Variant CJD and Surgery

Paul Beresford Excerpts

Tuesday 29th November 2016 Commons Chamber

[Sir Paul Beresford (Mole Valley) (Con)]

I am delighted to see the Minister in her place, and I am sure that she is delighted to be here—or at least she is trying to smile under the circumstances. She has probably been made aware of my long-term interest in, and deep concern about, this subject. I am sorry to inflict it on her this evening, but she is bearing up. I also declare a potential interest, as a very part-time dentist.

Variant CJD is a fatal neurodegenerative disease originating from exposure to bovine spongiform encephalopathy-like prions, prions being small particles of protein. Variant CJD prion infections are associated with a very long and clinically silent incubation, but when the disease strikes, it causes a fast, spongy degeneration of the brain, followed by a horrible and untimely death. It is probable, but not certain, that carriers might not produce the disease themselves, but it appears to have a potentially decades-long incubation. The long incubation period means that some will die of other causes first, but, as we live longer, we cannot be certain that in time —after decades—the disease might not strike all carriers, if they survive long enough. Carriers might also unwittingly pass on the prion through blood transfusion and via surgical instruments.

Variant CJD is an appalling disease with no cure. The number of asymptomatic individuals with variant CJD prion infection is unknown, but recent research estimates that the carriers number about one in 2,000 adults, which is a staggering number. The disease poses a risk to others, via blood transfusions, blood products, organ or tissue grafts and contaminated medical or dental instruments. The response of this and previous Governments has been bipolar. To give an exaggerated simplification, the first position of this bipolar response is that as we have not had many recent cases, there is no problem—but considering the long incubation period and some recent changes, this is a dangerous assumption. The second position is that there might be a problem so we should apply the precautionary principle in some areas. We cannot have both. I believe that waiting and an occasional application of the precautionary principle really do not hit the problem. If the Minister takes no action, I hope she will recognise that the absence of evidence is not evidence of absence.

As I have said, research says that one person in 2,000 is a carrier. The incubation period may well be decades, and some individuals appear to be more susceptible and some less so, although in time this could be proven wrong. A death from variant CJD in Edinburgh in January this year showed a potentially deeply worrying change. People are of various genotypes: they can be VV homozygotic, or MM homozygotic or MV homozygotic—and for the sake of Mr Deputy Speaker, I will not explain that. Until this case of the Edinburgh patient, all cases of variant CJD had been MM. The Edinburgh patient was the first MV patient that we have seen. It was thought that being MV or VV might offer some resistance, but this does not seem to be the case. We should bear it in mind that about 45% of the population are MV.

There is still no conclusive evidence, but there is a possibility that patients with the MV genotype may have a longer incubation period, which could lead to a second wave of variant CJD. The real point is that until recently it was hoped that MV patients might not show clinical signs, but in these early days this appears to have been put in deep doubt.

Research also shows that prions are transmissible by blood products and contaminated surgical instruments, and as the prions resist decontamination from stainless steel, we have a problem. Over the years, a precautionary principle has been applied—it is still being applied, but only partially. Much has been done slowly over many years. Leucodepletion was introduced, and synthesised clotting factors have been provided for haemophiliacs. A prion unit was set up at Queen Square. Single-patient use of stainless steel endodontic reamers was made mandatory, which I find quite interesting and will return to in a few moments. Non-UK blood supplies were sourced for those born after 1 January 1996.

What I found curious about the endodontic reamers is that if a patient requires endodontics, it is possible to use the stainless steel reamer but singly; but if the patient for some reason does not have endodontics, the tooth will have to be extracted using a stainless steel instrument that is used repeatedly, called a pair of forceps.

Very early on the Government established, through Medical Research Council funding, a prion unit at Queen Square under Professor Collinge. This unit was tasked with finding a test, finding ways of stopping or reducing transmission and hopefully even finding a cure. The prion unit with DuPont has produced a RelyOn soak, which deactivates the prion on stainless steel surgical instruments. Following the soak, there is then decontamination and a washing machine—a dishwasher-type machine—and then a full-blown steriliser, particularly a vacuum-based one. These instruments will bring about total sterilisation, from which the prion will be lost.

