Tricuspid Atresia

The entry below is from my other blog, from January 17, 2008. On that day we learned for the first time that Johnny had a challenged heart, Functional Tricuspid Atresia.

January 17

Our spirits had really been lifted from the appointment the previous week. We were optimistic. They had expressed some concern about our baby’s heart last week, now that they had a good chance to look at it since the fluid in his chest had dissipated so well. But it really didn’t seem that serious, just some words like “we can’t tell much for sure, so we will have a specialist take a look, it’s probably fine”. The woman that did the ultrasound at the last appointment was really kind and enthusiastic. Lea remembered her from our appointment when she was pregnant with Juliana. Her name was Patty. She would say things about our baby boy like “Oh, there’s his beautiful face” and “look at those beautiful feet and legs”.

It was easy to think our baby is making a comeback! Thanks be to God, a miracle! What we have been praying for! How could there now be a problem with his heart, after he had overcome so much. So we didn’t think too much about it.

The pediatric cardiologist came in and with another woman working the ultrasound, the exam began. They talked a lot during the exam, in hushed tones, working quickly when the baby was in good position for them to view what they needed to see. The doctor saying move there, over here, need to see this, and that. Then the words started to emerge, we couldn’t understand everything they were saying but words like “defect” and “missing” and “undersized chamber” began to be part of the whispered conversation. At one point I asked if everything was okay and the doctor said something about needing to work quickly, while they had a good view. So I sat down next to the bed Lea was on, and my heart began to sink. How could it be? After all this, how could there be a problem with his heart?

It seemed like the exam took over an hour. As I sat looking up at the monitor, I wondered how bad the news would be. Would he live? What were his chances? I couldn’t take my eyes off the monitor, even though it really didn’t mean much to me. It was a close-up of his heart, beating away, full of life and energy. It seemed beautiful to me.

The doctor left and they had Lea sit up. She said she would come back for us in a few minutes. We talked to the woman doing the ultrasound, she said everything else looked great and commented how well the baby was doing compared to the chart from a few weeks ago. We didn’t talk much about the heart.

The doctor came back and led us down the hall. As we were walking down the hall I became filled with dread. I knew where we were going. As you enter the hall from the reception area, there is a room off to the left, first door on the left down the hall, with a sign on the wall that says “Family Room”. Inside the room is a small loveseat sofa and chair. The furniture looks terrible. It looks like what you would find in the lobby of a Motel 6. There are two end tables that are beat up and some old lamps. There is a TV in the corner with an old VCR machine. There is only one video, it’s always there on the table – “Hoosiers”. There is a kleenex dispenser on the wall. The room seems depressing. Somehow we had avoided this room, despite the bad news back before Christmas.

She led us in the room and closed the door. Here we go, I thought. Take a deep breath. I sat on the loveseat by Lea. I said to the doctor that “it can’t be good that we are in here”. She didn’t smile. She produced a drawing of a heart. She began to tell us about our son’s heart condition. He has a condition called Tricuspid Atresia with several variations, a rare congenital heart defect. On a scale of 1 to 10, it ranks a 9 for seriousness. He will need surgery, the first one right after birth. It will be a long road. The final surgery, when he is around 2 years old, is the most complicated and serious (Fontan procedure). Some kids don’t survive one of the surgeries. Some have neurological problems from the anesthesia and having surgery when they are so young. Some kinds do quite well. If he survives, he will get tired when he is active. He will probably be limited from participating in competitive sports. He may not live past his 40’s or 50’s, although there are unknowns because the techniques of the surgery and treatment options continue to evolve.

The obvious question – is this related to the fluid problem he had on his chest? She said no, that the defect forms at 8 weeks of age, when the heart is formed. The heart defect probably occurred before the fluid problem occurred.

Lea took the news hard. At one point she said “Dave does triathlons and runs, our son will want to do those things with him.” This really broke my heart. I was thinking the same thing, but to hear Lea concerned about it was really tough.

From hear on out, they want to see us twice a week. The critical issue now is that he must go full term. Before, the plan was that if the fluid returned, they would deliver him early with chances increasing each week that he remains in the womb. Now if he were to deliver early, he would be too small to survive the first surgery he needs to live. The doctor had an ominous tone to her voice when we talked about this. She talked of the decision being a tough one that only the parents could make. God, please spare us from having to make this decision. I really can’t imagine. The fluid returns, and two difficult choices. Deliver him early to reduce the fluid but put our son at high risk to save his life through the surgery he desperately needs, or allow him to remain in the womb, knowing if the fluid does not reverse again, he will probably die of heart failure before delivery. God, please let him go full term!

The appointment ended. It’s unbelievable to me how with many of the appointments we seem to leave on an extreme high or low. There appears to be no middle ground. We were there for about 4 hours again. Always a long appointment. We left somewhat in shock. Another ice cold day outside, around zero F, to fit my mood. Mom and Dad were home taking care of Juliana. We got home and I brought them into another room to tell them the news, so Juliana would not hear it. It was tough. The news was still sinking in even as I shared the news. I just got off the internet, learning more about the heart problem our baby boy has. It’s tough to read this info and think about what our future holds. The journey down this long road continues.