions) or indirect transmission (deer-to-environment) by sharing or depositing infectious prion proteins in these preferred habitats. Epidemiology of CWD in the eastern U.S. is likely controlled by separate factors than found in the Midwestern and endemic areas for CWD and can assist in performing more efficient surveillance efforts for the region.

 

Conclusions. During the pre-symptomatic stage of CWD infection and throughout the course of disease deer may be shedding multiple LD50 doses per day in their saliva. CWD prion shedding through saliva and excreta may account for the unprecedented spread of this prion disease in nature.

 

see full text and more ;

 

Monday, June 23, 2014

 

*** PRION 2014 CONFERENCE CHRONIC WASTING DISEASE CWD

 


 


 

*** Infectious agent of sheep scrapie may persist in the environment for at least 16 years***

 

Gudmundur Georgsson1, Sigurdur Sigurdarson2 and Paul Brown3

 


 

New studies on the heat resistance of hamster-adapted scrapie agent: Threshold survival after ashing at 600°C suggests an inorganic template of replication

 


 

Prion Infected Meat-and-Bone Meal Is Still Infectious after Biodiesel Production

 


 

Detection of protease-resistant cervid prion protein in water from a CWD-endemic area

 


 

A Quantitative Assessment of the Amount of Prion Diverted to Category 1 Materials and Wastewater During Processing

 


 

Rapid assessment of bovine spongiform encephalopathy prion inactivation by heat treatment in yellow grease produced in the industrial manufacturing process of meat and bone meals

 


 

PPo4-4:

 

Survival and Limited Spread of TSE Infectivity after Burial

 

PPo4-4:

 

Survival and Limited Spread of TSE Infectivity after Burial

 

Karen Fernie, Allister Smith and Robert A. Somerville The Roslin Institute and R(D)SVS; University of Edinburgh; Roslin, Scotland UK

 

Scrapie and chronic wasting disease probably spread via environmental routes, and there are also concerns about BSE infection remaining in the environment after carcass burial or waste 3disposal. In two demonstration experiments we are determining survival and migration of TSE infectivity when buried for up to five years, as an uncontained point source or within bovine heads. Firstly boluses of TSE infected mouse brain were buried in lysimeters containing either sandy or clay soil. Migration from the boluses is being assessed from soil cores taken over time. With the exception of a very small amount of infectivity found 25 cm from the bolus in sandy soil after 12 months, no other infectivity has been detected up to three years. Secondly, ten bovine heads were spiked with TSE infected mouse brain and buried in the two soil types. Pairs of heads have been exhumed annually and assessed for infectivity within and around them. After one year and after two years, infectivity was detected in most intracranial samples and in some of the soil samples taken from immediately surrounding the heads. The infectivity assays for the samples in and around the heads exhumed at years three and four are underway. These data show that TSE infectivity can survive burial for long periods but migrates slowly. Risk assessments should take into account the likely long survival rate when infected material has been buried.

 

The authors gratefully acknowledge funding from DEFRA.

 


 


 

Friday, December 14, 2012

 

DEFRA U.K. What is the risk of Chronic Wasting Disease CWD being introduced into Great Britain? A Qualitative Risk Assessment October 2012

 

snip...

 

In the USA, under the Food and Drug Administration’s BSE Feed Regulation (21 CFR 589.2000) most material (exceptions include milk, tallow, and gelatin) from deer and elk is prohibited for use in feed for ruminant animals. With regards to feed for non-ruminant animals, under FDA law, CWD positive deer may not be used for any animal feed or feed ingredients. For elk and deer considered at high risk for CWD, the FDA recommends that these animals do not enter the animal feed system. However, this recommendation is guidance and not a requirement by law.

 

Animals considered at high risk for CWD include:

 

1) animals from areas declared to be endemic for CWD and/or to be CWD eradication zones and

 

2) deer and elk that at some time during the 60-month period prior to slaughter were in a captive herd that contained a CWD-positive animal.

 

Therefore, in the USA, materials from cervids other than CWD positive animals may be used in animal feed and feed ingredients for non-ruminants.

