What is the risk of chronic wasting disease being introduced into Great Britain? An updated Qualitative Risk Assessment March 2016
What is the risk of chronic wasting disease being introduced into Great
Britain? An updated Qualitative Risk Assessment March 2016
Summary
The previous assessment concentrated on the incursion of disease from North
America through the imports of animal feed or the movement of contaminated
clothing, footwear and equipment. The results suggested that import of pet feed
was a non-negligible risk, but given the unlikely contact of resident deer in GB
with such non-ruminant feed, this was considered overall a negligible to very
low risk. The movement of contaminated clothing, footwear or equipment
(particularly hunting equipment) could pose a very low risk, although the volume
of contaminated soil which would need to be ingested to give rise to an
infection is likely to be higher than would be present. There is a variable
level uncertainty in all these assessments.
The new assessment focuses on an additional potential route of entry: the
importation of natural deer urine lures. The main conclusions from this
assessment are:
In areas of North America where CWD has been reported, given that CWD is
excreted in faeces, saliva, urine and blood, and survives in the environment for
several years there is a medium probability that the deer urine in North America
contains CWD (high uncertainty; depends on the source of deer used for
production).
The risk of a deer in GB being infected per 30 ml bottle of urine
imported from the USA is very low, albeit with high uncertainty. Overall it is
concluded that the risk of at least one infection of deer in the UK with CWD per
year from deer urine lures imported from the USA is medium. This assumes a high
number of 30 ml bottles imported per year from all areas of the USA.
None of the species affected by CWD in North America are present in GB.
For a British species to become infected with CWD following exposure, the dose
and inherent susceptibility of the species will be important. Based on current
scientific evidence Red deer (Cervus elaphus elaphus) are susceptible to CWD,
Fallow deer (Dama dama) are likely to be less susceptible and Roe deer
(Capreolus capreolus) have a gene conferring susceptibility. Therefore, it is
likely that given exposure to an infectious dose of CWD, deer in GB could become
infected with CWD.
Overall, the probability of importing CWD into GB from North America and
causing infection in British deer is uncertain but likely to be negligible to
very low via movement of deer hunters, other tourists and British servicemen and
very low via imported (non-
2
ruminant) animal feed and medium for the use of lures. However, if it was
imported and (a) deer did become infected with CWD, the consequences would be
severe as eradication of the disease is impossible, it is clinically
indistinguishable from BSE infection in deer (Dalgleish et al., 2008) and
populations of wild and farmed deer would be under threat.
The USA has implemented a Herd Certification Programme for farmed and
captive cervids. So far, 29 States are approved for HCP status (APHIS, 2015).
The list includes States such as Colorado, where CWD is present, therefore it is
recommended that any sourcing of such natural urine lures should be not only
from States with an HCP programme, but also from a herd which is registered as
being regularly tested free of CWD.
Animal urine is not considered a commodity which is subject to animal
by-products legislation for imports. Internet sales are common and although a
license would be required, there are no conditions for the safe sourcing of such
products. Deer urine lures are also available in Europe and may be produced from
carcases of hunted deer. The use of deer urine produced from a species not
present in Europe (such as white tailed deer) is questioned for its value with
native GB deer according to the British Deer Society survey.
Background
Chronic wasting disease (CWD) is a highly infectious transmissible
spongiform encephalopathy (TSE) that is circulating in the wild and farmed
cervid populations of North America. It is the only TSE maintained in
free-ranging wild animal populations. A feature of CWD is that it is able to
transmit both directly (animal-to-animal) and indirectly via the contaminated
environment. In particular, CWD prions are able to bind to and survive in the
soil in a bio-available form for many years without any decrease in infectivity.
This makes eradication of the disease from a wild population increasingly
challenging. Thus far, there have been no reported cases of CWD or other TSE in
deer in Great Britain (GB). This is based on surveys of wild and farmed red deer
(Cervus elaphus elaphus) (EFSA, 2011). Given the consequences of CWD observed in
North America, it is of high importance that GB remains free of the disease.
Further, as the clinical signs of CWD in deer are similar to those of deer
experimentally infected with bovine spongiform encephalopathy (BSE), all
infected deer would need to be tested to differentiate if they were infected
with CWD or BSE to minimise the risk of BSE entering the human food chain via
affected venison. In 2015, the British Deer Society (BDS) carried out an online
survey of BDS and BASC members to gather evidence about the use of deer urine as
a lure. Fifteen percent of respondents (~1,800) answered yes about knowing that
deer urine was used as a lure. Of the respondents, less than 2% responded yes to
using such a product themselves. Of those that use the product, 50% had sourced
the product from the USA, while 20% use more than a litre in volume a year and
~70% is natural (as opposed to synthetic).
3
Hazard identification The hazard is identified as Chronic Wasting
Disease
Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy
(TSE) affecting cervids. It is the only TSE maintained in free-ranging wild
animal populations; other TSE’s are mostly restricted to captive domestic
livestock populations or humans. Chronic wasting disease was first identified as
a clinical disease of captive mule deer in Colorado in 1967 and later classified
as a TSE in 1978 (Williams & Miller, 2003). The origin of the disease is
unknown and may have been a spontaneous TSE that arose in deer. Currently,
natural infections of CWD have been reported in mule deer (Odocoileus hemionus
hemionus), black-tailed deer (Odocoileus hemionus columbianus), white-tailed
deer (Odocoileus virginianus), Rocky Mountain elk (Cervus elphus nelsoni),
Shira’s moose (Alces alces shirasi) and mule deer and white-tailed deer hybrids
(Hamir et al., 2008). Other species of elk may also be susceptible. The disease
is restricted to North America except for isolated cases of infected elk being
exported from Canada to South Korea (Williams & Miller, 2003). In the last
decade, with increased CWD testing, a more widespread distribution of CWD within
North America has been observed and the geographic distribution is increasing
(APHIS, 2015) (Figure 1).
Figure 1: Current range of CWD in North America (USGS, 2016)
4
This more widespread distribution may be due to enhanced surveillance but
also to natural migration of cervids and translocation of infected animals by
humans (EFSA, 2011). Within affected areas, the prevalence varies. In the
endemic area of Wyoming, for example, the prevalence of CWD in mule deer has
increased from approximately 11% in 1997 to 36% in 2007 (Almberg et al., 2011).
In such areas, population declines of deer of up to 30 to 50% have been observed
(Almberg et al., 2011). In areas of Colorado, the prevalence can be as high as
30% (EFSA, 2011).
The clinical signs of CWD in affected adults are weight loss and
behavioural changes that can span weeks or months (Williams, 2005). In addition,
signs might include excessive salivation, behavioural alterations including a
fixed stare and changes in interaction with other animals in the herd, and an
altered stance (Williams, 2005). These signs are indistinguishable from cervids
experimentally infected with bovine spongiform encephalopathy (BSE). Given this,
if CWD was to be introduced into countries with BSE such as GB, for example,
infected deer populations would need to be tested to differentiate if they were
infected with CWD or BSE to minimise the risk of BSE entering the human
food-chain via affected venison.
The duration of clinical disease is highly variable and death can occur
within 4 weeks but some infected animals may survive as long as a year
(Williams, 2005). The incubation period is a minimum of approximately 16 months
and is more likely to be between 2 and 4 years (Williams, 2005). In affected
elk, the incubation period is between 1.5 and 3 years after which they become
clinically affected and may succumb less than 12 months after initial clinical
signs appear (Miller et al., 1998). During the pre-clinical period, the animal
is infectious (Almberg et al., 2011).
The CWD agent or Prion Protein (PrPCWD) in affected animals is distributed
firstly in the gut associated lymphoid tissues, digestive tract (e.g. tonsils,
Peyer’s patches, mesenteric lymph nodes) and then in the brain and spinal cord
as the disease progresses (Sigurdson, 2008). Prions of CWD have also been found
in muscle tissue (Angers et al., 2006) (see Figure 2). The distribution and
levels of PrPCWD in tissues differ between species (e.g. elk versus deer).
5
Figure 2: Diagram displaying the main organs affected by CWD in infected
cervids (http://www.dnr.state.mn.us/mammals/deer/cwd/index.html)
Given its propensity to colonise the digestive tract, evidence suggests the
prion is excreted in faeces (Safar et al., 2008), urine and saliva potentially
leading to direct and indirect transmission between cervid species. Indeed, the
disease is transmitted horizontally with high efficiency and circumstantial
evidence suggests that environmental contamination with CWD prions contributes
to the maintenance of CWD in affected areas (Safar et al., 2008). The rate of
transmission of CWD has been reported to be as high as 30% and can approach 100%
among captive animals in endemic areas (Safar et al., 2008). The efficiency of
CWD transmission is unparalleled among TSE diseases (EFSA, 2011). Trifilo et
al., (2007), using a murine tg mouse model, established that CWD can be
transmitted via the oral route. Indeed, the distribution of PrPres in the orally
infected mice (e.g. in the spleen and lymph nodes) mimicked what has been
reported in deer developing CWD via natural infection (Trifilo et al., 2007).
Modelling studies also support the theory that transmission of CWD in deer herds
is maintained by contact with a prion contaminated environment (Almberg et al.,
2011). Scavenging of CWD-infected carcasses provides another route of releasing
the prion into the environment and exposure of non-cervid species (Sigurdson,
2008). This indirect transmission route is problematic as it not only increases
the basic reproductive number but also because there are very few effective
mitigation strategies for reducing the risk from indirect transmission. This is
due to the fact that the agent is extremely resistant in the environment and
able to bind to soil particles making eradication and control of CWD a major
obstacle in both farmed and free-ranging cervid populations.
Sheep and cattle may be exposed to CWD via common grazing areas with
affected deer but so far, appear to be poorly susceptible to mule deer CWD
(Sigurdson, 2008). In contrast, cattle are highly susceptible to white-tailed
deer CWD and mule deer CWD in experimental conditions but no natural CWD
infections in cattle have been reported (Sigurdson, 2008; Hamir et al., 2006).
It is not known how susceptible humans are to CWD but given that the prion can
be present in muscle, it is likely that humans have been exposed to the agent
via consumption of venison (Sigurdson, 2008). Initial experimental research,
however, suggests that human susceptibility to CWD is low and there may be a
robust species barrier for CWD transmission to humans (Sigurdson, 2008). It is
apparent, though, that CWD is affecting wild and farmed cervid populations in
endemic areas with some deer populations decreasing as a result.
Thus far, CWD is restricted to North America with the exception of imported
infected animals into South Korea from Canada. Surveys of wild and farmed cervid
populations in the European Union between 2006 and 2010 did not identify any
TSEs (EFSA, 2011). As part of this survey, 601 farmed and 598 wild red deer
(Cervus elaphus elaphus) were tested (EFSA, 2010). These included clinical/sick
animals, fallen stock, healthy shot/slaughtered animals and road killed animals.
Based on the survey results, it can be concluded that the prevalence of CWD in
the EU is less than 0.5%. It is important, therefore, to ensure that the disease
is not introduced into Europe and establish within the
6
EU wild and farmed cervid population as the probability of being able to
eradicate the disease would be very small.
Risk Question
This risk assessment considers the risk posed to the Great Britain (GB)
deer population if chronic wasting disease (CWD) was imported from North America
(i.e. Canada and the United States). The specific risk question addressed is:
What is the risk of CWD being introduced into Great Britain (GB) from North
America and causing infection in deer?
To answer the above question, the risk assessment follows the OIE framework
of release (or entry), exposure and consequence assessment. Specifically, it is
divided into the three key areas:
1. What is the probability of introducing CWD into GB from North
America?
2. What is the probability of a deer species in GB being exposed to the CWD
prion?
3. What is the probability of a GB deer species becoming infected with CWD
upon exposure to the prion?
Risk Assessment
Terminology related to the assessed level of risk
For the purpose of the risk assessment, the following terminology will
apply (OIE, 2004):
Negligible
So rare that it does not merit to be considered
Very low
Very rare but cannot be excluded
Low
Rare but does occur
Medium
Occurs regularly
High
Occurs often
Very high
Event occurs almost certainly
7
Entry assessment
The routes by which CWD may be introduced into GB from North America
include:
Importation of live deer
Importation of deer urine lures
Importation of meat and other products derived from cervid species (e.g.
trophy items including antlers, semen)
Importation of animal feed
Hunters and other tourists and British servicemen travelling from
affected areas to GB with contaminated equipment (e.g. boots, clothing,
knives)
Currently, according to the European Union Trade Control and Expert System
(TRACES) database, GB does not import live cervids, ‘other’ animal meat products
or raw hides and skins. This was the same conclusion drawn by EFSA (2004) who
stated GB does not import cervids or products from North America. Therefore, the
three routes which this assessment focuses on are:
1) importation of animal feed
2) importation of deer urine lures
3) importation of CWD prion on contaminated equipment and clothing/footwear
of hunters or other tourists and British servicemen
Importation of animal feed
Animal feed encompasses all feed fed to farmed livestock, horses, pets,
farmed fish, zoo and circus animals and also animals living freely in the wild.
Currently, legislation for animal feed relating to production, and labelling and
composition is harmonised at the EU level and, in GB, is the responsibility of
the Food Standards Agency (FSA). In addition, Defra is responsible for ABP
Regulations which includes pet food manufacturing regulation.
Pet food containing material of animal origin, according to EU Regulation
(EC) No. 142/2011 on Animal By-Products, must be derived from animals inspected
and passed as fit for human consumption prior to slaughter (Category 3
material). Further, the products are subject to strict microbiological criteria
for Enterobacteriaceae and Salmonella. Under the EU Regulation, imported pet
food produced using Category 3 processed animal proteins (PAP) must adhere to
the same standards as that produced within the EU. More specifically, the
imported pet food must satisfy the following criteria:
The PAP must have been produced in accordance with the same requirements
as PAP for placing on the market in the EU
The PAP must have been sampled and tested to satisfy certain
bacteriological criteria in accordance with the Regulations before release onto
the EU market
The product must enter the EU under correct Health Certification
The Health Certification signed by the veterinarian or official inspector
responsible for the rendering plant in the exporting country must verify that EU
standards of
8
sourcing of animal by-products, processing and sampling are met for each
consignment.
These requirements apply to canned pet food, processed pet food other than
canned pet food, dog chews, raw pet food and flavouring innards.
According to TRACES, GB imports processed pet food from Canada and the
United States of America (USA). In November and December 2015, for example, GB
imported 13.6112 tonnes of processed cat and dog food (including dog chews)
containing products of ungulate origin from Canada and USA. There are a limited
number of processed pet food products made in the USA containing (roasted)
venison for the cat and dog food market that are available in GB (e.g. Taste of
the Wild pet food). Venison is high in iron content and considered a good
alternative meat product for pets with intolerance to certain meat proteins. The
specific amount of pet food products imported into GB from North America
containing deer protein is unknown and not specified in the TRACES system, but
is likely to be a small percentage of the overall amount of processed pet food
imported. The TRACES database indicates that in the same period (Nov – Dec 2015)
751,418 tonnes of other products for pet food were imported into the UK from USA
and Canada.
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 cannot be determined, however, as it is not specified in TRACES. It may
constitute a small percentage of the very low tonnage of non-fish origin
processed animal proteins that were imported from US into GB.
*** Overall, therefore, it is considered there is a greater than negligible
risk that (non-ruminant) 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.
9
Movement of hunters, other tourists and British servicemen
Probability that the environment in North America is contaminated with
CWD
As outlined in Figure 1, there are 21 states and provinces in the USA and
Canada where CWD has been detected in farmed and wild cervids. These include:
Alberta (Canada), Arkansas, Colorado, Illinois, Kansas, Maryland, Minnesota,
Missouri, Nebraska, New Mexico, New York, North Dakota, Saskatchewan (Canada),
South Dakota, Utah, Virginia, West Virginia, Wisconsin and Wyoming. In these
areas, the environment is likely to be contaminated with CWD prions from direct
excretion of the prion in various bodily fluids of infected animals, and
leaching of prions into the soil from decaying carcasses of infected animals. A
summary of the current studies on CWD in faeces, urine, blood and other bodily
fluids or organs is summarised in Table 1.
Table 1: Summary of the studies on CWD prion excretion
Fluid/organ
Study summary
Reference
Faeces
Faeces were the source of CWD infection in Syrian hamsters under
experimental conditions.
Prions remain intact after being passed through the digestive tract and,
therefore, are a viable source of infectivity in the environment.
CWD prions are excreted in the faeces of infected mule deer 7 to 11
months prior to the onset of neurological signs (i.e. during the incubation
period)
Safar et al., (2008) Tamguney et al. (2009)
Saliva
During studies of oral transmission using a murine tg mouse model, it was
observed that prior to and during clinical disease, serous and mucous glands
contained PrPres.
Three naïve fawns were orally inoculated with 50ml of saliva from an
infected deer in 3 doses over a 3 day period. Eighteen months post inoculation,
CWD prions were detected in all 3 fawns during tonsil biopsy.
Pooled saliva from five terminally CWD infected white-tailed deer was
inoculated into nine tg1536 mice. Eight of the nine mice developed disease
consistent with a TSE at 342 ± 109 days post inoculation suggesting infectious
prions are present in saliva of infected cervids.
Trifilo et al., (2007)
Mathiason et al., (2006)
Haley et al., (2009)
Blood
Two naïve white-tailed deer were inoculated intraperitoneally with 250 ml
of frozen citrated blood and a further fawn with an intravenous transfusion of
250 ml freshly citrated whole blood. After 18 months post inoculation, all three
fawns had CWD prions in their tonsils and retropharyngeal lymph node.
Mathiason et al., (2006)
Urine
Pooled urine from five terminally CWD infected white-tailed deer was
inoculated into nine tg Haley et al., (2009)
10
mice. Two of the nine mice developed disease consistent with a TSE at 370
and 376 days post inoculation suggesting infectious prions are present in the
urine of infected cervids but at a lower infectivity than other bodily fluids
such as saliva.
Henderson et al. (2015) report urine from CWD-infected deer contained 1
intracerebral LD50 per 10-20 ml. But their LD50 is estimated from amyloid
formation rate and may be difficult to relate to an oral LD50 for live
deer.
Henderson et al. (2015) cite Gonzalez-Romero et al. 2008 which used PMCA
to show that levels of prions in neat urine were approximately equivalent to a
10-10 to 10-11 dilution of brain.
The levels in urine may be so low as to be difficult to quantify by
bioassay.
Assumed estimate below that the oral LD50 for a deer may be 1,000 litres
of urine from a CWD-infected deer. This assumes the oral route is 100,000-fold
less efficient than the ic route. Henderson et al. (2015)
Antler velvet
Antlers are covered by a layer of skin, velvet, which is shed after an
increase in testosterone and ossification of antlers. CWD is present at low, but
detectable, amounts in antler velvet from infected elk.
Angers et al., (2009)
It is clear from Table 1 that CWD is excreted in several different bodily
fluids and, as demonstrated in experimental studies, can be a source for onward
transmission to naïve animals. Infected carcasses decaying naturally in confined
areas can also lead to new CWD infections in naïve deer (Sigurdson, 2008). This
was proved, experimentally, by Miller et al., (2004) during a study of
environmental transmission. Specifically, 3 naïve mule deer were stocked in an
800m2 paddock in which a naturally infected mule deer had died and decomposed
approximately 1.8 years prior. In a second paddock, a further 3 naïve mule deer
were placed where infected mule deer had resided 2.2 years earlier and
contaminated the environment with their faeces (Miller et al., 2004). The
experiment was conducted in 3 replicates. In total, 3 out of 12 and 1 out of 9
deer were infected by being exposed to an infected decomposed carcass or
residual excreta, respectively.
The CWD prion has also been detected in water. Specifically, very low
levels (below infectious levels) were detected in a water sample from melting
winter snow-pack from an endemic area (Nichols et al., 2009). The data showed
persistence of CWD prions in water, accumulated levels of which, it is
hypothesised, may promote transmission within deer herds.
Once in the environment, TSE prions can bind to soil particles and remain
infectious (Saunder et al., 2010). Indeed, Johnson et al., (2006) demonstrated
that the disease-
11
associated form of the prion protein can bind to all soil mineral surfaces
and is preserved in a bioavailable form. Further, in a later study, Johnson et
al., (2007) observed that prions bound to the soil mineral montmorillonite (Mte)
significantly enhanced disease penetrance and reduced the incubation period
compared to unbound prions. The reason why binding to Mte or other soil
components enhances transmissibility is unclear but it may provide some
protection for the prion in the gut against denaturation allowing more agent to
be absorbed by the animal (Johnson et al., 2007). Further, binding to the soil
particles maintains prions near the soil surface increasing the probability of
animal exposure (Russo et al., 2009).
In addition to the enhanced infectivity, prions can remain in the soil for
several years as the agents are resistant to inactivation by most chemical
agents, radiation and heat (Johnson et al., 2006). Seidel et al. (2007), for
example, demonstrated that scrapie agent (strain 263K) remains persistent in
soil over a period of at least 29 months and remains highly infectious to Syrian
hamsters in oral inoculation experiments. In Iceland during an epidemiological
investigation of scrapie, a TSE of sheep and goats, Georgsson et al., (2006)
reported that the scrapie agent survived on a farm for at least 16 years.
However, Russo et al., (2009) demonstrated experimentally that reactive soil
components such as manganese oxides may contribute to the inactivation process
of TSE prions in soil. The authors did not study CWD prion specifically but the
study highlights the complexity of the effect the inorganic and organic
constituents in soil may have on prion survival and infectivity.
*** In summary, in endemic areas, there is a medium probability that the
soil and surrounding environment is contaminated with CWD prions and in a
bioavailable form. In rural areas where CWD has not been reported or only very
recently reported and deer are present, there is a greater than negligible risk
the soil is contaminated with CWD prion.
