why I wear purple every March 26th

 

re-posted from my guest appearance over at Team Aidan

 

 

I should establish up front that I have tendency to take the scenic route.  If you’re interested in gutting it out with me you might want to pause here so you can grab a cuppa joe.  A box of Kleenex wouldn’t hurt either.

 

 

:: wink ::  

 

 

Also, one quick thing before we take the plunge.  Though I’ll keep slipping into the first person “I did this” or “I did that” – the truth is – I’m not on this journey alone. My mom is not able to contribute as much recently due to health issues, but during the most dire times she was available at the drop of hat to help me.  In fact, Toby and Bristel lived with my parents while we spent almost a month in Detroit. I’m also connected with other IS families who are my virtual safety net and sounding board. We have support from our local community too – friends gave hotel points, air miles, money, many a ride to & from various sports for the big kids which allowed them to have a little slice of “normal” during some very “abnormal” stretches and even delivered meals when we finally came home after surgery. We have a plethora of prayer warrior support. Also, I married good. Jonathan is an amazingly tender husband. He supports me in many, many ways but a biggie has been taking night duty (Trevor would wake 5-10 times a night with clusters of seizures) which allowed me to have rest so I could face whatever battles the next day might bring. And of course, I have three amazing children who inspire me daily to love harder than I ever thought possible.

 

 

Okay then.

 

 

Take a breath.

 

 

Pull a tissue.

 

 

Cause here we go…

 

 

It so happens a new (to me) mom was at our weekly Homeschool Ice Skate.  And as we were chatting The Story came up.  To get the full effect of The Story one must imagine melodramatic music floating and weaving around the words.  As it all came tumble bumbling out of me (minus the music)…I was struck by the absurdity that is The Story.  Otherwise known as our life.  If I weren’t actually living it…I would totally be rolling my eyes at the crazy lady telling it.  Thoroughly convinced that she were an embellishing-spot-light-loving-drama queen.  Or maybe she has a nasty case of Munchausen by proxy.  But that’s just it.  Every crazy unbelievable thing I said is true.  Without a twitch of embellishment.  If anything I try to downplay the drama so I don’t scare potential mommy friends off.  I don’t know about you…but I find that The Story has a way of freaking people out.  Like it’s catchy.  Or that I’m somehow super human which makes me un-relate-to-able.  But these other moms were just listening.  A little slack jawed.  But listening.  And I couldn’t help myself – the irony and absurdity tickled my funny bone.  I started (inappropriately, I’m sure) laughing.

 

 

Like, double over.  Hold my side.  Belt it out.  Laughing.

 

 

The Story sounds so entirely fictional.

 

 

NY Times Bestseller wanna be fantasy.  Jodi Picoult don’t you dare get any ideas!

 

 

Even if it does read like Hollywood B movie material.

 

 

I mean…we have The Bush Factor.  My husband and I, along with our two oldest kiddos, were missionaries once upon a time.  Trevy too – but he was just a baby bump at the time. A time that feels like another life altogether but was really just a handful of years ago.  We still carry on rusty Swahili conversations with each other just because we can.  And oh but do we have stories to tell.  Malaria in the middle of nowhere filled tales.  Tales that draw Romantic hearts to foreign countries.  Stories that are fuzzy around the edges now.  Because they happened a life time ago.

 

 

Before Trevor.

 

 

Well, really…

 

 

before seizures.

 

 

Not the seizures that most people are familiar with.  Not the kind Hollywood is embracing lately.  Not the kind your favorite Aunt Sally has.  Unless she has the catastrophic kind.

 

 

I’m not trying to offend or diminish controllable epilepsy.  Any life with seizures brings with it stress.

 

 

But catastrophic epilepsy is another breed altogether.

 

 

We’re talking seizures on steroids.

 

 

Seizures that you don’t ever want to know exist.  And when you do, you hug your healthy children that much the tighter.  And if you already do, your heart is knitting with each word you read.

 

 

At seven months old Trevor had his first cluster of Infantile Spasms.

