HC Deb 21 June 2001 vol 370 cc273-80

Motion made, and Question proposed, That this House do now adjourn —[Mr. Kemp.]

7 pm

Dr. Julian Lewis (New Forest, East)

It is with some trepidation that I rise to address the House on the question of cardiac risk in the young, or sudden death syndrome. I say "trepidation" because it is a subject on which I am by no means an expert. Therefore, I am grateful to see that a number of colleagues are nevertheless remaining for the debate.

I wish to do justice to a fine young man whom I am proud to have as one of my constituents. His name is Adrian Woodhead. Adrian was married to Sarah and they were together for 10 years until, in 1997, just before Christmas, Sarah, who at the age of 28 had never been ill, had been very fit and was a non-smoker, suffered a massive heart seizure and died.

Adrian and Sarah had been hoping to start a family soon. He has movingly written to me about his feelings after that tragedy: I expected the house to be full of noise and life, as you get from children, not silent through death and loneliness … There is no structure to my life except for my work and my efforts to ensure that someone else might not have to go through this … I can do things for other people, but I can do nothing for myself. Indeed, Adrian does a great deal for other people. At the time of the tragedy, he was an assembly line worker at Ford's. Since then, with great grit and determination, he has retrained as a police constable. When he passed out, he was awarded a baton of honour. He is a passionate exponent of the need to warn people about sudden death syndrome and its risk for the young These are the three areas that Adrian has drawn to my attention: the need to raise awareness, the need for screening and the need for research. I shall address those in turn.

On the need to raise awareness, it is estimated that at least four young people in the United Kingdom die every week from sudden death syndrome; that is more than 200 tragedies every year. There is a great need to raise awareness among the public, so that, in those cases where symptoms begin to show themselves, they will be not lightly dismissed but investigated.

There is a need to raise awareness among general practitioners, so that, when young people go to see GPs with telling but not very severe warning symptoms, the GPs will act perhaps a bit more promptly than they do at present. In particular, GPs should be inquiring more whether other members of the family of the young person concerned have died young from heart failure, heart seizure or some other aspect of sudden death syndrome. If there is some history of that in tile family, the young people should be screened.

There is a need for greater awareness on the part of coroners. All too often, when tragedies happen and young people die unexpectedly, the cause of death is given as accidental, natural causes or even, incorrectly, epilepsy.

The great problem with sudden death syndrome—cardiac risk in the young, or whatever one chooses to call it—is that one does not usually see it coming, and when it happens it is too late. Screening is common policy for cervical smears and for breast tests. With such conditions people can see the disease coming and screening is accepted as a technique. It is a fact that an ordinary electrocardiogram will pick up the majority of the conditions constituting sudden death syndrome.

After the detection of such a condition, various prospects open up. There is the prospect of remedial action by implant, the prospect of remedial action by drugs, or the prospect of remedial action by changing one's life style. However, even in cases in which nothing is going to work and a fatal outcome cannot be averted, at least those who are subject to the genes responsible for those conditions can prepare themselves and their families for what will come, in the same way that families can prepare themselves when a child has a known terminal illness. People can do things now rather than postpone them, and they can choose what to do about whether to pass on the gene by having children.

Sudden death syndrome is a composite term for 10 main conditions. Hypertrophic cardiomyopathy, or HCM, accounts for about 30 per cent. of the cases; arrythmogenic right ventricular cardiomyopathy, or ARVC, accounts for about 20 per cent. of cases; and long QT syndrome accounts for another 10 per cent. Of those three categories, which account for 60 per cent. of the cases generically known as SDS, four fifths would be detected by ordinary electrocardiogram screening. If they were caught, the very least action that would be taken would be an echocardiogram—known as an ECHO—which is an ultrasound of the heart.

