Let’s talk Hydrocephalus

This week it’s Hydrocephalus awareness week in the UK and it’s the perfect opportunity to give a bit of background to our story. An opportunity to try and explain what this illness is and help to spread a little awareness of our own. I know from personal experience just how much this life changing condition is unknown to so many. We had no knowledge of this illness until it literally ripped our world apart, how is it that possible for an illness which is said to affect up to 1 in 1000 babies born every year to be so unknown? An illness which is just as common as Down’s Syndrome and more prevalent than Spina Bifida or brain tumours. Hydrocephalus is the most common reason for brain surgery in children so how is it I knew nothing of it until my baby was being prepped for surgery? Sadly, the lack of knowledge of the illness is not just limited to parents like us it is unfortunately very misunderstood by professionals too. Before our little boys’ surgery, I had no idea what a “hydrocephalus baby” looked like, I now look back at pictures of our baby roughly two years ago and realise with hindsight and knowledge the signs of hydrocephalus were there to see but sadly I didn’t have that knowledge then. So how did we get here?

There are two forms of hydrocephalus in children, congenital and acquired. Congenital hydrocephalus is when a baby is born with the condition as it has developed in the womb. Acquired is when it develops at some point after birth. In our case Charlie’s condition was acquired post birth but we may never know the reason why. Charlie was born at 36 weeks with a head measuring on the 50th centile, he had no development issues in the early months of life and that can sometimes be a source of great sadness, it is unbelievably hard to see your child develop skills only to lose them and have to work so hard to get them back. On reflection it was stated to us that issues with Charlie’s head growth should have been spotted at around eight weeks, unfortunately for us that was not the case. At roughly five months we started to notice he would outgrow hats; his head was large for his age and he couldn’t sit independently. Questions were asked and some of the responses included “big head big brains” and “does his dad have a large head?” in some cases we were reassured that his development may have been affected by his slight prematurity, this was certainly the case when it came to his digestive issues and the fact he was sick quite regularly but only small amounts. As Christmas approached the novelty hats were everywhere but the age appropriate hats didn’t seem to fit. We tried a first Christmas Santa hat that was intended for 6 to 12 months, one week it fitted very snugly and only a couple of weeks later Christmas Day it would no longer fit, no way no how, it was centimetres too small. At the time we marvelled at the size of his head and asked ourselves just how big was it? Underneath the light-hearted comments we were concerned and measured his head and discovered it was 54 CM, it was so large we could not even chart it on the baby growth chart. His fontanelle was huge, like a letter box and he still was unable to sit without support in fact he even struggled in highchairs. We were worried, this couldn’t be right so we went to see a doctor and told him our concerns but unfortunately he had limited knowledge of Hydrocephalus and stated he was a big boy and he was not overly concerned, he calmly stated that he would book an appointment at the hospital, the earliest available was in four weeks which he said was quite a quick turnaround. He didn’t seem bothered, said he couldn’t see any negative markers and said to bring him back if he deteriorated. He didn’t explain what deteriorate meant. Unfortunately for Charlie he was teething and the Calpol was in some ways masking the pain from the pressure in his head and we didn’t know what we were looking for, he was happy, he was smiling, and the doctor wasn’t worried. When he stopped holding his bottle, we thought it was a phase, we had never heard of sunsetting so didn’t realise he had a downward gaze, and nobody told us to watch for it. It breaks my heart I was so clueless, I feel I failed him and the thought of the pain he must have felt and smiled through makes me sob. I sometimes look back to that visit to the doctor and I’m angry I was so naïve, I should have fought harder, I should have insisted he was seen quicker but the truth is I didn’t know and I trusted someone who should have known, someone who should not have left him to suffer.
