Infantile Spasms Awareness...

Reposted with permission from Danielle...the super sexy, awe-inspiring, soon to join the "my son had brain surgery" group mom that advocates with all her heart on behalf of Infantile Spasms and her most adorable son, Trevor.


on raising awareness

Once upon a time...




In a land far away...



Where seizures were in remission and life was not so draining. There lived a mommy with fight in her heart. This mommy...together with her family...made a very special journey. At the request of a very important woman...who also hosts the heart of an advocate. That was how it came to be that the mommy testified on Capitol Hill against the tyrant Questcor...and their criminal abuse of the Orphan Drug Act.



See, a few months prior to the mommy's son being ensnared by the Seizure Monster...Questcor amplified the price of their drug. The front line drug. The drug indicated in the rescuing of her infant son from the Beast. Acthar gel. Over the course of a handful of days...Acthar's price point was increased from approximately $1200 a vial to $25,000 a vial. And it did not halt there. Today it can cost upwards of 30k per 5 ml vial. Most infants require a course of at least 4 vials.



Suddenly the mommy...seizure saturated son in her arms...was thrust fully into the war that rages between hospitals...insurance companies...drug corporations...and families holding broken children. Her insurance company initially denied coverage. The saga was intense for nearly a week as her son's neurologist went to battle beside her...writing scathing letters on behalf of the seizing baby boy.



In the end...they won that battle. And her son received treatment. Which helped for a time. But despite a price tag which could buy a decent family home even here in New England...Acthar is not a cure. Although it is one of the very very slim "chances" our babies of Infantile Spasms have to escape the Monster. If even for a time.



Which the mommy's son did. He was Seizure Free for a much too short spell. But the rage against the price-gouging tyrant did not lessen after the battle was won. It intensified. Posts were written. A few press corp attempted to tip the scales of right versus wrong. Capital Hill testimonies before Joint Economic Committees were passionately delivered. Interviews for books and even prominent televised news programs were given...although sadly most of those fell by the way side. Because, I suppose, she had no relation to Hollywood. But the mommy...whose advocate blood runs deep...continued to work together with other parents to increase awareness. To buck the system. To be the squeaky wheel and be heard.



But then on a different devastating day the seizure free time ran out. And day to day life became so altogether draining that the mommy had no energy left for advocating. It was all used up caring for her son. Who although injected with over a quarter of a million dollars worth of Acthar was not cured. But rather assaulted by 100s of seizures daily yet again. Because although I have shared this narrative in the framework of a fairy tale. It is anything but. And two weeks from this very day my beautiful little boy will be lying in a hospital bed. Wires protruding from his cranium. Connected to the Beast inside his skull. While we anxiously wait for the dawn of the following day. When doctors will preform what is closest to a real cure for seizures that we have at this time in history. Radical brain surgery. The removal of most of my little boy's left hemisphere. And pray to God...the Seizure Monster with it.



Quite possibly for a cost less than that of the Acthar gel with which he was injected. And proved not to be a cure. Though it may cost the same.



How ironic that Questcor...along with the Child Neurology Foundation...would be hosting an Infantile Spasms Awareness Week during the very month that Trevor (whose mommy once testified before the JEC against the tyrant Questcor) will be wheeled into a surgical room. Where the Seizure Monster their drug could not cure will be removed at last.



Also ironic that Don Bailey, CEO Questcor, with whom I have shared dialogue via email a time or two would not make me "aware" of their attempt at increasing awareness. Nor any other advocate parents with whom I am connected. In fact, I know of at least one parent who contacted Questcor in hopes of gaining an agenda for when this proposed awareness would be taking place...only to be directed to the Child Neurology Foundation. Who really had no clear answer.



Speaking of which...while monitoring my sitemeter I noticed someone from CNF spent hours combing my site. My silly hopeful heart. She actually entertained the thought that I'd shortly receive an email. And yet there has been no olive branch offered from that direction either.



Which leaves me wondering why?



Why would you not reach out to the parents whom you are cognizant have a passionate desire to increase awareness?



Perhaps it is not at all about increasing awareness? And I have no doubt my suggestion would not be remotely appreciated.



