We were overwhelmed after diagnosis. Many tears were shed, but we were determined to see the course through and be there for our child every step of the way. At 27 weeks along Ethan developed further complications called Pleural Effusions (fluid around the lungs). We were told he may not survive. After being closely followed Ethan was carried to full term and his effusions were very small. He was born in February. He needed no breathing assistance at birth and looked like a beautiful healthy baby. And from birth everyone talked about his amazing eyes. He could focus on you since the day he was born. His eyes showed so much expression and were the window to his soul. They showed his determination.
At 6 days old he had his first open heart surgery. The Norwood procedure with a Sano shunt. The Sano shunt was used instead of a BT shunt. Ethan had a surprise in surgery. He had a right sided aortic arch that went a did a full wrap (or ring) around his esophagus. Most rings are formed from one of the arteries that branch off the aorta. But his ring was his actual main aorta. Ethan already required extensive repair of his aorta from the HLHS, the ring made it a very complicated Norwood Procedure. He ended up with a trache from vocal cord paralysis from the aortic ring repair. We were told it would only be for a few months until the nerves healed and his vocal chords began to work. He also got a G-tube for feeding because they would not let him drink by mouth. There is high risk to aspirate formula into the lungs when his trachea and esophagus were healing from surgery.
Shortly after surgery his pleural effusions returned full force. Ethan endured several chest tubes to drain the fluid. Pleural effusions are a common complication from heart surgery, especially for single ventricles (half heart babies). However his problem was greater than most, and occurred even in utero, before surgery. They referred to Ethan as having “effusions of unknown origin”. Ethan also had wet lungs. His lungs absorbed too much. One philosophy behind his lung problems/effusions is that his PFO in utero was EXTREMELY small. Thus the pressures on the right side of his heart were too high and that puts high pressures on the lungs. They feel his lungs went “bad” in utero from this. Finally they put Ethan on a super nasty formula called Portagen (grateful for the G-tube at this point) that had no fat. They combined that with LOTS of diuretics to keep his lungs dry! His effusions finally got under control and he was able to come home at 9 weeks.
The first 2 weeks home were very stressful with his trache. I was denied home nursing care because he was too “fragile”. They wanted to send Ethan to Los Ninos, a nursing care center for children. But being a nurse, I would have nothing to do with that. I wanted him home with his family. Spending 9 weeks hardly seeing my other 3 children made me want to be home with him even more. Home he came and my kids just loved on him.
At 11 weeks of age he was able to have his trache removed after he had a heart cath. His vocal cords were working again. They made us stay a few days to watch how he did with no trache and then we headed home. We LOVED having him home the next couple of weeks. He was still hard to balance. Some days he required extra diuretics when his oxygenation would get too low. It was definitely a balancing act with him. But he thrived so much just in those few weeks. He was smiling, playing with toys, and loving his siblings. The bad news from his heart catheterization is that his Sano shunt was indeed narrowing. If his shunt were to close off he would die. Yet his next open heart surgery, the Glenn, would re-plumb blood directly to his lungs thus putting his already fragile lungs under higher pressure. We were walking a fine line and every one knew it.
On June 2, when Ethan was 3 ½ months old, we went ahead with his second open heart surgery, the Glenn procedure. He did well at first, everyone was surprised at how well he was tolerating the new circulation. He did get pleural effusions, but that was expected of Ethan. He was playing with toys and giving sheepish grins at 6 days post-op. However things quickly changed at about day 10. One of his chest tube sites got an infection and his heart grew collateral veins to relieve some of the new pressure. His pleural effusions became worse than ever. In a matter of 24 hours Ethan was clinging to life. He was re-intubated and his oxygenation was in the mid 60’s. He started putting out over 700ml of chylous fluid a day out of his chest tubes. That let to blood clots and even more complications. And of course more and more chest tubes as new locations needed to be reached or old tubes clogged.
Ethan became a balancing act for the next 6 weeks. I have never prayed so hard in my life. I felt complete despair at my childs suffereing, yet complete beauty and peace as I knew he was being closely watched over. I wish I could describe it better. But it’s too personal, so tangible to feel, but not possible to explain.
