Monday, May 19, 2008

SPORADIC CJD IN FARMERS, FARMERS WIVES, FROM FARMS WITH BSE HERD AND ABATTOIRS

SPORADIC CJD IN FARMERS, FARMERS WIVES, FROM FARMS WITH BSE HERD AND ABATTOIRS

‘The first farmer’ – August 1992

5.7 At the beginning of August 1992, Dr Will confidentially informed Dr Ailsa Wight (DH, senior medical officer with responsibility for TSEs), that a probable case of CJD had occurred in a 60-year-old farmer whose farm, in the Manchester area, had a history of BSE. Dr Wight passed on this information to Sir Kenneth Calman (CMO) on 13 August 1992, stating that the CJD patient was alive and had been visited by the CJDSU.188 Although unconfirmed, the diagnosis was considered likely to be CJD on clinical grounds. Dr Wight advised that: There is no direct evidence that the two events (BSE and CJD) are linked and Dr Will feels they are probably a coincidence. Despite the rarity of CJD, it was perhaps only a matter of time before this situation arose, given the large numbers of people employed in the agricultural and related industries, and the fact that BSE cases now total over 65,000.189

5.8 This ‘first case’ of CJD in a cattle farmer was discussed by SEAC190 at their 13th meeting on 15 October 1992.191 Dr Will informed the meeting that one of the farmer’s cows had confirmed BSE in 1989 and that the farmer had developed CJD two years later.192

5.9 Dr Will informed SEAC that he intended to publish a report of his study of this case in a scientific journal ‘which would probably draw the conclusion that there was no evidence that this was not a chance occurrence of normal disease’. Dr Will also reported that his studies at the CJDSU had failed to reveal a correlation between occupational backgrounds and CJD to date.193

5.10 On 22 October 1992, a minute from Mr Thomas Murray (SEAC DH Secretariat) informed the Secretary of State about the SEAC meeting and the fact that the farmer had now died.194 He noted that the diagnosis of CJD had been confirmed by pathology and that the CJDSU had also ruled out iatrogenic or familial CJD, as well as exposure to cattle brain. He commented that the SEAC meeting had come ‘to the view that all indications suggested that it was a typical sporadic case of CJD. However in view of the history it is hoped to carry out further laboratory studies to try to confirm this.’ 185

YB89/10.26/3.1 186 YB89/10.26/3.2 187 YB89/11.20/11.1 188 YB92/8.13/2.1–2.2 189 YB92/8.13/2.1–2.2 190

SEAC – Spongiform Encephalopathy Advisory Committee. This Committee was set up after advice from the Tyrrell Committee. Dr Will was a member of SEAC from its outset 191

YB92/10.15/2.1–2.8 192 YB92/10.15/2.4 193 YB92/10.15/2.4 194 YB/92/10.22/1.1–1.2

EMERGENCE OF VARIANT CJD 35 5.11

Dr Will published his report of the case, ‘Creutzfeldt-Jakob Disease in an Individual Occupationally Exposed to BSE’, as a letter in The Lancet on 6 March 1993.195 The letter concluded that ‘CJD in our case is most likely to have been a chance finding and a causal link with BSE is at most conjectural’. The letter noted that the only possible direct route of cross-contamination was that the farmer had drunk pooled milk from his herd which included that from the affected cow, but that epidemiological evidence had largely precluded milk as a route of transmission in spongiform encephalopathies.

5.12 This letter created much media interest over the following few days, and its contents were reported in The Times,196 Today,197 Daily Express,198 Daily Mail,199 and Daily Telegraph which also reported Mr Kevin Taylor (Assistant Chief Veterinary Officer, MAFF) stating ‘I don’t think that a link between this case and BSE is even conjectural’ and rejecting fears that the farmer might have contracted the illness from milk.200

5.13 On 10 March 1993, Mr Jimmy Young of BBC Radio 2, interviewed microbiologist Professor Richard Lacey, who commented that: The good news is that this farmer, I think, got it too soon. If BSE produces this disease in people it will take, perhaps, another 5 or 10 years. So I think this is a one-off coincidence and I don’t think this farmer got his disease, CJD, from BSE. But nevertheless the underlying worries remain and I think it’s reasonable that this issue should be discussed. 201

5.14 The Second Annual Report of the CJDSU, published in July 1993, concluded that: 202 This is most likely to have been a chance occurrence rather than indicating any causal link with BSE.

