What a way to start off the New Year...

ER visit (via ambulance), overnight video EEG, MRI, gobs of seizures, lots of medication changes and mood swings. All that on top of the normal this time of the year illnesses.

Let's just go straight to the seizures. Sophie had a relapse about 11 months post-op. We increased her Depakote and they immediately stopped. Then she relapsed again 2 months later. We again increased her Depakote and they immediately stopped. (But in the meantime, we scheduled a 24hr video EEG. But, by the time we got in for it, her seizures had stopped so nothing could be recorded on the EEG to determine where the seizures were originating from.)

Then she had her third relapse a few months later (it is now August). So instead of immediately increasing her meds again (hindsight...I believe this was STUPID), we decide to go in for another 24 video EEG to figure out what was really going on. Unfortunately, it took at least 6 weeks to finally get in. And they read absolutely nothing new on the EEG. So I (because I blame myself for everything) wasted all that time doing nothing while she continued to seize EVERY. SINGLE. DAY.

So we decide to wean the Dilantin* and start Vimpat. Seizures became worse. I was in a panic. I did not know what to do. I felt so completely lost. So depressed. So worthless. So helpless. So I turned to Dr. Chugani. We decided that we needed to see him in person to get some direction on what to do next.

*Through all the Depakote increases, her level remained in the mid 30s because of the Dilantin interaction. That's why we weaned it.

Which brings me to 2 weeks ago. A whirlwind of a week. Starting off with the ER visit because Sophie started nonstop trembling at 3am. Something she has never done before. Then we left for Detroit a few days later. Where she vomited at least 4 times in the middle of the night during her EEG. Which almost cancelled her MRI. Luckily, everything worked out and we completed all the testing and met with Dr. Chugani.

He got straight to the point. She was having seizures that originated in the left central region (the motor cortex on the side of the brain she had surgery...the only remaining tissue on that side). Since there was a focus we could opt for another surgery.


Hold up. Wait. Huh? What? Slow down. Breathe.


He did back up and say that we should try a few medications first. With surgery, it's like the brain resets itself. So we are able to go back and try medications that she originally failed. Because maybe those medications were controlling the seizures originating in the left central region but not the ones that were originating in the areas of the brain that was removed. So maybe this time around it would be different. Makes sense.

She is having Infantile Spasms (I hate that name...sounds way too mild). So we need to start over with the front line treatment for Infantile Spasms.


ACTH.


Which, at this point, sounds better than surgery. But it still makes the room spin and makes me want to vomit.


F**K.


That's what I'm thinking. This is f**cked up. You remove almost half her brain and it is not enough for those f**king seizures. They (seizures) want more. Well, they can't have more. She's had enough of her brain removed. ENOUGH. I feel so superficial for wanting to hold on to that little strip of brain. SO SUPERFICIAL. Because if it was any other area...I'd say take it. Which I did...they already have. This is all that is remaining.

So we decided not to jump onto the ACTH bandwagon just yet. We weaned the Vimpat and added back the Dilantin and reduced the Depakote. Wishful thinking really. It worked once. Maybe it will work again. We will see. We have the rest of this week to give it a chance. But our local neuro is already getting things started on the ACTH. It won't be so easy to administer this time around. She's 5 years old. She's 46 inches tall. She weighs 47 pounds. She is strong. Not so easy to inject her with a big ole needle in her thigh twice a day. It's not a quick easy poke.

So that's where we are at. We have to be very aggressive. We are fortunate the seizures have not yet spread to the right side of the brain which would be detrimental to her learning and development. So, if the ACTH fails, then we will try Vigabatrin. And if Vigabatrin fails, then...most likely...surgery.

But, I have to add... I DO NOT EVER REGRET Sophie having the first (and hopefully only) surgery. NEVER. Her developmental gain has been more than worth it. She is amazing. And she continues to amaze us. There isn't a day that goes by where I am just in awe of her. Her speech and language is so rich. And her memory is so clear. She talks in complete sentences about things that have happened months ago.

I am holding on to the hope that I do not have to put her through any more pain. I just don't know if my heart can take it. Did I mention that we are also in the midst of all the paperwork and testing for her to transition to kindergarten in the fall?!?!

