Sunday, December 23, 2007

July 2005


After beginning Vigabatrin wean, the spasms came back full force. Don't know what to make of things now and the doc refuses to up her ACTH dosage. She's now on Trileptal too, but who knows how effective it will be. On a good note, though, she is rolling both ways (right side preferred) and seems to want to try to crawl, but for her right arm getting stuck down at her side or underneath her belly.

July 26, 2005

It seems the 80 units did the trick to get rid of the spasms. She was at 80 for two weeks and we started her wean last Saturday. I can't wait to get her off of this stuff. She has gained so much weight and is so puffy. They had such a hard time trying to get an IV started in her at the hospital because she's so swollen, they ended up putting a PICC line in (small incision in the arm at the inside elbow with line going up through to above the heart). She went through a lot while she was in there. We were admitted Tuesday and left Friday. She had another MRI, a 4 day VEEG, and Ictal and Inter-ictal SPECT scans. It seemed she had almost every type of seizure while she was in there too. It was pretty frightening and confusing to watch her. We weaned her completely from the Vigabatrin, which may explain some of the new seizures. We were discharged with her being put on a higher dose of Klonopin, Topamax sprinkles, and of course the ACTH. She is so drugged up that she doesn't open her eyes much at all during the day. The Klonopin just wears her out and the ACTH has done its wonders too. The high dose of Klonopin is to try to keep away the grand mal seizures she has begun having. Most of her seizures resemble staring episodes. She will look straight up, left or right, or her eyes will dart back and forth. The rest of her body usually goes limp, but sometimes her arms and legs will shake or she will extend her arms out during the seizure. They usually last from 3-5 minutes with the tonic-clonics around the three minute mark. We go back to our NEW, WONDERFUL DOCTOR!!! on August 11th for a recheck. He seems to think her seizures are originating from the occiptal lobes. The MRI sort of indicated a thickening in the left occipital area, and a possible myelination issue in the right (if I remember correctly). So, his thinking is that it's either a delayed myelination or cortical dysplasia. Thankfully, there are no structural abnormalities. Hopefully, the SPECT scans and VEEG will help isolate things even more. I have a feeling though that her seizures are widespread and involve both hemispheres. We're not ruling out surgery just yet, but I wouldn't be surprised if she isn't a candidate. We'll try the Topamax and move on to the the Ketogenic Diet around one year of age (9/24) if necessary. We did see ophthamologists while we were there. He said the same thing the other two docs did: CVI. But, he feels that once she gains seizure control, her vision will improve vastly.

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Our peanut in a nutshell...

Hi everyone! Thanks for visiting my blog. My name is Jade and I am currently 8 years old. I have a big sister and a big brother whom I love very very much.

I have a very rare neurological disorder that isn't even named yet! It is a genetic mutation called CDKL5. It is considered a variant of Rett Syndrome and presently, I am only one of around 600 or so children in the world documented to have this mutation. Each child is unique in that his or her symptoms vary quite a bit. Unfortunately, I am one of those children who is affected much more severely than most of the kids.

Some of the problems I face include:

Reflux (in October 2006, I had surgery to have a Nissen Fundoplication done and to have a gastrostomy tube inserted for feedings). As of March 2012, I have a GJ tube for continuous feeds.

I am hypotonic - I have virtually no muscle tone and I find it difficult to even hold my head up on my own.

I have cortical vision impairment - my brain doesn't process the images it sees. Sometimes I see off to the side, sometimes, my vision is shuttered, sometimes I don't have any vision at all. The doctors and my Mom and Dad don't really know how or what I see because I can't tell them.

I cannot talk nor communicate with gestures.

I cannot walk, nor will I ever walk.

I grind my teeth. So much so, that some loosen during seizures.

I cannot grasp objects or use my hands for the simplest of tasks.

I cannot sit on my own. I can, however, sort of roll over from side to side.

I am choreoathetoid. I cannot control the jerky movements my arms and legs make.

I have life-threatening seizures every day. This is my greatest challenge.

So, now that you have gotten to know me, please feel free to take a stroll around my blog. Some of it you read may seem depressing, horrible, unfathomable, and downright crazy. But, this is my life.

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