Prion Disease
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First nvCJD baby feared as surge in cases is forecast
Three further nvCJD case reports from Leicestershire
Pigs with BSE
Wurst case scenario
First confirmed case of BSE in a Danish-born cow
Portugal frets over possible nvCJD case
8th vache folle in France
Swiss give ok to tainted blood
Inquiry statement of mother [#628, Ms Jean Crowe]
Half of US cattle harbor toxic E. coli strain

Tragedy of baby feared born with nvCJD

05 Mar 00 Jonathan Leake, Science Editor Sunday Times London
Terrible legacy: the mother, who has been diagnosed with CJD, and her four-month-old daughter Photograph: Simon Townsley

DOCTORS believe this may be the first baby to be infected with nvCJD, the human form of mad cow disease, by her mother. The woman had a caesarean section four months ago at a west Midlands hospital and has since been found to have the disease.

The hospital has confirmed that other expectant mothers and their babies were also accidentally exposed to nvCJD after contaminated instruments used on the sick woman continued in use on the maternity ward for two months afterwards. The Department of Health has launched an inquiry, but the hospital cannot be named for legal reasons. [Walsgrave Hospital, Coventry -- see 29 Jan 00 newspaper story -- webmaster]

The infected mother was showing many of the early symptoms of vCJD when her child was delivered, but was not properly diagnosed until later.

Doctors now believe that the woman's daughter has contracted the deadly disease. Brain scans indicate that she has characteristic tissue damage. If this is confirmed, it will establish that the disease can be passed from mother to child. The instruments used in the section were sterilised, but the infective agent, known as a prion, has been shown to be invulnerable to conventional sterilisation methods.

CJD experts told the hospital two months ago that up to 10 women were at risk. The director of public health said they had not contacted the women. "There are no tests for nvCJD and no cure. If we told them then they would have 10 or more years of anxiety." He said the authority was considering informing their GPs. [Tests exist but not this early-on. It would be very difficult in any case to say whether an infection came from the instruments or by eating beef. -- webmaster]

Professor John Collinge, head of the Medical Research Council's prion research unit at Imperial College in London, said: "There is no means of sterilising surgical instruments against prions." The infected woman, who is in her twenties, was formally diagnosed only two months ago. The illness has killed 51 people in Britain.

This weekend the woman was severely ill at home. One family member said: "Nobody knew what was wrong with her at the time. We are desperately sorry for all the women and babies who will have to spend years wondering if they are infected and then possibly go through the same tragedy as us."

First CJD baby feared as surge in cases is forecast

Mon, 28 Feb 2000  Sunday Times Jonathan Leake, Science Editor
Doctors are investigating Britain's first suspected CJD baby. The infant, born to a woman who has since been found to have new variant CJD (nvCJD), is thought to stand a high chance of having contracted the disease.

If tests confirm that the baby girl is also ill, it will mark the first case in which the disease has been transmitted from one generation to the next. All 51 people known to have died of the disease are believed to have contracted it by eating beef products made from animals suffering from BSE.

The case has also endangered medical staff attending the baby's birth because the mother's placenta contains high levels of prions, the disease-causing protein agent. The absence of any test means staff could face years of not knowing if they have the disease. All the staff are understood still to be working.

The revelation coincides with a decision by the Department of Health to make formal preparations for a likely surge in CJD cases. It is drawing up national guidelines telling health authorities and hospitals how to care for victims and to take precautions to minimise the risk of further infection. The move comes as the government-appointed BSE inquiry prepares to publish its report at the end of next month.

This weekend, a spokesman at the West Midlands hospital where the girl and her mother are being treated confirmed that intensive medical tests were being carried out on lymph tissue taken from the child's armpit. Other tests have already shown that her brain has been damaged in some way but the cause is uncertain.

"We have sent samples to St Mary's hospital in London, where they are being analysed [by Collinge lab]. We have not yet been informed of the results," said a spokesman. A court injunction means that the girl, her family and the hospital cannot be identified.

If the tests prove positive, it will mean that any woman incubating nvCJD who becomes pregnant could unknowingly transmit it to her children. Since almost everyone in Britain has been exposed to contaminated beef products and the disease is thought to take many years to develop, it could mean that any epidemic would last for generations.

It was maternal transmission that made the BSE epidemic in cows so long-lived. At its peak, more than 20,000 cows a year were found to have the disease but, because many had calved before symptoms showed, the spread was not curbed. Last year, despite beliefs that the epidemic was over, about 3,000 cows were found to have BSE. Undiagnosed cases are highly likely to be still being sent for slaughter. Maternal transmission is also the main means by which sheep transmit scrapie - considered by many experts to be far closer in behaviour to CJD in humans. [There is very little data supporting this view. Horizontal and vertical transmission are difficult to distinguish under field conditions. -- webmaster]

The exact size of any human epidemic in Britain is uncertain because there is as yet no reliable test to find out if people are incubating CJD. It is only in the past year that doctors have even agreed how to diagnose the disease. Professor John Collinge of Imperial College London, head of the Medical Research Council's prion unit, believes "it is logical to conclude that many more cases must result from the dietary exposure".

Last year, Dr Mary Jo Schmerr, chief scientist for prion diseases at the US National Animal Disease Center in Ames, Iowa, devised a test that could detect prions in animals. Doctors at the CJD Surveillance Unit in Edinburgh are now working with her on a new blood test for humans to enable scientists to measure the potential size of the epidemic.