Movement of deer hunters, other outdoor tourists and British servicemen
between North America and GB
The probability a person comes into contact with CWD prions varies
depending upon their place of residence and/or their involvement with outdoor
pursuits (e.g. hunting). In this assessment, focus is given to the following
groups of people:
Residents in CWD affected areas travelling to GB (particularly the
countryside) and British tourists travelling to CWD affected areas
North American hunters travelling to GB to hunt/stalk deer and British
hunters travelling to North America to hunt deer
British servicemen training in and/or near CWD affected areas
All other people (e.g. city tourists and residents) are considered to pose
a lower risk of being exposed to CWD in North America and, therefore, arriving
in GB with contaminated clothing, footwear and/or equipment.
There are limited data on the number of North American tourists, stratified
by location of residence, arriving in the UK. Therefore, it is not possible to
ascertain of the 3.89 million
12
visitors from the United States (US) to the United Kingdom (UK) in 2006,
for example, how many were from CWD affected areas. Likewise, there is no
breakdown of where in the USA the 4.5 million UK visitors travelled in 2008.
This is a significant data gap in the assessment.
Hunting in the US is a popular sport with 4% of the population (10.1
million) involved in deer hunting in 2006 (USFWS, 2011). Of these people, 4.7
million only hunted deer whilst the remainder hunted deer and other species
(e.g. small game, bears). Further, 58% of deer hunters were involved in wildlife
watching activities and other outdoor pursuits (e.g. hiking, fishing); this is
compared to 31% of the general public (USFWS, 2011; TAMS, 2007). Individuals
that hunted whilst on a trip were likely to come from northern and western
states (e.g. Alaska, Wyoming, North Dakota, South Dakota, Idaho and Montana)
compared to highly urbanised states. In Canada, those individuals participating
in hunting activities are most likely to do so within Canada with the majority
taking a trip within their own province or region (TAMS, 2006). Canadian hunters
were also more likely than other Canadian pleasure travellers to participate in
wilderness activities and hiking (TAMS, 2006). There are no data collated on the
number of hunters from North America travelling to the UK to stalk/hunt deer
and, vice versa, there are no data on the number of UK residents hunting in
North America. However, in order to hunt in GB with your own rifle, a visitor
firearms permit has to be obtained from the police force in one of the devolved
countries. In 2011, 123 licences were granted by the Scottish Police Force for
non-EU residents (BASC, pers. Comm., 2012). This includes not only individuals
from North America but also Norway and other non-EU countries (BASC, pers.
Comm., 2012). The number of hunters arriving without their own rifle and
participating in an organised hunting package/holiday is unknown. However, it is
likely the total number of hunters from North America is in the low hundreds;
the actual number, however, is highlighted as a significant data gap.
As well as tourists, British servicemen frequently move between North
America and GB. In particular, British Army servicemen use the Canadian Forces
base at Suffield, Alberta to take part in extensive training exercises. Suffield
is located in the southwest of Alberta and comprises of 2,690km2 of prairie
landscape. The eastern boundary is designated as a wildlife management area and
is south of a wildlife management area in which CWD was reported as recently as
2011 (www.srd.albert.ca). Consequently, the servicemen have the potential to be
in close contact with areas where CWD is present.
*** In summary, given the volume of tourists, hunters and servicemen moving
between GB and North America, the probability of at least one person travelling
to/from a CWD affected area and, in doing so, contaminating their clothing,
footwear and/or equipment prior to arriving in GB is greater than negligible.
For deer hunters, specifically, the risk is likely to be greater given the
increased contact with deer and their environment. However, there is significant
uncertainty associated with these estimates.
13
Probable amount of CWD prions on contaminated boots and equipment
Given that a hunter or tourist walks in areas which are contaminated with
CWD, it is possible that they will collect soil on their boots and other
equipment. This likelihood will increase if the hunter has shot and handled a
CWD infected deer resulting in contamination of the hunting equipment (e.g.
knives) and their clothing and they subsequently arrive in GB with this
equipment, footwear and clothing. Further, the soles of hiking boots tend to
retain more soil than those of normal shoes. Wilkinson (2010), for example,
removed 0.1 g of soil from hiking boots after returning to GB from a 2-month
research visit to Canada. The amount of CWD prion in this amount of soil will
depend upon the density of CWD infected animals excreting prions into the
environment and the type of soil; CWD prion binds to clay soil, for example.
Animal mortality sites could also be hotspots of CWD prion given the highly
infectious nervous system matter entering into the environment and soil
(Saunders et al., 2010).
Importation of deer urine lures
It is well established that urine from CWD-infected deer contains CWD
infectivity. The urine collected for deer lures has no processing and is
immediately refrigerated and bottled (Anon 2015b). There is no inactivation of
the CWD agent in the urine and thus all infectivity present at the point of
bottling in the USA will still be present at the point of use in the UK.
However, under EU rules, (EC Regulation 1069/2009), urine from farmed deer
should fall under the definition of “manure” and therefore the import of such a
product, if unprocessed, is not allowed, according to Regulation (EC) 142/2011.
Nevertheless, the processing required for bottling prior to retail is unlikely
to affect the CWD prion and if it did fulfil the requirements in 142/2011, the
active ingredient in the urine would no longer be effective. It is therefore
presumed that the urine is considered “unprocessed” under EU law. The import and
transit of urine from wild deer is not covered by 1069/2009.
A survey conducted by the British Deer Society (BDS) in July 2015 suggested
small number of hunters in GB were aware of and used deer urine lures (http://www.bds.org.uk/index.php/news-events/135-deer-urine-lure-survey-july-2015
).
For the purpose of the qualitative risk assessment developed here it is
necessary to estimate the probability that a 30-ml bottle of lure contains urine
from an infected deer. This requires an estimate of the proportion of deer herds
in the USA which are infected with CWD together with the within herd
prevalence.
The distribution map of CWD in US shows it is present mainly in central
states (Figure 1). However, Virginia in the east of the country has recorded
seven recent cases of CWD (Anon 2015a). Some US manufacturers claim to take
steps to prevent urine being taken from infected animals eg by sourcing from
farms where the deer are randomly tested for CWD (Anon 2015a). However, if
disease is already present and testing is not carried out regularly, captive
populations are not necessarily disease free (Strausser 2014). Urine-based deer
lures have been known to be collected from domestic white-tailed deer herds and
therefore there is a recognised risk. This is reflected by 6 US States which
have
14
banned the use of natural deer urine for lures, as the deer urine may be
sourced from CWD-endemic areas in the USA as well as from areas free of CWD. For
example, the US State of Virginia is banning the use of urine-based deer lures
on July 2015 and Vermont from 2016 due to the risk of spread of CWD. Alaska
banned their use in 2012 (Anon 2015a). Pennsylvania Game Commission has banned
urine-based deer lures and acknowledged that there is no way to detect their use
(Strausser 2014). On the basis of unpublished data (J. Manson, Pers. Comm.) it
appears that up to 50% of deer herds can be infected with 80-90% of animals
infected within some herds.
*** It is therefore assumed that probability that a 30-ml bottle of deer
urine lure imported from the USA is sources from an infected deer is
medium.
Exposure assessment
Importation of animal feed
Once in GB, the use of animal feed is subject to the TSE Feed Ban and ABP
Regulations. The BSE-related feed ban prohibits the feeding of PAP and gelatine
from ruminants to ruminants (including farmed deer) or non-ruminant farmed
animals. Further, ruminants must not be fed any animal protein or feedstuffs
which contain animal protein except for milk, milk-based products and colostrum,
eggs and egg products, gelatine from non-ruminants and hydrolysed proteins
derived from non-ruminants or from ruminant hides and skins. Therefore, in
accordance with the current ban, farmed deer should not be directly exposed to
(i.e. feed) imported animal feed containing any PAP. Therefore, assuming this
ban is adhered to correctly the risk of farmed deer being exposed to animal feed
containing deer protein from North America is considered negligible but with
associated uncertainty. ***However, given that non-ruminant feed produced in the
USA may contain deer and elk PAP, it is theoretically possible that wild deer
may be exposed to deer protein in legally imported non-ruminant feed. For this
to occur, wild deer would need to access non-ruminant feed (e.g. pig, fish and
chicken feed) on farms near their habitat. Alternatively, wild deer may be
exposed to CWD prion in the faeces of pets that have consumed and digested
imported, contaminated pet feed. ***The frequency in which these routes may
occur is unknown and is considered to be a greater than negligible risk with
associated uncertainty.
Movement of hunters, other tourists and British servicemen
The pathways by which naïve deer can be exposed to CWD contaminated soil
and prions on equipment and clothing from people arriving to GB from North
America are variable and highly uncertain. In principle, in order to expose a
deer to CWD prions, the traveller (hunter, tourist or serviceman) would need to
transfer the CWD prion from their clothing and/or equipment to the environment
in which deer habit. The latter will depend upon the behaviours of returning GB
residents or tourists and the probability of entering into and walking around
deer territory. In GB, there are two main deer populations (wild and
farmed
15
or park deer) each of which will have differing risks of exposure given the
type and frequency of contact with people. Each population type is considered in
turn.
Wild deer
There are 6 species of wild deer residing in GB including: Red deer (Cervus
elaphus), Roe deer (Capreolus capreolus), fallow deer (Dama dama), muntjac
(Muntiacus reevesi), sika (Cervus nippon), and Chinese Water deer (Hydropotes
inermis). The British Deer Society implemented a survey in 2007 to ascertain the
distribution of these deer species across the UK. The survey provides the
presence of deer on a standard template of 10km grid squares (www.bds.org.uk ).
A further survey was conducted in 2011 of which the results will be published
later in 2012. The deer distribution as ascertained from the 2007 survey is
summarised in Figure 3.
It can be seen from Figure 3 (below) that deer are widely distributed
across the UK with Roe deer being the most widespread. Chinese Water deer are
the smallest deer population with approximately 700 deer.
Deer hunters, particularly, are most likely to be in direct contact with
wild deer and their habitat compared to other tourists and returning GB
residents. During the stalking and/or hunting of deer, there is opportunity for
CWD prion on the hunter’s boots, clothing and/or equipment to be transferred to
the environment. The amount transferred will depend upon the measures taken to
remove soil etc from the equipment prior to stalking. Assuming that CWD prion is
transferred to the environment, there is an uncertain probability that a deer
will come into contact with the CWD prion.
16
Figure 3: Distribution of the six deer species in the UK in 2007 (British
Deer Society Deer Distribution Survey, http://www.bds.org.uk)
Farmed and park deer
Deer farming is a relatively recent enterprise. There are two systems
currently used for managing enclosed deer: park and farm deer systems. In the
park system, deer are raised in a park type setting and allowed to roam freely
and may be provided with some supplemental feed. Farmed deer, in contrast,
following conventional agricultural practices and may be housed in the winter
and nutritional supplements are provided where necessary. In this farming
system, there are several categories including calf rearers, calf finishers,
breeder finishers and producer/processors (www.bdfpa.org). In 2011, according to
the June Agricultural census, there were approximately 21,000 farmed deer on
commercial agricultural holdings in England. The Economic Report on Scottish
Agriculture (2011) cited that within Scotland, Wales, England and Northern
Ireland there were 30,910 farmed deer. It is less likely that tourists, deer
hunters and British servicemen will come into contact with conventionally farmed
deer compared to park deer. The total park deer population in GB is unknown.
However, in 2005, based on annual population control culling of about 8,000
animals, it was estimated that there were approximately 40,000 park deer. These
deer are distributed across several parks (some of which are famous
tourist
17
sites) where wild and/or exotic species of deer can roam and be viewed.
These are outlined in Table 2.
Table 2: Summary of parks in Great Britain where wild and/or exotic deer
roam (The British Deer Society, www.bds.org.uk)
Country Park Species
England Ashton Court, Bristol Red, Fallow Bolderwood Deer Sanctuary,
Minstead, Hampshire Fallow, Bradgate Park, Charnwood Forest, Leicestershire
Fallow, Red Bushy Park, Hampton Hill, London Fallow, Red Chatsworth Park,
Chatsworth, Derbyshire Fallow, Red Dunham Massey, Altrincham, Cheshire Dyrham
Park, Chippenham, Wiltshire Fallow Grimsthorpe Castle Park and Gardens, Bourne,
Lincolnshire Helmington Hall, Stowmarket, Suffolk Red Holkham Estate,
Wells-next-the-Sea, Norfolk Fallow Knole Park, Sevenoaks, Kent Fallow, Sika
Lodge Park & Sherborne Estate, Lyme Park, Disley, Cheshire Red, Fallow New
Forest Wildlife Park, Longdown, Hampshire European Bison, Red deer Petworth
Park, Petworth, Sussex Fallow Prinknash Deer and Bird Park, Cranham, Gloucester
Raby Castle, Staindrop, County Durham Red, Fallow
18
Richmond Park, Richmond, London Red, Fallow Snettisham Park Farm, King’s
Lynn, Norfolk Red South West Deer Rescue and Study Centre, Wayford Red, White
Red, Fallow, Axis, Roe, Japanese Sika Tatton Park, Knutsford, Cheshire Wentworth
Castle, Stainborough Red, Fallow Wildwood Trust, Hern Bay, Kent Roe, Fallow, Red
Woburn Abbey, Woburn Park, Bedfordshire Sika, Axis, Chital, Barasingha, Chinese
Water, Rusa, Pere David Wollaton Park, Woolaton, Nottinghamshire Red,
Fallow
Wales
Abergavenny Priory Deer Park, Abergavenny Dinefwr House, Kinefwr Park,
Llandeilo, Carmarthenshire Fallow Margam Country Park, Port Talbot Red, Pere
David, Chital, Hog, Barasingha, Roe, Muntjac, Chinese Water
Scotland
Beecraigs Country Park, Linlithgow, West Lothian Red Glengoulandie Deer
Park, Aberfeldy, Perthshire Red Highland Wildlife Park, Kingussie,
Inverness-shire Red, reindeer Jedforest Deer and Farm Park, Jedburgh The
Scottish Deer Centre, Cupar, Fife Nine species of deer
19
It is evident from Table 2 that there are several locations in GB where
tourists and returning residents may come into contact with park deer and, in
doing so, potentially expose the deer to CWD on their contaminated clothing
and/or footwear. Further, given the volume of tourists and other travellers
moving between North America and GB, there are potentially multiple
opportunities for CWD prions to be transferred from clothing, boots and/or
equipment to the environment. It has been observed that multiple exposures to
low levels of CWD prions in the environment and increased infectivity of CWD
when prions are bound to the soil are influential factors in transmission (Anger
et al., 2009). Given the nature of their management, there is a restricted area
(or environment) in which park deer inhabit enabling them to have a potentially
higher probability of coming into contact with any CWD transferred to the
environment by a tourist or returning GB resident compared to wild deer in a
free-ranging environment. ***Therefore, it is considered that farmed and park
deer may have a higher probability of exposure to CWD transferred to the
environment than wild deer given the restricted habitat range and higher
frequency of contact with tourists and returning GB residents.
Exposure of UK deer to infected urine
For the purpose of the qualitative risk assessment developed here it is
necessary to estimate the probability that some of the contents (i.e. 10-ml) of
each 30-ml bottle of deer urine lure is ingested by UK deer.
The degree of exposure depends on whether deer are likely to lick urine in
the woods, and where the urine is placed by the hunter. Some hunters claim it is
unlikely deer will lick the urine (Anon 2015a). The main risk reduction factor
is the fact that deer roam over a very large area and female deer (those not
attracted) would be unlikely to come in contact with it. The risk of exposure to
female deer therefore may be relatively low. This raises the question of deer
bucks which are attracted to the lure and whether they lick it. The aqueous
solvent component of the urine will evaporate or soak into the ground. However,
it is well known that TSE infectivity does not leach into the ground but
attaches to soil particles, which could be eaten by grazing deer. ***Although
the infectivity may be less bioavailable compared to a deer actually imbibing
the contents of a 30-ml bottle of deer urine lure the exposure of single bucks
attracted to the lure cannot be assumed to be negligible. ***Furthermore it
cannot be assumed that the lured deer are shot and the deer lure may continue to
attract deer for some time after the hunter/stalker has departed. It is not
known how long the lure would work for. CWD infectivity will persist in the
environment for long periods of time, and bind to the soil surface.
***Therefore, there is the possibility that the CWD infectivity in deer lure
urine may persist for months, such that deer could be exposed at a later date.
***Overall therefore, given the fact that the primary use of a deer lure is to
attract bucks, it is assumed here that the probability that some of the contents
(i.e. 10-ml) of each 30-ml bottle of deer urine lure is ingested by UK deer is
medium.
Given that a deer within GB is exposed to CWD bio-available prions in the
environment, the probability of becoming infected is dependent upon the
infectious dose and the susceptibility of the animal to the prion. The majority
of research into CWD has been
20
conducted in North America where it has been shown that the following
species are naturally infected with CWD (Hamir et al., 2008):
Mule deer (Odocoileus hemionus hemionus)
Black-tailed deer (Odocoileus hemionus columbianus)
White-tailed deer (Odocoileus virginianus)
Rocky Mountain elk (Cervus elphus nelsoni)
Shira’s moose (Alces alces shirasi)
None of these species are present in GB. However, EFSA (2010) considered
that red deer (Cervus elaphus), a species present in GB (see Figure 3), is
likely to be susceptible to CWD and was a species specifically targeted in the
EU survey for CWD. This stems from the fact that red deer have a close genetic
relationship to Rocky Mountain elk. This hypothesis has been supported by recent
experimental studies that have demonstrated red deer become infected with CWD
after oral inoculation of brain tissue from infected Rocky Mountain elk
(Balachandran et al., 2010). Specifically, two of the four 2-month old red deer,
showed clinical signs by 585 days p.i. and all deer had CWD prion in the brain,
spinal cord and other organs at necropsy (Balachandran et al., 2010). Further,
Martin et al., (2009) demonstrated in a similar study of four European red deer,
that red deer can become infected upon inoculation of 5g of infected brain
homogenate from four CWD elk and hence the species is susceptible to CWD.
Hamir et al., (2008) undertook a study to ascertain if fallow deer (Dama
dama), another British deer species, could be experimentally infected with CWD
brain suspension from infected elk or white-tailed deer. The authors concluded
that it is possible to transmit CWD to fallow deer via the intracerebral route
but the pathological features of CWD in the deer differs from those observed in
white-tailed deer or elk (Hamir et al., 2008). It was further concluded that it
might not be possible to transmit CWD via a more natural route or,
alternatively, a higher dose of inoculum is required leading to a longer
incubation period (Hamir et al., 2008).
Initial studies into the PRioN Protein (PRNP) gene variability in European
red deer and roe deer suggest that these species have a PRNP genetic background
that is compatible with TSE susceptibility, including CWD (EFSA, 2011). It is
important to note, however, that no experimental studies on roe deer have been
conducted verifying this hypothesis.
There are no data on the susceptibility of the other free-ranging deer
species present in Britain (muntjac (Muntiacus reevesi), sika (Cervus nippon),
Chinese Water deer (Hydropotes inermis)) to CWD. Further experimental studies
would be required to investigate the susceptibility of these species to CWD.
***Therefore, on the basis of current scientific understanding, it is likely
that given exposure to an infectious dose to CWD, deer in GB could become
infected with CWD. Whether the amount of CWD prion that could be transferred
from clothing, boots and/or other equipment into the deer’s environment is
enough to induce infection given that the infectious dose is extremely small
(Saunders et al., 2010) is uncertain. However, given that the amount of soil
ingested is likely to be very small, the probability of ingesting an infectious
dose via this route is considered negligible
21
to very low. The probability of ingesting an infectious dose via
consumption of non-ruminant feed is likely to be higher and may be very low,
with associated uncertainty.
The CWD agent is relatively dilute in deer urine compared to brain and
spinal cord material with 1 ic LD50 per 10 ml. The LD50 determined by Henderson
et al. (2015) is an intracerebral ID50 in cervidized transgenic mice. The oral
ID50 in deer would be in a much larger volume of urine, because the oral route
may be 100,000-fold less efficient than the intracerebral route in terms of TSE
transmission (Gale et al 1998). Thus, in terms of oral LD50, there may be one in
a 1,000,000 ml i.e. 1 m3 or 1,000 litre volume of deer urine. Therefore a deer
would have to ingest 1,000 litres of urine to have a 50% chance of being
infected through the oral route. Of course the CWD agent would be concentrated
as the urine evaporated from the tree trunk.
The main sources of uncertainty are:-
1. The amount of urine ingested by the deer. A bottle of Tink’s “69
Doe-in-Rut Buck” is about 30 ml and boasts 100% natural doe oestrous urine (Anon
2105d). A bottle from an infected doe would thus contain about 3 i.c. LD50
units. The bottles come with an easy to use squirt top, so it seems relatively
small amounts are used. There are also gel forms which do not freeze as fast,
last longer in the rain, and do not dry out so quickly.
2. The magnitude of oral/i.c. barrier
Assuming a deer drank 10 ml of urine from each 30 ml bottle, then that deer
would ingest 10-5 LD5. It is generally assumed that there is no threshold dose
for TSEs (Gale 2006) and the risk of CWD infection in GB deer per 30 ml bottle
imported is therefore 0.69 x 10-5 (Gale 1998). This is very low.
Table 1: Risk of infection of deer in GB per 30 ml bottle of deer urine
lure imported from the USA
Step in pathway Risk Uncertainty
Entry (probability a 30-ml bottle contains urine from an infected deer)
Medium Low
Exposure (probability some of 30-ml bottle is ingested by UK deer) Medium
High
Consequence (probability that deer is infected given exposure) Very low
Medium
Overall risk Very low High
Overall the risk of a deer in GB being infected per 30 ml bottle of urine
imported from the USA is very low, albeit with high uncertainty.
