Isla’s Treatment: Part 4

IMG_2283As I’ve mentioned previously, it’s quite uncommon to have a baby on ICU unventilated. From a breathing point of view Isla was now fine, but all her electrolytes were rock bottom. The heart uses extra electrolytes while in an arrhythmia to try and correct itself. Dr Ackerman advised to keep Isla’s potassium above 4.5 and give her spironolactone, a diuretic which would reduce the amount of potassium lost in her urine. At the time of her cardiac arrest, spironolactine had been stopped, without us knowing, and Isla’s potassium level was 2.6. The first few days back on ICU were iv after iv, with barely any gap between them to try and maintain a healthy level of potassium, let alone her other electrolytes which had also taken a major hit.

Even when her electrolytes reached a normal level, her heart was still in danger of going into Torsades as her Mexiletine/Lidocaine balance was no where near right. I had convinced the doctors to go up to the maximum amount of Mexiletine recommended for her weight but it still wasn’t enough and they weren’t prepared to go any higher. Cue Dr Ackerman.

We were in quite a bind treatment wise. Isla’s care was technically in the hands of the cardiology team but being on ICU we were also under their care. It seemed we were in limbo. We sometimes went days without seeing a consultant. No one seemed to be taking control of the situation. After having a breakdown and rant at the ICU consultants, telling them in no uncertain terms they didn’t know what they were doing, I emailed Dr Ackerman and updated him on the current state of play.

He informed me that some people in his clinic are what is known as ultra-rapid metabolisers and need near on double the recommended dose of Mexiletine. Given she was on the maximum allowed and it was only creating a level of 1.1, she was obviously one of these people. From a treatment point of view though, the doctors would only tell me that Isla was in the therapeutic range so she didn’t need any more. Dr Ackerman’s view was that Isla’s therapeutic level was clearly more than 1.1 and we needed to increase Mexiletine. Unfortunately, this fell on deaf ears.

This is another moment when Isla’s guardian angels played their hand. A nurse who had looked after Isla a good few times and knew her and us well, was looking after Isla and listened to me ranting and read my exchanges with Dr Ackerman. She told me if I forwarded her the emails she would print them out and staple them on the front of Isla’s notes. I had shown them the emails on my phone every chance I had, but this way there was a paper trail and that was hard to ignore. We loved all the nurses we had on ICU but Kerry was extra special to us. In fact, she helped give Isla her first proper bath in a washing up bowl! I have no doubt that without her offering to print Dr Ackerman’s emails and having faith in the work I’d done, Isla wouldn’t be here. Kerry joins Teryan and Alex in Isla’s hall of fame.

We had spent a long time pushing for Isla’s care to go under one person rather than being transferred to the consultant of the week each time. After all, it was the reduction in magnesium and ad hoc reduction in Lidocaine which contributed to her cardiac arrest. Dr Johnson, who was the only consultant we hadn’t seen, came to us and said he would be happy to lead Isla’s treatment from now on. I asked him what the plan was. He held up Dr Ackerman’s emails. I burst into tears. Finally. His attitude was if the world’s leading expert on LQT is telling us to do something, we had better do it.

From the moment Dr Johnson took over Isla’s care, she came on leaps and bounds. We’re led to believe his attitude to instantly double Isla’s medication raised eyebrows amongst the other consultants. He listened to the me and the advice I’d sought. He went against the text books and the standard dosing for an unknown drug. He had the balls to do what needed to be done, what I’d begged for to happen for weeks. His attitude saved Isla’s life.

Dr Johnson’s commitment to Isla was unfaltering. After a week we made it back to HDU. Isla was fitted with a broviac line (surgically burrowed central line) to ensure there was secure iv access in case of emergency. Some evenings we’d be in HDU with Isla and Dr Johnson would appear in running or cycling gear. He’d have just been cycling by the hospital and come in to check on Isla, or he’d have an epiphany or thought about her treatment. He was amazing and again a key part in Isla’s survival. Guardian angel number 4: Dr Johnson.

Of course we have had a rocky road even after his intervention, and there’s still a lot I’m learning about Mexiletine every day by chatting to other people who use it. Only recently Isla was readmitted to Alder Hey due to a few spells of Torsades. We are learning together as a team and devising plans by email, phone and clinic. I’m sure it won’t be the last time we need the help of the cardiac team at Alder Hey but she has definitely taught them a thing or two and hopefully, if there is another Isla in the future, they’ll be able to learn from Isla’s journey. So far to date, Isla’s story and unique rhythm – Torsades, have been presented at 2 national conferences and she’s been the subject of an article for a medical journal. Dr Jones told us she’s a once in a lifetime patient.

Dr Gladman initially gave her survival odds of 1 in a million, but told us she could be that one. She really is 1 in a million.

I know it’s been a very long post. It’s been very hard to write and I’ve cried a few times doing so. However, it’s been very liberating to write this piece so even if it’s bored you to tears, it’s helped me. If you’ve made it this far, thanks for reading!


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