No patient is ever really done and dusted. At least, that’s how we in paediatric neurosurgery see it. Anyone that passes through our doors is rarely more than six months or a year from returning for a check-up. It doesn’t matter what you came in for or in what condition you leave, there could be years of assessment before the full discharge notice is signed. Just because we’ve fixed one thing, it doesn’t mean it can’t cause other symptoms elsewhere in the body. Some brain conditions happen overnight. Others build up undetected for years before the diagnosis is made.
Many of the days when I’m not operating are spent dealing with outpatients in clinic or the day ward. I love it. It’s such a potpourri. Children can alter immeasurably in a year. Each time I step out into the waiting room and call a name, I have no idea which person is going to come forward – the cute little pigtailed girl from last year is now heavily made-up, complete with Goth outfit. She is still adorable, but I can’t say that to her now!
In theory, every operation we perform is intended to leave the patient in a better condition than before, or at least stop the situation worsening. Surgery is a big risk. I’d never do it unless there was a chance of success. Even the misjudgements arise from a desire to improve. The truth is, we can do everything right in theatre, achieve everything we set out to do, but we still don’t know if it’s successful until that patient wakes up. Even then it could be days, weeks or months before the benefits are fully revealed. Or the drawbacks. The truth is, it’s not always good news. And not everyone gets to walk away.
It’s a clinic day. I’ve seen the list of patients due to attend their annual or bi-annual check-up. Most of them were mine originally and the ones whose faces I still remember. Others were operated on by my colleagues. What I don’t know is what they’ll be like when they arrive. I’m always excited to see.
‘Clare?’ I call out to the packed room. ‘Clare Bennett?’
Let’s rewind to three months earlier. I was referred this young girl, whom the paediatrician had diagnosed with a condition called Chiari malformation, an abnormality of the back of the base of the brain where tissue extends down into the spinal canal. She was seven when we first saw her and a lovelier young bundle of happiness you would be hard-pressed to find.
Chiari malformation can be completely asymptomatic – that is, cause absolutely no problems in a patient. Trouble is, in the next one it can be extremely debilitating. If the spinal cord is getting squashed it can affect the function in your arms and legs, with poor balance, pain or funny feelings in the limbs, and often reduced strength even in walking on the flat. It can also affect the nerves supplying the lower part of your face, so chewing or swallowing, plus speech and even eye function, can all be affected.
Clare was born with some mild cerebral palsy (CP), causing weakness in her left side.
Chance had played a sneaky hand in Clare’s treatment. As she grew and struggled with walking and hand function, her parents and doctors, naturally, put everything down to her CP – this is what CP causes. It was only around the age of six or seven when it became obvious that she was suffering in both sides of her body, not just on her weaker left side, that someone said, ‘We’d better do a scan here to see what’s going on.’ They were expecting to see more problems in the brain, where CP causes its changes, but actually they found an abnormality in what we call the craniocervical junction, the crossing between the brain and the spine.
By the time she was referred to me, Clare had deteriorated further, with wobbly hand function, lots of headaches, and really struggling to walk. She was in a right state and getting progressively worse according to notes from her referring doctor. That, as I told her and her parents, was what we had to address.
‘I think we need to operate,’ I said to all three of them.
‘Can you fix me, Doctor Jay?’ Clare asked.
‘Maybe,’ I said. ‘But the main thing is to stop things from getting worse.’
I explained how the condition could exacerbate over time. The classical teaching is that the aim of surgery here is to stop a deterioration. So we tell the patient, ‘We can’t get back what’s already been lost. But we can protect the rest – the function you still have.’
Secretly, though, I hoped for more. The body has a way of healing in these cases. I wouldn’t have been surprised to see some motor function return within months. We have tended to operate earlier in the disease history than many units, and we have noticed a proportion of patients get a recovery and regain some of the lost function. But since it would be a bonus – and we couldn’t predict which patients would get the recovery, I don’t offer that up for the family – it wouldn’t be fair to make a decision on that basis.
There are various ways of treating Chiari malformation. Like all surgeries they come with risks. In this case there was stroke and paralysis, breathing and swallowing problems, meningitis, death, and a risk that the operation doesn’t work or has to be repeated – all of which I explained to Clare and her parents, but I got the thumbs up anyway. The majority of Chiari malformations respond to decompression surgery, so that’s what we did. To the sounds of The Stone Roses’ self-titled debut album, I removed a small piece of bone from the base of Clare’s skull and another from the top of her spine. I opened up the dura and buzzed away the bottom bit of the brain to give the spinal cord and the brainstem some space. The bits we attacked are the cerebellar tonsils, and unless you are a shark or similar, you don’t absolutely need them – a bit like the tonsils at the back of your throat. I was hopeful it would take effect immediately, as brain fluid could flow unimpeded into the spinal cord now that pressure on the brain had been reduced.
