Evaluation of the protection of primates transfused with variant Creutzfeldt-Jakob disease–infected blood products filtered with prion removal devices: a 5-year update
TRANSFUSION COMPLICATIONS
Evaluation of the protection of primates transfused with variant
Creutzfeldt-Jakob disease–infected blood products filtered with prion removal
devices: a 5-year update Nathalie Lescoutra-Etchegaray1,†, Nina Jaffré1,†,
Chryslain Sumian2, Valérie Durand3, Evelyne Correia3, Jacqueline Mikol3, Sophie
Luccantoni-Freire3, Audrey Culeux1, Jean-Philippe Deslys3 and Emmanuel E.
Comoy3,* Article first published online: 3 FEB 2015
DOI: 10.1111/trf.12999
© 2015 AABB
Issue
Transfusion Volume 55, Issue 6, pages 1231–1241, June 2015
Lescoutra-Etchegaray, N., Jaffré, N., Sumian, C., Durand, V., Correia, E.,
Mikol, J., Luccantoni-Freire, S., Culeux, A., Deslys, J.-P. and Comoy, E. E.
(2015), Evaluation of the protection of primates transfused with variant
Creutzfeldt-Jakob disease–infected blood products filtered with prion removal
devices: a 5-year update. Transfusion, 55: 1231–1241. doi: 10.1111/trf.12999
Author Information 1 Macopharma, Fontenay-aux-Roses, France
2 Macopharma, Tourcoing, France
3 Division of Prions and Related Diseases (SEPIA), CEA, Institute of
Emerging Diseases and Innovative Therapies (iMETI), Fontenay-aux-Roses,
France
† These authors contributed equally to the work.
*Address reprint requests to: Emmanuel Comoy, CEA, Division of Prions and
Related Diseases (SEPIA), Institute of Emerging Diseases and Innovative
Therapies (iMETI), 18 Route Du Panorama, BP6, 92265 Fontenay-aux-Roses, France;
e-mail: emmanuel.comoy@cea.fr.
This work was supported by a grant from Agence Nationale de la Recherche
(ANR), Project PRIONSECUR ANR05PRIB02302.
Publication History Issue published online: 13 JUN 2015 Article first
published online: 3 FEB 2015 Manuscript Accepted: 30 OCT 2014 Manuscript
Revised: 27 OCT 2014 Manuscript Received: 30 DEC 2013 BACKGROUND Analysis of
archived appendix samples reveals that one in 2000 individuals in the United
Kingdom may carry the infectious prion protein associated with variant
Creutzfeldt-Jakob disease (vCJD), raising questions about the risk of
transfusion transmission from apparently healthy carriers. Blood leukoreduction
shows limited efficiency against prions. Therefore, in absence of antemortem
diagnostic tests, prion removal filters, including the P-Capt filter were
designed to improve blood transfusion safety.
STUDY DESIGN AND METHODS We evaluated the performances of two filters, the
P-Capt and one prototype (PMC#005), with blood-borne infectivity in two
independent experiments. Blood was drawn twice from prion-infected macaques.
Corresponding RBCCs were prepared according to two different procedures: in
Study A, the leukoreduction step was followed by the filtration through the
P-Capt. In Study B, the leukoreduction and prion removal were performed
simultaneously through the PMC#005. For each study, two groups of three animals
were transfused twice with samples before or after filtration.
RESULTS Among the six macaques transfused with nonfiltered samples, five
developed neurologic signs but only four exhibited peripheral detectable
protease-resistant prion protein (PrPres) accumulation. In Study A, the three
animals transfused with P-Capt–filtered samples remain asymptomatic and devoid
of PrPres in lymph node biopsies 6 years after the transfusion. In Study B, one
animal transfused with PMC#005-filtered samples developed vCJD.
CONCLUSION After 5 to 6 years of progress, this ongoing study provides
encouraging results on the prion blood removal performances of the P-Capt filter
in macaques, an utmost relevant model for human prion diseases.
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.
Eurosurveillance, Volume 12, Issue 3, 18 January 2007 Articles Editorial
team1 + Author affiliations
--------------------------------------------------------------------------------
Citation style for this article: Editorial team. Fourth case of
transfusion-associated vCJD infection in the United Kingdom. Euro Surveill.
2007;12(3):pii=3117.
Available online: http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=3117
Date of submission:
--------------------------------------------------------------------------------
Fourth case of transfusion-associated vCJD infection in the United
Kingdom
Editorial team (eurosurveillance.weekly@hpa.org.uk), Eurosurveillance
editorial office
A case of variant Creutzfeldt-Jakob disease (vCJD) has recently been
diagnosed in a patient in the United Kingdom (UK), who received a blood
transfusion from a donor who later developed vCJD [1]. This is the fourth case
of probable transfusion transmission of vCJD infection in the UK. Three of the
four recipients developed symptoms of vCJD. The first symptomatic case of vCJD
associated with blood transfusion was identified in December 2003. This
individual developed vCJD six and a half years after transfusion of red cells
donated by an individual who developed symptoms of vCJD three and a half years
after donation.
A second case of vCJD 'infection' was identified a few months later in a
person who had received red cells from a donor who developed symptoms of vCJD 18
months after donation. This patient (the second case) died from causes unrelated
to vCJD five years after transfusion. Post-mortem investigations found abnormal
prion protein in the spleen and a cervical lymph node., However, prion protein
was not found in the brain, and no pathological features of vCJD were found.
A third case developed symptoms of vCJD six years after receiving a
transfusion of red blood cells, and died two years and eight months later. The
donor of the blood involved developed vCJD about 20 months after donating it.
These three cases have been published as case reports and in the findings
of the ongoing collaborative study between the National Blood Services, the
National CJD Surveillance Unit, and the Office for National Statistics. This
study aims to collect evidence about transmission of CJD or vCJD via the blood
supply [2,3,4,5].
The new, fourth case is in a patient who developed symptoms of vCJD eight
and a half years after receiving a transfusion of red blood cells from a donor
who developed vCJD about 17 months after this blood was donated [1]. The donor
to this case also donated the vCJD-implicated blood transfused to the third
case. As for all other reported clinical vCJD cases that have been tested for
genotype, this patient is a methionine homozygote at codon 129 of the prion
protein gene. The patient is currently alive.
All four cases had received transfusions of non-leucodepleted red blood
cells between 1996 and 1999. Since October 1999, leucocytes have been removed
from all blood used for transfusion in the UK. The effect of leucodepletion on
the reduction of the risk of transmission of vCJD from an infective donation is
uncertain.
