In October 1976, Dr Alan Dickinson, a veterinary scientist of the Agricultural Research Council, who is working on scrapie, telephones the MRC to alert officials to the risk of transmission of CJD through human growth hormone.

In a 1981 report of the NBTS Protein Fractionation Technology Working Party, we read of scientific advances in the manipulation of genetic material that led to technological developments introducing the possibility of large-scale production of proteins [of which Factor VIII is one] for therapeutic use from bacteria or from animal cells cultured in vitro [in the test tube or Petri dish].

There have been two major lines of work toward this end: "One has drawn on recombinant DNA techniques to construct bacteria capable of producing "human proteins". The alternative has been to change the characteristics of animal, including human, cells to make them more amenable to large-scale cultivation in vitro.

We are not surprised to see what criteria the NBTS PFT Working Party use to decide whether or not to implement genetic manipulation techniques:

"It is necessary to decide whether the principal plasma proteins could be generally available at a competitive cost and of proven safety in a time-scale which bears upon the development of BPL. If so it is important to decide whether the technology arising from genetic manipulation is one which BPL would logically become engaged in." (page 27, para 3)

"Given prompt action on the redevelopment of conventional fractionation facilities, the return on investment will have been achieved before genetically engineered products can have a major impact. Nevertheless failure to take account of them may lead to a crisis shortly after the new plant has commenced operation." (page 30, final para)

Note: From 1981, it took 13 years for recombinant clotting factors to be licensed for use within the UK (i.e. in 1994). We believe that this could have been done in far less time, like Hyland, who were running human clinical trials of their recombinant in 1987. We would also like to point out that it took Government until 2006 to fully 'roll-out' recombinant factor concentrate to all adult haemophiliacs across the UK.

From 1981, that's a grand total of 25 years of deferment.

In the minutes of the first SNBTS Factor VIII Study Group, it is clear that physicians have knowledge as early as 1982 of the viral risk of the Creutzfeldt-Jakob agent (CJD) in blood products. It should be noted that the agent referred to here is not the variant form (vCJD), which is not classified until March / April 1996.

Background:
Classical CJD or ‘sporadic’ CJD is the most common form of Human TSE. CJD was first described in 1921 by two German neurologists, Hans Gerhard Creutzfeldt and Alfons Maria Jakob. The disease is not related to food consumption. The neurologists do, however, describe a case of progressive fatal dementia in a female patient; accompanied by multiple neurological abnormalities.

The human growth hormone programme discontinues in 1985 following reports of the first deaths from CJD in the United States, after which synthetic hormone is used.

In the minutes of the First Meeting of the Advisory Committee on the Virological Safety of Blood (ACVSB) on 4th April 1989, we read the following in relation to the handling of Human Growth Hormone (hGH) recipients:

"Dr Pickles questioned whether it was more ethical for recipients of hGH to learn of the risks from the media rather than from their doctor." (see page 10, point 8, of PDF link below)

Ethical Considerations:
"This committee decided that there was no need to create anxiety among hGH recipients by telling them not to donate blood or carry donor cards and further action was considered unjustified as the risk of infection was so small and the risk of transmission through transfusion so uncertain."

"However concern had been expressed at the ethics of informing young people that they might have a lethal infection which could not be identified and for which there was no treatment."

NOTE: We would suggest that this is not the first time this concept has been used on patients where bad news has been disseminated, as if through osmosis, via the media. Ethics aside, it is clear that this practice suits both physicians and the Department quite nicely.

15.02.09 - Haemophiliac Contracts vCJD from Plasma:
We would like to draw attention to the news leak and general mishandling of the first case of a UK haemophiliac to contract vCJD who had been treated with plasma products. We understand that none of the agencies dealing with the haemophilia community, such as the Haemophilia Society, were informed of what had happened and none of the 4,000 people known to have received vCJD-implicated batches of plasma products were afforded the opportunity to have any help or counselling prior to this announcement.

In a draft paper for the January '91 meeting of the Advisory Committee on the Virological Safety of Blood (ACVSB), Dr Hilary Pickles demonstrates knowledge of the possible risk of prions being transmitted by blood donors.