Baby tested for CJD link to mother

THE GUARDIAN, London, Tuesday February 29, 2000
An investigation was under way yesterday into the possibility that a baby girl may have contracted the human form of mad cow disease from her mother. A department of health spokesman confirmed that tests were being carried out and that the possibility of maternal transmission of new-variant CJD was being investigated.

It is understood the baby girl, who has been ill since birth last year, is being treated in a Midlands hospital with her mother, who has been confirmed as suffering from new-variant CJD. The passing of new-variant CJD from mother to baby was previously thought to be very unlikely. [By whom? The placenta has long been known to be a very high titre tissue; maternal inheritance is suspected in scrapie, CWD, and BSE.-- webmaster] The spokesman said: "We understand that all the necessary investigations are under way by the NHS trust involved and the appropriate specialists."

It was reported late last month that a 24-year-old mother of a young baby was in a serious condition in Walsgrave hospital, Coventry, suffering from a suspected case of new variant CJD.

The hospital added that the woman's daughter, aged three months at the time, was also seriously ill, but that it was not thought the child had the same illness. Hospital staff would not comment yesterday on whether the cases were linked.

Opinion (webmaster): A large nvCJD epidemic is impossible to fathom in all its horrific implications for society; political interference with TSE research over the last 15 years is then especially appalling. The article gives a further basis for this concern though details are still sparse.

Agro-economic interests have completely controlled every aspect of this long-running drama, at great cost to the public health. At some point, England needs to transition from public relations damage control over to disease damage control . Releasing the long-delayed tonsil survey would establish the scope of necessary investment in research.

A bogus beef industry press release earlier in the week quoted a unnamed (that is, non-existent) "medical authority" to the effect that:

"Picking up in the French BSE-List, this new from the Press Association (Tuesday February 29, 2000 2:42 pm) This press agency reports that "Baby May Be Clear Of Mother's CJD " It announced that the tests done on the girl indicate she may not have the disease while the mother has been confirmed as suffering from nv CJD Even if there is a lack of tissue do run all tests, a medical authority reported that "the indications are this child doesn't have CJD".

The US FDA proposed contingency plans as far back as 1994 not to allow further American tourism to Britain. This would affect a larger industry than beef exports and goes far beyond mere blood donor exclusion. The travel ban was never implemented and even back then it was too little, too late. While nvCJD has not yet been confirmed in the US to date, there are likely many Americans who are infected and infectious. Single-minded promotion of beef exports has seriously compromised containment of the epidemic and resulted in the unnecessary globalization of both BSE and nvCJD.

Pregnancy and CJD at Medline:

1. Bovine spongiform encephalopathy. Media coverage had shortlived effect on beef consumption by pregnant women.

BMJ. 1996 Jul 20;313(7050):171. 
Wilson S, et al.
2. Creutzfeldt-Jakob disease in a pregnant woman with an implanted dura mater graft.
Neurosurgery 1994 Apr;34(4):737-9; discussion 739-40 
Lane KL, Brown P, Howell DN, Crain BJ, Hulette CM, Burger PC, DeArmond SJ
A 28-year-old woman with prior [Arnold-Chiari] neurosurgery involving the placement of a cadaveric dural graft developed a rapidly progressive neurodegenerative disorder with prominent cerebellar dysfunction that was proven at autopsy to be Creutzfeldt-Jakob disease. She represents the second American to develop Creutzfeldt-Jakob disease in association with a dural graft. The unusual features of the case include the patient's initial clinical presentation with cerebellar ataxia in the absence of dementia, the widespread presence of kuru-type amyloid plaques on a histological examination of the brain, the development of clinical symptoms during pregnancy, and the subsequent delivery of a child who remains healthy at the age of 3 years.

3. [Creutzfeldt-Jakob disease and pregnancy].

Berrebi A, Cohen M, Ayoubi JM
J Gynecol Obstet Biol Reprod (Paris) 1997;26(8):755-9  Article in French 
CHU La Grave, Toulouse
. Four cases of this transmissible spongiform encephalopathy have been described in pregnant women and none of the offspring, currently 22, 10, 7 and 3 years of age, have developed the disease. Vertical transmission of Jacob-Creutzfeldt disease cannot however be excluded as it occurs in animals and since injections of cord blood and placenta extracts of women with this disease into mice brains has proven the presence of the infectious agent. The rarity of Jacob-Creutzfeldt disease in women of reproductive age and the particularly long incubation period of this type of encephalopathy would incite prudence and long-term follow-up to determine outcome in children born from disease women or who developed signs of the disease during the years following delivery.

4. Demonstration of the transmissible agent in tissue from a pregnant woman with Creutzfeldt-Jakob disease.

N Engl J Med 1992 Aug 27;327(9):649 
Tamai Y, Kojima H, Kitajima R, Taguchi F, Ohtani Y, Kawaguchi T, Miura S, Sato M, Ishihara Y
5. Pediatric CJD is reviewed elsewhere.

nvCJD: three case reports from Leicestershire

J Neurol Neurosurg Psychiatry 2000 Mar;68(3):375-378
Allroggen H, Dennis G, Abbott RJ, Pye IF
Since a report in 1996 of 10 cases of Creutzfeldt-Jakob disease (CJD) with onset in a younger than usual age, a pattern of the disease has emerged. This includes early neuropsychiatric features and sensory symptoms and neurological signs such as ataxia and involuntary movements later in the course of the disease.