More than half of the 200-plus young people who die so tragically every year could be saved by medical intervention and change of life style, particularly because so many of those who die are very active. They are particularly likely to be engaged in serious and energetic sport, and sometimes they are engaged in professional sport. I recognise that we are talking about very serious conditions and that between one quarter and one third of those who are vulnerable will die regardless. In other words, between one quarter and one third of the 200-plus people who die each year from the conditions will probably suffer the same outcome regardless of whether their condition has been diagnosed in advance. Nevertheless, as I said, it is vital that people know what they have to contend with.

In professional tennis there is something called the Karen Krantzke sportsmanship award. It is given in memory of Karen Krantzke, an Australian doubles player who died at about age 30, in the mid-1970s, at the height of her powers. A friend of hers was Mrs. Alison Cox, who is the wife of Mark Cox—who as we all know was the No. 1 British tennis player in the 1970s.

In 1992, Mark and Alison's son Steven went to college in America on a sports scholarship. A few years earlier, a young man had died at that college from an SDS condition. Fearing future litigation, the college initiated a programme of automatic screening for young people on its intensive sports programmes. Consequently, it was discovered that Steven Cox, who was himself on the verge of a glittering tennis career, was suffering potentially from ARVC. Following that discovery and his decision to change his life style and forgo the professional tennis career that would otherwise have undoubtedly been his, he is still healthy at the age of 27, and it may well be that he owes his life to that screening in America.

It occurred to Alison Cox that not much had happened between the tragedy of Karen Krantzke in the mid-1970s and her own son's narrow escape in 1992, so she set up Cardiac Risk in the Young, which aims to raise awareness and to exert pressure for more screening, in the hope that conditions here will begin to match those in other parts of the world. For example, in this country there are only 17 implants per 1 million citizens. In Germany, there are more than 60, and in America more than 200.

The aim of CRY is to identify, screen and treat those most at risk: first, those in whose family there has been a death at a young age, and secondly, those who are engaged in serious sport. Its ultimate aim is the routine screening of those who participate in any serious sport, as is the case in Italy. So far, CRY's pilot programmes to test the effectiveness of ECG machines in detecting hidden heart conditions in young people have led to the placing of 26 machines in local communities, worth a total of £130,000. The organisation has also funded a £120,000 ECHO machine, which is needed for heart ultrasound—the vital next step after an ECG has detected the possibility of an SDS defect in a young person.

Section 64 of the Health Services and Public Health Act 1968 gives the Secretary of State power to make discretionary grants to voluntary organisations in England working in support of health objectives of which the Government approve. CRY has tried five times to get a grant for its important work, and I wonder whether the Minister will be able to give us any encouragement and lead us to hope that a future application will be more successful.

Adrian's third point concerned the need for the research. I have referred briefly to implants. Internal cardiac defibrillators, or ICDs, kick-start a heart that has gone into seizure and regulate it after the attack. Five years ago, they were the size of half a brick; now they are the size of a matchbox. Five years ago, they required major surgery; now they can be implanted under local anaesthetic. Five years ago, they had to be sited deep in the stomach; now they are sited very near the surface, just below the collar bone, so that they can easily be tweaked, adjusted and serviced as required.

Drugs to thin the blood and prevent clotting are another form of medical intervention. Is the Minister satisfied that enough is being done to increase the number of implants that are being supplied, which would have to be tripled to match the number in Germany, and that enough research is being done on drugs to alleviate the dangers?

We must also consider genetic research. One of the objections to screening is the expense of ECGs and ECHO tests. I would be grateful if the Minister would indicate whether progress is being made on identifying a simple blood test for the gene that causes those syndromes. Does she accept that if that could be done, the expense argument against screening would fall? If so, would some of her objections to screening be overcome?

I am coming to the end of my remarks, but refer to a letter that the Minister wrote to Earl Howe on 8 May. It is clearly designed to be helpful, but at one point it states that studies show that screening does not identify all those affected"— by SDS— and also that there is little evidence at present that treatment before the onset of symptoms alters the course of the disease. I feel that the Minister has been supplied with information that may be a little out of date. As we have seen, implants can make a considerable difference to significant numbers of people surviving an attack of that sort. It is true that sometimes there are symptoms before a potentially fatal attack but, all too often, they are not recognised, either by the potential victim or by his or her GP.