5.15 It further noted that: A farmer’s wife who was diagnosed in 1992 had worked on a small holding for over 20 years but there had not been a case of BSE in the herd (Wilesmith, Personal Communication).203

snip...

‘The second farmer’ – July 1993

5.16 In early July 1993, Dr Will informed DH of a ‘second’ case of CJD in a farmer with BSE in his herd. The diagnosis had been confirmed by brain biopsy.204

5.17 Dr Wight described the case in a minute sent on 12 July 1993 to the private secretaries to Baroness Cumberlege and Sir Kenneth Calman. The minute was copied to others in DH and to Mr Howard of MAFF. The 64-year-old dairy farmer from the West Country was thought to have had at least two BSE cases in his herd, which were diagnosed in 1992. He was also thought to have assisted in calving and to have drunk the milk from his herd. His clinical symptoms had begun in May 1993. She commented that the history did not suggest anything other than a sporadic case of CJD but that DH was taking expert advice on the case.205

5.18 On 19 July 1993, Mr Kevin Taylor (MAFF) minuted the private secretary to Mrs Gillian Shephard, the MAFF Minister, in a response to a request for more detailed briefing.206 He noted that neither Dr Will nor the CJDSU intended to publicise the case at that time unless it attracted media attention, as they intended to include the information in their Third Annual Report due in approximately one year, ie, July 1994.

5.19 The minute attached a briefing note for the Minister. This specifically mentioned the consideration of occupational exposure to BSE as discussed in the CJDSU’s Second Annual Report which concluded that: . . . current information does not suggest that occupation is linked to an increased risk of developing CJD and it includes occupations which might involve an increased exposure to the agent of BSE.207

5.20 On 20 July 1993, SEAC held a meeting to consider this ‘second case’.208 They decided that a connection between occupation and CJD was unlikely and no conclusions could be drawn from the available statistical information. A paper by Professor Smith was presented which concluded that ‘the observation of two cases in workers in dairy farms with BSE-infected herds is disquieting, but the evidence is insufficient at this stage to draw any definite conclusions’.209

5.21 On 12 August 1993, the Daily Mail and Today publicised the story of the ‘second case’ of CJD in a dairy farmer.210 Both named the farmer and reported a DH spokesman saying that the Government’s experts had considered the case and ‘agreed that there are no features that give cause for undue concern’. The spokesman had also commented that it was most unlikely that there was any direct link between BSE and CJD in the patient.

5.22 In September 1993, the case study of this ‘second farmer’ was published in The Lancet. This letter gave the farmer’s age as 54.211 204

YB93/7.12/1.1 205 YB93/7.12/1.1 206 YB93/7.19/1.1 207 IBD2 tab 6 p. 6 208 YB93/7.20/1.1 209 YB93/7.20/1.5 210 YB93/8.12/1.3–1.4 211 Davies, P.T., Jahfar, S., Ferguson, I.T. and Windl, O. (1993) Creutzfeldt-Jakob Disease in an Individual Occupationally Exposed to BSE, The Lancet, 342, 680

snip...

Her note had a separate heading for ‘Comparison with young onset cases in world literature’. Here she noted that Creutzfeldt’s first patient was 23 years old (reported in 1920), and that there were other cases of CJD in young people which predated the emergence of BSE. These were a 20-year-old female and a 16-year-old female in the US and a 19-year-old female in France.219

snip...

