Prior to the Better Blood Transfusion initiative in 1998, some work was introduced in the early to mid 1990s probably in response to a high level of transfusion errors involving blood transfusions of incompatible blood groups causing morbidity and mortality.8
There was also a changing legislative landscape following the establishment of the Consumer Protection Act 1987 and the introduction of the European Directive on blood, which led senior individuals working in the transfusion services to focus on the issue of blood transfusion.9
The emergence of variant Creutzfeldt-Jakob disease (“vCJD”) reinforced the need for vigilance.10
5.9 vCJD
This chapter describes vCJD and assesses the government response to the risks of its transmission by blood or blood products. It explores the contrast between the response to HIV in the 1980s and the response to vCJD in the 1990s/2000s.
Key dates
September 1985 first confirmed BSE case in cows.
March 1996 vCJD formally identified in humans; probable link between BSE and vCJD announced in Parliament.
August 1996 blood transfusion services required to ask about family history of CJD.
October 1997 CMO’s statement reporting that there are patients with vCJD who had been blood donors; MSBT decides that recipients of possibly infected transfusions do not need to be told; NBA recalls batches of Factor 8 and albumin after a donor develops vCJD.
November 1997 the Government decides on leucodepletion; UKHCDO recommends recombinant Factor 8 for all with Haemophilia A.
July 1998 leucodepletion programme is implemented.
November 1998 Government decides that blood products will no longer be made from UK plasma.
August 2000 CMOs set up the CJD Incidents Panel.
October 2000 BSE Inquiry report published and compensation is announced.
December 2002 the Government purchases US plasma collector.
December 2003 death of a patient thought to have been infected with vCJD through transfusion is announced. People who received transfusions from donors who subsequently developed vCJD are notified.
March 2004 exclusion of donors who had received blood transfusions after 1 January 1980.
July 2004 second case of transfusion-related vCJD is reported.
September 2004 haemophilia centres notify people who received factor products between 1980 and 2001.
People
Professor John Collinge head of the Medical Research Council Prion Unit, University College London Institute of Neurology
Dr Patricia Hewitt Standing Advisory Committee on Transfusion Transmitted Infections
Professor James Ironside professor, clinical neuropathology
Dr Robert Will professor, clinical neurology
Abbreviations
CJDIP CJD Incidents Panel
MSBT Microbiological Safety of Blood and Tissue
NCJDRSU National Creutzfeldt-Jakob Disease Research & Surveillance Unit
TMER Transfusion Medicine Epidemiology Review
Introduction
The central nervous system – brain and spinal cord – may degenerate over time. Neurodegenerative disorders may display themselves as losses of cognitive function, or motor capabilities, or both.
A variety of different causes may be responsible for this. Amongst them is a form of disease first identified by two neurologists – Hans Creutzfeldt and Alfons Maria Jakob – which thus became known as Creutzfeldt-Jakob Disease (“CJD”). This is a prion disease. Prion diseases form a group of degenerative brain diseases. They are always progressive and invariably fatal.
Prions are proteins. Many prion proteins sit on the surface of brain cells. This is normal. Such proteins are three dimensional in shape, and this 3D shape is essential for their function. Prion disease occurs when the protein does not fold into its normal shape but misfolds. It can do so in a way such that many individual prion protein molecules stick together, forming long chains or assemblies of protein. These form fibres of protein (technically referred to as amyloid). As the amyloid fibres grow, by recruiting more of the normal protein into the disease-associated form, they also fragment: and as they fragment they effectively produce more amyloid. Professor John Collinge described it as if there were “seeds in the brain ... which in [sic] then in turn grow ... fragment and form more seeds.”1524 It is thus a selfpropagating process.
And prion protein is not a virus: it has no DNA or RNA of its own. It is purely protein. Chemically, therefore, a misfolded protein is indistinguishable from a correctly folded protein. This creates particular challenges in distinguishing a diseased prion from a healthy prion protein. It also means that the immune system does not respond to the threat posed by the diseased prion. A normal defensive immune response begins with the recognition of a virus, other antigen, or protein as being “foreign”. 1525 Thus the body of a person who is infected with abnormal prion does nothing of itself which slows the inevitable progress of replication of the misfolded proteins, the development of amyloid, and the onset of symptoms.
These prions, which cause disease, arise in one of three forms (though the third has two subsets). The first, and commonest, is as a sporadic disease (sporadic CJD: “sCJD”). This occurs at random and it is similar in this respect to contracting cancer but is much more rare. About 1 in 5,000 people will develop this during their lifetime,1526 compared to 1 in 2 people developing some form of cancer.
Second, there are inherited forms: a genetic mutation of the prion protein gene, inherited from a parent, results in spontaneous mutation at some stage during adult life. The disease
1524 Professor John Collinge Transcript 13 May 2022 pp21-23 INQY1000206 1525 See chapter Blood and Transfusion for a fuller explanation. 1526 Professor John Collinge Transcript 13 May 2022 p23 INQY1000206 Infected Blood Inquiry | The Report vCJD 299
then progresses in the same way as sCJD. Inherited disease accounts for about 15% of UK patients.1527
Third are the forms of disease that are acquired as a result of some exposure. There are two subsets, depending on the routes of exposure. The first subset consists of medical accidents. Examples include the use of contaminated neurosurgical instruments for certain tissue grafts, or the use of particular hormones to treat growth deficiency (some batches of hormones derived from pituitary glands were contaminated with CJD prions because they were extracted from human tissues which had been pooled together). The second subset of acquired CJD is variant CJD (“vCJD”). This is usually due to dietary exposure to bovine spongiform encephalopathy (“BSE”) prions.1528 However, there have to date been five known cases where vCJD has spread from one infected person to another through the transfusion of blood or blood products.1529
The protein strains which cause classical CJD (the sporadic or inherited types) propagate almost exclusively in the brain and spinal cord. In vCJD, however, the development of diseased prions involves the lymphoreticular system. Thus it is thought that lymphoreticular tissues – such as the tonsils, and patches of lymphoid tissue in the gut – are likely to be the first places to be infected. A likely model is that prions then spread along the nerves in the lymphoid tissue, until they reach the spinal cord and then go up into the cranial nerves. Eventually, they get to the brain itself and begin to cause the neurological disease recognised clinically as vCJD. The whole lymphoreticular system is involved: spleen, lymph nodes around the body, tonsils, and gut-associated lymphoid tissue.1530
Developing knowledge of vCJD and reaction to it BSE, or “mad cow disease” as it became known, was first confirmed in September 1985 at a farm in Sussex. Further cases followed, rapidly rising in number. There was a risk that it might jump the species barrier and infect humans through their eating of beef or beef derivatives.1531 Such was the risk that by 1989 high-risk food stuffs such as offal were banned for human consumption, and many British consumers stopped buying beef because of their fears of what it might contain.
By April 1990, as the epidemic spread rapidly, a Spongiform Encephalopathy Advisory Committee (“SEAC”) was set up to advise the Department of Agriculture, Fisheries and Food and the Department of Health, and the National Creutzfeldt-Jakob Disease Research and Surveillance Unit (“NCJDSU”, later “NCJDRSU”) was set up to monitor cases of CJD in
1527 Professor John Collinge Transcript 13 May 2022 p24 INQY1000206
1528 Professor John Collinge Transcript 13 May 2022 p24-25 INQY1000206
1529 Expert Report to the Infected Blood Inquiry: Statistics September 2022 pp95-98 EXPG0000049
1530 Professor John Collinge Transcript 13 May 2022 pp27-28 INQY1000206
1531 Professor Collinge referred to Kuru, an acquired prion disease historically seen in a small area of the Highlands Region of Papua New Guinea. At mortuary feasts it was the practice in that area for the deceased to be consumed and this led to an epidemic of prion disease. Professor John Collinge Transcript 13 May 2022 pp25-26 INQY1000206
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the UK in order to identify any changes in the pattern of disease that might be attributable to the emergence of BSE.
The epidemic amongst cattle spread rapidly, reaching its height in 1992. A yet more worrying development was reported by Dr Robert Will and Dr James Ironside1532 on 8 March 1996 when the same disease as BSE was first formally identified as vCJD in humans. It had crossed from cows to people, and was labelled new variant CJD (“nvCJD”) because of its similarities to classical CJD, before being known simply as vCJD.1533
Twelve days after Drs Will and Ironside had presented their work (to SEAC), the Secretary of State for Health, Stephen Dorrell, announced in Parliament that there was a probable link between BSE in cows and the new variant (now known as vCJD) in humans.1534 This ministerial announcement was followed by a publication in The Lancet of the findings of Dr Will and Dr Ironside. The features were that sufferers tended to have a young age at onset, a long duration of illness, an absence of mutations in the prion protein gene (the PrP gene), and developed characteristic plaques involving extensive prion deposition throughout the brain which had not previously been seen in CJD.1535 This made for a clear distinction between classical CJD and vCJD.
vCJD seemed similar to BSE in cattle. In a similar disease (“scrapie”) in sheep to BSE in cattle the prions could be detected in the sheep’s tonsils. Could it, then, be found in human tonsils? In late 1996 the National Prion Clinic which had been set up under Dr Collinge checked to see. They discovered that it could quite easily be found. This realisation had implications: since prions could be detected quite readily in tonsils, lymph nodes and the spleen from patients who had died from vCJD, they did so in places where cells of the immune system, such as white blood cells, circulate freely. White blood cells, as the name suggests, also circulate in the bloodstream. This implied that the abnormal prions of vCJD might not only be transmissible by eating BSE-infected meat, but also through the blood circulation. It also implied that there was a general risk that surgical instruments might be contaminated, not only where surgery was being done on the brain, but also more generally in other body tissues where the abnormal prions might be present in lesser numbers, since prions stick “rather avidly to surgical stainless steel.”1536
1532 Subsequently both professors.
1533 Minutes of Spongiform Encephalopathy Advisory Committee meeting 8 March 1996 pp5-8 DHSC0004445_043. The first death from vCJD was found to have occurred on 21 May 1995, when 19-year-old Stephen Churchill died – although the UK Government continued to emphasise the safety of British beef and, in October 1995, concluded that there was “currently no scientific evidence” to link BSE and vCJD. The Chief Medical Officer (“CMO”) quoted in a Department of Health press release: Department of Health Press Release CJD Deaths in Line with Levels Worldwide 5 October 1995 p2 CABO0000292_013
1534 Department of Health Press Release CJD and Public Health: Stephen Dorrell Statement 20 March 1996 CABO0000383_036
1535 Will et al A new variant of Creutzfeldt-Jakob disease in the UK The Lancet 6 April 1996 p2 HSOC0010099. Dr Will and Dr Ironside both worked for the NCJDSU.
