The doctor who performed my cesarean initially announced Rex to the world with a very jovial ‘Congratulations you have a boy toddler!’
His toddler reference was because Rex was born a healthy (but not huge) 8lb,10 and very long, kicking strongly, crying loudly and with a rather large head. It was moments after the initial congratulations that the urgent whispered conversations between the medical staff began and it became obvious something was wrong…
So what is hydrocephalus?
Despite being one of the more common congenital conditions I am always surprised by how little coverage hydrocephalus receives. I had never even heard of it until it exploded into my life and changed everything. Hydrocephalus present at birth is called congenital hydrocephalus (there are other types). Simply put it is the build-up of cerebrospinal fluid (CSF) on the brain. The brain produces about a pint of CSF daily and it is used to protect the brain, remove waste products and provide nutrients. Normally CSF moves around the brain and skull and is absorbed into the blood stream and removed. Hydrocephalus occurs when the fluid is unable to move freely and, therefore, cannot be removed fast enough by the blood stream. The build-up of fluid causes ventricles (chamber type spaces inside the brain) to swell causing compression on the surrounding brain. In babies (whose skulls aren’t yet fused) this causes the head to enlarge. This pressure and swelling can result in temporary or permanent damage to the brain.
I was allowed a brief moment holding Rex before he was taken away for tests and less than two hours after giving birth I was told he had hydrocephalus. Still exhausted from a five day labour, hazy with the drugs and very much in shock I had asked if it was serious. The doctor responded with words that I will never forget ‘Yes, it’s about as serious as it can get’. I think part of me closed off in that moment and it’s taken many years to really re-open.
What causes it?
It isn’t always possible to find a reason but there are many known causes of congenital hydrocephalus ranging from associated conditions (notably spina-bifida), inherited X-linked genetic conditions, rare genetic disorders, arachnoid cysts, bleeds on the brain, certain maternal infections during pregnancy and premature births. Rex was two weeks late, all genetic tests came back okay, all infection tests came back negative, there were no bleeds on the brain, no cysts, no other health conditions at all. There was nothing that could explain why my son had developed hydrocephalus. In every other way he was a strong thriving baby. For several years not knowing the cause and not having an explanation really haunted me. I pursued every single medical avenue of exploration available and pushed doctors into doing all sorts of tests but to no avail. In my mind having an explanation would somehow have made it easier to accept in those early years. I still think it would be helpful to know but generally I don't wonder about it these days.
What is the treatment for hydrocephalus?
Congenital hydrocephalus is permanent. There is no cure, just treatments to manage it. Rex had brain surgery when he was five days old to fit a VP shunt (there are different types of shunts) just behind his right ear. This is basically a value fitted into the side of his skull. It enables the fluid to drain away into his stomach via a tube fitted under his skin. For some there is an alternative treatment to a shunt called an Endoscopic third ventriculostomy (ETV). An ETV involves the surgeon makes a hole in the floor of the brain to allow the trapped CSF to escape to the brain's surface, where it can be absorbed. There can be complications during surgery and afterwards; the holes can close over months or even years after surgery but there is less risk of infection than with a shunt.
ETVs aren’t suitable for everyone but it is an option for Rex. Shunt’s have a higher success rate with babies, which is why he had a VP shunt fitted initially but when his shunt fails (which it will) there is a decision to be made; whether to replace the shunt that has worked well for many years or try an ETV, which could have better long term benefits but is new to us and I am told carries more risk during surgery. Initially our surgeon recommended Rex had an ETV at two years old, I wasn’t aware at the time but this was because he assumed we, like many, would have experienced repeated issues with Rex’s shunt by that time.
So for two years I was in a state of constant count down to this planned brain surgery and I am sure in hindsight this looming threat was a significant factor in prolonging my period of shock and shut down. But when the time arrived Rex’s shunt was working so well there was no question of voluntarily undergoing brain surgery. We would simply have to wait for his shunt to fail in its own time before making the decision between replacing the shunt and the ETV. This was a turning point for me emotionally; I realised this waiting game was not going to end. It was a constant and would never ever go away. There is no permanent cure for hydrocephalus and even after this next surgery there would be more. This was the reality I had to accept so that Rex would too and could live without constantly feeling afraid.
