Baby Steps

Friday, December 7, 2012

Overdue update

I have been slacking on my updates, sorry!

The last update was about sleep, or lack thereof. Things in that department are still the same. On a good day I can get her to take two 30 min naps. She is still not sleeping through the night and wakes up at 2am ready to play. Since we have tried all of the natural sleep aids our neurologist said our next step would be pharmaceutical sleep aids. We want to avoid that for now and just try to manage. She doesn't have seizures at night when she wakes so I guess it could be worse.

Seizures: In November we increased the Sabril medication in hopes of cutting down the number of spasms that have been creeping back up again. It seemed to work initially but now we are back to the same pattern as before. No luck there. We don't have any other safe medication options until she is older so we are just trying to manage what she is doing now. Her seizures usually come frequently in the morning and taper off later in the day. I have changed all of her weekly appointments so that we are at home most mornings.

Procedures: she will be doing a double procedure next Friday. She is having a MRI and ERG done. We have done both of these before but it was this time last year. These are just to check to see if anything has changed. Pray this goes smoothly and for good results.

Other doctors: Our appointments have slowed down a lot now that we are established with our doctors. We go for routine check ups as needed. She had her 15m check up with pediatrician and she has improved developmentally. She has gone from 4m to 9m on the social aspect and from 4m to 6m on the physical aspect (gross and fine motor). She has made big improvements with feeding as well. She has learned to chew and can eat a larger variety of textures and foods. We are working on teaching her to eat with her hands. She is not that interested and would much rather have someone else do the hard work. We are just pleased that she is motivated by food :)

Another issue we are facing is crying and "temper tantrums". We are not sure if this is an age appropriate behavior or if she is having pain she cannot communicate to us. Since her main form of communication is crying, I find myself carefully trying to decipher what her cries mean. I haven't cracked the code yet... If this is an age appropriate behavior then its good in a weird way. Lets just say we pray for patience often. :)

Overall things are good and we are managing challenges as best as we can. She is growing and learning little by little. She has a smile and giggle that melts out hearts. We look forward to enjoying the holidays and making great memories.



Monday, October 22, 2012

Sleep

Sleep...it seems like a very distant memory. 14 months of sleep deprivation is really catching up with us. We feel like we have a newborn again and we are back to taking turns getting up with her. Piper has never been a good sleeper at night, she did sleep quite a bit during the day for a few months when she was little. Since we took her off phenobarbitial we have seen the least amount of sleep so far. We have been working on finally getting her on a schedule in hopes that she will sleep better. So far it hasn't helped much. We are also trying to get her to sleep in her crib in her room. On a good night she will sleep 4-6 hours not consecutively. She falls asleep easily but wakes after 3-4 hours and cries for 2-3 until she wears herself out again. We have tried sleep aids which help her fall asleep but don't help her stay asleep.

She has screaming meltdowns all day now and it's probably from being so tired. It's affecting her therapy sessions and we usually leave early because she is too tired.

We are doing a sleep study this evening and they need atleast 6 hours of sleep to get the data they need. Piper and I will go in at 7 and be released at 5:30am. If they don't get enough data we will have to repeat the study. She is not allowed to be held while we are doing the study so it should be interesting. I foresee a long night for both of us.

On a more positive note she is smiling an laughing a lot more. Justin can really make her giggle. It's awesome to see her so happy.

Wednesday, October 3, 2012

Changes and new challenges

I feel like I start every blog with saying its been a crazy few weeks, but again we have been busy.

We had a wonderful vacation and Piper was so happy the whole time. We began the phenobarbital wean last month so we started seeing real smiles around the time we went on vacation. Let's just say I have taken pictures non stop. It's been so long since we have seen our smiley pants and every smile just melts my heart. Piper has now been off of pheno for three days. We have seen a few more seizures but not a huge increase like last time. Hopefully it will just take a little while for her body to get adjusted. She is still smiling and has even laughed a few times. On really good days she is very vocal and her body is getting stronger. We didn't realize just how sedated she was.

On the other end of this she is finally able to feel the negative stuff too. Before she never cried unless she was having seizures. She never seemed to express any emotions. Boy, has that changed! Now she expresses them like never before. When she is tired, hungry, bored or hurting she just screams. I mean screaming at the top of her lungs. She screamed for three hours straight the other night and the second we got to therapy on Friday. We had to leave because we were disrupting the other kids. It's definitely something I have to get used to because she is normally so mellow. She had started screaming for attention and when she doesn't want to do something. She knows how to get what she wants!