This fourth case of vCJD infection associated with blood transfusion
further increases the level of concern about the risk of vCJD transmission
between humans by blood transfusion, although much remains unknown. This
reinforces the importance of the existing precautions that have been introduced
to reduce the risk of transmission of vCJD infection by blood and blood products
[6]. No cases of vCJD have been associated with fractionated plasma products.
The small group of living recipients of vCJD-implicated blood transfusion in the
UK have been informed of their potential exposure to vCJD by blood transfusion,
asked to take certain precautions to reduce the risk of onward person-to-person
transmission of vCJD during health care, and offered specialist neurological
evaluation and advice.
This article has been adapted from reference 1
References: Health Protection Agency. Fourth case of variant CJD associated
with blood transfusion (press release). Press release, 18 January 2007. (http://www.hpa.org.uk/hpa/news/articles/press_releases/2007/070118_vCJD.htm)
Llewelyn CA, Hewitt PE, Knight RSG, Amar K, Cousens S, Mackenzie J, et al.
Possible transmission of variant CJD disease by blood transfusion. Lancet 2004;
363:417-21. Peden AH, Head MW, Ritchie DL, Bell JE, Ironside JW. Preclinical
vCJD after blood transfusion in a PRNP codon 129 heterozygous patient. Lancet
2004 ; 364: 527-9. Wroe SJ, Pal S, Siddique D, Hyare H, Macfarlane R, et al
Clinical presentation and pre-mortem diagnosis of blood transfusion-associated
variant CJD. Lancet 2006;368:2061-67. Hewitt PE, Llewelyn CA, Mackenzie J, Will
RG. Creutzfeldt-Jakob disease and blood transfusion: results of the UK
Transfusion Medicine Epidemiology review study. Vox Sang. 2006;91(3):221-230.
Department of Health [London]. Further precautions to protect blood supply.
Press release 2004/0104, 16 March 2004. (http://www.dh.gov.uk/PublicationsAndStatistics/PressReleases/PressReleasesNotices/fs/en?CONTENT_ID=4076608&chk=MTwE%2Bl)
http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=3117
*** The UK first reported a presumptive clinical case of TTvCJD in 2003,
followed by two other clinical cases and two preclinical or subclinical
infections,..
Thursday, September 10, 2015
25th Meeting of the Transmissible Spongiform Encephalopathies Advisory
Committee Food and Drug Administration Silver Spring, Maryland June 1, 2015
Sunday, November 23, 2014
Confirmed Variant Creutzfeldt-Jakob Disease (variant CJD) Case in Texas in
June 2014 confirmed as USA case NOT European
Confirmed Variant Creutzfeldt-Jakob Disease (variant CJD) Case in Texas
Updated: October 7, 2014
CDC and the Texas Department of State Health Services (DSHS) have completed
the investigation of the recently reported fourth vCJD case in the United
States. It confirmed that the case was in a US citizen born outside the Americas
and indicated that the patient's exposure to the BSE/vCJD agent most likely
occurred before he moved to the United States; the patient had resided in
Kuwait, Russia and Lebanon. The completed investigation did not support the
patient's having had extended travel to European countries, including the United
Kingdom, or travel to Saudi Arabia.
***The specific overseas country where this patient’s infection occurred is
less clear largely because the investigation did not definitely link him to a
country where other known vCJD cases likely had been infected.
2015 PRION CONFERENCE
*** RE-P.164: Blood transmission of prion infectivity in the squirrel
monkey: The Baxter study
***suggest that blood donations from cases of GSS (and perhaps other
familial forms of TSE) carry more risk than from vCJD cases, and that little or
no risk is associated with sCJD. ***
P.164: Blood transmission of prion infectivity in the squirrel monkey: The
Baxter study
Paul Brown1, Diane Ritchie2, James Ironside2, Christian Abee3, Thomas
Kreil4, and Susan Gibson5 1NIH (retired); Bethesda, MD USA; 2University of
Edinburgh; Edinburgh, UK; 3University of Texas; Bastrop, TX USA; 4Baxter
Bioscience; Vienna, Austria; 5University of South Alabama; Mobile, AL USA
Five vCJD disease transmissions and an estimated 1 in 2000 ‘silent’
infections in UK residents emphasize the continued need for information about
disease risk in humans. A large study of blood component infectivity in a
non-human primate model has now been completed and analyzed. Among 1 GSS, 4
sCJD, and 3 vCJD cases, only GSS leukocytes transmitted disease within a 5–6
year surveillance period. A transmission study in recipients of multiple whole
blood transfusions during the incubation and clinical stages of sCJD and vCJD in
ic-infected donor animals was uniformly negative. These results, together with
other laboratory studies in rodents and nonhuman primates and epidemiological
observations in humans, suggest that blood donations from cases of GSS (and
perhaps other familial forms of TSE) carry more risk than from vCJD cases, and
that little or no risk is associated with sCJD. The issue of decades-long
incubation periods in ‘silent’ vCJD carriers remains open.
ran across an old paper from 1984 ;
***The occurrence of contact cases raises the possibility that transmission
in families may be effected by an unusually virulent strain of the agent.
***
From: Terry S. Singeltary Sr.
Sent: Saturday, November 15, 2014 9:29 PM
To: Terry S. Singeltary Sr.
Subject: THE EPIDEMIOLOGY OF CREUTZFELDT-JAKOB DISEASE R. G. WILL
1984
THE EPIDEMIOLOGY OF CREUTZFELDT-JAKOB DISEASE
R. G. WILL
1984
snip...
THE BAXTER STUDY...SEE MORE HERE ;
Saturday, May 30, 2015
PRION 2015 ORAL AND POSTER CONGRESSIONAL ABSTRACTS
Wednesday, December 11, 2013
*** Detection of Infectivity in Blood of Persons with Variant and Sporadic
Creutzfeldt-Jakob Disease ***
THE BAXTER STUDY...SEE MORE HERE ;
Thursday, September 10, 2015
25th Meeting of the Transmissible Spongiform Encephalopathies Advisory
Committee Food and Drug Administration Silver Spring, Maryland June 1, 2015
*** 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. ***
*** 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).***
*** Brain and lymphoid tissue contain the highest levels of the abnormal
prion protein, the marker for disease, and transmission in white tailed deer can
be accomplished by blood transfusion from experimentally infected deer to naive
deer. ***
Research Project: TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES: THE ROLE OF
GENETICS, STRAIN VARIATION, AND ENVIRONMENTAL CONTAMINATION IN DISEASE CONTROL
Title: Chronic Wasting Disease Prions in Elk Antler Velvet
Authors
item Angers, R - UNIVERSITY OF KENTUCKY item Napier, D - UNIVERSITY OF
KENTUCKY item Seward, T - UNIVERSITY OF KENTUCKY item Green, M - UNIVERSITY OF
KENTUCKY item Spraker, T - COLORADO STATE UNIVERSITY item O'Rourke, Katherine
item Balachandran, A - CANADIAN FOOD INSPCTN AG item Telling, G - UNIVERSITY OF
KENTUCKY
Submitted to: Emerging Infectious Diseases Publication Type: Peer Reviewed
Journal Publication Acceptance Date: February 1, 2009 Publication Date: May 1,
2009 Repository URL: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2687044/pdf/08-1458_finalR.pdf
Citation: Angers RC, Seward TS, Napier D, Green M, Hoover E, Spraker T, et al.