EEG results...

We met with the neurologist the morning following the hookup. It is the hospital's procedure to go over the findings and to be sure we got all the data we need before they disconnect. Of course we did, since she has these "movements" every single day. Usually, she has several of these episodes in a 24 hour period.


There was no consistency in the EEG pattern with these movements. But, they believe the movements look like seizures so they believe they are seizures. They believe that the scalp EEG is not able to pick them up. In the remaining tissue in her left hemisphere, she continues to have intermittent spiking when she is awake and frequent spiking when she is sleeping.


She is currently on Dilantin and Depakote. It is a good combination to be on coming out of surgery because they work through different channels in the brain. But the Dilantin speeds up the metabolism of Depakote, so we were never able to get her Depakote level to a therapeutic dose. With each relapse, we increased the Depakote which would temporarily solve the problem. So we are taking a different approach this time. We are weaning the Dilantin in hopes that it will increase her Depakote level and stop the seizures.


We are currently weaning Dilantin with no relief in seizures. In fact, they are more intense. I now have no doubt...they are definitely seizures and they are getting worse. I am not sure what to do. Either her brain needs the Dilantin -or- she is having withdrawal seizures that will eventually stabilize. In the past, EVERY SINGLE TIME we have weaned an anti-epileptic drug (AED), she had withdrawal seizures. Even if the drug did NOTHING to stop the seizures she was currently having.


So, we will play the "wait and see" game and hope the past holds true. I really do not want to add another AED. Dr. Chugani suggested Vimpat. That is one she has never tried so it's time to do my research.

Our Day in Pictures...

Happy 5th Birthday and 24 hour video EEG...

Holding her favorite sticker book (thanks Grandma).
If your child loves Sesame Street, I highly recommend this.
She is learning lots from this book.

No...she wasn't this happy.
I asked her to smile and she obliged.
But not one tear was shed.
We were able to easily distract her.
Especially with her birthday present she got moments before...an iPad.

A birthday sign she had made at school.

Her incredible nurse decorated her door with the Happy Birthday letters.

Aunt Diane came by for a visit and gave her some fun books to read.

The hospital (along with a gift bag put together from a local girl scout troop) gave her birthday presents and balloons.

She has never before been interested in birthday presents.
But she ripped right into these!!!

Sophie's nurse and someone from Child Life Services.

Uncle Jason brought Sophie her favorite meal (chicken caesar salad from Panera).

Sophie's beloved Grandma came and stayed the day with her.
There is no one in the world that she loves more.

Singing Happy Birthday to her.
She pretended to blow out candles.
Too cute!!!

She had some fun with the icing on her cookie cake.

Uncle Jake came by for a visit and brought her a fun birthday present.

Daddy and Sophie.

A Nice Surprise

The EEG hookup went AMAZINGLY well. Not one tear shed!!! I am not sure how that is possible. Sophie has had over a dozen EEGs (I have lost count) and she has NEVER done that well. Not even close. The one she had six months ago, she almost vomited from crying so hard. This is a true sign of her development. We were able to talk her through it and reason with her.


She sat on Brandon's lap while holding her baby dolls*. We explained everything to her that was about to happen. We let her touch and feel all the things they were going to use on her. She kept saying "hurt" in the saddest voice. But we kept reassuring her that it would not. There were several times where she almost lost it but we were able to calm her down before that happened. I think it was also very helpful that she was simultaneously doing everything to her baby dolls that they were doing to her. Maybe she felt in control of the situation. I wish they sold EEG play kits (kind of like a doctor's kit).


Once we got back to her room, she kept wanting to leave and did not want to wear the backpack. So we resorted to letting her munch on all her favorite snacks since she was not interested in ANY of the distractions I brought. Finally, we put in a CD her teacher from school gave her. That got her excited. It is such a cute CD and she immediately recognized the songs. She knew the movements and even sang along to some of the words to a few of the songs.


She also LOVED when the doctors came in to check her out. She sat so still as they examined her. And when they (there were quite a few) would leave she would say "doctor come back". Have I told you how much she loves playing pretend with her doctor's kit at home and at school...it is one of her favorite toys.