Fast forward four weeks and we entered the paediatrician’s office for what we thought was a routine appointment, it was the moment everything changed. The paediatrician immediately saw what the GP had not, and he saw the situation was serious. Looking back, he stayed so calm, I think mainly to keep me as calm as possible. He said that he needed to see why his head was so large, it was just routine, but he wanted to check. He said it could wait but as we were there he might as well see if someone could look at it now, he called a friend, he asked for a favour. He stated that his fontanelle was “wide open” and could they ultrasound his head to see if they could see the cause of the growth, he told us to go straight over. He said after the scan to go home and he would call us after clinic with the outcome. Charlie’s head was scanned, and the doctor said simply she could see a blockage and could we take a seat while she called the paediatrician, at this point I felt fear I started to cry. She came back and told us to return straight to the paediatric clinic, I asked if Charlie was ok. She explained briefly that something was stopping fluid from draining and they needed to find out why still nobody said hydrocephalus. Randomly I recall us stopping for coffee on the way back to the clinic, but I was scared and when we got to the waiting room I couldn’t stop the tears from falling. A lovely nurse called Emma took us into a room away from the main waiting area and tried to keep me calm while we waited to see the doctor. When he had finished with his patient we went back in and he told us the fluid around Charlie’s brain was not draining properly and they needed to do some tests to find out why, he told me not to worry and this was routine (I know now that is completely untrue) he said it would be quicker to carry out the tests as an inpatient and with this in mind could we pack a bag and go straight to the children’s ward in Halifax where a bed was waiting. It all seemed very surreal, we got the bag we went to the hospital. They asked questions about him, his behaviour and why we thought there was a problem. A nurse called Heather was so lovely as I struggled to grasp what was happening, I knew something was wrong, but nobody really said what. I asked a doctor if he would be ok and she said, “we can only hope”, that one phrase was terrifying nobody would tell me what was wrong and equally it was obviously so serious they couldn’t reassure me it would be ok. They said they needed to do an MRI and if we were happy to take him home for the night we could and bring him back at 6am, again mixed signals as surely they wouldn’t allow him home if he was seriously ill. We went home, we did normal family things, we watched the Gruffalo. Early the next morning we returned to the hospital, it was snowing and Charlie’s first snow experience it’s funny the things you remember. A cot was waiting and Heather, the nurse from the previous day, had switched wards to stay with us. It was explained they would give Charlie light sedation and then take him to be scanned and Leeds were awaiting the results, the ward sister took one look at him and said “bless look he’s sunsetting” that was the first time I had heard that and had no clue what she meant. They gave Charlie his sedative and he was placed on my lap on a wheelchair and wheeled to the scanner, they then took him from me, and he looked so small. We waited, hearing the loud clicks and buzzing of the scanners. I felt sick at the thought of my little boy alone in the scanner and it felt like a lifetime until they brought him back. What followed was a blur, travelling down the M62 at 80 miles an hour in an ambulance with Charlie in my arms, feeling dizzy and sick. We arrived in Leeds with me looking worse than Charlie, stripped off despite the cold weather and being sick into a paper hat. I was in shock I just didn’t know that at the time. I can honestly say everything happened so quickly I didn’t have time to think, a surgeon told us that he needed a shunt, that he had hydrocephalus, that they didn’t know why but they would operate first and look for reasons later. There was no time to waste, the situation was critical. We were handed a thin leaflet and in this flimsy document was an attempt to explain what hydrocephalus is, what a shunt does, what happens if it fails and things to avoid. I remember my husband being saddened to read heading a football was a no go, I recall him saying that he was a boy he would want to play football. At the time we didn’t understand the massiveness of the situation, what impact it would have on all our lives and that we had much bigger issues to face than heading a football, or not as the case may be. We handed our seven month old baby over for brain surgery, I was not at all brave and it was Daddy who held him as he fell asleep. We waited patiently, we had lunch, we went for a walk. Then we returned to find our baby with bandages around his head, having him back in my arms was accompanied by the biggest feeling of relief. The nursing staff handed us a small sample tube of hair, the surgeon had requested it be kept as it was his first haircut. It was such a small act of kindness and it meant so much, in the craziness we hadn’t even considered something so “normal” as a lock of hair. The snow came down heavily outside and that was a blessing as it meant my husband could stay with us through the night. I was terrified of the beep of the machines, the sight of him with his bandages, how pale and helpless he looked. Night finally became day, he was clearly in some discomfort but seemed happy, we watched Lilo and Stitch, Daddy read Room on the Broom. A further MRI took place and no abnormalities were found with his spine, we later discovered that due to the extent of his hydrocephalus they feared a spinal abnormality was stopping the cerebrospinal fluid (CSF) from draining down the spine as it normally would. Once a spinal issue was discounted the surgeon said they could see some evidence of a bleed and they believed this or a low grade infection at some point in the first days or weeks of his life had caused the blockage. It is beyond hard to not know what caused this and I don’t think I will ever be at peace with that level of ambiguity about something so serious, so life changing. We were told he would never be able to drain the CSF without the shunt and would need it for the rest of his life. The surgeon was happy with his recovery and suggested that due to the weather we may want to go home as a family. So that’s what we did, we wrapped our baby up against the cold, took note of the times of when the next doses of painkillers were due, when we could remove the bandages and left for home. We took our thin leaflet and our scant understanding of what had happened and took the first steps into the next chapter of our story. We knew very little, we didn’t know that the illness would affect every element of Charlie’s life, we thought he was fixed, we naively thought that’s what the shunt did. The following day we went to music class with him, we were thinking things would go back to normal now. He would catch back up and this would be a blip. Hydrocephalus is not a blip, it is all consuming, it changes everything. Sadly, we had to find our way without directions and for a little while we were lost in the dark with no torch. We stumbled, we got lost, but along the way we found some amazing people who either helped us to understand or who learnt with us and helped to rebuild Charlie’s life. It’s a rollercoaster, I am sad that I didn’t know the signs, I am sad that others didn’t see the problem until it was nearly too late, but I have also been enriched by the people we have met and the beauty in my child and his resilience. We have suffered difficulty, but we have also been blessed to meet people that believe in our boy and want to help him in any way they can. I want everyone to know what this illness is, to know the signs and symptoms but also to understand the fallout of this illness and its lasting impact. For the sake of our amazing little boy we strive to make change and hope we can make a difference. The handbook needs to be bigger, the directions for help clearer. Together we will move mountains.