Tonsil tests: minimum of 400,000 nvCJD cases
House of Lords probe of nvCJD
House of Lords debates 'sizeable nvCJD epidemic'
Minister warns of wait on CJD facts
Evidence of a CJD epidemic may still be missed
Tragic time in Japan: 100,000 Japanese received dura mater
French farmers furious at feed makers
EU to propose compulsory mad cow disease tests
Butchers face new rules on meat
April 9 2000 London Sunday Times Jonathan Leake, Science EditorScientists have moved one step closer to confirming that thousands of Britons could be infected with variant CJD, the human form of "mad cow" disease (BSE).
A government-funded study of appendix and tonsil tissues taken from 2,000 people since the 1980s is understood to have found a small number of samples which tested positive for the infective prion particles that cause the disease. If confirmed, the results suggest the number of people who might get the disease could reach thousands.
The researchers are, however, thought to be worried about the accuracy of the tests and are to carry out checks before announcing them to a government committee next month.
| Details of the work were discussed last week when
researchers into CJD, BSE and other prion diseases held a
private meeting at Keele University.
The event, organised by the agriculture and health ministries and by the government's Biotechnology and Biological Sciences Research Council, was conducted amid unprecedented security. It was not announced to the press and every scientist attending had to pledge not to reveal anything. The study was ordered to try to find out how many people were at risk of contracting variant CJD. Half of it is being carried out by Professor James Ironside at the CJD national surveillance unit in Edinburgh, and the other half is being done at Derriford hospital in Plymouth by Dr David Hilton. Both men attended last week's meeting and are understood to have held discussions with other scientists on the nature of their results. The research followed the death of Tony Barrett, a Devon coastguard, who died of variant CJD in 1998. His appendix had been removed in 1995. When it was re-examined after his death scientists found abnormal prion protein - showing that his illness could have been diagnosed before he became ill. |
Since then the two research centres have been collecting tonsil and appendix samples removed between the mid-1980s and late 1990s - the period when exposure to BSE-contaminated meat was at its peak. [Some 800,000 samples of tonsil and 45,000 samples of appendix have been removed and stored. -- webmaster]
If the tests do find any positive cases of variant CJD then the implications would be serious. Extrapolating the result to the population as a whole suggests that for each positive result picked up by the study, 30,000 people in the population would get the disease.
So far there have been more than 50 deaths from variant CJD with at least a dozen more people currently dying from the disease. Among them is a woman who gave birth just before she was diagnosed and whose child is now suspected of being the first case in which the disease has passed from mother to child.
Opinion (webmaster): This bizarre scientific meeting, at which mighty oaths of secrecy were sworn by all participants, took place on the secret dates of April 4, 5, 6 at Keele University. Little if any new science was presented according to attendees.
In fact, a specific number of infected tonsils and appendices was not given out at any of the sessions. No commitment has been to made to go public with the numbers next month after it is reviewed by various ethics and scientific committees. In other words, if they are bad, no word; if they are low, hold a press conference. Any suspicious cases at all in such a small sample is very bad news.
Earlier, Kenneth Calman, the government's chief medical officer said "If just one in 1000 cases proves positive, in a population of 50 million, that would imply that 50,000 people are infected." [Science 281 Sep 1998, pp. 1422].
Unfortunately, the situation is worse than this. A "small number" testing positive is consistent with the 8-10 range, meaning 300,000 to 500,000 cases (assuming half the positives are detectable with current technology). Other cases could be progressing but not be within the sensitivity limits of the tonsil test or be infected by other routes not manifesting in tonsils.
A great many delays have taken place. The use of tonsils as an early detection and survey tool was introduced 40 months ago [Hill AF et al., Lancet 349, # 9045 11 January 1997] but the government fought a long and bitter battle to keep the scope of the epidemic unknown. Many people will die unnecessarily because of the 15 years of interference with scientific work and underfunding of therapy research.
Will we see another 15 years of lies and denial or have things finally reached a turning point?
Update on nvCJD baby (webmaster): The reporter is in close contact with both family and researchers. On 11 Apr 2000 he posted this clarification to the BSE listserve:
"Just to be clear: There is still NO confirmation that the baby in question has CJD. She has had two MRI scans and a variety of blood tests and these are said to have shown strong indications of the disease but cannot be considered definitive.
I am told by her family that John Collinge, the Medical Research Council's professor of prion research who is involved in treating her, still believes she is likely to have CJD. He has, however, been unable to confirm or deny this because, as one would expect, he is unable to discuss individual patients.
There is a possibility that a tonsil or other biopsy - to which the family has agreed - will tell them one way or the other but I am told the child is currently too ill for this to be done safely. Additionally, her mother has deteriorated and the next few weeks and months could be the last they will have together. For both these reasons, I am told, the operation has been put back indefinitely
In the midst of the speculation it is important to remember that there are also many reasons why the baby may not have CJD. Among them is the fact that - so far as I know - no other prion disease of animals or humans is known to affect the young without an incubation period. Just this one fact alone would make it astonishing if this child really has been born with fully-fledged CJD. But maybe someone out there knows differently?"
Comment (webmaster): The number of babies born each year in the UK with undiagnosable neurological disease to healthy young mothers is very small, perhaps one in 5,000. Advanced prion disease in the mother might very well cause a loss of normal prion function or secondary brain or placental dysfunction that could adversely affect the baby without transmitted prion disease being responsible. The baby could survive the initial problem only to succumb to nvCJD at a later date. But these distinctions between direct and indirect disease are in many ways moot to the families involved.
Last update: 08 May 00. Questions posed by Lord Lucas of Crudwell and Dingwall, Organizer of an April 2000 debate in the House of Lords on the size of the nvCJD epidemic. Full text of debate: on-line version of Hansard by 4 Apr 00. See also continuation (question 44 and beyond)Question 1: Whether they will place in the Library of the House, or on the Internet, full details of each of the persons who have died in the United Kingdom of new-variant CJD, showing (a) the date of death; (b) the age at death; (c) the age at onset of symptoms; (d) identified exposure to possible sources of infection; and (e) the genetic makeup of the prion genes. (HL1549)
Response: The Parliamentary Under-Secretary of State, Department of Health (Lord Hunt of Kings Heath):
"To protect patient confidentiality, precise details cannot be supplied but the following information may be helpful: Table 1; new variant Creutzfeldt Jakob Disease (nvCJD) cases in the UK, by month and year of death, date of onset of symptoms and date vCJD was confirmed. To date the National CJD Surveillance Unit has no record of a case of new variant Creutzfeldt Jakob disease having arisen as a consequence of medical or surgical intervention."
| Case | Onset | Confirmed | Death | Duration | Trend |
|---|---|---|---|---|---|
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 | 06/94 01/95 12/94 02/94 07/94 08/94 03/95 01/95 08/95 01/94 01/95 07/95 12/95 12/95 12/94 10/95 03/96 03/94 03/96 01/96 11/96 10/96 01/96 02/97 09/95 03/96 05/96 07/97 07/97 05/96 10/97 11/97 07/97 11/97 11/97 04/98 03/98 05/98 08/97 12/97 02/98 12/97 12/97 10/97 05/98 06/98 01/99 12/96 07/98 01/99 05/98 04/99 | 09/95 01/96 01/96 01/96 01/96 03/96 03/96 02/96 03/96 04/96 07/96 09/96 * 06/97 07/97 05/97 02/97 06/97 09/97 06/97 10/97 11/97 09/96 03/98 05/98 06/98 05/98 10/98 10/98 10/98 11/98 11/98 11/98 12/98 02/99 02/99 * 10/99 02/99 03/99 02/99 09/99 06/99 05/99 07/99 08/99 11/99 09/99 01/00 12/99 12/99 02/00 | 05/95 11/95 11/95 01/96 01/96 02/96 02/96 02/96 05/96 06/96 06/96 09/96 11/96 02/97 02/97 03/97 03/97 05/97 05/97 06/97 07/97 10/97 12/97 01/98 03/98 04/98 05/98 08/98 08/98 10/98 10/98 10/98 10/98 10/98 11/98 11/98 11/98 12/98 12/98 12/98 01/99 02/99 02/99 02/99 07/99 08/99 08/99 09/99 10/99 10/99 11/99 01/00 | 11 10 11 23 18 18 11 13 9 29 17 14 11 14 26 17 12 38 14 17 8 12 23 11 30 25 24 13 13 29 12 11 15 11 12 7 8 7 16 12 11 14 14 16 14 14 7 33 15 9 18 9 |  |
*Cases not confirmed neuropathologically and therefore classed as 'probable nvCJD'
Duration in months, computed from supplied data, averaged 15.5 months.
Forty nine of the fifty two 'definite' cases to date have been methionine/methionine homozygous at codon 129, 3 cases not tested.
Results on one of the remaining cases are still awaited.
In the two remaining two cases no blood was taken on which to conduct the appropriate genetic tests.
Newly released data on confirmed but still living: case # date of onset 53 11/96 54 02/98 55 06/98 56 07/98 57 07/98 58 10/98 59 02/99 60 03/99 61 04/99 62 05/99 63 05/99 64 06/99 age at onset (case number not known): average 24.1 years, std dev 7.3 12/08 15/10 18/02 19/00 21/11 22/09 24/01 25/01 29/10 30/04 33/04 36/07[The following age at onset/age at death/duration in months data was also released for 36 nvCJD victims in a recent article: Lancet 2000 Apr 22;355(9213):1412-8
12.2 15 33
14.8 16 14
13.8 17 38
16.9 18 13
18.0 19 12
18.1 19 11
19.1 20 11
19.4 20 7
18.9 20 13
20.8 22 14
23.0 24 12
21.9 24 25
23.8 25 14
23.9 25 13
26.7 28 16
28.4 29 7
27.6 29 17
28.1 29 11
28.1 29 11
27.1 29 23
29.2 30 10
28.5 30 18
30.1 31 11
31.1 33 23
33.0 34 12
33.8 35 14
35.0 36 12
34.8 36 15
38.1 39 11
37.8 39 14
39.5 41 18
40.4 41 7
43.0 45 24
47.6 50 29
50.1 51 11
51.1 52 11
30 15.4 averages
10 7.1 std deviations
Question 2: What level of sterilisation they believe to be necessary to rid surgical instruments of the risk of carrying Transmissible Spongiform Encephalopathies; and whether they will place in the Library copies of the principal scientific papers underlying such belief. (HL1526)
Response: The Parliamentary Under-Secretary of State, Department of Health (Lord Hunt of Kings Heath):
"The nature of the Transmissible Spongiform Encephalopathy (TSE) agent is such that conventional methods of sterilisation such as autoclaving cannot always be relied upon to inactivate the agent completely. The Spongiform Encephalopathy Advisory Committee in September 1999 advised that rigorous implementation of washing, decontamination and general hygiene procedures were key measures in minimising the risk of TSE infection.
The nature of the Transmissible Spongiform Encephalopathy (TSE) agent is such that conventional methods of sterilisation such as autoclaving cannot always be relied upon to inactivate the agent completely. The Spongiform Encephalopathy Advisory Committee in September 1999 advised that rigorous implementation of washing, decontamination and general hygiene procedures were key measures in minimising the risk of TSE infection.
The Advisory Committee on Dangerous Pathogens (ACDP) / Spongiform Encephalopathy Advisory Committee (SEAC) publication "Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection", 1998, copies of which are available in the Library provides guidance on cleaning, decontamination and waste disposal and cites the following scientific papers:"
1. Taylor D M et al (1994). Decontamination studies with the agents of bovine spongiform encephalopathy and scrapie. Arch Virol 139 313-326.2. Taylor D M et al (1997). Inactivation of the 22A strain of scrapie agent by autoclaving in sodium hydroxide. Vet Microbiol 58 87-91.
Question 3: Whether gelatin, tallow, blood and protein of animal origin are permitted ingredients in feeds for cattle; and whether to the best of their knowledge any such ingredients are in current use in the United Kingdom.
Response: The Minister of State, Ministry of Agriculture, Fisheries and Food (Baroness Hayman):
"In the UK, a ban on feeding ruminant protein to ruminants was introduced in 1988. Since June 1994, the EU has prohibited the feeding of mammalien protein to ruminant species in all Member States including the UK.
Gelatin and blood products are exempt from the feed ban in both EC and UK legislation. Tallow, being fat rather than protein, is also a permitted ingredient in cattle feed. Tallow and gelatin for inclusion in animal feed, however, must be produced to standards laid down in Community law. Such materials are not widely used in animal feed in the UK."
