Fragile Lives Page 15

  ‘Roland, I need a Berlin Heart. The boy is ten but small for his age. There’s a chance that the heart might recover but he won’t last much longer. What will it cost me?’ I knew that the money would have to come out of my charitable funds.

  His response was as expected. ‘Let’s worry about that later. When do you need it?’

  There was a brief pause. ‘Could you get it to me by tomorrow morning, with one of your guys along to help?’

  Roland was only too pleased to help.

  The Lear Jet landed at Oxford airport at 8 am the following morning. In the interim I’d sent a message to our hospital chief executive with a copy to the medical director to announce my intentions. The insightful Nigel Crisp had moved on by then, and it was less than a month since I’d been threatened with the sack for saving Julie.

  Archer did the honourable thing and went to see them both to try to persuade them it was our only option. The collective body of medical opinion had agreed that the boy could be dead by the end of the day, he informed them, and he’d exhausted all of the conventional channels. No one would help. If Westaby had a solution, he continued, they were morally obliged to let him get on with it. Action first, recriminations later. Oh, and by the way, had they visited Julie Mills yet on the ward? A world’s first for Oxford, wasn’t it? And if not, why not?

  Archer was and remains a religious man. He held back from the ‘resurrection from the dead’ analogy and agreed with them that not only was Westaby not God, but that he might indeed even be an irritating son of a bitch. But wasn’t it his job to save lives? That’s all he was trying to do. So back off for now. Let the Germans come.

  For my own part, I held fast to the concept that it was ethical to save lives whatever it took. I didn’t need an anal ethics committee to cast doubt on that. And I didn’t care about being sacked. I needed to work where I could fulfil my potential, pushing the envelope. If Oxford couldn’t back it, I would go somewhere else!

  The Berlin Heart consisted of an orange-sized blood receptacle divided into two parts – blood on one side, air on the other, forced inflation of the air chamber driving blood onwards through the valved tubing. Simple, but highly effective. The pump chamber sat outside of the body and could be exchanged if blood clots were detected within it. Inflow and outflow tubes connected the pump to both sides of the heart, and all the tubes passed out through the abdominal wall from the failing heart to external pumps. Then both ventricles were bypassed and rested, with guaranteed blood flow to the lungs and body. Just what the doctor ordered, I guess.

  Now I needed to get Stefan down into the operating theatre. And not only that. The Lear Jet was waiting to take the German team home after the surgery and I was paying for it. Not exactly a black taxi cab with the meter running.

  Stefan had managed to survive the night without being put on the ventilator. Now he was physically exhausted and very scared. At his age he had an appreciation of his predicament – he could read the long faces and his mother’s tears – so there was an emotional separation in the anaesthetic room, the sort I prefer to avoid. Children’s anaesthetists deal with this every day, but for me it’s added pressure that I don’t need, so I took the German team to change into theatre gear. This was embarrassing in itself – a scruffy room jammed full of grey lockers, brown wooden benches with paint peeling off, plaster coming adrift from the walls of the toilets, discarded theatre shoes, masks and clothing everywhere. What shoes would they wear? We searched around until we matched a couple of pairs, then went into the perfusionists’ room to show them the kit.

  Desiree was already there, ready to learn, and the surgical registrars were waiting with Katsumata. There was already an air of frenetic excitement, a sense of breaking new ground, something to tell their partners and kids about when they went home. Would it be on the news tonight? No. Maybe the local Oxford news? No. Would I be getting the sack? Quite probably. That would make the news. But we’d say nothing at this stage. Let’s just get the boy better.

  Stefan was a sorry sight as he was wheeled in on the operating table – so thin, almost pathetic. By this point I was sure that he wasn’t suffering from viral myocarditis; it could only be severe chronic heart failure, with a heart muscle pathology that was unlikely to recover. Step one remained the same. First keep him alive, then take stock.

