The Naked Surgeon, page 16
Cystic fibrosis is a terrible condition. It is a congenital illness due to a faulty gene that is quite common, in that one in 25 of the population carry it. Fortunately, it is not a dominant mutation, which means that, if you simply carry the faulty gene, having inherited it from one of your parents, you do not acquire the disease. You only have cystic fibrosis if you inherit the faulty gene from both parents. When you are conceived, you get half a set of genes from your father and half from your mother, and that combination is what makes you (and makes you unique, unless there is an identical twin). When a child is born to two parents of whom just one (mother or father) is a carrier of the cystic fibrosis gene, there is no possibility of the child having cystic fibrosis. This is why:
Father’s genes: N (normal) plus CF (faulty)
Mother’s genes: N (normal) plus N (normal)
Mixing N-CF with N-N has these four possibilities:
N-N N-N CF-N CF-N
This means that, on average, half the children produced would be carriers, but not one would have the actual disease, because you need the combination CF-CF to have cystic fibrosis.
But if both the father and the mother are carriers, the situation changes:
Father’s genes: N (normal) plus CF (faulty)
Mother’s genes: N (normal) plus CF (faulty)
Mixing N-CF with N-CF has these four possibilities:
N-N N-CF CF-N CF-CF
This means that, if two carriers get together and make babies, on average, one in four will be normal, two will be carriers, and one in four will be unlucky and have the actual disease.
Cystic fibrosis affects many organs, but the most vulnerable of all are the lungs. Patients find it difficult to clear lung secretions, so that these build up and clog the airways, leading to infection after infection, each slowly but inexorably leaving some lung damage. In the 1960s, few children with cystic fibrosis made it past the age of ten. With massive effort from parents, physiotherapists, nurses, and doctors over the past few decades, survival has improved tremendously. Nowadays, cystic fibrosis patients receive regular and vigorous physiotherapy every single day, any lung infection they develop is aggressively treated, and they are kept under close review, with care given by parents, teachers, and a dedicated healthcare team in specialist clinics. With this treatment, survival is now well into the fourth decade, and many children with cystic fibrosis now can expect to live well beyond, into their 40s.
Why am I telling you about cystic fibrosis? The reason is simple. It is one of the specialties in which one would have thought that quality monitoring is impossible. The reasons, or excuses, are legion and the following have all been said by doctors specialising in this field and in other areas of medicine:
Our specialty is nothing like cardiac surgery.
We cannot make our patients better — it is a slowly progressive disease.
We don’t have an outcome measure. It’s so easy for you cardiac surgeons: death and survival are easy to define. We have no such thing in our specialty.
Our outcomes are so much more subtle.
Our patients are very different from one another in age, disease severity, and how fast the disease progresses.
In fact, none of these excuses holds water. Even a clinic that provides medical care to cystic fibrosis patients can have outcome measures. All we need to do is to ask a few basic questions:
What is the service trying to achieve?
Is there a reliable way of measuring that achievement?
Is there an acceptable target to aim for?
In fact, one could easily argue (and I often do) that if doctors do not know what they are trying to achieve, have no way of measuring whether it has been achieved, and no idea if the achievement is good enough, then what on Earth are they doing in medicine? They’d be better employed selling snake oil.
Let us come back to cystic fibrosis: once these three simple questions are answered, how to monitor becomes self-evident. So here goes:
What is the service trying to achieve?
We know that cystic fibrosis cannot be cured, and that the progress of the disease cannot be stopped, so the best that can hoped for is a slowing down of the inevitable deterioration. Put on the spot, doctors specialising in cystic fibrosis agree with this statement.
Is there a reliable way of measuring that achievement?
Of course there is: all cystic fibrosis patients are monitored using lung function tests (in which they blow into tubes so that the capacity and effectiveness of their lungs can be measured). These measurements are routinely taken at frequent intervals in the cystic fibrosis clinic, and they are safe and non-invasive tests.
Is there an acceptable target to aim for?
Here we need to defer to experts in the field. They have agreed that, in general, a good thing to aim for is that lung function, as measured by the tests, should not deteriorate by more than 5 per cent per year. Achieving that would count as a success of the treatment.
The above is precisely what Dr Diana Bilton, who ran the cystic fibrosis service at Papworth Hospital in the 1990s, worked out and implemented. To the best of my knowledge, the service was the first non-surgical service in the world to introduce robust, objective quality monitoring with an actual, credible, relevant, and objective performance indicator. She proved that such practice is possible outside the narrow field of heart surgery and the wider one of surgery in general.
The value of such an approach is manifold. First, it informs the doctors, nurses, and other healthcare professionals how well they are doing. Second, it allows for the detection and correction of any underperformance (if you don’t even know it’s broke, there is no incentive to try to fix it). Third, it allows units to compare their performance, so that those doing less well can learn from those doing better. Finally, it allows for the assessment and audit of any treatment modification or innovation. Shouldn’t all doctors in all specialties be doing this?
So, where are we now?
I am a tiny, insignificant, ignorant bit of carbon.
