VARIANT CJD (vCJD) or nvCJD

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Location: BACLIFF, Texas, United States

My mother was murdered by what I call corporate and political homicide i.e. FOR PROFIT! she died from a rare phenotype of CJD i.e. the Heidenhain Variant of Creutzfeldt Jakob Disease i.e. sporadic, simply meaning from unknown route and source. I have simply been trying to validate her death DOD 12/14/97 with the truth. There is a route, and there is a source. There are many here in the USA. WE must make CJD and all human TSE, of all age groups 'reportable' Nationally and Internationally, with a written CJD questionnaire asking real questions pertaining to route and source of this agent. Friendly fire has the potential to play a huge role in the continued transmission of this agent via the medical, dental, and surgical arena. We must not flounder any longer. ...TSS

Wednesday, July 23, 2014

After the storm? UK blood safety and the risk of variant Creutzfeldt-Jakob Disease

After the storm? UK blood safety and the risk of variant Creutzfeldt-Jakob Disease
 
18 July 2014 .
 
The Committee’s Second Report of Session 2014–15, After the storm? UK blood safety and the risk of variant Creutzfeldt-Jakob Disease, will be published on Thursday 24 July at 00.01 am (HC 327). Electronic embargoed copies of the Report will be available from 9.30 am on Monday 21 July.
 
Inquiry: Blood, tissue and organ screening Science and Technology Committee
 
Copies of the embargoed Report will be available to media representatives and witnesses only on request. Copies of the Report will be available in the following ways on publication: The Reports will be sent by post to all witnesses or, if preferred, may be collected from 14 Tothill Street. Copies of the Report may be obtained from the usual outlets, including The Parliamentary Bookshop and The Stationery Office. The Report will be posted on the Parliamentary internet on publication, and will be accessible via the Committee’s homepage.
 
 
EMBARGOED ADVANCE COPY Not to be published in full, or in part, in any form before 00.01am on Thursday 24 July 2014 HC 327
 
House of Commons Science and Technology Committee
 
After the storm?
 
UK blood safety and the risk of variant Creutzfeldt-Jakob Disease
 
Second Report of Session 2014–15
 
EMBARGOED ADVANCE COPY Not to be published in full, or in part, in any form before 00.01am on Thursday 24 July 2014
 
Summary
 
In the late 1990s, few diseases were as high profile, or as poorly understood, as variant Creutzfeldt-Jakob Disease (vCJD): the ‘human form’ of bovine spongiform encephalopathy (BSE). Invariably fatal and seemingly impossible to control, vCJD was an unusually enigmatic threat, leading prominent figures to warn of hundreds, even thousands of potential deaths, prompting widespread speculation that the handful of cases seen at the time were merely the tip of the iceberg.
 
Twenty years on, the feared epidemic has not materialised and vCJD has, to an extent, slipped from public consciousness. However, there remains much that we do not understand about vCJD and little to suggest that it should be dismissed as a threat. While cases of vCJD are now rare, recent studies indicate that tens of thousands of people in the UK might be ‘silent’ carriers of the prions responsible for the disease and could perhaps transmit those prions to others. The most likely form of onward transmission is through blood transfusion. Cases of transfusion-transmitted vCJD are known to have occurred in the past, and, while it remains to be seen whether or not widespread transmission via the blood supply is probable, evidence suggests that it is possible. In the absence of a validated test capable of detecting the presence of prions in blood, we simply cannot know how significant a threat to public health vCJD might be.
 
The Government acknowledges this risk and claims that, like its predecessors, it has taken a precautionary stance in response. However, while administrations in the late 1990s assumed the worst and took steps to prevent it from happening, the Government recently appears to have adopted a more optimistic approach in which the low incidence of identified cases of vCJD is used as justification for inaction. This is particularly evident in the Government’s less than enthusiastic response to emerging vCJD risk mitigation technologies such as prion filtration and the prototype vCJD blood assay recently developed by the MRC Prion Unit.
 
In this report we remind the Government that no evidence of harm is not the same as evidence of no harm. Cases of vCJD appear to be falling but, given the level of uncertainty regarding the potential for blood-borne transmission, precaution must remain the guiding principle in decision-making. Research intended to reduce this uncertainty should be pursued as a priority and, in the meantime, measures to reduce the risk of blood-borne transmission should be strengthened wherever possible.
 
The Government’s casual attitude to vCJD transmission is not confined to blood transfusion: it is also evident in its response to the risk of surgical transmission. It is known that classical CJD can be transmitted via contaminated surgical instruments and there is reason to believe that vCJD may also be transmissible via this route; however, development of a commercial technology capable of eliminating this risk has ceased in the absence of Government support and as a result of the NHS’s apparent lack of appetite for such technology. Without a technological solution, we cannot be confident that CJD is not being transmitted through surgery and we are disturbed by the Government’s apparent lack of concern about this issue.
 
Failure to adequately mitigate these risks means that some people have inadvertently been exposed to CJD or vCJD and may be at increased risk of developing the disease. This inquiry has exposed deficiencies in the level of support provided to these individuals and the system of surveillance through which they are monitored; both of which, in many cases, have effectively been outsourced. We consider this arrangement to be unacceptable and urge the Government to take greater care of, and responsibility for, those who have been accidentally exposed to CJD or vCJD. We were also disappointed to find that so few ‘at risk’ individuals have been asked for their consent to participate in research and recommend that the Government takes immediate steps to remedy this situation.
 
At the conclusion of this inquiry we are unconvinced that the Government has done all that it potentially could do to ensure that the UK blood supply is, and continues to be, free of dangerous pathogens. We therefore conclude by recommending that the Government commission a full assessment of the key risks, known and unknown, that the UK blood supply currently faces and might face in the future, so that it can identify and fill relevant knowledge gaps and support the development of appropriate risk reduction measures and technologies.
 
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Surgical transmission of prions
 
27. Blood transfusions are not the only source of secondary prion infection; transmission can also occur via other forms of medical intervention, notably surgery. The prions thought to be responsible for both classical and variant forms of CJD are known to be present in parts of the body that are accessed during surgical procedures.79 According to Professor John Collinge, MRC Prion Unit, prions are known to “stick very avidly to metal surfaces”, meaning that contaminated surgical instruments could potentially act as “a very efficient route” of person-to-person prion transmission.80 This is more than just a theoretical risk: Professor Richard Knight, Director of the National CJD Research and Surveillance Unit, confirmed that “a handful” of cases of classical CJD appeared to have been transmitted in this way.81 Professor Collinge added that there was “epidemiological evidence from several countries now that patients developing classical CJD are more likely to have had abdominal surgery beforehand”, suggesting a potential link between the procedure and the disease.82 Professor Collinge also considered it possible that some cases of vCJD had“been related to” surgical exposure, but members of the Department of Health’s Decontamination Science Working Group stated that these concerns were “exaggerated”.83 To date, there have been no cases in which it has been conclusively demonstrated that vCJD has been transmitted via surgery, although scientific evidence suggests that this would be possible.84
 
28. Speaking on behalf of the Government, Chief Medical Officer Dame Sally Davies, stated that she was “concerned about the transmission of disease” via surgical instruments and claimed that the Government had applied the precautionary principle in its management of this risk.85 The Government highlighted two key steps that it had taken:
 
• Since the mid-1990s, the Advisory Committee on Dangerous Pathogens (ACDP) has issued guidance on “the decontamination, quarantining and appropriate use of surgical equipment (including endoscopes), and on pre-surgical assessment of patients to identify and act on those with, or at risk of, all forms of human prion disease”.86
 
• In 2006, the National Institute for Health and Care Excellence (NICE) issued guidance on “patient safety and reduction of risk of transmission” of CJD via surgical procedures.87 This made several suggestions relating to the management and tracking of surgical instruments and recommended the use of new, unused instruments for certain groups, such as children undergoing high-risk procedures.
 
We did not receive any evidence on current levels of compliance with the ACDP guidance but, according to NICE, following publication of its 2006 guidance,the Department of Health became aware that implementation “had not proceeded satisfactorily”.88 A number of activities took place to address this and in 2008 NICE published additional resources to aid implementation, including “a checklist for acute Trusts to self-assess current practice against the guidance”.89NICE does not perform implementation audits for this type of guidance. However, a 2011 academic study examining decontamination procedures across a sample of NHS centres found that the guidance had only been “fully implemented” in ten (19%) of the organisations audited.90 Dame Sally stressed the importance of NICE’s “significant” guidance and stated that she was “not aware” that it had not been fully implemented and would consider it “unacceptable” if this were the case.91
 
29. The Government has acknowledged that contaminated surgical instruments are a potential source of prion transmission and states that it has taken a precautionary approach in its response to this risk. However, this response appears to rest heavily on guidance which, based on the available evidence, may not have been fully implemented.We recommend that the Government work with the National Institute of Health and Care Excellence (NICE) and the Advisory Committee on Dangerous Pathogens to better understand the extent to which the precautions recommended by these bodies have been implemented across the NHS. We ask the Government to provide us with an update on this work well before the dissolution of Parliament, together with an indication of the steps it will take if preliminary findings suggest that implementation has been incomplete.
 
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3 Technology evaluation and the role of the scientific gatekeeper
 
30. Given the risk posed by prion transmission and the inability of existing measures to fully mitigate this risk, efforts are continuing, both in the public and private sectors, to develop new technologies for prion detection, inactivation and removal. The primary customers for these technologies areUK Blood Services and the NHS, access to both of which is typically mediated by one of several scientific bodies responsible for assessing the evidence to support technology adoption. Through the discussion of three case studies, this chapter examinesthe Government’s approach to the evaluation of vCJD risk mitigation technologies, with particular focus on the role played by these scientific gatekeepers.
 
Case study 1: decontamination of surgical instruments
 
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37. Given the NHS’s resistance to change and the well-documented challenges associated with initiating a UK clinical trial, the Minister’s assessment that “no barriers” were put in the way of DuPont’s prion inactivation product does not reflect the reality of the situation. Where technologies are developed in direct response to Government need—and on the back of Government funding—the Government must be prepared to take steps to help companies overcome barriers to adoption. We ask the Government to set out how, in future, it will ensure that the directed research that it funds is better supported through the technology readiness pathway. In particular, we ask the Government to set out how it will ensure that promising clinical technologies are promptly trial led in an NHS setting, so that potential adoption challenges can be quickly identified and resolved.
 
38. We also question the value of a scientific review panel which has no mandate or power to ensure that the products that it recommends can be tested in, and eventually adopted by, the NHS. We see this as further evidence of the Government’s passive approach to technology uptake.We propose that the Rapid Review Panel (RRP) be given stronger powers to ensure that its recommendations open the door to in-use evaluation and stimulateNHS uptake.
 
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40. In our view, all Scientific Advisory Committees should adhere to both the 2010 ‘Principles of Scientific Advice to Government’ and the 2011 ‘Code of Practice for Scientific Advisory Committees’. We were disappointed to find that the Rapid Review Panel (RRP)failed to do so. We recommend that the Chief Medical Officer takes action to rectify current weaknesses. We request a progress report be sent to us well before the dissolution of Parliament.
 
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Case study 2: prion filtration
 
The technology: ProMetic’s P-Capt prion filter
 
41. Prion filtration is a process through which prions are physically removed from blood through the use of highly specific resin ligands, in order to “provide increased protection against the transmission of vCJD via blood and blood-derived products”.130 One group heavily involved in the development of this technology is the UK-based company ProMetic BioSciences (“ProMetic”). In 2002, ProMetic established a joint venture with the American Red Cross aimed at developing materials “with the ability to capture and remove prion proteins from a wide variety of biological source materials including blood, red cells, plasma and plasma proteins”.131 Four years later, following what ProMetic termed “extensive performance and safety testing”,the P-Capt prion filtration device obtained its CE mark,132 making it “the world’s first prion-filtration product acknowledged to increase the safety of red blood cell concentrate”.133 At this point, the product became subject to further scientific evaluation, led by the Advisory Committee on the Safety of Blood, Tissues and Organs (SaBTO).
 
The gatekeeper: the Advisory Committee on the Safety of Blood, Tissues and Organs
 
42. SaBTO is an independent scientific advisory committee responsible for advising “UK ministers and health departments”on “the most appropriate ways to ensure the safety of blood, cells, tissues and organs for transfusion/transplantation”.134 As part of its remit, SaBTO is specifically tasked with considering the “cost-effectiveness of interventions, including the introduction of new safety measures” such as prion filtration.135
 
43. In 2006, SaBTO initiated its evaluation of ProMetic’s P-Capt device.136 This consisted of three stages:
 
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45. We do not wish to question the scientific decision-makingof the Advisory Committee on the Safety of Blood, Tissues and Organs (SaBTO) and we respect its decision not to recommend adoption of prion filtration at present.However, we feel that the time taken to reach this decision was excessive and that the process, particularly in its latter stages, entailed an unnecessary level of uncertainty for the commercial developer. We have some sympathy for SaBTO’s desire to wait until more evidence was available before making a decision; however, if industry is to continue to develop innovative blood safety products for the UK market, SaBTO must introduce greater speed and predictability into its evaluation process. We recommend that, in future, when assessing a new technology, SaBTO agree with stakeholders at the outset what the evaluation will consist of, together with key dates, milestones and decision points. This ‘evaluation roadmap’, and any subsequent amendments, should be made publicly available to ensure maximum transparency and accountability.
 
46. We also consider it important that the health technology appraisals conducted by SaBTO—and all other SACs—use the same methodology and meet the same high standards as those undertaken by the UK’s centre of excellence for this activity: NICE. We therefore recommend that the Government Office for Science work with NICE over the next 12 months to develop and publish a standard methodology for all SACs tasked with conducting health technology appraisal.Until this guidance is published, we recommend that a NICE representative review and, where necessary, provide input to all such appraisals undertaken by, and on behalf of, SACs.
 
SaBTO’s relationship with Government
 
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50. Scientific Advisory Committees should be—and be seen to be—independent of the bodies to which they are providing advice. At present, the Advisory Committee on the Safety of Blood, Tissues and Organs (SaBTO) comprises members who are both contributing to, and acting on, the advice that it formulates. We consider that this could be damaging to its perceived independence and a source of potential conflicts of interest. We recommend that SaBTO’s terms of reference be amended to reflect the fact that it does, in effect, provide advice to UK Blood Services as well as the Government. We suggest that SaBTO’s current membership be reviewed and potentially revised in light of this change.
 