Three patients with varied clinical presentations and disease course seen at a single neurology unit are described. The first patient was characterised by cognitive and psychiatric symptoms together with neurological signs. The second patient presented with unusual behavioural disturbance and episodes of collapse. The third patient exhibited striking psychomotor retardation and had abnormal CSF and MRI findings. All patients succumbed in a state of akinetic mutism and myoclonus.

All three patients had the methionine/methionine genotype at codon 129 of the PrP gene and in two of the three patients a tonsil biopsy was performed with positive results. These two patients also tested positive for the 14.3.3. protein in the CSF.

Whereas late features of the disease seem very similar in all cases, the initial presentation was variable and underlines the uncertainty of the range of the clinical phenotype. Successful diagnosis demands a high index of clinical suspicion.

Comment (webmaster): It is not clear whether these cases are included in statistics released monthly by the Dept of Health. It seems that some cases are being processed by the Collinge lab, others by regional neurologists, it being uncertain if the Edinburgh CJD Surveillance Unit is fully in the loop.

First confirmed case of BSE in a Danish-born cow

2/28/2000 AP and correspondends
The first confirmed case of BSE in a Danish born cow was announced today. A cow with nervous symptoms was found on a dairy farm in Himmerland, probably some 4 weeks ago. The vet attempted treatment, which gave only a short term improvement, and it was decided to euthanize the cow.

As Denmark (like other EU countries) are obliged to examine a certain number of cows which are found with nervous symptoms for possible BSE, the vet (who did not suspect BSE) did nonetheless refer the head of the cow for further examination. As Danish vets were unable to rule out BSE, the case was referred on to somewhere with more expertise (apparently to the UK). The announcement today came after the results of this more thorough examination had confirmed that the cow had BSE.

A case of mad cow disease was reported Monday in northern Denmark, the government said. The four-year-old cow was immediately slaughtered while the rest of the herd was put under observation. [This makes no sense. The cow would have been slaughtered as part of the confirmation process. Because of horizontal transmission risks and common feed, countries such as France slaughter the entire herd as well. -- webmaster]

"It's totally incomprensible. In Denmark, we have done all that is possible to avoid this," Lennart Korsgaard Nielsen, chairman of the Danish Livestock and Meat Board, said in an interview with public radio. [Denmark may have imported substantial amounts of bone meal during the 1985-1992 era. -- webmaster]

It was the second known case of bovine spongiform encephalopathy, or mad cow disease, detected in this Nordic country. The first one occurred in 1992 when the disease was found on an animal imported from Britain. Government officials were not available for comment before an afternoon news conference, and more details were not immediately available.

The case was detected in a herd in the northern part of the Jutland peninsula, which has the largest cattle population in Denmark with 364,500 heads in 1998.

Panic swept Europe in the late 1980's and early 1990's after British-raised cattle contracted bovine spongiform encephalopathy and it was linked to a brain-wasting disease in humans. The European Union ruled in August that British beef was again safe for import, although France has maintained its ban.

Question (webmaster): What is the history of restrictions on MBM and live imports for Denmark? In regards to transshipment through Belgium and Holland, how much MBM is used locally compared to how much is generated locally? How many cattle are imported each year from somewhere? Is it true that they are not destroying the rest of herd?

Answer (Torsten Brinch): "I can only give a precise answer to your last question: It is not true. It has already been decided to cull the complete herd in which the case was found.

According to my sources, this herd ( "Brodhoejgaard", Skoerping in north Jutland, co-owned by Jens Hyldgaard and his 80 year old father) is a relatively small and particularly closed herd. That is, it has only had homebred animals (indeed for the last 25 years). And as yet, nothing unusual about its feeding history has appeared.

Denmark banned the feeding of ruminant protein to ruminants in June 1990. Since March 1990 no cows or calves have been imported from the UK.

In connection with the recent announcement it has been mentioned that MBM regulations were tightened in 1997 (as a result of EU regulations). This recent BSE case was born 4-5 years ago, i.e. before the 1997 initiative. Not unexpectedly it is now speculated that the herd can have been exposed to cattle feed which has been cross-contaminated with MBM on feed mills which may have produced feed for ruminants as well as for non-ruminants.

What is the likelihood that cows suffering from BSE have been imported and have thus entered into Danish feed production, and what is the likelihood, that contaminated feed has directly been imported and sold here? I don't know the number of imported potentially sick cattle, nor the tonnage of imported, potentially contaminated feed.

"It's totally incomprensible. In Denmark, we have done all that is possible to avoid this," Lennart Korsgaard Nielsen, chairman of the Danish Livestock and Meat Board, said in an interview with public radio.
I think this psychologically well covers the surprise and shock all involved have felt with the announcement. Everyone had thought that 'it can't happen here'. It is also clear that everyone perceives Denmark to have had a history of prompt and swift implementation of any reasonable measure imposed by circumstances and everything possible within EU regulations throughout the BSE history.

In the strictest of terms, everything possible -- in the physical sense-- has of course not been done. We could have banned cows,say, or incinerated all fallen stock, closed our borders for imports of foreign livestock and feed materials, battled any such decision in EU courts endlessly etc.etc.

