Prion Disease: Tonsil Tests Underway
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Tonsil test delays
15 new nv CJD cases announced
Full text of DoH/SEAC announcement
nvCJD case: hospital sets up helpline for mothers
We sentenced Rachel to death, say CJD girl's parents
Village lives in fear of the 'dust of death'
Pig farmers go to court for BSE aid
Germany bows to BSE-beef imports
Pithing of cows, sausage casings banned in France
Organs taken from 800 children without parental permission
CWD deer, elk not wasting Utah hunters

Tonsil test delays

Thu, 16 Mar 2000 SEAC press conference [hosted by MAFF]
Notes and opinions of an attendee;   SEAC press release page 
"1. Tonsil testing: The trials of tonsils and appendices have been proceeding but very slowly. Two retrospective trials, one in Scotland and one in the South West of England, have only tested about 1000 samples each out of a total of 18,000, in over a year. No results are yet available, but it was accepted that any positive results (i.e. indications of abnormal prions) would be put in the public domain 'promptly'.

The major prospective trial of 2,000 tonsils, run at Collinge's laboratory in London, has stalled for over a year while it waited for the relevant ethical committee to give it clearance.

The project has just been cleared this week -- the ethics committee have finally agreed that specimens will be tested anonymously (this gets round the difficulties of having to tell people they may have an incurable disease, but it means that no follow up of positive samples can be pursued, to see if the positives do actually develop the disease, somewhat defeating the purpose of a prospective study).

2. The Edinburgh-based national CJD surveillance centre has been following up on blood and blood products which had been donated by people who subsequently developed CJD (it wasn't clear if this was nvCJD or all-cause CJD). Products known to be on the shelves have been or are being withdrawn. Products that have been used have been traced to 'a very small' number of recipients.

The CJD centre has been instructed not to tell recipients that they have received possibly tainted products, although there is confusion about what to do if any of these recipients themselves decided to donate blood -- would they be barred from donating, and would

3. Among the 'probable' cases of nvCJD currently being investigated is the youngest yet case of a 13-year old. Diagnosis has yet to be confirmed post-mortem. The 15 new cases represent a 29% jump over the baseline of 52 cases (plus 3 in Ireland and France).

SEAC 15 February 2000 meeting notes were ironicaly released on 16 Mar 00. They include the following:

"The Committee... considered the recent publication in 'Transfusion' suggesting that infectivity was detectable after disease onset in the plasma of mice experimentally challenged with a mouse adapted human TSE. It also considered preliminary evidence which appeared to demonstrate that blood plasma, taken from mice with clinical disease caused by a mouse adapted BSE agent and inoculated into the brains of disease-free mice, gave rise to clinical disease in some of the recipient mice....

The Committee noted that the pathogenesis of the disease caused by BSE in mice was different to the pathogenesis of BSE in cattle but more similar to that of TSEs in sheep and humans. Given the difference in the pathogenesis of the disease in cattle and this mouse model, and taking account of the results of bioassays of cattle tissues in both mice and cattle, the Committee concluded that there were no implications for the safety of the food chain from these findings.

...The Committee recommended that work to ascertain the sub-clinical prevalence of vCJD in the human population should be taken forward with all possible speed. This might, for example, include large scale surveys testing tonsil and appendix tissue for evidence of infection....

Opinion (webmaster): This is a bizarre document that everyone in SEAC may have known to be fraudulent. Of course, 'proposed' tonsil tests had long been underways as the press conference today made clear. Indeed these tests were initiated and overseen by SEAC. It is inexplicable that only 1000 tonsils have been tested after a full year -- this many could be tested in a week. Only about 38% of the population has the high risk codon 129 met/met alleles so in effect only 380x2 were tested.

The survey design may be fatally flawed by intent, as happened in maternal BSE and hunting dog studies -- years later, it is 'oh well, have to start over.' Or there will be a mimimalist announcement that'they were all negative, with nothing ever published. For these reasons, study protocols and status (weekly updates) should be made available to scientists now.

The age distribution for nvCJD has not been released (apparently so as not to alarm the over-30's). However, the ages for the first 39 cases from Ghani et al 1999. Proc R Soc Lond B Biol Sci 267:23-29 are provided below. Note that 11 cases are 35 or over and 2 are even over 50. If these are ages at death, then the data are pushed out a couple of years from onset data. Ideally retrospective onset would be used so that still living cases could be included. The ages of the 15 new cases were not released except for the 13 year old girl.

0-14    0
15-19   5
20-24   9
25-29   8
30-34   6
35-40   6
40-44   2
45-49   1
50-54   2
55-74   0

15 new nv CJD cases announced

 Sat, 18 Mar 2000 Dept of Health press release

New CJD tests established

Fri, Mar 17, 2000 By Maxine Frith, Health Correspondent, PA News
Scientists have established new tests which for the first time will allow them to diagnose people with the human form of mad cow disease before they die. Until now doctors could only be sure a person had new variant CJD once they had died and a brain biopsy could be carried out. But medical advances and new research mean experts have now established criteria which enables them to say CJD is the "probable" cause of someone's illness.

Living, "probable" CJD victims will be now included in the Department of Health's monthly figures for the first time from April. The policy change was ordered by the Deputy Chief Medical Office Dr Pat Troop on the advice of the Spongiform Encephalopathy Advisory Committee (SEAC), the Government's expert body on BSE and CJD.

Interim figures show that there are 12 living patients in the UK with probable CJD and three other people who have died but whose relatives were still awaiting confirmation that the disease was the cause of death.

Dr Troop said: "Being able to count and record the number of living sufferers will allow us to make more accurate predictions about the future of this disease. This is now possible because diagnostic tests have been very accurate in identifying `probable' CJD in severely neurologically impaired patients. However there is no test available to detect those who may be infected with CJD in the pre-clinical (before people fall ill) stage. Developing such a test remains one of the Department of Health's research priorities."

Victims normally suffer from the progressively debilitating disease, which has no cure, for an average of 14 months but some live for more than three years. The new criteria mean that patients should be able to be identified as "probable" CJD sufferers within six months of the first signs. So far 52 people have died from new variant CJD since it was identified in 1995.

UK starts counting living 'probable' CJD cases

Friday March 17   Reuters Health
A combination of tests can now reliably detect the fatal neurological disorder, variant Creutzfeldt-Jacob disease (vCJD), in living patients, British officials announced on Friday. The disorder is a form of the disease linked to eating beef infected with bovine spongiform encephalopathy (BSE), also known as 'mad cow disease.'

Because testing for the disease is more reliable, the numbers of 'probable' cases of the disease can be counted, the British Department of Health said on Friday. The total number of such 'probable' cases in the UK includes 12 living patients and 3 deceased patients whose cause of death is yet to be pathologically confirmed, according to the UK governme

The announcement follows recommendations from the Spongiform Encephalopathy Advisory Committee (SEAC), which confirmed that the recently agreed criteria for diagnosing vCJD in living sufferers are reliable. In the past, the disease could only be confirmed at autopsy, but a combination of magnetic resonance imaging of the brain and a biopsy of tonsil tissue can determine a 'probable' case of the degenerative disorder, according to the SEAC.

