In 2007 my beloved son Andrew Black was unlawfully killed by
VCJD/Human BSE, know as the human form of mad cow disease.
He was just 24 years old. Since his untimely and avloidable
death I created a campaign to find out why my Andrew died
and who is responsible. Food and medicines should be safe and never kill.
This is my blog containing the history of my investigations, facts and information the UK
government have tried to supress. It is the history of my ongoing investigations, and how
millions of us remain at risk of developing Human BSE. The disease has not gone away
and continues to kill people and animals globally in 2017.
Saturday 2nd September 2017
Today would have been my only son Andrew’s 34th birthday.
I can’t imagine how he would look or be as a mature man. The life he should have led, the relationships he would have forged, the wonderful career he would have had in the media, the friends he would have made, the children and grandchildren with whom he may have been blessed. All those days, nights, months and years he has lost. All those holidays, family celebrations, he has missed.
The empty seat at the dinner table will never be filled, there is void in our family that grows bigger with the passing years.
Andrew (centre holdiing a bottle) celebrating his 23rd Birthday.
Forever Andrew will be a young guy in his early twenties, dying far too soon and young.
Today I will walk to the cemetery and place flowers on his grave. A journey I have made every week for ten years. The cemetery is a harsh and uncompromising place for parents who tend their children’s graves.
Time does not heal and until those responsible ( many named and shamed on this website) are punished for stealing my son’s life and future, there is no healing for me or any of those victims and families who have been affected by human BSE/ vcjd/cjd/ prion disease, the human form of mad cow disease.
Andrew aged 22 on his way to a shift at TalkSPORT National Radio, London
WEDNESDAY 23rd August 2017
TUESDAY 1st August 2017
The research below highlights how people suffering from vCJD the Human form of BSE have passed the diseased on-wards through blood and medical procedures. This research found that many other tissues and parts of the body contain the rogue prions that cause human mad cow disease which are infectious and could have be a danger during surgery.
As this research states we need a individual blood test for vcjd so that all patients before hospital procedures are screened and all blood donors are screened for the human form of mad cow disease.
The consequences of BSE remains a lethal threat for millions of people across the globe and generations to come. A blood screening test for vcjd would stop the re cycling of this deadly disease within our global blood supply and during hospital procedures.
The UK Department of Health continues to with hold the facilities to implement a blood test for vcjd as it knows the legal implications when 1 in 2,000 people could be carrying of incubating the human form of mad cow disease.
All those named and shamed on this website would face criminal proceedings and the ‘establishment’ would be rocked to its core. This is why a blood test for vcjd will continued to be blocked by the UK DOH.
Volume 23, Number 6—June 2017 Synopsis
Distribution and Quantitative Estimates of Variant Creutzfeldt-Jakob Disease Prions in Tissues of Clinical and Asymptomatic Patients
Jean Y. Douet, Caroline Lacroux, Naima Aron, Mark W. Head, Séverine Lugan, Cécile Tillier, Alvina Huor, Hervé Cassard, Mark Arnold, Vincent Beringue, James W. Ironside, and Olivier Andréoletti
Comments to Author Author affiliations: Institut National de la Recherche Agronomique, Toulouse, France (J.Y. Douet, C. Lacroux, N. Aron, S. Lugan, C. Tillier, A. Huor, H. Cassard, O. Andréoletti); University of Edinburgh, Edinburgh, Scotland, UK (M.W. Head, J.W. Ironside); Animal and Plant Health Agency, Loughborough, UK (M. Arnold); Institut National de la Recherche Agronomique, Jouy-en-Josas, France (V. Beringue) Suggested citation for this article
In the United-Kingdom, ?1 of 2,000 persons could be infected with variant Creutzfeldt-Jakob disease (vCJD). Therefore, risk of transmission of vCJD by medical procedures remains a major concern for public health authorities. In this study, we used in vitro amplification of prions by protein misfolding cyclic amplification (PMCA) to estimate distribution and level of the vCJD agent in 21 tissues from 4 patients who died of clinical vCJD and from 1 asymptomatic person with vCJD. PMCA identified major levels of vCJD prions in a range of tissues, including liver, salivary gland, kidney, lung, and bone marrow. Bioassays confirmed that the quantitative estimate of levels of vCJD prion accumulation provided by PMCA are indicative of vCJD infectivity levels in tissues. Findings provide critical data for the design of measures to minimize risk for iatrogenic transmission of vCJD
Most previous studies with tissue from vCJD patients have failed to identify consistent accumulation of the vCJD agent outside the nervous and lymphoreticular systems. However, data obtained in this study clearly demonstrate the presence of vCJD prions in a wide and unexpected variety of peripheral tissues.
Natural scrapie and experimental BSE in sheep are 2 models of orally transmitted prion diseases (24,25). In both diseases, the agent accumulates in the lymphoreticular system and the enteric nervous system during the early preclinical phase of the incubation period. Moreover, an early and persistent prionemia is observed in asymptomatic infected animals (26,27). These features were also observed in vCJD in humans and in view of the likely origin of vCJD (oral exposure to BSE agent), these similarities have led to a consensus that BSE and scrapie in sheep and vCJD in human have a common pathogenesis (28).
Although vCJD prions in a variety tissues, such as bone marrow, kidney, salivary gland, skeletal muscle, pancreas, liver, or heart, might be surprising, each of these tissue has already been demonstrated to accumulate prion infectivity or abnormal prion protein in TSE-infected sheep (29–33). Because low levels of infectivity have been reported in blood fractions from a vCJD-affected patient, such widespread tissue positivity might be derived from residual blood, rather than from the solid tissue in these samples (16). However, this proposal seems unlikely because in whole blood PMCA amplification inhibitors preclude detection of endogenous vCJD agent by this method (11,34–36).
The patient in our study who was infected with a prion containing PRNP gene codon 129 Met/Val is 1 of only 2 identified vCJD agent–infected persons known to have died of other causes before onset clinical symptoms of vCJD, and the only person who provided consent to sample autopsy tissues for research. For this patient, all previous investigations did not detect abnormal prion protein or infectivity in the brain (12,37). The negative PMCA results we obtained for cerebral cortex, dorsal root ganglia, and trigeminal ganglia tissue from this patient are consistent with a lack of central nervous system involvement at the time of death. However, PMCA seeding activity in the pituitary gland was surprising in this instance.