1536 The words are those of Professor Collinge. Professor John Collinge Transcript 13 May 2022 pp30-32 INQY1000206
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The worrying features of The Lancet publication were such that only three days later an ad hoc meeting was organised at the Royal College of Physicians of Edinburgh to discuss its implications for the blood transfusion services in the UK. Dr Ironside made a presentation. There was little information as to whether BSE/vCJD could be transmitted by the transfusion of blood and blood products. However, this could not be excluded. Nor could it be assumed that it would behave in a manner similar to that of classical CJD. The meeting noted a need to consider what plasma fractionators should do, and that because the level of BSE in the UK was significantly higher than in other countries “it may thus be appropriate to be proactive in this area.”1537
Proactive protective responses were also agreed. The transfusion services were to take urgent action to start direct questioning of blood donors. It was also thought essential to identify whether patients who had been identified as having had CJD had ever donated blood. It was agreed recommendations should be developed to consider what action should be taken where a new case of CJD was identified in a current or former donor. The feasibility of a form of “lookback” to assist in identifying the transmissibility of vCJD by blood needed to be assessed since it was recognised that it was necessary to investigate systematically whether reported cases of vCJD had themselves received transfusions of blood or blood productions prior to the symptoms of the disease. Possible quarantining of donations of frozen blood components was considered.1538
Thus, from 1 August 1996 blood transfusion services throughout the UK were required to ask all blood donors whether they had a family history of CJD. Where a close family member with CJD was a direct bloodline relative (eg parent, brother, child) the potential donor was to be advised not to give blood.1539
By June 1997, SEAC reported that the evidence favoured a conclusion that there was indeed a link between BSE and vCJD, but this was not (yet) sufficient to be regarded as scientific proof of a causative link.1540
1537 It was organised by Dr Angela Robinson (National Blood Authority) and Professor John Cash (Scottish National Blood Transfusion Service) and 16 of the major figures in blood transfusion in the UK were present. Notes of Royal College of Physicians of Edinburgh meeting 9 April 1996 p3 NHBT0115407
1538 Notes of Royal College of Physicians of Edinburgh meeting 9 April 1996 pp1-3 NHBT0115407
1539 National Blood Service Creutzfeldt-Jakob Disease: Information for Blood Donors August 1996 JPAC0000177_008. From 1 April 1998 donors who had had brain surgery before August 1992 were permanently deferred. Corneal transplants, an operation for a tumour or cyst on the spine before August 1992, or an injection of human pituitary extract such as growth hormones before 1987 were also grounds for deferral. (Growth hormones derived from pituitary glands were a source of acquired CJD). UKBTS/NIBSC Medical Assessment of Donors April 1998 p17, pp22-23, p33, p93 JPAC0000160_002
1540 Letter from the Department of Health to the Prime Minister 30 March 1997 CABO0000009_002, Department of Health Research Into Link Between New Variant CJD and BSE: Publication of Latest Scientific Advice 1 July 1997 DHSC0006880_072, Minutes of Spongiform Encephalopathy Advisory Committee meeting 23 May 1997 p13 NCRU0000248_058. The statement was prompted by a letter from the parents of Stephen Churchill, the first person to die of vCJD, asking for SEAC’s considered view on whether there was a causal link. Minutes of Spongiform Encephalopathy Advisory Committee meeting 15 April 1997 pp11-12 DHSC0046994_003
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By 16 September 1997, however, SEAC considered the results of two studies in Nature (in advance of their publication at the start of October 1997) and became convinced that the evidence of the link between BSE and vCJD had become “compelling”. 1541 Although the primary candidate for transmitting the disease was eating infected beef, it was theoretically possible that it might be transmitted between humans through blood. SEAC thus recommended in their next meeting that the National Blood Authority should take steps towards the leucodepletion of blood (the removal of as many white blood cells as possible from a donation by the available technology) as far as practicable pending the results of an assessment of the risk of transmitting vCJD by this means.1542
The Chief Medical Officer (“CMO”) issued a statement on 6 October 1997, which reported that three patients who had suffered from vCJD had been blood donors, and a fourth was suspected of having been one too. The statement noted that “there is no epidemiological evidence to suggest that classic CJD has been transmitted between humans through blood transfusions or the use of blood products. However we do not know whether the same will apply to nvCJD.”1543 Since the Blood Products Laboratory (“BPL”) manufactured blood products from donated plasma it was notified of these three donors. It was ascertained that between them they had provided seven donations of plasma, six of which had been included in fractionation pools for the production of blood products.1544
Recipients of the products made from pools containing this plasma had not been notified.1545
A debate began about whether recipients should be told of the risks they now faced. In successive days a working party of the Committee on Proprietary Medicinal Products, a European Union body to which the UK had recommended a policy of recall if a plasma pool was found to have been made in part from a donation from someone who had subsequently developed vCJD, agreed that there should be such a recall and SEAC agreed that the surveillance unit would set up a procedure to report cases.1546
This in turn was swiftly followed by a meeting of the Advisory Committee on the Microbiological Safety of Blood and Tissue (“MSBT”). This recommended that when the blood services were informed of suspected cases of vCJD confirmed as such by the NCJDSU the recipients of any donation from those people would have to be traced – a process which might seem similar to a “lookback”, save that it was forward-looking: it was not seeking to ask how
1541 Minutes of Spongiform Encephalopathy Advisory Committee meeting 16 September 1997 pp6-8 WITN3430070, Bruce et al Transmissions to mice indicate that ‘new variant’ CJD is caused by the BSE agent Nature 2 October 1997 DHSC0004125_011, Hill et al The same prion strain causes vCJD and BSE Nature 2 October 1997 DHNI0000041_123. The word “compelling” was used in SEAC’s description in its first annual report. First Annual Report of the Spongiform Encephalopathy Advisory Committee 1997-1998 p9 MHRA0020531
1542 Minutes of Spongiform Encephalopathy Advisory Group meeting 24 October 1997 pp7-12 NCRU0000174_001
1543 Department of Health CMO Statement on CJD 6 October 1997 p2 DHSC0041442_171
1544 vCJD, Blood Components and Plasma Products 30 January 2001 p13 NHBT0001722
1545 vCJD, Blood Components and Plasma Products 30 January 2001 p14 NHBT0001722
1546 Memo from Dr Jefferys to Dr Jones, Mr Kenny and Miss Casemore 23 October 1997 p1 DHSC0041442_050, Minutes of Spongiform Encephalopathy Advisory Group meeting 24 October 1997 pp11-12 NCRU0000174_001
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the suspected case came to be infected, by tracing back, but who they themselves had infected. It was agreed that it was important still to continue the (retrospectively focused) vCJD lookback.1547
The minutes of this MSBT meeting also record a debate about whether to inform recipients that they had been given a transfusion which increased the risks that they would suffer vCJD. The Committee on Proprietary Medicinal Products had advised that those who received blood products made from pooled plasma should be told. Should recipients of single-unit donations of blood also be told? A Regional Ethics Committee1548 considering whether to give ethical clearance for a proposed epidemiological review to examine a potential link between transfusion and infection with vCJD had previously advised against telling recipients that they had received a donation which might be infected. It was reported to the MSBT at this meeting that this Committee had been asked to review that decision in the light of the developments described above, but was understood to have upheld the existing line. The chairman of the MSBT “recognised an apparent inconsistency in following the CPMP [Committee on Proprietary Medicinal Products] advice on blood products but not telling patients, once traced, when labile components [ie blood] had been given to them.” The decisive reason for keeping quiet, at least so far as the chair was concerned, was “whether we could do anything about a situation”. Blood already transfused could not be recalled, but products in stock could be, on a precautionary basis.1549 Disagreement about whether people should be told that they were at risk continued for the next seven years, and is described more fully later in this chapter.
As to recall, there was no such debate. It was swiftly put in place. On 30 October 1997 the National Blood Authority issued a press statement to the effect that it had recalled albumin and Factor 8 from 26 sites within England on the basis that a blood donor who had developed vCJD had contributed to that batch.1550
In early November 1997, the Secretary of State for Health, Frank Dobson invited Dr Collinge to meet him in person to advise him about the risks of transmission of vCJD by blood and blood products. The meeting was attended by a number of senior civil servants, the CMO, and the chair of SEAC. Dr Collinge set out how he considered that on the basis of the evidence available at the time much of the infectivity in vCJD was likely to be whitecell-associated. He described the (still) theoretical risk that vCJD might be transmitted
1547 Minutes of Advisory Committee on the Microbiological Safety of Blood and Tissues for Transplantation meeting 27 October 1997 pp3-8 SBTS0000522
1548 Lothian Research Ethics Committee – concerned with Scotland, in particular the Edinburgh area, and the body to which the NCJDSU, which was based in Edinburgh, had to apply to obtain ethical clearance for proposed research. Approval was given by the Committee for a retrospective study to examine a possible link between CJD and blood transfusion (known as the “TMER study” – Transfusion Medicine Epidemiology Review), on the condition that anyone who was traced as a result of the TMER study would not be told of their exposure. Chronology on Precautions on Blood Protection: Transfusion Medicine Epidemiology Review (TMER) 10 January 2005 DHSC0038559_029
1549 Minutes of Advisory Committee on the Microbiological Safety of Blood and Tissues for Transplantation meeting 27 October 1997 p6, p8 SBTS0000522
1550 National Blood Authority Press Release National Blood Authority Issues Recall Notice on Plasma Products 30 October 1997 NHBT0005408_004
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through blood and blood products. Technology was available to filter out white cells from blood before it had been transfused (a number of countries already did this, at least to an extent, for reasons unrelated to vCJD, because it may also reduce the transmission of some viruses and unwanted immunological reactions as well as increasing the shelf-life of blood). After questioning Dr Collinge the Secretary of State decided that leucodepletion should take place before either transfusion, or the manufacture of blood products from the blood donated. The Secretary of State took the issue immediately to the Prime Minister (Tony Blair) who agreed straight off, and a decision was taken that day to set the wheels in motion for this.1551 In a press statement the Government said it had accepted the advice of SEAC to extend the use of leucodepleted blood and blood products as far as practicable, had commissioned an assessment of the risks of human-to-human transmission of nvCJD through blood and blood products, and instructed the National Blood Authority to start work towards the “possible extension of leucodepletion of blood in order that they are prepared in the event that the risk assessment indicates that this would be a sensible precautionary measure.”1552 As it happens, no case of transmission from leucodepleted blood or plasma has ever been recorded.