 

 

 

 

We were literally two weeks from returning to Tanzania.  Luggage half packed.  Vaccinations and passports - all systems go.  In fact, Jonathan was in Delaware loading a container with supplies we intended to use during our second term as (what we believed would be) career missionaries.  I stayed behind in Rhode Island with my parents.  Schlepping three rugrats across several states just didn’t sound fun.  If you’ve ever driven I-95 through NYC - you get it.  Also, I needed to take the kids for their flu shots and show the pediatrician these weird movements Trevor kept doing.  Jonathan was the brilliant one – he suggested I take that video clip the next time an “episode” happened.  I had tried verbally explaining what I was seeing – the doctor thought it was reflux.  A common misdiagnosis.  He told me to try a soy based formula.  Which, of course, did not help.  Trevor was becoming increasingly cranky during the day and sleeplessly irate at night.  Symptoms.  The first cluster we noticed was on a Monday.  By Wednesday I was a basket case.  I knew something was wrong – just not what.  How in a million years could I have known?

 

 

It took three phone calls to the pediatrician.  The first two were toned down because I didn’t want to sound like a crazy mommy.  The third time – the mirage no longer mattered  – I told the doctor he needed to see me now.  That I’m not a new mom and something is desperately wrong.

 

 

He agreed to see me after hours.

 

 

I could tell.  The way the lines on his face shifted as he watched the video clip.  He asked if he could show it to a fellow doctor because in his 20 years of practice he’d never diagnosed a child with what he was suspecting.  My stomach was in my toes.  Sometimes I try and picture myself sitting there.  All pins and needles.  And innocence.  I was such a different girl then.  Less serious than I am now.  More carefree.  Not as cranky.  Skinnier too.  I knew something was wrong – but I never imagined just how much our life was about to change.  Never.  Because until you face them, you can’t imagine the monsters hiding behind the name tag: Rare Disease.  When he came back in the room I teased,

 

 

“Just promise me it’s not Tourettes!” 

 

 

His smile was probably tighter than I remember noticing when he assured me it wasn’t Tourettes.

 

 

“Well then, promise me I’m not missing my flight back to Africa in two weeks.  Because as you can see, I really need to work on my tan…”

 

 

Humor is my nervous tic.

 

 

    The rain was cats and dogs (I warned you – B movie material).  It was late – six or seven.  When I called Jonathan.  He sounded busy and distracted because he was.  There is an art and madness to loading a container.  Making use of every crevice.  I was barely holding back the flood when I asked him…

 

 

“Do you still believe God has a Plan in all things?”

 

 

I knew what his response would be even as I told him he needed to come home.  Now. 

 

 

And he did.  He left the packing to others and drove through the rain.  Not stopping until he was with me.  It was sometime after midnight before we were holding each other.  I had already surfed more medical speak than any parent ever wants to know could sneak up and engulf their child.

 

 

Those beginning weeks are blurry now.  So much life has flowed since…eroding away bits of the past.  I know Trevor had an EEG which confirmed the diagnosis.  I know our dreams of bronzing away our life in Africa were shattered. 

 

 

(this was actually after he’d started treatment – note the ACTH puffy cheeks - but was the earliest EEG picture I have)

 

I also remember our insurance denied Trevor the frontline treatment.  There are only a few and ACTH was the most promising.  A handful of months before Trevy was diagnosed, a company (Questcor) who had not developed but had purchased the drug (Acthar) was successfully given the go-ahead to change the price point by utilizing the Orphan Drug Act.

 

 

Overnight the price increased from approximately $1,500 (just put in on my credit card) per vial to $23,000 (now we’re talking a home mortgage) per vial.

 

 

Which is another story altogether - I testified against Questcor before the Joint Economic Committee the summer of 2008.

 

 

Trevor’s course recommendation was for 5 vials.  Possibly more.

