Monday, July 30, 2012

"So, what's wrong with his heart?"

That's a question we get a lot.  It's not a short answer.  Many times, I've thought about doing a post describing Eli's defects.  Actually, I promised to do that in my very first blog.  One of our goals with the blog is to provide accurate information.  This may interest some of you and it may bore others, so read at your own risk! 

Everyone has had to deal with something medical at some point in their life.  Most people do so with some level of confusion.  Our situation is no different.  The heart is a very complex and amazing organ.  Heart defects (or anomalies) make the complex cardiovascular system (the heart and all blood vessels) very confusing.  It gets even more confusing when multiple defects are present, which can literally make each heart work in a unique way.  We've met a few other families whose situations are very similar to ours, but each child's path has been a little different.  Some of the differences are simply because individuals react differently to medicines, procedures, therapies, etc.  My point with all this is that this post is about ELI'S DEFECTS.  There are (unfortunately) many, many types of heart anomalies and I'm only posting what we understand to be true for Eli's heart.

Another thing:  Yes, I am an RN (and proud of that), but that doesn't mean I have special insight or abilities to deal with our situation.  In all honesty, my least favorite parts of nursing were cardiology and pharmacology.  And some people say God doesn't have a sense of humor?  Maybe I'm just overly sensitive to the issue, but please don't assume that I'm equipped to deal with this because I'm a nurse or that I always understand what is going on.  I don't.  Eli is not my patient, he is my child.  We can't absorb all the information we've been given.  There's so much to understand that sometimes we "choose" what information we retain or what questions we want answered.  One example is the fact that Eli will need a pacemaker.  The plan is for him to have that placed during his next surgery (possibly spring or summer 2013).  We've had several caring individuals ask questions such as "will he get a new one as he grows?" or "what kind of care is involved for a child with a pacemaker?"  These are good questions, but frankly we don't know the answers.  We haven't asked the questions yet.  We will ask when the time comes, until then we don't need to overload ourselves with information we won't use for almost a year.  Please don't be afraid to ask us questions and show us your concern, it really does mean the world to us, just don't look at us like we're nuts for not knowing every little detail of what to expect in his life. 
Whew!  Thanks for that little vent!  Now, to our lesson:

First I thought I should explain the parts of a "normal" heart and its flow so that when Eli's defects are explained you might have something to reference for the difference.
  • The heart has four chambers.  The upper chambers are called atriums and the lower chambers are ventricles.
*in medical terms, when specifying "right & left" it refers to the patient's right or left*
  • The two chambers on the right side are separated by the tricuspid valve.
  • The two chambers on the left side are separated by the mitral valve.
  • The right side of the heart has unoxygenated blood and the left side has oxygenated blood.
  • The right and left sides of the heart are separated by a wall of tissue called a septum.  The upper chambers are separated by an atrial septum and the lower chambers by a ventricular septum. 
  • The pulmonary artery carries unoxygenated blood from the right ventricle to the lungs to pick up oxygen.  
  • The pulmonary vein carries the newly oxygenated blood from the lungs and returns it to the left atrium.
  • The aorta transports the oxygenated blood to the body.
  • The vena cava transports unoxygenated blood from the body back to the heart.

The blood flow in a normal heart:
  1. Vena Cava brings unoxygenated blood from the body back to the heart
  2. Right Atrium
  3. through the Tricuspid Valve
  4. Right Ventricle
  5. Pulmonary Artery to the lungs
  6. Pulmonary Vein
  7. Left Atrium
  8. through the Mitral Valve
  9. Left Ventricle
  10. the Aorta carries blood away from the heart out to the body & its tissues
  11. returned to the heart via the Vena Cava
Clear as mud, right?  It's a beautiful system...when it works properly.  Now I'll list Eli's defects and a description of each:

Tricuspid Atresia:  instead of a tricuspid valve between the upper and lower chambers on the right side of Eli's heart, there's solid tissue.  He never formed a valve or opening of any kind between these chambers.  That results in no blood flow to the lower right side which makes it severely underdeveloped.  Normally, this defect alone is not conducive to life.  In blunt terms, one cannot live with this condition.  He should have died within the first 48 hours of life.  The reason he didn't is because of the next defect listed.

Ventricular Septal Defect (VSD):  a hole in the septum between his ventricles.  This results in a mixing of oxygenated and unoxygenated blood being sent to the body.  This defect is the only way Eli's right ventricle and body received any oxygenated blood.

