vCJD transfusion-associated Fourth Case UK

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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

Friday, November 13, 2015

No evidence of asymptomatic variant CJD infection in immunodeficiency patients treated with UK-sourced immunoglobulin ?

No evidence of asymptomatic variant CJD infection in immunodeficiency patients treated with UK-sourced immunoglobulin


M. R. Helbert1,*, C. Bangs1, M. Bishop2, A. Molesworth2 and J. Ironside2 Article first published online: 3 NOV 2015


DOI: 10.1111/vox.12358


© 2015 International Society of Blood Transfusion


Keywords:immunoglobulins; prions


Surveillance of 75 immunodeficiency patients exposed to UK-sourced immunoglobulin, including batches derived from donors who went on to develop vCJD, has not detected any clinical cases of vCJD, or of asymptomatic infection in 15 patients with available tissue samples of sufficient quality for testing.



‘’No evidence of asymptomatic variant CJD infection in immunodeficiency patients treated with UK-sourced immunoglobulin’’


in my opinion, that title should read ;


‘no evidence of asymptomatic variant CJD infection of immunodeficiency patients treated with UK-sourced immunoglobulin from 15 out of 75, of which 60 were NOT of sufficient quality for testing’’'


SO, the other 60 could/might have had a vCJD infection in that study?


Inquiry: variant Creutzfeldt-Jakob Disease (vCJD)


Submission by Dr Matthew Helbert on behalf of the UK Primary Immunodeficiency Network


1 Executive summary


Immunoglobulin is a type of plasma product. About 5000 PID patients in the UK rely on life long immunoglobulin infusions. In 1997 use of British plasma to manufacture immunoglobulin was stopped as a precautionary measure. PID patients subsequently received immunoglobulin manufactured from non UK sourced plasma. However by 1997, some PID patients had already been exposed to immunoglobulin manufactured from blood donated by people who went on to develop vCJD.


In 2004, an individual risk assessment was carried out, using tracking of which batches of immunoglobulin had been infused into which patients. The risk assessment suggested a low risk of exposure for any given PID patient. No special precautions were subsequently required for PID patients, who have thus not experienced stigmatisation or anxiety. Since 2006, ongoing surveillance, relying on tissue sampling, has shown no evidence of prion infection in PID patients. In the future, it would be difficult to recommend that patients choose immunoglobulin manufactured from UK sourced plasma, unless each donor had been screened for prion infection with a well evaluated blood or urine test. The safe reintroduction of UK sourced immunoglobulin would be further enhanced by electronic batch tracking, to facilitate individual risk assessment and product recall in the event of further prion infection (or other pathogen) outbreaks.




UKPIN ( the professional body for doctors, nurses and scientists involved in the care of patients with primary immunodeficiency disorders (PID). In 2006, UKPIN initiated a study on surveillance of prion infection in these patients, lead by Dr M Helbert. Contact: Dr Matthew Helbert, Consultant Immunologist, Central Manchester University Hospitals NHS FT. 0161 276 6468


3 Background to immunoglobulin and prions


Approximately 5000 primary immunodeficiency patients in the UK require lifelong injections with immunoglobulin. Immunoglobulin is manufactured from donated plasma, along with Factor VIII (for haemophilia). Manufacture of these plasma products (or 'blood products') requires pooling several thousand plasma donations. As a result, plasma products have transmitted blood borne pathogens and, in the past, immunoglobulin and has caused outbreaks of hepatitis C.


Factor VIII is manufactured slightly differently has been contaminated with HIV and hepatitis C virus. As a precautionary response to the BSE and vCJD epidemics, processing of UK sourced plasma ceased in 1997. Since then, PID patients have only received immunoglobulin manufactured from plasma imported from overseas. There has been no evidence of prion transmission via immunoglobulin, although there is some evidence that a haemophiliac patient was infected via Factor VIII.


4 2004 prion infection risk assessment


Once it became clear that some blood donors went on to develop vCJD, The CJD Incidents Panel initiated risk assessment programmes for patients who had received UK sourced plasma products, as part of the strategy to reduce the risk of secondary infection. UKPIN and patient representatives decided that an individual risk assessment approach was best for PID patients: Data were collected on how much of any given implicated batch of immunoglobulin each patient had received. A noteworthy observation is that it was often very difficult to track down which batches of immunoglobulin had been dispensed by pharmacies and infused into patients. To do this, paper records had to be searched by hand for evidence of batch tracking. However, the result of the individual risk assessments was that no PID patient was assessed at being at more than 1% risk of exposure to abnormal prions. Thus PID patients did not require special measures, such as quarantining of endoscopes after procedures. On the other hand, the haemophilia community chose to undergo a collective risk assessment, and when this established that some patients could be at risk, several thousand haemophiliacs faced difficulties arranging surgical and endoscopic procedures, along with stigmatisation and anxiety.


5 Immunoglobulin manufacture since 1997


In the UK, plasma is currently discarded (at a value of about £65 per donation – G Grazzini, Blood Transfusion, 2013). Immunoglobulin continues to be manufactured in the UK and abroad, using pooled plasma sourced overseas. Unlike any other blood borne infection, no laboratory screening test is available to rule out prion infection in donors. Donor screening questions are used to eliminate those perceived as being at high risk of prion infection, although given uncertainties of the biology of prion infection, the utility of these is very unclear. The manufacturing process includes steps to reduce the infectivity of plasma, should a donor have undiagnosed prion infection. Immunoglobulin products used in the EU contain statements about blood borne pathogens in their Summaries of Product Characteristics.


6 The impact of the vCJD epidemic on PID patients - 2004 onwards


British PID patients now only receive immunoglobulin manufactured from non UK plasma. This has placed PID patients at the mercy of a finite global immunoglobulin market, with occasional supply problems and increases in prices. The DH now operates a demand management scheme for immunoglobulins. Even though currently available immunoglobulins are perceived as safe from the point of view of prion infection, good practice is to inform patients of this theoretical risk as part of obtaining informed consent to start, or stay on, immunoglobulin. PID patients are not be able to be blood donors because they have received plasma product infusions, but also because of their underlying illness. Following the 2004 risk assessment, PID patients have not required other measures to reduce secondary infection. Despite these ongoing concerns, these is no evidence that prion infection has been a significant source of anxiety for British PID patients, including the subset exposed to UK sourced immunoglobulin prior to 1997. I am not aware of any prion – specific life insurance problems in this group.


7 PID Prion surveillance project 2006 onwards


In 2006, the DH funded a surveillance programme for PID patients exposed to UK sourced immunoglobulin. To date, 60 patients have given consent to participate and tissue samples are available on just over half of these. These samples were collected during routine surgical procedures or post mortem a median of 8 years after potential exposure to prion infection. No tissue samples show evidence of prion infection. The major limitations on these data are the long incubation period of prion infection after expsoure, the inevitable poor access to tissue samples and absence of a blood or urine test. Thus, these data can not yet reassure that no PID patients were infected with prions in the 1990s or, by inference, that it would be safe to re commence processing of British plasma.


8 Should UK sourced plasma be used to manufacture immunoglobulin?


There are no data on the opinions of PID patients or their clinicians on this question. However, any patient embarking on life long immunoglobulin treatment could receive either a product manufactured from plasma sourced overseas, from a country with no history of BSE or vCJD, or UK sourced plasma. In the UK there is thought to be a measurable rate of background asymptomatic prion infection, but no blood or urine test to screen individual donors. Even though prion transmission has not been shown to take place via immunoglobulin, it would be illogical for a patient to choose immunoglobulin manufactured from UK sourced plasma, until a sensitive prion screening blood or urine test is in routine use.


9 Lessons Learnt


9.1 If plasma processing is resumed in the UK, robust means of batch tracking are required for on going risk assessment. The process must enable rapid identification of which batches of product have been infused into which patients and also enable rapid product recall in the event of any future pathogen outbreaks.


9.2 Individual risk assessment was negative in all PID patients. No PID patients required expensive quarantining of equipment and there has been no lasting anxiety or stigmatisation. In the event of future outbreaks or donor infection, we recommend this approach over 'umbrella' risk assessment.


9.3 A blood or urine test for asymptomatic prion infection is a pre requisite for the re establishment of processing of UK sourced plasma. There is a reasonable chance that such a test would be adopted for screening plasma donors from outside the UK. A blood or urine test would be an invaluable research tool, for example to validate our prion surveillance study.


10 Response to TOR


Are UK policies governing who can donate blood and blood products, tissues and organs sufficiently evidence-based? Is NHS Blood and Transplant overly restrictive about who can donate, or should greater precautions be taken to further reduce risk?


Current policies are sufficiently evidence based given current technical and scientific constraints. A well evaluated prion blood or urine test would further mitigate risk. Is the Government and its scientific advisory structure sufficiently responsive to the threat posed by emerging diseases being transmitted through blood and blood products, tissues and organs?


Up until about 2011 there were clear lines of accountability and management. For example, had our study detected a PID patient with prion infection, I would have known who to contact the CJD Incidents Panel – and this was enshrined I the ethics application for our project. However, this is no longer the case and I do not know who to contact or how they would respond. Has the threat of ongoing transmission of vCJD through the blood and blood product supply been adequately mitigated?


Blood supply – the risk would be better mitigated with a well evaluated prion blood or urine test.


Blood products (plasma products) - currently, well mitigated, although with an expensive solution, ie using products manufactured from non UK plasma.


What are the strengths and weaknesses of NHS Blood and Transplant’s strategy, “Taking Organ Transplantation to 2020”? What further changes could be made to safely increase the supply of blood and blood products, tissues and organs?


No specific response.


What lessons could be learnt from the screening and donation practices of other countries? No specific response.



Inquiry: variant CreutzfeldtJakob Disease (vCJD)


Submitted by Primary Immunodeficiency UK ( Director: Dr Susan Walsh


1. About PID UK


PID UK is a national organisation working for patients is an organisation supporting people affected by primary immunodeficiencies in the UK.


Our aims are to help ensure that every individual and family affected by a primary immunodeficiency has the knowledge needed:


• To manage their condition effectively


• To ensure that their health needs are understood and addressed by those involved in policy and delivery of healthcare.


2. Our role in ensuring safety of plasma derived products for patients A large majority of patients with PIDs (approx 5000) need life‐long treatment with immunoglobulin (Ig) infusions. The safety of this product is of paramount importance to affected patients who cope with the health burden of these complex chronic conditions and for whom Ig infusion is an integral and essential medicine.


To ensure safety issues concerning plasma products is maintained we work with other patient groups, both national and international and UKPIN, the professional body for doctors, nurses and scientists involved in the care of PID patients. PID UK fully endorses the work of UK PIN and their work on the surveillance of prion infection in these patients, led by Dr M Helbert.


Currently PID patients receive Ig infusions derived from plasma ONLY from non‐UK sources.


This was put in place following exposure of PID patients in the UK to plasma from people who went onto develop vCJD.


3. Response to terms of reference PID UK fully endorses the response and recommendations made by UKPIN in response to this consultation.


3a. Are UK policies governing who can donate blood and blood products, tissues and organs sufficiently evidencebased?


Is NHS Blood and Transplant overly restrictive about who can donate, or should greater precautions be taken to further reduce risk?


The use of UK‐sourced plasma would be a cause of extreme anxiety to PID patients without full mitigation of risk of prion contamination. It would be essential to have in place a prion screening procedure. We understand that this would require a blood or urine test for asymptomatic prion infection if UK plasma were to be used in the future. Tracking of donations would be essential to ensure traceability back to individual donors.


3b.Is the Government and its scientific advisory structure sufficiently responsive to the threat posed by emerging diseases being transmitted through blood and blood products, tissues and organs?


PID UK understands from UKPIN that there are currently no clear lines of communication and accountability about relaying incidences of prion infection through their study.


3c. Has the threat of ongoing transmission of vCJD through the blood and blood product supply been adequately mitigated?


This has been well mitigated through the use of non‐UK sourced plasma. Through education of patients of the processes involved in sourcing donors and isolating Ig there is confidence within the community about its safety. Steps to mitigate risk using UK plasma would need to include essential screening procedures for prion contamination and robust traceability mechanisms.


Declaration of interests


PID UK over the last year has been in receipt of two donations from CSL Behring.



please see ;




*** RE-P.164: Blood transmission of prion infectivity in the squirrel monkey: The Baxter study


***suggest that blood donations from cases of GSS (and perhaps other familial forms of TSE) carry more risk than from vCJD cases, and that little or no risk is associated with sCJD. ***


P.164: Blood transmission of prion infectivity in the squirrel monkey: The Baxter study


Paul Brown1, Diane Ritchie2, James Ironside2, Christian Abee3, Thomas Kreil4, and Susan Gibson5 1NIH (retired); Bethesda, MD USA; 2University of Edinburgh; Edinburgh, UK; 3University of Texas; Bastrop, TX USA; 4Baxter Bioscience; Vienna, Austria; 5University of South Alabama; Mobile, AL USA


Five vCJD disease transmissions and an estimated 1 in 2000 ‘silent’ infections in UK residents emphasize the continued need for information about disease risk in humans. A large study of blood component infectivity in a non-human primate model has now been completed and analyzed. Among 1 GSS, 4 sCJD, and 3 vCJD cases, only GSS leukocytes transmitted disease within a 5–6 year surveillance period. A transmission study in recipients of multiple whole blood transfusions during the incubation and clinical stages of sCJD and vCJD in ic-infected donor animals was uniformly negative. These results, together with other laboratory studies in rodents and nonhuman primates and epidemiological observations in humans, suggest that blood donations from cases of GSS (and perhaps other familial forms of TSE) carry more risk than from vCJD cases, and that little or no risk is associated with sCJD. The issue of decades-long incubation periods in ‘silent’ vCJD carriers remains open.



ran across an old paper from 1984 ;


***The occurrence of contact cases raises the possibility that transmission in families may be effected by an unusually virulent strain of the agent. ***



From: Terry S. Singeltary Sr.