Case study 3: vCJD blood testing
 
The need for a vCJD blood test
 
51. A key strand in UK Blood Services’ strategy for preventing transfusion-transmitted infections is the use of blood tests to enable those donations carrying known pathogens to be identified and discarded.161 Unfortunately, this is not currently a viable strategy for mitigating the risk of vCJD transmission because no suitable high-throughput test currently exists. Witnesses were unanimous in their support for the development of such a test. Professor Sheila Bird, MRC Biostatistics Unit, expressed concern that the absence of a vCJD blood test meant that we could not protect the blood supply from prions in the same way that we can protect it from other pathogens, such as hepatitis B and C and HIV, and stated that development of a validated test should “undoubtedly” be a research priority.162 Professor Richard Knight, Director of the National CJD Research and Surveillance Unit (‘the surveillance unit’), agreed that development of a test was “extraordinarily important” and “would be a great boon in all sorts of ways”.163In addition to its potential screening applications, witnesses highlighted the role that a blood test could play in providing certainty to patients thought to be at risk of vCJD.Joseph Peaty, Tainted Blood, told us that, some years ago, it had “looked very much” as though he was suffering from the early signs of vCJD.164 He explained:
 
It would have been incredibly helpful if we had had access to [a test] at that point to identify, “Is this the onset of variant CJD, or is it where these viruses overlap and you’ve got HIV? Perhaps the medication, or perhaps hepatitis C, is affecting the brain in some way.” I had to go through brain scans and vigilance for a number of months. I had insomnia, where I hardly slept for three months. I was incredibly depressed and anxious.165 The Government did not explicitly state its support for the development of this technology but acknowledged that a test “may be advantageous”.166
 
52. The number of research groups working to develop a vCJD blood test has fallen in recent years. According to Professor Marc Turner, Advisory Committee on the Safety of Blood, Tissues and Organs (SaBTO), “looking back a decade or so ago, there were probably […] a dozen or more different research groups and commercial companies working” in this area. Now, however, “there are really only two or three”.167 Professor Turner stated that the “most advanced test by far” was the one currently being developed by the MRC Prion Unit, a publicly-funded research group led by Professor John Collinge.168 In addition, Prionics AG, a Swiss company, has continued to conduct work in this area, as has the Scottish National Blood Transfusion Service, working in partnership with the national surveillance unit.169
 
The technology: the Prionics blood test
 
53. Prionics AGis a developer of diagnostic tests for major livestock diseases.170 In 2001, when surveillance programs for BSE became mandatory in the European Union, Prionics “pioneered” the use of in-situ rapid diagnostic tests and, today, the company continues to develop diagnostic tools for prion diseases such as vCJD.171 According to Prionics, it has made a “significant investment” in this area, spending “€5 million to €10 million” on the development of prototype vCJD blood tests since 2002.172 In 2009, NHS Blood and Transplant (NHSBT) issued a tender for the development of a diagnostic test for use in vCJD blood screening. Following a successful bid, Prionics was awarded a framework contract pending further evaluation of its test by the National Institute of Biological Standards and Controls (NIBSC), the body tasked with maintaining and managing the distribution of rare vCJD blood samples.173
 
The gatekeeper: the National Institute of Biological Standards and Controls 54. The National Institute of Biological Standards and Controls (NIBSC) is a body of the Medicines and Healthcare Products Regulatory Agency, an Executive Agency of the Department of Health.174 It hosts the CJD Resource Centre, which exists to “help research scientists obtain characterised materials for studying and developing diagnostic tests” for all forms of CJD.175
 
55. In order to develop a diagnostic blood test, it is necessary for researchers to have access to blood samples from people who have suffered from the target infection. In the case of common blood-borne pathogens such as hepatitis B and HIV, such samples can be easily obtained. However, because vCJD is such a rare disease, patient samples are extremely scarce.176 In the UK, the majority of samples from confirmed vCJD cases are initially collected and stored at either the surveillance unit or the MRC Prion Unit.177 According to the NIBSC, following requests for access to these samples from several test developers in the mid-2000s, the Government concluded that access should be “controlled” and only granted to those developers whose tests were most likely to be successful.178In 2007,an Oversight Committee was established within the CJD Resource Centre to “perform evaluations” of prototype tests and “manage the distribution of samples” according to a standard protocol.179 According to Dame Sally Davies, Chief Medical Officer, the NIBSC currently holds samples from 16 individual vCJD patients: equivalent to approximately “one and a half tablespoons” of blood.180
 
56. In order to gain access to these samples, test developers require NIBSC approval. However, according to the NIBSC, “it was agreed at the start of the [CJD Resource] Centre’s existence” that the two primary centres of UK prion research—the national surveillance unit and the MRC Prion Unit—should be exempt from this process in order to avoid “unreasonably” restricting their research work.181 Additional samples are therefore currently held and used by these units and, on occasion, are provided directly to other test developers without recourse to the NIBSC evaluation process.182
 
57. Several witnesses expressed concern about the way in which access to vCJD samples was controlled in the UK. Christine Lord, mother of vCJD victim Andrew Black, pointed out that the Government held “all the keys” to vCJD test development and claimed that a “few select scientists and Government officials” held “a monopoly” over this research area.183 Mrs Lord added that relatives of victims had been “thwarted and blocked” in their attempts to share blood samples with foreign research groups.184 Dr Alex Raeber, Head of Research and Development at Prionics AG, agreed that, “as a foreign company”, Prionics was “not treated in the same way as other stakeholders” and had faced “big challenges” in obtaining access to samples.185 According to Dr Raeber, while the NIBSC had done “an excellent job” in setting up the test validation process, the number of samples made available through this process was “very limited”.186 Prionics’ test was evaluated on the basis of two samples from known vCJD patients and, on the basis of this evaluation, was deemed “not sufficiently fit for purpose”.187 The test was never used by UK Blood Services.
 
58. Dr Raeber criticised this evaluation process, stating that it was “really not adequate” for the NIBSC to validate the efficacy of his company’s test on the basis of only two samples188, particularly given that there was no guarantee that prions were present in these particular samples.189 Professor Sheila Bird, MRC Biostatistics Unit, agreed that the statistical significance of this evaluation was questionable and pointed out that “provision of fewer than five or six vCJD samples within a blind panel of 500” was an “inadequate—or very harsh” statistical assessment to which to submit a prototype test.190 In contrast, the test developed by the MRC Prion Unit (discussed below) has so far been validated on the basis of 21 samples from known vCJD cases, all sourced directly from its own collection of patient samples.191 In response to these criticisms, the NIBSC stated that its process was “open to all” and that, in fact, “most interactions” had been with non-UK developers rather than UK companies.192 It acknowledged that it was “not ideal that only two samples were made available” to Prionics, but stressed that this decision was made only after “substantial discussion in the Oversight Committee”.193
 
59. We understand the need to carefully control access to rare vCJD samples and commend the National Institute of Biological Standards and Controls (NIBSC) for putting in place a standard protocol for test validation. However, we are disappointed that so few samples are currently held by the NIBSC and consider its process to be undermined by the fact that the two major centres of UK prion research—the National CJD Research and Surveillance Unit and the MRC Prion Unit—can each use and distribute samples independent of NIBSC evaluation. All test developers should be given equal opportunity to gain access to the available samples and these should be distributed on the basis of merit alone. We recommend that access to all vCJD patient samples— including those currently held elsewhere in the UK—be managed through the NIBSC, according to a consistent set of test validation protocols.
 
60. We were also concerned by the apparent statistical weakness of past NIBSC evaluations. We recommend that the CJD Resource Centre Oversight Committee add to its membership an individual with expertise in biostatistics, who can provide it with expert advice on this matter during future deliberations.
 
The technology: the MRC Prion Unit blood test
 
61. The MRC Prion Unit was established in 1998 and is located at the UCL Institute of Neurology.194It was formed “to provide a national centre of excellence with all necessary facilities to pursue a major long-term research strategy in prion and related diseases”.195The Unit undertakes research across a wide-range of topics and aims to “seamlessly combine basic (laboratory) and clinical (patient-based) research” in order to enable “better early diagnosis, prevention, and effective treatment” of prion disease.196 It receives approximately £6 million per year from the Medical Research Council and is led by John Collinge, Professor of Neurology and Head of the Department of Neurodegenerative Disease at the UCL Institute of Neurology.197
 
62. In February 2011, the Unit announced that it had developed a prototype blood test capable of detecting “blood spiked with a dilution of vCJD to within one part per ten billion—100,000 times more sensitive than any other method developed so far”.198In this study, the prototype test returned no false positives from 100 control samples and accurately identified 15 of 21 samples taken from known vCJD patientsas positive, indicating that the test was 100% specific and approximately 70% sensitive.199In a larger follow-up study published in early 2014, the prototype was tested on 5,000 control samples (from US citizens considered not to have been exposed to BSE) and a subset of the vCJD samples previously used in the 2011 study. It again demonstrated 100% specificity and 70% sensitivity.200
 
63. Professor Collinge stated that the next logical step in the test’s development would be to carry out a larger ‘population prevalence’ study in which the prototype would be used to test 20,000 UK blood samples and 20,000 US blood samples, at an estimated cost of £750,000.201 According to Professor Collinge, if, during this study, the test returned positive results only from UK samples, two things could be concluded:
 
One is that our test is capable of detecting [vCJD] carriers, which we don’t formally know yet: we have simply looked at [vCJD] patients. Secondly, we would have confirmed that there is, indeed, a problem in the British donor core. In our view, that piece of research is required to make the case to progress that test further.202
 
A proposal for this study was considered by the MRC in March 2013, but was rejected, in part because of the test’s “low level of sensitivity”.203 According to the MRC: the Unit was advised to consider ways to improve the test sensitivity to provide greater confidence of identification of infected people, in order to make the test more accurate for prevalence studies and more attractive for development into a screening test.204
 
Professor Collinge disputed the MRC’s decision, claiming that the recommended steps constituted “test development work” which lay outside of his unit’s area of expertise.205 He added that,in the view of his Unit’s “statistical advisers”, the test’s sensitivity was “perfectly adequate to do the study that we propose to do” and that it may not be possible to further increase sensitivity because “it could be that only 70% of people with vCJD have prions in their blood”.206 Professor Collinge also highlighted that feedback from diagnostics companies was “very much” that they wanted to see the results of a larger study “before thinking about whether they would help us to take [the test] any further”—a view confirmed by several industry representatives.207
 
64. Expert witnesses strongly supported Professor Collinge’s proposal for a UK blood prevalence study; indeed, Dr Simon Mead, Association of British Neurologists, stated that there now appeared to be “scientific consensus” on this matter.208 Professor Marc Turner, SaBTO, agreed with Professor Collinge that the test’s sensitivity was “pretty good” and considered a blood prevalence study to be “the next logical step” in its development, while Dr Roland Salmon, Acting Chair of the Advisory Committee on Dangerous Pathogens, considered there to be “a great deal of scope” for the test to be used for research purposes in its current state.209 Dr Lorna Williamson, NHS Blood and Transplant (NHSBT), took a similar view:
 
I think we are all in agreement that the next step, if there were a medium throughput test available, would be to conduct a study of the UK population using blood samples to understand what the frequency of prion infection in the blood actually is.210
 
The Government, however, stated that there were “currently no tests suitable” for this purpose and was non-committal in its support for further test development work.211 Dame Sally Davies, Chief Medical Officer, stressed that the Government had “limited budgets for healthcare, public health and research” and that it had previously “given a lot of money to this area of prion research, particularly to Professor Collinge”.212 The Minister said that she was “open-minded to receiving advice” on this matter, but added that she was “pretty satisfied that, proportionate particularly to the number of cases and deaths over the last 10 years or so, there is a good body of work going on at the moment”.213
 
65. The incubation period of prion diseases such as vCJD can extend to several decades and it is therefore possible that individuals infected in the 1990s might not yet have developed symptoms. We do not follow the Minister’s logic that there should be a link between the number of cases seen in the last ten years and the level of resource dedicated to prion research. We simply do not know, at present, how many people have been exposed to prions and what the implications of this might be for the blood donor pool. There is an urgent need to reduce this uncertainty.
 
66. Based on the testimony that we have heard,we considerthat a vCJD blood prevalence study utilising a version of the prototype test developed by the MRC Prion Unit would be of considerable value, both for test development and research purposes. We recognise that significant public funds have already been directed towards the development of this test; we view this as even more reason to ensure that a return on this investment is realised. To cut off support now would be a false economy. Werecommend that the Government ensures that a large-scale vCJD blood prevalence study be initiated in the UK within the next 12 months.
 
4 CJD risk management and surveillance
 
CJD risk management and ‘at risk’ individuals
 
67. Both classical and variant forms of CJD214 are relatively rare and precautions are in place to prevent those known to be suffering from the disease from passing it on to others. However, CJD’s long incubation period—that is, the time between infection and the onset of symptoms—means that people could unknowingly carry the disease for many years before symptoms appear. During this time, they could participate in procedures which risk exposing others.215 To date, in the UK, over 6,000 people have been identified as being at increased risk of CJD as a result of this type of retrospectively recognised secondary exposure.216 Public Health England (PHE) divides these people into two groups:
 
• “individuals with a known link to a clinical case of vCJD (through donation or receipt of blood or blood products, receipt of certain pooled plasma products or following surgical exposure); and
 
• groups of individuals, not linked directly to a clinical case but who, on the basis of a risk assessment, are defined as likely to have been exposed to a high enough risk of exposure through their treatment with blood or plasma products to inform them about this risk, where possible, and to recommend that public health precautions concerning blood, tissues, organs and surgery are followed”.217 (These precautions are detailed in box 1.) Incidents leading to further additions to the ‘at risk’ list continue to occur and, until its dissolution in March 2013, were managed under the advice of the CJD Incidents Panel, a scientific advisory committee with expertise in CJD risk management.218 According to PHE, between January 2010 and March 2013, the CJD Incidents Panel was notified of 43 ‘CJD incidents’ and 70 lower-risk ‘CJD reports’.219
 
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Notification of ‘at risk’ individuals
 
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Nevertheless, the Government’s Chief Medical Officer, Dame Sally Davies, indicated that she was “confident” that local CJD management and reporting structures were robust and that ‘at risk’ individuals were receiving the necessary support.231
 
70. People who are notified that they may have been exposed to CJD will inevitably be alarmed by this information and will likely have questions that cannot be answered in the leaflets currently provided by Public Health England. We consider it totally inappropriate for this news to be communicated solely in writing. We recommend that the Government put robust measures in place to ensure that all individuals assigned this designation receive the news verbally, either from a healthcare provider or from a CJD specialist with experience in patient communication.
 
The impact of ‘at risk’ notification
 
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73. It is clear that the prototype vCJD blood test developed by the MRC Prion Unit cannot yet be relied upon for universal screening purposes. However, it could be of significant value to those people who have been notified that they are at increased risk of carrying the disease. Until the implications of a negative test result can be more firmly established, current precautions must remain in place for those considered to be ‘at risk’ of vCJD. However, the results of an imperfect test may provide comfort to some. We therefore recommend that‘at risk’ individuals be given the opportunity to participate in the blood prevalence study recommended in paragraph 66.
 
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CJD surveillance
 
snip...
 
77. The Government claims to be undertaking close surveillance of those it considers to be ‘at risk’ of CJD. Yet it cannot provide reliable data either on the total number of people designated ‘at risk’ or the number who have been notified of this fact. This is unacceptable. We recommend that the Government conduct an immediate audit of the entire ‘at risk’ cohort to establish whether any notifications remain outstanding and to ensure that appropriate support and follow-up is in place for all those affected.We also propose that the Government commission an independent review of the transfusion data pathway to ensure that, in the event of any future blood contamination incident, it can promptly trace, notify and provide support to affected recipients. 78. We were disappointed by the evident lack of support provided to those designated ‘at risk’ of CJD. We consider it inappropriate for the Government to have effectively delegated responsibility for the care and surveillance of a large proportion of these individuals to external bodies such as the UK Haemophilia Centre Doctors' Organisation—a charitable organisation with no formal relationship with the Executive. We recommend that the Government, through its public health agencies, assume direct responsibility for the surveillance and support of all those considered to be ‘at risk’ of CJD, with input from other specialist organisations as required.
 
snip...
 