  I ran the saw up his sternum and cranked the edges apart with the retractor. We opened the pericardium and tagged the edges to the skin, bringing the heart up towards us. Lots of straw-coloured fluid spilled out. I reckoned that about a quarter of his body weight was excess heart failure fluid, full of protein and salt, and now discarded by the sucker. I wondered if I was a fool to have become involved in this world of misery. There were easier jobs.

  Now I had a good view of the struggling, dilated organ. His right atrium was tense and blue, ready to burst from the high pressure in his veins, and swelling his liver. His right ventricle was distended, and I looked carefully at the right coronary artery to rule out the possibility that he had the same problem as Kirsty. But he didn’t, and in any case Archer would have spotted it if he had. There was no scarring in his huge left ventricle, just pale, fibrous-looking muscle that had given up. Nor was his heart swollen and inflamed like Julie’s. I’d biopsy the muscle, then we’d see precisely what the problem was under the microscope.

  The Germans were looking over the drapes at the top of the operating table. As two of Roland’s elite transplant team, they’d seen many similar struggling hearts in Berlin. They used the generic term ‘idiopathic dilated cardiomyopathy’, a quite unusual condition in a child of ten.

  It was already clear that Stefan would need both right-and left-sided support to keep him going. A left heart pump alone would deliver more blood to the body, but it would all come back through the veins to the right ventricle, which would then pack up because it couldn’t cope. So right-sided support was essential. There would be four tubes coming out through the abdominal wall to two air-driven prosthetic ventricles that would passively fill, then forcefully eject blood, providing a similar volume and pumping rate to a normal child’s heart. Good or what!

  I sensed that his buggered organ would not tolerate manipulation as we’d provoke rhythm changes, making things worse before the pumps could be connected. I’d put him on cardiopulmonary bypass first to keep him safe. I then tried to lighten the atmosphere with a joke.

  ‘I just deleted all the Germans from my phone.’ Pause. ‘Now it’s Hans free!’

  They didn’t get it. Nor did Katsumata. We all pressed on in silence, making the four stab holes to bring the cannulas out of the chest, fixing one end to the heart and the other to the pump. Most importantly, we emptied the system of air. I attempted another joke.

  ‘What’s the difference between a hippo and a Zippo? One is very heavy, the other a little lighter!’

  But once again, not a titter.

  Everything was completed according to plan and the time for the switch-on had come. The prosthetic pumps were like normal ventricles but sat outside the body, where you could watch them work – lub dub, lub dub, lub dub. Energetic and effective. Stefan’s own heart emptied like a deflated balloon and there was much better blood pressure now, with vigorous pulsation in the aorta and pulmonary artery. Lub dub, lub dub, lub dub. It was a ridiculously simple approach, but an outstanding result – a triumph of life over death. Although there was something more aesthetic and satisfying about pulsatile flow, with Stefan the pumps had to be external. At least continuous-flow devices were small enough to implant within the body.

  Katsumata took care to ensure that there was no bleeding, squirting biological glue around the incisions to stop the tedious oozing. We needed to leave two drains in Stefan’s chest to let the blood out, so there were now six tubes sticking out of his fragile little body. This meant he’d had multiple stab wounds, but they were all necessary. The usual thick, stainless steel wire stitches were used to draw the edges of the sternum together, pulled tight and twisted to
cover all the hardware inside.

  Stefan was then taken back to the paediatric intensive care unit, where his ventricular assist device was the first they’d ever encountered. It could have been intimidating for the nursing staff, but it wasn’t. We told them not to focus on the tubes or the knobs on the console and that there was no need to change anything. All they had to do was take care of the boy, particularly as he’d be alarmed and agitated when he woke up.

  We also emphasised that it was vital that Stefan didn’t pull on the tubes, as they were his lifelines. When he awoke it would be best just to sit him up, take him off the ventilator and remove the tracheal tube – one less cause of discomfort. Then it would be possible to reason with him and be easier to keep him calm. His parents could sit with him and Desiree would be close by to help, even when she was off duty.

