Fragile Lives Page 18

  He wasn’t too worried, it’s a perfectly rational response to a difficult set of circumstances. He wasn’t surprised by it. He advised me to try and think about what I was doing without trying to put me off. He challenged me – are you sure you mean it? I did mean it, but not sufficiently to overcome my fear of the actual process.

  My endearing cyborg was drifting in no man’s land. Seven and a half years after the implant we were way into unchartered territory. Previously no one had survived with a mechanical heart for more than four years. Peter said, ‘The procedure lands you in a position that no one has ever endured – what life on a battery does to you as a person. You’re an invented entity trying to cope with it, trying to deal with the emotional context of it. You become cold-hearted.’ He admitted that he now had a careless attitude towards money – ‘You don’t care if you’ve overspent on your credit cards or not. If you don’t have any time left you might as well enjoy it. You think, what the hell, if I want something I’ll have it.’

  Much of the money Peter raised charitably he spent on visits to international conferences, and at these he was a revered figure, a driving force behind the new technology. Yet the last paragraph in the Washington Post piece was revealing:

  Things get normalised. You no longer see yourself as odd or outside the norm. Being snatched from the brink of death and transformed into a poster child for cyborg life, despite serious psychological transformations, has been quite an experience. A roller coaster. Better than being dead, I think. Three days out of five.

  By now Peter had a job with the Birmingham Settlement to help homeless and deprived people. At the same time, he was working to establish a spiritual retreat in the mountains of Wales, had undertaken a ninety-one-mile charity walk, and hiked in the Swiss Alps and the American West. Our ‘dead man walking’ lived for almost eight years after his operation. As a result, the United States and many European countries adopted these miniaturised rotary blood pumps as an alternative to heart transplants. Many patients went back to work. Sixteen years later, with all our accumulated expertise in managing pump patients, we’re on course to achieve equivalent survival times between mechanical hearts and heart transplants.

  Peter died within weeks of the Washington Post article. I was away in Japan, working to introduce ventricular assist devices in a culture that did not accept transplantation. His death was not pump-or heart failure-related. He simply suffered a profuse nosebleed that caused his single diseased kidney to fail. He could easily have been put on dialysis – we’d previously dialysed him for a week after his first operation – but the local hospital declined to intervene. Without treatment, the levels of potassium and acid in his blood caused his own heart to fibrillate and the pump was switched off. Had I been in the UK we’d have taken over his care and treated him. I regarded this as a wholly unnecessary death.

  We asked Peter’s wife Diane for permission to perform an autopsy so we could investigate the long-term effects of pulseless circulation. The pump itself was pristine – there was no blood clot and minimal wear on the rotor bearings. We returned it to Rob Jarvik in New York, where it has continued to work for years on a bench testing apparatus. Peter’s own left ventricle remained hugely dilated, still functionally useless. The only finding related to the pump was the thinning of the muscle layer in the wall of his aorta. As he’d had little or no pulse pressure his aorta didn’t need the thickness of muscle that the rest of us have, a perfect example of how nature adapts to circumstances.

  Peter left an important legacy. His experience confirmed the enormous potential of mechanical blood pumps to provide a good quality of life for the many thousands of patients with severe heart failure who are not eligible for a transplant. There are few if any ethical dilemmas, however hard you search for them. The reality is that patients targeted for this treatment have short, wretched lives.

  Peter made it clear that extra life is not ordinary life. There’s a price to pay and a second dying to come. But he was the first to reveal the true potential of blood pump technology, and I was pleased to play my part in something that most people believed to be impossible. He was a truly remarkable man.

  11

  anna’s story

  Body and mind, like man and wife, do not always agree to die together.

  Charles C. Colton

  My work is to help others at the most vulnerable stage of their lives – after they’ve discovered that they have a serious heart problem. When they meet me it’s clear to all of them that they could die, indeed some expect to. One lady was so certain of it that she made it happen after a completely straightforward operation. Never underestimate the human mind. It’s a powerful thing.

  One thing is for certain. For the patient and family, every single professional contact is emotionally charged. This was never more the case than for Anna. She’d had a difficult start in life. Her mother died when she was just eleven months old, but she was fortunate to have two other strong characters in her life. Her father David brought her up in a peaceful Oxfordshire village close to the church – and not just geographically – and later on her husband Des supported her through thick and thin.

  Seven months after Anna was born her mother suffered an extensive stroke. It came completely out of the blue in her mid-thirties, and why it happened at such a young age was never explained. It was the last contact she had with her baby daughter. When David was told that his wife was dying he went straight home to wash the nappies.

  Yet Anna recalls a happy childhood – holidays in Yorkshire and Guernsey, Sunday afternoon walks, picnics and outings. David taught her about nature and the great outdoors, where she discovered an affinity for birds and plants. She worked hard at school, but religious and social activities in the village were much more appealing than books. Above all, she adored small children and babysitting. In church she was the one who held the new babies, as well as ringing the bells – a longstanding family tradition.