Question 4: Whether the practice of "pithing" cattle at slaughter results in the contamination of the carcass with brain material; and, if so, what is the level of such contamination. (HL 1523)
Response: The Minister of State, Ministry of Agriculture, Fisheries and Food (Baroness Hayman):
"The Government has funded research which found evidence of contamination of jugular blood by fragments of brain tissue in one out of 16 animals which were pithed following stunning. That research did not investigate whether any traces of brain tissue could be transported in the blood to the rest of the carcase. The results were published in the Veterinary Record of 16 October 1999 [Anil MH, et al. Potential contamination of beef carcases with brain tissue at slaughter. Vet Rec. 1999 Oct 16;145(16):460-2. ].
The Spongiform Encephalopathy Advisory Committee reviewed the research findings and advised that there is no reason on the basis of current data to change UK practices of stunning and pithing during slaughter of cattle."
Question 5: Whether they still expect BSE to "dwindle to insignificant proportions by the year 2001" (www.maff.gov.uk/animal/bse/index.html); or, if not, what are their current projections for the likely number of cases in United Kingdom cattle for each of the next ten years. (HL1698)
Response: The Minister of State, Ministry of Agriculture, Fisheries and Food (Baroness Hayman):
"Projections of the number of BSE cases, derived from the Veterinary Laboratories Agency's (VLA) computer model, indicate that the epidemic will continue to decline. The latest VLA forecast as at 4 January 2000 is given in the table below.
The expectation is that the outcome, as in recent years, will tend to be closer to the upper 95% confidence interval. Because of the increasing unreliability of more distant projections, neither the VLA nor the Wellcome Trust Centre for Epidemiology of Infectious Diseases make predictions beyond 2001."
| Year | Confirmed | 95% confidence |
|---|---|---|
| 1999 | 2083 | 1774-2392 |
| 2000 | 1114 | 889-1339 |
| 2001 | 470 | 325-615 |
Question 6: Whether there is the potential for transmission of Transmissible Spongiform Encephalopathies through the use of inadequately sterilised dentists' instruments. (HL1524)
Response: The Parliamentary Under-Secretary of State, Department of Health (Lord Hunt of Kings Heath):
"Although the possibility of person to person transmission of any theoretical Transmissible Spongiform Encephalopathy infectivity through the use of surgical instruments cannot be ruled out, the National CJD Surveillance Unit, as they investigate cases of CJD, undertake an examination of medical histories to look for common risk factors.
Analyses of cases to date do not provide evidence to suggest any increased risk with past dental surgical intervention. Nonetheless, guidance has been formulated on appropriate prudent practice, which is available from the BDA, and which takes into account advice from the Joint Working Group of the Spongiform Encephalopathy Advisory Committee and the Advisory Committee on Dangerous Pathogens. In addition, a Health Service Circular on best practice on decontamination of medical devices was issued in August 1999 to healthcare organisations including Dental Postgraduate Deans and Regional Dental Advisors."
Question 7: Whether they are aware of any cases in which it appears possible that a Transmissible Spongiform Encephalopathy has been transmitted by a human mother to her child in utero; and, if so, what is their current state of knowledge of such cases. (HL1525)
Response: The Parliamentary Under-Secretary of State, Department of Health (Lord Hunt of Kings Heath):
"The National CJD Surveillance Unit monitor the onset of all cases of Creutzfeldt Jakob disease in the United Kingdom. No confirmed or probable case has been reported to the Unit of a baby being affected by this disease.
Epidemiological evidence from Kuru - a human TSE occurring amongst the Fore people of Papua New Guinea - suggests human maternal transmission of that TSE disease does not occur. However animal studies have shown maternal transmission can occur in scrapie in sheep and there is evidence of a low rate of vertical transmission of BSE in cattle. We will continue to liaise closely with scientists at the National CJD Surveillance Unit in Edinburgh to monitor the onset of all cases of CJD and seek to identify any common features."
Question 8: Whether there is any evidence that any Transmissible Spongiform Encephalopathy in any species can be transmitted through blood; and whether they will place in the Library of the House copies of the principal relevant scientific papers. (HL1545)
Response: The Parliamentary Under-Secretary of State, Department of Health (Lord Hunt of Kings Heath):
"Some animal studies have shown that certain transmissible spongiform encephalopathies can be experimentally transmitted from animal to animal through blood components. However, the Spongiform Encephalopathy Advisory Committee at its February meeting reviewed recent research undertaken in this area and did not consider any measures were necessary, in addition to those already in place, to reduce any potential risk to public health from human blood and blood products.
Copies of the following relevant scientific papers are being placed in the Library:"
Brown P, 1995, "Can Creutzfeldt-Jakob Disease be transmitted by Transfusion?" Haematology 2: 472 - 477.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 CJD in humans. Transfusion Vol. 39, November/December 1169 - 1178.
Question 9: What progress has been made on evaluating the capillary electrophoresis test, developed by Mary Jo Schmerr of the National Animal Disease Center of the United States Department of Agriculture, for abnormal prions in blood; what tests are underway; and when it is expected that they will be completed. (HL1546)
Response: The Parliamentary Under-Secretary of State, Department of Health (Lord Hunt of Kings Heath):
"The test currently under development by Dr Schmerr has undergone some preliminary evaluation at: the Medical Research Council Prion Unit, London; the National Creutzfeldt-Jakob Disease Surveillance Unit, Edinburgh; and the Veterinary Laboratory Agency, Weybridge. An application for a full evaluation of this, and other, blood tests is being considered by the Government. Before a diagnostic test such as this can be used to determine whether people or animals are infected with a transmissible encephalopathy agent at a pre-clinical stage, it will be necessary for the test to be fully developed and scientifically validated. We are not yet able to predict when any of these blood tests currently being developed will be completed."
Question 10: What progress has been made on the programme of screening tonsils removed in routine operations for the presence of abnormal prions; and whether they will publish the results of the screening to date. (HL1547)
Response: The Parliamentary Under-Secretary of State, Department of Health (Lord Hunt of Kings Heath):
"Two retrospective studies to examine samples of tonsil and appendix tissue taken from 15,000 patients in the South West of England and 3,000 in the Lothian Region of Scotland for the presence of abnormal prions commenced in the spring of 1999.
The first preliminary results from these studies are likely to be available during the spring and will then be considered by the Department of Health, the Medical Research Council and relevant scientific experts including the Spongiform Encephalopathy Advisory Committee. An announcement will be made about the findings.
An additional prospective study proposed by the London Prion Unit at St Mary's Hospital, to look at 2,000 tonsil specimens, has recently obtained ethical approval from the London Multi-centre Regional Ethical Committee and this study should be under way shortly."
Question 11: Whether Mr Rod Griffiths, the West Midlands director of public health, has been correctly quoted as saying (in respect of instruments used in subsequent operations having been used on a woman with new-variant CJD) that the risk to other women was vanishingly small, as the prion that caused CJD was removed simply by washing before sterilisation; and, if so, whether they agree with him. (HL1548)
Response: The Parliamentary Under-Secretary of State, Department of Health (Lord Hunt of Kings Heath):
"We are unable to comment on the accuracy of the reporting of any statement by Professor Griffiths. However the George Eliot Hospital NHS Trust has advised that Caesarean section itself is considered to be low risk surgery for transmission. The Government agrees with advice from the Spongiform Encephalopathy Advisory Committee that rigorous washing, decontamination and general hygiene practices with regard to surgical instruments are key measures in minimising the risk of any infection being transferred from patient to patient.
To date there is no known case of variant Creutzfeldt Jakob disease having arisen as a consequence of medical or surgical intervention."
Question 12: Whether they are prepared to provide independent researchers with authenticated samples of new-variant CJD DNA; and, if so, what procedures such a researcher must go through, and what criteria they must satisfy, before such samples will be provided (HL1550)
Response: The Parliamentary Under-Secretary of State, Department of Health (Lord Hunt of Kings Heath):
"The Government have recently agreed over 1 million pounds to set up a biological resource centre at the National Institute for Biological Standards and Control to supply validated infectious material for the study of both classical and variant Creutzfeldt Jakob Disease. It is intended that this centre will also supply blood and other appropriate tissues for research. All these research materials will be obtained in full accordance with the Human Tissue Act 1961.
Any researchers are at liberty to apply for validated infectious material, but must be bona fide establishments adequately resourced to conduct the work and their proposed projects must be subject to the usual peer review mechanisms. Applications should be made in writing to the Director of the Centre (Dr. Philip Minor or telephone: +(44) 1707 646399 )."
Question 13: What research they are funding into (a) the normal function of prions; (b) early diagnosis of prion diseases; and (c) rogue conformer therapy; and what is the annual cost to the Government of such research. (HL1612)
Whether they will host a central prion disease research website to carry material such as detailed and current survey information, planned and ongoing experiments (including daily lab book entries) and meeting transcripts, to expedite the pace of discovery. (HL1614)
Response: The Parliamentary Under-Secretary of State, Department of Health (Lord Hunt of Kings Heath):
"All transmissible spongiform encephalopathy (TSE) research projects, current and completed, and funded by public monies, are already listed on the Medical Research Council website. The site provides a breakdown of the areas of research being covered, including those identified in the question, as well as the annual costs. The total Government funding on TSE research for 1999/00 is likely to be in excess of 26 million pounds.
The Spongiform Encephalopathy Advisory Committee (SEAC) routinely considers all relevant research findings from work on TSEs, whether funded by the UK Government or not, including any research topics considered by the Committee's sub-Groups. SEAC produces a public summary of each of its meeting. These are placed on the Ministry of Agriculture Fisheries and Food and Department of Health websites.
The Government believe that in the TSE research area, publication of the full detail of ongoing experiments such as entries into lab books would be counterproductive. The provision of such invalidated information outside its context and without suitable peer review would run contrary to accepted scientific procedures, encourage misinterpretation, add to costs, and impede rather than facilitate constructive research. It would also discourage researchers from bidding for future projects."
Question 14: What blood functions or derivatives, made from United Kingdom donated human blood, and "pooled" so that a given sample may contain elements from many different donors, are in current use in the National Health Service. (HL1615)
Response: The Parliamentary Under-Secretary of State, Department of Health (Lord Hunt of Kings Heath):
"All blood products manufactured by the National Health Service plasma fractionation centres are sourced from pooled non-UK plasma. However Fresh Frozen Plasma (FFP), a blood component for transfusion is sourced from UK donated blood in the same way as red cells and platelets. Like red cells FFP is not pooled before use, although samples from up to 10 donor units of FFP are pooled to enable Nucleic Acid Amplification testing for the earlier detection of hepatitis C infection."
Question 15: Further to the Written Answer by the Baroness Hayman on 17th March (HL1522), which test was used in the survey of 4,163 cattle; and (a) what were the results of this survey or (b) when will these results be made public. (HL1616)
Response: The Minister of State, Ministry of Agriculture, Fisheries and Food (Baroness Hayman):
"Of the brains removed, a total of 3,951 were in a suitable condition for examination by all the tests that were to be applied. The results from the microscopic analysis of brain tissue showed that 18 cattle had signs of BSE infection. 3,933 were negative and showed no sign of infection. These results were put to the House on 16th July 1999.
Subsequently, the Prionics western blot test has been used on all samples and this test has confirmed the initial findings. No additional positive brains were identified. Samples will also be examined using the DELFIA test. Testing by DELFIA, and consequential interpretation of the results has been delayed by the need to have the test evaluated by the European Commission. However all 18 of the positive brains identified so far are positive by DELFIA. Final interpretation of the data must await the completion of DELFIA testing and evaluation."
Question 16: Whether they intend, in conjunction with the devolved authorities, to eradicate scrapie from the United Kingdom. (HL1657)
Response: The Minister of State, Ministry of Agriculture, Fisheries and Food (Baroness Hayman):
" In their report on research and surveillance on TSEs and sheep published in April 1999 the Spongiform Encephalopathy Advisory Committee (SEAC) recommended that a programme for the long-term control and eradication of scrapie should be drawn up in consultation with the Scrapie Information Group - a joint initiative of industry, veterinary, and Government experts who have a day to day involvement in the surveillance and control of scrapie. This Group which, includes representatives of the devolved administrations, has held a number of deliberations on this subject and has scheduled further discussions during 2000. The development of long-term control and eradication strategies will be an ongoing process that will require close communication with this Group and the devolved administrations.