I have one life, and it is short
And unimportant
But thanks to recent scientific advances
I get to live twice as long as my great great great great uncleses and auntses.
Twice as long to live this life of mine
Twice as long to love this wife of mine
Twice as many years of friends and wine
Of sharing curries and getting shitty
At good-looking hippies
With fairies on their spines
And butterflies on their titties.
These lines are by the Australian comedian and musician Tim Minchin, from his beat poem ‘Storm’, possibly one the most eloquent (and funniest) eulogies to science and modern medicine that I have ever heard. It is also a viciously sharp and effective attack on alternative medicine and unscientific thinking in general. It makes you both think and laugh out loud at the same time.
A long time ago, medical treatment was simple and cheap, much of it was safe, and most of it was utterly useless. Today, medical treatment is complex and expensive, some of it can sometimes be highly dangerous, but most of it is very effective. The progress made by medicine and surgery in the past few decades has been awesome in depth, range, efficacy, and complexity.
That said, much of our enviable good health and longevity derives not from high-tech medical intervention, but from basic public health measures, such as safe and nutritious food, clean water, decent sewerage, healthy living environments, mass vaccination, and not smoking. More can be ascribed to basic healthcare such as antibiotics for infections. Quite a bit of our good health, however, comes from modern, high-tech, and expensive medicine. The contribution of modern medicine to the overall health standard of the population is still relatively small when compared with more basic public health measures, but it is undoubtedly growing.
Heart surgery alone can now fix what was unfixable with operations that were unthinkable just 30 years ago. In fact, with the rare exception of heart failure, there is now a surgical solution to almost every cardiac problem, from coronary disease to leaky valves and enlarged aortas, all the way even to surgical treatment of electrical rhythm disturbances of the heart. Even heart failure may soon become a thing of the past when the artificial heart becomes reliable and inexpensive, and that surely is only a matter of technology and time. Progress in other fields of medicine has been similarly exciting, with keyhole surgery to fix many problems in the abdomen, phenomenal orthopaedic treatments to replace worn-out joints, kidney transplants for renal failure, liver transplants for hepatic failure, and eye surgery to treat cataracts and refraction abnormalities. Even the brain, that impossibly complex organ, is beginning to yield to surgical attempts to fix it. The results of cancer treatment are massively better than they were 50 years ago, and many cancers can now be looked at as a chronic disease rather than an imminent death sentence.
Chances are that you, if you live long enough, will need and benefit hugely from one or the other of these high-tech medical solutions to problems. A substantial proportion of us, if we reach a ripe old age, will have a worn aortic valve replaced, receive a pacemaker, have a coronary bypass, and so on, and that is just in my field. More will have joint replacements, prostate treatment, operations for cancer, and procedures on the arteries and veins of the body outside the heart.
In this book, I have deliberately drawn attention to the foibles of my profession. We often work with incomplete medical knowledge. We occasionally make stupid decisions. We take unnecessary risks with our patients’ lives and wellbeing. The good news is, we are finally embracing the concept of ‘total quality management’, albeit half a century after the Japanese carmaker Toyota incorporated it into its production systems, and we are beginning to understand risk management and safety protocols two decades after civil aviation. We still have a long way to go.
Despite this, I believe that medicine has never been better than it is today. Our treatments are mostly effective. We can make patients feel better. We can help them live longer. Our practice is largely based on evidence. Quality assurance is rapidly becoming integral to our processes. We have started to learn from crashes and near misses, improving our methods and our systems. And there is increasing transparency in our work. In every single aspect of the improvements listed above, cardiac surgery has played the lead role. For that, my colleagues and I may perhaps be forgiven the indulgence of a little pride.
Tomorrow, or sometime in the future, you or someone you love will perhaps need a heart operation. My hope is that the material in this book will help you to ask the right questions, consider the options, and reach the right decision. If you decide to go ahead and place your life in my hands, or in the hands of one of my colleagues, you can do so with confidence. Medicine has never been as good as it is now, and nowhere is that truer than in the specialty of heart surgery.
Acknowledgements
Steve Large and Steve Bolsin generously provided me with heartfelt narratives of their experience of difficult times when they must have felt most vulnerable. My daughter Leila applied her phenomenal literary skills both to the manuscript and to the proposal, without which The Naked Surgeon might have never seen the light. Peter Tallack of the Science Factory is an amazing agent, whose remit extends way beyond mere representation, to include perceptive insight and excellent advice, given with passion and dedication. Philip Gwyn Jones and David Golding did a superb job in editing the final manuscript. To all of them, and to my partner, Fran, for her tireless and unwavering support, thank you.
Appendix A
Still Nobody Told the Parents
By Steve Bolsin
Bristol remains a charming city in an idyllic setting, and I was delighted to be appointed as a consultant anaesthetist for adult and paediatric cardiac surgery in 1988. Maggie and I planned to bring up our family there. The job content of adult and paediatric cardiac anaesthesia included some of the most challenging work in the specialty available at the time, with a mixture of intensive care medicine thrown in for good measure. I was excited by the prospects and opportunities. I had no idea that Bristol would become the graveyard of my dreams at the same time as it became the graveyard of 171 children who could have survived if they had been operated on anywhere other than Bristol. From the deaths of these children emerged the concept of clinical governance, which has saved thousands more lives in the UK and worldwide. Sadly the change in attitudes of the profession came too late for these small sacrificial lambs.