A further risk reduction measure was advocated by the United Kingdom Haemophilia Centre Doctors’ Organisation (“UKHCDO”), also in November 1997. It issued a press release recommending the use of recombinant Factor 8 for all. Where this was not available, it suggested that the risk of transmission of vCJD would be reduced by using concentrates prepared from using donor plasma collected in countries other than the UK where there were no recorded cases of either vCJD or BSE (eg the US).1553 The following year, the Department of Health made recombinant available to all children under 16 and new patients in England, matching what was already provided in Scotland, Wales and Northern Ireland, though Scotland and Wales went further.1554 In Scotland, the Scottish Office made sufficient funding available not only for children but also some adults.1555 In Wales, recombinant was made available for all patients from 1997.1556 Adults were increasingly funded through 1998 in Scotland and Northern Ireland.1557 However, it was not until the 2004/2005 financial year that all patients with Haemophilia A in the UK had access to recombinant Factor 8.1558
1551 Professor John Collinge Transcript 13 May 2022 pp16-17, pp62-63, pp65-66 INQY1000206
1552 Department of Health Press Release SEAC Advice on Safety of Blood and Blood Products Accepted 6 November 1997 p1 BART0002084_002
1553 Letter from Dr Christopher Ludlam to colleagues 25 November 1997 p3 HSOC0015139
1554 Department of Health Press Release Further Precautionary Measures on Blood Products Announced 26 February 1998 HSOC0015101_002, Haemophilia Society Press Release Haemophilia Society Wins Battle for Recombinant Factor VIII 26 February 1998 p2 HSOC0016980
1555 Memo from I Snedden of NHS Management Executive to PS/Minister of State 3 June 1996 SCGV0000116_166, House of Commons written answer on use of synthetic factor VIII for the treatment of haemophilia 10 July 1996 SCGV0000116_149
1556 Written Statement of Dr Saad Al-Ismail para 46 WITN3761005
1557 Haemophilia Society Press Release Haemophilia Society Wins Battle for Recombinant Factor VIII 26 February 1998 p1 HSOC0016980
1558 Written Statement of Dr Paul Giangrande para 97.1 WITN3311003. See the chapter on Access to Treatment.
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From early 1998, all cases of vCJD reported to the NCJDSU and diagnosed as having “probable” vCJD (not simply “confirmed” cases) resulted in a search of blood donation records to enable the destruction of any products made from those donations.1559 1998 is important for the taking of two major precautions: the actual start of the previously agreed programme of leucodepletion, and the acceptance of a recommendation not to use UK-sourced plasma to make blood products (instead, plasma for this purpose was to be imported).
As to the first, on 17 July 1998, the risks of transmission were determined to be high enough to justify starting a “£70 million” programme of removing most of the white blood cells from blood destined for transfusion: it became universal to the UK by October 1999.1560
As to the second, on 30 April 1998 the Committee on the Safety of Medicines recommended: “that manufactured blood products should not be sourced from UK plasma. Although it was accepted that some parts of the manufacturing process for blood products may separate prion proteins, the present state of the art means that these processes cannot be validated. Therefore the theoretical risk that nvCJD could be transmitted by blood products cannot be discounted.”1561 BPL and the Protein Fractionation Centre were to agree on a date after which no products would be made from UK plasma. The Government accepted that advice, and on 12 November announced a “£30 million programme” to ensure the use of UK-sourced plasma for the manufacture of blood products would be phased out, by the purchase of plasma from foreign sources believed to be BSE-free.1562 From the end of 1999 no UK plasma was used.1563
This went further still. The principal source of the plasma from outside the UK necessary to make blood products was the US, where there were signs that large pharmaceutical companies were increasingly acquiring smaller plasma collectors. This posed a potential risk to the continuation of supplies of plasma to BPL at reasonable cost. Accordingly, in December 2002 the UK Department of Health purchased the largest remaining independent plasma collector – Life Resources Incorporated – to ensure the continuity of supply without needing to rely on UK-sourced plasma.1564 It should be noted that this proactive step occurred before the first case of a person known to have contracted vCJD from transfusion
1559 Department of Health Press Release Further Precautionary Measures on Blood Products Announced 26 February 1998 p1 HSOC0015101_002, NBA Procedure for Responding to Information supplied by the CJD Surveillance Unit 14 August 1998 NHBT0008720_002
1560 House of Commons Statement by the Secretary of State for Health: Development in vCJD p2 ABHB0000181
1561 Appendix 1 to minutes of Committee on Safety of Medicines meeting 30 April 1998 p1 DHSC0041250_103
1562 House of Commons Statement by the Secretary of State for Health: Development in vCJD p2 ABHB0000181
1563 Minutes of Advisory Committee on the Microbiological Safety of Blood and Tissues for Transplantation meeting 28 October 1999 pp2-3 NHBT0004333
1564 Department of Health Press Release Department of Health Secures Guaranteed Long-Term Supplies of Plasma for NHS Patients 17 December 2002 HCDO0000266_021
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was reported. It involved time, effort and expense in order to take precautions against a threat which, though theorised, had never as yet materialised.
To return to the chronological story: in August 2000 the CMO (England), on behalf of the CMOs of all four nations, set up the CJD Incidents Panel (“CJDIP”). It was asked to develop a framework for the management of possible exposures to CJD through medical procedures which would then be subject to consultation with interested bodies and organisations.1565 This framework document was not published in its first edition until March 2004 and some sections – including establishing a confidential database without consent, estimates of the infectivity of blood components and plasma derivatives, and “Advice on the investigation and management of incidents involving blood” – were “greyed out” as they had not yet been finalised.1566 Consultation took place in the interim two and a half years or so. Much of that focussed on ethics and the risks and benefits to patients.1567 Before the framework document was established, however, the CMOs of each of the four nations were asked to approve it. They responded in June 2003.1568 Yet it was not for another nine months that the document was first published: during that interim, as described below, a highly significant event occurred.
The first death from vCJD linked to a transfusion
The account so far is that once it was suspected that BSE might have spread to humans, precautions were taken progressively to lessen the risk of transmission of vCJD through blood. These were:
(a) screening donors to exclude riskier donations
(b) recalling products made from batches to which someone later found to have had CJD had donated, and destroying them
(c) conducting a “lookback” to check if people with CJD had themselves received blood or blood products and if so from whom, and then if donations from those later shown to have CJD had caused symptoms in recipients
1565 Letter from Professor Michael Banner to Professor Sir Liam Donaldson 4 October 2002 DHSC0004806_026, CJD Incidents Panel Management of possible exposure to CJD through medical procedures 10 October 2001 NHBT0096710_001
1566 CJD Incidents Panel Management of possible exposure to CJD through medical procedures: Framework Document 15 March 2004 p6, pp30-37, pp44-45, pp46-48 PHEN0000383. The framework drew particularly on two risk assessments: one in respect of the Risk Assessment for Transmission of vCJD via Surgical Instruments: A Modelling Approach and Numerical Scenarios by the Economics and Operational Research Division of the Department of Health; and the second in respect of the Risk Assessment of Exposure to vCJD Infectivity in Blood and Blood Products produced by Det Norske Veritas for the Department of Health. Department of Health Risk Assessment for Transmission of vCJD via Surgical Instruments: A Modelling Approach and Numerical Scenarios January 2001 HSSG0000136_043, Det Norske Veritas Risk Assessment of Exposure to vCJD Infectivity in Blood and Blood Products for Department of Health February 2003 MHRA0007248
1567 “The Panel debated the ethical impact of its decisions many times over the years” was how Dr Nicola Connor put it in her written statement. Written Statement of Dr Nicola Connor para 20 WITN7091001
1568 Letter from Professor Sir Liam Donaldson to Professor Banner 9 June 2003 HCDO0000108_106
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(d) adopting leucodepletion
(e) phasing out the use of UK-sourced plasma in the manufacture of blood products
(f) using recombinant factor concentrates for some patients
Some of these precautions involved considerable expense and effort. Yet at the time they were adopted the risk of transmission from one person to another through blood or blood products was purely theoretical. There had been no known case of it happening in the seven years since it had become known that BSE caused vCJD in humans. In sharp contrast with both clinical and governmental reaction in the early 1980s to the risk of AIDS, the argument that there was no conclusive proof to show that blood transmitted the cause of the disease was not rolled out to justify inaction. Rather, the approach was to act first, just in case, even if it later turned out that some measures might have been unnecessary.1569
Things changed in 2003. On 17 December 2003 the Secretary of State for Health, Dr John Reid, told Parliament that earlier in the autumn a patient had died. He had received a donation of blood in March 1996 from an infected donor. That donor died some three years later of the disease. Dr Reid announced that: “In the light of the facts which I have outlined, it is therefore possible that the disease was transmitted from donor to recipient by blood transfusion, in circumstances where the blood of the donor was infectious, three years before the donor developed vCJD, and where the recipient developed vCJD after a six and a half year incubation period. This is a possibility not a proven causal connection.”1570
This possible case led to further precautionary measures, announced immediately. 15 people who had received transfusions of blood from donors who subsequently developed vCJD were to be told. Those who had received blood products made from human plasma, and were concerned that it may have contained vCJD prions were invited to call NHS Direct. The MSBT was asked to reassess whether donors who had previously received a transfusion should be permitted to donate blood. The MSBT was to discuss with the Medical Royal Colleges and NHS hospitals reducing the use of blood transfusions to situations where it was absolutely necessary for medical reasons – measures to achieve this had only been partially successful before then.1571
1569 Dr Angela Robinson, who was the National Blood Authority’s Medical Director at the time, described the change in approach as follows: “There was a shift around the time of vCJD when the concept of the ‘precautionary principle’ was introduced. At that stage we were doing enormous things at great cost which we had not done before” and she attributed that to the CMO Dr Kenneth Calman. Written Statement of Dr Angela Robinson para 306, para 311 WITN6926001. Dr Gail Miflin became Medical Director of NHSBT in 2016 and thought the response was also informed by earlier blood-borne infections, with the precautionary approach partly: “as a result of the difficulty in testing blood for vCJD, and partly as a result of the experience of HIV and HCV in the preceding decades.” Written Statement of Dr Gail Miflin para 1129 WITN0672006
1570 Statement of Secretary of State for Health regarding blood transfusion incident involving vCJD 18 December 2003 pp3-4 HCDO0000108_005. The case was the subject of a written report: Llewelyn et al Possible transmission of variant Creutzfeldt-Jakob disease by blood transfusion The Lancet 7 February 2004 NHBT0008743_013
1571 Statement of Secretary of State for Health regarding blood transfusion incident involving vCJD 18 December 2003 pp4-5 HCDO0000108_005
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The risk which had previously been purely theoretical may have manifested itself in practice. Further impetus was given to the need for precautions less than a week after Dr Reid’s statement by the publication of research findings. The findings, of what came to be known as “the first appendix study”, emphasised the risk. It suggested that there was a prevalence of infection of about 1 in 4,000 people, albeit from a study of limited size.1572 These were people with no known symptoms whose appendices or tonsils showed an accumulation of the misfolded prion.1573
The MSBT met in January 2004. It agreed that any donor who had received a blood transfusion after 1 January 1980 should be excluded from donating.1574 On 16 March 2004, Dr Reid made a ministerial statement accepting this recommendation.1575
What should people be told of their personal risk?
The recognition of the first known case of transmission of vCJD by blood stimulated a reconsideration of whether those who received blood from a donor who had later been found to suffer vCJD should be notified of this,1576 and indeed, whether to notify members of high-risk groups so that they did not donate blood, or have surgery without telling the surgeon of the increased risks there might be if surgical instruments used in their treatment were later used in the treatment of others.
The position which had been adopted back in 1996 was based on a number of factual premises. They are best illustrated by an exchange with Dr Patricia Hewitt, a member of the Standing Advisory Committee on Transfusion Transmitted Infections at the time it was proposing a study to check on recipients of those blood donors who later developed vCJD.1577 Around May 1996 she spoke informally to Professor (now Sir Ian) Kennedy, then a professor of medical ethics at King’s College London.1578 He understood at the time that there was no evidence that vCJD was transmissible by blood transfusion; there was no screening nor diagnostic test; and no treatment to be offered to those infected. She remembered his advice as being that on balance it favoured not notifying identified recipients. He considers, now, that the lack of scientific evidence that vCJD was transmitted by blood transfusion at the time was “crucial”. His view was and is that if such evidence became available, recipients should be identified and notified, since at that point their futures would be wholly changed,
1572 Hilton et al Prevalence of lymphoreticular prion protein accumulation in UK tissue samples Journal of Pathology 2004 DHSC0006581_004
1573 If infected there, the infection might then spread through the lymphoreticular system as described at the start of this chapter until it reached the brain.
