Sophie was kept heavily sedated on the ventilator for three weeks, which helped to keep her blood pressure under control. Then the dreaded fungus candida crept in under the radar. We discovered it first in the urinary tract, where it could prove life threatening if we were unable to control it. Now the so-called period of stability was turning into a nightmare, so we felt it was time to initiate some forward movement to reduce the risk of more complications. The pump was doing its job splendidly and it was time to let her wake up.
When the sedation was switched off, Sophie woke up promptly and responded to her parents. With awareness came higher blood pressure, which we could control, and there was still no bleeding. The poor lass was clearly terrified by her plight, and I suspected that there were signs of brain injury. We tried to explain why she had pipes full of rushing blood coming out of her belly, assuring her that she was safe and that we would shortly remove them. Soon afterwards the nurses noticed that she was not moving her left arm or leg – they were paralysed and didn’t respond to pain. Either infected material or blood clot from the damaged heart wall had found its way up the arteries to her brain. She was paralysed at fifteen. Why did we deserve that?
This was devastating for her parents, and the Battle of the Bulge was now beginning to take its toll on me. Why would anyone want to do this job, day after day, night after night? Healed heart, buggered brain was not what I had hoped for.
The following day I took Sophie back to the operating theatre to remove the cannulas from the left ventricle and aorta. I was delighted to find that there was no clot in the centrifugal pump or the rest of the system and that Sophie’s heart looked great on echo, apart from a residual crater beneath the mitral valve, which was now a blind-ended pouch. Miraculously, after all the cardiac massage and surgery, the repaired valve had held up and was still functioning well. I gave the chest and pericardium a good washout with antiseptic solution, while once again assiduously avoiding the back of the heart. Then we closed her up for the last time. This was the end of Sophie’s marathon surgery, the final phase of a long, bloody battle of relentless attrition. We had succeeded in keeping her alive, but at what cost?
For the family’s sake it was important to stay positive as they were all shell-shocked. Mother Fiona had witnessed all three resuscitation attempts, each time enduring the agonising wait to see if Sophie would come out of theatre to intensive care or to the mortuary, dressed in her white hospital gown or wrapped in a shroud. Right now it was Westaby 3, Grim Reaper 0. Did we have a plan to keep it that way? There was absolutely no precedent. It was a unique case.
As I saw it we needed to do two things. First, continue to screw down the blood pressure, with Sophie’s own heart supporting the circulation. Second, keep her brain well supplied with oxygen to minimise cerebral damage. The brain possesses more resilience than most people expect, and the majority of strokes recover substantially, with improvement more likely in a young person. This was the positive message that both the nurses and family needed to hear. They all needed some hope that she wouldn’t be bedridden for what remained of her life.
After considering the options, I decided to play safe by insisting that Sophie stay asleep on the ventilator for another two weeks. We would only move her for a brain scan when I was satisfied that we could do so without risk. In the meantime she was still not passing urine and needed continuous kidney dialysis. Kidneys don’t like sepsis and low blood pressure, but they always recover. With the hardware out, it was time to move her back to paediatric intensive care, a smaller unit with a more consistent nursing team and mercifully more peaceful nights.
Sophie’s brain scan showed several small areas of focal damage with swelling around them. These were probably caused by embolisation of infected debris from the heart, and it was important to try to prevent them turning into cerebral abscesses. She would be kept on the intravenous antibiotics regime for another six weeks, which is routine for endocarditis, and the inflammatory swelling around the damaged areas would undoubtedly disappear. Would Sophie remain paralysed? We received the standard answer from the neurologists – they hoped she wouldn’t but only time would tell.
On 1 April, the day of fools, Sophie’s sedation was finally withdrawn and she woke up promptly. She could breathe well enough and respond to commands, so we proceeded to remove the tube from her throat and raise the bed head. Mum and Dad sat on either side, holding her hands. When Dad squeezed Sophie’s left hand she responded with weak but unequivocal movement. But she was obviously having difficulty in finding words and articulating sentences – the brain scan had predicted this, showing a small but strategic defect in the speech area. There was light at the end of the tunnel. Her attempts to communicate definitely made sense, and my job was done.
Brain recovery is always slow. Sophie would need ongoing care from nurses, physiotherapists, occupational therapists and a host of different medical staff. They all pulled together to move her forward, then the community services rallied round. Over the following months the paralysis and speech impediment got better, then she went back to school and eventually on to college, with her considerable intellect intact. Against all the odds, Sophie survived. Even I find it difficult to understand how we succeeded over those weeks. Divine help, I suspect. And that piece of kit on loan, more charity-shop healthcare.
I wrote a case report about the rescue technique of emptying an injured left ventricle with an assist device and it was published in an erudite American journal so that others might use the method in desperation. Sophie’s case illustrated the very best of NHS hospital team work and the triumph of hope over misery. So many dedicated staff worked way beyond their prescribed hours in the struggle to save her. Ironically, our pioneering efforts were questioned every step of the way and they generated huge costs for the hospital. But what is healthcare there for? Ten years on, I still see Sophie and her family socially. On the tenth anniversary of the Battle of the Bulge we had dinner at Fiona’s Oxford college, Lady Margaret’s Hall, together with the eminent brain surgeon and author Henry Marsh. Heart surgeons and brain surgeons are very different, but we agreed on one thing. Life is precious. It was great to see an animated Sophie holding her own in that rarefied academic environment.
