A new chapter

Dari Taylor MP welcomes the government’s decision to include recommendations from her Cardiac Risk in the Young private member’s bill kin the National Service Framework

This Bill was informed by the research of cardiac specialists who have worked extensively on diseases such as hypertrophic cardiomyopathies and ion channelopathies, supported by an academic physiologist from the English Institute of Sport.  With this background, the bill is set out to challenge medical opinion that dismissed the fact, or undermined the belief, that fit young people can be victims of heart diseases that have the potential to be fatal.  Additionally, it aimed to use available research to prove the efficacy of certain treatments for diseases that cause cardiac risk in the young. 

My bill highlighted that symptoms occurring in all people without cause for concern can in some be a warning that a serious heart condition exists.  That, for example, when breathlessness disproportionate to activity or blackouts or dizziness occur in fit young people these symptoms should not be dismissed as something that will pass but should be taken seriously.  In these cases, screening should be considered.  If symptoms persist when a young person is engaged in a physical activity, then screening should be instantly offered to diagnose the cause.  From the research produced by cardiac specialists who work in this field of medicine there is clear evidence that early availability of screening achieves an accurate diagnosis of the problem and supports the establishment of a treatment that will reduce the risk of cardiac failure. 

My bill asks that this research-based knowledge is accepted and utilised to inform the medical procedures used to diagnose and treat young people whose symptoms are confirmed in the diseases outlined, that is, become “best medical practice.” 

I have referenced the two prime diseases that can cause sudden death in fit young people.  Added to these are arrythmogenic right ventricular cardiomyopathy, dilated cardiomyopathy, Long QT syndrome (Marfan’s and Wolfe Parkinson-White syndrome).  All in their different ways present a thickening of the wall muscle of the heart and an irregular heart beat.  All are acknowledged as difficult to diagnose and that is why I have emphasised the need for a relevant cardiac specialist – a trained eye and ear to screen and accurately interpret responses.  This emphasis in the bill and in my speech to the House pointed out the unacceptable and erroneous belief that killer heart conditions rarely exist in fit young people, the catalogue of misdiagnosis, or for many lack of diagnosis, as well as the complex nature of achieving an accurate understanding of the problem. 

The prime requirement, writ large in this bill, is that lives can be saved if the research in this field is accepted and is used to inform medical procedures.  The medical opinion that made me determined to articulate this bill in the House of Commons was given to a young active male who is now dead.  He was told that his condition, Wolfe Parkinson-White syndrome, was a “bit of a nuisance,” that the treatment was medically nasty and painful, “so just get on with your life” …he now has no life to get on with.  Of course, treatment can be difficult to put in place and painful, if, as is likely, an operation is required, but isn’t it obvious that a treatment would be chosen if the choice was treatment or possible death? 

The treatments available which, from research evidence, reduce the risk of sudden death include that which I believe is most difficult for a fit athletic young person, “a change of life style,” to the more medically complicated excision or ablations of the heart; to the more recognised use of pacemakers and implantable cardioverter defibrillator. 

Of course it was not the research-based knowledge that informs the screening procedure to diagnose and treat the outlined diseases that was the inspiration for the bill.  It was a tragedy that hit a close family friend.  Levon Morland, aged 22 and a keen sports person in demanding employment with a diagnosed heart condition (Wolfe Parkinson-White syndrome) died in his sleep.  Neither he nor his family were aware that his heart condition could be fatal.  They knew it could be treated but as this operation was described as nasty and his condition as only a nuisance the decision was that he would get on with his life.  Similar medical histories exist everywhere for young people; one tipped to have an Olympic future, another who was an international tri-athlete, others playing first class football, with one just starting in that profession.  Many had shown symptoms that were either ignored or misdiagnosed.  The point of absolute similarity is that all were young and have now died.  Their deaths were sudden, their families were left heartbroken and this was significantly added to when they learned too late that the death of their loved one may have been prevented. 

Let me be straight with you: when I worked out the substance of my bill – with the charity Cardiac Risk in the Young, a cardiac specialist in the field of diseases outlined earlier (Dr Sanjay Sharma) and a specialist sport physiologist (Dr Greg Whyte) – I did not believe that it would or should be made law.  My initial discussion with the Department of Health reflected this fact – it was strikingly argumentative.  In fact, if I had been easily put off, I would, without a doubt, have introduced a bill with a different subject matter.  However, my conviction remained firm.  A gap in medical service existed that was, indirectly or directly, causing sudden death in fit young people.  I believe then, as now, that I needed the support of Parliament and tens of hundreds of constituents to persuade the Department of Health to accept this and to take the substance of my bill seriously.  I needed it to acknowledge the efficacy of the research and the medical work that was and is being done by a few in this area of medicine. 

I am pleased to say that this gap was acknowledged by the minister.  The positive outcome of my bill is that a chapter on arrhythmias and cardiac risk in the young is to be written and added to the Cardiac National Service Framework.  The fact that the advisers to my bill, along with Alison Cox from Cardiac Risk in the Young, will make up part of a team that will write this chapter ensures that the substance of my bill will inform its content.  At long last we will see the establishment of:

• A set of national standards that will improve the quality of medical care and reduce unacceptable variation in medical service;

• A defined service model, in which screening will be central, that will put in place strategies to support implementation and delivery of performance measures, against which progress (within an agreed time scale) is measure and monitored

• An in inspection regime that will oversee the implementation of service guidelines. 

This National Service Framework in Arrhythmias and Cardiac Risk in the Young will produce an authoritative medical model that will be universally implemented throughout the NHS.  Fit young people displaying symptoms that suggest the existence of a potentially killer disease will be screened – and if this disease is confirmed, offer treatment that will reduce the risk of sudden death.  This medical model has been long fought for and is now within reach.  When working effectively, it will reduce the risk of heart diseases that each week kill upwards of eight fit and athletic, young people.  Thanks for this achievement goes to a small team of cardiac specialists, Cardiac Risk in the Young and especially to the parents of young people who were the victims of hypertrophic cardiomyopathy and the other diseases outlined in this Private Members Bill. 

My especial thanks is given to the many parents who have coped with the sudden death of a child and also to many Members of Parliament.  The constant and robust support from both has made a medical model of service delivery on cardiac risk in the young a reality. 

Dari Taylor is Labour MP for Stockton South and secretary to the Cardiac Risk in the Young APPG. 

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My bill, “Cardiac Risk in the Young (Screening)”, sets out with several aims and they are to achieve:

• Universal acceptance by the medical profession and the Department of Health that certain heart defects, hypertrophic-cardiomyopathies and ion channelopathies can fatally strike fit young people;

• Acceptance that effective screening by a relevant cardiac specialist would be instantly available to all, in this case young people. Who display specific symptoms that suggest the possibility that a serious and possibly fatal heart condition exists;

• Acknowledgement of the value of treatments that reduce the risk of a heart attack by all medics;

• The availability of screening for all first degree relatives of kin (genetically related) with a confirmed inherited cardiac condition or to first degree relatives when a sudden death of a genetically related kin has occurred and the pathology confirms an inherited disease;

• Acceptance that all young people with a confirmed inherited disease that has the potential to be fatal have this fact clearly communicated to them so that they can make a decision with regards to treatment based on fact.