The reactions to the case in Denmark has not unexpectedly been on two fronts, to adress consumer concerns, and to deal with possible repercussions for trade.

Already tomorrow all beef which may be contain high risk material (e.g. T-bone steaks and other cuts from the dorsal areas) will have been withdrawn from the human food supply in Denmark. Consumers have been instructed on TV to throw away any such cuttings which they have already purchased. A new slaugthering technique must be implemented within one week, such that risk material (nervous tissue, dorsal cord, lymphatic tissue, eyes) will be removed from the human food supply early during the slaugthering process.

This policy has been characterised by some, including by a top leader of a farmers association as draconic and as a gross overreaction. "The meat which is now being withdrawn is from perfectly healthy cattle, which has already been controlled by vets" "This may be only a single isolated sporadic case" "There is only reason to withdraw meat from cattle which has had contact with this particular herd etc. etc. But there seems to be widespread understanding of the necessity of very serious and tough measures among lay cattle farmers, and indeed this has strong support from the beef industry.

It is really common sense, because politically there is no other choice, than to go 'all the way' at once. Noone knows how this single case got the disease. And if it has got it from feed (which is the only plausible explanation), noone can believe that there will be no more cases. It would be a political nightmare to have a series of forced, gradually tightened measures each time a new case might be found in the future. So all the way it'll have to be, and at once.

Tonight it has been announced that Lithuania has immediately banned all imports of beef from Denmark. Norway has announced that it expects more information from the Danish authorities tomorrow -- if not, all imports of beef into Norway from Denmark will be suspended."

The neuropathology of experimental BSE in the pig.

J Comp Pathol 2000 Feb;122(2/3):131-143
Ryder SJ, Hawkins SA, Dawson M, Wells GA
In an experimental study of the transmissibility of BSE to the pig, seven of 10 pigs, infected at 1-2 weeks of age by multiple-route parenteral inoculation with a homogenate of bovine brain from natural BSE cases developed lesions typical of spongiform encephalopathy. The lesions consisted principally of severe neuropil vacuolation affecting most areas of the brain, but mainly the forebrain. In addition, some vacuolar change was identified in the rostral colliculi and hypothalamic areas of normal control pigs. PrP accumulations were detected immunocytochemically in the brains of BSE-infected animals.

PrP accumulation was sparse in many areas and its density was not obviously related to the degree of vacuolation. The patterns of PrP immunolabelling in control pigs differed strikingly from those in the infected animals.

Opinion (webmaster): This is one of very few published experimental studies of pigs; no slaughterhouse survey has been permitted. The report suggests transmitted disease could have been hard to detect using older methods; pigs also are often slaughtered at a young age. It seems probable that pigs have eaten a great deal of rendered cow, sheep, and pig rations over the years.

In the US, pigs were exempted from the downer cow feed ban on the argument that, although very closely related to cows as even-hoofed mammals and unlikely to have much of a species barrier, technically they were not ruminants. An incident of suspected porcine TSE in NY State was investigated but details were never published, according to Dr. Michael Hansen of Consumers' Union.

Portugal probes suspected mad cow death link

17 Feb 00 Reuters
Portuguese health officials were investigating the death of a 65-year-old woman from a brain disorder for a possible link with the human equivalent of mad cow disease, a Health Ministry source said on Thursday.

"There is a suspicion, but the case history suggests this was the classic strain of Creutzfeld-Jakob disease, rather than a new one related to BSE," the source said. The source added that results were expected by the end of this or next week.

A new variant of the human Creutzfeld-Jakob disease that has killed at least 48 people has been linked to the brain-wasting BSE (bovine spongiform encephalopathy), the so-called mad cow disease, from which hundreds of cows have died across Europe since tracking began in the early 1990s.

Portugal has yet to record a case of the new CJD variant, but is still banned from exporting its beef to the European Union due to the number of BSE cases registered in cows. The Agriculture Ministry reported a preliminary total of 162 BSE cases in 1999, which was higher than in 1998. But it said the disease's incidence had peaked because no cases had been reported for cows born since 1995 when officials banned the use of meat and bone meal in animal feed, which are widely thought to cause BSE.

Comment (webmaster): This case may simply be sporadic or familial CJD. The puzzling aspect of the story is that an average incidence of sporadic CJD in Portugal means that a fair number of cases are expected each year; why did this particular one get singled out? Portugal imported massive amounts of meal and bone meal from Britain; past reports of BSE incidence have dramatically understated the problem, meaning that trend data is useless.

Officials Find Eighth Case This Year of Mad Cow Disease In France

2/29/2000 PARIS (AP)
Officials said Monday they have detected a new case of mad cow disease in western France, the eighth discovered since the beginning of the year.

The milk cow and its herd of 100 were slaughtered as a precautionary measure, the Agriculture Ministry said in a statement. The affected animal was found in the Cotes dArmor region of Brittany. Last year, authorities discovered 31 cases of mad cow disease, or bovine spongiform encephalopathy, in France.

The Agriculture Ministry has said new cases of mad cow disease will break out in France until 2001, five years after stringent prevention measures were taken against the disease, which has an average incubation period of five years. [Wishful thinking. They would do better to test randomly at the slaughterhouse for a year and then consider an estimate. -- webmaster]

Officials have said they will soon begin tests of cattle destined for human consumption to determine how widespread the disease has become.