2009.
Chronic wasting disease prions in elk antler velvet.
Emerg Infect Dis 15(5):696-703.
Interpretive Summary: Chronic wasting disease (CWD) is a fatal neurologic
disease of deer and elk in the United States and Canada. The disease is
associated with accumulations of infectious proteins in the brain, nervous
system, blood, and a limited number of other tissues. In this study, the
investigators examined elk antler velvet, the covering that grows on elk antlers
every year. Antler velvet is rich in blood and nervous supply and may represent
a source of infectious material as the velvet is shed every year. Antler velvet
and brain tissue from four infected elk was examined by immunohistochemistry and
biochemical methods, with no evidence of the abnormal prion protein in antler
velvet. The same preparations were tested in genetically engineered mice
susceptible to CWD. Mice in both inoculated groups developed prion disease. This
finding demonstrates that antler velvet from CWD infected elk contain infectious
material and may represent a risk material to other elk. Technical Abstract:
Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy or
prion disease of captive and free ranging white tailed deer, mule deer, Rocky
Mountain elk and moose in the some parts of the United States and Canada. The
presence of the disease has sharply curtailed movement of captive animals and
reduced the domestic or international market for some cervid by-products. The
disease appears to be transmitted by Rocky Mountain elk relatively late in the
disease course, but the sources of the infectious material remain undefined.
Brain and lymphoid tissue contain the highest levels of the abnormal prion
protein, the marker for disease, and transmission in white tailed deer can be
accomplished by blood transfusion from experimentally infected deer to naive
deer. In this study, the investigators examined the transmission potential of
antler velvet, a highly vascularized and innervated epidermal tissue covering
the growing antler. Antler velvet is shed each year and is widely used as a
nutritional supplement. Genetically engineered mice susceptible to CWD were
inoculated with homogenates of paired brain and antler velvet from 4 elk with
CWD. Mice in both groups developed a transmissible spongiform encephalopathy.
These findings demonstrate prion infectivity accumulates in antler velvet and
may have impact on marketing of this product.
*** Brain and lymphoid tissue contain the highest levels of the abnormal
prion protein, the marker for disease, and transmission in white tailed deer can
be accomplished by blood transfusion from experimentally infected deer to naive
deer. ***
see;
Thursday, March 19, 2009
Chronic Wasting Disease Prions in Elk Antler Velvet (Nutritional
Supplements and CJD) 10.3201/eid1505.081458 Suggested citation for this article:
Angers RC, Seward TS, Napier D, Green M, Hoover E, Spraker T, et al.
Chronic wasting disease prions in elk antler velvet.
Emerg Infect Dis. 2009 May; [Epub ahead of print]
SNIP...
SEE MUCH MORE HERE ;
Wednesday, August 5, 2015 Federal judge enters permanent injunction against
Wisconsin dietary supplement manufacturers prohibited cattle materials BSE TSE
Prion
*** Singeltary Submission to BSE Inquiry on Nutritional Supplements
containing SRM 1998 ***
*** 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.***
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
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.***
From: Terry S. Singeltary Sr.
Sent: Saturday, November 15, 2014 9:29 PM
To: Terry S. Singeltary Sr.
Subject: THE EPIDEMIOLOGY OF CREUTZFELDT-JAKOB DISEASE R. G. WILL 1984
THE EPIDEMIOLOGY OF CREUTZFELDT-JAKOB DISEASE
R. G. WILL
1984
*** 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). (SEE LINK IN REPORT HERE...TSS) PLUS, THE CDC DID NOT PUT
THIS WARNING OUT FOR THE WELL BEING OF THE DEER AND ELK ;
snip...
85%+ of all human tse prion disease is sporadic CJD.
see what the NIH prion Gods say themselves ;
‘’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.’’
‘’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.’’
*** 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).***
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 ;
July's Milwaukee Journal Sentinel article did prod state officials to ask
CDC to investigate the cases of the three men who shared wild game feasts. The
two men the CDC is still investigating were 55 and 66 years old. But there's
also Kevin Boss, a Minnesota hunter who ate Barron County venison and died of
CJD at 41. And there's Jeff Schwan, whose Michigan Tech fraternity brothers used
to bring venison sausage back to the frat house. His mother, Terry, says that in
May 2001, Jeff, 26, began complaining about his vision. A friend noticed
misspellings in his e-mail, which was totally unlike him. Jeff began losing
weight. He became irritable and withdrawn. By the end of June, he couldn't
remember the four-digit code to open the garage door or when and how to feed his
parents' cats. At a family gathering in July, he stuck to his parents and
girlfriend, barely talking. "On the night we took him to the hospital, he was
speaking like he was drunk or high and I noticed his pupils were so dilated I
couldn't see the irises," his mother says. By then, Jeff was no longer able to
do even simple things on his computer at work, and "in the hospital, he couldn't
drink enough water." When he died on September 27, 2001, an autopsy confirmed he
had sporadic CJD.
In 2000, Belay looked into three CJD cases reported by The Denver Post, two
hunters who ate meat from animals killed in Wyoming and the daughter of a hunter
who ate venison from a plant that processed Colorado elk. All three died of CJD
before they were 30 years old. The CDC asked the USDA to kill 1,000 deer and elk
in the area where the men hunted. Belay and others reported their findings in
the Archives of Neurology, writing that although "circumstances suggested a link
between the three cases and chronic wasting disease, they could find no 'causal'
link." Which means, says Belay, "not a single one of those 1,000 deer tested
positive for CWD. For all we know, these cases may be CWD. What we have now
doesn't indicate a connection. That's reassuring, but it would be wrong to say
it will never happen."
So far, says NIH researcher Race, the two Wisconsin cases pinpointed by the
newspaper look like spontaneous CJD. "But we don't know how CWD would look in
human brains. It probably would look like some garden-variety sporadic CJD."
What the CDC will do with these cases and four others (three from Colorado and
Schwan from Upper Michigan), Race says, is "sequence the prion protein from
these people, inject it into mice and wait to see what the disease looks like in
their brains. That will take two years."