``We welcome SEAC's advice,'' said Dr. Pat Troop, the Department of Health's deputy chief medical officer, in a statement. ``Being able to count and record the number of living sufferers will allow us to make more accurate predictions about the future of this disease.''

Troop noted that there is still no way to detect vCJD before symptoms begin. Typically, an individual experiences hallucinations, difficulty walking and talking, and progresses to coma and death in anywhere from 7 to 38 months. The average duration of the disease is 14 months.

``A probable case would typically be diagnosed at about 6 months into the illness,'' according to the statement. More than 52 people in Britain have died of vCJD since British scientists identified the new strain of the disease in 1996.

Opinion (webmaster): These 15 new cases are catch-up cases that SEAC/MAFF has apparently been holding back to minimize perceptions of the nvCJD epidemic until beef exports to Germany could be resumed. It is curious to see a top public health official still taking marching orders from an agricultural committee. Revising old data of course has the effect of depleting future cases.

MRI, little used in CJD, is thrown in to mask the delay in use of tonsil data, which has been bitterly contested for several years on non-scientific grounds. How many 'false positives' would result simply from relying on amyloid plaque immunopositive for prion protein in tonsils? Do the 'false positives', say from scrapie brain, make better or worse blood donors? A few false positives don't really matter when the choice is between a few thousand and a few million cases of nvCJD.

The notice says that the 15 cases will be included in the 3 April 00 statistics but does not say how they will be back-allocated to year. An average disease course of 14 months with a 7-36+ month range is a useful disclosure of the skewed distribution. The article alse states that nvCJD is detectable within six months of the first signs (not defined) and asserts there is no pre-clinical test.

Since government interference, in the guise of a bogus hospital 'ethics' committee, continues today with the prospective tonsil test, it is clear enough why pre-clinical nvCJD is not detectable. In oral exposure, the tonsil is thoroughly infected long before signficant neuronal death (MRI detectibility, clinical sign) occurs. Perhaps a case does not get referred for tonsil testing until it becomes MRI-positive (routinely run at smaller hospitals for puzzling cases). At some point, concerned citizens may have to go to Europe to get themselves tested; England will not offer pre-clinical testing because it could interfere with beef exports.

For purposes of calculating year-to-year trends (a poor substitute for a removed-tonsil survey) as a non-lagging indicator, it is not completely clear how to consistently assign a date to each case retrospectively: first cliinical sign minus 6 months? first anomaly noted by parents or spouse? midpoint of disease course?

With exports of BSE beef resuming yesterday and cashiering of the MAFF head, opposition to dislosure has dramatically moderated. Yesterday saw the admission of two secret tonsil serveys and permission to go forward with a third; today, backing off from the lagging indicator (death + 4 months for study + 2 months for inclusion).

Still, we aren't quite there yet. Protocols and results of the two small scale, slow-moving tonsil surveys have not been disclosed a year after initiation; Collinge's tonsil survey has just been neutered; and the age distribution is still very closely held. Above all, no information on the scope of the epidemic has been released. In fact, the government is not allowing any research go forward to determine it.

No one actually knows the scope of the epidemic -- the government has fought off by fair means and foul all efforts to get a handle on the number of future cases. The biggest threat came from the Wellcome Trust because it funded Collinge and Anderson and was outside MAFF's control. However, the government had their top epidemiologist brought up on a gender charge and stopped their top experimentalist via a bizarre year-long delay at the hospital 'ethics' committee. (Identical issues come up and are resolved daily in medicine -- a year's delay is pure politics.)

If nobody knows what is going on, there is no worry over leaks or defections to the public interest side. But this leads to a most bizarre state of affairs: The top level of governmental itself does not know -- nor want to know -- the scope of the epidemic.

This is to establish 'plausible deniability' -- at a hypothetical second nvCJD Inquiry in 2005, when asked why research was such a low priority, they can say, 'oh, there were just 70 deaths by then and no disturbing trend or survey data, we didn't know it would get this bad.' No one is accountable this way, past or future; hopefully the bad news falls to a later administration of the opposite party.

Fifteen years have been wasted with delay and denial. If the scope is really bad, will there be enough research time remaining to do anything about it? This is a very dangerous chance to take with a difficult disease to which the entire population has been exposed.

Full text of DoH/SEAC announcement

Thursday 16th March 2000  (Issued 17th March) SEAC
SEAC advice on publishing 'probable' cases of new variant CJD accepted

 Following advice today from the Spongiform Encephalopathy Advisory
 Committee (SEAC), the Department of Health will in future publish the
 number of living patients known to be suffering from 'probable'
 variant Creutzfeldt-Jakob disease (vCJD), in addition to the number
 of confirmed vCJD deaths currently published monthly.

 Deputy Chief Medical Officer Dr Pat Troop said:

 "We welcome SEAC's advice which confirms that the recently agreed
 criteria for diagnosing vCJD in living sufferers is reliable enough
 for us to publish the number of 'probable' cases identified in this
 way. That number will now be included in the Department of Health's
 monthly statement of CJD disease figures. The next one is due to be
 published on 3 April 2000.

 "Interim figures show that there are 12 patients alive in the UK who
 are 'probable' sufferers of vCJD. In addition, there are a further 3
 'probable' vCJD cases who have died but are awaiting pathological
 confirmation that their deaths were 'definitely' attributable to this
 disease. Brain biopsy remains the only way to confirm this 'definite'
 vCJD status.

 "Being able to count and record the number of living sufferers will
 allow us to make more accurate predictions about the future of this
 disease. This is now possible because diagnostic tests have been
 very accurate in identifying 'probable' vCJD in severely
 neurologically impaired patients. However, there is no test
 available to detect those who may be infected with vCJD at the
 pre-clinical stage. Developing such a test remains one of the
 Department of Health's research priorities.

 "The average duration of the disease is 14 months, but can range from
 7 to 38 months. A 'probable' case would typically be diagnosed at
 about six months into the illness. Although this helps provide an
 indication of future numbers of deaths from the disease, we cannot
 predict when these will occur nor how many more 'probable' cases
 would be identified at any time."

 Dr Troop continued:

 "I would also like to thank SEAC's for its work reviewing studies
 involving plasma. We note that the Committee did not recommend any
 further public health measures additional to those already in place.
 The Department of Health has introduced measures to protect the
 safety of blood and plasma on a precautionary basis. These include:

 * As from 1 November 1999, all blood (red cells and platelets)
 collected is leucodepleted (white cells removed).

 * All blood products manufactured by the UK plasma fractionation
 centres are sourced from non-UK plasma.

 "Finally, we note SEAC's concern about the need to discover whether
 people might be incubating vCJD and taking forward related research
 with all possible speed. I am pleased to announce that ethical
 approval has now been given to begin a further study of 2,000 tonsils
 to see whether any abnormal prion protein is present. This will be in
 addition to the 2 retrospective studies currently underway to examine
 18,000 tonsils and appendices taken from patients in the South West
 of England and the Lothian Regions."