1574 Minutes of Advisory Committee on the Microbiological Safety of Blood and Tissues for Transplantation meeting 22 January 2004 p4 NHBT0035101
1575 Hansard extract on Developments in vCJD 16 March 2004 p1 DHSC0020695_173
1576 See the MSBT’s earlier approach in 1997, described above.
1577 Minutes of Standing Advisory Committee on Transfusion Transmitted Infections meeting 16 April 1996 p7 NHBT0000088_013
1578 She mentions this in a letter to Professor Kennedy: Letter from Dr Hewitt to Professor Kennedy 15 April 1999 NHBT0017407
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and they were entitled to know that.1579 He is clear that if it were ever established that vCJD could be transmitted through blood transfusions it would be wrong not to inform recipients. He stated that his general view is that, ordinarily, people/patients should be informed if there is a reason to believe they are at risk as regards emerging diseases. This is the case notwithstanding the fact that there is nothing that they can do in terms of treatment in response to the information because “once informed, they can at least adjust their lives and their relationships with others.”1580 Though he does not now recall the conversation, Dr Hewitt’s recollection closer to the time was that indeed he raised two important caveats – that if there was any change in the capacity to diagnose the disease, or if any intervention was possible, then the means to contact infected recipients must be in place.1581
Dr Hewitt wrote some three years later to Professor Kennedy, mentioning the conversation. By then the relevant circumstances had changed. A decision had been taken not to allow recipients of blood from people who later developed vCJD to become blood donors themselves.1582 In his evidence to the Inquiry Professor Sir Ian Kennedy commented (rightly, and obviously) that that contemplated a scenario in which a number of those recipients would be informed that they had received blood from someone who later developed vCJD:
“Quite apart from the relevant ethical questions in 1996 (which may or may not be answered differently in 1999 given the change of facts) there was a fundamental shift in the analysis. It was no longer a question of whether recipients should be informed, but instead, given that they (or some of them) will be informed given the policy at the time, how should they be informed? The answer to that, of course, is as carefully and sensitively as possible.” 1583
It is plain that had Professor Kennedy been available to respond to the letter (he was not)1584 his advice would have changed diametrically from his earlier informal advice as recorded in that letter, because the circumstances were now so different.
The Lothian Research Ethics Committee initially took the view that no attempt should be made to trace nor inform recipients of implicated donations, and after being asked to review its position in October 1997, reiterated its earlier advice. It took the view that it was possible that the very act of advising a recipient in such circumstances would itself be construed as an injury given the mental suffering that would undoubtedly result and the probable impact on the recipient’s status with regard to life/healthcare insurance.1585 By the end of 1997, an
1579 Written Statement of Sir Ian Kennedy para 46 WITN7007001
1580 The central principle which Sir Ian Kennedy describes himself as applying was the ethical principle of concern for the rights and interests of people/patients. In the vast majority of circumstances that would mean that people are to be informed of what is contemplated by way of healthcare so they can decide for themselves what they wish to do. Written Statement of Sir Ian Kennedy para 15 WITN7007001
1581 Letter from Dr Hewitt to Professor Kennedy 15 April 1999 p1 NHBT0017407
1582 Letter from Dr Hewitt to Professor Kennedy 15 April 1999 p2 NHBT0017407
1583 Emphasis in the original. Written Statement of Sir Ian Kennedy para 47 WITN7007001
1584 He was chairing the public inquiry into children’s heart surgery at the Bristol Royal Infirmary. Written Statement of Sir Ian Kennedy para 42 WITN7007001
1585 SACTTI Position Statement 16 December 1997 NHBT0004115
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awkward position had been reached in which it had been agreed: (a) to question donors in relation to any family history of CJD, (b) to investigate whether people with CJD had ever received transfusions or given blood, (c) to conduct a lookback to identify recipients of blood donations from donors who subsequently developed CJD, and (d) to recall batches of blood products to which someone with vCJD had contributed,1586 and yet it had also been agreed that no one should be told that they were at risk of either transmitting or themselves developing vCJD (or both) and why. When the blood services were informed of suspected cases of vCJD from any sources which were then confirmed by the NCJDRSU, the recipients would have to be traced – but not informed. The advice had been accepted that the leucodepletion of blood and blood products should be extended in order to protect the public against risk. In short, serious and costly steps to protect public health were taken on the basis that there was a real risk that blood and blood products might transmit the cause of vCJD – yet no attempt was to be made to advise individual recipients that that real risk applied in particular to them.
It is difficult, in principle, to justify a position based on there being no scientific evidence that a disease can be transmitted by blood and blood products (ie no meaningful risk) as a basis for dealing with patients whilst recognising that there is such a risk when introducing measures to protect public health. A sensible policy should not look in opposite directions at one and the same time.
A practical difficulty was recognised in applying the policy of non information. To meet the public health risk blood products might have to be recalled from patients. What should they be told? The advice from the NHS Executive to NHS medical directors on 6 February 1998 was that they had been told by ethics experts and advisory bodies that there was no need to inform patients of their exposure because:
• it was thought unlikely that vCJD would be transmitted this way
• there was no diagnostic test for vCJD
• there was no preventative treatment for vCJD
“In these circumstances the general view is that patients will not benefit from this knowledge, and that uncertainty created by informing patients could have the contrary effect causing unjustified worry and creating a permanent blight on their lives in relation, for example, to obtaining life or health care insurance.” The advice then added that it was for individual
1586 Minutes of Advisory Committee on the Microbiological Safety of Blood and Tissues for Transplantation meeting 27 October 1997 p3 SBTS0000522
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clinicians to decide whether to follow the ethical advice.1587 A letter in identical terms was sent by the Scottish Office to NHS trust medical directors on 23 April 1998.1588
What was to be said to those who came to donate blood and were told it could not be accepted? Professor Len Doyal wrote to Dr Hewitt of the National Blood Authority expressing his view that donors who were told their blood would not be used must be informed why that was, and that it would be illegal and immoral to allow someone to give blood when it was known that the donation would be destroyed. He identified the inconsistency:
“The problem is that the National Blood Authority has adopted a policy about the non-use of the blood of the recipients of potentially infected blood which entails that they must be informed that they are ineligible to give it. The Department [of Health] has also insisted that as the medium of potential transmission, white cells be removed from blood for transfusion. Both decisions suggest – and will certainly do so to the public – that there is evidence of transmissibility. Therefore, recipients or donors who are told that their blood cannot be used must be informed of the circumstances surrounding this decision. On the one hand, if they are given no explanation then they will rightly demand it. On the other hand, if they are told nothing and allowed give [sic] blood which is then simply destroyed, they would be doing so under false pretences. This is both immoral and illegal. If any thing should now be clear in the practice of health care in Britain, it is that deception is not an option for good clinical practice or public policy.” 1589
At the same time as Dr Hewitt had been speaking to Professor Doyal, the MSBT discussed how to manage donors known to have received blood from people who subsequently developed vCJD. In January 2000 the outcome was reported to Dr Angela Robinson in her role as medical director of the National Blood Authority. Although the letter recognised the current Department of Health policy that people who might have been exposed to vCJD through blood or blood products should not be informed of this, it said that that policy would be kept under review. It was noted that MSBT had agreed that “in the spirit of openness” and “contracts with donors” the blood services would need to consider telling, or offering to tell, the donor why their blood could not be accepted. However, discussions with such donors
1587 Letter from Dr Graham Winyard to NHS Trust medical directors 6 February 1998 p1 BART0002418. The full text deserves citation. It adds: “There may clearly be some circumstances where clinicians will decide to inform a particular patient of the reason for the product withdrawal, for example where a product involved in the recall is one that is generally held by the patient at home, or where the recall action has prompted an individual patient specifically to ask whether he/she has received the implicated blood product. In such circumstances it is for the clinician to decide how best to respond, having taken careful consideration of all aspects of his/her patients circumstances.” Letter from Dr Graham Winyard to NHS Trust medical directors 6 February 1988 p2 BART0002418. By saying what it did the NHS Executive might arguably be telling clinicians they were free to act unethically: although it must be true that each patient’s case is individual, and the treatment should be appropriate to the individual, so that other ethical considerations may come into play, this possibly represents a reluctance to interfere too much with “clinical freedom”.
1588 Letter from Sir David Carter to NHS Trust Medical Directors 23 April 1998 GGCL0000111_001
1589 Letter from Professor Doyal to Dr Hewitt 20 December 1999 p2 NHBT0004392_002
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were to be managed on a case-by-case basis, and the appropriate health department contacted in the first instance. A protocol to deal with this was to be developed.1590
In the meantime the continuing debate about whether or not people should be told that they had an increased risk of developing vCJD had a chilling effect on the continuation of the Transfusion Medicine Epidemiology Review (“TMER”) study to examine a possible connection between transfusion and the development of vCJD. On 30 January 2000 the Lothian Research Ethics Committee refused the NCJDRSU’s application for renewed ethical approval of the TMER.1591
Professor Will of the NCJDRSU made a further application on 23 May 2000 to reconsider ethical approval for the TMER on the basis that it was unethical not to do the study because it might be the only mechanism by which transmission of vCJD through blood or blood products could be identified.1592 On 31 May 2000, in response to this, the Lothian Research Ethics Committee then reinstated approval for the TMER – but this was on the basis that the decision whether to inform a person identified as being at risk by the TMER was left to the local health authority.1593 For four months the study had been without ethical approval.
