SuperEvan
May 24, 2000 started out like any other day toward the end of my first pregnancy. When I went into labor and left for the hospital, I distinctly remember thinking that I hoped I'd packed enough items to last 2 or 3 days in the hospital after birth (especially since I was only packing trial-size items that would be tossed after the stay so we wouldn't have to take as much home with us). That was my biggest concern going into the birth of my first-born child. The next day, after a long labor and no result, I had a c-section and my first child was born. He was 8 pounds, 10 ounces, and "perfect" (that's the word the pediatrician used).
Things were otherwise fairly normal for our first few days. I questioned his heavy sweating and breathing, and some other oddities. Each time I was told that this was completely normal newborn behavior. Not having any other children to compare to, I assumed what I was being told was true. Sure, I had a stepson, but he was 5 when I came into his life. I had no idea whether what we were seeing was "normal" and had to trust what I considered to be the medical experts. The morning of Evan's 3rd day of life, we were told that we could go home that day. It was 7:45 a.m. and we could leave at 10 (after shift changes) with our "perfect" baby. The nurse who brought my breakfast also took the small bassinet that Evan lay in. She was going to take him to the nursery to get him ready to go home. Thank goodness she did, because he coded in the hallway on the way to the nursery. Had he been in my room still, he would have died quietly in the bassinet and I'd not have known what was going on. As it was, she was walking in the hallway with him when he turned blue. This was a blessing that she was there, and that she'd taken him. His heart had completely stopped beating, and he was no longer breathing.
Evan was rushed into the nursery where medications were given to re-open a duct in his heart, and life-support measures were taken. A team from a children's hospital an hour away was brought up to look at him (he was far too fragile to transport). My "perfect" baby was born with an imperfect heart. We had no idea. Every ultrasound looked great. Every doctor visit went well. He was a planned baby! I took prenatal vitamins and we took every precaution during the pregnancy. We worked hard to do everything "right" for a perfectly healthy baby. How did this happen?!
The team from the children's hospital worked for over 4 hours. They took 3 polaroid photos of Evan, thinking these would be our last pictures of him "alive". In them, he is gray in color, his eyes just open enough to see that they'd rolled back in his head, and he is covered in machines, wires, and tubes. He hardly looks "alive" at all. He most certainly doesn't look like the baby that had slept in my arms for his first few nights, snuggling and seemingly healthy. After several more hours at the children's hospital, my husband (David) and I were brought into a room, away from our family who had, by that time, gathered. In the room was a cardiologist, a resident, and a nurse. They each sat in chairs across from us with very serious faces. David and I sat on a couch, next to a box of tissues. Sadly, this was likely not the first time they'd faced this scenario. They were ready for us.
We were shown a diagram of a normal heart. We were told all the parts, and the functions of each one. Then the doctor got out a diagram of a very different heart. He added some other changes with his pen, and then showed us the drawing. He laid it on a table to my left, and asked us to point out what we noticed was different. As we pointed to all the differences (how could a heart even LOOK like that?), he named each defect, explained what it was, and what it meant for our son. Evan had (among several other heart defects) narrowed transverse aortic arch, and critical valvar aortic stenosis. I wanted so badly for them to take my own heart out right then and give it to my son. I'd lived for 25 years and that would be plenty, just give my baby a chance to live. They cannot do this, so soon, they took us in to see our baby again. This might be the last time, we knew, so he was baptized immediately. Then, off to another, much larger, children's hospital he would go. Another ambulance ride, on only his 3rd day of life. What an eventful day it was. The nurse who'd been with us for that first consultation, for the diagnosis... she whispered in my ear before leaving to follow the ambulance (we weren't allowed to ride along), that no one could take away the memories of the 3 days I had with my son. I knew then that no one thought he was going to make it for yet another ride, to another hospital. But they were going to try. I remember thinking that I'd never heard of heart defects like this. I had no idea this could happen to a child just born, that their heart could be so distorted. I thought it must be so RARE! I had no idea that CHDs were actually the most common birth defect, and the most common birth-defect related death.
The entire way to the next hospital, I heard the nurse's words to me. She KNEW he wouldn't make it. I was beyond devastated. David, thankfully, never lost hope. We arrived at the second children's hospital, 2 hours from home at that point, late in the evening. We weren't told yet if he'd survived the trip, and it would be almost another hour before we would know. Evan, whose name is Welsh and means "young warrior", lived up to his name. He fought hard and made it, just barely, to get to the second children's hospital (his 3rd hospital in one day). He was very, very critical, however. We would not be allowed to hold him for many days. We were given a small room to sleep in for the night, and were awoken at 6:30 the next morning to sign permission for his first heart surgery. They were going to balloon Evan's aortic valve to try to get more blood flow to his body. We were told that Evan would be in the hospital for weeks, and probably even months. He had gotten so sick that it was likely he would require kidney dialysis after his heart healed. True to his name, however, Evan recovered well and was sent home just 2 1/2 weeks later (no kidney dialysis necessary).
When we got him home, we had to feed him high-calorie formula (added to what I was pumping for him, initially). What he was too exhausted to take by mouth, he had to take by naso-gastric tube (NG tube). It was a small tube that went into his nose and down to his stomach. Before every feeding, we had to use a stethoscope and syringe to be sure the tube was in his stomach and not his lungs (so we didn't drown him, we were warned). When he pulled the tube out, we had to place another one. "Do-it-yourself nursing" we called it. We didn't care, though, we were just glad to have our baby home! Things were relatively normal for the first couple of months. We had cardiology visits weekly, then bi-weekly, but still most days were about as normal as they come in the world of Congenital Heart Defects (CHD's -- Congenital meaning "born with").