  With that the Germans were gone and we were left on our own with the technology. No problem, as Stefan improved rapidly. His urine flowed again into the catheter bag and, as anticipated, he woke up in the early evening and had the tracheal tube taken out. He was pretty cross – even with his poor mother – but he was pink again, with rosy cheeks, warm legs and warm hands, which his parents held so tightly. He was just not keen on the aliens emerging from his belly, pulsating away in front of his nose – priceless, lifesaving technology, but pretty daunting for a kid.

  As the days passed I was eager to know what the biopsy showed so we could work out the next step. The Berlin Heart would keep him alive for weeks or even months, but could his own heart recover? I suspected it wouldn’t, so in the background we needed to plan for a transplant. Ever curious, I went to the pathology laboratory myself and asked to look at the processed specimens from Julie and Stefan, in the same spirit that I always went to the autopsies of patients of mine who died. The pathologists knew me well enough and they appreciated the clinical feedback.

  Julie’s heart muscle was densely infiltrated with a type of white blood cell called lymphocytes that respond to a viral infection. While viruses are too small to be visible under a light microscope, lymphocyte infiltration tells you that they’re there. There were millions of them present, and the muscle was swollen and oedematous with the inflammatory process.

  This was not so with Stefan, which came as a bit of a shock in a ten-year-old. Much of his muscle had been replaced with fibrous tissue, but not as a result of a lack of blood supply. There were no white blood cells at all. Stefan did have chronic idiopathic dilated cardiomyopathy – a long-term enlargement of the heart, cause unknown – and his condition would never improve simply with rest. He’d simply hit the buffers. Julie and Stefan had only one thing in common. We’d got there just in time. But the way forward was now clear – Stefan needed someone else’s heart to go home with.

  In those days – as now – neither an individual hospital nor a surgeon on their own could organise a heart transplant, not even if a suitably matched brain-dead donor was lying in the bed next to the patient needing the heart. There was a decision-making process to follow and an organisation to take on – the UK Transplant Service. They’d decided that, in order to make better use of scarce donor organs and to ensure equitable distribution, the ‘urgent’ category of patient should be abandoned. So, at the time, donor organs were offered to the transplant centres on a strict rotational basis. Many of those who received a donor heart were out in the community – not on a life support device like Stefan. We now know that these ambulatory patients gain little or no survival benefit from a transplant and many die from complications afterwards. Wasted organs, and one reason why I was driven to find an alternative. What’s more, if a heart transplant took place involving an organ offered informally, then the transplant unit concerned had to report it to the UK Transplant Service, who would then place them right at the bottom of the waiting list.

  I was growing increasingly concerned about finding a heart for Stefan and I had to get him into the Great Ormond Street system. I called the transplant surgeon Marc de Leval, whom I’d trained with and greatly respected, and who in turn was supportive of the fact that I’d developed a congenital heart service from nothing in Oxford. Over the years I’d send him any complex case where I felt he could do a better job than me, as there’s no place for pride or arrogance when operating on small people. I explained that we’d already tried to transfer Stefan before he’d taken a downward turn.

  Marc knew all about this and was willing to help. He was also interested to see a Berlin Heart. Although Stefan was now stable, he was in such a parlous and unpredictable state that he could be added to the Great Ormond Street transplant list, just as if we’d managed to transfer him the previous week.

  But there was a problem. Transferring him to London while he was on the Berlin Heart was going to prove unreasonably hazardous. When we asked the ambulance service to take him there, they couldn’t guarantee sufficient electrical power to cover the transfer time, given the risk of getting stuck in traffic or the vehicle having a mechanical breakdown. So we’d need to make preparations with the Oxford transplant coordinator, confirming the blood group, arranging the tissue typing and looking for unusual antibodies in his blood. If we found a suitable donor heart we’d transplant him in Oxford, meaning that the medical director would almost certainly have a stroke.

