HOUSE OF COMMONS
OFFICIAL REPORT
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 who 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 for 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 the 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 caradiomyopathy, 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-1970’s 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
objects 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 the
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 conditions 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 forms of 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 also 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 underestimation.
I take seriously the hon. Gentleman’s 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'’ 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.
Department 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 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.
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