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 ***
*** 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, Val erie 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 longe 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...TSS
===============
Saturday, January 31, 2015
European red deer (Cervus elaphus elaphus) are susceptible to Bovine
Spongiform Encephalopathy BSE by Oral Alimentary route
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 soon as possible for the following
reasons...
======
In the USA, under the Food and Drug Administrations 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.
======
31 Jan 2015 at 20:14 GMT
*** Ruminant feed ban for cervids in the United States? ***
31 Jan 2015 at 20:14 GMT
Sunday, October 25, 2015
USAHA Detailed Events Schedule – 119th USAHA Annual Meeting CAPTIVE
LIVESTOCK CWD SCRAPIE TSE PRION
Monday, October 26, 2015
FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED
VIOLATIONS OFFICIAL ACTION INDICATED OIA UPDATE October 2015
Abstract
Although Bovine Spongiform Encephalopathy (BSE) is the cause of variant
Creutzfeldt Jakob disease (vCJD) in humans, the zoonotic potential of scrapie
prions remains unknown. Mice genetically engineered to overexpress the human
prion protein (tgHu) have emerged as highly relevant models for gauging the
capacity of prions to transmit to humans. These models can propagate human
prions without any apparent transmission barrier and have been used used to
confirm the zoonotic ability of BSE. Here we show that a panel of sheep scrapie
prions transmit to several tgHu mice models with an efficiency comparable to
that of cattle BSE. The serial transmission of different scrapie isolates in
these mice led to the propagation of prions that are phenotypically identical to
those causing sporadic CJD (sCJD) in humans. These results demonstrate that
scrapie prions have a zoonotic potential and raise new questions about the
possible link between animal and human prions.
Subject terms: Biological sciences• Medical research At a glance
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
1: J Infect Dis 1980 Aug;142(2):205-8
Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to
nonhuman primates.
Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.
Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep
and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were
exposed to the infectious agents only by their nonforced consumption of known
infectious tissues. The asymptomatic incubation period in the one monkey exposed
to the virus of kuru was 36 months; that in the two monkeys exposed to the virus
of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the
two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively.
Careful physical examination of the buccal cavities of all of the monkeys failed
to reveal signs or oral lesions. One additional monkey similarly exposed to kuru
has remained asymptomatic during the 39 months that it has been under
observation.
snip...
The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie
by natural feeding to squirrel monkeys that we have reported provides further
grounds for concern that scrapie-infected meat may occasionally give rise in
humans to Creutzfeldt-Jakob disease.
PMID: 6997404
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract
Recently the question has again been brought up as to whether scrapie is
transmissible to man. This has followed reports that the disease has been
transmitted to primates. One particularly lurid speculation (Gajdusek 1977)
conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and
transmissible encephalopathy of mink are varieties of a single "virus". The U.S.
Department of Agriculture concluded that it could "no longer justify or permit
scrapie-blood line and scrapie-exposed sheep and goats to be processed for human
or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is
emphasized by the finding that some strains of scrapie produce lesions identical
to the once which characterize the human dementias"
Whether true or not. the hypothesis that these agents might be
transmissible to man raises two considerations. First, the safety of laboratory
personnel requires prompt attention. Second, action such as the "scorched meat"
policy of USDA makes the solution of the scrapie problem urgent if the sheep
industry is not to suffer grievously.
snip...
76/10.12/4.6
Nature. 1972 Mar 10;236(5341):73-4.
Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis).
Gibbs CJ Jr, Gajdusek DC.
Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0
Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)
C. J. GIBBS jun. & D. C. GAJDUSEK
National Institute of Neurological Diseases and Stroke, National Institutes
of Health, Bethesda, Maryland
SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey
(Macaca fascicularis) with an incubation period of more than 5 yr from the time
of intracerebral inoculation of scrapie-infected mouse brain. The animal
developed a chronic central nervous system degeneration, with ataxia, tremor and
myoclonus with associated severe scrapie-like pathology of intensive astroglial
hypertrophy and proliferation, neuronal vacuolation and status spongiosus of
grey matter. The strain of scrapie virus used was the eighth passage in Swiss
mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral
passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton,
Berkshire).
Friday, January 30, 2015
*** Scrapie: a particularly persistent pathogen ***
Thursday, March 26, 2015
Increased Infectivity of Anchorless Mouse Scrapie Prions in Transgenic Mice
Overexpressing Human Prion Protein
Increased susceptibility of human-PrP transgenic mice to bovine spongiform
encephalopathy following passage in sheep
J. Virol. doi:10.1128/JVI.01578-10 Copyright (c) 2010, American Society for
Microbiology and/or the Listed Authors/Institutions. All Rights Reserved.
Increased susceptibility of human-PrP transgenic mice to bovine spongiform
encephalopathy following passage in sheep.
Chris Plinston, Patricia Hart, Angela Chong, Nora Hunter, James Foster,
Pedro Piccardo, Jean C. Manson, and Rona M Barron* Neuropathogenesis Division,
The Roslin Institute and R(D)SVS, University of Edinburgh, Roslin, Midlothian,
UK; Laboratory of Bacterial and TSE Agents, Food and Drug Administration,
Rockville, MD, USA
* To whom correspondence should be addressed. Email:
rona.barron@roslin.ed.ac.uk .
Abstract
The risk of transmission of ruminant transmissible spongiform
encephalopathy (TSE) to humans was thought to be low due to the lack of
association between sheep scrapie and incidence of human TSE. However a single
TSE agent strain has been shown to cause both bovine spongiform encephalopathy
(BSE) and human vCJD, indicating that some ruminant TSEs may be transmissible to
humans. While the transmission of cattle BSE to humans in transgenic mouse
models has been inefficient, indicating the presence of a significant
transmission barrier between cattle and humans, BSE has been transmitted to a
number of other species. Here we aimed to further investigate the human
transmission barrier following passage of BSE in a sheep. Following inoculation
with cattle BSE, gene targeted transgenic mice expressing human PrP showed no
clinical or pathological signs of TSE disease. However following inoculation
with an isolate of BSE that had been passaged through a sheep, TSE associated
vacuolation and proteinase-K resistant PrP deposition were observed in mice
homozygous for the codon 129-methionine PRNP gene. This observation may be due
to higher titres of the BSE agent in sheep, or an increased susceptibility of
humans to BSE prions following passage through a sheep. ***However these data
confirm that, contrary to previous predictions, it is possible that a sheep
prion may be transmissible to humans and that BSE from other species may be a
public health risk.
Suspect symptoms
What if you can catch old-fashioned CJD by eating meat from a sheep
infected with scrapie?