Sent: Saturday, November 15, 2014 9:29 PM


To: Terry S. Singeltary Sr.


















Results We sampled blood 19 times from the inoculated monkeys at various stages of the disease over a period of 29 months, generating liters of vCJD-infected macaque blood. vCJD was confirmed in all inoculated macaques. After PMCA, PrPTSE was detected in plasma from infected monkeys, but not from uninfected animals. Both mouse models were more sensitive to infection with macaque-adapted vCJD agent than to primary human vCJD agent.



see much more here ;




Detection of cellular prion protein in exosome derived from ovine plasma


Authors: Elena Berrone1, Cristiano Corona2, Maria Mazza3, Elena Vallino Costassa4, Monica Lo Faro5, Francesca Properzi6, Chiara Guglielmetti7, Cristiana Maurella8, Maria Caramelli9, Maria Chiara Deregibus10, Giovanni Camussi11, Cristina Casalone12


VIEW AFFILIATIONS Affiliations: 1 1Istituto Zooprofilattico Sperimentale del Piemonte, Turin 2 2Istituto Zooprofilattico Sperimentale del Piemonte, Turin 3 3Istituto Zooprofilattico Sperimentale del Piemonte, Turin 4 4Istituto Zooprofilattico Sperimentale del Piemonte, Turin 5 5Istituto Zooprofilattico Sperimentale del Piemonte, Turin 6 6Istituto Superiore di Sanità, Rome 7 7Istituto Zooprofilattico Sperimentale del Piemonte, Turin 8 8Istituto Zooprofilattico Sperimentale del Piemonte, Turin 9 9Istituto Zooprofilattico Sperimentale del Piemonte, Turin 10 10University of Turin 11 11University of Turin 12 12Istituto Zooprofilattico Sperimetale del Piemonte, Turin


Published Ahead of Print: 22 September, 2015 Journal of General Virology doi: 10.1099/jgv.0.000291 Published Online: 22/09/2015 Prion protein (PrP) is present at extremely low levels in the blood of animals and its detection is complicated by the poor sensitivity of current standard methodologies. Interesting results have been obtained with recent advanced technologies that are able to detect minute amounts of the pathological PrP (PrPSc), but their efficiency is reduced by various factors present in blood. In this study, we were able to extract cellular PrP (PrPC) from plasma-derived exosomes by a simple, fast method without the use of differential ultracentrifugation and to visualize it by Western blotting, reducing the presence of most plasma proteins. This result confirms that blood is capable of releasing PrP in association with exosomes and could be useful to better study its role in TSEs pathogenesis.



Thursday, August 13, 2015


Iatrogenic CJD due to pituitary-derived growth hormone with genetically determined incubation times of up to 40 years



Wednesday, December 11, 2013


*** Detection of Infectivity in Blood of Persons with Variant and Sporadic Creutzfeldt-Jakob Disease ***




Sunday, December 14, 2014


*** ALERT new variant Creutzfeldt Jakob Disease nvCJD or vCJD, sporadic CJD strains, TSE prion aka Mad Cow Disease United States of America Update December 14, 2014 Report ***



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





Thursday, September 10, 2015


25th Meeting of the Transmissible Spongiform Encephalopathies Advisory Committee Food and Drug Administration Silver Spring, Maryland June 1, 2015




Thursday, October 1, 2015


Alzheimergate, re-Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy, Singeltary Submission to Nature






Sunday, May 18, 2008







Sunday, May 18, 2008


BSE, CJD, and Baby foods (the great debate 1999 to 2005)



Sunday, May 18, 2008





U.S. Imports for Consumption: December 1998 and 1998 Year-to-Date




U.S. Imports for Consumption: December 1998 and 1998 Year-to-Date (Customs Value, in Thousands of Dollars) (Units of Quantity: Kilograms)


<--- ---="" 1998="" country="" dec="" div="" quantity="" value="" ytd="">



WORLD TOTAL . . . . . . . 25,740 1,827 270,357 20,476


Belgium . . . . . . . . . 14 8 145 60


France . . . . . . . . . --- --- 134 60


Netherlands . . . . . . . --- --- 11 5


Switzerland . . . . . . . 10,462 597 86,101 5,894


United Kingdom . . . . . --- --- 335 62




U.S. Imports for Consumption: December 1998 and 1998 Year-to-Date (Customs Value, in Thousands of Dollars) (Units of Quantity: Kilograms)


<--- ---="" 1998="" country="" dec="" div="" quantity="" value="" ytd="">



WORLD TOTAL . . . . . . . 1,039 817 19,056 22,678


Austria . . . . . . . . . --- --- 9,194 18,707


Belgium . . . . . . . . . --- --- 22 15


Netherlands . . . . . . . 353 2 6,733 41


Switzerland . . . . . . . 374 218 1,084 440


United Kingdom . . . . . --- --- 1 4




U.S. Imports for Consumption: December 1998 and 1998 Year-to-Date (Customs Value, in Thousands of Dollars) (Units of Quantity: Kilograms)


<--- ---="" 1998="" country="" dec="" div="" quantity="" value="" ytd="">



WORLD TOTAL . . . . . . . 1,926 461 14,484 3,563...


United Kingdom . . . . . 2 8 464 192


3002.10.0040: FETAL BOVINE SERUM (FBS)


U.S. Imports for Consumption: December 1998 and 1998 Year-to-Date (Customs Value, in Thousands of Dollars) (Units of Quantity: Kilograms)


<--- ---="" 1998="" country="" dec="" div="" quantity="" value="" ytd="">



WORLD TOTAL . . . . . . . 2,727 233 131,486 8,502...


Belgium . . . . . . . . . --- --- 17 32


United Kingdom . . . . . 329 82 743 756




U.S. Imports for Consumption: December 1998 and 1998 Year-to-Date (Customs Value, in Thousands of Dollars) (Units of Quantity: Kilograms)


<--- ---="" 1998="" country="" dec="" div="" quantity="" value="" ytd="">



WORLD TOTAL . . . . . . . 88,467 27,343 944,412 309,947...


United Kingdom . . . . . 1,887 2,300 26,823 23,585






U.S. Imports for Consumption: December 1998 and 1998 Year-to-Date




<--- ---="" 1998="" country="" dec="" div="" quantity="" value="" ytd="">



WORLD TOTAL . . . . . . . 36,178 643 250,982 11,604...


United Kingdom . . . . . 584 39 11,292 588



snip...see more here ;








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


Subject: TSE Advisory Committee October 14-15, 2004


Date: Fri, 10 Sep 2004 16:34:24 –0500


From: "Terry S. Singeltary Sr."


To: Bovine Spongiform Encephalopathy


Transmissible Spongiform Encephalopathies Advisory Committee


Committee Roster


Suzette Priola PhD, National Institute of Allergy & Infectious Diseases (Chair) 1/31/05 Val Bias, National Hemophilia Foundation 1/31/07 Lynn Creekmore DVM, U.S. Department of Agriculture 1/31/07 Lisa Ferguson DVM, U.S. Department of Agriculture Animal & Plant Health Inspection Service 1/31/04 R. Nick Hogan MD/PhD, University of Texas Southwestern Medical School 1/31/07 Rima Khabbaz MD, National Center for Infectious Disease Shirley Walker, Dallas Urban League (Consumer Representative) 1/31/04 John Bailar MD/PhD, University of Chicago 1/31/05 Arthur Bracey MD, St. Luke's Episcopal Hospital 1/31/07 Stephen DeArmond MD/PhD, University of California San Francisco School of Medicine 1/31/05 Pierluigi Gambetti MD, Case Western Reserve University 1/31/05 Richard Johnson MD, Johns Hopkins University Schoo of Medicine 1/31/06 Stephen Petteway PhD, Bayer (Non-voting Industry Representative) 1/31/05 Sidney Wolfe MD, Public Citizen's Health Research Group 1/31/04 William Freas PhD, FDA (Executive Secretary)


Tentative Meetings


October 14-15, 2004


Upcoming Meetings Date


Plasma-Derived Product Label Claims For TSE Testing; BSE Diagnosis, Food Rules; vCJD Transmission


Plasma-Derived Product Labeling Claims For TSE Clearance To Be Discussed By Cmte.


FDA s Transmissible Spongiform Encephalopathies Advisory Committee will discuss labeling claims for TSE clearance studies for plasma-derived products on Oct. 14.


The committee will also receive updates on U.S. Department of Agriculture licensed tests for the diagnosis of bovine spongiform encephalopathy, FDA BSE food safety rules, and the BSE situation worldwide.


Presumptive transfusion transmissions of variant Creutzfeldt-Jakob Disease and FDA recommended safeguards will also be addressed by the committee.


To watch a webcast of this meeting, click the button below. To arrange for live videoconferencing, or to order videotapes & DVDs, email or call 800-627-8171.


Posted: Friday, September 10, 2004


 Sign up to view this meeting.


 October 14, 2004




Previous Meetings Date Mad Cow Disease: FDA Recommendations To Minimize Risk From TSE Agents In Medical Products -Day 2


February 13, 2004




Mad Cow Disease: Risk Models For Bovine Sourcing For Medical Products; First U.S. Case


February 12, 2004




TSE Removal From Medical Devices and Equipment


July 18, 2003




Bovine Bone Gelatin Safety; BSE In Canada


July 17, 2003




Variant Creutzfeldt-Jakob Disease Guidance Implementation


February 20, 2003




Blood Donor Deferral Implementation


June 27, 2002




CJD Transmission Risk Reduction Draft Guidance


June 26, 2002




TSE/Blood Products Joint Cmte. Meeting


January 17, 2002




Creutzfeldt-Jakob Disease FDA Draft Guidance


October 25, 2001




For information about additional meetings, email or call Julie D. Robenson at (800) 332-1370.





talk about spooky. i was just going over a few old Advisory Committee meetings;






 Advisory Committee on:








December 18, 1998


Holiday Inn


8120 Wisconsin Avenue


Bethesda, Maryland


Proceedings by:


CASET Associates, Ltd


10201 Lee Highway, Suite 160


Fairfax, Virginia 22030




DR. ROOS: I have a question for Dr. Metters. I guess I just wanted to make sure that I understood the rationale for the present policies in the United Kingdom; that is that there is no pooled plasma products that have UK donors. Nevertheless, there is no curtailment of blood transfusions and labile products from UK individuals.


I just wanted to make sure I understood the rationale for that. That was just -- in other words, if there is a safety problem with a particular unit bearing CJD, then presumably those individual blood transfusions also carry that risk. Maybe you could explain that.


DR. METTERS: First of all, all blood products. At the moment there are, because we haven't completed the change-over from UK source to non-UK source. Once that change-over takes place, there will be no UK sourced blood products. We are making that change as soon as possible.


The reason why blood products may be different from blood is that, of course, blood products go into an enormous pool.


The potential disbursement of a unit that is contaminated with new variant depends on the size of the pool, whether it is a pool of 500 or 6,500 units.


As I said, we have to find an alternative source of blood products. The most units it would go to is three recipients.


The other point to make is that this relates to the follow up. By far, those who receive blood in the United Kingdom will die from the current disease for which they receive the blood within 12 to 18 months. That is a real problem when you come to holding it up, because of the attenuation and so on.


The blood products, that does present a disbursal factor. As I said, we do actually have steps to monitor who receives the blood, and are taking steps to out-source blood products.


DR. BROWN: Just to add, on the disbursal factor, we don't know what is going on in new variant. We don't know if there are a million infectious units per unit of blood. We just don't know.


I received this comment about CJD. If new variant is, indeed, like ordinary CJD, there is a logical curiosity about the disposal factor. Infectivity is a functional definition. We don't necessarily know what it is.


So, if there are 15 infectious units, we are talking about 15 transmissions of the disease. It is likely, after all the experimental and epidemiologic evidence that any level of infectivity in the blood of normal CJD is very, very low.


It doesn't much matter if that 100 infectious units is distributed to 10,000 or a million. There are going to be 100 transmissions. The notion that you can dilute out infectivity has no scientific basis.


The other thing, if the unit of blood that is donor is fractionated with an infectious agent, then disbursal may be higher. Unfortunately, we don't know the answer to that yet.


DR. SCHONBERGER: I am wondering if our colleagues from the United Kingdom can tell us what type of screening for blood donors is done to reduce the chances of new variant disease specifically in the donor group.


Is there any kind of screening specifically directed toward new variant CJD.


The other issue, again, of screening, is there any screening that is done in the United Kingdom that is focused specifically on ruling out somebody who is symptomatic, for example, with new variant.