In the meantime, although scrapie has been present in the national flock for over 250 years without a confirmed link with human illness we have a range of precautionary health controls in place, introduced after advice from the Spongiform Encephalopathy Advisory Committee (SEAC). These comprise the destruction of specified risk materials (whole heads, excluding the tongue, and spleen from all sheep and goats and spinal cord and tonsils of older sheep) and compulsory slaughter, with compensation, of any sheep or goats suspected of having a scrapie-like illness that cannot be ruled out as something else."
Question 17: What action they intend to take as a result of the studies on the heat resistance of hamster-adapted scrapie agent. (HL1658)
Response: The Minister of State, Ministry of Agriculture, Fisheries and Food (Baroness Hayman):
"The Spongiform Encephalopathy Advisory Committee (SEAC) monitors all relevant scientific publications. Members will evaluate the findings reported in this publication at their next meeting and recommend action if they feel it is necessary. It may be, however, that further details will be required from the author before a definitive conclusion can be reached."
Question 18: Why an ethics committee has become involved in Professor Collinge's proposed prospective study of tonsil samples; and whether the involvement of this committee will contribute to the speedy determination of whether, when and how abnormal prions in tonsils led on to clinical disease. (HL1659)
Response: The Parliamentary Under-Secretary of State, Department of Health (Lord Hunt of Kings Heath):
"Ethics committees exist to review any proposed research projects involving human subjects with the aim of ensuring that the dignity, rights, safety, and well-being of all actual or potential research participants are safeguarded. Such committees will consider the potential impact of any such research on the subjects both in the short term and, where relevant, in the long term. The Government believe that the goals of research, however important or time critical, should not be permitted to override the health, well-being, and care of research participants.
As researchers make headway and results in due course start to emerge from this study, they will at the same time need careful interpretation before any conclusions are published. Findings from the study will have to be subject to the normal processes of validation and peer review by the relevant scientific experts, including the Spongiform Encephalopathy Advisory Committee."
Question 19: Why patients who have received blood from people who subsequently developed CJD are allowed to donate blood. (HL1660)
Response: The Parliamentary Under-Secretary of State, Department of Health (Lord Hunt of Kings Heath):
"There is strong epidemiological evidence to suggest that classic Creutzfeldt Jakob disease (CJD) is not transmitted through blood. Donors who have received blood or blood products from people who subsequently developed classic CJD are therefore not barred from giving blood. However, there is far less knowledge about the routes of transmission of variant CJD and, as a precautionary measure, no blood from donors who received blood from people who subsequently developed variant CJD enters the blood supply. As an additional precaution against the theoretical risk that variant CJD may be transmitted through blood, all blood taken from United Kingdom donors is leucodepleted (the white cells are removed) and all blood products are made with plasma sourced from outside the UK."
Question 20: Whether they will permit insurance companies to require that candidates for life insurance be tested for incipient new-variant CJD.[HL1661].
Response: The Parliamentary Under-Secretary of State, Department of Health (Lord Hunt of Kings Heath):
"Insurance companies would be unable to introduce such a step, as no acceptable test currently exists for the demonstration of infection before the onset of clinical symptoms."e.
Question 21: Whether any studies are in progress or contemplation to screen brain tissue from food animals other than cattle and sheep for the presence of Transmissible Spongiform Encephalopathies. (HL1893)
Response: The Minister of State, Ministry of Agriculture, Fisheries and Food (Baroness Hayman):
"No studies are in progress, and currently there are no plans, to screen brain tissue from food animals other than cattle and sheep for the presence of Transmissible Spongiform Encephalopathies."
Question 22: Whether they intend to test sheep for BSE; and, if so, how and when. (HL1772)
Response: The Minister of State, Ministry of Agriculture, Fisheries and Food (Baroness Hayman):
"As SEAC reported in a News Release of 16 March, agent strain typing is underway on isolates from over 130 brains of sheep with natural scrapie in order to determine whether the BSE agent profile exists. Although incomplete (each study takes up to two years) the analysis of over 30 isolates is advanced enough to suggest that they do not have the characteristics associated with the BSE agent."
Question 23:Whether any contingency plans have been made, or are being made, for a cull of sheep if BSE is diagnosed in the national flock. (HL1773)
Response: The Minister of State, Ministry of Agriculture, Fisheries and Food (Baroness Hayman):
"SEAC has suggested that MAFF should draw up operational plans to deal with the possibility of BSE occurring in sheep. SEAC has not recommended a cull, or called upon MAFF to plan for one."
Question 24: How many cattle born in the United Kingdom in each month since 1st January 1996 have died of BSE in each year since 1996, including the current year to date. (HL1865)
Response: The Minister of State, Ministry of Agriculture, Fisheries and Food (Baroness Hayman):
"In the UK to 10 April 2000 there has only been one case of BSE confirmed in an animal born on or after 1 January 1996. This animal was born in January 1996, slaughtered as a suspect in December 1999, and the disease confirmed in February 2000."
Question 25: How many cases of BSE in cattle have been strain-typed; and what were the results of these experiments. (HL1866)
Response: The Minister of State, Ministry of Agriculture, Fisheries and Food (Baroness Hayman):
"Samples from the brains of 9 cows with natural BSE have been strained-typed in panels of mice. These all gave incubation periods and lesion profiles consistent with the presence of one strain of BSE. In addition, the lesion profiles within the brains of cattle with natural BSE have been monitored in birth year cohorts, starting with the 1992 cohort. The lesion profile in cattle has remained constant indicating that only one strain is present."
Question 26: Whether it is considered by those investigating the occurrence of new-variant CJD that a sufficient number of cattle with BSE have been strain-typed to provide an accurate assessment of the nature of the presumed infective agent. (HL1931)
Response: The Minister of State, Ministry of Agriculture, Fisheries and Food (Baroness Hayman):
"Strain typing would not identify the nature of the presumed infective agent, it identifies the strain of TSE present. Scientists working in the field have concluded that there is one strain of BSE as identified by mouse bioassay and molecular typing of the prion protein. The link with vCJD was strengthened when it was shown that the molecular typing of vCJD was the same as BSE. The strain of BSE identified by mouse bioassay and PrP typing remains constant when animals other than cattle are infected with BSE. In addition, the lesion profile within the brains of cattle with natural BSE has been monitored in birth year cohorts starting with the 1992 cohort. The lesion profile in cattle has remained constant indicating that only one strain is present."
Question 27: Further to the Written Answer by the Lord Hunt of Kings Heath on 27th March (WA 55-56), whether they will provide data on those who have died from, or been diagnosed with, new-variant CJD in the United Kingdom as to - (a) the status of nucleotide -21 preceding the prion ATG start codon; (b) the status of codons 26, 56 and 174 of doppel; (c) the dates of onset and confirmation for those patients diagnosed with new-variant CJD but still living; (d) the definition of "onset"; and (e) the age of the patients at onset to the nearest month. (HL1807)
Response: The Parliamentary Under-Secretary of State, Department of Health (Lord Hunt of Kings Heath):
"(a)The genetic information requested is not available.
(b)However extensive studies of polymorphisms in and around the prion protein and doppel genes have been underway for some time at the St Mary's Prion Unit, London. The results of these investigations will be published in the scientific literature, subject to peer review, in due course.
(c) Confirmation of a diagnosis of vCJD is currently obtained by post mortem neuropathology. There are therefore no "confirmed" patients still living. The dates of onset for patients still living and defined as "probable", to the nearest month, are as follows: [given above]
(d) "Onset" is defined by the National CJD Surveillance Unit as the timing and nature of the first symptoms reported by the patients' relatives and obtained from the patient's medical, psychiatric and general practitioner case notes. On average, some 8 months elapse between date of onset and a patient being diagnosed 'probable'.
(e) Age at onset for the twelve probable vCJD patients who are still alive (years/months) is as follows:"[given above]
Question 28: Further to the Written Answer by the Lord Hunt of Kings Heath on 27th March (WA 59), what is the "strong epidemiological evidence to suggest that classic CJD is not transmitted through blood"; which of the many variants of "classic CJD" this evidence applies to; and whether they will place copies of the relevant papers in the Library of the House. (HL1864)
Response: The Parliamentary Under-Secretary of State, Department of Health (Lord Hunt of Kings Heath):
"The following, peer reviewed, articles, relate to this subject and will be placed in the Library:
These studies cover all types of CJD. Sporadic (classic) CJD however accounts for some 85 per cent. of non-variant cases.T F G Esmonde et al, 1993, "Creutzfeldt-Jakob disease and blood transfusion" Lancet 341; 205 - 207. P Brown, 1995, "Can Creutzfeldt-Jakob disease be transmitted by transfusion?" Current Opinion in Haematology, vol 2, pp 472 - 477 C M van Duijn et al, 1998, "Case-control study of risk factors of Creutzfeldt-Jakob disease in Europe during 1993 - 95" Lancet 351; 1081-1085 Ricketts MN et al: Is Creutzfeldt Jakob Disease transmitted in blood? Energ Infect Dids 1997:3 155-166 Heye N et al: Creutzfeldt Jakob Disease and blood transfusion. Lancet 1994;343;298-299
The European Committee for Proprietary Medicinal Products (CPMP) reviewed the evidence in December 1995 and advised that there was no experimental or epidemiological evidence that classical CJD is transmitted by blood transfusions or plasma-derived products. A recall policy was not considered justified for plasma derived products from plasma pools incorporating a donation implicated for classical CJD. It reaffirmed that advice in March 1997.
CPMP concluded on the basis of currently available information from epidemiological and experimental studies that there is no scientific justification for changing from the current CPMP position on classical CJD. CPMP further stated there to be no evidence that classical CJD is transmitted via blood or plasma derived products. This issue was also subsequently considered by the US Food and Drugs Administration who came to the same conclusion."
Question 29: How many cases of new-variant CJD in humans have been strain-typed; and what were the results of these experiments. (HL1867)
Response: The Parliamentary Under-Secretary of State, Department of Health (Lord Hunt of Kings Heath):
"The following two published papers address these questions. Further research is underway:"
1. 'Transmissions to mice indicate that 'new variant' CJD is caused by the BSE agent' (Bruce et al); 2. 'The same prion strain causes vCJD and BSE' (Hill et al) (Nature: Vol 389: 2nd October 1997).
Question 30: Whether any studies are under way to determine how far mice, that express only human PrP with methionine only at codon 129, and such mice which are heterozygous for valine and methionine at this codon, are susceptible to infection with BSE. (HL189
Response:The Minister of State, Ministry of Agriculture, Fisheries and Food, (Baroness Hayman):
This has been identified as a research priority by MAFF. A call for proposals to perform these studies has been included in the TSE Research Requirement's Document which was published by MAFF on 4 April 2000. Suitable BSE brain samples have also been supplied to a UK laboratory which has developed such transgenic mice.
Question 31: Whether any large scale studies are in progress for the molecular strain typing of scrapie in sheep. (HL1892)
Response:The Minister of State, Ministry of Agriculture, Fisheries and Food (Baroness Hayman):
Molecular strain typing needs to be performed under rigorous conditions in a laboratory to give interpretable results. MAFF has supported several studies which have attempted to repeat or scale up this process. MAFF has recognised the need to further explore the definition of strain both in mouse bioassay and by molecular methods and has included this in their Research Requirement's document that was published on 4 April 2000. In addition, the strains present in the brains from 141 sheep with natural scrapie are currently being determined using mouse bioassay and a further 94 are planned.
Question 32: Whether any studies in humans or animals are under way with the object of determining whether infectious diseases and gastrointestinal disorders play a part in determining susceptibility to BSE infection by the oral route. (HL1891)
Response:The Minister of State, Ministry of Agriculture, Fisheries and Food (Baroness Hayman):
MAFF funded studies are currently underway to look at early disease processes in the guts of mice and cattle following oral infection with BSE. A study of the gut in sheep with natural scrapie is also ongoing in which any signs of infection other than scrapie are being noted together with any association with prion protein.
Question 33: What validation has been carried out of the methodology being used in the new-variant CJD tonsils and appendix surveys; and what rate of (a) false positives and (b) false negatives they currently expect in the results. (HL2031)
Response: The Parliamentary Under-Secretary of State, Department of Health (Lord Hunt of Kings Heath):
Validation of the methodology and data arising from the tonsil and appendix surveys falls to the Medical Research Council/Department of Health steering group for studies of detectable abnormal prion protein. That group will consider these issues at its future meetings. It is not possible at present to provide any meaningful forecast of the rate of false positives or false negatives that may, or may not, arise.