Participation in research
 
snip...
 
81. In our view, the decision to participate in research should always rest with the individual or, in exceptional circumstances, their loved ones. Nevertheless, samples contributed by those potentially exposed to CJD are of immense scientific value and we are disappointed that more has not been done to obtain consent from those willing to participate in research. We recommend that the Government consider ways to increase the number of ‘at risk’ individuals giving consent for research participation, particularly post-mortem. We ask that the Government summarise its plans for achieving this in its response to this Report.
 
The National CJD Research and Surveillance Unit
 
Classification and reporting
 
snip...
 
87. We are confident in the integrity of the National CJD Research and Surveillance Unit and have not seen any evidence to corroborate claims of deliberate underreporting or misclassification. However, we share our witnesses’ concerns that cases could be missed due to misdiagnosis, particularly in the elderly. We recommend that the Government lend its support to research intended to give greater clarity over the causes of atypical dementiain the elderly and, through this, the potential rate of undiagnosed CJD.
 
snip...
 
5 After the storm?
 
snip...
 
94. SaBTO’s decision not to recommend the adoption of prion filtration, taken alongside the other evidence that we have gathered during this inquiry, in our view signals a change from what was a genuinely precautionary approach to vCJD risk reduction in the late 1990s to a far more relaxed approach today. Much of the uncertainty surrounding prions, their potential modes of transmission and the possible rate of undetected infection and disease remains: recent evidence that subclinical prevalence could be as high as one in 2,000 people would suggest that a precautionary approach is now more warranted than ever.
 
95. Our fearis that the Government’s current attitude is driven less by the available scientific evidence than it is by optimism: a hope that the storm has now passed and that vCJD is no longer the threat to public health that it once was. In the current economic environment, this attitude is not surprising. However, it is not justified. For all we know, the storm may well be ongoing. We conclude this report by recommending that the Government take a more precautionary approach to both vCJD risk mitigation and blood safety more generally, in order to safeguard against future infections. We suggest that it begin by assessing the key risks, known and unknown, that the UK blood supply currently faces and might face in the future, so that it can identify and fill relevant knowledge gaps and support the development of appropriate risk reduction measures and technologies. The Government should initiate this work immediately and we ask that it provide us with an update on its progress well before the dissolution of Parliament.
 
Conclusions and recommendations
 
Risks to the UK blood supply
 
1. Blood transfusions save lives and we should be proud, as a nation, of our long tradition of altruistic donation. In recent years, the UK blood supply has proved to be extremely safe and, in the vast majority of cases, the benefits of receiving a transfusion will far outweigh the risk of acquiring a transfusion-transmitted infection. However, we urge against complacency and stress the need for UK Blood Services to remain vigilant to the threat posed by blood-borne pathogens. (Paragraph 9)
 
2. The evidence that we have heard suggests that we cannot be confident that prions are not present in the blood supply. There remains considerable uncertainty about the potential implications of such contamination. We consider it imperative that a precautionary approach to this risk be maintained until further evidence becomes available. (Paragraph 17)
 
3. We echo concerns that population-level risk assessment could lead to inaccurate and potentially discriminatory judgements being made about the risk posed by individuals, particularly men who have sex with men. We recommend that the Advisory Committee on the Safety of Blood, Tissues and Organs (SaBTO) reconsider the feasibility of a move to more individualised risk assessment as part of its 2015 work programme, following completion of the current UK blood donor survey. (Paragraph 22)
 
4. Pathogens are constantly emerging and evolving; novel pathogens will therefore always pose a threat to the blood supply. In the past, it has often taken multiple cases of transfusion-transmitted infection before these threats have been recognised and mitigated. This will remain the case as long as risk mitigation measures remain pathogen-specific. We urge the Government to take steps to support the development of broader spectrum technologies with the potential to mitigate the risk of both known and unknown pathogens. (Paragraph 26)
 
Surgical transmission of prions
 
5. The Government has acknowledged that contaminated surgical instruments are a potential source of prion transmission and states that it has taken a precautionary approach in its response to this risk. However, this response appears to rest heavily on guidance which, based on the available evidence, may not have been fully implemented. We recommend that the Government work with the National Institute of Health and Care Excellence (NICE) and the Advisory Committee on Dangerous Pathogens to better understand the extent to which the precautions recommended by these bodies have been implemented across the NHS. We ask the Government to provide us with an update on this work well before the dissolution of Parliament, together with an indication of the steps it will take if preliminary findings suggest that implementation has been incomplete. (Paragraph 29)
 
52After the storm? UK blood safety and the risk of variant Creutzfeldt-Jakob Disease EMBARGOED ADVANCE COPY Not to be published in full, or in part, in any form before 00.01am on Thursday 24 July 2014
 
Case study 1: decontamination of surgical instruments
 
6. Given the NHS’s resistance to change and the well-documented challenges associated with initiating a UK clinical trial, the Minister’s assessment that “no barriers” were put in the way of DuPont’s prion inactivation product does not reflect the reality of the situation. Where technologies are developed in direct response to Government need—and on the back of Government funding—the Government must be prepared to take steps to help companies overcome barriers to adoption. We ask the Government to set out how, in future, it will ensure that the directed research that it funds is better supported through the technology readiness pathway. In particular, we ask the Government to set out how it will ensure that promising clinical technologies are promptly trialled in an NHS setting, so that potential adoption challenges can be quickly identified and resolved. (Paragraph 37)
 
7. We also question the value of a scientific review panel which has no mandate or power to ensure that the products that it recommends can be tested in, and eventually adopted by, the NHS. We see this as further evidence of the Government’s passive approach to technology uptake. We propose that the Rapid Review Panel (RRP) be given stronger powers to ensure that its recommendations open the door to in-use evaluation and stimulate NHS uptake. (Paragraph 38)
 
8. In our view, all Scientific Advisory Committees should adhere to both the 2010 ‘Principles of Scientific Advice to Government’ and the 2011 ‘Code of Practice for Scientific Advisory Committees’. We were disappointed to find that the Rapid Review Panel (RRP) failed to do so. We recommend that the Chief Medical Officer takes action to rectify current weaknesses. We request a progress report be sent to us well before the dissolution of Parliament. (Paragraph 40)
 
Case study 2: prion filtration
 
9. We do not wish to question the scientific decision-making of the Advisory Committee on the Safety of Blood, Tissues and Organs (SaBTO) and we respect its decision not to recommend adoption of prion filtration at present. However, we feel that the time taken to reach this decision was excessive and that the process, particularly in its latter stages, entailed an unnecessary level of uncertainty for the commercial developer. We have some sympathy for SaBTO’s desire to wait until more evidence was available before making a decision; however, if industry is to continue to develop innovative blood safety products for the UK market, SaBTO must introduce greater speed and predictability into its evaluation process. We recommend that, in future, when assessing a new technology, SaBTO agree with stakeholders at the outset what the evaluation will consist of, together with key dates, milestones and decision-points. This ‘evaluation roadmap’, and any subsequent amendments, should be made publicly available to ensure maximum transparency and accountability. (Paragraph 45)
 
10. We also consider it important that the health technology appraisals conducted by SaBTO—and all other SACs—use the same methodology and meet the same high standards as those undertaken by the UK’s centre of excellence for this activity: NICE. We therefore recommend that the Government Office for Science work with NICE over the next 12 months to develop and publish a standard methodology for
 
After the storm? UK blood safety and the risk of variant Creutzfeldt-Jakob Disease53 EMBARGOED ADVANCE COPY Not to be published in full, or in part, in any form before 00.01am on Thursday 24 July 2014
 
all SACs tasked with conducting health technology appraisal. Until this guidance is published, we recommend that a NICE representative review and, where necessary, provide input to all such appraisals undertaken by, and on behalf of, SACs. (Paragraph 46)
 
11. Scientific Advisory Committees should be—and be seen to be—independent of the bodies to which they are providing advice. At present, the Advisory Committee on the Safety of Blood, Tissues and Organs (SaBTO) comprises members who are both contributing to, and acting on, the advice that it formulates. We consider that this could be damaging to its perceived independence and a source of potential conflicts of interest. We recommend that SaBTO’s terms of reference be amended to reflect the fact that it does, in effect, provide advice to UK Blood Services as well as the Government. We suggest that SaBTO’s current membership be reviewed and potentially revised in light of this change. (Paragraph 50)
 
Case study 3: vCJD blood testing
 
12. We understand the need to carefully control access to rare vCJD samples and commend the National Institute of Biological Standards and Controls (NIBSC) for putting in place a standard protocol for test validation. However, we are disappointed that so few samples are currently held by the NIBSC and consider its process to be undermined by the fact that the two major centres of UK prion research—the National CJD Research and Surveillance Unit and the MRC Prion Unit—can each use and distribute samples independent of NIBSC evaluation. All test developers should be given equal opportunity to gain access to the available samples and these should be distributed on the basis of merit alone. We recommend that access to all vCJD patient samples—including those currently held elsewhere in the UK—be managed through the NIBSC, according to a consistent set of test validation protocols. (Paragraph 59)
 
13. We were also concerned by the apparent statistical weakness of past NIBSC evaluations. We recommend that the CJD Resource Centre Oversight Committee add to its membership an individual with expertise in biostatistics, who can provide it with expert advice on this matter during future deliberations. (Paragraph 60)
 
14. The incubation period of prion diseases such as vCJD can extend to several decades and it is therefore possible that individuals infected in the 1990s might not yet have developed symptoms. We do not follow the Minister’s logic that there should be a link between the number of cases seen in the last ten years and the level of resource dedicated to prion research. We simply do not know, at present, how many people have been exposed to prions and what the implications of this might be for the blood donor pool. There is an urgent need to reduce this uncertainty. (Paragraph 65) 15. Based on the testimony that we have heard, we consider that a vCJD blood prevalence study utilising a version of the prototype test developed by the MRC Prion Unit would be of considerable value, both for test development and research purposes. We recognise that significant public funds have already been directed towards the development of this test; we view this as even more reason to ensure that a return on this investment is realised. To cut off support now would be a false
 
54After the storm? UK blood safety and the risk of variant Creutzfeldt-Jakob Disease EMBARGOED ADVANCE COPY Not to be published in full, or in part, in any form before 00.01am on Thursday 24 July 2014
 
economy. We recommend that the Government ensures that a large-scale vCJD blood prevalence study be initiated in the UK within the next 12 months. (Paragraph 66)
 
CJD risk management
 
16. People who are notified that they may have been exposed to CJD will inevitably be alarmed by this information and will likely have questions that cannot be answered in the leaflets currently provided by Public Health England. We consider it totally inappropriate for this news to be communicated solely in writing. We recommend that the Government put robust measures in place to ensure that all individuals assigned this designation receive the news verbally, either from a healthcare provider or from a CJD specialist with experience in patient communication. (Paragraph 70)
 
17. It is clear that the prototype vCJD blood test developed by the MRC Prion Unit cannot yet be relied upon for universal screening purposes. However, it could be of significant value to those people who have been notified that they are at increased risk of carrying the disease. Until the implications of a negative test result can be more firmly established, current precautions must remain in place for those considered to be ‘at risk’ of vCJD. However, the results of an imperfect test may provide comfort to some. We therefore recommend that ‘at risk’ individuals be given the opportunity to participate in the blood prevalence study recommended in paragraph 66. (Paragraph 73)
 
CJD surveillance
 
18. The Government claims to be undertaking close surveillance of those it considers to be ‘at risk’ of CJD. Yet it cannot provide reliable data either on the total number of people designated ‘at risk’ or the number who have been notified of this fact. This is unacceptable. We recommend that the Government conduct an immediate audit of the entire ‘at risk’ cohort to establish whether any notifications remain outstanding and to ensure that appropriate support and follow-up is in place for all those affected. We also propose that the Government commission an independent review of the transfusion data pathway to ensure that, in the event of any future blood contamination incident, it can promptly trace, notify and provide support to affected recipients. (Paragraph 77)
 
19. We were disappointed by the evident lack of support provided to those designated ‘at risk’ of CJD. We consider it inappropriate for the Government to have effectively delegated responsibility for the care and surveillance of a large proportion of these individuals to external bodies such as the UK Haemophilia Centre Doctors' Organisation—a charitable organisation with no formal relationship with the Executive. We recommend that the Government, through its public health agencies, assume direct responsibility for the surveillance and support of all those considered to be ‘at risk’ of CJD, with input from other specialist organisations as required. (Paragraph 78)
 
20. In our view, the decision to participate in research should always rest with the individual or, in exceptional circumstances, their loved ones. Nevertheless, samples contributed by those potentially exposed to CJD are of immense scientific value and
 
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we are disappointed that more has not been done to obtain consent from those willing to participate in research. We recommend that the Government consider ways to increase the number of ‘at risk’ individuals giving consent for research participation, particularly post-mortem. We ask that the Government summarise its plans for achieving this in its response to this Report. (Paragraph 81)
 
21. We are confident in the integrity of the National CJD Research and Surveillance Unit and have not seen any evidence to corroborate claims of deliberate under-reporting or misclassification. However, we share our witnesses’ concerns that cases could be missed due to misdiagnosis, particularly in the elderly. We recommend that the Government lend its support to research intended to give greater clarity over the causes of atypical dementia in the elderly and, through this, the potential rate of undiagnosed CJD. (Paragraph 87)
 
Conclusion
 
22. SaBTO’s decision not to recommend the adoption of prion filtration, taken alongside the other evidence that we have gathered during this inquiry, in our view signals a change from what was a genuinely precautionary approach to vCJD risk reduction in the late 1990s to a far more relaxed approach today. Much of the uncertainty surrounding prions, their potential modes of transmission and the possible rate of undetected infection and disease remains: recent evidence that subclinical prevalence could be as high as one in 2,000 people would suggest that a precautionary approach is now more warranted than ever. (Paragraph 94)
 
23. Our fear is that the Government’s current attitude is driven less by the available scientific evidence than it is by optimism: a hope that the storm has now passed and that vCJD is no longer the threat to public health that it once was. In the current economic environment, this attitude is not surprising. However, it is not justified. For all we know, the storm may well be ongoing. We conclude this report by recommending that the Government take a more precautionary approach to both vCJD risk mitigation and blood safety more generally, in order to safeguard against future infections. We suggest that it begin by assessing the key risks, known and unknown, that the UK blood supply currently faces and might face in the future, so that it can identify and fill relevant knowledge gaps and support the development of appropriate risk reduction measures and technologies. The Government should initiate this work immediately and we ask that it provide us with an update on its progress well before the dissolution of Parliament. (Paragraph 95)
 
56After the storm? UK blood safety and the risk of variant Creutzfeldt-Jakob Disease EMBARGOED ADVANCE COPY Not to be published in full, or in part, in any form before 00.01am on Thursday 24 July 2014 Formal Minutes Wednesday 16 July 2014
 
 
 
From: TSS
 
Subject: THE MANIPULATION OF BSE/TSE SCIENCE IN PEER REVIEW JOURNALS ?
 