I understand that new variant's onset can be subtle and not really very apparent for a while.


DR. BROWN: So, what you are asking is whether or not there are any special criteria that are in place or being thought of to reduce the risk of a new variant patient who is either -- according to Bob Will's criteria -- either probable or definite. I can't imagine a definite, but shall we say a suspect.


DR. METTERS: I think the general answer has to be no. There is nothing you can ask somebody. There is, on the other hand, the donor is at least asked about their general health.


Then if there is any suspicion at the time that they are not 100 percent fit, and they have their blood count done before they are accepted.


If someone is physically unwell in any way, hopefully they will not get through the screening system. So, while it isn't specific to that, it is -- I would be doing a very bad job if I let someone who was unhealthy in any way to get through our screening system.


DR. SCHONBERGER: So, there is no set of questions that is standard --


DR. METTERS: The questions of about CJD are there, right. To avoid getting classes of donors, you may be able to avoid getting classes of donors.


I would be very interested if any of you at the table could answer the question that was asked.


We haven't yet had one who has been identified that in the time that they were labile, was a donor.


DR. METTERS: As you know, most of the patients, or many of them, have psychiatric onsets. So, if their response to the very first question you ask is moo, you know to be suspicious.


DR. ROHWER: I have a question for Dr. Ferguson. When the provisions against importations from the United Kingdom were extended in February of 1998, was there any attempt by the USDA to go back to see what level of exposure the United States had to European bovine products and cattle since 1980, for example, or since 1988 when the provisions were put in place for the United Kingdom?


For example, between 1980 and 1989, apparently we imported some 500 cattle. Now that we have recognized that there may have also been a risk from imports from Europe and others before this change in policy, have we gone back and looked at how exposed we were from that risk? I mean, how many imports were there, and what kind of things were imported.


DR. FERGUSON: Yes, actually we have gone back and looked at live animal imports from continental Europe at that same time.


They were fairly restricted at that point in time because cattle in Europe were infected with other animal diseases, such as FMB.




MR. SUDIERI(?): My name is Sal Sudieri. I am the vice president for medical affairs at the New York Blood Center.


Regarding this section A, there is a piece of information that I think is important for you to have.


For the last 25 years, the American Blood Center has had a program with Switzerland, Holland and Germany, where centers that produce plasma derivatives in this country collect units of whole blood from volunteer donors.


They became licensed centers, collection centers, from the New York Blood Center, by our FDA license, and they will ship us the red cells, where we do the processing, dedicate the plasma and the plasma is fractionated.


About 30 percent of the blood, or about 200,000 units of red cells a year, come to New York through this method.


DR. BROWN: Is the committee happy about what everybody considers and knows to be an other BSE country or do we want to get clarification of that?


DR. LEITMAN: Clarification.


DR. BROWN: Are we talking about the United Kingdom plus France, plus Portugal, plus the whole of Europe? What are we talking about?


DR. LURIE: Who is to make that decision?


DR. BROWN: That is what we want to know. Is that a decision that is going to be made? If they can't tell us what is meant by other BSE country, we can't really answer the question.


DR. LURIE: The procedural approach would be to vote on it separately. I think the vote is more providing guidance.


DR. BROWN: Good suggestion. Ditto for periods of higher and lower risk, I suppose, and ditto for possible versus probable. We can move along in that way. That is a good idea.


DR. LURIE: Another parallel type suggestion would be, I think the question that we do need some clarification on is the definition of reside.


While obviously it is more efficient to exclude residents from Britain and visitors from Britain because, a, there are presumably fewer residents than there are visitors, and the duration of exposure and presumably severity of exposure would be different.


DR. BROWN: Maybe the best way to do it is to go piecemeal and nibble, in which case we might, for example, phrase the first question, should the FDA recommend excluding donors who are British citizens and see what you get in answer to that, and see just how far the committee is willing to go.


On the other hand, that is going to require about 117 votes this afternoon.


DR. HOEL: There is another approach. First, we have to answer the first question first.


DR. BROWN: I know. That was going to be my next point. Depending on our answer to one, we can either dismiss A through E or take them up. I think that is why Dr. Epstein phrased these two questions in this way. Maybe I am wrong, but that is the way it is going to be done.


If the committee is ready to vote without further discussion on question one -- not A, B, C, D and E, but just question one as a question -- we will then vote and see what we then have to do, or we can have a little, a moderate or a large amount of discussion before we get to that.


DR. LEITMAN: I have always had a problem with reducing a theoretical risk or reducing a hypothetical risk or reducing a potential risk, because perhaps, as I was talking to one of my colleagues earlier today, perhaps it is a speculative risk and not a theoretical risk that, in actuality, hasn't occurred.


How do we reduce speculative risk? How do you reduce zero?


DR. BROWN: That is an interesting kind of semantic question. It is the virtual reduction of a theoretical risk. Does anyone want to get into semantics?


DR. CLIVER: Clearly question one turns on the perception of nvCJD as a food borne disease that is somehow derived from cattle.


I think I am prepared to accept that. The period of emphasis ending at 1990, though, I think is not indicated. The observations that Dr. Detweiler had before, the data on this would have been very valuable for risk on people on farms, however, there is no imputation here that the risk was associated with people on farms with cattle.


[try 15 times more likely to get CJD...TSS]









now story changes from;


SEAC concluded that, if the fourth case were confirmed, it would be worrying, especially as all four farmers with CJD would have had BSE cases on their farms.




This is not unexpected...


was another farmer expected?



4th farmer, and 1st teenager





DR. BROWN: The question, Robert, is there any instance of maternal transmission of new variant CJD?


DR. WILL: The answer is no, although tragically, at least one of the individuals was pregnant when diagnosed with new variant CJD.


MS. HARRELL: Was she delivered?


DR. WILL: She did delivered. The child is alive and well, but we are only talking two or three years. Of course, therefore, we rely on previous evidence, which does not suggest that there was maternal transmission.


DR. BROWN: It is an interesting question. If it were to have occurred, it would be one more very striking example of a particular biological behavior of new variant from sporadic.


We have information about half a dozen children born to patients who were sick with CJD on delivery, who now have -- they have lived for as long as 30 years after that event -- that is, after they were born -- and are quite healthy...




DON'T count your chickens again before they hatch there Dr. Brown, remember the 38 years incubation period on the iCJD of a small dose;




Creutzfeldt-Jakob disease 38 years after diagnostic use of human growth hormone


E A Croes, G Roks, G H Jansen, P C G Nijssen, C M van Duijn




J Neurol Neurosurg Psychiatry 2002;72:792-793


We describe the second patient with hGH related CJD in the Netherlands. The patient developed the disease 38 years after hGH injections. To our knowledge, this is the longest incubation period described for any form of iatrogentic CJD. Furthermore, our patient was _not_ treated with hGH, but only received a _low_ dose as part of a diagnostic procedure...


and this was not a case of nv/v CJD, but spordic CJD...TSS





6.1 Information is held on a especial data base from the initial BSE survey in 1989. More current products, which were vetted during their initial licensing assessment, after the UK BSE quideline was published, may not appear in the listing, since BSE was considering in their initial licensing assessment.



RESTRICTED-COMMERCIAL..............CSM/BIOLS/94/6th Meeting










1.1 The chairman reminded the Sub-Committee that the papers and proceedings were confidential and should not be disclosed...






4.6.1 There was a discussion of the problem of the paper trail audit, and countries of sourcing for bovine sera. Concern was expressed about the fact that bogus certificates of origin had been produced and circulated. It was noted that in the USA bovine protein was still being fed to cattle. Sera from the USA could be used in the initial production and subsequent large scale manufacture of hybridomas, rDNA products and vaccines (some of which may be used in healthy children)...









(7 blank pages to follow...TSS)




7.4 The Committee was informed that, since the publication of Hunter et al. (2002), two further sheep had succumbed to BSE; one of these sheep had been transfused with buffy coat, while the other had been transfused with whole blood. Of the remaining 20 sheep that received transfusions, one died of unrelated causes and 19 animals remain apparently healthy. The healthy animals are at varying times post-transfusion ranging from less than 100 to over 1000 days.


7.5 Members were informed that among the 21 sheep transfused with blood from scrapie infected animals (761 to 1080 days of age), 4 had developed scrapie between 614 and 737 days post-transfusion. One animal received the buffy coat preparation from the blood of an animal with clinical disease. The remaining 3 animals received whole blood from donors not yet showing clinical signs. Of the remaining 17 transfused sheep, one died of unrelated causes and two further sheep were showing clinical signs of scrapie; one of these had received buffy coat, while the other had received whole blood.


7.6 The Committee noted that it would not be possible to confirm that the negative controls are free of TSEs until the end of the study, when they would be culled and analysed post mortem for signs of subclinical infection. Of the 10 positive controls that received BSE-infected cattle brain homogenate intravenously, 5 have developed disease or appear to be in the early stages of the disease.


3 Hunter, N., Foster, J., Chong, A., McCutcheon, S., Parnham, D., Eaton, S., MacKenzie, C. and Houston, F. (2002). Transmission of prion disease by blood transfusion. Journal of General Virology 83.


CJD: Transmission


Lord Lucas asked Her Majesty's Government:


Further to the Written Answer by Lord Hunt of Kings Heath on 27 March (WA 59), what is the "strong epidemiological evidence to suggest that classic CJD is not transmitted through blood"; which of the many variants of "classic CJD" this evidence applies to; and whether they will place copies of the relevant papers in the Library of the House.[HL1864]


The Parliamentary Under-Secretary of State, Department of Health (Lord Hunt of Kings Heath): The following, peer reviewed articles, relate to this subject and will be placed in the Library:


T F G Esmonde et al, 1993, Creutzfeldt-Jakob disease and blood transfusion, Lancet 341; 205-207;


P Brown, 1995, Can Creutzfeldt-Jakob disease be transmitted by transfusion?, Current Opinion in Haematology, vol 2, pp 472-477;


C M van Duijn et al, 1998, Case-control study of risk factors of Creutzfeldt-Jakob disease in Europe during 1993-95, Lancet 351: 1081-1085;


11 Apr 2000 : Column WA32


Ricketts MN et al: Is Creutzfeldt-Jakob Disease transmitted in blood?, Energ Infect Dids 1997:3, 155-166; and


Heye N et al: Creutzfeldt-Jakob Disease and blood transfusion, Lancet 1994; 343; 298-299.


These studies cover all types of CJD. Sporadic (classic) CJD, however, accounts for some 85 per cent of non-variant cases.


The European Committee for Proprietory Medicinal Products (CPMP) reviewed the evidence in December 1995 and advised that there was no experimental or epidemiological evidence that classical CJD is transmitted by blood transfusions or plasma-derived products. A recall policy was not considered justified for plasma derived products from plasma pools incorporating a donation implicated for classical CJD. It reaffirmed that advice in March 1997. CPMP concluded on the basis of currently available information from epidemiological and experimental studies that there is no scientific justification for changing from the current CPMP position on classical CJD. CPMP further stated there to be no evidence that classical CJD is transmitted via blood or plasma derived products. This issue was also subsequently considered by the US Food and Drugs Administration, which came to the same conclusion.


New-variant CJD


Lord Lucas asked Her Majesty's Government:


Further to the Written Answer by Lord Hunt of Kings Heath on 27 March (WA 55-6), whether they will provide data on those who have died from, or been diagnosed with, new-variant CJD in the United Kingdom as to:


(a) the status of nucleotide-21 preceding the prion ATG start codon;


(b) the status of codons 26, 56 and 174 of doppel;


(c) the dates of onset and confirmation for those patients diagnosed with new-variant CJD but still living;


(d) the definition of "onset"; and


(e) the age of the patients at onset to the nearest month.[HL1807]


Lord Hunt of Kings Heath: The genetic information requested is not available. However, extensive studies of polymorphisms in and around the prion protein and doppel genes have been under way for some time at the St Mary's Prion Unit, London. The results of these investigations will be published in the scientific literature, subject to peer review, in due course.


Confirmation of a diagnosis of vCJD is currently obtained by postmortem neuropathology. There are therefore no "confirmed" patients still living. The dates of onset for patients still living and defined as "probable" to the nearest month are as follows:



25 Apr 2002 : Column WA58


Bovine Embryos and Live Cattle: Imports from North America


The Earl of Caithness asked her Majesty's Government:


When the ban on the importation of embryos and live cattle from North America will be lifted; and [HL3912]


What is the scientific evidence for the imposition of a ban on the importation of embryos and live cattle from North America. [HL3913]


Lord Whitty: Her Majesty's Government have not imposed a ban on imports of bovine embryos and live cattle from North America.


The European Parliament and European Council introduced legislation in May last year laying down rules for the prevention, control and eradication of certain transmissible spongiform encephalopathies (TSEs). The legislation was introduced in response to the recommendations of the Office International des Epizooties (OIE the international animal health organisation) and advice from the Commission's scientific comittees. The legislation (and the transitional measures which came into effect in October last year) includes requirement that imports into the EU of bovine embryos and live cattle must be accompanied by certification confirming that the feeding of ruminants with protein derived from mammals has been banned and that the ban has been effectively enforced. Some exporting countries, such as Canada and the USA, are currently unable to meet these new requirements.