‘The third farmer’ – December 1994

5.33 A ‘third case’ associated with farming where cattle in the herd had contracted BSE concerned a farm worker from Cornwall who had died in early December 1994, aged 54. There had been two confirmed cases of BSE on the farm, in August 1991 and October 1992. Additionally, a cow sold off the farm in December 1987 had been diagnosed with BSE in September 1988.224

5.34 On 1 December 1994, the case was reported in the local newspaper, The Cornishman, while the patient was still in hospital.225

5.35 On 19 December 1994, Mr Charles Lister, DH, minuted the private secretary to Baroness Cumberlege with information about this possible ‘third case in farmers/ farm workers who have had BSE cases in their herds’.226 This minute enclosed the article from The Cornishman and was copied to DH officials and to Mr Eddy at 219

YB94/1.14/1.2 220 YB94/1.26/3.1 221 YB94/1.26/2.3; YB94/1.14/1.2 222 YB94/1.26/2.2 223

The report formed Annex 2 to the CJDSU’s Third Annual Report (IBD2 tab 8) 224

YB94/12.19/3.1 225 YB94/12.19/3.4 226 YB94/12.19/5.1

EMERGENCE OF VARIANT CJD 39 MAFF. He noted that diagnosis would not be confirmed until post mortem, but the Surveillance Unit thought it highly likely to be CJD.

5.36 On the same day, Mr Thomas Eddy, MAFF secretary to SEAC, passed the newspaper article and basic information about the case on to MAFF Ministers and officials.227

5.37 On 13 January 1995, SEAC held a special meeting to discuss the significance of this third case of CJD in a farmer in the first four years of surveillance.228 Dr Sheila Gore, an epidemiologist from the MRC Biostatistic Unit, was invited as an independent expert.

5.38 Detailed consideration was given to the case itself and the epidemiological implications. Dr Will commented that the post-mortem results were not yet available, but it was highly likely that the diagnosis of CJD would be confirmed. He stated that the man had no significant medical history and that he had worked as a farm labourer on the same dairy farm since 1955: The man was known to have assisted with calving but never with any operative procedure; he rarely drank unpasteurised milk and never from BSE-affected animals. It was not known if he had ever eaten cattle feed.229

5.39 As to the epidemiological significance of the case, the members recalled the advice given by Professor Smith after SEAC had considered the second case of CJD in a farmer: Professor Smith had advised that if four cases arose in the first 5 years of the surveillance scheme the possibility of an association which was not due to chance had to be given very serious consideration.230

5.40 Dr Gore commented that: If the adult incidence of sporadic CJD in the UK was taken as one case per million (the figure used by Professor Smith) and if the same incidence applied to workers on dairy farms with BSE-affected herds, then the probability of observing three or more definite CJD cases in such workers in England and Wales in 5 years was low: 4 in 1,000. The probability was higher if the calculation was made using the total number of dairy farm workers in England and Wales. However, this was considered to be less relevant as the only reported cases of CJD in dairy farm workers since 1990 had been in lifetime dairy workers all with BSE-affected herds.

snip...

5.63 On 29 September 1995, various newspapers reported the third case of CJD in a dairy farmer.255 Reference was made to a letter published in The Lancet (dated 30 September 1995) by Dr (now Professor) Smith (LSHTM).

5.64 The letter reported: The occurrence of CJD in another dairy farmer with a potential occupational exposure to BSE is clearly a matter of concern. Statistical analysis indicates that the probability of discovering three or more dairy farmers with CJD by chance since 1990 in England and Wales ranges from 0.09 to 0.0002, depending on the occupational denominator (individuals who work on farms to full-time workers on BSE-affected dairy farms).256

5.65 Statistics for CJD in European farmers were also reported in the 30 September 1995 edition of The Lancet.257 The paper concluded that ‘there is no differential increase in the risk of CJD to farmers in the UK through potential occupational contact with cases of BSE’. On the continent there was also a slightly higher proportion of cases of CJD arising in farmers.258 This indicated that in the UK, CJD in farmers had probably not arisen from transmission of BSE.259

The fourth farmer – September 1995

5.66 On 28 September 1995, Dr Wright minuted the private secretary to the CMO about a probable fourth case of CJD in a farmer. The 59-year-old beef farmer lived in North Wales and was alive when the case was reported to the CMO. The farm, which had a 70-strong suckler herd, had a confirmed case of BSE about four years previously in a 4½ to 5-year-old cow.260

5.67 The minute recorded the urgency of dealing with the issue as the case was in the public domain and BBC Wales were making a programme referring to the case.261 An urgent meeting of SEAC was called for the following week.

