We have been going to Denver almost monthly since Isabel was released form the hospital at the end of January. I think I could make the 5 hour drive blindfolded at this point.
It has been great being able to describe how I was feeling and what we were going through with Isabel’s birth. I also know it has been a little confusing since everything happened a few months ago but I am just now writing about them. To make things even more confusing for everybody today’s post is about something that is happening in the future.
Tomorrow morning we are leaving Crested Butte heading to Denver for a few doctors appointments for Isabel. We have been going to Denver almost monthly since Isabel was released form the hospital at the end of January. I think I could make the 5 hour drive blindfolded at this point. Denver is about 20 degrees warmer than it has been in Crested Butte so I am looking forward to taking a pair of shorts with me. Isabel and Addison do extremely well in the car and I am very thankful for that.
Tomorrow afternoon Isabel will have a checkup on her G-Tube and then Friday she will have her second Catheterization. This Cath. will let the doctors know how much her arteries have grown. This is important because it will give us a better idea on when the next surgery can be scheduled. The first surgery Isabel had back in January was to make sure there was blood going through the pulmonary arteries giving them the opportunity to grow. The next surgery will hopefully be the one that fixes her heart by closing the VSD and placing a new valve or conduit connecting the pulmonary artery to her heart.
They are planing on her staying the night in the hospital after the Catheterization. They also asked us to stay in town for at least 24 hours after the procedure to be closer to a hospital just incase something was to pop up. As long as everything goes well, we are planing on spending a few hours at the Zoo with both the girls while we are in Denver. Hopefully we will have some fun pictures to share next week.
I was a great distraction for us to have Addison still in town for Isabel’s surgery. We had lunch downstairs with her and Mamaw PJ before they headed back to Crested Butte. One of the nurses would come out and talk to us or give us a call every 45 minutes or so to let us know how things were going. She told us that the surgeon decided to go with a procedure where he used a cadaver artery as a conduit connecting the right ventricle to an area of the pulmonary artery that had some blood flow already. One of the rules they told us is “If it flows it grows”, so the goal was to provide more blood through her pulmonary artery in helping the artery grow. We received word that the surgery was a success and we would be able to go see Isabel in her room.
I am going to talk in detail about what Isabel looked like when we got back to her room and include some pictures as well. They are hard to look at and I completely understand if you don’t want to see a week old baby like that. I am including them because it is what we went through and is part of the experience. You will not offend me if you don’t continue reading.
When we got to her room she was on her bassinet and there was a nurse and doctor that were making sure everything was setup exactly right. She had a blanket over her and the nurse told us that we are welcome to move the blanket and sit around her but they didn’t want us to move her. We could touch her head and let her know that we were there but that was about it.
We removed the blanket and she had a 4 inch long bandage over the incision on her chest. She had 2 chest tubes for drainage coming out from below her ribs and 2 wires attached to an external pacemaker coming from just above where the tubes were. She was in 3rd degree heart block. This means that her heart would not beat normally and she was completely reliant on the pacemaker. The doctor told us that they were a concerned about it because they did not cut on the heart in any places where it would have cause the issue she as experiencing. They were going to keep a very close eye on it and normally little kids start to come out of the heart block a few hours or even a few days after. If it did not go back to normal after a week then they might have to replace the external pacemaker with an internal one.
We spent the next few hours making sure Isabel knew that we were there and watching the nurses and doctors. This surgery was the first of what will be many that she ultimately has in her life. I don’t know if I will ever get used to seeing my little girl like this but it is much better than any alternatives.
Over the weekend we had multiple doctors letting us know the different options for surgery. Jen and I decided that we would have enough time to have Addison and Mamaw PJ come spend a day or two in Denver before the surgery. This had been the longest Jen had been without being around Addison and we both missed her a lot. We were told that they were scheduling the surgery for Friday, January 10th at 7:30 am.
Mamaw PJ and Addison arrived in Denver on Monday and we were able to spend some much needed time with Addison. We were even able to go through our normal bedtime routine Monday evening. It was really nice to have something kind of normal go on for once in over a week. On our way home from lunch Tuesday afternoon we received a call from the hospital. They had a surgery get canceled on Wednesday and they needed us to get all the paperwork filled out so that Isabel’s surgery could be bumped up to that spot.