28 Mar 01
Most doctors believe that sCJD is caused by a prion protein deforming by
chance into a killer. But Singeltary thinks otherwise. He is one of a number of
campaigners who say that some sCJD, like the variant CJD related to BSE, is
caused by eating meat from infected animals. Their suspicions have focused on
sheep carrying scrapie, a BSE-like disease that is widespread in flocks across
Europe and North America.
Now scientists in France have stumbled across new evidence that adds weight
to the campaigners' fears. To their complete surprise, the researchers found
that one strain of scrapie causes the same brain damage in mice as sCJD.
"This means we cannot rule out that at least some sCJD may be caused by
some strains of scrapie," says team member Jean-Philippe Deslys of the French
Atomic Energy Commission's medical research laboratory in Fontenay-aux-Roses,
south-west of Paris. Hans Kretschmar of the University of Göttingen, who
coordinates CJD surveillance in Germany, is so concerned by the findings that he
now wants to trawl back through past sCJD cases to see if any might have been
caused by eating infected mutton or lamb...
2001
Suspect symptoms
What if you can catch old-fashioned CJD by eating meat from a sheep
infected with scrapie?
28 Mar 01
Like lambs to the slaughter
31 March 2001
by Debora MacKenzie Magazine issue 2284.
FOUR years ago, Terry Singeltary watched his mother die horribly from a
degenerative brain disease. Doctors told him it was Alzheimer's, but Singeltary
was suspicious. The diagnosis didn't fit her violent symptoms, and he demanded
an autopsy. It showed she had died of sporadic Creutzfeldt-Jakob disease.
Most doctors believe that sCJD is caused by a prion protein deforming by
chance into a killer. But Singeltary thinks otherwise. He is one of a number of
campaigners who say that some sCJD, like the variant CJD related to BSE, is
caused by eating meat from infected animals. Their suspicions have focused on
sheep carrying scrapie, a BSE-like disease that is widespread in flocks across
Europe and North America.
Now scientists in France have stumbled across new evidence that adds weight
to the campaigners' fears. To their complete surprise, the researchers found
that one strain of scrapie causes the same brain damage in mice as sCJD.
"This means we cannot rule out that at least some sCJD may be caused by
some strains of scrapie," says team member Jean-Philippe Deslys of the French
Atomic Energy Commission's medical research laboratory in Fontenay-aux-Roses,
south-west of Paris. Hans Kretschmar of the University of Göttingen, who
coordinates CJD surveillance in Germany, is so concerned by the findings that he
now wants to trawl back through past sCJD cases to see if any might have been
caused by eating infected mutton or lamb.
Scrapie has been around for centuries and until now there has been no
evidence that it poses a risk to human health. But if the French finding means
that scrapie can cause sCJD in people, countries around the world may have
overlooked a CJD crisis to rival that caused by BSE.
Deslys and colleagues were originally studying vCJD, not sCJD. They
injected the brains of macaque monkeys with brain from BSE cattle, and from
French and British vCJD patients. The brain damage and clinical symptoms in the
monkeys were the same for all three. Mice injected with the original sets of
brain tissue or with infected monkey brain also developed the same
symptoms.
As a control experiment, the team also injected mice with brain tissue from
people and animals with other prion diseases: a French case of sCJD; a French
patient who caught sCJD from human-derived growth hormone; sheep with a French
strain of scrapie; and mice carrying a prion derived from an American scrapie
strain. As expected, they all affected the brain in a different way from BSE and
vCJD. But while the American strain of scrapie caused different damage from
sCJD, the French strain produced exactly the same pathology.
"The main evidence that scrapie does not affect humans has been
epidemiology," says Moira Bruce of the neuropathogenesis unit of the Institute
for Animal Health in Edinburgh, who was a member of the same team as Deslys.
"You see about the same incidence of the disease everywhere, whether or not
there are many sheep, and in countries such as New Zealand with no scrapie." In
the only previous comparisons of sCJD and scrapie in mice, Bruce found they were
dissimilar.
But there are more than 20 strains of scrapie, and six of sCJD. "You would
not necessarily see a relationship between the two with epidemiology if only
some strains affect only some people," says Deslys. Bruce is cautious about the
mouse results, but agrees they require further investigation. Other trials of
scrapie and sCJD in mice, she says, are in progress.
People can have three different genetic variations of the human prion
protein, and each type of protein can fold up two different ways. Kretschmar has
found that these six combinations correspond to six clinical types of sCJD: each
type of normal prion produces a particular pathology when it spontaneously
deforms to produce sCJD.
But if these proteins deform because of infection with a disease-causing
prion, the relationship between pathology and prion type should be different, as
it is in vCJD. "If we look at brain samples from sporadic CJD cases and find
some that do not fit the pattern," says Kretschmar, "that could mean they were
caused by infection."
There are 250 deaths per year from sCJD in the US, and a similar incidence
elsewhere. Singeltary and other US activists think that some of these people
died after eating contaminated meat or "nutritional" pills containing dried
animal brain. Governments will have a hard time facing activists like Singeltary
if it turns out that some sCJD isn't as spontaneous as doctors have
insisted.
Deslys's work on macaques also provides further proof that the human
disease vCJD is caused by BSE. And the experiments showed that vCJD is much more
virulent to primates than BSE, even when injected into the bloodstream rather
than the brain. This, says Deslys, means that there is an even bigger risk than
we thought that vCJD can be passed from one patient to another through
contaminated blood transfusions and surgical instruments.
Like lambs to the slaughter
Thursday, December 20, 2012
OIE GROUP RECOMMENDS THAT SCRAPE PRION DISEASE BE DELISTED AND SAME OLD BSe
WITH BOVINE MAD COW DISEASE
Monday, November 30, 2009
USDA AND OIE COLLABORATE TO EXCLUDE ATYPICAL SCRAPIE NOR-98 ANIMAL HEALTH
CODE
Monday, April 25, 2011
Experimental Oral Transmission of Atypical Scrapie to Sheep Volume 17,
Number 5-May 2011
Friday, February 11, 2011
Atypical/Nor98 Scrapie Infectivity in Sheep Peripheral Tissues
Monday, June 27, 2011
Comparison of Sheep Nor98 with Human Variably Protease-Sensitive
Prionopathy and Gerstmann-Sträussler-Scheinker Disease
Thursday, October 22, 2015
Former Ag Secretary Ann Veneman talks women in agriculture and we talk mad
cow disease USDA and what really happened
Sunday, October 18, 2015
*** World Organisation for Animal Health (OIE) and the Institut Pasteur
Cooperating on animal disease and zoonosis research
Friday, October 23, 2015
CJD FOUNDATION CREUTZFELDT JAKOB DISEASE TSE PRION QUESTIONNAIRE UPDATE
OCTOBER 2015
Self-Propagative Replication of Ab Oligomers Suggests Potential
Transmissibility in Alzheimer Disease
Received July 24, 2014; Accepted September 16, 2014; Published November 3,
2014
*** Singeltary comment ***
Thursday, October 1, 2015
Alzheimergate, re-Evidence for human transmission of amyloid-β pathology
and cerebral amyloid angiopathy, Singeltary Submission to Nature
Tuesday, August 26, 2014
Blood reference materials from macaques infected with variant
Creutzfeldt-Jakob disease agent
Results We sampled blood 19 times from the inoculated monkeys at various
stages of the disease over a period of 29 months, generating liters of
vCJD-infected macaque blood. vCJD was confirmed in all inoculated macaques.