There remains the question about the ethics of screening and whether or not it is a good thing. In the country as a whole, 10,000 to 15,000 people may be at risk, whereas "only" 200-plus per year will suffer the fatal outcome. There remains the ethical question whether it is right to worry the large number to save some of the small number. I believe that the evidence is in the affirmative. Alison Cox told me that of the hundreds of people affected by cardiac risk with whom she has dealt, only one mother said that she wished she had not known that her child was vulnerable. I am glad to say that, so far, her child has not suffered an attack; long may that continue.

The final word on the ethics of screening should go to Adrian Woodhead, the young man to whom I referred at the beginning of my speech. He told me: I'd have loved to have had a couple of children with Sarah: even if I'd known they'd have been with me for a limited time. It doesn't matter what condition you have—you just deal with it. But to deal with it, you've got to know. On Adrian's behalf and on behalf of all the young people who are vulnerable to the condition, I hope that the Minister will have something positive to say this evening.

7.18 pm
The Parliamentary Under-Secretary of State for Health (Yvette Cooper)

I congratulate the hon. Member for New Forest, East (Dr. Lewis) on securing an early Adjournment debate in the new parliamentary Session and on choosing to bring these important issues to the attention of the House. He spoke movingly about the plight of his constituent and his constituent's family. This is an area that does not always get the attention that it deserves, but one that can be devastating for families when an apparently fit and healthy young person suddenly dies without warning. In an age when we have come to associate death with old age, rather than youth, young people being struck down before their potential is fulfilled, without the time for people to adjust and say goodbye, can be almost too much to bear for grieving families, including parents who lose loved children and young people.

I pay tribute to the dedicated work of Cardiac Risk in the Young and, indeed, other voluntary organisations that provide a counselling network to help families who suffer such a loss. Support from people with real experience of the condition can certainly be a remarkable comfort to those who live on.

CRY and other organisations with an interest in the conditions have made a plea for greater awareness of risk factors among health professionals. The hon. Gentleman mentioned the need to increase awareness and to provide more information. I agree wholeheartedly with the importance of doing so and I have asked officials to investigate the potential for using the new media that are now available to us for communicating with health professionals and members of the public— including, for example, NHS Online and the electronic library for health—to provide updated information about risk factors.

Unfortunately, data on the true incidence of cardiomyopathy are hard to come by. Some studies put the incidence of hypertrophic cardiomyopathy at one person in 500. That is one of the problems that we encounter when we assess a proper policy response to the condition. Too little is known in many areas. As the hon. Gentleman rightly pointed out, little is known about the number of deaths caused by cardiomyopathies. The Office for National Statistics recorded 35 deaths due to sudden adult death syndrome in 1999, but this is almost certainly an underestimate. I take seriously his point about the problems with the coroner system and the inability to make a full inspection of the heart tissue in a post mortem following a sudden adult death. He may be aware that the Home Office has announced a full review of the antiquated coroner system, which includes public health specifically in its terms of reference. I shall ensure that the points raised this evening are passed on to that review.

Research is being carried out at the Imperial college school of medicine under Professor David Woods into the incidence of sudden adult death syndrome, and I believe that the research will be published later this year. We look forward to its conclusions to enlighten the policy process further.

The hon. Gentleman mentioned a national screening programme of young people to identify those with cardiomyopathy and those who are at increased risk of sudden death, and CRY has also done a lot of work on that issue. The national screening committee has looked at those proposals very carefully. For a screening programme to be practicable, there needs to be a clear means of defining cases of the disease and the ability to make a prognosis on the basis of that definition. In addition, there needs to be good evidence that early intervention can improve the prognosis. In other words, we need evidence that a national screening programme would make a real difference to health outcomes rather than simply provide information that might have no impact on those outcomes.

