Stiripentol Update

[Epileptic Boy's Mom]

Hi all, just thought I would post an update about my son's experience with Stiripentol. For the most part I think it's working . (He did go into Status Epilepticus on day 3 of the medication but I think the seizure was due to him being sick - chest x-ray showed some hazing and radiologist thought it might have been the beginnings of pneumonia.) so it has now been 2 weeks since his last seizure and we are noticing a huge difference! He is now wanting to walk and is taking lots of steps!!! And he is starting to be more vocal now (good or bad thing I'm not sure yet ) it may be a bit premature but we are feeling more hopeful than we have been in a very, very long time. One side effect that I am noticing though is that he is a bit more aggressive - rips my glasses off my face, pulls my hair, squeezes super tight when we hug him etc. But I think I can live with these side effects if it means no more seizures! If anything changes I'll be sure to update!

 

[shmuel]

Hi!
Our son 3 1/2 has a missence mutation on the SCN1A gene. He has been on Stripentol for 12 months. If you read the booklet that comes with the strippentol you will see that the side effects include loss of appetite/ anarexia/ ataxia. In the booklet I received with the medication (called Diakomit- imported from France to Israel) the side effects are catagorized as very commom- common and less common (or to that effect). So it looks like your child is responing really well- very encouraging! Our Noam was on Keppra previously. Now he is on Valporal/ Frisium and Strepentol. I understand that Stripentol is not a stand alone drug but rather a performace booster for vlaparol. We had massive problems earlier in the year as he was very Atactic and all togehter out of it. The neurologist halved the amount of Frisium and things improved dramatically. Topomax was also a disaster for us. In the US they are currently testing a new drug called Epidolex that supposedly has less side effects. In any case hang in there and good luck!

 

[shmuel]

I should add that our son displays the same side effects- he is agressive and cranky at times and also squeeses when we hug. We were told that the squeezing when hugging is more emotional. We are talking about little ones that have been through a lot of trauma so they need to "feel" affection- so I squeeze back. We make sure he has a teddy bear when he sleeps. I also have a salt lamp in our room (he sleeps with us) to make a relaxed environment. His brothers sometimes give him a massage with wheat germ oil and lavander oil which is also great. I put a drop of lavander oil on his sheet when he goes to sleep so he associates the smell with the massage in order to relax him for a good nights sleep.

 

[jimj]

Do either of you have a Dravet diagnosis for your kids? SCN1a is typically thought of the Dravet genes (shmuel), but there are many kids without a SCN1a mutation that have a Dravet diagnosis based on their clinical presentation (Epileptic Boy's Mom).

The Dravet foundation website and Facebook group have the most Dravet information, I think you'd find a lot of information there relevant to your situations:
http://www.dravetfoundation.org/
https://www.facebook.com/groups/117762871617179/

I keep a few notes on Dravet syndrome here:
https://docs.google.com/document/d/...0Ti8DA67QLzL01y4c/edit#heading=h.r970t4a6f0qs

 

[Epileptic Boy's Mom]

No Oliver does not have Dravet - he has generalized tonic clonic seizures. I am noticing that his seizures tend to happen only when he is sick. Thought the stiripentol was really working as he went 3 weeks and 2 days without having a seizure (it still might be doing something for him but not making him seizure free like we hoped). So hard when he can't tell is what is happening and what he is feeling

 

[jimj]

No Oliver does not have Dravet - he has generalized tonic clonic seizures. I am noticing that his seizures tend to happen only when he is sick. Thought the stiripentol was really working as he went 3 weeks and 2 days without having a seizure (it still might be doing something for him but not making him seizure free like we hoped). So hard when he can't tell is what is happening and what he is feeling

It is hard when they can't communicate. Sorry you have to go through this.

It doesn't matter to me how you classify Oliver's epilepsy, but getting an accurate diagnosis does help in choosing treatments and getting support. I'm no doctor and I obviously don't know Oliver's entire history, but you've posted several things that are red flags for Dravet and I haven't seen anything that would exclude Dravet. Dravet kids have all types of seizures including generalized tonic clonic.