The new medicine

Piper started vigabatrin on Wednesday. It has decreased the number and severity of her spasms which is awesome. She did have more yesterday but we hope as the dosage increases they will continue to decrease. We don't expect for the spasms to go away completely but a decrease is great. We pray it continues to work.

The medicine makes her extremely sleepy, she slept almost 19 hours the first day. It worried me a little because I have never seen anything sleep like that. The only way I could get her to wake up and eat was to rub an ice cube on her belly or put her in a cold bath. (mean old mommy)The doctor decreased her dose and we are going to gradually work our way up so her body can get used to it slowly. It was great to see her catch up on all the sleep she has missed in the past three months. Justin said, "imagine how you would feel if you didn't sleep well for three months." He quickly withdrew that statement after the look on my face. Of course my retort was "try six months." hahaha! We are actually able to put her down for a nap, this is so foreign to us we just sit there and watch her because we aren't used to putting her down at all! It makes me so happy to watch her sleeping peacefully and uninterrupted.

Her muscle tone seems to be increasing and she is getting stronger. She has become extremely squirmy and is constantly kicking and what I call "windmilling" her arms around. We have both received several blows to the face with these little arms. She moves her head around constantly but is still very wobbly. She is pretty irritable and angry right now but we take advantage of that and put her on her belly. She will try her best to lift her head and scream at us! She seems to be stronger every day.

We haven't seen any smiles or laughs yet. I was hoping I could get a picture of her smiling for her 6 month picture yesterday but she is just not ready yet. I'll keep waiting. She is still not making a lot of eye contact or responding to us. She will listen to our voices if we catch her in the right mood. We are still not sure how well she can see. We do know that her ERG was normal for her age but it really just tests light detection. We have also done a lot of research on vigabatrin and how to protect her eyes. They research we found says to keep her in dim light most of the time and away from bright lights. Since it will be warm soon and we love being outdoors I bought her some Julbo infant protective sunglasses. She may be wearing these all the time.

As far as everything else goes, there may be some other changes in the near future. Justin's job may include new opportunities and responsibilities which may lead us out of Chattanooga.
We have started doing home improvement projects around the house to get it ready to put on the market.It has made me feel like myself for the first time in months. Those of you that know me well, nothing makes me happier than a house project. I'm the weirdo that is elated over a can of paint, a room to organize and something to fix. Now that Piper is taking naps I can actually get things done. I even watched Hoarders for a couple hours the other day for inspiration. Nothing motivates me more than the thought of living like that!

Vigabatrin....prayers please!

I just got the phone call that Piper's first supply of her new medicine vigabatrin will be here tomorrow. Tomorrow will be exactly 3 months to the day since her first seizure. We need this medicine to make some significant changes but we are so nervous about this medication because the side effects are so scary. We had a follow up with the Opthamologist here in Chattanooga and he gave me a lecture about the risks. He also told me he has a patient who has permanent central vision loss from taking the drug. I have talked to other parents whose daughters have experienced vision loss as well. There are also many other children who have not had any problems so far. The scary part is it has the potential to have side effects 6 months after she stops taking it. So if they do notice damage and she stops taking the drug it will take 6 months to be in the clear. Just the thought of me giving her a pill that could make her go blind is beyond terrifying. It honestly makes me sick to my stomach.

We know that vision loss is a lot less severe compared to the amount of seizures she is having. She is continuing to have increasing amounts in the past week. She is up to the 350-400 mark. Last night we had a particularly bad night. She wasn't able to fall asleep until 4am because she just had cluster after cluster all night. She had 250 in a 6 hour period when she normally has around 40.It was one of her worst nights so far. In a weird way it was a good thing because I felt so helpless and frustrated I would have done anything to help her (like give her vigabatrin). I guess God is giving me a sign.

So, for all of you who want to help us, now is the time. We need prayers!! More specifically..

-pray for our peace while we give our daughter the new medicine.

-pray the medicine works with no side effects.

-pray she can get out of the fog and sadness and enjoy things again.

-Justin is in New Hampshire this week so pray for safe travels and that he can rest a little.

Thank you!

Playing the waiting game

Not much has changed around the Koozer household. Piper is still having around 300 seizures a day. We were weaning her off of one medication but started to see an increase in seizures so now she is on a low dose of this medicine until we can get started on the vigabatrin. On the lower dose of topamax we have seen a small increase in muscle tone and strength. She is turning her head to the left side again (she stopped doing this when she started the med) and using her left side a little more. We still have a lot of work to do to get her neck strong enough to hold her head up. Her smile has not returned but we keep patiently waiting. Sometimes she smiles when she is having seizures so at least we know she still can but just doesn't want to.She does not recognize faces and we have accepted that she may never do so again but still pray it's just a side effect. I do think she recognizes Justin and my voice which makes us happy. She has been doing better during tummy time ,mostly because she is angry and lifts her head to scream at me. I guess whatever works is good. :)

**on a side note, i hate the drug topamax and would recommend trying other things first if you or someone you know is in this situation. It has crappy side effects and horrible withdrawal symptoms when you stop using it. It is sometimes prescribed for migraines so be careful.

The new drug is vigabatrin and is called the "wonder drug" in the Aicardi community. I don't really want to think of it that way because I really don't want to get my hopes up. It is a long process to be approved for the drug because it can cause permanent vision loss. Our recent visit to Emory for the ERG is the first step in the process. It takes 10 days for the test results and our doctor can process the request. It has been 6 days since the test. We may not start the new meds until March. Playing the waiting game is the hardest part right now. I want to fast forward to March but at the same time I want time to freeze so she can have a chance to catch up if that's even possible. Having patience is extremely hard, especially when you watch your baby struggle every day.

I decided to finally get on the Aicardi website and read stories of other girls ( I have put this off for a long time). It was something that needs to be done although it was not that encouraging. Most of the stories are very sad. Most of the girls are not able to walk, non verbal and have many health problems. There are a few that function well physically but have extreme cognitive difficulties. These girls are on the mild end of the spectrum and do not have as many seizures as Piper does.Every child is different so we don't know what the future holds, but we do know that unless we get her seizures under control her prognosis will not be so hopeful. Please pray with us that the new medicine will help her.

I joined the discussion forum to be able to have support from other families.
It will be good to talk to people who have gone through or are going through the same situation. They have a family conference in July in St. Louis that helps families learn and support each other. We are going to try and save up "vacation" money to go to the conference.This is the website if anyone is interested in reading more

http://www.aicardisyndrome.org/site/node/13

Thank you so much for our friends and family that read the blog and continue to stay updated and praying for us. It makes it so much easier when people already know what is going on so we don't have so many questions to answer. It helps us have a little more normalcy in our lives, which is so important to us.