Everything That Makes Us Human

by Jay Jayamohan

  With one puncture we could have drained the sac, sewn the boy up and waited for him to regain some semblance of function a few days later. In doing so, however, we’d be inviting future complications. Whatever is causing the cyst and the fluid build-up within would continue to do so. Within six months, the boy would be back reporting similar symptoms.

  So that is why it’s not enough to make one hole. I have to make several – half a dozen if I can. The body has this annoying habit of healing any scars – usually a good thing, but in this case an inconvenience of life-changing proportions. Make two holes and the body might heal one within six months and the other in a year. Make six and you’d have to be seriously unlucky for them all to heal.

  Voodoo needlework done, I stitch up the dura, then lower the link bracelet of the spine down and secure it into place. It has to slot in perfectly to maintain the integrity of the spine’s biomechanics. If it’s misplaced, a growing boy could end up with curves in his spine or other problems.

  It takes an hour to rebuild the jigsaw and close. Everything is finally connected back to where it was before. Confident we’ve done as much as could be expected, I call an end to the surgery. The scrub team can then prepare for whichever operation they were meant to be doing for the last couple of hours. I need to speak to my patient’s parents, then wait. And wait.

  I have never guaranteed a surgical outcome. By the same token, I’ve rarely intimated results that were anything other than likely. I’m Mr Middle of the Road. I believe that patients should be as informed as I am. No hyperbole, just plain probability. I’d warned this little boy that we may be able to reverse his condition, but it was by no means guaranteed. I’d told his mum and his dad the same thing.

  The whole procedure took about four hours. The patient was declared safe and moved to a ward. We’d started late afternoon and it was the middle of the evening by the time we were done. On a non-surgery day, as today originally was, I should have been home in time for family supper. If I rushed, I could possibly still catch the dying embers of the meal. But as tasty as the boss’s cooking is, I couldn’t leave without updating the parents.

  ‘We’ve done what we set out to do,’ I said.

  ‘Have you fixed him?’

  Ah, the most common question. ‘We’ve disrupted the sac, but the spinal cord is a very intricate part of the body. We should have stopped the damage progressing, but I don’t know if we will see an actual improvement.’

  Slowly, the boy stirred. I tried to get him to move his legs, but there was nothing. Still early days, I thought. He was so tired after the ordeal of the day that he nodded straight back off to sleep. That’s enough for one day. I said goodbye to my team and the parents, and hit the road.

  The following morning I did my ward rounds as usual. There were eleven cases within our purview, each of them as important as the next. Everyone was as I expected them to be, even the ones in the ICU. Two I was able to sign off for discharge. Another pair I prepared for surgery later in the day. But the person I was secretly most anxious to reach filled me with nothing but disappointment.

  I said ‘Good morning’ to Mum and Dad and then my patient. It’s always important to include them in any discussion. I asked how the night had been, then opened the question up to the nurses. ‘Anything you need to report?’ I asked.

  The nurse in charge replied that there was nothing of import. ‘Temperature standard, heart rate normal, sleeping pattern regular. No leakage from the wound.’

  The boy was awake, smiling in between bursts of discomfort, which always gives hope to the coldest of hearts. But when I asked, ‘Can you move your feet for me?’ his reply was ‘No’.

  ‘Are you sure? Are you sure you can’t wiggle your big toe?’

  He stared down at his feet and concentrated. Nothing happened.

  I touched them gently, but he couldn’t feel them either. He was exactly the same as his preoperative state.

  ‘It’s okay,’ I said, as much to his parents as to him. ‘It can take some time.’

  The following day produced the same response. Dad was beginning to go crazy. He wanted a result.

  ‘As I said, the surgery went as well as we anticipated. How it manifests itself is less clear. But it’s early days.’

  ‘Early days? How long are we expected to wait?’

  ‘As long as it takes.’

  Every day I walked the wards and every day it was the same story. ‘Can you wiggle your toes?’

  ‘No.’