Date: July 11, 2005 at 11:23 am PST - 10 - 19.
 
NEW BRAIN DISORDER
 
3. WHAT ABOUT REPORTS OF NEW FORM OF BSE?
 
THE VETERINARY RECORD HAS PUBLISHED AN ARTICLE ON A NEW BRAIN DISORDER OF CATTLE DISCOVERED THROUGH OUR CONTROL MEASURES FOR BSE. ALTHOUGH IT PRESENTS SIMILAR CLINICAL SIGNS TO BSE THERE ARE MAJOR DIFFERENCES IN THE HISTOPATHOLOGY AND INCUBATION PERIODS BETWEEN THE TWO. MUST EMPHASISE THAT THIS IS NOT BSE.
 
4. IS THIS NEW BRAIN DISORDER A THREAT?
 
WE DO NOT EVEN KNOW WHETHER THE AGENT OF THIS DISEASE IS TRANSMISSIBLE. IN ANY CASE, CASES SO FAR IDENTIFIED HAD SHOWN SIMILAR SYMPTOMS TO THOSE OF BSE, AND THEREFORE HAVE BEEN SLAUGHTERED AND INCINERATED, SO THAT IF A TRANSMISSIBLE AGENT WERE INVOLVED IT WOULD HAVE BEEN ELIMINATED. .......
 
 
 
2. The Collinge/Will dispute appears to rumble on. Dr. Collinge had told Dr. Tyrrell that Dr. Will's response to his criticism about sharing material had been ''quite unacceptable'' (in spite of it's apparently conciliatory tone). Apparently Professor Allen was now going to try and arrange a meeting to resolve the dispute. No action here for MAFF, although Mr. Murray may be interested.
 
3. Dr. Tyrrell regretted that the Committee had not seen the article on BBD. However he felt that for the time being NO specific action was called for. The most important need was to consider the possibility that the condition might be transmissible. As we have discussed, I suggested that we might circulate a paper to the members of the committee giving our appreciation of this condition (and perhaps of other non-BSE neurological conditions that had been identified in negative cases) and of any necessary follow up action. IF any Committee member felt strongly about this, or if the issue CAME TO A HEAD, we would call an interim meeting. He was happy with this approach. I would be grateful if Mr. Maslin could, in discussion with CVL and veterinary colleagues draft such a note, which will presumably very largely follow what Mr. Bradley's briefing paper has already said, taking account of DOH comments, We can then clear a final version with DOH before circulating it to Committee members.
 
 
 
IN CONFIDENCE
 
This is a highly competitive field and it really will be a pity if we allow many of the key findings to be published by overseas groups while we are unable to pursue our research findings because of this disagreement, which I hope we can make every effort to solve.
 
 
 
COLLINGE THREATENS TO GO TO MEDIA
 
6. Trouble has been brewing for some time. Dr. Collinge is lobbying hard, and threatening to go to the media, claiming Dr. Will is blocking his research by denying him access to the material and case histories collected as part of the RDD-funded surveillance. Encouraged by us, the MRC is trying to arbitrate, and a meeting of the research funders is being called by the MRC for the 11th January. Our natural sympathies lie with Dr. Will, who is a much easier character and one of our advisers on the Tyrrell committee on spongiform encephalopathies. On the other hand, we and the MRC do not want to hold up research.
 
7. Lord Peyton has been briefed to raise this at the lunch with DRD today.
 
8. The recommended line to take is :
 
* the HD priority is not to jeopardise the national surveillance of CJD
 
* research is very important, but a secondary issue, must be conducted according to the highest scientific and ethical standards
 
* a compromise solution should be sought, and a meeting under the auspices of the MRC is planned to effect this
 
* research, patients, their relatives and the national surveillance programme could all be damaged by comments to the press. This could backfire on the researchers.
 
[9. Whilst this matter is not at the moment directly concerned with the iatrogenic CJD cases from the hgH, there remains a possibility of litigation here, and this presents an added complication. There are also results to be made available shortly (1) concerning a farmer with CJD who had BSE animals, (2) on the possible transmissibility of Alzheimer’s and (3) a CMO letter on prevention of iatrogenic CJD transmission in neurosurgery, all of which will serve to increase media interest.]
 
92/12.16/5.3
 
 
 
"BSE" - ATYPICAL LESION DISTRIBUTION __ (RBSE 92/21367)
 
A 6 year old, home bred (HB), Friesian x Holstein cow in a dairy herd in Aberdeenshire, submitted as a suspect BSE case in the negative study (SE0203), has been diagnosed as BSE negative on standard, statutory (obex only), diagnostic criteria at CVL.
 
Further examination by Dr Jeffrey at Lasswade, as required by the project design, has revealed vacuolar change in the septal nucleus and putamen which co-Iocalised with PrP immunoreactivity. No significant lesions were found in any other part of the brain, neither was PrP found in the medulla.
 
It is important to note that examination of four brain blocks used earlier in the epidemic would not have detected the lesion but a 16 block study (as used in the very early days of BSE) would.
 
FURTHER INFORMATION
 
1. The herd of origin has had 15, HB, suspect cases of BSE since July 1989 and a further case is still alive.
 
2. Of the 15, eight have been confirmed by standard histopathology and seven diagnosed negative (including the above case).
 
3. Fixed brain tissue from the negative cases exists at Lasswade (because they always collect whole brain in Scotland) but has not so far been examined further. No frozen tissue was collected so neither SAF nor PrP detection (by immunoblotting) has been attempted.
 
4. Mr Wells agrees with Dr Jeffrey's and Dr Simmons' findings.
 
FURTHER ACTION IN PROGRESS
 
1. The brain tissue from the negative cases will be examined in detail by conventional histopathology and ICC.
 
2. Kevin Taylor and his veterinary colleagues have been alerted to the situation.
 
OTHER RECOMMENDED ACTIONS
 
TRANSMISSION Attempt transmission from the 'case’ to standard mice strains. (Note: In regard to strain typing, formalin may have modified strain phenotype - we need to discuss with NPU). Further transmission studies (eg in cattle) might be suggested if primary transmission in mice fails. These proposals have funding implications.
 
93/2.17/1.1
 
PrP GENOTYPING-AIthough only fixed brain tissue is available we are considering genotyping from parents/offspring/fixed brain. As a first step we are attempting to extract DNA from the fixed brain and to amplify the PrP gene by PCR.
 
3. John Wilesmith has interrogated the data base for the herd history. Other than the high proportion of negative cases nothing significant is apparent.
 
4. Familial relationships between suspect (including all positive and negative) cases in this herd could be examined and tracings of breeding animals initiated.
 
5. Consideration might be given to collecting frozen spinal cord from new cases in this herd or in dispersals from it (for SAF /PrP examination).
 
CONCLUSIONS
 
1. At present it is unclear whether or not this is a singleton incident or whether the other negative cases in this herd show a similar lesion.
 
2. The discovery might indicate the existence of a different strain of BSE from that present in the general epidemic or an unusual response by an individual host.
 
3. If further atypical lesion distribution cases are revealed in this herd then implications of misdiagnosis of 'negative' cases in other herds may not be insignificant.
 
4. If this is a new strain all the implications need to be considered including whether or not to proceed with the further investigation of future cases negative for BSE on obex examination alone and from which whole brains are available (as in Scotland) or collected in the future. Also perhaps investigation of the tissue distribution of infectivity in these animals might be considered.
 
5. Animal and public health controls in place should be sufficient since all tissues (other than brain for diagnosis) are incinerated.
 
We observe that Dr. Tyrrell would wish to be informed of this at an early opportunity and that the SEAC would wish to discuss it at their meeting in April.
 
R BRADLEY M DAWSON 17 February 1993
 
CVO – for information and comment on further action please
 
cc Mr K C Taylor Dr B J Shreeve
 
93/2.17/1.2
 
This minute is re-issued with a wider distribution. The information contained herein should be disseminated furhter except on the basis of ‘’need to know’’.
 
R BRADLEY 9 March 1993
 
Mr JM Scudamore Mr RC Lowson Dr D Mattews Mr I Robertson Dr K MacOwan Mr C Randall Mr J W Wilesmith Mr GAH Wells Dr M Jeffrey Dr. M Simmons
 
93/2.17/1.3
 
 
 
IN CONFIDENCE
 
BSE ATYPICAL LESION DISTRIBUTION
 
 
 
snip...
 
On 18th February, 1987 (YB87/2.18/1.1) I reported to Dr Watson and Dr Shreeve on a further case which we had received from Truro VIC. The brain had shown neuronal vacuolation and in brain extracts there were fibrils that were similar in size and appearance to SAFs from sheep with scrapie. The Virology Department was studying the brain further and considering a transmission study. A few weeks before this, I had discussed the possibility of a transmission study with Michael Dawson, a research officer in the Virology Department and an expert in viral diseases in sheep, and we were considering carefully the safety aspects. In my note I raised the question of whether we should disclose the information we had more widely to the VIS because this may assist in getting any other cases referred to CVL but there was the difficulty that we knew very little about the disorder and would be unable to deal with queries that might be raised.
 
20. On 23rd February, 1987 (YB87/2.23/1.1) I sent Mr Wells a note asking him to prepare a statement for publication in Vision, the in-house newsheet prepared by the VIS for the SVS, setting out details of what we had discovered. On 24th February, 1987 (YB87/2.25/2.1) Gerald Wells indicated in a note to me that he had discussed the proposed article with Mr Dawson and they both believed that it could be damaging to publish anything at that stage. They believed cases would be referred to CVL in any event because they were unusual and they did not feel "Vision" was an appropriate publication because its confidentiality was questionable and might lead to referrals to veterinary schools rather than CVL. Gerald Wells was also concerned about the resources available in his section to deal with referred cases. I replied (YB87/2.25/2.1) indicating a draft statement was needed by the Director before a decision on publication could be made. Gerald Wells prepared a draft statement (YB87/3.2/2.1) and sent it to me on 2nd March, 1987. In his cover note (YB87/3.2/1.1) he commented that he believed the distribution of any statement about the new disease outside of CVL to be premature because there was so little information available about the new disease. I passed on a copy of Gerald Wells' note to Dr Watson (YB87/3.2/3.1). I discussed the matter of publication with Dr Watson. No decision had been taken to publish any material at that stage and I sent a note to Gerald Wells letting him know the position and confirming that his views and those of Michael Dawson would be taken into account when a decision was taken.
 
- 11 -
 
21. In March, 1987 serious consideration was given to possible transmission (e.g. to hamsters) and other experiments (other than the collection of epidemiological data by the VIS and clinicopathology which had been in progress since the first cases were recognised in November, 1986).
 
22. On 23rd April, 1987 I sent a report (YB87/4.23/1.1) to Dr Watson and Dr Shreeve informing them that nine control brains were being examined for SAFs and a cow which appeared to be affected with BSE had been purchased for observation. The cow had come from the farm where the original cases had developed and had arrived at CVL on 22nd April, 1987.
 
23. On 15th May, 1987 Dr Watson informed me that the proposed "Vision" draft would be circulated to VICs in England and Wales if it was approved by management. On 22nd May, 1987 I was copied in on a note (YB87/5.22/2.1) from B.M Williams, (who I believe was Head of the VIS at this time but retired shortly after this), to Dr Watson. This confirmed that the draft prepared for publication in Vision was approved but that the final paragraph should be amended to make it clear that knowledge of the new disease should not be communicated to other research institutes or university departments. At a meeting with Dr Watson on 2nd June, 1987 he informed me that no communication should be made with NPU until after the meeting with the CVO on 5th June, 1987 (see my note of 3rd June, 1987 – YB87/6.3/1.1). We needed much more data and information to answer inevitable queries. ...
 
 
 
snip...see full text ;
 
Wednesday, August 20, 2008
 
Bovine Spongiform Encephalopathy Mad Cow Disease typical and atypical strains, was there a cover-up ?
 
 
Tuesday, November 17, 2009
 
SEAC NEW RESULTS ON IDIOPATHIC BRAINSTEM NEURONAL CHROMATOLYSIS (IBNC) FROM THE VETERINARY LABORATORIES AGENCY (VLA) SEAC 103/1
 
 
NEW RESULTS ON IDIOPATHIC BRAINSTEM NEURONAL CHROMATOLYSIS "All of the 15 cattle tested showed that the brains had abnormally accumulated PrP" 2009
 
 
========
 
NOW, what about the 'obex only' mode of testing used by the USDA et al for TSE, prions $$$ works for them too, a sure fire way NOT TO FIND MAD COW DISEASE $$$
 
NOW, read the following please, and then ask yourself, WHY the USDA et al were ONLY TESTING THE OBEX PART OF THE BRAIN in USA cattle for BSE $$$
 
BECAUSE they knew that would be the least likely way to find BSE/TSE in USA cattle $$$...TSS
 
=========
 
Discussion
 
In the five cats in this study with a spongiform encephalopathy, fibrils were observed by electron microscopy and their major protein, Prpsc, was identified by SDS-PAGE and Western blot. The fibrils were similar to those described in sheep with scrapie (Rubenstein and others 1987, Gibson and others 1987, Scott and others 1987, Dawson and others 1987), cattle with bovine spongiform encephalopathy (Wells and others 1987, Hope and others 1988, Scott and others 1990) and humans with Creutzfeldt-Jakob disease (Merz and others 1984).
 
In sheep with scrapie, fibrils can be readily detected in several areas of the brain, including cerebral cortex (Stack and others 1991).
 
By contrast, the frequency with which fibrils were detected in cattle with BSE, DEPENDED ON THE REGION OF THE BRAIN SAMPLED; THE HIGHEST YIELD BEING OBTAINED FROM MEDULLA, MIDBRAIN, THALAMUS AND BASAL NUCLEI WHERE VACUOLA CHANGES ARE PRESENT (Scott and others 1990). This correlation between PrPsc accumulation and vacuolar pathology is also well established in laboratory animal models of scrapie (Bruce and others 1989). Because of the widespread distribution of changes in FSE (Whatt and others 1991) and the requirement, in the present study, not to compromise the histopathological examination of the brain, the frontal region of the cerebrum was therefore selected for fibril and PrPsc examinations. However, studies of the sensitivity of fibril detection in different parts of the brain in cats with FSE are required to determine whether detection can be made as readliy in other regions as in the frontal cerebral cortex.
 
IT IS OF INTEREST, that fibrils were detected in the brains of 3 cats (cases 9, 13, & 18) WITHOUT histopathological evidence of spongiform encephalopathy, and that in only one of them, (case 9), a Western blot for modified PrP was positive. There are precedents for the occurrence of abnormal PrP in the organs of animals incubation scrapie prior to clinical signs and/or spongiform encephalopathy...
 
snip...
 
(please see full text (and one might start downloading these documents for future use, as some disappear never to re-appear, as in some of the FDA's. ...TSS)
 
 
PLEASE NOTE *
 
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...
 
 
AND THE USDA ET AL KNEW IT TOO ;
 
"These 9,200 cases were different because brain tissue samples were preserved with formalin, which makes them suitable for only one type of test--immunohistochemistry, or IHC."
 
