Home Sweet Home

We are home.  We actually got home late Thursday night.  Sorry for the lack of timely updates.  Sophie is doing well.  She is getting stronger by the day.

Everything has caught up to me and I am just completely exhausted.  I am trying to get back into the routine.  And with the holidays right around the corner, I have a lot on my To Do list.  But I am just soaking in these days...being back at home with my family.  I missed Elsa and Charlie a lot.  And it feels good to be cuddled up next to Sophie in my own bed (did I mention that I slept 7 straight nights in a hospital bed with Sophie!).

Happy Holidays!

A True Fighter...

She is doing Ah-May-Zing!!!  What a difference a couple of days have made.

Here's a picture just a few hours after surgery on Friday...

Then she slept for the next 3 days...

Physical Therapy and Occupational Therapy came this morning.  She was MAD.  I had to leave because she kept crying for me.  So, of course, I went down to the gift shop to buy her a prize...cute little slipper socks.  But, by the time I came back up, she was already in bed looking great.  She said "Mommy, I did good".  She was so proud of herself.  They had her (with support) walk down the halls of the hospital.  She is still weak but I know it will only be a matter of time before she gains her strength back.

Looking so awake and alert after therapy...

Do you see that piece of graham cracker she is holding?  She is eating and drinking, though we are taking it VERY slowly.  She gets a few sips of water an hour and has ate a little over half a graham cracker square.

After therapy, these clowns came by...

She is holding a magic wand in her RIGHT hand and popping bubbles.  What a great therapy idea!

I am hopeful that we will be home by the end of the week!!!

Thank you all for your love, support and prayers.  It really has lifted me in spirit.  You all have been on this journey with us and have been an important part of the healing process.  Thank you from the bottom of my heart.

One day at a time...

The most important thing... Sophie is seizure-free!!! I lost focus of that a few times in the last couple of days. She surprised us coming out of surgery on Friday. She was so awake and alert and thirsty. We tried to take things slowly to make sure she did not get sick. And she did not. She was a little swollen which was to be expected. She was doing amazingly well, all things considered. So I got my hopes up high that she was going to recover quickly this time around. She was already sitting up on her own, talking and drinking. I felt like we were already coasting. She showed a slight weakness on the right side of her body but I know she will regain most (if not all) of it with therapy so I was not all that concerned. She also did not seem to be in any pain. Things were beyond all expectations. But Saturday and Sunday were different. She has been sleeping almost nonstop. Though she was awake but not interactive for a period of time this afternoon. She refuses to take a sip of anything and continues to vomit or dry heave almost every time she sits up. But those things are minor and she will come around. I just need to be patient and focus on the big picture.

Surgery is over!!!

Dr. Asano and then Dr. Sood came in and talked to us. They both said all went well. They removed the cushion that they planned on and then performed the MST over the entire sensi-motor cortex. When they replaced the grids, they did not see any spiking. Therefore, they did not feel it was necessary to remove any additional tissue which will give her a better chance at preserving the motor function in her right hand. This is GREAT news so far!!! Now it is a "wait and see" game to see how much weakness she has and how much therapy she needs. Just like we wanted Sophie to have lots of seizures to be captured during the grid monitoring, we are also wanting to see weakness immediately after surgery. But we are hoping it will only be temporary and she should regain her strength within two weeks.

Thank you for all your prayers, support and love.

Game Plan

This is hard.  So hard.  Because I know she is going to come out with some weakness.  I just don't know how much yet.  And I do not know how much of it will be permanent.  They said to expect her to get frustrated and that she will need the support of her family to get through it.  Those are difficult words to hear.

Doesn't she look great?

Plans are different than what we thought they would be before we started this whole process again.  Initially, we thought she would just need Multiple Subpial Transection (MST).  But the intracranial subdural grids showed a focus.  And for a lack of better terms, you can say she has focal hypsarrhythmia in the cushion surrounding the lower bottom half of the motor cortex.   So, Dr. Sood is going to resect the cushion on the entire left side of the motor cortex.  Then, perform the MST on the entire sensi-motor cortex.  They will once again put the subdural grids back on and monitor the brain wave activity.  If they are still seeing spiking in the lower half of the motor cortex (which controls the facial muscles), they will then resect that tissue.  And then close her up and hope to God that she never has a seizure again.