  ‘Are you sure? None of them? Can you wiggle your big toe?’ It’s stupid, but every time I ask someone to do that, I remember it being said in Quentin Tarantino’s Kill Bill: Volume 1 film. Except in that case it’s the patient, played by Uma Thurman, ordering herself to do it.

  Anyway, back at John Radcliffe, he couldn’t. ‘That’s okay. There’s always tomorrow.’

  As each day passed by, Mum became more resigned, more sanguine. Dad, by contrast, was increasingly wound up. He wanted answers, he wanted results. ‘When will he be able to run? When will he cycle? When will he be able to take up boxing?’ At least that is what came out of his mouth. What he really sought, however, was absolution. He was haunted by the idea that he was responsible for his son’s condition. ‘Why didn’t I listen to him when he said his tummy hurt? Why didn’t I notice when his walking changed? Maybe if I’d woken him up an hour earlier, then we could have picked it up an hour earlier and you might have saved something.’

  It was as painful watching him torturing himself as it was registering zero improvement in my patient. Nothing was Dad’s fault. It’s human nature to say to a child who’s complaining about anything to ‘run it off’ or ‘go to the toilet’ or ‘get an early night’. They’re all quick fixes that tend to work more often than they don’t.

  ‘Listen, there was absolutely nothing you did wrong. How on earth could you have known this was going on? You need to forgive yourself and spend your energy on him. It’s early days,’ I reassured him. ‘We have to be patient.’

  Rehabilitation is as much about your state of mind as it is about your state of body. You’ve got to really work at it and want to work at it and put the effort in. Whether you’re four years old or forty, it’s all too easy just to lie there and go, ‘Ah, it’s never going to happen.’ And you’d be right. We know that the psychological state of a patient has a direct relationship to the recovery they can enjoy. We tell patients that they ‘need to stimulate the nerves back into action’. But what four-year-old can do that with a father chuntering ‘It hasn’t worked, it hasn’t worked’? I wasn’t just working on my tiny patient. I had to treat Dad as well.

  Four days turned into five, turned into six, turned into ten. I was so concerned at that point that I ordered another set of scans. What if a blood clot had occurred on the operation site since I’d been in? That could explain everything. But there was no sign of this. It all seemed fine.

  On day eleven I made the same pass through the building. Mum and Dad barely looked up when my team and I swanned in. Eau de guilt filled the room. My solidarity with my patient’s parents was being tested. I would have given anything to have them feel less guilty.

  ‘Good morning,’ I said, just as I’d done on the previous eleven days. ‘Do you have any news for me?’

  ‘No,’ my young patient replied. ‘Sorry.’

  ‘That’s all right, completely okay. But as I’m here, could you just try to wiggle your big toe for me? If you can’t you can’t, that’s okay.’

  I’m not sure I was even paying that much attention. It was mainly the reaction of the boy himself that shook me into life. He was giggling, laughing, pointing.

  And why? His big toe was wiggling. Just like Uma Thurman’s in 2003. In fact, all of his toes were moving. The extremities of both feet were alive. It was crazy. The most heroic scene that ward had experienced for ages.

  Yes, you could say it’s a relatively minuscule part of the body. But the toes are also the furthest from the brain. If th
e signal is forcing its way down to them, through whatever blockage there had been, then there is a very good chance it is reaching out to the various points in between. In this instance, legs and possibly bowel and bladder.

  But that was getting ahead of ourselves. Right now we had a small win on our hands. I was choking with happiness. My trainee and other staff were besides themselves. Mum was gobsmacked. Dad looked like he just wanted to cuddle someone. Of course, his enthusiasm didn’t stop there. ‘Come on, let’s get you up and walking!’

  ‘Please,’ I said, ‘you have to give him time. Some people in his condition regain control of their ankles and that’s it. That’s where it stops. You can’t force him without making him feel bad.’

  He looked so contrite. ‘Yes, yes, of course, you’re totally right.’