THIS WAS DONE FOR A REASON!
 
THE IHC test has been proven to be the LEAST LIKELY to detect BSE/TSE in the bovine, and these were probably from the most high risk cattle pool, the ones the USDA et al, SHOULD have been testing. ...TSS
 
USDA 2003
 
We have to be careful that we don't get so set in the way we do things that we forget to look for different emerging variations of disease. We've gotten away from collecting the whole brain in our systems. We're using the brain stem and we're looking in only one area. In Norway, they were doing a project and looking at cases of Scrapie, and they found this where they did not find lesions or PRP in the area of the obex. They found it in the cerebellum and the cerebrum. It's a good lesson for us. Ames had to go back and change the procedure for looking at Scrapie samples. In the USDA, we had routinely looked at all the sections of the brain, and then we got away from it. They've recently gone back. Dr. Keller: Tissues are routinely tested, based on which tissue provides an 'official' test result as recognized by APHIS.
 
Dr. Detwiler: That's on the slaughter. But on the clinical cases, aren't they still asking for the brain? But even on the slaughter, they're looking only at the brainstem. We may be missing certain things if we confine ourselves to one area.
 
snip.............
 
Dr. Detwiler: It seems a good idea, but I'm not aware of it. Another important thing to get across to the public is that the negatives do not guarantee absence of infectivity. The animal could be early in the disease and the incubation period. Even sample collection is so important. If you're not collecting the right area of the brain in sheep, or if collecting lymphoreticular tissue, and you don't get a good biopsy, you could miss the area with the PRP in it and come up with a negative test. There's a new, unusual form of Scrapie that's been detected in Norway. We have to be careful that we don't get so set in the way we do things that we forget to look for different emerging variations of disease. We've gotten away from collecting the whole brain in our systems. We're using the brain stem and we're looking in only one area. In Norway, they were doing a project and looking at cases of Scrapie, and they found this where they did not find lesions or PRP in the area of the obex. They found it in the cerebellum and the cerebrum. It's a good lesson for us. Ames had to go back and change the procedure for looking at Scrapie samples. In the USDA, we had routinely looked at all the sections of the brain, and then we got away from it. They've recently gone back.
 
Dr. Keller: Tissues are routinely tested, based on which tissue provides an 'official' test result as recognized by APHIS .
 
Dr. Detwiler: That's on the slaughter. But on the clinical cases, aren't they still asking for the brain? But even on the slaughter, they're looking only at the brainstem. We may be missing certain things if we confine ourselves to one area.
 
snip...
 
FULL TEXT;
 
Completely Edited Version PRION ROUNDTABLE
 
Accomplished this day, Wednesday, December 11, 2003, Denver, Colorado
 
END...TSS
 
==========
 
Subject: USDA OIG SEMIANNUAL REPORT TO CONGRESS FY 2007 1st Half (bogus BSE sampling FROM HEALTHY USDA CATTLE)
 
Date: June 21, 2007 at 2:49 pm PST
 
Owner and Corporation Plead Guilty to Defrauding Bovine Spongiform Encephalopathy (BSE) Surveillance Program
 
An Arizona meat processing company and its owner pled guilty in February 2007 to charges of theft of Government funds, mail fraud, and wire fraud. The owner and his company defrauded the BSE Surveillance Program when they falsified BSE Surveillance Data Collection Forms and then submitted payment requests to USDA for the services. In addition to the targeted sample population (those cattle that were more than 30 months old or had other risk factors for BSE), the owner submitted to USDA, or caused to be submitted, BSE obex (brain stem) samples from healthy USDA-inspected cattle. As a result, the owner fraudulently received approximately $390,000. Sentencing is scheduled for May 2007.
 
snip...
 
Topics that will be covered in ongoing or planned reviews under Goal 1 include:
 
soundness of BSE maintenance sampling (APHIS),
 
implementation of Performance-Based Inspection System enhancements for specified risk material (SRM) violations and improved inspection controls over SRMs (FSIS and APHIS),
 
snip...
 
The findings and recommendations from these efforts will be covered in future semiannual reports as the relevant audits and investigations are completed.
 
4 USDA OIG SEMIANNUAL REPORT TO CONGRESS FY 2007 1st Half
 
 
FOR IMMEDIATE RELEASE Statement May 4, 2004 Media Inquiries: 301-827-6242 Consumer Inquiries: 888-INFO-FDA
 
Statement on Texas Cow With Central Nervous System Symptoms
 
On Friday, April 30 th , the Food and Drug Administration learned that a cow with central nervous system symptoms had been killed and shipped to a processor for rendering into animal protein for use in animal feed.
 
FDA, which is responsible for the safety of animal feed, immediately began an investigation. On Friday and throughout the weekend, FDA investigators inspected the slaughterhouse, the rendering facility, the farm where the animal came from, and the processor that initially received the cow from the slaughterhouse.
 
FDA's investigation showed that the animal in question had already been rendered into "meat and bone meal" (a type of protein animal feed). Over the weekend FDA was able to track down all the implicated material. That material is being held by the firm, which is cooperating fully with FDA.
 
 
 
THE USDA JUNE 2004 ENHANCED BSE SURVEILLANCE PROGRAM WAS TERRIBLY FLAWED ;
 
CDC DR. PAUL BROWN TSE EXPERT COMMENTS 2006
 
In an article today for United Press International, science reporter Steve Mitchell writes:
 
Analysis: What that mad cow means
 
By STEVE MITCHELL UPI Senior Medical Correspondent
 
WASHINGTON, March 15 (UPI) -- The U.S. Department of Agriculture was quick to assure the public earlier this week that the third case of mad cow disease did not pose a risk to them, but what federal officials have not acknowledged is that this latest case indicates the deadly disease has been circulating in U.S. herds for at least a decade.
 
The second case, which was detected last year in a Texas cow and which USDA officials were reluctant to verify, was approximately 12 years old.
 
These two cases (the latest was detected in an Alabama cow) present a picture of the disease having been here for 10 years or so, since it is thought that cows usually contract the disease from contaminated feed they consume as calves. The concern is that humans can contract a fatal, incurable, brain-wasting illness from consuming beef products contaminated with the mad cow pathogen.
 
"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.
 
Despite this, Brown said the U.S. prevalence of mad cow, formally known as bovine spongiform encephalopathy, or BSE, did not significantly threaten human or cattle health.
 
"Overall, my view is BSE is highly unlikely to pose any important risk either in cattle feed or human feed," he said.
 
However, Jean Halloran of Consumers Union in Yonkers, N.Y., said consumers should be troubled by the USDA's secrecy and its apparent plan to dramatically cut back the number of mad cow tests it conducts.
 
"Consumers should be very concerned about how little we know about the USDA's surveillance program and the failure of the USDA to reveal really important details," Halloran told UPI. "Consumers have to be really concerned if they're going to cut back the program," she added.
 
Last year the USDA tested more than 300,000 animals for the disease, but it has proposed, even in light of a third case, scaling back the program to 40,000 tests annually.
 
"They seem to be, in terms of actions and policies, taking a lot more seriously the concerns of the cattle industry than the concerns of consumers," Halloran said. "It's really hard to know what it takes to get this administration to take action to protect the public."
 
The USDA has insisted that the safeguards of a ban on incorporating cow tissue into cattle feed (which is thought to spread the disease) and removal of the most infectious parts of cows, such as the brain and spinal cord, protect consumers. But the agency glosses over the fact that both of these systems have been revealed to be inadequately implemented.
 
The feed ban, which is enforced by the Food and Drug Administration, has been criticized by the Government Accountability Office in two reports, the most recent coming just last year. The GAO said the FDA's enforcement of the ban continues to have weaknesses that "undermine the nation's firewall against BSE."
 
USDA documents released last year showed more than 1,000 violations of the regulations requiring the removal of brains and spinal cords in at least 35 states, Puerto Rico and the Virgin Islands, with some plants being cited repeatedly for infractions. In addition, a violation of similar regulations that apply to beef exported to Japan is the reason why Japan closed its borders to U.S. beef in January six weeks after reopening them.
 
Other experts also question the adequacy of the USDA's surveillance system. The USDA insists the prevalence of mad cow disease is low, but the agency has provided few details of its surveillance program, making it difficult for outside experts to know if the agency's monitoring plan is sufficient.
 
"It's impossible to judge the adequacy of the surveillance system without having a breakdown of the tested population by age and risk status," Elizabeth Mumford, a veterinarian and BSE expert at Safe Food Solutions in Bern, Switzerland, a company that provides advice on reducing mad cow risk to industry and governments, told UPI.
 
"Everybody would be happier and more confident and in a sense it might be able to go away a little bit for (the USDA) if they would just publish a breakdown on the tests," Mumford added.
 
UPI requested detailed records about animals tested under the USDA's surveillance plan via the Freedom of Information Act in May 2004 but nearly two years later has not received any corresponding documents from the agency, despite a federal law requiring agencies to comply within 30 days. This leaves open the question of whether the USDA is withholding the information, does not have the information or is so haphazardly organized that it cannot locate it.
 
Mumford said the prevalence of the disease in U.S. herds is probably quite low, but there have probably been other cases that have so far gone undetected. "They're only finding a very small fraction of that low prevalence," she said.
 
Mumford expressed surprise at the lack of concern about the deadly disease from American consumers. "I would expect the U.S. public to be more concerned," she said.
 
Markus Moser, a molecular biologist and chief executive officer of Prionics, a Swiss firm that manufactures BSE test kits, told UPI one concern is that if people are infected, the mad cow pathogen could become "humanized" or more easily transmitted from person to person.
 
"Transmission would be much easier, through all kinds of medical procedures" and even through the blood supply, Moser said.
 
© Copyright 2006 United Press International, Inc. All Rights Reserved
 
 
 
CDC - Bovine Spongiform Encephalopathy and Variant Creutzfeldt ... Dr. Paul Brown is Senior Research Scientist in the Laboratory of Central Nervous System ... Address for correspondence: Paul Brown, Building 36, Room 4A-05, ...
 
 
PAUL BROWN COMMENT TO ME ON THIS ISSUE
 
Tuesday, September 12, 2006 11:10 AM
 
"Actually, Terry, I have been critical of the USDA handling of the mad cow issue for some years, and with Linda Detwiler and others sent lengthy detailed critiques and recommendations to both the USDA and the Canadian Food Agency." ........TSS
 
 
OR, what the Honorable Phyllis Fong of the OIG found ;
 
Audit Report Animal and Plant Health Inspection Service Bovine Spongiform Encephalopathy (BSE) Surveillance Program ­ Phase II and Food Safety and Inspection Service
 
Controls Over BSE Sampling, Specified Risk Materials, and Advanced Meat Recovery Products - Phase III
 
Report No. 50601-10-KC January 2006
 
Finding 2 Inherent Challenges in Identifying and Testing High-Risk Cattle Still Remain
 
 
THIS is just ONE month report, of TWO recalls of prohibited banned MBM, which is illegal, mixed with 85% blood meal, which is still legal, but yet we know the TSE/BSE agent will transmit blood. we have this l-BSE in North America that is much more virulent and there is much concern with blood issue and l-BSE as there is with nvCJD in humans. some are even starting to be concerned with sporadic CJD and blood, and there are studies showing transmission there as well. ... this is one month recall page, where 10 MILLION POUNDS OF BANNED MAD COW FEED WENT OUT INTO COMMERCE, TO BE FED OUT. very little of the product that reaches commerce is ever returned via recall, very, very little. this was 2007, TEN YEARS AFTER THE AUGUST 4, 1997, PARTIAL AND VOLUNTARY MAD COW FEED BAN IN THE USA, that was nothing but ink on paper. i have listed the tonnage of mad cow feed that was in ALABAMA in one of the links too, this is where the infamous g-h-BSEalabama case was, a genetic relation matching the new sporadic CJD in the USA. seems this saga just keeps getting better and better.......$$$
 
10,000,000+ LBS. of PROHIBITED BANNED MAD COW FEED I.E. BLOOD LACED MBM IN COMMERCE USA 2007
 
snip...see full text ;
 
Tuesday, November 02, 2010
 
IN CONFIDENCE
 
The information contained herein should not be disseminated further except on the basis of "NEED TO KNOW".
 
BSE - ATYPICAL LESION DISTRIBUTION (RBSE 92-21367) statutory (obex only) diagnostic criteria CVL 1992
 
 
2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006
 
 
Sunday, June 29, 2014
 
*** Transmissible Spongiform Encephalopathy TSE Prion Disease North America 2014 ***
 
 
IF you really want to know, what they are feeding cows and other livestock for human and animal consumption, please see my latest review of the OIA’s under the mad cow feed ban for 2013. please be aware, the mad cow feed ban of 1997, was nothing but ink on paper. the tonnage of banned mad cow feed that has gone into commerce is phenomenal, it’s in the 100s if not 1000s of tonnages. it’s flat out shocking...
 
Sunday, December 15, 2013
 
FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OIA UPDATE DECEMBER 2013 UPDATE
 
 
Blood product, collected from a donor who was at risk for variant Creutzfeldt-Jakob disease (vCJD), was distributed.
 
 
 
 
 
just a few months I checked. frightening. you can go through each month yourself and just see how much Blood product, collected from a donor who was at risk for variant Creutzfeldt-Jakob disease (vCJD), was distributed has been distributed ;
 
 
*** 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.
 
 
*** PPo3-7: Prion Transmission from Cervids to Humans is Strain-dependent
 
*** Here we report that a human prion strain that had adopted the cervid prion protein (PrP) sequence through passage in cervidized transgenic mice efficiently infected transgenic mice expressing human PrP,
 
*** indicating that the species barrier from cervid to humans is prion strain-dependent and humans can be vulnerable to novel cervid prion strains.
 
PPo2-27:
 
Generation of a Novel form of Human PrPSc by Inter-species Transmission of Cervid Prions
 
*** Our findings suggest that CWD prions have the capability to infect humans, and that this ability depends on CWD strain adaptation, implying that the risk for human health progressively increases with the spread of CWD among cervids.
 
PPo2-7:
 
Biochemical and Biophysical Characterization of Different CWD Isolates
 
*** The data presented here substantiate and expand previous reports on the existence of different CWD strains.
 
 
Envt.07:
 
Pathological Prion Protein (PrPTSE) in Skeletal Muscles of Farmed and Free Ranging White-Tailed Deer Infected with Chronic Wasting Disease
 
***The presence and seeding activity of PrPTSE in skeletal muscle from CWD-infected cervids suggests prevention of such tissue in the human diet as a precautionary measure for food safety, pending on further clarification of whether CWD may be transmissible to humans.
 
 
>>>CHRONIC WASTING DISEASE , THERE WAS NO ABSOLUTE BARRIER TO CONVERSION OF THE HUMAN PRION PROTEIN<<<
 
*** PRICE OF CWD TSE PRION POKER GOES UP 2014 ***
 
Transmissible Spongiform Encephalopathy TSE PRION update January 2, 2014
 
Wednesday, January 01, 2014
 
Molecular Barriers to Zoonotic Transmission of Prions
 
*** chronic wasting disease, there was no absolute barrier to conversion of the human prion protein.
 
*** Furthermore, the form of human PrPres produced in this in vitro assay when seeded with CWD, resembles that found in the most common human prion disease, namely sCJD of the MM1 subtype.
 
