Fragile Lives Page 14

  Word of the bizarre operation in Theatre 5 soon spread. The curious gathered to watch and the camera kept on filming. We had to ensure that all air was removed from the heart, otherwise it could be ejected into the blood vessels of the brain and cause a stroke. After that, all that remained was to defibrillate and try to restore normal heart rhythm.

  ‘We’re done,’ I announced. ‘Try 20 joules.’

  Zap! The heart stopped quivering, and for what seemed like an age there was no spontaneous electrical activity. I poked the muscle with forceps and it contracted in response. This time there was a flicker on the blood pressure trace. Miraculously, the black banana had ejected blood into the aorta.

  Mike looked again at the echo. ‘It certainly looks different. Shall we try using the pacemaker?’

  I was already sewing the fine pacing wires into place. We arbitrarily set the pacing box rate at 100 beats per minute and switched it on. I told the perfusionist to cut back on the pump flow rate and leave blood in the heart to see if it would eject consistently. It did. What’s more, the echo showed that the mitral valve no longer leaked. At this point I felt that we were in with a chance. Life really does depend upon physics and geometry.

  It was now after midday. Kirsty had been on the bypass machine for more than three hours and we needed to get her off it. As if timed to perfection, her own heart rhythm suddenly broke through the pacing. Coordinated natural heart rhythm is much more efficient than electrical pacing, providing much better blood flow and pressure.

  It was like switching on a light in the operating theatre. Gloom changed to elation. My own adrenaline kicked in and the fatigue suddenly lifted. We gave Kirsty an infusion of adrenaline to help her heart take over from the bypass machine. Finally I gave the instruction to ‘come off slowly’. We still expected her blood pressure to fade, yet the curiously reconfigured little heart kept on pumping.

  ‘Off bypass. I don’t believe it,’ said Mike.

  I remained silent, but I looked over my mask at Katsumata. He knew that I’d had enough by now.

  ‘Let me finish,’ he said.

  ‘Sure.’

  I took a last, disbelieving glance at the little black banana pumping away, then turned to the echo screen, where the incomprehensible flashes of white, blue and yellow were also reassuring, like a blazing fire. We could see blood streaming through the new left coronary and a double jet entering the left ventricle through the mitral valve – a curiously reconfigured baby’s heart that finally worked.

  After the encounter at the theatre door both Archer and the parents believed that Kirsty had died. This created an unprecedented and awkward situation, but one that I was too knackered to deal with. I asked the anaesthetic nurse to bleep Dr Archer and tell him to come down yet again. She did this, then offered to get me a coffee.

  Katsumata made certain that there was no bleeding, then meticulously closed the chest.

  ‘Never been done before,’ he said, looking over at me.

  Soon afterwards Becky came down to the paediatric intensive care unit in shock. She put her hand on Kirsty’s little foot and exclaimed, ‘It’s warm. It’s never been warm.’ When she started to cry I left. It had been a long day.

  Dee, my endearing and eccentric secretary, drove me home to Bladon, some twenty minutes outside Oxford. I was restless, with a mixture of elation and fatigue. A huge, red, wintery sun was setting over Blenheim Palace. To wind down I set out to run around the lake with Max, our German shepherd dog. Through the ancient oaks we scattered rabbits and fortunate pheasants who’d survived the shooting season. The shadows lengthened. Hissing swans told Max to piss off. The sun slipped away as I staggered by the water’s edge. I left the park at the Bladon gate, crossed the road and went into the grounds of St Martin’s Church.

  Winston Churchill is buried in the graveyard. Facing his tomb – invariably surrounded by dead flowers with their heads dipped in reverence – is a wooden seat donated by the Polish resistance from the Second World War. Hot, and gasping for breath, I sat down to talk with the great man, less than ten feet away in his box. Morbidly, I tried to visualise how his corpse looked now and contemplated how Kirsty at that moment could so easily have been stiff and cold in the hospital mortuary. But I’d followed his dictum – never surrender.

  Max irreverently cocked his leg on a neighbouring tomb. Now I needed to sleep. I hoped that the phone would leave me in peace. It did. Kirsty survived.

  We followed her carefully over the next ten years, using echocardiography to watch her heart develop. She was a perfect little girl, happy, outgoing and energetic, the only clue to her extraordinary internal metamorphosis being the faint stripe up the middle of her chest.

  When we felt she was mature enough to discuss it we asked for her permission to carry out a magnetic resonance imaging scan to show us how the remodelled heart had developed. What we found was quite extraordinary. Apart from the double-orifice mitral valve her heart appeared normal, as did her new left coronary artery. Only a thin scar showed the position of the line of stitches up the heart. Remarkably, all other scar tissue had disappeared. The whole inner lining of her left ventricle had been pure white scar tissue – now all gone.

  This provided some of the first evidence that an infant’s own cardiac stem cells can regenerate heart muscle and actually remove fibrous tissue. Adult hearts cannot recover in the same way. But what if we could identify and culture stem cells that could do just this for an adult heart? Could it provide a solution for the hundreds of thousands of adult chronic heart failure patients with coronary artery disease? My grandfather was someone who might have benefited. We could inject the cells at the same time as their coronary bypass surgery, or get them in through a catheter within the heart. What cells would we use, where would we find them, and how could we preserve and transplant them? One day I’d find out.