  As it turns out, and not for the first time, I was totally wrong. It was like turning on a tap. When I swung by for the next morning’s meeting, I was thrilled to see a young boy sitting upright and looking downright bored. He had significant function in his legs. In twenty-four hours he’d gone from wiggling his big toe to flexing his ankle, bending his knee and arching his hips. Remarkable progress even for us old pros.

  He was discharged after about three weeks. I was confident by then that full motor performance had returned to his legs and elsewhere. And how did I know? Because on his last day at John Radcliffe he walked, unaided, out of the door. Some days are full of sunshine. We save such memories to balance out against the rainy ones.

  Just because you’re out of my ward, it doesn’t mean you’re off my radar. Every six to twelve months, most patients call back in for check-ups so I can monitor progress and see how the physio is going and whether the rehabilitation is on track. Sometimes everything is so perfect that we say goodbye after a year, sometimes three years. Sometimes it’s ongoing forever.

  Four years after this lad hobbled back to Northampton, he visited me for the final time. He could barely remember a time when he couldn’t walk as well as everyone else. If it weren’t for my notes I’m not sure I’d have believed it either. Happily, I have no need to see him ever again. But in the meantime, there are the Christmas cards.

  CHAPTER NINE

  THE TESCO TEST

  There are very few times when the music goes off. Often, I have differing tastes to my colleagues and team (in fact, swap ‘often’ to ‘always’, and ‘differing’ to ‘totally opposite’). If I am doing something that really takes my concentration, then I need to hear music. Generally, colleagues know that and they leave the sound system alone. Sometimes, if we are doing a less demanding procedure, the team will sneakily change it to a different playlist. I can usually manage to cope with something more socially acceptable – I think I am getting better at this now that my kids force me to listen to the streaming version of the Sunday afternoon Top 40 (how I fondly remember those days – finger ready on the ‘tape record’ button).

  There’s only one scenario where the soundtrack gets unplugged and I don’t complain. In fact, it’s because I’ve shut it down, which is never, ever a good sign.

  A child’s head isn’t solid skull. When you’re born, you’ve got various plates that make up a skull and there are growth lines between them. Often, you’ll see the middle part of a baby’s head – the diamond-shaped area known as the fontanelle – pulsate up and down because there isn’t actually bone there. It’s part of the membrane in which the bone grows. It’s a bit like ice forming in water. The plates of bone are like ice and they grow towards each other over time.

  The bone itself is fairly firm. But the joining bits, the membranes, are quite soft. They need to be for two reasons. Without the plates of the bone being able to ride over each other, the baby’s head could never make a vaginal delivery. It would be too big. The second thing is that those junctions between the different plates are where more bone can be laid down and the skull can grow. The growth during the first two years is beyond rapid. By the time you’re two, your head size is about 80 per cent of an adult’s.

  If you think of the changes that a child goes through in those first two years, the demands of a massively expanding brain, it’s no wonder the skull needs to grow so quickly. Whereas a baby giraffe, for example, comes out of its mum pretty much fully wired because it can walk and feed independently, human babies are useless. But not for long. They go from being completely helpless squishy things, to angry, sometimes-capable-of-shouting-back-and-throwing-stuff squishy things in no time at all. They crawl, they eat, they communicate, they toddle, they develop fine motor skills – they become tiny people exceptionally quickly. Nought to sixty in a matter of months. No wonder David Attenborough calls them the most impressive creature in the wider animal kingdom. A lot of remarkable brain development occurs in a brief time period – and the plates have to keep up.

  But what if they don’t? What if those junctions between the different plates fuse too early? It’s called craniosynostosis and it happens, sometimes while the baby is still developing within the womb. If it does, it can create a small but firm head that is still able to be delivered normally. More often the plates will fuse after birth. But whenever it occurs, it’s often bad news. If the whole skull fuses as one, then there is total restriction of head growth. If just a section of the junctions start to harden then, as the brain demands space to develop and expand, other parts of the skull will grow in compensation. So instead of pushing on the left, for example, which it now can’t do because there’s fusion of the suture, the brain pushes double strength on the right, resulting in a distinct distortion in the shape of the head.