5.68 On 4 October 1995, SEAC held a special meeting to discuss this further suspected case of CJD in a cattle farmer.262 Professor Smith (LSHTM) and Dr Cousens (LSHTM) were in attendance to provide the Committee with expert epidemiological advice.263 5.69 Dr Will advised that although the Unit had initially clarified the case as probable CJD, he felt that it was more appropriate to look at it as a suspect case. Consideration was given by SEAC to European data that showed 12 cases of BSE in France, along with a progressive neurological disease in a farmer associated with 255

YB95/9.29/12.1; YB95/9.29/10.1; YB95/9.29/14.1 256

Smith, P.E., Zeidler, M., Ironside, J.W., Estibeiro, P. and Moss, T.H. (1995) Creutzfeldt-Jakob Disease in a Dairy Farmer, The Lancet, 346, 898 257 Delasnerie-Laupretre, N., Poser, S., Pocchiari, M., Wientjens, D.P. and Will, R. (1995) Creutzfeldt-Jakob Disease in Europe, The Lancet, 346, 898 258 T71 p. 115 259 T24 p. 95 260 YB95/9.28/3.1 261 YB95/9.28/3.1 262 YB95/10.4/1.1–1.8 263 YB95/10.04/1.1

EMERGENCE OF VARIANT CJD 45 one of those cases. (In the eventuality, this farmer was not diagnosed with CJD. At the beginning of January 2000, there had been no reported cases of CJD in farmers in France where BSE had been found in that farmer’s herd.)

5.70 Mr Wilesmith gave SEAC information about the farm associated with the possible UK fourth case of CJD under discussion. The farm had not been visited by MAFF. It had one case of BSE in a purchased animal which died in September 1991. From available information, the animals had not been fed on concentrates (although this had not been double-checked). It was thought, however, that the farm did have a big poultry battery unit, which may have meant that ruminant-derived feed was available on the farm.

5.71 Dr Cousens made a presentation of the epidemiology.264 He had calculated age specific mortality rates for sporadic CJD from 1990 to 1994 and applied these to data on farmers to calculate the expected number of sporadic CJD cases in farmers. The following conclusions were reached: i. there had been an alarming number of cases in farmers who had had contact with cattle with BSE. However, other occupational groups, expected to carry greater risk (eg, abattoir workers, veterinary surgeons), did not appear to be affected; ii. it was now difficult to explain the cases as a chance phenomenon. Yet the absolute risk still remained extremely low; iii. it was unclear whether the possible risk factor might be associated with cattle with BSE or the food given to them; and iv. as there was a problem with establishing a causal link, transmission studies would be extremely important.

5.72 At this meeting, Dr Wight invited members of SEAC to make a fairly clear statement on how they viewed the significance of a fourth case and to consider whether they were satisfied that nothing else needed to be done in terms of practical measures.265 In evidence to the Inquiry, Dr Wight said that trying to get a clear statement as to what would be a significant number of cases in farmers was bound to be difficult. She said, ‘I do not think that SEAC any more than anybody else had any idea how to make sense of this at this stage.’266 At the meeting, Dr Tyrrell’s response was that although numbers were higher than expected, they were still extremely small. It would be irrational to take specific measures at the moment. Members of SEAC agreed to draw up a statement which the Department of Health could issue in response to media inquiries.267 The text of the statement included the following:268 The Committee concluded that it was difficult to explain this simply as a chance phenomenon. There is a statistical excess in cattle farmers compared with the general population but the absolute risk, even for farmers, is extremely low at about 2 cases per million per year. There may be other explanations for such an association besides infection with BSE, and the Committee noted that there are no recorded cases in other occupational 264