France has provoked a spat within the European Union by keeping in place it's ban on beef from Britain, where the brain-wasting disease first started an international scare in 1996. The EU ruled in August that British beef was again safe for import, but France has maintained its ban.

Swiss give ok to tainted blood

Xinhua, 28 Feb 2000 [promed edited]
The Swiss Federal Health Office has decided _not_ to ban people living in Britain between 1980 and 1986 from giving blood, Swiss Radio International reported Sunday.

The decision came despite claims a ban could help control the spread of new variant -Creutzfeldt Jacob Disease (nv-CJD), which is the human variant of BSE, (Bovine Spongiform Encephalopathy) commonly known as mad cow disease, a fatal brain disorder in cattle. It is thought humans can contract nv-CJD by eating contaminated beef, although this has never been formally proven.

The Swiss health authorities believed existing blood filtration systems had provided sufficient protection for patients, adding a ban on donations from former British residents is unnecessary. It admitted, however, this method does not provide a 100 percent guarantee that blood products are safe, saying this would require a new test capable of detecting the infectious agents which cause BSE and nv-CJD. As a further precaution, donors will be asked if there have been cases of CJD within their families, and to state whether they have undergone corneal implants.

Britain was badly hit by the BSE epidemic. As a result, the United States and Canada have imposed bans on blood donations from people who were living in Britain when the disease was at its height.

Although they hope these precautions will help limit the spread of nv-CJD, experts admit the benefits of such bans are impossible to quantify as it is not yet known whether the disease can actually be transmitted through blood products.

Statement No 628 Ms Jean Crowe

The BSE Inquiry.  29/02/2000
1. I am the mother of Lisa Crowe born 22 January 1969 and died on 13 December 1998 at 11.00 a.m. from new variant Creutzfeldt-Jakob Disease (nvCJD).

2. I make this statement as an element of the investigation into the untimely death of my daughter, Lisa Jane Crowe. My husband and I now seek to investigate whether Lisa’s death is attributable to the ingestion of beef and beef products contaminated with bovine spongiform encephalopathy (BSE).

3. Upon leaving school, Lisa worked for a while as a trainee hairdresser but left after a couple of years. She got a full-time job at Great Mills DIY in Bromley where she worked until she met and fell in love with Sion who came from North Wales.

4. After a while, Lisa moved to North Wales to set up home with Sion. She never had a proper job in Wales, just a few different part-time jobs in various shops.

5. Lisa loved Wales but missed the family terribly. We were always a very close family and Lisa was very family orientated. She loved visiting all the family and would always take the younger members out. She loved children. Lisa and Sion’s relationship did not always run smoothly; there were many problems and I think Lisa always felt isolated from her family. When things were bad though she would come home for awhile but after a few weeks, she would always return to Wales.

6. She really loved Sion. There were a lot of times when they were truly happy especially when the baby was born. Lisa was so proud of Shannon. She was looking so forward to being a part of Shannon’s growing up. However, circumstances took over and all the time Lisa had with her young daughter was two short years.

7. In late June/early July 1998, when the first signs of Lisa’s illness became apparent, she came back home from Wales to stay with her father and me.

8. We all thought at first that Lisa was suffering from post-natal depression which the doctors had failed to diagnose. She became withdrawn, forgetful, constantly tired and lost a lot of weight. She also constantly complained about pains in her legs.

9. During the early stages of her illness, until about late August 1998, Lisa managed to look after Shannon. She did everything for her young daughter. Lisa adored Shannon and it was always very upsetting seeing her become less able to do things for her.

10. Lisa was under the care of our local General Practitioner (GP), who prescribed the anti-depressant drug, Prozac, for Lisa because he diagnosed that she was suffering from depression. However, when there was no improvement in Lisa’s condition, the doctor eventually, under pressure, gave us a letter to take with us to our Acute Hospital Trust.

11. Lisa was assessed in the Casualty Department of the Acute Hospital Trust. The doctors arranged for a Consultant Psychiatrist, to make home visits to see Lisa. It was shortly after this that Sion left his job and moved in with us to help look after Lisa and their baby.

12. Lisa was still deteriorating. She was now hallucinating. She thought that she had a second baby. She would walk around the house looking for this baby.

13. Lisa was next admitted to the psychiatric ward of a Mental Health Trust. Her treatment here was not what we would have hoped it would be. Nobody knew what was wrong with her. She was just subjected to a lot of tests and apart from that, she was just left to get on with things in general.

14. By this time, Lisa was totally confused. She could hardly walk. She suffered panic attacks when she could not breathe. She suffered from headaches and had trouble with her vision. She was constantly rubbing one of her eyes.

15. After having a Barium test, Lisa was particularly bad. She could not control herself, was incontinent, but the only help she was given was to have a commode in her room and a giant roll of toilet paper was supplied. This treatment went on for three days with the room foul-smelling. Lisa had to sleep in that room. She needed help. She could not do much for herself. We could take no more and we brought her home but after awhile we had to take her back to the hospital again.

16. By now, the doctors on the psychiatric ward had decided that Lisa had a medical problem. They tried without success to get her transferred to a medical ward at the Mental Health Trust. The medical doctors did not want to take her so we brought her home again.

