Everything That Makes Us Human

by Jay Jayamohan

  I look around the waiting room. It’s not so packed this time. Clare has the first appointment. I scan the closest faces. Listen for the kerfuffle of rubber stoppers being deployed on the shiny NHS floor. I hear nothing and for a second I’m pleased. It would make my day if Clare arrives without crutches.

  She does. So why am I not smiling?

  The double doors at the back of the room swing open and in comes my patient and her doting parents. Dad is pushing the wheelchair.

  I really hope I manage to look as pleased to see them as they do to see me. Inside I’m in turmoil. What the hell has happened? When I first met Clare she was walking, albeit with a struggle. The second time, after my first intervention, she needed crutches. Now, after the operation that was meant to reverse the original misfortune, she can’t even do that. She’s in a wheelchair. What on earth is going on? What have I done?

  Fast-forward ten years and Clare is no longer in her chair. She can walk short distances and has mastery over her hands for the most part. And she’s still smiling – with good reason. She is now a proud mother herself. Since turning eighteen she passed to the care of my colleagues in the adult section of the hospital. To this day I can’t honestly say why she didn’t respond to the treatment. It was textbook, the same process I’ve done many, many times with solid results. Nothing on her scans or her charts indicated anything out of the ordinary. And yet there she was. Getting worse and worse and worse.

  You feel so powerless in these situations. I consulted with everyone I knew. No one could spot a flaw in the surgical treatment. The failing was somewhere between modern medical knowledge and Clare’s personal DNA. My colleagues used the same platitudes that I offer them in other cases – ‘life sucks sometimes’, ‘remember it’s not you, it’s the disease causing this’, and ‘you can’t fix everyone’. They are right, of course, which is why we take so much time to consent before operating. Still, it doesn’t help very much.

  At least in her case I was able to try. At least when she’d first arrived I hadn’t had to say ‘Sorry, there’s nothing I can do.’ Because, trust me, that feels a damn sight worse.

  CHAPTER THIRTEEN

  MY LAST PATIENT DID REALLY TERRIBLY

  Patients presenting similar symptoms can have wildly different causes. A child suffering from a sudden loss of leg function can be the result of a blood clot or a stroke. It can also be due to a tumour. Or it can be multiple sclerosis.

  Many children who arrive with a bleed from a vascular malformation in the spinal cord don’t get significantly better. Unsurprising odds, I’d say, for a condition that sees sudden rupturing of abnormal vessels which then spurt blood, under pressure, into the spinal-cord tissue itself. These are children who have been absolutely fine until the moment they’re not. One minute they’re haring around the house or their school and suddenly they drop to the floor with total loss of leg function. No warning. Complete paralysis. They’re taken to hospital and a massive blood clot is discovered in the spinal cord.

  It’s not often you find yourself hoping for a tumour, but sometimes it can prove to be the least worst option. Loss of leg function caused by tumorous clot is attackable. We can operate. Go in, get the bastard, retreat. Job done. Vascular or blood vessel problems sound less terrifying, but they’re harder to put right.

  If you think about it, whatever blood vessel has ruptured, the clot has to some extent been contained by the fact that it’s held within the spinal column, compressing the cord. Hence the loss of lower-limb function. It sounds obvious to try to unblock the passageway, but if you do, as well as being accurate you also have to be quick. When you open up the spinal dura, you’re essentially opening the very structure that is containing this vascular malformation. All the blood vessels can swell out of control in front of you in a very tight, narrow space. Suddenly, the cord is being pushed out of the dural opening, and precious, vital nerve tissue is coming out, and you have to apply pressure to hold it in, but without damaging the spinal cord any further. In short, the situation can quickly turn into a disaster if you aren’t careful. And that’s being optimistic.

  The current thinking with this type of problem, with the attendant risks of operating, is often very much ‘let’s wait and see’, unless there is active deterioration in function. In other words, we’re trusting in luck and our old friend, time.