I certainly agree with Marissa's Dad that increasing awareness would best happen among those not already intimate with the disease. The pediatricians for instance. The educators who think every seizure is a grand mal. And such. Yet it is our understanding that this so-called IS Awareness Week will take place amongst neurologists. Whom I dare-say should have a solid level of awareness already. Which no doubt will be divulged over platters of fruit and cheese.



Which brings me to my own suggestion?



If Questcor (who makes very ambitious claims regarding the efficacy of their Acthar gel) truly stands behind their drug as a cure? If they truly boast a deep seeded desire to provide awareness for the families suffering this very rare and unknown disease.



I think an ingenious way to create a buzz would be to do something truly dramatic. Truly generous. Truly grounded in the belief that their drug is more than effective...but rather a cure...80% of the time.



I submit to you...short of decreasing the cost of their drug to bring it more in-line with other rare disease treatments...



Why not offer a reimbursement program to those who trial their drug and find it not to be a cure after all?



If Acthar fails - Questcor will reimburse



It's not a super sexy slogan. May I remind you that my creative energy levels are running on fumes as I prepare for my son to endure radical brain surgery.



Call me crazy, but I do believe that would generate some awareness.

Turning the corner...

...And there is a big wide open field. No wall. Sophie's behavioral issues are getting better and we have seen a spurt in her development. Especially this weekend.

So as the weekend unfolded and the blogworthy news kept building, I knew I just had to take the time to sit down and type it out...as quickly as possible because the clock just ticks way too fast during the day.

It all started Saturday morning. I was watching Sophie from the sidelines in her second swim lesson. The instructor, Sara, put a floating device around Sophie's waist and got her into the water. As Sara was holding Sophie, she told her to hold on to the edge of the pool and then she let go of her. And she held on!!! Just like that. She understood. In that moment, she looked so old. So...dare I say...typical. Just a 3 year old, holding on to the edge of the pool, chilling out. By the way, I will definitely remember my camera next week.

But it didn't stop there. She told Sophie to close her eyes. She did. Squeezed them so tightly together...too cute. She told Sophie to put her nose in the water. She did. She told Sophie to kick her legs. She did.

I was so proud of her that I had to fight back the tears!!!

Then came dinner time. Brandon went to the baseball game so it was just me, Sophie, Elsa, Charlie and Grandma Debbie (aka NaNaw). We decided to be brave and go out to dinner. Taking Elsa and Charlie...easy. But when you add Sophie to the mix...well nothing short of EXHAUSTING. But we were up for the challenge.

And Sophie did great. The I'm so proud, I could cry moment was when she dipped a piece of fish in tartar sauce. First of all, she requested dip by saying "dip, dip, dip". She learned that from Elsa. Elsa LOVES dip and will eat almost anything if you dip it in something. But it wasn't the requesting as much as the action. It was such a controlled and purposeful movement. She delicately dipped the edge of a piece of fish and then popped it into her mouth. In that moment, she looked so...typical (I don't like using that word but I don't know how else to describe it). Not that she looks so typical to the outside world...sitting in a highchair that looks way too small for her while obsessively blowing raspberries in the air and of course her very cute do*.

*Once, a waitress took one look at Sophie and made the comment...it looks like someone got a hold of the scissors.

There is more...

Late Sunday morning, Sophie said "dough dough" out of the blue. So I said, if you want to get a doughnut, you have to put your shoes on**. Getting a doughnut is a treat in our house. Occasionally, we will take the kids to the doughnut shop and go to the park. So she walked out of the family room, through the kitchen and grabbed a shoe out of the back closet and brought it to me. Usually, Sophie gets distracted in these situations and never quite makes it to where she initially plans on going. So for her to make it somewhere AND come back is huge. Most of the time, I continuously verbally redirect her or hold her hand in order to get her to follow through. And half the time, this doesn't even work because she will have a meltdown in the midst of it all. This time, there was no redirecting her since I really did not feel like taking her...it was raining outside and she just finished eating a big breakfast. But I was too proud of her to not follow through. Well...actually...Brandon took both her and Elsa.

**She hates wearing her shoes. You can't blame her. Who wants to wear hard plastic orthotics all day long. Just not comfy. But necessary.