YB95/10.4/1.2–1.4 265 YB95/10.4/4.5 266 T71 pp. 135–6 267 YB95/10.4/4.5 268 YB95/10.4/4.9


VARIANT CJD

46 groups such as veterinarians who might be expected to be similarly exposed. They also noted that the surveillance of CJD elsewhere in Europe has shown a similar incidence of CJD in farmers, including dairy farmers, in countries with no or very few cases of BSE. They therefore felt that it was important to undertake further epidemiological studies to detect any particular risk factors which might be involved, and reiterated their advice that the UK cases of CJD in cattle farmers and the strain of agent recovered from them should be studied in detail. The Committee have asked for further work to be done, but have not altered their advice to Government on the precautions necessary to protect either the public health, including farmers, and animal health.

5.73 Mr Eddy minuted the MAFF Minister and Parliamentary Secretaries advising them of the outcome of the SEAC meeting.269 He commented that SEAC had concluded that it would be worrying if the fourth case of CJD in a farmer from a BSE farm was confirmed. The chances of four CJD cases occurring randomly in farmers with BSE in their herds was . . . [since 1990] around 3/10,000. The Committee therefore concluded that it was difficult to explain the incidence as a chance phenomenon. This is a change to the Committee’s position; it had said that the most likely explanation of the three previous cases of CJD in dairy farm workers was that they were chance phenomena.270

5.74 Mr Eddy also stated that the SEAC did not recommend changes to any of the measures currently in place to protect human and animal health, including those of farmers and others handling cattle and BSE suspects.

5.75 On the same day, Mr Eddy prepared a second minute which was sent to Dr Matthews and Mr Keith Meldrum (CVO) amongst others about discussions during the SEAC meeting.271 Mr Eddy included a list of four ways in which the farmers might have been exposed to BSE that might have then led to their infection with CJD: i. cattle were excreting the agent in some form – no evidence for this; ii. meat and bone meal (MBM) in cattle feed – if so this would affect pig and poultry farmers equally (these feeds also contained MBM); iii. normal food – unclear why this discriminated in favour of farmers, although farmers could have been exposed to foods that other people might not have been routinely exposed to, such as unpasteurised milk; and iv. contact with animals – possibly animals killed on the farm.

snip..

5.93 Dr Will updated SEAC on CJD surveillance results at their 23rd meeting on 5 January 1996.292 He ‘reaffirmed that the incidence of CJD in dairy farmers in Europe showed an excess over the incidence for the population as a whole’. He confirmed that a 52-year-old abattoir worker from York was suspected of having CJD. The patient had worked mainly as a stockman in a mixed abattoir for 18 months in the late 1980s, and had occasionally pithed animals but had much less exposure than other abattoir workers. Dr Will believed that the patient was no more than a suspect at that stage.

5.94 The minutes of the meeting record that Professor Smith commented on this case: He [Professor Smith] felt that it was not possible to come to any conclusions on the basis of this case alone even if CJD is confirmed. Nevertheless, taking into consideration the affected farmers as well, and even though the abattoir worker was in an apparently relatively low risk category, the ‘box’ of ‘at 289 S61D Will para. 4 290 T138 p. 34 291 S61D Will para. 18 292

YB96/1.5/1.6–1.8; S61D Will paras 19–22

EMERGENCE OF VARIANT CJD 51 risk’ occupations was getting full compared to expectation on pure chance and could not be dismissed.293

snip...

Update on cases of CJD in farmers

5.152 During the period 1986–96, much attention and publicity was focussed on four cases of CJD in farmers (see above). Although those four cases were regarded as likely to be more than might be expected for the known population frequency of the disease, analysis of CJD in Europe showed the incidence of disease in farmers was similar to that in the UK.373 In addition, the clinical and pathological features of these cases were no different to those found in classical sporadic CJD.