In the past couple weeks she has really shown an interest in sitting. She actually will pull herself up from laying down to a sitting position without using her hands. She is determined to sit and gets very angry if she can't. It's a good thing because she is so stubborn and has already made progress. We should be getting a chair for the house that will help her learn to sit.

We got two pieces of equipment yesterday that took about 6 months to get. She now has a wheelchair/stroller that is very supportive and she already loves it. We took her out last night and she was able to see things around her and was very happy. We like it too, but its pretty heavy. It weighs 42lbs which is about twice as heavy as the chicco stroller we used before. I may have to lift some weights to stay strong enough to move it around all the time! Thanks to Piper I'm in the best shape of my life!! We also ordered a "stander" but our insurance denied it because they said its not medically necessary. We have started the appeal process so we will see what happens. Luckily, the company we use has extra equipment to loan out while people go through appeals. So were able to borrow a stander! This is really great because it will help her hips and feet since they are not working they way they should. She really likes it and was in it for over an hour yesterday.

Last week I took her to get fitted for leg braces. She will have braces that will help her feet stay flat. She will wear the braces all day. They will also be helpful when she is in the stander because they will force her legs and feet to be in the correct position. We will see how she will tolerate those!

In October she has a few procedures planned. She will be doing a sleep study to check for sleep apnea. We noticed that she is snoring at night and sometimes makes unusual noises. Children with low muscle tone don't always gasp for air while snoring the way they should. I will be taking her to Children's hospital for an overnight study to see what she is doing when she sleeps. It may be a challenge because since she is off the pheno she never sleeps. She doesn't take naps and only sleeps about 7-8 hours on a good night. Saturday and Sunday she slept 8 hours total. We have tried everything but she just doesn't sleep. Hopefully she will sleep for the study.

If she is not having sleep apnea we will have a follow up ERG. An ERG measures how your eyes are processing light. She has had one before and has to do one every 6 months to stay on her medication. They had to push our appointment back because if she does have sleep apnea then it is dangerous to sedate her for the ERG. We will see how the sleep study goes to determine the next step.

Overall, she has had a really good 3 weeks. She has a personality again which is great....even when she screams. She is very alert, playful and seems happy. Honestly, we just want her to be happy and as healthy as possible. We are very blessed!

Tuesday, September 11, 2012

Piper lately...

Wow, it's almost been a month since I've posted anything! Time is
flying and Piper is keeping us on our toes. Since the weekend of her
birthday she hasn't been herself at all. Up until last week she wasn't
playing or eating much and really just seemed miserable. We noticed
that she has 4-6 teeth coming in so we thought that was the cause. Her
teeth have still not come through so now we think she was having
trouble with allergies. Whatever the cause it has really taken a toll
on her. With being sick she has a huge increase in seizures. The new
med seems to be preventing the big seizures so she just has spasms.
The problem is she is back up to having 150-200 a day. This leaves her
no time to feel like playing or exercising. She had no head control up
until just a few days ago. She basically regressed back to where we
started from.

This week she started playing a little and her appetite came back
until yesterday evening. She was up all night coughing, wheezing,
runny nose , sneezing and threw up twice. She had a fever this morning
and we took her to the pediatrician. He seems to think its a sinus
infection so we started an antibiotic and hopefully she won't be
allergic like I am. We hope this will help with the breathing issues,
which are terrifying especially since she has poor head control and
the fear of her choking on all the junk in there. Let's just say none
of us slept well last night. She seems a little happier now so we will
see how she does the rest of the week. We are supposed to go on a
family vacation to the beach on Friday so praying we are able to go.
We sure do need one!

As far as seizure control we met with he neurologist last week. Since
she is basically just having spasms we don't have many options at this
point for new meds. We are going to up her dose of the med that helps
these to see if we can control them better. Our only other option is a
medicine that has a high death rate in children under 2. Uh, I think
we will pass on that for now. We are finally taking her off
phenobarbital which we have been trying to do for months now. Praying
we will see a happier little girl. Last time we started the wean we
saw an enormous spike in seizures so we are a little scared.

It's a lot to keep up with, believe me! Just keeping track of medicine
and dosage is a full time job now she has an antibiotic, sleeping aid
and multivitamin on top of everything else.

We just ordered her wheelchair/stroller which will really help us out.
Thanks to the donations from the west high alumni and other friends we
are able to cover the cost for her equipment and her new car seat. We
can't thank you guys enough!!!