The surgeon I worked with was Mr James Wisheart. He was soon to become the senior cardiac surgeon in the South West region, and head of the department in Bristol. At the time, I had no idea how good or bad the results in paediatric cardiac surgery were anywhere in the UK. Nobody except the surgeons did. However, what I did know was that, in Bristol, the operations were taking a long, long time. That, by itself may not have been a bad thing, but two other factors worried me even more. Firstly, the long times on the cardiac-bypass pump led to much more postoperative organ damage than in London hospitals. Secondly, the length of time the heart was starved of oxygen during the operations in Bristol was also much longer than London, and this led to severe heart damage and children dying in much greater numbers.
A few months after I had started in Bristol, an audit meeting of the paediatric cardiac surgical specialists confirmed my suspicions that the death rates in Bristol were abnormally high. The meeting was held specifically to discuss the operation of repair of ventricular septal defect (or VSD: a hole in the heart). The mortality rate for this operation at the Royal Brompton Hospital in London and around the world was very low, at around 1 per cent. Figures were presented that indicated the mortality in Bristol for this simple operation was incredibly high, as much as 30 per cent. The problem in Bristol was not that surgeons and cardiologists were unaware of the high mortality rates; the problem was that they were not prepared to do anything to get the death rates down. In those days, holding audit meetings was revolutionary, forward-thinking, and probably ahead of many other centres with better results. However, failing to act on the evidence of these audit meetings and annual reports was typical of the attitudes in Bristol.
The paediatric unit in Bristol had been under a cloud for many years. An expert paper produced for the Department of Health in 1980 had insisted that paediatric cardiac surgeons needed to do at least 50 operations each year on children less than one year of age (infants) to maintain their skills. Units doing more than 50 operations in young children had lower mortality rates than those that did fewer than 50. Bristol had never achieved that number of operations, and had not been considered in the first list of centres to be given the prestigious ‘Supra-Regional Paediatric Cardiac Surgery Centre’ status by the Supra-Regional Services Advisory Group (SRSAG), chaired by Dr Norman Halliday. The description by the Committee of the Bristol unit at that time was that it ‘did not shine like a star’ and was ‘not one of the leading lights’. Bristol was designated to fail, but was added to the list of Supra-Regional centres in 1983, as an afterthought, in response to its geographical location in the west of England. There was no consideration of high mortality rates at this stage.
The city of Cardiff, in Wales, is 44 miles from Bristol. In 1986, the senior cardiologist in Cardiff, Professor Andrew Henderson, negotiated a special arrangement with the paediatric cardiac centre in Southampton (138 miles away) to assess and treat children from Cardiff and Wales. In doing so, he was replicating a deal that the paediatricians in Plymouth (120 miles from Bristol) had negotiated with Southampton (152 miles away), also in 1986, to assess and treat their children as well. Both arrangements were specifically made to ensure that children were not treated in the ‘high-mortality’ Bristol centre. Nobody told the parents in Plymouth, Cardiff, or Bristol. At that time, the criticisms of Bristol were so strident the Chief Medical Officer for Wales, Professor Gareth Crompton, discussed the matter with the Chief Medical Officer for England Sir Donald Acheson. Professor Crompton was advised to speak to Dr Norman Halliday.
Also in 1986, a report co-authored by Mr Terence English had confirmed that Bristol did not have the number of operations necessary to achieve low mortality rates, but Dr Halliday’s SRSAG continued its designation of Bristol as a Supra-Regional centre. By 1987, the criticism was so loud that the broadcaster BBC Wales aired a scathing documentary about the survival of children from south Wales in the Bristol centre.
My problem from 1989 was that operations on tiny babies less than one month old were the highest risk in Bristol. After consulting a senior colleague, in June 1990, I decided to express my concerns, and wrote to Dr John Roylance, who was about to become the chief executive of the Bristol Royal Infirmary. The response to my letter was swift. First, a phone call from the chief executive dismissed the concerns. Second, a meeting with the chairman of the Hospital Consultants Committee concluded that I had been manipulated by a senior colleague. Later, I attended a meeting that was much more sinister. An obviously infuriated Mr Wisheart explained that writing to the chief executive was not the way to behave in cardiac surgery, and he delivered a potent threat: ‘If you wish to remain in Bristol you should not disclose the results of paediatric cardiac surgery to people outside the unit ever again.’ The venomous and intense manner of the delivery of the message added to its sombre content. This was reinforced at an anaesthetic audit meeting held to discuss paediatric heart surgery some weeks later. The president of the Association of Anaesthetists of Great Britain and Ireland, himself a cardiac anaesthetist, advised caution in dealing with the issue, because, as he put it, ‘Steve had already put his head above the parapet and had a “pot shot” taken at him’. This was colourful language from a colourful character, but very sobering feedback for a junior anaesthetist like me who desperately wanted to do the best for his patients.