  Now aged eighteen, Kirsty is a vivacious and athletic teenager, but had she died we’d never have known about this exciting possibility for heart regeneration. Her case could potentially save countless other lives.

  9

  domino heart

  I will give you a new heart and put a new spirit in you; I will remove from you your heart of stone and give you a heart of flesh.

  Ezekiel 36:26

  Barely a day went by without a visit to the paediatric intensive care unit to see the babies or small children I’d operated on, and to reassure their parents that one day soon things would get better. More often than not these visits provided a window onto other personal tragedies. Nothing could be worse than the black gangrenous limbs of infants with meningitis that required amputation. Or children injured on the roads with multiple injuries or brain death. Or the untold complications of cancer and chemotherapy. Why should kids get cancer? How can that be fair? Or hydrocephalus – babies with huge skulls full of fluid, their heads heavier than their bodies, unable to lift them from the floor? Fragile, miserable lives.

  It was three weeks after our success with Julie. I’d been summoned by the paediatric cardiologists, who wanted to discuss an urgent case. Would I come straight away?

  Several doctors were standing at the foot of a boy’s bed, sifting through charts and test results. His desolate mother sat hunched beside him, her face strained with the anxiety of it all, holding his sweaty hand and staring at the heart monitor. He was propped up on pillows at a forty-five degree angle, eyes closed, chest heaving and making grunting sounds with every breath, coughing intermittently. I could see that he was deathly pale and limp, his eyes closed, his head tipped back, neck extended, struggling to breathe and quite obviously emaciated with that yellow tinge of terminal cancer. He was somewhere else in his mind.

  So why did they need me? Maybe he had a tumour in his heart. Rare – certainly – but I’d operated on several heart tumours in children. Or maybe cancer had spread from a kidney or bone to the pericardium, causing fluid to compress his heart. I’d often been asked to make a window in the pericardium for such cases so that the flu
id would drain into the chest cavity, where it did less harm.

  Whatever the problem, it looked pretty desperate. I went unnoticed for a while – unusual for a cardiac surgeon – and just stood behind them and listened.

  The boy’s name was Stefan, and he was ten but looked younger. His mother had described him as ‘not right’ for some time – he couldn’t keep up with his friends and wasn’t concentrating at school. He’d even stopped playing football, because if he ran a few yards he was left gasping for breath.

  Over the school holidays his parents had become increasingly concerned and he soon became really very unwell. The GP who’d listened to his chest said it sounded ‘wet’, then sent him straight up to the hospital for an X-ray. The news was bad. His lungs were wet because he had a huge heart with left ventricular failure and fluid on the lungs, what we call pulmonary oedema. This all came out of the blue as there was no previous medical history – no congenital heart disease, nothing to explain why he was now clearly dying.

  The whole atmosphere was so tense that I needed to break the ice.

  ‘Good morning,’ I said. ‘Can I help with anything?’

  This was met by the usual response from Archer. ‘Ah, Westaby, what kept you? Can I show you an echo?’

  Stefan was concentration camp thin, with no fat on his chest wall, which told me that he’d been sick for months. His mum wasn’t thin, so this wasn’t deprivation. But the good news, if there was any, was that thin provides good echo pictures, and the problem was obvious when I looked at these. Both ventricles were dilated, worse on the left. The huge left ventricle barely moved and the mitral valve leaked. The two mitral leaflets were pulled apart, as what should have been a conical heart had turned into a sphere. A rugby ball heart, much like Kirsty’s.

  My thoughts went galloping on ahead. They were going to ask me to repair the valve and take the pressure off the lad’s lungs. But surely this wasn’t primarily a mitral valve problem. It was end-stage heart muscle disease, causing the valve to leak as a secondary issue. Any attempt at conventional cardiac surgery would finish him off, although I didn’t state this out loud so as not to alarm his parents. Then I realised where this conversation was heading. It was about pumps.

  By now everyone knew about Julie, who was still in the hospital but recovering well. We were beginning to get calls for help from all over the country. Viral myocarditis with chronic heart failure was Stefan’s probable diagnosis, but because he’d been sick for months not days he was unlikely to recover as Julie had.

  My immediate response was that he needed a new heart. Soon. Very soon. At the time only Great Ormond Street were doing children’s transplants. I knew the surgeons well, as I used to work there. So let’s get Stefan into their system and onto the urgent waiting list. Simple.

  But not so simple, actually. Our people had already talked to the London transplant doctors and been told, sorry, but they were pressed for beds and already had several urgent patients waiting. And no, there was no chance at all of a transplant by order. Not in a child. Of course they’d get back to us when the situation improved, but in the meantime ‘Do your best.’

  Stefan was already receiving high doses of intravenous drugs to make his heart pump harder and diuretics to draw the water off his lungs. Without adequate blood pressure the kidneys won’t work, and they were already struggling. He was on the verge of falling off the edge into the abyss. One of the paediatric cardiologists around his bed then asked the direct question: could I use another AB-180? With Julie we had set the precedent. If it really was myocarditis we might save him – and save his own heart. Or at least keep the lad going until Great Ormond Street could take him. It was the family’s last hope.

