Bad news closes 1998
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nvCJD cases up estimated 30% in 1998
US military blood donors: first-hand account
Worst year yet for diagnosed cases of BSE in Ireland
Change in disease status of Liechtenstein because of BSE
FDA urged to defer donations by UK residents
UK haemophilic patients ok so far
500 delegates attend secret meeting in Brussels
BSE Inquirey: "Draft Factual Accounts"
Beef on the bone decision to be based on health
2,837,582 cattle slaughtered so far; 173,126 confirmed BSE cases

nvCJD cases up 30% in 1998

4 Jan 98 webmaster commentary
The total for the first 10 months of 1998 -- 10 cases -- is equal to the total for the whole year for 1997 and 1996. In other words, the rate in 1998 is estimated to be slightly higher ["20%"], with about 12 expected for the year. 1996-97 may be slightly inflated due to 'catch-up' relative to 98. Changing or improved ascertainment (14-3-3 or tonsil or brain biopsy) may make comparisons invalid.

Newspapers have been saying 34 UK cases + 1 French case, which, if reliable, would make at least 11 UK cases for 1998. The next table will be published on Monday 4 January 1999 (though these are often late) and will cover data to 30 Nov 98. The table after that will be published on Feb 2, 1999. That one will have official full year statistics for 1998. The December 1998 issue (No.29) of the BSE Enforcement Bulletin, just published, is said to give a more up-to-date total than that. It states that, as at 21 December, there were 34 confirmed cases of nvCJD. No date has been set for release of statistics from tonsil or appendectomy samples.

There is still no detailed word on genotypes or karyotypes of people affected. We could simply be looking at rather separate epidemics of chromosome 20 partial trisomics, followed by an enhanced promoter allele subpopulation, followed by a much larger epidemic in the main population. The first two classes are symptomatic earlier due to a prion protein dosage effect (not confirmed in humans).

Thus, until more is known, it is simply not possible to infer anything meaningful about the whole epidemic [which is the sum of all the individual epidemics] from incidence numbers or their trends because we have no idea what subpopulation they represent. People with met/met at codon 129 show symptoms earlier than val/val because of the like/like rule (BSE cattle are always met/met). The graphic is for conceptual and illustrative purposes only as there is no data past 1998.

Assuming that the account below is accurate, the UK has been running close to 150 referals a year with 1 in 5 confirmed as nvCJD or 30 cases per year. At 200 referals per year, this would be a rate of 40 cases per year for 1999 or 33% increase.

The DoH page has 134 referals for 1996, 160 for 1997, and 111 for first 10 month of 1998 [133 annualization evidently low] which are compatible with the story. Possibly people have been misreading this table, not realizing that the confirmed column does not relate deaths to the referal column of that year but to several years earlier.

The implication is that the referal column has proven to be a fairly reliable leading indicator when divided by 5. There have been 910 total referals since 1990 so 182 cases of nvCJD would emerge from waiting out the present set of referals according to this interpretation. If this reading is correct, then there is bad news on both the epidemic 'to date' and in the newly seen possible rate increase for the coming year.

Another reason, beyond not trying to read too much into data that a GS-5 clerk puts on the DoH site, beyond poly A variability etc, for not trying predicting the course of the epidemic is the very sharp spiking of onset time (1%) seen over and over in many labs in rodents (some not all that inbred). For purposes of simple-minded comparison, a group of mice that come down in 182 days 2 days corresponds to a group of isogenic people aged 40 160 days. You would not be able to infer a whole lot about the epidemic by the time this group had reached 38, any more than you could about the mice at day 173.

Here are today's [4 Jasn 98] DoH released statistics to 30 November 1998 with the October and September data beneath them. There were evidently 2 confirmed cases of nvCJD in the month of November relative to a referal rate of 26 and 4 confirmed cases in October relative to 34. I expect the DoH total for 1998 will be in the 13-15 range, making the DoH epidemic total 36-38.

In the last 2 months, there have been 22 confirmed CJD of which 6 were nvCJD, 14 sporadic, 1 growth hormone, and 1 familial.

1998   137   32        1         2        0       12     47    Nov 98
1998   111   27        1         2        0       10     40    Oct 98
1998    77   18        0         1        0        6     25    Sep 98

Total number of definite and probable  cases of nvCJD = 35.
The next table will be published on Monday 1 February 1999.

