The cardiac ward at Alder Hey agreed to take her in. Although seizures are not a cardiac issue, her heart condition makes Isla a very complex patient who a lot of hospitals or even wards aren’t equipped to cope with.
The other reason that Isla was really a cardiac case in this instance is the fact that some of the side effects of Isla’s Mexiletine, the only thing that keeps her alive, are: nausea, vomiting, diarrhoea, upset stomach, constipation, headaches, tremors and seizures. Seizures are an extreme reaction when the dose becomes toxic, which is the line we play with to keep Isla safe, so we always knew there was a potential for them to happen. We just weren’t prepared for them to happen.
It turned out that Isla’s Mexiletine blood serum level had jumped to 3.6. A normal range for the drug is between 1 and 2. Upon discussion with Dr Ackerman in America, he was convinced this was the cause of her seizures. We began reducing her Mexiletine dose while carefully monitoring her heart, and added in an anti-seizure medicine to help control the side effects. The Alder Hey doctors consider this an American solution to medical issues; treat the problem, then treat the side effects and so on.
After trying 3 different anti-epilepsy drugs, with numerous iv rescue drugs and one ill-fated rectal rescue drug (poor nurse Katie) where nothing worked, we were told Isla had irretractible epilepsy and it was likely no drug would actually control her seizures. This was heartbreaking news. Now our baby girl with a complex heart issue also had epilepsy. I remember wanting to punch the smug junior doctor who consoled Kerry, who’d told us it was epilepsy all along and she should be on Keppra. Of course I didn’t, and wouldn’t, but I felt so angry and lost.
Unsurprisingly, I didn’t accept the diagnosis of epilepsy. There was a reason for her seizures. There was a reason I could time her seizures almost to the minute. There was a reason I could tell the baby massage crew that they were welcome to come and give Isla a massage, but she would have a seizure in 15 minutes (She did by the way). To me that wasn’t epilepsy. We charted all of her seizures and what she did afterwards. To me it was still a stomach and pain issue as she was totally against having any milk and would arch and squirm to avoid it. After each seizure she’d have a big release of wind and then settle down. We had a brief spell at home before a return to Bolton Royal and then a stay on the Neuro ward at Alder Hey… fortunately we made it home the day before her 1st birthday and for Christmas.
It wasn’t until I’d fully explored the route of gastroenterology that things started making sense with Isla’s seizures. I, and I say I, in the way that I convinced doctors that there was valid cause, subjected Isla to a vast range of tests and drugs. For a long time I was convinced it was Small Intestinal Bacterial Overgrowth, which is a result of slow moving food, causing and feeding bacteria in the gut creating excess gas production. The gas then bubbles back up into the stomach and leads to bloating and pain. Even the neurologists believed that this was a possible scenario and the pain may be at the root of her seizures. The gastroenterologists even signed off on the drug to treat it, however, the one they prescribed I rejected as it could prolong her qt interval. Finally, we were assigned a gastric consultant who agreed to do an endoscopy. Anaesthetic for Isla is incredibly risky so we didn’t take the decision to go through with the procedure lightly; I was convinced that the camera would show Isla’s stomach to be a mess and causing her pain.
It turns out I was wrong… it wasn’t the first time… and it won’t be the last. However, my pushing for the endoscopy was the key for the cure.
After one failed attempt to arrange the scope, due to a lack of communication between the cardiology team and the gastro team, we had an incredibly scary day at Alder Hey. As I mentioned, Isla is very difficult to anaesthetise as most anaesthetics prolong the qt interval and so she needs to be carefully monitored during any procedure. The first time it was arranged a cardiac anaesthetist hadn’t been informed and when he was, he wasn’t happy for the procedure to go ahead without careful thought and planning. When things like this happen you realise the complexities of what actually goes on in a hospital. So many teams are involved and there’s an element of risk doing any surgery, you appreciate the amount of work that goes into what is in essence a simple, routine procedure.
That was a little off track… so… to have a procedure or surgery, the patient has to be nil by mouth for a certain length of time prior to it. This meant that Isla couldn’t have any food or milk alongside her Mexiletine or anti-epilepsy drugs, both of which are recommended to be taken with food. Just as we were in the waiting room for the surgeons to come and take Isla in, she had a tonic clonic seizure so bad I had to rescue her from it. It was the first time I’d had to do this myself. Despite being in a hospital, and surrounded by nurses, it was down to me to give Isla the rescue medication. I remember getting so hot and shaking so much, I thought I was going to faint before I’d have chance to give it do her. Isla came out of the seizure and was stable enough following the midazolam to have the endoscope.
The fact she had such a violent seizure, following Mexiletine and indeed her anti-epilepsy medication, on an empty stomach, led me down a new thought process and changed the way I thought about Mexiletine; It wasn’t about what happened when we gave it, or what happened after we gave it, it’s what happened in the hour before we gave it, that was key.
All the pictures that accompany the seizure posts are Isla, post-seizure. Wow, that’s confusing!