In history books 2020 will always be the year of ‘The Pandemic’. A year so many lost their lives to this horrific virus. A year we applauded our NHS front line workers every night for a few weeks and then apparently forgot them just as quickly. Condolences to anyone who lost loved ones this year. Much love to those furloughed and who lost their jobs. It’s been a very harsh year. However, for me and Isla, looking back with 20:20 vision, this year wasn’t all bad…
For me, 2020 will always be the year Isla started school properly, something I wasn’t sure would ever happen. Isla’s life hangs in the balance daily and given the severity of her condition, it’s seems most in the cardiac world had secretly written her off. Sad but true. Starting school was a massive milestone for Isla, I know she’s been in nursery in the same school setting, and is in the same classroom, but it’s different (if different means the same).
This last year has been a rollercoaster medically. The only admissions Isla’s had this year have been planned ones… that’s something pretty special! First time in 5 years there’s been no emergencies. I say no emergencies… she’s had a good number of arrhythmias, a few descending into cardiac arrests (he says casually), 4 planned admissions (one cut short by London’s failing sewer systems), 3 life saving shocks, 2 cardiac surgeries, 1 new medication, and a partridge in a pear tree. Any other year we would have been admitted to hospital following the cardiac arrests but due to Covid and because she’s got the ICD it was safer to stay at home. The no emergency admissions then is a slight misrepresentation but given they’d have only monitored anyway and we coped (survived) at home is something to be positive about.
On the other hand (Whisper) The last 6 months of 2020 have been cardiac arrest free! How amazing is that!? The longest period ever. Ever. This is most likely due to starting the combi therapy of Mexiletine+Flecainide. Something Isla’s doctors were skeptical of but given the denervations did nothing but give her dry hands (well they might have done more but who knows, she still had events and shocks after them), it was to them a Hail Mary. I will be eternally grateful to Georgia Brugada for advocating this course of action. I think it’s amazing that someone as busy as her responds to anxious parents like myself. I have had email conversations with pretty much every world renowned long qt doctor available, and all have given their two cents and advice, which again is amazing and I am thankful for their input; I don’t think there’s many medical conditions where this kind of support from the top dogs is available. It was around two years ago, before Isla eventually got her ICD, that I first contacted Georgia and she took the time to discuss with Dr Johnson her patient who presented similarly to Isla and shared her treatment plan. Ultimately, it didn’t happen, but it was something I always kept in the back of my mind as Mexiletine alone never gave Isla the safety I could be happy with. Although both sodium channel blockers they work at different stages in the electrical current (ok we might be talking 100th of a millisecond) and we know from Isla trying Flecainide at 7 weeks old that it did something, for me, the combination of the 2 drugs might be the answer. Great Ormond Street didn’t want to try this until the tried and tested bilateral denervations were done, but when they didn’t work were prepared try ‘out the box’ thinking. Without Georgia Brugada and her work and reputation there is no way this would have happened. Absolutely no way. Although she’s in Spain and we may never meet her, she’s now ingrained in Isla’s story and one of her guardian angels. Thank you Georgia, for us, you’ve saved 2020.
Alongside having the denervations and adding Flecainide, part of the compromise and plan was to add Isla to the transplant list. It is pretty rare and extreme to be listed for transplant due to ‘irretractable arrhythmias’ but then Isla is a pretty rare and extreme child. This was, without question, the hardest part of 2020. I remember Luke ringing and saying that this is what they wanted to do. I got off the phone and cried like I’ve never cried before. I was heartbroken. It was effectively them giving up. So many people had their say on transplants but until you’ve had that discussion with the transplant team you can’t really have an opinion. It really is a last resort, and don’t get me wrong, if it has to happen and Isla needs it, I would rip my own out and she could have it, but a transplant buys you time, it doesn’t fix everything. My hope is that in 10 years time technology will overtake biology and Isla can have an Ironman heart which will not only last forever without need for anti rejection medications, but it’ll give her unlimited strength and power. I’m not ready to put my faith in that hope yet though. Isla will remain a constant feature on the transplant list since she is still super high risk of cardiac arrest, which is kind of a relief to have as a safety net, I’m just holding on to the hope that the medications improve and Isla’s long qt can be managed enough to never need an actual donor heart.
You only have to look through the pictures from the last year to see the progress Isla has made and how happy she is. I have no doubt that the reduction in Mexiletine, allowed for by the addition of Flecainide, is responsible for Isla’s steps in development. She is so much more aware, responsive, interactive; it’s amazing to see. Her Mexiletine dose is still way way way above normal so we know it’s affecting the neurological sodium channels, but at the moment there’s nothing we can do about that. She needs to be alive to develop and at the moment these levels of medications are keeping her alive so I’m in no rush to change things for the chance of a word out of her. She will do things at her own pace regardless! Fingers crossed there are new drugs in development which will be less toxic for Isla and still keep her safe, we just need to keep her alive until then. Isla is still making leaps and bounds in her development and smashing her targets and goals at school. She’s communicating much more effectively and certainly let’s you know what she wants and likes. She can be a real madam now, which is great because it means she has an opinion and can express it.
On a personal level, 2020 has been tough and I wouldn’t have made it through without my friends, family and some of you guys reading this. Through lockdown (and all my life to be honest) my mum has been Wonder Woman. She is listed as one of Isla’s carers and my god does she care about Isla. She’s here every day without fail (arthritis and all) to see Isla and help me. She’s simply amazing and goes way above and beyond for me, especially on days where I’m feeling defeated. This year I took up the option of speaking to a psychologist again, initially once a fortnight but this has increased to weekly. I’ve done psychology appointments before and found them so-so. I guess the increase of video calls as a result of Covid has made it easier and more appealing to speak to someone about my mental health and struggles but I’m glad I tapped into it again. After the transplant chat I’ve had some dark days and while I still do, it has helped and things do seem brighter. Isla is the light of my light and it is the ‘what ifs’ about that light going out which breaks me. I know everyone can have these fears but when it’s a constant threat and can happen any second it’s not easy to live with.
It’d be very easy for me to focus on the negatives of 2020 but there’s so much to be grateful for too. Much love to our front line workers working tirelessly in masks to save lives, despite being stiffed of a pay rise while those failing to lead the country reward themselves for a job badly done. Huge love to Georgia Brugada for advocating for Isla’s course of treatment. Thank you to you all for your messages of support this year, it means a lot, even if I only acknowledge them with a like sometimes. Most of all thank you to Isla for smiling every day and giving me perspective when I lose sight of what’s important. It’s another year where Isla’s been battered and bruised by her condition but she still finds the time to laugh and find joy in life. Let’s hope 2021 gives everyone more joy than 2020 but whatever happens find something to smile or laugh about each day.
Happy New Year!
The Long Cutie 