 
 
Exploring the zoonotic potential of animal prion diseases: In vivo and in vitro approaches
 
 
*** These results would seem to suggest that CWD does indeed have zoonotic potential, at least as judged by the compatibility of CWD prions and their human PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests that if zoonotic CWD occurred, it would most likely effect those of the PRNP codon 129-MM genotype and that the PrPres type would be similar to that found in the most common subtype of sCJD (MM1).
 
 
as I said, what if ?
 
*** our results raise the possibility that CJD cases classified as VV1 may include cases caused by iatrogenic transmission of sCJD-MM1 prions or food-borne infection by type 1 prions from animals, e.g., chronic wasting disease prions in cervid. In fact, two CJD-VV1 patients who hunted deer or consumed venison have been reported (40, 41). The results of the present study emphasize the need for traceback studies and careful re-examination of the biochemical properties of sCJD-VV1 prions. ***
 
 
Thursday, January 2, 2014
 
*** CWD TSE Prion in cervids to hTGmice, Heidenhain Variant Creutzfeldt-Jacob Disease MM1 genotype, and iatrogenic CJD ??? ***
 
WHAT IF ?
 
 
Sunday, August 25, 2013
 
HD.13: CWD infection in the spleen of humanized transgenic mice
 
***These results indicate that the CWD prion may have the potential to infect human peripheral lymphoid tissues.
 
Oral.15: Molecular barriers to zoonotic prion transmission: Comparison of the ability of sheep, cattle and deer prion disease isolates to convert normal human prion protein to its pathological isoform in a cell-free system ***However, they also show that there is no absolute barrier ro conversion of human prion protein in the case of chronic wasting disease.
 
PRION2013 CONGRESSIONAL ABSTRACTS CWD
 
Sunday, August 25, 2013
 
***Chronic Wasting Disease CWD risk factors, humans, domestic cats, blood, and mother to offspring transmission
 
 
*** REPORT OF THE MEETING OF THE OIE TERRESTRIAL ANIMAL HEALTH STANDARDS COMMISSION Paris, 19–28 February 2013
 
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.
 
 
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 ***
 
 
*** Chronic Wasting Disease CWD in cervidae and transmission to other species
 
 
Envt.06:
 
Zoonotic Potential of CWD: Experimental Transmissions to Non-Human Primates
 
Emmanuel Comoy,1,† Valérie Durand,1 Evelyne Correia,1 Aru Balachandran,2 Jürgen Richt,3 Vincent Beringue,4 Juan-Maria Torres,5 Paul Brown,1 Bob Hills6 and Jean-Philippe Deslys1 1Atomic Energy Commission; Fontenay-aux-Roses, France; 2Canadian Food Inspection Agency; Ottawa, ON Canada; 3Kansas State University; Manhattan, KS USA; 4INRA; Jouy-en-Josas, France; 5INIA; Madrid, Spain; 6Health Canada; Ottawa, ON Canada
 
***In contrast, bovinized and humanized transgenic mice showed signs of infection, suggesting that CWD-related prion strains may be capable of crossing the cattle-to-primate species barrier. Comparisons with transmission results and incubation periods obtained after exposure to other cattle prion strains (c-BSE, BSE-L, BSE-H and cattle-adapted TME) will also be presented, in order to evaluate the respective risks of each strain.
 
Envt.07: Pathological Prion Protein (PrPTSE) in Skeletal Muscles of Farmed and Free Ranging White-Tailed Deer Infected with Chronic Wasting Disease
 
Martin L. Daus,1,† Johanna Breyer,2 Katjs Wagenfuehr,1 Wiebke Wemheuer,2 Achim Thomzig,1 Walter Schulz-Schaeffer2 and Michael Beekes1 1Robert Koch Institut; P24 TSE; Berlin, Germany; 2Department of Neuropathology, Prion and Dementia Research Unit, University Medical Center Göttingen; Göttingen, Germany
 
*** Tissue-blot-analyses revealed that PrPTSE was located in muscle- associated nerve fascicles but not, in detectable amounts, in myocytes. 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.
 
 
***********CJD REPORT 1994 increased risk for consumption of veal and venison and lamb***********
 
CREUTZFELDT JAKOB DISEASE SURVEILLANCE IN THE UNITED KINGDOM THIRD ANNUAL REPORT AUGUST 1994
 
Consumption of venison and veal was much less widespread among both cases and controls. For both of these meats there was evidence of a trend with increasing frequency of consumption being associated with increasing risk of CJD. (not nvCJD, but sporadic CJD...tss)
 
These associations were largely unchanged when attention was restricted to pairs with data obtained from relatives. ...
 
Table 9 presents the results of an analysis of these data.
 
There is STRONG evidence of an association between ‘’regular’’ veal eating and risk of CJD (p = .0.01).
 
Individuals reported to eat veal on average at least once a year appear to be at 13 TIMES THE RISK of individuals who have never eaten veal.
 
There is, however, a very wide confidence interval around this estimate. There is no strong evidence that eating veal less than once per year is associated with increased risk of CJD (p = 0.51).
 
The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).
 
There is some evidence that risk of CJD INCREASES WITH INCREASING FREQUENCY OF LAMB EATING (p = 0.02).
 
The evidence for such an association between beef eating and CJD is weaker (p = 0.14). When only controls for whom a relative was interviewed are included, this evidence becomes a little STRONGER (p = 0.08).
 
snip...
 
It was found that when veal was included in the model with another exposure, the association between veal and CJD remained statistically significant (p = < 0.05 for all exposures), while the other exposures ceased to be statistically significant (p = > 0.05).
 
snip...
 
In conclusion, an analysis of dietary histories revealed statistical associations between various meats/animal products and INCREASED RISK OF CJD. When some account was taken of possible confounding, the association between VEAL EATING AND RISK OF CJD EMERGED AS THE STRONGEST OF THESE ASSOCIATIONS STATISTICALLY. ...
 
snip...
 
In the study in the USA, a range of foodstuffs were associated with an increased risk of CJD, including liver consumption which was associated with an apparent SIX-FOLD INCREASE IN THE RISK OF CJD. By comparing the data from 3 studies in relation to this particular dietary factor, the risk of liver consumption became non-significant with an odds ratio of 1.2 (PERSONAL COMMUNICATION, PROFESSOR A. HOFMAN. ERASMUS UNIVERSITY, ROTTERDAM). (???...TSS)
 
snip...see full report ;
 
 
Thursday, October 10, 2013
 
*************CJD REPORT 1994 increased risk for consumption of veal and venison and lamb**************
 
 
CJD9/10022
 
October 1994
 
Mr R.N. Elmhirst Chairman British Deer Farmers Association Holly Lodge Spencers Lane BerksWell Coventry CV7 7BZ
 
Dear Mr Elmhirst,
 
CREUTZFELDT-JAKOB DISEASE (CJD) SURVEILLANCE UNIT REPORT
 
Thank you for your recent letter concerning the publication of the third annual report from the CJD Surveillance Unit. I am sorry that you are dissatisfied with the way in which this report was published.
 
The Surveillance Unit is a completely independant outside body and the Department of Health is committed to publishing their reports as soon as they become available. In the circumstances it is not the practice to circulate the report for comment since the findings of the report would not be amended. In future we can ensure that the British Deer Farmers Association receives a copy of the report in advance of publication.
 
The Chief Medical Officer has undertaken to keep the public fully informed of the results of any research in respect of CJD. This report was entirely the work of the unit and was produced completely independantly of the the Department.
 
The statistical results reqarding the consumption of venison was put into perspective in the body of the report and was not mentioned at all in the press release. Media attention regarding this report was low key but gave a realistic presentation of the statistical findings of the Unit. This approach to publication was successful in that consumption of venison was highlighted only once by the media ie. in the News at one television proqramme.
 
I believe that a further statement about the report, or indeed statistical links between CJD and consumption of venison, would increase, and quite possibly give damaging credence, to the whole issue. From the low key media reports of which I am aware it seems unlikely that venison consumption will suffer adversely, if at all.
 
 
Thursday, October 10, 2013
 
*** CJD REPORT 1994 increased risk for consumption of veal and venison and lamb
 
 
PLUS, THE CDC DID NOT PUT THIS WARNING OUT FOR THE WELL BEING OF THE DEER AND ELK ;
 
Thursday, May 26, 2011
 
Travel History, Hunting, and Venison Consumption Related to Prion Disease Exposure, 2006-2007 FoodNet Population Survey
 
Journal of the American Dietetic Association Volume 111, Issue 6 , Pages 858-863, June 2011.
 
 
NOR IS THE FDA recalling this CWD positive elk meat for the well being of the dead elk ;
 
Wednesday, March 18, 2009
 
Noah's Ark Holding, LLC, Dawson, MN RECALL Elk products contain meat derived from an elk confirmed to have CWD NV, CA, TX, CO, NY, UT, FL, OK RECALLS AND FIELD CORRECTIONS: FOODS CLASS II
 
 
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 ;
 
 
PRION CONFERENCE 2014 HELD IN ITALY RECENTLY CWD BSE TSE UPDATE
 
> First transmission of CWD to transgenic mice over-expressing bovine prion protein gene (TgSB3985)
 
PRION 2014 - PRIONS: EPIGENETICS and NEURODEGENERATIVE DISEASES – Shaping up the future of prion research
 
Animal TSE Workshop 10.40 – 11.05 Talk Dr. L. Cervenakova First transmission of CWD to transgenic mice over-expressing bovine prion protein gene (TgSB3985)
 
 
P.126: Successful transmission of chronic wasting disease (CWD) into mice over-expressing bovine prion protein (TgSB3985)
 
Larisa Cervenakova,1 Christina J Sigurdson,2 Pedro Piccardo,3 Oksana Yakovleva,1 Irina Vasilyeva,1 Jorge de Castro,1 Paula Saá,1 and Anton Cervenak1 1American Red Cross, Holland Laboratory; Rockville, MD USA; 2University of California; San Diego, CA USA; 3Lab TSE/OBRR /CBER/FDA; Rockville, MD USA
 
Keywords: chronic wasting disease, transmission, transgenic mouse, bovine prion protein
 
Background. CWD is a disease affecting wild and farmraised cervids in North America. Epidemiological studies provide no evidence of CWD transmission to humans. Multiple attempts have failed to infect transgenic mice expressing human PRNP gene with CWD. The extremely low efficiency of PrPCWD to convert normal human PrPC in vitro provides additional evidence that transmission of CWD to humans cannot be easily achieved. However, a concern about the risk of CWD transmission to humans still exists. This study aimed to establish and characterize an experimental model of CWD in TgSB3985 mice with the following attempt of transmission to TgHu mice.
 
Materials and Methods. TgSB3985 mice and wild-type FVB/ NCrl mice were intracranially injected with 1% brain homogenate from a CWD-infected Tga20 mouse (CWD/Tga20). TgSB3985 and TgRM (over-expressing human PrP) were similarly injected with 5% brain homogenates from CWD-infected white-tailed deer (CWD/WTD) or elk (CWD/Elk). Animals were observed for clinical signs of neurological disease and were euthanized when moribund. Brains and spleens were removed from all mice for PrPCWD detection by Western blotting (WB). A histological analysis of brains from selected animals was performed: brains were scored for the severity of spongiform change, astrogliosis, and PrPCWD deposition in ten brain regions.
 
Results. Clinical presentation was consistent with TSE. More than 90% of TgSB3985 and wild-type mice infected with CWD/Tga20, tested positive for PrPres in the brain but only mice in the latter group carried PrPCWD in their spleens. We found evidence for co-existence or divergence of two CWD/ Tga20 strains based on biochemical and histological profiles. In TgSB3985 mice infected with CWD-elk or CWD-WTD, no animals tested positive for PrPCWD in the brain or in the spleen by WB. However, on neuropathological examination we found presence of amyloid plaques that stained positive for PrPCWD in three CWD/WTD- and two CWD/Elk-infected TgSB3985 mice. The neuropathologic profiles in CWD/WTD- and CWD/Elkinfected mice were similar but unique as compared to profiles of BSE, BSE-H or CWD/Tg20 agents propagated in TgSB3985 mice. None of CWD-infected TgRM mice tested positive for PrPCWD by WB or by immunohistochemical detection.
 
Conclusions. To our knowledge, this is the first established experimental model of CWD in TgSB3985. We found evidence for co-existence or divergence of two CWD strains adapted to Tga20 mice and their replication in TgSB3985 mice. Finally, we observed phenotypic differences between cervid-derived CWD and CWD/Tg20 strains upon propagation in TgSB3985 mice. Further studies are underway to characterize these strains.
 
PRION 2014 CONFERENCE
 
CHRONIC WASTING DISEASE CWD
 
A FEW FINDINGS ;
 
Conclusions. To our knowledge, this is the first established experimental model of CWD in TgSB3985. We found evidence for co-existence or divergence of two CWD strains adapted to Tga20 mice and their replication in TgSB3985 mice. Finally, we observed phenotypic differences between cervid-derived CWD and CWD/Tg20 strains upon propagation in TgSB3985 mice. Further studies are underway to characterize these strains.
 
We conclude that TSE infectivity is likely to survive burial for long time periods with minimal loss of infectivity and limited movement from the original burial site. However PMCA results have shown that there is the potential for rainwater to elute TSE related material from soil which could lead to the contamination of a wider area. These experiments reinforce the importance of risk assessment when disposing of TSE risk materials.
 
The results show that even highly diluted PrPSc can bind efficiently to polypropylene, stainless steel, glass, wood and stone and propagate the conversion of normal prion protein. For in vivo experiments, hamsters were ic injected with implants incubated in 1% 263K-infected brain homogenate. Hamsters, inoculated with 263K-contaminated implants of all groups, developed typical signs of prion disease, whereas control animals inoculated with non-contaminated materials did not.
 
Our data establish that meadow voles are permissive to CWD via peripheral exposure route, suggesting they could serve as an environmental reservoir for CWD. Additionally, our data are consistent with the hypothesis that at least two strains of CWD circulate in naturally-infected cervid populations and provide evidence that meadow voles are a useful tool for CWD strain typing.
 
Conclusion. CWD prions are shed in saliva and urine of infected deer as early as 3 months post infection and throughout the subsequent >1.5 year course of infection. In current work we are examining the relationship of prionemia to excretion and the impact of excreted prion binding to surfaces and particulates in the environment.
 
Conclusion. CWD prions (as inferred by prion seeding activity by RT-QuIC) are shed in urine of infected deer as early as 6 months post inoculation and throughout the subsequent disease course. Further studies are in progress refining the real-time urinary prion assay sensitivity and we are examining more closely the excretion time frame, magnitude, and sample variables in relationship to inoculation route and prionemia in naturally and experimentally CWD-infected cervids.
 
Conclusions. Our results suggested that the odds of infection for CWD is likely controlled by areas that congregate deer thus increasing direct transmission (deer-to-deer interactions) or indirect transmission (deer-to-environment) by sharing or depositing infectious prion proteins in these preferred habitats. Epidemiology of CWD in the eastern U.S. is likely controlled by separate factors than found in the Midwestern and endemic areas for CWD and can assist in performing more efficient surveillance efforts for the region.
 