30 Dec 98 - CJD fears put watchdog under strain

By Steve Connor, Science Editor
Independent ... Wednesday 30 December 1998
An increase in the number of people suspected of suffering from Creutzfeldt Jakob disease is straining the national surveillance system for the human version of "mad cow" disease. Bob Will, head of the National CJD Surveillance Unit in Edinburgh, has consulted the Department of Health about the need to increase the unit's resources to cope with the extra workload.

Dr Will, a clinical neurologist, said the past few months had seen a higher number of people being referred to the unit by doctors concerned that their patients were suffering from CJD, some of whom may be suffering from the human form of bovine spongiform encephalopathy (BSE). The unit, which was set up in 1990, was designed to cope with a maximum of 150 referrals a year. However, if present trends continue , the unit will have to deal with up to 200 referrals in 1999 .

"If it gets higher than 150 referrals we will need extra staff and if it gets higher again we'd have to consider the way the whole system works," Dr Will said. He has resigned from the government's Spongiform Encephalopathy Advisory Committee, on which he has served since 1990, to concentrate on the surveillance programme.

There have been 34 deaths from new variant CJD - human BSE - and scientists have not seen a significant increase in cases to indicate an epidemic. However, the first indication of an epidemic would be an increase in referrals to the unit .

"It's significant in terms of the workload. In terms of meaning something in relation to CJD I think that is impossible to say," Dr Will said. "We have been quite busy in the past few months but I don't think you can conclude that means something definite epidemiologically at the moment."

For every five patients with suspected new variant CJD, only one proves definitely to have it - cases are usually confirmed by post-mortem tests on the patient's brain.

"We've had a few more referrals than our previous experience in the past few months," Dr Will said. He added that the unit looks particularly carefully at suspected cases of CJD in the under-50s, who are at greater risk of the new variant of the disease.

Scientists believe it is too early to say whether there will be an epidemic of "human BSE" because of the uncertainty over both the time it takes for the disease to incubate and the amount of infected material that entered the human food chain.

Note: "Preliminary-opinion of the SSC on a method to assess the geographical BSE-Risk of Countries or Regions (adopted on 10 December 1998, open for comments until 15 January 1999)." is now downloadable (pdf).

Open Letter to Inquiry Secretariat

"Hello Inquiry Secretariat ,

During his evidence at the Inquiry, Dr Robert Will, Director of CJD Surveillance Unit, showed slides of graphs of incidence of nvCJD plotted against CJDSU's best estimates of dates of clinical onset.

Although Will described them in words while displaying them, the graphs have not been made publicly available on the Inquiry Web site.

The published nvCJD statistics only show annual figures for deaths. Attempts to carry out any calculations or draw inferences from these figures are hampered because of unknown variable incubation periods, and wide variability in durations of clinical illness. The calendar year is far too coarse a time-scale, and best estimate of date of clinical onset is far more meaningful than date of death expressed on any time-scale.

The data for best estimates of dates of clinical onset would therefore be of far greater public interest than the official statistics of annual deaths. Accordingly, I would request that the graphs presented to the Inquiry by Robert Will be made available on the Inquiry Web site.

Regards,
J Ralph Blanchfield
4 Jan 98"

Worst year yet for diagnosed cases of BSE in Ireland

Dec. 24/98 The Irish Times Sean MacConnell, Agriculture Correspondent
This has, according to this story, been the worst year on record for Bovine Spongiform Encephalopathy (BSE) in the Irish herd, it emerged last night with the publication of the December figures. These statistics showed that the 10 new cases diagnosed in December brought to 83 the total of BSE-infected cows found in the State this year. Last year there were three fewer cases and in the previous year, 1996, which was a crisis year for the European beef industry, there were 74 cases. Department of Agriculture officials last night pointed out that the infection rate in the Republic of Ireland is very low given the fact that there are 7.8 million cattle in Ireland, an infection rate of 1:100,000 animals.

A veterinary source was quoted as saying, "The number of cases we have had in Ireland in the last nine years is only 352. That figure is equal to what the British authorities were finding in a single week when the disease was at its height in that country."

Experts were cited as believing that the disease will disappear from the Irish herd in about three years when the last animals to have been fed on contaminated cattle rations are diagnosed.

The British authorities had, according to this story, expected to be rid of the disease by 2000 but they discovered that cross-contamination of cattle rations had continued at mills where pig and poultry rations were being processed by the same rollers.

