When being extraordinary is a bad thing…

We’ve been mentally and emotionally preparing ourselves for Ellie’s first heart surgery.  We’ve been told how much of a difference the banding of the pulmonary arteries can make.  We’ve been preparing ourselves for how she’d look (which is always a hard thing) and for possible risks and such associated with the surgery.  We know that Ellie has plenty working against her here, and this whole time we’ve been so hopeful that the banding (and trach) would be the surgeries that would turn things around for her.  We’re still very hopeful of this, but our world has been rocked, for sure.

Yesterday morning, Dr. Morales (our surgeon) came into our CICU room to chat with me about Ellie’s upcoming surgery.  I made a list of questions and called B and my parents to see if they had any additional questions to add to the list so that I was astute and well prepared for this meeting.  This poor man has the impossible job of delivering hard facts and heartbreaking realities, though.  He also saves lives, by the grace of God… but this part of his job has to be awful.

He said that Ellie was an exceptional case… an exceptionally difficult case.  He reviewed that she’d be a 1-ventricle repair, but told me that single ventricle repairs were incredibly risky in kids with trisomy 21 (they always say trisomy 21 here instead of Down syndrome).  Because of some of Ellie’s physical characteristics and restrictions, a very complicated surgical journey becomes MUCH more complicated and more likely to fail.  Most of the worry he has concerns Ellie’s extremely high pulmonary vascular resistance (PVR), which is the high pressure in the arteries to her lungs.  He said that high PVR isn’t terribly uncommon in kids with Down syndrome, but the thickening of the arterial walls usually starts, at the earliest, around 10 months.  This thickening is usually driven by airway instability in other kiddos.  Ellie managed to thicken her arterial walls and restrict her blood flow between two and three months of age.  This is not something our surgeon had seen before.  He's one of the top surgeons in the world, and this is new.  Regarding her future surgeries, he said, “It doesn’t meant it can’t be done… and it doesn’t mean this has never been done… but I want you to know that the risk is much higher than normal here and the outcome is less likely to have the effects we want it to have”.

The only way to measure PVR is through a heart catheterization, so there was no way of knowing that we were dealing with this before she went in for the cath on December 3^rd.  Since then, the surgical and cardiac teams have been discussing her case to determine a reasonable course of action for Ellie.  From here, we know that banding is the first step.  If the banding isn’t wildly successful, Ellie doesn’t have other options for surgery.  She’s not eligible for the Glenn or Fontan procedures (which bypass her heart to dump her un-oxygenated blood into her pulmonary arteries).  She’s not eligible for a transplant, either.  If Ellie is not able to relax the pressure in her pulmonary arteries… she will have to live with the very broken heart she was born with.  The outlook down that road is very grim.  Unthinkably grim.  My-heart-has-been-ripped-out-and-stomped-on kind of grim.  Ugh.

So what I’m saying here… this banding HAS to be the answer.  There’s no other option.  By the grace of God, this little girl HAS to respond perfectly to this procedure in order to have any hope for a future.  Heavy stuff, I know.  Now, to the nitty gritty of the procedure…

The surgery is set to start at 8am tomorrow morning.  The surgery itself only takes 2-3 hours, but with prep (about an hour and a half) and recovery (about 2 hours), it’ll likely be 7 hours or so between the time we kiss her in the OR and the time we see her again back up in the CICU.  We will get updates through the day.  This surgery, itself, isn’t incredibly risky because they’re not cutting into heart tissue.  But any time they open up a sternum, there is risk, of course.  During the prep period, they’ll put in a central line (an IV like tube that goes in her neck and down to her heart to measure pressures and such).  They’ll also put in an arterial line in her arm (another tube that taps into an artery in her arm and constantly and accurately measures blood pressure).  They'll remove her trach and intubate her (hate this).  She’ll be getting chest tubes to help drain off excess fluids, too.  She'll be extubated within the first 24 hours and her trach will be put back in.  The chest tubes will come out two or three days post-op.  The central and arterial lines will stay in until she leaves the CICU.  The band itself will be adjusted in the OR to restrict Ellie’s pulmonary arteries enough to relieve some pressure, but not so much that she’s blue.  We were warned that she may be pretty blue after surgery.  There is an acceptable level of blueness here… and they’ll have to adjust the band so that she’s not SO blue that it causes damage to her brain and organs and other cells.  Eeeek!  I asked about the material that the band is made of and how it grows with Ellie.  Dr. Morales said, “It doesn’t.  It’ll need to be readjusted as she grows.”

After surgery, she’ll need to recover in the CICU for a week or two until she balances out her vitals.  Once stable, we’ll be transferred to the TCC (transitional care center) so that we can start our intense trach training.  Dr. Morales thinks we’ll be able to go home with her after we’ve put in our time in the TCC (likely 6-8 weeks).  Then… it’s a waiting game.  We’ll be watching for signs that the PA band is doing its job (still need to get a grasp of what those signs are), and she’ll be back to get heart catheterizations again to see if her PVR has dropped.  If this happens (and we pray to God it does… and does so relatively quickly), then we can move on to surgical options.

Dr. Morales and the current attending cardiologist (didn’t catch his name, but he’s British) both talked to me about how incredibly risky the following surgeries will be for Ellie.  Because she was such an over achiever in protecting her lungs, giving her high PVR, and because she has a nice little trifecta of risks (Down syndrome, high PVR, unstable airway), the risks involved in the next surgeries are much higher than normal.  They so kindly said, the risk of complications here (including mortality) is quite high.  The risk of not doing any surgeries is absolutely fatal… we have no idea how long her heart will hold out with just the banding.  To reinforce the importance of the PA band being successful... if it does not lower the PVR, she's not eligible for a Glenn, she's not eligible for a Fontan, she's not eligible for a transplant.

So our baby girl has to be strong… has to respond to the banding… and by golly, has to stop trying to be so darn extraordinary.  I do NOT like hearing a world class surgeon say, “she’s an exceptionally complex case”.  This is NOT where we want to be exceptional, Ellie.  This is not where we want to pave new ground.  This is where we want to be ordinary and run-of-the-mill.  Let’s stop being extraordinary for a while, okay??

Pray for our baby girl.  Pray for God to lead the surgeon’s hand.  Pray for a miracle.  Pray for strength for her and for us.  We can’t walk this road alone, we need prayer.  We need a miracle… and I know those happen every single day.  Let’s let the last day of 2014 be the day that changes everything (for the better) for our sweet girl.  Let this make all the difference and let 2015 be a year full of hope and promise for Ellie.  By golly, this little fighter deserves a break!