After PMCA, PrPTSE was detected in plasma from infected monkeys, but not from
uninfected animals. Both mouse models were more sensitive to infection with
macaque-adapted vCJD agent than to primary human vCJD agent.
Mad deer disease can infect normal human brains in laboratory tests
Monday, May 8, 2000
www.ems.org has further information and facts on CJD. Jennifer Kelly or Amy
Leska, EMS 202/463-6670 Washington, D.C.
Public health advocates are demanding that the Food and Drug Administration
close loopholes in animal feed regulations to prevent the spread of U.S. mad
cow-type diseases now at epidemic levels in Western deer and elk that might
infect people who eat meat. In a letter sent today to the FDA, the Center for
Food Safety (CFS), the Humane Farming Association, and families of U.S. victims
of the human version of mad cow disease, Creutzfeldt-Jakob disease (CJD) are
demanding new efforts to protect public health and food safety. The FDA was
asked to respond to a legal petition filed in January 1999 that would change
U.S. animal feed regulations to prevent the spread of U.S. mad cow-type diseases
already occurring in deer, elk, sheep and humans, and suspected in pigs and
cattle.
Under current FDA regulations animals known to be infected with mad
cow-type disease such as deer and elk infected with 'chronic wasting disease'
and scrapie-infected sheep, can be legally fed to pigs, chickens and pets, which
in turn can be rendered and fed to cows. Billions of pounds of slaughterhouse
waste in the form of rendered animal by-products are fed to US livestock every
year as fat and protein supplements, despite this practice being the known route
of transmission of British mad cow disease.
A fatal 'mad deer' disease called chronic wasting disease or CWD is
occurring at epidemic levels in deer and elk in Western states and on game
farms, CFS legal director Joseph Mendelson wrote in the letter to the FDA. This
may already be claiming human lives as is suggested by the alarming appearance
of unusually young victims of Creutzfeldt-Jakob disease.
*** Today at the first CJD Foundation conference in Miami, government
researcher Byron W. Caughey, Ph.D., announced that in laboratory tests CWD from
deer can infect human brain tissue at rate similar to British mad cow disease.
In Britain 56 people have died of human mad cow disease, the death toll is
climbing and some scientists suspect it will claim hundreds of thousands of
lives in the decades ahead. Caughey's research on US mad deer disese was
conducted at the National Institutes of Health Rocky Mountain Laboratories in
Hamilton, Montana, and has not yet been published.
The most recent suspected victim of US mad deer disease is Jay Dee Whitlock
II of Oklahoma, who died of CJD on April 7, 2000. Whitlock, 28, was an avid deer
hunter and venison consumer. He is the second young hunter to die of CJD in the
past year.
John Stauber, co-author of Mad Cow USA and a speaker at the CJD Foundation
conference, said, "The announcement that US mad deer disease can infect the
human brain, and that it happens at a rate similar to British mad cow disease,
is extremely disturbing. A deadly human dementia might be already spreading from
deer and elk into hunters in Western states, and the policies of the FDA and
other agencies are completely inadequate to protect public health.
Could 'mad cow type epidemics happen in the US
John Stauber, talk given 7 May 00
*** CJD Foundation conference Miami Center for Media & Democracy
520 University Avenue, Suite #310 Madison, WI 53703
"Three years ago Sheldon Rampton and I were finishing our book Mad Cow USA
published in November of 97. In our introduction we wrote that ours is not a
biology book nor a diet guide, but a "book about politics and how it operates in
the real world. It explains how government officials have placed concerns for
the food industry over human health and welfare. In addition to telling the
story of an exotic, mysterious and frightening disease, we have written this
book to report on equally dangerous legal and political trends which threaten
not only our physical health, but also our fundamental democratic rights..." The
title of this session asks a question: "Could 'mad cow type epidemics happen in
the US.?" My answer is not only could they, but they are. Sheep scrapie arrived
in the US a half century ago and thanks to government bungling is now widely
spread throughout the US, with dozens of different strains. Chronic wasting
disease may have begun as sheep scrapie but now it is spreading through deer and
elk in western states and on game farms.
In the past two years two western state hunters under the age of 30 have
died of CJD, and some of us suspect they may be human victims of a new strain of
TSE in sheep, deer or elk that has begun claiming young human victims in the US.
Since 1964 researchers have suspected that transmissible mink
encephalopathy TME in the US is from a hidden TSE in cattle, and the late Dr.
Richard Marsh, to whom we dedicate our book, believed that he isolated that TSE
in mink in Wisconsin in 1985, a year before the British strain of TSE dubbed mad
cow disease was observed.
As our book was going to completion, we discovered that USDA inspectors
felt they had identified a TSE in pigs way back in the the 1970s, when few
researchers other than a small group in what Paul Brown calls the Club were very
concerned or aware of TSE diseases.
Of course, British mad cow disease and the looming specter of hundreds of
thousands of people condemned to death in the decades ahead has changed
everything. Everything, that is, except public policy here in the US. For
despite the fact that two Nobel prize winners in this area of research are from
the US, there is a public relations cover-up of massive proportions in this
country that is preventing us from effectively establishing public policies that
can monitor, prevent and eventually treat TSE diseases.
Please note that I said a public relations cover-up; that's important. I
suspect that if TSE diseases were spread by mosquitoes, we'd be spraying
pesticides all over the US to try and irradiate sheep scrapie and chronic
wasting disease. But these diseases are spread by agribusiness through animal
livestock products fed to people and animals, and thus instead of putting the
concerns of people first, we have see that consistently governments have put the
concerns of industry first.
We've heard eminent and hard working scientists talk about TSE diseases in
terms that dizzy the head of even other scientists. This is a very contentious
and mysterious area of research because TSE diseases break many rules.
Luckily, my co-author and I are not experts or scientists. However, we did
have the benefit of being able to interview top TSE researchers like Richard
Marsh and Dr. Gajdusek, have them review parts of our manuscript, and thus
deliver a book that is based on solid, sound science regarding what is known,
and what is unknown, what is proven, and what is not.