 

The amount of animal PAP that is of deer and/or elk origin imported from the USA to GB can not be determined, however, as it is not specified in TRACES. It may constitute a small percentage of the 8412 kilos of non-fish origin processed animal proteins that were imported from US into GB in 2011.

 

*** Overall, therefore, it is considered there is a __greater than negligible risk___ that (nonruminant) animal feed and pet food containing deer and/or elk protein is imported into GB.

 

There is uncertainty associated with this estimate given the lack of data on the amount of deer and/or elk protein possibly being imported in these products.

 

snip...

 


 

2003D-0186 Guidance for Industry: Use of Material From Deer and Elk In Animal Feed

 

EMC 1 Terry S. Singeltary Sr. Vol #: 1

 


 


 

see my full text submission here ;

 


 

*** Susceptibility of UK red deer (Cervus alaphus elaphus) to oral BSE transmission Project Code: M03024 ***

 

02/08/2011

 

The project confirmed that U.K red deer are susceptible to both oral and intra-cerebral inoculation with the cattle BSE agent. Six clinically positive (from 26-42 months post inoculation) i.c inoculated and one (56 months post inoculation) orally dosed deer that tested positive for TSE by immunohistochemistry and Western blotting using several primary antibodies demonstrated widespread accumulation of disease specific prion protein in the central nervous system, peripheral nervous system and enteric nervous system but none in lymphoreticular system. All showed several brain sites positive for disease specific prion protein and presented immunohistochemistry and Western blotting phenotypes with similarities to BSE in sheep, goats and cattle but unlike those seen in chronic wasting disease (CWD) in elk or scrapie in sheep. The vacuolar pathology and distribution of disease specific prion protein in red deer resembled that of CWD in most major respects however we have shown that BSE can be clearly differentiated from CWD by existing immunohistochemical and biochemical methods that are in routine use.

 

The knowledge gained as a result of this work will permit rapid and accurate diagnosis should a TSE ever be detected in European red deer and will also enable effective disease control methods to be quickly put in place.

 


 

Results

 

We confirmed that U.K red deer are susceptible to both oral and intra-cerebral inoculation with the cattle BSE agent. Six clinically positive (from 26-42mpi) i.c inoculated and one (56mpi) orally dosed deer that tested positive for TSE by IHC and WB using several primary antibodies demonstrated widespread accumulation of disease specific PrP in CNS, PNS and ENS but none in LRS. All showed several brain sites positive for disease specific PrP and presented IHC and WB phenotypes with similarities to BSE in sheep, goats and cattle but unlike those seen in CWD in elk or scrapie in sheep. The vacuolar pathology and distribution of PrPd BSE in red deer resembled that of CWD in most major respects however we have shown that BSE can be clearly differentiated from CWD by existing immunohistochemical and biochemical methods that are in routine use.

 

Final technical report MO3024 01/04/2003 – 31/03/2010 Susceptibility of UK red deer (Cervus elaphus elaphus) to oral BSE transmission. Stuart Martin - VLA Lasswade Pentlands Science Park Bush Loan Penicuik EH26 0PZ Page 2 of 21 Further work undertaken August 2009 – March 2010. Genetic analysis - Wilfred Goldmann; Roslin NPD.

 

Negative controls and the remaining 5 orally dosed deer culled at 72mpi tested negative by IHC and Western blot however analysis of the PrP ORF of these deer (kindly carried out by Wilfred Goldmann of the Roslin NPD) identified a Q to E polymorphism at codon 226 that may influence the efficiency of oral transmission (not published).

 

In the experimental BSE challenge of red deer six out of six deer succumbed to BSE when challenged by intracerebral routes but only one of six deer challenged by the oral route succumbed to infection. Deer killed at 190 days or 365 days post oral challenge showed no evidence of abnormal PrP accumulation when tested by immunocytochemistry. The PrP gene of red deer includes a Q to E polymorphism at codon 226. The table shows the distribution of these codon 226 polymorphisms within experimental challenge groups.

 

snip...

 


 

Sunday, December 15, 2013

 

*** FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OIA UPDATE DECEMBER 2013 UPDATE

 


 


 

 

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