Piper did get to have her first "play date" with her buddies Max and
Rylan this weekend and it was a lot of fun. Piper slept most of the
time but we had fun!

I'll keep everyone updated!







Sunday, August 19, 2012

One year reflection...you may need tissues

I have struggled for days on what to write about for this post. I have gone back and forth on whether or not I should just write a happy post or a truthful post. I finally decided to combine the two.

Birthday's are a huge celebration and should be a very happy day with lots of fun and love from people who care about you. We are celebrating Piper's birthday 3 times this week so I think we have that part covered. We are very excited and are celebrating her birthday but also celebrating that we have made it this far. It's a bittersweet day for me.

I made the mistake of reading all of my blog posts yesterday which brought back so many emotions. One of the hardest posts to read was the one I wrote a couple days after she was born. I was so happy,positive and hopeful in that post. We really thought everything was going to be okay. I had no idea how drastically our lives would change in just a few months. The reason I read the posts is because I thought it would help me see how much a I have grown over the past year. What it made me realize is I am not happy, hopeful and positive like I used to be. Not saying I don't have those feelings, they are just extremely different than they used to be. I have been conditioned to not be overly happy or overly sad...I guess it's my defense mechanism against the stress.

The other reason I took a trip down memory lane is to see how much she has grown and changed. This was also a little bittersweet too. She has definitely grown, and by that I mean physically. She is huge! She has gone from a tiny adorable baby into such a beautiful little girl. On the other side of this her development has not grown much. We all know that she may never meet those milestones we want her to and we have learned to accept that. It's something we all deal with and try to not let it upset us too much. It's just so hard to know an entire year has gone by and how long it has taken for her to do things a typical child would learn in a week. It's heartbreaking. To know that I have spent the past year just trying to teach her how to play seems crazy. I guess this really opened my eyes to how fast time flies but in her world most days time stands still.

This year has changed us so much. Some of the changes have made us much better people but some have really affected us in negative ways. Piper has made an impact in the hearts of many people in just a year. She has taught us unconditional love. She has taught us how to fight and defend our baby girl. She has taught us how to be even more educated, persistent, and stubborn. This experience has also made us feel hopeless, angry, bitter, disappointed and lost many times. They say the first year is the hardest. I think the first year is the hardest because it takes that long to be able to learn how to live your new life.

This year has been an enormous challenge. I wouldn't trade it for the world. I love this girl more than anything. We have no clue what the future holds for Piper but we thank God that he has allowed us to be her parents. She is amazing and even though it's so hard most of the time she is worth it.

HAPPY BIRTHDAY PIPER!!!!!



Friday, August 3, 2012

Doctors, Doctors and more Doctors.

The past two weeks have been crazy. Piper had an EEG, Swallow study, visit to the ENT, GI and will visit the Neurologist today. My nerves are running high today. We will be discussing the results of the EEG and since she did not have a new seizure during the study we know there won't be a lot of new information. We will be deciding what medicine to try next. We have already done our research and know what we want to do. We will also be expressing our concerns with our experience at this office so far. To say the least we are extremely disappointed. In my opinion the neurologist is the top dog when it comes to Piper's well being. This person should be able to be our guide and making suggestions and decisions for us. We are  not doctors and are growing tired of making all the decisions. Piper's seizures have increased a lot this week and we are very concerned. We need the doctor to share our concern and act quickly. After going to the conference and speaking with parents about how awesome their neurologists are we know that there is room for improvement. If it means going somewhere else, then we go.

We did have some wonderful news after Piper's swallow study. Back in November her first study showed that she aspirated (breathed in liquids) and we had to thicken her food. Now it shows that her muscles have gotten stronger and she no longer aspirates. This is amazing!!! I was shocked and asked them to check again. This means she can start speech therapy and learn how to eat new foods. This also means that those dreaded feeding tubes won't be needed right now. I pray they never will.

We met with the ENT to get established just in case. They checked Piper's hearing and she has no problems there. Once again, great news!

We also met with her GI for the first time. GI issues are second to seizure control for girls with Aicardi. Feeding issues are normal and most girls are on feeding tubes. Another issue is constipation which can also lead to feeding tubes if it cannot be managed. Piper will be taking a mixture of medicine and pedialite to help her be more regular. She will also be doing a barium colon study next week to make sure her colon is working properly. The doctor said, she is a plump little doll...looks like she is eating just fine. Once again I pray she continues to be a good eater.

