Hard to swallow...

Last night, our little bug was hooked up to a video EEG machine.  Around 3am, she started to have one of her fits.  I hit the alert button on the machine and talked to it (it records voice and video, btw) describing Ellie’s fit.  Someone from neurology was in our room within minutes to observe this episode.  He watched as Ellie failed about, arched her back and increased her heart rate, body temp and breathing rate.  He held her down and checked to see if she could focus.  He was able to restrain her limbs (unlike what he’d expect in a seizure) and noted that she was responsive with her eyes (again, unlike a seizure).  She did calm down for a few minutes, but once she was given her heart medicine, she started another fit.  They gave her a dose of Ativan (which is a sedative used to treat seizures and autonomic storming, alike).  Within 3 or 4 minutes, Ellie was out.  Ick – I do not like that… but it was nice to see her breathing and heart rate return to normal so quickly.  He said they’d have to assess the EEG, but was almost certain this was not a seizure.  So we talked about autonomic storming again.  I told the neurologist that when we’re at home, the only thing that consistently calms her down is laying her on our cold living room floor.  He said that made sense because in an autonomic storm, the brain sort of misfires and then ramps back up and causes the body to release adrenaline (which is why her heart races, her breaths are fast and shallow, she sweats, and her arms and legs flail).  This progresses until something resets the system (like a manual reboot on your computer).  He said that laying her on a cold surface sends the message of cold back to the brain (the same area that’s causing these episodes also regulates temperature) and this coldness sort of resets that area of the brain and calms the storm.  Hmmm… we had no idea what we were doing putting her on the floor, we just knew that it worked… but now it makes sense why it worked. 

Shortly thereafter, the cardiac team made rounds and they talked to us about the fits and a plan moving forward.  Since it looked like the EEG was ruling out seizures, the next likely explanation is that these are autonomic storms (exactly what we’d talked with neuro about).  They would need to do an MRI to confirm this.  However, for Ellie to have an MRI, she’d have to be sedated again… and with her reaction last time, they’d need to have the cardiac anesthesia team present for this.  They also let us know that she’d possibly need to hang out the in Cardiac ICU after the MRI, so they weren’t rushing this… they wanted to make sure they could schedule the right anesthesia team and such. 

Almost as soon as the cardiac team left our room, Dr. Morales (our surgeon) came in to do the surgery consult that we were supposed to do next week.  We were so excited to get to meet him… but that excitement turned to a feeling of total defeat in a matter of minutes.  He said that Ellie’s echo showed that her AV Canal defect was unbalanced.  Unbalanced to the point where her right ventricle looked inadequate to function (even after surgery).  He gave us a quick anatomy lesson and reminded us that the non-oxygenated blood from the body dumps into the right atrium from the superior vena cava (SVC), which brings blood from the head and upper body, and the inferior vena cava (IVC), which brings the blood up from everything below the heart.  In Ellie’s heart, the volume that is dumped into the right atrium will not fit into her tiny right ventricle (where the blood is held and then pumped to the lungs to pick up oxygen).  So, the solution is a different surgery than we were originally thinking.  He’d have to do what is called a Glenn Operation first, where he will disconnect the SVCs (Ellie actually has 2 of these instead of one… overachiever) and connect them directly to the pulmonary arteries (arteries that carry blood from the right ventricle to the lungs).  If Ellie does well with this, and her right ventricle can still handle the volume of blood from the IVC, then she’d be good for a few years before additional surgery would be needed.  If Ellie’s right ventricle cannot handle the volume of blood from the lower part of her body, then an additional procedure would be needed (called a Fontan Operation).  This procedure would direct the blood from the IVC to the pulmonary arteries, as well.  Essentially, Ellie’s right ventricle is useless.  These surgeries would make it so that Ellie’s heart will work as just one pump instead of two.  Her heart will only pump blood to her body, like it’s just using the left side of her heart.  Is this scary?  Terrifying!  The scariest part about this for me is that if the operations are not successful, the only other option is a heart transplant.  So… these operations HAVE TO WORK.  Below is a diagram of each procedure, if that helps you understand my mumbo jumbo here.

 

Before we can think about surgery, we have to get to the bottom of these episodes, or storms.  A storm would have catastrophic consequences on her post-surgery body.  I don’t know why we didn’t think about this before… but it was suddenly so obvious when he brought it up.  A newly mended heart simply couldn’t handle the stress that’s put on her heart and lungs during these episodes.  That is our top priority right now… to figure these out and treat them.  Priority number two is to make sure Ellie isn’t under respiratory distress and doesn’t have any airway obstruction.  We know she has this now, we know that it’s very common in kiddos with Down syndrome.  We also know that it makes the heart procedures we’re now looking at much riskier.  If there is too much pressure in her pulmonary arteries, the blood will not be able to passively flow into them (remember, blood will no longer “pump” into the lungs).  Ugh!

For now, they’re putting Ellie on a beta blocker called propranolol as a maintenance medication to prevent the autonomic storms, at least until we can get her in for an MRI sometime next week.  Dr. Morales told us to expect to be here for at least another week – maybe two so that we can get to the bottom of a few things.  They’re also going to do a heart catheter sometime early next week to get a better idea of what her pulmonary hypertension looks like.  Sooo… it’s time to get comfy.  We went home quickly today to pack up additional supplies and to visit with the boys for a while.  Then we headed back to Cincinnati to set up camp.  It’s not bad here, it’s about the size of a college dorm room.  Except this time, our roommate is a beautiful 10-pound ball of stress and the tests DO actually have an impact on real life.  This is the hardest day we’ve had, in my opinion… and so far I’ve melted into B and my dad and let the stress of it all run down my cheeks and onto their shirts.  Tomorrow is a better day, I know that.  In a weird way, I am so incredibly thankful that she did not do well with sedation on Wednesday.  If she had, we would have been sent home and we would be dealing with respiratory distress at home.  Also, these fits she’s been having (which everyone kept saying was just colic wouldn’t have been addressed.  Like I said earlier, not addressing these would be catastrophic post-op).  So, pray for our baby, please… this is a harder road than we originally thought we’d be traveling… and the first road wasn’t all that much fun.  We’re hanging in there, though… we know that God’s got this and He has a plan for our little bug.

Thank you for your prayers and for following along with our story.