If Ellie was noodley before the phenobarb, while on it, she practically oozed.  She hardly ever woke up.  I couldn't interact with her at all.  I felt like the worse mother in the world, because I was having trouble bonding with her.  I mean, I loved my baby, but who the heck was Ellie?  The worst part of it was, the phenobarb didn't help the seizures.  So, after another hospital stay of a few days, Ellie was put on Dilantin.

 

Dilantin and phenobarb together is a dangerous combination, as we soon found out. 

 

It was a weekend, and Ellie just didn't seem right.  By this time, she was 3 months old, and even though she slept most of the time, she was getting more and more alert.  She had even started rolling over!  (This stopped after the seizures got very bad, and didn't start again for months...that really sucked.)  But on this day, she was completely non-responsive, and couldn't even nurse.  Ellie has NEVER had problems nursing; she nursed right after she was born and has kept going ever since.  I had been a mom long enough now to trust my instincts, and I told my mom I was taking Ellie to the hospital; something was wrong. 

 

The three of us piled into the car, and went off the TGH yet again.  There, we were greeted by name by the nurses and ER techs.  This is not a pleasant experience.  These are not people you want to know you by name unless they are your friends!  They all agreed Ellie didn't seem like herself.  They took some blood (she still had veins that gave blood at this point), and the results came back...

 

She had no phenobarb in her system, and her Dilantin levels were poisonous!  She could have died if I'd given her the next dose of Dilantin!  This was our first clue that Ellie's metabolism was a little quirky.

 

So, we spent the night and went home off the Dilantin and only on the phenobarb, which still wasn't working.

 

That was in August, and by the end of September, Ellie had had two CAT scans, and MRI, a spinal tap, and a muscle biopsy.  Her floppiness had become just as much of a concern as her seizures.  Dr. Gieron was seeing us every two weeks.

 

During one of those office visits, Dr. Gieron saw one of Ellie's seizures, which by now had progressed from just her hand trembling to her entire body crunching up like she was doing a sit up, followed by her drooling, then getting completely limp for hours at a time.  This was the kind of seizure Dr. Gieron saw.  We had previously discussed the type of epilepsy Ellie might have, and we were all hoping it was benign infantile seizures, but this looked more like infantile spasms.

 

Infantile spasms is a terrible form of epilepsy, with a not very good prognosis for mental quality of life.  This was the worse news ever.

 

We went home that day, and I made myself come to terms with the fact that Ellie was "handicapped", and would always be that way.  She was not going to grow out of her seizures.  She would probably be retarded.  I wasn't going to be able to share the world with her.

 

This is what broke my heart the most.  I thought I wouldn't be able to share the world with her; to see her smile at a bird; to see her look up in awe at a tree.  That had been what I most looked forward to about being a mom.  I wanted so much to share in the joy of a new person discovering the world, and now I was being told Ellie would never even recognize me as her mom, let alone realize the world was there.

 

But something about this diagnosis didn't ring true, either for me or Dr. Gieron.  Since Ellie's seizures weren't getting any better, the next time we went to the ER, they admitted her, and I refused to leave the children's floor until they helped Ellie.  At TGH, the parents can stay in the room with the baby, so Ellie and I lived in a succession of smaller rooms at TGH for a month.  This was Ellie's last hospital stay.

 

During this time, Ellie's preliminary muscle biopsy results came back showing a non-specific myopothy.  (Later results showed no mitochondrial disease, thank God.)  When Dr. Gieron saw that, she was actually relieved...the floppiness finally had somewhat of an explanation.  We also started Ellie on the treatment for Infantile Spasms at this time: ACTH shots.  These are shots of a steroid that helps kids with Infantile Spasms.  Like so much else in the medical world, they don't know why, but they do know that after a week of shots, the seizures should lessen, if not stop.

 

Well, Ellie got these shots for a week, gained a bunch of weight and actually drank from a bottle because she was so hungry all the time.  What didn't happen was the seizures stopping.  Toward the end of our stay, I asked Dr. Gieron if there wasn't a daily form of Valium Ellie could take, because the only thing that stopped her seizures was the Diastat I gave her at home when they lasted more than 30 seconds.

 

Lo and behold, there was!  Ellie was prescribed Clonazepam and Topamax.  Both of these are not medications that are usually prescribed for infants, and are considered the drugs of last resort for most adults, as well.  But, since Ellie's seizures were what Dr. Gieron calls "refractory" to medication, meaning they don't respond (ie, stop) to most treatments, she was willing to listen to the mom and give this stuff a try.

 

And don't you know, they worked.  Within three days, Ellie had her first seizure free day.  After we went home, it was a seizure free week, then a month, and then I couldn't  even remember when Ellie's last seizure was! (Editing to add: Ellie has been seizure-free since the spring of 2003)  There were some meds adjustments here and there as she grew, but eventually her seizures became so mild, I didnt even know she'd had one.  Her post-ictal (after seizure) symptoms began to last only a few minutes compared to a few hours before. (Editing to add: as of January 2004, Ellie has been taken off Klonapin completely and is in the process of having her Topamax dosage reduced.  Another page of her story will be posted shortly.)