 Notes to Editors

 1. Detail of the new diagnostic criteria are set out in at Annex A:

 2. The SEAC conclusions, on diagnosing vCJD in the living, follow on
 from work carried out by scientists at the National CJD Surveillance
 Unit at Edinburgh, at the St Mary's Hospital Prion Unit in London,
 and elsewhere. Experts at the Spongiform Encephalopathy Advisory
 Committee's Epidemiological Sub-Committee meeting on 9 February were
 able to confirm that the new criteria were robust enough to be
 included in future within the regular statements on CJD figures
 published by the Department of Health. SEAC subsequently endorsed
 these findings.

 3. Reliable tests can now be carried out on those with suspected
 symptoms of the disease, by means of (i) magnetic resonance imaging
 of the brain, or (ii) tonsillar biopsy. When taken together with
 other criteria (at Annex A), the results can enable clinicians to
 determine with reasonable confidence whether or not an individual is
 suffering from vCJD.

 4. Statistics on all types of CJD disease are reported to the
 Department of Health by the National CJD Surveillance Unit in
 Edinburgh, who undertake their surveillance work in collaboration
 with colleagues in the neurological community and other scientific
 and medical experts both within the UK and worldwide.

 5. The CJD figures are due to be published on 3 April 2000.

 6. For more information please contact Emily Hands: Department of
 Health media centre - 0171 210 5233.








 PROBABLE: I and 4/5 OF II and III A and III B  or I and IV A
* depression, anxiety, apathy, withdrawal, delusions.
** this includes both frank pain and/ or unpleasant dysaesthesia
*** generalised triphasic periodic complexes at approximately one per second
**** spongiform change and extensive PrP deposition with florid
 plaques, throughout the cerebrum and cerebellum.


 The Spongiform Encephalopathy Advisory Committee (SEAC) met at the
 offices of the Ministry of Agriculture, Fisheries and Food (MAFF),
 Page Street, London on 15 February. The Committee thanked outgoing
 member Dr Peter Goodfellow who had retired from the Committee to take
 up other duties in relation to human genetics.

 The Committee conducted its regular review of research findings and
 epidemiological information on BSE and vCJD. It considered the recent
 publication in Transfusion Brown et al 1999, Further studies of
 blood infectivity in an experimental model of transmissible
 spongiform encephalopathy, with an explanation of why blood
 components do not transmit Creutzfeldt - Jakob disease in humans.
 Transfusion Vol. 39, November/December 1169 - 1178. suggesting that
 infectivity was detectable after disease onset in the plasma of mice
 experimentally challenged with a mouse adapted human TSE. It also
 considered preliminary evidence which appeared to demonstrate that
 blood plasma, taken from mice with clinical disease caused by a mouse
 adapted BSE agent and inoculated into the brains of disease-free
 mice, gave rise to clinical disease in some of the recipient mice.
 The Committee concluded that priority should be given to carrying out
 a study on mice infected with a mouse adapted BSE agent at various
 stages before development of disease. However, the Committee did not
 recommend any measures in addition to those already in place to
 reduce any potential risk to public health from human blood and blood

 The Committee noted that the pathogenesis of the disease caused by
 BSE in mice was different to the pathogenesis of BSE in cattle but
 more similar to that of TSEs in sheep and humans. Given the
 difference in the pathogenesis of the disease in cattle and this
 mouse model, and taking account of the results of bioassays of cattle
 tissues in both mice and cattle, the Committee concluded that there
 were no implications for the safety of the food chain from these

 The Committee recommended that work to ascertain the sub-clinical
 prevalence of vCJD in the human population should be taken forward
 with all possible speed. This might, for example, include large scale
 surveys testing tonsil and appendix tissue for evidence of infection.

 The Committee was informed that the total number of people who had
 died of vCJD stood at 52. Although the final number of deaths from
 vCJD during 1999 once all confirmations were complete was expected to
 show a fall from the number who had died in 1998, it remained too
 soon to make predictions with any confidence about the likely number
 of deaths in the coming years.

 The Committee noted the outcome of the meeting of its Epidemiology
 Sub-Group on 9 February. On the definitions used for confirmed and
 probable vCJD, the Committee endorsed the view of the Sub-Group that
 robust scientific criteria now existed. These definitions would
 enable live probable cases to be separately identified for the first
 time. The number of cases meeting these criteria could then be
 included within the data on vCJD published by the Department of

 The Committee conducted a review of key research related to scrapie
 and BSE in sheep. A representative from the Institute of Animal
 Science and Health, Lelystad, Netherlands presented some of their
 recent work on the pathogenesis of scrapie. Progress reports on the
 work at the Institute of Animal Health and Veterinary Laboratories
 Agency on the pathogenesis of BSE in sheep and screening brains from
 sheep with spongiform encephalopathy for the presence of BSE were
 also presented to the Committee.

 Members noted that the preliminary and interim results of the studies
 of experimental BSE pathogenesis in sheep tend to support the
 conclusions reached by SEAC in July 1996 and July 1998 that BSE in
 sheep is likely to behave in a very similar manner to the natural
 disease of sheep scrapie. The latest work on the pathogenesis of
 scrapie confirms earlier findings that the disease appears to infect
 peripheral tissues including the lymph nodes, spleen and parts of the
 intestine as well as the central nervous system. It also indicates
 that the spread of the infection is likely to be from the gut via
 peripheral nerves or via innervations of the lymphatics to the
 central nervous system.

 Agent strain typing is underway on isolates from over 130 brains of
 sheep with natural scrapie in order to determine if the BSE agent
 profile exists. Although incomplete (each study takes up to two
 years) over 30 isolates are advanced enough to suggest that they do
 not have the characteristics associated with the BSE agent. Members
 also noted strain typing of isolates from a pool of brains from
 scrapie affected sheep which had been collected for a rendering
 experiment between 1990 and 1992. The Committee agreed that further
 work needed to be done on screening sheep for evidence of infection
 with the BSE agent but concluded that at present there was no need
 for further public health controls.

 The Committee further noted the existence of an EU proposal on
 specified risk materials. The proposal would classify individual
 Member States according to the incidence of BSE in cattle. Members
 noted that to set out SRM controls for sheep and goats on this basis
 would not be consistent with the likely routes of exposure of sheep
 to BSE infection. This is because the risk of exposure would vary
 according to feeding practices assuming that sheep had in fact been
 infected from feed. It would also depend on if, and how infection and
 disease associated with the BSE agent could be propagated in sheep.

 Members further agreed that, in the event that the EU proposal to add
 intestine to the list of specified risk materials from sheep or goats
 were adopted by Member States, they would not advise against this but
 would advise that the whole intestine from sheep of all ages should
 be classified as specified risk material.

