Fragile Lives Page 23

  As a further measure she was given the drug amiodarone in an attempt to calm the electrical storm – a good move, but after thirty shocks she still reverted back to ventricular fibrillation. With the situation looking desperate, the on-call cardiologist, Dr Bashir, arrived. He looked carefully at the patient and changed one thing: the position of the electrodes on the chest. He placed one on the front of her chest, over the right ventricle, and one on her back, directly behind the left ventricle.

  One shock of 200 joules, and normal heart rhythm was restored. With adrenaline on board, her blood pressure immediately rose to above-normal levels, but although this had the benefit of increasing blood flow to the traumatised heart muscle it also increased electrical instability. The result? Repeated fibrillation that needed more shocks and a high dose of a beta-blocker to counter the stimulant. Once the electrodes were in the correct position the shocks worked each time. Dr Bashir, an experienced electrophysiologist, then prescribed a combination of powerful rhythm-stabilising drugs in high doses.

  Around two hours after she’d collapsed the girl’s erratic rhythm began to settle and she became stable enough to perform an echo study to obtain pictures of the heart. Whatever this showed would be important. Only a handful of problems cause sudden death in young people. One of the possibilities is an inherited condition of thick heart muscle, but the echo soon ruled that out as both ventricles were normal in size and thickness.

  By now her right ventricle was suffering visibly after the prolonged cardiac massage and electric shocks. It was dilated and contracting poorly, although the heart valves all appeared normal. Very rare coronary artery abnormalities can cause ventricular fibrillation, but from what could be seen of these small vessels they also appeared normal.

  Was she suffering from a primary ventricular dysrhythmia, electrical instability in a structurally normal – albeit now battered – heart? This can present as fainting attacks or sudden cardiac death without any identifiable genetic syndrome. It’s not related to exercise or stress but probably arises from within the heart’s own electrical system, and it can take place in short bursts of electrical instability or as a full-blown ‘electrical storm’.

  If it settled it could be treated by electrical mapping to locate its origin, followed by destruction of the irritable source. This was Dr Bashir’s specialty and would be performed in the catheterisation laboratory. It can be done during the electrical storm if the patient’s circulation can be sustained in the meantime, but this is not easy to organise at night as it needs a highly trained support team.

  The plan was to move her from the accident department to the cardiac intensive care unit. The intensive care consultants were already involved, working to normalise her blood chemistry after three hours of resuscitation. They were anxious that she was sliding into heart failure and wanted my opinion as to whether she’d need mechanical circulatory support.

  I arrived at the accident department at 9.30 pm to find a crowd around her bed in the resuscitation area, most of them spectators doing nothing. The cardiac massage machine was still in place but had thankfully been switched off while her rhythm was normal. Personally, I disliked it. Cardiac massage has its place, but the heart is a delicate organ, and one I don’t like to see being pulped by a machine. By now the intensive care doctors had her well sedated and ventilated, and her blood chemistry had improved because normal rhythm had given her much better blood flow. The cardiology registrar lingered nervously by the defibrillator.

  I’d only been there three minutes when she fibrillated again. This time no pounding on the chest was administered, just a finger on the trigger of the defibrillator. Zap! Her heart returned to its normal sinus rhythm. I suggested that we take her round to the cardiac intensive care unit, away from the circus, and get the sledgehammer back into the ambulance away from her broken ribs.

  After seventy electrical shocks we settled on that diagnosis of idiopathic ventricular fibrillation. By this stage she was beginning to respond to the anti-dysrhythmic drugs, so perhaps it was wise not to move her to the catheterisation laboratory while we appeared to be winning. As the shocks became less frequent, the heart was easier to defibrillate.

  We stayed with her in intensive care, right by her bedside. During the night her parents and boyfriend arrived after a terrible journey down from the north of England, all poleaxed by grief and anxiety. This was the worst part for me. I was watching as the nurses told them the story away from the bed, then witnessed the shock on their faces when they first saw her – on the ventilator, pale with blue lips, big drips in her neck, arms and wrist. This is how intensive care always looks, but it comes as a terrible shock the first time, and even more terrible when it’s your child hovering between life and death.