  The endgame came sooner than we expected, but we were ready for it. Stefan had improved day by day, still feeble physically but no longer in heart failure. The following weekend we received the transplant alert. Only thirty miles down the motorway, Harefield Hospital were preparing for a heart and lung transplant in a teenager with cystic fibrosis who was severely debilitated and dying from lung failure. She’d been on home oxygen for several years but was now bedbound, blue and gasping for air, with high pressure in the lung circulation and regularly coughing up blood. When she received a heart and lung transplant her own strong heart could be given to Stefan. This was the plan, a procedure like this being known as a domino heart, for obvious reasons. Domino transplants were rare then, non-existent now.

  The cystic fibrosis patient was brought into Harefield while the organ retrieval team stood by. The logistics were nothing if not complex – the donor was many miles away and four separate surgical teams would be involved, for heart and lungs, liver and two kidneys. These would all be heading for different cities and were like vultures hovering above their prey, ready to consume the best bits of the body, albeit with the noblest intentions. They were all travelling at night and their journeys were not without danger, airborne transplant teams having been lost on occasion in bad weather.

  When it was established that the heart was a tissue match for Stefan, and that he and the cystic fibrosis patient shared the same blood group, the process was scheduled to kick off during Saturday night. Could anything be better than that? We’d operate in Oxford on a quiet Sunday morning with minimal fuss.

  Better still, the heart was not subject to the adverse physiological consequences of donor brain death. Head injury donors had often been subject to fluid restriction and diuretic therapy to reduce the pressure in their skull, and this, together with pituitary gland damage, often prompted the need for resuscitation with several litres of fluid. Many needed massive drug support to maintain adequate blood pressure, so as a result these compromised donor hearts often failed in the post-transplant period. I’d worked at Harefield for three years and knew the score.

  The Great Ormond Street transplant coordinator would keep us abreast of the timing. The domino heart would be removed from the heart and lung recipient at around 7 am, and by the time it arrived in its plastic bags and cool box Stefan’s chest would be reopened and ready. He’d go onto the heart–lung machine to remove the Berlin Heart, and we’d chop out Stefan’s useless organ, cannulas and all. My team would be in early and raring to go.

  Ten was a difficult age to face something like this, but Stefan understood the situation, expressing relief at the prospect of having the aliens out, and then resignation. And trepidation. He hated havi
ng four tubes the size of hose pipes sticking out of his belly, blue blood streaming through one pair, then bright red in the other, as well as noisy, pulsating discs in front of his nose. We’d originally told him that he might need to spend months with all this equipment on, so the early transplant was a happy release.

  But we didn’t tell him the risk of not getting through, which in those days was 15 to 20 per cent following failure of the donor heart, infection or rejection. But this domino heart – from a live person with a normal brain – was particularly strong. And it was a reasonable match on the tissue typing. No alarm bells. We just needed to get on with it. Stefan’s parents had been sitting with him since 6 am, and had spent most of the night awake, getting more and more anxious despite their underlying hope. As the tension mounted for them, they transmitted this anxiety to their son.

  I took Marc to meet them. By now they were with Stefan in the anaesthetic room, where there was not a lot of space with the equipment and everything else. Marc’s eyes kept flicking back and forth to the Berlin Heart, at this stage the only ventricular support system suitable for small children. Great Ormond Street needed to get one so that lives were not wasted.

  Katsumata appeared at the door with news – the domino heart had left Harefield. With the light Sunday morning traffic it would reach Oxford in thirty minutes, so it was time for Stefan to be put to sleep. The moment of parting had now come, acutely distressing for the parents and – briefly – for Stefan. Kate, the anaesthetist, was poised and ready. Anaesthetic injected into the drip and soon his mental anguish was gone. A nod from Louise, the anaesthetic nurse, and the parents shuffled out of the door. Huddled together, their distress would continue for a while. As if they hadn’t had enough already.

  After that things happened quickly. My scrub nurses Linda and Pauline painted his chest with pink chlorhexidine antiseptic solution and dried it because the liquid is inflammable. They then covered him in sterile green drapes. Marc, Katsu and I scrubbed, gowned and gloved. The clock was ticking.