BSE: US Export of Specified Risk Material


Lord Kennet asked Her Majesty's Government:


Whether the United States contends that under the provisions enforceable by the World Trade Organisation the European Union may not ban the import into Europe from the United States of "specified risk material" (that is, material at possible risk of BSE infection).


Lord Donoughue: Yes. But their position on the Specified Risk Material legislation is based on the assumption that the United States can safely be regarded as a "BSE free" country. Their case for such treatment has not been accepted by the EU Commission's Scientific Veterinary Committee.



Baroness Masham of Ilton: My Lords, as blood products which infected haemophiliacs with HIV came from the USA, is the Minister confident that something else nasty may not come again from imported blood from the USA? Is he aware that there are ways of cleaning blood to make it safer? I know that that is done in Vienna, in Austria. Will the Minister look into that? Following the question asked by the noble Lord, Lord Clement-Jones, about people using their own blood, I am sure that, when this Statement goes out into the wider community, people will want to know that information.



However, the Bio Products Laboratory who produce plasma products did export surplus products, under the Income Generation Regulations for the NHS, and used the income for the benefit of the health service.[21]



43. Do you sell any of it abroad at all?


(Mr Gorham) No. The only circumstances in which we would export blood would be if there was an approach to the British Government and the British Government felt that it was appropriate to support an international emergency or something like that. We do supply the British Forces. We occasionally help out our colleagues in Wales and Scotland and they would reciprocate with us if that was appropriate. At the moment it is more or less totally contained within the United Kingdom.



Blood and Blood Products


Mr. Hinchliffe: To ask the Secretary of State for Health what estimate he has made of the number of persons who have been inoculated with blood or blood products over the past three years in the United Kingdom. [61681]


Ms Jowell [holding answer 2 December 1998]: It is estimated that about one million people in the United Kingdom receive blood and blood products every year.



CJD blood products given to 3,000 patients




UP TO 3,000 people treated in 100 British hospitals may have been injected with blood products taken from a donor who died six weeks ago from new variant Creutzfeldt-Jakob disease, the human form of BSE. None of them is to be told because the Health Department believes the risk of them developing the disease is so slight that there is no reason to cause alarm.


Although hospitals have been advised to return the product, used in X-ray screenings to detect lung disease, so far only 15 per cent has been recovered. There is no order obliging hospitals to return it and some clinicians may go on using up stocks on the basis that patients are far more likely to die from infections or cancer that can be diagnosed with the product than from CJD.


Another 268 patients in Ireland are known to have been given injections from the same batch of the product. The Irish Health Ministry has decided to notify all the patients concerned.


Even though the identity of all those who have been given an injection of the product is known, it was decided not to tell them because there is no evidence that the illness can be transmitted through the blood or the serum derived from it to make the product and the risk of developing CJD is regarded as negligible.


"You are putting an enormous burden on people by telling them they have a remote risk of contracting the disease," the department said last night. "The ethics committee which advises us on these matters decided it was just not appropriate to tell them."


The blood from the donor was sent 18 months ago to the National Blood Authority laboratory, where it was split into a number of different products. The donor's plasma was mixed with some taken from 49,000 other donors to make 8,174 bottles of albumin, the water-soluble protein found in blood.


Many were exported but 210 of the 50ml bottles remained in Britain and were sent to eight different hospitals and companies. Some of the bottles were used intravenously to rehydrate burn victims.


One bottle was sent to Nycomed Amersham which used it to produce 14,000 vials of Amerscan Pulmonate II, an agent which is injected into the lungs so that infections and cancer show up under X-ray. The company sent almost 3,700 vials to 100 British hospitals.


At the end of October the European Committee on Proprietary Medicinal Products called for the withdrawal of blood products derived from donors who were confirmed CJD cases. On November 1 the Blood Transfusion Service was notified that one of its donors had died from the disease so the Amersham company was told.


In turn the company got in touch with the Medicines Control Agency which informed the Health Department and it recommended withdrawal of the product on November 17.


Despite regular alarms, there has never been any convincing evidence that blood or blood products can transmit CJD (Nigel Hawkes writes). Unless new variant CJD, the human form of "mad cow" disease, is more easily transmitted than classic CJD via blood or blood products, there does not appear to be any cause for concern. [This is no medical basis for this statement -- webmaster]


For classic CJD the risk seems negligible. About 50 people a year die of the disease, so it is certain that every year some of them give blood after they have the infection but before its symptoms appear. Studies show that classic CJD can be passed on in human tissue, but not - so far as we know - in blood. [This is not an accurate summary of current scientific knowledge -- webmaster]



South Africa has been exporting plasma for many years. From 1983-86, human plasma was falsely labelled "animal plasma" and illegally exported to Europe. This illegal practice resulted in a court case and one conviction in Belgium. In 1996, Austrian police seized 4000 L of infected blood from a Linz-based company, Albovina.


The Sunday Times report said that Austrian officials have investigated two British companies, based in Guernsey and Berkshire. Police are still trying to find out where the companies' plasma products were used. Johann Kurz, head of unit for biologicals with the federal Ministry of Social Security & Generation in Vienna, Austria, told The Lancet, "We suspect that in 1996 and earlier, some products coming from South Africa were transferred to India, China, and Hong Kong, among other countries". The products were albumin and intravenous immunoglobulins.


Nevertheless, Luc Noel, Coordinator of Blood Safety with the WHO in Geneva, Switzerland, emphasised that: "There should not be any confusion of the transfusion services in South Africa--which are now world class--with these criminal activities". While it is important that this trafficking is exposed, the public can be confident in their blood services, Noel added.


Sanjay Kumar


Kirsten Myhr, MScPharm, MPH Bygdøy alle 58B 0265 Oslo, Norway Tel.: +47 22 56 05 85, fax: +47 22 24 90 17





Thursday, September 10, 2015


25th Meeting of the Transmissible Spongiform Encephalopathies Advisory Committee Food and Drug Administration Silver Spring, Maryland June 1, 2015



Sunday, December 14, 2014


*** ALERT new variant Creutzfeldt Jakob Disease nvCJD or vCJD, sporadic CJD strains, TSE prion aka Mad Cow Disease United States of America Update December 14, 2014 Report ***



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







Please send your reply to the Food and Drug Administration, Attention: Tyra S. Wisecup, Compliance Officer, at the address on the letterhead. If you have questions regarding any issues in this letter, please contact Ms. Wisecup at (612) 758-7114. Sincerely, /S/ Michael Dutcher, DVM Director Minneapolis District




[1] Specified risk materials include the brain, skull, eyes, trigeminal ganglia, spinal cord, vertebral column (excluding the vertebrae of the tail, the transverse processes of the thoracic and lumbar vertebrae, and the wings of the sacrum), and dorsal root ganglia of cattle 30 months of age and older, and the tonsils and distal ileum of the small intestine of all cattle. - Page Last Updated: 11/15/2012



I made a submission to the BSE Inquiry long ago during the BSE Inquiry days, and they seemed pretty interested. see on down ;


Sender: "Patricia Cantos"


To: "Terry S Singeltary Sr. (E-mail)"


Subject: Your submission to the Inquiry


Date: Fri, 3 Jul 1998 10:10:05 +0100


3 July 1998


Mr Terry S Singeltary Sr.


E-Mail: Flounder at


Ref: E2979


Dear Mr Singeltary,


Thank you for your E-mail message of the 30th of June 1998 providing the Inquiry with your further comments.


Thank you for offering to provide the Inquiry with any test results on the nutritional supplements your mother was taking before she died.


As requested I am sending you our general Information Pack and a copy of the Chairman's letter. Please contact me if your system cannot read the attachments.


Regarding your question, the Inquiry is looking into many aspects of the scientific evidence on BSE and nvCJD. I would refer you to the transcripts of evidence we have already heard which are found on our internet site at ;



Could you please provide the Inquiry with a copy of the press article you refer to in your e-mail? If not an approximate date for the article so that we can locate it?


In the meantime, thank you for you comments. Please do not hesitate to contact me on...




everyone I tell this too gets it screwed up...MY MOTHER WAS NOT TAKING THOSE SUPPLEMENTS IPLEX (that I ever knew of). this was my neighbors mother that died exactly one year _previously_ and to the day of sporadic CJD that was diagnosed as Alzheimer’s at first. my mother died exactly a year later from the Heidenhain Variant of Creutzfeldt Jakob Disease hvCJD, and exceedingly rare strains of the ever growing sporadic CJD’s. _both_ cases confirmed. ...kind regards, terry


TSEs i.e. mad cow disease's BSE/BASE and NUTRITIONAL SUPPLEMENTS


IPLEX, mad by standard process;


vacuum dried bovine BRAIN, bone meal, bovine EYE, veal Bone, bovine liver powder, bovine adrenal, vacuum dried bovine kidney, and vacuum dried porcine stomach.




what about potential mad cow candy bars ?


see their potential mad cow candy bar list too...


THESE are just a few of MANY of just this ONE COMPANY...TSS








Friday, January 19, 2001 snip...


17 But I think that we could exhibit some quite


18 reasonable concern about blood donors who are taking dietary


19 supplements that contain a certain amount of unspecified-


20 origin brain, brain-related, brain and pituitary material.


21 If they have done this for more than a sniff or something


22 like that, then, perhaps, they should be deferred as blood


23 donors.


24 That is probably worse than spending six months in


25 the U.K.




3681t2.rtf(845) page 501



Docket Management Docket: 96N-0417 - Current Good Manufacturing Practice in Manufacturing, Packing, or Holding Dietary Ingredients a Comment Number: EC -2 Accepted - Volume 7




see full text ;



snip...see full list of potential mad cow products ;


Wednesday, August 5, 2015


Federal judge enters permanent injunction against Wisconsin dietary supplement manufacturers prohibited cattle materials BSE TSE Prion


Singeltary Submission to BSE Inquiry on Nutritional Supplements containing SRM 1998



Tue, 13 Feb 2001 JAMA Vol. 285 No. 6, February 14, 2001 Letters


Diagnosis and Reporting of Creutzfeldt-Jakob Disease � � To the Editor:


In their Research Letter in JAMA. 2000;284:2322-2323, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.


Terry S. Singeltary, Sr Bacliff, Tex



To the Editor:


At the time of my mother's death, various diagnoses were advanced such as "rapid progressive Alzheimer disease," psychosis, and dementia. Had I not persisted and personally sought and arranged a brain autopsy, her death certificate would have read cardiac failure and not CJD.


Through CJD Voice1 I have corresponded with hundreds of grief-stricken families who are so devastated by this horrific disease that brain autopsy is the furthest thing from their minds. In my experience, very few physicians suggest it to the family. After the death and when families reflect that they never were sure what killed their loved one it is too late to find the true cause of death. In the years since my mother died I think that the increasing awareness of the nature of CJD has only resulted in fewer pathologists being willing to perform an autopsy in a suspected case of CJD.


People with CJD may die with incorrect diagnoses of dementia, psychosis, Alzheimer disease, and myriad other neurological diseases. The true cause of death will only be known if brain autopsies are suggested to the families. Too often the physician's comment is, "Well, it could be CJD but that is so rare it isn't likely."


Until CJD is required to be reported to state health departments, as other diseases are, there will be no accurate count of CJD deaths in the United States and thus no way to know if the number of deaths is decreasing, stable, or increasing as it has recently in the United Kingdom.


Dorothy E. Kraemer Stillwater, Okla


In Reply:


Mr Singeltary and Ms Kraemer express an underlying concern that our recently reported mortality surveillance estimate of about 1 CJD case per million population per year in the United States since 1985 may greatly underestimate the true incidence of this disease. Based on evidence from epidemiologic investigations both within and outside the United States, we believe that these national estimates are reasonably accurate.


Even during the 1990s in the United Kingdom, where much attention and public health resources have been devoted to prion disease surveillance, the reported incidence of classic CJD is similar to that reported in the United States. [The elderly demented in a country with a medical system like England's rarely reach a neurologist. However, it is precisely the elderly where the disease is concentrated. -- webmaster]


In addition, in 1996, active US surveillance for CJD and new variant (nv) CJD in 5 sites detected no evidence of the occurrence of nvCJD and showed that 86% of the CJD cases in these sites were identifiable through routinely collected mortality data. [This again was merely "death certificate" CJD. -- webmaster]


Our report provides additional evidence against the occurrence of nvCJD in the United States based on national mortality data analyses and enhanced surveillance. It specifically mentions a new center for improved pathology surveillance. We hope that the described enhancements along with the observations of Singeltary and Kraemer will encourage medical care providers to suggest brain autopsies for more suspected CJD cases to facilitate the identification of potentially misdiagnosed CJD cases and to help monitor the possible occurrence of nvCJD.


Creutzfeldt-Jakob disease is not on the list of nationally notifiable diseases. In those states where surveillance personnel indicate that making this disease officially notifiable would meaningfully facilitate collection of data that are needed to monitor the incidence of CJD and nvCJD, including the obtaining of brain autopsy results, we encourage such a change. However, adding CJD to the notifiable diseases surveillance system may lead to potentially wasteful, duplicative reporting because the vast majority of the diagnosed cases would also be reported through the mortality surveillance system.