 The Committee noted a proposal by MAFF to extend the survey of the
 brains of cattle in the Over Thirty Month Scheme in the next year.
 They felt it was important to continue this work as it provided an
 independent check on models of the progress of the BSE epidemic. The
 performance of diagnostic tests was seen as critical to the outcome
 of such surveys. To that end the Committee welcomed the intention of
 the Commission to support a study to evaluate the usefulness of the
 recently validated tests that can efficiently detect prion protein
 (PrP) in the central nervous system (CNS) of clinically affected
 animals, for the detection of PrP in healthy cattle during the
 incubation period. This study will use CNS tissues from cattle in the
 study of pathogenesis of experimental BSE in cattle using the tests
 previously evaluated for sensitivity and specificity compared with
 conventional diagnostic methods. The study is expected to indicate
 how long before the onset of clinical signs the test would be
 reliable to detect PrP which, by inference, would indicate that a
 positive animal be infected. Members thought this was a key factor
 which must be taken into account when designing the surveys.

nvCJD case: hospital sets up helpline for mothers

Tue, Mar 14, 2000  By Matthew Cooper, PA News
A hospital today set up a telephone helpline for women who were treated with surgical instruments used to deliver a baby whose mother was suffering >from the human form of mad cow disease. The George Eliot Hospital in Nuneaton, Warwickshire, issued the number for mothers who underwent Caesarean sections at the hospital's maternity ward between October 26 last year and January 29 this year.

The instruments, which may have been contaminated with the agent which causes new variant Creutzfeld Jakob Disease, were re-used in seven Caesarean sections before the mother was diagnosed as having the disease.

George Eliot's chief executive Nick Carver said the hospital was relieved that a court injunction banning identification of the hospital had been varied. Mr Carver added: "We are pleased that the injunction has been relaxed, enabling any women who have any concerns at all to contact us directly. "As soon as we were aware that the woman concerned had a potential diagnosis of new variant CJD we removed the kit from circulation."

Mr Carver said the helpline, which can be contacted between 6am and midnight on 024 7686 5555, had received 70 calls today. He added that "advised scientific opinion" was that the risk of any of the women being infected with CJD was extremely low. The equipment which was re-used was properly washed and sterilised between operations. [Mr. Carver is very likely aware that hospital washing and sterilization procedures have little effect on transmissible prions and have failed in other instances.-- webmaster]

Earlier this month, Dr Rod Griffiths, the West Midlands' Director of Public Health, said the risk that the women had been infected with CJD during the subsequent births was "vanishingly small". Dr Griffiths added that staff at the hospital had followed proper procedures, removing the surgical instruments from use as soon as the mother's condition was known. The doctor added that it was theoretically possible for the prion which causes CJD to remain on instruments after operations, but it was usually removed simply by washing prior to sterilisation. [Dr. Griffiths is very likely aware that study has ever been conducted that supports this assertion, to the contrary.-- webmaster]

Asked to quantify the risk to those mothers exposed to the instruments, Dr Griffiths added: "When something has never happened, you don't know whether it's one-in-a-million or one-in-a-thousand."

The mother diagnosed as having CJD gave birth last autumn, but she cannot be named for legal reasons. Her baby daughter is also thought to be suffering from a neurological condition and doctors are understood to be awaiting the result of tests to see if she also has CJD.

The mother and baby were transferred to another hospital after the birth and staff at the George Eliot acted within hours of learning of the CJD diagnosis. [The mother displayed early signs of nvCJD prior to onset of pregnancy; subsequent severe symptoms were treated as depression. At least 11 months passed without correct diagnosis. -- webmaster]

CJD victims display symptoms of dementia, becoming unsteady, confused and losing their memory. Death normally occurs about six months after the onset of the disease. CJD is the human form of so-called mad cow disease, bovine spongiform encephalopathy. New variant CJD has been linked to the consumption of beef infected with BSE.

Village lives in fear of the 'dust of death'

To the people of Blyton, it is known as the dust of death. Their previously unremarkable village in the middle of Lincolnshire is now home to a depot which is going to store thousands of tons of granulated cattle, rendered down to manageable proportions as part of the Government's "mad cow" clampdown.

Parents are fearful for their children's future and, in the eyes of many, they have good reason. For what the long-term effects of exposure to the dust may be, no one knows for sure.

Dissatisfied with official reassurances, the people of Blyton have formed an action group and are picketing the storage site with placards bearing slogans such as "Blair's Dust of Death" and "Lincs says No to BSE Waste".

The dust, from Government stocks of Meat and Bonemeal (MBM), is officially described as an "infectious agent" and strict transport and storage regulations should keep it safety secured.

Yet this week protesters were able to scoop up a handful from the back of a transporter lorry. Rachel Marriott lives about 400 yards from the depot and has two children, Bethany, three, and Katie, 18 months.

She said: "Nothing we have been told convinces us that this stuff won't have a long-term effect on our children's health. We worry when they are in the garden. When the lorries are going in and out, the doors of the shed are open. I worry that the dust might be flying in and out.

"There must be safer ways of storing the stuff than in heaps. We have asked them to bag it up, but they said no, I suspect on the basis of cost." Blyton is being used as a staging post for the storage of thousands of tons of the MBM, which is going to be incinerated at nearby Glanford power station. The heat-rendered meat and bone - not only banned from the human food chain but also cattle feed and fertiliser - is protected by the official Control of Substances Hazardous to Health Regulations.

Sian Jaehrig, mother, solicitor and member of the action group, told The Express: "We have seen dust on the trailers as they come out of the site. They're not wearing protective clothing, they are being quite provocative.

"We are not saying 'You must never dump it here.' We're saying 'If you must dump it here, please do it in such a way as to make it safe for our village and particularly our children.'" A Health and Safety Executive spokesman said: "As an infectious agent MBM would be covered by three separate sets of regulations on the carriage of dangerous goods."

But a local manager for the "pollution control" company running the Blyton dump told The Express: "The material itself does not present any health and safety risk."

The intervention board, the agency managing the three-year disposal of 600,000 tons of MBM from more than 2.5 million culled cattle, has told residents that everything has been done to ensure safety as the waste is transported to the building on the edge of the village.

"Any person who carries controlled waste has a duty to take all reasonable measures to prevent the escape of waste," the board said. "The penalty for failing to comply is a heavy fine." The board's MBM operations manager also said: "All loose material will be removed from lorries before they exit the building. I do not accept that airborne contamination is inevitable."

But Steve Fisher, chairman of Blyth Action Group, was watched by The Express this week as he scooped up the equivalent of several teaspoonfuls of dust with his finger from the back of a lorry. Mr Fisher, who lives opposite the site and has three children, including a son Michael, 12, and two baby grandchildren, said: "This confirms what we have been saying.

"They know that the Press is here, and that local residents are outside. Even in circumstances when you would expect the lorries to be perfectly clean, they are not. This supports our case that these lorries cannot be cleaned to the extent we would wish, and bearing in mind the grey areas which still exist about this disease, this is not acceptable.

"We feel it also supports what we have asked for all along, which is for the waste to be bagged before it is transported." But Jack Cleghorn, sales and marketing manager for pH Pollution Control Ltd, dismissed local concerns, saying: "This is a 'not in my back yard' protest and other sites are operating in a similar fashion without protest." He told The Express: "There is a clean-down procedure for the vehicle inside the building before it leaves. Part of that process involves the use of air to blow any large amounts of material off the back end of the truck."