The presence of abnormal prion protein accumulation in the pituitary gland and other circumventricular organs before deposition of PrPres in surrounding brain has been reported in TSE-infected sheep (38). However, this phenomenon in animals does not represent the main route for neuroinvasion and is a probable consequence of hematogenous dissemination of the TSE agent through the fenestrated capillary system of the circumventricular organs, which is substantially more permeable than the other capillaries in the brain (blood–brain barrier). Therefore, this finding might be a consequence of the hematogenous route of secondary vCJD in this person (by transfusion of packed erythrocytes from a vCJD-infected donor), in contrast to the oral route of infection in primary clinical vCJD cases (12).
Andrew (centre sitting down) aged 23 one year before he died of vcjd
vCJD prions were detected in certain peripheral tissues from the patients infected with a prion containing the PRNP gene codon 129 Met/Val. Although distribution of vCJD seeding activity in lymphoreticular tissues was similar to that observed for symptomatic vCJD patients, several tissues that were positive in clinically affected patients were negative in this heterozygous asymptomatic person. These findings suggest that involvement of some peripheral tissues might occur at a later stage in the incubation period than others, or that they could involve recirculation of the agent from the central nervous system (i.e., centrifugal spread in a late state). However, we cannot discount the possibility that that these differences in tissue distribution are caused by the hematogenous route of infection in this person (as opposed to the probable oral route in patients with clinical vCJD) or the difference between the PRNP gene codon 129 genotype of the asymptomatic vCJD–affected person (PRNP gene codon 129 Met/Val) and persons with clinical vCJD (PRNP gene codon 129 Met/Met).
Irrespective of the actual explanation for these differences, the presence of vCJD agent in peripheral tissues of patients during preclinical and clinical stage of the disease indicates the potential for iatrogenic transmission of this fatal neurologic condition by surgical procedures. Furthermore, this finding shows that, for certain peripheral tissues, a level of infectivity equivalent to an end stage titer (and attendant risk) is reached at a preclinical stage.
Several hundred cases of iatrogenic CJD have been reported worldwide. These cases appear to result from transmission of sporadic CJD, and most cases have occurred in recipients of human dura mater grafts or after administration of human growth hormone extracted from cadaveric pituitaries (39). Although in sporadic CJD the distribution of the agent is largely restricted to the nervous system (central and peripheral), the wide distribution of the vCJD agent in the asymptomatic infected patient we report might serve to increase the range of medical procedures, including dentistry, organ transplant, and surgery involving nondisposable equipment, that might result in iatrogenic transmission of vCJD (40–43).
Nevertheless, >20 years after identification of the first vCJD patients, only 5 cases that are a probable consequence of iatrogenic vCJD transmission are known, all in the United Kingdom and associated with blood and blood products. These cases were caused by transfusion of non–leukocyte-depleted erythrocyte concentrates or by treatment involving large amounts of pooled plasma from the United Kingdom that were known to include donations from persons who later showed development of vCJD (12,44–46).
None of the 220 other vCJD cases identified worldwide have been linked to any other medical or dental procedure. Whereas this fact is reassuring, it would be unwise to disregard the threat that vCJD still poses for public health. Despite the relatively low number (n = 178) of vCJD clinical cases observed in the United Kingdom, the most recent epidemiologic studies indicate that ?1 of 2,000 persons in the United Kingdom could be infected with the vCJD agent (as indicated by the presence of abnormal prion protein detected by immunohistochemical analysis of lymphoid follicles in the appendix). Each asymptomatic vCJD-infected person represents a potential source of secondary infection. The data in our report offer an opportunity for refining measures that were implemented in many countries to limit the risk for vCJD iatrogenic transmission. The apparent concordance between PMCA biochemical and infectivity bioassay data, and the higher analytical sensitivity of PMCA, suggest that future research need not rely exclusively on time-consuming and costly animal bioassay.
Our results indicate the need for vCJD screening assays. After more than a decade of effort, several vCJD blood detection tests have reached a stage in their development that could enable their evaluation as screening or confirmatory assays (11,47,48). In particular, there is now a strong case for use of PMCA in a highly sensitive and specific blood test for vCJD, as indicated by our previous studies (11,16) and studies by Bougard et al. (35) and Concha-Marambio et al. (36). The relationship shown here between PrPres amplification by PMCA and detection of infectivity by bioassay indicates that PMCA seeding activity is a good surrogate marker of infectivity and could provide a sound basis for a vCJD blood test for use with blood or tissue donors.
Dr. Douet is a research scientist and assistant lecturer in ophthalmology at the National Veterinary School of Toulouse, Toulouse, France. His primary research interests are the pathogenesis of the prion disease with special emphasis on the iatrogenic risk of transmission.
Andrew as a baby just after having vaccines which I didn’t know were made from BSE infected cattle
This study was supported in part by the Department of Health Policy Research Programme and the Scottish Government. The National CJD Research and Surveillance Unit is supported by the Policy Research Program of the Department of Health and the Scottish Government (DH121/5061). The Edinburgh Brain Bank is supported by the Medical Research Council (MRC grant G0900580). The Unité Mixte de Recherche 1225, Ecole Nationale Vétérinaire de Toulouse was supported by the European Union FEDER/INTERREG (EFA282/13 TRANSPRION), the Institut National de la Recherche Agronomique Institut Carnot en Santé Animale, and an Agence Nationale Recherche grant (Unmasking Blood Prions; ANR-15-CE18-0028).
WEDNESDAY 7th JUNE 2017
Chronic wasting disease or CWD is the equivalent of BSE in cattle and human mad cow disease/vCJD in people. CWD is killing deer, elk and animals that are hunted and are eaten by individuals and groups across the USA and Canada.
CWD in deer herds is the equivalent of BSE in cattle, and has the same lethal consequences to the animals and also to humans that eat or ingest infected meat from deer/animals suffering from CWD.