  Then I heard the quiet descent into recrimination. How could this have happened? She seemed so happy at Brookes. Did she get the condition from us? This was the time for me to ask her parents questions, but I asked my registrar to do it as I just couldn’t face it. I hovered in the background. Had anyone in the family died suddenly? Was there any history of heart disease? Had she had problems before? Each drew a blank.

  I knew what to expect next, which is why I stayed, although I hoped that it wouldn’t happen. When the adrenaline wore off, the electrical irritability lessened but her blood pressure started to creep down, and by the early morning it was worryingly low. Meanwhile the pressure in her veins drifted upwards as the right ventricle – battered and bruised, now buggered – struggled to cope. The urine flow tailed off, as it always does in these circumstances, and the acid in the blood started to rise, produced by the muscles as their blood flow dwindled.

  She needed more shocks, and unfortunately there was no time to usher her parents away. This was a grim reminder to them that she was actually dying. Her hands and legs were cold with the onset of cardiogenic shock that hadn’t come from the dysrhythmia but from the heavy-duty cardiac massage and repeated electric shocks, and certainly not helped by the high-dose beta-blocker she’d needed to counteract the adrenaline.

  I asked for another echo, this time taken through a probe in the gullet. The camera sits right behind the heart so the pictures are much better. Things had changed dramatically for the worse, as both the left and right ventricles were contracting poorly. This is when the ‘what ifs’ kick in. Would this have happened if the defibrillating electrodes had been positioned differently? What if they’d just brought her straight into the hospital so she could be treated sooner by those who could make the diagnosis, then target the drug treatment as my colleague had done? What she’d needed was expertise and drugs, not a mechanical sledgehammer out in the town.

  ‘What ifs’ are no good in cardiac surgery – they simply don’t help. We just have to get on and treat what lies in front of us. I knew what she required now. Her struggling heart was still recoverable but she needed circulatory support, and the only thing we could do quickly was to insert an intra-aortic balloon pump, in full awareness of the fact that it was pretty useless in shocked patients. It went in anyway and slightly improved the blood pressure on the monitor. But she needed more blood flow and the balloon doesn’t provide that. We had to give her the vasopressor drug noradrenaline to keep the pressure above 70 mm Hg, but that soon triggered further episodes of ventricular fibrillation.

  What I meant when I said she needed circulatory support was a ventricular assist device to take over the circulation, the sort of pumps we had before the money ran out. In this case we needed a system called extracorporeal membrane oxygenation – or ECMO for short. This combines a centrifugal blood pump with an oxygenator and is similar to the oxygenator in the heart–lung machine, except that it’s engineered for long-term use and is safe for days or weeks until the heart gets better. We needed this because she had both left and right ventricular failure and her lungs were deteriorating in response to the shock. But we didn’t have one. Only a handful of UK units were funded to use it, primarily for young patients with severe lung disease.
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  By now my own blood was beginning to boil as I saw the despairing parents by the bedside, as I watched the watery spring sunshine break over the horizon, as normal, healthy people were beginning their day, just as she did yesterday at Brookes.

  So what did the recent NICE guidelines for acute heart failure have to say? They said that one should ‘ask advice from a hospital that has circulatory support equipment’. We did. My surgical colleagues who I’d trained said she needed ECMO. But what were the prospects for safe transfer of a dying girl who was suffering ventricular fibrillation at regular intervals? Who’d been shocked seventy times? Whose heart was toast? The odds of getting her to another centre in safety were negligible. No one disputed that fact.

  Given our track record of innovation, my colleagues expressed surprise that we had no ECMO system and said that I simply had to get the equipment brought to Oxford by the company representative as soon as possible. We couldn’t locate the supplier until 8.30 am, by which time her blood pressure had sagged again, with a rise in pressure in the veins. As a result her tissues were poorly perfused, blood flow within the vital organs was critically impaired and acid levels started to climb.