Conclusions. During the pre-symptomatic stage of CWD infection and throughout the course of disease deer may be shedding multiple LD50 doses per day in their saliva. CWD prion shedding through saliva and excreta may account for the unprecedented spread of this prion disease in nature.
 
P.28: Modeling prion species barriers and the new host effect using RT-QuIC
 
Kristen A Davenport, Davin M Henderson, Candace K Mathiason, and Edward A Hoover Prion Research Center; Colorado State University; Fort Collins, CO USA
 
The propensity for trans-species prion transmission is related to the structural characteristics of the enciphering and heterologous PrP, but the exact mechanism remains mostly mysterious.
 
Studies of the effects of primary or tertiary prion protein www.landesbioscience.com Prion 37 structures on trans-species prion transmission have relied upon animal bioassays, making the influence of prion protein structure vs. host co-factors (e.g. cellular constituents, trafficking, and innate immune interactions) difficult to dissect.
 
As an alternative strategy, we are using real-time quaking-induced conversion (RT-QuIC) to investigate the propensity for and the kinetics of trans-species prion conversion. RT-QuIC has the advantage of providing more defined conditions of seeded conversion to study the specific role of native PrP:PrPRES interactions as a component of the species barrier.
 
We are comparing chronic wasting disease (CWD) and bovine spongiform encephalopathy (BSE) prions by seeding each prion into its native host recPrP (full-length bovine recPrP, or white tail deer recPrP) vs. into the heterologous species.
 
Upon establishing the characteristics of intra-species and inter-species prion seeding for CWD and BSE prions, we will evaluate the seeding kinetics and cross-species seeding efficiencies of BSE and CWD passaged into a common new host—feline—shown to be a permissive host for both CWD and BSE.
 
*** We hypothesize that both BSE prions and CWD prions passaged through felines will seed human recPrP more efficiently than BSE or CWD from the original hosts, evidence that the new host will dampen the species barrier between humans and BSE or CWD. The new host effect is particularly relevant as we investigate potential means of trans-species transmission of prion disease.
 
 
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...
 
 
Thursday, July 03, 2014
 
How Chronic Wasting Disease is affecting deer population and what’s the risk to humans and pets?
 
 
Tuesday, July 01, 2014
 
CHRONIC WASTING DISEASE CWD TSE PRION DISEASE, GAME FARMS, AND POTENTIAL RISK FACTORS THERE FROM
 
 
Saturday, June 14, 2014
 
Rep. Rosa DeLauro (D-CT) Calls for Briefing on Beef Recalled for Mad Cow Potential Rep. Rosa DeLauro (D-CT)
 
 
Monday, June 02, 2014
 
*** Confirmed Variant CJD Case in Texas ***
 
 
Tuesday, March 11, 2014
 
Science and Technology Committee Oral evidence: Blood, tissue and organ screening, HC 990 Wednesday 5 March 2014 SPORADIC CJD
 
Actually, it is nearer 2 per million per year of the population will develop sporadic CJD, but your lifetime risk of developing sporadic CJD is about 1 in 30,000. So that has not really changed. When people talk about 1 per million, often they interpret that as thinking it is incredibly rare. They think they have a 1-in-a-million chance of developing this disease. You haven’t. You’ve got about a 1-in-30,000 chance of developing it.
 
 
Sunday, January 19, 2014
 
*** National Prion Disease Pathology Surveillance Center Cases Examined1 as of January 8, 2014 ***
 
 
Saturday, April 19, 2014
 
Exploring the zoonotic potential of animal prion diseases: In vivo and in vitro approaches
 
 
Sunday, July 06, 2014
 
*** Dietary Risk Factors for Sporadic Creutzfeldt-Jakob Disease: A Confirmatory Case-Control Study ***
 
Conclusions—The a priori hypotheses were supported.
 
*Consumption of various meat products may be one method of transmission of the infectious agent for sCJD.
 
 
Monday, June 23, 2014
 
*** PRION 2014 CONFERENCE CHRONIC WASTING DISEASE CWD
 
 
Monday, June 23, 2014
 
*** PRION 2014 CONFERENCE TYPICAL AND ATYPICAL BSE AND CJD REPORT UPDATES
 
 
Transmissible Spongiform Encephalopathy TSE Prion Disease North America 2014
 
Transmissible Spongiform Encephalopathy TSE Prion Disease have now been discovered in a wide verity of species across North America. typical C-BSE, atypical L-type BASE BSE, atypical H-type BSE, atypical H-G BSE, of the bovine, typical and atypical Scrapie strains, in sheep and goats, with atypical Nor-98 Scrapie spreading coast to coast in about 5 years. Chronic Wasting Disease CWD in cervid is slowly spreading without any stopping it in Canada and the USA and now has mutated into many different strains. Transmissible Mink Encephalopathy TME outbreaks. These Transmissible Spongiform Encephalopathy TSE Prion Disease have been silently mutating and spreading in different species in North America for decades.
 
The USDA, FDA, et al have assured us of a robust Triple BSE TSE prion Firewall, of which we now know without a doubt, that it was nothing but ink on paper. Since the 1997 mad cow feed ban in the USA, literally tons and tons of banned mad cow feed has been put out into commerce, never to return, as late as December of 2013, serious, serious breaches in the FDA mad cow feed ban have been documented. The 2004 enhanced BSE surveillance program was so flawed, that one of the top TSE prion Scientist for the CDC, Dr. Paul Brown stated ; 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. see ; http://www.upi.com/Health_News/2006/03/15/Analysis-What-that-mad-cow-means/UPI-12841142465253/
 
The BSE surveillance and testing have also been proven to be flawed, and the GAO and OIG have both raised serious question as to just how flawed it has been (see GAO and OIG reports). North America has more documented TSE prion disease, in different documented species (excluding the Zoo BSE animals in the EU), then any other place on the Globe. This does not include the very likelihood that TSE prion disease in the domestic feline and canine have been exposed to high doses of the TSE prion disease vid pet food. To date, it’s still legal to include deer from cwd zone into pet food or deer food. Specified Risk Material i.e. SRM bans still being breach, as recently as just last month.
 
nvCJD or what they now call vCJD, another case documented in Texas last month, with very little information being released to the public on about this case? with still the same line of thought from federal officials, ‘it can’t happen here’, so another vCJD blamed on travel of a foreign animal disease from another country, while ignoring all the BSE TSE Prion risk factors we have here in the USA and Canada, and the time that this victim and others, do spend in the USA, and exposed to these risk factors, apparently do not count in any way with regard to risk factor. a flawed process of risk assessment.
 
sporadic CJD, along with new TSE prion disease in humans, of which the young are dying, of which long duration of illness from onset of symptoms to death have been documented, only to have a new name added to the pot of prion disease i.e. sporadic GSS, sporadic FFI, and or VPSPR. I only ponder how a familial type disease could be sporadic with no genetic link to any family member? when the USA is the only documented Country in the world to have documented two different cases of atypical H-type BSE, with one case being called atypical H-G BSE with the G meaning Genetic, with new science now showing that indeed atypical H-type BSE is very possible transmitted to cattle via oral transmission (Prion2014). sporadic CJD and VPSPR have been rising in Canada, USA, and the UK, with the same old excuse, better surveillance. You can only use that excuse for so many years, for so many decades, until one must conclude that CJD TSE prion cases are rising. a 48% incease in CJD in Canada is not just a blip or a reason of better surveillance, it is a mathematical rise in numbers. More and more we are seeing more humans exposed in various circumstance in the Hospital, Medical, Surgical arenas to the TSE Prion disease, and at the same time in North America, more and more humans are becoming exposed to the TSE prion disease via consumption of the TSE prion via deer and elk, cattle, sheep and goats, and for those that are exposed via or consumption, go on to further expose many others via the iatrogenic modes of transmission of the TSE prion disease i.e. friendly fire. I pondered this mode of transmission via the victims of sporadic FFI, sporadic GSS, could this be a iatrogenic event from someone sub-clinical with sFFI or sGSS ? what if?
 
Two decades have passed since Dr. Ironside first confirmed his first ten nvCJD victims in 1995. Ten years later, 2005, we had Dr. Gambetti and his first ten i.e. VPSPR in younger victims. now we know that indeed VPSPR is transmissible. yet all these TSE prion disease and victims in the USA and Canada are being pawned off as a spontaneous event, yet science has shown, the spontaneous theory has never been proven in any natural case of TSE prion disease, and scientist have warned, that they have now linked some sporadic CJD cases to atypical BSE, to atypical Scrapie, and to CWD, yet we don’t here about this in the public domain. We must make all human and animal TSE prion disease reportable in every age group, in ever state and internationally, we must have a serious re-evaluation and testing of the USA cattle herds, and we must ban interstate movement of all cervids. Any voluntary effort to do any of this will fail. Folks, we have let the industry run science far too long with regards to the TSE prion disease. While the industry and their lobbyist continues to funnel junk science to our decision policy makers, Rome burns. ...end
 
REFERENCES
 
[all scientific peer review studies and other scientific information I have put into blogs, to shorten reference data. I DO NOT advertise or make money from this, this information is for education use...lost my mom to the hvCJD, and just made a promise, never forget, and never let them forget. ...TSS]
 
Sunday, June 29, 2014
 
*** Transmissible Spongiform Encephalopathy TSE Prion Disease North America 2014 ***
 
 
UNITED STATES OF AMERICA
 
FOOD AND DRUG ADMINISTRATION
 
CENTER FOR BIOLOGICS EVALUATION AND RESEARCH
 
+ + + + +
 
TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES ADVISORY
 
COMMITTEE
 
MEETING
 
THURSDAY,
 
FEBRUARY 12, 2004
 
Sunday, March 09, 2014
 
A Creutzfeldt-Jakob Disease (CJD) Lookback Study: Assessing the Risk of Blood Borne Transmission of Classic Forms of Creutzfeldt-Jakob Disease
 
FDA TSEAC CIRCUS AND TRAVELING ROAD SHOW FOR THE TSE PRION DISEASES
 
 
 
 
Singeltary Submission 2001 to FDA et al
 
Advisors and Consultants Staff
 
2001 Advisory Committee (short version).
 
I understand the reason of having to shorten my submission, but only hope that you add it to a copy of the long version, for members to take and read at their pleasure, (if cost is problem, bill me, address below). So when they realize some time in the near future of the 'real' risks i speak of from human/animal TSEs and blood/surgical products. I cannot explain the 'real' risk of this in 5 or 10 minutes at some meeting, or on 2 or 3 pages, but will attempt here:
 
remember AIDS/HIV, 'no problem to heterosexuals in the U.S.? no need to go into that, you know of this blunder:
 
DO NOT make these same stupid mistakes again with human/animal TSE's aka MADCOW DISEASE.
 
snip...
 
I am beginning to think that the endless attempt to track down and ban, potential victims from known BSE Countries from giving blood will be futile. You would have to ban everyone on the Globe eventually? AS well, I think we MUST ACT SWIFTLY to find blood test for TSE's, whether it be blood test, urine test, eyelid test, anything at whatever cost, we need a test FAST. DO NOT let the incubation time period of these TSEs fool you.
 
To think of Scrapie as the prime agent to compare CJD, but yet overlook the Louping-ill vaccine event in 1930's of which 1000's of sheep where infected by scrapie from a vaccine made of scrapie infected sheep brains, would be foolish. I acquired this full text version of the event which was recorded in the Annual Congress of 1946 National Vet. Med. Ass. of Great Britain and Ireland from the BVA and the URL is posted in my (long version).
 
U.S.A. should make all human/animal TSE's notifiable at all ages, with requirements for a thorough surveillance and post-mortem examinations free of charge, if you are serious
 
see full text Singeltary 2001 submission to the FDA et al ;
 
 
Owens, Julie
 
From: Terry S. Singeltary Sr. [flounder9@verizon.net]
 
Sent: Monday, July 24, 2006 1:09 PM
 
To: FSIS RegulationsComments
 
Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE) Page 1 of 98
 
 
response to Singeltary
 
 
Sent: Friday, December 01, 2006 2:59 PM
 
Subject: Re: TSE advisory committee for the meeting December 15, 2006 [TSS SUBMISSION
 
snip...
 
ONE FINAL COMMENT PLEASE, (i know this is long Dr. Freas but please bear with me)
 
THE USA is in a most unique situation, one of unknown circumstances with human and animal TSE. THE USA has the most documented TSE in different species to date, with substrains growing in those species (BSE/BASE in cattle and CWD in deer and elk, there is evidence here with different strains), and we know that sheep scrapie has over 20 strains of the typical scrapie with atypical scrapie documented and also BSE is very likely to have passed to sheep. all of which have been rendered and fed back to animals for human and animal consumption, a frightening scenario. WE do not know the outcome, and to play with human life around the globe with the very likely TSE tainted blood from the USA, in my opinion is like playing Russian roulette, of long duration, with potential long and enduring consequences, of which once done, cannot be undone.
 
These are the facts as i have come to know through daily and extensive research of TSE over 9 years, since 12/14/97. I do not pretend to have all the answers, but i do know to continue to believe in the ukbsenvcjd only theory of transmission to humans of only this one strain from only this one TSE from only this one part of the globe, will only lead to further failures, and needless exposure to humans from all strains of TSE, and possibly many more needless deaths from TSE via a multitude of proven routes and sources via many studies with primates and rodents and other species. ...
 
Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518
 
snip... 48 pages...
 