Question 34: Whether they are aware of any human nutritional supplements on sale in the United Kingdom which contain material from bovine or ovine glands, brain or other nervous tissue.[HL1777]
Response: Lord Hunt of Kings Heath:
There are no specific compositional rules on food supplements and no prior approval system exists to assess their composition. Nutritional supplements, like other foods, are subject to the general provisions of the Food Safety Act 1990 which make it an offence to sell food which is injurious to health.
Certain bovine and ovine tissues are however covered by the controls on specified risk material, the purpose of which is to ensure that no food containing such material may be sold for human consumption in the United Kingdom.
Specified risk material includes the brain and spleen of all sheep and the spinal cord and tonsils of cattle slaughtered in the UK aged over six months and the thymus and intestines of all cattle slaughtered in the UK for human consumption.
Question 35: Whether red deer antlers are designated as specified risk material in draft European Union Commission Document XXIV/2905/99.[HL1966; Lady Saltoun of Abernethy]
Response: Lord Hunt of King Heath:
The document to which the noble Lady refers would designate only tissues from cattle, sheep and goats as specified risk material.
Question 36: Why they are unwilling to state the genetic make-up of the infant born to a mother with new-variant CJD when this information is readily available from other sources.[HL2030]
Response: Lord Hunt of Kings Heath:
Individual case details are regarded as confidential between patients, their families and the healthcare practitioners involved.
Question 37: What validation has been carried out of the methodology being used in the new-variant CJD tonsils and appendix surveys; and what rate of (a) false positives and (b) false negatives they currently expect in the results.[HL2031]
Response: Lord Hunt of Kings Heath:
Validation of the methodology and data arising from the tonsil and appendix surveys falls to the Medical Research Council/Department of Health steering group for studies of detectable abnormal prion protein. That group will consider these issues at its future meetings. It is not possible at present to provide any meaningful forecast of the rate of false positives or false negatives that may, or may not, arise.
Question 38: Whether they believe that the public will be better prepared for the eventuality of a sizeable epidemic of new-variant CJD if they (a) are not aware of developments in research into the disease or (b) have all available information offered to them at all stages.[HL2033]
Response: Lord Hunt of Kings Heath:
Details of government funded research projects currently under way into all transmissible spongiform encephalopathy issues are available to the public through the Medical Research Council website. In accordance with standard procedures supported by reputable scientific journals, all data resulting from government-funded research projects must be validated and peer reviewed. Results are then published once that process has been completed.
Question 39: Whether they are aware of any children born to mothers who have, within three years of the birth shown clinical signs of new-variant CJD, and, if so, whether any of them are displaying signs of a neurological condition that might be caused by a transmissible spongiform encephalopathy; and, if so, how many and whether they are homozygous or heterozygous at codon 129 of the prion protein.[HL2057]
Response: Lord Hunt of Kings Heath:
All reported cases of progressive intellectual or neurological deterioration in children are investigated for potential variant Creutzfeldt Jakob disease and details of any suspect cases are passed to the National CJD Surveillance Unit, Edinburgh. No definite or probable cases of variant Creutzfeldt Jakob disease have been found in children under the age of 13.
Question 40: Whether, in their view, the study by Mackey and Derrick 'Contamination of deep tissues of carcasses by bacteria present on slaughter instruments or in the gut' (Journal of Applied Bacteriology 1979 Apr; 46(2): 355-66), and the study reported in the Lancet in September 1996 by Tam Garland, demonstrate that brain material becomes spread through the whole carcass of an animal after stunning. (HL1889)
Response: The Parliamentary Under-Secretary of State, Department of Health (Lord Hunt of Kings Heath):
Neither study, in our view, demonstrates that brain tissue becomes spread through the whole carcass of an animal after stunning. The work by Mackey and Derrick concerns contamination by bacteria and does not relate to brain tissue. Garland reported only the finding of brain tissue in the vessels carrying blood from the heart to the lungs following the use of a stunning device which injects air at high pressure into the cranium. This device is not used in United Kingdom abattoirs.
Question 41: Whether they will place in the Library of the House copies of the protocols of the new-variant CJD tonsil and appendix surveys currently under way, and the data gathered to date. (HL2034)
Response: The Parliamentary Under-Secretary of State, Department of Health (Lord Hunt of Kings Heath):
Data from the tonsil and appendix surveys currently underway will, as they arise, be assessed at meetings of the joint Medical Research Council/Department of Health Steering Group for Studies of Detectable Abnormal Prion Protein. The Department will in due course announce interim findings from the Steering Group's deliberations, and a copy of that announcement will be placed in the Library. Publication of the protocols and the detail of the study findings are matters for the researchers themselves.
Question 42: Whether they are aware of the basis on which AFSSA (the French food safety agency) has banned pithing in slaughterhouses; and whether they will place in the Library of the House their evaluation of the French decision and their reasons for differing from AFSSA's conclusion. (HL2035) Response: The Parliamentary Under-Secretary of State, Department of Health (Lord Hunt of Kings Heath):
AFSSA's recommendation that the practice of pithing should be banned in French abattoirs was based on its assessment that pithing presents a risk of contamination of the carcase by both nervous tissue, which AFSSA considered a risk factor for Bovine Spongiform Encephalopathy (BSE), and microbiological organisms. It is for the French Government to consider that recommendation and to take such action as it considers necessary.
On the BSE risk in the United Kingdom, the Government have accepted the advice from the Spongiform Encephalopathy Advisory Committee (SEAC) that there is no reason to change UK practices of stunning and pithing during slaughter of cattle. In giving that advice, SEAC took into account the measures in place in the UK to protect public health from BSE, notably the prohibition on the sale for human consumption of meat from animals aged over thirty months, as a result of which the number of infected animals at the late stage of the incubation period entering the food chain is now estimated to be very low.
There is no rule equivalent to the over-thirty-month rule in France and it would be quite understandable if a risk assessment carried out in a country without such a rule resulted in different advice. The risk of the introduction of microbiological contamination by pithing rods is small as long as good hygienic practices are followed.
Question 43: Why the recent meeting of Transmissible Spongiform Encephalopathy researchers in Keele was held in private; who was invited to it; what confidentiality agreements they were required to sign; and whether the minutes of that meeting will now be published. [HL 2032]
Response: Baroness Hayman:
A Joint Funders Workshop on TSE Research in the UK was held in Keele (4-6 April 2000). This comprised a meeting of funded scientists working in the field of TSEs, department/research council officials and five members of SEAC (Spongiform Encephalopathy Advisory Committee). Meetings of this type have been held annually by the BBSRC (Biotechnology and Biological Sciences Research Council) for a number of years.
The first joint meeting with the participation of other funders was held in 1998. The purpose of these meetings is to inform the funding bodies of the progress being made in the work they are supporting, to share results and encourage collaboration and networking between the scientists. The researchers present were funded by one or more of the following funding bodies: BBSRC, Department of Health, Medical Research Council and MAFF. Two international guest speakers were also invited.
This was a closed meeting as interim results were presented, and most of the research work discussed was pre-publication. For this reason, a confidentiality agreement was signed by conference delegates. Written reports covering all sessions of the workshop will be circulated to the funders. However, the proceedings will not be published.
Question 44: Whether human body parts such as skin, tendons, heart valves, bones and dura mater are imported into the United Kingdom for use in the National Health Service; and, if so, from which countries, with what safeguards, and at what annual cost. (HL2645)
Response: The Parliamentary Under-Secretary of State, Department of Health (Lord Hunt of Kings Heath):
The National Health Service is largely self-sufficient in tissues such as skin, tendon and heart valves, relying on material from tissue banks within the NHS. Exceptionally, small amounts of tissues are imported to meet individual patient's needs. Details of the quantity and cost of imported tissues are not held centrally.
Tissue banks accepting material from overseas must make sure that the Committee on Microbiological Safety of Blood and Tissues for Transplantation's "Guidance on the microbiological safety of human tissues and organs used in transplantation" has been followed by the bank of origin. If the tissues do not pass through a United Kingdom tissue bank, the clinician who uses the tissue is responsible for making sure the Guidance has been followed. Dura mater is no longer used in the NHS.
4 Apr 00 Highlights: See Lords Hansard Home Page for full textLord Lucas rose to ask Her Majesty's Government what preventive measures they are taking and what preparations they are making to deal with the possibility of a sizeable epidemic of new-variant CJD.
The noble Lord said: My Lords, I seek to persuade the Government to prepare for the worst. I can do no better than refer the Minister to the paper published by Professor Collinge in July 1999 setting out his views on the likely course of new-variant CJD in Britain. The conclusions he reached were that in all likelihood what we are seeing at present are not individuals who caught the disease at the time we knew that BSE existed but those who had caught the disease before that; and, taking a line from other TSEs in humans, we were likely to see the peak of this epidemic in 15 or 20 years' time. Therefore, considering the level of cases occurring now, we may be looking at a disease which will kill hundreds of thousands or even millions of us.
Should we ever reach that horrific moment when we have to admit that an epidemic will kill enormous numbers, I want the Government to be able to look back at what they did with pride and to say, "We did the best we could. We took all the measures we reasonably could take at the time". I want the rest of us to be able to agree with the Government on that.
I was a fly on the wall, as a very junior member of the Government in the Ministry of Agriculture, Fisheries and Food, when the BSE crisis boiled over. I look back at that period as a time of panic and a lack of preparedness. I look back at 1,001 things that we could have done better in the years running up to that point. But I also look back at a government composed of people who had the best of motives, took the best of advice, and did what they thought was best at the time.
There are two main reasons why the road to hell was paved with our good intentions. First, we were optimistic. Of course we were. Before knowing to the contrary, who would not wish that BSE could not transmit to humans? However, we allowed that optimism to cloud the need for pessimism. We concentrated on giving the public a reassuring message and we believed that doing so precluded us from taking precautions, openly and in public, to deal with the situation should our optimism turn out to be unjustified.
The second reason for getting things wrong was that we were secretive. There is, and always has been, a strong culture of secrecy in MAFF, but we allowed it to take over. All the research data, conclusions and direction were confined within MAFF. There was little public knowledge outside of what was happening. All the decisions were taken internally. Such a closed attitude leads to bad, slow, misdirected research.
The other aspect of secrecy was that we took our decisions in private. We did not publicly share them or the reasons for taking them. That meant that if later the decision turned out to be wrong or questionable, it was hard to go back on it. As it had not been accepted by the public when it was taken, to go back on it (having asked them to believe in it) risked precipitating a crisis which might not be justified. Sadly, that was not borne out by experience.
Optimism and secrecy meant that we could not obtain proper funding from the Treasury for preventive measures and for research. The Treasury was not told the full extent of the fears about what might be going wrong. We could not prepare our European and other colleagues for the worst and we could not prepare our citizens for what might happen. Therefore, when the crisis broke, it was devastating.
The reason I have asked for tonight's debate is that when I look at what the Government are doing about new-variant CJD I recognise the same symptoms in what we did during the run-up to the BSE crisis. I see the same optimism and secrecy.
If we were making preparations for the worst, we would expect to see a large, open, public research programme. We would expect to see scientists all over the world swapping information openly and freely. In the best and most open scientific areas there is real work to be done at the fundamentals of science. That is certainly true in respect of new-variant CJD when we do not even know the basic disease mechanism.
The best examples publish their laboratory workbooks daily on the Internet. We have to wait a year, or a year and a half, for results on new-variant CJD to be published in a review paper. Asking the Government for information can be like drawing teeth. I asked the Minister a Question about the ages of people who have died of new-variant CJD and was told to the nearest 10 years. How can one use such data to draw a graph and see what is happening, given the number of people who have died of the disease? Even the basic, most simple, least important data is being held back and delayed by the Government. That is entirely familiar to me as we did exactly the same thing.
We ought to be looking at a research programme which will clearly and to everyone's knowledge provide a good picture of what is happening. The foundation of that must be prospective studies. It must be gathering data on a large group of us, testing us, seeing what is happening to us and then waiting to see how many of us die of the disease. Unless that is done, we shall lack the basic predictive data which enables us to work on the disease if and when it becomes relevant.
There is only one sizeable prospective study. It is a tonsil test covering 1,000 people, but it has been delayed for more than a year by a hospital ethics committee. The sense of urgency does not exist. I ask the Minister what progress has been made in evaluating the capillary electrophoresis test developed by Mary Jo Schmerr. He may remember that the answer he gave was that the test had undergone some preliminary evaluation and that an application for full evaluation is being considered.