DuPont is no longer producing the soak, because there is no market. And there is no market simply because hospitals, clinics and surgeries in this country are not required to use it; if they were, there would be a market. That is quite extraordinary considering that this country has the greatest deposit, if I may use that term, of people carrying the prion.

In a surgery washer, the disinfectant would do the job. Recently, Professor Collinge became aware that the Department of Health had announced funds for research into prion-disinfecting stainless steel instruments. I believe the prion unit has applied and will hopefully get a grant. The problem with the wash was that it meant an extra stage, which slowed everything down in the hospital, but if DuPont or another manufacturer could produce it in the form of a tablet, a powder or a liquid that would go into the dishwasher without frothing, that step would be taken away, we would get rid of the prion and there would be no time wasted. Those instruments would be prion-free.

Incidentally, the Minister may be aware that there is some evidence that a protein may—and I stress the word “may”—be responsible for the occasional transmission of Alzheimer’s disease. If she wants a little bit of help on moving with RelyOn, I can tell her that RelyOn would disinfect instruments with this protein as well.

Another major failure relates to the sourcing of blood products. People born after 1 January 1996 who needed blood products—for instance, a transfusion—could get non-UK-sourced plasma that was almost certainly prion-free. Those born before that date would get UK plasma, and would have to pray earnestly that the donor was not the one in 2,000. As a parent, I can imagine having two children born on either side of that date. If for some horrible reason they both needed blood transfusions, one child would get the prion-free plasma and the other would take the risk, as would elderly people like us.

With a test, we could be fairly sure of excluding that one in 2,000. Professor Collinge and his prion unit team have developed such a test. They tried it out in this country and subsequently went to the United States, where they checked it with an extensive research programme to make sure that it produced no false positives. They were successful. They then returned to this country. The final stage of the research needs to be tested on a large batch of anonymised UK blood samples, but the Medical Research Council will not fund it. At least, that is the case so far.

If we had that test, blood donors who were carriers would be sorted out and their blood not used, and special measures could be taken for surgery patients who proved to be carriers. In respect of the latter line, the Minister’s Department introduced new guidance in July this year. I understand that it requires separate instruments to be used on high-risk tissues in the case of patients born before and after 1 January 1997 respectively. That is sensible reasoning, because it is thought that people born since 1 January 1997—I thought that it was 1996—have had less exposure to prions via the food chain. Those people form a group who are at lower risk of prion diseases, and thus less likely to contaminate surgical instruments with prions.

The instruction from the National Institute for Health and Clinical Excellence on a risk-reduction strategy requires every hospital and clinic to have separate pools of instruments to be used for high-risk surgery. It distinguishes between patients who were born before 1 January 1997 and those who were born on or after that date. The instruments must be kept separately, and notated. Although I consider that instruction to be eminently sensible, it will add greatly to the costs to hospitals of instrument provision, storage, and the required regular re-sterilisation. Tracing and tracking of instruments has also proved costly, and some hospitals are etching all instruments with identification numbers to ensure that they can carry out the process properly.

I have only been able to obtain one figure, but I understand that since, I think, July, observing the new guidance has cost the National Hospital for Neurology and Neurosurgery in Queen Square an extra £120,000. A little further down the road, the cost to a hospital specialising in children will be considerably higher. If RelyOn were developed so that it could be used, that difficulty would be removed.

I have three small asks of the Minister. First, we must recognise that all patients need to be treated equally in respect of blood products. As one person in 2,000 is thought to be a carrier, until we have a variant CJD test everyone should receive non-UK plasma. Secondly, rather than chasing a new product for sterilisation, the Department of Health, through whatever means, should fund the manufacturer of RelyOn to produce it in a more user-friendly form. If NICE or the Care Quality Commission made the use of such a product mandatory, there would be a market potential, which might be sufficient to persuade DuPont or some other manufacturer to produce such a user-friendly product without the need for funding, because it would be sold and used every time sterilisation pouches went through the dishwasher. Thirdly, funding the last stage of the testing of the prion unit system for prion detection would enable carriers to be taken out of the blood transfusion pool, and would also ensure a more sensible separation of surgical instruments. The cost savings would be vast.