22
The next question is how much deer urine is imported into GB from the USA
per year. If there are n 30-ml bottles, then the risk is calculated as:-
pn_bottle = 1 - (1 – pone_bottle)n
Hundreds of gallons of urine are sold every year just by one company in the
USA (Anon 2015b). It is difficult, however, to estimate the volume of deer urine
imported into the UK per year from the USA. That some respondents to the BDS
survey reported they used upwards of 1litre per year, is consistent with
importation of a high number of 30-ml bottles. Assuming that the number, n, of
30-ml bottles imported annually is high, then the risk of CWD infection in at
least one deer in the UK per year will be medium (given the probability, of
infection of a UK deer from one 30-ml bottle is very low (see Table 1)).
***Overall it is concluded that the annual risk of at least one infection
of deer in the UK with CWD from deer urine lures imported from the USA is
medium. This assumes a high number of 30 ml bottles imported per year from all
areas of the USA.
Control and risk management options
In order to reduce the potential amount of CWD prion entering GB on boots
and clothing, it is important to meticulously clean off all adherent material
prior to departing from North America. As CWD is a highly resistant agent, where
possible, equipment should be soaked in a solution of bleach that has 20,000
parts per million of active chlorine for one hour. However, it is acknowledged
it is impractical to soak hunting boots, clothing or firearms, for example, in
strong concentrations of bleach.
For deer urine lures, the import of unprocessed natural deer urine is not
allowed under the EU Animal By-Product legislation. However the import of such
commodities including internet sales would still require licenses, but it would
be a voluntary action on behalf of the importer to ensure the urine is sourced
from safe herds. One option is to ensure the sourcing is from herds in States
which are currently registered under the National CWD Herd Certification
Programme which has recently been introduced in the USA. Otherwise the use of
synthetic of European-produced lures should be promoted by the industry and
stakeholder groups.
Conclusions
There is significant uncertainty associated with estimating the risk of CWD
entering the UK via movement of people (tourists, hunters and British
servicemen) and importation of animal feed. This partly stems from the lack of
data on these two importation routes. Given this uncertainty, the probability of
importing CWD into GB from North America and causing infection in British deer
is likely to be negligible to very low via movement of deer hunters, other
tourists and British servicemen and very low via imported (non-ruminant) animal
feed.
23
***However the risk of natural deer urine lures from the USA containing CWD
PrP is considered high (reflected by some US States banning the use) and the
probability of such a commodity, if used in significant volumes, leading to CWD
infection in GB populations is considered to be medium (lack of susceptibility
in certain species and limited use by hunters and stalkers in GB) but with a
high level of uncertainty.
The consequences of CWD, however, are severe with the minimal possibility
of eradicating the disease from a wild cervid population and populations of wild
and farmed deer would be under threat.
Current research indicates that of the six free-ranging deer species in the
UK, red deer are susceptible to CWD. This deer species is concentrated in
distinct areas of the country (e.g. North of Scotland) and one of the key
species which hunters, in particular, stalk. It is important, therefore, that
the risk of this species being exposed to CWD is minimised by taking appropriate
precautionary measures.
References
Almberg, E.S., Cross, P.C., Johnson, C.J., Heisey, D.M. & Richards,
B.J. (2011) Modeling routes of chronic wasting disease transmission:
environmental prion persistence promotes deer population decline and extinction.
PLos ONE, 6(5), e19896. Angers, R.C, Browning, S.R, Seward, T.S., Sigurdson,
C.J., Miller, M.W., Hoover, E.A., & Telling, G.C. (2006) Prions in skeletal
muscles of deer with chronic wasting disease. Science, 311, 1117. Angers, R.C.,
Seward, T.S., Napier, D., Green, M., Hoover, E., Spraker, T., O’Rourke, K.,
Balachandran, A., & Telling, G.C. (2009) Chronic wasting disease prions in
elk antler velvet. Emerging Infectious Diseases, 15 (5), 696-703. Anon (2015a)
http://www.fieldandstream.com/blogs/field-notes/virginia-bans-deer-urine-lures
Anon (2015b) http://www.bloomberg.com/bw/articles/2012-08-31/odd-jobs-deer-urine-farmer
Anon (2015d) Tink’s natural attraction http://www.trophybucklure.com/ APHIS
(2015) Voluntary National Herd CWD Certification Programme. See APHIS website at
Link Accessed August 2015. Balachandran, A., Harrington, N.P., Algire, J.,
Soutyrine, A., Spraker, T.R., Jeffrey, M., Gonzalez, L., & O’Rourke, K.I.
(2010). Experimental oral transmission of chronic wasting disease to red deer
(Cervus elaphus elaphus): Early detection and late stage distribution of
protease-resistant prion protein. Canadian Veterinary Journal-Revue Veterinaire
Canadienne, 51, 169-178.
24
Dalgleish, M.P., Martin, S., Steele, P., Finlayson, J., Siso, S., Hamilton,
S., Chianini, F., Reid, H.W., Gonzalez, L., & Jeffrey, M. (2008)
Experimental transmission of bovine spongiform encephalopathy to European red
deer (Cervus elaphus elaphus). BMC Veterinary Research, 4, 17. EFSA (2004) Annex
to the EFSA Journal (2004) 70 On the Opinion on a surveillance programme for
Chronic Wasting Disease in the EU. EFSA Panel on Biological Hazards. (2011)
Joint Scientific Opinion on any possible epidemiological or molecular
association between TSEs in animals and humans. EFSA Journal, 9(1), 1945. EFSA
Panel on Biological Hazards. (2010). Scientific Opinion on the results of the EU
survey for Chronic Wasting Disease (CWD) in cervids. EFSA Journal, 8(10), 1861.
Gale, P. (1998) Quantitative BSE risk assessment: relating exposures to risk.
Letters in Applied Microbiology, 27, 239-242. Gale, P., Young, C., Stanfield, G.
& Oakes, D. (1998) Development of a risk assessment for BSE in the aquatic
environment. Journal of Applied Microbiology, 84, 467-477. Gale, P. (2006) The
infectivity of transmissible spongiform encephalopathy agent at low doses: the
importance of phospholipids. Journal of Applied Microbiology, 101, 261-274.
Georgsson, G., Sigurdarson, S., & Brown, P. (2006) Infectious agent of sheep
scrapie may persist in the environment for at least 16 years. Journal of General
Virology, 87, 3737-3740. Gross, J.E. & Miller, m.W. (2001) Chronic wasting
disease in mule deer: disease dynamics and control. Journal of Wildlife
Management, 65, 2001. Haley, N.J., Seelig, D.M., Zabel, M.D., Telling, G.C.,
& Hoover, E.A. (2009) Detection of CWD prions in urine and saliva of deer by
transgenic mouse bioassay. PLoS One, 4(3), e4848. Hamir, A.N., Kunkle, R.A.,
Nicholson, E.M., Miller, J.M., Hall, S.M., Schoenenbruecher, H., Brunelle, B.W.,
& Richt, J.A. (2008) Preliminary observations on the experimental
transmission of chronic wasting disease (CWD) from elk and white-tailed deer to
fallow deer. Journal of Comparative Pathology, 138, 121-30. Hamir, A.N., Kunkle,
R.A., Miller, J.M., Greenlee, J.J., & Richt, J.A. (2006) Experimental second
passage of chronic wasting disease (CWDmule deer) agent to cattle. J. Comp.
Path, 134, 67-73. Hartley, M., Voller, F., Murray, T. & Roberts, H. (2012)
Qualitative veterinary risk assessment of the role of wild deer in the
likelihood of incursion and the impact on effective disease control of selected
exotic notifiable diseases in England. Eur J. Wildl Res DOI
10.1.1007/s10344-012-0647-7.
25
Henderson, D.M., Davenport, K.A., Haley, N.J., Denkers, N.D., Mathiason,
C.K. & Hoover, E.A. (2015) Quantitative assessment of prion infectivity in
tissues and body fluids by real time quaking-induced conversion. Journal fo
General Virology 96: 210-219. Johnson, C.J., Philips, K.E., Schramm, P.T.,
McKenzie, D., Aiken, J.M., & Pedersen, J.A. (2006) Prions adhere to soil
minerals and remain infectious. PLoS Pathogens, 2 (4), e32. Johnson, C.J.,
Pedersen, J.A., Chappell, R.J., McKenzie, D., & Aiken, J.M. (2007) Oral
transmissibility of prion disease is enhanced by binding to soil particles. PLoS
Pathogens, 3 (7), e93. Martin, S., Jeffrey, M., Gonzalez, L., Siso, S., Reid,
H.W., Steele, P., Dagleish, M.P., Stack, M.J., Chaplin, M.J., &
Balachandran, A. (2009) Immunohistochemical and biochemical characteristics of
BSE and CWD in experimentally infected European red deer (Cervus elaphus
elaphus). BMC Veterinary Research, 5:26. Mathison, C,K., et al., (2006)
Infectious prions in the saliva and blood of deer with chronic wasting disease.
Science, 314, 133-136. Miller, M.W., Wild, M.A., & Williams, E.S. (1998)
Epidemiology of chronic wasting disease in captive rocky mountain elk. Journal
of Wildlife Diseases, 34 (3), 532-538. Miller, M.W., Williams, E.S., Hobbs,
N.T., & Wolfe, L.L. (2004) Environmental sources of prion transmission in
mule deer. Emerging Infectious Diseases, 10(6), 1003-1006. Nichols, T.A.,
Pulford, B., Wyckoff, A.C., Meyerett, C., Michel, B., Gertig, K., Hoover, E.A.,
Jewell, J.E., Telling, G.C., & Zabel, M.D. (2009) Detection of
protease-resistant cervid protein in water from a CWD-endemic area. Prion, 3,
171-183. OIE, (2004). Handbook on import risk analysis for animals and animal
products. World Organisation for Animal Health, 12, rue de Prony, 75017 Paris,
France, p.59. Rural & Environmental Science & Analytical Services
(2011). Economic Report on Scottish Agriculture 2011 Edition. Scottish
Government: Edinburgh. Russo, F., Johnson, C.J., Johnson, C.J., McKenzie, D.,
Aiken, J.M., & Pedersen, J.A. (2009) Pathogenic prion protein is degraded by
manganese oxide material found in soils. Journal of General Virology, 90,
275-280. Safar, J.G., Lessard, P., Tamguney, G., Freyman, V., Deering, C.,
Letessier, F., DeArmond, S.J., & Prusiner, S.B. (2008) Transmission and
detection of prions in feces. Journal of Infectious Diseases, 198, 81-89.
Saunders, S.E., Bartz, J.C., Vercauteren, K.C., & Bartelt-Hunt, S.L. (2010)
Enzymatic digestion of chronic wasting disease prions bound to soil. Environ.
Sci. Technol., 44, 4129-4135.
26
Seidel, B., Thomzig, A., Buschmann, A., Groschup, M.H., Peters, R., Beekes,
M., & Terytze, K., (2007) Scrapie agent (strain 263K) can transmit disease
via the oral route after persistence in soil over years. PLoS Pathogens, 5,
e435. Sigurdson, C.J. (2008) A prion disease of cervids: Chronic wasting
disease. Veterinary Research, 39, 41. Strauser, K. (2014) Detection of
Urine-Based Deer Lures to Mitigate CWD Transmission in Pennsylvania Keystone
Journal of Undergraduate Research 2(1): 1-7. 2014 Tamguney, G., Miller, M.W.,
Wolfe, L.L., Sirochman, T.M., Glidden, D.V., Palmer, C., Lemus, A., DeArmond,
S.J. & Prusiner, S.B. (2009) Asymptomatic deer excrete infectious prions in
faeces. Nature, 461, 529-590. TAMS (2006) Travel activities and motivations of
Canadian residents: An overview. Ministry of Tourism. TAMS (2007) Travel
activities and motivations of U.S. residents: An overview. Ministry of Tourism.
Trifilo, M.J., Ying, G., Teng, C. & Oldstone, M.B.A. (2007) Chronic wasting
disease of deer and elk in transgenic mice: oral transmission and pathobiology.
Vaccine, 365, 136-143. U.S. Fish & Wildlife Service (2011). Deer hunting in
the United States: Demographics and Trends. Addendum to the 2006 National Survey
of Fishing, Hunting, and Wildlife-associated recreation. Report 2006–10.
Wildlife and Sport Fish Restoration: Arlington, VA. USGS (2016) Chronic Wasting
Disease (CWD) http://www.nwhc.usgs.gov/disease_information/chronic_wasting_disease/
Accessed 05/04/2016. Williams, E.S. (2005) Chronic wasting disease. Vet Pathol.,
42, 530-549. Williams, E.S., & Miller, M.W. (2003) Transmissible spongiform
encephalopathies in non-domestic animals: origin, transmission and risk factors.
Rev. Sci. Tech. Off. Int Epiz., 22, 145-156. Wilkinson, D.M. (2010) Have we
underestimated the importance of humans in the biogeography of free-living
terrestrial microorganisms? Journal of Biogeography, 37, 393-397.
What is the risk of chronic wasting disease being introduced into Great
Britain? An updated Qualitative Risk Assessment March 2016
From: Terry S. Singeltary Sr.
Sent: Thursday, April 07, 2016 1:02 PM
To: psi@nationalarchives.gsi.gov.uk
Cc: helen.roberts@apha.gsi.gov.uk ; jan.opgenoorth@efsa.europa.eu ;
Press@efsa.europa.eu ; SCER.PublicConsult.55@efsa.europa.eu ;
scer@efsa.europa.eu ; publicmeetings@efsa.europa.eu ;
Gemma.Smith@wales.gsi.gov.uk
BCC: ...
Subject: What is the risk of chronic wasting disease being introduced into
Great Britain? 6 April 2016
Greetings DEFRA MAFF, EFSA, Welsh Government et al,
I kindly wish to again the urgent need to address CWD in the USA and risk
factors to the world there from.
I see ‘What is the risk of chronic wasting disease being introduced into
Great Britain?’ was published recently 6 April 2016 and would kindly like to
send this additional information.
What is the risk of chronic wasting disease being introduced into Great
Britain? An updated Qualitative Risk Assessment March 2016
Since my last correspondence with you, CWD has exploded in new states, with
Arkansas topping them all. Texas is reluctant to report in a timely manner, just
how bad CWD is in the captive industry, but of course, Texas did the same thing
with mad cow disease, covering up the last case for 7 months before it took an
act of Congress to finally have that sample retested, which did confirm the mad
cow, all this documented on the internet. with that said, I do not have any hope
that CWD surveillance and timely reporting to the public will be any different.
In two months Arkansas went from a few cases to a total of 56 cases in just two
months, from a very small sample survey. also, I have pleaded with the FDA et al
to please close the damn mad cow feed loop hole for cervids. FDA has finally
readdressed the old Docket, updated it in March 2016, HOWEVER IT’S NON-BINDING,
STILL NOTHING BUT INK ON PAPER. things don’t look good here at all. cwd is out
of control, and corporate America is and will continue to let cwd spread
further. as you all well know, our politicians are too worried about who’s
‘richard’ is bigger than the others, while Rome burns. I am embarrassed about
our country and it’s politicians, so I shall apologize to the world for the
sheer stupidity of it all. ...
I wish to kindly give you an update on this CWD TSE Prion situation here in
the USA.
Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer
and Elk in Animal Feed Singeltary Submission
*** URGENT UPDATE MARCH 2016 OF THIS OLD DOCKET ***
however, it’s still non-binding $$$
insane!!! imo...
Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer
and Elk in Animal Feed Singeltary Submission
Greetings again FDA and Mr. Pritchett et al,
I would kindly like to comment on ;
Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer
and Elk in Animal Feed Singeltary Submission
#158
Guidance for Industry
Use of Material from Deer and Elk in Animal Feed
This version of the guidance replaces the version made available
September15, 2003.
This document has been revised to update the docket number, contact
information, and standard disclosures. Submit comments on this guidance at any
time.
Submit electronic comments to http://www.regulations.gov. Submit written
comments to the Division of Dockets Management (HFA-305), Food and Drug
Administration, 5630 Fishers Lane, Rm. 1061, Rockville, MD 20852. All comments
should be identified with the Docket No. FDA-2003-D-0432 (formerly 03D-0186).
For further information regarding this guidance, contact Burt Pritchett,
Center for Veterinary Medicine (HFV-222), Food and Drug Administration, 7519
Standish Place, Rockville, MD 20855, 240-402-6276, E-mail:
burt.pritchett@fda.hhs.gov.
Additional copies of this guidance document may be requested from the
Policy and Regulations Staff (HFV-6), Center for Veterinary Medicine, Food and
Drug Administration, 7519 Standish Place, Rockville, MD 20855, and may be viewed
on the Internet at either http://www.fda.gov/AnimalVeterinary/default.htm
or http://www.regulations.gov.
U.S. Department of Health and Human Services Food and Drug Administration
Center for Veterinary Medicine March 2016
Contains Nonbinding Recommendations
2
Guidance for Industry Use of Material from Deer and Elk in Animal Feed
This guidance represents the current thinking of the Food and Drug
Administration (FDA or Agency) on this topic. It does not establish any rights
for any person and is not binding on FDA or the public. You can use an
alternative approach if it satisfies the requirements of the applicable statutes
and regulations. To discuss an alternative approach, contact the FDA office
responsible for this guidance as listed on the title page.
I. Introduction
Under FDA’s BSE feed regulation (21 CFR 589.2000) most material from deer
and elk is prohibited for use in feed for ruminant animals. This guidance
document describes FDA’s recommendations regarding the use in all animal feed of
all material from deer and elk that are positive for Chronic Wasting Disease
(CWD) or are considered at high risk for CWD. The potential risks from CWD to
humans or non-cervid animals such as poultry and swine are not well understood.
However, because of recent recognition that CWD is spreading rapidly in
white-tailed deer, and because CWD’s route of transmission is poorly understood,
FDA is making recommendations regarding the use in animal feed of rendered
materials from deer and elk that are CWD-positive or that are at high risk for
CWD.
In general, FDA’s guidance documents do not establish legally enforceable
responsibilities. Instead, guidances describe the Agency’s current thinking on a
topic and should be viewed only as recommendations, unless specific regulatory
or statutory requirements are cited. The use of the word should in Agency
guidances means that something is suggested or recommended, but not
required.
II. Background
CWD is a neurological (brain) disease of farmed and wild deer and elk that
belong in the animal family cervidae (cervids). Only deer and elk are known to
be susceptible to CWD by natural transmission. The disease has been found in
farmed and wild mule deer, white-tailed deer, North American elk, and in farmed
black-tailed deer. CWD belongs to a family of animal and human diseases called
transmissible spongiform encephalopathies (TSEs). These include bovine
spongiform encephalopathy (BSE or “mad cow” disease) in cattle; scrapie in sheep
and goats; and classical and variant Creutzfeldt-Jakob diseases (CJD and vCJD)
in humans. There is no known treatment for these diseases, and there is no
vaccine to prevent them. In addition, although validated postmortem diagnostic
tests are available, there are no validated diagnostic tests for CWD that can be
used to test for the disease in live animals.
Contains Nonbinding Recommendations
III. Use in animal feed of material from CWD-positive deer and elk
Material from CWD-positive animals may not be used in any animal feed or
feed ingredients. Pursuant to Sec. 402(a)(5) of the Federal Food, Drug, and
Cosmetic Act, animal feed and feed ingredients containing material from a
CWD-positive animal would be considered adulterated. FDA recommends that any
such adulterated feed or feed ingredients be recalled or otherwise removed from
the marketplace.
IV. Use in animal feed of material from deer and elk considered at high
risk for CWD Deer and elk considered at high risk for CWD include: (1) animals
from areas declared by State officials 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 immediately before the time of slaughter were in a captive herd that
contained a CWD-positive animal.
FDA recommends that materials from deer and elk considered at high risk for
CWD no longer be entered into the animal feed system. Under present
circumstances, FDA is not recommending that feed made from deer and elk from a
non-endemic area be recalled if a State later declares the area endemic for CWD
or a CWD eradication zone. In addition, at this time, FDA is not recommending
that feed made from deer and elk believed to be from a captive herd that
contained no CWD-positive animals be recalled if that herd is subsequently found
to contain a CWD-positive animal.
V. Use in animal feed of material from deer and elk NOT considered at high
risk for CWD FDA continues to consider materials from deer and elk NOT
considered at high risk for CWD to be acceptable for use in NON-RUMINANT animal
feeds in accordance with current agency regulations, 21 CFR 589.2000. Deer and
elk not considered at high risk include: (1) deer and elk from areas not
declared by State officials to be endemic for CWD and/or to be CWD eradication
zones; and (2) deer and elk that were not at some time during the 60-month
period immediately before the time of slaughter in a captive herd that contained
a CWD-positive animal.
3
Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer
and Elk in Animal Feed Singeltary Submission
Greetings again FDA and Mr. Pritchett et al,
MY comments and source reference of sound science on this very important
issue are as follows ;
Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer
and Elk in Animal Feed Singeltary Submission
I kindly wish to once again submit to Docket No. FDA-2003-D-0432 (formerly
03D-0186) Use of Material from Deer and Elk in Animal Feed.
Thank you kindly for allowing me to comment again, ...and again...and
again, on a topic so important, why it is ‘NON-BINDING’ is beyond me.
this should have been finalized and made ‘BINDING’ or MANDATORY OVER A
DECADE AGO.
but here lay the problem, once made ‘BINDING’ or ‘MANDATORY’, it is still
nothing but ink on paper.
we have had a mad cow feed ban in place since August 1997, and since then,
literally 100s of millions of pounds BANNED MAD COW FEED has been sent out to
commerce and fed out (see reference materials).
