Do No Harm: Stories of Life, Death and Brain Surgery (18 page)

19

AKINETIC MUTISM

n.
a syndrome characterized by the inability to speak, loss of voluntary movement and apparent loss of emotional feeling.

Neuroscience tells us that it is highly improbable that we have souls, as everything we think and feel is no more or no less than the electrochemical chatter of our nerve cells. Our sense of self, our feelings and our thoughts, our love for others, our hopes and ambitions, our hates and fears all die when our brains die. Many people deeply resent this view of things, which not only deprives us of life after death but also seems to downgrade thought to mere electrochemistry and reduces us to mere automata, to machines. Such people are profoundly mistaken since what it really does is upgrade matter into something infinitely mysterious that we do not understand. There are one hundred billion nerve cells in our brains. Does each one have a fragment of consciousness within it? How many nerve cells do we require to be conscious or to feel pain? Or does consciousness and thought reside in the electrochemical impulses that join these billions of cells together? Is a snail aware? Does it feel pain when you crush it underfoot? Nobody knows.

An eminent and eccentric neurologist who had sent me many patients over the years asked me to examine a woman I had operated on a year earlier who was in a persistent vegetative state. I had operated for a ruptured arterio-venous malformation after she had suffered a life-threatening haemorrhage and I had operated as an emergency. It had been a difficult operation and although it had saved her life it could not undo the damage done to her brain by the haemorrhage. She had been in a coma before the operation and remained in a coma for many weeks afterwards. She had been transferred back to her local hospital some weeks after the operation where she had been under the care of the neurologist who now wanted me to see her in the long-term nursing home in which she had ended up. Before she was transferred to the nursing home, I had carried out a shunt operation for hydrocephalus which had developed as a late after-effect of the original bleed.

Although the shunt operation had been a relatively minor one – one I would usually delegate to my juniors – I remembered it well because I had carried it out at the local hospital and not in my own neurosurgical centre. I scarcely ever operate away from my own theatres, except when I am working abroad. I had gone to the local district hospital where she was a patient with a tray of instruments and one of my registrars. I had gone vainly thinking that the visit of a senior neurosurgeon to the hospital – since brain surgery was not normally performed there – would be an event of some importance and be of some interest, but apart from the desperate family everybody else in the hospital seemed scarcely to notice my arrival. The local neurologist, who was away at the time of my visit, had told the family that the operation might relieve her persistent vegetative state. I was less optimistic, and said so, but there was little to be lost by trying and so after discussing this with them I went down to the operating theatres where, I was told, the staff were ready for me to operate.

The nurses and the anaesthetists greeted me with total indifference, which I found quite disconcerting. I had to wait two hours before the patient was brought down for the operation, and when she eventually came into the theatre, the staff all worked in sluggish and sullen silence. The contrast to my own friendly and energetic neurosurgical theatres was remarkable. I had no way of knowing whether they felt that I was wasting their time by operating on a human vegetable or whether this was just their normal way of behaving. So I operated, reported back to the family afterwards, and drove back to London.

As the months passed after this second operation, it became clear that the shunt had made no difference to her condition, and her neurologist wanted me to examine the patient and see if the shunt was working or if it had blocked. It seemed a little cruel and unnecessary to drag her all the way to my hospital in an ambulance just for my opinion so I had agreed – a little reluctantly, as I knew that I could not help – to visit her in the nursing home that now cared for her.

Patients in persistent vegetative state – or
PVS
as it is called for short – seem to be awake because their eyes are open, yet they show no awareness or responsiveness to the outside world. They are conscious, some would say, but there is no content to their consciousness. They have become an empty shell, there is nobody at home. Yet recent research with functional brain scans shows this is not always the case. Some of these patients, despite being mute and unresponsive, seem to have some kind of activity going on in their brains, and some kind of awareness of the outside world. It is not, however, at all clear what it means. Are they in some kind of perpetual dream state? Are they in heaven, or in hell? Or just dimly aware, with only a fragment of consciousness of which they themselves are scarcely aware?

There have been several high-profile court cases in recent years as to whether treatment that keeps these people alive – since they cannot eat or drink – should be withdrawn or not, whether they should be left to die or not. In several cases the judges decided that it was reasonable to withdraw treatment and let the vegetative patients die. This does not happen quickly – instead the law, solemn and absurd, insists that the patients are slowly starved and dehydrated to death, a process that will take several days.

