Monday, December 09, 2013

Against All Odds

I have the most ridiculous smile on my face. My world seems a bit lighter. I can now let go of the anticipation of this day, as it is now here.  A day that did not seem possible. Today marks 2 years since Sophie's second round of brain surgeries. Two years. It's hard to believe.  

On May 10, 2006, our world as we knew it was changed forever. That day was a little over seven and a half years ago and I still get a rush of emotions when I think about it. It takes my breath away and makes my heart beat a little bit faster. I can feel the anxiety rising. When I close my eyes, I can vividly picture that day. I remember the exact spot in the office where I was standing...where I soon crumbled. I remember the stream of tears that I could not seem to stop from continuously flowing down my face. I remember driving straight to the hospital. I remember waiting at admissions to be checked in. I remember a staff person coming to get us to walk us to our room. I remember looking at Sophie and feeling so helpless. That day will forever be etched in my memory. The pain of that day will always be with me.  

But to fully appreciate today, that day and so many other pivotal moments along this journey that lead us to today must be remembered.  

After that day, I spent an obscene amount of hours on the internet to research all I could about Infantile Spasms. For days on end, every single night after Sophie went to bed, I sat down in front of my computer to research. I struggled to find any good news and often ended up in tears. The words uttered by our neurologist just moments after we were given the devastating diagnose seemed to hold true. We were told that she had a 70% chance of being mentally retarded and we were to start her on a drug that had a side effect of death. I am not sure if all this information was necessary at that exact moment but I guess one might believe to just pile it all on at once.  

That drug that we were to put all our hope into is called Acthar gel (a natural product derived from a bovine or porcine source of the adrenocorticotropic hormone or ACTH). While we were in the hospital, we were taught how to inject this drug into our daughter's muscle in her upper thigh. In other words, I held her down while Brandon injected an obnoxious size needle into her leg every single day for a little over two months. Soon her thighs looked like they were sprinkled with freckles from all the little scars from the injection sites.

ACTH was a failure as it did not stop her seizures. Though they did reduce the number and severity of the seizures while she was on the drug, the seizures slowly crept back up and only got worse from there. We were given options of other drugs but were told the possibility of any other drug stopping the seizures was low. But we tried anyway...Topamax, Vigabatrin, Zonegran, Depakote, Clonazepam, Clobazam, Ketogenic Diet, Keppra, Depakote (again), IVIG, ACTH (again), Banzel and Felbatol...all over the course of three years.

As Sophie's numerous EEGs never showed a focal point in which the seizures were originating nor did her initial MRI show any abnormalities, we were never given any other options but to give her these medications that were failing her.

In the meantime, I met an amazing mom online through an Infantile Spasms support group who has a son a few years older than Sophie. It was because of her that I found out that I should pursue brain surgery as an option for stopping the seizures. At the time, it sounded barbaric to me. I mean how could you possibly remove large portions of the brain...entire lobes, maybe even the entire hemisphere. How could a person, a child...my child...survive that?! Your brain, in essence, is who you are. Once it is removed, there is no going back.

I soon learned through her that a chaotic EEG showing generalized seizures and a normal MRI did not automatically rule out brain surgery. That even though Sophie's MRI (which shows the structure of the brain) was normal, that she needed a PET scan (which shows the function of the brain). She also told me about Dr. Harry Chugani, a pediatric neurologist known all over the world for specializing in Infantile Spasms and brain surgery.

When Sophie was 11 months old, we traveled to Detroit for our first visit to the Children's Hospital of Michigan for a PET scan, 24 hour video EEG and an appointment with Dr. Chugani. In order to be a candidate for resective brain surgery for epilepsy, only one side of the brain must be affected. Unfortunately, Sophie's initial PET scan showed not only a large area of hypometabolism in the left hemisphere but also a small area of hypometabolism in the right hemisphere. Even though Sophie was not a surgical candidate at this time, we kept in touch with Dr. Chugani via e-mail. He is an amazing doctor like that...as busy as he is, he always finds time to quickly (usually within 24 hours) respond to e-mails from parents all over the world even if your child is not a patient of his.  

As our list of failed medications grew, we decided to make another trip to Detroit when Sophie was one month shy of her 3rd birthday. This time, the PET scan only showed hypometabolism in the left hemisphere and the right hemisphere appeared normal. In other words, we had one foot in the door for Sophie to become a surgical candidate. Though more testing needed to be done, so we returned to Detroit the following month. With our hopes up high, it was devastating to find out that the test results were not conclusive enough for Sophie to become a surgical candidate at this time. We were left with instructions to repeat the EEG in six months to find out whether a focus would appear. I was too exhausted to travel to Detroit again, so we did the overnight EEG at our local hospital six months later. I got a copy of the EEG on a disc to send to Dr. Chugani before we even left the hospital. The disappointment of finding out that Sophie was not a surgical candidate last time weighed so heavily on me, that I waited almost three weeks to mail the CD.