17. After awhile, arrangements were made for a medical doctor to see Lisa to assess her condition. We went to the Mental Health Trust. After a wait of seven hours and no sighting of a doctor, we gave up and came home. We were then told to return on the following morning and to be there by 8.00 a.m. when a doctor would see us.

18. After a long wait the next morning, and unsuccessfully paging a doctor, we were getting very distressed, especially Lisa, and we were taken to a room where we could sit and wait.

19. Four hours later, when the doctor still had not appeared, we were then told that they had been able to make an appointment for us at a London Teaching Hospital. Lisa was to be seen by a neurosurgeon that afternoon.

20. We returned home. We were now all very distraught. We went to the London Teaching Hospital in the afternoon. Lisa was seen by a Consultant Neurologist. It was he who was the doctor who said that he thought that Lisa could be suffering from the new variant form of CJD. We were devastated at the news even though we were already suspecting that this might be the diagnosis.

21. Lisa was admitted to the London Teaching Hospital just over a week later. The Consultant Neurologist and his team were great but Lisa’s treatment was still not as good as it should have been. The nurses did not really seem to care. They did not know how to cope with Lisa.

22. Lisa by this time had become quite difficult to handle. She would over-react to everything, sometimes she would be aggressive. She could not walk without help. She did not always know what she was doing. She also seemed to lose all sense of pain.

23. Because Lisa did not seem to be getting the care she deserved, I started taking time off work so that I could be with her at the hospital for most of the day. I helped to look after her. We also took a wheelchair into the hospital for Lisa so we could take her for walks.

24. Lisa loved visits from her daughter, Shannon, but she would always become very agitated if Shannon did not sit still.

25. Lisa was being nursed in a mixed ward which was not very nice for her. She did not have any privacy and when she used to become very agitated and confused, I felt as though everybody was looking at her. The whole scenario was very distressing.

26. However, the Consultant Neurologist and his team could not have done more for Lisa and for us. They were there if we wanted to talk. They never made a decision without consulting us first and did their level best for Lisa.

27. After numerous tests again, Lisa eventually went to a London specialist Teaching Hospital in Paddington, for a tonsil biopsy.

28. By now, Lisa was difficult to understand as her speech was often slurred and she was often incoherent. Her treatment at the London specialist Teaching Hospital was first class.

29. The specialist nurse who looked after Lisa whilst she was there, was excellent; she had a very caring nature. At last it seemed as though somebody really cared about our Lisa.

30. After the tonsil biopsy, we took Lisa home.

31. We had already been in touch with Social Services who supplied us with a special chair for Lisa together with other aids and equipment to help her. Sion, who had all this time been doing most of the caring for Lisa and the baby whilst she was at home, did not receive a penny in help. Both my husband and I had full-time jobs which we were trying to keep going and without Sion’s help, we could never have managed to look after Lisa.

32. Our local GP, arranged for a nurse to keep calling in on Lisa and also for a carer to come in and get Lisa washed and dressed in the mornings. These two ladies were very good. However, once when we called the doctor out to see Lisa because we were worried about her condition, he did not seem to want to touch her. He listened to her chest through her jumper. You could tell from his attitude that he really did not want to be there and that he had no idea how to handle the situation. We had to ask the Consultant Neurologist if he would phone the GP and explain the disease to him.

33. We had Lisa home for just a couple of weeks but she deteriorated so much during that time. She lost all speech and just sat there looking vacant. In the end, Lisa could not eat or drink. We were then advised that she would have to go back into hospital again where she could be made more comfortable.

34. Lisa was readmitted to the London Teaching Hospital but this time she was admitted to a side room which was much better for her and for us. She was commenced on intravenous liquids. We said that we did not want Lisa to be force fed. She was also heavily sedated.

35. We made sure that somebody was with Lisa all the time. We took it in turns to stay with her overnight. All we could do though was just watch her waste away. She looked nothing like the Lisa we all knew and loved. Lisa was once so beautiful but now she just looked like a skeleton with skin on. We tried to keep moistening her lips but this would make her face contort into the most awful grimaces whereupon she would shut her mouth so tight, one could not open it.

36. Her hospital room in the last couple of weeks was always full of family and friends. I would like to think that perhaps Lisa was aware and knew just how much everybody loved her. We would take it in turns to sit with her and to hold her hand.

37. I took time off work so that I could stay at the hospital all the time with Lisa but sadly I was not with her when she died. The nurses came in to wash and to turn her in the bed so I went downstairs to see my husband off as he had to go home. When I returned to the room, Lisa had just passed away.

38. It was 11.00 a.m. on 13 December 1998.

39. Lisa was cremated on 23 December 1998.

40. Lisa’s death has left a great void in our lives which we are all still trying to come to terms with. We all miss her terribly; her smile, her phone calls from Wales, her visits home, in fact, everything. It feels like a part of me has gone and I only wish that I could change places with her now. She had so much to look forward to.

41. This despicable disease which has robbed Lisa of her life (she was only 29 years of age when she died) has also robbed us of a daughter who we loved dearly, our son of his sister and Sion of his partner, and Shannon, dear Shannon has lost her mother. This is something that should never have happened, it was not God’s will but man’s greed that killed Lisa.

42. We just hope that Lisa has gone to a better place. I just wait now for the day when I can join her. My life will never be the same again. I do not feel that I have any right to be happy or to enjoy myself ever again. Any celebrations are particularly difficult for all of us.

