Eight months. It’s no age. No age to die. No age to be born. Not like this.
Not even eight months old. This is eight months BC – Before Caesarean. Before Coming into this world. It’s tragic.
The baby had popped up on my radar antenatally, after Mum complained of not feeling any kicks or movements or any of those other things that pregnant women can sense and enjoy. Her local hospital did a scan of the foetus. Often it turns out to be something that’s fixable. That’s what they were looking for. In this instance they found that Baby had a brain tumour.
Breaking that news to the parents of a five-year-old is hard enough. But telling expectant parents that the unborn child they haven’t even met yet might have a terminal disease is crushing.
‘A tumour?’ Mum says, her face white in shock. Her husband clutches her hand. It won’t help either of them to learn that the condition is relatively rare. That there were just over 400 brain tumours in under-eighteen-year-olds in 2018 – in the UK. Maybe this was the only baby to be diagnosed at this stage in the whole country. It’s all irrelevant to them.
Dad looks at his wife, his hand drifts to her tummy. He looks at me, then back to them. ‘Oh my God,’ he says, ‘our baby’s got cancer.’
You can’t blame him. When people hear the word ‘tumour’ they automatically think of the ‘Big C’. It’s understandable, but not what the word means.
A tumour is a lump. An area of tissue that has experienced accelerated growth. If the tumour is the sort that tends to stay in the same location and slowly gets bigger within the same place, and just pushes the brain out of the way, that tends to be what we call benign. As opposed to the tumours which can move around the bloodstream or the cerebrospinal fluid between the brain and the spinal cord. These tumours have the ability to detach a portion of themselves off, float off, reattach in various locations and start to grow there as well. Those are what we call cancer cells.
There are lots and lots of different types of cells that work together to form the brain. It’s not all just nerve cells. These are very delicate and require an elaborate scaffolding of supporting – or glial – cells to give them the ability to make the connection with each other. There are supporting cells and insulating cells, cells that are there to supply energy and oxygen, and cells that the neurons themselves hang onto.
The texture and the appearance of a tumour will very much depend on which cell type it comes from. Tumours in the nerve cells are actually relatively rare. The most common are tumours of the glial cells – they make the scaffolding for the nerves. These are cells which tend to replicate and grow over time, replacing each other. In most people it all goes to plan. Occasionally, the cells replicate but misread the recipe book, forget to add an ‘off switch’ and just keep growing into what becomes a tumour.
With neonatal cases there’s an added layer of confusion. At that age cells are still in a state of flux, they’re literally growing right in front of you. What you have to ask yourself is: are you looking at rapidly growing cancer cells or just cells that are growing because they’re still supposed to be in utero? There’s much we still don’t understand.
MRI scans revolutionized medical treatment. In the case of tumours, however, they can only do so much. You can’t tell a malignant tumour from a benign one just by looking. A tumour comprising 10 per cent of the brain can prove more toxic than one covering 20 per cent. Or, in this instance, 50 per cent. Whether they’re highly malignant or benign, there’s no way of knowing at first glance. Bottom line: they all look bad till you get inside.
Still, there are always options.
The later you leave a termination, the harsher it is for the carrier. You can dress it up however you like, but basically the medical team will often end the baby’s life, with the mother still needing to deliver the body. I can’t think of a much more traumatic scenario than a mother having to give birth to a child whose medical condition was so serious, she agreed to terminate their life. There’s no hearing the first cry, there’s no first feed. It’s unimaginably distressing. But sometimes it’s the way that parents choose to go.
This baby was just past eight months of development when her condition was flagged. Way past the ‘normal’ dates when mothers decide to have an abortion. But these conditions were anything but normal. In situations where birth can be injurious to the health of the mother, or a baby is really sick, then termination remains an option throughout pregnancy.
But the mother needs to decide. Although fathers are often involved in such a momentous decision, legally it is only the mother who can make the final call either way.
Baby’s mum hears the evidence, takes in the severity of her unborn child’s condition, understands the real likelihood of her baby dying, if not in childbirth then shortly after. She hears it all and, after discussion with her husband, decides to press ahead with the pregnancy anyway.
I’m there at the birth, a month after the dreadful news had been broken. The mother had to go through that four weeks in constant stress and anxiety – every time she couldn’t feel the baby move, she must have been so worried.
Scans can only tell you so much. I need to see with my own eyes what I will be dealing with later. Will I have to operate tonight or do I have a couple of weeks to prep the case properly and study the baby?