 

 

We spent over a week fighting with our insurance company for coverage.  At one point they even stopped shipment.  They also threatened to not cover any costs incurred from side effects should we move forward without approval.  It was hell.  Emotional hades.  Absolutely gutted and helplessly holding our broken, seizure saturated infant.  Knowing that each seizure was stealing his cognitive and physical development.  Unable to access the drug which the doctors were telling us could possibly save his life. 

 

 

We were a wreck.

 

 

Ultimately, and after hundreds of unnecessary seizures and emotional turmoil, we finally had the treatment in hand.  Liquid Gold, Jonathan called it.  I thought Hope in a Syringe.  Acthar is a steroid gel which is injected into muscle mass – typically the thigh.  I delegated the needle handling to Jonathan.  Because I’m a wimp.  He always said he was more afraid of dropping the $5,000 syringe than actually giving the injection. 

 

 

(in the small boxes up front are two 5ml vials)

 

There is no describing how it feels to swaddle your baby as tight as you can.  Leaving one little thigh exposed before laying your body over his bitty one.  The goal being - eliminate as much of the struggle as possible.  I would close my eyes and hold my breath until I heard the scream.  The needle is so very big.  And the gel so very thick.  Jonathan says it took more force than he was prepared to use.  It goes against every paternal instinct.  Your heart hardens over time…but that first injection had everyone (home health nurse included) in tears.  We had to give him one injection daily for 5 weeks. 

 

 

Four shots in, Trevor was seizure free.

 

 

He did experience some known side effects including crankies, weight gain…

 

 

(he earned his nick name Heavy Trevy)

 

along with high blood pressure which required medication to keep controlled.  Try explaining to the pharmacist why your 7 month old needs Enalapril.

 

 

But he was seizure free.  The benefit outweighed the risk.

 

 

I wish that were the end of the story.

 

 

We did go on to have several months of seizure freedom.  During those months Trevor continued to make developmental progress in spite of his abnormal EEG.  Which remained intermittently spikey over the left side.  I never had a peace settle over me that our journey was over.  I always had this hunted feeling.  Like we were being stalked by the Seizure Monster.  But our neurologists insisted he was on a positive path – so I set my heart towards trying to believe them.  Speech was our biggest tangible concern back then.  He never seemed to respond to language – Early Intervention assured us speech delay was normal with boys.  Of course, Trevor was my 3rd child so I had some experience with Language Development.  Toby and Bristel were early bloomers.  Both speaking in sentences before they were 2.  I wasn’t convinced Trevor was just going to be my pokey talker.

 

 

I was right.

 

 

The majority of his subclinical seizure activity was occurring throughout his left hemisphere but appeared to primarily be originating in the temporal lobe region.  The left hemisphere is responsible for speech.  When he was 13 months old, a Boston Children’s neurologist brought up the topic of removing his left temporal lobe if speech continued to be significantly delayed.  He said as long as the “broken brain” was in there it would try to do the job and would never be successful.  If we removed it – it would allow the miracle of rewiring to the healthy right brain to occur.  The younger the patient – the better the outcome.  The idea of brain surgery wasn’t new to me.  I poured hours into research and various support forums and had already learned of several IS children who’d had portions of their brain removed.  Many of them in Detroit with Dr. Chugani (whom I fondly call Dr. Rockstar).  It was radical.  But so is catastrophic epilepsy.

 

 

When he was 17 months old our worst nightmare became reality -

 

 

his Infantile Spasms relapsed.   

 

 

 

 

The next year plus some was immersed in seizures and med trials.  Testing this.  Trying that.  Cocktailing these.  Titering those.  Nothing worked.  His EEG was no longer only spikey over the left brain.  It was a generalized (involving both sides of the brain) hypsarrhythmia (specific EEG pattern associated with Infantile Spasms) mess.  The spiking on the screen looked like a toddler scored a box of crayons.