Piper is now eating normal foods and seems to be getting used to new formula again. She is still not very interested in most solid foods but likes apples and avocados. She doesn't seem so fond of vegetables, she may have inherited one thing from me. We will have a speech evaluation sometime soon so we can get started with speech therapy to help her learn how to eat new things.

Next week Piper will be getting fitted for braces for her stander. We have ordered some equipment for her but it takes a long time to process the orders so there is no telling when we will get the stuff. One of the things we have ordered is a special needs car seat which we need very soon! She has just a few weeks left in her infant seat so hopefully we can get the seat soon!


We celebrated Justin's birthday on the lake last Saturday and had a great time. Piper seemed to enjoy being on the boat and having the wind in her hair. We also learned that a life jacket is an awesome tool to support her when sitting. I will be adjusting one to make it a little more comfortable so we can use it at home!


                                                                      get me a new seat!





                                                    Are you going to feed me or just sit there?



Thanks for reading and supporting us!

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

Medicine/Seizures:
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:
http://professionals.epilepsy.com/page/generalized_tonic.html

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!



Equipment:
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!!!!

 Chair/Stroller

Stander


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 10pm....it 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

Friday, June 8, 2012

Please say a prayer

I have just recently started branching out and joining groups of other parents with Aicardi girls. This is something that has been extremely difficult for me because its very hard for me to hear about the struggles other people have and most of the struggles hit home for us. I have avoided joining these forums and Facebook groups because I tend to be a little pessimistic and really am not good at meeting people. I did start talking to a few moms this week and introduced Piper to the Aicardi community. We will be attending the conference in July and I figured this was a good time to start meeting other people.

In just a few days I have already talked with parents who have experiences so similar is it almost scary. I am not used to having anyone truly understand what I am talking about and it has been really nice to share stories and concerns. I can tell this will be beneficial to have support and be able to support others.

On the other hand, it has been extremely emotional and overwhelming. In just a few short months three sweet Aicardi girls have passed, one did just this morning from an infection her body just couldn't fight. My heart is ripped to pieces for this family. Although we have never met, I feel connected. I cannot imagine the pain they are experiencing right now. These girls are such precious gifts and struggle so much. It breaks my heart. I have cried all morning thinking about these people. I know that life is precious and we have no idea what the future is for any of us but it just seems so unfair for these girls and their families. I have just sat here staring at Piper thanking God for the months we have had with her so far.

When things like this happen its a smack in the face and quickly reminds me of what is important in life. It angers me at how quick I am to complain over things that are ridiculous. I woke up this morning in a bad mood over something petty and should have been thankful instead. Being Piper's mom has made me full of joy, hope and love but also fear, sadness and worry.


Please pray for this family, our family and all the other families struggling to be strong parents of such precious girls.

Monday, June 4, 2012

vacation and other stuff

We had an amazing time on our trip to Hilton Head. Piper was awesome in the car and we only had to stop a few times. We made it there in 7 hours which is pretty good when you have a 9 month old. We stayed in Hilton head for 4 nights and drove down the Charleston to see family for a night. We had a great time, I am still amazed at how well Piper did. She is always a good baby but she was great the whole trip. Of course we went to the beach while there was a tropical storm so we had a few days of good weather and the other days we went shopping. Piper wasn't sure about the ocean, it was still a little cold and windy on the beach. She did enjoy laying on a towel under an umbrella and relaxing. We brought our jogging stroller which turned out to be the perfect place for her to relax and stay cool under the canopy. We went to the pool a few times and she really enjoyed the water. She smiled more on this trip than we have seen in the past months combined. I guess somebody loves the beach as much as us! I had some good friends who were on a trip there too so we all were able to go to dinner and catch up. It's sad that I have to go out of town to catch up with friends who live right down the road!! I guess I get a little busy.









In the past few weeks Piper seizures have stayed fairly consistent. Some days she has more than others and we are not sure why, there could be many factors. Since she is on the ketogenic diet for seizure control we have to keep her ketones in the highest level for best results. We have put her on a very strict feeding schedule to help her maintain high ketones but she seems to be lower in the mornings and throughout the day she will jump back up to where she needs to be. She does have stronger episodes in the morning so this may be why. We have included a night time feeding to help with the fluctuation of ketones. She does get to eat solid foods that we make from a special ketogenic recipe. She has had avocado, carrots, peaches, cucumber, beets, apples and green beans. She seems to like all of them and is learning how to eat from a spoon. We are very proud of her!

