This blog post is all about my first experience visiting Great Ormond Street Hospital, for my (at the time) 5 month old son, as an outpatient for his heart problems. I take you through my thoughts, feelings, questions and anticipations before, during and after our appointment.
Jaxon has had appointments with a specialist cardiologist once a month since they discovered his hypertrophic cardiomyopathy (enlarged heart). He saw them in hospital when it was diagnosed (I just heard the words no new mother should ever hear..), once in December and then recently in January.
In December his cardiologist explained it wasn’t getting any better but from the diagnosis talk in November I hadn’t even thought it getting better was on the cards. I was under the impression we were hoping he just managed with it. She increased his medication dosage for the 4th time and advised she would send the images of his heart to Great Ormond Street (GOSH).
In January I returned and she said GOSH had written back to her that they hadn’t seen this type of hypertrophic cardiomyopathy before either. How has Great Ormond Street never seen this before? So Jaxon has two rare conditions (cytomegalovirus & 1p36 deletion syndrome) and a heart condition that so far no specialist at two of the best hospitals in the UK have seen? How?! After scanning his heart again his cardiologist expressed that she was extremely concerned and that the phone call she’d nipped outside during our appointment to make was to GOSH asking them to see him within the next two weeks. She was that worried about him.
This confused me a little because, if you recall, my first meeting with her with the diagnosis, she had told me he was at high risk of SIDS (sudden infant death), that there was nothing they could do about it, and it was just a waiting game to see how he coped with his large heart. It doesn’t get much worse than being told your child could die at any moment. So by this point I couldn’t quite understand why we were more worried now? I’d been worried, I’d accepted the risk, I’d learnt to get on with it and just enjoy the fact we weren’t in hospital anymore. Hoping for the best but aware of worst. So why was she now more concerned? I didn’t think there was anything more concerning. Apparently previously it appeared the left side of his heart muscle was thickened but now it appeared the right side was also. I asked “doesn’t that mean it’s progressive?” and she said she’d have to look back at previous scans to know for sure.
So I’m en route to GOSH. It’s another long day. Jaxon developed a temperature of 39.3 two nights ago which led to a 111 call before midnight followed by a 999 call when he started shaking and had blue hands and feet. Despite me listing his medical conditions it took 4 hours for an ambulance to arrive. We got to the hospital at around 3am and I didn’t sleep until we left the hospital late last night. The more I think about it the more I don’t know what we’re going to GOSH for. Jaxons cardiologist had briefly mentioned a heart transplant in passing. Was that what we would be discussing today? Was it going to be more bad news? I had no idea what to expect.
We arrived at GOSH. I didn’t realise how central it is. 10 minute walk from St.Pancras. At least it wasn’t a hassle to find. There’s plenty of staff at GOSH to guide you to where you need to be making your appointment smooth. Jaxon was checked in to an outpatient clinic where he had his observations measured (temperature, blood pressure, heart rate etc). We were taken to cardiology by some kind of tour guide for an ECG (this checks the heart’s rhythm and electrical activity) and an echocardiogram (this is a sonogram that creates an image of the heart, like an ultrasound). Here were we told GOSH do their own scans every time so they know it’s the most recent and accurate, which was reassuring. From there we were taken back to the original clinic to see the cardiologist. This whole process took 2-3 hours, there was a lot of waiting in between. Before seeing the cardiologist a nurse took us into a room and went through a family history.
When the cardiologist saw us he went through the stages; the diagnosis, what this means, what treatment there is and prognosis. The diagnosis, what this means and treatment were all repeating what I’d heard before. He explained this was a specific hypertrophic cardiomyopathy clinic and team and for a hypertrophic cardiomyopathy there were mild and severe cases. Jaxon was closer to severe than mild looking at todays scans (so moderate?). Again, they couldn’t tell me the cause but they had decided amongst themselves that this must be a rare outcome of Jaxons rare 1p36 deletion syndrome (I mean, really kid?!). This didn’t surprise me, no one seemed to have a clue so guesswork made sense. Or should I say, assuming it was related to the rare condition that no one fully understands would make sense. If not many people had the condition, its not so crazy that he would be the first to display a previously unseen symptom.
