Overview

We had what was thought to be a normal 20 week visit to the OBGYN.  It turned out to be anything but, when we learned that there was something wrong with our baby's heart. The news was overwhelming and difficult to hear. The weeks that followed have been a roller coaster ride of emotions and each appointment has yielded new findings.

If you are just hearing this for the first time, I apologize for the poor delivery of bad news! We have kept the information close to ourselves and family, as we've needed more time for doctors to provide us with more accurate information.

I wanted to start a blog to keep friends, family, and others in our lives updated on the journey and health of Baby Joey. This blog will be a means of sharing information with a large group, which will help us to not have to explain things multiple times. We want to keep everyone update on what is going on as I know a lot of people are concerned.

The Diagnosis: 
The doctors believe that Baby Joey has three defects of the heart, and one of the abdomen:

• Transposition of the Main Vessels  (Aorta and Vena Cava are switched)
• AV Canal Defect  (Holes in the Atrial and Ventricle Walls)
• Partial Anomaly of the Pulmonary Veins  (2 of the 4 PV's going to the wrong chamber)
• Total Abdominal Situs Inversus  (organs in the abdomen are in opposite sides than normal)

The combination of defects along with the Situs Inversus, doctors rule this as Heterotaxy Syndrome. There are different degrees of Heterotaxy and each cases is different. I have spent numerous hours on the internet googling this term and I have learned to stay off the computer! Heterotaxy is extremely rare and the outcome depends on the severity of the heart defects. In many cases babies do not live to see their first birthday, but others lead very long and normal lives.  

The Prognosis:

Ultra sound technology is good, but its can only tell us so much. The doctors will not have a full understanding of what is going on with Joey's heart or body until he gets here.

From all the information we have, the doctors and surgeon believe that Baby Joey will need to have two open-heart surgeries. The first will probably happen during the first week of life. The initial surgery will be repairing the Partial Anomaly of the Pulmonary Veins. This will make it so that Joey is receiving enough oxygenated blood to his heart and rest of body. When these veins are not hooked up correctly they can become obstructed very quickly, which is why immediate repair is needed.

The surgeon is hopeful that after the first surgery, Baby Joey will be well enough to come home for a few weeks. The recovery from surgery is estimated to be about 10 to 14 days in the Open Heat Unit. This is not guaranteed and we are not expecting this to happen, but if it does it would be amazing to have our little guy home for Christmas.  

The second surgery is expected to be 6 weeks later where the doctors will correct the other two defects.  The AV Canal Defect is normally done at 7-9 weeks, because it allows the heart tissues to strengthen a little bit before surgery.  Both the Transposition and AV Canal defects can be fixed in the same surgery, but it'll be a first for Dr Ilbawi, the surgeon caring for Joey. 

Outlook: 

The outlook for Joey depends on the success of the heart surgeries.  The heart surgeon seems very confident that he can fix Joey's heart. Although Joey has a very complex heart, each isolated defect is a routine procedure with a high success rate.
  

From what we have been told the stomach being on the opposite side of the body is a "non-issue". Apparently, people are born with this all the time and things function normally with little to no correction needed.

Once the heart defects are corrected, the doctors anticipate Joey to have a very normal life. He should reach all of his milestones and have no restrictions on diet or exercise. He will need to follow up with a cardiologist on a yearly basis. Dr Ilbawi has said that the objective is to give our son a fully functioning, circulatory heart.   

Positives: 

In light of all the bad news and grim circumstances, we have found a way to look at the positive things going for us:

• We have, who is considered by most, to be the best Pediatric Heart Surgeon in the entire county who will operate on Joey. Dr. Ilbawi has been quoted as a "miracle worker" and even has procedures named after him. The countless awards for "Surgeon of the Year" and "Doctor of the Year" in his office are very impressive.  
• We know about this ahead of time.  There are only a handful of ultrasounds done over the 40 week pregnancy. Thankfully, we had a skilled doctor and she was able to discover the issues with Joey's heart at 20 weeks. This has allowed us preparation to ensure he is treated properly at birth in an controlled environment. In so many of these cases babies are not diagnosed until after they are born.
• Despite the several defects, Baby Joey has some good things going on with his heart too. The babies that are reported of dying with heterotaxy are due to having very poor hearts. Our baby has all 4 chambers, 2 sides that pump, and a value that is not leaking very much. The heart surgeon seems confident that he can fix it. 
• The heart is fixable. Typically, when a baby is diagnosed with a heart defect, there is a chromosomal issue as well. We were able to do extensive genetic testing and everything came back as normal.  A heart is fixable, but a chromosome issue is permanent.

How are we doing:

We are doing as well as anyone can be in our position. We are worried, scared, excited, and hopeful. At this time we are in a good place. We have been able to accept our situation for what it is.

Trust me, it took me awhile to get here.  At first I was upset, then I was angry, and then I did the "why me?".  I quickly learned that these things will get you nowhere fast and they did not make the situation any better.

I have no idea why this is happening to us and there is nothing that I can do to change it. I am trying to be patient and wait for all of our questions to be answered. In a situation like this, you cannot plan for anything and things can change day-to-day. I am learning that nothing is in my control and that I need to be flexible. Going through this experience has made mine and Eric's relationship much stronger and we have a better appreciation for life. We have gotten all of the information that we can and are ready to take this on. We are bracing for the worst and hoping for the best. If this little guy is anything like Eric or I, I know that he'll be a fighter. 

At this point in the game, I have no other choice, but to be positive. As much Joey consumes my mind every day, I have not let it take over. I still have job to do at work, a house to keep clean, an active two year old to care for, a husband to love, and myself. I am fully in trusted in God plan for us. I am a firm believer that everything happens for a reason. A person could drive themselves nuts with all of the “what if” possibilities.  Eric and I are both much more logical then emotional people. This has helped us to remain calm amidst the chaos.  There's a belief that God gives the harder tasks to the stronger individuals to overcome. He wouldn’t have placed us in this situation if we he knew we could not handle it. So now we wait, have faith, trust in his plan, and hope that outcome is what we want it to be.