The official Advisory Committee on Dangerous Pathogens (ACDP) says in its Guidance for Handling Meat and Bone Meal Material that such debris should be removed by industrial vacuum cleaner, before being bagged and incinerated. The ACDP warns: "Rendering processes may not completely inactivate the BSE agent and there may be residual infectivity in MBM.

"The ACDP opinion is that minimising exposure is prudent. The most likely routes for entry are through dusty material contaminating wounds and open lesions on the skin, splashing of mucous membranes (eyes and mouth) or possibly accidental ingestion." So far, 52 people have died from variant Creutzfeldt-Jakob Disease, the incurable human version of Bovine Spongiform Encephalopathy, which has an incubation period of between four and 40 years.

While the intervention board says the risks from MBM are "negligible," the cull of all cattle over 30 months, rendering, storage and incineration have already cost billions, and the board has told Blyton residents: "Proper handling, according to HSE guidelines, will minimise any risk of infection to those handling the material, and is a requirement of pH's contract."

However, Mr Cleghorn said it was not possible - or necessary - to make sure there was no dust on the trucks leaving the site. "It has to be realised," he said, "we are dealing with a large piece of equipment. It can in no way, shape or form be 100 per cent guaranteed that no material whatsoever will leave the site.

"We have not given a guarantee of 100 per cent no dust leaving the building. That would be impractical. However, the villagers' concerns would suggest that they have concerns with the material itself, and we would re-emphasise that the material itself does not present any any health and safety risk.

"If there was a risk that the material posed which necessitated 100 per cent cleanliness, then we would be doing things differently. The material itself does not pose a risk which requires us to clean to that degree. That would be impractical and completely unnecessary.

"This is an over-30 months scheme. It is not BSE-infected material. It is removed from the food chain as a precaution. All the independent assessments suggest the material is safe and is not a risk to human health." But the intervention board says: "The Control of Substances Hazardous to Health assessment recognises that MBM is an organic material and, by its nature, has the potential to pose a very small risk of containing infections."

People working with MBM are supposed to wear disposable overalls, gloves, safety glasses and dust mask, wellington boots and hard hats and, in some cases, air-fed respirators.

Another villager, Tony Codd, says he and his son followed one of the trucks last week after it had emptied its load, and watched the end of its tarpaulin flapping in the wind. He said: "The tarpaulin on the top is supposed to be fastened all the way round with straps. But the straps at the back weren't fastened at all.

Pig farmers go to court for BSE aid

Tue, Mar 14, 2000 By John Aston, PA News
The Government is facing a High Court challenge over allegations that it is guilty of discrimination against the pig industry because of its failure to provide aid following the BSE crisis. A judge has ruled the pig farmers have "an arguable case" which should go to a full hearing as a matter of urgency.

Today Mr Matthew Atkin, chairman of the British Pig Industry Support Group (BPISG), announced the legal action was under way and welcomed the judge's decision to put it on the fast track. Mr Atkin said: "Expedition is important as the pig industry continues to suffer, with businesses going bankrupt all the time. We estimate that, up until the end of February, we have slaughtered 30% of the UK sow herd. In human terms that represents the loss of many thousands of jobs."

The group is applying for judicial review against agriculture minister Nick Brown, arguing that the Government has unlawfully and unfairly failed to provide funding to help it cope with its continuing losses as a result of the BSE crisis.

Mr Atkin said the EC Commission had recognised that the BSE crisis amounted to an "exceptional occurrence" justifying state aid. But the Government, while granting aid to the beef and sheep industries, was continuing to refuse similar funding to pig farmers. The BPSG will contend in court that Mr Brown is under a legal duty to grant aid in order to avoid acting in a discriminatory manner. Last Friday, Mr Justice Jackson considered the point arguable and gave leave to seek judicial review.

Mr Atkin said the cattle industry - and to a lesser extent sheep - had so far received nearly 2 billion pounds in aid. [This is a staggering amount of charity to industry and completely dwarfs expenditures on research for a cure for nvCJD. Victims' families have receives little or now help from the government and often must care for the affected family member at home.

Pig farmers estimated that BSE controls, applied since 1996, had so far cost them up to 270 million. This, along with the cost of higher animal welfare standards in the UK and sterling appreciation against the Euro, had made the industry disastrously uncompetitive, with imports flooding in from other countries.

Blair vows to look at aid for dairy farmers

Wed, Mar 15, 2000 By Martin Evans, Political Staff, PA News
The Prime Minister today pledged to do what he could to help the crisis-hit dairy industry. Mr Blair told the Commons he would meet farmers' representatives at the end of the month to discuss problems facing all agricultural sectors. His pledge during Prime Minister's Question Time came as dairy farmers across the UK were lobbying parliament over the crisis.

He said: "On March 30 I have a meeting with representatives of the farmers union and representatives of farmers from every part of the UK. "We will see what measures we can take to help both the dairy industry and other sectors like the pig industry that are experiencing real difficulty. But I do say, as I have constantly said, that there is a limit to the amount of public subsidy that we can give. We are putting in a very significant amount of money. We understand that the position has worsened particularly for the dairy sector recently and we will do what we can."

Liberal Democrat Andrew George said that dairy farmers had suffered because of the "gross negligence" of the Tories over BSE and the failure of the Government to protect them from exploitation from large processors and supermarkets. "Do you believe that eight pence a pint for our dairy farmers is enough for them to survive on?" he asked.

Mr Blair replied: "We are aware of the problems but we need solutions that are long term as well as solutions which simply get them over a short term crisis." He highlighted problems experienced by dairy farmers in Northern Ireland who had suffered disproportionately compared with their mainland counterparts. He said Northern Irish farmers had seen their income drop 79% since 1995 as compared with 60% in the UK..

Germany bows to BSE-beef imports

18 Mar 00 news wires

Minister Urges German States to Lift British-beef Ban

3/14/2000 AP
Germany's agriculture minister urged state leaders Tuesday to lift a ban on British beef when the issue comes to a vote in parliament's upper house, or risk high fines from the European Union, but several state leaders said they would not go along.

Agriculture Minister Karl-Heinz Funke warned in an interview published Tuesday in the Neue Osnabrueck Zeitung that Germany faces heavy fines if it does not lift the ban in line with an EU decision August 1. He said consumers could be assured that beef from Britain was safe, and that it would be clearly labeled as British. [This is false. British beef will simply be trans-shipped through Holland and will not be labelled. Who would buy it? -- webmaster]

Chancellor Gerhard Schroeder's Cabinet approved lifting the ban on February 2, after adopting stringent new EU rules for British beef. But it must be approved by the upper house, where the states are represented.

But the German states of Bavaria, Baden-Wuerttemberg and North-Rhine Westphalia reiterated their intention to vote against lifting the ban. The states of Saarland, Thuringia and Hesse also were expected to resist. The northern city-state of Bremen said it would let its representatives in the upper house, or Bundesrat, decide for themselves. Saxony state said its Cabinet was still debating how to vote. A majority of 35 votes is needed in the 69-seat Bundesrat, which includes Germany's 16 state governors and other state representatives.