We recommended a birth by Caesarean section as we weren’t really sure how Baby’s head would cope with the pressure of standard delivery. Her tumour accounted for a significant part of the brain mass and there were too many variables to risk.
The birth goes well and Baby emerges breathing and functioning as you would hope. No way had that been a given. Such a large tumour could easily have pressed hard enough on the brain to block vital passageways. On top of that, the pressure outside the womb is different to inside. There was no telling how she was going to react physically, but not only is she functioning, she is thriving. Watching Mum hold her, you would have no idea there was a problem.
But we both knew there was. From my point of view, I could see I had time to fully explore what had to be done. To get blood cross-matched for a transfusion, to do MRI scans, to really get a sense of what we were dealing with.
If things hadn’t gone to plan, we weren’t exactly on DEFCON 1 but we were prepared. We would have had theatre ready within the day. We could have drained any fluid build-up inside the head, any number of things. It isn’t necessary. Even so, Baby is sent to SCBU – Special Care Baby Unit – while I request the MRI.
Tumours appear for different reasons. First guess historically has always been genetics. Our tests found no obvious cause in either parent’s lineage. These days there is a whole area called epigenetics, which studies the way that the environment changes your genes, even in utero. It’s interesting stuff, but not particularly helpful when you’re staring at a tumour that’s only going to keep growing.
Despite their bad press, it can be malignant brain tumours that pose a lesser threat than their ‘benign’ cousins. Or, at least, a more treatable one. Due to their cells’ rapid replication, they often prove susceptible to chemotherapy and can be treated without invasive surgery, just a biopsy. I know I’m a surgeon, but I’m never happier than if I don’t have to operate at all on a newborn. There’s always someone else who does need an operation.
Tumours in the brain differ from elsewhere in the body. Or, more accurately, our responses to them do. At face value, people think that having a malignant tumour must be terrible because it can spread around and it’s the benign tumour you pray for. In neurosurgery that distinction is anything but obvious. Sometimes more important than the type of cells that form the tumour is its location. A benign tumour in a very important structure of the brain can do the most damage.
For example, right in the centre, in the midbrain, the ‘clockwork’ of the brain which controls stuff like wakefulness, blood-pressure and breathing, a benign tumour can cause immense difficulties. Partly as it grows and squeezes important nerve endings, but also when we come to try to remove it. Accessing and then cutting at something in such a delicate area is like walking a tightrope. The odds of injuring an important structure can be greater than the chances of removal.
The majority of tumours present by affecting the brain around them and causing a problem with the function. So we may see children who have developed a weakness in the limbs or poor core balance, or difficulties with eating or with vomiting after they eat. Problems with their speech, their vision. Anything that you can think of that the brain controls can go wrong if there’s enough pressure on the cells in charge. Seizures are common. Even something like epilepsy can be a result of a growth muscling in on the brain.
It gets worse.
While tumours that grow from outside can constrict the brain and do serious damage, they do tend to be more easily removable. Sometimes, when you’ve removed such a tumour, the brain will expand back to fill the space.
With those tumours the biggest question is the quality of the boundary between the tumour and the brain. If it’s a very good one, as in ‘this bit is brain, this bit is tumour’ and they’re just lying next to each other rather than entwined or incorporated within each other, then you can hope to achieve a fairly clean break. Other than the brain vessels coming in to feed the tumour, you can work carefully around the whole lump, cutting it up piece by piece.
In brains, and in children especially, those neat little packages are rare. The worst types are what we call ‘intrinsic’ – that is to say, they’re within the brain itself. Not next to it or around it, but part of it. The issue then is how much brain tissue is within the tumour as opposed to how much tumour tissue is within the brain? On the edges of the tumour, how much is the interface between tumour and brain? How well delineated is it? How large is it? Can you make out brain from tumour with the naked eye?
There’s one type of tumour known as DIPG – Diffuse Intrinsic Pontine Glioma – which grows within the middle or ‘clockwork’ part of the brain. It’s insidious, completely embedded. Good cells grow networked with bad cells. They’re impossible to separate. In those cases you just have to hope chemotherapy and radiation will treat the worst of it.
Imagine two hands together with interlocking, overlapping fingers. Where does one finger end and the other begin? Which one is a brain cell and which one is a tumour cell? All you see is tumour interwoven with brain. It gives you, as a surgeon, a decision to make. ‘Do I cut off from the left knuckle? In which case I’m leaving those fingers of tumour behind. Or do I cut off at the right knuckle? In which case I’m taking all the tumour, but all those bits of brain as well.’