In this 1991 background paper entitled "CJD Agent/Prions and Blood Donors" the risk factors for blood and blood products in relation to CJD are discussed:

"CJD is not a recognised complication of treatment by blood products as in haemophilia, even though viral-inactivating procedures are unlikely to be adequate to deal with the CJD agent." (Page 3, point 3.)

Despite the above statement that CJD is not a recognised complication with blood products, we are extremely concerned to learn that a Government Advisory committee clearly had knowledge in 1991 of the risk of prions to blood products for the treatment of haemophilia and that the viral-inactivating measures were probably going to be inadequate to deal with the CJD agent.

Background: Sadly, by September 2004, the Health Protection Agency, is required to facilitate a tracing exercise of approximately 101 batches of vCJD-implicated factor VIII & IX manufactured in the UK by Bio Products Laboratory (BPL) and the Protein Fractionation Centre (PFC), Scotland. We believe that more could have been done to protect recipients of blood products between 1981; when genetic engineering of plasma proteins such as factor VIII was discussed and 1996; when vCJD was first documented.

Note: We need to draw attention to the fact that this paper relates to CJD (as in sporadic or classical CJD) and not specifically vCJD (Variant CJD) which was not documented until 1996.

In a briefing note by John Sloggem intended for Professor Gull for the Biologicals Subcommittee (CSM) meeting of 1st November 1995, CJD and plasma-derived blood products are discussed as follows:

J. SLOGGEM: "There is no experimental evidence in animals to suggest that CJD is transmitted by whole blood or plasma. Plasma being cell free is thought safer because scrapie infectivity is tissue associated and sheep plasma - cell free - appears clear of scrapie infectivity."
..."There is no epidemiological evidence to suggest that there is a higher incidence of CJD in groups of patients receiving plasma derived products regularly. They are closely monitored by clinicians presumably any major excess number of cases would be spotted."

J. SLOGGEM: "Basically the risk is theoretical."

..."Besides classical blood products, anything with a blood derivative could be considered "risky" and mean that vaccines, other biological and biotechnological products would be withdrawn."

"The risk/benefit ratio was not considered in favour of withdrawal on the basis of to-date theoretical risk."

Background: From these statements, it appears as if John Sloggem neither read nor absorbed Dr Pickles paper on Prions and Blood Products ('ACVSB 9/9') from February 1991 - which incidentally was over 4 years earlier. Mr Sloggem should have been able to recall from the ACVSB background paper that CJD was going to be a problem in blood products, especially since Dr Pickles (see related entries link below) recorded that viral-inactivating procedures were unlikely to be adequate to deal with the CJD agent. We would hardly call this 'theoretical'.

NOTE: It is also possible to see from the above comment on 'monitoring' exactly why people with haemophilia have very much been ignored and left ill-informed over vCJD.

A new disease, vCJD, is first described and documented in April 1996. The first cases of Variant CJD (vCJD) actually appeared in 1995, but it was not until 1996 that the new variant of CJD was recognised.

A letter was sent from Leeds NHS Executive headquarters to all NHS Trust Medical Directors and Health Authority Chief Executives dated 6 February 1998. The letter, which discussed nvCJD and patients who have received implicated blood products, stated: "...there have been a number of recall exercises over recent months…" and advises doctors not to tell patients why their plasma products have been recalled.

"The advice which the Department has received from ethics experts and other advisory bodies is that there is no need to inform patients because:

1. ...it is thought unlikely that nvCJD will be transmitted in this way;
2. ...there is no diagnostic test for nvCJD;
3. ...even if a test was available, there is no preventative treatment that could be offered."

The letter went on to explain that: "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 of health care insurance."

BACKGROUND: This letter was undisclosed and the haemophiliac community and campaign groups knew nothing of it until Carol Grayson and her husband Pete Longstaff received some leaked letters around September 2000. Carol informed the Guardian, the Newcastle "Journal" and "Private Eye" in the year 2000 and an item on the subject later went out on the BBC's "Watchdog" programme. As a result of these media articles, UK physicians eventually wrote to their patients in 2001 asking them if they wished to be informed of their exposure to vCJD.