She then ate a great lunch and now she is sleeping peacefully with her Daddy cuddled up right next to her. Child Life just came by and is getting together some toys for us. So hopefully, between my bag of tricks and the "new" toys, she will be distracted enough.


Thank you Uncle Jake for the yummy cookies and stopping by for a visit. Thank you Grandma Debbie for also visiting and watching the little ones for us. Brandon and I were able to have a nice lunch outside at one of my favorite restaurants down the street from the hospital.


*My sweet little 2 year old Elsa ran and got her beloved Dora doll for Sophie to take to the hospital with her when she found out Sophie had to stay the night in the hospital. And we also brought along baby Chou Chou...her favorite.

Look at that sweet, innocent face just moments after being hooked up. No bright red eyes. No tear stained cheeks. Just my sweet, little angel of a girl. All that stress...worrying...knots in the stomach...sleepless night...guilt...all for nothing. Thank goodness!!!

Guilt

That is what motivated my shopping trip today. Causing me to buy 3 bag full of goodies for Sophie. New toys, puzzles, DVDs and her favorite snacks are what filled those bags.


Because those bright red, tear-filled eyes are already haunting me. They are already breaking my heart.


Tomorrow, she goes in for a 24 to 48 hour EEG. Most likely 24 hours but they threw in the 48 hours for "just in case". She HATES getting the leads glued to her head. HATES it. HATES it. HATES it. It is pure torture for her. You should see the fit she throws for me when I want to brush her hair. Her short little bob cut with no tangles hair. She is very sensitive to people* messing with her head...as you can only imagine given her history. Because I still flinch when her mouth comes close to my arm in remembrance of her biting sprees. So I can only imagine the nightmares that must flash through her mind. They still flash through mine.


We are going in because she had been doing some "odd movements" that look eerily familiar. We upped her medication twice and things are looking much better. But we still have to know what is going on.


* Except for her 8 year old cousin Colin. In which, she would do ANYTHING for him...brush her teeth, lay still during diaper changes, brush her hair, take her medicine, etc. She absolutely adores everything and anything he does. He is the coolest person in the world to her. Colin can you please hop on a plane and be here by 8:30am tomorrow?

More on our 24 hour video EEG...

The hookup was horrible. She was crying so hard that she was making herself gag. Not that the EEG tech wasn't great, it is just that Sophie is now very sensitive to anyone touching her head. Can't blame the girl.

Once she was hooked up, she did surprisingly well. She had my and Brandon's full attention. And the attention of any visitor or doctor that came into the room. There was this one doctor, in his fellowship, that came by to get familiar with Sophie's history. She was standing on the bed and he was standing in front of her. She put her arms in the air and kept saying up. So he would lift her up high in the air. Over and over and over again. He was an average size guy and I am sure he was getting tired of lifting all 38 pounds of her over his head. But he obliged until finally I grabbed her so he could make a quick exit.

Around 9pm, Brandon went home for the night. And Sophie and I snuggled up in bed for the night. She slept very well. But by the morning, she was ready to get out of there. I think 24 hours is her limit.

Here are a few video clips...

More than I let myself believe...

So we got the preliminary results of the EEG. The news was not perfect. But it was pretty darn close. Amazing considering we are less than 5 months post-op. Though I could only dream of a perfect EEG, I truly do not know if that is possible. That is a great question I will have to follow up with Dr. Chugani. Not that it could ever be completely normal since almost half her brain was removed. But I wonder what the best case scenario is for her now.

There was a neurologist from our local children's hospital that followed Sophie's care while we were in-patient. He came by on Thursday to get her complete history and then came by after he read the EEG on Friday. There were also a few residents that came by to get her history. Everyone seemed quite interested in her case and I am more than willing to share the knowledge I have gained over the last 3 years to anyone who is willing to listen.


So the results are...

Sophie's right hemisphere looks healthy and there was NO SPIKING on the right hemisphere!!! There was SOME spiking near the motor cortex on the left hemisphere. SOME is so much better than CONSTANT. It is unknown whether it is truly coming from the motor cortex or tissue surrounding the motor cortex that was disconnected. I am not sure if we will ever know.


