The history of variant CJD exposure in haemophiliacs given factor concentrate treatment (pooled plasma products) for an inherited bleeding disorder and the fight for Recombinant synthetic treatment are very closely inter-twined. As soon as they became aware that vCJD could be in the UK blood supply, they knew they were at risk and must look for an alternative treatment.
On the 15th February 1996, the Independent newspaper ran a short article with the headline BSE “cause of death” where a Newcastle virologist Dr Harash Narang was making an extraordinary claim and that was that, “Mad Cows Disease” could be passed on to humans, the article stated,
Peter Hall, of Chester-Le-Street, Co Durham, died last week just days before his 21st birthday after being ill for two years.
Dr Harash Narang, a clinical virologist formerly with the public health laboratory service, said last night Mr Hall had died from Creutzfeldt- Jakob Disease, while Mr Hall’s parents, Derek and Frances Hall, called for more tests to be carried out.
The official Government view is that Mad Cow Disease – or bovine spongiform encephalopathy (BSE) – cannot be transmitted to humans.
Just a month later, on the 20th March 1996, SEAC (Spongiform Encephalopathy Advisory Committee) announced a probable link between BSE in cows and vCJD in humans where 10 cases were reported.
For Peter Longstaff, born with severe haemophilia (less than 1% clotting factor) and this author (his wife), this was a very worrying development. For some years they had watched news reports of distressed and sometimes suicidal farmers, staggering cows with BSE and burning pyres as herds of cattle were destroyed to avoid contaminating the food chain. John Gummer, agriculture minister, 1989-93 was famously seen back in 1990 feeding his daughter a beef burger to help restore public confidence but the public were becoming more and more alarmed.
The current NHS website last updated in 2021, states,
There’s clear evidence that variant CJD (vCJD) is caused by the same strain of prions that causes bovine spongiform encephalopathy (BSE, or “mad cow” disease).
In 2000, a government inquiry concluded that the prion was spread through cattle that were fed meat-and-bone mix containing traces of infected brains or spinal cords.
The prion then ended up in processed meat products, such as beef burgers, and entered the human food chain.
On the 29th March 1996, Longstaff wrote to his then Consultant Dr Peter Jones, Royal Victoria Infirmary, Newcastle, requesting Recombinant, as opposed to human plasma products from UK Blood Products Laboratory (BPL) which it was feared might contain the prion vCJD. This letter has been submitted to the Infected Blood Inquiry chaired by Sir Brian Longstaff who is looking at how haemophiliacs (including Longstaff and his younger brother) came to be infected with HIV and hepatitis viruses through their plasma treatment which included products imported from the US from “high risk” donors, such as prisoners, gay men and prostitutes.
Longstaff was turned down for Recombinant on the grounds of cost (Recombinant could be twice as expensive as human factor concentrates and what we learnt years later was that in the Autumn of the same year (1996) he had 12 exposures to vCJD then classed as “a relatively new and rare neurological disease, classified as a Transmissible Spongiform Encephalopathy (TSE)”.
Although it was years before Longstaff learnt of his exposure to vCJD, we later discovered that haematologists had published a paper in the Lancet about the risks to patients and on the 1st December 1997, Nature Journal, ran with the title, UK accused over risk of CJD in plasma and highlighted the following,
Foot-dragging by the British government is exposing haemophiliacs to an avoidable risk of infection with the new variant of Creutzfeldt-Jakob disease (vCJD) from contaminated blood products. That was the warning issued last week by the organization that represents the directors of 109 centres that treat haemophilia and other complex blood diseases.
The government has acknowledged that blood products pose a potential risk of transmission. But it has so far limited action to commissioning a detailed risk assessment of the problem, and asking the National Blood Authority to consider removing lymphocytes from the blood, given that the causative agent of vCJD may occur in these and other lymphoreticular tissues (see Nature 390, 105; 1997).
In a letter to The Lancet, however, the UK Haemophilia Centre Directors’ Organization says the potential risk is already obvious. It calls for immediate action to protect haemophiliacs by switching them from blood products prepared from UK plasma supplies to either recombinant alternatives or products prepared from donor plasma collected in countries with no recorded cases of bovine spongiform encephalopathy (BSE) or vCJD.