In June 2000 the options were discussed at a meeting. It was agreed, after what the minutes suggest was a wide-ranging discussion, that the questionnaires which were always completed regarding a donor’s medical history should have a question built into them which would effectively allow patients to make an informed choice about whether they would like to be told of risks to their health. They would be told that their blood could be rejected for a range of reasons from the very minor to a major health concern. It also agreed that full counselling would be available to the patient if they made the decision to be informed of any risk to their health that emerged.1594
As part of risk management the CJDIP sought to balance the individual’s “right to know” and “right not to know” about possible exposure to risk.1595 Although the CJDIP were close to finalising their framework for the management of possible exposures to CJD through medical procedures for publication by the time the Secretary of State announced in December 2003 that 15 people would be informed that they had received transfusions from donors who subsequently developed vCJD, the section on managing blood incidents was marked as not yet finalised.1596 The Department of Health asked the Health Protection Agency (“HPA”) to take the lead in notifying people. The HPA were informed about 19 people who should be notified, 2 of whom were in Scotland and 2 of whom transpired to have died. The HPA
1590 Letter from Dr Mike McGovern to Dr Robinson 12 January 2000 NHBT0004310
1591 Letter from Dr Ian Starkey to Professor Robert Will 30 January 2000 NHBT0004364_004
1592 Letter from Professor Will to Dr Ian Starkey 23 May 2000 p2 NCRU0000112_068
1593 Letter from Dr Ian Starkey to Professor Will 31 May 2000 p1 NCRU0000112_069
1594 Minutes of NHS Blood and Transplant meeting 16 June 2000 pp5-6 NHBT0009063_002
1595 Minutes of NHS Blood and Transplant meeting 16 June 2000 p4 NHBT0009063_002
1596 CJD Incidents Panel Management of possible exposure to CJD through medical procedures Framework Document Draft 1 December 2003 pp47-49 DHSC0020839_003, Statement of Secretary of State for Health regarding blood transfusion incident involving vCJD 18 December 2003 p4 HCDO0000108_005
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decided that the local health protection team and the GP should decide the most appropriate way to inform and support each person.1597
Mark Buckland was a young man who was infected with vCJD in September 1997 as a result of receiving a blood transfusion during surgery. He received a letter from the HPA in early January 2004 as part of the national notification exercise, informing him that he had been identified as having received a significant volume of blood transfusions that carried a theoretical risk of vCJD.1598 Mark’s father, Peter Buckland, subsequently discovered that the blood service had known since 18 August 2000 that one of the donors of a unit transfused into his son in 1997 had died of vCJD.1599 Dr Hewitt explained that the initial decision not to inform recipients was made by the Department of Health.1600
The impact of the delay in informing Mark Buckland of this was considered during the inquest into his death. The Coroner heard (from Dr Stephen Wroe, a consultant neurologist) that had Mr Buckland been in regular contact for review with the National Prion Clinic then the diagnosis of vCJD would have been made between 6 and 18 months earlier than it was. This in turn would have allowed Mark Buckland’s earlier entry onto the trial of quinacrine. Moreover, earlier information would have allowed Mark, his family and friends more time to come to terms with the diagnosis rather than being left to struggle with an (erroneous) diagnosis of chronic fatigue syndrome. It was Dr Wroe’s view, which was shared by the Coroner, that Mark Buckland should have been informed that he had received blood from a donor with vCJD as soon as the Department of Health became aware of that fact in 2000. As the Coroner explained, “patients should have the opportunity of receiving appropriate assessments, advice and treatment if they wish and being able to deal with the possible future, doing what they may wish to do and helping their families to come to terms with the future as well.”1601 Mark’s father Peter told the Inquiry that withholding the information from Mark was wrong: “he would have thought to himself, ‘Okay, if this is the case and I don’t know, but if this is the case I’ll make sure I live a full life’, I would have told him to do that, instruct him, I’m sure he’d have thought the same. At least he would have known the truth.”1602
1597 Health Protection Agency CJD Team Interim Report on incident involving blood components and vCJD and the patient notification exercise conducted from December 2003 to January 2004 21 January 2004 pp4-9 PHEN0000104. Following the diagnosis of vCJD in two more former donors, another ten people who had received transfusions were notified in 2005. Minutes of CJD Incidents Panel meeting 7 September 2005 pp6-7 PHEN0000629
1598 A copy of a similar letter and a patient information sheet is at Letter from Health Protection Unit to Patients 31 December 2003 PHEN0002392_005, Health Protection Agency CJD Team Interim Report on incident involving blood components and vCJD and the patient notification exercise conducted from December 2003 to January 2004 21 January 2004 pp27-28 PHEN0000104
1599 Peter Buckland Transcript 6 June 2019 pp71-73 INQY1000015
1600 Written Statement of Dr Patricia Hewitt para 11 WITN3101002. This advice is recorded in the letter set out above: Letter from Dr Graham Winyard to NHS Trust medical directors 6 February 1998 p1 BART0002418
1601 Written Statement of HM Deputy Coroner for Brighton & Hove Arthur Hooper 16 August 2006 p6 WITN0694008. The Coroner wrote to the Secretary of State for Health to express his concern that Mark Buckland had not been told of the position at the earliest possible stage. Letter from Arthur Hooper to Dr Patricia Hewitt 26 September 2006 p4 WITN0694002. Caroline Flint responded on behalf of the Secretary of State. Letter from Caroline Flint to Arthur Hooper 12 October 2006 PRIU0000015
1602 Peter Buckland Transcript 6 June 2019 p74 INQY1000015
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It was not known in 1997, when Mark Buckland was transfused, that the donor suffered from vCJD. It was known in 2000. By then, the risk that blood transfusions might transmit vCJD was understood, sufficiently for the several measures set out above to have been taken to protect people from this happening. Given this, given what Professor Doyal said in 1999, given the views that Professor Sir Ian Kennedy has, then I am clear that these views of Dr Wroe, the Coroner and Peter Buckland, are right. Dr Hewitt now feels that the blood services may have been “erring on the side of not acting soon enough to impart potentially devastating news in terms of possible exposure to HCV and vCJD.”1603 She too is right.1604
Half a year after the first set of notifications to people who had received transfusions, the CJDIP produced a report in July 2004 recommending which groups should be told they had an increased risk of vCJD for public health purposes from implicated plasma, and setting out notification timelines.1605 This referred to the announcement the previous December of the first transfusion-related case of vCJD. So far as patients who received plasma products were concerned, they were regarded as being “at risk” if they were assessed as having received sufficient blood products to be considered exposed to a 1% or greater risk of infection, on top of the general risk to the UK population from eating beef.1606
More cases come to light
A second case of transfusion-related vCJD was reported in July 2004.1607
On 7 September 2004, the CJDIP finalised their advice, with a three-stage categorisation of the likelihood of patients being “at risk” of vCJD for public health purposes – “High”, “Medium” or “Low”. “High” was where the amount of potential infectivity was high enough for patients to be considered “at-risk” following the administration of a very small dose (eg one treatment with Factor 8 or 9); “Medium” where the amount of potential vCJD infectivity in product batches was not low enough to be ignored but substantial quantities of the material
1603 Written Statement of Dr Patricia Hewitt para 105 WITN3101006. Dr Hewitt told the Inquiry that “With hindsight, I think the difficult issues and strongly held views from both sides (those who supported notification of the possibly affected, despite the potential for psychological harm, and those who felt that such harm outweighed the benefits) may have led to erring on the side of not acting soon enough to impart potentially devastating news in terms of possible exposure to HCV and vCJD”. In her oral evidence she referred to another case where a recipient developed vCJD, whose family had said that “If they had known that he had been at risk, his last few months would have been dealt with differently … they would have known what they were dealing with, or what they were likely to be dealing with.” Dr Patricia Hewitt Transcript 10 December 2021 p129 INQY1000171
1604 However, because the question is essentially an ethical one, I think she is being too careful in introducing her view with the words “With hindsight”. Though the answer to ethical questions may often be difficult to tease out, they were expressed at the time; they were capable of being expressed at the time; and in this case – that of the failure to notify Mark Buckland earlier – the proper course was not followed.
1605 vCJD and Implicated Plasma Products Notification Road Map 23 July 2004 LOTH0000082_007
1606 The threshold of 1% was consistent with the threshold for patients exposed through surgical instruments. Report on Notification of potential exposure to vCJD through plasma products 7 January 2005 p5 PHEN0000721
1607 See: Health Protection Agency Variant Creutzfeldt-Jakob Disease (vCJD) and Plasma Products: Clinical Information 7 September 2004 p2 HCDO0000650, House of Commons Notice of Written Ministerial Statement on Blood Donation and vCJD 9 September 2004 p2 HCDO0000660
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would need to be administered for patients to be considered “at-risk” (eg several infusions of intravenous immunoglobulin, or large doses of albumin 4.5%); “Low” where the amount of infectivity was so low that the likelihood of the patient being considered at potential additional risk of vCJD could realistically be ignored. These were specifically thresholds for public health purposes, and not estimates of assessed individual risk.1608 CJDIP also wrote to those responsible for tracing vCJD-implicated plasma batches setting out tables of implicated batch numbers.1609
Two days later, Dr Reid, the Secretary of State for Health, announced in Parliament that clinicians had been given the information to enable them to identify potentially infected batches, and said that they would then as a precaution notify any patient identified as “at risk” as having received product from that batch. He commented: “Although there are now two reports of possible transmission of vCJD via blood, the risk of transmission via plasma products, which will have been derived from large pools of plasma donated from many thousands of people - and therefore heavily diluted - is uncertain. But it cannot be excluded.” He added that: “Throughout this exercise we have been concerned to ensure that the results of the risk assessment are communicated to patients by the clinicians responsible for their day to day care, so that appropriate supporting information can be provided.”1610
The HPA and the Scottish Health Protection Centre for Infection and Environmental Health (“SCIEH”) had consulted on how to notify patients:
“The UKHCDO and Haemophilia Society argued that since a single dose of implicated plasma concentrate would be sufficient to place a recipient ‘at-risk’ and because future batches were likely to be implicated … all patients with bleeding disorders treated with UK-sourced pooled factor concentrates or antithrombin between 1980 and 2001 should be considered ‘at-risk’ and asked to take public health precautions, rather than just those who had received the known implicated batches”. 1611
People with a bleeding disorder were informed by their haemophilia centre in September 2004 and given an opportunity to discuss the implications and to find out if they had received an implicated batch if they wished.1612
1608 Assessment of exposure to particular batches of variant Creutzfeldt-Jakob disease (vCJD) implicated plasma products 7 September 2004 HCDO0000647
1609 Note addressed to those responsible for tracing vCJD implicated plasma product batches in the UK 7 September 2004 HCDO0000649
1610 House of Commons Notice of Written Ministerial Statement on Blood Donation and vCJD 9 September 2004 pp2-3 HCDO0000660
1611 The UK Primary Immunodeficiency Network preferred an individual approach of informing only those patients who had received an implicated batch. Report on Notification of potential exposure to vCJD through plasma products 7 January 2005 pp7-9 PHEN0000721
1612 See for example letters sent by the Newcastle Haemophilia Centre, including a leaflet jointly produced by the HPA, SCIEH, National Public Health Service for Wales and the Northern Ireland Department of Health, Social Services and Public Safety. Letters from Dr John Hanley and Dr Kate Talks to Patients or Parents 20 September 2004 NTHT0000012. Letter from Dr Frank Hill to Haemophilia Centre Doctors 9 September 2004 HCDO0000658. See also the evidence of Professor Charles Hay who
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By January 2005 the HPA reported that approximately 4,000 people with bleeding disorders had been told they were at risk from vCJD, and that it had identified 12 other patients who by reason of their conditions and exposure to plasma products were at sufficient risk to require public health precautions to be taken (notifying them, so that they could then notify their families and any treating doctor). By this time, 9 plasma donors were known to have developed vCJD and had made 23 blood donations which had been made into 187 batches of various plasma products.1613
By July 2005 it had been identified that three patients had developed vCJD almost certainly as a consequence of receiving blood transfusions. Between them, they had received the blood of 110 donors. Those then had to be traced, and advised that they should not give any further donations, nor should their tissues or organs be donated.1614
In January 2007 a fourth case of vCJD transmission was associated with blood transfusion and was reported to the press.1615
Thus far, despite the umbrella approach which had been taken for notifying everyone with a bleeding disorder who had attended a haemophilia centre for treatment between 1980 and 2001 that they were “at risk”, none of the identified cases related to a person with a bleeding disorder. However, on 7 September 2009 it was confirmed that a post mortem carried out on a man with haemophilia found the vCJD prion in his spleen. He did not die of vCJD; nor was it present in the brain. However, the probability was that he had been infected and that the likeliest cause was his treatment with a plasma product.1616 An early report of this case in The Sunday Telegraph on 15 February 2009 prompted an update being urgently sent to people with bleeding disorders saying that “The information from this case does not change the public health ‘at risk’ status of any patients with bleeding disorders”. 1617
Whereas people who had received transfusions were only notified if a donor went on to be diagnosed with vCJD, patients undergoing surgery or endoscopy were now to be asked if they had received blood transfusions from 80 or more donors since 1980 and considered at increased risk if so.1618
was vice chair of UKHCDO in 2004 and later chair. Written Statement of Professor Charles Hay paras 144.1-150.2 WITN3289039
1613 Report on Notification of potential exposure to vCJD through plasma products 7 January 2005 p2, pp15-16 PHEN0000721. On individual assessment, none of the patients with primary immunodeficiencies were assessed to be at-risk for public health purposes.