When Evan was 3 months old, he had to have his first procedure repeated. He was losing weight and not getting enough blood flow to his body again. This time, the procedure failed completely. Before he was even out of the procedure room, we were told that he'd be having open-heart surgery (the one they wanted to do when he was 20 years old) the following month. He would be just 4 1/2 months old. There is no way, really, to prepare yourself to hand your child over for open-heart surgery. I washed his chest with the special soap the night before surgery. I remember looking at his tiny chest thinking it would never look the same. If he even made it through the surgery. I hadn't really noticed it at all, but he had gotten so skinny that one could immediately tell he was sick, just by looking. It happened so gradually, though, that I didn't see it until much later, looking back at pictures.
The day of the open-heart surgery came, and eventually we had to hand our son over. They would removed a very badly defective aortic valve, replace it with another of his own valves (his pulmonary), and then put a new donor valve in place of the pulmonary valve they had moved. This was a very complicated surgery, involving so many steps (and there was actually another step, two procedures in one -- the Ross-Konno Procedure it was called when done together). Two surgeons were present, and we knew that if he had to have this done, he was in the best possible hands. Still, it was so difficult to hand him off to the nurses, never knowing if we'd see him breathing again. Toward the end of surgery, it was time to start taking Evan off the heart-lung bypass machine. This is a machine that pumps all blood and oxygen for a patient while the doctors work on their heart. Evan had already been on this machine more than twice as long as they want anyone (of any age, any health status) on it, but his surgery was so involved that it was necessary. And yet, he was having difficulty coming off of it. What this meant is that he might have to go on ECMO -- a longer-term (2 weeks at most) life support that would do the same thing. Only 10% of people who go on ECMO live to get off of it. Thankfully, Evan somehow got better and eventually came through the surgery with flying colors. An expected "2 or 3 week" stay ended up being just 6 days. Our "young warrior" had done it again!
When he was 7 months old, I asked Evan's cardiologist what he thought of us that first day. The day he showed us the pictures of the two hearts. One "normal" and one very distored -- Evan's heart. He said that he honestly didn't think he would see us again. He said that babies that sick don't make it back, but he tries to get them to the bigger hospital for help. His honest feeling, though, was that it was far too late for our baby. I was so glad that he was wrong (even though it made the nurse right, the one who had whispered in my ear).
Time went by and all seemed so normal in the world. Evan got older. He gained a few more siblings (all heart healthy). Then Evan started to gain weight, and have hardly any energy to be a kid. So in the spring of his first grade year (May 2008) he had a heart cath to see what was going on. We were told that there was something "new" going on, as a result of his intial heart issues. Evan had "left ventricular diastolic dysfunction" -- this meant that the left side of his heart did not relax. It could not fill properly with blood. It was very rare, however, so we need to see someone with more experience -- the head of cardiac transplant! Transplant?! Our Evan?! This was frightening, but we went. What we heard was horrible news. It was likely that he would require a transplant, and if things continued to worsen, it would be that he'd need both a heart AND lungs. Odds of that happening are so slim, the doctor said only 2 of his 6 patients who'd gotten to that point made it. In an effort to avoid a transplant, Evan was allowed to try some medication that *might* help with the problem. An evaluation was done in Dec. 2008 and the meds had worked! It was time to replace Evan's donor pulmonary valve to see if they could force more blood to the left side of his heart, and avoid a transplant for good. With Evan's first open-heart surgery, David and I only had to worry about our own fears. This time? We had to explain to an 8 year old child that he was going to have to under-go open-heart surgery. We had to contain our fear. We had to show him that we had absolute faith that everything would go well. I wasn't near as sure as I let on, but I had to remain strong on the outside for Evan. On the morning of February 4, 2009, Evan ran (literally) into the hospital and received a new donor pulmonary valve. Once again, he had difficulty coming off the heart-lung bypass machine. Could this really be happening AGAIN?! Soon, we would learn that it was due to an allergic reaction (an anaphylactic reaction, to latex). Once the anaphylactic reaction was under control, he came off the bypass machine and began his remarkable recovery. Evan was sent home just 3 days after his surgery. He was back at school in just two weeks time, and back to gym class and physical therapy in four weeks. It is thought that Evan will not need a transplant (though no one can say for sure), and that this may have been his last open-heart surgery. They are hopeful that they can replace his valve next time by catheter. His heart will never be "fixed" completely, and he will require new valves for the rest of his life. But he is a 'young warrior' and he will get through this. He is our SuperEvan.
While Evan's story has a happy ending, there are far too many stories like his that do not. David and I knew, once we learned exactly how common heart defects are, that we *NEVER* wanted another family to learn about them for the first time the way we did -- when being told that your child probably isn't going to live through the day. How can this be the most common birth defect in the world, and THAT is how people hear of it for the first time?! It doesn't make sense. How can these defects take twice as many kids each year than all forms of childhood cancer combined, yet no one knows that this is happening?! This is an outrage and no child's death should be in vain. Every child matters, perfect heart or not! Parents should not be burying their babies! It is indescribable the pain that is felt when another "heart family" loses their child. It could just as easily be us. We realize this and try to not take even one moment for granted. It happens so suddenly, and unfortunately it happens far too often. We have only been in the world of CHD's for 9 years, and have seen so many parents lose their children. My hope is that in Evan's lifetime, there will be more awareness, and more research. I want no other family to learn of CHD's for the first time when they are told that their dying child has exactly that!
Thank you,
Kathaleen Deam (proud to be Evan's mom)