I finished my outpatient clinic at eight and drove out of London in the early autumnal evening. It was quite late by the time I reached the neurologist’s home. He drove me in his own car to the nursing home a few miles away. This was a pleasant country house, surrounded by tall and ancient trees. It was night by now, and once we had parked the car I could see the friendly lights of the nursing home through the dark branches of the trees as we walked across a derelict tennis court covered in dry, fallen leaves. The home was run by Catholic nuns and devoted to people with catastrophic brain damage. Inside all was clean and tidy, and the staff clearly very caring and friendly. The contrast with the hospital where I had carried out the shunt operation a year earlier could not have been greater. The devout Catholic staff did not accept the grave lesson of neuroscience – that everything we are depends upon the physical integrity of our brains. Instead, their ancient faith in an immaterial human soul meant that they could create a kind and caring home for these vegetative patients and their families.

The sister took me up a grand staircase to see my patient. I wondered who had lived in the house originally – an Edwardian capitalist perhaps or lesser aristocrat, with a small army of servants. I wondered what he would have thought of the use to which his imposing home had now been put. On the first floor there was a wide, carpeted corridor which we walked down, passing many patients in their rooms on either side. The doors were all open and through the doorways I could see the motionless forms of the patients in their beds. Beside each door was an enamelled plaque with the patient’s name; because they are there for so many years, until they die, they can have proper plaques rather than just the paper labels you find in an ordinary hospital. To my dismay I recognized at least five of the names as former patients of mine.

One of the senior neurosurgeons who trained me, and a man I revere, told me a story once of the famous, knighted surgeon with whom he, in his turn, had served as an apprentice.

‘He used to remove acoustic tumours with a periosteal elevator, an instrument normally used for opening the skull,’ he told me. ‘An operation that would take most surgeons many hours took him thirty or forty minutes. Inevitably this would sometimes lead to disaster. I remember one woman with a large acoustic – he caught the vertebral artery with the elevator and there was torrential haemorrhage. The woman was obviously done for. I had to close up and that was that. Nevertheless, I always had to ring him up every evening at seven o’clock on the dot to let him know how all the patients were doing. So I went through the list of all the inpatients. At the end I mentioned the woman with an acoustic. Mrs B she was called, I can still remember the name. Mrs B is slipping away, I said, or words to that effect. “Mrs B?” he said. “Who’s that?” He had forgotten her already. I wish I had a memory like that,’ my boss said wistfully. ‘Great surgeons,’ he then added, ‘tend to have bad memories.’

I hope I am a good surgeon but I am certainly not a great surgeon. It’s not the successes I remember, or so I like to think, but the failures. But here in the nursing home were several patients I had already forgotten. Some of them were people I had simply been unable to help, but there was at least one man who, as my juniors put it in their naive and tactless way, I had wrecked.

I had ill-advisedly operated on him many years earlier for a large tumour in a spirit of youthful enthusiasm. The operation had gone on for eighteen hours and I had inadvertently torn the basilar artery at two in the morning – this is the artery that supplies the brainstem, and he never woke up again. I saw his grey curled-up body in its bed. I would never have recognized him were it not for the enamelled plaque with his name by the door.

The patient I had come to see lay mute and immobile, limbs rigid, her eyes open in an expressionless face. She had been a journalist for a local newspaper, full of life and energy, but had then suffered the haemorrhage which caused the damage which my operation could not undo. There were happy, smiling photographs of her before this terrible event on the walls around the room. She made occasional mewling sounds. It took me only a few minutes to test the shunt by putting a needle into it through the skin of her scalp and establish that it was working. There was nothing I could do to help.

She communicated, apparently, via a Morse-code buzzer, as it seemed that she could move one finger. A nurse was sitting beside her and patiently listened to the bleeping sounds, concentrating very hard with a slight frown. She interpreted them for me. The patient asked me, the nurse explained, about the shunt and then she thanked me and wished me goodnight.

Her mother was there and came out of the room with me, accosting me a little desperately in the wide corridor outside. We spoke for a while. She talked about letters that her daughter had been sending – transcribed by one of the nurses from her Morse-code bleeping. She expressed some doubts as to whether her daughter had really said the things transcribed by the nurses.

There is no way of knowing, of course. The woman’s mother lives in a nightmare, a labyrinth of uncertainty and of hopeless love, her daughter both alive and dead. Behind her daughter’s rigid, expressionless face, is she in fact awake? Is she aware, in some way, of what is going on outside her paralysed body? Are the nurses inventing her letters – wittingly or unwittingly? Does their faith deceive them? Can we ever know?

20

HUBRIS

n.
arrogant pride or presumption; (in Greek tragedy) excessive pride towards or defiance of the gods, leading to nemesis.