There have been many cases in the USA/Canada of people who hunt or have eaten deer developing CJD or dementia type disease. These cases have been dismissed by the authorities as being spontaneous and nothing to do with the individuals close proximity or having ingested meat from deer. The families of those killed by CJD/dementia type disease, who have been active hunters for many years insist that the symptoms suffered by their loved ones are exactly the same as my Andrew’s symptoms and other victims of vCJD. Both BSE and CWD are prion diseases and can have long incubation periods. All of these bereaved families concerns have been dismissed by the authorities.
Hunting of deer across the USA and Canada is a huge sporting activity with deer meat prized in many communities. There would be huge financial and legal implications if links were made between CWD in Canadian and USA deer herds and vCJD within the deer hunting community.
In the White Paper below experts highlight the ticking health time-bomb that CWD poses for deer/elk herds across the USA/Canada and also its deadly risk to humans. As many deer in the USA and Canada and across the globe are wild this also has huge implications for other wildlife and of course agriculture.
Most recently in the USA a deer herd was found to have CWD, the animals who were chronically diseased were destroyed and meat from the rest of the herd was distributed to ‘deprived people in the area’. As experts know, animals can be infectious with the lethal prions that cause vCJD in humans but be symptom free.
This is appalling as it means those poorer people are being allowed to eat meat from a herd infected with CWD, meat that is not deemed fit/safe enough to feed richer and more prosperous people in society.
So are these poorer folks being experimented upon to see how many of them develop vCJD in the next decades? In the same way that Margaret Thatcher and her government allowed BSE infected material to feed UK children in their free school meals and also vaccines made from BSE infected herds? Is this why so many younger people then died of vCJD in the UK and continue to die?
THE COVER UP CONTINUES.
CHRONIC WASTING DISEASE CWD TSE PRION ZOONOSIS ZOONOTIC INSIDIOUS AND DIRE CONSEQUENCES AHEAD
Alliance for Public Wildlife Living Legacy White Paper
The Challenge of CWD: Insidious and Dire Only immediate action will avoid catastrophic outcomes
Valerius Geist, Professor Emeritus, University of Calgary David Clausen, (former) Chair, Wisconsin Natural Resources Board Vince Crichton, (former) Co-Chair, Canada’s National Wildlife Disease Strategy Darrel Rowledge, Director, Alliance for Public Wildlife
Download this White Paper Download this white paper and other related publications at www.apwildlife.org/publications For more information To order copies of this white paper or receive information about other related publications, please contact Alliance for Public Wildlife at firstname.lastname@example.org
CWD is now deemed to be the largest-ever mass of infectious prions in global history, and experts sum up the threat (to wildlife, agriculture, our economies, and potentially to human health) in two words: “insidious and dire.” Current policy and apathy toward the levels of CWD consumption by people has been described as “one of the most outrageous human susceptibility experiments in history.
We have a problem. A big problem. Chronic Wasting Disease (CWD), a sister to BSE or ‘mad cow,’ is threatening our deer and elk. Unfortunately, CWD has broad implications. Without immediate action, we are heading for worst cases outcomes that include severe population impacts, extinctions, crashing economies, and, although unlikely, potential transfers of CWD to people.
Chronic Wasting Disease is an incurable, always fatal degeneration of the brain. Technically, it’s a Transmissible Spongiform Encephalopathy (TSE), but there are a number of quite different versions, depending on species. They include in humans kuru and fatal familial insomnia, as well as some with even more unpronounceable names, such as the dreadful human Creutzfeldt-Jakob Disease (CJD) and Gerstmann–Sträussler–Scheinker Disease (GSS). The largest TSE epidemics have been in domestic or captive animals: such as Scrapie in domestic sheep, Bovine Spongiform Encephalopathy (BSE), or so-called ‘mad cow’ disease, Transmissible Mink Encephalopathy (TME) on mink farms, and CWD in captive deer and elk.
CWD emerged as a particular nasty variant, because it can be transmitted by body fluids of infected animals (urine, feces, and saliva). Unlike BSE, CWD is highly contagious and can spread to and through wild ungulate herds. The infective agents are mis-folded proteins called prions; they are virtually indestructible, can persist in the environment, and tiny quantities can transmit the disease. Prion diseases have repeatedly jumped species barriers—most alarmingly in the United Kingdom, when BSE-infected beef killed 229 people.
As CWD spread, naturally and through trade, the U.S. in 2001 officially declared a “State of Emergency.” Every factor has since gotten worse. It has now been confirmed in 24 US states, 3 Canadian provinces, South Korea, and recently in Norway. Field studies are confirming potentially severe impacts on wildlife populations. So far no transmission to humans has been documented, but the risk is not zero. Non-human primates and transgenic (humanized) mice have been infected. In many jurisdictions, a lack of awareness and availability of free, rapid, and convenient testing of harvested deer has led to significant level of human exposure. Estimates show 7,000 to 15,000 CWD-infected animals are being consumed by hunter families every year, and this number continuing to rise by as much as 20% per year. The combination of threats is sobering. CWD has been shown to persist and remain infectious in the environment, including in clay-based soils that can dramatically increase infectivity (up to 680 times). Decomposing carcasses create contaminated “super-sites.” Prions are extremely resilient, known to resist disinfectants, alcohol, formaldehyde, detergents, protein enzymes, desiccation, radiation, freezing, and incineration >1100°F. Facilities infected with CWD have resisted all efforts at removing the infective agent. Canadian officials report that even on premises thought to be very low risk, restocking with healthy animals led to a 50% re-occurrence of CWD.
Transmission occurs animal to animal, soil to animal, mother-to-offspring, and from exposed plants or other surfaces including tools or surgical instruments (even autoclaving is ineffective). Now there is evidence the infective agent is taken up via the root systems of plants growing in contaminated soils, with transfer to stems and leaves. These were shown to be infective via inter-cerebral injection (oral tests are ongoing).
Left unchecked, the prospects for wildlife are bleak. CWD has clear population impacts; some models suggest extinction. Disproportionate impact on mature males carries implications for hunters and wildlife economies let alone populations. Still more bad news: Efforts for vaccines have failed, and evolutionary or adaptive salvation is unlikely and would be too late in any case. CWD is now deemed to be the largest-ever mass of infectious prions in global history, and experts sum up the threat (to wildlife, agriculture, our economies, and potentially to human health) in two words: “insidious and dire.” Current policy and apathy toward the levels of CWD consumption by people has been described as “one of the most outrageous human susceptibility experiments in history.”
