Earlier this year, experts on variant Creutzfeldt-Jakob Disease (v CJD) gave evidence at the Infected Blood Inquiry in relation to haemophiliacs with an inherited bleeding disorder who were exposed to the prion disease through administration of UK factor concentrate treatment, injected into their veins to prevent and stop bleeding episodes. Haemophiliacs were infected with HIV and hepatitis viruses in the 1970 and 80s which is the main focus of this inquiry however v CJD is also being investigated.
The Inquiry heard from Professor John Collinge, Professor of Neurology and Head of the Department of Neurodegenerative Disease at the UCL Institute of Neurology who is also director of the UK Medical Research Council’s Prion Unit. In addition, Professor James Ironside, Emeritus Professor at the Centre for Clinical Brain Sciences at the University of Edinburgh who was made Commander of the British Empire (CBE) for Services to Medicine and Healthcare in 2006 also testified.
Back in May 2009, the Independent reported,
More than 800 people with haemophilia have received contaminated blood products putting them at heightened risk of developing vCJD, the Government has disclosed for the first time.
The figures were revealed in a parliamentary answer from Lord Darzi, a Health minister, following the death of a haemophiliac who had received infected blood products. A post-mortem examination on the man, who was in his seventies when he died last November, revealed traces of variant Creutzfeldt-Jakob disease (vCJD) in his spleen, although his death was due to an unrelated cause.
The development has increased the likelihood that haemophiliacs who received infected blood could develop the brain disease, Chris James, chief executive of the Haemophilia Society, said yesterday. The CJD Incidents Panel is to consider the implications of the finding at its meeting today.
“It now looks like there is a real possibility of a link between receiving blood products and developing vCJD. What was a theoretical risk is now a suspected causal link. That has raised the threat for other haemophiliacs who may have dismissed it,” Mr James said.
The complete article can be read on the following link:-
On the 3rd August 2021, The European Centre for Prevention and Control published its Risk Assessment, titled, The risk of variant Creutzfeld disease transmitted via blood and plasma- derived medicinal products manufactured from donations obtained in the United Kingdom which can be read here:
However, this author spotted an article in Science just days earlier titled, France issues moratorium on prion research after fatal brain disease strikes two lab workers (28th July 2021) which does not appear to have been considered in the publication. References to France in the European Centre report from page 8 onwards are focused specifically on blood donors and recipients as this example demonstrates,
In France, three patients that developed signs of vCJD in 2004 and 2005 were blood donors. A total of
42 recipients of blood components donated by these donors were identified, with 17 recipients still alive in 2006
. The most recent vCJD case with blood donation history was reported in Italy in 2016 (Figure 3).
What chilled this author was an introduction in Science regarding lab accidents where workers were exposed to CJD as follows,
PARIS—Five public research institutions in France have imposed a 3-month moratorium on the study of prions—a class of misfolding, infectious proteins that cause fatal brain diseases—after a retired lab worker who handled prions in the past was diagnosed with Creutzfeldt-Jakob disease (CJD), the most common prion disease in humans. An investigation is underway to find out whether the patient, who worked at a lab run by the National Research Institute for Agriculture, Food and Environment (INRAE), contracted the disease on the job.
If so, it would be the second such case in France in the past few years. In June 2019, an INRAE lab worker named Émilie Jaumain died at age 33, 10 years after pricking her thumb during an experiment with prion-infected mice. Her family is now suing INRAE for manslaughter and endangering life; her illness had already led to tightened safety measures at French prion labs.
What is disturbing is that lab worker Émilie Jaumain appears to have been infected during her daily activities and the implications for haemophiliacs. This further significant passage is likely to worry haemophiliacs,
In Jaumain’s case, there is little doubt she was infected on the job, according to a paper published in The New England Journal of Medicine (NEJM) in 2020. She had variant CJD (vCJD), a form typically caused by eating beef contaminated with bovine spongiform encephalopathy (BSE), or mad cow disease. But Europe’s BSE outbreak ended after 2000 and vCJD virtually disappeared; the chance that someone of Jaumain’s age in France would contract food-borne vCJD is “negligible or non-existent,” according to the paper.