ENFORCEMENT OF SAID BINDING REGULATIONS HAS FAILED US TOO MANY TIMES.
so, in my opinion, any non-binding or voluntary regulations will not work,
and to state further, ‘BINDING’ or MANDATORY regulations will not work unless
enforced.
with that said, we know that Chronic Wasting Disease CWD TSE Prion easily
transmits to other cervid through the oral route.
the old transmission studies of BSE TSE floored scientist once they figured
out what they had, and please don’t forget about those mink that were fed 95%+
dead stock downer cow, that all came down with TME. please see ;
*** PLEASE SEE THIS URGENT UPDATE ON CWD AND FEED ANIMAL PROTEIN ***
Sunday, March 20, 2016
Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer
and Elk in Animal Feed ***UPDATED MARCH 2016*** Singeltary Submission
see what the UK GOV warns about this;
Saturday, April 02, 2016
TEXAS TAHC BREAKS IT'S SILENCE WITH TWO MORE CASES CWD CAPTIVE DEER
BRINGING TOTAL TO 10 CAPTIVES REPORTED TO DATE
Friday, February 26, 2016
TEXAS Hartley County Mule Deer Tests Positive for Chronic Wasting Disease
CWD TSE Prion
Division of Ag researchers move ahead determining prevalence, distribution
of Chronic Wasting Disease in state deer, elk By Ryan McGeeney U of A System
Division of Agriculture April 1, 2016
Fast Facts:
CWD detected in deer in Newton County
Experts working to determine prevalence CWD not transmissible between
species (732 words)
LITTLE ROCK — Researchers with the University of Arkansas System Division
of Agriculture said this week that 18 out of 48 white-tailed deer tested in
Newton County in March were found to have Chronic Wasting Disease, also known as
CWD.
Don White, Jr., professor and wildlife ecologist for the Division of
Agriculture, said that number was probably not representative of the disease’s
actual prevalence in the state’s wildlife herds.
“Those samples we had contained what’s known as ‘focal sampling,’” White
said. “Those animals were either road-killed or seemed to display the symptoms
of CWD, so it’s not random, it’s not representative of the whole.”
White said the samples were collected by Arkansas Game and Fish Commission
biologists.
CWD is a transmissible form of spongiform encephalopathy, which kills
members of the deer family by altering proteins known as prions in the animals’
brains. Symptoms include weight loss to the point of emaciation, excessive
salivation, loss of coordination and death. White said there is currently no
vaccine for the disease, which results in 100 percent fatality.
White and he and Game and Fish Commission biologists had collected sample
tissue from 265 deer within the Newton County surveillance zone, harvesting
tissue post-mortem from the animals’ brain stem and lymph nodes, areas that tend
to accumulate the proteins that cause CWD. The samples are being sent in batches
to a veterinarian laboratory in Madison, Wis., which takes about 10-14 days to
analyze them, White said.
Once all of the results from the 265 deer are available, prevalence of CWD
in the population of deer within the surveillance zone can be determined. Once
prevalence is known, researchers will begin working to determine the
distribution of CWD in the general area where it has been detected.
By establishing the distribution of the disease, White said, researchers
can begin to address areas where the disease is concentrated, which may require
looking at some human practices, as well.
“Some researchers have seen a relationship between the presence of CWD and
the density of the deer herd,” White said. “So the more animals per unit of
space, the greater the prevalence of CWD. One approach may be to reduce deer
density, and keep it low in those CWD-positive areas.
“CWD is transmitted from deer to deer via body fluids such as saliva,
urine, and feces. Because deer feeding and baiting concentrate deer into small
areas, perhaps we will need to address the issue of feeding and baiting in
Arkansas,” he said. “But we really can’t develop a management approach to CWD
until we know prevalence rates and the distribution of the disease in the
state.”
Although various animals, and even humans, host their own specific versions
of a transmissible spongiform encephalopathy (in humans, it is known as
Creutzfeldt-Jacob Disease), the respective diseases aren’t transmissible between
species.
Additionally, humans cannot contract CWD by ingesting mean from an infected
deer.
“People in CWD-positive areas in Colorado, Wyoming, and Wisconsin have
undoubtedly consumed millions of tons of venison over the past few decades,”
White said. “A human has never contracted a TSE by eating deer or elk meat.”
Nevertheless, there are carcass-handling guidelines available for hunters
concerned with coming into contact with animals that may be carrying CWD. Becky
McPeake, professor and extension wildlife specialist for the Division of
Agriculture, is currently finalizing a publication that outlines basic safety
precautions, which include:
Do not harvest animals exhibiting clinical signs of CWD or any other
disease. When processing harvested game, hunters should wear gloved protection,
and avoid contact with nervous system tissues. Don’t consume brain or organ
meats, especially lymph nodes of the head of cervids. Bone out the meat, and
make sure to minimize contact with the brain and spine, as they constitute the
bulk of the nervous system where prions tend to concentrate. Dispose of all
non-consumable parts securely and in a location where other cervids will not
encounter the carcass (such as an approved landfill or buried at a depth of at
least 6’). Cleaning processing equipment in a 50% chlorine bleach solution will
destroy prions, but is very detrimental to most equipment. Standard cleaning
with hot soapy water is typically sufficient, given the limited health risks to
humans. Wipe down processing surfaces with the same solution on a clean cloth.
For more information on Chronic Wasting Disease or other animal health issues,
contact your local Cooperative Extension Service agent or visit www.uaex.edu.
The University of Arkansas System Division of Agriculture offers all its
Extension and Research programs and services without regard to race, color, sex,
gender identity, sexual orientation, national origin, religion, age, disability,
marital or veteran status, genetic information, or any other legally protected
status, and is an Affirmative Action/Equal Opportunity Employer.
# # #
Media Contact: Mary Hightower Dir. of Communication Services U of A
Division of Agriculture Cooperative Extension Service (501) 671-2126
mhightower@uaex.edu
Friday, April 01, 2016
ARKANSAS CHRONIC WASTING DISEASE CWD TSE PRION CASES EXPLODE BY 27 NEW
CASES WITH 50 CASES TOTAL TO DATE
Friday, April 01, 2016
Arkansas confirms six more cases of CWD bringing total to 56 since first
reported 2 months ago
Tuesday, March 29, 2016
Maryland Department of Natural Resources Five Deer Test Positive for
Chronic Wasting Disease ONE OUTSIDE CWD MANAGEMENT ZONE
Friday, March 18, 2016
Michigan confirms additional CWD-positive free-ranging, white-tailed deer,
bringing the total to seven
Wednesday, March 16, 2016
Wisconsin CWD sample survey 2015 confirms 290 cases of Chronic Wasting
Disease TSE Prion
Thursday, March 10, 2016
WYOMING RIDE EM COWBOY HELICOPTER WRANGLING RAMBO STYLE DEER BULLDOGGING
RODEO FOR CWD VIDEO
CHRONIC WASTING DISEASE: The Final Epidemic
Tuesday, March 08, 2016
Oklahoma Chronic Wasting Disease CWD of Deer and Elk Surveillance, Testing,
and Preparedness ???
Wednesday, March 02, 2016
Kansas Chronic Wasting Disease CWD TSE Prion 52 cases 2015 updated report
'ALARMING'
Sunday, March 06, 2016
Missouri 2015-2016 CWD Surveillance Summary to Date, with confirmed cases
mounting
Thursday, March 31, 2016
*** Chronic Wasting Disease CWD TSE Prion Roundup USA April 1, 2016
***
I strenuously once again urge the FDA and its industry constituents, to
make it MANDATORY that all ruminant feed be banned to all ruminants, and this
should include all cervids, as well as non-ruminants such as cats and dogs as
well, as soon as possible for the following reasons...
31 Jan 2015 at 20:14 GMT
*** Ruminant feed ban for cervids in the United States? ***
31 Jan 2015 at 20:14 GMT
see Singeltary comment ;
*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics
of BSE in Canada Singeltary reply ;
Wednesday, July 15, 2015
Additional BSE TSE prion testing detects pathologic lesion in unusual brain
location and PrPsc by PMCA only, how many cases have we missed?
***however in 1 C-type challenged animal, Prion 2015 Poster Abstracts S67
PrPsc was not detected using rapid tests for BSE.
***Subsequent testing resulted in the detection of pathologic lesion in
unusual brain location and PrPsc detection by PMCA only.
*** IBNC Tauopathy or TSE Prion disease, it appears, no one is sure ***
Posted by Terry S. Singeltary Sr. on 03 Jul 2015 at 16:53 GMT
*** PLEASE SEE THIS URGENT UPDATE ON CWD AND FEED ANIMAL PROTEIN ***
Sunday, March 20, 2016
Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer
and Elk in Animal Feed ***UPDATED MARCH 2016*** Singeltary Submission
Evidence That Transmissible Mink Encephalopathy Results from Feeding
Infected Cattle Over the next 8-10 weeks, approximately 40% of all the adult
mink on the farm died from TME.
snip...
The rancher was a ''dead stock'' feeder using mostly (>95%) downer
or dead dairy cattle...
Monday, April 04, 2016
Limited amplification of chronic wasting disease prions in the peripheral
tissues of intracerebrally inoculated cattle
Saturday, February 6, 2016
*** Secretary's Advisory Committee on Animal Health; Meeting [Docket No.
APHIS-2016-0007] Singeltary Submission
RE-What is the risk of chronic wasting disease being introduced into Great
Britain? 6 April 2016
What is the risk of chronic wasting disease being introduced into Great
Britain? A Qualitative Risk Assessment October 2012
I strenuously once again urge the FDA and its industry constituents, to
make it MANDATORY that all ruminant feed be banned to all ruminants, and this
should include all cervids, as well as non-ruminants such as cats and dogs as
well, as soon as possible for the following reasons...
31 Jan 2015 at 20:14 GMT
*** Ruminant feed ban for cervids in the United States? ***
31 Jan 2015 at 20:14 GMT
Terry Singeltary Sr. comment ;
*** PLEASE SEE THIS URGENT UPDATE ON CWD AND FEED ANIMAL PROTEIN ***
Sunday, March 20, 2016
Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer
and Elk in Animal Feed ***UPDATED MARCH 2016*** Singeltary Submission
Monday, March 28, 2016
National Scrapie Eradication Program February 2016 Monthly Report
Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer
and Elk in Animal Feed Singeltary Submission
Greetings again FDA and Mr. Pritchett et al,
I would kindly like to comment on ;
Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer
and Elk in Animal Feed Singeltary Submission
#158
Guidance for Industry
Use of Material from Deer and Elk in Animal Feed
This version of the guidance replaces the version made available
September15, 2003.
This document has been revised to update the docket number, contact
information, and standard disclosures. Submit comments on this guidance at any
time.
Submit electronic comments to http://www.regulations.gov. Submit written
comments to the Division of Dockets Management (HFA-305), Food and Drug
Administration, 5630 Fishers Lane, Rm. 1061, Rockville, MD 20852. All comments
should be identified with the Docket No. FDA-2003-D-0432 (formerly 03D-0186).
For further information regarding this guidance, contact Burt Pritchett,
Center for Veterinary Medicine (HFV-222), Food and Drug Administration, 7519
Standish Place, Rockville, MD 20855, 240-402-6276, E-mail:
burt.pritchett@fda.hhs.gov.
Additional copies of this guidance document may be requested from the
Policy and Regulations Staff (HFV-6), Center for Veterinary Medicine, Food and
Drug Administration, 7519 Standish Place, Rockville, MD 20855, and may be viewed
on the Internet at either http://www.fda.gov/AnimalVeterinary/default.htm
or http://www.regulations.gov.
U.S. Department of Health and Human Services Food and Drug Administration
Center for Veterinary Medicine March 2016
Contains Nonbinding Recommendations
2
Guidance for Industry Use of Material from Deer and Elk in Animal Feed
This guidance represents the current thinking of the Food and Drug
Administration (FDA or Agency) on this topic. It does not establish any rights
for any person and is not binding on FDA or the public. You can use an
alternative approach if it satisfies the requirements of the applicable statutes
and regulations. To discuss an alternative approach, contact the FDA office
responsible for this guidance as listed on the title page.
I. Introduction
Under FDA’s BSE feed regulation (21 CFR 589.2000) most material from deer
and elk is prohibited for use in feed for ruminant animals. This guidance
document describes FDA’s recommendations regarding the use in all animal feed of
all material from deer and elk that are positive for Chronic Wasting Disease
(CWD) or are considered at high risk for CWD. The potential risks from CWD to
humans or non-cervid animals such as poultry and swine are not well understood.
However, because of recent recognition that CWD is spreading rapidly in
white-tailed deer, and because CWD’s route of transmission is poorly understood,
FDA is making recommendations regarding the use in animal feed of rendered
materials from deer and elk that are CWD-positive or that are at high risk for
CWD.
In general, FDA’s guidance documents do not establish legally enforceable
responsibilities. Instead, guidances describe the Agency’s current thinking on a
topic and should be viewed only as recommendations, unless specific regulatory
or statutory requirements are cited. The use of the word should in Agency
guidances means that something is suggested or recommended, but not
required.
II. Background
CWD is a neurological (brain) disease of farmed and wild deer and elk that
belong in the animal family cervidae (cervids). Only deer and elk are known to
be susceptible to CWD by natural transmission. The disease has been found in
farmed and wild mule deer, white-tailed deer, North American elk, and in farmed
black-tailed deer. CWD belongs to a family of animal and human diseases called
transmissible spongiform encephalopathies (TSEs). These include bovine
spongiform encephalopathy (BSE or “mad cow” disease) in cattle; scrapie in sheep
and goats; and classical and variant Creutzfeldt-Jakob diseases (CJD and vCJD)
in humans. There is no known treatment for these diseases, and there is no
vaccine to prevent them. In addition, although validated postmortem diagnostic
tests are available, there are no validated diagnostic tests for CWD that can be
used to test for the disease in live animals.
Contains Nonbinding Recommendations
III. Use in animal feed of material from CWD-positive deer and elk
Material from CWD-positive animals may not be used in any animal feed or
feed ingredients. Pursuant to Sec. 402(a)(5) of the Federal Food, Drug, and
Cosmetic Act, animal feed and feed ingredients containing material from a
CWD-positive animal would be considered adulterated. FDA recommends that any
such adulterated feed or feed ingredients be recalled or otherwise removed from
the marketplace.
IV. Use in animal feed of material from deer and elk considered at high
risk for CWD Deer and elk considered at high risk for CWD include: (1) animals
from areas declared by State officials 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 immediately before the time of slaughter were in a captive herd that
contained a CWD-positive animal.
FDA recommends that materials from deer and elk considered at high risk for
CWD no longer be entered into the animal feed system. Under present
circumstances, FDA is not recommending that feed made from deer and elk from a
non-endemic area be recalled if a State later declares the area endemic for CWD
or a CWD eradication zone. In addition, at this time, FDA is not recommending
that feed made from deer and elk believed to be from a captive herd that
contained no CWD-positive animals be recalled if that herd is subsequently found
to contain a CWD-positive animal.
V. Use in animal feed of material from deer and elk NOT considered at high
risk for CWD FDA continues to consider materials from deer and elk NOT
considered at high risk for CWD to be acceptable for use in NON-RUMINANT animal
feeds in accordance with current agency regulations, 21 CFR 589.2000. Deer and
elk not considered at high risk include: (1) deer and elk from areas not
declared by State officials to be endemic for CWD and/or to be CWD eradication
zones; and (2) deer and elk that were not at some time during the 60-month
period immediately before the time of slaughter in a captive herd that contained
a CWD-positive animal.
3
Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer
and Elk in Animal Feed Singeltary Submission
Greetings again FDA and Mr. Pritchett et al,
MY comments and source reference of sound science on this very important
issue are as follows ;
Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer
and Elk in Animal Feed Singeltary Submission
I kindly wish to once again submit to Docket No. FDA-2003-D-0432 (formerly
03D-0186) Use of Material from Deer and Elk in Animal Feed.
Thank you kindly for allowing me to comment again, ...and again...and
again, on a topic so important, why it is ‘NON-BINDING’ is beyond me.
this should have been finalized and made ‘BINDING’ or MANDATORY OVER A
DECADE AGO.
but here lay the problem, once made ‘BINDING’ or ‘MANDATORY’, it is still
nothing but ink on paper.
we have had a mad cow feed ban in place since August 1997, and since then,
literally 100s of millions of pounds BANNED MAD COW FEED has been sent out to
commerce and fed out (see reference materials).
ENFORCEMENT OF SAID BINDING REGULATIONS HAS FAILED US TOO MANY TIMES.
so, in my opinion, any non-binding or voluntary regulations will not work,
and to state further, ‘BINDING’ or MANDATORY regulations will not work unless
enforced.
with that said, we know that Chronic Wasting Disease CWD TSE Prion easily
transmits to other cervid through the oral route.
the old transmission studies of BSE TSE floored scientist once they figured
out what they had, and please don’t forget about those mink that were fed 95%+
dead stock downer cow, that all came down with TME. please see ;
It is clear that the designing scientists must also have shared Mr Bradleys
surprise at the results because all the dose levels right down to 1 gram
triggered infection.
it is clear that the designing scientists must have also shared Mr Bradleys
surprise at the results because all the dose levels right down to 1 gram
triggered infection.
Evidence That Transmissible Mink Encephalopathy Results from Feeding
Infected Cattle
Over the next 8-10 weeks, approximately 40% of all the adult mink on the
farm died from TME.
snip...
The rancher was a ''dead stock'' feeder using mostly (>95%) downer or
dead dairy cattle...
To further complicate things, we now know that science has shown that
plants and vegetables can uptake the TSE Prion, and that the Scrapie agent can
still be infectious from soil 16 years later. a frightening thought with the CWD
running rampant now in North America (please see source reference materials
below).
IF we don’t not do this, we have failed, and the TSE Prion agent will
continue to spread, as it is doing as we speak.
I strenuously once again urge the FDA and its industry constituents, to
make it MANDATORY that all ruminant feed be banned to all ruminants, and this
should include all cervids, as well as non-ruminants such as cats and dogs as
well, as soon as possible for the following reasons...
31 Jan 2015 at 20:14 GMT
*** Ruminant feed ban for cervids in the United States? ***
31 Jan 2015 at 20:14 GMT
see Singeltary comment ;
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...
36% in 2007 (Almberg et al., 2011). In such areas, population declines of
deer of up to 30 to 50% have been observed (Almberg et al., 2011). In areas of
Colorado, the prevalence can be as high as 30% (EFSA, 2011). The clinical signs
of CWD in affected adults are weight loss and behavioural changes that can span
weeks or months (Williams, 2005). In addition, signs might include excessive
salivation, behavioural alterations including a fixed stare and changes in
interaction with other animals in the herd, and an altered stance (Williams,
2005). These signs are indistinguishable from cervids experimentally infected
with bovine spongiform encephalopathy (BSE). Given this, if CWD was to be
introduced into countries with BSE such as GB, for example, infected deer
populations would need to be tested to differentiate if they were infected with
CWD or BSE to minimise the risk of BSE entering the human food-chain via
affected venison.
snip...
The rate of transmission of CWD has been reported to be as high as 30% and
can approach 100% among captive animals in endemic areas (Safar et al., 2008).
snip...
In summary, in endemic areas, there is a medium probability that the soil
and surrounding environment is contaminated with CWD prions and in a
bioavailable form. In rural areas where CWD has not been reported and deer are
present, there is a greater than negligible risk the soil is contaminated with
CWD prion.
snip...
In summary, given the volume of tourists, hunters and servicemen moving
between GB and North America, the probability of at least one person travelling
to/from a CWD affected area and, in doing so, contaminating their clothing,
footwear and/or equipment prior to arriving in GB is greater than negligible.
For deer hunters, specifically, the risk is likely to be greater given the
increased contact with deer and their environment. However, there is significant
uncertainty associated with these estimates.
snip...
Therefore, it is considered that farmed and park deer may have a higher
probability of exposure to CWD transferred to the environment than wild deer
given the restricted habitat range and higher frequency of contact with tourists
and returning GB residents.
snip...
please see further ;
REFERENCE MATERIALS
*** Docket No. APHIS-2007-0127 Scrapie in Sheep and Goats Terry Singeltary
Sr. Submission ***
Monday, November 16, 2015
*** Docket No. APHIS-2007-0127 Scrapie in Sheep and Goats Terry Singeltary
Sr. Submission ***
Friday, March 18, 2016 CFSAN
Constituent Update: FDA Announces Final Rule on Bovine Spongiform
Encephalopathy BSE MAD COW TSE PRION Center for Food Safety and Applied
Nutrition - Constituent Update
Tuesday, March 15, 2016
Docket No. FDA-2016-N-0321 Risk Assessment of Foodborne Illness Associated
with Pathogens from Produce Grown in Fields Amended with Untreated Biological
Soil Amendments of Animal Origin; Request for Comments, Scientific Data, and
Information Singeltary Submission
Saturday, February 6, 2016
Secretary's Advisory Committee on Animal Health; Meeting [Docket No.
APHIS-2016-0007] Singeltary Submission
Friday, August 14, 2015
*** Susceptibility of cattle to the agent of chronic wasting disease from
elk after intracranial inoculation
PRION 2015 CONFERENCE FT. COLLINS CWD RISK FACTORS TO HUMANS
*** LATE-BREAKING ABSTRACTS PRION 2015 CONFERENCE ***
O18
Zoonotic Potential of CWD Prions
Liuting Qing1, Ignazio Cali1,2, Jue Yuan1, Shenghai Huang3, Diane Kofskey1,
Pierluigi Gambetti1, Wenquan Zou1, Qingzhong Kong1 1Case Western Reserve
University, Cleveland, Ohio, USA, 2Second University of Naples, Naples, Italy,
3Encore Health Resources, Houston, Texas, USA
*** These results indicate that the CWD prion has the potential to infect
human CNS and peripheral lymphoid tissues and that there might be asymptomatic
human carriers of CWD infection.
