Zann at 5 weeks, post COA repair

Our son was born at home with a midwife on the evening of April 27th, 2007. He had a strong cry and took to nursing immediately. He weighed 8 pounds 5 ounces and was 21 inches tall. We thought all was ok. Sure, his hands and feet would get a bit blue from time to time but when we put socks on him it went away. Sometimes his breathing seemed fast, but I would hold him and it would slow. Besides he has a pediatrician appointment soon, so I will just ask about it then. He held his head up for the first time at two days! He just felt so strong.

Then on May 8th everything changed. The day before he had stopped eating well and was gassy, but we were all getting over a stomach bug and thought he had it too. It was early morning and time to nurse him again and I could hear him making what I thought were sucking sounds. His face looked a little funny, but when I opened his pajamas, his entire torso was purple. His legs and arms were too. He would open his eyes but only briefly. I thought he must be severely dehydrated from being off his feed and took him into the hospital. As soon as the triage nurse saw him she rushed him back and told me to go to the insurance window. I heard them page and pediatrician to the ER STAT and when the lady at the desk didn't even bother to copy my insurance cards I knew I was in trouble.

I went back and there were two doctors and three nurses working on my son. He was on oxygen but still breathing for himself. His first blood glucose came back 4. His rectal temp was 95 degrees. Then 93 and the nurse said. "We're losing ground". I sat in the chair in the corner and just about passed out from the shock. His pediatrician was there and she explained that she thought he might be septic and was going to do cultures and a spinal tap and that his liver was very swollen. I said ok, do whatever you have to. They came in and took a chest X-ray to check for pneumonia. Then she did the spinal tap. I called my husband to come up to the hospital as he was home with our two year-old. We only have one car but my brother-in-law drove him up. He didn't realize how sick Zann was. My pastor is an oncall chaplain at the hospital and he came to be with me.

The x-ray tech came and got my ped. Moments later the ped walked in and said that Zann's heart was enlarged and she needed to send him out to UAB. I said ok, and had no idea what UAB was or where it is as we are an Air Force family and not from the area. She said she thought he had a heart defect but couldn't tell me anything more. My husband still wasn't here at this point. I called my brother-in-law's cell phone and they were on the way. Josh said they had to stop somewhere first and I told him there was no time, there is something wrong with our baby's heart and get up here NOW!

We waited several hours for the flight team. The staff at the ER told us to go pack our bags and get something to eat. I reluctantly agreed as our pastor was there to stay with the baby and he was stable by this point. We ran home, ransacked our house, and I grabbed my pump. We then ran to the commissary and BX and bought a cooler for my milk and two sandwiches because it was almost lunchtime and we had not eaten for the day. When we got back to the hospital, the transport team had arrived.

The nurse was very nice and said they had to stabilize him before they could make the flight. They put him on a ventilator. His bicarb was eight and it had to be brought up before transfer. She gave me a picture of the plane they were going to fly him to Birmingham in and told me she would call me when they had landed and things settled down. When they left we left and drove the two hours to Birmingham, speeding just a little.

As we are entering Birmingham the nurse called and said that he had made the flight safely and that the cardiologist were already looking him over. We arrived at the hospital shortly after that and they already had his main diagnosis, coarctation of the aorta. He would need surgery and the next forty-eight hours were going to be shaky as to whether or not he would survive. We were told he might have brain, kidney, or intestinal damage from the prolonged period of acidosis. He is not brain damaged but he does have some kidney and intestinal damage that he should grow out of. We spent the next few days on the phone and at his bedside. My daughter stayed with my sister and we had never been apart from her so it was hard for us all.

On Friday May 11th Zann had his coarc repaired. The surgeon did a wonderful job but was not able to get to all the narrowing without putting him on bypass; however what he did repair seems to be enough. His coarc was odd because it would narrow and balloon and basically does this the whole way down. By Saturday he was on O2 via nasal cannula and Sunday afternoon he was transferred off the CICU and back to the NICU. Later in the week we resumed nursing and after some weight gain he was able to come home the following Saturday. His only medication is Lasix, 8mg twice per day.

In June we went back to the cardiologist and were told that although the coarc repair is looking wonderful, his left side is not. His right side measures 13mm, the left only 5. On top of that, his VSD blows over into the left side, further obstructing blood flow. We have decided to watch it for now but if it does not grow by the end of the summer he will have to have surgery. It is not for sure but it would probably be all the procedures leading up to the Fontan. We are really hoping for growth, because we know that, as it is, it is placing to much strain on his heart at 5mm. We tried to wean him off Lasix, cutting back to 4mg twice per day, but he quickly developed respiratory distress.

So that is us.