 

 

(EEG with hypsarrhythmia)

 

When it should have been closer to this…

 

 

(healthy normal EEG of a baby)

 

 

All that disorganized chaos reflected the electrical storm waging under the crop of curls and super cute face.  So many seizures.  Hundreds of uncountable seizures.  Every single day.  From the time he woke up until the time he went to sleep.  He was a walking seizure.  He would cluster all day long.  Each cluster could last from 5 – 30 minutes and involve as many as hundreds of back to back seizures.  I stopped trying to track them.  It was exhausting.  Yet rest did not bring him respite.  Infantile Spasms is most aggressive when the brain tries to rest.  Sleep is so important to growing babies because this is when the brain takes all the information it has processed throughout the day and organizes it.  Learning cements during sleep.  This aggressive increase in seizure activity during that precious learning time arrests development.  And causes developmental regression.  A growing brain cannot sustain perpetual insult without resulting in arrest and eventually loss of function.  We watched Trevor’s global development slip from a month-ish behind his peers to significant delay.  When he was 28 months old, an evaluation placed him globally functioning around 10 months.  His body was growing bigger but he was developmentally frozen at the point his spasms relapsed.

 

 

We had exhausted medications.  Sure there were more to try.  But the likelihood of being effective was nil to none.

 

 

We had reached the desperation point.

 

 

Which I guess, is where you need to be when you finally decide to passionately pursue surgical intervention.  I had flirted with Dr. Chugani in the past and did so again.  Because I needed Hope.  He is the rare breed of doctor who cares enough to offer himself via email to parents of children with catastrophic epilepsy.  There are many, many parents whose children are not his patients and yet he takes the time to answer their questions.  Offer guidance.  On this very dark, murky road.  Jonathan’s dad passed away from Pancreatic Cancer during this time.  My parents kept the kids so we could attend the funeral together.  It was shortly after the ceremony that my cell phone vibrated.  Chugani was on the other end.  And I can’t deny, it felt like a sign.  I was already eyes weepy and hands shaky as he expressed feeling that Trevor could be a surgical candidate. Based on my shared data and the single test (a PET scan) he studied for me pro bono.   Having not actually preformed any testing himself – it was just his guess. 

 

 

But it was enough.  To get my hopes up.

 

 

It’s just that Detroit is an awfully long way from Rhode Island.

 

 

So we decided to pursue surgery locally first.  The best choice was Boston Children’s.

 

 

Where we were sorely disappointed.  The neurologist we were referred to wouldn’t even present Trevor’s case to the surgical board.  He said his seizures were generalized and that his EEG was trending towards Lennox-Gastaut Syndrome (LGS).  This was the same guy who once mentioned removing a lobe.  But that was before the relapse.  Now, he was telling us the most we could hope for was some form of seizure control but that Trevor would never be seizure free and would likely never talk.

 

 

It’s not even that we were surprised.  Or that any of the information was new. 

 

 

It’s just that the amount of emotional energy it takes just to survive these meetings is astronomical.  All of the hope we’d poured into pushing for surgery – dashed – just like that.  Appointments like these have a tendency to leave me little more than a puddle on the floor.

 

 

But that hope.  Dr. Chugani had breathed that hope in my heart.  And I knew I had to pick up the shattered pieces and move forward.  I knew I didn’t want to waste any more time.  “The younger they are – the better the outcome” echoed inside my head at all times.  Which meant it was time to get serious and schedule an appointment with Chugani himself.  I also knew it wasn’t going to be easy to convince our insurance to approve this way out of state & network facility and neurologist.  Especially, when our local hospital clearly disagreed.  And in fact, Trevor’s primary neurologist quietly referred us (meaning, she didn’t run it by her superiors) to prevent any further delays or barriers. 

 

 

And it was no easy feat.  I lost track of the hours I spent on the phone.  The number of letters written.  Arguments made.  Sleep lost.  Pounds gained.  Tears shed.

 

 

Somewhere over those months my passion for surgery morphed.  In the beginning, it was entirely (selfishly) about the cognitive benefits it could bring him.  We had already watched him make developmental progress before - when the seizures were controlled.  But as time flowed on,  it became more about giving him a better quality of life.  I would have given anything to stop those damn seizures from attacking him.  Saturating him.  Slowly stealing him way. 

