New to the Foyer

[Paorrin]

My daughter Abigail is 11yo she was diagnosised with subcortical band heteratopia ( migrational disorder of the brain) at age 4 had her first seizure at 5 1/2 was dx with lennox gastaut syndrome @ 7yo is on depakote, klonipin, zonagen, topamax. She was on lamictal but made siezure worse keppra made her crazy and hallusinate the best med that made he seizure free was banzil but after 1 month she had a severe allergic reaction and was taken off. She had a VNS placed 2/11 with some improvment. We have tried to implament the ketoginic diet but she is very stubborn and wants the same food as her siblings.

Developmentaly she is far behind...she has lost most of her alphabet and numbers. She was fully potty trained during the day but now has many accidents. She talks in short 2 word sentences, walks but get tiered easly for long distances.

Seizure wise she has drop seizure (atonic), complex, and absent seizures multiple times a day now on the increase again. After our last VEEG her doctor talked to us about SUDEP that was scary and very hard on my wife..... She has refered us to NYU for eval of possoble corpus callasotomy. We are starting Felbatol this week and weaning off zonagan and decreasing topamax.

Now the best part... Abigail is a beautiful girl with long blond hair. Loves to laugh and smile. Loves giving great big hugs. She is truely warm loving child. Loved by her sister 9 and brother 7.

I am glad to have found this group as i belong to other groups about her diagnosises that have helped in the past. I welcome any advise and knowledge that you may have.

Thanx
Robert

 

[Dutch mom]

Welcome Robert,

My 12-year old special needs son has LGS as a result of post-natal braindamage. He is multiple and complex disabled, as well cognitive, physical as visual (can't walk, can't talk, functions as a 2 year-old.)
Before he started the ketogenic diet at age 5 he had 10-100 seizures a day (absences, tonics/stiff cramps, clonic shocks, atonic head drops and falls, grand mal tonic-clonics, myoclonics in sleep.)
He has tried many AEDs: valporate (depakote, orfiril), topamax, ethymal & zarontin (made him hallucinate), clobazam, clonazepam, lamictal (made szs worse), keppra, phenobarbital, felbamate. All without positive effect on his seizures. We realized he is resistant to all medications and decided to wean off all of them as he only suffered from many side effects.

At age 5 we started him on the ketogenic diet. For once his handicaps were a benefit because he only eats what we give him and doesn't realize his siblings are getting other food. In the first 2 weeks on the diet, even before adequate ketosis, we saw a significant seizure reduction, which made us convinced we had to make this diet a succes. In the first 6 months his seizures reduced 90% and his EEG improved >70% without AEDs He is on the diet for over 7 years now and he only has (tonic-clonic) seizures when he is ill or when blood ketosis is <3.5.

I understand your daughter is starting on Felbatol this week. My son had a non convulsive absence status (NCSE) in the first 2-3 days on felbamate (brand name Taloxa in the Netherlands) ending up in hospital. Because of the serious (possible lethal!) side effects (irriversible reduction of white bloods cells, aplastic anemia and liver failure) we decided to stop the felbamate and to try the ketogenic diet. In his case it was a golden choice!

My son's story as written 5 years ago for Matthews Friends http://site.matthewsfriends.org/index.php?page=joni-s-story

 

[Dutch mom]

Hi Robert,

I was wondering: was rufinamide (brand name Banzel in US, Inovelon in Europe) considered yet?

For years Felbamate was 'the' last option for LGS. Because of the devestating impact of the syndrome, the serious side-effects were considered worth the risk.

But since 2008-2009 (2009-2010 in the Netherlands) Rufinamide is a new drug used and especially effective for LGS with less serious side-effects.
In the Netherlands rufinamide is prefered over felbamate to try first, as well because of effectiveness as because of side-effects.

