We were driving to Scotland for a pre-Christmas holiday in December 2018 when we got the phone call. It was 9am so I imagine the Paediatricians first call of the day. She confirmed that Riley’s x-ray had shown a premature fusion of the sutures in his skull and we were going to be referred to Great Ormond Street Hospital (GOSH). His condition is called Craniosynostosis and it occurs in one in 2000 births.
After that I remember crying.
Riley was born by emergency caesarean in the early hours of 13 August. After a ten hour labour I pushed for two hours and he just didn’t want to come out. The doctors and midwives said he bounced further back every time I pushed and after a quick consult in the operating theatre they decided the only course of action was to get him out the sunroof. Within minutes he was born and screaming.
After a few weeks of being home Riley’s dad and I noticed he never seemed to turn his head to the left and always seemed a bit uncomfortable. When I mentioned this to the health workers or doctors at check ups they said it can happen with caesarean babies as they’re not “ironed out” when they’re born. After doing a lot of research we decided to take Riley to The Oesteopathic Centre for Children (OCC) as they’d helped so many other babies in the same situation. It was on our first consultation with them when Riley was a couple of months old that we first started to think there may be something else going on.
On our second visit to the OCC we were approached by a Senior Oestopath, Christine, to talk about Riley’s head shape. She had concerns about it being a bit oval and the tightness of the sutures in his skull. I recalled during a scan at 37 weeks the ultrasound technician mentioned that Riley’s head was oval in shape but as he was transverse, this wasn’t a problem as it may just not have rounded out yet. Christine suggested we visit our GP and get them to take a further look.
Being an anxious new mother I arranged an appointment for the following day. The Doctor I met with was lovely and after doing all the standard measurements assured me there was nothing to be concerned with but would book me an appointment for a weeks time to repeat the measurements just to be sure. We were visiting the OCC once every two weeks for treatment so the GP visits happened in between appointments. After the follow up with the doctor I felt confident there was nothing to worry about. I had been “assured” by the doctor… Christine and the other oesteopaths weren’t so sure. She pressed me to see a different doctor. So I did. Same thing. Measurements were done and everything was fine. We were being dramatic. At every OCC appointment I could see the concern on the therapists faces. The sutures weren’t softening and Riley’s head was still oval. As he was growing his forehead was becoming more pronounced. Christine pushed again and told us to go to the GP and simply demand an appointment with a paediatrician. I passed this task on to my partner, Riley’s dad. I’m too much of a push over whereas he comes across a bit more intimidating than me and if we were going to get a referral to a specialist then he was better suited to fight for this.
I didn’t go with to the GP appointment but he said the doctor he met with, a third GP who did took the same measurements as all the others, could understand ours and the OCC’s concerns and would refer us to a paediatrician. Done!
We waited what felt like weeks for the referral but when Riley was about four months old we finally got to see a specialist. She chatted to us and again, did all the same measurements as the other doctors, as well as having a good prod of his head. Once again, we were assured that even though the sutures were tight there was nothing to be concerned about. I will never ever forget the words that came out her mouth next. Since we were already at the hospital we should “go for an x-ray just as a precaution but really, there’s nothing to worry about”. She said she only makes calls if it’s bad news so not to expect a call but rather wait for a letter in the post confirming that the appointment and x-ray had taken place. I left feeling relieved. We had absolutely nothing to worry about. A specialist had confirmed this and Riley had a precautionary x-ray as proof.
And then came the phone call…
We used the trip to Scotland and Christmas as a welcome distraction waiting for the referral to GOSH to come through. I’d find myself carrying on with my day and then having a breakdown when I’d see Riley being his beautiful, perfect little self. He may not have been technically perfect but to me regardless of whatever was going on, he will always be perfect. Then one day, out of the blue, I received a phone call from the Craniofacial specialist nurse to say they had a cancellation that afternoon and would we like the appointment. Without hesitation I said yes and then phoned my partner to tell him to rearrange his afternoon as we were going to GOSH. A sense of relief came over me. I felt like we were finally getting somewhere and a step closer to getting the help we needed. I also felt like we finally weren’t fighting to get answers anymore. Finally the doctors were on our side.
The team at GOSH had been forwarded all of Riley’s x-rays and medical history so it felt like our first consult with them was really a discussion on a course of action. It was clear to them that Riley would need to undergo surgery. Riley’s dad and I discussed on the way to the hospital that this wasn’t the time to become emotional and that if surgery was recommended, which after talking with the team at OCC we were convinced it would be, then choosing to save him the pain of an operation now meant long term pain down the line. It was made clear to us that the best course of action was for Riley to have springs inserted into the skull and pushed apart, to treat Sagittal Craniosynostosis – a premature fusing of the sagittal suture which runs down the middle of the skull from front to back – before he was six months old. This surgery is less invasive but can only be done on babies younger than six months due to the nature of the skull. After this age the surgery become more involved and recovery takes longer. As Riley was already nearing the five month mark, it was imperative the team prioritise his case.