Then a few days after mailing the CD, I received a call saying they were going to present Sophie's case to the surgical committee. I was in complete shock. Unbelievable. I could not believe this day was here.

On May 29, 2009, at the age of 3.5 years old, Sophie had a left subtotal hemispherectomy. A surgery that removed her left parietal, occipital, temporal and frontal (except the motor/sensory cortex) lobes. She went from having hundreds of seizures a day to none! She went from being virtually nonverbal to talking up a storm! All those therapy sessions where we saw very little developmental gains were finally paying off. Our child's brain now had the ability to make new connections and her cognitive development was nothing short of amazing to watch.  

Somewhere around ten months after surgery, Sophie's seizures relapsed. I knew it had but I was in denial. I did not want to believe it. I think it took me about a month to come to terms with it. I was exhausted and lost. Elsa just turned 2 years old and Charlie was about 8 months old. We tried adjusting medications which would temporarily work but the seizures were relentless. We even tried another round of ACTH. We had to pull Sophie from preschool since her immune system was suppressed from the ACTH. I don't know if it was from the exhaustion of having three kids or the depression of the relapse in seizures, but this time in my life was a blur.

After we exhausted another long list of medications, we decided to pursue brain surgery again. Though this time it was easier to convince them to go in. The biggest challenge we faced this surgery was that there was not much tissue remaining in the left hemisphere. We had a high possibility that they would want to remove the left motor/sensory cortex which would have left her with a physical deficit on top of all the cognitive challenges she was already facing. In the end, it turned out all the seizures were originating from a 1cm by 1in piece of cushion that was surrounding the motor/sensory cortex. They removed that piece of tissue and did extensive Multiple Subpial Transection (MST) over the remaining tissue.

Which...finally...brings us to today. Two years since that last surgery. TWO YEARS OF BEING SEIZURE FREE!!! Sophie's latest EEG was about 9 months ago which showed NO SPIKING and NO SHARP WAVES. This was the first ever EEG she has had that did not show any epileptic or pre-epileptic activity.

By the way, we moved to a Chicago suburb a little over a year ago (for Brandon's job). Sophie is now in 2nd grade and is doing amazing. She splits her time between the general education classroom and instructional classroom. She receives OT, PT, ST along with instructional minutes at school. She also has private OT, ABA, vision therapy and music therapy.

She is considered an emerging reader. She recognizes all the letters in the alphabet, knows the sounds they make and has memorized a long list of sight words, though she struggles with decoding (sounding out words). She still loves pretend play and likes to act things out with her Disney figurines. Her fine motor skills are getting stronger. She is able to draw stick figure people and write her first name.

Her biggest obstacle in learning is her ADHD. We have tried a few medications (Tenex and Strattera). We just started back up on Therapeutic Listening. We have thought about trying Tenex again or possibly Focalin (she has another doctor's appointment on Tuesday to discuss these options).

And though sometimes I felt lost and that I had lost hope, there was always a small part of me that kept fighting on behalf of Sophie through all of this. Sometimes I had no idea what I was fighting for, but I knew I had to keep moving forward for her. For her perseverance in life has taught me so much.  Her smiles through it all has held me together. I still take each day as it comes as I know all too well that the possibility for relapsing is there (especially when hormones start changing). So I will enjoy today...the first time in her 8 years of life to be able to say she has reached the two year anniversary of being seizure-free. It only took us two rounds of brain surgeries to get here! 

Monday, March 12, 2012

A whole new ballgame...

Sophie has been through a LONG list of anti-seizure medications.  I can name off a dozen without even blinking an eye.  Somehow, I have gained comfort in the knowledge that I have built up over the years when it comes to anti-seizure medications.  But now that she is seizure-free, I am changing my focus to ADHD medications.



I meant to do a whole post on Friday, March 9th...her 3 month seizure-free anniversary.  I can't believe it...3 months already!!!  Absolutely amazing.  So this is what her hair looks 3 months post-op.  By the way, I love when people ask if she got a hold of some scissors so that I can drop the "No she had brain surgery" bombshell.  For me, it's all about raising awareness.  And I hope I put people at ease by talking so candidly about it.


She is 6 years old and in kindergarten, in other words, it is important for her to have the ability to focus on tasks that are demanding of her.  But learning about a whole new set of drugs is exhausting.  There are stimulants, non-stimulants, long lasting, short lasting, side effects and the list goes on.  And, really, I don't want to have to start trialing a whole list of drugs.  I have to trick her into taking these drugs and then she doesn't have the ability to tell me how they make her feel.  I hate that.  It makes me sad to see what the side effects of these drugs do to her.  I want a magic wand to wave and have "the" drug poof from thin air.  You know, "the" one that will help her focus without having horrible side effects.