 
Wednesday, January 15, 2014
 
*** INFECTION PREVENTION AND CONTROL OF CJD, VCJD AND OTHER HUMAN PRION DISEASES IN HEALTHCARE AND COMMUNITY SETTINGS Variably Protease-Sensitive Prionopathy (VPSPr) January 15, 2014
 
 
Sunday, April 06, 2014
 
SPORADIC CJD and the potential for zoonotic transmission there from, either directly or indirectly via friendly fire iatrogenic mode, evidence to date
 
 
Thursday, January 23, 2014
 
Medical Devices Containing Materials Derived from Animal Sources (Except for In Vitro Diagnostic Devices) [Docket No. FDA–2013–D–1574]
 
 
Wednesday, December 11, 2013
 
*** Detection of Infectivity in Blood of Persons with Variant and Sporadic Creutzfeldt-Jakob Disease ***
 
 
Thursday, November 14, 2013
 
Prion diseases in humans: Oral and dental implications
 
 
Tuesday, May 28, 2013
 
Late-in-life surgery associated with Creutzfeldt-Jakob disease: a methodological outline for evidence-based guidance
 
 
Sunday, June 9, 2013
 
TSEAC March 14, 2013: Transmissible Spongiform Encephalopathies Advisory Committee Meeting Webcast
 
 
Tuesday, May 21, 2013
 
CJD, TSE, PRION, BLOOD Abstracts of the 23rd Regional Congress of the International Society of Blood Transfusion, Amsterdam, The Netherlands, June 2-5, 2013
 
 
Tuesday, March 5, 2013
 
Use of Materials Derived From Cattle in Human Food and Cosmetics; Reopening of the Comment Period FDA-2004-N-0188-0051 (TSS SUBMISSION)
 
FDA believes current regulation protects the public from BSE but reopens comment period due to new studies
 
 
Sunday, June 9, 2013
 
TSEAC March 14, 2013: Transmissible Spongiform Encephalopathies Advisory Committee Meeting Webcast
 
 
Friday, July 19, 2013
 
Beaumont Hospital in Dublin assessing patients for CJD
 
 
Saturday, September 21, 2013
 
CJD CONFIRMED in patient at New Hampshire Department of Health and Human Services (DHHS), Catholic Medical Center (CMC), and the Manchester Health Department (MHD)
 
 
Thursday, September 26, 2013
 
Minimise transmission risk of CJD and vCJD in healthcare settings Guidance
 
 
Wednesday, June 19, 2013
 
Spreading of tau pathology in Alzheimer's disease by cell-to-cell transmission
 
 
Sunday, October 27, 2013
 
A Kiss of a Prion: New Implications for Oral Transmissibility
 
 
Thursday, January 17, 2013
 
TSE guidance, surgical, dental, blood risk factors, Part 4 Infection control of CJD, vCJD and other human prion diseases in healthcare and community settings (updated January 2013)
 
 
Thursday, October 25, 2012
 
Current limitations about the cleaning of luminal endoscopes and TSE prion risk factors there from
 
Article in Press
 
 
Wednesday, February 1, 2012
 
CJD and PLASMA / URINE PRODUCTS EMA Position Statements Alberto Ganan Jimenez, European Medicines Agency PDA TSE Safety Forum, 30 June 2011
 
 
ENFORCEMENT REPORT
 
Enforcement Report - Week of August 29, 2012
 
Class II, Blood products, collected from a donor who was at risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed. Belle Bonfils Memorial Blood ...
 
 
Thursday, August 16, 2012
 
Blood products, collected from a donor who was at risk for variant Creutzfeldt-Jakob disease ( vCJD) USA JUNE, JULY, AUGUST 2012
 
 
Sunday, June 3, 2012
 
A new neurological disease in primates inoculated with prion-infected blood or blood components
 
 
Friday, June 29, 2012
 
Highly Efficient Prion Transmission by Blood Transfusion
 
 
Thursday, August 16, 2012
 
Blood products, collected from a donor who was at risk for variant Creutzfeldt-Jakob disease ( vCJD) USA JUNE, JULY, AUGUST 2012
 
 
price of prion poker goes up again $$$
 
Monday, June 11, 2012
 
Guidance for Industry Draft Guidance for Industry: Amendment to “Guidance for Industry: Revised Preventive Measures to Reduce the Possible Risk of Transmission of Creutzfeldt-Jakob Disease and Variant Creutzfeldt-Jakob Disease by Blood and Blood Products”
 
 
Saturday, August 18, 2012
 
RedCross Request Jerome H. Holland Laboratory is collecting small volumes of blood from patients afflicted with various forms of transmissible spongiform encephalopathies (TSE)/prion diseases and their blood-related family members 2012
 
 
Monday, August 13, 2012
 
Summary results of the second national survey of abnormal prion prevalence in archived appendix specimens August 2012
 
 
Friday, August 24, 2012
 
Iatrogenic prion diseases in humans: an update
 
 
Thursday, September 06, 2012
 
HANSARD, vCJD, blood, FFP, 5 Sep 2012 : Column 353W SaBTO
 
 
Thursday, April 12, 2012
 
Health professions and risk of sporadic Creutzfeldt–Jakob disease, 1965 to 2010
 
Eurosurveillance, Volume 17, Issue 15, 12 April 2012
 
Research articles
 
 
Saturday, January 16, 2010
 
*** Evidence For CJD TSE Transmission Via Endoscopes 1-24-3 re-Singeltary to Bramble et al
 
Evidence For CJD/TSE Transmission Via Endoscopes
 
From Terry S. Singletary, Sr flounder@wt.net 1-24-3
 
Terry S. Singeltary Sr., P.O. BOX 42, Bacliff, Texas 77518 USA
 
 
Professor Michael Farthing wrote:
 
*** Louise Send this to Bramble (author) for a comment before we post. Michael
 
 
Tuesday, July 31, 2012
 
11 patients may have been exposed to fatal disease Creutzfeldt-Jakob Disease CJD Greenville Memorial Hospital
 
 
Thursday, August 02, 2012
 
CJD case in Saint John prompts letter to patients Canada CJD case in Saint John prompts letter to patients
 
 
Saturday, February 12, 2011
 
Another Pathologists dies from CJD, another potential occupational death ?
 
another happenstance of bad luck, a spontaneous event from nothing, or friendly fire ???
 
 
Monday, February 7, 2011
 
*** FDA’s Currently-Recommended Policies to Reduce the Possible Risk of Transmission of CJD and vCJD by Blood and Blood Products 2011 ???
 
 
Thursday, July 08, 2010
 
GLOBAL CLUSTERS OF CREUTZFELDT JAKOB DISEASE - A REVIEW 2010
 
 
Sunday, May 10, 2009
 
*** Meeting of the Transmissible Spongiform Encephalopathies Committee On June 12, 2009 (Singeltary submission)
 
 
Thursday, January 29, 2009
 
Medical Procedures and Risk for Sporadic Creutzfeldt-Jakob Disease, Japan, 1999-2008 (WARNING TO Neurosurgeons and Ophthalmologists) Volume 15, Number 2-February 2009 Research
 
 
Sunday, August 09, 2009
 
CJD...Straight talk with...James Ironside...and...Terry Singeltary... 2009
 
 
Tuesday, August 18, 2009
 
* BSE-The Untold Story - joe gibbs and singeltary 1999 - 2009
 
 
Tonometer disinfection practice in the United Kingdom: A national survey
 
 
Tuesday, August 12, 2008
 
Biosafety in Microbiological and Biomedical Laboratories Fifth Edition 2007 (occupational exposure to prion diseases)
 
 
Monday, December 31, 2007
 
Risk Assessment of Transmission of Sporadic Creutzfeldt-Jakob Disease in Endodontic Practice in Absence of Adequate Prion Inactivation
 
 
Subject: CJD: update for dental staff
 
Date: November 12, 2006 at 3:25 pm PST
 
1: Dent Update. 2006 Oct;33(8):454-6, 458-60.
 
CJD: update for dental staff.
 
 
Re: vCJD in the USA * BSE in U.S. 15 November 1999 Terry S Singeltary, NA
 
CWD is just a small piece of a very big puzzle. I have seen while deer hunting, deer, squirrels and birds, eating from cattle feed troughs where they feed cattle, the high protein cattle by products, at least up until Aug. 4, 1997. So why would it be so hard to believe that this is how they might become infected with a TSE. Or, even by potentially infected land. It's been well documented that it could be possible, from scrapie.
 
It was proven in Oprah Winfrey's trial, that Cactus Cattle feeders, sent neurologically ill cattle, some with encephalopathy stamped on the dead slips, were picked up and sent to the renders, along with sheep carcasses.
 
 
U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well...
 
2 January 2000 Terry S Singeltary
 
The exact same recipe for B.S.E. existed in the U.S. for years and years. In reading over the Qualitative Analysis of BSE Risk Factors-1, this is a 25 page report by the USDA:APHIS:VS. It could have been done in one page. The first page, fourth paragraph says it all;
 
"Similarities exist in the two countries usage of continuous rendering technology and the lack of usage of solvents, however, large differences still remain with other risk factors which greatly reduce the potential risk at the national level."
 
Then, the next 24 pages tries to down-play the high risks of B.S.E. in the U.S., with nothing more than the cattle to sheep ratio count, and the geographical locations of herds and flocks. That's all the evidence they can come up with, in the next 24 pages.
 
Something else I find odd, page 16;
 
"In the United Kingdom there is much concern for a specific continuous rendering technology which uses lower temperatures and accounts for 25 percent of total output. This technology was _originally_ designed and imported from the United States. However, the specific application in the production process is _believed_ to be different in the two countries."
 
A few more factors to consider, page 15;
 
"Figure 26 compares animal protein production for the two countries. The calculations are based on slaughter numbers, fallen stock estimates, and product yield coefficients. This approach is used due to variation of up to 80 percent from different reported sources. At 3.6 million tons, the United States produces 8 times more animal rendered product than the United Kingdom."
 
"The risk of introducing the BSE agent through sheep meat and bone meal is more acute in both relative and absolute terms in the United Kingdom (Figures 27 and 28). Note that sheep meat and bone meal accounts for 14 percent, or 61 thousand tons, in the United Kingdom versus 0.6 percent or 22 thousand tons in the United States. For sheep greater than 1 year, this is less than one-tenth of one percent of the United States supply."
 
"The potential risk of amplification of the BSE agent through cattle meat and bone meal is much greater in the United States where it accounts for 59 percent of total product or almost 5 times more than the total amount of rendered product in the United Kingdom."
 
Considering, it would only take _one_ scrapie infected sheep to contaminate the feed. Considering Scrapie has run rampant in the U.S. for years, as of Aug. 1999, 950 scrapie infected flocks. Also, Considering only one quarter spoonful of scrapie infected material is lethal to a cow. Considering all this, the sheep to cow ration is meaningless. As I said, it's 24 pages of B.S.e.
 
To be continued...
 
Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA
 
 
Letters
 
JAMA. 2001;285(6):733-734.
 
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
 
Terry S. Singeltary, Sr Bacliff, Tex
 
Since this article does not have an abstract, we have provided the first 150 words of the full text.
 
KEYWORDS: creutzfeldt-jakob disease, diagnosis. 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.
 
References 1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323.
 
 
Published March 26, 2003
 
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States
 
Terry S. Singeltary, retired (medically)
 
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?
 
Published March 26, 2003
 
 
14th ICID International Scientific Exchange Brochure - Final Abstract Number: ISE.114
 
Session: International Scientific Exchange
 
Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009
 
T. Singeltary Bacliff, TX, USA
 
Background: An update on atypical BSE and other TSE in North America. Please remember, the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been documented in North America, along with the typical scrapie's, and atypical Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these TSE in different species have been rendered and fed to food producing animals for humans and animals in North America (TSE in cats and dogs ?), and that the trading of these TSEs via animals and products via the USA and Canada has been immense over the years, decades.
 
Methods: 12 years independent research of available data
 
Results: I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc.
 
Conclusion: I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.
 
 
The Lancet Infectious Diseases, Volume 3, Issue 8, Page 463, August 2003 doi:10.1016/S1473-3099(03)00715-1
 
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 ...
 
 
 
-------- Original Message --------
 
Subject: Tracking spongiform encephalopathies in North America LANCET INFECTIOUS DISEASE Volume 3, Number 8 01 August 2003
 
Date: Tue, 29 Jul 2003 17:35:30 –0500
 
From: "Terry S. Singeltary Sr." Reply-To: Bovine Spongiform Encephalopathy
 
To: BSE-L@uni-karlsruhe.de
 
Volume 3, Number 8 01 August 2003
 
Tracking spongiform encephalopathies in North America
 
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 disease (CJD). So he decided to gather hundreds of documents on transmissible spongiform encephalopathies (TSE) and realised that if Britons could get variant CJD from bovine spongiform encephalopathy (BSE), Americans might get a similar disorder from chronic wasting disease (CWD)the relative of mad cow disease seen among deer and elk in the USA. Although his feverish search did not lead him to the smoking gun linking CWD to a similar disease in North American people, it did uncover a largely disappointing situation.
 
Singeltary was greatly demoralised at the few attempts to monitor the occurrence of CJD and CWD in the USA. Only a few states have made CJD reportable. Human and animal TSEs should be reportable nationwide and internationally, he complained in a letter to the Journal of the American Medical Association (JAMA 2003; 285: 733). I hope that the CDC does not continue to expect us to still believe that the 85% plus of all CJD cases which are sporadic are all spontaneous, without route or source.
 
Until recently, CWD was thought to be confined to the wild in a small region in Colorado. But since early 2002, it has been reported in other areas, including Wisconsin, South Dakota, and the Canadian province of Saskatchewan. Indeed, the occurrence of CWD in states that were not endemic previously increased concern about a widespread outbreak and possible transmission to people and cattle.
 
To date, experimental studies have proven that the CWD agent can be transmitted to cattle by intracerebral inoculation and that it can cross the mucous membranes of the digestive tract to initiate infection in lymphoid tissue before invasion of the central nervous system. Yet the plausibility of CWD spreading to people has remained elusive.
 
Part of the problem seems to stem from the US surveillance system. CJD is only reported in those areas known to be endemic foci of CWD. Moreover, US authorities have been criticised for not having performed enough prionic tests in farm deer and elk.
 
Although in November last year the US Food and Drug Administration issued a directive to state public-health and agriculture officials prohibiting material from CWD-positive animals from being used as an ingredient in feed for any animal species, epidemiological control and research in the USA has been quite different from the situation in the UK and Europe regarding BSE.
 
Getting data on TSEs in the USA from the government is like pulling teeth, Singeltary argues. You get it when they want you to have it, and only what they want you to have.
 
Norman Foster, director of the Cognitive Disorders Clinic at the University of Michigan (Ann Arbor, MI, USA), says that current surveillance of prion disease in people in the USA is inadequate to detect whether CWD is occurring in human beings; adding that, the cases that we know about are reassuring, because they do not suggest the appearance of a new variant of CJD in the USA or atypical features in patients that might be exposed to CWD. However, until we establish a system that identifies and analyses a high proportion of suspected prion disease cases we will not know for sure. The USA should develop a system modelled on that established in the UK, he points out.
 
Ali Samii, a neurologist at Seattle VA Medical Center who recently reported the cases of three hunterstwo of whom were friendswho died from pathologically confirmed CJD, says that at present there are insufficient data to claim transmission of CWD into humans; adding that [only] by asking [the questions of venison consumption and deer/elk hunting] in every case can we collect suspect cases and look into the plausibility of transmission further. Samii argues that by making both doctors and hunters more aware of the possibility of prions spreading through eating venison, doctors treating hunters with dementia can consider a possible prion disease, and doctors treating CJD patients will know to ask whether they ate venison.
 
CDC spokesman Ermias Belay says that the CDC will not be investigating the [Samii] cases because there is no evidence that the men ate CWD-infected meat. He notes that although the likelihood of CWD jumping the species barrier to infect humans cannot be ruled out 100% and that [we] cannot be 100% sure that CWD does not exist in humans& the data seeking evidence of CWD transmission to humans have been very limited.
 
 
Singeltary submission to PLOS ;
 
No competing interests declared.
 
see full text ;
 
 
*** PLOS Molecular, Biochemical and Genetic Characteristics of BSE in Canada
 
Singeltary reply ;
 
 
Monday, July 24, 2006 1:09 PM
 
FSIS Regulations
 
Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE) Page 1 of 98
 
 
FSIS, USDA, REPLY TO SINGELTARY
 
 
 
Creutzfeldt-Jakob Disease Public Health Crisis
 
 
 
 
layperson
 
MOM DOD 12/14/97 confirm ‘hvCJD’ just made a promise to mom, NEVER FORGET! and never let them forget. ...
 
Terry S. Singeltary Sr.