The EU Commission said February 15 it had started legal action against Germany over its continued refusal to lift its ban. Legal action also has started against France, which has kept its ban in place. The EU introduced the British beef ban at the peak of the scare over so-called mad cow disease in 1996 after scientists established a link between bovine spongiform enceplhalopathy, then ravaging British cattle, and a fatal human brain disease

German state paves way to ending British beef ban

Reuters World Report  Wed, Mar 15, 2000
The German state of Saxony said on Wednesday it plans to support an end to a ban on British beef imports, in a move that should open the door for the federal authorities to lift the national embargo. A spokesman for the state health ministry in Saxony, one of 16 states represented in the upper house of parliament, told Reuters it had changed its position. Until now, the upper house had blocked government attempts to lift the ban, but the Saxon move should shift the majority in the government's favour.

The European Commission last year ordered a ban on British beef imports be lifted, but a majority of states -- which are responsible for food safety -- in the upper house cited fears of mad cow disease in preventing legislation from being passed. Chancellor Gerhard Schroeder's government has urged the states to drop the import ban, warning there could be heavy fines handed down from the EU if the embargo were not lifted. But until Wednesday opponents had enough votes to thwart that. "We will vote with the federal government's position at the Bundesrat meeting on Friday," a spokesman for the health ministry in Saxony said, referring to the next upper house session on Friday.

Reinhold Bocklet, Bavaria's minister in the Bundesrat, told Reuters there was now a sufficient majority of votes in favour of lifting the ban in the upper house. Bavaria is also voting to end the boycott. The EU opened legal proceedings against Germany last month because of the failure to lift the ban. France and Germany both failed to comply with an EU ruling that the block on British beef imports should be lifted after more than three years.

The commission's top scientific advisers have ruled British beef safe following France's claim of new evidence that it could not be guaranteed to be free of mad cow disease or BSE -- bovine spongiform encephalopathy. [There is no direct testing of British beef either in Britain or Germany, even though sensitive tests are now available. -- webmaster] Germany lifts ban on British beef

Fri, Mar 17, 2000 By Geoff Meade, European Editor, PA News in Brussels
Germany lifted the ban on British beef today - leaving France isolated as the only European country still blocking imports from the UK. A majority vote of the German parliament's upper house was welcomed by Europe's food safety commissioner David Byrne, who called a halt to legal moves launched against Berlin to force a resumption of beef trade with Britain.

The German decision will have little impact on British beef farmers, however. Germany imports about 100 tons of British beef a year and consumer resistance is likely to continue despite the opening up of markets. Nevertheless Mr Byrne hailed the result as a political boost to the Commission's campaign to restore British beef to the entire EU market place.

The ban should have been lifted on August 1 last year, when a three-and-a-half year trade blockade imposed by Brussels because of so-called mad cow disease came to an end.

Comment (Roland Heynkes To the person writing about the safety and control of baby food in Germany. and the use of SRM/SBO in baby food in the Germany system: no German law would inhibits the use of SRM/SBO in baby food although there may be a regulation. But relevant is what a company actually does. They often have their own regulations that are much more strictly and this is especially the case for baby food producers.

Hipp for example uses only German cattle that have had no meat bone meal or growth hormone inducers. But you can ask them if they also do not use milk exchangers, if they slaughter the cattle with or without bolt pistol and pithing, and if they use anything else than meat.

Brain material is allowed in some low quality and a few medium quality German meat prodducts. Baby food is of course a high quality product, but I am not sure if it is allowed to have brain tissue in it. By the way, babies can live and growth without any meat products. I tested this with my own daughter.

There is no German law that regulates the use of a pithing rod. Therefore even pure meat is likely to bear traces of brain if pithing was done in order to reduce the risk for the slaughterhouse workers. But even when only stunning with a bolt pistol was done without pithing, brain material could have reached the muscle.

Norway, Lithuania, Latvia, Estonia Ban Danish Meat

Wednesday March 11, 2000 AP
Norway banned most Danish meat imports and stepped up customs controls Wednesday, two days after neighboring Denmark reported a case of mad cow disease. Norway joined the three Baltic countries of Lithuania, Latvia and Estonia in barring imports of Danish beef. Major Swedish supermarkets began voluntarily pulling Danish meat products from their shelves on Tuesday.

Denmark on Monday recalled all meat products that could be contaminated by bovine spongiform encephalopathy, or mad cow disease, after finding the illness in a Danish-born cow.

Norway and the other nations want products stopped because contaminated meat has been linked to a deadly human brain ailment, Creutzfeldt-Jakob disease. The Norwegian Food Control Authority said it was banning many types of high-risk Danish fresh meat, including beef, sheep and goat products that contain bones and other organs that could harbor the disease.

Norway lifts mad cow ban on Danish meat imports

Reuters World Report  Tue, Mar 21, 2000
Norway said on Tuesday it had lifted a ban on Danish meat imports after Denmark showed it was ready to combat an outbreak of mad cow disease.

"Internationally, the Danish measures are considered very satisfactory," the Norwegian state food safety authority SNT said in a statement. The ban, which covered beef, mutton and goat meat, was introduced on March 1 after Denmark found one milk cow afflicted with bovine spongiform encephalopathy (BSE), the scientific name for the deadly brain-wasting disorder known as mad cow disease. It was the first case of BSE in Denmark since 1992.

Many Norwegians travel to Denmark on weekend ferry trips to stock up on everything from alcohol to meat, which is far cheaper than in Norway.

BSE scare leads to Saudi boycott of Danish milk

COMTEX Newswire Tue, Mar 14, 2000
The BSE scare is threatening to cost the Danish dairy group MD Foods half a billion Danish crowns per year. According to Danish radio news, reported by the news agency Ritzau, Saudi Arabia has suspended its cooperation with the dairy for fear of the disease.

The 'Mad Cow Disease' has not previously been associated with milk products and MD Foods is puzzled by the Saudi reaction. "It is the first time 'Mad Cow Disease' has been linked to milk products and we find it difficult to understand the boycott," MD Foods' head of information Peter Kjeldstrup told Ritzau. "'Mad Cow Disease' is not carried by milk." [Only a small amount of ineffectual research was ever carried out in milk -- webmaster.]

Although the dairy group has not been contacted directly by its Saudi partners the local authorities have issued a statement saying that Danish dairy products can no longer be imported into Saudi Arabia.

Oman bans Danish beef on ``mad cow'' fears

Reuters World Report Tue, Mar 14, 2000 
Oman has banned the import of Danish beef and dairy products due to fears of contamination by "mad cow" disease, a health official said on Tuesday. "We have already informed all food importers not to distribute Danish meat and dairy products and issued a public notice asking consumers not to buy these products," a senior health inspector told Reuters.

Other Gulf Arab states including the United Arab Emirates and Saudi Arabia have already banned beef imports from Denmark. Denmark said in February that a milk cow had been infected by mad cow disease, or bovine spongiform encephalopathy (BSE), a fatal brain-wasting disorder.

Pithing of cows banned in France

Reuters World Report  Tue, Mar 14, 2000
France's food safety agency approved on Tuesday a proposed ban on "pithing," a method widely used in slaughterhouses which experts fear may increase the risk of "mad cow disease" spreading to humans through infected meat. It involves firing a metal bolt into the brain to stun the animal before killing it.