The good news
There is, of course, much more—but we need to get to the good news: There is hope, beginning with the fact that CWD is relatively new—not a long-standing or indigenous disease of our wildlife. The vast majority of our herds are still disease-free. We have considerable expertise, leading-edge technologies, and the benefit of experience. We faced a crisis on this scale once before, almost exactly a century ago, when the very existence of wildlife on this continent was threatened by the severest of over-exploitation. Hunters and conservation organizations led the efforts to avert disaster. With the courage and foresight of presidents and prime ministers enlisting the best and ablest on both sides of the US/Canada border to enact science-based policies, they turned our greatest tragedy into a ‘triumph of the commons.’ Anchored in the public trust doctrine, and now recognized as the North American Model of Wildlife Conservation, it replenished an entire continent with wildlife.
We need, today, nothing less than a similar effort to manage the Chronic Wasting Disease crisis. We have the benefit of experience and principles for success. Following the Roosevelt Doctrine, the same concerned hunter and conservation organizations must once again be the standard-bearers of principled, science-and evidence-based leadership in wildlife conservation. We must be relentless in following the leading science and scholarship, tracking the evidence, and engaging in comprehensive analysis to foresee the implications. We understand how policies affect the spread of diseases, as documented in the scientific and historical record summarized below. This threat is dire, and immediate action is warranted.
While details and methods must be guided by science and evidence, there is significant agreement on critical needs; and we have assurances from leading experts and labs that we have the capacity to meet this challenge. We must secure mandate and funding to:
- Contain the geographic spread of CWD by enacting and enforcing an immediate ban on the movement of all live cervids, all potentially CWD-infected carcasses, animal parts, products, exposed equipment, trailers, or other sources of infectious materials.
- Mandate and implement for hunters, convenient, cost-free, rapid testing of all animals harvested from CWD-affected areas.
- Ensure that no CWD-infected material reaches the food or feed chains, and that it is instead properly disposed of.
- Establish and fund accountable research and science-based policy to protect public interest (health, wildlife and related industries, agriculture, our economies and communities).
The issues are numerous, serious, and complex, but complacency is not an option. The sooner we act, the greater the prospects to protect our greatest living legacy. Further details, discussion, citations, and scientific references follow.
Potential Risk of Transfer to People There are few considerations which require greater foundational context than questions of zoonotic risk of infectious diseases. Combining public policy and science is very much a matter of addressing uncertain risks that are dynamic, evolving, and with complex, even profound consequences.
The reality is that most (~70%) emerging zoonotic diseases have come from animals.150, 151 Each presented uncertain risks, and in every instance there was a point in history where the animal to human transfer of that particular pathogen had not yet occurred. Such absence of evidence or ‘proof’ can often elicit false inferences that dismiss or underestimate the risk. The UK example of BSE (unexpectedly) transferring to people as vCJD, is but one recent example.152 The impacts were complex, extending far beyond immediate victims, bringing serious and prolonged socioeconomic and health consequences that included suicides tied to the severe economic impacts of BSE on the agricultural economy.153 There are persisting uncertain zoonotic risks related to the BSE that remain to this day. For example, findings in lymphoreticular tissue (archived through appendix samples) indicate that 1 in 2,000 of the UK population are asymptomatic carriers infected with abnormal PrP.154 The long term implications are unknown.
Zoonotic risks are neither static nor merely historic phenomena: “it is estimated that approximately 75 per cent of ‘new’ human pathogens reported in the past 25 years have originated in animals and the risk of zoonoses is predicted to continue to increase.”155 As status quo matters of public policy they require consideration of known and potential consequences. “The Global Burden of Disease Study estimates that, in the year 2000, infectious diseases were responsible for 22% of all deaths and 27% of disability-adjusted life years worldwide.”156
Such risk profiles can only be considered as snapshots in dynamic, evolving landscapes, where observation and evidence of variability—indicating change or evolution—is a vital consideration. This underscores the very essence of the precautionary principle, and nowhere is it more requisite than with respect to infectious pathogens. Inadequate policy or regulatory failures can result in pandemics that kill thousands or even millions of people or other animals, causing enormous damage on economies and ecosystems.
The Precautionary Principle
Where there is a potential for severe or irreversible harm, especially to public wellbeing and interest, an absence of scientific consensus or proof of harm cannot be used to allow or maintain policies or actions underlying the risk. In such cases, the burden to ‘prove safety’ falls on those advocating the potentially harmful policy or action.157
The standard of “severe or irreversible harm” is a very high bar; yet one CWD has long surpassed regarding public wildlife. It is only against that backdrop that the potential transference of CWD to people can be reasonably considered. We must consider risk, consequences, and even worst case scenarios. The fact is that prion diseases are described by physicians and victim’s families as aggressive, horrific, and dreadful.
Faced, in his medical practice, with the reality of human prion and neurodegenerative diseases, a leading scientists like Dr. Neil Cashman, (former) Scientific Director, PrioNet Canada, have long warned that CWD is “an emergency in slow motion.” At the “On The Horizon” PrioNet Research Conference Dr. Cashman summarized the background and urgency as follows:
“CWD is spreading like wildfire. From a few foci in Saskatchewan, it has now come to involve deer and elk in Alberta and Saskatchewan and there are no geographical barriers. It will spread until it infects the entire continent. It also spreads across species. …It can persist in water; it can persist in soil. It’s spreading without check. It’s arguably the most contagious prion disease, and the human health impact is unknown. We just frankly do not know if humans are susceptible to chronic wasting disease. It’s an emergency in slow motion.”158
This combination of growth, spread, changing risk, and extreme consequence explains the near unanimity of caution in available zoonotic analyses: “Although the zoonotic potential of CWD is considered low, identification of multiple CWD strains and the potential for agent evolution upon serial passage hinders a definitive conclusion.”159
Assessing zoonotic risk of new, emerging, and especially fatal diseases, is challenged by the inability to experimentally test susceptibility in people. Indeed, the ethical challenges are formidable enough regarding potential treatments. Yet questions of susceptibility require new approaches combining epidemiological and laboratory analyses (both in vitro and in vivo), as well as considerations of known and probable human exposure. Questions of appropriate policy and regulatory responses must be weighed against all implied consequences (biological, social, and economic), and the entire range of outcomes. This must include potential worst case scenarios even if they are thought extremely unlikely, not just because of evolving risk, but because market, media, and societal responses are often based more on perception than on science or reality.
