An Anniversary and Update

Today marks two years since Eli's fist open heart surgery.  Monday, January 10, 2011.  The days leading up to it were a blur of exhaustion, a roller coaster of emotions, and gathering information about our baby that no parent wants to explore.  If we didn't proceed with surgery, Eli would be dead by the weekend.  If he had surgery, he may not live through it.  If he lived through it he might be on a heart-lung bypass machine.  If he lived through all that, we had no idea how long he'd live or what his quality of life would be before he required a transplant.  Too many unknowns and the only certainty was that we had to do something.  This was our baby.  Despite his deteriorating condition we could still see his perfection.

The last few hours before he was taken for surgery were filled with tears and pleading to God that our baby live through the operation.  We were so scared he was going to die.  Of course we were hoping he would live, but our faith was so rocked by the news of his diagnosis that it was hard to be hopeful.  I prayed nicely, I gave praise, I questioned Him, I mentally yelled at Him, I begged, and pleaded, knowing the whole time that God "owed" us nothing.  I finally stopped with the words and just asked God to hear the prayers in the silence of my heart.  Thankfully the plan was for Eli to make it through that surgery (and the other surgeries, heart catheterizations, and procedures he's survived since then). We are so grateful that he's still with us.  We don't know how long we have him so we cherish each day the four of us have together...even when he's playing in the toilet water.  As I was typing this paragraph Zachary hollered for me to help him.  I walk into the bathroom to see Eli splashing in the "just used" toilet bowl.  Now, after a quick bath and change of clothes, I'm rereading those words and already laughing about how Eli chose to celebrate this occasion!  Timing is everything!

It's been a long two years, but the emotions of the days leading up to that first surgery and the time he was in the OR will never leave me.  They seem especially fresh as we are starting some preparations for his next surgery.  Eli's last cardiac check up was in December.  His oxygen saturation was about 75%.  As expected, his levels are slowly but steadily declining.  The goal was for him to reach 25-30lbs before the third surgery and his last documented weight was 25lbs 9oz.   His steady weight gain and declining sats are leading us to think his surgery will likely be this spring.  He's scheduled for an echocardiogram (ultrasound of the heart and its vessels) and and EKG (electrocardiogram, measures the electrical activity/rhythm of the heart).  Those will be done with our Evansville cardiologist in mid-February.  Those results will be sent to our main cardiologist in Indy and they will decide when he needs the Fontan.

We celebrated New Year's Eve 2010 with friends who are like family and were excited to begin 2011 as a family of four.  2011 wasn't the year we expected and was filled with stress, to say the least.  We were ready to say goodbye to 2011 and have a fresh start in 2012.  It was a good year for us, no major medical procedures other than a maintenance heart cath and, most importantly, no surprises!  As 2013 begins, I'm filled with apprehension.  I know Eli will have another open heart surgery sometime this year.  I know more about his condition.  I know more of the positive outcomes and the negative outcomes.  Not only are we on an emotional ride this time, we have more knowledge and that is helpful yet it comes at a price.  And of course we're not only worried for Eli but for Zachary as well.  We've never hidden Eli's condition from him, but it's hard to explain to a little kid.  He's older now and has more questions.  He understands enough to know that it's serious but not enough to fully comprehend what could happen.  It's hard trying to explain the truth without scaring him...or me.

Something that's become more and more obvious to me is that we need more awareness about Congenital Heart Defects.  Most people reading this blog, myself included, were pretty much clueless about the prevalence of CHDs prior to Eli's diagnosis.  Even now, as I do more research, I am shocked at some of the statistics I've come across.  I have shared a few of those with you before and will be doing a lot more soon.  I want to ask that you mark something on your calendars:  February 7-14 is CHD Awareness Week.  I will be doing some things to promote that week.  I'm not doing any fundraisers for it, my goal this year is to simply raise awareness.  I will let you know what we have in store as we make more plans  I want you to put it on your calendar when you finish reading this.  As you're going about your daily life try to mention CHD Awareness to someone new each week as CHD Awareness Week approaches.  My next post will have more information and statistics.  You don't have to memorize it, but just remember something from it that you didn't know before and share it with someone each week.