Two years ago, following advice from the British authorities, Ireland moved to separate pig and poultry feed processing and now the plants are, according to this story dedicated ones and are not allowed to mix the processing. This should eliminate the main cause of the disease unless farmers feed pig and poultry rations to their cattle, which could perpetuate BSE.

Change in disease status of Liechtenstein because of BSE

Dec. 24/98
Federal Register
Volume 63, Number 247
Page 71209-71210
[DOCID:fr24de98-1]
AGENCY: Animal and Plant Health Inspection Service, USDA.
ACTION: Interim rule and request for comments.

SUMMARY: We are amending the regulations by adding Liechtenstein to the list of regions where bovine spongiform encephalopathy exists because the disease has been detected in two bovine animals in that region. The effect of this action is to prohibit or restrict the importation of ruminants that have been in Liechtenstein and meat, meat products, and certain other edible products of ruminants that have been in Liechtenstein. This action is necessary to reduce the risk that bovine spongiform encephalopathy could be introduced into the United States.

DATES: Interim rule effective December 18, 1998. Consideration will be given only to comments received on or before February 22, 1999.

ADDRESSES: Please send an original and three copies of your comments to Docket No. 98-119-1, Regulatory Analysis and Development, PPD, APHIS, Suite 3C03, 4700 River Road Unit 118, Riverdale, MD 20737-1238. Please state that your comments refer to Docket No. 98-119-1.

Comments received may be inspected at USDA, room 1141, South Building, 14th Street and Independence Avenue SW., Washington, DC, between 8 a.m. and 4:30 p.m., Monday through Friday, except holidays. Persons wishing to inspect comments are requested to call ahead on (202) 690-2817 to facilitate entry into the comment reading room.

FDA urged to defer donations by UK residents

 THE LANCET, Volume 353, Number 9146, 2 January 1999 Alicia Ault
[This seems to be a recommended change from the 9/8/98 FDA Change to the Guidance Entitled "Revised Precautionary Measures to Reduce the Possible Risk of Transmission of Creutzfeldt-Jakob Disease (CJD) by Blood and Blood Products". which says, website fact sheet: "

It is FDA's current thinking that, consistent with the procedures specified in the December, 1996 memorandum, plasma derivatives should be retrieved, quarantined, destroyed and consignees notified only in the event that in-date products were manufactured from a donor who developed new variant CJD." ie that the blood from all other CJD donors is somehow ok. This website strongly disagrees.

A majority of a US FDA advisory committee recommended on Dec 18 that the agency should develop a new policy outlining deferral of blood donations from people who might have resided in or visited the UK during the peak years of the bovine spongiform encephalopathy (BSE) epidemic.

The panel stopped short of saying how to limit these donors, but urged the US blood-banking industry to start surveying donors to determine the potential level of exposure to BSE. Donors should be asked whether they lived in or visited the UK between 1980 and the present, and how long they were there, said the panel.

BSE casts a long shadow

The concern is that these donors could transmit variant Creuztfeldt-Jakob disease (variant CJD) through transfusions of whole blood or components, or through plasma products. The panel heard from UK and other experts that there has been no such disease transmission to date in any country.

If any donor is found to develop variant CJD, blood components should be recalled, said the advisors. They said that plasma derivatives do not need to be withdrawn.

The US blood-banking industry said blocking donations from anyone who had lived in or visited the UK during the past two decades could be disastrous. According to preliminary surveys by the American Red Cross, 10.7%, or one million units, of the current blood supply would be lost, and one million new donors would have to be found.

Retrospective neuropathological review of prion disease in UK haemophilic patients.

Thromb Haemost 1998 Dec;80(6):909-11
Lee CA, Ironside JW, Bell JE, Giangrande P, Ludlam C, Esiri MM, McLaughlin JE
[Haemophiliacs, because they receive frequent injections of specialty blood products, have been the unwilling canaries in the coal mine for previous blood-borne illnesses such as AIDS and hepatitis. They do not receive injections of material derived from cattle. For BSE to have reached them by now, a blood donor would need to be in an undiagnosed stage of nvCJD, infectious material would need to survive various purification steps, and many years of incubation for a low titre exposure would need to pass. Note haemophiliacs, like others in the UK, could also have received oral doses from meat. Higher oral doses are balanced by lower efficiencies of infection. -- webmaster]

In 1996, the CJD surveillance unit in Edinburgh, UK described nvCJD which was thought to be the human equivalent of bovine spongiform encephalopathy (BSE). The identification of prion protein in the tonsil of an affected individual has raised the question of transmission of nvCJD via blood products. This study examines the post mortem brains of 33 patients who were treated with clotting factor concentrate of predominately UK donor source during the years 1962-1995.

