Well you can probably tell by the title of this thread that the Vigabatrin did not turn out to be our wonder drug. Infact it was quite the opposite. Zaks seizures increased and we started to see new ones developing...not what we were hoping for.
The increase in Epilim was not doing the job, so it was decided that he needed something else. That was Lamotrigine.
Now, we had been warned that Lamotrigine can have a lot of very nasty side effects. One of which was the earily named Lamotrigine rash. Zak had to be weaned on to it very slowly to try to avoid any adverse reaction.
It was successful to a large degree.
After the Vigabatrin Zak was having several new seizures. One of his more worrying ones was one called a Tonic Axial seizure. His entire body would go very stiff, his head would pull up and in line with his body, he would jerk a couple of times and then fall unconscious. The first time we saw this one was back in December 07, but at the time we thought it was an isolated incident.
Along with this, he now had Myoclonic seizures, absences and drop seizures. But thank goodness this new concoction of drugs seemed to be taking the edge off things.
His seizures come and go in severity. He has seizures every single day still, and some days are better than others.
On average Zak has about 30 seizures a day. The majority of them are Myoclonic and absences, but he likes to throw in a couple of the bigger ones every now and then just to keep us alert.
Now, because of this new pattern of seizure (the different seizure types and their frequency) Zak had to go for yet another EEG, just to see what was going on behind the scenes. The news was not good.
Zaks brain is constantly in some sort of seizure activity. Wether he is having a clinical seizure or not doesn't seem to make any difference, his brain is still seizuring on regardless.
His 'normal' background activity is now a modified Hypsarrythmia. But on top of that he now has what they call a 'slow spike and wave' output. This combined with his seizure pattern and his very profound developmental delay/regression, gives him the new title of having Lennox Gastaut Syndrome.
I did the foolish thing when I got home from the Neuro appointment, and googled LGS....how silly!
It did have an upside though. Because of the seriousness of this condition along with the Cerebral Palsy, it now meant that Zak could get access to the local childrens hospice, and we could also get help from a charity called The Rainbow Trust. I will tell you a bit more about these further in to the blog.
Friday, 2 April 2010
Wednesday, 31 March 2010
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....
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....
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