When we got to Isabel’s room there were doctors waiting for us. Each doctor that was going to be involved in the surgery came into our room and explained their part and made sure any questions we had got answered. Also, since it is a teaching hospital there were a few of the doctors asking if Isabel could be a part of their study. I have always believed that as long as the study doesn’t require any extra procedures then I am fine with it. Who knows, maybe the data they learn from Isabel would help save somebodies life or maybe even her life in the future. The last person we talked to was the surgeon. Like every surgeon I have ever talked to, he was quick and to the point. He said that he had 3 different types of procedures he might use. He wouldn’t know exactly which one he will be doing until he can see exactly what her heart looks like. He made sure to explain that one is not better than the others and that he would perform the procedure that works the best for her anatomy.
During the winter months the hospital doesn’t allow little kids in the CICU because of the risk of spreading flue and other winter time viruses. Since Isabel was going into surgery the next day, we talked to our nurse and they worked out a way that we could take Isabel out of the CICU so Addison could spend a few minutes with her sister. They allowed us to get Addison into a private waiting room just outside the CICU where she was able to see and hold her sister for a few minutes. It was wonderful and we were very thankful to the nurses for letting our family be together for a little while.
They took Isabel back around 7:30 the next morning. It was a very scary time. We loved on her for a while before and then watched them take her down the hall and through the surgery doors. It was an emotional time sitting in Isabel’s room empty room and not knowing what could happen.
The doctors were amazing at explaining what was going on. They would sit down with us and most of the time draw pictures of what they thought was going on.
The doctors were amazing at explaining what was going on. They would sit down with us and most of the time draw pictures of what they thought was going on. After the Cath the doctor gave us an exact drawing of what Isabel’s Heart looks like. Here are some of those pictures. I am also throwing in one of when Addison was able to come visit us in Denver after the Cath.
Isabel is now a week old and is getting ready to have her first major procedure done. The doctor that was going to perform the Heart Catheterization came in and discussed the details of the procedure. They took her back and we waited. It it only took about an hour but it was one of the longest hours of my life.
The procedure was successful. We now know what Isabel’s anatomy looks like. Isabel was born with Pulmonary Atresia (PA), a Ventral Septal Defect (VSD), Major Aorto-Pulmonary Collateral Arteries (MAPCAs) and a Right Aortic Arch (RAA). Holy poop thats a mouth full. Honestly, I had to ask Jen for the terms because she is so much better at remembering the technical stuff. For those of you that my not have been paying attention during “Super Specific Heart Stuff 101”, here is a quick overview of what is going on.
In Isabel’s case this means that she does not have a pulmonary valve and has a very small main pulmonary artery that does not contain any blood flow. The pulmonary artery supplies un-oxygenated blood to the lungs from the right ventricle. She does have both the right and left Pulmonary Arches that are providing some blood to the lungs but, they are being supplemented by the MAPCAs, described below, and not the Pulmonary Artery.
Ventral Septal Defect
Isabel has a hole between the right and left ventricles of her heart. Oxygenated blood is mixing with non-oxygenated blood and then being pumped through the Aorta to the rest of her body. This causes the oxygen level in her blood (~80 %) to be lower than normal (between 95-98 %).
Major Aorto-Pulmonary Collateral Arteries
This is cool and one of the reasons I absolutely love the human body. As Isabel grew her body realized that it was going to need to get blood to her lungs. It wasn’t going to get there through the pulmonary valve since she doesn’t have one, so the body created MAPCAs in order to get the blood where it needed to go. She has many small arteries that move the blood from the Aorta to the Pulmonary Arches and the lungs. These are what kept my daughter alive! Mind Blown…
Right Aortic Arch
Most people have an Aortic Arch that goes up and to the left supplying the blood to the head and arms and then it goes behind the heart and down to supply the legs and other organs like the liver and kidneys. Isabel’s actually goes up and to the right but still supplies the blood to the same parts of the body. There are people that live with a RAA and never know it.
The Next Step
Another good thing we found from the Cath was that they were able stop the Prostaglandin they had started her when we first arrived because it was not the Patent Ductus Arteriosus (PDA) that was keeping her alive like they thought when we arrived. The PDA connects the Pulmonary Artery to the Aorta while the baby is still in utero. Normally after the first few breaths the PDA closes forcing blood to the lungs. Prostaglandin’s keep the PDA open.
Now knowing Isabel’s anatomy, the doctors are able to start planning her surgery. For the next few days we are waiting to hear from the surgeon about the options and when they schedule the surgery.