Reality Check

Today is the 1st Annual World Moyamoya Day to help raise awareness about this rare (and pretty scary) disease and to celebrate its survivors.

Peter and I were talking last night about Claire, her diagnosis and prognosis, and what the future holds for her.  I had to clear up some misconceptions he had... and thought I would take the opportunity today to do the same for all of you, dear Readers.

1.  Claire is not cured.

This seems to be the biggest misconception.  Claire’s surgery did not miraculously “fix” her.  Claire’s surgery was an attempt to provide the means for her body to fix itself.  Because Claire is a pediatric patient and her vessels are not big enough for a full bypass procedure -- Claire had an indirect surgery, which should allow for her body to create new blood supply to the affected areas of her brain over a period of time.

2. Over a period of time...

That’s a very important point there.  It will be six months to two years before these new blood supplies develop in Claire’s brain.  June 7 will be Claire’s six month anniversary and June 12 she will have a procedure to determine how things are progressing.  During this period of time, Claire is still at risk for stroke, seizure, TIA, and other scary brain things.

3.  How at risk?

Well... high risk.  Very high.  Claire had at least three strokes before her surgery.  I say “at least” because all the strokes happened in so-called quiet areas of her brain and she had no outward signs of them.  So three strokes show clearly on some of the many brain scans Claire has had done, and two more are questionable.  So, our fearless warrior child could have had as many as five strokes before she had surgery.

Claire also recently had a trans ischemic attack (TIA or “mini-stroke”).  During a TIA, a blockage occurs, disrupting the flow of blood to the brain.  A rare complication of a TIA is a brain hemorrhage -- which can be fatal.

Claire can, and likely will continue to have “episodes” while her brain is healing itself and developing new blood flow.  No one knows what the probability is -- this disease is so rare, not much is known about it at all.  And Claire is an anomaly in so many ways that she’s even harder to diagnose.

4.  Claire is not the same as she was.

Because Claire looks the same, and mostly acts the same, and because I treat her the same as “before” -- people assume she is the same.  She isn’t.  This diagnosis and her treatment have caused major changes in who and what Claire is and will be.  She is not worse -- she is simply a different Claire now.  I’m not asking anyone to treat her different (as I said -- I don’t) but I would like it if people would understand that she’s been through a major trauma and it has affected her. It will continue to affect her.  And we are all doing our best to deal with that.  Claire requires a bit more patience than “before”.  So does her mother... and her brother... and others who love her.  

5.  What does this all mean?

I hate answering this question.  HATE.  It’s scary and I don’t like to talk about.  I’m not sailing away on the Good Ship Lollipop wearing my rose-colored glasses when it comes to Claire’s prognosis -- but I also don’t particularly enjoy thinking about or talking about the death of children.  Any children.  Especially my child.  I prefer people digest the facts themselves and come to their own conclusions, or Google it.  But, that’s not been working out so well (see Exhibit 1. Peter) so... here goes.

Claire could die from this.

There. I said it.  She could die. Of course, she could get run over by a runaway Dial-A-Ride bus too -- there are no guarantees about how long we get to bless this world with our presence. But there it is. Claire could have a massive stroke today, tomorrow, or (hopefully) never.  The surgery may be a success, or it may not.  This could develop on the other side of her brain, or it might not.  We. Just. Don’t. Know.  I’ve been told a lot of scary facts, and figures, and I don’t like to talk about them. Or even particularly think about them to be honest.  No one does.  

You don’t want to come up to me in the grocery store and ask, “How’s Claire?” and have me respond with, “Well, there’s an upwards of 67% chance that she could have a massive stroke and die within the next six months.”  What’s the proper response to that?  “Ummm... I think I read somewhere that carrots are good brain food and I think they’re on sale this week.”?

Nope.  That’s not a conversation I want to have with anyone. Ever. So instead I focus on the ups and try to mitigate the downs.  

6.  So what are you going to do?

We are going to do what our neurosurgeon suggested we do... Live.  Claire’s life has been forever changed but we are still going to enjoy and celebrate that life as it is now.  We are going to celebrate the small victories (staying on honor roll despite all she’s been through) and the large ones (surviving a TIA).  She is still going to have to clean her room and eat her vegetables.  We are not going to mourn all the things she can’t do (ride a roller coaster, go on a water slide) but instead choose to enjoy the things she still can (ride a bike, swim).  For however long we can, we are going to live... and hopefully, hopefully, live well.