  I debated whether to take her to theatre and put her on a conventional heart–lung machine. Yet this could have been a disaster for a number of reasons. It would further damage the lungs and the ability of the blood to clot. Bleeding is the commonest life-threatening complication during ECMO, and after prolonged standard cardiopulmonary bypass the risks would be even greater.

  But there was one other option that would buy us some time, a powerful heart failure drug called Levosimendan that we’d used in the past. It helps link calcium to the muscle molecules and makes the force of contraction greater, doing so without increasing tissue oxygen uptake or ventricular irritability. I asked the intensive care doctors to start an infusion of the drug, only to be told that we didn’t keep it any more as the hospital said it was too expensive. All we had were drugs to constrict the blood vessels and make the heart more irritable, or drugs that would flog the heart and make things worse.

  The ugly truth was that we were desperately trying to keep this young woman alive without the equipment or the drugs she needed. It was a tense, miserable morning watching the clock tick away, while attempting to reassure the poor parents that we were doing everything we could. We waited for the ECMO equipment to arrive, in the meantime infusing vials of sodium bicarbonate to neutralise the acid and watching her pupils. Did they still react to light? Was her brain getting enough oxygen? Higher doses of arterial constrictor drugs would briefly elevate her blood pressure, hopefully increasing flow to her brain – but all at the expense of her limbs and gut. Her hands and legs were already cold and white, and she had critically low blood flow, with acid pouring into the circulation from muscles starved of oxygen.

  By midday I couldn’t watch any longer. I went round to the operating theatres and told them that we had to put her on cardiopulmonary bypass, hopefully just for a short time until the ECMO equipment reached the hospital. Then someone asked the inevitable. Who was going to pay for ECMO? Who was going to look after it at night? What if?

  I was tired and irritable so I let rip. Who on earth were they to question our efforts to save a twenty-year-old? So bloody what if we weren’t a transplant centre. She didn’t need a transplant. Her own heart just needed a rest from the battering it had received over the last twenty-four hours. Why was this so-called ‘centre of excellence’ unable to save a kid who’d collapsed within a mile of the hospital? It certainly wasn’t through lack of effort by the medical staff.

  Just as I was about to lose it altogether I heard that the equipment had arrived. Our patient was already on her way round to theatre, so I went to meet the company representative who’d made an enormous effort to come and help us. He’d already been in Oxford for more than an hour, stuck in traffic while trying to get into the hospital, then driving round in circles to find a parking space – all with mounting levels of frustration and anxiety. Lost time, lower chance of survival. He knew it and was extremely pissed off.

  Once the equipment was ready it took just minutes to establish the ECMO circuit via blood vessels in each groin. Ultrasound visualisation showed the femoral artery to be narrow, and because of this I chose to surgically expose it and join a vascular graft to its side. This would guarantee that the leg still received adequate blood flow. The femoral vein in the opposite groin was cannulated directly using a needle and guidewire. The long cannula was advanced into her right atrium and positioned carefully using an echo probe in her oesophagus.

  When the pump was turned on her blood pressure immediately rose to 110/70 mm Hg, while the pressure in her veins fell from 25 mm Hg to 5 mm Hg. Although we’d introduced a kidney dialysis cannula in her neck, her urine flow improved in response to increased blood flow. She’d been transformed by the ECMO system within a few minutes – better colour, better chemistry, different patient. I was jubilant, and her parents finally relaxed.

  For the first few hours her pupils remained responsive to light. Then late in the afternoon, when her heart had improved substantially, her pupils suddenly dilated widely and became unreactive to light. My complete nightmare scenario – body better, brain buggered. Starved of blood and oxygen, her brain had started to swell. The pressure within the bony confines of her skull went up and her brain stem herniated into the spinal canal. Medical jargon for fucking disaster.

