Ulan Ude; Dealing with the Unimaginable

I recently purchased the Hamilton musical.  I've never been a fan of rap, but the music resonates with me strongly.  Bonus, the boys ask lots of questions about the American Revolution.  But then there's this:

"It's Quiet Uptown" is a song from the Broadway musical Hamilton performed by Renée Elise Goldsberry (Angelica), Lin-Manuel Miranda (Hamilton), Phillipa Soo (Eliza).

ANGELICA:

There are moments that the words don’t reach

There is suffering too terrible to name

You hold your child as tight as you can

And push away the unimaginable

The moments when you’re in so deep

It feels easier to just swim down

ANGELIC/ENSEMBLE:
The Hamiltons move uptown
And learn to live with the unimaginable

My child is not dead.

My child does not have cancer.

My child is not recovering from a catastrophic car accident.

I started crying when I first heard this song.  I am living the unimaginable.  Dr. Ahn had called to tell me the results of the weekly vascular neurosurgery conference.  And instead of giving me a timeline for gamma knife surgery followed by a Chiari decompression, he told me they felt it was better to operate and microsurgically dissect the AVM.  The team was concerned his headaches were getting worse, they felt he needed a Chiari decompression in order to alleviate the headaches, but they were worried opening the skull without first removing the AVM was too risky.  It could lead the AVM to rupture.  Gamma knife surgery takes 2-3 YEARS to resolve the abnormal blood vessels, it doubles the risk of rupture during the first year, and it carries some of the same complication risks for surrounding healthy brain tissue as old-fashioned surgery.  So, Dr. Rafael Tamargo, the resident AVM expert at Johns Hopkins and Director of Cerebrovascular Neurosurgery, will take the lead and dissect out Christopher's AVM.  Also of note:  his AVM is straddling the central sulcus line, with the nidus in the sensory cortex, not the motor strip.  So while there is some risk of temporary damage to his left leg and even his whole left side, it isn't the catastrophic damage they originally thought operating in that region could cause.

Image 1--The tangled ball of yarn at the top center is Christopher's arteriovenous malformation.  You can see that it has two main feeder arteries and that it clearly looks different than the branching vessels around it.

I wasn't thrilled the first time we were told Christopher might need skull base surgery, but I'd been mollified with the thought that it was "the most routine of all possible surgeries". And really, it just barely qualifies as brain surgery.  The idea is that the surgeon widens the skull and C1 where they pinch the brain, and then opens the dura, adding an extra large patch so that cerebrospinal fluid can flow freely again.  The brain itself usually isn't touched.  (Reference this post for more details.)  That made it seem like a reasonable approach, especially if amitriptyline couldn't control his headaches.  At least, it was better than asking a 9 year old to live the life of an invalid.

This is different.  This is bona fide, crack your skull open and extirpate brain tissue, sit in an OR waiting room all day while your child's brain is open on a table, brain surgery.  I've always been taught that the brain is a sealed system, something we shouldn't open.  And now they want to open it twice.

Figure 1--In an AVM resection, vascular neurosurgeons, working with a stereoscope,  use specialized instruments to first close off and detach the feeder arteries.  Image Courtesy of The Aneurysm and AVM Foundation.

Figure 2--In the next stage of surgery, the core of the AVM, the nidus, is removed, along with any brain tissue it encloses.  Then the draining veins are closed off in the final step.  Image Courtesy of The Aneurysm and AVM Foundation.

For more details on the procedure, visit The Aneurism and AVM Foundation.

But the alternative is to wait until the AVM ruptures.  At a 2.5% annual risk, 25% over a 15-year period, it will almost certainly rupture in his lifetime.  The rule of thumb is 

105 - age at diagnosis = 96% 

for Christopher.  One-third of rupture patients die before they reach the hospital.  The remainder vary between those with the worst headache of their life and permanent, debilitating loss of neurological function.  As much as I hate the idea of a craniotomy and microsurgery, I am rational enough to appreciate the superiority of a calculated preventative procedure to an emergent, all-hands-on-deck trauma. 

My unimaginable may not be as terrible as the worst case scenario, and two weeks later, I have learned to live with it, thanks largely to my incredible support network of friends and family.

So instead of focusing on the unimaginable, I try to focus on this rallying cry for Christopher instead, cheesy though he thinks it is:

“Fight Song” by Rachel Platten 

Like a small boat

On the ocean

Sending big waves

Into motion

Like how a single word

Can make a heart open

I might only have one match

But I can make an explosion

And all those things I didn't say

Wrecking balls inside my brain

I will scream them loud tonight

Can you hear my voice this time?

This is my fight song

Take back my life song

Prove I'm alright song

My power's turned on

Starting right now I'll be strong

I'll play my fight song

And I don't really care if nobody else believes

'Cause I've still got a lot of fight left in me