==================
***These results indicate that the CWD prion has the potential to infect
human CNS and peripheral lymphoid tissues and that there might be asymptomatic
human carriers of CWD infection.***
==================
P.105: RT-QuIC models trans-species prion transmission
Kristen Davenport, Davin Henderson, Candace Mathiason, and Edward Hoover
Prion Research Center; Colorado State University; Fort Collins, CO USA
Conversely, FSE maintained sufficient BSE characteristics to more
efficiently convert bovine rPrP than feline rPrP. Additionally, human rPrP was
competent for conversion by CWD and fCWD.
***This insinuates that, at the level of protein:protein interactions, the
barrier preventing transmission of CWD to humans is less robust than previously
estimated.
================
***This insinuates that, at the level of protein:protein interactions, the
barrier preventing transmission of CWD to humans is less robust than previously
estimated.***
================
*** PRICE OF CWD TSE PRION POKER GOES UP 2014 ***
Transmissible Spongiform Encephalopathy TSE PRION update January 2, 2014
*** 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.
*** 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).***
*** 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.
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 is so rare in people under age 30, one case in a billion (leaving out
medical mishaps), that four cases under 30 is "very high," says Colorado
neurologist Bosque. "Then, if you add these other two from Wisconsin [cases in
the newspaper], six cases of CJD in people associated with venison is very, very
high." Only now, with Mary Riley, there are at least seven, and possibly eight,
with Steve, her dining companion. "It's not critical mass that matters,"
however, Belay says. "One case would do it for me." The chance that two people
who know each other would both contact CJD, like the two Wisconsin sportsmen, is
so unlikely, experts say, it would happen only once in 140 years.
Given the incubation period for TSEs in humans, it may require another
generation to write the final chapter on CWD in Wisconsin. "Does chronic wasting
disease pass into humans? We'll be able to answer that in 2022," says Race.
Meanwhile, the state has become part of an immense experiment.
I urge everyone to watch this video closely...terry
*** you can see video here and interview with Jeff's Mom, and scientist
telling you to test everything and potential risk factors for humans ***
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.
Prions in Skeletal Muscles of Deer with Chronic Wasting Disease Rachel C.
Angers1,*, Shawn R. Browning1,*,†, Tanya S. Seward2, Christina J. Sigurdson4,‡,
Michael W. Miller5, Edward A. Hoover4, Glenn C. Telling1,2,3,§ snip...
Abstract The emergence of chronic wasting disease (CWD) in deer and elk in
an increasingly wide geographic area, as well as the interspecies transmission
of bovine spongiform encephalopathy to humans in the form of variant Creutzfeldt
Jakob disease, have raised concerns about the zoonotic potential of CWD. Because
meat consumption is the most likely means of exposure, it is important to
determine whether skeletal muscle of diseased cervids contains prion
infectivity. Here bioassays in transgenic mice expressing cervid prion protein
revealed the presence of infectious prions in skeletal muscles of CWD-infected
deer, demonstrating that humans consuming or handling meat from CWD-infected
deer are at risk to prion exposure.
***********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 ;
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.
http://web.archive.org/web/20030511010117/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf
*** 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).***
O.05: Transmission of prions to primates after extended silent incubation
periods: Implications for BSE and scrapie risk assessment in human populations
Emmanuel Comoy, Jacqueline Mikol, Valerie Durand, Sophie Luccantoni,
Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys
Atomic Energy Commission; Fontenay-aux-Roses, France
Prion diseases (PD) are the unique neurodegenerative proteinopathies
reputed to be transmissible under field conditions since decades. The
transmission of Bovine Spongiform Encephalopathy (BSE) to humans evidenced that
an animal PD might be zoonotic under appropriate conditions. Contrarily, in the
absence of obvious (epidemiological or experimental) elements supporting a
transmission or genetic predispositions, PD, like the other proteinopathies, are
reputed to occur spontaneously (atpical animal prion strains, sporadic CJD
summing 80% of human prion cases). Non-human primate models provided the first
evidences supporting the transmissibiity of human prion strains and the zoonotic
potential of BSE. Among them, cynomolgus macaques brought major information for
BSE risk assessment for human health (Chen, 2014), according to their
phylogenetic proximity to humans and extended lifetime. We used this model to
assess the zoonotic potential of other animal PD from bovine, ovine and cervid
origins even after very long silent incubation periods.
*** We recently observed the direct transmission of a natural classical
scrapie isolate to macaque after a 10-year silent incubation period,
***with features similar to some reported for human cases of sporadic CJD,
albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked
in humanized mice (Cassard, 2014),
***is the third potentially zoonotic PD (with BSE and L-type BSE),
***thus questioning the origin of human sporadic cases. We will present an
updated panorama of our different transmission studies and discuss the
implications of such extended incubation periods on risk assessment of animal PD
for human health.
===============
***thus questioning the origin of human sporadic cases***
===============
***our findings suggest that possible transmission risk of H-type BSE to
sheep and human. Bioassay will be required to determine whether the PMCA
products are infectious to these animals.
==============
Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF
TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES
Title: Transmission of scrapie prions to primate after an extended silent
incubation period
Authors
item Comoy, Emmanuel - item Mikol, Jacqueline - item Luccantoni-Freire,
Sophie - item Correia, Evelyne - item Lescoutra-Etchegaray, Nathalie - item
Durand, Valérie - item Dehen, Capucine - item Andreoletti, Olivier - item
Casalone, Cristina - item Richt, Juergen item Greenlee, Justin item Baron,
Thierry - item Benestad, Sylvie - item Hills, Bob - item Brown, Paul - item
Deslys, Jean-Philippe -
Submitted to: Scientific Reports Publication Type: Peer Reviewed Journal
Publication Acceptance Date: May 28, 2015 Publication Date: June 30, 2015
Citation: Comoy, E.E., Mikol, J., Luccantoni-Freire, S., Correia, E.,
Lescoutra-Etchegaray, N., Durand, V., Dehen, C., Andreoletti, O., Casalone, C.,
Richt, J.A., Greenlee, J.J., Baron, T., Benestad, S., Brown, P., Deslys, J.
2015. Transmission of scrapie prions to primate after an extended silent
incubation period. Scientific Reports. 5:11573.
Interpretive Summary: The transmissible spongiform encephalopathies (also
called prion diseases) are fatal neurodegenerative diseases that affect animals
and humans. The agent of prion diseases is a misfolded form of the prion protein
that is resistant to breakdown by the host cells. Since all mammals express
prion protein on the surface of various cells such as neurons, all mammals are,
in theory, capable of replicating prion diseases. One example of a prion
disease, bovine spongiform encephalopathy (BSE; also called mad cow disease),
has been shown to infect cattle, sheep, exotic undulates, cats, non-human
primates, and humans when the new host is exposed to feeds or foods contaminated
with the disease agent. The purpose of this study was to test whether non-human
primates (cynomologous macaque) are susceptible to the agent of sheep scrapie.
After an incubation period of approximately 10 years a macaque developed
progressive clinical signs suggestive of neurologic disease. Upon postmortem
examination and microscopic examination of tissues, there was a widespread
distribution of lesions consistent with a transmissible spongiform
encephalopathy. This information will have a scientific impact since it is the
first study that demonstrates the transmission of scrapie to a non-human primate
with a close genetic relationship to humans. This information is especially
useful to regulatory officials and those involved with risk assessment of the
potential transmission of animal prion diseases to humans. Technical Abstract:
Classical bovine spongiform encephalopathy (c-BSE) is an animal prion disease
that also causes variant Creutzfeldt-Jakob disease in humans. Over the past
decades, c-BSE's zoonotic potential has been the driving force in establishing
extensive protective measures for animal and human health.
*** In complement to the recent demonstration that humanized mice are
susceptible to scrapie, we report here the first observation of direct
transmission of a natural classical scrapie isolate to a macaque after a 10-year
incubation period. Neuropathologic examination revealed all of the features of a
prion disease: spongiform change, neuronal loss, and accumulation of PrPres
throughout the CNS.
*** This observation strengthens the questioning of the harmlessness of
scrapie to humans, at a time when protective measures for human and animal
health are being dismantled and reduced as c-BSE is considered controlled and
being eradicated.
*** Our results underscore the importance of precautionary and protective
measures and the necessity for long-term experimental transmission studies to
assess the zoonotic potential of other animal prion strains.
***This information will have a scientific impact since it is the first
study that demonstrates the transmission of scrapie to a non-human primate with
a close genetic relationship to humans. This information is especially useful to
regulatory officials and those involved with risk assessment of the potential
transmission of animal prion diseases to humans.
***This observation strengthens the questioning of the harmlessness of
scrapie to humans, at a time when protective measures for human and animal
health are being dismantled and reduced as c-BSE is considered controlled and
being eradicated. Our results underscore the importance of precautionary and
protective measures and the necessity for long-term experimental transmission
studies to assess the zoonotic potential of other animal prion strains.
why do we not want to do TSE transmission studies on chimpanzees $
5. A positive result from a chimpanzee challenged severly would likely
create alarm in some circles even if the result could not be interpreted for
man. I have a view that all these agents could be transmitted provided a large
enough dose by appropriate routes was given and the animals kept long enough.
Until the mechanisms of the species barrier are more clearly understood it might
be best to retain that hypothesis.
snip...
R. BRADLEY
”The occurrence of CWD must be viewed against the contest of the locations
in which it occurred. It was an incidental and unwelcome complication of the
respective wildlife research programmes. Despite it’s subsequent recognition as
a new disease of cervids, therefore justifying direct investigation, no specific
research funding was forthcoming. The USDA veiwed it as a wildlife problem and
consequently not their province!” page 26.
In Confidence - Perceptions of unconventional slow virus diseases of
animals in the USA - APRIL-MAY 1989 - G A H Wells
3. Prof. A. Robertson gave a brief account of BSE. The US approach was to
accord it a very low profile indeed. Dr. A Thiermann showed the picture in the
''Independent'' with cattle being incinerated and thought this was a fanatical
incident to be avoided in the US at all costs. ...
MAD COW DISEASE HAS BEEN IN THE USA FOR DECADES, AND I BELIEVE IT WAS IN
THE USA FIRST, PLEASE SEE ;
Evidence That Transmissible Mink Encephalopathy Results from Feeding
Infected Cattle Over the next 8-10 weeks, approximately 40% of all the adult
mink on the farm died from TME.
snip...
The rancher was a ''dead stock'' feeder using mostly (>95%) downer
or dead dairy cattle...
PL1
Using in vitro prion replication for high sensitive detection of prions and
prionlike proteins and for understanding mechanisms of transmission.
Claudio Soto
Mitchell Center for Alzheimer's diseases and related Brain disorders,
Department of Neurology, University of Texas Medical School at Houston.
Prion and prion-like proteins are misfolded protein aggregates with the
ability to selfpropagate to spread disease between cells, organs and in some
cases across individuals. I n T r a n s m i s s i b l e s p o n g i f o r m
encephalopathies (TSEs), prions are mostly composed by a misfolded form of the
prion protein (PrPSc), which propagates by transmitting its misfolding to the
normal prion protein (PrPC). The availability of a procedure to replicate prions
in the laboratory may be important to study the mechanism of prion and
prion-like spreading and to develop high sensitive detection of small quantities
of misfolded proteins in biological fluids, tissues and environmental samples.
Protein Misfolding Cyclic Amplification (PMCA) is a simple, fast and efficient
methodology to mimic prion replication in the test tube. PMCA is a platform
technology that may enable amplification of any prion-like misfolded protein
aggregating through a seeding/nucleation process. In TSEs, PMCA is able to
detect the equivalent of one single molecule of infectious PrPSc and propagate
prions that maintain high infectivity, strain properties and species
specificity. Using PMCA we have been able to detect PrPSc in blood and urine of
experimentally infected animals and humans affected by vCJD with high
sensitivity and specificity. Recently, we have expanded the principles of PMCA
to amplify amyloid-beta (Aβ) and alphasynuclein (α-syn) aggregates implicated in
Alzheimer's and Parkinson's diseases, respectively. Experiments are ongoing to
study the utility of this technology to detect Aβ and α-syn aggregates in
samples of CSF and blood from patients affected by these diseases.
=========================
***Recently, we have been using PMCA to study the role of environmental
prion contamination on the horizontal spreading of TSEs. These experiments have
focused on the study of the interaction of prions with plants and
environmentally relevant surfaces. Our results show that plants (both leaves and
roots) bind tightly to prions present in brain extracts and excreta (urine and
feces) and retain even small quantities of PrPSc for long periods of time.
Strikingly, ingestion of prioncontaminated leaves and roots produced disease
with a 100% attack rate and an incubation period not substantially longer than
feeding animals directly with scrapie brain homogenate. Furthermore, plants can
uptake prions from contaminated soil and transport them to different parts of
the plant tissue (stem and leaves). Similarly, prions bind tightly to a variety
of environmentally relevant surfaces, including stones, wood, metals, plastic,
glass, cement, etc. Prion contaminated surfaces efficiently transmit prion
disease when these materials were directly injected into the brain of animals
and strikingly when the contaminated surfaces were just placed in the animal
cage. These findings demonstrate that environmental materials can efficiently
bind infectious prions and act as carriers of infectivity, suggesting that they
may play an important role in the horizontal transmission of the disease.
========================
Since its invention 13 years ago, PMCA has helped to answer fundamental
questions of prion propagation and has broad applications in research areas
including the food industry, blood bank safety and human and veterinary disease
diagnosis.
see ;
Wednesday, December 16, 2015
Objects in contact with classical scrapie sheep act as a reservoir for
scrapie transmission
Objects in contact with classical scrapie sheep act as a reservoir for
scrapie transmission
Timm Konold1*, Stephen A. C. Hawkins2, Lisa C. Thurston3, Ben C. Maddison4,
Kevin C. Gough5, Anthony Duarte1 and Hugh A. Simmons1
1 Animal Sciences Unit, Animal and Plant Health Agency Weybridge,
Addlestone, UK, 2 Pathology Department, Animal and Plant Health Agency
Weybridge, Addlestone, UK, 3 Surveillance and Laboratory Services, Animal and
Plant Health Agency Penrith, Penrith, UK, 4 ADAS UK, School of Veterinary
Medicine and Science, University of Nottingham, Sutton Bonington, UK, 5 School
of Veterinary Medicine and Science, University of Nottingham, Sutton Bonington,
UK
Classical scrapie is an environmentally transmissible prion disease of
sheep and goats. Prions can persist and remain potentially infectious in the
environment for many years and thus pose a risk of infecting animals after
re-stocking. In vitro studies using serial protein misfolding cyclic
amplification (sPMCA) have suggested that objects on a scrapie affected sheep
farm could contribute to disease transmission. This in vivo study aimed to
determine the role of field furniture (water troughs, feeding troughs, fencing,
and other objects that sheep may rub against) used by a scrapie-infected sheep
flock as a vector for disease transmission to scrapie-free lambs with the prion
protein genotype VRQ/VRQ, which is associated with high susceptibility to
classical scrapie. When the field furniture was placed in clean accommodation,
sheep became infected when exposed to either a water trough (four out of five)
or to objects used for rubbing (four out of seven). This field furniture had
been used by the scrapie-infected flock 8 weeks earlier and had previously been
shown to harbor scrapie prions by sPMCA. Sheep also became infected (20 out of
23) through exposure to contaminated field furniture placed within pasture not
used by scrapie-infected sheep for 40 months, even though swabs from this
furniture tested negative by PMCA. This infection rate decreased (1 out of 12)
on the same paddock after replacement with clean field furniture. Twelve grazing
sheep exposed to field furniture not in contact with scrapie-infected sheep for
18 months remained scrapie free. The findings of this study highlight the role
of field furniture used by scrapie-infected sheep to act as a reservoir for
disease re-introduction although infectivity declines considerably if the field
furniture has not been in contact with scrapie-infected sheep for several
months. PMCA may not be as sensitive as VRQ/VRQ sheep to test for environmental
contamination.
snip...
Discussion
Classical scrapie is an environmentally transmissible disease because it
has been reported in naïve, supposedly previously unexposed sheep placed in
pastures formerly occupied by scrapie-infected sheep (4, 19, 20). Although the
vector for disease transmission is not known, soil is likely to be an important
reservoir for prions (2) where – based on studies in rodents – prions can adhere
to minerals as a biologically active form (21) and remain infectious for more
than 2 years (22). Similarly, chronic wasting disease (CWD) has re-occurred in
mule deer housed in paddocks used by infected deer 2 years earlier, which was
assumed to be through foraging and soil consumption (23).
Our study suggested that the risk of acquiring scrapie infection was
greater through exposure to contaminated wooden, plastic, and metal surfaces via
water or food troughs, fencing, and hurdles than through grazing. Drinking from
a water trough used by the scrapie flock was sufficient to cause infection in
sheep in a clean building. Exposure to fences and other objects used for rubbing
also led to infection, which supported the hypothesis that skin may be a vector
for disease transmission (9). The risk of these objects to cause infection was
further demonstrated when 87% of 23 sheep presented with PrPSc in lymphoid
tissue after grazing on one of the paddocks, which contained metal hurdles, a
metal lamb creep and a water trough in contact with the scrapie flock up to 8
weeks earlier, whereas no infection had been demonstrated previously in sheep
grazing on this paddock, when equipped with new fencing and field furniture.
When the contaminated furniture and fencing were removed, the infection rate
dropped significantly to 8% of 12 sheep, with soil of the paddock as the most
likely source of infection caused by shedding of prions from the
scrapie-infected sheep in this paddock up to a week earlier.
This study also indicated that the level of contamination of field
furniture sufficient to cause infection was dependent on two factors: stage of
incubation period and time of last use by scrapie-infected sheep. Drinking from
a water trough that had been used by scrapie sheep in the predominantly
pre-clinical phase did not appear to cause infection, whereas infection was
shown in sheep drinking from the water trough used by scrapie sheep in the later
stage of the disease. It is possible that contamination occurred through
shedding of prions in saliva, which may have contaminated the surface of the
water trough and subsequently the water when it was refilled. Contamination
appeared to be sufficient to cause infection only if the trough was in contact
with sheep that included clinical cases. Indeed, there is an increased risk of
bodily fluid infectivity with disease progression in scrapie (24) and CWD (25)
based on PrPSc detection by sPMCA. Although ultraviolet light and heat under
natural conditions do not inactivate prions (26), furniture in contact with the
scrapie flock, which was assumed to be sufficiently contaminated to cause
infection, did not act as vector for disease if not used for 18 months, which
suggest that the weathering process alone was sufficient to inactivate prions.
PrPSc detection by sPMCA is increasingly used as a surrogate for
infectivity measurements by bioassay in sheep or mice. In this reported study,
however, the levels of PrPSc present in the environment were below the limit of
detection of the sPMCA method, yet were still sufficient to cause infection of
in-contact animals. In the present study, the outdoor objects were removed from
the infected flock 8 weeks prior to sampling and were positive by sPMCA at very
low levels (2 out of 37 reactions). As this sPMCA assay also yielded 2 positive
reactions out of 139 in samples from the scrapie-free farm, the sPMCA assay
could not detect PrPSc on any of the objects above the background of the assay.
False positive reactions with sPMCA at a low frequency associated with de novo
formation of infectious prions have been reported (27, 28). This is in contrast
to our previous study where we demonstrated that outdoor objects that had been
in contact with the scrapie-infected flock up to 20 days prior to sampling
harbored PrPSc that was detectable by sPMCA analysis [4 out of 15 reactions
(12)] and was significantly more positive by the assay compared to analogous
samples from the scrapie-free farm. This discrepancy could be due to the use of
a different sPMCA substrate between the studies that may alter the efficiency of
amplification of the environmental PrPSc. In addition, the present study had a
longer timeframe between the objects being in contact with the infected flock
and sampling, which may affect the levels of extractable PrPSc. Alternatively,
there may be potentially patchy contamination of this furniture with PrPSc,
which may have been missed by swabbing. The failure of sPMCA to detect
CWD-associated PrP in saliva from clinically affected deer despite confirmation
of infectivity in saliva-inoculated transgenic mice was associated with as yet
unidentified inhibitors in saliva (29), and it is possible that the sensitivity
of sPMCA is affected by other substances in the tested material. In addition,
sampling of amplifiable PrPSc and subsequent detection by sPMCA may be more
difficult from furniture exposed to weather, which is supported by the
observation that PrPSc was detected by sPMCA more frequently in indoor than
outdoor furniture (12). A recent experimental study has demonstrated that
repeated cycles of drying and wetting of prion-contaminated soil, equivalent to
what is expected under natural weathering conditions, could reduce PMCA
amplification efficiency and extend the incubation period in hamsters inoculated
with soil samples (30). This seems to apply also to this study even though the
reduction in infectivity was more dramatic in the sPMCA assays than in the sheep
model. Sheep were not kept until clinical end-point, which would have enabled us
to compare incubation periods, but the lack of infection in sheep exposed to
furniture that had not been in contact with scrapie sheep for a longer time
period supports the hypothesis that prion degradation and subsequent loss of
infectivity occurs even under natural conditions.
In conclusion, the results in the current study indicate that removal of
furniture that had been in contact with scrapie-infected animals should be
recommended, particularly since cleaning and decontamination may not effectively
remove scrapie infectivity (31), even though infectivity declines considerably
if the pasture and the field furniture have not been in contact with
scrapie-infected sheep for several months. As sPMCA failed to detect PrPSc in
furniture that was subjected to weathering, even though exposure led to
infection in sheep, this method may not always be reliable in predicting the
risk of scrapie infection through environmental contamination. These results
suggest that the VRQ/VRQ sheep model may be more sensitive than sPMCA for the
detection of environmentally associated scrapie, and suggest that extremely low
levels of scrapie contamination are able to cause infection in susceptible sheep
genotypes.