Experts say the bolt could release prions, the proteins which cause mad cow disease or BSE, from the brain while the animal was not yet dead, allowing them to spread into the meat. The bolt, which is reused, might also carry the prions from one animal to another, the AFSSA food safety agency said in an opinion made public last week.

Meat from cattled infected with BSE -- bovine spongiform encephalopathy -- is blamed for human cases of new variant Creutzfeldt-Jakob Disease or nvCJD, a deadly, brain-wasting disorder for which there is no known cure. Slaughterhouses use pithing so that animals do not flail their limbs while being butchered, injuring workers.

Comment (Roland Heynkes): The older type of captive-bolt pistol uses an explosive charge that pushes the bolt through the skull, whereas newer types do this with compressed air or CO2. But the captive-bolt pistols of the US-company Hantover pneumatically inject air through the penetrating bolt into the brain. This destroys the brain to a much greater extend than the bolt alone.

Anil et al. write that "no pithing is required due to air injection into spinal canal" (Anil,M.H. et. al. Potential contamination of beef carcases with brain tissue at slaughter. - Veterinary Record 1999 Oct 16; 145(16): 460-2).

Opinion (webmaster): A ban on pithing is long overdue. Dr. T Garland's results on captive bolt stunning were absolutely appalling, big plugs of brain ending up in the lungs and circulatory system. Regardless of the slaughter method, nobody in their right mind should eat meat products from a BSE cow, even if the brain and spinal cord are carefully segragated.

France considers banning use of cows' intestines

March 11, 2000, Agence France Presse English, PARIS
Agriculture Minister Jean Glavany was cited as saying on Saturday that France is considering banning the use of cows' intestines in food because of fears that it could spread BSE or mad cow disease. The minister was cited as telling France Inter radio that an investigation should first be carried out at the European level, but "the ban will not come in the next few days."

Cows' intestines are, according to this story, used mainly as the skin for sausages, including chitterling sausages. A ban is already in place on a part of the large intestine, but France's food health agency (AFSSA) was cited as believing that other parts of the intestine could also bring risks.

The AFSSA earlier this month was cited as recommendingc a ban on a technique of cattle-slaughter known as pithing because of fears it could help spread Bovine Spongeiform Encephalopathy. As an interim measure until the ban is in place, the agency was cited as advising the government to ensure that the blood and lungs of cattle killed by pithing be removed from the human and animal food chains.

The agricultural ministry's food chief, Marion Guillou, was cited as tellling the same radio programme that France planned "restrictive measures on trading and importing" cows' intestines, adding, "It would be illogical to forbid French intestines and allow intestines from other countries with equal or higher risks to be brought in," also calling for a harmonisation of the European position.

A ministry spokesman was cited as telling AFP that the French government was discussing the issue with the "concerned parties" and a ban would only come into place if no other alternative could be found. No other European Union member state is, according to this story, currently planning to ban the use of the entire intestine. Glavany also was cited as telling France Inter that he wanted a more coordinated effort in Europe to battle BSE. The current approach to the disease varied from country to country, he said.

Organs taken from 800 children without parental permission

17 March 2000 By Ian Herbert, Northern Correspondent, L. Times
Opinion (webmaster): This story raises the issue of organs possibly taken for sale in transplants. In the US, no permission is needed from the family and items such as corneas are taken from cadavers in large numbers. There is a definite risk of spreading CJD in this manner because obviously no family history is taken.

The chairman of a Liverpool children's hospital which stockpiled the organs of more than 800 children without their parents' permission resigned yesterday after an admission that a child's heart, brain and lungs have been lost.

The Health minister Lord Hunt of Kingsheath demanded and received the immediate resignation of Frank Taylor from Alder Hey hospital after the parents of 10-day-old Stephen White-Conlin, who died eight years ago, were told that the organs could not be found, 48 hours before the second funeral they had planned for him this afternoon.

Lord Hunt demanded a report into the lost organs by the end of today and said the treatment of the child's family was "totally unacceptable". Lord Hunt said: "I will not tolerate this kind of poor management in any NHS hospital."

Stephen's mother, Julie White, 33, of Crosby, Merseyside, said undertakers had informed her on Tuesday that Alder Hey was ready to release the organs. "I am disgusted. It is just like reliving everything," she said.

The White-Conlins found out only last year that Stephen's organs had been removed ands aid his second funeral was planned after the hospital had told them, in September, that the organs were available.

The hospital was unable to explain how it could have lost them in the intervening six months. "At this stage, we cannot say precisely what happened but we believe these organs, which were very small, have been mistakenly disposed of," a spokeswoman said.

Last December, Alder Hey apologised for the removal of organs, without parental permission, by a pathologist, Professor Dick van Velzen. The organs, from children who died between 1988 and 1995, were stored in a cellar.

A public inquiry into the affair ordered by the Secretary of State for Health began hearing evidence last month and the Chief Medical Officer is investigating the issue of organ retention. The hospital has returned organs to 250 families since September.

UK necropsy guidelines updated in wake of organ-retention scandals

Lancet 2000; 355: 1082 - 1084
The UK's Royal College of Pathologists issued new guidelines for the retention of tissues and organs at necropsy on March 23, which includes a new model consent form that gives relatives the right to agree or refuse permission for organs of the deceased to be used for research, examination, or education.

The guidelines were issued partly in response to heightened public and media interest in organ-retention procedures from the hearings into paediatric cardiac surgery at the Bristol Royal Infirmary (see Lancet 1999; 353: 987) and the recent disclosures from the Alder Hey hospital (see Lancet 1999; 354: 2055). Last year it was revealed that hundreds of children's organs were retained for research at the Alder Hey hospital without the parent's consent.

The College now recommends that hospitals and medical schools must provide training for doctors and other health-care staff in requesting and obtaining agreement for necropsies and in dealing with relative's concerns about tissue and organ retention.

Hospital pathology practices questioned in Ireland. Irish police are continuing their investigation into what the chief executive of the Coombe Women's Hospital in Dublin called a "sinister" incident when the remains of seven miscarried foetuses were reported stolen on March 16 and later found hidden in the hospital mortuary.

The Parents for Justice group, which pushed for a public inquiry into hospital pathology practices at all Irish hospitals following the discovery that their dead children's organs were removed without consent, said the incident was another blow to their confidence in hospitals. The Minister of Health is expected to give the details of the inquiry into necropsy practices within days. Karen Birchard

Doctors must also liaise with pathologists to determine the reasons for retaining tissues so that relatives can make informed decisions. The College has drafted an information pamphlet for relatives to explain the medical benefits of tissue and organ retention and relatives of the deceased will now be given a copy of the signed consent form.

The College suggests that relatives are given a wide range of necropsy options on the updated consent form, which now allows relatives to limit the extent of the necropsy; agree or disagree to have organs being taken for further examination; have tissue or organs disposed lawfully and respectfully by the hospital or taken away for personal burial; donate tissue, fluids, or organs for research for an unlimited period;

Last week, the chairman of the Alder Hey hospital, Frank White, resigned after doctors at the hospital admitted they could not find the organs of a baby boy that had been removed without the parent's consent.