The brains were examined by conventional histological methods and also for the prion protein using monoclonal antibodies KG9 and 3F4. No evidence of spongiform encephalopathy was found and the immunocytochemistry was negative for PrP in all cases. It is concluded that, at present, there is no evidence for the transmission of nvCJD via clotting factor concentrate to patients with haemophilia.

European Parliament holds secret 30 Nov 98 conference

J Ralph Blanchfield  Listserve 4 Jan 99
"The just-published December issue (no.29) of the BSE Enforcement Bulletin contains a report that the European Parliament held a conference on lessons to be learned from the BSE crisis in Brussels on 30 November and 1 December 1998, attended by 500 delegates. Copies of some of the papers are said to be available on the DGXXIV Web site at which, however, has so far proved inaccessible. [The EC search engine shows many BSE documents]

The European Commission has issued its Second Bi-Annual BSE Follow-up Report in pdf only, 59 pages, dated 18 November 1998. It updates action taken in respect of research, inspections and control, implementation of Community law, the UK beef ban, legislation, trade issues, CJD, and fraud and financial aspects. It includes tables showing, country-by-country and year-by-year the number of BSE cases, with totals to 5 November 1998.

The EU commission has published an updated 3rd edition of its BSE "Vademecum" -- information for consumers in pdf format, 63 pages long.

The 500 delegates are described as "representing consumer, farming and industry interests; food scientists and academics and MEPs, as well as members of the public". The "24 principal speakers included the President of the European Commission, Jaques Santer, Commissioners Fischler and Bonino, Vice President of the European Parliament, David Martin, and the EU rapporteurs on BSE, Mrs Roth Behrendt and Mr Boge". It is stated that The outcome of the conference will be taken account of in the final report on the BSE crisis to be prepared by the EU rapporteurs and presented to the Parliament before its term ends in June 1999".

I wonder how any of the 500 delegates knew about this conference or how to register for it. It seems to have been arranged with extraordinary secrecy and complete lack of any prior announcement. I note the mention of "food scientists", and I can say that IFST received no notification whatsoever of this event."

Roland Heynkes writes:

"I was there on 30 November, but there was nothing worth to make a paper from it. The politicians said nothing new and Prof. Anderson demonstrated that he thinks into the right direction but lacks the necessary knowledge about TSEs. Interesting was only the possiblity to meet some people after the conference.I accidently heard it from a coworker of MEP Roth Berendt and registered by email."

Draft Factual Accounts


 The BSE Inquiry Web site at 
now carries the first three of what will be a series of Draft Factual
Accounts, which can be accessed and downloaded.  Those already there relate
to the Southwood Committee (111 pages), the Tyrell Committee (52 pages) and
SEAC (66 pages). A list is given of twenty other topics of DFAs still to
come.

Draft Factual Accounts

The BSE Inquiry is publishing a number of draft factual accounts for consultation. The accounts will cover the work of the main scientific advisory committees, key events such as the ruminant feed ban and the specified bovine offal ban, slaughter and compensation policy, CJD surveillance and care, risk assessment, bovine by-products, statistics, scientific research and several other areas.

The documents are designed to be full accounts of the factual evidence on certain topics which the Inquiry has gathered in the last twelve months and to help the Inquiry in the rest of its work. They are not intended to deal with every aspect of the evidence and they will not include judgements or conclusions.

The summaries are not intended to cover all the areas of the evidence, to make any judgements about the implications of the facts or to point to any conclusions. These will be for the Committee"s report to Ministers on 30 June 1999. The Draft Factual Accounts (DFAs) are intended to help the Committee of Inquiry in their further work.

You are invited to point out any errors or material omissions. If you have any comments which do not involve amendments to the factual account please put them in a separate document.

