West Syndrome

The thing with West syndrome is that no-one has ever heard of it. Unless you know someone directly aflivted, then chances are it will never be mentioned in your life time. And that goes for doctors as much as it does for you and I.
For months the doctors thought that Zak was just suffering the effects of a lactose intollerance, and severe reflux. But a Mum knows her own child, and what Zak was doing was NOT reflux. It would happen after a feed, during, before or nowhere near a feed.
He went through such a hard time before final diagnosis. He started with just the odd individual spasm, that would make his whole body jerk violently to the left. He looked terryfied by them, and they just got worse.
As time went on these individual jerks developed into small clusters. They came in waves like hiccups, but would force his knees up, his arms in and his head swung to the left. He'd have a cluster of around 12 at a time before we decided that enough was enough, and he had to see someone.
The Neurologists appointment came quickly, and it didn't take long for me to realise that she was worried.
When a Neurologist tells you your child needs an EEG, and he needs it NOW, it puts a multitude of thoughts in your head.
We went directly to the hospital, and he had his EEG.
By now we were on first name terms with the radiologist, and I felt confident that her opinion was usually pretty spot on. She could tell me right there that his EEG was far from normal, and to confirm West Syndrome she was looking for a specific pattern called Hypsarrythmia. In her opinion, it was present but of course we had to wait for the results to be sent off to be read officially, and a diagnosis would be made on their return.
We knew well before the results came in that Zak was going to have Epilepsy of some description. Little did we know how bad that diagnosis would be.
West Syndrome is rare (thank god). It is made up of a triad of symptoms. one, the presence of Hypsarrythmia on EEG, Two, The Infantile Spasms, and three, developmental delay. Zak had all 3.
The day the results came in we had to go directly to hospital, where we spent the next three weeks trying to stop the Hypsarrythmia and the spasms. It wasn't easy. Zak was on a steroid called Prednisalone, and it was so hard going. His body went through hell, literally. He spasmed and arched, he was sick, off his food, couldn't sleep for longer than 3 hours, even his physiotherapist couldn't bend him. He would be layed in bed and his back never made contact with the matress. And he screamed constantly.
But slowly, after about 2 weeks he turned a corner. He chilled out, the spasms were slowing and he would eat. Best of all he started to sleep a bit better at night.
By the end of the 3 weeks, we went home with a different boy, and because of his new hospital routine, he had a better sleep pattern too.
Now if I have repeated myself throughout this blog, you will have to forgive me as I have a caringbridge page set up for Zak, and I may have got confused between which one I am supposed to be writing about......
Anyway, where was I?
Zaks spasms stayed away for a whole 10 days! At Christmas he had a major Tonic clonic seizure which found us calling for an ambulance for the first time in Zaks short life. It was not fun to see, but worst of all it also saw the return of the spasms and the Hypsarrythmia. So here we went again.
Another 3 week stay in hospital, but this time not quite the success we had seen before.
Zak was put on another drug to try to get rid of the Hypsarrythmia, this time it was Vigabatrin.
Now, for many people Vigabatrin is a wonder drug that can conquer the hardest of Epileptic tendancies....Not for Zak. It had the absolute oposite effect.
Zak went in to hospital having in the region of 6 to 8 clusters of Infantile spasms a day. By the end of the second week, we lost count after about 300!!!
The poor boy was going through hell, and to top it off he started to develope other signs of seizure. He started to twitch. First it was his bottom lip, then it was his chin, then his head and shoulders.
To cut a long story short, he was not responding well to the treatment, and we were going to have to find something else. But first we had to see the Vigabatrin through to the end. If we didn't then there would always be what if's,, and if you know me at all, I don't do what if's.
So we saw it through to the full dose, and he couldn't take any more. The spasms and twitches were constant, and he was exhausted.
We weaned him off the Vigabatrin and increased his Epilim.....and held our breath....