43. Since Lisa’s death we have not eaten any beef at all. I don’t think we ever will eat beef again. Lisa used to love beef burgers and whenever she was out we would always go to MacDonalds. At home, she always used to eat a lot of chicken, but beef certainly featured most in her diet.

44. The matters referred to in this statement are within my own knowledge except where stated otherwise.

45. I believe that the facts stated in this witness statement are true.

More Cattle Have Ecoli Than Thought

3/1/2000 By: Philip Brasher AP Farm Writer 
About half the cattle at the nation's feed lots carry the deadly E. coli bacteria during the summer making it at least 10 times more common than previously thought, government research shows. The study by Agriculture Department scientists doesn't mean that E. coli O157:H7 is any more likely to show up in the supermarket. But USDA officials, who outlined the findings Tuesday, said they are considering new controls on cattle production and beef processing.

The research requires us to re-examine our policies and standards for dealing with this difficult organism, Thomas Billy, administrator of USDA's Food Safety and Inspection Service, said at a conference in Arlington, Va.

The bacteria, which is most commonly found in ground beef, kills about 60 people each year and sickens an estimated 73,000 more, according to the Centers for Disease Control and Prevention. The departments findings, which will be published in April's Proceedings of the National Academy of Sciences, are based on detection methods that are far more sensitive than previously used.

The occurrence of E. coli in feed lots drops to 1 percent during the winter, but scientists found that 83 percent of the cattle they studied had been exposed to the bacteria at some point. Calves can pick up the bacteria during the birth process, while other cattle get it from manure, scientists say. Changes in feeding methods and transportation have been shown to reduce the incidence of E. coli.

Consumer groups say the government needs to require far more extensive testing of cattle and beef to prevent people from being exposed to the germs. Testing is now required only on ground beef, a program that started after tainted hamburger killed several children in Washington state in 1993.

"Like throwing darts at a dart board, although the government hits the target occasionally, it is clearly missing a lot of the problem," said Caroline Smith DeWaal of the Center for Science in the Public Interest.

The incidence of E. coli in cattle is believed to vary by region as well as time of the year. An industry-funded study released Tuesday found a wide variation of infection rates at packing plants. At least 18 percent of the cattle headed for slaughter at a dozen plants were carrying the bacteria. Two of the plants had no infected cattle, and the average rate for the 12 facilities was 3.56 percent.

The bacteria was found on 0.44 percent of the fresh carcasses sampled in the study. But no E. coli showed up once the carcasses had gone through the plants usual cleaning process, which generally involves steam, hot water or organic acid rinses. The study shows that E. coli testing should be done earlier in the packing process, so infected meat is caught before it is processed, industry officials said.

"It is our hope that this data will encourage USDA to re-evaluate its ground-beef sampling program," said James H. Hodges, president of the American Meat Industry Foundation, which sponsored the research. A carcass testing program for E. coli O157:H7 is more practicable and will help ensure that the safest and most wholesome product possible enters commerce.

Scientists are working on additional methods of treating meat to kill a variety of pathogens, including E. coli. One is an anti-microbial agent, known as lactoferrin, that is a naturally occurring protein in the milk of mammals, including humans.

Laboratory research presented at Tuesday's USDA meeting indicated lactoferrin was effective against more than 30 different kinds of harmful bacteria, including E. coli, salmonella and campylobacter. Lactoferrin does not change the taste, flavor, color or appearance of meat and isnt harmful to humans, the researchers said.

"We have borrowed a page from Mother Nature," said A.S. Naidu, a medical microbiologist at California State Polytechnic University-Pomona, where the treatment was developed. "We have taken a natural compound with anti-microbial properties and discovered a way to make it work on meat surfaces to provide a protective barrier against harmful bacteria.

The government recently approved the use of irradiation to treat raw meat, but it is unclear how customers will react to that.

Opinion (webmaster): This hardly just a new 'strain' of E. coli. It is really more of a chimera. A megabase of extra DNA is another whole bacterial genome -- a thousand genes. Has some unsuspected process in the cattle industry favored selection of this oddity or has it been there undetected all along?

Chromosome of the enterohemorrhagic Escherichia coli O157:H7; comparative analysis with K-12 MG1655 revealed the acquisition of a large amount of foreign DNAs.

DNA Res 1999 Dec 31;6(6):361-8
Ohnishi M, Tanaka C, Kuhara S, Ishii K, ... Terawaki Y, Hayashi T
A complete Xba I and Bln I cleavage map was constructed for the chromosome of an enterohemorrhagic Escherichia coli (EHEC) O157:H7 strain isolated from an outbreak in Sakai City, Japan, in 1996. A comparative chromosome analysis with E. coli K-12 strain MG1655 was made. The EHEC chromosome was approximately 5600 kb in length, 1 Mb larger than that of MG1655. Despite the marked difference in chromosome length, the location and direction of seven rRNA operons of the EHEC strain were similar to those for MG1655. Overall organization of genes common in both strains is also highly conserved. Chromosome expansion was observed throughout the EHEC chromosome, albeit in an uneven manner. ... Sample sequencing of 3627 random shotgun clones suggested the presence of approximately 1550 kb strain-specific DNAs on the EHEC chromosome, most of which are likely to be of foreign origin.