However, The Times of 3rd October--six months ago--reported a MAFF spokesman as saying that a team had been set up to evaluate the test and then begin a screening programme. In six months we have gone backwards. We are not looking at a government with a sense of commitment or urgency to analyse what is going on with the disease.
If we arrive at a point of crisis, we must have diagnostics, cures and prospective studies well under way and co-ordinated; and we, the public, must know what is going on. If not, we shall risk a catastrophe of confidence in the institutions of this country and in our future as individuals.
Another symptom of optimism and secrecy is things left undone. The noble Lord will have seen the article in the Sunday Times picking up the fact that we in this country are still feeding calves with blood, gelatin and tallow. We now know--we might not have known at the time the regulations were promulgated by the EU--that all those substances carry infection when the original animal is carrying BSE. How can we be feeding such substances to calves, which is the most vulnerable stage of a cow's life? How can we continue to do so when we have taken expensive precautions to try to reduce the risk of humans transmitting new-variant CJD in their blood, a course which has not been proven? We have ignored the certainty and alighted on the great uncertainty.
When dealing with BSE and new-variant CJD, we have always had a problem as regards the lack of co-ordination between MAFF and the Department of Health. Having responsibility for the one disease sited in two ministries was always a questionable decision, but this Government have gone further and sited it in three departments. The Food Standards Agency is to have its own axe to grind on the subject. The possibilities of co-ordination have been immensely reduced.
One of the basic things we need to know about TSEs is the strain of disease we are facing. The strain of TSE makes an enormous difference to the symptoms and to the progress of the disease. Presuming that the disease has come from cattle, which is almost certain, we need data about the strains of BSE in cattle. We have always been told that there is only one, but that assertion has been based on strain-typing nine cows--nine out of the hundreds of thousands which have died of the disease. There could be a significant level of infection from a different strain attacking the human population. We would not know about it because we have not done the basic research into the strains which exist in cattle.
One of the key actions to take at this stage in the BSE epidemic is to examine what is happening to the tail in order to make sure that there are no transmission mechanisms in cattle that we should be dealing with, thereby ensuring that the original source is gone for ever. But MAFF will no longer make predictions for the BSE epidemic beyond 2001; it is not even looking at the question of what will happen beyond that. When the Sunday Times asked it about those cattle born in 1996 which had contracted the disease--that is supposed to be at the end of the epidemic--MAFF refused to say in which month those cattle had been born.
That is symptomatic of a determination not to pick up the details in the hope and pervading optimism that we shall not need to know them because everything will turn out all right. We are not properly testing cattle at slaughter to discover whether they are carrying BSE. We are not running trials in sheep to discover whether they can catch BSE, what the symptoms are, and how we can pick them up in the national herd.
We are not looking at the brains of other species of food animals to see whether they, too, have TSEs or have been exposed to TSEs or whether, through our practice of feeding cows to them, they have picked up BSE in their turn. All those little things are being left undone because there is no stream of pessimism in the Government's thinking, any more than there was in ours.
Another symptom is evasive answers. I asked about blood donations. The noble Lord replied that there is strong epidemiological evidence that classic CJD is not transmitted through blood. That is not true. There is no evidence one way or the other, but there is certainly no evidence that it is transmitted through blood. However, even a combination of all available studies is not statistically significant. In many of them there are severe problems with the selection of controls, and some studies show that receiving blood from a person who has CJD protects that person against CJD. The selection of controls has been bad enough to allow that result through. The answer given by the noble Lord--in the best of faith, I am sure--does not accord with the evidence. It is a distortion of the evidence. We got away from that for a year or two after the break of the BSE crisis when there was a pathological attachment to openness and honesty. That seems to have gone, but it needs to be recreated.
Again, I asked about transmission from mother to child in utero of new-variant CJD in humans. The noble Lord replied that no confirmed or probable case had been reported. I read the newspapers. I know about this child, who appeared to have neurological symptoms after his mother had been diagnosed with new-variant CJD. I know that their symptoms are different from classic new-variant CJD. However, perhaps the noble Lord can tell me--I am sure that the Box could tell him--whether that child is met/met at codon 129 or whether that child is met/val? If that child is met val, one would expect different symptoms and a different disease. Those data, which I am sure the Government have, have not been released.
What do I want the Government to do? I want the Government to be open. I want to make sure that all the Government's data are available to all and any researchers. I want people who research with government money to be open on a daily basis about what they are doing. I want the Government to take their decisions in public so that their errors may be exposed quickly and so that what, at the time, are reasonable decisions gain public support at the time so that they are not blamed if those decisions turn out to have been wrong. I want the Government to prepare for the worst. I want them to think through what it would be like if we had a major epidemic of new-variant CJD in this country.
Then I want the Government to talk that through with the Treasury so that proper funding is in place for preventive measures; with the European Union so that we do not have the "kick Britain" attitude that we had at the time that the BSE crisis broke; with the United States, whose attitude to trade and passage of peoples between the UK and America will be enormously important; and with life insurers, who will have a great deal to say if people start dying in large numbers from this disease.
I want the Government to be able to look back and say that everything that they have done is all that it should have been. I believe that if they follow the route of openness and preparedness, they will be able to do that and that we shall have a research programme that we all believe to be right and adequate and of which we can be proud. I believe that we would then have good decisions in place of the frightened and bad decisions that we seem to get at the moment. Above all, I believe that if the crisis breaks with this Government in charge, rightly or wrongly they will be able to blame us for BSE and get away with it. If this Government, with our example in front of them and in the clear knowledge of all that we did wrong, make the same mistakes, we shall suffer enormously as a country and this Government will be damned and doubly damned in the eyes of their people for ever.
Lord Clement-Jones: ... The debate this evening mainly provides an opportunity to question this Government's approach to prevention and treatment of new-variant CJD. On these Benches we welcome a number of the steps already taken by the Government, such as the setting up of the BSE inquiry in the first place. However, I believe that the results of the inquiry are now nine months overdue. Can the Minister indicate when it will report?
Another matter that we welcome is the guidance issued in June 1999 on the danger of cross-infection from contact lenses. We also welcome the announcement of improvements to the care of new-variant CJD patients announced in December by the Minister of State responsible for Health, John Denham. However, will he follow up those February 1999 recommendations on the care and information needs of new-variant CJD patients? The Minister will recall that there is a strong recommendation for a key worker to be appointed at a very early stage as soon as new-variant CJD is diagnosed. I believe that those care and information recommendations should be a strong part of the new procedures that the Minister announced in December.
We also welcome the reissuing of guidance to coroners and others regarding the treatment of those who have died from new-variant CJD in the face of evidence of distress caused to families by inappropriate restrictions placed on funeral arrangements. That was a matter raised recently in the other place by my honourable friend Nick Harvey. We also welcome the agreement of the CMOs reached last December to monitor closely the incidence of new-variant CJD, as we do the new system of reporting by SEAC announced this March which will track the incidence of possible new-variant CJD among living patients, not only the deaths arising from CJD. I welcome the fact that the research appears to show that the tracking system is watertight. ...
... What tests are available for use in the NHS? The noble Lord, Lord Lucas, also asked what tests are available to detect CJD. Is the tonsil test generally available in the NHS? Is there urgent evaluation of any other tests? How is the research which is being carried on by Imperial College and St Mary's Hospital proceeding on the matter of testing? How watertight is the current system of tracking cattle and their offspring? Is it really correct that some 90,000 cattle have simply "gone missing"? Are reports elicited by the noble Lord, Lord Lucas, that cattle are still being fed cattle products made of tallow, blood and gelatine actually correct? Does the Minister agree with Professor Collinge, who wrote in The Lancet last July: "that the risks of blood and blood products cannot be quantified".
If so, how can the Government claim that they are taking a precautionary approach? What guarantee do we have that the events of the past few years will not happen again?
Earl Howe: My Lords, the whole House should be indebted to my noble friend Lord Lucas for having secured a debate today on the subject which he has made it his business to research in depth. It is a deeply troubling subject, and if there is a single message that my noble friend would wish distilled from his admirable opening speech, it is--at the risk of misrepresenting him--that if ever there was an issue on which there is absolutely no room for complacency whatever, this is it. I hope and believe that the Minister's speech will reassure us that no such complacency exists, politically, scientifically or medically. But the issue of new-variant CJD does not stand still. As my noble friend has emphasised, we must constantly examine all new data and constantly look ahead.
The Question on the Order Paper urges us to look ahead to the possibility of a major epidemic of new-variant CJD at some point in the future. Our difficulty is getting our bearings on the likely scale of such an epidemic or indeed on whether any sort of epidemic will occur at all. At this stage we simply do not know what will happen. It is not even possible to make an educated guess, despite the theory of a causal link between BSE and CJD being now a virtual certainty. Shortly after the last election I attended a presentation given by senior statisticians from City University. Their research indicated that the total number of variant CJD cases was likely to be fewer than 100. That conclusion was based on an analysis of new-variant CJD cases to date and on a number of assumptions, the most significant being that the incubation period for new-variant CJD was relatively short.
However, that conclusion is by no means universally accepted. It is clear from published data that we cannot be at all certain that the incubation period for the disease is a short one. On an analogy with kuru, it may extend over a period of 20 or 30 years. While we can estimate the number of BSE-infected cattle that may have entered the human food chain in the early to mid-1980s, we do not know how much infected meat was consumed, nor the extent to which the species barrier between cattle and people has inhibited such infection. Similarly, there is some evidence that genetic susceptibility plays a part in the onset of CJD, but that does not mean that people with a different genetic make-up may not be infected with the disease and incubate it for longer. The fact that there have to date been only 52 deaths positively attributable to new-variant CJD is no indication at all that the optimists are right and the pessimists wrong. The Chief Medical Officer has warned that we could still be facing a large number of cases over several decades.
Therefore, predicting the course of the disease must be a priority. Currently there are programmes in train to devise a non-invasive test for BSE infection in live cattle and for pre-clinical new-variant CJD in the human population. I hope that the Minister can tell us something about these. We can also look forward to the results of biopsies of tonsils and appendices. Some 18,000 such samples taken from patients in the south west of England and the Lothian regions are being examined for the presence of abnormal prion protein. I was pleased to see a few days ago that ethical approval had been given to begin a further study of 2,000 tonsils. Will the Minister tell us about the timescale of this work and when the results are likely to be published?
Meanwhile, the paramount duty of government is to protect human health from the risk of infection. There are perhaps two principal spheres of action. The first is to ensure that food is safe. The measures that are in place relating to cattle are well known: the ban on animal protein in ruminant feed; the rule that cattle over 30 months cannot enter the human food chain; the cull of offspring of BSE-affected cows; the removal of specified risk material from all cattle at slaughter. Those measures are only as good as the rigour with which they are enforced.
I understand that the Meat Hygiene Service is confident that procedures at slaughter to ensure that banned meat and offal does not slip through the net are being observed conscientiously. However, will the Minister confirm that the checks on cattle at slaughter still include an inspection of teeth? Irrespective of the ear-tag and the date on the cattle passport, there is a need for a belt-and-braces approach to prevent over-age animals from entering the food chain. What level of error or deliberate non-compliance on the part of farmers has been uncovered by the Meat Hygiene Service in recent months? What procedures are in force to check the safety of imported meat?
However, there are some worrying areas of uncertainty. It is still possible that BSE may have been transmitted to other agricultural species; notably, sheep. The symptoms of scrapie and BSE are indistinguishable. If BSE were to be found in the sheep population, the consequences would be extremely serious, not least because, unlike its occurrence in cattle, BSE infects almost the whole animal and not just certain organs. Is the Minister in a position to say whether large-scale tests are now under way; whether there are any plans to tag sheep; and whether, if BSE were to be found in sheep, consideration would be given to a cull? If he cannot comment in any detail now, will the Minister undertake to keep the House closely informed of developments under that heading?
I mentioned action in two spheres. The second sphere is to eliminate, as far as possible, the risk of transmitting new variant CJD from person to person by medical means. Much has been done to forestall what is still only a hypothetical possibility. Procedures are in place to remove white cells from blood intended for transfusions and to source blood plasma from abroad. There are strict rules to prevent blood entering the blood supply from donors who themselves received blood from people who later developed new-variant CJD.
... My noble friend laid great stress on the need for openness by government. I endorse that view without hesitation. His opinion of the culture within MAFF as one which inhibits such openness is perhaps, with all deference to him, somewhat jaundiced. I should not go that far. I believe that in MAFF there exists a desire to ensure that whatever information reaches the public domain is both robust and fully considered....