We also are concerned about the Phenobarbital she is on. When attempted weaning her off of it a month ago and saw amazing results as far as smiles, alertness and increase in muscle tone. Unfortunately her seizures increased dramatically so we had to put her back on the medicine. We have done research and decided we wanted to try another medicine in hopes that it would control seizures but not have the nasty side effects. We are starting a new medicine for two weeks and will start the weaning process of Pheno once again after the new medicine is in her system. Hopefully we will see better results with minimal side effects. It's just a chance we have to take because we have no idea what it will do for seizure control. If you are praying for us, this would be an excellent thing to pray about! It makes us nervous not knowing if the new medicine will work.


She has still not started physical therapy here (UGH) because they are so busy that she will not be able to be evaluated until the end of June. This is nearly a 2 month lapse in time from her previous sessions. This is beyond frustrating and I can't do anything about it. I have done more research about exercises and massages to do to help her while we wait and that is all I can do for now. It's amazing how much you can find on the internet when you take the time to look. She will also be getting vision therapy and occupational therapy at some point. I am just a naturally impatient person so it's driving me crazy! The good thing is she is still motivated to sit  and I just do everything I can to facilitate her interest. I can see a difference already and hopefully she will continue to get stronger.

Everything else is pretty normal with us. Justin is traveling like crazy and is only here on the weekends. He was out of town 3 weeks in May, Will be out 3 in June, and will be out almost 3 weeks out of every month until October. I told him the next time I see him I will be 32 years old.


 

Monday, May 14, 2012

Knoxville

Get ready, this post will be an unorganized rant of information! A lot has happened in a short time so here goes.....

Moving: we rented our house in Chattanooga and they needed to moved in ASAP so I packed up our whole house and we moved last weekend! We are renting a house in west Knoxville and are happy to back. The enormous task of unpacking is almost done and was much easier with help from grandparents and friends. We still have a lot of work but I'm taking my time (for once)

Justin: he has starting taking on some responsibilities from his new position so he has been traveling nonstop. He is in San Antonio this week. He was in Canada and Texas the past two weeks. He somehow got lucky and was out of town when we had to pack up the house and when we had to unpack. Hmm... How convenient :)

Me: I had a wonderful first mother's day! Justin cooked the family brunch before taking off on his trip. Piper had a good day and we spent most of it relaxing and watching movies.

Piper: the past few weeks have consisted of some ups and downs for her. We had planned on weaning her off of phenobarbital because it has such harsh side effects. The first decrease went great. The second decrease seemed to be great too.After the second decrease we saw increased muscle tone and alertness, her smiles returned and she was very happy. Then she started having a lot of seizures again, some of them were a type we haven't seen in a while. This was pretty disappointing because we knew we would have to put her back on the med. She is now back on the medicine but at the dosage of the first decrease. Her seizures are back to 40-50 a day and she is still playful. She stopped smiling again but is still happy and playful. I did take about a million pictures of her smiling so at least I know she still can.

She is getting stronger and more persistent every day. She is trying so hard to sit by herself and is surprising us every day! We got a piece of adaptive equipment that supports her and helps her sit correctly. It's called a kidcart but we call it her "johnny 5" she loves being able to sit up and stroll around. Since her vision isn't great this allows her to see more around her. She loves it!! I think it will really help her but it is not easy to pack up and take with me! I may look like a body builder soon.

I have been working hard to get all of her doctors and therapies set up here but things don't move as fast as I would like. Luckily, I have plenty of things to do with her and she seems to be doing just fine.

Thursday, April 12, 2012

Some beach...somewhere

The Koozers need a vacation more than ever! We need a sit on the beach doing absolutely nothing for a week vacation. Whew. We are juggling more than ever now with Justin transitioning to his new position, selling/renting the house, finding doctors in Knoxville, keeping up with therapies and appointments for Piper, spending every waking hour implementing strategies, routines and exercises with her and I could probably go on forever. I'm surprised I have time to write this! I thought teaching would be the most decision making I would do in a day but I guess it was just training for the decision making Olympics we are in now.

Piper has an interventionist that meets with us once a week to help us try things to promote her motor, social and cognitive development. We spend hours working on helping her do things that most babies do naturally, for example we are working on getting her to bat a toy. She did this all the time before her seizures started but hasn't shown much interest in her surroundings until recently. She is starting to notice toys around her which is a good sign. We are also working on teaching her to hold her bottle, hold toys, play with her hands and feet, and reach out for people or objects.