  I was conscious that the poor mother was listening to every word. The nurse had a hand on her shoulder, trying in vain to keep her calm. All eyes were on me. I went quiet and thought for a moment. Yes, we did have a second AB-180, but no, it wouldn’t work. That inflow cannula was too big and too stiff to fit into a child’s left atrium.

  I shared this response with the gaggle of physicians. Their long, serious faces showed obvious disappointment, while the boy’s mother started to cry. Archer had already suggested to her that the pump was the only outstanding option and that if the situation deteriorated further – as it was bound to – his course would be rapidly downhill, with an all-too-obvious terminus. So I’d just delivered the death sentence.

  Stefan was an ordinary kid from a working-class family. He had his whole life ahead of him, and should have been in the school playground with his mates, not propped upright and petrified in an intensive care bed surrounded by white coats and long faces. He must have felt totally exhausted just lying there, the simple effort of breathing on its own leaving him worn out. Then that tedious cough with the tight feeling in his throat, like being strangled. He felt cold, yet his sheets were damp with perspiration. Strangers were sticking sharp needles into his arms and neck, sucking out his blood, shoving rubber pipes up his private parts, things he should never have to contemplate at that age. Seeing his mum and dad upset clearly disturbed him, and he heard words that he didn’t understand. Soon he started to become light-headed, reality began to fade and things slowly seemed to drift away into the far distance. Morphine was taking the dread away.

  His mum and dad slumped on either side of the bed, leaning forward to be closer to him, both tense but emotionally drained. They should have been at work, not in the hospital – in fact they’d have rather been anywhere else but here, with neither control nor influence as their only son lay dying.

  How could this have happened just out of the blue? What did they do wrong? They’d now been told the brutal facts – that the odds were poor – and they’d heard the ‘transplant’ word. Great Ormond Street had also been mentioned. But nothing happens quickly. They could see that Stefan was in shock and that his organs were failing. Time was their enemy. Fear gripped their throats and lay heavily on the chest to accompany their heart-rending, gut-wrenching misery. Sentences became difficult, then words, and soon it became impossible for them to speak without an outpouring of emotion. But they tried not to cry in front of the lad. Leave that for the end.

  Archer was by this stage stressed and frustrated. He knew the Great Ormond Street doctors well, and although he understood that miracles are hard to come by – other children were waiting in the same situation with their own desperate parents – it would soon be too late. He sifted through the blood tests. The potassium was rising and so was the lactic acid, but he could neutralise this with sodium bicarbonate. Stefan would soon need kidney filtration. Archer did everything to prevent a catastrophic change in heart rhythm, which would certainly precipitate death. What else could he do in this hopeless situation?

  The intensive care consultant waited in the background. Although he’d seen it all before, had looked after many children who’d died, he’d do his best. But what on earth would that be? Soon Stefan would need the ventilator as he was gasping for air, his breathing further depressed by the morphine. So the consultant hovered with anaesthetic drugs and breathing tube at the same time as he was waiting to do the ward round. He had nine other sick babies and children to worry about.

  And then there was Stefan’s nurse. Paediatric intensive care nurses are a special breed, as not everyone is cut out for the job of facing heart-rending anxiety and distress at work, day in, day out. A mature lady with children of her own, she liked to look after my heart surgery babies because they got better. She really didn’t like to watch children die. Clearly she felt for Stefan’s parents – the strain was beginning to show – and someone needed to do something drastic or it would be too late, her young patient’s life ebbing away. She was the one who pressed Archer to find me.

  The atmosphere around the bed was now as thick as fog, with a sense of impending doom. No one can snatch a donor heart out of thin air, especially for a child. Only a handful of children’s heart transplants are done each year, so they’d looked to me for an a
lternative. But it wasn’t going to happen.

  I stared at the grieving parents and felt bloody useless. How would I feel in their position, if one of my kids were in the shit like this, struck down without the slightest warning? Their last hopes had just been dashed. Undoubtedly, having children of my own sensitised me to the plight of anxious families. By then my daughter Gemma was twenty and I had a son, Mark, at school in Oxford.

  Viewing Stefan as if he were one of my children would be to empathise. Then he’d become a person, not just another patient. Some would suggest that empathy is the key to being a good doctor, the ‘key to compassionate care’, whatever that means. But if we really considered the enormity and sadness of every tragedy played out in this unit we’d all drown. That’s why my intensive care colleague needed to get on with his ward round and not get sucked into the whirlpool that was Stefan’s imminent demise.

  Now I was rattled. At the time there was only one ventricular assist device suitable for children. It was called the Berlin Heart and had just recently been introduced by Professor Roland Hetzer at the Deutches Herzzentrum in Berlin. This was fortunate as he was a great friend – one of the benefits of scientific meetings – so I was going to call him and ask for a big favour, maybe tell him that Stefan was German. It certainly sounded that way. Added to all this, Roland was an Anglophile.

  Fortunately he was right there in his office, so I got through at the first attempt. We exchanged the usual pleasantries then I came directly to the point.