From my perspective, this is the most important point and perspective to
keep in mind:
For the past thirty years a massive unregulated experiment in creating new
strains of TSE disease has been undertaken by the livestock industry, and we're
the guinea pigs. The experiment is ongoing. The experiment began as a really
neat idea: lets take the billions and billions of pounds of slaughterhouse
waste, blood and offal that is produced every year from cattle and pigs and
sheep and roadkill and pets and zoo animals, and let's recycle it. Let's turn it
into protein fat supplements, and let's feed it back to the livestock we eat.
It seemed like a good idea. Unfortunately, what it didn't take into account
was the infectious prion agent. As Dr. Gibbs has pointed out, probably every
mammalian species has a TSE disease at some minute level: people, pigs, cows,
sheep, mink, cats and dogs, deer, elk. Grind up the most infectious parts of
millions and millions of animals, concentrate them into feed supplements, and
you are creating an environment not only for spreading and amplifying existing
agents such as scrapie in sheep, but also for creating an untold number of new
strains of TSE.
This is especially important to grasp. In laboratory experiments, when TME
or scrapie or CJD is injected into new animal species, whole new strains of TSE
are created, and they can have different potential to infect new host species.
The process of rendering animal waste into animal feed has been a massive and
ongoing experiment in the creation of new TSEs, one of which emerged in Britain
in the mid 1980s as mad cow disease, has clearly spread into humans claiming
over fifty so far, and in my guess will in the years ahead be founded to have
infected hundreds of thousands of Britons.
I said that this outbreak of BSE in Britain changed everything, but what
really changed everything was what caused that outbreak: feeding rendered animal
by-products back to animals as food.
I wish I could tell you that this practice has been stopped, but it has
not. I wish I could tell you that well informed and courageous officials in the
FDA, the USDA and the CDC have taken the mad bull by the horns, and are right
now executing a precautionary policy to insure that what has happened in Britain
does not happen in the US. Unfortunately, that is not the case.
Dr. Hansen will shortly explain why the much heralded FDA feed regulations
on rendered animal by-products announced in 1997 are in many ways a farce. For
instance, in the US calves are literally being weaned on cattle blood protein,
and scrapie infected sheep can be used as feed for pigs which can be used as
feed for cattle.
But the problem is beyond just the poor US animal feed regulations. You
need look no further than your favorite vitamin and nutritional supplement
stores. Right now, with the full knowledge of the FDA, the NIH, the CDC,
millions of Americans are popping over the counter as glandular supplements.
These unregulated products contain the most infectious parts of slaughtered
animals, the very parts that make up the so-called Specified Bovine Offal that
should be banned from cattle feed, such as the brain, pituitary, and glandular
system. These pills are pooled, collected from hundreds of thousands of animals
and taken daily by untold numbers of Americans, probably millions given the
popularity of supplements.
Again, this amounts to an unregulated human experiment, minus laboratory
controls or knowledgeable consent, feeding humans the most infectious parts of
animals, possible creating new TSEs by passaging animal TSE from pooled
glandular products.
I've brought along four different bottles of the pills I'm talking about,
and I've asked that they be pass around so you can look at them yourself and
read the label.
Some would say that FDA's hands are tied, that thanks to lobbying by the
nutritional supplement industry the FDA lacks the power to prevent sales of
these glandular. Actually this points to another frightening disease rampant
among otherwise good people who populate government agencies which I call BCD
for Bureaucratic Cowardice Disease. FDAs hands might be tied and they can't yank
these products from the market, but their mouths are not gagged and concerned
officials should be speaking out loudly warning the American public that they
are consuming animal brains and glands and might want to consider the potential
risks.
There is a reason why scientists in agencies and universities, not to
mention corporations, avoid sticking their necks out, and we need look no
further than Richard Marsh, a dedicated and conservative scientist who took it
upon himself to speak up in 1993 in an interview in Wisconsin's largest farm
paper. Richard Marsh warned Wisconsin farmers, thousands of whom were feeding
rendered cow by-products back to their cows, that given the mad cow outbreak in
Britain they should, despite the reassurances from USDA that such feeding was
safe, stop.
The day after that article appeared Dr. Marsh was literally called on the
carpet in the office of the dean of the school of agriculture at the University
of Wisconsin, and read the riot act. he was told that industry funders were
threaten to sue him, sue the school, cut off money for research, and who did he
think he was?
Within months the school scrambled and pulled together a symposium on the
subject, which served to dismiss and marginalize Dr. Marsh's views. When Dr.
Marsh died of cancer in 1997, after the British announcement that mad cow
disease was killing young people, the same University had the gall to praise Dr.
Marsh and his work as in the best tradition of the University. Some of us feel
that the stress and abuse heaped on Dr. Marsh> from 1993 to 1997 had an
impact on his health that contributed to his demise.
This is what happens to scientists who break ranks and speak out, and it is
a great loss to us today because I think if Dick Marsh was still alive he would
be leading the charge to confront the ignorance and the cow-towing to industry
that typifies this issue.
Dr. Marsh was a colleague and contemporary of many of the researchers in
this room. As Paul Brown whom he once worked with might say, he was a member of
the club. But Dr. Marsh was able to make realizations that I'm afraid most of
the club members haven't yet come to, and he was able to put his sense of
scientific obligation to society in front of concerns about his funding, or even
his personal and professional safety.
I first began investigating mad cow type diseases in the early 1990s when I
was working in Wisconsin organizing farmers and consumers opposed to Monsanto's
genetically engineered bovine growth hormone, the cattle equivalent of human
growth hormone which when injected into cattle forced them to produce more milk.
Well, its not quite that biologically simple as I'm sure any woman here who has
had children can imagine.
It was brought to my attention by a retired industry veterinary researcher
that in order to make the hormone work, cows need to be fed additional fat and
protein supplements, and that the cheapest supplements were rendered byproducts
from other cows. This veterinarian told me this was very bad because it was
exactly what had cause the outbreak of BSE in Britain.
I investigated and found this was true - massive feeding of cows to cows
was going on in the US, despite the fact that as early as the Fall of 1987
British epidemiology had shown that is was this practice that spread mad cow
disease.
I remember one of my first meetings with Dr. Marsh. We talked about his
believe that a US BSE agent, different than the British strain, was in cattle at
low levels and was the cause of occasional outbreaks of TME. I know that eminent
researchers in this room dispute that, but frankly Dr. Marsh never doubted it
and I think the evidence and commons sense remains in his favor.
I had just been to a holistic chiropractor for my chronically bad lower
back, and had been sold a bottle of adrenal gland extract which I showed to Dr.
Marsh - he practically fell out of his chair and I immediately stopped taking
them.
