Mum wants justice after CJD cover up
Campaigner whose son died of human form of mad cow disease to lobby MLAs
By Joe Oliver Sunday, 11 January 2009
CJD victim Jonathan Simms and his father Don.
A campaigning mum is set to visit Northern Ireland in her quest for justice after the devastating death of her son from the human form of ‘mad cow’ disease.
Freelance journalist Christine Lord is planning to address members of the Assembly and family groups — as fears grow over a new wave of the killer brain bug.
She also hopes to set up a private meeting with the family of west Belfast man Jonathan Simms — the world’s longest-known survivor of variant Creutzfeldt-Jakob Disease (vCJD).
At 24, Jonathan is the same age as her son Andrew who died just over a year ago.
Since then — and following Andrew’s dying request — Christine has campaigned relentlessly to expose the key players behind the BSE scandal and the horror of vCJD.
Christine, from Southsea, Hants, recently petitioned Prime Minister Gordon Brown in Downing Street to release confidential documents about Government policy regarding BSE.
She told Sunday Life: “The cover up has gone on too long — it’s time we had justice. My beautiful, gentle boy was reduced to a shell. He had a terrible and distressing death.
“He was blind, deaf, quadriplegic and unable to recognise or remember anything or anyone. If the Government and senior officials had not ignored evidence and withheld crucial information, then all the victims of vCJD, including my son, would still be alive.”
Christine has already been in touch with former first minister Ian Paisley and was overwhelmed by the letters of support she received from Northern Ireland since telling her harrowing story in a BBC documentary.
“I wrote to Dr Paisley because I was aware that one of the victims of vCJD in Northern Ireland was a member of his party,” she said.
Andrew Hunter, who was just 27, was a DUP member of Newtownabbey Borough Council and died from the disease at his home in Rathcoole in April 2002.
The first person from the province to contract vCJD was 30-year-old Maurice Callaghan from west Belfast. He died in 1995, leaving a wife who was pregnant with their second child, and a daughter.
The first wave of vCJD, caused by eating infected beef products in the 1980s and early 1990s, has been responsible for 164 deaths.
All victims belonged to a gene type known as MM. Clinical tests suggest that new sufferers have an MV gene type, although this cannot be confirmed until a brain biopsy is carried out after death.
But the possibility has raised concerns that the illness may have a longer incubation period.
Christine does not know how Andrew contracted vCJD, but revealed that she stopped giving her children meat when the first fears where raised.
She believes he may have been carrying it in his system before 1994, although he showed no symptoms until the end of 2006 when he was initially told he was “depressed”.
Her daughter Emma (18) has shown no sign of infection.
“The disease affects predominantly young people in their teens and twenties,” she said.
“Its agent, BSE, was given free rein again and again to enter the food chain, wreck lives, families, careers, homes and futures. There was a wealth of scientific knowledge that BSE was harmful to humans, but they still allowed the most toxic materials into the food chain to be ingested by infants and children.
“They did nothing to stop the threat to school meals, baby food and the infected serum in childhood immunisations.”
Christine decided against giving her son pentosan polysulphate — the blood-thinning and anti-inflammatory therapy previously only tested on animals.
“I know the Simms family in Belfast had to fight a battle in the courts to get it for their son and I am genuinely delighted to hear the consensus that he is no longer terminally ill,” she said.
“But it was something I discussed with my son and one of the reasons I would very much like to meet the Simms family.
“When I’m in Northern Ireland I want to meet the political parties across the whole spectrum and, of course, I want support for my petition which already has thousands of signatures.”
She added: “My son’s death was totally avoidable.
“Those in the Conservative government at the time and their advisors knew at the earliest stages that BSE had severe implications for human life.
“They held the smoking gun that killed my son and many others. I want them publicly and legally held accountable.
“That way I can finally win justice for Andrew and all victims of vCJD past, present and, tragically, those still to come.”
The Government’s chief adviser on vCJD warned recently there could be a second wave of deaths over the coming years involving anything between 50 and 350 people.
l To visit Christine’s website click onto www.justiceforandy.com
JUSTICE FOR ALL TSE VICTIMS !!!
