Isabella's un-diagnosed siezure disorder

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NAS

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Well,

I have taken a wile to figure out how I was going to go about posting this thred.

My daughter's name is Isabella, she is now turning 6 at the end of this month. At the age of 2 1/2 She was completly normal. Behavior, speech, potty training every thing normal. She is the second of 3 atthe time (4 in total)

In the care of my mother-in-law she suffered a near drowning. It is unknown how long she was out. Her heart had stopped, and was subsiquently revived by CPR.

Within days of the event she started peeing her pants at night and during the day (we chalked it up to stress). Slurring words, stumbling, falling, forgetting things, not "listening".

Within 2 months we started seeing absance siezures (staring spells) that lasted 30 seconds-2 minutes. on average 20 an hour. She was classified as "un-diagnosed" Siezure disorder.

It has gotten progressively worse since. She had no sense of time, word recall problems, severe behavior issues, and was unable to complete extended requested tasks (like go up stairs, get jammies on, brush teeth and go to bed).

She progressed to have Complex-Partial siezures that always effected only her left side. Walking in circles, falling to the left, Left eye going "Squirly", drooping facial expressions, left arm spazams, listing to the left and more.

She also has Atonic-Clonic (im not sure on the spelling or if the name is right) siezures where she would drop un-concious. They ranged from 5 seconds to 3 minutes. They would also cause her to pee randomly.

She has classic abcance siezures including drooling, and eyes fluttering/rolling up and to the left.

She also has Clonic-tonic (Grand mal's?) that usually last 2-3 miuntes.

Here is the kicker. She is greatly effected by her discharges and the subsiquent siezures. The worst eeg they recorded 156 discharges/siezures in one hour. That included over 50 siezures proper that lasted 3 second to 2 minutes.

We have gone throught a wide specturm of medications all offered some control. But we have never had all 4 types of siezures controled at the same time. It has now coming up on 3 years since the accident. It is unclear wether it's the cause or not.

We are awaiting the response to the testing for Ring chromosome 27 (i think that's what it's called) ---the genetic testing.

They have been unable to locate any brain damage by mri or ct.

There you have it. I think the most important thing here is the effect that it has had on our family. I will get into that shortly. For now im out of time.
 
Wow, you have been through so much. I'm very sorry your family is struggling so. Is she seeing an epi?
 
Hi NAS,

My son started with myoclonic seizures during sleep and absences (petit mals) during the day at age 1,5. Growing older he developed other types of szs: complex partials, tonics (stiff cramps), atonics (head drops) and tonic-clonics (grand mals.) Just like your daughter his EEG was a disaster: 90-99% epileptic activity all day.
At age 3 he was diagnosed with Lennox Gastaut syndrome, his EEG showed a typical pattern for this diagnosis. He has braindamage so the cause is known.
Like with your daughter we never could control all seizure types with medication. It seemed as if one med helped against type A but the epilepsy then came out as type B. After trying 10 different med combinations we realized his E. is resistant to AEDs. That's why we decided to try the ketogenic diet. We didn't have much expectations but the diet worked in the first week and still does after 5 years. Weaning him of meds only did him well.
 
Again, I'm so sorry to hear of all the heartache you all have been through. My Katie was normal, too, until her 1st seizure last September. Her diagnosis is Doose Syndrome which involves absence, myclonics, & drop seizures, she's also had the tonic clonics, too. She has been declared "intractable" since she hasn't really responded to any of the 6+ meds she's tried. We just started the Ketogenic Diet last weekend and are noticing improvements every day! She hasn't had any potty accidents either on the diet (she used to have them several times a week). Dutchmom & Rackel's mom also have good Keto stories for their kids.

You've definitely found a good place for support!

Kristi
 
The neurologist wanted to try the clonazapam in conjunction wtih the Valporic acid. In May last year her seizures quickly came down to the best ever. That is, 3 discharges under 2 seconds and no siezures in one hour.

From that point she quickly accelerated to a normal kid, with the exception of behavior and "bad" days associated with her being sick. We have had aroud 8 months of decent progress. We are nearing the top end of the dosage for the clonazapam. Just now we are starting to see more seizures again.

It all sounds like doom and gloom, and trust me we have had our share. At this poit the neurologist are not entertaining lenox-gastaut syndrome. (thank god!)

The are still leaning towards a brain injury as well, but figure is dispersed or small. We just want to keep the siezures at bay as much as possible.

I have turfed the idea of "Hope". Every time in the past the concept was the cause of a great deal of anguish. Hope it will go away, hope they will find a cause and cut it out, hope she will grow out of it, hope we will find the magic bullet medication. It's a waste of time. The discovery to me that the key was her development and quality of life is the goal. So, now we are keeping with the meds, and planning forward to be ready for the Keto diet. We are all taking it in stride and moving forward.

Feel free to ask any questions.
 
The neurologist wanted to try the clonazapam in conjunction wtih the Valporic acid. In May last year her seizures quickly came down to the best ever. That is, 3 discharges under 2 seconds and no siezures in one hour.

From that point she quickly accelerated to a normal kid, with the exception of behavior and "bad" days associated with her being sick. We have had aroud 8 months of decent progress. We are nearing the top end of the dosage for the clonazapam. Just now we are starting to see more seizures again.

It all sounds like doom and gloom, and trust me we have had our share. At this poit the neurologist are not entertaining lenox-gastaut syndrome. (thank god!)

The are still leaning towards a brain injury as well, but figure is dispersed or small. We just want to keep the siezures at bay as much as possible.