The announcement in March 1996 that there was a prima facie link between BSE and new-variant CJD should not have come as such a shock when, for the previous 10 years, the precautionary measures put in place by government had been designed to anticipate that very finding. ...
The Parliamentary Under-Secretary of State, Department of Health (Lord Hunt of Kings Heath): My Lords, I thank the noble Lord, Lord Lucas, for having raised the issue of new variant CJD. He has spoken in a sense from experience, as well as from a great deal of knowledge. The friendly warning which he has given to the Government in relation to the dangers of complacency, optimism and secrecy is one which I well take...
One of the particularly distressing features of variant CJD is the number of young people who have suffered terribly. Since May 1997 the British Paediatric Surveillance Unit of the Royal College of Paediatrics and Child Health has been working with the CJD Surveillance Unit and Addenbrookes Hospital, Cambridge to try to identify any possible variant CJD cases at the first possible moment. Each month, a survey is carried out of all 2,000 consultant paediatricians, to ask if they have come across any cases of progressive intellectual or neurological deterioration in under 16 year-olds. There is currently a 90 per cent response rate, including some nil returns.
Of the 602 cases investigated to date, only 3 definite or probable cases of variant CJD have been found. Results are published in the annual report of the British Paediatric Surveillance Unit...
I was asked about those who may be incubating the disease but have yet to show any clinical symptoms. How many are there and who are they? With variant CJD we are much hampered by the lack of a simple diagnostic test. Last September the Spongiform Encephalopathy Advisory Committee, or SEAC, identified the development of such a test as a priority. Although no imminent results are in prospect, studies are under way as part of the 26 million-worth of government-funded research to look into CJD and BSE issues during the coming year.
Meanwhile, and in answer to the comments made by the noble Earl, Lord Howe, analyses are taking place retrospectively of some 18,000 tonsil and appendix samples taken from routine surgical operations in the South West and Scotland. In that study, scientists are looking for the presence of abnormal prion protein, as there is some evidence that that may be detectable at the pre-clinical stage, although we do not know when. Results from the first 2,000 samples will be presented to a meeting of medical and scientific experts under the auspices of the Medical Research Council and the Department of Health later this month. An announcement about the findings will be made shortly thereafter....
The noble Lord, Lord Lucas, asked what progress had been made on evaluating the test developed by Mary Jo Schmerr of the National Animal Disease Center of the US Department of Agriculture. I regret that I am not in a position to provide any further information....
The noble Lord asked about information relating to the ages of new-variant CJD cases. I have to tell the noble Lord that one of the issues in relation to this information is that because of clinical confidentiality such information cannot be released in a form which could be linked to particular individuals. Furthermore, in response to the noble Lord's comments on the secrecy surrounding research in this area, I have to tell the noble Lord that, while I fully accept the need for openness, it is important to note that all TSE research projects currently completed and funded by public moneys are already listed on the MRC website. The site provides a breakdown of areas of research being covered, including those identified with the issues before us tonight, as well as issues concerning the annual costs of such research projects. ...
The noble Lord asked about a case reported about the possibility that TSE had been transmitted by a mother to a child in utero. I cannot report on individual cases. The case in question is a confidential matter between clinicians, patients and families. However, my understanding is that no child under the age of 13 has been reported as a probable or definite case of new-variant CJD.
I was asked by the noble Lord, Lord Lucas, about the feeding of tallow, gelatin and blood products to calves. I understand that United Kingdom rules on feeding stuffs are much tighter than those elsewhere in the EU. We ban the feeding of mammalian meat and bonemeal to all farm animals whereas the EU rules only prevent the feeding of MBM to a selected area. Certainly in accordance with EU rules, we allow the feeding of milk, tallow, gelatin and blood products to ruminants and we believe that is in line with SEAC's advice.
I have dealt with the issue of blood transmissions. The noble Lord asked me about genetic testing. All probable and definite cases of new-variant CJD so far have been found to have been from methionine/methionine homozygous genotype and of the same genetic sub-type as the population. However, I cannot divulge the details of individual cases.
The noble Lord, Lord Lucas, also asked me about the testing of other animals for BSE. Of course, extensive research has been undertaken into the issue of sheep and BSE. My understanding is that BSE has not been confirmed as occurring in animals from the national flock...
Mon, Apr 3, 2000 By Andrew Evans, Lords Staff, PA NewsIt may be "at least a year or two" before scientists can predict whether the variant of Creutzfeld Jakob Disease, the human equivalent of BSE, will become widespread in the UK population, a health minister said tonight. There have been 67 confirmed or probable deaths in the past five years, nearly half of them occurring in people in their 20s. But it is not known how long the incubation period is.
Lord Hunt of Kings Heath said statistical modellers were analysing current trends. "But it may be at least a year or two yet before we are in a position to rule out a significant number of people eventually succumbing to the disease."
Replying to a Lords debate, he said the first results of separate tests on some 18,000 tonsil and appendix samples, to test for the prevalence of CJD in Scotland and South West England, would be available later this month. Results from the first 2,000 samples would be reported to a meeting of medical and scientific experts, under the auspices of the Medical Research Council and the Health Department. "An announcement about the findings will be made shortly thereafter."
The minister added: "The Government will shortly be publishing some new guidance, which is expressly intended for those health care professionals caring for CJD victims, and it will mention the importance of having a named key worker in place as soon as possible."
Tory former agriculture spokesman Lord Lucas, opening the debate, had warned: "We may be looking at a disease which will eventually kill hundreds of thousands or maybe even millions of us." Lord Lucas, who had served in the department at the time the BSE crisis was at its height, accused the Government of complacency, over-optimism and evasiveness on the issue.
"When I look at what this Government is doing," he said, "I recognise the same symptoms of what we did in the run-up to the BSE crisis. I see the same optimism and the same secrecy." Lord Hunt denied complacency and justified such secrecy as there was on the grounds of patient confidentiality and to avoid unnecessary panic.
BMJ 2000;320:1011 ( 8 April 00) James Lowe, professor, Division of Pathology, University of Nottingham Medical SchoolThe United Kingdom faces the possibility of an epidemic of new variant Creutzfeldt-Jakob disease as a result of bovine spongiform encephalopathy,1 but the referral of brain tissue to neuropathologists to determine the cause of degenerative diseases of the brain has virtually stopped. Public concerns over the retention of tissues for investigation after postmortem examination make it is easy to see how this has happened.2
In the United Kingdom most postmortem examinations on adults are performed because they are directed by law. The main reason is to ascertain a natural medical cause of death for certification, thereby removing the need for an inquest.
In the past, retaining the brain for examination was a routine part of the practice of pathologists, but it is now clear that many relatives of the deceased person may not have been aware of this. Because of concerns over the legitimacy of organ retention the true cause of a degenerative brain disease is now hardly ever being established after a postmortem examination, even though the brain has been examined by the inadequate method of slicing during the necropsy.
Simply certifying that a person has died of a disease such as pneumonia, in the face of a decline as a result of a degenerative brain disease, is giving a mechanism of death rather than its true cause. This has implications for estimating misclassification rates in epidemiological studies.3
I suspect that many families will be concerned that they were not given the opportunity to benefit from appropriate investigations. Guidelines from the Royal College of Pathologists (www.rcpath.org) will be released soon, but medical and public confidence has already been lost.
So far, the cases of new variant Creutzfeldt-Jakob disease have been in a distinct subset of the population.1 We have no knowledge of the ways in which new variant Creutzfeldt-Jakob disease may become manifest, especially in elderly people. Unless a positive statement is made to reassure the public, medicolegal authorities, and pathologists, the situation of "not looking" will prevail and we run the risk of missing any emerging epidemic.
1. Collinge J. Variant Creutzfeldt-Jakob disease. Lancet 1999; 354: 317-323[Medline]. 2. Woodman R. Storage of human organs prompts three inquiries. BMJ 2000; 320: 77[Full Text]. (8 January.) 3. Majeed A, Lehmann P, Kirby L, Knight R, Coleman M. Extent of misclassification of death from Creutzfeldt-Jakob disease in England 1979-96: retrospective examination of clinical records. BMJ 2000; 320: 145-147[Abstract/Full Text]. 15 January.)
Reuters World Report Tue, Mar 28, 2000 By David EvansThe European Commission is set to propose compulsory tests on cattle for mad cow disease in a bid to build a realistic picture of a disease many fear is under-reported, EU officials said on Tuesday. The proposal, to be endorsed by a full Commission meeting on Wednesday, will force all European Union countries to undertake random tests, focusing on fallen stock [downers] -- those animals that die on the farm for no apparent reason.
"We hope the programme will provide better information on the incidence of BSE in member states," Beate Gminder, spokeswoman for EU Food Safety Commissioner David Byrne told Reuters.
Bovine spongiform encephalopathy (BSE), or mad cow disease, continues to cause controversy in Europe, years after it was first discovered in Britain in the late 1980s. Scientists say it is still too early to say how many people will die >from a new form of the brain-wasting Creutzfeldt-Jakob Disease (nvCJD), believed to be caused by eating BSE-infected meat. The nvCJD death toll in Britain has risen above 67, and may eventually reach thousands, experts say.
All British beef exports were banned by the Commission in March 1996. The embargo was lifted last July after millions of cattle were slaughtered at a cost running to billions of pounds. Defying EU law, France refused to lift its ban on British beef, an action which sparked a fierce anti-French media campaign in Britain and damaged cross-Channel relations. Paris has now been taken to the European Court over the ban.
There was evidence of a thaw in relations on Tuesday as Britain's Prince Charles and one of France's top chefs Raymond Blanc joined forces to showcase British beef at the Frenchman's Michelin-starred restaurant near Oxford, England.
France itself has come in for recent criticism over its own BSE incidence and health controls. Twelve cases have been reported so far this year and Paris has recently announced its own national testing programme.
An EU report last month said traces of meat and bone meal continued to be found in French animal feed despite a July 1996 ban on cows being fed animal proteins. France will test 40,000 cattle out of its huge national herd of 21 million, and French Farm Minister Jean Glavany has warned the results may produce unwelcome news. The report follows earlier criticism that sewage sludge had also been added to cattle feed. France said the practice had stopped but the news further fuelled British indignation.
Under the EU proposal, member states will be able to choose one of three tests on the market -- one from France, one from Switzerland and another from Ireland. Fears that cases still go unreported, with potentially infected meat entering the food chain have been highlighted by research in Switzerland, where tests uncovered three BSE cases among 7,000 supposedly safe cattle heading for the slaughterhouse.
Thu, Apr 6, 2000 COMTEXThe European Union (EU) has decided to adopt a Swiss test as one of the three to be used in fight against the BSE, know as the mad cow disease, Swiss Radio International reported Thursday. The test, developed by the University of Zurich's spin-off company Prionics, uses prion protein analysis to detect the disease in dead animals.
Switzerland has been using the test since 1999 as its standard means to check for BSE in randomly sampled cattle, including cows which did not die after displaying BSE-like symptoms. However, the EU is not planning to adopt as rigorous an approach as Switzerland.
Of the 41 million cattle in the EU aged over two years, one percent is estimated to be at risk from BSE. Of this group of around 400,000,one sixth of the animals will be surveyed in what are termed "rapid post-mortem tests". The cows surveyed will be ones which have shown BSE-like symptoms.
The relatively small study included approx. 5000 tests on regularly slaughtered cattle. In Switzerland, the incidence in this group in 1999 was one in approx. 2500 animals. The fact that nothing was found in Germany in 5000 animals makes it seem unlikely that Germany has a similar BSE-problem like Switzerland.
In the meantime we know from the Swiss surveillance with the Prionics-Test that (at least in Switzerland) emergency slaughtered cattle and fallen stock represent a higher BSE-risk category than normal slaughtered cattle. Future European surveillance programs will therefore be likely to incorporate these cattle categories. But even with the surveillance carried out so far in Germany it is probably safe to assume that this country is unlikely to be among the major BSE-countries. More data however will be needed to substantiate how much less BSE (if at all) there is in Germany than e.g. in Switzerland."
Lancet 2000; 355: 1250 - 1252The European Commission has approved proposals from the Commissioner for Health and Consumer Protection, David Byrne, requiring all member-states to undertake systematic testing for bovine spongiform encephalitis (BSE) in cattle that are classified as fallen stock. These include animals that die unexpectedly on the farm, sick animals slaughtered in emergencies, or animals displaying neurological symptoms. There are about 400 000 fallen-stock animals each year and the Commission wants member states to check at least 10% of them.