She also has a physical therapist that sees her once a week. She has taught me exercises to do with her to strengthen her neck, arms and trunk. She is actually pulling her body up to sit but her head still wobbles. Her head control is much better and when she is alert she does a really good job. She is getting stronger so the exercises are working. Now that she is getting so big (she is 18lbs 10oz and 28.5 in!!!) I am not physically able to support her body in sitting position and support her head too. We are looking at some adaptive chairs to help her sit and be engaged as well.

All of the information from both of these people is great but finding time to do it all is a challenge. I have to decide what exercise or strategy is most important and what she will benefit the most from at a certain time. I race the clock and most days we barely have enough time. My OCD tendencies are back in full force, some days this is a blessing, others not so much! And just ask Justin, telling me to take a break or not to worry will only get a "not so nice" response from me. With Piper, her routine is crucial and we can tell a difference on the days that are off schedule. (yes, I'm that crazy mom)

Piper will have an evaluation for adaptive technology as well. Our TI feels that Piper is motivated by bright colorful toys that make noise. She does not respond to stuffed animals that are not bright colors. We realized that we have a ton of stuffed animals and not very many bright toys that make noise. They will be making toys for her that are more sensitive to touch so that she can make the connection when she is playing.

Overall, she is doing really well and is making progress. We pray it continues. Piper and I both have a cold which made me very nervous because this is her first "normal" sickness!! I have no idea what to do!! Lol. I was primarily worried about an increase in seizures (which is typical) but she seems to be okay so far. Hopefully she has her daddy's immune system and will get over it quickly.

As far as everything else goes, we are showing the house to rent or sell, whatever comes first. We are going through a broker so background checks,etc will be done on possible renters. We actually have two showings tomorrow. You have no idea how hard it is to keep a house clean to show with a baby, two dogs and a cat!!! It's a never ending job :) One of us has the bright idea of having a yard sale this weekend too.....sure why not? It's not like we are busy or anything.

It's a good thing Justin and I both don't know how to sit still for long and thrive when we have a million things going on.

Monday, April 2, 2012

Post race update

We had an amazing weekend in Knoxville. It was great to see friends and family we haven't seen in a while. The race on Sunday was awesome and feel so blessed to have so many people supporting and praying for sweet Piper. I am shocked I was actually able to finish the half marathon considering my training consisted of a couple long walks with a stroller. I had planned on walking the whole thing and I ended up running half of the race. My time was under 3 hours so I beat my goal by 30 minutes. This is the third race I have ever done and the first half marathon so I'm pretty proud of myself! The night before the race Piper decided to stay up all night to remind me what I was going to be running for. I guess she had pre race jitters too.

While Justin was running he had a man ask him who he knows with Aicardi. Justin told him it was his daughter and asked the man if he was familiar with the syndrome. He said he is a pediatric neurologist in Knoxville and has worked with a girl who has Aicardi too. Justin told him we were getting ready to call and make an appointment in the next few weeks and he said he can't wait to meet her. What are the chances that out of over 7000 people in the race that the doctor we have been talking about seeing happens to run next to Justin and introduce himself?? Hello God, we hear you!

Piper was in a great mood all weekend. She barely slept she was so excited to see and hear people. The medicine is still keeping her seizures around 50 a day and they are still mild. She is slowly getting stronger but we have noticed that she is able to hold her head up for longer periods of time and can almost roll over again. It is definitely a slow process and we are continuing to be patient and celebrate everything she does. She will be getting physical therapy once a week along with visits from our early interventionist. We have many activities and exercises to promote physical, social, and cognitive skills. So far she is progressing faster in the physical area. We haven't noticed much of a change in the other areas but will keep working hard. No smiles yet but we can tell when she is happy by the "talking" she does. She is a blessing and constantly reminds us to be thankful for everything God has blessed us with.

We have decided that this race is just the beginning of many that we will do. We are going to continue to sell shirts and raise awareness for Aicardi. We have extra tee shirts if anyone did not get a chance to order. You can email me at Anniekoozer22@gmail.com or send me a Facebook message.

Once again, thank you so much for the prayers, donations, kind words and love that you have given us. We would not be where we are without you.