Experts weigh in
Given their own and the risk analyses of others, leading scientists are expressing concern: “The increasing levels of CWD exposure are highly concerning.” “As a matter of policy, I believe all animals taken from CWD-infected areas should be tested before consumption and people should definitely not be consuming any infected material.”181
Qingzhong Kong, PhD, Case Western Reserve University
“The CWD situation and increasing levels of CWD exposure is a concern for cervid and human public health.” “CWD-testing should be conducted on animals harvested from CWD-infected areas prior to consumption.”182
Candace Mathiason, PhD, Colorado State University
“ … The more opportunity to expose humans to this stuff, the more we’re potentially playing with fire, in terms of these strain adaptations. It wouldn’t take very many cases of human prion disease that were linked back to chronic wasting disease, to where this whole conversation could change, fairly dramatically, and pretty much overnight. So I think while we have the opportunity, to get out in front of this … where we can as best we can, we should probably take advantage of that.”183
Michael Miller, PhD, CO Division of Wildlife
The full spectrum
The scope of human exposure to CWD is broader than is generally appreciated. It includes some direct exposures that have been largely ignored, lessons from history notwithstanding. When early suspicions of BSE being spread through consumption of blood, bone, and nerve tissues were confirmed in 1988,184 it led to bans on feeding meat and bone meal (MBM) supplements.185 Yet the potential risk of CWD in velvet antlers (i.e., blood, bone, and nerve tissue) sold for human consumption continued to be ignored long after both the confirmation of BSE being transferred to people as vCJD,186 and the repeated findings of CWD on game farms.187
A Risk Assessment of TSE products (dated June 2000) undertaken for Health Canada identified “pharmaceutical products containing high risk tissues and elk antler velvet food supplement” as the highest ranking risks.188 Under public pressure the Canadian Food Inspection Agency pledged in October 2000 to destroy velvet antler from CWD-infected animals. However, no recalls nor warnings to potential consumers have ever been issued.189 The difficulty of recalling product widely distributed throughout Asia is accepted; but it also illustrates the challenge and the consequences of failing to detecting potential zoonotic transfer. Furthermore, this passive approach regarding velvet antler continues even after confirmation of PrPd in velvet antler in 2009.190
As evident from challenges in achieving a CWD vaccine for cervids, there is little hope that breakthrough treatments would soon emerge. CWD has been in the shadows; but the toll of other protein misfolding diseases in people (Alzheimer’s, Parkinson’s, Huntington’s, ALS, CreutzfeldtJakob, etc.) affects tens of millions of Americans and cost hundreds of $billions per year. Yet the scale of complexity and level of difficulty is such, that, even after decades of research, there are no cures, and few effective treatments for any of them.191 Moreover, and as with any disease, containing and managing risk of CWD will demand an understanding and acceptance of the relentless capacity of this disease, as it continues to grow, spread, persist, and evolve. “Prions are distinguished from other amyloid diseases both by their infectious character and the observed exponential growth of infectious material.”192
With its growth and spread, human exposure from all sources has been increasing exponentially, and we would do well to consider the implications of both known and unknown factors. For example, prion load has been shown to be relevant, but note the title of McLean and Fryer’s 2011 work “There is No Safe Dose of Prions.” Analysis of 4,338 mice showed “that infection is possible at the very low dose of a 1000-fold dilution of the dose that infects half the challenged animals (ID50).” 193
After pointing out that bank voles (which are circumpolar) are described as the ‘universal acceptor for prions,’ Sigurdson asks if the sequence in the human ?2-?2 loop creates a permissive host PrPC sequence that is converted by prions from other species, despite sequence mismatches.194 With multiple avenues of direct and indirect human exposure, potential bioaccumulation, the potential role of co-factors, stressors, and potentially consequential passage to or through intermediate species, caution remains prudent. Moreover, it’s becoming clear that our understanding will be well served by looking beyond mammals. Quite apart from the work documenting mammalian prions taken up or adhering to plants, Susan Lindquist’s team has recently shown the “first protein from the plant kingdom with bona fide prion attributes.”195
Science, our greatest ally
These analyses outline more than risk: they offer hope. Lindquist has long been at the front of breakthrough prion research with yeast, and has not only documented many collaborative interactions, but key evolutionary and epigenetic analyses to help explain the phenotypic benefits that have conserved prion existence for 800 million years. These and other insights 196 into prion function may well open opportunities to prevent, limit, or potentially even reverse prion disease.197
However hopeful those breakthroughs might be, they are distant, and the levels of human exposure to CWD are already into the UK’s range of 1,000—10,000 BSEinfected carcasses sufficient to result in BSE transferring to a person.198 North American hunter families are consuming some 7,000—15,000 CWD-infected animals per year, and the number is growing exponentially.199 Though deer are much smaller in mass than cattle, this is more than offset by the fact that CWD prions are spread far more broadly than BSE prions in tissues most likely to be consumed.200 Prion load per animal may thus be higher in deer, despite the difference in mass. And while retail markets for beef reach more genetically susceptible consumers, that is less comforting when considering that the entire deer is often consumed by one hunter family.
Overall, the zoonotic risk profile of CWD is complex, uncertain and evolving. Without exception, dozens of experts consulted for this work concur with the current Director, Prion Diseases Program for the Public Health Agency of Canada, Dr. Michael Coulthart, who describes the risk of CWD transferring to people as “far from negligible.”201
Implications are broad, deep, and longterm
What is clear to policy analysts is that even a single transfer of CWD to a person will carry catastrophic implications in reactions of the public, in markets, and in public policy and international trade, regardless of how the disease manifests. And we cannot ignore the reality that CWD is highly contagious in deer.202
It is not inconceivable that a possible transfer to people could result in similar prion shedding in urine, feces, and saliva. Such an occurrence is beyond any known, practical means of containment or treatment, or avenues to curtail the economic fallout. If less dramatic, the risk profile is broad, complex, and with potentially dire outcomes at every turn. As to one minor example: consider the risk of CWD transfer through pet food. That was included in the UK’s updated, March 2016 Qualitative Risk Assessment regarding chronic wasting disease being introduced into Great Britain.203 The assessment asks: What is the risk of CWD being introduced into Great Britain (GB) from North America and causing infection in deer?