It is noted that the skin was broken and drew blood. Two further passage state,
For Jaumain, who worked at INRAE’s Molecular Virology and Immunology Unit in Jouy-en-Josas, outside Paris, that long period of uncertainty began on 31 May 2010, when she stabbed her left thumb with a curved forceps while cleaning a cryostat—a machine that can cut tissues at very low temperatures—that she used to slice brain sections from transgenic mice infected with a sheep-adapted form of BSE. She pierced two layers of latex gloves and drew blood. “Émilie started worrying about the accident as soon as it had happened, and mentioned it to every doctor she saw,” says her widower, Armel Houel.
In November 2017, Jaumain developed a burning pain in her right shoulder and neck that worsened and spread to the right half of her body over the following 6 months, according to the NEJM paper. In January 2019, she became depressed and anxious, suffering memory impairment and hallucinations. “It was a descent into hell,” Houel says. She was diagnosed with “probable vCJD” in mid-March of that year and died 3 months later. A postmortem confirmed the diagnosis.
So to sum up, a lab worker had an accident at work and had direct exposure between infected material and a cut which drew blood. Haemophiliacs had the blood of multiple blood donors infected with the prion disease v CJD injected directly into their veins. Surely these 2 lab worker cases must raise concerns regarding infected material/blood entering the body and directly causing prion infection?
Here is the remainder of the article which surely has implications for haemophiliacs especially as the incubation period is much longer than was first realized and could be decades before infection becomes apparent. Why weren’t these French cases mentioned in the European report? Also in addition an Italian lab worker died of v CJD in 2016 as highlighted below.
INRAE only recently admitted the likely link between Jaumain’s illness and the accident. “We recognize, without ambiguity, the hypothesis of a correlation between Emilie Jaumain-Houel’s accident … and her infection with vCJD,” INRAE chair and CEO Philippe Mauguin wrote in a 24 June letter to an association created by friends and colleagues to publicize Jaumain’s case and lobby for improvements in lab safety. (Science has obtained a copy of the letter, which has not been made public.)
Jaumain’s family has filed both criminal charges and an administrative suit against INRAE, alleging a range of problems at Jaumain’s lab. She had not been trained in handling dangerous prions or responding to accidents and did not wear both metal mesh and surgical gloves, as she was supposed to, says Julien Bensimhon, the family’s lawyer. The thumb should have been soaked in a bleach solution immediately, which did not happen, Bensimhon adds.
Independent reports by a company specializing in occupational safety and by government inspectors have found no safety violations at the lab; one of them said there was a “strong culture” of risk management. (Bensimhon calls the reports “biased.”)
The government inspectors’ report concluded that Jaumain’s accident was not unique, however. There had been at least 17 accidents among the 100 or so scientists and technicians in France working with prions in the previous decade, five of whom stabbed or cut themselves with contaminated syringes or blades. Another technician at the same lab had a fingerprick accident with prions in 2005, but has not developed vCJD symptoms so far, Bensimhon says. “It is shocking that no precautionary measures were taken then to ensure such an accident never happened again,” he says.
In Italy, too, the last person to die of vCJD, in 2016, was a lab worker with exposure to prion-infected brain tissue, according to last year’s NEJM paper, although an investigation did not find evidence of a lab accident. That patient and the lab they worked at have not been identified.
After Jaumain’s diagnosis, “We contacted all the research prion labs in France to suggest they check their safety procedures and remind staff about the importance of respecting them,” says Stéphane Haïk, a neuroscientist at the Paris Brain Institute at Pitié-Salpêtrière Hospital who helped diagnose Jaumain and is the corresponding author on the paper. Many labs tightened procedures, according to the government inspectors’ report, for instance by introducing plastic scissors and scalpels, which are disposable and less sharp, and bite and cut-resistant gloves. A team of experts from the five research agencies is due to submit proposals for a guide to good practice in prion research to the French government at the end of this year.