  Of course, many babies have ‘funny-shaped’ heads when they pop out, especially if forceps or other tools have been used in the birth. Parents may not initially spot an issue, being so excited about their little new addition. A midwife sometimes will. If the problem doesn’t kick in until later, then it’s the parents who are most likely to notice it first. Luckily, with the power of the Internet, at the slightest concern they can hit Google, do a bit of research, print off a sheaf of papers and quickly run things past their health visitor or GP. Sadly, the medical profession tends to switch off when patients say they’ve seen something online. I can see why: a simple headache can seem like a brain tumour on certain sites. But parents do tend to know. More than a few have arrived at my clinic saying, ‘Thank you for taking me seriously. Everyone else said I was a paranoid, crazy parent.’ Easy for me, though, isn’t it – the diagnosis has already been made.

  Whether the problem is picked up pre-birth or ten months afterwards, there’s often no real point doing anything about it until around the twelve-month mark. Though it’s true that for every day you delay treatment there’s the chance of pressure building inside the restrictive prison that is the skull, operate too early and you’ll only have to go back in and repeat the process further down the line. It’s not a universally accepted time frame, if I’m honest. Compared to a lot of units around the world we do tend to do ours relatively late. But our procedure is much more extensive and definitive, I would say.

  If physical signs aren’t recognized, then behavioural flags can be. If there is a problem with pressure inside the head, then babies can become irritable, not want to lie down, not sleep properly. Sometimes their feeding goes off. If it’s particularly serious, and goes from symptomatic to developmental problems, then they can start to miss their milestones. Maybe they’re not sitting up at the age they’re expected to. Or they’re not reaching out when they should do. They might not be growing at a reasonable rate. Perhaps they have problems with vision. Particularly in genetic or inherited cases they may get double vision and not develop the ability to have 3D stereoscopic vision.

  Regardless of the tells or the family history, it always boils down to the same thing: a skull meshing too early and often squeezing the brain. And that’s when we’re called in.

  Our next patient was six weeks old, referred to us from another hospital with the note: ‘This baby clearly has craniosynostos
is.’ The plan was to get her into clinic in the next few weeks, observe, check and make a plan. That was all agreed when the other hospital first called us.

  They phoned back after a couple of days. ‘Actually, it’s more than craniosynostosis. She’s started vomiting a lot. Can you see her earlier? We’ll transfer her across to you. We think she has Crouzon’s syndrome.’

  Crouzon’s is a genetic condition in which there’s a change to a specific receptor on the cells of the skull that affects the way the bone is laid down. It means you get rapid fusion of the skull sutures as well as changes in the brain anatomy, opening the door for hydrocephalus – a build-up of fluid in the middle of the brain. So not only might you have a small skull, which can raise the pressure inside itself, but the hydrocephalus can also increase the contents of the skull through the build-up of water, thereby raising the pressure even further.

  We had a look at her. Clinically, it appeared that she did have Crouzon’s syndrome, plus problems with pressure inside her head. When you’ve got fusion of the sutures, the brain still needs somewhere to go. If all of the sutures start to fuse, in actual fact, the weak spot starts to become the centre of the plates of the skull rather than in the joints. Even though the bone is hard, and the brain is soft, the brain starts to wear away the bone as it pushes slowly out, just like water eroding rock.

  The head takes on a very classical shape as this wee mite demonstrated. It can become quite small, wide on the sides and tall at the front. The head is described as a clover-leaf skull because that is the shape it grows to look like. Obviously, these changes take time, much longer than six weeks. Clearly the transition had begun in utero, building up for months possibly. But, just as clearly, we knew it wasn’t a case that could wait twelve or eighteen months.

  As well as the build-up of pressure, I was worried the baby was susceptible to Chiari malformation, and not a mild version of it. This is where the bottom part of the brain (which should remain in the skull) pushes into the top part of the spinal canal, causing compression of the important brainstem structures and contributing to blocking the fluid pathways, hence the hydrocephalus.