The analysis focuses on three routes of potential CWD introduction:
- importation of animal feed
- importation of deer urine lures
- importation of CWD prion on contaminated equipment and clothing/footwear of hunters or other tourists and British servicemen
The assessment cites the European Union Trade Control and Expert System (TRACES), which confirmed that “in November and December 2015, for example, GB imported 13.6112 tonnes of processed cat and dog food (including dog chews) containing products of ungulate origin from Canada and USA.”
The UK Assessment points out that while the U.S. Food and Drug Administration (FDA) recommends that CWD positive deer or elk, or even such considered at risk, may not enter the animal feed system, it is only a recommendation. They therefore conclude that CWD risk material “may constitute a small percentage of the very low tonnage of non-fish origin processed animal proteins imported from the U.S. into GB.” therefore considers that “there is a greater than negligible risk that (nonruminant) animal feed and pet food containing deer and/or elk protein is imported into GB.” (Emphasis in the original.)
As more information becomes known, and major market players get involved, that status quo is unlikely to stay. Pet food regularly includes a variety of rendered animals and animal parts, including road kills. Sept. 15, 2003 the FDA issued guidance that “Material from CWD positive deer and elk may not be used in any animal feed.” That may have seemed a solid precautionary measure, however, Dr. Dave Clausen (from the CWD positive state of Wisconsin) explained that the results soon turned perverse: “Since results of CWD tests would typically take a few days or weeks, renderers in the CWD areas cite this section as the reason to not accept any deer carcasses that have been tested for CWD. Fear was that a positive test would compromise their interest and/or shut down operation. Thus renderers will take untested carcasses just not tested ones.”204
Then, with CWD continuing to extend its range to 24 states and 2 provinces, the FDA recently took further steps, adding a section to cover: “deer and elk considered at high risk for CWD”205 But as the UK Assessment pointed out, this is mere guidance, and does not establish legally enforceable responsibilities.206 Moreover, FDA rules and guidelines for ensuring composition requirements, compliance, monitoring and enforcement are weak, and interstate and international transport of pet foods is widespread and poorly regulated.207
Given their experience with BSE, the UK assessment of risk via pet food is somewhat surprising. Their experience included the world’s first documented instance of interspecies transfer of prion diseases in 1990, when a house cat developed scrapie-like skin irritation so fierce he licked himself bare. Dubbed “Mad Max” by the British press, the cat died from Feline Spongiform Encephalopathy (FSE), a toll eventually reaching 89 domestic cats in UK, one in Northern Ireland, one in Norway, one in Switzerland, and one in Liechtenstein.
Virtually all species of large cats in zoos were similarly infected, including: five cheetahs, three pumas, three ocelots, three tigers, five lions, and one Asian Leopard Cat.208 All were instances of simple oral ingestion of BSEcontaminated feed. The news of transfer across species barriers immediately rocked public confidence and affected markets, which were damaged further with the subsequent admission that people were dying of vCJD from consuming infected beef.209
The UK assessment outlines a similarly “greater than negligible” risk of importation of CWD prion on contaminated equipment and clothing/footwear of hunters, and that “the annual risk of at least one infection of deer in the UK with CWD from deer urine lures imported from the USA is medium.” But whereas official assessments of CWD threats to European wildlife have been measured, largely unseen, and potentially understated, concerns raised by NGOs and the media have been blunt, as in an article in The Times headlined: “Disease from the U.S. could wipe out all the deer in Britain.”210
Any news of international transfer of CWD by any means, to any species whether deer, rodents, pets, or livestock (such as domestic sheep), would almost certainly have severe consequences. The economic implications would be felt most acutely in North American CWD affected areas.
The UK experience with vCJD is instructive, but key differences are noteworthy. Despite recent confirmation of PrPd in saliva of BSE cattle,211 the absence of (efficient) lateral transfer of BSE between living animals limited growth and spread of the disease,212 and it allowed the ruminant feed ban to eventually halt both the epidemic and the subsequent trade embargo. This underscores a contrast of some significance: CWD with its prolific prion shedding in saliva, feces and urine, is a highly contagious, extremely persistent disease, which has established unprecedented reservoirs in public wildlife and the environment. Compared to BSE, CWD offers no apparent ‘off switch.’
Lessons from BSE
From a public policy perspective, the experience with BSE offers vital lessons—from the foundational frame to the conclusions and recommendations by the official inquiry (paraphrased for brevity):
- “At the heart of the BSE story lie questions of how to handle hazard — a known hazard to cattle and an unknown hazard to humans.”
- “BSE developed into an epidemic as a consequence of intensive farming practice(s) … unchallenged over decades, (that) proved a recipe for disaster.”
- “Government was preoccupied with preventing an alarmist over-reaction … (they) believed that the risk was remote. It is now clear that this campaign of reassurance was a mistake.”
- “Public was repeatedly reassured that it was safe to eat beef.” 213
- “Repeated statements that ‘there is no evidence that BSE is transmissible to humans’ does not explain that such evidence would take many years to emerge.”214
- “Even when risk to humans seems remote, all reasonable precautions must be taken.”
- “There should be more checks on possible pathways of transmission, and on occupational risks.”215
- “Where there is uncertainty, government must not shrink from saying “we are not sure.”216
The analyses and recommendations of the UK experience with BSE are founded on failures of governments to uphold public trust and the precautionary principle. The lessons are directly applicable to CWD: Without immediate, science-based intervention by North American governments to contain and limit the spread, growth, evolution, and exposure of CWD, the likelihood of ‘worstcase’ outcomes will continue to increase, and wildlife is in the cross hairs in every scenario.
Monday 15th May 2017
CATTLE IN SPAIN WITH MAD COW DISEASE
On the 28th April 2017 it was confirmed in that cow from Cantabria, Spain born in 2002 (aged 15 years old) was diagnosed with BSE. See report below from the Spanish Government.
Over 50 other animals in the same herd are at risk. The Spanish authorities state that it ‘poses no risk to human health?’ I am afraid that cattle do not develop BSE in isolation, what about the fields these animals have been grazing on? What about the milk and butter that has already gone into the human food chain? Also what about the meat from other animals in the herd which is now in supermarkets and shops?