  Like my own mother, Anna left school and became a bank clerk. She started early in the morning and often worked late. She put her heart and soul into everything. As her father put it, ‘Anna’s inner strength and perseverance most likely came from my influence, and I’m proud to accept it is so.’

  Anna met her husband Des when he was out walking his dog in the village. They fell in love, got married in July 1994 and bought a house. She was twenty-five, and was happy both at home in the village and at the bank.

  Then, very suddenly, seven weeks or so after their wedding, she started to feel tired, at times absolutely worn out. She put it down to the long hours at work. Then there were inexplicable bouts of sudden severe breathlessness that were attributed to panic attacks. Out of the blue a sore red spot appeared on her toe. It blistered and became infected, and although antibiotics fixed the infection she wondered what could have caused the blistering, which still remained. Unbeknownst to her at the time, these were the classic symptoms of a rare and life-threatening condition, the same one that her mother had suffered from. But no one took the trouble to find out, and life took over.

  At 9 am on 29 August 1994 Anna was in bed nursing a violent headache. Not a hangover – she didn’t drink. Des was reading the newspaper downstairs and she recalls that Skippy the Bush Kangaroo was on the television. The room suddenly started spinning, and she felt that she was losing her grip on reality and going to a strange and different place in her head. She just managed to shout out to Des downstairs to call the doctor before everything went black. Anna could hear Des on the telephone and the anxiety in his voice worried her. She felt she needed an ambulance. Her brain knew what she wanted to say but her voice and mouth wouldn’t cooperate. It was as if her brain had been separated from her body, which was now lifeless and unresponsive. The experience was terrifying for her.

  Anna was rushed directly to the John Radcliffe Hospital in Oxford, where she appeared to be unconscious and paralysed on arrival. The paramedics wheeled her straight into the resuscitation area. ‘Airway, breathing, circul
ation’ is the rescue mnemonic, the medical ABC. Every doctor, nurse and paramedic learns this.

  The doctors passed a tube into her windpipe to prevent her choking to death, then started to breathe for her with the mechanical ventilator. Her pulse was steady and strong, her blood pressure elevated – high blood pressure that goes with brain injury. So the circulation part was fine. Or was it? Did anyone listen to her heart or notice her blistered toe? Was her mother’s death factored into the equation? To be fair there had been no time to look into the family history yet. It was a matter of saving Anna’s life first, then afterwards establishing the cause of the catastrophe.

  Diagnosis is like a jigsaw puzzle. You need to find the pieces first, then fit them together. Only then does the full picture emerge. Anna had clearly suffered a sudden catastrophic brain injury. In young people this is usually caused by a bleed into the brain from a congenitally weakened and ruptured blood vessel.

  But there’s a second possibility, an event known as a paradoxical embolism. An embolus is a piece of foreign material floating around in the bloodstream; broken bones can release globules of fat from the bone marrow and blood clots can detach from a deep vein thrombosis in the legs then float to the lungs. Should air enter the circulation through a cannula and drip it can block blood vessels leading to the brain or cause an air lock in the heart. A paradoxical embolism is where a blood clot breaks off from the veins in the legs or pelvis, but instead of floating to the lungs it passes through a hole in the heart to reach the brain, which can cause a sudden and sometimes fatal stroke. Anna needed a brain scan with a view to emergency brain surgery. There was one positive sign, however. Her pupils were normal size and reacted to light. She was not brain dead.

  The brain scan was performed with injected dye to show the arteries within the brain. This reveals its magnificent architecture, like the branches of an oak tree – but a tree of life with a branch sawn off, as one blood vessel had come to a premature stop, although there was no bleeding. It was an embolus lodged in a critical artery supplying the brain stem, cutting off flow to that vital nerve centre.

  A crucial mass of white matter was already dead or damaged, including the nerves to the arms and legs, the nerves controlling speech, together with those that govern the body’s automatic reflexes. She appeared to be deeply unconscious, and probably blind.

  Yet how could Anna be able to hear and think when she appeared to be completely gone? This is something straight out of a horror movie, like being buried alive in a coffin with a window – the dreaded ‘locked in’ syndrome, involving the complete paralysis of the voluntary muscles in all parts of the body except those that control eye movement. And worse still, only vertical movement of the eyes and blinking remain. Yet there’s no damage to the thinking brain – the cerebral cortex or grey matter – and the patient remains alert and fully conscious, still able to think but mute and immobile. It’s a nightmare scenario.

  Anna never did lose consciousness. Her vocal cords were not paralysed but her ability to coordinate breathing with speaking had gone. So while to the outside world she seemed to be in a deep coma, from Anna’s perspective her hearing and thought processes carried on as normal. Understandably, this trapped new life was terrifying to her. She could see out – although those around her were complete strangers – and she could hear a persistent, intermittent beep, which was the monitor. As her nervous system lost control she felt cold inside, even though she was covered in warm blankets. It was as if her body had been frozen and strapped down.