10
resilience
23 October 2009
NHS CONSENT FORM 2
Parental Agreement to Investigation or Treatment for a Child or Young Person
NHS organisation: John Radcliffe Hospital, Oxford
Patient’s name: Oliver Walker
Date of birth: 11 February 2003
Name of proposed procedure or course of treatment: Excision of Cor Triatriatum membrane – salvage procedure
Statement of health professional
The intended benefits: to sustain life – at present death is inevitable without surgery.
Serious or frequently occurring risks: surgical mortality, at least 30%, bleeding, the damaging effects of cardiopulmonary bypass, need for reoperation.
Other procedures: blood transfusion, echocardiography.
I handed the form to Richard, Oliver’s trembling father. His mother Nicky was already in meltdown. She was watching her son die from a rare congenital heart condition that had taken the NHS six years to diagnose. Not in the Highlands of Scotland or far-flung west Wales. Not even in Scunthorpe, but in central London. Easy for me to criticise, since I never have to make a diagnosis – the cardiologists do that for me, I’m just the plumber. But one thing I did understand by then was always listen to the mother, because no one knows her child better. If Mum insists there is something seriously wrong, you can bet your life there will be.
For Nicky it had been a long, hard grind to persuade the doctors. Now, years later, a flash Harry of a surgeon who had just walked in from Heathrow Airport was pushing her son down the corridor to the operating theatres as fast as the bed, ventilator, drips and drains would move. We were that close to los
ing him to something he had been born with, a bog-standard congenital heart condition.
Oliver had been delivered at St Mary’s Hospital, Paddington, one of our great London teaching hospitals, in the same maternity unit where our royals choose to have their babies. It was a normal but noisy birth, and Oliver appeared to be pink and robust. His heart rate was a little rapid, but after the shock of being squeezed through a greasy rabbit hole into the cold world, no one at the hospital thought anything of it. Nicky said he didn’t feed at all well – tugging on her nipples made him breathless and agitated – but he wasn’t a ‘blue baby’, just very chesty. So damned chesty and breathless, in fact, that the little family were in and out of St Mary’s on a regular basis, as if it were a football ground and they had season tickets. It seemed that any cough or cold could become life-threatening.
Eventually it became embarrassing for them, a case of the ‘not you again’ syndrome, and in the casualty department they were known as ‘frequent fliers’. Yet St Mary’s was a top hospital and this was a family who were deeply concerned for their child. Nicky grew paranoid about being labelled a neurotic mother who couldn’t cope, because on many occasions they were sent away from the hospital or general practice having been told that the boy was fine and there was nothing wrong with him. So often did they return in distress that Oliver was investigated repeatedly, with many distressing attempts to draw blood, then frequent X-rays so that he almost glowed in the dark. Every conceivable lung condition was considered: bronchiolitis, cystic fibrosis and pneumonia, on numerous occasions. Sure, his little lungs sounded congested, but nothing came up positive. They considered a stomach hernia with gastro-oesophageal reflux and inhalation of his gastric contents. He didn’t have that either. There was nothing to find, yet he was constantly struggling for breath.
Oliver now had a younger brother two years his junior. The fact that he couldn’t keep up with him caused Oliver extreme frustration. There was a twenty-minute walk to his primary school, which a four-year-old should be able to manage. Yet two-year-old Charlie would often climb out of the buggy and walk, which allowed ‘lazy’ Oliver to sit down and be pushed to school. Clearly that wasn’t right. Others could see that there was a problem, but the doctors had no answer. What causes breathlessness at the slightest provocation, failure to thrive and perpetual lack of energy? A boy who couldn’t play football with his friends or walk to the park, who sat in the corner looking dejected at birthday parties? This was all heartbreaking for Nicky. But however many times she was brushed aside by the medical profession, she couldn’t possibly give up, and I greatly admired her for this.
Eventually Oliver was referred to the chest physicians at the Royal Brompton Hospital. There was nowhere to go after that. It was the National Heart and Lung Institute, and they said that the only things that could cause his symptoms were his heart or lungs – but his lungs were normal and his heart was not enlarged on the chest X-ray, ostensibly ruling out the usual causes of heart failure in children. So goodbye again, even from this world-famous hospital.
Soon Nicky was on the phone once more, pleading for her poor boy’s life. They must have missed something. Oliver simply couldn’t run or play with the other kids at school. The teachers had remarked upon it, other mothers could see it. Sure, he looked normal sitting in front of the doctor, but that was all he could do. Just sit. So the Brompton doctors decided to watch him run up and down the hospital corridor, then checked his heart rate – he couldn’t manage to complete this easy exercise test and his heart rate went through the roof even after a few steps. With such negligible exercise tolerance there simply had to be something wrong.