Transposition of the Great Vessels:  the aorta and pulmonary artery are deemed the Great Vessels.  One takes unoxygenated blood to the lungs to receive oxygen and one takes the oxygenated blood to the body to use the oxygen.  Eli's aorta and pulmonary artery were switched.  That resulted in unoxygenated blood being transported to his body.  We were told that Eli's extremities were probably only receiving about 20% of the oxygen they should have been receiving. 

Coarction of the Aorta:  Eli's aorta had several narrow areas or "kinks" in it which resulted in a restricted flow of blood

Hypoplastic Right Heart:  fancy term used to group some of the previously mentioned defects.  Because he has no tricuspid valve, the blood flow to his right ventricle was severely restricted to whatever leaked in because of his VSD, therefore the lower right portion of his heart did not develop.  It isn't something that can "catch up."  It did not develop and it will not develop.  Eli has one working ventricle in his heart.  Sometimes the term "Single Ventricle Heart" is used instead of the more specific hypoplastic right heart.  

What can be done about these defects?  Eli is currently undergoing a series of three surgeries.  They are as follows:
  • Eli's first surgery was a Norwood procedure.  During this surgery, Eli's kinked and narrowed aorta and his pulmonary artery were combined into one vessel that takes (mostly oxygenated) blood throughout his body.  A shunt was placed between this reconstructed aorta and his lungs to provide blood flow to the lungs.   Because of the extensive reconstruction of the aorta that must be done, this operation is one of the most challenging heart surgeries in pediatrics.  This surgery is typically done within the first week of newborn life, but because Eli wasn't diagnosed until he was ten weeks old, his heart was very enlarged and his condition was rapidly deteriorating.  He had a 30% chance of not surviving the surgery.  It was almost certain that IF he survived, he would be on ECMO (ExtraCorporeal Membrane Oxygenation), which is a machine that acts as his heart and lungs so that his organs can rest and heal.  Survival in such situations is often felt to be 20% or less.  Fortunately for us/by the grace of God/prayers were answered/Eli is too stubborn (you choose any or all of the previous statements), Eli did not require ECMO.  He amazed everyone by being strong enough to function on his own after surgery.  He's pretty amazing!  (He = God and Eli!)
  • His second surgery was a hemi-Fontan.  Eli was 10 months old at the time of this surgery.  He was much bigger and healthier than he was prior to the first surgery and that gave us great confidence going into this one.  Of course there were still risks, but we knew Eli was as ready as he could possibly be for this.  During this surgery, the superior vena cava (vessel that returns unoxygenated blood to the heart from the upper extremities, head and neck) is removed from the heart and sewn directly to the pulmonary artery.  The shunt that was placed in the first surgery is removed.  Basically, this surgery reroutes how unoxygenated blood from the upper half of his body is returned to the heart.  
  • The third stage is called a Fontan completion and will probably happen within a year.  Our doctors have told us they would like Eli to be close to 30lbs before proceeding with this surgery.  During the Fontan operation, the inferior vena cava (blood vessels returning blood to the heart from the lower half of the body) is connected directly to the pulmonary artery. Until now this blood has bypassed the lungs and has been pumped directly to the body resulting in oxygen levels lower than normal.  After this procedure, Eli's oxygen levels should be in the lower 90s (93% or higher is considered normal for most people). 
What's after the three surgeries?  These three surgeries are not fixes.  Eli's heart cannot be fixed.  The goal is to make his heart as functional as possible for as long as possible.  A heart with this level of reconstruction is not expected to last nearly as long as a normal heart.  A single ventricle heart works much harder than the average heart and therefore it ages much faster.  As of now, once his reconstructed heart starts to fail, our only option is a heart transplant.  How long before that happens?  We don't know.  We're hoping he at least gets to his teens before requiring one.  Of course we'd be thrilled if it's longer!  And of course we're praying for more discoveries in the field of pediatric cardiology.

Even more confused?  It's okay, we're not quizzing you.  If anyone has any questions, please ask us! We love that people are interested in his condition and we want to help you understand as much as you want.  Some people want to know more and that's partially why I did this. It's taken me a few days to put this together because of all the double checking I'm doing to make sure my info is correct.  If it helps anyone understand even one thing about a normal heart or Eli's heart, I'm happy.  It was good for me to do this too.  Sometimes I forget just how complicated his system is and this has helped me. All I know for sure is that, for the time being, his heart beats....and we cherish Every Little Beat.