Written by Melissa, Lucy's mom
We found out at my 20 week ultrasound that Lucy had a heart defect. My OB noticed it on the ultrasound so she sent us to a cardiologist in St. Cloud who did an echo and confirmed that Lucy had Tricuspid Atresia with a hypo plastic right heart (read about that here). We had then started going to the U of M to see the fetal cardiologists. They had a plan for us to be induced one week early so that they could have the doctors on staff that Lucy would need. Lucy was born September 12, 2011 at 7:49 she was 10 lbs 5oz and 20.5 inches. It was a long labor that ended in a c-section due to her size.
Lucy was brought to the NICU immediately so that they could do an echo to confirm what they saw prenatally. Things were as they believed. They waited a couple of days for her PDA to close to see how her heart would circulate her blood. Things seems fairly well so they decided they could do the Rashkind procedure (A cardiologist threads a catheter through the belly button or a vein in the leg, to the heart, and creates a hole between the upper chambers. The hole allows oxygenated blood from the left side of the heart and deoxygenated blood from the right side to mix, increasing the amount of oxygen in the blood pumped to the body) instead of having to put in a shunt. This was a good thing since that meant not having to open her chest. After a few days she seemed to be doing very well. She was eating like she should and her oxygen was in the high 80's to low 90's which was wonderful. They had moved us to another room because they had planned on sending us home.
Lucy started to not eat as much which they thought was ok due to what she had gone through with the Rashkind procedure. They told us she could not go home until she ate a certain amount every 4 hours. On the 20th we had gone home for the night when we got a call at 4am that her oxygen was dropping dramatically into the 40%. They were giving her meds to keep it up. They ended up putting in a central pick line to get meds directly to her heart. This took hours because they could not get it in they finally had to take her to radiology to get it done. After another day and no improvements they move her to the PICU so they could watch her better. On the 22nd her pressures started to drop dramatically. It was like a circus in her room trying to give her meds to keep it up. At about 11pm that night the surgeon came in saying our only option was ECMO and it was very risking because she was in heart failure and very sick with only half of a heart. We signed the papers and we watched them take her away not knowing if she would make it back. She got back around 1 am and things went well. The doctors were not sure why she went into heart failure. We had multiple doctors coming and going looking at her and doing tests. They sent her tests to the children's hospital in Cincinnati for their input as to what they thought may be wrong. They were not sure if it was cardiomyopathy or myocarditis. Lucy was on ECMO for about a week and things were not really changing they decided to try the Berlin heart which would be another risky procedure due to how sick she was and that she was on ECMO. They put in the Berlin heart and things went well during the surgery but she only had it for less then a day. Since she had only half a heart they had to also put a shunt in for the Berlin heart to work and place it in differently then they would normally and it did not work for her. Lucy went back onto ECMO. They told us she would have to be put on the transplant list immediately as a 1A recipient in order to have any chance but even with a transplant they told us she had a very low percentage that should would make it thorough because she was so sick. At this point Lucy still had the doctors unsure as to what was wrong with the left side of her heart. Lucy went on the transplant list September 28th. They had attempted to close her chest on the 6th which would be the first time since the 22nd. They did get her chest closed but she was bleeding more then she ever had. They did not know if she would make it through the night. At 1pm our surgeon Dr. Bryant called me to say they had a heart!! We were so excited but they told us getting her through this was not going to be easy. She got her new heart October 7th. She made it through the surgery great but when they tried to taking her off of bypass her pressures kept dropping again. They tried 3 different times for at least 4 hours and finally they had to put her back on ECMO. They believe that the heart was good but was on the small side and Lucy was so sick that it could not catch up. They kept her on ECMO for a week then they tried taking her off and the first time it did not work so they tried again two days later and she was able to do it on her own. Now the problem was her kidneys took a hit while being on bypass so she was full of fluid and was not peeing so they put her on dialysis. So they had to put her through another procedure to start the dialysis. They would fill her with the fluid and let it sit for 30 min then drain the fluid. Every time they would empty the fluid her pressures would drop. So they had to stop the dialysis for a couple of days and try it again. Once they started again it worked better and did not seem as hard on her. They were getting rid of some of the fluid but still not enough to close her chest. On October 19th they told us her liver tests were coming back extremely elevated so they were going to be watching it more closely. So now it was her heart, kidney's, and liver. October 20th I was waiting for the tests to come back to see if it had gotten better. One test stayed the same and one was a little better. This was also my husband's and mine 10th anniversary so after we got the results back we decided to go out to eat. When we got back Lucy was doing fairly well just going through her dialysis. She was starting to have some urine too so things seem to be looking good. But at about 10:15 when they were draining the fluid her pressures dropped as they had been but they always went back up right away. This time they dropped and did not seem to be going up as normal. They gave her meds and some blood products and it went back up for about a second but i stood there watching the monitors as her pressure suddenly dropped in half and never went back up. They hit they code blue and the room instantly filled up. They worked on he for about 45 min and she never came back. The surgeon told us that they could try ECMO one more time but he gave it about a 1% chance to work since she had already been on it 3 times and her chest had been open for over 4 weeks so anything we did not would be for us and not for Lucy anymore. He also told us that because her oxygen had dropped so low for a long time that she may have brain damage if she were to even make it through. My husband wanted to be the one to tell the nurses to stop so we went back into the room and he asked them to all stop. She never came back to us. It was the hardest thing we ever had to do.. we always knew this could happen but always before she was having another procedure not just what seemed out of the blue. I miss her so much. We only were able to hold her that first week but she was able to hold our hand and she would squeeze our hand every time we held it. She would always open her eyes and move her head when she heard me it was wonderful. The nurses loved her and I think the whole staff was amazed at her strength and how long she actually hung on. She was a fighter!!
They did not find out what was wrong with her native heart till after it was removed for the transplant. They discovered it was Hypertrophic cardiomyopathy (. Due to Lucy being born with only half a heart it put to much pressure on the other side which caused the heart failure to progress so quickly.
I miss her and love her so much but am thankful for the time we had and glad she no longer is suffering.