The next day it seemed like a lifetime passed before we finally heard from the doctor. I stayed in constant contact with Josh that morning. I sat helpless, feeling a million miles away in my hospital bed waiting...and praying.
Finally after 1:00 in the afternoon, the cardiologist, Dr. Law came in to talk to Josh and the rest of our family.
I texted Josh during that time and he told me that the doctor was in there explaining everything to them and he would call me as soon as the doctor left.
I told my mom and my sister who were in my room with me and we prayed together. I spent the remainder of that time praying and speaking scripture. I was terrified but doing my best to put my faith in God and trust that good news was coming. It seemed like years passed before Josh called. When the phone finally rang, I thought that my heart was going to leap out of my chest. It was one of the most serious moments of my life.
Josh explained to me what Dr. Law had just explained to him. At the time it was a lot of information and it was confusing and scary. Since then I have had plenty of time to study Jack's condition and fell comfortable with the terminology.
Here is everything Dr. Law explained to Josh and our family that day. It's precise in detail because they videoed him speaking and I wrote it all down straight from the video. It's complicated but pretty much explains it all in good detail. Here it is.
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Jack's heart is built different from a normal heart. First of all, there is no way for blood to go from the left atrium to the left ventricle because the valve that separates the two chambers didn't form. This is called Mitral Atresia. So, in Jack's heart, blood is having to take a longer route to get from the lungs back into the heart and back out into the body. Also when this blood is coming back into the heart it can go one of two places. It can go across the pulmonary valve or into the aorta because there is a hole between the bottom chambers of the heart (the right ventricle and the left ventricle.) This is called a Ventricular Septal Defect or VSD.
They are not able to make Jack's heart into a normal heart, but they have ways to treat his heart as it is. They will have to re-route the blood flow as time goes by. This is because the left ventricle is too small and also because the valve that separates the left atrium and left ventricle didn't form.
At the time, Jack was on a medicine called prostaglandins which was being administered through an IV. This medicine was keeping his Patent Ductus Arteriosus or PDA open. All babies are born with their PDA open which allows blood to flow between the pulmonary valve and the aorta. In normal babies, the PDA closes within the first few days of life. They needed to keep that open in Jack's heart for now with the prostaglandins. This was ensuring that there was enough blood flow going to his lungs.
At this time, the first question that needed to be answered was wether or not Jack needed the PDA to remain open. If the PDA closed and he was still getting a sufficient amount of blood flow to the lungs then he didn't really need it to remain open. Even in Jack's heart, blood could get out into the lungs from the heart, however, his pulmonary valve was a little bit smaller and had a narrowing at the opening (called Pulmonary Stenosis) which would make it more difficult for blood to flow through, leaving his cardiologist to wonder if the amount of blood that could flow through there would be enough. The question he didn't know the answer to at that time was if it was too small or too narrow and he couldn't tell that while the PDA was still open.
So, what he recommended was that the RNICU doctors turn off the prostaglandins at 6am the next morning. The reason why he wanted to wait until the next day was that sometimes turning off the medicine could create an emergent situation (like if they turn it off and Jack's body wasn't getting enough blood to his lungs on his own.) In that case, those doctors would have to decide what to do. The RNICU doctors were not cardiologists, nor did they know Jack's case particularly well. So, Dr. Law, Jack's cardiologist, wanted to wait until the morning when he would be there in case they needed him. It was just safer and easier for everyone that way.
They needed to take this step (turning off the prostaglandins and allowing the PDA to close) so that they could see if there was enough blood flow to the lungs without the medicine because Jack couldn't stay in the RNICU forever on the medicine. They had to figure out how to ensure that he was getting enough blood flow. They would turn it off and see how his oxygen saturation looked over the next few days as the PDA closed. An acceptable oxygen saturation was usually in the 80s.
If they turn off the prostaglandins and allow the PDA to close and there is enough blood flow to the lungs to keep his oxygen levels in the 80s on his own without the PDA then he would get to go home. We would follow up closely with the cardiologist in the pediatric cardiology clinic at UAB over the next few months. If they allow the PDA to close and his oxygen levels begin to drop, he would require a surgery to fix that problem before we could leave the hospital with him.
This surgery, called the Norwood Procedure, would consist of placing a shunt in his heart to allow a sufficient amount of blood to flow to the lungs. Basically they would replace the PDA with another tube that connected blood flow from the pulmonary artery to the aorta.
Either way, wether he needed the first surgery or not, Jack would be sent home to grow over the next few months. Then in a few months(3-6 months) he would need the next surgery.
If he didn't have to have the first procedure, then he would have a heart cath before the second and third surgeries in the series. The cath would tell the doctors if the pressure or resistance in the lung arteries is high, if it is high then the second surgery will not work. Dr. Law said that he didn't feel like that would be a problem but they have to check anyway.
The next surgery in the series is called a Bidirectional Glenn. Jack would be put on pulmonary bypass for this procedure. This surgery would connect the blood that comes into the heart from the head and arms (blood from the superior vena cava) into the pulmonary artery (lung artery). This allows the blue blood that comes from the head and arms to go into and back out of the lungs.
After this surgery, Jack would be sent home to grow again. The only issue now is that there is still some blue blood going out to the rest of his body and his oxygen levels still wouldn't be normal. So after he has had a few years to grow they would perform a final procedure called the Fontan procedure.
There isn't a specific time frame for the Fontan procedure. They usually perform it at around 2-5 years of age. Dr. Law said that we (Jack's parents) would be the ones who would dictate when Jack's surgery would be based on his endurance levels and coloring. He said once Jack starts running and playing as he gets bigger, he would begin to lose endurance and start to lose some of the pinking in his color. (i.e. he would turn blue and lose his breath easily.) When that starts to happen to him, wether he is 2 or 5, that will be the time when he needs the surgery. Even if it doesn't happen to him within those years, they wouldn't allow him to go past the age of 5 because they think its good to get the surgeries over with while they are young enough that they don't remember them when they are older.