1614 Hewitt et al vCJD Donor Notification Exercise: 2005 Clinical Ethics 2006 p1, p7 RLIT0000156
1615 Health Protection Agency Draft Press Release 4th case of variant CJD infection associated with blood transfusion 16 January 2007 HCDO0000131_006
1616 The surveillance form is dated the same day: UKHCDO/Department of Health surveillance form 7 September 2009 p2 HCDO0000131_056. Written Statement of Professor James Ironside para 8(a) viii WITN7034001, Peden et al Variant CJD infection in the spleen of a neurologically asymptomatic UK adult patient with haemophilia Haemophilia 2010 pp4-6 HCDO0000799
1617 Email chain entitled vCJD running on Telegraph website 15 February 2009 15 February 2009 pp3-4 DHSC5198184, Template letter from UK Haemophilia Centre Doctors to patients February 2009 p8 CVHB0000011_015
1618 Letter from Dr Hester Ward, Mr David Pryer and Professor Jeffries to Chief Executives of NHS Boards July 2009 p1 RLIT0001222
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In July 2011 the Advisory Committee on Dangerous Pathogens Transmissible Spongiform Encephalopathy Risk Assessment Sub-Group met for the first time. It advised the Department of Health that the policy adopted thus far had relied on the precautionary principle, and was thus largely driven by the “‘worst case’ scenarios”. It was not surprising that there would be changes as more information accrued over time. Prediction of future infection would change, and policy would evolve accordingly. The models which had been used to predict the risk of future infection indicated rates of infection which were higher than those actually seen. There might thus be a need to adjust the models to account for this.1619
A consequence of this reasoning, and a subsequent paper from the Department of Health recalculating the level of risk,1620 was that in 2013 those who had received plasma products only between 1980 and 1989 should be de-notified – ie told they need no longer consider themselves as more of a risk than other members of the public, and need not declare any additional risk to doctors or dentists as they had been doing.1621
By 2013, 11 patients who had received transfusions from more than 80 donors and were due to undergo surgery had been identified.1622 The threshold was revised by 2014 so that only patients who had received transfusions donated by an aggregate total of 300 or more donors were to be considered at risk for public health purposes.1623
By mid summer 2014, it appeared that the worst fears of a rapid rise in the number of cases of vCJD had not been realised. It seemed possible to entitle an official report by the House of Commons Science and Technology Committee on UK blood safety and the risk of vCJD: “After the storm?” But what remained uncertain was also recognised: there was a question mark after the word “storm”.
1624 The report concluded:
“in the vast majority of cases, the benefits of receiving a transfusion will far outweigh the risks of acquiring a transfusion-transmitted infection. However, we urge against complacency and stress the need for UK Blood Services to remain vigilant to the threat posed by blood-borne pathogens … We consider it imperative that a precautionary approach to [the risk that prions remain present in the blood supply] be maintained until further evidence becomes available … Pathogens are constantly emerging and evolving; novel pathogens will therefore always pose a threat to the blood supply. In the past, it has often taken multiple cases of transfusion-transmitted infection before these threats have been recognised
1619 Minutes of Advisory Committee on Dangerous Pathogens Transmissible Spongiform Encephalopathy meeting 14 July 2011 pp6-7 DHSC5235271
1620 Blood-Borne Transmission of vCJD: Revisions to Risk Assessment 31 August 2011 PHEN0000600
1621 Letter from Dr Katy Sinka to Dr Gerard Dolan 24 January 2013 WITN3775004, Letter from Dr Dolan to UKHCDO colleagues 25 April 2013 pp3-5 LGFT0000020
1622 Written Statement of Dr Nicola Connor para 103 WITN7091001
1623 ie the aggregate of the number of single units they had received, many of which might have been given on repeated occasions: the reference to “donors” indicates that some multiply transfused patients may have received more than one unit from the same donor, but this only counted once. Template letter from Dr Sinka to doctors of patients at increased risk of vCJD 2014 WITN7091009
1624 The full citation is: House of Commons Science and Technology Committee After the Storm? UK blood safety and the risk of variant Creuzfeldt-Jakob Disease 24 July 2014 TSTC0000052
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and mitigated. This will remain the case as long as risk mitigation measures remain pathogen-specific. We urge the Government to take steps to support the development of broader spectrum technologies with the potential to mitigate the risk of both known and unknown pathogens.” 1625
In its final paragraph the Committee wrote:
“We conclude this report by recommending that the Government take a more precautionary approach to both vCJD risk mitigation and blood safety more generally, in order to safeguard against future infections. We suggest that it begin by assessing the key risks, known and unknown, that the UK blood supply currently faces and might face in the future, so that it can identify and fill relevant knowledge gaps and support the development of appropriate risk reduction measures and technologies.” 1626
The position as at the date of this Report
There have been 178 cases of vCJD in the UK identified as definite (supported by post mortem) or probable (no post mortem), though the latter category is virtually certain.1627 No new case of vCJD has been identified since 2016.1628 No one now living in the UK has been diagnosed with vCJD.
Although no case of vCJD has manifested itself since 2016, a review of three studies conducted between 1995 and 2014 of the prevalence with which abnormal prion proteins seen in samples of appendices removed at operation (hence “appendix studies”, as they are known) found that 1 in 2,000 of the population had abnormal prion protein in their appendix.1629 The exact implications of this remain unclear.1630 Thus it is not known whether these individuals are in a carrier state, such that they might unwittingly pass on the abnormal proteins through the use of surgical instruments, or by giving blood – though as to the latter, leucodepletion seems to have successfully prevented this happening so far.
Leucodepletion was, however, introduced too late to prevent the transmissions which occurred in the four symptomatic cases in which there has been known to be transmission
1625 House of Commons Science and Technology Committee After the Storm? UK blood safety and the risk of variant Creuzfeldt-Jakob Disease 24 July 2014 p53 TSTC0000052
1626 House of Commons Science and Technology Committee After the Storm? UK blood safety and the risk of variant Creuzfeldt-Jakob Disease 24 July 2014 p57 TSTC0000052
1627 Professor James Ironside Transcript 17 May 2022 p28 INQY1000207
1628 NCJDRSU 26th Annual Report 2017 Creutzfeldt-Jakob Disease Surveillance in the United Kingdom 2017 p11 RLIT0001605, Expert Report to the Infected Blood Inquiry: Statistics September 2022 p95 EXPG0000049
1629 Diack et al Public health risks from subclinical variant CJD Public Library of Science Pathogens 30 November 2017 p2 RLIT0002363
1630 Written Statement of Professor Robert Will pp34-35 WITN7098001
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of vCJD via blood and blood components, and the one other case which was via blood products and asymptomatic.1631
Research has shown that there is genetic variation between individuals relating to the make-up of the prion protein. One of two chemicals – methionine or valine – is involved. The possible combinations were likened by Professor Ironside in his evidence to those of blood groups: just as blood may be grouped, as A, B, AB, or O depending upon the genetic coding of the individual concerned, so the prion protein may be MM, MV, or VV. In all but one case of vCJD so far identified, the patient has been MM. The current working supposition is that MM individuals are more likely to have the shortest incubation period, and VV the longest, with MV somewhere in between.1632 If this is so, and if indeed some people who are asymptomatic are carriers who are capable of transmitting their abnormal prions, some of the risk may not yet have materialised. The prospects are encouraging, but vigilance remains essential.
Steps as yet untaken: missed opportunities or steps too far?
It was apparent after 1997 that if the risk of transmissibility from one human to another were to be borne out in practice, there were two particular implications for public health.
First, there was a risk that surgical instruments used in abdominal surgery might transmit the disease from one person to another since prions stick “rather avidly”1633 to the surface of surgical stainless steel. There was thus a risk that surgery on the gut and internal organs would, just as in the case of surgery on the brain, give rise to a risk of the transference of prion disease, unless the instruments could successfully be decontaminated. Second, it was essential to establish as accurately as possible in the UK those who were incubating prion disease even if they had not yet suffered symptoms, because in such a pre-symptomatic state they might unwittingly transmit the disease to others, and give rise to an epidemic.