The mandate comes amidst allegations that some countries have under-reported or misdiagnosed BSE. The Commission is asking farm ministers to agree to this first community-wide programme to establish BSE. It notes that such a testing programme in Switzerland led to a "significant improvement in monitoring of BSE".
Byrne said the scheme would help build up a better view of the scale of the disease within the EU. "I expect that the proposed new monitoring will provide significantly better information on the incidence of BSE in the community than we have at present", he said.
Wed, Apr 5, 2000 AP/Dow JonesThe European Union on Wednesday agreed to start E.U.-wide testing for mad cow disease as of Jan. 1, 2001, focusing on animals that die on farms, are killed because they are sick or are suspected of suffering from neurological disorders. A panel of E.U. veterinary experts endorsed a European Commission proposal for random testing of 65,000 animals a year that die before reaching the slaughterhouse.
"The test results should provide a more accurate picture" of the incidence of mad cow disease in the union, E.U. Consumer Affairs Commissioner David Byrne said in a statement. He said the program would be evaluated after six months. The new tests will focus on the 41 million cattle in the E.U. that are over two years old. One percent is at risk of mad cow disease and of those 400,000, at least 65,000 will be surveyed in "rapid post-mortem tests."
Byrne again urged E.U. governments to adopt his proposal of last fall to remove such cattle parts as the brain, spleen and spinal cord. When processed into animal feed, these "specific risk" parts are seen as key to infecting cattle with mad cow disease.
Only eight of the 15 E.U. governments have made the removal from the food chain mandatory. There is currently no such systematic E.U.-wide testing for mad cow disease.....
March 31, 2000 Kyodo news agencyComment (webmaster): The headline here is highly misleading, yet fairly typical of news stories worldwide. First, the patients had various kinds of CJD, not nvCJD or other bovine transmitted diseases. Second, the caseload is not for one year but is the cumulative total for 15 years. The population of 124 million suggests reported CJD of 124 cases per year, though Japan is plagued with higher levels of dura mater iatrogenic CJD.
The number of patients suffering from or having once suffered from Creutzfeldt-Jakob disease (CJD), the human equivalent of mad cow disease, topped 1,000 for the first time in Japan last year, a Health and Welfare Ministry survey showed Thursday. The survey showed the number of CJD patients reached 1,029 at the end of last year, up from the 939 registered as of March 31, 1999. CJD is a rare, fatal brain disorder which causes rapid, progressive dementia and associated neuromuscular disturbances.
The ministry survey, which covered patients between 1985 and March 31 last year, confirmed 939 patients, and 90 more patients were added to the list between April and December 1999, ministry officials said. Mad cow disease first made headlines in March 1996 when British authorities announced a possible link between the cow disease and CJD.
Mon, 3 Apr 2000 Los Angeles Times By SONNI EFRON, Times Staff WriterAn unknown number of people who received brain tissue implants may now be exposed to a killer virus [sic]. Amid the finger-pointing, no one is taking responsibility.
KoseichO, Japan--At first, she thought it was a bad case of jet lag. Then her vision began to blur, and she got lost on the walk to the nearby post office. Her handwriting became childlike, and she was tormented by hallucinogenic nightmares. Within four months of her first headache, Takako Tani quite literally lost her mind to Creutzfeldt-Jakob disease, or CJD, the human form of the fatal brain-wasting malady known as "mad cow" disease. It was 1996. She was 41.
What horrifies Japan most about what happened to Tani is that it almost certainly was preventable. She is one of at least 65 Japanese believed to have been infected through contaminated brain tissue collected from European corpses. For reasons that are being probed in the Japanese media, courts and parliament, the imported tissue continued to be used in Japan even after 1987, when regulators in the U.S. and Britain banned it. Most of Japan's known victims were infected before 1987, but Tani received her implant during routine neurosurgery in 1989.
Unlike British victims thought to have contracted CJD by eating contaminated meat, the Japanese are believed to have been infected through implants of the dried brain tissue. Known as dura mater, the tissue was widely used as a surgical patch for the membrane covering the brain until synthetic substitutes were developed. [Sheep and cattle dura mater may also have been used extensively -- the surgeon employed by the Lyodura company to supply dura mater himself died of CJD -- webmaster]
During the 1980s, as many as 100,000 Japanese received dura, and an untold number of them may have been exposed to CJD. Statistically speaking, 35 to 40 more people are likely to develop the disease during the coming decade, said Dr. Takeshi Sato, a neurologist and head of a commission that investigated the outbreak.
But it is impossible to know who may be infected. There is as yet no screening test and, like HIV, CJD has a long incubation period: It has been known to lie dormant in the brain for 16 years or longer before it awakens to kill its host.
Japan's CJD outbreak reinforces worldwide concerns about the danger of contamination of blood, organs and biological products by emerging infectious diseases--especially emerging viruses that may not be detected until long after their hosts have been distributed to and used in dozens of countries.
In addition to HIV--the virus that causes AIDS--and CJD, other viruses with long incubation or latency periods include herpes, chickenpox, hepatitis B and hepatitis C. The latter was identified only in 1989 and is a concern in blood and organ transplants, said Dr. Lawrence Mintz, an infectious-diseases specialist and professor at UC San Francisco's medical school. "It's inevitable that more long-incubation viruses will be found," Mintz said. "It's a survival strategy for viruses."
Of particular concern is the potential for unknown viruses to be transmitted from animals to humans through genetically engineered biopharmaceuticals. Some companies use animals as factories for producing human substances, such as insulin or hormones, by inserting human genes into the animals.
"When you put a human gene into a mouse, you can't be sure that there isn't something mousy in that final product that hasn't yet been discovered or isn't appreciated as a pathogen in man but may turn out to be so," Mintz said. "The reputable companies do a good job at anticipating this, and there's a lot of oversight, but the technology is just exploding" and so is the number of donor species, he said. The globalization of the medical industry means that small countries must grapple with these issues--even if they lack the regulatory resources to keep up.
"If HIV can happen, if mad cow disease can happen, I can't rule out the possibility of any other infectious disease of a similar nature occurring in any country," said Dr. Ermias Belay, an epidemiologist and CJD expert at the Centers for Disease Control and Prevention in Atlanta. "The only way to potentially control emerging infectious diseases is by anticipating them and getting ready for the unknown."
What worries critics in Japan is whether their nation's regulatory and medical establishments are up to the job... In a country where the ritual apology has been elevated to an art form but accountability remains elusive, she notes that no one has yet apologized or accepted responsibility for the CJD affair.
Not the German manufacturer, B. Braun Melsungen, which maintains that there is no proof that its product, Lydodura, caused CJD. This despite findings by the Centers for Disease Control and Prevention that at least 85 patients worldwide have developed CJD after dura implants and that the vast majority of cases were associated with Lydodura.
Not the Japanese distributor, which has said it never received an order from B. Braun to recall Lydodura from hospital shelves here in 1989, after the second report of a CJD case in a dura recipient.
Not the Health and Welfare Ministry, which admits that it received a February 1987 report from the Atlanta centers that Lydodura was the suspected culprit in a CJD case involving a 28-year-old Connecticut woman, a safety alert two months later from the U.S. Food and Drug Administration announcing that it was banning Lydodura imports to America, and subsequent reports of more deaths. The ministry says it had no duty to ban Lydodura sales in Japan because the CJD link was unproven.
Not the Japanese medical establishment, which says the manufacturer is responsible because it did not recall the Lydodura that was used in Tani's surgery in January 1989 and a 61-year-old woman's operation in 1991. Their surgeries occurred after articles had appeared from August 1987 onward in prominent Japanese medical journals about the emerging threat of CJD from dura.
The families of 15 CJD patients--only three of them still alive, including Tani--have filed civil lawsuits in Tokyo and Osaka against B. Braun, its Japanese distributor, and the Health and Welfare Ministry. Tani's husband, Sanichi, also has filed a criminal complaint of attempted murder against the current and former presidents of the distributor, Nihon BSS. So far, however, prosecutors have not indicted anyone, nor has any official in the health ministry or any doctor been reprimanded.
"Everyone says, 'We didn't know, we had no idea,' " said Tetsuji Abe, one of the attorneys for the plaintiffs. "But if they had learned anything from AIDS, they should have known."
1,500 Infected in Hemophilia Scandal: The CJD revelations come on the heels of a scandal in which about 1,500 Japanese hemophiliacs were infected with human immunodeficiency virus after the health ministry allowed pharmaceutical firms to sell high-risk, unsterilized blood products even after safer substitutes were available.
The Japanese language does not include a word for "accountability." But lately the English term has become a buzzword here among Japanese frustrated by a system that has stumbled from crisis to crisis in the 1990s but appears impervious to attempts at change. Reformers say that's partly because individual decision-makers are rarely held responsible for the behavior of their corporations or government agencies, though symbolic resignations and apologies are expected from a top official in order to quell scandals.
"The basic philosophy of the Japanese bureaucracy is to evade all responsibility," said Dr. Takashi Kitamura, a former researcher at the National Institute for Infectious Diseases in Tokyo, who translated into Japanese the U.S. warnings about the risks posed by dura in light of the Connecticut woman's death and published them in 1987.
A spokesman for the health ministry, Koichi Ando, conceded in an interview that his agency's response to CJD was "not necessarily adequate." He outlined steps that have been taken to improve information dissemination and oversight. However, he said the ministry, which had licensed sales of Lydodura in Japan, had no legal duty to act in 1987 based on a U.S. report of a single death, when the link to CJD had not been scientifically proved.
The Japanese media have had a field day revealing the embarrassing details of just how uninformed regulators were. The Asahi newspaper filed Freedom of Information Act requests in the United States--an increasingly common journalistic strategy here because it's nearly impossible to pry unflattering documents out of Japanese agencies--and got hold of an urgent fax sent by a senior Japanese health ministry official to the FDA in 1996.
The official wanted to know whether it was true that the FDA had issued safety alerts against Lydodura in 1987, and asked what other measures the Americans had taken to deal with dura mater. Japan finally banned dura imports in 1997.
The cases here, as with most such lawsuits, focus mainly on what B. Braun and regulators knew and when they knew it. These questions would be of purely legalistic interest were it not for the issue of how quickly authorities will be able to intercept peculiar infectious outbreaks in the future.
CJD transmission was first reported from a cornea transplant, in 1974. By 1978, when it was learned that the disease had also been transmitted by electrodes placed inside the cranium during tests, researchers began discussing the fact that the mysterious agent responsible for CJD wasn't killed off by usual sterilization methods.
The question for the courts is whether B. Braun or others knew or should have known the risks in time to save any of the Japanese patients, the majority of whom had surgery in the mid-1980s.
"It is not clear in this case that they knew," said Kiyohiko Katahira, a medical information specialist at Tokyo Medical and Dental University and an expert witness for the CJD patients. What is clear is that they could have known the risks, certainly as early as 1978, he said. "They could have known by reading published reports in leading medical journals."
But Sato, the neurologist who chaired the investigative commission, said CJD was such an obscure disease that it wasn't likely to register on the Japanese epidemiological radar. A neurologist might only see one case in 10 years, if that, so it is not realistic for people to have had a sense of urgency about a threat from dura," he said. Sato suggested that the Japanese media, which have focused on the lapses of the domestic establishment, should also probe the failure of German regulators to police the manufacturing practices of B. Braun, which he considers to be the main culprit.
According to epidemiologist Belay, Sato and FDA documents, B. Braun collected brain membranes from corpses in a number of European countries and dumped them together into a tank for processing. That meant that tissue from a single CJD-infected donor could contaminate an entire batch. Moreover, the company did not have a system for tracking individual donors, and German authorities later learned that the causes of death listed in some of the records that B. Braun did have were false, according to Japan's NHK television and Nakagawa's office.
By contrast, U.S. manufacturers of dura were identifying donors by number and separately sterilizing tissues taken from each corpse to reduce the risk of disease transmission. Unlike Japan, the U.S. had never approved Lydodura for sale, and only two CJD deaths believed to have been caused by the product have occurred in the States, Belay said. Cases have also been reported in Australia, France, Germany, Italy, New Zealand, Spain, Britain and Canada. Worldwide, all but half a dozen dura cases are linked to exposure to Lydodura, Belay said.
