|Posted by Sherri Viner on April 30, 2015 at 9:25 PM|
We went to meet the surgeon today along with the neonatologist. The day started out great, Alexander was in good spirits, weather was cooperating with no rain in sight and loads of sunshine, and Jimmy was able to spend the morning with us before we had to go to our appointments. Once we got there, that was a bit of another story.
We checked into the cardiology department and I was anxious to meet the surgeon. As we were waiting, the toys in the waiting room were too alluring for Alexander and he had to go play with them. Jimmy let him loose and let him out of his stroller. Yikes- I just kept thinking what a bad idea this was. How were we ever going to get him back into the stroller when we were called because I know that he will not like the fact that we have to pull him away from his new beloved toys? I played it out in my mind, it was going to be a disaster and sure enough that’s when all the craziness began.
We strapped him in as he was protesting all the while. Some children have almost a cute little whine... If you can imagine the world’s largest set of lungs and then imagine the world’s loudest screams coming out of those lungs you might get an idea of what we were up against. I thought his behavior was deplorable at our previous ultrasound; this was something on a whole new level. I could have sworn the devil possessed our sweet little boy for about 3 hours today.
We pushed him into the room where Dr. Haw was waiting for us. Right from the beginning Alexander decided to introduce himself in a very expressive way and demanded he get out of his stroller. I reluctantly gave in as a means to try to calm him so that we’d be able to go on with our meeting. Mistake #2! Alexander decided to do his I’m made of jello and collapses his body in such a way so that I couldn’t pick him up, it’s so frustrating.
Dr. Haw was just as I expected. He was very pleasant, soft spoken, and made us feel very comfortable. He was really understanding of the fact that we were both struggling to keep our toddler from destroying the entire room. It couldn’t have been easy for him. Trying to make sure we understood what to expect by diagraming out and explaining some very complicated procedures with a little one throwing the longest tantrum in history.
We discussed the 3 surgery set again which ultimately leads to the Fontan circulation. He went into further details of the other defects that were seen in the ultrasound along with some of the issues that we could face at each surgical step. It was also the first time that right atrial isomerism was mentioned. It brought me back to the day we first heard of heterotaxy because I had read about right and left isomerism. I just remember thinking then that I was hoping it wouldn’t be right. Everything I read said that right atrial isomerism had very poor prognosis and mortality rates were really high. So when the doctor brought that up it was a bit of a shock to the system. This poor little guy can’t seem to get a break and he isn’t even born yet.
The above picture is the findings from the April 16 Ultrasound- I’m no medical expert but I tried to explain from what I know in the parentheses
1. Heterotaxy syndrome, findings consistent with right atrial isomerism
2. Discordant cardiac apex and the stomach; levocardia with stomach on the right (Heart is on the correct side of the body but the related structures are not- in our case the stomach presents on the right)
3. Left AV valve atresia (The atrioventricular connections are abnormal as in closed or absent)
4. Sever hypoplasia of the left-sided ventricle (underdevelopment or incomplete development of the left ventricle)
5. Great vessels transposed; aorta anterior, arising from the main ventricle (The two major vessels that carry blood away from the heart -- the aorta and the pulmonary artery -- are switched.
6. Pulmonary atresia with ductal supply of the confluent branch pulmonary arteries (the valve that lets blood out of the heart to go to baby's lungs isn’t form correctly. Blood from the baby's heart can't go back to the lungs to pick up oxygen.)
7. Left aortic arch (ventricular atrial connections are abnormal. Aortic arch is leftward based on position of trachea with branching anomalies)
8. Bilateral superior vena cavae (the SVC returns the blood from upper part of body drains into the right atrium)
9. Pulmonary venous connections are not determines (Normally, pulmonary venous return carries oxygenated blood from the lungs to the left atrium where it can then be pumped to the rest of the body)
10. Normal venous flow profiles, no evidence of significantly altered fetal circulation
The biggest variable in all of this is of course Nicholas. Nothing will be confirmed until he is actually born and they are able to do scans on him. Things could be oriented in a manner that the doctors hadn’t expected and things could show up from complications of heterotaxy like malrotation of intestines. It’s too difficult to tell everything in utero, but with that said I’m so amazed and thankful of what they can identify. We are so much better prepared and able to get the appropriate medical professionals assembled for Nicholas’ care.
We were finished with Dr. Haw. He was so awesomely patient with us during our appointment and I felt relieved for him that we were going to leave! Of course as we got Alexander in his stroller and walked out of the room you would never know he just had a 1 ½ hour long tantrum.
One more appointment to go, but by this time Jimmy and I were both exhausted. We got to the area for our second appointment and were set into a conference room where we would meet our social worker person and the Neonatologist. Luckily this was more of a short and sweet type of meeting. We got all of our questions answered and the visit was really so that we could get a good picture of the events following Nicholas’ birth. What would happen to him, where he would go, who would care for him, how would they care for him, etc. It’s a real team effort among the neonatologists and nurses and I felt pretty good about it all. The social worker will be our main contact from the time of birth through Nicholas’ discharge.
The really nice thing is that all the different parts of the hospital are linked. So from the delivery area to the NICU would literally take about 2 minutes to get to. We went on a tour of the NICU areas. That part was when I started to feel that familiar sadness set back in. I was hoping I wouldn’t see any babies in there but of course I did and it made me almost lose it. That will be our little one and where we will live for a while. Everything up to this point was theoretical, but in that place - in that moment, everything was so real and that was hard to handle.
We finally left the hospital and Alexander fell asleep almost as soon as he was placed in his car seat. I left feeling low, like the first time we found out something was wrong with Nicholas. I was a little surprised I found myself back there again but I’m sure I’ll be going back and forth on this emotional rollercoaster many more times.
Our objective at this point now is to go full term, throwing a premature baby on top of everything else could further complicate matters. So despite the fact that I’m only 6 months pregnant and look like I’ve swallowed a semi-truck, I’ll gladly slap a wide load sticker on my back and call it a day! Our next doctor’s appointment is May 21st where we’ll be getting another echocardiogram so that the cardiologist can take another look along with some of the normal pregnancy things like the glucose test.
Categories: April 2015