I went to Marks & Spencer in Wimbledon in the morning and bought a boxful of fruit and chocolates for the theatre staff. I had gone through my CD collection and picked out enough disks to last at least all day and much of the night as the operation was going to be a long one. I had only been a consultant for four years but I already had a very large practice, larger than any other neurosurgeon’s I knew. The patient was a schoolteacher in his late fifties, tall and bespectacled, who walked with a stick and was a little stooped. He had been seen by a local neurologist who had arranged a brain scan and as a result he had been sent to see me. It was in the days of the old hospital and I saw him in my office, with its row of windows looking out onto a little copse of birch trees. One of the local foxes would sometimes look in at me with a thoughtful expression as it passed by. I sat the patient down in the chair by my desk with his wife and son next to him and took the films of his brain scans – which he had brought with him – over to the viewing box on the wall. Computers were still a long way away.

I already knew what the scans would show but I was still startled by the sheer size of the tumour growing from the base of his skull. All of the brainstem and the cranial nerves – the nerves for hearing, movement, sensation for the face, and for swallowing and talking – were stretched over its sinister hump-backed mass. It was an exceptionally large petro-clival meningioma. I had only seen tumours this size before in the textbooks. In later years I was to see many such in Ukraine when patients with terrible tumours from all over the country would come to seek my opinion. I was not sure whether to feel excited or alarmed.

I went back to my desk and sat down next to him.

‘What have you been told about this?’ I asked.

‘The neurologist said it was benign,’ he replied ‘And that it was up to you as to whether it should be removed or not.’

‘Well, it’s certainly benign but it’s also very large,’ I said. ‘But they grow very slowly so we know it’s been there for many years already. What led you to having the scan in the first place?’

He told me how he had noticed that his walking had slowly been becoming a little unsteady in recent years and that he was also starting to lose hearing in his left ear.

‘But what will happen if it stays there?’ his son asked.

I replied cautiously, telling them that it would go on growing slowly and that he would slowly deteriorate.

‘I’ve already decided to take early retirement on medical grounds,’ he said.

I explained that surgery was not without risks.

‘What sort of risks?’ asked the son.

I told them that the risks were very serious. There were so many brain structures involved with the tumour that the dangers of surgery ranged from deafness or facial paralysis to death or a major stroke. I described what surgery would involve.

The three of them sat in silence for a while.

‘I’ve been in contact with Professor B in America,’ the son said. ‘He said it should be operated upon and he said he could do it.’

I was not sure what to say. I was only at the beginning of my consultant career and knew that other surgeons were more experienced than I was. In those years I was in awe of the big names of international neurosurgery, who you could hear giving keynote lectures at conferences where they showed cases like the man in front of me, and the amazing results they achieved, quite beyond anything that I had yet done.

‘But it would cost over 100,000 dollars,’ the patient’s wife added. ‘And we can’t afford that.’

The son looked a little embarrassed.

‘We’re told that Professor M is the best neurosurgeon in the country,’ he said ‘And we’re going to see him for a second opinion.’

I felt humiliated but knew that any operation was going to be exceptionally difficult.

‘That’s a good idea,’ I said. ‘I’d be very interested to hear what he thinks.’ They left the room and I continued with my outpatient clinic.

‘I’ve got Professor M on the line for you,’ said Gail two weeks later, looking into my office.

I picked up my phone to hear the professor’s booming, confident voice. I had known him briefly when I was a trainee and he was certainly a superb surgeon whom all the trainees hoped to emulate. Self-doubt had never seemed to be one of his weaknesses. I had heard that he would soon be retiring.

‘Ah, Henry!’ he said ‘This chap with the petro-clival. Needs to come out. He’s starting to have some difficulties with swallowing so it’s only a matter of time before he gets aspiration pneumonia and that will be the end of him. It’s a young man’s operation. I’ve told them you should do it.’

‘Thank you very much Prof,’ I replied, a little surprised but delighted to have been given what felt like a papal dispensation.

So I made arrangements for the operation, which I expected to be a long one. This was many years ago, when hospitals were different places, and all I had to do was ask the theatre staff and anaesthetists to stay on longer than usual. There were no managers whose permission had to be sought. The operation started in an almost festive spirit. This was man-sized brain surgery – a ‘real Big Hit’ as the American registrar assisting me put it.

As we opened the man’s head, we talked about the big names of neurosurgery in America.

‘Prof B’s a really fantastic surgeon, amazing technician,’ my registrar said, ‘but do you know what he was called by his residents before he moved to his present job? They called him “the Butcher” because he trashed so many patients as he perfected his technique with these really difficult cases. And he still gets some terrible complications. Doesn’t seem to trouble him much though.’

It’s one of the painful truths about neurosurgery that you only get good at doing the really difficult cases if you get lots of practice, but that means making lots of mistakes at first and leaving a trail of injured patients behind you. I suspect that you’ve got to be a bit of a psychopath to carry on, or at least have a pretty thick skin. If you’re a nice doctor you’ll probably give up, let Nature takes its course and stick to the simpler cases. My old boss, who was really nice – the one who operated on my son – used to say ‘If the patient’s going to be damaged I’d rather let God do the damage than do it myself’.