A bit of history...

During Sophie's grid surgery, through a burr hole on the right side, a strip of grids were placed to monitor her right hemisphere. They were concerned about her right hemisphere because there was an area of concern that showed up on the PET scan. Her EEGs always showed constant spiking on both hemispheres. And since her EEGs were constantly chaotic, they were unable to tell where her seizures were originating. That is until the subdural grid monitoring. Those results showed spiking on the right hemisphere but no seizures originating from the right hemisphere.

There was a big question going into surgery. Since there was constant spiking on her left motor cortex, we did not know if they were going to remove it, perform the MST on it or leave it alone. After the other areas of her brain were removed, there was no spiking on her motor cortex so they did absolutely nothing to it. Which is amazing considering the amount of spiking there was.


All in all...

I am so incredibly grateful that her right side is normal but a bit surprised about the spiking on the left. Honestly, I really do not know what the expectations were post-op besides controlling any possible break-through seizures with meds. I cannot wait to get Dr. Chugani's thoughts on all of this.

Dr. Chugani should have the recording by Wednesday. I am quite tempted to hop on a plane to Detroit right now and stand by his side (or Dr. Asano) and have them explain to me what they are actually seeing on the screen. If I only had my own personal EEG interpreter. I have read this and I am tempted to buy this or this. Because I have many of Sophie's prior EEGs along with her latest EEG saved on my hard drive. So if any of you white coatless moms or dads have found anything you would recommend, please let me know.

How beautiful does this clean sheet of paper look???

Chilling out with Daddy.

Getting ready to get the markers out with Mommy.

Thanks Aunt Cindy and Aunt Diane for visiting.

This sink height was way too perfect...EEK. We went through 3 outfit changes before I finally figured out to put the tray table in front of it. Though, I initially let her play in it for about 30 minutes. Any chance of getting electrocuted?

Snuggling up with NaNaw.

Having a blast coloring.

A fun visit with Red Robin.

Yet another EEG...

The med game continues. Except we are on the other side. Before we were always trying to find the right combo to stop the seizures. Now we are trying to find the right dose to make sure they don't come back. Trust me...I realize what side I want to be on. But the thought of a 24 video EEG has my stomach all in knots. Especially dealing with all of Sophie's behavioral issues. So I am nervous about her getting hooked up...tomorrow morning at our local children's hospital.

After the grid surgery, Sophie was put on Dilantin and Depakote. We had issues getting her levels up while we were in the hospital. They did quite a few boluses. They eventually got the levels up to the lower end of the therapeutic range. We got her blood drawn two months after surgery. They were really low. Dilantin was 1.3 (therapeutic range is between 10 - 20). Depakote was 26 (therapeutic range is between 50 - 100). So I e-mailed Dr. Chugani and he suggested we increase the Depakote and check levels again in six weeks. So we did. Dilantin stayed the same and Depakote increased a measly 4 points. So I e-mailed Dr. Chugani again and he suggested that Sophie get a 24 hour video EEG before making any more med changes.

I know it is only one night in the hospital. Seems like nothing compared to brain surgery. But I am still stressed. I not only have to think about myself and Sophie but there is also Elsa and Charlie. I am also anxious to see what her EEG is going to look like. Is it too much to hope for a clean EEG? She has never had a clean EEG. They were always constantly chaotic. I do not even know if it is possible to have a clean EEG after getting almost half her brain removed.

By the way, if anyone in the area wants to come by and say hi, give us a call on our cellphones.

5 Star Treatment...

Never would I have ever imagined that things would go as smoothly as they did...even with a little mishap along the way.

First of all, we were able to do the EEG locally. There was no worrying about plane trips or places to stay. The hospital, St. Louis Children's Hospital, is only 20 minutes from our house. I, of course, still overpacked and worried myself crazy the night before.

I was immediately impressed when we walked into our room. It was a private, spacious and well lit room...the nicest hospital room we have ever stayed in by far. We even had a view as we could see the Gateway Arch. Everyone we met was so incredibly nice...the nurses, EEG technician, in-house pediatrician, epileptologist, etc. I was amazed at having so many people check in on us.