Furthermore, making CJD a notifiable disease may not necessarily help identify undiagnosed CJD cases. The unique characteristics of CJD make mortality data a useful surrogate for ongoing surveillance. Unlike many other neurologic diseases, CJD is invariably fatal and in most cases rapidly progressive and distinguishable clinically from other neurologic diseases. [Essentially all elderly dementia is "fatal" in the sense that no one gets better. -- webmaster]


Because CJD is least accurately diagnosed early in the course of the illness, notifiable disease surveillance of CJD could be less accurate than mortality surveillance of CJD. In addition, because death as a condition is more completely and consistently reported, mortality surveillance has the advantage of being ongoing and readily available.


The absence of CJD and nvCJD from the list of nationally notifiable diseases should not be interpreted to mean that they are not important to public health; this list does not include all such diseases. We encourage medical caregivers to report to or consult with appropriate public health authorities about any diagnosed case of a transmissible disease for which a special public health response may be needed, including nvCJD, and any patient in whom iatrogenic transmission of CJD may be suspected.


Robert V. Gibbons, MD, MPH Robert C. Holman, MS Ermias D. Belay, MD Lawrence B. Schonberger, MD, MPH Division of Viral and Rickettsial Diseases National Center for Infectious Diseases Centers for Disease Control and Prevention Atlanta, Ga


Opinion (webmaster): Behind the polite words, CDC is waging a bitter battle to prevent CJD incidences from becoming known, to protect its clients in the blood and transplant industries. The best available study is that of Boller et al. from 1989. In this study 7.5% of Alzheimer patients were actually CJD. Numerous other diseases were confused as well.


With CJD, the real issue is how many people die with it, rather from it in the sense of the death certificate. Even if the true cause of death was a heart attack, as a hypothetical, who would want an untested cornea from someone with preclinical CJD ?


Diagnosis of dementia: clinicopathologic correlations. Neurology 1989 Jan;39(1):76-79 Boller F, Lopez OL, Moossy J Based on 54 demented patients consecutively autopsied at the University of Pittsburgh, we studied the accuracy of clinicians in predicting the pathologic diagnosis. Thirty-nine patients (72.2%) had Alzheimer's disease, while 15 (27.7%) had other CNS diseases (four multi-infarct dementia; three Creutzfeldt-Jakob disease; two thalamic and subcortical gliosis; three Parkinson's disease; one progressive supranuclear palsy; one Huntington's disease; and one unclassified). Two neurologists independently reviewed the clinical records of each patient without knowledge of the patient's identity or clinical or pathologic diagnoses; each clinician reached a clinical diagnosis based on criteria derived from those of the NINCDS/ADRDA. In 34 (63%) cases both clinicians were correct, in nine (17%) one was correct, and in 11 (20%) neither was correct. These results show that in patients with a clinical diagnosis of dementia, the etiology cannot be accurately predicted during life.




 26 March 2003


Views & Reviews


Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States


Ermias D. Belay, MD, Ryan A. Maddox, MPH, Pierluigi Gambetti, MD and Lawrence B. Schonberger, MD


+ Author Affiliations


From the Division of Viral and Rickettsial Diseases (Drs. Belay and Schonberger and R.A. Maddox), National Center for Infectious Diseases, Centers for Disease Control and Prevention, Atlanta, GA; and National Prion Disease Pathology Surveillance Center (Dr. Gambetti), Division of Neuropathology, Institute of Pathology, Case Western Reserve University, Cleveland, OH.


Address correspondence and reprint requests to Dr. Ermias D. Belay, 1600 Clifton Road, Mailstop A-39, Atlanta, GA 30333.



26 March 2003


Terry S. Singeltary, retired (medically) CJD WATCH


I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?



The Pathological Protein:


Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases


Philip Yam


''Answering critics like Terry Singeltary, who feels that the US undercounts CJD, Schonberger _conceded_ that the current surveillance system has errors but stated that most of the errors will be confined to the older population''....end






Clinically Unsuspected Prion Disease Among Patients With Dementia Diagnoses in an Alzheimer’s Disease Database


Ryan A. Maddox, PhD1⇑ J. L. Blase, MPH1 N. D. Mercaldo, MS2,3 A. R. Harvey, MSPH1 L. B. Schonberger, MD1 W. A. Kukull, PhD3 E. D. Belay, MD1 1Division of High-Consequence Pathogens and Pathology, National Center for Emerging and Zoonotic Infectious Diseases, Centers for Disease Control and Prevention, Atlanta, GA, USA 2Department of Biostatistics, Vanderbilt University, Nashville, TN, USA 3National Alzheimer’s Coordinating Center, University of Washington, Seattle, WA, USA Ryan A. Maddox, PhD, Centers for Disease Control and Prevention, 1600 Clifton Road, Mailstop A-30, Atlanta, GA 30333, USA. Email:


Abstract Background: Brain tissue analysis is necessary to confirm prion diseases. Clinically unsuspected cases may be identified through neuropathologic testing.


Methods: National Alzheimer’s Coordinating Center (NACC) Minimum and Neuropathologic Data Set for 1984 to 2005 were reviewed. Eligible patients had dementia, underwent autopsy, had available neuropathologic data, belonged to a currently funded Alzheimer’s Disease Center (ADC), and were coded as having an Alzheimer’s disease clinical diagnosis or a nonprion disease etiology. For the eligible patients with neuropathology indicating prion disease, further clinical information, collected from the reporting ADC, determined whether prion disease was considered before autopsy.


Results: Of 6000 eligible patients in the NACC database, 7 (0.12%) were clinically unsuspected but autopsy-confirmed prion disease cases.


Conclusion: The proportion of patients with dementia with clinically unrecognized but autopsy-confirmed prion disease was small. Besides confirming clinically suspected cases, neuropathology is useful to identify unsuspected clinically atypical cases of prion disease.


prion disease Creutzfeldt–Jakob disease Alzheimer’s disease dementia diagnosis



> Results: Of 6000 eligible patients in the NACC database, 7 (0.12%) were clinically unsuspected but autopsy-confirmed prion disease cases.


those figures seem large... tss


***Miracles do happen. it just took 3 decades of denial from these authors cdc et al to finally come up with this conclusion, during that time period how many humans have been exposed due to their continued denial over those decades?


see full text with old studies confirming misdiagnosis figures over the decades...tss Wednesday, September 2, 2015


Clinically Unsuspected Prion Disease Among Patients With Dementia Diagnoses in an Alzheimer’s Disease Database



Thursday, August 13, 2015


Iatrogenic CJD due to pituitary-derived growth hormone with genetically determined incubation times of up to 40 years



Self-Propagative Replication of Ab Oligomers Suggests Potential Transmissibility in Alzheimer Disease


Received July 24, 2014; Accepted September 16, 2014; Published November 3, 2014



*** Singeltary comment ***



Thursday, October 1, 2015


Alzheimergate, re-Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy, Singeltary Submission to Nature



Thursday, November 12, 2015


Evaluation of the protection of primates transfused with variant Creutzfeldt-Jakob disease–infected blood products filtered with prion removal devices: a 5-year update



2 January 2000


British Medical Journal


U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well



15 November 1999


British Medical Journal


vCJD in the USA * BSE in U.S.



The Lancet Infectious Diseases, Volume 3, Issue 8, Page 463, August 2003 doi:10.1016/S1473-3099(03)00715-1Cite or Link Using DOI


Tracking spongiform encephalopathies in North America




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 ...



Suspect symptoms


What if you can catch old-fashioned CJD by eating meat from a sheep infected with scrapie?


28 Mar 01


Most doctors believe that sCJD is caused by a prion protein deforming by chance into a killer. But Singeltary thinks otherwise. He is one of a number of campaigners who say that some sCJD, like the variant CJD related to BSE, is caused by eating meat from infected animals. Their suspicions have focused on sheep carrying scrapie, a BSE-like disease that is widespread in flocks across Europe and North America. Now scientists in France have stumbled across new evidence that adds weight to the campaigners' fears. To their complete surprise, the researchers found that one strain of scrapie causes the same brain damage in mice as sCJD.


"This means we cannot rule out that at least some sCJD may be caused by some strains of scrapie," says team member Jean-Philippe Deslys of the French Atomic Energy Commission's medical research laboratory in Fontenay-aux-Roses, south-west of Paris. Hans Kretschmar of the University of Göttingen, who coordinates CJD surveillance in Germany, is so concerned by the findings that he now wants to trawl back through past sCJD cases to see if any might have been caused by eating infected mutton or lamb...



cwd to humans, iatrogenic, what if ?



Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery.


Gibbs CJ Jr, Asher DM, Kobrine A, Amyx HL, Sulima MP, Gajdusek DC. Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892.


Stereotactic multicontact electrodes used to probe the cerebral cortex of a middle aged woman with progressive dementia were previously implicated in the accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger patients. The diagnoses of CJD have been confirmed for all three cases. More than two years after their last use in humans, after three cleanings and repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were implanted in the cortex of a chimpanzee. Eighteen months later the animal became ill with CJD. This finding serves to re-emphasise the potential danger posed by reuse of instruments contaminated with the agents of spongiform encephalopathies, even after scrupulous attempts to clean them.



Tuesday, May 26, 2015


*** Minimise transmission risk of CJD and vCJD in healthcare settings ***


Last updated 15 May 2015




***however in 1 C-type challenged animal, Prion 2015 Poster Abstracts S67 PrPsc was not detected using rapid tests for BSE.


***Subsequent testing resulted in the detection of pathologic lesion in unusual brain location and PrPsc detection by PMCA only.


IBNC Tauopathy or TSE Prion disease, it appears, no one is sure


Posted by flounder on 03 Jul 2015 at 16:53 GMT



31 Jan 2015 at 20:14 GMT


*** Ruminant feed ban for cervids in the United States? ***


Singeltary et al


31 Jan 2015 at 20:14 GMT



Monday, October 26, 2015







Using in vitro prion replication for high sensitive detection of prions and prionlike proteins and for understanding mechanisms of transmission.


Claudio Soto


Mitchell Center for Alzheimer's diseases and related Brain disorders, Department of Neurology, University of Texas Medical School at Houston.


Prion and prion-like proteins are misfolded protein aggregates with the ability to selfpropagate to spread disease between cells, organs and in some cases across individuals. I n T r a n s m i s s i b l e s p o n g i f o r m encephalopathies (TSEs), prions are mostly composed by a misfolded form of the prion protein (PrPSc), which propagates by transmitting its misfolding to the normal prion protein (PrPC). The availability of a procedure to replicate prions in the laboratory may be important to study the mechanism of prion and prion-like spreading and to develop high sensitive detection of small quantities of misfolded proteins in biological fluids, tissues and environmental samples. Protein Misfolding Cyclic Amplification (PMCA) is a simple, fast and efficient methodology to mimic prion replication in the test tube. PMCA is a platform technology that may enable amplification of any prion-like misfolded protein aggregating through a seeding/nucleation process. In TSEs, PMCA is able to detect the equivalent of one single molecule of infectious PrPSc and propagate prions that maintain high infectivity, strain properties and species specificity. Using PMCA we have been able to detect PrPSc in blood and urine of experimentally infected animals and humans affected by vCJD with high sensitivity and specificity. Recently, we have expanded the principles of PMCA to amplify amyloid-beta (Aβ) and alphasynuclein (α-syn) aggregates implicated in Alzheimer's and Parkinson's diseases, respectively. Experiments are ongoing to study the utility of this technology to detect Aβ and α-syn aggregates in samples of CSF and blood from patients affected by these diseases.




***Recently, we have been using PMCA to study the role of environmental prion contamination on the horizontal spreading of TSEs. These experiments have focused on the study of the interaction of prions with plants and environmentally relevant surfaces. Our results show that plants (both leaves and roots) bind tightly to prions present in brain extracts and excreta (urine and feces) and retain even small quantities of PrPSc for long periods of time. Strikingly, ingestion of prioncontaminated leaves and roots produced disease with a 100% attack rate and an incubation period not substantially longer than feeding animals directly with scrapie brain homogenate. Furthermore, plants can uptake prions from contaminated soil and transport them to different parts of the plant tissue (stem and leaves). Similarly, prions bind tightly to a variety of environmentally relevant surfaces, including stones, wood, metals, plastic, glass, cement, etc. Prion contaminated surfaces efficiently transmit prion disease when these materials were directly injected into the brain of animals and strikingly when the contaminated surfaces were just placed in the animal cage. These findings demonstrate that environmental materials can efficiently bind infectious prions and act as carriers of infectivity, suggesting that they may play an important role in the horizontal transmission of the disease.




Since its invention 13 years ago, PMCA has helped to answer fundamental questions of prion propagation and has broad applications in research areas including the food industry, blood bank safety and human and veterinary disease diagnosis.



see ;








98 | Veterinary Record | January 24, 2015




Scrapie: a particularly persistent pathogen


Cristina Acín


Resistant prions in the environment have been the sword of Damocles for scrapie control and eradication. Attempts to establish which physical and chemical agents could be applied to inactivate or moderate scrapie infectivity were initiated in the 1960s and 1970s,with the first study of this type focusing on the effect of heat treatment in reducing prion infectivity (Hunter and Millson 1964). Nowadays, most of the chemical procedures that aim to inactivate the prion protein are based on the method developed by Kimberlin and collaborators (1983). This procedure consists of treatment with 20,000 parts per million free chlorine solution, for a minimum of one hour, of all surfaces that need to be sterilised (in laboratories, lambing pens, slaughterhouses, and so on). Despite this, veterinarians and farmers may still ask a range of questions, such as ‘Is there an official procedure published somewhere?’ and ‘Is there an international organisation which recommends and defines the exact method of scrapie decontamination that must be applied?’