‘In the
US
,’ my registrar continued, ‘we’re a bit more can-do, but we have a commercial healthcare system and nobody can afford to admit to making mistakes.’

The first few hours of the operation went perfectly. We slowly removed more and more of the tumour, and by midnight, after fifteen hours of operating, it looked as though most of it was out and the cranial nerves were not damaged. I started to feel that I was joining the ranks of the really big neurosurgeons. I would stop every hour or two, and join the nurses in the staff room and have something to eat and drink from the box I had brought and smoke a cigarette – I stopped smoking some years later. It was all very convivial. Music played continuously while we operated – I had brought all sorts of CDs in that morning ranging from Bach to Abba to African music. In the old hospital I always listened to music when operating and although my colleagues found some of my choices a little strange they seemed to like it, especially what we called ‘closing music’ which meant playing Chuck Berry or B.B. King or other fast rock or blues music when stitching up a patient’s head.

I should have stopped at that point, and left the last piece of tumour behind, but I wanted to be able to say that I had removed all of the tumour. The post-operative scans shown by the big international names when they gave their keynote lectures never showed residual tumour so surely this was the right thing to do, even if it involved some risk.

As I started to remove the last part of the tumour I tore a small perforating branch off the basilar artery, a vessel the width of a thick pin. A narrow jet of bright red arterial blood started to pump upwards. I knew at once that this was a catastrophe. The blood loss was trivial, and easy enough to stop, but the damage to the brainstem was terrible. The basilar artery is the artery that keeps the brainstem alive and it is the brainstem that keeps the rest of the brain awake. As a result the patient never woke up and that was why, seven years later, I saw him curled into a sad ball, on a bed in the nursing home.

I will not describe the pain of seeing his unconscious form on the
ITU
for many weeks after the operation. To be honest I cannot remember it well now, the memory has been overlain by other, more recent tragedies, but I do remember many anguished conversations with the family as we all hoped against hope that he would wake up again one day.

It is an experience unique to neurosurgeons, and one with which all neurosurgeons are familiar. With other surgical specialties, on the whole, the patients either die or recover, and do not linger on the ward for months. It is not something we discuss among ourselves, other than perhaps to sigh and nod your head when you hear of such a case, but at least you know that somebody understands what you feel. A few seem to be able to shrug it off, but they are a minority. Perhaps they are the ones who will become great neurosurgeons.

Eventually the poor man was sent back to his local hospital, still in a coma but no longer on a ventilator, and at some point he had been sent on to a nursing home where he had remained ever since. This was the man I had seen and scarcely recognized on my visit to see the girl with akinetic mutism.

For the next few years whenever I saw similar cases – which was only on a few occasions – I deemed the tumours inoperable and left the unfortunate patients to go elsewhere or to have radiation treatment, which is not very effective for very large tumours of this kind. These were also the years when my marriage fell apart and the old hospital was closed. I am not sure whether I realized it then, but this was the time when I became a little sadder but, I would like to think, much wiser.

Nevertheless, I gradually regained my courage and used what I had learned from the tragic consequences of my hubris to achieve much better results with tumours of this kind. I would, if necessary, operate in stages over several weeks, I would operate with a colleague, taking the operating in turns with an hour on and an hour off, like drivers in a military convoy. I would not try to remove all of the tumour if it looked as though it would be particularly difficult. I would rarely let an operation take longer than seven or eight hours.

The problem remains, however, that such tumours are very rare. In Britain, with a culture which believes in the virtues of amateurism, and where most neurosurgeons are very reluctant to refer difficult cases on to a more experienced colleague, no individual surgeon will ever gain as much experience as some of our colleagues do in the US. In America there are far more patients, and therefore more patients with such tumours. The patients are less deferential and trusting than they are in Britain. They are more like consumers than petitioners, so they are more likely to make sure that they are treated by an experienced surgeon.

After twenty-five years I would like to think that I have become relatively expert – but it has been a very long, slow advance with many problems along the way, though none as awful as that first operation. A few years ago I operated on the sister of a famous rock musician with a very similar tumour and, after a difficult time for the first few weeks after the operation, she made a perfect recovery. Her brother gave me a large sum of money from the charitable fund he runs which has helped fund my work in Ukraine and elsewhere ever since, so perhaps I can say that some good came out of that wretched operation many years ago.

There were two other lessons that I learned that day. One was not to do an operation that a more experienced surgeon than me did not want to do; the other was to treat some of the keynote lectures at conferences with a degree of scepticism. And I can no longer bear to listen to music when operating.