Wednesday, July 24, 2013

Not What We Expected

Monday did not go as expected.  We planned on going to another cardiology appointment.  Then we planned on meeting with the cardiac surgeon who will perform the surgeries on our baby boy's heart.  Then we planned on meeting with the hospital family coordinator who would give us a tour of the hospital where we are planning on delivering.

Things kind of fell apart at the cardiology appointment.  We had a (nearly) 2 hour long fetal echocardiogram.  By the time it was over we should have been meeting with the surgeon.  My parents came to meet the surgeon and tour the hospital, but since they were already there they came in to meet with the cardiologist too.  I'm glad they were there to hear the news too, because Jeff and I were having a hard time.  It always helps to have multiple ears when you are hearing bad news.

This is what we learned about Baby Ethan's heart:  (You can click on this picture to make it bigger.)



1. They could actually see his left atrium today, which seems like a good thing, but it's not.  Since they've never been able to see it before, this meant that there was blood flow going to it that wasn't there before.  They learned that his pulmonary veins were actually attached to this side, so blood is going in, but there is a lot of pressure in this chamber and the blood is not able to get out as well as it should.  This hypertension in the left atrium indicates a problem.

2.  Most (94% or more) HLHS kids have another defect which is a hole between the right and left atrium.  This is called an Atrial Septal Defect (ASD).  Ethan's heart does not have this.  (Who would've thought that we'd actually WANT him to have another defect?)  If his heart had this hole it would relieve this hypertension in the left atrium and would make his life a little easier.  As I've researched this a little more, I've found that they call this an Intact Atrial Septum (IAS) or Restricted Atrial Septum (RAS).  

3.  Remember how I said before that HLHS kind of starts a chain reaction of problems in babies?  Well since Ethan's heart has no ASD, his body tried to make up for that by growing a new vein going from the left atrium to the Superior Vena Cava.  This "decompressing vein" was kind of Ethan's life line.  It should have solved the problem by draining the blood from the left atrium back into his system.  The problem is that this "decompressing vein" is obstructed and can't do it's job.  They looked closely at this vein last month and measured the pressure of blood going through it.  This month the amount of pressure has more than doubled.

4.  All of this extra blood pressure building up is also starting to cause problems for Ethan's lungs.  Because all this blood in his left atrium cannot drain fast enough, it has started to flow backwards through the pulmonary veins toward his lungs.  This can easily and quickly damage his lungs.  

5.  With all this going on inside me, they are a little bit concerned about Ethan's health inside the womb.  Before they said that he would be okay until his due date, but now they aren't so sure about that.  I will now have to be monitored more frequently to make sure the baby is not in fetal distress.  And I am now more paranoid if it's been more than 10 minutes since I've felt him move.  Great.

This unique set of problems is very rare.  HLHS itself is rare, occurring in about .04% of births.  IAS occurs in only about 1% of all HLHS cases.  And honestly, it's a very lethal combination.  The outlook is not good for our baby boy.  We have been presented with three options to chose from to try and fix this problem and give our baby boy a chance at life.  All of these three options are considered "high risk."  None of these options are good, but they are the only chance we have.  

1.  Our cardiologist recommended sending our case to Boston Children's Hospital where they have the #1 children's heart center in the U.S.  There they are performing fetal intervention for cases like ours where the baby does not have an Atrial Septal Defect.  They would stick a needle in my belly and through the baby's chest to get to his heart.  They would then be able to create a hole between the right and left atria.  This surgery is high risk to me, and to the baby.  It can also sometimes cause pre-term labor, and HLHS babies born premature have almost no chance at life.   By sending our case to Boston, our cardiologist is hoping that they can shed some light on other cases they've seen like ours and what research they have gathered.  He's a little concerned that since this surgery is still in the research phase and a baby with this specific anatomy is so rare, that they will be very enthusiastic about having us come to Boston and give it a shot.  (In his words, "If the only tool you have is a hammer, you're going to want to use it." Even if a hammer is not the best tool to use.)  He said he should hear back from them within a couple days and then he would get in contact with us to tell us what they said.

2.  The second option he presented to us is an emergency Norwood procedure.  This is the first open heart surgery that all HLHS babies have.  They typically like to wait as long as possible to do this surgery (about a week after birth) so the baby has at least a few days to gain some strength and get stable.  This would not be the case with Ethan.  If we choose this option, he would be taken immediately after birth, have an echocardiogram to see what shape his heart is in, then the surgery would be performed immediately.  This surgery includes removing the atrial septum between the left and right atria and enlarging the aorta by connecting it to the large main pulmonary artery.  This seems like a great option.  The cardiac surgeon came and visited with us and explained the whole procedure as well.  But he said in his 26 years of experience, when he has performed and emergency Norwood, the babies have not done well.  They don't recover well and typically have other complications that arise from it.



3.  Our third option is to have a different emergency surgery.  This would mean that Ethan would have two open heart surgeries within the first week of his life (this one and then the Norwood).  In this surgery they would create a whole between the left and right atria and also place a couple bands around his pulmonary veins to keep the blood flowing to his body and not as much toward his lungs.  When the surgeon talked us through the different surgery options, it seemed like this one was the "best" option.  It is still high risk, but maybe a little bit less-so than the others.

With the last two options, we are most likely going to have to have an induced labor in a very controlled setting so that "all hands are on deck."  That means I will likely be induced about a week early, so September 23.

We need your prayers.  We need your prayers so that Ethan will be safe inside me for 9 more weeks and won't go into fetal distress.  We need your prayers so that Jeff and I can be strengthened and have a clear mind to make the right decision for our sweet baby boy.  We need your prayers so that we can have hope and faith that whatever outcome is in our future we can be okay with.  We need lots of encouragement and kind words.  It's hard to describe what this feels like for first-time parents.  We love you all and are go grateful for all the support we've gotten from you so far.  Thank you.