From a European perspective, it is difficult to find a treatment that could be applied, especially in relation to the disinfection of surfaces in lambing pens of affected flocks. A 999/2001 EU regulation on controlling spongiform encephalopathies (European Parliament and Council 2001) did not specify a particular decontamination measure to be used when an outbreak of scrapie is diagnosed. There is only a brief recommendation in Annex VII concerning the control and eradication of transmissible spongiform encephalopathies (TSE s).


Chapter B of the regulation explains the measures that must be applied if new caprine animals are to be introduced to a holding where a scrapie outbreak has previously been diagnosed. In that case, the statement indicates that caprine animals can be introduced ‘provided that a cleaning and disinfection of all animal housing on the premises has been carried out following destocking’.


Issues around cleaning and disinfection are common in prion prevention recommendations, but relevant authorities, veterinarians and farmers may have difficulties in finding the specific protocol which applies. The European Food and Safety Authority (EFSA ) published a detailed report about the efficacy of certain biocides, such as sodium hydroxide, sodium hypochlorite, guanidine and even a formulation of copper or iron metal ions in combination with hydrogen peroxide, against prions (EFSA 2009). The report was based on scientific evidence (Fichet and others 2004, Lemmer and others 2004, Gao and others 2006, Solassol and others 2006) but unfortunately the decontamination measures were not assessed under outbreak conditions.


The EFSA Panel on Biological Hazards recently published its conclusions on the scrapie situation in the EU after 10 years of monitoring and control of the disease in sheep and goats (EFSA 2014), and one of the most interesting findings was the Icelandic experience regarding the effect of disinfection in scrapie control. The Icelandic plan consisted of: culling scrapie-affected sheep or the whole flock in newly diagnosed outbreaks; deep cleaning and disinfection of stables, sheds, barns and equipment with high pressure washing followed by cleaning with 500 parts per million of hypochlorite; drying and treatment with 300 ppm of iodophor; and restocking was not permitted for at least two years. Even when all of these measures were implemented, scrapie recurred on several farms, indicating that the infectious agent survived for years in the environment, even as many as 16 years after restocking (Georgsson and others 2006).


In the rest of the countries considered in the EFSA (2014) report, recommendations for disinfection measures were not specifically defined at the government level. In the report, the only recommendation that is made for sheep is repopulation with sheep with scrapie-resistant genotypes. This reduces the risk of scrapie recurrence but it is difficult to know its effect on the infection.


Until the EFSA was established (in May 2003), scientific opinions about TSE s were provided by the Scientific Steering Committee (SSC) of the EC, whose advice regarding inactivation procedures focused on treating animal waste at high temperatures (150°C for three hours) and high pressure alkaline hydrolysis (SSC 2003). At the same time, the TSE Risk Management Subgroup of the Advisory Committee on Dangerous Pathogens (ACDP) in the UK published guidance on safe working and the prevention of TSE infection. Annex C of the ACDP report established that sodium hypochlorite was considered to be effective, but only if 20,000 ppm of available chlorine was present for at least one hour, which has practical limitations such as the release of chlorine gas, corrosion, incompatibility with formaldehyde, alcohols and acids, rapid inactivation of its active chemicals and the stability of dilutions (ACDP 2009).


In an international context, the World Organisation for Animal Health (OIE) does not recommend a specific disinfection protocol for prion agents in its Terrestrial Code or Manual. Chapter 4.13 of the Terrestrial Code, General recommendations on disinfection and disinsection (OIE 2014), focuses on foot-and-mouth disease virus, mycobacteria and Bacillus anthracis, but not on prion disinfection. Nevertheless, the last update published by the OIE on bovine spongiform encephalopathy (OIE 2012) indicates that few effective decontamination techniques are available to inactivate the agent on surfaces, and recommends the removal of all organic material and the use of sodium hydroxide, or a sodium hypochlorite solution containing 2 per cent available chlorine, for more than one hour at 20ºC.


The World Health Organization outlines guidelines for the control of TSE s, and also emphasises the importance of mechanically cleaning surfaces before disinfection with sodium hydroxide or sodium hypochlorite for one hour (WHO 1999).


Finally, the relevant agencies in both Canada and the USA suggest that the best treatments for surfaces potentially contaminated with prions are sodium hydroxide or sodium hypochlorite at 20,000 ppm. This is a 2 per cent solution, while most commercial household bleaches contain 5.25 per cent sodium hypochlorite. It is therefore recommended to dilute one part 5.25 per cent bleach with 1.5 parts water (CDC 2009, Canadian Food Inspection Agency 2013).


So what should we do about disinfection against prions? First, it is suggested that a single protocol be created by international authorities to homogenise inactivation procedures and enable their application in all scrapie-affected countries. Sodium hypochlorite with 20,000 ppm of available chlorine seems to be the procedure used in most countries, as noted in a paper summarised on p 99 of this issue of Veterinary Record (Hawkins and others 2015). But are we totally sure of its effectiveness as a preventive measure in a scrapie outbreak? Would an in-depth study of the recurrence of scrapie disease be needed?


What we can conclude is that, if we want to fight prion diseases, and specifically classical scrapie, we must focus on the accuracy of diagnosis, monitoring and surveillance; appropriate animal identification and control of movements; and, in the end, have homogeneous and suitable protocols to decontaminate and disinfect lambing barns, sheds and equipment available to veterinarians and farmers. Finally, further investigations into the resistance of prion proteins in the diversity of environmental surfaces are required.






98 | Veterinary Record | January 24, 2015



Persistence of ovine scrapie infectivity in a farm environment following cleaning and decontamination


Steve A. C. Hawkins, MIBiol, Pathology Department1, Hugh A. Simmons, BVSc MRCVS, MBA, MA Animal Services Unit1, Kevin C. Gough, BSc, PhD2 and Ben C. Maddison, BSc, PhD3 + Author Affiliations


1Animal and Plant Health Agency, Woodham Lane, New Haw, Addlestone, Surrey KT15 3NB, UK 2School of Veterinary Medicine and Science, The University of Nottingham, Sutton Bonington, Loughborough, Leicestershire LE12 5RD, UK 3ADAS UK, School of Veterinary Medicine and Science, The University of Nottingham, Sutton Bonington, Loughborough, Leicestershire LE12 5RD, UK E-mail for correspondence: Abstract Scrapie of sheep/goats and chronic wasting disease of deer/elk are contagious prion diseases where environmental reservoirs are directly implicated in the transmission of disease. In this study, the effectiveness of recommended scrapie farm decontamination regimens was evaluated by a sheep bioassay using buildings naturally contaminated with scrapie. Pens within a farm building were treated with either 20,000 parts per million free chorine solution for one hour or were treated with the same but were followed by painting and full re-galvanisation or replacement of metalwork within the pen. Scrapie susceptible lambs of the PRNP genotype VRQ/VRQ were reared within these pens and their scrapie status was monitored by recto-anal mucosa-associated lymphoid tissue. All animals became infected over an 18-month period, even in the pen that had been subject to the most stringent decontamination process. These data suggest that recommended current guidelines for the decontamination of farm buildings following outbreaks of scrapie do little to reduce the titre of infectious scrapie material and that environmental recontamination could also be an issue associated with these premises.






Thorough pressure washing of a pen had no effect on the amount of bioavailable scrapie infectivity (pen B). The routine removal of prions from surfaces within a laboratory setting is treatment for a minimum of one hour with 20,000 ppm free chlorine, a method originally based on the use of brain macerates from infected rodents to evaluate the effectiveness of decontamination (Kimberlin and others 1983). Further studies have also investigated the effectiveness of hypochlorite disinfection of metal surfaces to simulate the decontamination of surgical devices within a hospital setting. Such treatments with hypochlorite solution were able to reduce infectivity by 5.5 logs to lower than the sensitivity of the bioassay used (Lemmer and others 2004). Analogous treatment of the pen surfaces did not effectively remove the levels of scrapie infectivity over that of the control pens, indicating that this method of decontamination is not effective within a farm setting. This may be due to the high level of biological matrix that is present upon surfaces within the farm environment, which may reduce the amount of free chlorine available to inactivate any infectious prion. Remarkably 1/5 sheep introduced into pen D had also became scrapie positive within nine months, with all animals in this pen being RAMALT positive by 18 months of age. Pen D was no further away from the control pen (pen A) than any of the other pens within this barn. Localised hot spots of infectivity may be present within scrapie-contaminated environments, but it is unlikely that pen D area had an amount of scrapie contamination that was significantly different than the other areas within this building. Similarly, there were no differences in how the biosecurity of pen D was maintained, or how this pen was ventilated compared with the other pens. This observation, perhaps, indicates the slower kinetics of disease uptake within this pen and is consistent with a more thorough prion removal and recontamination. These observations may also account for the presence of inadvertent scrapie cases within other studies, where despite stringent biosecurity, control animals have become scrapie positive during challenge studies using barns that also housed scrapie-affected animals (Ryder and others 2009).


***The bioassay data indicate that the exposure of the sheep to a farm environment after decontamination efforts thought to be effective in removing scrapie is sufficient for the animals to become infected with scrapie. The main exposure routes within this scenario are likely to be via the oral route, during feeding and drinking, and respiratory and conjunctival routes. It has been demonstrated that scrapie infectivity can be efficiently transmitted via the nasal route in sheep (Hamir and others 2008), as is the case for CWD in both murine models and in white-tailed deer (Denkers and others 2010, 2013).


Recently, it has also been demonstrated that CWD prions presented as dust when bound to the soil mineral montmorillonite can be infectious via the nasal route (Nichols and others 2013). When considering pens C and D, the actual source of the infectious agent in the pens is not known, it is possible that biologically relevant levels of prion survive on surfaces during the decontamination regimen (pen C). With the use of galvanising and painting (pen D) covering and sealing the surface of the pen, it is possible that scrapie material recontaminated the pens by the movement of infectious prions contained within dusts originating from other parts of the barn that were not decontaminated or from other areas of the farm.


Given that scrapie prions are widespread on the surfaces of affected farms (Maddison and others 2010a), irrespective of the source of the infectious prions in the pens, this study clearly highlights the difficulties that are faced with the effective removal of environmentally associated scrapie infectivity. This is likely to be paralleled in CWD which shows strong similarities to scrapie in terms of both the dissemination of prions into the environment and the facile mode of disease transmission. These data further contribute to the understanding that prion diseases can be highly transmissible between susceptible individuals not just by direct contact but through highly stable environmental reservoirs that are refractory to decontamination.


The presence of these environmentally associated prions in farm buildings make the control of these diseases a considerable challenge, especially in animal species such as goats where there is lack of genetic resistance to scrapie and, therefore, no scope to re-stock farms with animals that are resistant to scrapie.


Scrapie Sheep Goats Transmissible spongiform encephalopathies (TSE) Accepted October 12, 2014. Published Online First 31 October 2014



Monday, November 3, 2014


Persistence of ovine scrapie infectivity in a farm environment following cleaning and decontamination





Detection of Environmentally Associated PrPSc on a Farm with Endemic Scrapie


Ben C. Maddison,1 Claire A. Baker,1 Helen C. Rees,1 Linda A. Terry,2 Leigh Thorne,2 Susan J. Belworthy2 and Kevin C. Gough3 1ADAS-UK LTD; Department of Biology; University of Leicester; Leicester, UK; 2Veterinary Laboratories Agency; Surry, KT UK; 3Department of Veterinary Medicine and Science; University of Nottingham; Sutton Bonington, Loughborough UK


Key words: scrapie, evironmental persistence, sPMCA


Ovine scrapie shows considerable horizontal transmission, yet the routes of transmission and specifically the role of fomites in transmission remain poorly defined. Here we present biochemical data demonstrating that on a scrapie-affected sheep farm, scrapie prion contamination is widespread. It was anticipated at the outset that if prions contaminate the environment that they would be there at extremely low levels, as such the most sensitive method available for the detection of PrPSc, serial Protein Misfolding Cyclic Amplification (sPMCA), was used in this study. We investigated the distribution of environmental scrapie prions by applying ovine sPMCA to samples taken from a range of surfaces that were accessible to animals and could be collected by use of a wetted foam swab. Prion was amplified by sPMCA from a number of these environmental swab samples including those taken from metal, plastic and wooden surfaces, both in the indoor and outdoor environment. At the time of sampling there had been no sheep contact with these areas for at least 20 days prior to sampling indicating that prions persist for at least this duration in the environment. These data implicate inanimate objects as environmental reservoirs of prion infectivity which are likely to contribute to disease transmission.



the tse prion aka mad cow type disease is not your normal pathogen.


The TSE prion disease survives ashing to 600 degrees celsius, that’s around 1112 degrees farenheit.


you cannot cook the TSE prion disease out of meat. you can take the ash and mix it with saline and inject that ash into a mouse, and the mouse will go down with TSE.