A7 and A8 represents the cushion surrounding the lower bottom half of the motor cortex. 

Speaking of seizures, she has not had a visible one since 11:55am yesterday.  Just 3 hours prior to that, they had started her on Fosphenytoin (IV form of Dilantin).  Very interesting but that doesn't change the surgery.  The focal hypsarrhythmia and spiking still showed on the EEG.

During her left subtotal hemispherectomy (that she had 2.5 years ago), Dr. Sood removed all the tissue in her left hemisphere except the sensi-motor cortex plus one centimeter of tissue (cushion) that surrounds it.

Okay, let me try to explain this in simple terms because I know when I tried to explain it to my family last night, it became confusing to them.

Think of Sophie's sensi-motor cortex as a slice of white bread.  Now draw a vertical line down the middle of the piece of bread.  The left side of the bread is the motor cortex.  The right side of the bread is the sensory cortex.  The vertical line is a major artery.  The crust that surrounds the slice of bread, that is the cushion.  Between the cushion and sensi-motor cortex are small arteries that supply blood to both areas.  The closer you get to the sensi-motor cortex, the higher the risk of impairment.  Thus, the reason for the cushion.

Now think of taking a scalpel and separating the crust from the white part of the bread without actually removing any of the white part and avoiding all the arteries.  Not easy.  Now turn the crust of the bread into a squiggly line.  Even more difficult.  Dr. Sood will be performing this surgery microscopically.  

The part of the cushion they are removing is attached to the motor cortex that controls her right hand and right side of the face.  Therefore, there will most likely be weakness in this area.  But not total loss of control.  She will probably lose 20% of the function of her right hand.  But she should regain all of her facial muscles because the head is bilaterally controlled by the brain.

I believe this change in plan is good.  I *think* it gives her a higher chance of seizure freedom.  Even though it does give her a higher risk of motor impairment, the higher chance of seizure freedom outweighs that risk.

Does this all make sense?  If not, ask away.

By the way, the first incision was made at 10:40am.

So far, so good.

Sorry for the lack of updates on my blog.  Part 1 of the surgery went well.  Grids are placed and she is being monitored.  She has woke up a few times and seems to be doing well.  Very thirsty but no pain.

She lost a lot of blood and looks really pale.  They gave her some blood while in the OR and they are about to give her some more.  

She had a big cluster of seizures around 10:20pm, so they will have plenty of data to review tomorrow.

Brandon is taking the night shift and I am getting ready to head back to the hotel.

Day 1 (Take 2)

Her eyes widen as I carried her into the operating room. The lights were bright. Everything was white except for the few things that were draped in the sterile blue cloths. Everyone was wearing surgical gowns and masks on their face.  

It was much harder this time around. To kiss her goodbye. She is much more aware. I could feel the sense of panic in her. As I set her on the operating table, she did not want to lay down but she did. Then came the bubble gum scented mask that would put her to sleep. She fought it hard. Tears started pooling up in her eyes. It broke my heart. I sang her We're Off To See The Wizard as she drifted to sleep. I was hoping she would dream about it while she was under. It is her all time favorite movie. She has watched it so many times that she has most of the movie memorized. Her Grandma Debbie bought her Wizard of Oz puppets and a pop-up book that she treasures. She loves to act out the movie with those puppets almost on a daily basis.

I kissed her goodbye around 9:15am (Eastern time) and surgery started at 10:21am.

Here are pictures from this morning...

Hospital Owl and Hootie along with her own owl shirt.
Can you tell we have a theme going on here!

Reading the Wizard of Oz pop-up book with Daddy.

Thank you all for your love, support, thoughts and prayers. It means the world to us. It warms my heart and soul to know that so many people care.

My beautiful babies...