Clinical Results

FDA Approval
FDA approval of Banzel was based on the results of a single clinical trial. This multicenter, double-blind, placebo-controlled, randomized, parallel-group study enrolled 138 subjects, between 4 and 30 years of age, with inadequately controlled seizures associated with LGS. All subjects were being treated with 1 to 3 concomitant stable dose anti-epileptic drugs. After completing a 4-week baseline phase on stable therapy, the subjects were randomized to have Banzel or placebo added to their ongoing therapy during a 12 week double-blind phase. This double-blind phase consisted of two periods: the Titration Period (1 to 2 weeks) and the Maintenance Period (10 weeks). During the Titration Period, the dose was increased to a target dosage of approximately 45 mg/kg/day (3200 mg in adults of > 70kg), given on a twice daily schedule. Final doses at titration were to remain stable during the maintenance period. The median percentage reduction in total seizure frequency from baseline was greater in the rufinamide therapy group than in the placebo group (32.7% versus 11.7%; p<0.002). The rufinamide-treated subjects had 42.5% median percentage reduction in tonic-atonic seizure (drop attack) frequency per 28 days from baseline as compared with 1.4% increase in the placebo-treated subjects (p<0.0001). The rufinamide group had a statistically significant improvement in seizure severity (p<0.005) and a higher percentage of subjects who experienced at least a 50% reduction in tonic-atonic seizure frequency per 28 days compared with placebo (42.5% versus 16.7; p=0.002).

http://www.medilexicon.com/drugs/banzel.php

More about Rufinamide:

http://www.ncbi.nlm.nih.gov/pubmed/21351809

http://www.banzel.com/about-banzel.aspx

About Felbamate:

http://www.drugs.com/mtm/felbamate.html

Felbamate can cause a decrease in many types of blood cells (white cells, red cells, platelets). Call your doctor at once if you have any unusual bleeding, weakness, or any signs of infection, even if these symptoms first occur after you have been using the medication for several months.
Felbamate may also cause liver damage. Call your doctor if you have symptoms such as loss of appetite, stomach pain, or jaundice (yellowing of the skin or eyes).
You may have thoughts about suicide while taking this medication. Your doctor will need to check you at regular visits. Do not miss any scheduled appointments.
[...]
Felbamate can cause serious side effects and is usually given only to people with severe epilepsy when the need for seizure control outweighs the risk of side effects. You may be asked to sign a consent form after you and your doctor have discussed the risks and benefits of taking felbamate.

 

[Nakamova]

Hi Robert --

I just wanted to welcome you to CWE. I hope you can find a way to help Abigail get better seizure control and quality of life. Dutch Mom gives good advice -- perhaps the dietary approach can make a difference. As I'm sure you know, a corpus callosotomy is a major procedure, so be sure to ask your NYU docs plenty of questions about the risks and benefits.

Best,
Nakamova

 

[Paorrin]

Thanx Dutch mom & Nakamova for your responces.....

We tried bazel it was the best med ever!!!! Not one seizure but after about one month she developed a severe allergic reaction ( Steven-johnson rash) that has left scaring on her legs to this day.

My daughter is mentally chalanged but is very aware what everyone else is eating and would go into the pantry and take food or steal it from someone elses plate when they were eating. That is why the keto diet hasnt worked for us.

When we went to NYU for eval of corpus callosotomy the epitologist explained everything very clear and answered every question we had that is why we went with felbatol first before going any further with surgery. The doctor had a great show while we were there as Abigail had three atonic seizures in the office, she really put on a show for him.

So for now we hope the febatol works.


-Robert

 

[Dutch mom]

Too bad as well the ketogenic diet as rufinamide were no options for your daughter since these treatments are the best options for LGS. Especially since rufinamide made her seizure free. What a disappointment it is to stop with an effective med because off side effects. The impact of LGS on childhood development and skills is so devestating. I do know all about it, my son is doing so much better since the keto diet has brought him significant seizure reduction. I do hope felbamate will make the difference for Abigail and she won't suffer from serious side effects. Good luck to your girl!