This diseased cow was 15 years old how long had it been ill?
Human BSE remains a deadly risk for us all and this new case of BSE in Spain, highlights that the disease has not gone away and continues to affect cattle globally and Human BSE continues to kill people.
As the report says below the origins are unknown, so how many other cattle in the area may be suffering from BSE but not showing symptoms when they enter the slaughterhouses?
The report below ends ;THIS EVENT IS RESOLVED’ ??? HOW CAN IT BE RESOLVED BY THE DESTRUCTION OF ONE COW, WHEN 50 PLUS ARE AT RISK AND THE REST OF HERD MAY ALSO BEEN INFECTED.
— SPAIN OIE Bovine Spongiform Encephalopathy atypical L-type Camargo, CANTABRIA
Subject: SPAIN OIE Bovine Spongiform Encephalopathy atypical L-type Camargo, CANTABRIA
|Bovine spongiform encephalopathy , Spain|
Information received on 12/05/2017 from Dr Valentín Almansa, Director General, Sanidad de la Produccion Agraria, Ministerio de Agricultura, Alimentación y Medio Ambiente, Madrid, Spain
|Source of the outbreak(s) or origin of infection||
|Epidemiological comments||On April 28th, 2017, the Central Veterinary Laboratory at Algete (National Reference Laboratory for transmissible spongiform encephalopathies accredited under UNE-EN ISO/IEC 17025:2005) received a brainstem sample suspected to be BSE-positive from the regional accredited animal health laboratory of Cantabria (official regional laboratory) after a positive result was obtained through the Bio-Rad TeSeE SAP rapid test.
The National Reference Laboratory undertook the confirmatory tests authorized according to Regulation (EU) No. 1148/2014. The selected assays associated were Western blot (Prionics) and ELISA (TeSeE SAP Bio-Rad). Following positive results to both assays, the National Reference Laboratory performed assays to discriminate the BSE strains by immunoblotting with results for atypical BSE type L on May 5th, 2017.
The sample was taken within the national TSE surveillance program (sampling of dead or non-slaughtered animals for human consumption over 48 months old). The animal was a crossbred (conjunto mestizo) female born on 25 February 2002.
|Measures to be applied||
Diagnostic test results
|Laboratory name and type||Central Veterinary Laboratory, Algete ( National laboratory )|
|Tests and results||
|The event is resolved. No more reports will be submitted.|
WEDNESDAY 19th April 2017
BRAZIL FACES 63 INDICTMENTS FOR YEARS OF UNSAFE TOXIC MEAT FLOODING GLOBAL MARKETS
Huge corruption within Brazils Ministry of Agriculture has meant toxic lethal meat has flooded the global food market for years. Federal Auditors for the Brazilian Government and Ministry of Agriculture at meat processing plants took bribes for years in exchange for false health and safety permits for beef and poultry and domestic animals for human consumption.
63 indictments are being prepared by Brazil’s Federal Police force.
Across Brazil people continue to die of vcjd. These cases of human BSE are all pushed under the carpet by the South American authorities.
Brazil is one of the biggest exporters of meat globally and this corruption means that even more animals w infected with BSE would have also flooded the human food chain for years. JBS the biggest meat packer in the world had one of its staff in Brazil suspended over his involvement with members of the Ministry of Agriculture.
MOY PARK. EUROPES LARGEST POULTRY PROCESSOR WITH 12,000 STAFF ACROSS UK, FRANCE AND NETHERLANDS WAS BOUGHT BY JBS FOR £944 MILLION IN JUNE 2015.
POULTRY GO TO SLAUGHTER BEFORE THEY CAN SHOW SYMPTOMS OF ANY DISEASE INCLUDING THE EQUIVALENT OF BSE.
Mad cow continues to kill people. Food corporations and governments globally profits grow to billions of pounds whilst, thousands of people, young men and women, mums, dads, sisters, grandparents, brothers and children continue to die or suffer the long term lethal effects of the human form of mad cow disease.
Brazil Meat Scandal: 63 Indictments
By Greg Henderson April 17, 2017 | 3:07 pm
Federal Police in Brazil has indicted 63 people for their role in a vast corruption scheme within the Ministry of Agriculture. The charges allege federal auditors at meat processing facilities took bribes for years in exchange for fraudulent sanitary permits.
The probe into Brazil’s meat corruption was launched March 17, 2017, by Brazil’s Federal Police. Brazil, the world’s largest beef and poultry, and the fourth largest exporter of pork, saw its exports drop to near zero within a week of the scandal’s announcement, though most export sales have resumed.
The suspects in the case are charged with falsifying medical records and certificates, tampering with food products, conspiracy and corruption. One employee at a JBS processing plant in Brazil was included in the investigation, allegedly due to his relationship with federal inspectors. The employee was suspended.
Allegations also include selling spoiled meat and injecting water to sell poultry at higher prices. Police also reported that the producers under investigation used ascorbic acid and other chemical ingredients, in quantities far above the legally permitted amount, in order to “disguise the physical aspect or smell of rotten meat.”
No JBS brands or products are associated with product tampering nor has JBS been accused of selling tainted or rotten meat. The majority of this investigation, particularly the food quality issues, focused on the actions of smaller companies in Brazil that supply the domestic market, not JBS.
JBS is the largest meat packer in the world, with subsidiary JBS USA the second largest beef packer in the U.S., with nine beef plants and estimated 2016 sales of $14 billion. JBS USA is not involved in the Brazilian scandal. JBS USA is a “leading processor of beef, pork and lamb in the U.S., a leading processor of beef in Canada and the largest cattle feeder in the world.”
Monday 13th March 2017
A cow born in 2003, over 14 years old has been diagnosed with BSE in Spain March 2017, ( see news links below) this cow produced many calves all of which would have entered either the human food or medicine chain for over a decade.These calves would have been used for milk, butter, meat and in some cases their cells/tissue used for vaccines and blood products. The cow with BSE milk would also have been sold to supermarkets and shops.