The potential risk to patents was also stated…
The potential risk for haemophiliacs is much higher than for other transfused patients, as they receive multiple transfusions of products prepared from pools of plasma derived from 20,000 to 50,000 individual donations. “There is therefore obviously a much increased risk of getting at least one infected donation in the pool, and one batch of factor VIII may go to 50 haemophiliacs,” says Ludlam.
The article concluded,
Ludlam’s frustration is shared by many researchers who feel that the risk from blood products is receiving insufficient attention. “I took part in the discussion in the early 1980s over HIV and blood, and I have that awful sinking feeling all over again; the issues are so similar,” says Ludlam. “All we need is one haemophiliac to get vCJD and all blood products will be removed from the market.”
The following year 1998, when Longstaff continued to request Recombinant, the BMJ stated that the trust refusing to provide Longstaff with safer treatment, was “following the Department of Health’s guidelines issued in March 1998 that only patients with haemophilia A aged under 16 and those not previously treated with plasma based blood clotting products should be given the genetically engineered recombinant factor VIII.”
Moving on to the year 2000, by chance, Grayson read a letter on v CJD in the Newcastle Journal from Dr Harash Narang who had worked at the Public Health Laboratories in Newcastle. Grayson responded in writing and this was noticed by health journalist Louella Houldcroft who wanted to learn more about what had happened to the haemophilia community. After meeting Grayson and Longstaff, she then invited them to work jointly with her to set up the “Bad Blood” campaign, seeking truth and justice for haemophiliacs given contaminated blood.
Grayson and Longstaff also met up with Dr Narang. To their surprise he stated he already recognized Longstaff’s name from the Newcastle labs as he had tested his blood for hepatitis C. What Longstaff learnt from Narang, was that this occurred years before he knew he was being tested. Seemingly blood had been taken to check clotting factor levels in the blood but was used without his permission to test for HCV in 1992 and apparently before this date. Longstaff was only told he was positive in 1994 in the presence of his wife.
Regarding BSE, Narang explained that he had teamed up with a local business man, Ken Bell, who was in the meat trade and had offices just a 7 minutes walk from the couple’s home. Bell was also concerned about BSE and inadequate safety regulations and was now sponsoring Narang’s work.
Back in 1996, on the 10th January, the Independent wrote about Narang, who was viewed as a controversial figure by some but was sought out by the families of those already dying from what became known as variant CJD. He felt the government were covering up over BSE and the use of MBM so as not to have a negative impact on the British beef industry. MBM refers to meat and bone meal given to cows that are herbivores, eating grass. They have one stomach with 4 compartments for this process and were never meant to eat meat. Milk Means More website explains the process of digestion for a cow as follows:-
Parts of a cow’s stomach:
- Rumen – This is the first part of the cow’s stomach. It helps break down complex plant products like grass.
- Reticulum – Here the food mixes with the cow’s saliva and produces cud. Cows burp up the cud into their mouths and chew it to help break it down more. When you see a cow that looks like she is chomping on bubble gum, really she is chewing her cud.
- Omasum – Here all the water is absorbed out of the food.
- Abomasum – Here is where the food is finally digested, similar to what happens in a human stomach.
The Independent wrote on the working relationship between Narang and Bell stating,
Harash Narang, a clinical virologist at the Public Health Laboratories in Newcastle upon Tyne, was the man Mr Bell chose to sponsor. Having done much original work on spongiform disease over 25 years, Dr Narang had devised a slaughterhouse test to diagnose BSE in cattle that hadn’t yet shown clinical symptoms of the disease. But in 1990 when he applied for an official grant to develop the tests, he was turned down. Beef was safe, the Ministry of Agriculture told him.
Mr Bell was incensed: “Here was a scientist who had some answers. Someone who was prepared to meet the problem head on. And yet the old boy network wouldn’t let him work on the test. They didn’t want some little Indian guy to come along and prove them all wrong.”