However. It was the prognosis I wanted to know. But once again, the prognosis was the same disappointing; “we don’t know”. We came all this way for that? The doctor asked if he had told me anything different and I had to be honest. I told him politely, no it was all pretty similar to what our cardiologist had said previously in Nottingham. I explained my confusion “I was told back in November he’s at high risk of sudden death, I can’t understand why we’re now more worried? I’ve heard the worst, it doesn’t make any sense to me, why are we here now?”. At this point I was surprised to hear he would never have said he was at high risk of sudden death to me. This doctor explained that in fact, Jaxon was not at risk of sudden death but instead that symptoms would get worse over time and we would see a gradual deterioration. He explained that they could increase the beta-blocker Jaxon was on to lower his heart rate (the one that had already been increased 4x) and there were other medications to manage different symptoms like his shortness of breath but I explained Jaxon was unwell with a urine infection and so how he appeared right now was not his normal. It was agreed that medication would be left as it is for now. My mum mentioned the heart transplant, she knew our cardiologist in Nottingham had mentioned it. The doctor advised we weren’t there yet. Well, actually, the first thing he said was that baby’s hearts don’t become available very often, in fact hardly ever (which was something that had tormented me when I had thought about the possibility it would be suggested – nobody wants a baby’s heart to become available!). And that was that, they’ll send us an appointment to return in 3 months whilst we keep seeing out cardiologist in Nottingham on a monthly basis.
I couldn’t fault the care at GOSH, everyone was welcoming and helpful. It was a long day but all hospital appointments were long days. But on the train back, trying to piece it all together, all I had gained was that Jaxon wouldn’t suddenly die in his sleep. I felt relieved, but at the same time, not at all relieved. It was such a confusing feeling. Great, my son won’t suddenly die, but they didn’t tell me he won’t die. They just said it would be gradual. Is that even any better? Could that be worse? Pain, suffering, struggling? Other than that, all I had was a piece of mind that he was under Great Ormond Street, supposedly the best doctors he could have. Then why didn’t we get anywhere today? Why have I learnt nothing? Why didn’t they have answers? Why didn’t they have solutions? I knew I had been scared of them saying he needed a heart transplant but was it better to have no option except to sit and wait. The same “we’ll know better if he manages his first year” was mentioned. What did this even mean?
One thing I have to note post appointment is that whenever doctors write to each other about Jaxon, I always receive a copy and I always read them. I was shocked to read in a paragraph about discussions with “mum”, in the doctor’s letter from GOSH to Nottingham, that a heart transplant would not be a suitable option. Now there is an alarming difference between “not right now” and “never”. That threw me a little and will be at the top of my agenda on our next visit in April.
Te letter also confirmed Jaxons abnormal need to be rare..
It appears I’m between a consultant cardiologist from a very reputable cardiac centre (Glenfield hospital) who is extremely concerned, and a consultant cardiologist who specialises in HCM at a renowned children’s hospital who is a lot less concerned. Is that because he (the GOSH specialist) sees it all day everyday and therefore knows more in that field? Is that a good thing because he’s knowledgable in the area or a bad thing because he’s actually just used to it and not so emotive? My consultant during pregnancy was very blasé which was great at keeping me calm and optimistic but that didn’t prove to mean he was right and everything was okay with my baby, did it?
Find out more about Great Ormond Street hospital here: Great Ormond Street Hospital
If you would like more information on a Hypertrophic Cardiomyopathy click here :Childrens Heart Federation HCM Factsheet (Please do not be confused by the information in the factsheet regards to Jaxon. The factsheet is an overall overview, Jaxons case is very extreme and rare and does not relate directly.)
Thank you for reading.
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Please comment with your thoughts and opinions reading this. Your feedback is what keeps me posting and writing is proving to be a helpful therapy for me right now. A therapy I wouldn’t be indulging in without your interest.