Baby Steps

Monday, July 23, 2012

EEG and another new seizure

I am sitting in the hospital with Piper for her EEG. We are having this done to find out more about her new seizures. She now has 2 new types of seizures so a total of 3 seizure types that look very different. While we were at the conference we noticed that she randomly started smiling a lot. Since she never smiles we thought this was odd. At first we thought maybe she was just happy to be around other girls like her but they quickly became cyclical and repetitive like her spasms. So we spent the first 2 hours of the conference on the phone with the doctor trying to figure out what to do. We are concerned because she does it all day long so it seems like she is just constantly seizing. So we were not able to focus or really talk to a lot of people because we were busy trying to figure out what was going on. She has had 2 of the 3 seizures since we started the EEG but of course not the one that we really need info on. It is extremely frustrating because our next step with meds is based on the new info which we don't have. She has been a trooper as always but it's already been a long day for both of us.

She is now completely off the ketogenic diet and this could possibly be why we are seeing new seizures but who really knows. There are too many variables to be sure. Regular formula seems to upset her stomach so she throws up a little every time we feed her. She has a lot more energy now so that is a good thing.

We had a good time at the conference and were able to talk to some people and learn more. Piper had a great time, she spent the first two days talking and playing the whole day. She did music therapy and loved it! We saw a lot of real smiles (I think!) and she was very happy.

Monday, July 16, 2012

New Seizure, meds and diet

As I mentioned in the last post we have seen a what looked like a new seizure in the past month. She doesn't have one every day, the pattern is every 5-7 days. We talked to our neurologists and they both think she is having partial tonic seizures along with the infantile spasms. It is considered partial because one one side of her brain is causing the activity and tonic because she freezes up when she has one.

Here is a link to learn more about tonic seizures:

If you remember, back in November when Piper had multiple EEG tests done we learned that the right side of her brain is responsible for the seizure activity. At that time the left side was not affected (probably because she is missing the corpus callosum that connects the two hemispheres) and looked relatively normal for someone with ACC. We are not sure if that is still the case but think its possible since she is having partial tonic seizures. The only way to know more is to have another EEG done and hope that she has a new seizure during the procedure. Since she is having them randomly, the chances of that happening are pretty slim. The doctor cannot make any logical changes in medicine until he knows exactly what is going on. This pushes our phenobarbital wean back to who knows when which is frustrating since this is medication that makes her smiles go away. We sure do miss those smiles but just have to be patient one again. We have a 6 hour EEG scheduled for Monday so hopefully she will have one while we are there. The doctor also did not think we saw a change when we put her on Zonegran so we are taking her off it now. We will know more at our appointment in August after the EEG is done. This sounds terrible but please pray she has a seizure while we have the procedure done!

We met with a physiatrist last week who will be working with Piper. She is a rehab doctor that works with bone, muscle and nerve issues and how it affects mobility. She has ordered Piper some pieces of adaptive equipment to help her mobility. Since the stroller doesn't support her well we will be getting a supportive chair/stroller and a stander for when she gets stronger. What is a stander? Justin says its the thing that they wheel Hannibal Lecter in on in Silence of the Lambs (and yes he said this to the doctor)
Hope we don't offend anyone but we find humor in every situation and it sure does make things easier. It does look pretty similiar, it basically supports her in standing position and helps her muscles learn how to work while standing. The stander will require her to wear braces on her feet so help her stand correctly. We are going to get fitted for braces in two weeks. We will also be building a room in our next house for her equipment!!! Along with these things, she has also outgrown her car seat and will need a new one soon. We have searched everywhere for one that she can use and finally found one for "special needs" and it looks like it will work well. Since it can be ordered along with the other stuff we may be able to get insurance to pay for it, if not it's $700!!! Ridiculous!!!!



Ketogenic diet:
She is now almost off of the ketogenic diet. We spoke with the dietician today and Piper can go back to regular formula starting tomorrow! Her solid food will still be the 2:1 ratio but will go to regular food next Monday if we don't see any problems. We are very excited about giving her regular food!! We will also begin speech therapy now that she is off the diet. At this age they help with feeding and strengthening her muscles to help her eat correctly. She had a swallowing test done back in November as well and she aspirates when she eats (she breathes in liquids) so we have to thicken her food. We are not sure if she is still doing this so we will be doing another test to see if her muscles have gotten stronger or weaker. We pray she has gotten stronger so that she will be able to eat normal food. The things we will be able to feed her will depend on the results of this study.

Everything else:
We will be traveling to St. Louis for 5 days this week to attend the Aicardi Conference. We are excited and a little nervous to meet other families and learn more about the syndrome. Justin is already traveling and will meet us there. He rented us a mini-van to drive because we there is no way we can fit everything in a car. No offense to all you mini-van driving moms but the thought of it makes me cringe! I vowed never to drive one....Justin said he is going to fly a white flag on the back. Hahaha. I told him chances are I will love having more room and want one when we come back. We will see!!

That is all for now...thanks for keeping updated with all the craziness!

Monday, July 2, 2012

Changes in medicine and diet

It has been another busy and stressful month for us. I have tons of information to share so this will be a long post!