He was shocked to learn that such glandular were sold. Imagine what he
might think today, that at this late stage of the game with kids in their 20s in
the US dying of CJD, that these glandulars are sold without warning.
Of course, I may be taking a risk myself in saying this. After all, we are
in Florida, one of 13 states in the US in which the Animal Feed Industry
Association members have succeeded in lobbying into law a food product
disparagement bill. I could end up like Oprah Winfrey and her guest Howard
Lyman, forced to spend millions and millions of dollars (which in my case would
be thousands and thousands and then bankruptcy) to convince a jury that my
remarks today are based on sound science.
Having written two books in the past five years, one on political PR and
the other on the politics of food, I can tell you that a major reason why there
has not been more news media coverage of this issue is the multi-million dollar
PR campaign and litigation threats of the food industry which we document
extensively in our book.
I find it ironic that this weekend as we're meeting here President Clinton
announced Saturday a new initiative to address the safety of hot dogs. Philip
Brasher of the Associated Press speculates that as he is leaving office the
President wants to burnish his image with food safety initiatives.
The irony is that it is under this administration that the food industry
has launched an all out attack on our first amendment rights by lobbying food
libel laws onto the books, and the administration has remained silent. Despite
knowing of the risks of cow cannibalism since the beginning of his presidency,
his administration did nothing until 1997, and as we reveal in our book and Dr.
Hansen will explain, the regulations are severely inadequate.
I am circulating a letter being delivered by the Center for Food Safety
urging the agency to respond to the petition filed by that group and many of the
families in this room that calls on FDA to severely tighten its regulations.
Yet, probably to avoid publicity, the FDA stonewalls.
A couple weeks ago I was in Washington and I attended a conference that was
paid for in part by the lobbyists responsible for putting food censorships laws
on the books, the Animal Feed Industry Association and the law firm of Ollsen
Frank and Weeda that drafted the model bill that was then lobbied for at the
state level by the American Farm Bureau. I picked up this glossy brochure in
which the FDA is slapping itself on the back for its food safety initiatives. In
it I read that the FDA's BSE Educational Activities have Gained an award from
the vice president:
The US feed regulations and the FDA were honored with VP Al Gore's Hammer
Award. Dr. Stephen F. Sundlof, the directory of FDA's Center for Veterinary
Medicine, the man who since January 1999 has been unable to respond to the legal
petition to close gaping loopholes in the FDA regulations stated: "thanks to the
development of the BSE regulation, we can continue to say that there has not
been a single case of BSE reported in the United States. Educational efforts ...
will help assure that we can continue to make that claim."
In researching our books we had access to documents obtained through the
Freedom on Information Act. One 1991 document of the USDA was titled "BSE Public
Relations," and if someday attorneys in a class action lawsuit on behalf of CJD
patients in the US are looking for a smoking gun demonstrating that concerns for
industry were place over concerns for people, this is it.
(p.148-149) According to this document, the mere perception that BSE might
exist in the United States could have devastating effects on our domestic
markets for beef and dairy products." The report examined how the British
handled their PR, and it fretted over a story in the British magazine The
Economist which, quote, "could potentially create alarm among US consumers,"
unquote, because it reported that, quote,"many veterinarians and medical experts
have come around to the belief that humans could catch the mystery brain
disease."
This USDA PR document approvingly noted a quote in the Washington Post by
Dr. Joseph Gibbs at NIH in 1990 saying "I don't think there is any danger in
consuming British beef." Remember, this was in 1991. It had known for four years
that it was feeding cows to cows that was spreading BSE in Britain. More and
more were fearing that humans would die. Meanwhile, in the US, billions of
pounds of cow by-products were still being fed, in fact the amount was
increasing each year.
Was there no one inside the USDA or FDA who saw the lunacy, indeed the
criminality, in knowing this and letting cannibalistic feeding go one in the US?
Some did. The report notes that "some (USDA APHIS) staff members... argue that
because there is evidence that pigs, cats, mink, deer and a variety of
experimental animals may b e susceptible to trans. spongiform. encephalopathy,
the only prudent policy is to not feed products that contain these agents to ANY
SPECIES OF ANIMAL."
That's it. That's what we should have done then, and that's what we should
be doing now, but are not. Instead we are still exposing ourselves and US
livestock to a massive TSE experiment.
So, in 1991 the USDA and FDA rejected this advice by some anonymous
staffers, as they do today. This 1991 USDA PR report admitted that a more
cautious policy would be, quote, "to prohibit the feeding of sheep and
cattle-origin protein products to all ruminants, regardless of age. The
advantage of this option is that it minimized the risk of BSE. The disadvantage
is that the cost to the livestock and rendering industries would be
substantial."
In fact, absolutely nothing of substance was done until August of 1997,
when FDA announced its sham feed regulation, winner of the coveted Al Gore
hammer award, designed as a PR fig leaf so that FDA can say 'we are keeping
British mad cow disease out of our livestock."
Let me say that the only bright spot in this story of corporate and
government collusion, irresponsibility and censorship has been a few brave souls
like Richard Marsh, Richard Lacey in England, others who have bucked the tide
and spoken out, and those of you who are the families and loved ones of CJD
victims.
*** Through CJD Foundation and CJD Voice you have found each other, and you
will eventually force responsibility onto government and industry because if you
don't no one else will.
In conclusion, we need a massive commitment in the United States to address
TSE diseases. We need to start by admitting the shortcomings of our failed
federal feed policies and change them. We need to dedicate hundreds of millions
of research dollars, if necessary, to try to eradicate scrapie in sheep,
eradicate CWD in deer and in elk, and investigate, research, test and monitor
for TSEs in humans and animals. We need money for research to develop
treatments, because while this disease used to be rare disease, I suspect we are
about to see it emerge full blown in Britain with hundreds of thousands of
victims, and more and younger victims appearing in the United States, as a
result of our thirty year experiment in animal cannibalism.
The leadership for this will not come from politicians in bed with the
agribusiness industry. It will not come from researchers who while brilliant are
stuck back in pre-BSE days, still viewing this as a rare unusual disorder. It
will come from average citizens who husbands and mothers and children are dying
of this bizarre class of dementia diseases in increasing numbers, and who are
not afraid to demand that the right policies be implemented and funded. It is
upon your shoulders that leadership falls, and so far you are doing admirably."
snip...please see this and more here ;
Friday, October 23, 2015
CJD FOUNDATION CREUTZFELDT JAKOB DISEASE TSE PRION QUESTIONNAIRE UPDATE
OCTOBER 2015
Friday, November 13, 2015
No evidence of asymptomatic variant CJD infection in immunodeficiency
patients treated with UK-sourced immunoglobulin ?
posted by Terry S. Singeltary Sr. | 11:31 AM
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