Tuesday, March 13, 2012

Tee shirt information

WOW!! We have had an awesome response to the tee shirt design and have already received a lot of orders. We thank you so much for your support! In order to streamline the process we decided to post this blog with all the information.

The following will be available:

Toddler cotton tee (black) sizes-2T,3T,4T $10
Youth cotton tee (black) sizes-YXS,YS, YM, YL $10
Adult cotton tee (black) sizes-S,M,L,XL,2XL,3XL, 4XL $13
Adult dry fit tee (black) sizes-S,M,L,XL,2XL,3XL $15

Ordering informtation:
All orders must be received by Friday, March 16th
Orders can be submitted by facebook or email- AnnieKoozer22@gmail.com
Please specify shirt type and size and how I can get the shirt to you (more info below) If you are out of town and need your shirt shipped to you, please include your address.

*If you have already sent me a message I have you down, you don't need to send another. I will contact you if I have a question.

Payment information:
Payment can be submitted by online donation (see link below) or check/cash to our address or in person.
Our address

6452 White Tail Drive
Ooltewah, Tn 37363

How to get your shirt:
Christway friends-We will bring them to church April 8th
Knoxville friends- We will be at the Marathon Expo (Expo and Convention center) on Saturday, March 31st from 10:00am to !2:00pm.
Out of town friends- Include you address with your order and you shirt will be mailed to you.


THANK YOU SO MUCH FOR THE SUPPORT!!! It means so much to us that we have so many people helping us raise awareness and supporting our family.

Here is the design for the front of the shirt.....the back will be a surprise!




Click here for online payments:





Friday, March 9, 2012

Team Piper!

We have recently received the official diagnosis of Aicardi Syndrome. We have suspected this for a couple months so it came as no surprise. We joined the Aicardi website and forum for families and plan on supporting the foundation. They provide a yearly conference for families to meet and learn more about Aicardi. They provide hotel rooms and food for all families who attend the conference. The donations they receive provide the funds to do so. Many families would not be able to attend without this assistance.

With that being said, we would like to help raise money for the foundation and for Piper. Justin and I, her grandparents and friends will be running the Knoxville Covenant Health half marathon for team Piper. The race is on April 1, which ironically is the same day we were told there was an abnormality in her brain last year. It will be an emotional day for us. We want to raise awareness for Aicardi syndrome because it is so rare, with only 800 reported cases in the U.S. We would like to donate half of the money to the foundation so they can continue to unite and provide support for families. The other half will go to medical expenses for Piper. As Justin likes to say, we are putting many doctors' children through college.

If you are already running and would like to purchase a Team Piper tee shirt let me know. We are so blessed to have such supportive friends and family! We can't wait to see you in Knoxville. Look for me, I will be the one crawling over the finish line.


For donations click below








Thursday, March 1, 2012

Big announcement!


No, I'm not pregnant again..haha!

Justin got a promotion!!!! We are very excited for him and his new responsibilities and opportunities with his job. He will be traveling more so we feel it would make sense for us to......I never thought I would say this.....move back to Knoxville! He was gone for a long trip and realized it added a lot of stress worrying about what I would do if there was an emergency. It just makes sense for us to be close to family. We will miss our friends that we have made here very much. Our church family has been such a huge support system and we are so thankful God put us here and provided us with an amazing church.

We are putting the house on the market in the next month so we are busy doing home improvement projects. Since we are not sure what the future holds for Piper we are considering building a house in Knoxville that is wheelchair accessible. We will know more as she gets older and we can see if she has the potential to walk. It's just a thought right now. We will have to save up some money before we are able to do this. The good thing is we are on no schedule at all so we will just see what happens with this house first.

As far as doctors go, we don't have a problem switching. So all you Knoxville people...I will need recommendations on a pediatrician, pediatric neurologist, Opthamologist, Pt/Ot etc. Since we are only going to Emory once a month we feel it won't be a big deal to drive another hour.

Piper is doing a lot better on her new medicine. Her spasms have decreased, she still having episodes but they are not as intense. She is still very sleepy during the day but she is more active than before when she is awake. We are working on helping her get stronger, she is still having trouble holding her head up. Her torso is very strong and she kicks her legs all the time so that is great. She is "talking" more and looking around. She is still not smiling which is disappointing because we thought once the seizures decreased her smile would come back. Overall, we have seen huge improvements and are so thankful. The prayers are working!

Monday, February 20, 2012

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!




Tuesday, February 14, 2012

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!

Thursday, February 9, 2012

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.