  The decision-making in these scenarios is about probabilities. About cause and effect. On the one hand, what are the chances that if you go in and remove this blood clot you will recover leg function? On the other, what are the chances that this is a very abnormal collection of blood vessels and so any surgical procedure may set off a tsunami of reactions with the haemorrhage spreading higher up the cord. In trying to save the knees, you could risk the hips. In trying to save the hips, you could put the upper torso at risk.

  It sounds doom and gloom, but it’s not always. If a local hospital says they’ve got a child with incomplete loss of function and it’s been around four hours, there’s still enough time for you to potentially save some function. Once there’s been twelve hours of absolutely no function, your window of opportunity is beginning to close.

  There’s an element of chance. Some parents won’t dial 999 until a leg actually falls off. They come from that stock of people who don’t want to ‘trouble’ the emergency services. Their child starts falling over? They pack him off to bed with some Calpol and assume everything will be okay in the morning. Or maybe something happens during the night while the boy’s asleep. Next day, Mum just thinks he’s too lazy to get up, especially if it’s a weekend.

  Any number of reasons can delay a patient getting to hospital. Once there, the dice need to be rolled again. A lot of children’s departments have very good A&E set-ups. Even so, it can be a decent amount of time before you get in front of the person with the authority to say, ‘We need a scan.’

  Or maybe they don’t do that. Maybe they jump straight on the phone to their local specialist hospital. The John Radcliffe has one of the twenty or so stand-alone paediatric neurosurgical departments in the country. Where potential loss of function in children is concerned, it’s our policy to have them shipped over immediately – we need to see them and ensure the information on which we are making such major decisions is accurate. I can’t vouch for the way that other departments are run – I know from my medicolegal work that not all of the units around the country do this. So there’s another element of chance based on geography. And all of it depends on another condition: the clock.

  My phone rings one day. ‘Jay, we’ve got incoming.’

  ‘Who? What? Where?’

  ‘Five-year-old boy, loss of leg function, day before yesterday. No scans done. Northampton rerouted straight here.’

  ‘Okay, get him magnetized as soon as he arrives.’ I tend to make up a lot of stupid words – this was my way of asking for an MRI scan.

  The problem with a patient coming in ‘hot’ like this is that we’re blind. The time element is worrying. Two days is a long time to have these sorts of symptoms.

  The boy arrives with his parents. A registrar greets them and then gets the scans done. He’s already had a look by the time he hands them over to me.

  ‘What do you think?’ I ask.

  ‘Not good.’

  ‘Yes, thanks, Doctor House! What else?’

  ‘I don’t think it’s a tumour. The history seems wrong for that – either a vascular lesion or of course it could be a bleed into a tumour. It’s a bit too fast for something like demyelination.’ The latter term refers to a condition like multiple sclerosis, which can present similarly but has a very different treatment.

  ‘How long since he’s been well?’

  ‘Maybe thirty-six hours.’

  I pause. ‘I fear the horse may have bolted.’ But I don’t tell the family that. Not yet. Not till I have all the information. Naturally, they’re beside themselves.

  ‘We’re so sorry, we didn’t realize it was so serious,’ Mum sobs. ‘I
thought he was playing up.’ Another common phrase where children are concerned. What kid hasn’t feigned injury at some point for attention or to get out of chores or just for a laugh? Navigating the mindset of a young boy or girl can be a minefield for parents.

  ‘It’s not your fault,’ I say. ‘You didn’t cause this.’ Not directly anyway. Perhaps there’s a genetic link. I run through a few questions. I want to know the family history, who’s had what. It will help to narrow down the likely causes. Neither Mum nor Dad can offer any magic pointers from their family lines.

  ‘What do you think it is?’ Dad asks.

  ‘It appears your son has a blood clot in his spinal cord caused by a burst vessel. The pressure has shut off power to everything south of that spot. Like a roadblock on the motorway. Nothing can get past.’

  ‘Will he be all right? Can you cure him?’