But the best part has yet to come...for all of you who have been through a 24 hour video EEG or longer you will appreciate this. In the ceiling, there was a little dome with a camera in it. In another room, there was a technician who monitored the camera and EEG reading at all times. This allowed Sophie to move freely about the ENTIRE ROOM!!! The cord was so long that she was even able to step right outside the room. She was given a backpack to wear to hold the box that all the wires were attached to. At every other hospital, we were confined to the bed or one foot beside the bed in order to stay within camera view. No one ever checked in on us. If a lead came loose on Sophie's head, we had to notice it on the EEG reading and notify someone. There was no backpack so we had to constantly be hunched over Sophie so that every time she moved one of the wires didn't pop out of the box.

The following morning, the epileptologist reviewed the EEG and then spent at least 20 minutes explaining everything to us and answered all of our questions. She seemed to genuinely care and took the time to review Sophie's previous MRI and PETscan. Which amazed me since Sophie is not her patient nor is she a patient of a doctor at this hospital. They took a complete history of Sophie including every treatment she has ever tried. The epileptologist recommended trying felbatol and explained all the risk and side effects to us.

Our one minor mishap... Sophie loves to throw her body around like crazy. She thinks it is funny. She gets in these moods and becomes a complete wet noodle. It is completely on purpose because she only does it when she is in a really silly mood. So as I was waiting at the elevators to head down to the cafeteria the second morning we were there, I heard a loud scream. I just knew it was Sophie. So I headed back to the room (which is around a corner, down a hall and around another corner...that is how loud she was crying). When I walked in the room, Brandon was holding her and she was sobbing. He said that he was holding her hand and she just threw her body backwards and he heard a pop. So I held her, thinking that once she calmed down that she was going to be okay. Well, she refused to move that arm. I knew she had to be in a lot of pain because she has a very high pain threshold. Brandon got the nurse, who got the resident pediatrician, who then got the head pediatrician. She examined Sophie's arm, moved it around a bit while Sophie screamed her head off. She said that Sophie's elbow was popped out of socket and that she just popped it back in. She said that if she still wasn't moving it around within a few minutes that she would need x-rays. Well, within 5 minutes, Sophie was back to normal like nothing had ever happened.

Special thanks to Grandma Debbie for taking such great care of Elsa. She even brought her up to the hospital for a few hours which Sophie absolutely loved. It was calming to know that Elsa was in such great hands.

Special thanks also to Sophie's old ABA therapist, Jen. She came to visit us in the hospital and brought Sophie her favorite chips and a book. Sophie was excited to see her and even showed off some new words for her. Jen always had a way of getting Sophie to do things that no one else could.

Another EEG...

I'm anxious. Tired. Just feeling overall gloomy. In about 7 hours we will be leaving for the hospital for Sophie's 24 hour video EEG. I hate staying the night in the hospital. I hate the thought of being confined to a hospital room. I hate the thought of the tears that will be rolling down Sophie's face as the leads are put on her head.

It is going to be a gorgeous day tomorrow. And we are going to miss it. And it makes me mad. Then I think of Zoey and so many other children that have long extended hospital stays. I think of all the gorgeous days they miss. It is not fair.

But we are suppose to be grateful that there are hospitals for our children to go to...to become well. And I am grateful. Just sometimes, there are so many more mixed emotions that go along with it. But, between all of Sophie's hospital visits and all the testing, nothing has made her better. She continues to have seizures everyday. We are now at 600mg of Banzel (we increased to 500mg last Wednesday and 600mg yesterday). Her seizures are not any better. Maybe even a little worse. They are longer. Lasting up to 10 seconds per seizure. But she does seem to have a bit more clarity when she is having a good moment. It doesn't make any sense.

It is hard for me to let go. To know that I have no control. I just try to do my best in getting Sophie the best medical treatment possible. But ultimately I must leave things up to Him. To enjoy all the great things that Sophie does do and not dwell on the medical issues. But it is hard. It is hard watching your child suffer.

So if anyone is in the area and has any desire to come by and say hi...we will be there...just give us a call on our cellphones. We will be counting down the minutes until we get to enjoy some of the gorgeous weather that has come our way.