All of Me...

Ellie had the video swallow test and the upper GI test done today where they push barium through her NG tube to make sure that her upper GI tract is functioning and structured properly… it is!  Then they had her drink the barium mixture through a bottle to see if she was able to swallow without aspirating.  With a preemie nipple on the bottle, she had great success.  If we stepped up even to a level 1 (used by most newborns), Ellie couldn’t handle the bolus and a little trickled into her trachea.  So… preemie nipple it is for her! 

After the study, I talked in length to the nurses, GI docs, speech pathologists, ENT docs, general surgeons and cardiology folks about our sweet bug.  Then I sat alone with my thoughts for a while, jotting notes and doing a little research on my own.  Before you ask, I only look at medical journals and reputable sources (.edu and .org websites… never .com ones).  I was inundated with facts, charts, graphs, you name it.  And if you know me and what I do for a living, you know that these kinds of things speak to me.  First, I looked at single ventricle congenital heart defects and learned that they’re so incredibly rare (I knew this).  The chance of having a baby with a hypoplastic right heart (which we now know Ellie has) is 5 in 100,000 births.  The chance of having a baby with Down syndrome is about 1 in 700.  The chance of your baby having autonomic storms that are not related to a brain injury… there aren’t numbers on that (at least not that I could find) because almost all autonomic storming is related to traumatic brain injuries.  So if we just factor in Down syndrome (1:700), plus the chance of having a baby with hypoplastic right heart (5:100,000), we had a 7 in 1,000,000 chance of getting both.  That’s not taking into account the odds of having a baby with an AV canal defect or any of the other “surprises” she’s brought forth.  With those odds (if we were gambling folks), we should be playing the lottery, right? 

I didn’t know that an underdeveloped right ventricle was much rarer than an underdeveloped left ventricle (as in, 95% of hypoplastic heart defects impact the left side… only about 5% impact the right).  Both are the most complex forms of heart defects and both are the most complex to fix, surgically.  The procedures have to be done in a series of steps (so if we go with the plan they currently have for Ellie, she’ll end up having at least 3 open-heart surgeries).  The procedures have been around for about 40 years.  I haven’t learned yet, however, that when you come across the words “average life expectancy post-op” that I need to STOP reading.  That number is scary.  REALLY SCARY.  However, I have to take into account that these procedures have only been around for 40 years (only is relative, right?) and that the data on survival rates is based on some of the earlier procedures, and they’ve come a LONG way.  The data shows that typically, patients live for 15-30 years after these procedures... more if they go for a heart transplant down the road.  That’s a horrifying statistic… but one that I know Miss Ellie is going to leave in the dust.  She’s going to blow by that no problem, she’s tough like that!

With the structure of Ellie’s heart, we have 3 surgical repair options:  1) reroute some of her deoxygenated blood, allowing her heart to function like it has 1.5 ventricles, 2) reroute all of her deoxygenated blood, allowing her heart to function like it has just the left ventricle, and 3) a heart transplant.  However, in Ellie’s current state, we have exactly zero options.  None of these surgical procedures will work unless Ellie has low pulmonary vascular resistance (low blood pressure in the arteries to her lungs).  Right now, Ellie has extremely high vascular resistance.  These procedures simply won’t work right now.  The hope is that after she gets a tracheostomy this week, her vascular resistance will go down and the constriction of her arteries will relax.  Once that happens and it is confirmed with another heart cath (in 3-4 weeks), they’ll be able to put a band around her pulmonary artery, thus mechanically restricting the blood flow.  Ellie’s body is actually restricting flow on its own right now (a protective measure the body does to keep from destroying her lungs but is rarely seen in a child as young as her).  If this procedure is successful, then we can proceed to surgically repair the heart.  If the procedure is not successful, the outlook is even worse.  Babies with this type of heart defect who are not eligible for surgery (where Ellie is now), have a life expectancy of just a few years.  I know… this sounds morbid… but this is what I talked to the docs about today and I know that I have to know what we’re up against… and pray, pray, pray that this is not the road we have to travel.

Typically, a baby born with an underdeveloped right ventricle (hypoplastic right heart) need to undergo surgery immediately after birth (or within a week).  Ellie is doing so well (again, a relative term) because of the way her heart developed (or rather, didn’t develop).  The only thing that is Ellie’s saving grace right now is that she is missing most of her septum.  This allows the left chambers to kind of accommodate the blood that the right ventricle is too small to hold.  Sometimes, it’s possible to kind of “grow” the right ventricle a bit.  This is why they reroute the blood from the superior vena cavas (this is plural because Ellie has 2 of these veins instead of 1) first.  This allows the blood from the inferior vena cava to still dump into the right ventricle and gives this ventricle a chance to grow and develop a little to maybe accommodate that blood volume.  This isn’t always possible, but it is something we can hope for.  This would result in the 1.5 ventricle repair I mentioned earlier.  Okay… I can process this and mentally prepare now.

The cardiologist then asked me if anyone had gone over the length of our stay.  I told him that I knew she’d be getting a g tube and a tracheostomy this week and that after those procedures; we’d need to spend about 5 days in the CICU, then go to the 3^rd floor to transition home.  He agreed, but said the CICU stay could be more like 7 days and that when we’re transferred to TCC (transitional care), we could be there for a month or more… easily 2 months if she needs ventilator support.  Whoa!  I wasn’t ready for that.  I said, “So what you’re telling me is that I need to go get a Christmas tree for our room, right?”  Ha!  We both chuckled a little bit.  Then I looked at Ellie and said, “you have an interesting way of turning an hour and a half appointment into several months in the hospital, young lady”.  He said… “Yes, you’re kind of in a Gilligan’s Island situation, right?”.  I sang, “A three hour tour… a three hour tour”.  And he said that an ideal situation for Ellie is that upon reevaluation with a heart cath in 3-4 weeks, we’ll find that she’s ready to start her surgical journey, which would mean much, MUCH more time here.  He said that if she does well (which is of course what we’re rooting for), we could start the surgeries and such after the heart cath… but that would extend our stay significantly.  That would make the stay 4-5 months (or more).  So yeah – we might as well set up residence in the Ronald McDonald house at this point (we do have a room there now).  We’ll be here for a while. 

In other news, I had followed a story of Matt Hammit several years ago (he’s a Christian artist for those of you who might not know).  About 3.5 years ago, he came out with a song called “All of Me” that he wrote while his wife was pregnant with their 3^rd child, Bowen.  Bowen would be born with a hypoplastic left heart (very similar to Ellie’s hypoplastic right heart) and they were told that he might not make it.  If he did, they’d have a long road of risky surgeries ahead.  Bowen underwent his 1^st open heart surgery when he was 5 days old.  Bowen is now 3.5 years old and has had several more open heart surgeries – so this is super encouraging.  Anyhow, Matt Hammit wrote an entire album about the emotions and struggles they endured with Bowen, the most popular track is “All of Me” and it sure captures what parenthood feels like.  I hope that you love it as much as I do.  So, pray for our sweet Ellie… pray for healing and that the trach is a HUGE step in the right direction for her.  Pray that this opens the door for surgical options that she desperately needs… and pray that she beats the odds that are stacked against her.  I know I’m asking a lot here… but I know that prayer is the most powerful tool we have… and I know she’s lifted by hundreds (or more) prayer warriors every day.  Thank you – we love you – we are constantly humbled by your thoughtfulness.  Enjoy this song!