And we are going to try... TRY to listen to what Bob Marley said:

“In every life we have some trouble.  When you worry, you make it double.  Don't worry. Be happy.”

Worst Case Scenario

A friend of mine recently wrote, "Sometimes I think that we should have a special warning system for bad news when it comes. A different ring tone, a blinking light - something that says take a breath before you pick up the phone. Sit down before you speak a hello."

She's right.

This past August, Claire suffered an excruciating headache on the way home from visiting her dad in Minnesota.  She was in terrible pain for over eight hours.  As Quinn and I both suffer from migraines (I started getting them at eight years old, as did Quinn.  Claire is currently nine), I assumed she was following the family path as well.  The only curious thing was that her "migraine" lasted so long and was very different from how mine, and her brother's, are -- but I discounted that with a simple, "every headache is different."

Two weeks later when Claire got another headache, I was a little more concerned.  I decided to start keeping a headache diary for her.  Two days after that, when she got another headache, I took her to the local ER.  The doctor noted that she had no sensitivity to light or sound, was showing no neurological distress, and had no fever and therefore sent us home.  Two days after that, the day before we had our follow-up appointment with the pediatrician, Claire suffered another headache.

When we met with the pediatrician, I explained how I suspected Claire may be getting migraines, but mentioned that her symptoms were completely unlike mine or Quinn's.  Fortunately (although I didn't know at the time how fortunately), my pediatrician recommended that Claire get an MRI done.

A week later we received a phone call to come into the office to discuss the results.  Me, being the worrier that I am, was immediately concerned.  If everything was okay, why wouldn't they just say so? So, obviously (to me) everything was NOT okay, and that's why there were calling me in.

The morning of Claire's appointment I received a phone call:  The doctor was sick and we needed to reschedule the appointment for later in the week.  Trying to keep the hysteria out of my voice I indicated that I did not want to wait more than one day to find out the results.  They kindly agreed to squeeze me in the next day.

Peter and I were sitting in the office, looking at this photograph of a group of what must be the ugliest babies I have ever seen.  I'm staring at these children and this horrible thought popped into my head. The kind of thought you wish you could unthink because it feels like you are tempting the fates to even think it...

"This is the moment before our whole lives change forever..."

Moments later, Claire's doctor walks in, sits down, and gives us the news: "Claire has lesions on her brain."

Lesions. On her brain.  This can't be good.

For the next few moments I could only hear a buzzing sound as blood whooshed through my ears.

Lesions. Leeeesions. LeEsiOns.

I heard the doctor say that it was probably nothing to worry about, but that she was referring us to DeVos Children's Hospital to "get things checked out."  I heard the words "demyelization" and "MS".  And lesions. On. Her. Brain.

We had to wait a month before we could get in to see the pediatric neurologist at Helen DeVos Children's Hospital (HDVCH).  It was a painfully long month, full of me attempting to act normal, but hearing the echo in my head near constantly...  Lesions. Lesions. Lesions.

Our appointment was scheduled for Monday, October 22nd.  We arrived at the neurology department bright and early -- only to be told that, due to a schedule snafu, they had canceled my appointment.

I cried.  For those who know me best, you know this phenomenon is one of the most frightening to witness. I. Don't. Cry.  I hate crying. Abhor it. Believe it is a sign of sissies and weakness.  I am intolerant, bigoted, prejudiced and hateful towards tears.  It is simply not done.

And I sat in the neurology scheduling office and cried for a half hour.  Peter was frantic. Claire was terrified.  Mama crying is a very bad thing.  The stress of a month's worth of hearing that echo in my head had gotten to me. I had tried being stoic.  I had tried being funny - going so far as to make comments that Claire would definitely survive a zombie apocalypse as zombies probably don't like the taste of lesion-y brains.  But I had passed the point of no return -- I was losing it. Lesions. On. Her. Brain.

Tonya, the Special Processing person, went above and beyond the call of duty by getting us overnight accommodations at Ronald McDonald house and worked her tail off to get us a 7:45 appointment the next day.

We met with the chief of pediatric neurology that day, Dr. Stephen DeRoos.  He was amazing with Claire.  He was kind, friendly, approachable, and thorough.  He ran Claire through a series of neurological tests... squeeze his hands, run through the halls, look at the light... those kinds of things.  He had me run through the series of events that led to us being there.  Then he got down to brass tacks - lesions.