5.153 It is understood that since 20 March 1996, at least two further cases of sporadic CJD in a relevant occupational group have been reported to the CJDSU, one in a farmer and another in an abattoir worker.374 Recent transmission studies in mice indicate that the causal agent in these cases has transmission characteristics (incubation period and neuropathology) which are distinct from both vCJD and BSE, and that the protein deposited in the brain in all of these cases has a glycosylation pattern distinct from the type 4 pattern observed in vCJD and BSE.

snip...

Is occupation a risk factor in vCJD?

5.192 One of the original proposals in the CJD surveillance project was to monitor occupational groups exposed to BSE-affected cattle and their products. Such groups include farmers, veterinarians, slaughtermen and butchers. This part of the project was given a low priority by the Tyrrell Committee and was not implemented. It was felt that rather than set up longitudinal study of a fixed number of individuals in each group, together with matched controls, it would be adequate to take an occupational history of each CJD case at the time of referral.

5.193 From 1990 to 1996, the CJDSU had referred to it four farmers affected with CJD who were known to have had cases of BSE on their farms. Assuming a total of 155,000 dairy farmers in the UK,384 the number of observed CJD cases is significantly higher than expected from population estimates. Counting only those farmers with affected cattle, the probability of observing four or more confirmed cases of CJD is estimated at less than one in 10,000.385 In addition, two farmers’ wives were known to have CJD from farms in which clinical BSE had not been reported (although preclinical cases of BSE on these farms might have been expected). 384 Gore, S. (1995) More than Happenstance: Creutzfeldt-Jakob Disease in Farmers and Young Adults, British Medical Journal, 311, 1416–8 385 Ibid.

EMERGENCE OF VARIANT CJD 79

5.194 The affected farmers were aged between 54 and 64 and had signs and symptoms typical of sporadic CJD. Two had EEG changes typical of the sporadic disease and all four had type 2 glycosylation patterns. Three farmers were homozygous for methionine at codon 129 and the fourth was a valine homozygote. None conformed to the phenotype characteristic of vCJD. The findings remained unexplained, although a European collaborative study showed a similar increased incidence in deaths due to CJD in farmers in several member states. It was noted that unexpected numbers of affected individuals occurred in other occupational groups, such as the clergy, but numbers in each occupation remained small.

5.195 Among occupational groups exposed to BSE, farmers remain unusual in having such an excess over the incidence of CJD for the population as a whole. No cases of CJD have been reported amount veterinarians exposed to BSE. Four people in the meat industry (butchers, abattoirs, rendering plants, etc) have been reported to have vCJD.386 The present evidence has been accepted by some as reassuring in that such occupations may not pose as serious a risk as might have been expected.



http://collections.europarchive.org/tna/20080102111737/http://www.bseinquiry.gov.uk/pdf/volume8/chapter5.pdf



This was not simply another farmer but the third farmer......


http://collections.europarchive.org/tna/20080102141416/http://www.bseinquiry.gov.uk/files/yb/1995/06/21002001.pdf



suspect case of CJD in a farmer who has had a case of BSE in his beef suckler herd.


http://web.archive.org/web/20030331213802/http://www.bseinquiry.gov.uk/files/yb/1995/10/23006001.pdf




cover-up of 4th farm worker ???


http://web.archive.org/web/20030516083454/http://www.bseinquiry.gov.uk/files/yb/1995/10/23006001.pdf




http://web.archive.org/web/20030330175323/http://www.bseinquiry.gov.uk/files/yb/1995/10/20006001.pdf




CONFIRMATION OF CJD IN FOURTH FARMER


http://collections.europarchive.org/tna/20080102203608/http://www.bseinquiry.gov.uk/files/yb/1995/11/03008001.pdf



now story changes from;


SEAC concluded that, if the fourth case were confirmed, it would be worrying, especially as all four farmers with CJD would have had BSE cases on their farms.


to;


This is not unexpected...

was another farmer expected?


http://web.archive.org/web/20030728074919/http://www.bseinquiry.gov.uk/files/yb/1995/11/13010001.pdf




4th farmer, and 1st teenager


http://collections.europarchive.org/tna/20080102235004/http://www.bseinquiry.gov.uk/files/yb/1996/02/27003001.pdf




2. snip...