There is now a so called ATYPICAL BSE WHICH AUTHORITIES ACROSS THE GLOBE ARE USING TO FUDGE THE TRUE NUMBER OF CATTLE DYING OF MAD COW DISEASE. The authorities falsely reassure the public that ATYPICAL BSE is almost a natural disease in the same way that SPORADIC CJD IS IN HUMANS. Both are falsehoods, dodgy accounting by governments to keep the true numbers of BSE cases in cattle lower and deaths of vcjd the human form of mad cow disease lower in humans.
Before BSE there was no such thing as ATYPICAL BSE IN COWS. This new title for BSE is used to protect the global food and pharmaceutical industries and the farmer in Spain who for over a decade had been selling milk from a cow with BSE, selling the cows offspring for meat and medical use. All of these animal products would have entered the human food and medicine chain.
Its a crude ‘damage limitation’ by the authorities in Spain saying the cow had Atypical BSE. (which means it was an odd case and old before BSE was diagnosed) hoping that the public will believe that ATYPICAL means ‘safe’. Unproven science to falsely reassure the public that beef from this BSE cow from this farm in Spain was and is ‘safe for human consumption’. BSE is lethal to humans and animals, sometimes animals or humans have long incubation periods before they become ill, but they are still toxic to other animals or humans.
This cow which was 14 years old in Spain was found to have BSE and that is a lethal risk to human health. This cow also developed BSE in the same region where a few years ago a mother aged 60 and her son in his 40’s died of vcjd. There have also been other human cases of vcjd in the area of Spain where this cow was recorded as having BSE.
The global cover-up regarding animal BSE and human mad cow disease continues!
ABC NEWS AND OTHER MEDIA REPORTS.
Wednesday 1st March 2017
Had two brilliant radio interviews this week, one with presenter Cheryl Fergus-Ferrell at Croydon Radio, London and another with Pippa Jones at TalkRadio Europe which was broadcast across Spain. Both were extended items, talking about my Andrew the campaign and documentary ‘Cash. cows and cover-ups’. See link below to the podcast Croydon Radio.
Monday 13th February 2017
We have released a trailer of our shocking new one hour indpendent documentary,
’ Cash, cows and cover-ups’
Please share the link below. I will let you know when the documentary in full is being broadcast. I have worked for over 18 months researching and filming the documentary and have uncovered some shocking new facts and information that the UK government and other establishments wanted to keep secret. Partickular Films are the great production company which has filmed, edited, and directed this great film. It has been produced with passion, professionalism and facts, with many world exclusive revealed. Director Joseph Andrew Mclean, cameramen Martin Heron, Dale McEwan and a great crew in the USA.
We have filmed across the UK and in the USA and the documentary is for release to a national and international audience.
This campaign and film is not just about the unlawful death of my son Andrew and all victims of human BSE but about you and your families. I want to know that the food that children and families in 2017 are eating and the medicines they are ingesting are safe and not a poisoned chalice. Sadly our governments and the global food companies care little for animal or human health with profits the driving force.
If you care about the food you eat, the scientists you put your trust in and the politicians you vote for please watch the trailer and support the documentary when its broadcast.
This concerns everyone, I want to make sure your children are safe, your friends, family and you do not become infected with BSE, do not become disabled or die through human mad cow disease.
‘cash, cows and cover-ups’ and justice4andy campaign is about keeping your family safe too!
The trailer is also on YouTube
The link is now on Vimeo: https://vimeo.com/203115042
Monday 6th February 2017
This evening I am appearing on BBC1 ‘INSIDE OUT’ at 7.30pm its a shocking investigation into diseases that can be transmitted from domestic animals to humans. How the UK government in 2017 continue to cut funding regarding testing farm animals for disease and its on-going effect on food safety.
Below is latest research which highlights the on going ticking time-bomb which is human BSE and how millions remain at risk of dying of the human form of mad cow disease.
With this blog is photo of me filming in snowy countryside on a farm. I talk about BSE and my fight to protect the UK and global public from the government/ corporate greed and corruption that unlawfully killed my son Andrew and how BSE will continue to kill people for generations. I also discuss other zoonotic diseases which will flourish all the time government policy condones profit before human health.
Many more people could still die from mad cow disease in the UK
Wednesday 1st February 2017
Roger Hiorns Turner nominated artist has an exhibition of his work at the world famous IKON gallery in Birmingham, UK.
The show is on for 3 months December 2016- March 2017 and includes items from www.justice4andy.com campaign and also Roger’s art expressing the tragedy and corruption surrounding the BSE epidemic and its fatal human consequences. Please go along if you are in the area.
Photo with this blog is of Christine and Roger at his show. My book ‘Who killed my son?’ is also available at the IKONs book store and their catalogue. See link below.
Monday January 30th 2017
This footage on Youtube (see below) of a cow staggering and dying of BSE in Belgium , highlights the ticking health-time bomb that is mad cow disease. It has not gone away and is endemic in cattle. With the closing of animal autopsy facilities in England and the UK no longer testing for BSE. How many hundreds of cattle here in the UK and globally are still entering the human food chain that are infected with mad cow disease?
The footage of the cow unable to walk due to deadly mad cow disease and the Belgium officials off-hand response that:
‘Only one cow has BSE’ and that it’s therefore safe and ok for Belgium families and their children to consume bovine material is false. Totally appalling that profit before lives always dominates governments agendas.
BSE in cattle is never isolated to just one case. What about the cows that had already gone to slaughter and not showing symptoms? they would be toxic and infectious too!
How many cows in the Belgium herd were incubating or carrying mad cow disease and entered the human food and medicine chain?
Of course people who consume this toxic material will not show symptoms for many years, by then the farm may well be owned by another farmer and the officials who say eating beef is safe moved on to another post.
All of the cattle in the affected herd should have been immediately destroyed including calves and parents of the cows.
Also all meat and milk that had been sent from the farm should have beentraced and consumers informed.
The culture of global ministerial and official secrecy which killed my son Andrew and hundreds innocent victims, disabled thousands and put millions at risk continues to condone and hide the truth/ facts about BSE and its human on-going deadly consequence vcjd
Photos with this blog of me and my son Andrew aged 24 at Southampton Hospital the day he was diagnosed with human mad cow disease, and 6 months before he died of human BSE. The other photo taken just a short while before of Andrew healthy and well in San Francisco USA.