Now they really did have to look inside the heart. With the stethoscope, the valves sounded fine but perhaps quieter than they should have been – lub, the mitral valve closes; dub, the aortic valve closes – but the heart rate was so fast that it was impossible to detect a murmur. That was Friday. Concerned that there must be something seriously wrong with his heart, Nicky was asked to bring Oliver back yet again on Monday for an echocardiogram. This would define the anatomy in detail and incorporate an ECG to detect any heart rhythm problem. Because the valves sounded normal and the left ventricle appeared small on the X-ray, Nicky hoped that nothing serious would be found. She arranged to meet her sister for lunch in Chelsea after the tests, as a treat for her son.
By now Oliver was used to hospitals. Moreover, he had been reassured that there would be no needles this time, just sticky jelly on his chest and a slippery probe sliding around. He lay perfectly still on the couch while the young lady echocardiographer watched the images flashing on the screen and chatted away cheerfully to Nicky. All seemed relaxed and normal until the lady stopped moving the probe and suddenly went quiet, her gaze fixed. In the space of an instant her expression changed from unconcerned to dumbfounded.
‘What is it?’ asked an alarmed Nicky, but there was no response.
The girl was mesmerised by an abnormality on the screen, but had no idea what she was looking at. On the third time of asking, she heard the tone of desperation in Nicky’s voice. All she could say was, ‘Oh I’m sorry, I need to fetch the consultant.’ Oliver lay there oblivious to the sudden air of panic, while Nicky’s circulation was flooded with adrenaline and she started to have a panic attack. She felt the urge to call her husband at work but had nothing to tell him yet, except that the woman had found something terrible inside Oliver’s heart. It had to be terrible, otherwise she wouldn’t have rushed off like that.
For six long years Nicky had been sure that there was something seriously wrong with Oliver but had always been relieved when told she was mistaken. Not any more, although at least the consultant was calm. He returned the echo probe to Oliver’s bony chest and started to work back through the images. The right atrium was mildly dilated, the right ventricle thickened and the pulmonary artery dilated, none of which had been picked up on the many chest X-rays. Put together, this suggested that there was some sort of obstruction to the blood flow through Oliver’s lungs. What’s more, the left atrium was dilated, while the left ventricle seemed small and underfilled. This would normally suggest narrowing of the mitral valve, as in patients with rheumatic fever, but the mitral valve leaflets looked thin and normal.
The experienced consultant knew precisely what he was looking at, yet the condition was so exceptionally rare that this was the first time he had diagnosed it himself. Inaudibly he muttered, ‘Cor triatriatum, that explains it!’, although that left neither Nicky nor the echocardiographer any the wiser. Blood was swirling around within the left atrium but not filling the left ventricle in the way that it should. Why? Because there was a thin membrane obstructing the left atrium and all four veins from the lungs, so blood couldn’t get to the orifice of the mitral valve. The only way through was a tiny aperture 3 mm in diameter, contrasting starkly with the normal mitral valve orifice of 18 mm for a six-year-old boy.
Cor triatriatum translates from the Latin as ‘heart with three atrial chambers’. Oliver’s blood was dammed up behind the obstruction, causing congestion in otherwise normal lungs, so there was no way that he could increase blood flow to his body while exerting himself because his entire cardiac output had to squeeze through the restriction. All of Oliver’s symptoms were caused by this congenital anomaly. His very existence had been a nightmare of untold physical and psychological misery, while his poor parents had been sent away time after time with misinformation. But at least he was still alive. For now, that is.
The paediatric cardiologist was justifiably shocked by the severity of the blockage and immediately called a surgical colleague. Oliver had suffered quite long enough and needed that membrane removed as a matter of urgency. In the meantime, poor Nicky had disintegrated. Richard was at work, waiting for news. She managed to dial his mobile number but was too emotional to articulate the problem or explain that Oliver needed open heart surgery as soon as possible. The kind cardiologist tried to put things
in perspective for Richard, telling him that at least they had finally identified the problem. Although it was rare, there were experienced cardiac surgeons at the hospital who would deal with it at low risk. Rare did not necessarily mean difficult, then after the surgery Oliver should catch up and have a normal life. Nicky’s sister came to the Brompton to support her, and everyone headed home to take it all in. But they had now moved house some distance away from central London, to Beaconsfield.
Oliver went back to school the following morning, his parents trying to maintain a semblance of routine before the operation. The teachers were warned of his situation. Suddenly he had a serious heart condition, which explained why he couldn’t keep up with the other kids. Some felt guilty for the things they had said about him, others because they had tried to push him too hard. Children with congenital heart disease often suffer like this. They have two arms and two legs, but their engine doesn’t work. They are the blue kids who squat in the playground to relieve their breathlessness. Other kids poke fun at them and they always come last on sports day. Their parents burn up inside, but try to smile and keep things normal for them.
21 October 2009. I was giving a talk about ventricular assist devices at the European Society of Cardiothoracic Surgeons in Vienna, so there was no children’s heart surgeon back in Oxford. Meanwhile Nicky was at home in Beaconsfield, trying to get her three children ready for school. Two of them were looking after themselves, but poor Oliver had a barking cough and couldn’t get out of bed. With a temperature exceeding 38°C, he was already panting for breath with an imperceptibly rapid heart rate, and his skin had turned yellow.
The Knife's Edge Page 19