He didn't think they were that big of a deal.  Apparently, it's somewhat common - these lesion-y things showing up on an MRI.  She was too young to have MS and she wasn't showing any symptoms of, well, anything.

Whew! Yay! Hurrah!  Everything is okay!!

Well... no.  Because the MRI uncovered that Claire had more blood vessels on one side of her brain vs. the other.  Okaaaaay... what does that mean?

The doctor didn't know without further testing.  It could be this, it could be that, "it could be something called Moyamoya disease but that's really rare and unlikely and we're getting ahead of ourselves..."

We left the hospital with a diagnosis of "intracranial vascular disease", an order for another MRI/MRA, and the word "Moyamoya" now echoing through my brain, replacing lesions.  I googled intracranial vascular disease before we left the hospital parking lot.  Okay... this isn't good.  But the prognosis is excellent and... okay. We'll be okay.  It's not great news but... Claire is going to be okay.

I then googled Moyamoya, looked over the information for about 30 seconds, then promptly shut the browser down so I didn't scare myself to death.  "That's worst case scenario - not gonna happen."

Claire went back to DeVos this past Sunday to get her MRI/MRA done.  She enjoys this particular hospital - they go above and beyond to make it kid friendly.  The gift shop is phenomenal, she adores the garden, and the staff is exemplary.  We are so very lucky to be so near this amazing medical facility.

The hospital really needs better pjs

After Claire's testing was complete, the tech told us to expect to hear from the doctor within three to five days.  I left feeling like if it were really bad news, they wouldn't be letting us go.  Good enough.

At 4:30 yesterday afternoon - the next day, I wish I would have had that warning bell, that blinking light, that... something... that would have told me to take a moment before I picked up the phone. Take a breath. Picture something happy.

Claire has Moyamoya disease.  Our lives did change forever in that moment.

One the rarest forms of occlusive cerebrovascular disorders encountered in neurosurgery is Moyamoya disease. Fragile blood vessels proliferate around a blocked artery in an attempt to bypass an occlusion and their appearance on a cerebral angiogram resembles a "puff of smoke" or "moyamoya," a term coined by a Japanese team who first described the disease. Symptoms are TIAs, strokes, headaches and seizures. There is currently no drug treatment that is effective for moyamoya disease and surgery is aimed at bypassing the blockage with another artery to restore normal blood flow.

Fuck.

My son was born with a ventricular septal defect, which was discovered at his 3 week check up.  He spent almost a week in the hospital at the age of four months, during which time I slept in the oxygen tented baby crib with him.  He's had more shots, and tests, and pokes, and prods, and surgeries, and stitches than your average twelve year old -- to the point that he used to give himself stress migraines just going to the pediatrician for check ups.

Claire was my "healthy" child.  Sure -- she has asthma -- but that's been controlled for over two years now.  She was the robust one, the picture of health.  Finding out I have one bad heart kid and one bad brain kid -- it's just too much. I'm on overload.

This... sucks.  And I'm not good at this.  I'm not patient or understanding or wait-and-see.  I'm manic and demanding and let's do something about this NOW.  But nothing good happens NOW in a health crisis.  Nothing.

I am so MAD. I am just... angry... and pissed... and terrified... and mad. I appreciate all the kind words, and thoughts, and prayers. But if God's plan is anything other than her growing up to have a fabulous life, a beautiful wedding to a wonderful man (or woman) who adores her, giving birth to perfect children (if she wants to), and being loved and adored and contented and happy 'til the ripe old age of 97... I don't want to hear about it. Fuck God's plan. She is my BABY and I don't want to plan whether or not I should bury her or cremate her or where we would spread her ashes and what to say at her funeral. No. Fuck No. She is my baby and she is going to have a LIFE. A fabulous wonderful perfect everything she ever wanted LIFE. God. Dammit.

She has a mother, and father, and brother, and grandfather, and almost stepfather, and aunts, and uncles, and cousins, and friends who LOVE her.  As my best friend said, "This is Claire.  CLAIRE.  The world needs that little nut."

So. Screw you, fates, and world, and Moyamoya.  We aren't going down without a fight.  We are strapping on a fabulous sparkly purple cape, gearing up, and heading in to battle.

Because when the zombies come, my little lesion-y brain kid is our ace in the hole.