What we can’t do is operate down to the cellular level. We can’t go in and take individual cells out and leave alone those other cells in between. Chemotherapy can go some way towards a similar result, but the scalpel capable of the type of incisions necessary has yet to be invented. And neither, I suspect, has the surgeon.
The question is: what should this child’s treatment comprise? While the decision will ultimately be mine and the family’s, I like to take advice from my learned colleagues. Every week I sit down with the oncology, pathology and radiology departments and we work through the results for our upcoming and ongoing patients. With the right people it’s a healthy and productive way to work. And, luckily, we have the right people.
I might have four or five ‘hot’ cases in the pipeline, each at various stages of treatment, and the oncologist will have a similar number. We present each case and use each other as sounding boards. At best we’re trying to find the best possible treatment for each individual patient. At the very least we want to avoid the scenario where you’ve attempted a risky operation only for the radiologist to say, ‘Why the hell did you operate? It was obviously a very chemo-sensitive tumour.’
Some situations are black and white. After a sample biopsy or a bigger removal, the pathologists will present the slides of what they’ve seen and say as categorically as they can, ‘This is tumour X.’ Then the oncologist will say, ‘Okay, we now need to proceed with this type of treatment’ or ‘Let’s do another scan because it looks like it’s a GRT.’ This means a Gross Total Resection – it’s always so good to hear that we have removed the tumour completely. There’s little to argue with following such assertions.
It’s the cases where a biopsy has yet to be performed where opinions come more into play. Based on scans, the radiologists will give us the top two or three likelihoods. If their feeling is that a tumour is chemo-sensitive (one that will likely respond well to chemotherapy – drugs that we can give by mouth or injection) I take that on board, especially if the tumour is in a dangerous location. But if I can see that it’s in a really easy-to-get-to-place with minimal risk to the patient, then sometimes I will advocate doing the operation. Why subject anyone to months of ravaging chemo when a ‘short, sharp shock’ style of treatment can get them on the mend in days?
It’s all about consensus. About minimizing risk for the patient and providing the best care. Sometimes getting there can feel like a battle, but usually it arrives fairly organically. As it does with my latest case.
Scans show that the entire left side of Baby’s brain is taken up by tumour. Malignant or not, I put her likely lifespan, if untreated, at no more than a fortnight. The oncologist agrees. As does the radiologist. And the pathologist.
The fact that the patient is so tiny, so frail – born a month premature – means she might not survive multiple invasions. Even if we had the time. But then again, she has about as much blood as in a small glass of wine in her whole body. We need to have a clear plan when to stop – and this is likely to be decided by blood loss. Again, it’s a balance.
But it’s decided. We’re going in. And we’re going do as much as we can in one hit.
Of course, the parents ask me about the chances of success. They always do. I make it a personal point never to paint a rosier picture than we have. I don’t pull punches. In this case I am not able to say that the success rate for this type of operation is high.
Later that night, I ask myself, ‘Why do it?’ We’re looking at a patient who should, by rights, still be in the womb. What’s the point of subjecting her to the nightmare of surgery if it’s too risky? Surely she should have some chance at life. Her family should have some time with her, even if it’s only days or weeks. Without a decent track record, it could be said that there would be no justification for putting babies or their families through that kind of agony.
Let’s say I’m quietly confident we can help. But trust Dad to ask the one question I never answer. ‘Can you cure her?’ he says.
I shake my head. I won’t ever use the word ‘cure’. There are too many variables. So I say what I always say in these circumstances: ‘We can certainly aim to treat her.’
The anaesthetist is ready. His heart-monitoring equipment has been bleeping monotonously for the last ten minutes. Two large screens stand between him and my scrub nurse. On one we have the scans displayed for reference. On the other is a live view from an ultrasound machine. Between my knowledge of anatomy and the image the camera will give, I’ll have a kind of sat-nav to guide me through the brain.
First we have to get in. As Iron Maiden’s greatest hits strike up, I shave the section of the baby’s head that I need to access. Then I cut very carefully into the soft skull. At this stage, it’s so soft that we can use a pair of scissors to open it up. Carefully, I lift off the pentagon of bone and see with my own eyes what the scans had already shown. Except it’s not my own eyes.
You’ve probably seen the ridiculously exaggerated glasses that surgeons wear. If they look like little telescopes over each eye it’s because that’s what they are. They’re called ‘loupes’ and come with immense magnification. Perfect for when a millimetre out of place can mean the difference between a patient walking or not.