Professor Collinge described how his unit1634 addressed both concerns. First, his unit was directed in the early 2000s to research methods of decontamination, and was funded by the Government to do so. It developed by trial and error what he said could be thought of as a “sort of biological washing powder” which cleaned surgical instruments of prions – it was a mixture of enzymes which would break up proteins, together with things that enabled those enzymes to access the surface to be cleaned: a form of detergent with enzymes. He also was able to develop an assay which showed whether the “washing powder” had
1631 For details of these see the evidence of Professor Ironside: Professor James Ironside Transcript 17 May 2022 pp31-35 INQY1000207. See also: Expert Report to the Infected Blood Inquiry: Statistics September 2022 pp95-98 EXPG0000049
1632 Professor James Ironside Transcript 17 May 2022 pp23-25 INQY1000207
1633 Professor John Collinge Transcript 13 May 2022 p32 INQY1000206
1634 The Medical Research Council Prion Unit, established in 1998 at the University College London Institute of Neurology under Professor Collinge “to provide a national centre of excellence with all necessary facilities to pursue a major long-term research strategy in prion and related diseases”. House of Commons Science and Technology Committee After the storm? UK blood safety and the risk of variant Creutzfeldt-Jakob Disease 24 July 2014 p34 TSTC0000052
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actually been effective in any given case in achieving decontamination. This showed that his unit had succeeded in developing a product which could show a reduction of prions on a metal surface by “about a millionfold”.1635 It would best be used to soak instruments prior to autoclaving. For it to be produced in reasonable quantities at an acceptable cost for use by NHS Trusts required commercial involvement. DuPont, who had marketed a high-level disinfectant for surgical products called Rely+On Perasafe since 1998, were interested in incorporating the unit’s technology into it, by making Rely+On Prion Inactivator. In 2008 and 2009 the use of the product was evaluated by the Government’s Rapid Review Panel. This accepted that basic research and development had been completed, and that the product had “potential value” but should be further evaluated by trials in an NHS setting. In 2010, DuPont put further development of the product on hold, because it had proved difficult in practice to initiate a UK trial, and because the Rapid Review Panel indicated that because its use would involve a change of practice in established decontamination procedures it was unlikely to find widespread acceptance unless the risk of vCJD became a greater concern than it was by then.1636
The NHS has not adopted the use of this prewash product.1637 Rather, their present approach is to rely upon quarantining surgical instruments to ensure that those on whom they had been used do not, within the quarantine period, show signs of CJD.1638 This in turn has led to access to some surgical procedures being difficult for patients who are considered to be in a class which is generally at higher risk than the general public, in particular people with bleeding disorders and people who had multiple transfusions: the Inquiry has heard reports of Trusts being unwilling to conduct some operations (or of delaying operations) because of a worry that the expensive equipment required will have to be quarantined, meaning that further sets of the equipment would then need to be purchased for use in the meantime. They regard this as simply too expensive and inconvenient.1639 It is plainly not acceptable that
1635 Professor John Collinge Transcript 13 May 2022 pp67-70 INQY1000206
1636 House of Commons Science and Technology Committee After the storm? UK blood safety and the risk of variant Creutzfeldt-Jakob Disease 24 July 2014 pp22-25 TSTC0000052
1637 The reasons given by the Department of Health for this are set out in Morwenna Carrington’s statement. The product was not adopted by the NHS in 2007 “as it did not achieve a category 1 rating from the RRP [Rapid Review Panel]. All manufacturers of products are required to have a category 1 rating to be suitable for the NHS. Other manufacturers developing similar pre-soaks also did not achieve a category 1 rating from the RRP and were not recommended for use in the NHS.” Written Statement of Morwenna Carrington para 3.25 WITN7590001, NHS National Institute for Health and Clinical Excellence Patient safety and reduction of risk of transmission of CreutzfeldtJakob disease (CJD) via interventional procedures November 2006 SCGV0002357. It was not adopted later because (as it appeared to Morwenna Carrington from reviewing the documents on the evaluation of the pre-wash product), “it appears that the product required further development to ensure adequate decontamination and implementation for use in the NHS. The product was not resubmitted for review and other companies did not achieve a category 1 rating. At that time, pre-soaks were not considered viable products for use in the NHS and, as it was not resubmitted and further development work was not continued by DuPont, it is my understanding that it was not implemented, and other decontamination methods were used.” Written Statement of Morwenna Carrington para 3.33 WITN7590001
1638 Professor John Collinge Transcript 13 May 2022 pp70-72 INQY1000206
1639 Guidance was issued in 2006 by the Advisory Committee on Dangerous Pathogens’ Transmissible Spongiform Encephalopathy subgroup entitled “Assessment to be carried out before surgery and/ or endoscopy to identify patients with, or at increased risk of, CJD or vCJD”, which was updated from time to time. The 2014 iteration of this guidance makes it clear that patient care should not
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people who have been infected as a result of NHS treatment should then find themselves further disadvantaged and put at risk by having operations or investigations deferred or denied, and steps should have been taken to ensure that this does not continue to happen.
Second, the implication was that a test for the presence of the misfolded prion was needed. This was also achieved by Professor Collinge’s team. In 2011, they reported the development of a prototype blood test to diagnose vCJD in symptomatic individuals which could be further developed into a large-scale screening test for asymptomatic vCJD prion infections. In trials on a US population, supposed to be free of vCJD (since the US was free of BSE) the tests produced no false positives. Data showed it was likely to detect successfully three out of every four cases of vCJD that came before it.1640
The test has remained as a prototype. In order to be rolled out as a screening test for blood the test kit would need to be manufactured on a commercial scale. A commercial company was unlikely to develop and market such a test without first seeing the results of a study comparing large populations of those people presumptively exposed to vCJD with those presumptively unexposed (a large-scale comparison of 20,000 to 50,000 people in the UK and 20,000 to 50,000 people in the US). A study of this size is costly. Professor Collinge found that companies would not invest in such a study without being assured that there was a sufficiently significant problem with prions in blood in the UK to create a profitable market for the test. His team were unable to secure a commercial partner to make such an investment. The Medical Research Council, which had funded the initial research, also failed to finance such a study. The House of Commons Science and Technology Committee report in 2014 considered that a vCJD prevalence study utilising a version of this prototype test would be “of considerable value, both for test development and research purposes”
be prejudiced as a result of the procedures to be followed in identifying patients at an increased risk of vCJD. Annex J to Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection August 2017 p1 WITN7080005. Despite this, the Inquiry has received many accounts of the impact of this. Barbara ANON described how her late husband, Leigh, was refused endoscopies after he was diagnosed with liver cancer in consequence of infection with Hepatitis C because he had received a notification letter about vCJD. “We ended up paying thousands of pounds for Leigh to undergo the necessary procedures. It not only cost us money but it cost Leigh valuable time.” Written Statement of Barbara ANON p9 WITN2555001. Ian ANON needed his gallbladder removed having been infected with Hepatitis C and the surgeon told him “You are an expensive patient. We had to archive everything in the theatre because you have a variant CJD risk”. Ian said: “I felt deeply embarrassed and dirty at the time.” Written Statement of Ian ANON para 21 WITN2072001. Another man was refused a liver biopsy as part of care for Hepatitis B and C. Written Statement of ANON para 112 WITN5343001. Another man infected with Hepatitis C told the Inquiry: “The greatest impact of this whole saga has been the problems that I have experienced with regard to my suspected ‘infection’ with vCJD. Having been told that I was ‘at-risk’ for public health purposes in 2004 I noticed a significant change in how I was treated by the medical profession … It happens so often and it drives me mad, especially because of the number of appointments I have to attend. I have been made to feel dirty and these last minute changes of plan are incredibly frustrating, not to mention the problems of actually physically getting myself to these places because of my other health issues … It really upsets me.” Written Statement of ANON paras 38-51 WITN5407001. Mr AN told the Inquiry of having to insist upon procedures being carried out in the face of unwillingness from clinicians, and of then being made to wait around until the end of the patient list before being seen, and of having had procedures cancelled on the basis that there was no suitable equipment available. Mr AN Transcript 16 October 2019 pp32-33 INQY1000042, Written Statement of ANON WITN1387016
1640 Edgeworth et al Detection of prion infection in variant Creutzfeldt-Jakob disease: a blood-based assay The Lancet 5 February 2011 NHBT0033626
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and recommended that there be a large-scale vCJD prevalence study within the following 12 months – it considered other candidates, and concluded that Professor Collinge’s offer was the most promising.1641 However, to date, this recommendation has not been given effect.
The BSE Inquiry
Within two years of the announcement that there was a probable link between BSE and vCJD, an inquiry was set up to “establish and review the history of the emergence and identification of BSE and new variant CJD in the United Kingdom”. 1642 It started work just before Christmas 1997, and reported nearly three years later on 26 October 2000. Though reference should be made to the report itself for the full record of its findings and conclusions, some have particular resonance for this Inquiry. In particular, amongst these are the following:
“• The rigour with which policy measures were implemented for the protection of human health was affected by the belief of many prior to early 1996 that BSE was not a potential threat to human life.
• The Government was anxious to act in the best interests of human and animal health. To this end it sought and followed the advice of independent scientific experts – sometimes when decisions could have been reached more swiftly and satisfactorily within government. …
• At times bureaucratic processes resulted in unacceptable delay in giving effect to policy. …
• The Government did not lie to the public about BSE. It believed that the risks posed by BSE to humans were remote. The Government was preoccupied with preventing an alarmist over-reaction to BSE because it believed that the risk was remote. It is now clear that this campaign of reassurance was a mistake. When on 20 March 1996 the Government announced that BSE had probably been transmitted to humans, the public felt that they had been betrayed. Confidence in government pronouncements about risk was a further casualty of BSE.” 1643
In a section of its conclusions relating to communication of the risk posed by BSE to humans, the report added that:
“The public was repeatedly reassured that it was safe to eat beef. Some statements failed to explain that the views expressed were subject to proper
1641 House of Commons Science and Technology Committee After the storm? UK blood safety and the risk of variant Creutzfeldt-Jakob Disease 24 July 2014 pp34-37, pp41-42 TSTC0000052
1642 The Interim Response to the Report of the BSE Inquiry February 2001 p5 MHRA0021312
1643 The BSE Inquiry Report Volume 1 Findings and Conclusions 26 October 2000 pp1-2 CABO0000141_004
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observance of the precautionary measures which had been introduced to protect human health against the possibility that BSE might be transmissible. These statements conveyed the message not merely that beef was safe but that BSE was not transmissible … The impression thus given to the public that BSE was not transmissible to humans was a significant factor leading to the public feeling of betrayal when it was announced on 20 March 1996 that BSE was likely to have been transmitted to people.” 1644
Financial support
On the same day that the report of the BSE Inquiry was published in October 2000, the Government announced, first, that it would set up a fund for the care of victims of vCJD to ensure a speedy response to diagnosis and improvement in the quality of care for such patients. The NCJDSU was to coordinate this. Second, it was to set up a compensation scheme to operate through a special trust fund.1645 It was set up in April 2001 and interim payments of £25,000 would be made without delay on an ex gratia basis to families of those who had been diagnosed with vCJD.1646 By October 2001, Alan Milburn, Secretary of State for Health, had announced further details of the compensation scheme. It was to be administered by an independent body, the vCJD Trust, and would provide payments of up to a maximum of £55 million for the first 250 cases with a discretionary fund capped at £5 million. The scheme would be updated if the number of cases exceeded 250. In addition, the Government was to pay an additional £50,000 to each victim or their family. This was to take account of legal and other difficulties the first families had had to encounter and the additional pressures they had had to bear.1647
If averaged out between the 250 expected cases this effectively provided for around £300,000 per family. The payment was not to be taken into account for the purposes of calculating income-related social security benefits nor be subject to “claw back” under the social security compensation recovery scheme.
The vCJD Trust
The Trust has the following features of particular relevance to this Inquiry:
(a) The chair of the Board of Trustees is, and has been throughout, a High Court Judge. Shortly after his appointment to the High Court Bench in 2001, Sir Robert Owen was invited to be chair of the Board of Trustees. Though he retired from the bench in 2014, he remains the chair of the Board. He believes that he was appointed because he had particular expertise in personal injury work, in particular having been involved in litigation concerning the effect of human growth hormone derived
1644 The BSE Inquiry Report Volume 1 Findings and Conclusions 26 October 2000 CABO0000141_008
1645 Hansard extract on BSE Inquiry Report 26 October 2000 p2 MHRA0021233
1646 Briefing note on the vCJD No-fault Compensation Scheme 8 May 2001 p3 DHSC0004343_029
1647 Department of Health Press Release Compensation Scheme for Variant CJD Victims Announced 1 October 2001 pp1-2 NHBT0008988
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from pituitary glands harvested during post-mortem examinations, a number of recipients of which developed CJD at a young age.1648
(b) He was consulted about the other six original appointments to the Board, which included two family trustees whose contribution to the work “both in terms of their personal experience and as a channel of communication with the victims and their families, has been essential.”1649 It also included legal expertise from both north and south of the border. The trustees are paid on the standard scale applicable to nondepartmental bodies appointed to advise the Secretary of State. There has been a continuity of trusteeship which in the view of Sir Robert “has been of considerable benefit given the complexity of the Scheme and the reducing workload over the 20 years for which the Trust has been functioning.”1650
(c) Rather than have administrative staff, solicitors were and are engaged to undertake the necessary administrative work, as well as provide legal advice where needed.1651
(d) The Trust uses the records they maintain of the claims made and determined to help ensure that decisions are consistent over time and with each other.1652
(e) The Trust is under no obligation to report to the Department of Health; it is entirely independent of it. Its only involvement is where an amendment or variation is proposed to the terms of the Trust deed.1653
(f) The NCJDRSU, to which a treating clinician is advised to report any suspected case of vCJD is required to ensure that the victim and/or his/her family are made aware of the vCJD Trust and the possibility of compensation. In effect, the NCJDRSU acts as a “gatekeeper”. 1654
(g) No claim is means-tested. A beneficiary’s circumstances and household income are not taken into account.1655
Level of payments
There is a basic sum paid to all. In addition, there are payments made within a fixed range applicable to some heads of claim.