Thank you for reading, for praying, for all the encouraging comments, hugs, and support we've received.  I'll close with a few pictures of Eli on that day two years ago and now.

Bryan's parents brought Zachary to visit the day before surgery.  Sweet Zachary wanted to lay next to his baby brother before they left.  He wasn't phased at all by Eli's tubes, monitors, and equipment.  Look how young Z looks in this picture!  He was just a baby himself!

A kiss on the head for Eli.  I was so afraid this was the last time my two boys would be together.

A picture of the four of us before Zachary left with his grandparents.  I think we were looking at 6 different cameras because so many people wanted to capture this moment for us (the staff bent the visitation rules for us a little bit).

One of the last pictures of us holding Eli before surgery.  Neither of us slept the night before and there were lots of tears and hugs.  We kept smiles on our faces to keep from breaking down.

Our little hero!  This was taken about 4 hours post operatively.  He was swollen and covered in tubes, monitors, tape, and wires, but we really didn't see much of that.  We were enamored with our strong baby who had fought the odds and was surprising his healthcare team with how well he was doing. 

Eli today.  If you ask him where his smile is he will crinkle his nose and give this huge goofy smile that cracks us up every time we see it.  He's definitely a comedian.

Now go mark CHD Awareness Week on your calendars.  It's February 7-14th.  Spread the word!!!!

It's okay to ask

It's been happening more often.  That question.  It's asked by very innocent, well-meaning strangers.  I appreciate the fact they are just making conversation or even worried about the baby they see in front of them.  I really do.  I just don't know how to answer them without causing some amount of heartache for the questioner.

If you're kind enough to still be reading, you're probably wondering what in the world I'm talking about.  The question.  Or maybe I should say those questions.  The point is that I don't always know how to answer properly.

The questions are "Has your baby been eating a popsicle?" "Is your son okay?  His lips are blue."  "Do you need any help?"  We've expressed before that we are so grateful for the concern and support all of you show us in many ways.  We're also grateful for the concern of strangers, but I just don't know how to handle their concern.

For example, the other night the four of us took my mom to Red Lobster as an early birthday dinner.  Eli is unable to sit through any meal (home or restaurant).  You'd think he was a busy toddler or something.  He & I left the table to go for a walk.  It was very crowded that night and hot outside so I took him to the ladies room.  There was room to walk and girls to flirt with....let's face it, that's all he thinks he needs in life.  One lady was talking to him (yes, he was answering some of her questions) and she asked him if his belly was full.  He nodded yes.  Then she asked him if he ate something blue because his lips were so blue.  I would've just "ignored" the question, but she was looking directly at me and asked again, "did he eat something blue?"  I simply said, "No, he has heart problems and his lips are often blue."

Immediately her face fell and she started apologizing.  I assured her I wasn't upset and thanked her for her concern.  I know why strangers apologize in this situation, I probably would too, but it's still kind of funny to me.  It's not like she caused Eli's defects or has blocked us from receiving wonderful medical care or anything.  It's just a lack of knowing what to say when told the beautiful baby you've been conversing with in a Red Lobster ladies room has such hardships.  It's an apology that, through no one's fault, the same beautiful baby and his (equally beautiful) family have to endure such a tough situation.

Sometimes people will ask more detailed questions and I'm happy to answer them.  Sometimes people leave it at that and the conversation ends.  Sometimes (most often) they ask one more question, "Is he going to be okay?"  Another fully loaded question that really can't be answered honestly without some explanation they're probably not ready to hear.  Usually if we're asked "is he going to be okay?" I'll simply say, "He's doing well for now and we've got a lot of people praying for us."

Again, I love the questions.  Knowledge is power.  It doesn't upset me to talk about it.  I just don't know how much a stranger wants to hear, even though they're asking the questions.  You can see on their faces how much they're encouraging me to say that, yes, it's been tough, but he's healed and will live a very active life until he passes at a ripe old age.  But I can't do that.  I hate to disappoint them, but the truth is the truth.  In my mind the most positive thing that comes from the occasional question from a stranger is that more prayers might be offered up for Eli and other kids like him.

"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.