Within weeks surgery had been booked and Riley and I would stay overnight in the hospital. As the procedure is considered relatively simple, albeit still major, unless there were complications we wouldn’t need an extended hospital stay so we were headed home the day after with a very swollen and sad little man.
All children born with this condition in the UK are monitored till the age of eight with a ophthalmology check up every six months to see whether there is any pressure on the optic nerves, and a neurosurgeon check up annually. Craniosynostosis, if not treated it can lead to increased pressure on the brain which can result in visual impairment, sleeping impairment, eating difficulties, or an impairment of mental development combined with a significant reduction in IQ so these check ups are vital. It was thanks to a check up earlier this year that we discovered Riley wasn’t in the clear just yet.
On separate occasions, both Riley’s dad and I had noticed him straining his eyes when looking at something, almost like he was trying to focus. I guess as a parent, especially one who has a child with a condition like Riley’s, you notice these things more. If we hadn’t been through what we had, I probably would have brushed it off but we knew we had a GOSH check up due so we thought it best to contact the Craniofacial team and let them know. Due to Covid-19 hospital check ups are being done online which meant that Riley wasn’t due to have a face-to-face appointment but as we had concerns, the team wanted him to come in so necessary tests could be done.
And there it was. Pressure on the optic nerve. A CT scan was ordered for the following week which meant Riley would need to be admitted to hospital and put under a general aesthetic during a day procedure. His dad took him to this as only one parent was allowed due to Covid regulations. This was a Thursday and by Tuesday we had the results. Another of the sutures had partially closed – the coronal suture running from temple to temple.
I didn’t take this news well. I felt dreadful for Riley who was about to celebrate his second birthday. I cried. I cried continuously for about four hours. It took the rest of the week before I was able to talk to people.
A 2/3 remodelling of his skull was needed. I won’t go into graphic detail of what the surgery involves but what you’re imagining is probably right. The second surgery was more complicated than the first and involved a full week in hospital and a longer recovery period. Riley was admitted on Monday morning and the operation took about four hours to complete. Fortunately his dad and I were able to be at the hospital together during this which was comforting. Do to Covid regulations, leading up to the surgery only one of us was allowed to be with Riley during surgery and recovery in hospital but thankfully due to a change in the rules only a week before it meant that we were both able to attend. I cannot imagine how families during the one parent rule coped. The stress of that alone was making our household unbearable and Riley hadn’t even been admitted yet.
Riley’s recovery after surgery wasn’t easy. He didn’t simply bounce back with a bit of swelling as he had after the first one. During his week long stay in hospital he became so swollen, which was to be expected, that his eyes closed for 24 hours and he was unable to see anything. There was a lot of screaming and by the end of the week every time a nurse came into his room to take his blood pressure etc, he would start to cry and need comforting. Needless to say we were all very happy to go home and be in our own space again. I’ve never seen someone so happy to see his own toys and as soon as we walked in he was organising his cars on the dining room table. This is a daily occurrence with Riley and it made us so happy to see.
We can’t say that Riley’s cranio-journey is over. We just don’t know. And I find myself watching him constantly, overanalysing everyday things like rubbing his head. I doubt my anxiety around his wellbeing will ever go away. I can say that it is extremely rare for a child with craniosynostosis to have to undergo a second operation. Riley was just the unlucky one. But saying that we have been extremely lucky. We’ve been lucky to find the OCC and have people push us to get answers when the GPs all said it was nothing (I must clarify that it’s not due to a lax attitude but rather than people simply don’t know about this condition and what to look out for). We’ve been lucky to have the level of care we have at Great Ormand Street Hospital and at no point ever needed to put a cost to Riley’s situation. Riley’s dad and I are originally from South Africa where healthcare is privatised and expensive. For a condition like his – rare – there’s no saying whether his treatment would have even been covered. So many rare diseases aren’t. I don’t believe that parents should ever be put in the situation to decide whether they can afford to give their child a normal life. Parents will do anything it takes to give their children the best opportunities they can – sadly even if it means going into debt themselves to do it. And what if you simply can’t afford the surgery? You have to watch your child grow, potentially developing disabilities knowing that if you had the money they would never have to go through this. No parent should ever be put in this situation and no child should have to suffer.
September is Craniosynostosis month and to give back, we set up a just giving page in Riley’s name with all the proceeds going to GOSH (link below). This place changes the lives of children and their families and the people who work there deserve our utmost gratitude and respect. It’s a happy place. A hopeful place. And a devastatingly sad place. To choose to be a part of a team that cares for sick children, in any capacity, is a worthwhile charity.
Written by Denise (mum)
If you would like donate to a just giving page in Riley’s name, please click here. All the proceeds will be going to GOSH.