  That word again. My instinctive response is to recall the last chap I had in suffering from the same condition. I was too late to help then. In his own sweet time, over the course of nearly twelve months, he started recovering, but only a bit. But I can hardly tell this family that ‘my last patient did really terribly’. How’s that going to help them?

  What I can do – and what I will do – is to give them some hope. Positivity can go a long way to aiding or prompting recovery. There are numerous studies written about it. I want the parents to believe that there is a chance their boy can recover. And I need him to believe it, too. So I tell them about the body’s natural healing qualities. How it can, over time, fix little glitches. Little glitches that have massive repercussions.

  ‘Are you saying that you can’t do anything at all?’ Mum asks.

  ‘I have to be honest with you. I don’t know if we’ll ever be able to get back to square one. But our best bet is to just wait and let nature take its course. We’re going to run some more tests, but I’m afraid any operation we might try to do runs a high risk of making things worse.’

  ‘Worse?’ Mum says. ‘Worse than not being able to walk? What’s worse than that?’

  The fact that so much time has passed gives clarity to my thoughts. If the collapse had happened just a few hours ago, I’d have a ton of quick decisions to make: ‘Do I think I can help? Is the situation likely to deteriorate if I don’t act? Is there a chance that, in trying to help, I’m going to make things worse?’ And finally: ‘Can an operation in this area be safely performed on a child of this size?’

  The time that has elapsed means none of those questions are viable any more. The moment has passed. Pandora’s box is open.

  I look at the little guy tucked up in bed. You’re on your own now …

  Size matters. If you stand on a full-flowing hosepipe in the garden, then take your foot off it, the build-up of water comes shooting out at top speed for a second, then settles back to normal flow. What doesn’t happen is that you run out of water. That’s because there’s a considerable supply in the local water pipes, enough to cope with such shenanigans for hours, days and weeks.

  A human body has a finite amount of blood. The average adult woman carries around 8 pints inside her. A man, depending on size, can have anything up to 10 pints or just over 5 litres. Children obviously have less, again dependent on size. By the time you get down to babies, you’re looking at tiny amounts. At a few months old, a baby’s blood volume will be between 250 ml to 350 ml – somewhere between a large glass of wine and a can of Coke. They don’t have much to spare.

  Any operation runs the risk of blood loss, but vascular difficulties, blood clots and the like are trebly dangerous. If a surgeon goes flying in looking for a clot, just disturbing it can cause the vessel to start spraying, especially an artery carrying oxygenated blood from the heart. It takes seconds for a baby to lose a harmful amount and not much longer for it to be devastating. A small five-year-old would also bleed out in a dangerously quick time. So you have to weigh up the desire to treat with the potential to actually make things worse.

  As a general surgeon you wouldn’t go in and tackle a vascular malformation in the liver if the whole organ is involved. But you may take the risk if there’s just a small portion affected. You might say, ‘We’re going to cut out this bit of the lung’ or ‘We’re going to remove this section of bowel with the vascular changes in it’ – all perfectly sensible. Whatever it throws up should be manageable. But would you say, ‘We’ll take a piece of spinal cord?’ knowing that rapid blood loss could make things go south very, very quickly? Probably not. Not on a child. Not unless there were no other options.

  And that’s without the normal problems associated with removing any part of the central nervous system. The long and the short of it is that you don’t mess with anything hot and vascular unless you have a reasonably good shot at success. And with this kid, I didn’t.

  I’ve never felt so impotent. I’m a consultant at one of Britain’s best paediatric neurosurgical hospitals (we think). I have resources that many units in other countries can only dream of. I have access to some of the greatest minds in the profession. But at the end of the day, we’re all limited by science and physiology. Until someone devises a treatment for this condition, we just watch and we wait. And we hate every minute.

  It’s eighteen months later. I’m having a good clinic so far. Everyone I’ve seen since lunch has shown massive improvements since the last time they were here. There are few sights more life-affirming than families who believe they’ve been given a second chance. It’s almost as though they’ve had a religious epiphany and seen the light.