I have turfed the idea of "Hope". Every time in the past the concept was the cause of a great deal of anguish. Hope it will go away, hope they will find a cause and cut it out, hope she will grow out of it, hope we will find the magic bullet medication. It's a waste of time. The discovery to me that the key was her development and quality of life is the goal. So, now we are keeping with the meds, and planning forward to be ready for the Keto diet. We are all taking it in stride and moving forward.

Feel free to ask any questions.

Websters defines Hope as someone or something on which hopes are centered, a desire accompanied by expectation of or belief in fulfillment, to cherish a desire with anticipation. A synonym is listed as hope against hope : to hope without any basis for expecting fulfillment.

I remember the days when it was just a distant thought she might make it through an entire day without needing Diastat. Then hoping she wouldn't need surgery. Hoping surgery would work. Hoping meds would work after the surgery. Hope can be a very elusive thing. During those days when Rachel was in status more often than not and we were in and out of the PICU because she coded during status, all I had was Hope against Hope. But it was still there, a tiny glimmer. Not that she would be sz free, but that she would live. Missing and mourning my little girl as the drugs slowly robbed her of her laughter and the smiles, no longer could she seem to understand things as before, she was so drugged she only wanted to sleep. With each drug we would have a "honeymoon period" as you have described. I was at the point of giving up and accepting my little sweetie was gone, under a haze of behaviors I couldn't understand and sz's that robbed her of her childhood.

Then came the day the epi said, her only hope is the Keto diet. It might help for a while until we can take her back for more surgery. Boy were they wrong! She is 100% sz and med free! She is relearning much of what she lost and is doing fantastic.

I tell you this because Hope is never a waste of time, it is always there, waiting for you. I had some dear friends who reminded me of this when I was so low I couldn't get up. They reminded me that truly my hope is in the Lord and with God all things are possible! That is such a true statement, I have seen my daughter go from death's door to running and playing, learning and making friends again in a period of 3 years.

Blessings and Prayers,

Ann
 
about the benzo's as a regular drug in combination therapy

Hi Nas,

Oh have we been there: the benzodiazepines. Nasty stuff, especially for kids.

My son was on clobazepam and clobazam for over 3 years in combination with different AEDs (including valporate.) Another mom once explained to me in a very clear way why succes with the benzo's often is temporarely and why we've had so many problems to wean them off.

The benzo's can be very effective in stopping seizures but usually in a transient way, unless the dose keeps on being increased as tolerance to the drug occurs. For that reason they are best kept for emergency meds for prolonged seizures.

The benzo's act as a 'top up' to the GABA in the brain, a neuro inhibitory chemical, meaning everything is 'subduced' when the GABA level is increased, including seizure activity. But the tolerance aspect comes about when a compensatory mechanism starts occuring, that is -due to the regular artifical top up- the body starts to produce less natural GABA, meaning the overall level drops down again, requiring another med increase to keep the status quo, the natural GABA drops down again, another med increase is required, and so on...

Apart from the tolerance problem if using them as regular AEDs, the benzo's can become less effective in an emergency situation, as the brain is already 'used to' this class of med, and so a lot more is required to have the same effect.

Weaning of benzo's which are used on a regular base can be awful, which is also tied into the GABA levels. As you reduce the dose, the GABA levels drop down below what they were previously; the brain is in a neurologic excitory state, and so the seizure threshold is lowered until the natural GABA production kicks back in once the brain releases it as a shortfall. That is when they (hopefully) stabilise. And then you do the next reduction, go through it all again, and so on.

Three years we've been adding/reducing/weaning benzo's (mainly clobazam) in combination with valporate and several 3rd drugs. When the ketogenic diet appeared to be effective, we had no problems weaning of the current two AEDs. But weaning of clobazam took us a whole year, a dramatic process. We were weaning of 10 mg with only 0,5 mg per 3-6 weeks and still he was having breakthrough seizures for a week each time we weaned of 0,5 mg.

I know parents who swich between the used benzo's to prevent this tolerance effect.
Seems tricky, but however this study about the 'benzo switch' is worth reading.

http://highwire.stanford.edu/cgi/medline/pmid;18754904?
[FONT=verdana,arial,helvetica,sans-serif]'Using the "benzodiazepine switch" in difficult childhood epilepsy'.[/FONT]
[FONT=verdana,arial,helvetica,sans-serif]R Chatha, M Huyton, D Hindley, and M Clarke[/FONT]
[FONT=verdana,arial,helvetica,sans-serif]Dev Med Child Neurol, August 1, 2008; 50 ( 8 ) : 635-6 [/FONT]
[FONT=verdana,arial,helvetica]University of Manchester, Manchester, UK.[/FONT]

You do have to be registered to read the article.
I received a text copy (Dutch translation) but I don't have an English copy.
 
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It's off to the Neurologists tomorrow.

We are off tomorrow for the neurologists, again!

Looking into the keto diet... and more meds.
 
I also would consider discussing with the Neurologist the possibility of trying neurofeedback. It has a great history of taking an instable brain and bringing it back into balance. I can not tell from your story how old she is today, but they do work with young children, and also with brain damage.

It has been a great resource in my daughters control.
 
Hi Nas,

Looking forward to hearing what happens at the neurologists today. Praying for you and yours for direction and strength...

Blessings,
Ann
 
Good Luck today. I hope the doctor is open to listening.

There are other "diets" that are showing positive results. Perhaps not clinically studied results but if you research them, families are seeing great improvement. So don't rule them out just because they are not the "standard".
 
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