Warrior of the Week

Introducing this week’s warrior – With the best smile ever -the gorgeous Eddie !

In some ways we were lucky, or at least forewarned. A large porencephalic cyst was found on the left side of Eddie’s brain in utero at 35 weeks gestation. The initial diagnosis was porencephaly, a rare neurological disorder which can cause a wide range of neurological problems, from minor to severe. But following an MRI in utero it was thought that as the cyst was ‘isolated’ it might not be too damaging. Indeed a local pediatric consultant told us to go home, enjoy the last few weeks of my pregnancy and to be ‘quietly optimistic.’ And in many ways we were, as Eddie did everything he was supposed to do in the first few months. A little later, but he reached his early milestones. As the months passed Eddie was diagnosed with Cortical Vision Impairment, and then Right Hemiplegia. But still at his 6 month review we all felt, well, quietly optimistic! But at 7 months the seizures started. I knew enough to video what I saw and so he was diagnosed within days. But of the 6 professionals who I showed it to, from the health visitor through to three pediatricians, only one was sure it was IS. But except for the health visitor everyone agreed that it was worth checking ‘given the cyst’. We started on vigabatrin but that didn’t work, so a course of prednisilone followed. This too failed. We had expected he would try ACTH next but were told by both our local hospital and local children’s hospital that it was ‘too expensive’ and there was no evidence it would work if prednisilone hadn’t. In the year that followed we sought a second opinion from Great Ormond Street who assessed Eddie for a hemispherectomy (he isn’t suitable), tried three AED’s, all with their own side effects, weaning on and off times etc, and started Eddie on the ketogenic diet. We had two ‘honeymoon’ periods where it looked as though he was responding to the treatments and the seizures decreased, only to increase after a week or so. On average Eddie would have around 7 clusters in a 24 hour period, he was also diagnosed with myoclonic jerks.

Once we reached the one year anniversary of the seizures starting I think something broke in both of us, and we decided to try CBD although it was a terrifying decision to make. We value the support of the team around Eddie and hated going it alone. And although we saw an improvement in his awareness and mood almost immediately the seizures remained. We started fighting for ACTH again but then, incredibly, the seizures stopped. It’s been 6 weeks now since we last saw a cluster! His last EEG just before the seizures stopped showed modified hypsarrhythmia still but it had improved. We are hoping he will have another in a week or so.

Eddie will be 2 in 2 weeks time, there’s no doubt that he is hugely delayed and will likely be affected by the IS throughout his life. Arguably we might have expected this given his underlying neurological issues but we didn’t, we wanted and hoped for an easier, and better outcome for him, but despite it all he has made very slow, and important, developmental gains, he can hold his own drink, feed himself finger food and most recently has started trying to roll again. Since we got the diet ratio right and added the CBD he has been so much happier, and is now a very cuddly, loving, happy boy with a laugh that makes everything better!

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