Over a 5 year period, which is the time period on which the advice from Professor Smith and Dr. Gore was based, and assuming a population of 120,000 dairy farm workers, and an annual incidence of 1 per million cases of CJD in the general population, a DAIRY FARM WORKER IS 5 TIMES MORE LIKELY THAN an individual in the general population to develop CJD. Using the actual current annual incidence of CJD in the UK of 0.7 per million, this figure becomes 7.5 TIMES.

3. You will recall that the advice provided by Professor Smith in 1993 and by Dr. Gore this month used the sub-population of dairy farm workers who had had a case of BSE on their farms - 63,000, which is approximately half the number of dairy farm workers - as a denominator. If the above sums are repeated using this denominator population, taking an annual incidence in the general population of 1 per million the observed rate in this sub-population is 10 TIMES, and taking an annual incidence of 0.7 per million, IT IS 15 TIMES (THE ''WORST CASE'' SCENARIO) than that in the general population...


http://web.archive.org/web/20030516181226/http://www.bseinquiry.gov.uk/files/yb/1995/01/31004001.pdf



CJD FARMERS WIFE 1989



http://collections.europarchive.org/tna/20080103005651/http://www.bseinquiry.gov.uk/files/yb/1989/10/13007001.pdf




http://collections.europarchive.org/tna/20080102155149/http://www.bseinquiry.gov.uk/files/yb/1989/10/13003001.pdf




20 year old died from sCJD in USA in 1980 and a 16 year old in 1981. A 19 year old died from sCJD in France in 1985. There is no evidence of an iatrogenic cause for those cases....


http://web.archive.org/web/20030330212925/http://www.bseinquiry.gov.uk/files/yb/1995/10/04004001.pdf




USA 2008

Prion surveillance in cattle has been reduced by 90% (from about 470,000 to 40,000 in the U.S. in 2007 out of about 35 million cattle slaughtered). Termination of human prion surveillance would therefore remove the second line of surveillance, thereby eliminating prion surveillance in the U.S. entirely. This development would be extremely worrisome in view of recent reports that precautions to limit the spread of the prion infectious agent may not have been followed in some slaughter houses in the U.S. Cattle affected with bovine spongiform encephalopathy (BSE) continue to be discovered in Canada, which has more rigorous BSE surveillance than the U.S. At the same time, Canada imposes few limitations in the trade of potentially prion-infectious cattle with the U.S.

snip...

Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.

please see full text with additional comments and links @ ;


http://prionunitusaupdate2008.blogspot.com/



SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM 1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype of 'UNKNOWN' strain growing. ...


http://www.cjdsurveillance.com/resources-casereport.html



There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.

He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.


http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm


http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf



PLEASE NOTE IN USA CJD UPDATE AS AT JUNE 2007, please note steady increase in ''TYPE UNKNOWN''. ...TSS

1 Acquired in the United Kingdom; 2 Acquired in Saudi Arabia; 3 Includes 17 inconclusive and 9 pending (1 from 2006, 8 from 2007); 4 Includes 17 non-vCJD type unknown (2 from 1996, 2 from 1997, 1 from 2001, 1 from 2003, 4 from 2004, 3 from 2005, 4 from 2006) and 36 type pending (2 from 2005, 8 from 2006,

*** 26 from 2007)


http://www.cjdsurveillance.com/pdf/case-table.pdf



Diagnosis and Reporting of Creutzfeldt-Jakob Disease

Singeltary, Sr et al. JAMA.2001; 285: 733-734.