Keywords: classical scrapie, prion, transmissible spongiform
encephalopathy, sheep, field furniture, reservoir, serial protein misfolding
cyclic amplification
Wednesday, December 16, 2015
*** Objects in contact with classical scrapie sheep act as a reservoir for
scrapie transmission ***
Circulation of prions within dust on a scrapie affected farm
Kevin C Gough1, Claire A Baker2, Hugh A Simmons3, Steve A Hawkins3 and Ben
C Maddison2*
Abstract
Prion diseases are fatal neurological disorders that affect humans and
animals. Scrapie of sheep/goats and Chronic Wasting Disease (CWD) of deer/elk
are contagious prion diseases where environmental reservoirs have a direct link
to the transmission of disease. Using protein misfolding cyclic amplification we
demonstrate that scrapie PrPSc can be detected within circulating dusts that are
present on a farm that is naturally contaminated with sheep scrapie. The
presence of infectious scrapie within airborne dusts may represent a possible
route of infection and illustrates the difficulties that may be associated with
the effective decontamination of such scrapie affected premises.
snip...
Discussion
We present biochemical data illustrating the airborne movement of scrapie
containing material within a contaminated farm environment. We were able to
detect scrapie PrPSc within extracts from dusts collected over a 70 day period,
in the absence of any sheep activity. We were also able to detect scrapie PrPSc
within dusts collected within pasture at 30 m but not at 60 m distance away from
the scrapie contaminated buildings, suggesting that the chance of contamination
of pasture by scrapie contaminated dusts decreases with distance from
contaminated farm buildings. PrPSc amplification by sPMCA has been shown to
correlate with infectivity and amplified products have been shown to be
infectious [14,15]. These experiments illustrate the potential for low dose
scrapie infectivity to be present within such samples. We estimate low ng levels
of scrapie positive brain equivalent were deposited per m2 over 70 days, in a
barn previously occupied by sheep affected with scrapie. This movement of dusts
and the accumulation of low levels of scrapie infectivity within this
environment may in part explain previous observations where despite stringent
pen decontamination regimens healthy lambs still became scrapie infected after
apparent exposure from their environment alone [16]. The presence of sPMCA
seeding activity and by inference, infectious prions within dusts, and their
potential for airborne dissemination is highly novel and may have implications
for the spread of scrapie within infected premises. The low level circulation
and accumulation of scrapie prion containing dust material within the farm
environment will likely impede the efficient decontamination of such scrapie
contaminated buildings unless all possible reservoirs of dust are removed.
Scrapie containing dusts could possibly infect animals during feeding and
drinking, and respiratory and conjunctival routes may also be involved. It has
been demonstrated that scrapie can be efficiently transmitted via the nasal
route in sheep [17], as is also the case for CWD in both murine models and in
white tailed deer [18-20].
The sources of dust borne prions are unknown but it seems reasonable to
assume that faecal, urine, skin, parturient material and saliva-derived prions
may contribute to this mobile environmental reservoir of infectivity. This work
highlights a possible transmission route for scrapie within the farm
environment, and this is likely to be paralleled in CWD which shows strong
similarities with scrapie in terms of prion dissemination and disease
transmission. The data indicate that the presence of scrapie prions in dust is
likely to make the control of these diseases a considerable challenge.
Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF
TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES
Title: Scrapie transmits to white-tailed deer by the oral route and has a
molecular profile similar to chronic wasting disease
Authors
item Greenlee, Justin item Moore, S - item Smith, Jodi - item Kunkle,
Robert item West Greenlee, M -
Submitted to: American College of Veterinary Pathologists Meeting
Publication Type: Abstract Only Publication Acceptance Date: August 12, 2015
Publication Date: N/A Technical Abstract: The purpose of this work was to
determine susceptibility of white-tailed deer (WTD) to the agent of sheep
scrapie and to compare the resultant PrPSc to that of the original inoculum and
chronic wasting disease (CWD). We inoculated WTD by a natural route of exposure
(concurrent oral and intranasal (IN); n=5) with a US scrapie isolate. All
scrapie-inoculated deer had evidence of PrPSc accumulation. PrPSc was detected
in lymphoid tissues at preclinical time points, and deer necropsied after 28
months post-inoculation had clinical signs, spongiform encephalopathy, and
widespread distribution of PrPSc in neural and lymphoid tissues. Western
blotting (WB) revealed PrPSc with 2 distinct molecular profiles. WB on cerebral
cortex had a profile similar to the original scrapie inoculum, whereas WB of
brainstem, cerebellum, or lymph nodes revealed PrPSc with a higher profile
resembling CWD. Homogenates with the 2 distinct profiles from WTD with clinical
scrapie were further passaged to mice expressing cervid prion protein and
intranasally to sheep and WTD. In cervidized mice, the two inocula have distinct
incubation times. Sheep inoculated intranasally with WTD derived scrapie
developed disease, but only after inoculation with the inoculum that had a
scrapie-like profile. The WTD study is ongoing, but deer in both inoculation
groups are positive for PrPSc by rectal mucosal biopsy. In summary, this work
demonstrates that WTD are susceptible to the agent of scrapie, two distinct
molecular profiles of PrPSc are present in the tissues of affected deer, and
inoculum of either profile readily passes to deer.
White-tailed Deer are Susceptible to Scrapie by Natural Route of Infection
Jodi D. Smith, Justin J. Greenlee, and Robert A. Kunkle; Virus and Prion
Research Unit, National Animal Disease Center, USDA-ARS
Interspecies transmission studies afford the opportunity to better
understand the potential host range and origins of prion diseases. Previous
experiments demonstrated that white-tailed deer are susceptible to sheep-derived
scrapie by intracranial inoculation. The purpose of this study was to determine
susceptibility of white-tailed deer to scrapie after a natural route of
exposure. Deer (n=5) were inoculated by concurrent oral (30 ml) and intranasal
(1 ml) instillation of a 10% (wt/vol) brain homogenate derived from a sheep
clinically affected with scrapie. Non-inoculated deer were maintained as
negative controls. All deer were observed daily for clinical signs. Deer were
euthanized and necropsied when neurologic disease was evident, and tissues were
examined for abnormal prion protein (PrPSc) by immunohistochemistry (IHC) and
western blot (WB). One animal was euthanized 15 months post-inoculation (MPI)
due to an injury. At that time, examination of obex and lymphoid tissues by IHC
was positive, but WB of obex and colliculus were negative. Remaining deer
developed clinical signs of wasting and mental depression and were necropsied
from 28 to 33 MPI. Tissues from these deer were positive for scrapie by IHC and
WB. Tissues with PrPSc immunoreactivity included brain, tonsil, retropharyngeal
and mesenteric lymph nodes, hemal node, Peyer’s patches, and spleen. This work
demonstrates for the first time that white-tailed deer are susceptible to sheep
scrapie by potential natural routes of inoculation. In-depth analysis of tissues
will be done to determine similarities between scrapie in deer after
intracranial and oral/intranasal inoculation and chronic wasting disease
resulting from similar routes of inoculation.
see full text ;
PO-039: A comparison of scrapie and chronic wasting disease in white-tailed
deer
Justin Greenlee, Jodi Smith, Eric Nicholson US Dept. Agriculture;
Agricultural Research Service, National Animal Disease Center; Ames, IA USA
White-tailed deer are susceptible to the agent of sheep scrapie by
intracerebral inoculation
snip...
It is unlikely that CWD will be eradicated from free-ranging cervids, and
the disease is likely to continue to spread geographically [10]. However, the
potential that white-tailed deer may be susceptible to sheep scrapie by a
natural route presents an additional confounding factor to halting the spread of
CWD. This leads to the additional speculations that
1) infected deer could serve as a reservoir to infect sheep with scrapie
offering challenges to scrapie eradication efforts and
2) CWD spread need not remain geographically confined to current endemic
areas, but could occur anywhere that sheep with scrapie and susceptible cervids
cohabitate.
This work demonstrates for the first time that white-tailed deer are
susceptible to sheep scrapie by intracerebral inoculation with a high attack
rate and that the disease that results has similarities to CWD. These
experiments will be repeated with a more natural route of inoculation to
determine the likelihood of the potential transmission of sheep scrapie to
white-tailed deer. If scrapie were to occur in white-tailed deer, results of
this study indicate that it would be detected as a TSE, but may be difficult to
differentiate from CWD without in-depth biochemical analysis.
2012
PO-039: A comparison of scrapie and chronic wasting disease in white-tailed
deer
Justin Greenlee, Jodi Smith, Eric Nicholson US Dept. Agriculture;
Agricultural Research Service, National Animal Disease Center; Ames, IA USA
snip...
The results of this study suggest that there are many similarities in the
manifestation of CWD and scrapie in WTD after IC inoculation including early and
widespread presence of PrPSc in lymphoid tissues, clinical signs of depression
and weight loss progressing to wasting, and an incubation time of 21-23 months.
Moreover, western blots (WB) done on brain material from the obex region have a
molecular profile similar to CWD and distinct from tissues of the cerebrum or
the scrapie inoculum. However, results of microscopic and IHC examination
indicate that there are differences between the lesions expected in CWD and
those that occur in deer with scrapie: amyloid plaques were not noted in any
sections of brain examined from these deer and the pattern of immunoreactivity
by IHC was diffuse rather than plaque-like.
*** After a natural route of exposure, 100% of WTD were susceptible to
scrapie.
Deer developed clinical signs of wasting and mental depression and were
necropsied from 28 to 33 months PI. Tissues from these deer were positive for
PrPSc by IHC and WB. Similar to IC inoculated deer, samples from these deer
exhibited two different molecular profiles: samples from obex resembled CWD
whereas those from cerebrum were similar to the original scrapie inoculum. On
further examination by WB using a panel of antibodies, the tissues from deer
with scrapie exhibit properties differing from tissues either from sheep with
scrapie or WTD with CWD. Samples from WTD with CWD or sheep with scrapie are
strongly immunoreactive when probed with mAb P4, however, samples from WTD with
scrapie are only weakly immunoreactive. In contrast, when probed with mAb’s 6H4
or SAF 84, samples from sheep with scrapie and WTD with CWD are weakly
immunoreactive and samples from WTD with scrapie are strongly positive. This
work demonstrates that WTD are highly susceptible to sheep scrapie, but on first
passage, scrapie in WTD is differentiable from CWD.
2011
*** After a natural route of exposure, 100% of white-tailed deer were
susceptible to scrapie.
White-tailed Deer are Susceptible to Scrapie by Natural Route of Infection
Jodi D. Smith, Justin J. Greenlee, and Robert A. Kunkle; Virus and Prion
Research Unit, National Animal Disease Center, USDA-ARS
Interspecies transmission studies afford the opportunity to better
understand the potential host range and origins of prion diseases. Previous
experiments demonstrated that white-tailed deer are susceptible to sheep-derived
scrapie by intracranial inoculation. The purpose of this study was to determine
susceptibility of white-tailed deer to scrapie after a natural route of
exposure. Deer (n=5) were inoculated by concurrent oral (30 ml) and intranasal
(1 ml) instillation of a 10% (wt/vol) brain homogenate derived from a sheep
clinically affected with scrapie. Non-inoculated deer were maintained as
negative controls. All deer were observed daily for clinical signs. Deer were
euthanized and necropsied when neurologic disease was evident, and tissues were
examined for abnormal prion protein (PrPSc) by immunohistochemistry (IHC) and
western blot (WB). One animal was euthanized 15 months post-inoculation (MPI)
due to an injury. At that time, examination of obex and lymphoid tissues by IHC
was positive, but WB of obex and colliculus were negative. Remaining deer
developed clinical signs of wasting and mental depression and were necropsied
from 28 to 33 MPI. Tissues from these deer were positive for scrapie by IHC and
WB. Tissues with PrPSc immunoreactivity included brain, tonsil, retropharyngeal
and mesenteric lymph nodes, hemal node, Peyer’s patches, and spleen. This work
demonstrates for the first time that white-tailed deer are susceptible to sheep
scrapie by potential natural routes of inoculation. In-depth analysis of tissues
will be done to determine similarities between scrapie in deer after
intracranial and oral/intranasal inoculation and chronic wasting disease
resulting from similar routes of inoculation.
see full text ;
Monday, November 3, 2014
Persistence of ovine scrapie infectivity in a farm environment following
cleaning and decontamination
PPo3-22:
Detection of Environmentally Associated PrPSc on a Farm with Endemic
Scrapie
Ben C. Maddison,1 Claire A. Baker,1 Helen C. Rees,1 Linda A. Terry,2 Leigh
Thorne,2 Susan J. Belworthy2 and Kevin C. Gough3 1ADAS-UK LTD; Department of
Biology; University of Leicester; Leicester, UK; 2Veterinary Laboratories
Agency; Surry, KT UK; 3Department of Veterinary Medicine and Science; University
of Nottingham; Sutton Bonington, Loughborough UK
Key words: scrapie, evironmental persistence, sPMCA
Ovine scrapie shows considerable horizontal transmission, yet the routes of
transmission and specifically the role of fomites in transmission remain poorly
defined. Here we present biochemical data demonstrating that on a
scrapie-affected sheep farm, scrapie prion contamination is widespread. It was
anticipated at the outset that if prions contaminate the environment that they
would be there at extremely low levels, as such the most sensitive method
available for the detection of PrPSc, serial Protein Misfolding Cyclic
Amplification (sPMCA), was used in this study. We investigated the distribution
of environmental scrapie prions by applying ovine sPMCA to samples taken from a
range of surfaces that were accessible to animals and could be collected by use
of a wetted foam swab. Prion was amplified by sPMCA from a number of these
environmental swab samples including those taken from metal, plastic and wooden
surfaces, both in the indoor and outdoor environment. At the time of sampling
there had been no sheep contact with these areas for at least 20 days prior to
sampling indicating that prions persist for at least this duration in the
environment. These data implicate inanimate objects as environmental reservoirs
of prion infectivity which are likely to contribute to disease transmission.
Veterinary Pathology Onlinevet.sagepub.com Published online before print
February 27, 2014, doi: 10.1177/0300985814524798 Veterinary Pathology February
27, 2014 0300985814524798
Lesion Profiling and Subcellular Prion Localization of Cervid Chronic
Wasting Disease in Domestic Cats
D. M. Seelig1⇑ A. V. Nalls1 M. Flasik2 V. Frank1 S. Eaton2 C. K. Mathiason1
E. A. Hoover1 1Department of Microbiology, Immunology, and Pathology, Colorado
State University, Fort Collins, CO, USA 2Department of Biomedical Sciences,
Colorado State University, Fort Collins, CO, USA D. M. Seelig, University of
Minnesota, Department of Veterinary Clinical Sciences, Room 339 VetMedCtrS,
6192A (Campus Delivery Code), 1352 Boyd Ave, St Paul, MN 55108, USA. Email
address: dseelig@umn.edu
Abstract
Chronic wasting disease (CWD) is an efficiently transmitted, fatal, and
progressive prion disease of cervids with an as yet to be fully clarified host
range. While outbred domestic cats (Felis catus) have recently been shown to be
susceptible to experimental CWD infection, the neuropathologic features of the
infection are lacking. Such information is vital to provide diagnostic power in
the event of natural interspecies transmission and insights into host and strain
interactions in interspecies prion infection. Using light microscopy and
immunohistochemistry, we detail the topographic pattern of neural spongiosis
(the “lesion profile”) and the distribution of misfolded prion protein in the
primary and secondary passage of feline CWD (FelCWD). We also evaluated cellular
and subcellular associations between misfolded prion protein (PrPD) and central
nervous system neurons and glial cell populations. From these studies, we (1)
describe the novel neuropathologic profile of FelCWD, which is distinct from
either cervid CWD or feline spongiform encephalopathy (FSE), and (2) provide
evidence of serial passage-associated interspecies prion adaptation. In
addition, we demonstrate through confocal analysis the successful
co-localization of PrPD with neurons, astrocytes, microglia, lysosomes, and
synaptophysin, which, in part, implicates each of these in the neuropathology of
FelCWD. In conclusion, this work illustrates the simultaneous role of both host
and strain in the development of a unique FelCWD neuropathologic profile and
that such a profile can be used to discriminate between FelCWD and FSE.
prion chronic wasting disease immunohistochemistry interspecies cat feline
spongiform encephalopathy transmissible spongiform encephalopathy adaptation
species barrier
Monday, August 8, 2011 Susceptibility of Domestic Cats to CWD Infection
Oral.29: Susceptibility of Domestic Cats to CWD Infection
Amy Nalls, Nicholas J. Haley, Jeanette Hayes-Klug, Kelly Anderson, Davis M.
Seelig, Dan S. Bucy, Susan L. Kraft, Edward A. Hoover and Candace K.
Mathiason†
Colorado State University; Fort Collins, CO USA†Presenting author; Email:
ckm@lamar.colostate.edu
Domestic and non-domestic cats have been shown to be susceptible to one
prion disease, feline spongiform encephalopathy (FSE), thought to be transmitted
through consumption of bovine spongiform encephalopathy (BSE) contaminated meat.
Because domestic and free ranging felids scavenge cervid carcasses, including
those in CWD affected areas, we evaluated the susceptibility of domestic cats to
CWD infection experimentally. Groups of n = 5 cats each were inoculated either
intracerebrally (IC) or orally (PO) with CWD deer brain homogenate. Between
40–43 months following IC inoculation, two cats developed mild but progressive
symptoms including weight loss, anorexia, polydipsia, patterned motor behaviors
and ataxia—ultimately mandating euthanasia. Magnetic resonance imaging (MRI) on
the brain of one of these animals (vs. two age-matched controls) performed just
before euthanasia revealed increased ventricular system volume, more prominent
sulci, and T2 hyperintensity deep in the white matter of the frontal hemisphere
and in cortical grey distributed through the brain, likely representing
inflammation or gliosis. PrPRES and widely distributed peri-neuronal vacuoles
were demonstrated in the brains of both animals by immunodetection assays. No
clinical signs of TSE have been detected in the remaining primary passage cats
after 80 months pi. Feline-adapted CWD was sub-passaged into groups (n=4 or 5)
of cats by IC, PO, and IP/SQ routes. Currently, at 22 months pi, all five IC
inoculated cats are demonstrating abnormal behavior including increasing
aggressiveness, pacing, and hyper responsiveness.
*** Two of these cats have developed rear limb ataxia. Although the limited
data from this ongoing study must be considered preliminary, they raise the
potential for cervid-to-feline transmission in nature.
AD.63:
Susceptibility of domestic cats to chronic wasting disease
Amy V.Nalls,1 Candace Mathiason,1 Davis Seelig,2 Susan Kraft,1 Kevin
Carnes,1 Kelly Anderson,1 Jeanette Hayes-Klug1 and Edward A. Hoover1 1Colorado
State University; Fort Collins, CO USA; 2University of Minnesota; Saint Paul, MN
USA
Domestic and nondomestic cats have been shown to be susceptible to feline
spongiform encephalopathy (FSE), almost certainly caused by consumption of
bovine spongiform encephalopathy (BSE)-contaminated meat. Because domestic and
free-ranging nondomestic felids scavenge cervid carcasses, including those in
areas affected by chronic wasting disease (CWD), we evaluated the susceptibility
of the domestic cat (Felis catus) to CWD infection experimentally. Cohorts of 5
cats each were inoculated either intracerebrally (IC) or orally (PO) with
CWD-infected deer brain. At 40 and 42 mo post-inoculation, two IC-inoculated
cats developed signs consistent with prion disease, including a stilted gait,
weight loss, anorexia, polydipsia, patterned motor behaviors, head and tail
tremors, and ataxia, and progressed to terminal disease within 5 mo. Brains from
these two cats were pooled and inoculated into cohorts of cats by IC, PO, and
intraperitoneal and subcutaneous (IP/SC) routes. Upon subpassage, feline-adapted
CWD (FelCWD) was transmitted to all IC-inoculated cats with a decreased
incubation period of 23 to 27 mo. FelCWD was detected in the brains of all the
symptomatic cats by western blotting and immunohistochemistry and abnormalities
were seen in magnetic resonance imaging, including multifocal T2 fluid
attenuated inversion recovery (FLAIR) signal hyper-intensities, ventricular size
increases, prominent sulci, and white matter tract cavitation. Currently, 3 of 4
IP/SQ and 2 of 4 PO inoculared cats have developed abnormal behavior patterns
consistent with the early stage of feline CWD.
*** These results demonstrate that CWD can be transmitted and adapted to
the domestic cat, thus raising the issue of potential cervid-to- feline
transmission in nature.
www.landesbioscience.com
PO-081: Chronic wasting disease in the cat— Similarities to feline
spongiform encephalopathy (FSE)
FELINE SPONGIFORM ENCEPHALOPATHY FSE
Wednesday, October 17, 2012
Prion Remains Infectious after Passage through Digestive System of American
Crows (Corvus brachyrhynchos)
Chronic Wasting Disease Susceptibility of Four North American Rodents
Chad J. Johnson1*, Jay R. Schneider2, Christopher J. Johnson2, Natalie A.
Mickelsen2, Julia A. Langenberg3, Philip N. Bochsler4, Delwyn P. Keane4, Daniel
J. Barr4, and Dennis M. Heisey2 1University of Wisconsin School of Veterinary
Medicine, Department of Comparative Biosciences, 1656 Linden Drive, Madison WI
53706, USA 2US Geological Survey, National Wildlife Health Center, 6006
Schroeder Road, Madison WI 53711, USA 3Wisconsin Department of Natural
Resources, 101 South Webster Street, Madison WI 53703, USA 4Wisconsin Veterinary
Diagnostic Lab, 445 Easterday Lane, Madison WI 53706, USA *Corresponding author
email: cjohnson@svm.vetmed.wisc.edu
We intracerebrally challenged four species of native North American rodents
that inhabit locations undergoing cervid chronic wasting disease (CWD)
epidemics. The species were: deer mice (Peromyscus maniculatus), white-footed
mice (P. leucopus), meadow voles (Microtus pennsylvanicus), and red-backed voles
(Myodes gapperi). The inocula were prepared from the brains of hunter-harvested
white-tailed deer from Wisconsin that tested positive for CWD. Meadow voles
proved to be most susceptible, with a median incubation period of 272 days.