In the Fontan procedure they would take the blood that comes from the lower part of the body and routes it into the lung artery. (This would connect the inferior vena cava to the pulmonary artery.) Then after these surgeries his oxygen levels would be normal.
There wouldn't be any more necessary surgeries or interventions required. He will require lifelong cardiology follow-up and will be watched closely throughout his life in the cardiology clinic. He will go onto live his life but will be challenged in areas of stamina and endurance. Dr. Law joked that Jack probably won't be a star athlete but neither was he and he turned out just fine.
To sum it all up, Jack has a condition called Single Ventricle, sometimes referred to as Hypo-Plastic Left Heart Syndrome. Mitral Atresia, VSD, and Pulmonary Stenosis are all covered under that main term of Single Ventricle.
Dr. Law said that many children go through these procedures and do really well after the surgeries. He can't guarantee that Jack won't need a heart transplant one day but he wants to just focus on what is immediately in front of us right now. He said there is no way to know what he will need in 20-30 years but for now he thinks that Jack will do well with these procedures. He said that Jack is going to have to go through a lot and every step has risks, but he thinks Jack is in a low-risk category for most everything at this point in time.
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To make a long story a little bit longer...
The next morning at 6am I was up, showered and ready to go. I was closely monitoring what was going on with Jack via texting with Josh while I waited for my doctor to come by and sign my discharge papers. I was on pins and needles waiting to get out of that place. When she finally came by, she gave me a quick once-over and set me free. We got to UAB in one minute flat.
My mom and sister dropped me off at the door where Josh was waiting with a wheelchair and they went to find a pharmacy to fill my prescriptions. We couldn't get up to the RNICU fast enough. I was dying to see my baby again. It had been two long days and a lot of tears since I handed him to that nurse.
When I finally got to him, my heart melted. He was sleeping so sweetly, all bundled up on a tiny little NICU bed. I burst into tears. Josh held me as I cried and let out every emotion that I had been feeling for two days. The nurse came in and wrapped up all of his little cords and finally handed him to me. It was the most glorious moment. I was finally holding my sweet boy again, something that, for a time, I was scared I might never get to do again. Tears streamed down my face as I held him and started into his sweet little face. My boy. Mommy's boy.
Later that day Dr. Law, the cardiologist, came by to see me and ask if I had any questions. Of course I had hundreds but none that I was emotionally ready to face. That day I just wanted to hold my boy and forget that his heart was sick. I held him in that chair all day long. I finally got the chance to feed him for the first time. I won't go into detail about how a male nurse helped me and my husband learn to breast feed our son for the first time. Awkward.
Throughout that day, the nurses came in to check on us and I got acquainted with my new hospital home. The RNICU at UAB is amazing. Every baby has their own little private room with a chair and a couch. Josh and I never had to leave his side for the remainder of our stay. I am eternally grateful for that and for the sweet demeanor of the nursing staff that worked with us that week. They loved Jack too and were rooting for him with us. I'll never forget them.
Over the next few days we watched Jack's monitor like there was an award winning movie playing on that screen. Every time he moved it beeped and every time it beeped I panicked and every time I panicked I called for a nurse and every time a nurse came they had to reassure me that he was fine. It was a never ending cycle of me making sure those poor nurses were having to work for their pay. They probably weren't too sad to see us go. Haha
The
Now, I'll have you know that since then I have done quite a lot of research and have yet to find one serious case of HLHS where they child didn't need the first surgery. That's
Later that day, after waiting about 8 hours for one shot that Jack had to have, we were finally discharged. We packed up everything we had brought and our families had brought to us, which basically looked like we were moving, and we dressed Jack in his sweet little going home outfit. They finally removed all of his cords and wires and he was completely free for the first time in his little life. The nurse made me get back into a wheelchair even though I had been walking around there for nearly a week just fine, then she handed me my baby and wheeled me out to the car. We loaded our little bundle of joy into his car seat for the first time, and then we took him home.
There are many stories I could share with you about our week in the RNICU. Some amazing moments of joy, some moments of fear and even terror, and some moments where we truly felt the peace of God settle in the room with us. I'm sure that as time passes, I will come back to that week and share some of those moments with you in this blog. For now, its just important for you to know that we experienced our first real victory that week. God allowed Jack's body to perform a miraculous task which he is still performing right now. He is breathing on his own thanks to an adequate supply of blood flow to his lungs. Something that his doctor wasn't sure he would be able to do because of several heart defects that he was born with.
Today Jack is thriving and surprising his cardiologist every time we visit him in clinic. He is not only breathing well on his own but is eating and gaining weight. This is another true miracle. Praise God.
God has done so much for my sweet boy in just his short two and a half months of life. That first week was the beginning of an amazing show of God's power. God is still a miracle worker and if you ever doubt that, just look at my sweet son. If it weren't for a nurse telling me that Jack was going to weigh over a pound more than he actually weighed I wouldn't have chosen to have a C-section. If I hadn't had a C-section Jack could have died during child birth because his little heart wouldn't have been able to take the stress. Then also if it weren't for a nurse noticing his trouble breathing and then another nurse hearing his heart murmur while in the same room, they might not have found his heart problems and he would have died within a few days without much explanation. This is the absolute show of God's miracle working power. I'm not sure what God's plan is for Jack but I know He has a BIG one. I am certain that Jack is going to grow into a strong man of God and is going to do great things for the kingdom of God.
Now to him who is able to do immeasurably more than all we
ask or imagine, according to his power that is at work within
us. Ephesians 3:20