Opinion (Terry S. Singeltary Sr, Bacliff, Texas USA): Most of you will remember the debate myself and the Cleveland Eye bank had, and them taking cornea's from victims without their consent, and passing those potentially TSE infected corneas to someone else, thus passing it to who knows who, through the medical instruments used to transplant those potentially infect corneas. As we now also know, they are doing this all across the U.S., and evidently in most other countries. Also, as we now know, they are allowed to legally take any organ without consent in the U.S., due to the legal loopholes.

Although by doing this, without the knowledge of the family's past medical history, not knowing whether any family history of CJD or other disease, due to the way the organs were taken, no formal questionnaire about past medical history could have been done. Would it not be reasonable to be concerned from these organs, due to what little we still know about human/animal TSE? Should there be a questionnaire regarding these issues, before the organs are harvested or stolen?

We sentenced Rachel to death, say CJD girl's parents

Wed, 16 Feb 2000  By Tahira Yaqoob, PA News
A 33-year-old woman was "sentenced to death" by her parents when she contracted a rare form of CJD from the medical treatment they put her through. Rachel Gwilliam, of Drybrook, Gloucestershire, was injected with a growth hormone from the age of seven to 16. But unknown to doctors and her family, she was injected with a hormone contaminated with Creutzfeldt-Jakob Disease, the disease commonly associated with BSE.

Today Rachel's parents, Brenda and Roger Preece, said after an inquest into her death: "We in ignorance sentenced our daughter to death. We are left with nothing except guilt that we agreed to the treatment."

Gloucester Coroner's Court heard mother-of-one Rachel's condition was not linked with mad cow disease. Professor Seth Lane, a CJD expert from Frenchay Hospital in Bristol said Rachel was one of just tens of cases to have contracted the rare strain. Rachel's family are now involved in a battle for compensation from the Department of Health for her death from the disease.

Prof Lane told the hearing the growth hormone Rachel was given from 1973 to 1982 was extracted from people who had died. He said: "It needs just someone who had undiagnosed CJD to pass on the disease. It was not the human growth hormone that was the problem, it was the contaminant. The relative number of deaths that might have been occasioned by this is tens of cases. [This is a massive display of ignorance from someone claiming to be an expert on CJD. --webmaster]

The Preeces' lawyer Jonathan Glasson told the hearing he knew of 35 similar cases including a series of eight in Edinburgh and one in Southampton.

Mrs Preece told the coroner's court she first took Rachel for treatment when she showed no signs of growing as a toddler. As a one-year-old, Rachel weighed just 15 lbs, despite weighing a healthy 6lb 7oz when she was born. After consulting a paediatrician, Rachel was put on a growth hormone treatment in 1973. Mrs Preece told the hearing she injected the treatment herself at six-month intervals.

But she claimed the only side effect she was told about was the possibility of Rachel becoming infertile. She said: "It was a difficult position. Our GP drew attention to the problems Rachel would have by being short. We must have signed a consent form. I cannot remember being made aware of any other risk."

Rachel made trips for scans and X-rays at Great Ormond Street Hospital in London. She carried on the treatment until 1982 when she was 5ft 2ins tall, the hearing heard. But it was not until the mid-80s that Mrs Preece heard that the drug Rachel was injected with had been withdrawn in America after a health scare.

She said: "I got the impression there had been two deaths. I told my husband and we decided not to tell Rachel. We were all too aware if there was a need for anxiety, it was too late so why worry Rachel."

But she told the inquest Rachel spotted a newspaper article in 1991 and went to see her doctor, fearing for her life. Mrs Preece said she was angry with her parents for not telling her. But the family decided she was over the worst when Rachel reached the age of 30. Mrs Preece said: "We thought we were home and dry."

Her daughter began developing problems with her balance and her work deteriorated after Christmas 1997. Mrs Preece told the inquest she became tired, performed badly at work and by September 1998, she was unable to drive. Her health took a worse dip when she became confused, could not feed herself and had difficulty walking and carrying things.

Her family began to worry that Rachel's son James, who was then just two years old, was not safe as she seemed unable to cope. The inquest heard Rachel was eventually diagnosed with CJD in October 1998 after she became incontinent and was frequently falling over. She was admitted to Dilke Memorial Hospital in Cinderford, Gloucestershire, when her family realised she was going to die.Rachel died six months later in October 1999 of pneumonia triggered by CJD.

Gloucester coroner David Gibbons recorded a verdict of death by misadventure. He said: "Medicine has come a long way but how often do we stop and consider the human cost and suffering of these advances? What started as being a most successful treatment and gave joy to her family has turned so tragically into her death."

CWD deer, elk not wasting Utah hunters

Salt Lake Tribune  07 Mar 00 BY TOM WHARTON
Utah deer and elk hunters apparently don't have to worry that the animals they are killing might in turn kill them. A study in Utah, Maine, South Dakota and Oklahoma released Tuesday showed no trace of chronic wasting disease in deer and elk.

According to the Utah Department of Agriculture, the tests were conducted in response to concerns that the deaths of three hunters in Utah, Maine and Oklahoma might have been related to CWD infected deer and elk. All three hunters died of the degenerative nerve disorder known as Creutzfeldt Jakob disease (CJD). A variant of CJD is associated with mad-cow disease.

Hunters in Utah became concerned two years ago when news reports linked the death of a Kaysville man, afflicted with CJD, and the consumption of game with chronic wasting disease. The Utah study included 404 Utah deer and 196 elk. All tests for chronic wasting disease were negative.

"These test results should lay to rest any notion that the Utah wildlife hunter died from his contact with Utah deer and elk," said State Veterinarian Michael Marshall. "There are few deer and elk in the country that are infected, and there is no connection between chronic wasting disease in wildlife and CreutzfeldtJakob disease in humans." [NE Colorado is reporting up to 15% of its deer have CWD. No one has the slightest idea whether CWD can transmit to humans nor how long the incubation period is. No tonsil surveys have been conducted in Colorado hunters or in consumers of game farm elk. -- webmaster]

The two diseases produce similar symptoms in animals and humans but are considered separate diseases. Both are forms of spongiform encephalopathy that produce vacuoles, or holes, in the brain. Testing requires collecting a brain stem from animals at least 2 years old. Biologists from the Utah Division of Wildlife Resources operated checkpoints during hunting season to collect the samples.

Bill Morrill, president of the Nevada-based Mule Deer Foundation-- which is holding its annual meeting next week in Salt Lake City, said the results should be good news for deer hunters. "Hunters have the main interest in mule deer and supply most of the money for management of mule deer," he said. "I would hate to see anything that would hurt hunting and knock a hole in state game agencies' ability to fund management." Some cases of chronic wasting disease have been found in elk and deer in a nine-county area in southeast Wyoming and northeast Colorado.

Marshall said many Utah hunters call his office each year about abnormalities they observe in deer or elk. "Quite a few hunters called last fall that may have had a question about an animal that didn't appear too healthy," he said. "We advised them not to eat those."

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