Mr Bell offered Dr Narang pounds 20,000 to develop two cattle tests. “I want to be able to practise ‘due diligence’ when I trade meat,” Mr Bell explains. “The sooner BSE is weaned out of the cattle market, the better.” There is also a personal motive behind Mr Bell’s crusade: his brother, a butcher, died in 1983 of a dementing brain disease. “He ate a lot of animal heads”, is all Mr Bell will say on the matter.
Bell did however make a comparison between the safety of British beef and that from Namibia stating,
Namibian beef has a better flavour and is reared naturally, he says: no forced fattening, no dark sheds, no dangerous chemicals. And he says he can guarantee that it is BSE-free. The British beef industry, by comparison, is in a mess and as a meat trader he wants no part in it.
“We are feeding cattle diseased food, then feeding diseased meat to our people … I know some meat traders feel that I have betrayed the industry, but I believe you have to sell what is safe. I don’t know if BSE can be passed on to humans. But I’m not prepared to find out by carrying out an experiment using real people.
A government inquiry into the BSE scandal in 2000 deemed as a whitewash” by some did conclude as mentioned earlier that the spread of BSE “may have been amplified by the feeding of contaminated MBM to cattle.” Those eating BSE contaminated meat then became infected with variant CJD who then donated their blood without realizing their status. These new blood safety concerns prompted Longstaff to begin a very public treatment strike refusing any human plasma to treat his haemophilia.
Narang was keen to further his own research into vCJD but claimed to have been hounded out of his job because he knew too much and because of racism. Among other claims of his were that cases of BSE were known years earlier than government had announced and that he was being blocked on developing a test for vCJD and that his character was being deliberately defamed to silence him. A year before we met Narang, the Independent had now run another very different story on the scientist, expressing doubt over his findings, detailed below,
“ How a scientist obtained thousands for a BSE test he could not prove”
In 2000, the Observer reported that France was so concerned over the health risk from beef they were threatening to sue over BSE,
With most British and French experts agreeing that BSE was a disease ‘exported’ from the UK to France, lawyers believe there would be a case for French families of variant CJD victims to sue the British government
The Philips BSE report published last month confirmed that the spread of the disease was most probably caused by animal feed made from meat and bone meal which contained infected cattle. Britain banned the practice in July 1998, but figures from Customs and Excise reveal that in 1989 France imported more than 15,000 tonnes of meat and bone meal (MBM). By 1990, the amount had dropped to just over 1,000 tonnes and to practically zero the year after.
In 2001 the following appeared in the press, detailed in an old email of this author,
One of the government’s vCJD advisers, Prof. Michael Banner of King’s College in London, said the handing of the situation was a shambles. He said the government was seriously misleading the public about the risk of vCJD because the incubation could be as long as 20 years.
It is important to note that although over 800 haemophiliacs were exposed to vCD through blood from a donor with the prion, due to delays in notifying haemophiliacs that they could be harbouring v CJD which has a very long incubation period, they had no representation at the Philips Inquiry in 2000. There was no test available which could identify those that had vCJD and it was years before most haemophiliacs were notified of their exposure.
The result of cheap cattle feed, BSE in cows, people eating infected beef, donating blood even as they unknowingly incubated vCJD put the national blood supply at risk as Brits could no longer use UK plasma and safety restrictions had to be put in place. This was played down as the beef industry was already nearing collapse over BSE with famers committing suicide and in addition the nation’s blood supply was now compromised through careless policy and the unethical practice of cannibalising cows!
The symptoms of v CJD can be read on the following NHS link.
A Timeline of BSE can be read in New Scientist as follows,
Soon after the Philips Inquiry reported, Grayson was sent a leaked letter stating that haemophiliacs had been exposed to v CJD but “don’t tell them” plus she obtained a 4th of November 1997 product recall letter from BPL containing affected factor concentrate batch numbers given to haemophiliacs.
At this time, Longstaff and Grayson began phone and e-mail contact with Frances Hall, the mother of Peter Hall, who died aged 20. Hall campaigned on vCJD and they were introduced to other families who believed there was a massive cover-up over BSE, vCJD. In many ways this echoed the Contaminated Blood scandal putting profit before safety, cheap feed for cattle and cheaper plasma products for patients at the risk of their health. Grayson and Longstaff were also in touch with Professor Ironside at the CJD Surveillance Unit in Edinburgh.