So many brain surgeries
I was told by doctors that most children with hydrocephalus will have undergone approximately four brain surgeries by the time they are five years old. Rex is nearly at his ninth birthday and has not had any further brain surgery, although he has had a few other associated procedures including a lumbar puncture (spinal tap), CT and MRI scans. The reason for the number of surgeries is that shunts come with complications. They can get infected, malfunction intermittently, over drain/under drain or block completely. Symptoms can appear slowly and be mild in nature initially or they can be sudden and extreme.
Shunt failure? Who knows...
It is the intermittent malfunctions that scare me the most. These can be very unpredictable and hard to recognise. They can cause reoccurring periods of headaches, nausea and dizziness, sometimes regularly sometimes with weeks or even months in between. They could be extreme and involve passing out or they could be fairly mild and pass as a ‘normal’ ache or bug. Other symptoms to look for include tiredness, irritability and changes in character. Just imagine how hard it is to use these every day behaviours to identify shunt failure in a child, in particular a tired stroppy toddler or a moody lethargic teenager. Ideally you need to undergo an MRI whilst experiencing the symptoms to know for certain if the issues are shunt related but if they are irregular and short lived this often isn't possible.
Two years ago out of the blue Rex suddenly said his head was exploding in pain, three minutes later he lay down on the floor and went to sleep. He wasn’t unconscious but I couldn’t keep him awake either. He was floppy and unresponsive. I took him straight to hospital but by the time we got there he was waking up and whilst in A&E he perked up and felt ok again. His surgeon thinks it was likely to be a temporary large blockage in his shunt that caused a build-up of fluid resulting in sudden pain and sleepiness. The pressure exerted by the fluid build-up could had pushed the blockage through the shunt and away down the tubing meaning the pain receded and Rex felt ok again. Thankfully it has never happened since but often once a shunt starts to block it can be a sign it is damaged and will need replacing. Again though without a scan during that critical period there is no way of knowing for certain. It could have been a migraine or some other non-hydrocephalus related issue. This was a one off but imagine if that happened every few weeks or even days. You try to get a scan undertaken but with timescales, distances and availability of equipment that can be really difficult. At what point do you and the surgeon make a judgement call that the probability of it being shunt related is high enough to put your child through brain surgery? Thankfully I have not been in this position yet but many parents whose children have hydrocephalus find themselves faced with this prospect.
Side effects of hydrocephalus?
In addition to the shunt complications and side effects congenital hydrocephalus is also associated with learning difficulties affecting concentration, reasoning, short-term memory, co-ordination, motivation, organisational skills and language. Physical effects may include visual and motor skills problems, hormonal issues and early puberty in children. The consequences of hydrocephalus can vary hugely from one individual to another. There are no definites where hydrocephalus is concerned.
My advice to other parents
I have two pieces of advice to new parents whose child has been diagnosed with hydrocephalus:
Do not make any assumptions based on the experiences of others. Some children will have numerous challenges and surgeries to overcome whereas others, like Rex, can have very few problems. Accept this and just watch your child grow. See what they can do and what they need help with. That is the only way to know how it will impact your child. The sooner you accept that, the sooner you can stop comparing and predicting and begin to enjoy being a parent to your child.
It is a permanent condition and the temptation is to worry endlessly. Do the opposite. Let it teach you to not stress the little things. Be grateful for all the good things you do have and don't forget your baby will still grow up. Don't miss precious moments because you are focused on something that you will never be able to change. I wish I had learnt this sooner. Hydrocephalus feels insurmountable initially but trust me however many challenges it causes, your child is so much much more than this one health condition. Enjoy and celebrate them. .
Interested to read some more?
The National Health Service (NHS) provides a short summary of Hydrocephalus and the UK charity SHINE provides really useful fact sheets and resources for individuals, carers and schools.
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