Greetings to ALL human CJD/TSE victims and to their families,
THERE is more to this story than the UKBSEnvCJD adolescents only theory of CJD, and the rest i.e. 85%+ are just a happenstance of bad luck, or simply a flipped out protein. while i applaud Christine Lord's campaign to expose the key players behind the BSE scandal and the horror of vCJD, sadly, we are only speaking of a very small part, of a much larger global problem with human and animal TSEs, and by ignoring these other TSEs, you are only allowing this agent to spread through a multitude of proven routes and sources. Some of us have been fighting for over a decade to expose a much larger global cover-up of all strains of human and animal TSE, especially, the sporadic CJD's. sporadic CJD is not a single strain, but a multitude of many yet unknown strains, another of which was discovered (or announced) last year in 2008 here in the USA.
IT'S called STRAINS !
Sunday, December 28, 2008
MAD COW DISEASE USA DECEMBER 28, 2008 an 8 year review of a failed and flawed policy
8 YEARS IN REVIEW OF THE MAD COW DEBACLE IN THE USA ;
Tuesday, January 06, 2009
CWD Update 93 December 29, 2008
Monday, December 1, 2008 When Atypical Scrapie cross species barriers
USA PRION UNIT BLOG
Sunday, April 20, 2008 Progress Report from the National Prion Disease Pathology Surveillance Center April 3, 2008
Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.
see full text ;
Friday, December 12, 2008
The prion strain phenomenon: Molecular basis and unprecedented features
Wednesday, December 10, 2008
Evaluation of FSIS Management Controls Over Pre-Slaughter Activities (Audit Report 24601-7-KC) USDA CERTIFIED DEADSTOCK DOWNER COW SCHOOL LUNCH PROGRAM
Wednesday, August 20, 2008 Bovine Spongiform Encephalopathy Mad Cow Disease typical and atypical strains, was there a cover-up ?
A New Prionopathy OR more of the same old BSe and sporadic CJD
Communicated by: Terry S. Singeltary Sr.
[In submitting these data, Terry S. Singeltary Sr. draws attention to the steady increase in the "type unknown" category, which, according to their definition, comprises cases in which vCJD could be excluded. The total of 26 cases for the current year (2007) is disturbing, possibly symptomatic of the circulation of novel agents. Characterization of these agents should be given a high priority. - Mod.CP]
There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.
He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.
sporadic Fatal Familial Insomnia
THE PATHOLOGICAL PROTEIN
Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9
BY Philip Yam
CHAPTER 14 LAYING ODDS
Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.
The Lancet Infectious Diseases, Volume 3, Issue 8, Page 463, August 2003
doi:10.1016/S1473-3099(03)00715-1Cite or Link Using DOI
Tracking spongiform encephalopathies in North America
Original TextXavier Bosch
"My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem."
49-year-old Singeltary is one of a number of people who have remained largely unsatisfied after being told that a close relative died from a rapidly progressive dementia compatible with spontaneous Creutzfeldt-Jakob disease (CJD). So he decided to gather hundreds of documents on transmissible spongiform encephalopathies (TSE) and realized that if Britons could get variant CJD from bovine spongiform encephalopathy (BSE), Americans might get a similar disorder from chronic wasting disease (CWD)the relative of mad cow disease seen among deer and elk in the USA. Although his feverish search did not lead him to the smoking gun linking CWD to a similar disease in North American people, it did uncover a largely disappointing situation.
Singeltary was greatly demoralized at the few attempts to monitor the occurrence of CJD and CWD in the USA. Only a few states have made CJD reportable. Human and animal TSEs should be reportable nationwide and internationally, he complained in a letter to the Journal of the American Medical Association (JAMA 2003; 285: 733). I hope that the CDC does not continue to expect us to still believe that the 85% plus of all CJD cases which are sporadic are all spontaneous, without route or source.
Until recently, CWD was thought to be confined to the wild in a small region in Colorado. But since early 2002, it has been reported in other areas, including Wisconsin, South Dakota, and the Canadian province of Saskatchewan. Indeed, the occurrence of CWD in states that were not endemic previously increased concern about a widespread outbreak and possible transmission to people and cattle.