http://jama.ama-assn.org/http://www.neurology.org/cgi/eletters/60/2/176#535



JOURNAL OF NEUROLOGY

MARCH 26, 2003

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob

disease in the United States

Email Terry S. Singeltary:

flounder@wt.net

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to

comment on the CDC's attempts to monitor the occurrence of emerging

forms of CJD. Asante, Collinge et al [1] have reported that BSE

transmission to the 129-methionine genotype can lead to an alternate

phenotype that is indistinguishable from type 2 PrPSc, the commonest

sporadic CJD. However, CJD and all human TSEs are not reportable

nationally. CJD and all human TSEs must be made reportable in every

state and internationally. I hope that the CDC does not continue to

expect us to still believe that the 85%+ of all CJD cases which are

sporadic are all spontaneous, without route/source. We have many TSEs in

the USA in both animal and man. CWD in deer/elk is spreading rapidly and

CWD does transmit to mink, ferret, cattle, and squirrel monkey by

intracerebral inoculation. With the known incubation periods in other

TSEs, oral transmission studies of CWD may take much longer. Every

victim/family of CJD/TSEs should be asked about route and source of this

agent. To prolong this will only spread the agent and needlessly expose

others. In light of the findings of Asante and Collinge et al, there

should be drastic measures to safeguard the medical and surgical arena

from sporadic CJDs and all human TSEs. I only ponder how many sporadic

CJDs in the USA are type 2 PrPSc?


http://www.neurology.org/cgi/eletters/60/2/176#535



THE PATHOLOGICAL PROTEIN

Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9

June 2003

BY Philip Yam

CHAPTER 14 LAYING ODDS

Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.


http://www.thepathologicalprotein.com/



USA 2007-2008 sporadic CJD statistics revised to 1 in 9,000 in ages 55 and older !

The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.


http://www.cjdfoundation.org/fact.html



Sent: Monday May 28, 2007

Subject: THE BIG LIE SPORADIC CJD AND MAD COW DISEASEs i.e. TSE

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0705&L=sanet-mg&T=0&P=25276


Sunday, March 16, 2008

MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L, and or Italian L-BASE

http://bse-atypical.blogspot.com/2008/03/mad-cow-disease-terminology-uk-c-bse.html


Subject: HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory Date: January 29, 2006 at 9:03 am PST



HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory

TSEs have been rampant in the USA for decades in many species, and they all have been rendered and fed back to animals for human/animal consumption. propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2007. With all the science to date refuting it, to continue to validate this myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, surgical, blood, medical, cosmetics etc. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.

This would further have to be broken down to strain of species and then the route of transmission would further have to be broken down. Accumulation and Transmission are key to the threshold from sub-clinical to clinical disease, and key to all this, is to stop the amplification and transmission of this agent, the spreading of, no matter what strain. In my opinion, to continue with this myth that the U.K. strain of BSE (one strain TSE in cows), and the nv/v CJD (one strain TSE humans) and that all the rest of human TSE are just one single strain i.e. sporadic CJD (when to date there are 6 different phenotypes of sCJD, and growing per Gambetti et al), and that no other animal TSE transmits to humans, to continue with this masquerade will only continue to spread, expose, and kill, who knows how many more in the years and decades to come. ONE was enough for me, My Mom, hvCJD i.e. Heidenhain Variant CJD, DOD 12/14/97 confirmed, which is nothing more than another mans name added to CJD, like CJD itself, Jakob and Creutzfeldt, or Gerstmann-Straussler-Scheinker syndrome, just another CJD or human TSE, named after another human.

WE are only kidding ourselves with the current diagnostic criteria for human and animal TSE, especially differentiating between the nvCJD vs the sporadic CJD strains and then the GSS strains and also the FFI fatal familial insomnia strains or the ones that mimics one or the other of those TSE? Tissue infectivity and strain typing of the many variants of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...



Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518 flounder9@verizon.net

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