  She recalled an olive-skinned man in a green top and trousers who was trying to put a tube into a vein in the back of her hand. He seemed to be digging about and it hurt. She couldn’t move a muscle or make a sound, but she was screaming inside. He didn’t speak to her, and it was as if they were in completely separate worlds. Anna wondered if she was dead but being experimented on. Where was God or Heaven now?

  And where had the embolus come from? If it had originated from veins in her leg there would have to be a hole in the heart to let it through from the right side of the heart to the left. Many healthy people have a small hole between their right and left atria that’s left over from the foetal circulation in the womb; it’s there to divert blood from the right heart to the left before the lungs expand at birth. Anna needed an echocardiogram. Indeed all stroke patients should have one, not least so further similar episodes can be prevented by closure of the communication.

  Anna’s scan told the story, linking her own condition to her mother’s early demise. There was a huge tumour filling her left atrium. Although it was fragile looking, like a delicate piece of seaweed, it was forced into the mitral valve every time the atria contracted, effectively obstructing the left side of her heart. This would explain her breathlessness and tiredness.

  Her infected toe also began as an embolus, a small piece of the fragile tumour having broken off as it pounded into the valve. The next fragment went north, not south, directly through the carotid artery to the basilar artery and the brain stem – a catastrophic route that a self-destructive sat-nav couldn’t have selected any more accurately.

  I’d operated on many heart tumours, rare though they tend to be. Anna had a myxoma, which are common but benign. They’re often fragile like hers, so bits break off. Many cause a stroke as the first symptom and are operated on immediately upon discovery for this very reason. Fortunately, most myxomas never come back after removal.

  The cardiologists were called to see her. Dr Forfar wanted me to remove the tumour as a matter of urgency. I was moved by Anna’s story and by the sight of her lying paralysed in bed. Her eyes were open, with a blank stare – no movement, no response. Ironically, when I held a stethoscope to her chest I could hear the murmur of her obstructed mitral valve and the ‘plop’ of the myxoma into the orifice. Had no one listened to her heart before? At that stage we didn’t really know about her neurological prognosis. We tend not to operate on patients after a recent stroke as the anticoagulation for the heart–lung machine can cause more bleeding into the brain, but then again there was the very real risk that soon more fragments of her tumour would embolise and prove fatal.

  It was really a decision for Des and David, Anna’s husband and father. Did they want me to do it even if the prognosis was poor? This was very difficult for them – they were shell-shocked, and David had already lost his wife; now his precious daughter was in the same situation. They both wanted Anna to have a chance. What did I think, they wondered. I felt there was absolutely nothing to lose by operating, so when they decided that we should go ahead with the operation I took her to theatre that same afternoon.

  Anna had a small, vigorous heart that was beating away and looking completely normal from the outside. On the inside, however, it was a land mine primed and ready to explode. It was important not to touch it and disturb the delicate fronds of tumour before their escape route could be blocked by a clamp across the aorta.

  First we went onto cardiopulmonary bypass to support the circulation and empty the heart. Next I applied that clamp to halt blood flow to the coronary arteries, with cardioplegia solution stopping it altogether. With the little heart lying flaccid and cold, I opened the right atrium. Heart surgery is simple – or it should be.

  The myxoma was attached to the other side of the partition between the right and left atria known as the atrial septum. The safest way to approach it was to cut away the septum and locate the base of the myxoma. There’s often a short stalk between the septum itself and the mass floating in the blood, the aim being to remove the whole thing so it can’t grow back. This is best done in two steps – cut the stalk and lift the fragile lesion out gently without breaking bits off, then excise the whole base, which is precisely what we did. I proudly dropped the tumour into a container of formaldehyde preservative, a present for the pathologist to check that there was no malignancy. I’d operated on patients where the benign myxoma grew back and turned malignant. Rare, but it can happen.

  With the tumo
ur gone, Anna’s heart separated easily from the bypass machine and we closed her up, leaving her badly wounded but safe from further damage. The surgery itself was not the greatest challenge. As a quadriplegic patient Anna’s ability to get over the operation remained in question. She couldn’t respond to instructions, and we’d no idea if she could breathe independently or whether she could cough. To lie flat and immobile is a recipe for chest infection and pulmonary embolism from thrombosis of veins in the legs.

  We’d have to work hard to bring Anna through this journey, and as well as us it was a task for the physiotherapists and her friends and family. They were encouraged to talk to her and play her music, even though she gave no sign of being aware of anything whatsoever. When Des put earphones on her head to play her music from the local radio station there was no response at all.

  Remarkably, however, Anna was aware of everything around her. As the anaesthetic drugs wore off she could see and hear again but still couldn’t move. Worst of all, she felt pain that she was unable to communicate. To any observer in the outside world she remained in a deep coma.

  One night when Anna was lying there sweating, a new nurse changed the sheets on her bed. In kindness she stroked Anna’s head and said, ‘I’m sorry I can’t do anything more for you.’ Anna panicked inside, taking this compassionate comment to mean that she was dying. On another occasion a less sympathetic nurse said, ‘She looks dead!’