To date, experimental studies have proven that the CWD agent can be transmitted to cattle by intracerebral inoculation and that it can cross the mucous membranes of the digestive tract to initiate infection in lymphoid tissue before invasion of the central nervous system. Yet the plausibility of CWD spreading to people has remained elusive.
Part of the problem seems to stem from the US surveillance system. CJD is only reported in those areas known to be endemic foci of CWD. Moreover, US authorities have been criticized for not having performed enough prionic tests in farm deer and elk.
Although in November last year the US Food and Drug Administration issued a directive to state public-health and agriculture officials prohibiting material from CWD-positive animals from being used as an ingredient in feed for any animal species, epidemiological control and research in the USA has been quite different from the situation in the UK and Europe regarding BSE.
Getting data on TSEs in the USA from the government is like pulling teeth, Singeltary argues. You get it when they want you to have it, and only what they want you to have.
Norman Foster, director of the Cognitive Disorders Clinic at the University of Michigan (Ann Arbor, MI, USA), says that current surveillance of prion disease in people in the USA is inadequate to detect whether CWD is occurring in human beings; adding that, the cases that we know about are reassuring, because they do not suggest the appearance of a new variant of CJD in the USA or atypical features in patients that might be exposed to CWD. However, until we establish a system that identifies and analyses a high proportion of suspected prion disease cases we will not know for sure. The USA should develop a system modeled on that established in the UK, he points out.
Ali Samii, a neurologist at Seattle VA Medical Center who recently reported the cases of three hunterstwo of whom were friendswho died from pathologically confirmed CJD, says that at present there are insufficient data to claim transmission of CWD into humans; adding that [only] by asking [the questions of venison consumption and deer/elk hunting] in every case can we collect suspect cases and look into the plausibility of transmission further. Samii argues that by making both doctors and hunters more aware of the possibility of prions spreading through eating venison, doctors treating hunters with dementia can consider a possible prion disease, and doctors treating CJD patients will know to ask whether they ate venison.
CDC spokesman Ermias Belay says that the CDC will not be investigating the [Samii] cases because there is no evidence that the men ate CWD-infected meat. He notes that although the likelihood of CWD jumping the species barrier to infect humans cannot be ruled out 100% and that [we] cannot be 100% sure that CWD does not exist in humans& the data seeking evidence of CWD transmission to humans have been very limited.
he complained in a letter to the Journal of the American Medical Association (JAMA 2003; 285: 733). I hope that the CDC does not continue to expect us to still believe that the 85% plus of all CJD cases which are sporadic are all spontaneous, without route or source. < < <
actually, that quote was from a more recent article in the Journal of Neurology (see below), not the JAMA article. ...TSS
JOURNAL OF NEUROLOGY
MARCH 26, 2003
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States
Email Terry S. Singeltary:
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  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?
Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.
Terry S. Singeltary, Sr Bacliff, Tex
1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323. FREE FULL TEXT
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.
Creutzfeldt Jakob Disease
CJD TEXAS (cjd clusters)
USA WRITTEN CJD QUESTIONNAIRE ???
The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.
Attending Dr.: Date / Time Admitted : 12/14/97 1228
UTMB University of Texas Medical Branch Galveston, Texas 77555-0543 (409) 772-1238 Fax (409) 772-5683 Pathology Report
FINAL AUTOPSY DIAGNOSIS Autopsy' Office (409)772-2858
FINAL AUTOPSY DIAGNOSIS
I. Brain: Creutzfeldt-Jakob disease, Heidenhain variant.
Friday, December 12, 2008
Creutzfeldt-Jakob disease (CJD) update report Emerging Infections/CJD Published on: 12 December 2008
HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory
Sunday, May 18, 2008
MAD COW DISEASE BSE CJD CHILDREN VACCINES
TIP740203/l 0424 CONFIDENTIAL
Monday, May 19, 2008 SPORADIC CJD IN FARMERS, FARMERS WIVES, FROM FARMS WITH BSE HERD AND ABATTOIRS
Sunday, May 18, 2008 BSE, CJD, and Baby foods (the great debate 1999 to 2005)
BSE Inquiry DRAFT FACTUAL ACCOUNTS DFA's
Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518