The loupes sit low on the nose so I can see down at my work while being able to peep over the top at my colleagues and the information on the screens. I look up only occasionally, however, just to confirm I’m where the sat-nav thinks I am. Even then I don’t move my head, just my eyes, for fear my hands will follow. It’s enough.
The baby’s brain is the size of my fist. It looks complete, rippled like a walnut. To a layman it’s probably exactly everything you’d expect from a ‘brain’. Except everyone in the room knows full well that around 50 per cent of what we’re looking at should not be there.
We know, from our anatomical training, what those ripples should look like and where those ripples should be. Through the loupes I can make out where they become distorted. That doesn’t always mean I’m looking at the tumour, just its impact. In this case it’s both.
There are many ways to proceed depending on the work ahead. In this instance, I won’t be using a scalpel. Not unless I need to. The patient is too delicate, the risks too high. Not so long ago we wouldn’t have had alternative options. Luckily for us all, we do now.
The nurse hands me a thin cylinder attached to a cable, the tip about the size of the cartridge inside a Bic pen. It’s an ultrasonic aspirator. It’s actually two cylinders, one inside another. When held against a tumour, the inner cylinder vibrates at such a frequency as to disrupt and eventually dissolve the tissue. At the same time, a thin shaft of water from between the cylinders irrigates the area to form a micro-slush, and then a vacuum sucks up the liquidized result into the inner tube. It is without doubt the safest approach we have, easily the gentlest, and, at something like £40,000 per unit, among the most expensive.
And, I think, as I look at my 12-inch-long patient, worth every penny for the lives it saves. And the quality of those lives.
I begin the work. Before my magnified eyes the tumour begins to disintegrate. The first piece is bottled and sent immediately to pathology for analysis. Then it’s back to hoovering up the rest. It’s a satisfying process, like window-cleaning or demisting a car. You can see exactly where you’ve been and where you need to go.
I move from the outside edge inwards, occasionally flicking my eyes at the screen to check there aren’t any vascular blood vessels or other crucial pathways hidden within the mess. Occasionally, I glance at the heart monitor. No change. Nothing out of the ordinary. Which is good.
For an hour I move the magic pen across the dubious mass. As I reach the border with the brain itself, I hear the theatre doors open. A registrar stands at my shoulder. The pathology results are in. ‘It looks malignant,’ she says.
‘Thanks.’ I keep working. But inside, I think Bollocks. I’m pretty sure I said it out loud as well. But we can’t give up. Not yet.
I’m virtually done with the majority of the tumour. Now for the tricky part. Even with my super vision I can’t make out where the lump ends and the brain begins. It’s not the best analogy, but if you’ve ever sat with fussy eaters during a steak course, you’ll often see them lopping off huge chunks …
‘Why are you doing that?’
‘I don’t like fat.’
‘That’s not fat.’
‘It is.’
‘It isn’t. Try it.’
Et cetera, et cetera …
With neurosurgery, you can’t just ‘try it’ and then spit it out if you don’t like it. The damage is done by then. Literally. But you can do other things. An ultrasound scanner is very good at picking up the subtle differences in tissue mass, which is key where there’s a danger of blood vessels or major junctions. For example, at the spot where tumour and brain tissue meet.
The ultrasound sits directly on the brain. The pictures appear on the screen. One look up, one down and I shave off a couple of millimetres. Another look up, another down and a few more go. I can see a corridor of maybe an inch that should be safe for me to dissolve. When I get close to the join, I might need a microscope. Precision is all. I don’t want to jeopardize Baby’s function too much, if it is malignant.
I shave as close to the border as I dare. Further down the line, when Baby is out of the woods and strong enough, a course of chemo might be able treat anything I don’t take today. The aim of today’s operation is to remove the overwhelming pressure on the brain to the best of our abilities. We’ve done that.
It’s all about playing the odds. And right now, as I declare our patient ‘treated’, those odds are in our favour. I won’t know for sure how well surgery has gone until Baby wakes up, but I feel good about it. As I stand back to let my registrar begin the clear-up and the replacing of the skull piece, the dulcet tones of Rage Against the Machine scream in my ear. It’s been a good day. Our work here has given this child, with an initial life expectancy of two weeks, an extended shot at life, an increased chance of being the baby daughter her parents dreamed of.
I’m satisfied we did all we could. More importantly, we didn’t overreach. I didn’t overreach. Aiming for the moon when you’re still on the ground is very tempting, but it can have catastrophic results. As I know from personal experience …