I know, I know...they are not babies anymore.  But they are mine and I love them so much.  They are perfect...each in their own unique way.  I am going to miss each and every one of them this week.  It breaks my heart just thinking about it.  Even though Sophie will never physically be very far away from me, she will just as well be a million miles away when she is in surgery.  I love her so much.  I know I am making the right decision, even when I doubt myself.  She is going to be in trusting hands that will be guided by Him.  I know she is going to be alright but it doesn't make it any easier.

Packing sucks!

We leave tomorrow morning for our trip to Detroit.  I am trying to stay strong...for Sophie.  And a piece of me is already starting to miss Elsa and Charlie.  I hate this.  I hate it so much.  But the possibilities that this trip may bring...it is worth every moment.

So my current thought...

What do I tell her?

We have told her we are going on a trip to see Dr. Chugani.  She knows who he is and she is kind of excited to stay in a hotel.  But we haven't told her anything beyond that.  Last time around, I didn't worry about this.  She wouldn't have understood anyway.  But this time?  This time is a whole different ballgame.  She is so smart.

I mean I have to tell her something.  Can you imagine waking up from brain surgery and not knowing what the heck just happened?!?!  So I have to prepare her somehow.  I just don't know how.

Take Two

Starting the blog post is sometimes the hardest.  I wait so long between postings that I have way too much to say and don't know where to start.  But I'll just jump right in.

Sophie is scheduled to have another surgery.  Another two-stage brain surgery.  In the first stage, they will place the grids to monitor the seizure activity.  Then, two days later, they will perform the actual surgery...Multiple Subpial Transection (MST).

I can talk about it so matter-of-factly at times.  With no emotions.  But the closer we get to the date, the more real it feels.  I am starting to feel the weight of it all.  I think what scares me the most is how Sophie is going to react to everything.  This time around, she is much more aware.  But I talked with another mom, Emma, whose son, Alex, had a second surgery when he was 7 years old.  She was able to put my mind at ease a bit after hearing how well (relatively speaking) he did the second time around.

But, it is brain surgery.  And when I sit down and think about it...really think about it...it is difficult.  More difficult this time around than the first.  I am already starting to feel the panic.  We are leaving in exactly two weeks from today.  Just typing that...seeing it in writing...brings tears to my eyes.  The packing, organizing and what not of getting ready to go out of town is NOT what is putting me in panic mode.  It's the fact that I want to soak up every last second of time I have with Sophie before I have to kiss her sweet little forehead goodbye, once again, before I walk out of the operating room.  That moment...when you have to pull yourself away...is the hardest.

Picture perfect...

She rode the Special Education bus.  It's not the bus I had ever envisioned her riding to her first day of kindergarten.  Well, at least not before she was born...when all my hopes and dreams for her were of those of typical parents.  And then there was a time in my life that I didn't even know if she would go to school.  So, riding the Special Education bus is...in fact...a huge milestone for her.

My dreams for her have changed.  It was a hard thing to accept.  It still is.  And it is an ever-changing dream.  Because I still don't know what her future holds.  It's not that any parent knows what the future holds for their child.  But when you have a medically complicated child, there is so much more unknown about the future.  Sophie still has daily seizures.  They are affecting her.

At least when she was seizure-free, I kind of knew where she stood and where she was going.  I knew that she would always be developmentally delayed (she did have the majority of her left hemisphere surgically removed).  But she was making tremendous gains.

We have tried a whole list of medications since her latest relapse.  Nothing is working.  Zonegran did help but the positive effects have worn off.  Her seizures are at an all time high since surgery.  

Her speech and language are surprisingly amazing.  She is incredibly social.  And, of course, the most adorable 5 year old girl.  So the combination of those three traits...well...she can pull off appearing typical at times.  And I am SO GRATEFUL for that.  Not for the fact that she can pull off appearing typical but that her speech, language and social skills are at that level.  So I am trying my hardest to enjoy these days.  The days where she is five, six and maybe even seven years old and her immature personality will still seem that of a typical child of that age.  I know I am probably pushing anything past kindergarten since I already see the "hmmm something is not quite right here" look in the eyes of her peers.  Because even though she is incredibly social it's not always appropriately social.  One of the reasons I loved preschool so much is that it was a mix of three, four and five year olds...so being immature was easier to blend in.  I hope that as she grows with the kids in her class that they will just accept her for who she is.