Most children are pretty unwell after this procedure – the foramen magnum decompression – with nausea, vomiting and feeling seasick, and in pain, for about seven days and it can take up to six months to get fully back to their preoperative state. But this time, the day following the operation, I was amazed to see that my young patient was not only awake but also back to her usual smiley self.
‘Did you do it, Doctor Jay?’ she asked, before I could get a word in. ‘Did it work?’
‘We did what we set out to do,’ I said. ‘Fingers crossed it worked.’
‘When will we know?’ Mum asked.
‘Hard to say. I’m confident we’ve addressed the actual problem, if you like, so Clare shouldn’t get any worse. But she’s going to take time to actually mend from the operation, that could take weeks or months. You’ll know before I do.’
I was really hoping for a recovery. It would not happen today or tomorrow or even next month, but I was thinking that it could come in part or in full eventually. We sent her home to recover from the operation, wobbly, but walking on her own. The question was, would she also recover from the Chiari itself?
And now, three months later in clinic, I was about to find out.
‘Clare?’ I call again.
I’m on the verge of returning to my clinic room, thinking, how unusual it was that she had DNA’d – ‘Did Not Attend’ – when I spy movement at the back of the waiting area. There’s a bit of a kerfuffle as someone on crutches struggles to their feet. They don’t quite have the strength to support themselves on their arms. I’m about to offer to help when I realize. It’s her. It’s Clare. What the hell has happened?
The whole family are as delightful as always. They’re as mystified as I am as to why Clare has continued to deteriorate. Usually my clinics are for chatting and learning more than ‘doing’, but I am desperate to find some answers.
‘Do you mind if we take some more scans, Clare?’ I ask. ‘It’s possible that I didn’t fully release the area during the last operation, or that things have scarred back up.’ Both of these are potential causes of an ongoing problem. So I send her off for some urgent scans and book another clinic visit.
I wish it helps. But it doesn’t. Everything on the scan looks exactly as I’d hope – there was loads of space around the brain now. So why is she getting worse? Another textbook operation with an unfathomable outcome. I sit there for a minute, my fingers pressed together in the ‘church’ pose. I’m thinking. Thinking and thinking.
‘All I can suggest,’ I say eventually, ‘is that we have another go.’
All three of them – Mum, Dad and Clare – jump at the idea. For all their positivity, I think they are in dire need of a ‘quick fix’. I need to temper this straight away.
‘The facts of the matter are that we performed a good operation that should have gone a long way to stabilizing you. That hasn’t happened. The scans look clear, but maybe there’s something “invisible” to the scan that’s in the base of your skull and doesn’t want to go away – some small strands of scar tissue that are holding everything down, but which are too fine to see on the MRI. I would like another chance at finding it, but you should be aware of the risks.’
I trot them out: damage to the spinal cord, damage to the brainstem, damage to the brain, contraction of meningitis or some other serious disease.
Dad shrugs. ‘Paracetamol comes with worse warnings.’ I smile ruefully. Although this may be true, the fact is that the risks of a reoperation are almost always more than the first one, because we don’t have the usual anatomical markers and layers to guide us.
‘What would you do if it were your daughter, Doctor Jay?’ Clare asks.
I think of my three girls sitting at home. ‘You know what? I’d go for it. Yes, I don’t know what I’m looking for. Yes, it’s basically a fishing expedition. A suck-it-and-see mission. But given what’s happened, I can’t see an alternative, other than to accept that the deterioration may progress to paralysis.’
We perform the surgery. As predicted, it’s more difficult the second time round. I wanted to double-check that the spine didn’t need untethering, that there was free-flowing fluid and that the mobility of the skull and spine weren’t being impeded or impacting on crucial nerves. But I find nothing. Zero. Squat.
The best I could do was to retrace my original footsteps and ‘redo’ the cuts I’d made. Perhaps we’d get lucky. That was the stage we were at. Calling on good fortune to dig us out.
Once again, I am surprised by just how ebullient Clare is in recovery. Her eyes sparkled with the joys of life. If I could have played the Joker card on anyone’s health, I’m pretty sure I would have chosen her. She deserved it.
I report back to the family and confirm, once again, that all we can do now is wait. ‘How long?’ Mum asks.
‘Let’s say three months,’ I reply. ‘I really hope we’ll see changes by then.’
And I was right …
‘Clare? Clare Bennett?’
It’s the moment of truth. Nearly four months have in fact passed since I performed the second operation on the young girl with Chiari malformation and CP complications. I can’t pretend that I thought about her every day – impossible given the number of people I treat each week – but she’s not been far from my mind. More than anything I just want to know how she’s doing.
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.