The visit to Boston went well. Jack did great on the airplane. He has decide that private jets are the only way to travel and he never wants to ride coach!! Uncle Ed even put one of the seats down so we could use it as our changing table.
Meeting Dr. Breitbart was awesome. He was as nice and thoughtful in person as he has been via email and phone. The day of our appointment started out rough. We had orders that Jack had to stop eating by 8:30 am (formula), 10:30 am (breast milk) and could not sleep past noon for our 2 pm appointment. You have all seen my son...he loves to eat!!! So, this was no walk in the park. He screamed a lot!!! This child has never been deprived of food a day in his life, so he was very confused as to why I was just watching and holding him instead of feeding him. I cried off and on the whole morning...it was very hard listening and watching your child cry, knowing how to fix it, but not being allowed to do anything about it. We found the hospital easily, thanks to Aunt Suzan, who had worked at the hospital many years ago. It was clinic day for the cardiology department, so it was a mad house inside the office. It was standing room only...with a lot of sick little kiddos. Once we got back to our room, Jack had to be weighted and measured so the nurse could know exactly how much medicine to give him to make him sleep. He weighted in at 16 pounds 5 ounces, so this meant a good amount of medicine. The nurse told us of the risk before, during and after the sedation. We had to sign a bunch of papers...and the process started. I had to have Brian hold his hands while the nurse administered the chloral hydrate into his mouth. I, of course, was crying and Aunt Suzan was hugging me during the whole procedure. The sweet nurse told us that it could take up to 20 minutes to take effect. Not for my son...the child who was food and sleep deprived fell off to la, la land in under 2 minutes!!! I just held him as he fell asleep in his little hospital gown. I then had to lay him down on the hospital bed so they could begin the sedated echocardiogram. Brian, Aunt Suzan, and I got to be in the room the whole time the echocardiogram was being performed. The tech worked for 1.5 hours getting picture after picture, measuring arteries and veins, and watching each chamber pump. It really is quite amazing how modern technology works!!! During the procedure, Jack oxygen level dropped to 79%...the nurse quietly turned on the supplemental oxygen and laid it in front of his nose and it immediately went up. Later she told us that this is completely normal because the tech has to push down on the stomach so hard to get different angles for the pictures that it affect the way babies breathe and their oxygen levels. So, after the long procedure...Dr. Breitbart came into the room to go over the findings.
First he started by saying that Jack is perfectly “balanced” right now. He is very pleased at where he is right now and could say that he has never seen a kiddo with his condition doing this well. He confirmed that Jack has double inlet single ventricle with pulmonary stenosis along with L-TGA (self corrected transposition of the great arteries). This means that his right ventricle on the left side is not large enough to be considered a working ventricle, so his heart is working as one (or single) ventricle. This diagnosis rules out any possibility of a double switch procedure. So, the surgery that will be performed on Jack will be the modified Fontan procedure. The next step, Dr. Breitbart wants to take is to have a cardiac catheterization done next month to measure the pressure in his pulmonary artery. From the echocardiogram, the pulmonary artery seems to be in perfect balance with the stenosis. Dr. Breitbart said any more narrowed or wider and he would not be “balanced”. He wants to measure the pressure of the artery because this will determine if Jack has to have the Glenn procedure (stage 2 out of 3). If the pressure reads too high or too low then Jack will have the Glenn procedure around 6 months of age. The Glenn procedure is done to give a child’s body more time to grow and develop while they wait to have the Fontan procedure. In the Glenn procedure, they will connect the superior vena cava to the pulmonary artery. By doing this, pressure is taken off of the single ventricle to pump enough oxygen poor blood to the lungs. As I have mentioned before, the problem with Jack’s condition is the fact that the single ventricle gives out over time. Well, the doctors do not want any damage to his single ventricle before Jack is 2 years old and can have the Fontan procedure so the Glenn procedure is done as a preventative to protect the single ventricle. So, we will know after the cardiac catheterization if he will need his first surgery, the Glenn procedure, or not. Dr. Breitbart feels like the cardiac catheterization is going to reveal that his pulmonary artery is in perfect balance, but he wants to be for sure.
One great thing we learned from the visit is that Dr. Breitbart and Dr. Meyer (the surgeon) perform the modified Fontan operation in a different way then Dr. Pearce had told us. There are two ways to perform the modified Fontan operation...the lateral atrial tunnel Fontan and the extracardiac conduit Fontan. In both methods a total cavopulmonary connection is created, but by different means. With the lateral tunnel a baffle is placed in the right atrium to partition systemic from pulmonary venous blood. Because this partition comprises less than half the circumference of the pathway from inferior vena cava to pulmonary artery, the remaining native atrial tissue can grow to accommodate increased systemic venous return over time. The growth potential makes this type of Fontan operation suitable for smaller children without the need for future enlargement of the pathway. In the extracardiac conduit type of Fontan, one end of a synthetic tube graft is connected to the inferior vena cava and the other end to the pulmonary artery confluence. This pathway has obvious size limitations since the graft cannot grow. Brian and I were told that UAB would only do the conduit type and I was unaware of any other way to perform the Fontan. So, I am very excited about the lateral atrial tunnel approach!!!
Brian and I are so thankful that we got a second opinion and that we are going to Boston!! All and all the trip was very informative and I am so glad we went and had Aunt Suzan with us. We could not have made it through the day without her love and support every minute of the long procedure!!! It was very emotionally hard on me...it was so hard seeing my little, precious boy lying lifeless on the hospital bed with his little hospital gown on. I cried a lot. I think I cried more because of what is to come for him...I wish this one test was all he had to endure, but this is just the beginning...and that is hard to take as a mother. My heart breaks at the thought of all the future tests and surgeries his little, perfect body is going to have to go through. I do gain peace of mind knowing that he will be in good hands and that he is doing so well right now. I still pray every night that Jack will glorify God and He will use him for His glory!! But I am very selfish, though, and I want a miracle so he does not have to endure the pain of any surgery, the medication that will follow, and the lifetime of doctor appointments...and the scars that will remind him everyday that his perfect body has an imperfect heart.
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