A Journeyman video from 2000s can be viewed on the following link which paints the picture of the time, a country in fear over the BSE crisis. Dr Narang can be seen on the video.
BSE: Biting the hand that feeds you
Longstaff began his legal fight for Recombinant, access to which had become a “post code lottery” with treatment being phased in by age, aimed at the children first. Longstaff fully supported the children having early access but he also wanted to speed up access for haemophiliacs that were already suffering badly due to their earlier infections with HIV and hepatitis C. Thus began a series of letters back and forth to their local Health Authority pleading for the safer treatment with well reasoned arguements but although some doctors supported the couple, the answer from managers was always in the negative. In exasperation, Longstaff launched a Judicial Review in the High Court.
Haemophiliac patient goes to High Court to get synthetic factor VIII
The case came to be heard in 2003. The Northern Echo reported,
As a result of contracting various infections whilst being treated at the Royal Victoria Infirmary, Newcastle, Mr Longstaff decided in 2000 to refuse further treatment with Factor VIII derived from human blood plasma – made from thousands of individual blood donations – on the grounds that it had proved impossible to guarantee the safety of the product.
At a recent hearing, a judge was told the case was urgent as his situation was ”gradually deteriorating” and time was important.
The judge also heard 95 haemophiliac patients treated at the Royal Victoria Infirmary, Newcastle, had tested positive for HIV infection in recent years, and 78 had died.
Mr Longstaff was in ”the surviving group of 17”.
He had also contracted hepatitis B, diagnosed in the mid-1980s, hepatitis C in 1994. Hepatitis G and the HIV Aids virus were diagnosed about 1985.
Although the judge understood and was sympathetic to the reasons why Longstaff was refusing treatment and wanted a safer alternative to human plasma, he explained that if he allowed his case to go through, it would open the floodgates far beyond haemophilia to other treatment areas.
During this time, the couple met James Meikle from the Guardian, who wrote extensively on BSE and vCJD. They began to collaborate on articles and the couple shared letters regarding their fight to ensure there was no cross contamination to or from others when medical instruments were used in hospital. The government looked at single use instruments however the alternative did not go down well with doctors who found them less precise and at least one death was reported.
A policy was then devised to put haemophiliacs exposed to vCJD at the end of the day for invasive hospital procedures keeping one set of instruments for those incubating or exposed with vCJD. It is much more difficult to kill a prion compared to HIV or hepatitis C so Grayson was concerned that her husband might be exposed to vCJD yet again if the same instrument was used on multiple persons with vCJD. Meikle went on to win a first prize…the General Medical Consumer News category at the prestigious Norwich Union Healthcare Medical Journalism Awards 2003 for his piece on CJD.
On the 6th September, 2004, Grayson wrote a long email to a journalist writing on v CJD, one passage detailed a recent incident that showed how little the government had prepared haemophiliacs and their families to deal with their exposure,
I do have real cause for concern, my husband recently had a burst vein resulting in blood spillage, and the advice from professional organizations on dealing with blood spillage because of concerns over vCJD in blood was to send two men in full bio hazard suits to our home to remove our carpet which was then sent to be disposed of at a special incinerator in Leeds. I photographed the whole operation and this appeared on a local BBC News feature. As a carer of a severe haemophiliac who sometimes bleeds copius amounts of blood, I have still not received guidelines on how to deal with a blood spillage in the community with regard to v CJD risk, yet there are guidelines for hospitals and guidelines on how to deal with HIV, hep C blood spillage in the community. This incident was referred by infection control at our local hospital to the CJD incident panel about a year ago. I am still waiting for guidelines for blood spillage in our community.
Through the early 2000s Grayson and Longstaff did many media articles on vCJD and Grayson established positive contact with the Liberal Democrats, in particular the late Patsy Calton MP. On 22nd September 2004, the Guardian published an article titled, Lib Dems call for v CJD blood inquiry, this was announced at their yearly conference, the paper stated,
The Lib Dems call for an inquiry into transfusions comes after it was announced that patients who received blood products from donors who subsequently died from vCJD were to be warned they could be carrying the disease.