There were more of these moments. I just can't think right now. All in all, it was a refreshing weekend. Something I have been longing for. And it could not have come at a better time. With all that was going on with Julia this weekend. I hope this good news gives this family hope. That Julia's surgery will be a success. That her parents will soon be blogging about their I'm so proud, I could cry moments. Please keep this family in your thoughts as Julia is in her last few moments of surgery.

Nothing but love, hugs and warm thoughts sent to you...sweet Julia.

The sweeter side...

Note: I wrote this late Sunday night and was interrupted by a screaming Elsa that I had to get back to sleep before I could publish. So "Today" refers to Sunday.

Today was a good day. Sophie and I had a moment. Where we totally connected. Where she was focused. Where she was calm. Actually, it was more than a moment...more like 15 minutes.

Elsa and Charlie were napping. Haley was on the computer. Brandon was playing hockey. Perfect setup to do some much needed one-on-one bonding time with Sophie.

Lately, it has been very hard for me to get Sophie to have some down time. She is constantly on the go. I miss cuddling with her. She was so cuddly and snuggly pre-op. Even post-op for awhile. But when the behavioral issues were kicked into high gear, the sweet cuddly Sophie disappeared. She just could not sit still.

Until today.

We snuggled in bed together...sharing a pillow...laying face to face. I asked her to point to my nose. She did. I asked her to point to your nose. She did. That is a new thing she has learned recently...the meaning of my and your. So we went through all the facial features. She not only pointed to all of them but she said them all too. My heart was beaming with joy. To have this moment. Then we had a little conversation, that went a little like this...

Sophie: Daddy?

Me: Daddy play hockey.

Sophie: Hockey.

Me: Daddy hockey.

Sophie: Hockey.

Me: Daddy hockey.

This went back and forth a few more times. We are working really hard to get her to put two words together, so sometimes we speak in very short phrases.


Sophie: Bock?

Me: Big Rock upstairs.

Bock is Big Rock Candy Mountain DVD that we usually play on a little TV we have in my room but we moved all of that upstairs into their room where Elsa was currently sleeping. This conversation also went back and forth a few more times.


Sophie: Night Night.

Me (singing my made up Night Night song): Night Night Sophie. Night Night Elsa.

Sophie: Row Row.

Me (singing Row, Row, Row Your Boat): Row, Row, Row Your Boat.

Sophie: Night Night.

Me (singing): Night, Night...

Sophie: Row Row.

Me (singing): Row, Row...

This went back and forth quite a few more times. With a HUGE smile on Sophie's face. And even a BIGGER smile on my face. See...our nighttime routine begins with reading books. Then I turn off the lights and lay in bed with Sophie and Elsa and start singing the Night Night song. Elsa, not wanting to go to sleep just yet, will immediately cut me off and starts demanding (in her cute sort of way) another song. Once I start singing another song, she immediately cuts me off again and demands another song. Usually, it goes back and forth between Night Night, Row Row and Monkey (5 Silly Monkey Swinging in the Tree). I humor her a few times and then I say "last song" which she inevitably requests Night Night and they both fall asleep. Well, this was the first time Sophie has ever played this "game" with me. She is getting "it". She is starting to understand things on a deeper level. Simply amazing!!!


Well, our bonding time ended when she started obsessively poking me in the eyes and then went on to obsessively slapping herself in the mouth with the palm of her hand with her tongue hanging out.

But it left my heart full of happiness. Her eyes would light up and she would get all excited every time she knew I understood what she was saying. I am so proud of her. And these moments are what get me through the not so proud moments I have with her. Which I will post about soon...her behavioral issues.

But in the meantime, here's a video of Sophie and Elsa being silly one night right before bedtime. Sophie keeps saying hand throughout the video because she wants to hold Elsa's hand.

Sweet Julia...

Through the wonderful world of the internet, the paths of so many journeys are crossed. This time it is the journey of sweet little Julia. I met her mother, Lisa, about a month ago. She stumbled upon my blog through a link on Ken's blog, Blogzilly. Julia is scheduled for the two part brain surgery that is very similar to Sophie's. The grid surgery is scheduled for September 18th and the resection surgery is scheduled for September 22nd at Children's Hospital of Michigan with Dr. Chugani's team.