 

 

And after months of advocating…our insurance agreed to let us pursue surgery with Dr. Chugani’s Dream Team.

 

 

Toby’s (my oldest) baseball coach donated his air & hotel points for our surgical consult flight to Detroit.  I won’t go into all the gory details…but trust me…the trip was filled with B-movie drama of it’s own including severe weather almost causing us to miss our connection!  I can’t resist sharing one particular incident.  We had successfully secured seats on THE LAST flight from Philly to Detroit and some yahoos were trying to guilt trip us into giving them up.  “But we’re headed to a funeral”, they said.  “We’re en route to see if a neurosurgeon will agree to remove half of this beautiful little two year old boy’s brain as a radical last measure to control the hundreds of seizures he survives on a daily basis”, we responded.  They moved along and we made it to Detroit late that night.  We returned home even more weary and promise-less.  Later learning there was much debate over Trevor’s case when the surgical board met.  Because Trevor has no tangible findings on his MRI along with his generalized (meaning seizures occurring in both hemispheres of the brain) EEG – it was not a clear cut decision.  Dr. Chugani argued his case – and finally persuaded the team.  He would later tell us during post-op rounds that he couldn’t let a child continue to have 100s of daily seizures if there was even a small chance.

 

 

August 17th, 2009 was the day we learned that Trevor was a candidate for radical brain surgery (a left subtotal hemispherectomy with MST to motor cortex) with Team Detroit.

 

 

I’m not superstitious or anything, but I had stopped cutting Trevy’s hair when we made the decision to chase surgical intervention.  Over the months he’d grown a mop of beautiful surfer’s curls. 

 

 

(snapped on the day we were waiting to hear about the Surgical Team’s decision)

 

Just a handful of weeks after our consult appointment, the OR nurses took turns running their fingers through his beautiful surfer curls before shaving most of them off.  And sweetly saved a baggie for us.

 

 

There are so many details and emotions to share about those Nightmare Miracle days…weeks.  Too many, really.

 

 

  (our first cuddle after his Hemi)

 

A friend recently asked me how we made the decision to have half of his brain removed.

 

 

I don’t know that I can adequately answer that question.  At least not in a way that someone who hasn’t walked this road would be able to wrap her heart around.  Certainly, everything I’ve shared already played a role in leading us towards surgery.  It was truly the final resort (though, I personally believe neurologists treating catastrophic epilepsies should consider surgery more aggressively – rather than forcing willing but emotionally exhausted parents to find the strength to push for it).  We were out of viable options.  If surgery wasn’t on the table…our story would be different.  But it was.  And Jonathan and I had made a promise together.  During those early days just after we heard the words Infantile Spasms for the first time.  We held each other and promised that we would always choose the most aggressive treatments, no matter how scary, to give Trevor the best chance.

 

 

That doesn’t mean we have an extra supernatural dose of courage.  Or are immune to deep, bone shaking fear.

 

 

The same fear that quaked us to the core when we gave him that first injection of Acthar quaked us to the core again when we kissed him goodbye.  And as we held each other weeping and watching his bed roll away to the surgical suite.

 

 

(the actual scan of Trevy’s brain post surgery – obviously words added by mommy)

 

Of course, we’re on the Healing Side now.  As I like to call it.  The surgery is two years behind.  So are Infantile Spasms.  We have watched the Trevy we knew was locked underneath those seizures blossom these past two years.  It’s easier to smile now and relax into knowing we made the right choice.  For Trevy.  Of course, he’s only just about to turn five which means our journey is far from over.  I’m still advocating hard for him.  It’s still exhausting.  I’m still researching and pushing.  I can still be found stress eating.  He still has epilepsy.  Though thankfully more responsive to medications.  We’ve walked down the road a little bit further now.  Some of the “what ifs” have been answered.  Some of the drama already lived. 

 

 

People love the idea that he’s such a Miracle.

 

 

I love knowing that he has been all along… 

 

 

…danielle