In 1999, the Department's national screening committee commissioned Dr. Stuart Logan of the Institute of Child Health to examine the feasibility of population screening for hypertrophic cardiomyopathy. His recommendation was that population screening would not meet those key criteria. He argued that the most commonly used case definition—left ventricular hypertrophy on echocardiography—would miss some people at risk and would identify significant numbers of people whose life span is likely to be no different from that of the general population. He also concluded that studies have suggested that starting treatment before the onset of symptoms—the point that the hon. Gentleman quoted from my letter—makes no difference to the course of the disease. In other words, no clear benefit would accrue to those told as a result of screening that they had a particular condition. Dr. Logan' s conclusions were accepted and endorsed by the national screening committee.

Similarly, the role of genetic screening for hypertrophic cardiomyopathy remains to be determined. The hon. Gentleman mentioned genetic screening, which has huge potential in many areas and its development may take us in many directions. I understand, however, that in an editorial in the July edition of the journal Heart, which will be published next month, the leading expert in the field, Dr. Michael Vincent of the university of Utah, offers a conclusion on the present evidence base with regard to genetic testing for these conditions. The article states: Knowing the diagnosis, genotype or mutation type does not clearly influence treatment options or outcomes. Thus, there seems to be no compelling reason to perform genotyping for these clinical groups. In other words, on the basis of what we know at this stage, Dr. Vincent concludes that information from gene testing would not be of practical use, given the limitations of our knowledge about how to improve outcomes.

I take very seriously what the hon. Gentleman said about not simply assuming that it is better for people not to know about their condition. It is wrong to make such judgments on behalf of other people. We must accept that giving people information about a condition that we may not be able to improve or for which we cannot offer alternative treatments or life styles can have psychological consequences. However. I agree that we cannot make presumptions about what people should know about their own health. My experience is that people increasingly want more information about their health and about the options open to them. They at least want the chance to decide for themselves what happens.

We must also bear in mind the evidence that exists about whether there is any positive benefit that should be taken into account. The 1999 study showed that there was insufficient evidence that the sort of diagnosis that would flow from a screening programme would be of benefit, but that is not to say that we should accept the findings of that study and do nothing to alter the status quo.

The hon. Gentleman asked whether implantable cardioverter defibrillators would take us further and change the position, and I shall ask the national screening committee to advise me on that. The National Institute for Clinical Excellence issued guidance in September 2000 on the use of such defibrillators. Implementation of that guidance in the NHS is expected to cause implantation rates to rise from the present 17 per 1 million people to around 50 per 1 million. Additional resources have been made available through health authority allocations to support the implementation of all the NICE recommendations.

Whether that will change the position in terms of screening depends on whether there is evidence that outcomes showed an improvement for people diagnosed with the condition at an early stage. We simply do not have evidence about how many casualties of sudden death syndrome would have benefited from ICDs, nor about how many people who might be diagnosed as a result of a screening process would also benefit from the implants. We need to know the answers to those critical questions to decide whether a screening programme was a good idea.

We also need more information about appropriate ways to treat the condition in general. The Cardiomyopathy Association, working with Professor William McKenna of St. George's hospital medical school, has been developing guidelines on the diagnosis and management of hypertrophic cardiomyopathy. We await those guidelines with interest, and will consider how best to disseminate them when they are published.

I shall ask the national screening committee to consider whether there has been sufficient development—in terms of evidence and new treatment possibilities—for it to update the 1999 review. I will write to the hon. Gentleman on that subject, as I consider it important for us to keep an open mind and to review matters as research uncovers further evidence.

Finally, I agree with the hon. Gentleman that research is the way forward. He asked about section 64 funding for CRY. Departmental officials will meet CRY later this summer, and I hope that they will be able to discuss the priorities that should be taken into consideration when section 64 funding is awarded. The hon. Gentleman will know that that the contest for that funding is sharp every year.

I thank the hon. Gentleman for raising these important matters, which are extremely serious for the families involved and especially for the constituent to whom he has referred this evening. I shall write to him with more information.

Question put and agreed to.

Adjourned accordingly at half-past Seven o'clock.