Medicine: We visited the neurologist in Knoxville back in June and he suggested taking Piper off phenobarbital because it causes such dramatic changes in muscle tone. She has very low muscle tone and is still not strong enough to sit, push up with her arms or keep her back straight. It has become increasingly difficult to hold and carry her. She is 22lbs and 30 inches and we have to cradle her to carry her. I'm not sure how much longer I can safely carry her and getting her from the car seat to stroller is starting to get a little overwhelming. We put her on another medicine (Zonegran) and the plan was to wean her off of phenobarbital and hopefully see a stronger baby. We had to take a time out on this wean due to another change. See below

Ketogenic diet: Piper goes to Emory hospital in ATL every six weeks for a ketogenic clinic and meets with her neurologist there. Justin was out of town for the most recent visit so I made the trip with a sweet friend who kept me company. We left at 10am and got home at was a long day for all of us. It is definitely a harder trip to make now that we are in Knoxville. With that being said we really had to make some tough decisions. We have not seen clear cut evidence that the diet is working for Piper. There are too many factors and it is impossible to tell what exactly is working what isn't. The diet works wonders for some people but we didn't see a huge change and the side effects once again outweigh the benefits in our situation. We talked with the doctor at Emory and decided that they only way we would be able to determine if the diet is working is to wean her off it and see if her seizures increase. So we started the wean on June 13th.

 So here is where it gets really confusing (if you aren't lost already!) Piper had a "new" seizure the night before I went to Emory. It looked like what you would consider a "normal" seizure but it also looked like more severe spasms. The tricky part is this happened before we started the wean. After the wean began (the following day) she did not have an increase or change in seizures for three days. We thought we were in the clear....until Father's day. Piper had two big "new seizures" father's day morning that lasted longer than we have seen so far. Poor Justin, not the first father's day I had planned for him :( Of course it was on a Sunday and we couldn't call the doctor so once again we become the doctors and made decisions of what to do next. We decided to continue with the wean since we saw this new seizure before we started the wean. The wean takes about 2 months to do and right now we are about halfway through. So far Piper has had 5 "real" seizures since we started. We have come to the conclusion that it is not the diet but possibly from her getting older and we are starting to see things change. You have no idea how much time we spend talking, looking at our research (yes we have all of her seizures in an excel file) and coming to these conclusions. One thing I have learned through all of this is my husband is the smartest person I know and I thank God every day for that!! I can barely read the seizure graph he made......

So long story long, we are continuing with the wean and praying things stay somewhat consistent. We really want Piper on a more nutritious diet and it would help with some of the stress. Knowing that I don't have to carry a gram scale around in my purse and force feeding her to make sure she eats all her food would really help me out! Once we are off of the diet we will start the phenobarbital wean and maybe we will see a stronger healthier baby.

Therapy:When I was teaching I had a few "persistent" parents and many times I had trouble understanding their special requests. I am that parent now. I get it.
I think God made me stubborn, persistent and "slightly" crazy for a reason.
How does this apply to therapy? Let me tell you. It took nearly a month for Piper to get evaluated for therapy. She just now started OT and will start PT soon. I have called nearly every day to see if there are open appointments because it is so busy that they can't find a spot for her. I'm sure the lady who does the scheduling would like to block my number but I would probably just drive down there and talk to her in person. Seriously, I have no problem with that. The point is my days are filled with trying my best to get the services she needs even if it means calling someone a hundred times or sharing my opinion about the situation in the nicest way possible. She has OT this week and PT next week, we have already learned some new activities from her first OT session. I look forward to learning more. She will also start speech therapy once she is off of the diet and eating regular food.

Developmental: Piper has made some progress and is physically around 4 months old. She can hold her head up and look around while on her belly. She has started rolling from belly to back and is starting to use her arms. She loves being on her back and kicking her legs and swatting toys on her toy bar. She plays independently for long periods of time. We work on sitting and helping her back get stronger all day. 

We are working on helping her use her hands to reach out for toys and play. She does not seem interested in doing this but we have found a few things that get her attention. I actually taught her to reach out for my hand and she will do that if she is in a good mood. Her vision impairments probably have a lot to do with the disinterest in playing with toys so we use high contrast and light to motivate her. I use Christmas lights and bright colors against a black background and she has started paying attention more. Our vision specialist suggested a lot of activities to help train her brain to focus and track objects.

She has become more vocal since we started the wean off the diet. She does not babble or make specific sounds but is experimenting with different noises. She reacts to situations with different sounds and you can tell when she is happy. It may be the most basic forms of communication but we are able to know her mood based on these noises.

With all of the changes it is nearly impossible to have much normalcy! Piper's seizure episodes consist of 15-20 minutes of the loudest screaming and crying you have ever heard and it really helps if we have a place to lay her down and make her feel comfortable. You can't pick her up or soothe her so you basically just wait it out. So things like church, going out to dinner and going to the store become the biggest challenge of our day. Its a lot easier when Justin and I go places together because one of us can take her out to the car while the other takes care of what we were doing. I rarely go places alone, and if I do I have my escape plan ready.  It's just a part of life and I am gradually becoming accustomed to it.

I will keep everyone posted on how the diet is going. Thanks for reading this!

                                          Her new high contrast room to help with her vision impairments

                                                         Watching baby Einstein on the Ipad

Working out on the exercise ball