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


the TSE prion agent also survives Simulated Wastewater Treatment Processes.


IN fact, you should also know that the TSE Prion agent will survive in the environment for years, if not decades.


you can bury it and it will not go away.


The TSE agent is capable of infected your water table i.e. Detection of protease-resistant cervid prion protein in water from a CWD-endemic area.


it’s not your ordinary pathogen you can just cook it out and be done with.


that’s what’s so worrisome about Iatrogenic mode of transmission, a simple autoclave will not kill this TSE prion agent.


cwd to humans, consumption, exposure, sub-clinical, iatrogenic, what if ?


Saturday, January 31, 2015


European red deer (Cervus elaphus elaphus) are susceptible to Bovine Spongiform Encephalopathy BSE by Oral Alimentary route



*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics of BSE in Canada Singeltary reply ;



Saturday, March 21, 2015


*** Canada and United States Creutzfeldt Jakob TSE Prion Disease Incidence Rates Increasing



 *** O.05: Transmission of prions to primates after extended silent incubation periods: ***Implications for BSE and scrapie risk assessment in human populations


Emmanuel Comoy, Jacqueline Mikol, Valerie Durand, Sophie Luccantoni, Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys Atomic Energy Commission; Fontenay-aux-Roses, France


Prion diseases (PD) are the unique neurodegenerative proteinopathies reputed to be transmissible under field conditions since decades. The transmission of Bovine Spongiform Encephalopathy (BSE) to humans evidenced that an animal PD might be zoonotic under appropriate conditions. Contrarily, in the absence of obvious (epidemiological or experimental) elements supporting a transmission or genetic predispositions, PD, like the other proteinopathies, are reputed to occur spontaneously (atpical animal prion strains, sporadic CJD summing 80% of human prion cases). Non-human primate models provided the first evidences supporting the transmissibiity of human prion strains and the zoonotic potential of BSE. Among them, cynomolgus macaques brought major information for BSE risk assessment for human health (Chen, 2014), according to their phylogenetic proximity to humans and extended lifetime. We used this model to assess the zoonotic potential of other animal PD from bovine, ovine and cervid origins even after very long silent incubation periods.


*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period,


***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold longe incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014),


***is the third potentially zoonotic PD (with BSE and L-type BSE),


***thus questioning the origin of human sporadic cases. We will present an updated panorama of our different transmission studies and discuss the implications of such extended incubation periods on risk assessment of animal PD for human health.




***thus questioning the origin of human sporadic cases...TSS







***Moreover, L-BSE has been transmitted more easily to transgenic mice overexpressing a human PrP [13,14] or to primates [15,16] than C-BSE.


***It has been suggested that some sporadic CJD subtypes in humans may result from an exposure to the L-BSE agent.


*** Lending support to this hypothesis, pathological and biochemical similarities have been observed between L-BSE and an sCJD subtype (MV genotype at codon 129 of PRNP) [17], and between L-BSE infected non-human primate and another sCJD subtype (MM genotype) [15].












Zoonotic Potential of CWD Prions


Liuting Qing1, Ignazio Cali1,2, Jue Yuan1, Shenghai Huang3, Diane Kofskey1, Pierluigi Gambetti1, Wenquan Zou1, Qingzhong Kong1 1Case Western Reserve University, Cleveland, Ohio, USA, 2Second University of Naples, Naples, Italy, 3Encore Health Resources, Houston, Texas, USA


*** These results indicate that the CWD prion has the potential to infect human CNS and peripheral lymphoid tissues and that there might be asymptomatic human carriers of CWD infection.




***These results indicate that the CWD prion has the potential to infect human CNS and peripheral lymphoid tissues and that there might be asymptomatic human carriers of CWD infection.***




P.105: RT-QuIC models trans-species prion transmission


Kristen Davenport, Davin Henderson, Candace Mathiason, and Edward Hoover Prion Research Center; Colorado State University; Fort Collins, CO USA


Conversely, FSE maintained sufficient BSE characteristics to more efficiently convert bovine rPrP than feline rPrP. Additionally, human rPrP was competent for conversion by CWD and fCWD.


***This insinuates that, at the level of protein:protein interactions, the barrier preventing transmission of CWD to humans is less robust than previously estimated.




***This insinuates that, at the level of protein:protein interactions, the barrier preventing transmission of CWD to humans is less robust than previously estimated.***





From: Terry S. Singeltary Sr.


Sent: Saturday, November 15, 2014 9:29 PM


To: Terry S. Singeltary Sr.










*** The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04). (SEE LINK IN REPORT HERE...TSS) PLUS, THE CDC DID NOT PUT THIS WARNING OUT FOR THE WELL BEING OF THE DEER AND ELK ;





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



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






> 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)



Friday, August 14, 2015


*** Susceptibility of cattle to the agent of chronic wasting disease from elk after intracranial inoculation



July's Milwaukee Journal Sentinel article did prod state officials to ask CDC to investigate the cases of the three men who shared wild game feasts. The two men the CDC is still investigating were 55 and 66 years old. But there's also Kevin Boss, a Minnesota hunter who ate Barron County venison and died of CJD at 41. And there's Jeff Schwan, whose Michigan Tech fraternity brothers used to bring venison sausage back to the frat house. His mother, Terry, says that in May 2001, Jeff, 26, began complaining about his vision. A friend noticed misspellings in his e-mail, which was totally unlike him. Jeff began losing weight. He became irritable and withdrawn. By the end of June, he couldn't remember the four-digit code to open the garage door or when and how to feed his parents' cats. At a family gathering in July, he stuck to his parents and girlfriend, barely talking. "On the night we took him to the hospital, he was speaking like he was drunk or high and I noticed his pupils were so dilated I couldn't see the irises," his mother says. By then, Jeff was no longer able to do even simple things on his computer at work, and "in the hospital, he couldn't drink enough water." When he died on September 27, 2001, an autopsy confirmed he had sporadic CJD.


In 2000, Belay looked into three CJD cases reported by The Denver Post, two hunters who ate meat from animals killed in Wyoming and the daughter of a hunter who ate venison from a plant that processed Colorado elk. All three died of CJD before they were 30 years old. The CDC asked the USDA to kill 1,000 deer and elk in the area where the men hunted. Belay and others reported their findings in the Archives of Neurology, writing that although "circumstances suggested a link between the three cases and chronic wasting disease, they could find no 'causal' link." Which means, says Belay, "not a single one of those 1,000 deer tested positive for CWD. For all we know, these cases may be CWD. What we have now doesn't indicate a connection. That's reassuring, but it would be wrong to say it will never happen."


So far, says NIH researcher Race, the two Wisconsin cases pinpointed by the newspaper look like spontaneous CJD. "But we don't know how CWD would look in human brains. It probably would look like some garden-variety sporadic CJD." What the CDC will do with these cases and four others (three from Colorado and Schwan from Upper Michigan), Race says, is "sequence the prion protein from these people, inject it into mice and wait to see what the disease looks like in their brains. That will take two years."


CJD is so rare in people under age 30, one case in a billion (leaving out medical mishaps), that four cases under 30 is "very high," says Colorado neurologist Bosque. "Then, if you add these other two from Wisconsin [cases in the newspaper], six cases of CJD in people associated with venison is very, very high." Only now, with Mary Riley, there are at least seven, and possibly eight, with Steve, her dining companion. "It's not critical mass that matters," however, Belay says. "One case would do it for me." The chance that two people who know each other would both contact CJD, like the two Wisconsin sportsmen, is so unlikely, experts say, it would happen only once in 140 years.


Given the incubation period for TSEs in humans, it may require another generation to write the final chapter on CWD in Wisconsin. "Does chronic wasting disease pass into humans? We'll be able to answer that in 2022," says Race. Meanwhile, the state has become part of an immense experiment.



I urge everyone to watch this video closely...terry


*** you can see video here and interview with Jeff's Mom, and scientist telling you to test everything and potential risk factors for humans ***



Sunday, October 25, 2015


USAHA Detailed Events Schedule – 119th USAHA Annual Meeting CAPTIVE LIVESTOCK CWD SCRAPIE TSE PRION



Sunday, October 18, 2015


World Organisation for Animal Health (OIE) and the Institut Pasteur Cooperating on animal disease and zoonosis research



31 Jan 2015 at 20:14 GMT


*** Ruminant feed ban for cervids in the United States? ***


Singeltary et al


31 Jan 2015 at 20:14 GMT



Monday, October 26, 2015






Mad deer disease can infect normal human brains in laboratory tests


Monday, May 8, 2000 has further information and facts on CJD. Jennifer Kelly or Amy Leska, EMS 202/463-6670 Washington, D.C.


Public health advocates are demanding that the Food and Drug Administration close loopholes in animal feed regulations to prevent the spread of U.S. mad cow-type diseases now at epidemic levels in Western deer and elk that might infect people who eat meat. In a letter sent today to the FDA, the Center for Food Safety (CFS), the Humane Farming Association, and families of U.S. victims of the human version of mad cow disease, Creutzfeldt-Jakob disease (CJD) are demanding new efforts to protect public health and food safety. The FDA was asked to respond to a legal petition filed in January 1999 that would change U.S. animal feed regulations to prevent the spread of U.S. mad cow-type diseases already occurring in deer, elk, sheep and humans, and suspected in pigs and cattle.


Under current FDA regulations animals known to be infected with mad cow-type disease such as deer and elk infected with 'chronic wasting disease' and scrapie-infected sheep, can be legally fed to pigs, chickens and pets, which in turn can be rendered and fed to cows. Billions of pounds of slaughterhouse waste in the form of rendered animal by-products are fed to US livestock every year as fat and protein supplements, despite this practice being the known route of transmission of British mad cow disease.


A fatal 'mad deer' disease called chronic wasting disease or CWD is occurring at epidemic levels in deer and elk in Western states and on game farms, CFS legal director Joseph Mendelson wrote in the letter to the FDA. This may already be claiming human lives as is suggested by the alarming appearance of unusually young victims of Creutzfeldt-Jakob disease.


*** Today at the first CJD Foundation conference in Miami, government researcher Byron W. Caughey, Ph.D., announced that in laboratory tests CWD from deer can infect human brain tissue at rate similar to British mad cow disease. In Britain 56 people have died of human mad cow disease, the death toll is climbing and some scientists suspect it will claim hundreds of thousands of lives in the decades ahead. Caughey's research on US mad deer disese was conducted at the National Institutes of Health Rocky Mountain Laboratories in Hamilton, Montana, and has not yet been published.


The most recent suspected victim of US mad deer disease is Jay Dee Whitlock II of Oklahoma, who died of CJD on April 7, 2000. Whitlock, 28, was an avid deer hunter and venison consumer. He is the second young hunter to die of CJD in the past year.


John Stauber, co-author of Mad Cow USA and a speaker at the CJD Foundation conference, said, "The announcement that US mad deer disease can infect the human brain, and that it happens at a rate similar to British mad cow disease, is extremely disturbing. A deadly human dementia might be already spreading from deer and elk into hunters in Western states, and the policies of the FDA and other agencies are completely inadequate to protect public health.


Could 'mad cow type epidemics happen in the US John Stauber, talk given 7 May 00 CJD Foundation conference Miami Center for Media & Democracy 520 University Avenue, Suite #310 Madison, WI 53703


"Three years ago Sheldon Rampton and I were finishing our book Mad Cow USA published in November of 97. In our introduction we wrote that ours is not a biology book nor a diet guide, but a "book about politics and how it operates in the real world. It explains how government officials have placed concerns for the food industry over human health and welfare. In addition to telling the story of an exotic, mysterious and frightening disease, we have written this book to report on equally dangerous legal and political trends which threaten not only our physical health, but also our fundamental democratic rights..." The title of this session asks a question: "Could 'mad cow type epidemics happen in the US.?" My answer is not only could they, but they are. Sheep scrapie arrived in the US a half century ago and thanks to government bungling is now widely spread throughout the US, with dozens of different strains. Chronic wasting disease may have begun as sheep scrapie but now it is spreading through deer and elk in western states and on game farms.


In the past two years two western state hunters under the age of 30 have died of CJD, and some of us suspect they may be human victims of a new strain of TSE in sheep, deer or elk that has begun claiming young human victims in the US.


Since 1964 researchers have suspected that transmissible mink encephalopathy TME in the US is from a hidden TSE in cattle, and the late Dr. Richard Marsh, to whom we dedicate our book, believed that he isolated that TSE in mink in Wisconsin in 1985, a year before the British strain of TSE dubbed mad cow disease was observed.


As our book was going to completion, we discovered that USDA inspectors felt they had identified a TSE in pigs way back in the the 1970s, when few researchers other than a small group in what Paul Brown calls the Club were very concerned or aware of TSE diseases.


Of course, British mad cow disease and the looming specter of hundreds of thousands of people condemned to death in the decades ahead has changed everything. Everything, that is, except public policy here in the US. For despite the fact that two Nobel prize winners in this area of research are from the US, there is a public relations cover-up of massive proportions in this country that is preventing us from effectively establishing public policies that can monitor, prevent and eventually treat TSE diseases.


Please note that I said a public relations cover-up; that's important. I suspect that if TSE diseases were spread by mosquitoes, we'd be spraying pesticides all over the US to try and irradiate sheep scrapie and chronic wasting disease. But these diseases are spread by agribusiness through animal livestock products fed to people and animals, and thus instead of putting the concerns of people first, we have see that consistently governments have put the concerns of industry first.