Immunoblotting and immunohistochemistry confirmed the presence of PrPd in the
brains of all challenged meadow voles. Subsequent passages in meadow voles lead
to a significant reduction in incubation period. The disease progression in
red-backed voles, which are very closely related to the European bank vole (M.
glareolus) which have been demonstrated to be sensitive to a number of TSEs, was
slower than in meadow voles with a median incubation period of 351 days. We
sequenced the meadow vole and red-backed vole Prnp genes and found three amino
acid (AA) differences outside of the signal and GPI anchor sequences. Of these
differences (T56-, G90S, S170N; read-backed vole:meadow vole), S170N is
particularly intriguing due its postulated involvement in "rigid loop" structure
and CWD susceptibility. Deer mice did not exhibit disease signs until nearly 1.5
years post-inoculation, but appear to be exhibiting a high degree of disease
penetrance. White-footed mice have an even longer incubation period but are also
showing high penetrance. Second passage experiments show significant shortening
of incubation periods. Meadow voles in particular appear to be interesting lab
models for CWD. These rodents scavenge carrion, and are an important food source
for many predator species. Furthermore, these rodents enter human and domestic
livestock food chains by accidental inclusion in grain and forage. Further
investigation of these species as potential hosts, bridge species, and
reservoirs of CWD is required.
please see ;
Monday, April 04, 2016
*** Limited amplification of chronic wasting disease prions in the
peripheral tissues of intracerebrally inoculated cattle
I7
Early Trafficking and Dissemination of CWD Prions in Deer
Edward A. Hoover1*, Clare E. Hoover1, Davin M. Henderson1, Nathaniel D.
Denkers1, Kristin A. Davenport1, Shannon Bartelt-Hunt2, Alan M. Elder1, Anthony
E. Kincaid3, 4, Jason C. Bartz3, Mark D. Zabel1, Candace K. Mathiason1 1Prion
Research Center, Department of Microbiology, Immunology, and Pathology, Colorado
State University, Fort Collins, Colorado, USA 2Department of Civil Engineering,
University of Nebraska-Lincoln, Prion2015 Program Guide 10 invited
speakers
PRION 2015
Efficient horizontal infection is a hallmark of chronic wasting disease
(CWD) in freeranging cervids. The mechanisms and pathways that enable this
remarkable process, however, remain incompletely understood--in particular the
facile transmucosal entry, exit, and environmental persistence of CWD prions. We
have focused on trans-mucosal CWD infection in white-tailed deer, specifically
on early prion tissue tropism and later stage prion shedding and association
with environmental constituents using modifications of real-time quaking-induced
conversion combined with amplified immunohistochemistry. We have documented very
early trans-mucosal prion passage (within hours), followed by uptake and
amplification in upper digestive tract lymphoid t i s s u e s ( 4 w e e k s ) ,
and dissemination to more distant lymphoid and non-lymphoid tissue sites (8-12
weeks). We have used quantitative approaches to realtime conversion to estimate
the relatively low (i.e. vs. tissues) prion concentrations in body fluids and
excreta; i.e. >100 (cervidized mouse) LD50 are shed daily in the urine of one
CWD infected deer. Using similar methods, we have also demonstrated and
quantified the impressive affinity of CWD prions for both silty clay loam (a
major environmental soil constituent) and metal surfaces. Mucosal uptake,
excretion, and environmental interactions are central to this most transmissible
of prion diseases.
PMCA Detection of CWD Infection in Cervid and Non-Cervid Species
Hoover, Edward Arthur Colorado State University-Fort Collins, Fort Collins,
CO, United States
ABSTRACT Chronic wasting disease (CWD) of deer and elk is an emerging
highly transmissible prion disease now recognized in 18 States, 2 Canadian
provinces, and Korea.
***We have shown that Infected deer harbor and shed high levels of
infectious prions in saliva, blood, urine, and feces, and in the tissues
generating those body fluids and excreta, thereby leading to facile transmission
by direct contact and environmental contamination.
***We have also shown that CWD can infect some non-cervid species, thus the
potential risk CWD represents to domestic animal species and to humans remains
unknown.
Whether prions borne in blood, saliva, nasal fluids, milk, or excreta are
generated or modified in the proximate peripheral tissue sites, may differ in
subtle ways from those generated in brain, or may be adapted for mucosal
infection remain open questions.
The increasing parallels in the pathogenesis between prion diseases and
human neurodegenerative conditions, such as Alzheimer's and Parkinson's
diseases, add relevance to CWD as a transmissible protein misfolding disease.
The overall goal of this work is to elucidate the process of CWD prion
transmission from mucosal secretory and excretory tissue sites by addressing
these questions:
snip...
Public Health Relevance Chronic wasting disease (CWD) of deer and elk is an
emergent highly transmissible prion disease now recognized throughout the USA as
well as in Canada and Korea. We have shown that infected deer harbor and shed
high levels of infectious prions in saliva, blood, urine, and feces thereby
leading to transmission by direct contact and environmental contamination. In
that our studies have also shown that CWD can infect some non-cervid species,
the potential risk CWD may represents to domestic animal species and humans
remains unknown. The increasing parallels in the development of major human
neurodegenerative conditions, such as Alzheimer's and Parkinson's diseases, and
prion diseases add relevance to CWD as a model of a transmissible protein
misfolding disease. Understanding how infectious misfolded proteins (prions) are
generated and transmitted will aid in interrupting, treating, and managing the
risks associated with these agents and the diseases they cause.
Prion Amplification and Hierarchical Bayesian Modeling Refine Detection of
Prion Infection
Infected animals shed prions into the environment through saliva, feces,
urine and even antler velvet15,20,21,22,23,24. Studies have successfully
transmitted PrPCWD through a single dose of urine or feces from animals
displaying signs of CWD, indicating that at the time of clinical disease
sufficient prions are shed to result in an infectious dose24,25. However, at
what stage(s) of disease animals shed prions into the environment remains
unclear. If shedding occurs early in disease, a sub-clinical animal may not only
shed prions into the environment, increasing the infectious reservoir, but may
also transmit CWD horizontally to their associates.
Keywords: RT-QuIC, chronic wasting disease, diagnosis, feces, prion,
surveillance, urine
Authors: Theodore R. John, Hermann M. Schätzl and Sabine Gilch
Theodore R. John Department of Veterinary Sciences; University of Wyoming;
Laramie, WY USA
Hermann M. Schätzl Department of Veterinary Sciences; University of
Wyoming; Laramie, WY USA; Department of Molecular Biology; University of
Wyoming; Laramie, WY USA; Faculty of Veterinary Medicine; Department of
Comparative Biology and Experimental Medicine; University of Calgary; Calgary,
AB Canada
Sabine Gilch Corresponding author: sgilch@ucalgary.ca Department of
Veterinary Sciences; University of Wyoming; Laramie, WY USA; Faculty of
Veterinary Medicine; Department of Ecosystem and Public Health; University of
Calgary; Calgary, AB Canada
Abstract:
Chronic wasting disease (CWD) is a prion disease of captive and
free-ranging deer (Odocoileus spp), elk (Cervus elaphus nelsonii) and moose
(Alces alces shirasi). Unlike in most other prion diseases, in CWD prions are
shed in urine and feces, which most likely contributes to the horizontal
transmission within and between cervid species. To date, CWD ante-mortem
diagnosis is only possible by immunohistochemical detection of protease
resistant prion protein (PrPSc) in tonsil or recto-anal mucosa-associated
lymphoid tissue (RAMALT) biopsies, which requires anesthesia of animals. We
report on detection of CWD prions in urine collected from pre-symptomatic deer
and in fecal extracts by using real time quaking-induced conversion (RT-QuIC).
This assay can be useful for non-invasive pre-symptomatic diagnosis and
surveillance of CWD.
Received: February 7, 2013; Accepted: March 24, 2013; Published Online:
April 10, 2013
snip...
Introduction
Chronic wasting disease (CWD) is to date the most contagious prion disease
and affects captive and free-ranging elk, deer and moose in North America.1,2
The disease is caused by the accumulation of an abnormally folded isoform of the
cellular prion protein PrPC, denominated PrPSc.3,4 CWD is the cervid equivalent
of bovine spongiform encephalopathy (BSE), scrapie in sheep and goat5 or
Creutzfeldt-Jakob disease (CJD) in humans.6 Although transmission studies of CWD
prions to humanized transgenic mice or non-human primates suggest a strong
species barrier,7-9 recent in vitro studies have demonstrated that human PrP can
be converted by CWD prions into PrPSc upon adaptation.10 Therefore, a potential
for zoonotic transmission, as exemplified by BSE,11 cannot be completely
excluded.
A huge body of evidence suggests that CWD can be efficiently transmitted
horizontally within and between cervid species,12 which may be the reason for
geographical spread and increase in case numbers. Horizontal transmission is
explained by the rather unusual peripheral distribution of prions in CWD
affected animals and the high susceptibility to the disease by oral
infection.13,14 Unlike in most other prion diseases, CWD prions can be found in
a wide variety of tissues, such as skeletal and cardiac muscle15,16 or kidney,17
in addition to the lymphoreticular system and blood.18 Furthermore, they are
shed in significant amounts in saliva,18,19 urine19 or feces,20 which enables
oral infection of animals by foraging on contaminated pastures.
In addition, it has been demonstrated that prions can persist in soil21 and
that water in endemic areas can contain CWDassociated PrPSc.22
snip...
We demonstrate that CWD prions are detectable in urine of orally infected
deer prior to the onset of clinical symptoms. Furthermore, we show that fecal
extracts can be used as a seed in RT-QuIC assays. Thereby, we were able to
detect CWD prions in fecal extracts collected at later stages of the disease.
This study provides the first evidence that RT-QuIC can be successfully used for
the preclinical diagnosis of CWD in specimens that are available by non-invasive
methods.
snip...
In summary, we demonstrate that CWD prions can be detected by RT-QuIC in
urine of orally infected white-tailed deer and mule deer at a pre-symptomatic
stage of the disease.
snip...
2008
Excretion of Transmissible Spongiform Encephalopathy Infectivity in
Urine
Luisa Gregori, Gabor G. Kovacs, Irina Alexeeva, Herbert Budka, and Robert
G. Rohwer
To the extent that results from the hamster model can be generalized to
other TSE infections (and it has so far proven highly predictive), then even the
very low concentrations of infectivity measured here could result in substantial
environmental contamination. Several liters of urine and several thousand doses
of TSE infectivity may be excreted daily over the course of the illness; even
higher titers might be excreted by an animal with nephritis. The high stability
of TSE infectivity would account for its persistence in pasture years after
infected animals are removed (31). Recent studies have shown that infectivity
that is adsorbed and immobilized by soil minerals (32) can still infect hamsters
by oral exposure 29 months later (33). Our study also warns of a possible risk
from TSE contamination to fertility hormones and other medicinal products
extracted from human urine.
Sunday, November 10, 2013
LARGE CJD TSE PRION POTENTIAL CASE STUDY AMONG HUMANS WHO TAKE DEER ANTLER
VELVET WILL BE ONGOING FOR YEARS IF NOT DECADES, but who's cares $
Saturday, February 6, 2016
Secretary's Advisory Committee on Animal Health; Meeting [Docket No.
APHIS-2016-0007] Singeltary Submission
Friday, March 18, 2016 CFSAN
Constituent Update: FDA Announces Final Rule on Bovine Spongiform
Encephalopathy BSE MAD COW TSE PRION Center for Food Safety and Applied
Nutrition - Constituent Update
Tuesday, March 15, 2016
*** Docket No. FDA-2016-N-0321 Risk Assessment of Foodborne Illness
Associated with Pathogens from Produce Grown in Fields Amended with Untreated
Biological Soil Amendments of Animal Origin; Request for Comments, Scientific
Data, and Information Singeltary Submission ***
Monday, March 28, 2016
National Scrapie Eradication Program February 2016 Monthly Report
*** Docket No. APHIS-2007-0127 Scrapie in Sheep and Goats Terry Singeltary
Sr. Submission ***
Monday, November 16, 2015
*** Docket No. APHIS-2007-0127 Scrapie in Sheep and Goats Terry Singeltary
Sr. Submission ***
”The occurrence of CWD must be viewed against the contest of the locations
in which it occurred. It was an incidental and unwelcome complication of the
respective wildlife research programmes. Despite it’s subsequent recognition as
a new disease of cervids, therefore justifying direct investigation, no specific
research funding was forthcoming. The USDA veiwed it as a wildlife problem and
consequently not their province!” page 26.
In Confidence - Perceptions of unconventional slow virus diseases of
animals in the USA - APRIL-MAY 1989 - G A H Wells
3. Prof. A. Robertson gave a brief account of BSE. The US approach was to
accord it a very low profile indeed. Dr. A Thiermann showed the picture in the
''Independent'' with cattle being incinerated and thought this was a fanatical
incident to be avoided in the US at all costs. ...
MAD COW DISEASE HAS BEEN IN THE USA FOR DECADES, AND I BELIEVE IT WAS IN
THE USA FIRST, PLEASE SEE ;
Evidence That Transmissible Mink Encephalopathy Results from Feeding
Infected Cattle Over the next 8-10 weeks, approximately 40% of all the adult
mink on the farm died from TME.
snip...
The rancher was a ''dead stock'' feeder using mostly (>95%) downer
or dead dairy cattle...
=====================================================================
From: Gemma.Smith@wales.gsi.gov.uk
Sent: Wednesday, February 17, 2016 3:41 AM
To: flounder9@verizon.net
Subject: RE: Welsh Government and Food Standards Agency Wales Joint Public
Consultation on the Proposed Transmissible Spongiform Encephalopathies (Wales)
Regulations 2013
Dear Mr Singeltary,
Thank you for your recent email update on Transmissible Spongiform
Encephalopathies (TSEs) and in particular the Chronic Wasting Disease (CWD)
prion. The prevention of all TSEs are a priority for the Welsh Government when
protecting human and animal health, and the development of cases will continue
to be monitored and acted on accordingly.
Many thanks and kindest regards,
Gemma
Gemma Smith Office of the Chief Veterinary Officer / Swyddfa'r Prif Swyddog
Milfeddygol Welsh Government / Llywodraeth Cymru
From: Terry S. Singeltary Sr. [mailto:flounder9@verizon.net]
Sent: 05 February 2016 19:52
To: Endemics
Cc: WAG-EN; Jones, Carwyn (Ministerial);
Correspondence.Alun.Davies@Wales.gsi.gov.uk; Correspondence Mail - CS;
Correspondence Mail - EH; Correspondence Mail - HL; Correspondence Mail - JH;
Correspondence.John.Griffiths@Wales.gsi.gov.uk; Correspondence Mail - LG;
Correspondence Mail - MD; Correspondence.Jeff.Cuthbert@Wales.gsi.gov.uk;
Correspondence Mail - VG; Correspondence Mail - KS;
Correspondence.Gwenda.Thomas@Wales.gsi.gov.uk
Subject: Re: Welsh Government and Food Standards Agency Wales Joint Public
Consultation on the Proposed Transmissible Spongiform Encephalopathies (Wales)
Regulations 2013
update for you all, it’s not pretty. that bse mrr policy will come back to
haunt everyone...kindest regards, terry
Subject: TEXAS NEW CHRONIC WASTING DISEASE CWD CASE DISCOVERD AT CAPTIVE
DEER RELEASE SITE
snip...end...tss
From: Terry S. Singeltary Sr.
Sent: Thursday, February 18, 2016 11:52 AM
To: URL Bernhard
Cc: Press@efsa.europa.eu ; SCER.PublicConsult.55@efsa.europa.eu ;
scer@efsa.europa.eu ; publicmeetings@efsa.europa.eu
Subject: re-Revisiting EFSA@EXPO: emerging issues in animal and plant
health
re-Revisiting EFSA@EXPO: emerging issues in animal and plant health
Greetings Dr. Url and EFSA EXPO Revisiting EFSA@EXPO: emerging issues in
animal and plant health et al,
with the mad cow follies seemingly never to end since the BSE MRR policy
i.e. the legal trading of the TSE PRION was adopted around the globe, I wish to
kindly submit the following, and I urge all of you to finally put to bed once
and for all, bury it, the infamous UKBSEnvCJD only theory. this theory, myth,
corporate BSE, call it what you like, has helped fuel the spread of the TSE
prion disease, of which, North America is now awash in. I urge you to take heed
to your own demise of which you went through during the BSE debacle the first go
around. what we are seeing in North America is the same thing, except our
governments are just better at covering it up with junk science from the USDA,
CFIA, and OIE. with CWD TSE Prion in cervid spreading, maybe on plants too, and
the continued cover up of BSE in cattle in North America, I would be very
concerned if I were the EFSA et al imo. ...
with kindest regards, terry
Report of the Committee on Wildlife Diseases FY2015 CWD TSE PRION
Detections in Farmed Cervids and Wild
snip...end...tss
From: Terry S. Singeltary Sr.
Sent: Sunday, October 25, 2015 5:19 PM
To: EFSA Press ; URL Bernhard
Cc: SCER ; EFSA Press ; SCER.PublicConsult.55
Subject: Re: re-Wasting disease is threat to the entire UK deer population
URGENT UPDATE USA TEXAS ON CWD
Greetings again Dr. Bernhard Url, EFSA et al,
thought you all might want to see this. I do not advertise on the blogs, or
make money doing this. the blogs are for educational use on the tse prion
disease.
CWD TSE prion update update for your files, as follows ;
snip...end...tss
From: Terry S. Singeltary Sr.
Sent: Tuesday, August 18, 2015 11:46 AM
To: sp.info@scottish.parliament.uk
Cc: enquiries@swt.org.uk ; admin@jmt.org ; info@johnmuiraward.org ;
nicky.mcclure@jmt.org ; broadcasting@scottish.parliament.uk ;
racce.committee@scottish.parliament.uk ; Rob.Gibson.msp@scottish.parliament.uk ;
Graeme.Dey.msp@scottish.parliament.uk ; tom.edwards@scottish.parliament.uk ;
wendy.kenyon@scottish.parliament.uk ; SPICe@scottish.parliament.uk ;
racce.committee@scottish.parliament.uk
Subject: re-Wasting disease is threat to the entire UK deer population
URGENT UPDATE USA TEXAS ON CWD
Chronic Wasting Disease CWD TSE Prion is threat to the entire UK deer
population URGENT UPDATE USA TEXAS ON CWD August 2015.
Greetings Honorable Scottish Parliament et al,
I wish to kindly update you on the mad deer disease debacle in Texas. we
have lost Texas to CWD, and TAHC et al are making it worse by catering to the
industry. same as they did with the mad cow they tried to cover up for 7 months
in Texas.
I am just trying to warn you again, please take heed. this is an explosive
situation, that Texas has taken very lightly, which I believe could potentially
have global ramifications, to your wild herds as well ;
Angus MacDonald (Falkirk East) (Scottish National Party): To ask the
Scottish Government what action it is taking to prevent Chronic Wasting Disease
(CWD) in wild deer from entering the country and what discussions it has had
with the EU on the possible impact of CWD on (a) country sport tourism and (b)
sales of venison.
snip...end...tss
From: EFSA Press
Sent: Wednesday, August 19, 2015 5:34 AM
To: 'Terry S. Singeltary Sr.' ; URL Bernhard
Cc: SCER ; EFSA Press ; SCER.PublicConsult.55
Subject: RE: re-Wasting disease is threat to the entire UK deer population
URGENT UPDATE USA TEXAS ON CWD
Dear Mr Singeltary,
Dr. Bernhard Url would like to thank you for your message and asked me to
reply on his behalf.
Going through your email, we have noticed that you are also running a blog
and therefore we thought it would be best if Media Relations acknowledges
receipt of your mail.
I will take care that your mail is distributed to the appropriate
scientific Unit(s) within EFSA. If further questions arise these scientific
Units will get in touch with you.
Kind regards,
Jan
Description: cid:image003.png@01D0C89D.0E1DA970
Jan Op Gen Oorth Team Leader Media Relations Communications & External
Relations Department
European Food Safety Authority Via Carlo Magno 1A 43126 Parma - Italy
Direct: +39 0521 036 474 Email: jan.opgenoorth@efsa.europa.eu
======================
Description: Description: cid:image001.png@01CFCC2F.614429A0
Dear Mr Singeltary
Thank you for your email, which was received by Public Information at the
Scottish Parliament. We provide information about the Scottish Parliament, its
membership, business and procedures. As an impartial information service, we are
unable to take matters forward on your behalf, but we hope that the following
may be of use.
You may be interested in this page on the Scottish Government’s website
about chronic wasting disease in deer. As you may know, the Scottish Parliament
and Scottish Government are separate organisations. The Scottish Parliament is
the law-making body for a wide range of devolved matters such as rural affairs
and the environment, and its role is to scrutinise the work of the Scottish
Government. The Scottish Government is the government in Scotland for devolved
matters, and it is responsible for defining and implementing policy in these
areas.
The webpage above mentions Scottish Natural Heritage, which is funded
through the Scottish Government’s Environment and Forestry Directorate of.
Scottish Natural Heritage has information about this disease on their website.
Scottish Natural Heritage may therefore be the best people for you to contact
about this issue. They have a good contact details section on their website,
which includes contacts for senior staff.
I hope this information is useful. Please contact us again if you have any
questions about the Scottish Parliament.