Grandma Debbie bought this for Elsa for her birthday a few months ago...it was the only one that was big enough for Sophie to still fit it.  And she loves it, though she is a crazy driver.  For the cost of these power wheels, you would think they would come with a remote control.


Pure joy on Sophie's face.  Charlie, on the other hand, is not so sure about her driving skills ;)


So we started her on Strattera.  We started with 10mg in the morning but it was making her tired so we switched to the evening.  Within an hour of taking it, she is conked out.  No late nights for her but she was always great about going to bed anyway.  This past Friday night, we increased it to 18mg.  I can't say that I have seen any improvements yet.  It has made her more sensitive, as in, the tears come streaming if she gets her feelings hurt over any little thing.



Happy belated Valentine's Day.


But, for the most part, she is happy.  She is full of imagination these days.  She has always been great at pretend play but she has taken it to a whole other level.  The other day, I heard her in the other room playing with her hand puppets when I heard her talking in this deep voice.  That was the first time I heard her change the sound of her voice while playing with puppets.  Melted my heart.



She was going to a friend's birthday party where you were suppose to wear flip-flops.  She wasn't so sure about that.  They lasted all but a few seconds on her feet.  But she had a blast at the birthday party.  Her friends in kindergarten are so great about looking out for her.  They love her to pieces and truly care about her.  In some ways, I don't want her to get any older.  I wish I could freeze her at this age.


We are working on pre-reading skills.  She recognizes all her upper and lower case letters.  She also knows the sounds of most of the letters.  Fine motor skills are still very difficult for her.  But luckily (for her...I know how important it is for others), handwriting skills are not as important as they use to be.  We will continue to work on pre-handwriting skills (circle, squares, etc), but when the time is right we will introduce an adaptive keyboard.  Her expressive communication is still one of her strongest areas.  According to the chart below (thanks to Danielle for the posting this), I would say that she is solidly in the 4-5 year age range and would be in the 5-6 year age range if her speech was more fluent on a consistent basis.  Because you should hear the sentences and words that flow freely and naturally when she is playing with her Wizard of Oz hand puppets.


Thanks to Wee Talk Speech & Language Services, LLC for the above chart.

Friday, January 27, 2012

She is doing GREAT!!!

Oh goodness, I am horrible.  Sorry for the lack of updates.  Life has been...well...crazy but in a good sort of way.  I mean, I am a stay-at-home mom of three kids.  One that is recovering extremely well from brain surgery.  Another that is a keep-me-on-my-toes-sensory-seeking two year old.  And a just turned four year old that is a highly demanding momma's girl.  Between that and me having a hard time letting Sophie out of my sight, I have been physically and mentally exhausted.  It's hard for me to even think beyond what is right in front of me.  






But all of that has changed this week.  At the end of the day today, it will mark Sophie's first full week back at school.  Her energy level is there and so is my comfort level.  I know I have said this before but she is just AMAZING.  She really is.  I am just in awe of her.  She is one determined little (umm big...but it's hard for me to accept that she is getting so big) girl.





She remains seizure-free.  There isn't a day (or night) that goes by that I am not incredibly grateful for that.  A huge weight has been lifted from me.  It's hard to even think about my emotional state of mind prior to surgery.  Or, actually, prior to finding out that she was a surgical candidate (because the few weeks prior to surgery is a whole other story).  I will never be able to find the words to express the heartbreak of having to hold my child every single night while she had a cluster of seizures.  It was like clockwork.  Usually around midnight and then again around 4am.




My heart still pounds a little faster when I hear her moving around in the middle of the night.  Usually, she is just fishing for my hair.  Once she finds it, she will fall right back to sleep.  Yep...she sleeps with me.  But I feel my comfort level growing there too.  I think she is ready to sleep in her own bed again.  She was a great sleeper until she was 18 months old.  That was when the drop seizures started and she would wake up in the middle of the night with bloody lips (even with all the padding I put on her crib).  So she started sleeping right smack dab in the middle of Brandon and me.  And she stayed there until she was seizure-free after her first surgery (when she was 3.5 years old).  Then back to her own bed she went until her seizures relapsed eleven months post-op.




But this time is different.  It just has to be.  I am once again filled with so much hope.  Seizure-freedom seems so real.  So permanent.  Why not?  Right?  Miracles do happen.

Saturday, December 17, 2011

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!

Tuesday, December 13, 2011

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.

Sunday, December 11, 2011

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.

Friday, December 09, 2011

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.