Comments on this Draft Factual Account should be sent to:

The Secretary
The BSE Inquiry
6th Floor
Hercules House
Hercules Road
London SE1 7DU

E mail to : inquiry@bse.org.uk

Comments should reach the Secretariat by 21 January for them to be most
useful to the Inquiry.

nvCJD and U.S. Service Personnel

17 Dec 1998  Terrence C Goodwin USAF Retired  
"To Whom It May Concern: "Although I realize I missed the deadline for submitting comments to the TSE Advisory Committee (I did not receive the notice of this meeting until 05 Dec 98), I hope you will take the time to review my comments." "I am an Air Force retiree who has been following Creutzfeldt-Jakob Disease and TSE for several years due to my study of human biology in college. I am one of an estimated 50,000 or more military personnel and their families who were stationed for various lengths of time in the United Kingdom in the 1980s and 1990s. It was not unusual for the military "chow halls" and "in-flight kitchens" to serve British beef."

"Additionally, British beef was part of the menu at private eating establishments either directly on base (i.e., Burger King as well as native British establishments) or in close proximity to U.S. bases. Finally, British beef was also sold at the various shoppettes and commissaries on U.S. bases. It seems obvious that a number of service personnel and their families consumed British beef during their assignments to the United Kingdom. Whether that beef was infected (sic) with BSE will not be known until nvCJD symptoms become noticeable in these personnel." "It should also not come as a surprise that U.S. military personnel probably volunteer as blood and blood product donors at a higher rate than the general population. Many see it as a duty to help out their local communities not unlike serving their country. It does not require a large jump in deduction to realize that a number of personnel who were stationed in Britain and ate British beef have since returned to the U.S. and have continued to be active blood donors." "If you doubt the efficacy of these statements let me provide you with a brief history of my own case. I began donating blood soon after joining the Air Force in 1976 and continued donating on a pretty regular basis up until this last summer (1998). Between 1982 and 1994, I traveled and was stationed in Britain close to a dozen times or more. These temporary duty assignments lasted anywhere from three to four days up to 90 days at a time. I can't think of a single time that I was in Britain that I did not eat British beef. I would be surprised if there were not thousands of other service personnel who could tell you a very similar story as my own. Once I was convinced of the correlation between BSE infected beef and the development of TSE or nvCJD in humans, I could no longer conscientiously donate blood knowing that I may be carrying and thus passing on the TSE pathogen (sic) to others through my donated blood." "In my opinion, given the causal relationship between BSE and TSE in humans, screening those blood donors who have resided in Great Britain during the 1980s and early 1990s is a prudent course to take. Currently individuals who lived in Africa since 1977 are screened from giving blood to prevent the possibility of passing on the HIV virus. Though TSE does not destroy the infected individual as quickly as AIDS does from HIV, CJD/TSE is no less lethal in its course and outcome, and as yet, I know of no known drugs to slow down its course (unlike HIV). Thank you."

Sincerely,
 Terrence C Goodwin
USAF Retired

Beef on the bone decision to be based on health

Tue, Jan 5, 1999 Reuters World Report
Oxford, England - Britain will decide on whether to lift its ban on the sale of beef on the bone on the basis of expert health advice, not political considerations, farm minister Nick Brown said on Tuesday.

Addressing the annual Oxford Farming Conference, he stressed the serious consequences of the - albeit slim - chance of humans catching so-called 'mad cow' disease BSE from eating infected cattle.

"We're not talking about stomach ache. We're talking about death with new variant Creutzfeldt Jakob Disease," he said, referring to the human version of BSE.

In December British scientists said the risk of bovine spongiform encephalopathy (BSE) transmission from the bone marrow and dorsal root ganglia of cattle had declined compared with a year ago and now was very small, raising hopes of an early end to the ban, imposed by Brown's predecessor Jack Cunningham the previous December.

Scientists on the government's Spongiform Encephalopathy Advisory Committee (SEAC) estimated that there will be 43 BSE-infected cattle entering the food chain in Britain in 1999. This compares with an estimated 99 cases in 1998 and 184 in 1997. At the peak of the BSE epidemic in 1989, an estimated 200,000 infected animals were slaughtered for human consumption in Britain.

Welcoming the report at the time, minister Brown predicted that the government would soon be able to lift the ban on the sale of beef on the bone.

BSE Enforcement Bulletin #29 of Dec 1998

6 Jan 99 MAFF web site
MAFF'sBSE Enforcement Bulletin No 29:

Up to 27 November, 2,646,173 cattle participated in the over 30 months slaughter scheme plus 181,409 (6.4%) went through the incinerator queue, total 2,837,582 animals.

There were a total of 173,126 confirmed cases of BSE of which 37,779 (21.8%) were born after the ruminant ban.

The total number of new cases for 1998 (to 27 November) was 2,239 (cf. 4,312 for 1997, 8,016 for 1996).

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