B. Braun, in a statement, said it improved its donor screening and sterilization procedures in 1987, as soon as the first CJD case was reported, and ordered all distributors worldwide to recall the products in 1989, after a second death was reported in New Zealand.
The challenge now facing health officials is how to protect the blood supply and other biological products from contamination caused by diseases with such long incubation periods. One of the CJD victims in Japan is a 17-year-old boy who had brain surgery for a tumor in 1983 as an infant, was pronounced completely cured, then developed his first symptom 15 years later.
There are no confirmed cases of CJD transmission by blood, said Belay, but nevertheless blood donors in the U.S. and Japan now are asked about CJD risk factors. In Japan, those who report having received dura implants are not allowed to donate. Japanese authorities have identified eight cases of blood donation from people who later developed CJD, but they were able to trace and recall only four of the blood lots.
There is nothing to be done for Takako Tani, who lies in a coma at home here in Koseicho, a rural town about a 30-minute train ride from Kyoto. She is cared for around the clock in shifts by her husband and three daughters, who are now in their early 20s. The nightmare period, when she could not find the toilet in her own home and screamed in terror at night in the barred room of the psychiatric hospital where she was finally diagnosed, is over.
Though she has shown no signs of recognizing her family for nearly four years, they refuse to warehouse her in a hospital. Classical music plays all day in her sunny room, which is decorated with bright paper cutouts made by her daughters, cards and posters from well-wishers and the pink flowers she loved. On the wall is a family picture from the day in the spring of 1996 when she climbed Diamond Head in Hawaii, just before falling ill.
Sanichi Tani, 51, and his daughters bathe Takako every three days, massage her limbs, put cream on her clenched hands, feed her through a tube, suction out her nose up to 30 times a day, and take her to sit outside in good weather. She is unresponsive, though sometimes she moans. Still, Tani wants to believe this tender care has kept his wife alive for four years, whereas most CJD patients are dead within one.
"I don't know how much love I have, but I am trying," he said. "She did everything for our family for 20 years, so I feel I must repay her as best I can." His only remaining hope is that Takako will live long enough for him to report to her a successful verdict in her lawsuit. A decision is expected late this year or early next.
In lieu of her testimony, Tani has made public his wife's last diary entry, in disintegrating handwriting that careens across the page: "May 26. Sunny. Not very hot. Last night I had a scary dream. They were trying to erase all my dreams. It became pitch dark and I was afraid."
Experts are claiming that the cases, several of which are the subject of court actions by relations, are part of a "tragic time bomb" that will claim many more victims around the world. Figures show that there have been at least 23 CJD deaths caused by infected brain grafts and an estimated six people in Britain are thought to have caught the human equivalent of "mad cow" disease after having tissue implants taken from infected corpses. Several more cases have emerged in the United States.
The British victims include Pauline Nuttall, 45, who died in May 1991 eight years after having a brain tissue implant following surgery for a benign tumour. Another, Brian Bowler, 30, died in 1990 at Luton and Dunstable Hospital four years after the tissue, called dura mater, was used to close a gap at the base of his skull following surgery.
Professor Robert Will, director of the Government's CJD Surveillance Unit based in Edinburgh, said that the practice of using the tissues of corpses in brain surgery had been carried out in many countries for many years. It had been thought that the risk of infection was remote.
The tissue was used to heal a patient's brain membranes after surgery. But increasing concern over CJD, triggered by rising alarm that a new form was looming linked with eating infected beef, prompted several countries, including Britain, to ban the use of cadaver brain tissue in surgery in the very early 1990s.
Professor Will said that brain surgeons in Britain had switched to using tissue taken from a patient's leg. But critics claim that the Japanese authorities failed to act swiftly to reduce the risk despite reports >from the United States in 1987 linking transplants of dura mater with CJD.
Japan ordered the withdrawl of the cadaver-derived tissue only in March 1997. The Japanese cases, by far the largest number of any country in the world, have been linked with a German company, B Braun Melsungen, which supplied the cadaver membranes under the brand name Lydodura. Professor Will said that of the six British CJD cases linked with brain surgery, five had been linked to products from Germany. He said it was suspected that vats containing brain tissue from various corpses had come into contact with some from a single infected cadaver.
Sanichi Tani, whose wife Takako is suffering from terminal dementia after becoming infected following surgery in 1989, is suing the German manufacturer, its Japanese distributor and the Japanese Health and Welfare Ministry. Mr Tani has also brought a criminal complaint of attempted murder against the former and present heads of Nihon BSS, the distributors.
Tetsuji Abe, one of the lawyers for the families, said: "Everyone says that they did not know and had no idea. But if they have learnt anything from Aids, they should have known".
Wed, Apr 5, 2000 Reuters World ReportFrench animal feed makers sought to defend themselves on Wednesday as farmers and consumers grew increasingly angry over the country's growing number of cases of mad cow disease. Animal feed cooperative trade group Syncopac said it was worried by the number of cases of mad cow disease, or bovine spongiform encephalopathy (BSE), reported this year in France.
But it rejected criticism in French newspapers and magazines saying its members were tired of being the target of suspicion. "Syncopac will no longer tolerate the suspicion that has hit the animal feed profession while there are major uncertainties over the causes of contamination," the group said. It noted that legal proceedings already under way had not shed any light on who might be responsible for the growing number of cases.
That echoed comments made last week by another animal feed makers' group, SNIA, which attacked a lawsuit filed by farmers accusing as-yet unnamed parties of helping spread BSE. "This new strategy will only heap discredit on your production and will sow the seeds of trouble among consumers," SNIA said in a letter addressed to farmers.
France has reported 13 cases of BSE this year, bringing to 93 the total number of cases discovered since 1991. The number of cases as of early April represented about half the number reported in all of 1999, putting France on course for another record year of BSE.
French livestock producers have increasingly vented their frustration on animal feed manufacturers as the number of cases showed no signs of slowing down. Last week, some 200 farmers protested outside the gates of a feed manufacturer in the Ain region of east-central France after the region's first case of BSE was discovered. The Ain farmers' union also filed a lawsuit accusing unnamed parties of "fraud, falsifications and spreading a disease."
France's farm ministry has said the number of fresh BSE cases should die down after 2001 as the disease's incubation period is five years and tougher controls were placed on animal feed in late 1996.
Mon, Apr 3, 2000 Reuters World ReportA herd of 33 cattle was destroyed in northern France after the discovery of the country's 13th case of mad cow disease this year, the French Agriculture Ministry said on Monday. The discovery marked the 93rd case in France since 1991 when the first cases of the fatal brain-wasting illness bovine spongiform encephalopathy (BSE) -- so-called mad cow disease -- were first diagnosed.
However, some experts believe that cases of the illness might be going unreported among the country's 21 million cattle, infecting meat still entering the food chain. France is due to hold a tender later week for a suitable large-scale testing method for mad cow disease.
BSE, which forced hundreds of thousands of cattle to be destroyed in Britain, has been linked to new-variant Creutzfeldt-Jakob Disease -- a similar illness in humans for which there is no known cure. The European Commission, the EU executive body, has taken France to court over its refusal to lift a ban on British beef even though EU states agreed in August to end an export embargo.
The case came amid concern at the failure to arrest a steady increase in the number of affected animals in France over the past two years. France recorded 18 cases of mad cow disease in 1998, 30 in 1999, and so far this year there has been on average nearly a new case every week.
A possible explanation, according to the ministry, is that until 1996 contaminated animal feed, still legal for chickens and pigs, was mistakenly fed to cows.
The average BSE incubation period is five years which means cows that ate the contaminated feed in 1996 could develop BSE up to the end of 2001, the ministry said.
COMTEX Newswire Thu, Mar 30, 2000Germany Thursday formally ended its import ban on British beef, the Environment Ministry in the regional government of North Rhine-Westphalia said in a statement. The ban had been imposed because of fears about BSE, or mad cow disease. The ministry said it had received a federal regulation which permits the import of UK beef with immediate effect.
The upper house of the German parliament had approved the end of the ban Mar 17. The regulation puts parliament's decision into effect. Baerbel Hoehn, the Environment Minister in the North Rhine-Westphalia regional government, said direct imports from the UK will be labeled XEL. But Hoehn is concerned that re-exports of UK beef from other EU countries will not necessarily be labeled, a ministry spokesman said. She advised German consumers to ask about the country of origin of beef in the shops.
Mon, Apr 3, 2000 By Rachael Crofts, Consumer Affairs Correspondent, PA NewsButchers who sell raw and cooked meats are to be subject to strict licensing regulations to reduce food poisoning, the industry's newly-appointed watchdog announced today. The Food Standards Agency also aims to reduce salmonella in chickens by 50% and to name and shame food producers and retailers who fail to meet safety standards, its chairman said .
Chairman Professor Sir John Krebs told reporters at a press conference in London that the new body would be unique in its openness and transparency. The agency will hold its meetings in public and will also produce league tales of how environmental health departments perform across the country.
Deputy chair Suzi Leather described the agency, which came into force on April 1, as a "holy grail" for consumer groups which would banish the "cloud of secrecy" that had "characterised the food industry for so long". Sir John said regulations for the licensing of butchers would be put before Parliament today, with the statutory scheme coming into operation in the autumn.
More than 12,000 butchers across the UK will receive training on health issues before being issued with a licence which will be reviewed on a 12-monthly basis. The cost to butchers will be 100 pounds a year, which covers the annual inspection by local authority health inspectors, who will also have powers to revoke the licence from any premises failing to meet standards, he said. "The licensing and the associated training of butchers will ensure that the highest standards of food handling are maintained."
The agency also committed itself to reducing the levels of salmonella in UK-produced chicken by 50% over the next five years. The agency said the infection was found in 20% to 30% of chickens on retail sale, despite widespread claims by supermarkets and retailers that it had been virtually eliminated. The figure represents a drop from 1993/94 when the bacteria was evident in 34% to 44%, the agency said.
Sir John said: "We will aim to reduce levels of salmonella in UK-produced chickens on retail sale by at least 50% during the next five years. This will involve working with the industry to set new and ever higher standards." The agency will champion the interests of consumers and will make transparency a key part of its work.
Sir John said: "The way we do things will be different from in the past. As well as putting the consumer first, we will be open and accessible. "We will set and defend new standards for openness. We will work in a way that will encourage people to contribute to our thinking and see how decisions are made."
... He said the agency also vowed to stamp out misleading labelling such as "country style" and "85% fat free" and would also focus on the issue of food and nutrition in the poorer sectors of society. It has also taken over responsibility for the Meat Hygiene Service and will conduct a review of BSE controls to be published by the end of the year.
The agency, which has an annual budget of 150 million, will be funded from the public purse to the tune of 110 million and the remaining 40 million will be raised through levied charges within the industry, Sir John said.
The Consumers' Association welcomed the new agency and urged it to act on behalf of consumers. Association director Sheila McKechnie said: "It is essential that the agency does not repeat the mistakes of the past and promotes the highest standards for UK consumers.
"As the board of the agency will have limited consumer representation, it is going to be essential that the agency quickly establishes ways of finding out about consumers' concerns - and takes action to address them. "The British public want to have confidence in the food they eat. The agency must not let consumers down."
Mon, Apr 3, 2000 By Rachael Crofts, Consumer Affairs Correspondent, PA NewsPlans by the new Food Standards Agency to impose strict licensing regulations on butchers were tonight described as unnecessary and poorly thought out by one meat industry organisation. The Guild of Q Butchers (correct) said the scheme would force another "unnecessary financial burden" on its members.
The new agency, which came into force on April 1, announced plans today for the training scheme and annual licence to reduce cases of food poisoning. It also vowed to reduce salmonella in chickens by 50% and to name and shame food producers and retailers who failed to meet safety standards. Chairman Professor Sir John Krebs said that the new body would be unique in its openness and transparency.
But the Guild of Q Butchers chief executive Richard Jones said: "As an organisation of quality retailers committed to the highest standards of food safety, product quality and customer service, we support any move which improves standards in the industry and helps regain consumer confidence in meat and meat products. "However if the new agency is to continue the string of poorly thought out initiatives such as licensing, compulsory beef labelling and metrication it will quickly lose its credibility." He said Guild members already paid 215 a year for an inspection which exceeded legally required standards and no purpose would be served by making them pay a further 100 for another inspection.
Under the agency's plans, more than 12,000 butchers across the UK will receive training on health issues before being issued with a licence which will be reviewed on a 12-monthly basis. Professor Krebs said: "The licensing and the associated training of butchers will ensure that the highest standards of food handling are maintained...."