Yours sincerely
George Clark Public Information and Publications The Scottish Parliament
Our contact details
==================================
Description: Description: Description: Description: Description:
Description: Description: Description: Description: Description: Corp ID for
Signatures
Dear Mr Singeltary,
Thank you for your email, which was received by Public Information at the
Scottish Parliament. We provide impartial information about the Scottish
Parliament, its membership, business and procedures.
The Scottish Parliament’s Rural Affairs, Climate Change and Environment
Committee has been looking into deer management, as you can see from the
following press release, and your email has been forwarded to the committee for
information:
You may be interested in following the committee’s work. Papers for
committee meetings and transcripts of past meetings (called Official Reports)
appear on the following webpage:
I hope this information is of use to you. Please contact us again if you
have any questions about the Scottish Parliament.
Yours sincerely,
George Clark Public Information and Publications The Scottish Parliament
Our contact details
Description: Description: TwitterButtonJuly2012
Please note that any links to external websites are suggestions only. The
Scottish Parliament is not responsible for the content of external websites.
From: Stuart MacDiarmid (Stuart)
Sent: Monday, May 12, 2014 5:12 PM
To: Terry S. Singeltary Sr. Cc: Stuart MacDiarmid (Stuart) ; Alex Thiermann
Subject: RE: Member Country’s detailed justification for listing of chronic
wasting disease of cervids (CWD) against the criteria of Article 1.2.2., the
Code Commission _recommended_ this disease be reconsidered for listing.
Dear Mr Singeltary, I apologise for the lengthy delay in replying to your
email; I have been unwell. I assume that you are writing to me in my capacity as
an elected member of the OIE's Terrestrial Animal Health Standards Commission.
On more than one occasion our Commission has received a request from a Member
Country to list CWD as a disease notifiable to the OIE. However, it is not our
practice to specify which Member Countries make specific requests to us. All
countries which submit national comments to us at our February and September
meetings are listed in the reports of our meetings. However, the country names
are not linked to specific comments or requests. The most recent submissions on
CWD, in February 2013 and September 2013, were in response to the work of an ad
hoc group which met in 2012 to examine all currently listed diseases against the
recently-adopted criteria for listing published in the Terrestrial Animal Health
Code. The report of that ad hoc group and its recommendations were circulated
for Member Country comments in the report of the Terrestrial Animal Health
Standards Commission. The ad hoc group had recommended the delisting of certain
diseases because they do not meet the criteria for listing. However, in
commenting on the recommendations, a Member Country made a submission proposing
the listing of CWD. That was in February 2013. The same country made the same
proposal to the September meeting of the Terrestrial Animal Health Standards
Commission (TAHSC). The TAHSC passed the recommendation to the Director General
of the OIE with a request that next time an ad hoc expert group is convened to
consider issues of listing or delisting they may also evaluate CWD against the
OIE's criteria. That is where the situation stands at present. Next time an ad
hoc group is convened to consider issues of listing and delisting, CWD will be
evaluated. I have no idea of time frames. Because I am off work I do not have
access to my usual resources, otherwise I could have given you URLs to the
various reports and also the Terrestrial Animal Health Code chapter which gives
the criteria for listing a disease as notifiable to the OIE. However, I think if
you search the OIE web site you should be able to find everything. Regards,
Stuart C MacDiarmid Terrestrial Animal Health Standards Commission
--------------------------------------------------------------------------------
From: Terry S. Singeltary Sr. [flounder9@verizon.net]
Sent: Thursday, 8 May 2014 10:07 a.m.
To: Stuart MacDiarmid (Stuart)
Subject: Member Country’s detailed justification for listing of chronic
wasting disease of cervids (CWD) against the criteria of Article 1.2.2., the
Code Commission _recommended_ this disease be reconsidered for listing.
Subject: Member Country’s detailed justification for listing of chronic
wasting disease of cervids (CWD) against the criteria of Article 1.2.2., the
Code Commission _recommended_ this disease be reconsidered for listing.
> In response to a _Member Country’s_ detailed justification for listing
of chronic wasting disease of cervids (CWD) against the criteria of Article
1.2.2., the Code Commission _recommended_ this disease be reconsidered for
listing.
Annual report of the Scientific Network on BSE-TSE EFSA, Question No
EFSA-Q-2013-01004, approved on 11 December 2013 *** Further, it was addressed
that recently discussions have being held at OIE level on Chronic Wasting
Disease of cervids. page 6; http://www.efsa.europa.eu/en/supporting/doc/532e.pdf
Greetings Prof. Stuart MacDiarmid,
I kindly wish to ask a question please. as you know, I have been very
concerned since 1997, of the TSE prion disease, since the death of my mother to
the hvCJD. I have watched keenly the spread of cwd in North America and Korea.
In the links above, it states that there was a possibility that CWD Chronic
Wasting Disease of cervids, was to be brought to the table for possible listing
by OIE, and that some Country had brought this to the table for reasons I am not
sure about. wisely so though.
could you please tell me which Country brought this to the table and for
what reasons ?
and since the code commission recommended this, what happened since then
?
was it finally listed ?
if not, what were the reasons for not listing it ?
Thank You Kindly,
terry
Dear Mr Singeltary,
Further to Professor MacDiarmid’s reply, and in acknowledgement of the same
email sent to a variety of OIE recipients, including Dr Vallat, the urls to the
Terrestrial Animal Health Standards Commission reports that Professor MacDiarmid
refers to are:
February 2013 (Item 5 Criteria for listing diseases):
September 2013 (Item 5 Criteria for listing diseases):
Thank you for your interest and support of OIE’s work.
Yours sincerely
OIE International Trade Department
Thank you for the links, and Thank you for reconsidering CWD as a listed
disease.
I am writing up a report on cwd TSE prion disease, in North America and the
USA, risk factors there from, and game farms, I will be submitting it to you at
a later date for consideration or just for your files later...thanks again,
terry
snip...end...tss
From: Terry S. Singeltary Sr. [mailto:flounder9@verizon.net]
Sent: 23 November 2013 03:39
To: OfficialReport
Cc: Public Information
Subject: Fw: Wasting disease is threat to the entire UK deer
population
snip...end...tss
======================================
From: Terry S. Singeltary Sr.
Sent: Sunday, July 21, 2013 1:44 PM
To: endemics@wales.gsi.gov.uk
Cc: wag-en@mailuk.custhelp.com ; Carwyn.jones@wales.gsi.gov.uk ;
Correspondence.Alun.Davies@Wales.gsi.gov.uk ;
Correspondence.Carl.Sargeant@Wales.gsi.gov.uk ;
Correspondence.Edwina.Hart@Wales.gsi.gov.uk ;
Correspondence.Huw.Lewis@Wales.gsi.gov.uk ;
Correspondence.Jane.Hutt@Wales.gsi.gov.uk ;
Correspondence.John.Griffiths@Wales.gsi.gov.uk ;
Correspondence.Lesley.Griffiths@Wales.gsi.gov.uk ;
Correspondence.Mark.Drakeford@Wales.gsi.gov.uk ;
Correspondence.Jeff.Cuthbert@Wales.gsi.gov.uk ;
Correspondence.Vaughan.Gething@wales.gsi.gov.uk ;
Correspondence.Ken.Skates@wales.gsi.gov.uk ;
Correspondence.Gwenda.Thomas@Wales.gsi.gov.uk
Subject: Welsh Government and Food Standards Agency Wales Joint Public
Consultation on the Proposed Transmissible Spongiform Encephalopathies (Wales)
Regulations 2013
July 21, 2013
Subject: Welsh Government and Food Standards Agency Wales Joint Public
Consultation on the Proposed Transmissible Spongiform Encephalopathies (Wales)
Regulations 2013
endemics@wales.gsi.gov.uk; fenris@caramail.com;
Greetings Welsh Government and Food Standards Agency Wales,
With great urgency, I would kindly like to comment on ;
Number: WG18417
Joint Public Consultation on the Proposed Transmissible Spongiform
Encephalopathies (Wales) Regulations 2013
The European Food Safety Authority (EFSA) and the European Centre for
Disease Prevention and Control jointly advised in 2011 that BSE is the only
animal TSE that has been shown to be a risk to human health and that there is no
epidemiological evidence to suggest that classical scrapie is a risk to human
health.
and
What are the main issues under consideration?
2.1 The main issues under consideration relate to changes in BSE testing
requirements; more proportionate measures for controlling classical scrapie in
sheep flocks and goat herds in which classical scrapie is confirmed; and more
proportionate controls on animal feed. The Welsh Government also wishes to
consult on proposed amendments to the BSE cattle compensation system in light of
identified anomalies in the current system, in addition to a variety of other
proposed technical and procedural amendments to the 2008 Regulations.
2.2 The key specific amendments are summarised and then considered in more
detail below. Other proposed technical amendments, which are considered to have
a negligible impact or have already been implemented administratively, are
listed at Annex A. What are the main issues under consideration?
2.1
* The main issues under consideration relate to changes in BSE testing
requirements;
* more proportionate measures for controlling classical scrapie in sheep
flocks
* and goat herds in which classical scrapie is confirmed; and
* more proportionate controls on animal feed.
Greetings again Welsh Government and Food Standards Agency Wales, with
another TSE prion medical blunder happening just this past week ;
Friday, July 19, 2013
Beaumont Hospital in Dublin assessing patients for CJD
I STRENUOUSLY urge you take my submission with the greatest urgency. My
submission and concerns as follows, and in part mixed in and throughout the
WG18417 Proposed Transmissible Spongiform Encephalopathies (Wales) Regulations
2013. First and foremost, I think it is very important for the Welsh Government
and Food Standards Agency Wales, to take a full inventory of your imports from
North America, and other Countries, especially your pet and fish foods, for
reasons and scientific facts I have supplied below. I think the attempt by
Governments around the globe to do away with the Transmissible Spongiform
Encephalopathy TSE prion disease, via weakening of the BSE TSE prion
surveillance, lowering of age limits in testing, weakening the feed bans, caving
in to industry, ignoring all the science of the past 3 decades, ignoring these
different atypical TSE prion disease breaking out, making up new names for these
TSE prion disease, and by the way, what ever happened to the IBNC BSE, and the
pathology there, and what about testing there from?
The USDA, CFIA, with the help of the OIE, have made it there goal to
extinguish all BSE TSE prion trade barriers, before all the science on the TSE
prion disease is in, and are working hard to exempt all TSE prion in all species
from any trade barrier, and the OIE is working right along with them. This
happened December 2003, when the USA lost it’s OIE BSE Gold Card, when BSE was
first documented in the USA, after a long hard fought battle trying to cover mad
cow disease up. This proven time and time again by the OIG and the GAO of the
USA. I will supply url links of submissions I have made to the USDA et al over
the years, since the death of my Mother to the Heidenhain Variant of Creutzfeldt
Jakob Disease i.e. hvCJD ‘confirmed’ 12/14/97. just another happenstance of bad
luck they tell me, that it only happens in the UK, and that it’s a UK disease,
this mad cow disease, that no other animal species TSE prion disease in the
world, at no other location, can transmit a TSE prion disease to a human, and
then basing all trading protocol from country to country, based on this junk
science, destroys the past 3 decades of trying to eradicate the damn disease,
and the OIE, USDA, CFIA, and all the other countries that know better, that just
goes along with this due to trade purposes, just because it’s a long incubating
disease, just because the science is still in it’s infancy, does not mean we
should start ignoring what early science has taught us, and start weakening any
safety protocols there from.
North America has the most documented TSE prion disease in the wild and in
farmed livestock than any other country in the world, excluding the TSE prion
disease documented in zoo animals. Chronic Wasting Disease CWD in cervids is
running rampant, the USA and Canada can’t stop it, while Mexico has not a clue
of any TSE prion disease. The shooting pens, and CWD there from in the USA, is a
real risk factor, one the game farms refuse to admit they are a big problem with
the spreading of the CWD TSE prion disease, fighting tooth and nail completely
ignoring the evolving science on the CWD TSE prion disease, and how it’s spread,
and these antler mills are multiplying from state to state in big numbers. There
is ample evidence of CWD transmission to humans, to warrant a warning to the
world, of the IATROGENIC potential for this TSE prion disease in cervids, via
the multitude of potential routes of infection, via the medical, dental,
surgical, blood, tissue. CWD has now mutated to multiple strains. The science is
there to warrant this very real concern, it’s just the same as with what
happened in the U.K., the industry and USDA inc., are stopping these concerns to
be made public, with the same watered down junk science used in the beginning of
the BSE blunder. you should all be very aware of this, if you come abroad to
North America. DEFRA has put out a warning on CWD TSE prion disease in the USA
and have put out a document I supplied with additional risk factors from North
America, this is supplied below as well.
Another concern is with your assumptions that typical classical scrapie is
not a risk factor for humans, when there _is_ evidence to show otherwise, that
indeed typical scrapie is a risk factor for humans, as with atypical Scrapie.
The OIE, USDA inc, and the CFIA, have come to the conclusion that neither
typical scrapie nor the atypical Nor-98 scrapie are neither a risk factor for
humans, they have urged the OIE to conclude that atypical Nor-98 to be exempt
from any trading protocols, and indeed have made the Nor-98 atypical scrapie
EXEMPT, and made it legal to trade, and they are also in the works to make
typical scrapie exempt.
typical scrapie consist of many different strains of scrapie, not just one.
and the atypical Nor-98 has very similar features with human
Gerstmann-Sträussler-Scheinker Disease GSS and Variably Protease-Sensitive
Prionopathy VPSPr. I have not seen anywhere in the Bible, or the scrolls of the
Dead Sea, where it was stipulated that indeed typical c-BSE is the only zoonosis
Transmissible Spongiform Encephalopathy TSE prion disease. This is ludicrous in
2013 to still believe this junk science.
With the science to date of the 1st 10 nvCJD victims of Dr. Ironside et al,
and the diagnostic criteria then to diagnose the nvCJD, compared to what Dr.
Gambetti et al diagnosed in their 1st 10, of which young victims are being
diagnosed, but yet changed the name to VPSPr type CJD human TSE, is not
scientific in my opinion. I believe that the UKBSEnvCJD only theory is bogus, it
is not scientific, and should be put to bed once and for all. you cannot have
your cake and eat it too. either Ironside was wrong, or Gambetti is wrong. to
continue this UKBSEnvCJD only myth, will only help continue spread the TSE prion
agent long and far.
typical Scrapie has been studied for decades and decades, and has proven to
be transmitted to non-human primates by their NON-FORCED oral consumption (Gibbs
et al). when you write in absolute terms as this is fact that ‘’BSE is the only
animal TSE that has been shown to be a risk to human health and that there is no
epidemiological evidence to suggest that classical scrapie is a risk to human
health’’ may be true in terms of documentation, but in terms of science to date,
I think you are wishing. I kindly wish to submit the following in good faith
;
snip...end...tss
=============
Friday, November 22, 2013
Wasting disease is threat to the entire UK deer population
Wasting disease is threat to the entire UK deer population CWD TSE PRION
disease in cervids
***SINGELTARY SUBMISSION
The Scottish Parliament’s Rural Affairs, Climate Change and Environment
Committee has been looking into deer management, as you can see from the
following press release,
***and your email has been forwarded to the committee for information:
Sunday, July 21, 2013
Welsh Government and Food Standards Agency Wales Joint Public Consultation
on the Proposed Transmissible Spongiform Encephalopathies (Wales) Regulations
2013
*** Singeltary Submission WG18417
PREVIOUS ASSESSMENT 2012
This assessment has been provided as an update to a previous version
carried out in 2012, due to new evidence about the import of lures for deer
hunting which contain deer urine. The previous assessment is available at:
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
Tuesday, July 17, 2012
O.I.E. BSE, CWD, SCRAPIE, TSE PRION DISEASE Final Report of the 80th
General Session, 20 - 25 May 2012
Monday, January 4, 2016
Long live the OIE, or time to close the doors on a failed entity?
*** Needless conflict ***
Nature 485, 279–280 (17 May 2012) doi:10.1038/485279b
Published online 16 May 2012
Terry S. Singeltary Sr. said:
I kindly wish to submit the following please ;
"The fact the Texas cow showed up fairly clearly implied the existence of
other undetected cases," Dr. Paul Brown, former medical director of the National
Institutes of Health's Laboratory for Central Nervous System Studies and an
expert on mad cow-like diseases, told United Press International. "The question
was, 'How many?' and we still can't answer that." Brown, who is preparing a
scientific paper based on the latest two mad cow cases to estimate the maximum
number of infected cows that occurred in the United States, said he has
"absolutely no confidence in USDA tests before one year ago" because of the
agency's reluctance to retest the Texas cow that initially tested positive.
USDA officials finally retested the cow and confirmed it was infected seven
months later, but only at the insistence of the agency's inspector
general.
"Everything they did on the Texas cow makes everything they did before 2005
suspect," Brown said.
Thursday, October 22, 2015
*** Former Ag Secretary Ann Veneman talks women in agriculture and we talk
mad cow disease USDA and what really happened ***
Thursday, January 14, 2016
*** EMERGING ANIMAL DISEASES Actions Needed to Better Position USDA to
Address Future Risks Report to the Chairman, Committee on Energy and Commerce,
House of Representatives December 2015 GAO-16-132
GAO
Thursday, March 24, 2016
FRANCE CONFIRMS BOVINE SPONGIFORM ENCEPHALOPATHY BSE MAD COW (ESB) chez une
vache dans les Ardennes
Monday, March 28, 2016
National Scrapie Eradication Program February 2016 Monthly Report
Thursday, February 25, 2016
U.S. Food & Drug Administration (FDA) FDA/CFSAN Cosmetics Update:
Cosmetics Program; Import and Domestic and Transmissible Spongiform
Encephalopathy TSE Prion Disease Risk Factors
***WARNING TO ALL CONSUMERS AND COUNTRIES AROUND THE WORLD***
***Note: FDA labs do not conduct BSE analysis and thus no sampling guidance
is issued for BSE. ***
Friday, February 05, 2016
Report of the Committee on Wildlife Diseases FY2015 CWD TSE PRION
Detections in Farmed Cervids and Wild
Saturday, February 6, 2016
*** Secretary's Advisory Committee on Animal Health; Meeting [Docket No.
APHIS-2016-0007] Singeltary Submission ***
Tuesday, March 15, 2016
Docket No. FDA-2016-N-0321 Risk Assessment of Foodborne Illness Associated
with Pathogens from Produce Grown in Fields Amended with Untreated Biological
Soil Amendments of Animal Origin; Request for Comments, Scientific Data, and
Information Singeltary Submission
Friday, March 18, 2016
CFSAN Constituent Update: FDA Announces Final Rule on Bovine Spongiform
Encephalopathy BSE MAD COW TSE PRION
Center for Food Safety and Applied Nutrition - Constituent Update
PLEASE REMEMBER, IN 55 YEARS AND OLDER, THE RATE OF DOCUMENTED CJD JUMPS TO
ONE IN 9,000. but officials don’t tell you that either. carry on...
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14,
2001 JAMA
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
To the Editor: In their Research Letter, Dr Gibbons and colleagues1
reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD)
has been stable since 1985. These estimates, however, are based only on reported
cases, and do not include misdiagnosed or preclinical cases. It seems to me that
misdiagnosis alone would drastically change these figures. An unknown number of
persons with a diagnosis of Alzheimer disease in fact may have CJD, although
only a small number of these patients receive the postmortem examination
necessary to make this diagnosis. Furthermore, only a few states have made CJD
reportable. Human and animal transmissible spongiform encephalopathies should be
reportable nationwide and internationally.
Terry S. Singeltary, Sr Bacliff, Tex
1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob
disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323.
26 March 2003
Terry S. Singeltary, retired (medically) CJD WATCH
I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment
on the CDC's attempts to monitor the occurrence of emerging forms of CJD.
Asante, Collinge et al [1] have reported that BSE transmission to the
129-methionine genotype can lead to an alternate phenotype that is
indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD
and all human TSEs are not reportable nationally. CJD and all human TSEs must be
made reportable in every state and internationally. I hope that the CDC does not
continue to expect us to still believe that the 85%+ of all CJD cases which are
sporadic are all spontaneous, without route/source. We have many TSEs in the USA
in both animal and man. CWD in deer/elk is spreading rapidly and CWD does
transmit to mink, ferret, cattle, and squirrel monkey by intracerebral
inoculation. With the known incubation periods in other TSEs, oral transmission
studies of CWD may take much longer. Every victim/family of CJD/TSEs should be
asked about route and source of this agent. To prolong this will only spread the
agent and needlessly expose others. In light of the findings of Asante and
Collinge et al, there should be drastic measures to safeguard the medical and
surgical arena from sporadic CJDs and all human TSEs. I only ponder how many
sporadic CJDs in the USA are type 2 PrPSc?
2 January 2000
British Medical Journal
U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well
15 November 1999
British Medical Journal
vCJD in the USA * BSE in U.S.
The Lancet Infectious Diseases, Volume 3, Issue 8, Page 463, August 2003
doi:10.1016/S1473-3099(03)00715-1Cite or Link Using DOI
Tracking spongiform encephalopathies in North America
Original
Xavier Bosch
“My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my
mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever
since. What I have found is that we have not been told the truth. CWD in deer
and elk is a small portion of a much bigger problem.” 49-year—old Singeltary is
one of a number of people who have remained largely unsatisfied after being told
that a close relative died from a rapidly progressive dementia compatible with
spontaneous Creutzfeldt—Jakob ...
Terry S. Singeltary Sr. Bacliff, Texas USA 77518 flounder9@verizon.net
posted by Terry S. Singeltary Sr. at
3:45 PM
1 Comments:
This comment has been removed by a blog administrator.
Post a Comment
Subscribe to Post Comments [Atom]
<< Home