The measures are being taken after it emerged last year that a patient had died after a transfusion from someone with the disease.
That was thought to be the first time vCJD has been transmitted from person to person.
The conference also heard concerns over the handling and treatment of donated blood, the failure to alert patients to known risks and the failure to take action when the risks become apparent.
Junior Lib Dem health spokeswoman Patsy Calton said all those affected by the contamination of blood products, including haemophiliacs put at risk of exposure to HIV and hepatitis C, as well as vCJD, were entitled to an inquiry.
“The families of the over 780 individuals who died as a result of the failing in blood products deserve answers,” she said.
“To provide closure to the victims and to ensure there are no further scandals in this area, the government should hold a full independent inquiry into the failures of the past.”
An early day motion was also circulated by the Libdems as follows:-
That this House notes the severe illness and deaths of haemophiliacs and some partners from HIV and hepatitis C, caused by the use of contaminated blood products, and the potential for future deaths from vCJD; and supports the Haemophilia Society in its call for an independent public inquiry into the contamination of haemophilia treatment since the 1970s, in particular the failure to make the UK self-sufficient in blood products, the failure in some parts of the UK to make a timely switch to virally inactivated treatment when this became available from 1985 and the failure to make timely provision of genetically-engineered recombinant Factor VIII and IX for all haemophiliacs.
Just 2 days later, 26th September 2004, the Guardian reported… CJD risk letters go to 6,000 patients. Haemophiliacs that had not already hounded their doctors like Grayson and Longstaff to find out if they had been exposed to the prion, received official letters asking them if they wished to know if they had been exposed or not and directions for how to proceed.
On the 13th October 2004, reports appeared in the press with regard to Grayson contacting the Food and Drug Administration (FDA) after she discovered an English and French case where Europeans had sold their blood in the US this compromising the safety of the US blood supply regarding vCJD. UPI, reported,
The Agency’s William Freas, who serves as Executive Secretary of the TSE Advisory Committee, confirmed he received Grayson’s remarks, but said it was up to Committee members whether her concerns would be discussed at the meeting.
They’re going to receive her concerns, but the individual members are independent from the FDA and only they can decide whether to discuss the issues raised by Grayson. Freas told UPI, he couldn’t say anymore due to FDA’s very tight restrictions on what we can say” before advisory committee meetings.
Grayson said in her letter she is concerned that if any of the foreign nationals who donated blood or plasma in the United States do subsequently develop vCJD, US patients who received the blood or blood products derived from their donation may never be notified,
In 2005, Recombinant was finally phased in for haemophiliacs of Mr Longstaff’s age group. Despite a sustained and exhausting effort, he never got his safe treatment and died in a hospice with his wife beside him not long after his 47th birthday. His last selfless act had been to organize for his brain and other tissue to be donated to the CJD Surveillance Unit at Edinburgh for research to be carried out by Professor Ironside in order that other haemophiliacs might benefit and increase their knowledge of vCJD.
Last week at the Infected Blood Inquiry, it was distressing to those infected and affected to hear v CJD being portrayed as an “accident”. This is far from reality. A series of choices led to to vCJD and a crossing of unethical and unnatural boundaries in the use of MBM feed. If this is not examined and accepted then how do we know something similar won’t happen again?
This week it is Professor Ironside giving evidence. He is remembered as being approachable, helpful, and willing to give his time. I hope those listening will hear some of what Phillips failed to deliver in 2000, only with truth and justice can haemophiliacs and their families begin to heal and finally find some peace.
800 HAEMOPHILIACS GIVEN TAINTED BLOOD AT RISK OF VCJD (Independent, 20th May, 2009)
Carol Anne Grayson is an independent writer/researcher on global health/human rights/WOT and is Executive Producer of the Oscar nominated, Incident in New Baghdad. She was a Registered Mental Nurse with a Masters in Gender Culture and Development. Carol was awarded the ESRC, Michael Young Prize for Research 2009, and the COTT ‘Action = Life’ Human Rights Award’ for “upholding truth and justice”. She is also a survivor of US “collateral damage”