Lovers of German sausage risk exposure to BSE

Debora MacKenzie New Scientist News Sat, 4 Mar 2000
Source: Journal of Food Protection (vol 63, p 258)
MILLIONS of Germans could be eating brain tissue in cooked meat products that are supposed to be "brain-free", a new test reveals. German scientists have developed the first test for brain and spinal cord material in products such as pâté and sausages.

Their tests reveal that up to 15 per cent of German liverwurst and mettwurst, also known as bologna, may contain undeclared brain material. The scientists say that the use of such tissue is "unacceptable with regard to the development of new variant CJD".

Countries which recognise that their cattle may have BSE destroy brains and spinal cords from slaughtered animals, as these are the most infectious parts. The European Commission wants all member countries of the European Union to do the same, in case BSE is lurking in countries that are officially free of it. In December, the Commission's Scientific Steering Committee (SSC) concluded that "failure to do so is likely to expose a large number of consumers to an unnecessary risk".

But Germany refuses. "We have no BSE in our cattle," says Dorothe Heidemann of the Action Committee for German Meat, which represents farmers. "And there is no wide use of brain in meat products."

"We questioned this," says Ernst Lücker of Giessen University. "Brain is cheap material for sausages." But, he says, "we were astonished to discover there was no way to test for it in cooked meat products." So Lücker and his team decided to find a way. They collected 440 samples of local ready-to-eat sausage and 126 samples of liverwurst from all over Germany.

First, they measured cholesterol. Brain is rich in it, but so are other potential ingredients, such as eggs. They then used monoclonal antibodies to test those sausages with unusually high cholesterol for protein from glial cells in the brain [GFAP] and for an enzyme called enolase, which comes from neurons.

Those results, plus more recent data, says Lücker, revealed that blood sausage and nearly all frankfurters were brain-free. But brain matter turned up in 9 per cent of liverwurst and 15 per cent of mettwurst.

It is not illegal to put brain in food in Germany. "But these were the highest-quality products, which may not contain any innards but liver," he says. One company using brain repeatedly has already been fined as a result of the tests. This poses no risk if the brains are from pigs, or uninfected cattle, but Lücker's test cannot distinguish species.

"If there is any brain when the label says there should be none, we should be cautious," he says. The SSC is expected to announce shortly that BSE cannot be ruled out in Germany. In addition, Germany imports some of its sausage meat, says Lücker. Meat from countries with BSE should not contain brain, but without a test it has been impossible to tell if the ban is enforced. Now, the Swiss Federal Veterinary Office in Bern plans to use Lücker's test to monitor imported and domestic sausage.

Comment (Debora MacKenzie Europe correspondent, New Scientist; Thu, 2 Mar 2000):

"I would like to comment on the remarks that have been exchanged concerning the article I wrote for New Scientist this week.

The sausages tested were not traditional recipes using brain, and they were legally not allowed to contain it. It is not in question that there are some sausages in Germany that openly contain brain. It is legal to eat brain in Germany, though the meat industry insists it is not "in widespread use" and is used only in the traditional recipes calling for it. But the sausages tested were not supposed to contain it, and these, not the traditional kinds meant to contain brain, are the varieties consumed in the largest quantity. Meanwhile the existence of the analytical method raises other interesting possibilities.

It is illegal to put any internal organ but liver in sausages labelled "Spitzenqualitaet", "Kalbsleberwurst", and other quality designations which are defined and regulated under German food law. These were the products tested in this survey. Indeed, one company found in the course of the study to be adding CNS to such sausage repeatedly was fined.

As for whether it is proper to eat CNS in countries "without" BSE, I refer you to the conclusions of the Commission's advisory committee in December, which said that, just in case there might be some BSE lurking undetected or undeclared in countries nominally free of it, SRM bans should be applied. Germany is affected by the presence of BSE in neighboring countries, its history of importation of British cattle, MBM, etc. A bit like Denmark. But just for interest's sake, the team in Giessen calculates that one infected brain used in these products by one large sausage producer could reach 44,000 people.

The analytical method described in the article (which is sensitive to 0.01% CNS added to control sausage preparations) is designed to detect the deliberate, bulk addition of CNS to cooked meat products, not to detect what must be the tiny (although I agree not necessarily clinically irrelevant) amount of CNS contaminant in blood that results from stunning.

Obviously such minute contamination, while important, cannot possibly be as dangerous as the wholesale addition of up to 3 per cent CNS material to sausages legally supposed to be free of it, unless we have abandoned all notion of dose response ratio.

The interesting thing about this information is that there was never before any test to determine whether CNS was added to cooked meat products, and now there is. Indeed, tests based on the detection of the same proteins have only recently been developed for fresh meat. Minced and sausage meat in the EU is exempt from the labels of origin all other meat must carry from this year. This makes some test to determine what it really contains of obvious interest.

Such meat from countries that admit to having BSE is not supposed to contain CNS tissue, but as the British experience showed, the extent to which the specified risk materials ban may be enforced could be open to question. It can now be policed. The Swiss are designing sampling protocols to enable them to use the test to monitor imported sausage and minced meat. I wonder if they will find CNS in French or Belgian meat as they found DES in American beef?

Luecker is very interested in obtaining samples from elsewhere -- perhaps places that have an SRM ban, and especially samples of meat products dating from the 1980s in Britain. It was widely assumed there was CNS in those, but how much? It might be interesting to know. He has not yet received a reply to his enquiries about such material from MAFF.

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