[Jim Shannon (Strangford) (DUP)]

I congratulate the hon. Gentleman on making such a compelling case for those with CJD. In 2001, the Government set some money aside for a compensation scheme for UK victims of variant CJD. A trust fund was set up in April 2001 and compensation payments of £25,000 were made to the most affected families. Does the hon. Gentleman feel the Government should reconsider the compensation scheme and upgrade it for 2016 for those who, clearly from what he says, will probably fall into that category—although I hope not—in years to come?

[Sir Paul Beresford]

The hon. Gentleman makes a good point, but what I would really like to do is get the Government to take some action that is sitting, waiting, readily available to prevent it; otherwise, in time to come I believe we are going to have a chance of a considerable flood of variant CJD disease, but we do not know, and if this test was there we would know if the figure of one in 2,000 is right or wrong, or if we can separate patients out, so that those who have it have special instruments and the rest of us are all right, and we can also start using blood products in this country, because we will only be using products that do not have the prion on board.

In effect, the Minister needs to think about this: I do not want my grandchildren to be the generation that sees the re-emergence of variant CJD and for them to turn to me, if I am still around, and say, “Why didn’t we do something about it?” That is not a very big ask.

[The Parliamentary Under-Secretary of State for Health (Nicola Blackwood)]

I congratulate my hon. Friend the Member for Mole Valley (Sir Paul Beresford) on securing this important debate on variant CJD and surgery. It is clearly an area on which he has a great deal of knowledge. I recognise that prion disease is the causative agent of transmissible spongiform encephalopathies such as variant CJD. It remains in many ways obscure and there are many aspects of these rare diseases that we are still in the early stages of researching. However, one thing I am confident about is that the UK system for ascertaining CJD case numbers has been for the past 20 years reliable and accurate. Our national CJD research and surveillance unit, which is based in Edinburgh and funded by the Department and the Scottish Government, leads pan-European work and has leadership from expert clinicians and scientists who, in 1996 following BSE, were the first to identify variant CJD as a separate form of the condition.

The Department recognises the potential seriousness of secondary—person-to-person—transmissions of vCJD and has since the late 1990s introduced a series of measures to reduce the risk of such spread, whether by blood transmission or by surgery. We are reassured that there have to date been no cases attributable to surgical transmission and only three cases of clinical disease attributable to blood transfusions, all of which occurred in or before 1999. However, our risk assessment models, which we use in our impact assessments of potential risk reduction measures, continue to take into account the potential for secondary—person-to-person—transmissions by both routes, in people with all genotypes and over potentially very long incubation periods, which my hon. Friend mentioned. This is why the scientific advice we have is that the surgical instrument measures in place are sufficient irrespective of the genotype.

My hon. Friend was right to mention the recent case. It was always anticipated on the basis of a wide body of published scientific work that, following the BSE epidemic, further cases of vCJD, including MV cases, might arise from time to time. We have seen that with similar diseases, such as kuru in Papua New Guinea, and studies suggest MV cases could be seen in small numbers for more than 30 years after exposure. Having reviewed the information about the case of vCJD in a patient with MV genotype, the Advisory Committee on Dangerous Pathogens has advised that no changes in the current risk reduction measures are needed at present. It advises that the measures in place are sufficient, irrespective of genotype, although of course the matter will remain under review.

It is important to stress that modern surgical equipment in the UK is very safe and that robust guidance is in place for the NHS on procedures and practices to reduce the risk of contamination of any kind, including the use of single-use instruments where possible and of decontamination practices. Where it is not possible to use single-use instruments in higher-risk procedures, there are processes in place to track the use of specialist equipment. As my hon. Friend will know far better than I do, there is a potential risk of vCJD transmission via dental surgery, and this has been recognised by the UK’s chief dental officers. In 2007, they issued letters to all dentists to advise that endodontic root canal reamers and files should be used as single-patient or single-use instruments.

[Sir Paul Beresford]

I am a little worried that the Minister appears to accept that surgical procedures are as good as they can be, given that the Department is inviting research to find a RelyOn equivalent, to improve the situation. The Department must therefore see a flaw in what we have at present.

[Nicola Blackwood]

My hon. Friend anticipates my next words as only an experienced Member of Parliament can do. I think it is right to say that there has so far been no evidence of any secondary, person-to-person, vCJD transmissions via surgery or dentistry. Nevertheless, we are maintaining and updating our precautionary approach. Surgical instruments guidance has recently been refreshed to support health organisations in delivering the required standard of decontamination of surgical instruments and to build on existing good practice to ensure that high standards of infection prevention and control are developed and maintained. My hon. Friend mentioned a number of these points.