  At the time I was lying on the sofa in my office, hoping that the battle was at last over. Sue, my secretary, knocked tentatively on the door before she headed for home. The intensive care unit were asking that I go back, a message that always gives me that sinking feeling. No one calls with good news – it’s always trouble. I expected bleeding, or something that I could sort out. But when I reached the bed space the curtains were completely pulled around.

  Her parents were sitting on either side of her, each holding a hand, and now completely exhausted both physically and emotionally. I needed to know what the issues were before I disturbed them. The distraught nurse looking after her came out to talk. Her pupils had blown quite quickly, and I needed to know the cause straight away – whether she had intra-cerebral bleeding from the heparin anticoagulation or a swollen brain through lack of oxygen.

  A brain surgeon might be able to help with the first, by removing the blood clot. The second would more than likely signal a fatal conclusion to our efforts, just when we’d beaten off the ventricular fibrillation. Four hours had passed since the last shock, and now we needed to go to the brain scanner as soon as possible. I went to arrange it myself, then asked a brain surgeon colleague to come and look at the results with me.

  Brian scans emerge slice by slice, showing multiple cross-sections through convoluted grey and white matter. It’s complex but well-charted anatomy, each part responsible for part of our lives, some parts more important than others. The skull is a rigid box, so when the brain swells something has to give. The fluid spaces are compressed and disappear, the delicate brain appendages and nerves are distorted, and eventually parts of the brain stem are pushed out of the skull, hence the loss of the pupils’ reaction to light. And when brain stem reflexes are lost, the patient is dead.

  The whole scan was finished in minutes, then the slices were computed into a three-dimensional reconstruction of the whole organ. It told a story I didn’t want to hear. ‘Severe brain swelling with herniation of brain stem through the foramen magnum,’ was the official radiologist’s report. I tried to persuade the brain surgeons to take the top of the skull off to decompress the brain. They were sympathetic but said it was too late. Sorry about that. But not as sorry as I was.

  We pushed her back to the intensive care unit. This was quite an undertaking in itself with all the equipment – ECMO circuit, ventilator, balloon pump, monitoring equipment – and we moved slowly now in a sad procession.

  What were we left with? All her other organs were recovering. She was warm and pink, flo
oded with well-oxygenated blood from the machine, kidneys making urine, gut absorbing food and liver removing toxins. All organs need blood and oxygen, and ECMO – this simple, inexpensive technology – supplied both in abundance. But it had been too late for the brain. The cells that we’d failed to save were the cells that mattered the most.

  I was very bitter about this. No other team in the UK had our breadth of experience, had put in the same amount of hard graft in the laboratory, had made the important discoveries. But that didn’t matter. What mattered was that we weren’t a transplant centre, so we weren’t eligible for funding. What mattered was keeping down costs. Death comes cheap.

  I couldn’t face telling her parents. I took the coward’s way out and went back to my office, black as thunder. The intensive care doctors did their best to treat the brain swelling with drugs but they were just going through the motions. The die had already been cast. ECMO was withdrawn after forty-eight hours because of the brain death issue. I took the tubes out myself. By now her heart was working well – good blood pressure, normal rhythm, no ventricular fibrillation. That battle had been won.

  After formal tests for brain stem death, the issue of organ donation was raised with the desolate parents. Apparently the girl had previously expressed the wish to donate her organs in the event of her premature death, and her parents were in accord with her wishes. Before this I went to see her while they were still there. The nurse who’d helped us battle for her life was also at the bedside as she wanted to stay to the end, to see it through and support Mum and Dad. Uncommon decency. That takes moral fibre and courage.

  What could I say at this point? I was really sad. My son was a fellow Brookes student of similar age. How would I have felt in their shoes? I didn’t have to think about that – I’d faced so many bereaved parents that I already knew. What I told them was this. I was really sorry for their loss. Given the difficult circumstances, an experienced team of consultants had fought day and night to turn things around. All of my colleagues were devastated by the outcome and we appreciated the kind offer to donate her organs. It was a gesture that would transform the lives of others.