And even though I have accepted who she is, I still do everything I can (when I have the energy) to make sure she lives a well balanced life with just enough therapy but not too much.  It's a fine line.  And Charlie and Elsa definitely help me with that!

And so does my mother-in-law, Debbie.  I can't even begin to list the number of things that she has taught Sophie over the years in such a fun and exciting way.  They have an amazingly special bond and she has always been there for Sophie (and me!).

We are living our lives the best we can.  She tries our patience to the fullest extent almost on a daily basis.  She pushes me over the edge to where I say and do things that I regret so much that it hurts my soul.  And at other times, she can make me so proud that I cry tears of pure joy.

The first two weeks of school have been a huge adjustment...for me, for Sophie, for her teachers.  But we are all beginning to find our way.

Is it weird that...

I thought Sophie's little pee-drenched footprints were oh so cute???


It was 5 minutes before midnight. I was in the bathroom brushing my teeth when I heard Sophie coming down the steps. I knew it was her (Elsa had just woke up moments before and already crawled into our bed). But her footsteps sounded a little different (as I hear them every single night right around this time). I knew something was off.


Her pajama pants were soaked.


It has been so long since she has had an accident that I can't even remember. It still makes my heart skip a beat (in a happy way) that she is potty-trained. So proud of her.


Anyway... So Brandon and I cleaned her up and slipped some new pjs on her. And I went on the hunt for the pee spot...thinking it was probably going to be in her bed. Nope. It was those cute little pee-soaked footprints in front of her bathroom door. She tried. Just didn't make it.


(By the way, I just think I am in an extremely good mood tonight because I went to a workshop on ADHD and met a very knowledgeable psychiatrist that is willing to take us on as a new patient even after explaining Sophie's complicated history. Her ADHD has been wearing on me but even more so is trying to find the help/support that we need to deal with this issue. It seems the medical community is more focused on her epilepsy and how complicated and different her case is because of her brain surgery. The psychiatrist did admit that he has not treated anyone like Sophie but that it didn't mean that he wouldn't try his best to help us. See...it doesn't take much to make me happy.)

The flip side...

Before I continue on with my positive streak (I know one positive post doesn't constitute a positive streak but that is the direction I am going to try to go in), I will update on meds and seizures.

Since Sophie had her third relapse since surgery, we have made the following med changes... Weaned Dilantin. Added Vimpat. Weaned Vimpat. Reintroduced Dilantin. Weaned Depakote. Weaned Dilantin. Added ACTH. Weaned ACTH. Added Prednisone. Added Zonegran. Weaned Prednisone. Added Vigabatrin.

So, currently, she is on 225mg of Zonegran at night and 500mg of Vigabatrin twice a day.

She is tired. She is very wobbly. She is not talking as much.

She is only having one small, very mild cluster in the early morning.

We are waiting to increase Vigabtrin in hopes that some of the side effects will subside. We may have to decrease the Zonegran. And I hope with every last ounce of my being that Vigabatrin will work. Because the next option is to seriously consider surgery. And something as simple as putting on her backpack will no longer be simple. And I cannot bear to go there.

The little things...

Well, maybe they aren't so little if you had almost HALF OF YOUR BRAIN removed.

But here is my attempt to show you all the amazing things that Sophie can do now. Starting with something as seemingly simple as putting on her own backpack. Though, she does make it look easy...don't ya think?

Make an on-line slideshow at www.OneTrueMedia.com

The 3rd time is NOT a charm*...

I have written several posts over the last few months. Never to be published. I have so much anger and sadness and guilt with this relapse. It's exhausting and consuming. So I'll update as quickly as possible and hopefully this one will be published.


We tried ACTH. It FAILED.


I was worried that with the wean of ACTH (actually we switched to Prednisone for the wean) that we would see a steady increase in her seizures. And, at first, we did. But once we got to a higher dose of Zonisamide, the intensity and number of seizures within a cluster started to decrease. She is still having around two clusters a day. Small clusters. Very mild clusters. But seizures they are. On a daily basis.