The basic sum as at May 2022 was £141,400 for victims diagnosed after 31 March 2021. It is uprated annually in line with the Consumer Prices Index.1656
1648 Written Statement of Sir Robert Owen para 9 WITN6441001 1649 Written Statement of Sir Robert Owen para 11 WITN6441001 1650 Written Statement of Sir Robert Owen para 19 WITN6441001 1651 Written Statement of Sir Robert Owen para 18 WITN6441001 1652 Written Statement of Sir Robert Owen para 20 WITN6441001 1653 Written Statement of Sir Robert Owen para 24, para 26 WITN6441001 1654 Written Statement of Sir Robert Owen para 37, para 40 WITN6441001 1655 Written Statement of Sir Robert Owen para 47 WITN6441001 1656 Written Statement of Sir Robert Owen para 54 WITN6441001
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As to some of the heads of claim, the trustees have fixed a level of £15,000 for those who have suffered particular emotional hardship; three levels of fixed award for those who have particular financial hardship (£10,000, £25,000 or £40,000); carers’ loss of earnings at four levels (the same three as those relating to financial hardship, plus a lower, £5,000 level); and the same levels for victims’ loss of earnings.1657
The original scheme contained a mix of fixed discretionary and ongoing claims, some of considerable complexity and thus costly to administer: Sir Robert’s view a year after appointment was that the scheme the Trust was tasked to administer was a model of how a scheme ought not to be set up; he added, however, that it had been greatly improved by amendments since. Such was the complexity of the scheme that it had been costly to administer, a matter which has caused some concern to trustees. The views of Sir Robert expressed in his written statement are particularly valuable:
“there are considerable benefits in operating such a scheme by means of a Trust.
First and foremost the decision making has been informed by the involvement of Trustees of relevant expertise, and experience, in particular that of the family Trustees. Secondly, it gave the Trustees independence from the Government, which has been beneficial in terms of gaining the trust of the beneficiary community. Thirdly, and although some elements of the Scheme were unduly complex particularly in its pre 2010 form, the carefully defined provisions governing each head of compensation have ensured fair and consistent decisions over the years, and have kept disputes and complaints to a minimum. Finally … The Trust Deed ultimately gave the Trustees the flexibility to make decisions on matters which had not been contemplated when the Deed was first drafted to ensure victims/ families were adequately compensated and supported.” 1658
He went on to observe that when the initial 117 families negotiated the terms of the Trust deed they had successfully argued for several “discretionary awards”, payable based on the particular circumstances of the individuals claiming. He commented: “In fact, it was these elements of the Scheme which proved most costly, time consuming and upsetting to family groups.”1659
Commentary
There is a dramatic, informative contrast between the way in which the transmission of both HIV and hepatitis were dealt with by clinicians, advisory and government bodies, and regulators and the way the risk of vCJD was handled.1660 Did this lead to a difference in the
1657 Written Statement of Sir Robert Owen para 58 WITN6441001
1658 Written Statement of Sir Robert Owen para 74 WITN6441001
1659 Written Statement of Sir Robert Owen para 77 WITN6441001
1660 This comment is made by way of comparison. It does not intend to suggest that the reaction to the risk of vCJD was a perfect model to follow – some evidence to the Inquiry suggests there would have been room for improvement: see, for example, the evidence of Professor Collinge, and that of Peter Buckland.
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consequence? There has certainly been such a difference. The extent to which vCJD has been seen to occur as a result of the transfusion of blood or receipt of blood products is undoubtedly orders of magnitude less than in the cases of HIV and hepatitis.
However, there has to be a word of caution before accepting too easily that the difference in the approach to risk caused the difference in outcome – for obvious reasons, it has been impossible and unethical to conduct a study on people to establish what might have happened to them if the precautions had not been taken, compared with what did actually happen. This is one of the difficulties that any effective public health system will face. If it works as well as it might, the threats with which it deals will never seem to be real threats in the popular mind, but rather phantom menaces – for they will either not materialise at all or do so in a very limited number of cases. There may indeed be some “threats” which in time turn out to have been overstated. This in turn can lead to an absence of interest in financing and empowering a public health approach to many dangers – for those who in fact identify real dangers may be seen to be “crying wolf”, since the failure of previously imagined dangers to materialise (albeit as a result of successful precautionary measures) may suggest there are few real ones to overcome, and limit the will to resource public health adequately. The consequences of a failure to take enough measures, sufficiently strongly, sufficiently early, universally and sufficiently supported by the population, are clear when the failure materialises in disease and death, and will be the subject of complaint by many if they are not taken quickly. If however the system works, such that there is little or no disease and few if any deaths, then there is little complaint but a risk of much complacency.1661 There is, however, much less disease.
The main similarities and contrasts in response
Although some of the deficiencies in the approach taken initially to the risks of human beings being infected with vCJD as a result of eating beef were criticised by the BSE Inquiry as being reassuring rather than accurate and candid, and that trust in the authorities was sacrificed as a result, repeating the pattern seen in respect of HIV and hepatitis infections, in other respects there was a marked difference.
There are many points of similarity between HIV and vCJD as to transmissibility by plasma and plasma products. Both infections could not initially be detected before symptoms became apparent. Both had a long incubation period. Both had no cure: both could be fatal (though vCJD invariably so). Some of these similarities were shared with hepatitis, which initially could not be tested for, and manifested its worst effects after a long incubation period. There was treatment, however, available for Hepatitis C in due course, though the treatment brought its own problems as described elsewhere in this Report.
1661 The House of Commons Science and Technology Committee appears to have thought this had happened with the continuing response to vCJD – it regarded the NHS as resistant to change. See its report in 2014: House of Commons Science and Technology Committee After the Storm? UK blood safety and the risk of variant Creutzfeldt-Jakob Disease 24 July 2014 p25 TSTC0000052
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However, there were two important differences between vCJD on the one hand, and HIV and hepatitis on the other. Firstly, those suffering from vCJD did not have to suffer stigma of the nature endured by those with HIV in particular, but also by many with Hepatitis C; and, secondly, they did not have to wait so long for substantial financial support to be given, or for that matter for an Inquiry to be set up.
Other differences highlight the inadequacies of the response in particular to HIV infection. In the case of vCJD significant precautionary and protective steps were taken to prevent transmission through blood and blood products. Thus blood donations from those who might pose a risk were declined even whilst the risk remained purely theoretical. Expensive steps were taken to ensure the leucodepletion of blood. UK-sourced plasma ceased to be used as a basis for blood products. The Government went so far as to purchase a US plasma collector to ensure a continued supply of plasma which was likely to be free of the problem prion, and did so before there was a single known case, anywhere in the world, of the infection being transmitted by blood. The principle of “no evidence of harm is not evidence of no harm” was in effect applied1662 in respect of vCJD, at least after 2006, so far as recipients of blood and plasma products were concerned.
By contrast, in the case of AIDS, Professor Arthur Bloom appeared to suggest to the public that the (cause of the) disease was not transmissible by blood, and pointed out that there had been no case of it in the UK as a reason for inaction – though there had been a number of such cases in the US. When one case of vCJD arose which was probably caused by transfusion, it spawned further protective measures, all designed to minimise the risk as far as possible. By contrast, very little happened to prevent the further transmission of a probable cause of AIDS by transfusion once it became known that there were one or two cases of infection in the UK in 1983 from that route,1663 and (worse still) after the first reported death of AIDS in a victim almost certainly infected through blood products. Whereas the taking of precautions, in particular probably leucodepletion, may well have prevented significant numbers being infected with vCJD through transfusion or the receipt of blood products, the failure to take precautions against the risk that HIV was transmitted by blood, as described elsewhere in this Report, contributed to the significant number of deaths and serious infections with HIV in the UK.
There are also comparisons to be made in respect of the financial response of the government towards those who suffered the infections. Both the scheme for vCJD and the schemes for HIV and Hepatitis C were expressly ex gratia. But the sums awarded to the Trust to be paid to the families of vCJD victims dwarf the sums paid to those suffering from HIV and Hepatitis C. Moreover, an express component of the payments made to the families of initial victims of vCJD was made to reflect the struggles they had to find the disease recognised as a consequence of animal food policy. By contrast, those who were infected with either
1662 No one described the principle in these terms, but it makes for a pithy summary of part of the basis on which the approach taken was adopted.
1663 To administer blood products is in essence the same process as to transfuse blood – the characteristics of one person’s blood are transferred to the circulation of another with a view to the restoration of their health.
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or both HIV and Hepatitis C had a long struggle for recognition of the moral case they had for financial support, and through much of that period had to contend with the corrosive effects of stigma on most aspects of their daily lives. When, eventually, the Alliance House organisations and special payment trusts were used as a vehicle to pay some sums in support to victims of blood-borne HIV and Hepatitis C, those trusts and schemes lacked the advantageous features of the vCJD scheme identified above. Though Sir Robert was concerned about the complexity of many of the provisions governing the Trust, in other respects it was a model solution compared to the trusts and schemes described in the chapters on the Alliance House organisations.1664
In both the cases of vCJD on the one hand, and HIV and Hepatitis C on the other, there was a misplaced reluctance to tell people that they might be particularly at risk of infection for fear that it might cause panic or in some way affect future donations to the blood system. The views described above of Professor Sir Ian Kennedy and Professor Doyal in respect of telling people they were at a raised risk of vCJD were appropriate. People should have been told. The reluctance to operate on the basis that individuals were at a raised risk of vCJD resonates with the desire in the early days of the government handling of the potential spread of AIDS through blood and blood products to reassure the public.
The communication of risk to the public, to the extent many felt betrayed, drew particular criticism from the BSE Inquiry. It is a further tragedy that the lessons of the handling of communicating the risks of HIV/AIDS appear not to have been learned by the time of the BSE Inquiry. However, perhaps the bigger tragedy still is that in the face of unknown diseases which were potentially transmissible by blood the government was able in the 1990s and early 2000s to demonstrate that taking a proactive, precautionary approach could avert much disease – it will have left many people infected and affected by Hepatitis C or HIV both disappointed and angry that such an approach could not have been taken in response to earlier blood-borne infections.
1664 See the chapters on the Macfarlane Trust, Eileen Trust, Skipton