NOTE: How can the NHS Executive even attempt to justify not informing patients? We don’t think much of their ‘expert ethical advice’! We feel it is quite scandalous to learn that if Carol Grayson hadn't got hold of the leaked documents we would never have learnt of just how long the NHS, DOH and even BPL had already sat silently on the information since February 1998. Also, it is clear that they certainly appeared to have had no intention of ever telling people with haemophilia & other bleeding disorders directly. Without intervention from the press there would never have been the series of vCJD look-backs.

It is clear from this behaviour that the authorities were quite happy to lie to our faces. So when, exactly, would they have informed us of our exposure?

In an article by James Meikle and Alex Bellos in the Guardian on 5th February 2001, the following was stated:

Blood products donated by three people who were later struck down with the human form of BSE have been sold to 11 countries. Thousands of patients worldwide, and an unknown number of haemophiliacs in Britain, might have received treatments with the products between 1996 and last year. The risk of infection - which health department officials insist is only theoretical - has now been closed off by restricting blood sources to the US.

BPL had informed appropriate regulatory bodies abroad that nvCJD patients had donated blood. It had also written to wholesalers in each country and had confirmation they had told their relevant ministries. "We have tried our very best", said a spokesman.

WHERE IT WENT:

• Ireland: Polio vaccine - 83,500 doses
• Brazil: Albumin - 44,864 vials, immunoglobulin 80 vials
• Dubai: Albumin - 2,400 vials
• India: Albumin - 953 vials
• Turkey: Immunoglobulin - 840 vials
• Brunei: Albumin - 400 vials
• Egypt: Albumin - 144 vials
• Morocco: Albumin - 100 vials
• Oman: Immunoglobulin - 100 vials
• Russia: Factor VIII - 23 vials
• Singapore: Immunoglobulin - 3 vials

The Health Protection Agency, Colindale, provides those responsible for tracing vCJD-implicated blood products with tables listing batch numbers of plasma products which have been manufactured in the UK by Bio Products Laboratory (BPL) and the Protein Fractionation Centre (PFC).

The World Federation of Hemophilia website refers to this as "a precautionary measure", "as there is no known case of transmission of vCJD by a plasma-derived treatment product and no one with hemophilia has been diagnosed with vCJD."

On 24th February 2009, one day after the release of the report of the Archer Independent Inquiry, we find Dr Charles R. M. Hay making disturbing comments in the Telegraph. Dr Charles Hay, chairman of the UK Haemophilia Centre Doctors Organisation, stated the following:

"it is a misconception that the risk of contracting Hepatitis C was lower if patients were given UK products instead of American although it would have reduced the risk of catching HIV."

We would suggest that this statement is fundamentally flawed. It is the risk factors of the individual donor population that determine the overall level of risk, not the pool size during fractionation. UK sourced products often came from specially selected volunteer donor panels. Dr Hay goes on to state:

"...the irony was that if self sufficiency had been achieved then a larger proportion of haemophiliacs would have been exposed to donations from donors who went on to develop vCJD."

We absolutely disagree with this statement and pose the following questions as to who was responsible for allowing CJD (BSE) to enter into the human food chain: Who ignored all the warnings of scientists such as Dr Dealler? Who disregarded the significance of prions in blood products as shown in the minutes/background paper of the ACVSB meeting of January 1991? Who failed to develop and implement genetically-engineered factor products, i.e. recombinant, at the earliest opportunity?

We find it disturbing to see the chairman of the UKHCDO making such comments in the Daily Telegraph, since this was the very paper that, only 9 days earlier, had leaked devastating news about the first haemophiliac found to have vCJD at post-mortem. This patient was technically in the care Dr Hay in his role as chairman of the UKHCDO. We find it completely mystifying that on release of this article he did not speak out.

Comment: We feel the need to stress that BSE should never have made its way into the human food chain in the first place. It is, however, even more regrettable that Dr Charles Hay failed to seize this opportunity to apologise to the haemophilia community about the manner in which this news was broken. We are appalled to see him use the word irony in connection with a human tragedy of this magnitude.