So we are still fighting. We have Vigabatrin in the waiting (just received the package yesterday) along with the ketogenic diet (our appt is in April). We just increased Zonisamide on Monday night so we will give that a chance and see what happens.

On a good note. Actually...a GREAT note. We have seen improvement with Sophie's behavior. There has been a lot going on with medications since surgery. After surgery, she was on Dilantin and Depakote. Since her latest relapse in seizures we have...weaned Dilantin, started and stopped Vimpat, reintroduced and weaned Dilantin, weaned Depakote, started and stopped ACTH, started and stopped Prednisone and started Zonisamide. So right now, she is only on Zonisamide. With all that said, I think Dilantin was making her aggressive. And I think Zonisamide is making her calmer. She has been playing independently. She LOVES her iPad. She has been singing a lot lately. She has become attached to a few possessions and seeks them out. I have never seen her do that before. She has a Dora doll with Boots in a pouch, a magic wand and a music box. She'll put all three items in this little shopping cart and push them around. Or she'll just want to hold them while watching TV. I think this is a huge developmental milestone.

And speaking of milestones...we had her IEP yesterday. I PROMISE to update very soon with all the progress she continues to make (despite having daily seizures).


*This was the third time we have tried ACTH. And this is the third time it has failed. I just knew it was going to work this time. And why not?!?! She has had the majority of her left hemisphere removed...including her left frontal lobe where all the seizures were originating from (according to the grids that were placed prior to the resection). So, I just knew that ACTH would be powerful enough to stop the seizures from this small remaining area that is still active with seizures. I mean...it was powerful enough to make her gain 6 pounds in less than two weeks, have her face puff up with dark circles under her eyes, have wicked mood swings including roid rage, suppress her immune system, lower her potassium level (she had to be put on a supplement) and so much more ugly stuff. All this for the small sum of $130,000 from a company that has HORRIBLE customer service (and I am just talking about this round of ACTH).

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?!?!

Another blood draw...

I hate the night before Sophie has to get her blood drawn. Because I know what I have to do the moment she wakes up...get her dressed and out the door before she starts demanding food. Then the moment she sees the chair, I have to try my hardest to keep her calm. Then the moment from when they start checking out her veins, I have to use every muscle in my body to keep her screaming, fighting, thrashing body still enough so they can draw her blood. All the while, trying to keep my emotions in check because I am heartbroken in the inside seeing the tears just pour out of her eyes and the snot running down her face. Because this is how much she HATES getting her blood drawn.


But NOT today.


Today was different.


Today was blogworthy.


Today started off as usual. I told her she had to go bye-bye with mommy. I didn't tell her where we were going. No need to unless she asked. And she was just happy to be going somewhere. So we pulled into the hospital parking lot. She was excited to get out of her carseat. As we are walking down the hall, she seems to recognize the place. She said to me "we going to see the doctor". I said "yes" which actually did not turn out to be a lie (this is the same building her neuro is in which we ended up running into). We got to the outpatient laboratory and I signed us in. She said "where's the doctor". I said "we have to wait until they call our name". So they called us back and as I was giving them our insurance information, she recognized the chair. She looked at me with the saddest eyes and said "I get shot". I said "yes you are...I am so sorry baby girl". So as we walk toward the chair, she hesitates. Her face is full of worry. I pick her up and tell her it is going to be okay. She sits on my lap and I put my arms around her. They take her arm and tie the band around it. She is remaining calm. As they stick her with the needle, she screams out and flinches. But no tears and she is calm. I tell her to sit really still so they can finish quickly. And she does. She is sitting there watching the entire thing. She says to me "all done". I say "not yet, there is one more vial to go after this (they are collecting four)". They switch out to the last vial and she says "band-aid". I tell her "yes they will give you a band-aid". They finish and she is okay...a little hesitant but still very calm. She picks out a sucker and we are on our way out.


I tell her how proud I am of her.


And she says "I brave".