We've heard eminent and hard working scientists talk about TSE diseases in terms that dizzy the head of even other scientists. This is a very contentious and mysterious area of research because TSE diseases break many rules.


Luckily, my co-author and I are not experts or scientists. However, we did have the benefit of being able to interview top TSE researchers like Richard Marsh and Dr. Gajdusek, have them review parts of our manuscript, and thus deliver a book that is based on solid, sound science regarding what is known, and what is unknown, what is proven, and what is not.


From my perspective, this is the most important point and perspective to keep in mind:


For the past thirty years a massive unregulated experiment in creating new strains of TSE disease has been undertaken by the livestock industry, and we're the guinea pigs. The experiment is ongoing. The experiment began as a really neat idea: lets take the billions and billions of pounds of slaughterhouse waste, blood and offal that is produced every year from cattle and pigs and sheep and roadkill and pets and zoo animals, and let's recycle it. Let's turn it into protein fat supplements, and let's feed it back to the livestock we eat.


It seemed like a good idea. Unfortunately, what it didn't take into account was the infectious prion agent. As Dr. Gibbs has pointed out, probably every mammalian species has a TSE disease at some minute level: people, pigs, cows, sheep, mink, cats and dogs, deer, elk. Grind up the most infectious parts of millions and millions of animals, concentrate them into feed supplements, and you are creating an environment not only for spreading and amplifying existing agents such as scrapie in sheep, but also for creating an untold number of new strains of TSE.


This is especially important to grasp. In laboratory experiments, when TME or scrapie or CJD is injected into new animal species, whole new strains of TSE are created, and they can have different potential to infect new host species. The process of rendering animal waste into animal feed has been a massive and ongoing experiment in the creation of new TSEs, one of which emerged in Britain in the mid 1980s as mad cow disease, has clearly spread into humans claiming over fifty so far, and in my guess will in the years ahead be founded to have infected hundreds of thousands of Britons.


I said that this outbreak of BSE in Britain changed everything, but what really changed everything was what caused that outbreak: feeding rendered animal by-products back to animals as food.


I wish I could tell you that this practice has been stopped, but it has not. I wish I could tell you that well informed and courageous officials in the FDA, the USDA and the CDC have taken the mad bull by the horns, and are right now executing a precautionary policy to insure that what has happened in Britain does not happen in the US. Unfortunately, that is not the case.


Dr. Hansen will shortly explain why the much heralded FDA feed regulations on rendered animal by-products announced in 1997 are in many ways a farce. For instance, in the US calves are literally being weaned on cattle blood protein, and scrapie infected sheep can be used as feed for pigs which can be used as feed for cattle.


But the problem is beyond just the poor US animal feed regulations. You need look no further than your favorite vitamin and nutritional supplement stores. Right now, with the full knowledge of the FDA, the NIH, the CDC, millions of Americans are popping over the counter as glandular supplements. These unregulated products contain the most infectious parts of slaughtered animals, the very parts that make up the so-called Specified Bovine Offal that should be banned from cattle feed, such as the brain, pituitary, and glandular system. These pills are pooled, collected from hundreds of thousands of animals and taken daily by untold numbers of Americans, probably millions given the popularity of supplements.


Again, this amounts to an unregulated human experiment, minus laboratory controls or knowledgeable consent, feeding humans the most infectious parts of animals, possible creating new TSEs by passaging animal TSE from pooled glandular products.


I've brought along four different bottles of the pills I'm talking about, and I've asked that they be pass around so you can look at them yourself and read the label.


Some would say that FDA's hands are tied, that thanks to lobbying by the nutritional supplement industry the FDA lacks the power to prevent sales of these glandular. Actually this points to another frightening disease rampant among otherwise good people who populate government agencies which I call BCD for Bureaucratic Cowardice Disease. FDAs hands might be tied and they can't yank these products from the market, but their mouths are not gagged and concerned officials should be speaking out loudly warning the American public that they are consuming animal brains and glands and might want to consider the potential risks.


There is a reason why scientists in agencies and universities, not to mention corporations, avoid sticking their necks out, and we need look no further than Richard Marsh, a dedicated and conservative scientist who took it upon himself to speak up in 1993 in an interview in Wisconsin's largest farm paper. Richard Marsh warned Wisconsin farmers, thousands of whom were feeding rendered cow by-products back to their cows, that given the mad cow outbreak in Britain they should, despite the reassurances from USDA that such feeding was safe, stop.


The day after that article appeared Dr. Marsh was literally called on the carpet in the office of the dean of the school of agriculture at the University of Wisconsin, and read the riot act. he was told that industry funders were threaten to sue him, sue the school, cut off money for research, and who did he think he was?


Within months the school scrambled and pulled together a symposium on the subject, which served to dismiss and marginalize Dr. Marsh's views. When Dr. Marsh died of cancer in 1997, after the British announcement that mad cow disease was killing young people, the same University had the gall to praise Dr. Marsh and his work as in the best tradition of the University. Some of us feel that the stress and abuse heaped on Dr. Marsh> from 1993 to 1997 had an impact on his health that contributed to his demise.


This is what happens to scientists who break ranks and speak out, and it is a great loss to us today because I think if Dick Marsh was still alive he would be leading the charge to confront the ignorance and the cow-towing to industry that typifies this issue.


Dr. Marsh was a colleague and contemporary of many of the researchers in this room. As Paul Brown whom he once worked with might say, he was a member of the club. But Dr. Marsh was able to make realizations that I'm afraid most of the club members haven't yet come to, and he was able to put his sense of scientific obligation to society in front of concerns about his funding, or even his personal and professional safety.


I first began investigating mad cow type diseases in the early 1990s when I was working in Wisconsin organizing farmers and consumers opposed to Monsanto's genetically engineered bovine growth hormone, the cattle equivalent of human growth hormone which when injected into cattle forced them to produce more milk. Well, its not quite that biologically simple as I'm sure any woman here who has had children can imagine.


It was brought to my attention by a retired industry veterinary researcher that in order to make the hormone work, cows need to be fed additional fat and protein supplements, and that the cheapest supplements were rendered byproducts from other cows. This veterinarian told me this was very bad because it was exactly what had cause the outbreak of BSE in Britain.


I investigated and found this was true - massive feeding of cows to cows was going on in the US, despite the fact that as early as the Fall of 1987 British epidemiology had shown that is was this practice that spread mad cow disease.


I remember one of my first meetings with Dr. Marsh. We talked about his believe that a US BSE agent, different than the British strain, was in cattle at low levels and was the cause of occasional outbreaks of TME. I know that eminent researchers in this room dispute that, but frankly Dr. Marsh never doubted it and I think the evidence and commons sense remains in his favor.


I had just been to a holistic chiropractor for my chronically bad lower back, and had been sold a bottle of adrenal gland extract which I showed to Dr. Marsh - he practically fell out of his chair and I immediately stopped taking them.


He was shocked to learn that such glandular were sold. Imagine what he might think today, that at this late stage of the game with kids in their 20s in the US dying of CJD, that these glandulars are sold without warning.


Of course, I may be taking a risk myself in saying this. After all, we are in Florida, one of 13 states in the US in which the Animal Feed Industry Association members have succeeded in lobbying into law a food product disparagement bill. I could end up like Oprah Winfrey and her guest Howard Lyman, forced to spend millions and millions of dollars (which in my case would be thousands and thousands and then bankruptcy) to convince a jury that my remarks today are based on sound science.


Having written two books in the past five years, one on political PR and the other on the politics of food, I can tell you that a major reason why there has not been more news media coverage of this issue is the multi-million dollar PR campaign and litigation threats of the food industry which we document extensively in our book.


I find it ironic that this weekend as we're meeting here President Clinton announced Saturday a new initiative to address the safety of hot dogs. Philip Brasher of the Associated Press speculates that as he is leaving office the President wants to burnish his image with food safety initiatives.


The irony is that it is under this administration that the food industry has launched an all out attack on our first amendment rights by lobbying food libel laws onto the books, and the administration has remained silent. Despite knowing of the risks of cow cannibalism since the beginning of his presidency, his administration did nothing until 1997, and as we reveal in our book and Dr. Hansen will explain, the regulations are severely inadequate.


I am circulating a letter being delivered by the Center for Food Safety urging the agency to respond to the petition filed by that group and many of the families in this room that calls on FDA to severely tighten its regulations. Yet, probably to avoid publicity, the FDA stonewalls.


A couple weeks ago I was in Washington and I attended a conference that was paid for in part by the lobbyists responsible for putting food censorships laws on the books, the Animal Feed Industry Association and the law firm of Ollsen Frank and Weeda that drafted the model bill that was then lobbied for at the state level by the American Farm Bureau. I picked up this glossy brochure in which the FDA is slapping itself on the back for its food safety initiatives. In it I read that the FDA's BSE Educational Activities have Gained an award from the vice president:


The US feed regulations and the FDA were honored with VP Al Gore's Hammer Award. Dr. Stephen F. Sundlof, the directory of FDA's Center for Veterinary Medicine, the man who since January 1999 has been unable to respond to the legal petition to close gaping loopholes in the FDA regulations stated: "thanks to the development of the BSE regulation, we can continue to say that there has not been a single case of BSE reported in the United States. Educational efforts ... will help assure that we can continue to make that claim."


In researching our books we had access to documents obtained through the Freedom on Information Act. One 1991 document of the USDA was titled "BSE Public Relations," and if someday attorneys in a class action lawsuit on behalf of CJD patients in the US are looking for a smoking gun demonstrating that concerns for industry were place over concerns for people, this is it.


(p.148-149) According to this document, the mere perception that BSE might exist in the United States could have devastating effects on our domestic markets for beef and dairy products." The report examined how the British handled their PR, and it fretted over a story in the British magazine The Economist which, quote, "could potentially create alarm among US consumers," unquote, because it reported that, quote,"many veterinarians and medical experts have come around to the belief that humans could catch the mystery brain disease."


This USDA PR document approvingly noted a quote in the Washington Post by Dr. Joseph Gibbs at NIH in 1990 saying "I don't think there is any danger in consuming British beef." Remember, this was in 1991. It had known for four years that it was feeding cows to cows that was spreading BSE in Britain. More and more were fearing that humans would die. Meanwhile, in the US, billions of pounds of cow by-products were still being fed, in fact the amount was increasing each year.


Was there no one inside the USDA or FDA who saw the lunacy, indeed the criminality, in knowing this and letting cannibalistic feeding go one in the US? Some did. The report notes that "some (USDA APHIS) staff members... argue that because there is evidence that pigs, cats, mink, deer and a variety of experimental animals may b e susceptible to trans. spongiform. encephalopathy, the only prudent policy is to not feed products that contain these agents to ANY SPECIES OF ANIMAL."


That's it. That's what we should have done then, and that's what we should be doing now, but are not. Instead we are still exposing ourselves and US livestock to a massive TSE experiment.


So, in 1991 the USDA and FDA rejected this advice by some anonymous staffers, as they do today. This 1991 USDA PR report admitted that a more cautious policy would be, quote, "to prohibit the feeding of sheep and cattle-origin protein products to all ruminants, regardless of age. The advantage of this option is that it minimized the risk of BSE. The disadvantage is that the cost to the livestock and rendering industries would be substantial."


In fact, absolutely nothing of substance was done until August of 1997, when FDA announced its sham feed regulation, winner of the coveted Al Gore hammer award, designed as a PR fig leaf so that FDA can say 'we are keeping British mad cow disease out of our livestock."


Let me say that the only bright spot in this story of corporate and government collusion, irresponsibility and censorship has been a few brave souls like Richard Marsh, Richard Lacey in England, others who have bucked the tide and spoken out, and those of you who are the families and loved ones of CJD victims.


*** Through CJD Foundation and CJD Voice you have found each other, and you will eventually force responsibility onto government and industry because if you don't no one else will.


In conclusion, we need a massive commitment in the United States to address TSE diseases. We need to start by admitting the shortcomings of our failed federal feed policies and change them. We need to dedicate hundreds of millions of research dollars, if necessary, to try to eradicate scrapie in sheep, eradicate CWD in deer and in elk, and investigate, research, test and monitor for TSEs in humans and animals. We need money for research to develop treatments, because while this disease used to be rare disease, I suspect we are about to see it emerge full blown in Britain with hundreds of thousands of victims, and more and younger victims appearing in the United States, as a result of our thirty year experiment in animal cannibalism.


The leadership for this will not come from politicians in bed with the agribusiness industry. It will not come from researchers who while brilliant are stuck back in pre-BSE days, still viewing this as a rare unusual disorder. It will come from average citizens who husbands and mothers and children are dying of this bizarre class of dementia diseases in increasing numbers, and who are not afraid to demand that the right policies be implemented and funded. It is upon your shoulders that leadership falls, and so far you are doing admirably."




snip...please see this and more here ;


Friday, October 23, 2015




Thursday, November 12, 2015


Evaluation of the protection of primates transfused with variant Creutzfeldt-Jakob disease–infected blood products filtered with prion removal devices: a 5-year update


























Terry S. Singeltary Sr.