  The next name on my list is the young boy with the loss of leg function from a year and a half earlier. I remember when they were discharged. For every positive word that came out of my mouth, one of the parents countered it with some shrugged negativity. Even young Clare Bennett would have struggled to keep her countenance in their company. They didn’t mean to, of course. They were in pain. Still blaming themselves and possibly each other for not acting sooner. And neither of them believed a word I’d said about time sorting everything out.

  Their mood hasn’t really improved in the interim. Or perhaps it has changed. They seem less angry and more resigned. Resigned to their lot. Resigned to their son being disabled for the rest of his life. Resigned to the fact that they think they could have done more.

  I know they feel that I could have done more. They say it within the first few minutes. I reply how I always do: ‘Most patients’ bodies self-correct. It’s still our best option. Surgery for this condition carries too many risks.’

  For all the pain I can see the parents clearly suffering, the star of the show – the little fella who’s actually suffering physically – just smiles at me. He’s not blaming anyone.

  ‘Mum, Dad,’ he says. ‘Stop arguing. I’m all right.’

  I look at him and smile back. I offer my hand in the sort of clumsy fist bump that my daughters hate. ‘Yes, big man,’ I say. ‘You are.’

  CHAPTER FOURTEEN

  SMASH A HOLE IN THE WINDOW

  Who’d be a GP? You’re having to deal with 64,000 different conditions from any site inside or outside the body, and every patient who comes in complaining of this pain or that may potentially have something that’s about to go bang. What do you do? You can’t send everybody to hospital for an urgent assessment even if you wanted to. It would bankrupt the NHS in an afternoon. You’re a gatekeeper with your hands tied behind your back. So sometimes you have to make a call.

  And sometimes you make a mistake.

  A family went to see their GP with the general complaint that their three-month-old baby didn’t seem quite right. ‘Can you be more specific?’ the GP asked.

  ‘Well, he just doesn’t seem to be like our other kids were.’

  The GP had seen thousands of overcautious parents. ‘Look, don’t worry, every child is different. They all develop at their own pace.’

  And so it went on. A month later the family returned. This time they were adamant. ‘He doesn’t move like the othe
rs did. He can barely turn his head.’

  Once again the GP found nothing wrong, other than overanxious parents. And again he prescribed ‘not to worry about it’.

  You can picture the scene. I imagine this GP has probably just seen somebody with a heart condition beforehand, someone with a gammy leg prior to that and who knows what to come. He sees this child, he’s got ten minutes to make an assessment, get a history off the family, do an examination, bang, bang, bang. Kiddie looks fine, he’s probably okay, no obvious danger. But in this instance, there was. There really, really was.

  Another fortnight passed by and the family had become seriously concerned. They knew they weren’t making it up. This time they went to their local A&E. The doctor on call took one look at the boy and said, ‘Blimey, he’s got a big head’ and then called in the paediatrician. Soon after, they were on their way to me. I took one look: ‘Hydrocephalus.’

  Hydrocephalus – from the Greek words meaning ‘water’ and ‘head’ – is a condition which sees a build-up of fluid in the brain, causing an increase in pressure in the head. In adults it can become serious quite quickly, as the brain pushes against the skull and has nowhere to go.

  Diagnosing infants should – in theory – be a bit more straightforward. Because babies’ skulls aren’t fixed – they are basically a series of hovering plates that over time will fuse together – as their brains ‘swell’ they push the bones out and the entire head above the face balloons. On something as tiny as a baby, the difference in appearance can be extraordinary.

  It’s not the fluid itself that is the problem. All brains produce cerebrospinal fluid (CSF). It’s necessary as a lubricant for the brain to sit in, like a kind of bath. Without it, every time you turned your head, your brain would bash against your skull like a tennis ball in a tin. It doesn’t, though, because of the CSF.