My one and only regret in Ethan’s care came at the end of July. He was finally making progress to be weaned off the ventilator. He seemed to be doing better, yet he started having fevers. Infectious disease was stumped. Their only explanation became that maybe his body was reacting to having so many IV lines and meds or reacting to the damage that occurred to his lungs during this period. Dr. Cleveland approached me and said it is time to trache him again. Despite his progress he had a LONG road ahead. I said no. I asked if we could just give him a chance to breath on his own. They agreed to try. It was not a good choice. He did breath on his own. And after he went through the withdrawls (he had been under sedation for nearly the whole 6 weeks), he was even smiling again. I was soooo happy. I thought he did it. . . He fought back. He is going to come home.
The good days were very few and soon he became very fussy and skinny! They had to increase his diuretics to keep his lungs dry enough so they wouldn’t have to reintubate. I think they tried too hard to keep from putting him back on the ventilator. By this point everyone was in Ethan’s cheering section. All the doctors, nurses, and respiratory team wanted him to stay off the breathing machine. But one night he stopped being able to oxygenate well enough. He was re-intubated and many more test were run. Ethan’s echo’s showed his heart was functioning properly, but a blood test called BNP that determines heart failure came back at over 10,000. BNP is the chemical your heart produces when it is stressed or in failure. The number should be below 100. This chemical, as well as all the diuretics caused him to go into kidney failure as well. The BNP was most likely the cause of his fevers this whole time.
The team got him stable and then Dr. Cleveland came in to give us a talk. Ethan was too hard to balance. There was nothing they could do but try to get him more stable and then he would need a heart transplant, and most likely a heart and lung transplant. Heart and lung are very hard to come by and don’t have nearly the success of just a heart. I was devastated. I had researched this before birth. Combining that with being a nurse made me walk through my own personal hell for the next few weeks. I knew my child had suffered so much, and still was not even close to being out of the woods.
I began to realize that despite the amazing advances in medical care, that Ethan’s life was not ever going to be one with much quality. I was fine with delays or handicaps, as long as he could be home. But he was a constant balancing act. I finally realized what they told me when he was diagnosed in utero. He was a VERY SEVERE HLHS. I love all my children and only want what’s best for them. At this point I started to realize that there were worse things than death. . . and my Ethan was living it. Even the look in his eyes were changing. The fire was fading. He had endured so much.
My husband and I were ready to move forward and try for transplant. We would have to move out of state because St Joseph’s was not Transplant Certified at this time. But I was also ready to move forward on another very important decision. . . I wanted to make my child a NO CODE, or DNR (do not resuscitate). My husband wasn’t ready for that though. We have strong religious beliefs and know there is life beyond the grave. We KNOW we will be with Ethan after this life. But my husband felt if we made that decision it was like we were giving up on Ethan.
Over the next few weeks Ethan received another tracheotomy and swelled up to the size of the Pillsbury dough boy since they had to take him off his diuretics to let his kidneys recover. His fevers were real bad. More bad news kept coming. His initial blood work ups for Transplant showed he had a rejection factor in the 90th percentile. There was also initial talk that he may not even be a transplant candidate. We did all we could to comfort our sweet baby. He held our hands/fingers, we rubbed his beautiful head, and we sang to him. He LOVED it when we sang to him. Since the Glenn surgery I can count about 10 times that my husband and I were able to hold him. So finger holding and singing became our bonding time with Ethan.
I approached my husband again about making him a DNR. We decided to make him a “short code”. We told them if he were to need full resuscitation that we would want them to try for 10-15 minutes and then let him go if he wasn’t responding by then. Less than a week after this decision a middle of the night phone call came. My husband had been with him and left just before midnight. Ethan was doing great so daddy decided to come get some sleep in his own bed. An hour after dad left, Ethan left. A short code was unsuccessful. Ethan’s milrinone (IV heart failure med) had been turned off that evening. He was just on an oral one now. His ventilator was switched to auto mode (meaning he was breathing on his own). They were going to take him off the ventilator in the morning. With his fragile heart and lungs this deemed too much change for him. He got a little bit off. He had been way worse at other times. But not this time. Just a little bit off. Like I said, he was ready. And more importantly he knew mom and dad were finally going to be okay with his exit. Not all will be able to choose their time to leave this mortality, but Ethan chose his time.
It would not be fair to end this story without acknowledging the amazing outpouring of support our family, and Ethan, received through this journey. We came to know so many wonderful people in the heart world. And our friends before this journey began, have become like family. We were shown love and service beyond measure. I still shed tears almost daily for Ethan. Some are tears of joy, and many for sorrow. But we are so grateful for the six months he gave us.
