1 in 10. That’s the survival rate for an out of hospital cardiac arrest. Well figures place it around 8% to be exact. Given the odds Isla’s overcome so far in her little life that doesn’t actually sound too bad. Although we had the defibrillator we weren’t quick enough with it – you can’t shock a heart that isn’t beating. CPR isn’t something a parent should ever have to perform on their child. I still have nightmares about that day and I’m thankful for the training I received from the resus team at Alder Hey when Isla was first discharged. Ironically we had been organising a refresher course prior to Isla’s arrest but never got round to it. Fortunately, I obviously remembered enough. I’ve since had a refresher course but hopefully now I will never need to use it. I know I’m in a different boat because Isla is vulnerable but if you haven’t had recent CPR training, please get some. It might not be someone in your family that you save but you never know when it might come in handy and you might just save the life of someone’s loved one.
Isla had her ICD fitted 3 weeks ago and we are now adjusting to a new ‘normal’. I am no longer part of Isla’s medical team, I am no longer required! That’s a massive weight off my shoulders. We don’t have to worry about being quick enough with the defib now or carting it around (it isn’t light) so it is a relief that she has one inside her, waiting and watching. I don’t have to do daily or even weekly ECGs anymore. I could but there’s not really any point. It will show that her QT is really long, but they aren’t bothered about that anymore, it’s just a number. I’m not saying I won’t do any… but the necessity for me to do it to know if her meds are working as they should is no longer there.
The team at GOSH have completely changed the philosophy around Isla’s treatment. If you made it through my post about the different types of Long QT (and why wouldn’t you have? It was riveting!) you’ll remember Isla’s Long QT type, type 3, happens when the heart rate suddenly drops. My analogy for type 3 was about a car doing an emergency stop. For Isla I probably need to amend it slightly. Her heart was like a Ferrari in city centre traffic. Fast acceleration. Slow down. Fast acceleration. Slow down… etc. It is the repeated rapid change in her heart rate which seems to be her trigger for the Torsades. Although reducing the QT interval would reduce the likelihood of a badly mistimed beat, Isla’s fluctuating heart rate seems to have been the issue. Her QT could have been medicated with Mexiletine to the point where it was normal but she could have still gone wrong. The GOSH team have taken a different approach and basically prevented her own rhythm ever taking over. It seems to be working as since having the device implanted she has only had 4 ectopic beats. I’ve probably had more myself. The dose of beta blocker she is on though is insane, even for an adult, the fact she copes with it is amazing!
Isla’s Mexiletine dose is still higher than they would like. She’s still getting around 22mg/kg. It is significantly less than she was on… but significantly more than anyone would want (which is around 5mg/kg). It probably is the cause of her developmental delay but it is also what keeps her alive. It is a new balance for her and hopefully now she’s being monitored by her device she will be able to outgrow her dose and it’ll come down in toxicity. Mexiletine is a sodium channel blocker and helps keeps her electrolyte ion channels within normal timings. However, it isn’t cardio-selective so it also blocks the sodium channel in her brain. What impact that has on her, no one knows, but we have seen an improvement in her cognitive ability since her dose has been reduced.
I’ve called this post ‘Isla 2.0’… in reality she was 2.0 when she had her pacemaker fitted. It served its purpose and it was the only option at the time but her new device should really change things for her and it’ll give us more wiggle room with meds. She now automatically uploads data every Monday night and the team in London can see any events or trends her heart is doing. It’s really clever stuff! I’m really hoping once her dose has come down to ‘normal’ levels through her weight gain, if her heart allows and she doesn’t need it increasing, that we will see even more of ‘Isla’. With a fully functioning and flowing sodium channel in her brain we should get Isla 3.0 but that should come with that really long word; time. In September Isla will be starting nursery part time and more support should be starting to fall in to place for her. 2019 should be the year of Isla with lots of things to look forward to.
Now the ICD is in it starts a new chapter in Isla’s journey. Isla 2.0.
The Long Cutie 