The scientific community has long recognized that handling prions is dangerous and an occupational risk for neuropathologists, says neuropathologist Adriano Aguzzi of the University of Zurich. Aguzzi declined to comment on the French CJD cases, but told Science his lab never handles human or bovine prions for research purposes, only for diagnostics. “We conduct research only on mouse-adapted sheep prions, which have never been shown to be infectious to humans,” Aguzzi says. In a 2011 paper, his team reported that prions can spread through aerosols, at least in mice, which “may warrant re-thinking on prion biosafety guidelines in research and diagnostic laboratories,” they wrote. Aguzzi says he was “totally shocked” by the finding and introduced safety measures to prevent aerosol spread at his own lab, but the paper drew little attention elsewhere.
The moratorium will “obviously” cause delays in research, but given the very long incubation periods in prion diseases, the impact of a 3-month hiatus will be limited, Comoy says. His research team at CEA also works on other neurodegenerative diseases, including Alzheimer’s disease and Parkinson’s disease, and will shift some of its efforts to those.
Although Jaumain’s diagnosis upset many in the field, it hasn’t led to an exodus among researchers in France, Haïk says: “I know of only one person who resigned because they were so worried.”
Robert Herriman, a microbiologist who hosts Outbreak News can be viewed discussing the Émilie Jaumain case on the video link below,
The recent report on compensation for those who received Contaminated Blood, Compensation and redress for the victims of infected blood: recommendations for a framework published on the 7th June 2022 suggests that no compensation will be paid for exposure to v CJD and that anyone that develops symptoms will then have to access the government CJD fund. As yet however there is no test available for haemophiliacs who must live with the daily fear that they might develop the prion disease at any time. The recent lab infection cases suggest CJD might be more of a risk than haemophiliacs are currently being told… and lets face it, they have been there before with the playing down of hepatitis risks!
This is also of great concern to the partners and carers who looked after haemophiliacs and may also have been exposed to their blood. For years, government and local health authorities failed to provide safety guidelines for those in the community despite strict safety guidelines being applied in hospitals to protect staff in the work environment. Prions are much harder to kill than the HIV or hepatitis viruses and conflicting advice was given on what to do with bloody laundry if at all as this author discovered and highlighted in an earlier blog.
Once again this flags up haemophiliacs as a unique patient group due to the multiple viral infections they were given through their treatment including HIV and hepatitis viruses and exposure to prions, the fact that haemophiliacs were re infected through the use of large plasma pools (up to 400,000 donors with US plasma pools) and the infection of multiple family members that had haemophilia plus partners and children were sometimes infected also. This is a very different scenario to that of other individual patients without an inherited bleeding disorder.
Government must now recognize the psychological distress ALREADY caused to haemophiliacs with v CJD exposure on which this author has written earlier and pay compensation for over 2 decades of unresolved fear that exposure to v CJD may at some point lead to incubation and having to deal with the many symptoms of an horrific prion disease. Yes if they develop the disease they can apply to the CJD Trust and government must address now the additional stress that haemophiliacs are NOW living with, without relief and no test yet to change that position.
There is one case of a payment being made for psychological harm in the case of a doctor with a needle stick injury and fear of HIV exposure. However in her case she received compensation for stress despite the fact that in her case, there was a test for HIV, she was found not to be infected and could gain relief and return to work, even if not the same work. The case can be read here,
£465,000 Compensation Payout For A Syringe Needle-Stick Injury / Infection? – Case Study
So where does that leave current blood safety with regards to vCJD. Government have recently revised guidelines re v CJD and blood. Can we be certain though that the experts have factored in all the relevant data and safety information or is our blood supply again at risk?
Here is the latest update from government,
Research and analysis
Creutzfeldt-Jakob disease (CJD) update (data to end of December 2021)
Updated 21 June 2022
It is important to continue to ask questions regarding blood safety, experience has warned us government made many mistakes on blood safety in the past and we can not assume lessons have always been learnt in the present or for the future.
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”