The major change in this latest revision takes account of recent changes to the Advisory Committee on Dangerous Pathogens transmissible spongiform encephalopathy subgroup’s general principles of decontamination. This establishes a move towards in-situ testing for residual proteins on instruments. Residual protein is important because of the potential risk of the transmission of prions, though vCJD has not been shown to have been transmitted person-to-person in this way. The guidance provides information on how sterile services departments can mitigate the patient safety risk from residual protein with the objective of reductions in protein contamination levels through the optimisation of decontamination processes.

The ambition is that all healthcare providers engaged in the management and decontamination of surgical instruments used in acute care will have implemented this guidance by 1 July 2018. However, providers whose instruments are likely to come into contact with higher-risk tissues—for example, neurological tissue—are expected to give the guidance higher priority and to move to in-situ protein detection methodologies by 1 July 2017.

The chief medical officer has also recently written to NICE supporting the need to update its guidance on patient safety and the reduction of risk of transmission of CJD via interventional procedures, to ensure that it is fit for purpose, appropriately targeted, and can command the confidence of those who use it. We would expect this to take account of available evidence, including decontamination methods that are safe and effective against human prions; the epidemiology of CJD, including data on the prevalence of vCJD infectivity in the UK population from the appendix prevalence studies; and the availability and performance of single-use instruments in high-risk specialties. We would also expect the guidance review to be considered in the context of the latest research on prevalence, particularly for those born after 1996, who are currently considered unlikely to have been exposed to the BSE agent.

My hon. Friend is right, however, to say that adopting the precautionary principle alone is not sufficient. That is why successive UK Governments have been supportive of the development of new measures that might help in vCJD risk reduction. The Department of Health has provided over £70 million for CJD-related research in the last 15 years. That research has focused on infectivity, pathogenesis and transmission risk; decontamination of surgical instruments and the development of more sensitive methods to detect residual proteins to improve instrument cleaning; test development, treatment and diagnosis; and surveillance, screening, epidemiology and case finding.

[Sir Paul Beresford]

I accept everything that my hon. Friend says. However, a test solution is on the market and waiting to come through. The prion unit test has reached the point at which it just needs a final run to ensure that it does come through. I hope that I can count on the Minister to back me in persuading the MRC to support that last round of testing. If we could test blood, we would not have to import blood products from overseas. We could separate out the one in 2,000 or whatever the figure is and cut down on the costs of instrument storage.

[Nicola Blackwood]

That is one reason why the Department has continued in difficult financial times to ring-fence £5.5 million a year for CJD-related research. We are keen to see safe, evidence-based, cost-effective measures to reduce the risk of vCJD. At the moment, however, there is no validated diagnostic blood test that can be used before the onset of CJD symptoms to diagnose whether someone is infected or incubating the disease. We will of course take advice from the ACDP and the Advisory Committee on the Safety of Blood, Tissues and Organs on the use of any potential test in any proposed Department of Health-funded research study or deployment by UK blood services, but there are established systems for applying for research funds. We have put such funds out there, and any applications for those funds must go through the standard processes. To do otherwise would be to undermine the reputation for research excellence that the UK scientific community has fought hard to establish.

To that end, we recently launched an open competition, inviting proposals for research to further inform our risk-management and health-protection measures, including our understanding of vCJD infection in the UK population, the development of a test able to detect pre-clinical levels of infection in blood, and the development of decontamination technologies for reusable medical instruments. I understand that Professor Collinge’s RelyOn is one application that is currently going though that process, so it would be inappropriate for me to intervene.

I assure the House that the Department recognises the fatal consequences of all forms of clinical CJD and the devastating cost to individual patients, their families and carers, which my hon. Friend described movingly. That is why we set up the vCJD Trust in 2001 in recognition of their wholly exceptional situation and the fact that the Government are their last resort for help. The trust provides a no-fault compensation scheme for vCJD patients and their families, providing payments to be made in respect of 250 cases from a trust fund of £67.5 million. Over £41 million has been paid out by the trust to date.