My thoughts go out to this family as I know all too well the emotions they must be going through right now. Please keep them in your thoughts and prayers.

You can follow their story on their blog page Daniel and Julia.


The adorable little Julia...

Bear with me...

I am trying HARD to sort through my thoughts and feelings. Life is not as bliss as I thought it would be post-op. So many emotions. So many issues. So many things that I wish not to talk about because there is too much guilt wrapped up into it. But I am not being honest with myself. It is what it is and I am trying to push the guilt aside. Because I have to. For Sophie. I have to deal with these issues and stop pretending they are temporary. Because it has been more than 3 months post-op.

She remains seizure-free. Again, I never know how to write that. When I type it and see it written before me...it looks like a simple statement. But it is so much more than that.

Before Sophie's subtotal hemispherectomy, these were my thoughts... If we could just stop the seizures and focus just on development, life would be great. We just have to get through this surgery successfully. Everything here on out will be manageable. WRONG.

And that is where the guilt lies. Because if I were to complain about her post-op issues, it would seem like I do not appreciate the fact that she is seizure-free. But I do. More than ever. So for this post, I am going to try hard to put the guilt aside and be honest. Because these things need to be said. I'm scared, though. I'm scared these words will someday haunt me. Because if her seizures were to ever return, these things I am about to write about will be trivial.

Deep breath, here I go...

Sophie is out of control. Everything I do with her feels like a monumental task. Every...little...thing. I try so hard to be patient with her. Because I realize there is a lot going on in her brain right now that she is unable to comprehend. That I am unable to comprehend.

Her behavioral issues are such that we added 12 hours of ABA therapy to preschool. That is why she goes a full day. Her pediatrician talked to me about the possibility of needing to put her on an ADHD drug in the future. Dr. Chugani suggested maybe putting her on a behavioral med called Risperdal. None of which any of us are ready to do at this moment...just something to think about. I will discuss all of this in length with Dr. Chugani at her follow up visit in 3 months.

The magnitude of her behavioral issues are hard to explain. Each little act may not seem significant. But it is the fact that it is one act after another after another.

I am so glad that she was in preschool for almost a full year prior to surgery. Because her teachers and therapists know what she was capable of before surgery. They are a great support system for me.

Now her behavioral issues are not to be confused with her development. Because she continues to make stride developmentally. The number of words she is able to say increases by the day. Her awareness of her surroundings continue to increase. Her sense of humor amazes me.

Please, please, please don't think I am not grateful for all that she has overcome in these last 3 months. I DO appreciate it. I AM grateful for it. And I am sorry to those who I may have offended for complaining about things that seem so trivial.

A quickie...

I am taking the approach that a quick post is better than no post at all. Sophie remains seizure free. I write it so matter-of-factly but I truly want to scream it for the world to hear...especially to those doctors that said she would not be a surgical candidate.

August 30th marked her 3 month seizure free anniversary. One that I am too scared to celebrate. Too scared to get too comfortable.

Sophie started school 3 weeks ago. She goes full day Monday through Thursday. That is a whole different post that I will hopefully write about soon (among many other topics). In the meantime, here are some random pictures...

No...not another EEG.
I had to wrap her head at night to stop her from picking at her suture site. She would pick and pick and pick which caused it to bleed and bleed and bleed. For weeks on end, Sophie slept in-between us with one of us semi-sleeping so we could stop her from picking. Because even with this nice looking head-wrap, she was still able to get to it. It is finally almost healed.

First day of preschool. This was the best picture I could find. She was in a daze when I started snapping away.

First day riding the bus (3 days after school started). She rides the bus to school and then I pick her up after school. She is the last stop and the school is only a mile away so she is only on it for 5 minutes...the guilty part of me felt the need to say that. She cried the first day or two but she happily gets on the bus now.

Elsa's first day of preschool. And yes...this is also the best picture I could find. Why are my kids in such a daze on their first day of school once I get the camera out???

Sophie getting a much needed sensory fix. She loves to rub her inner arm up and down stubbly chins. She will actually go up to a complete stranger and do this.