By Sarah Virginia
It is both a hard and a perfect time to reflect on Shane’s heart journey, and mine alongside it. He is still in the throes of post-OHS recovery, and the path ahead looks unclear at best. But one thing that is clear to me is that his life is not only worth living, but also he has probably done more in his short life to inspire faith and kindness than I have in all 37 years. I’m just so incredibly grateful to be his mom and can’t wait to see the person he will become.
We learned about Shane’s Down Syndrome diagnosis at around 11 weeks of pregnancy. I was in my office, just leaving a meeting, and got a call from my doctor’s office. I was excitedly expecting a gender call, and ran to my office and closed the door. But the voice on the other end sounded immediately somber, and told me that she had some “bad news”--our baby’s prenatal testing results had come back with an 81% chance of Trisomy 21. I was in tears, not knowing the first thing about what this meant for us. I asked to know the gender and she said, are you sure? That moment I knew that we would always be fighting to show the worthiness of Shane’s life.
I was referred to a geneticist who told me I was “so brave” for considering taking on a child with Down Syndrome, and then proceeded to list all the things that can go wrong health wise. She was absolutely lovely, but there’s something that’s just so wrong about the first things you hear from a medical professional about your future child being all the things that could go wrong. What about all the things that can go wrong for neurotypical children? That’s what we sign up for as parents–the good, the bad, and everything in between.
In any event, I learned that there was a 50% possibility that my baby boy would have a congenital heart defect and that about 50% of those children with CHD require a surgical intervention. Only about 50% of those surgeries are “complicated.” Shane, of course, had to go the fanciest route and make life extra interesting by landing in that small percentage of kids with very complex hearts. I didn’t know that then, but I accepted all the possibilities.
At our 16-week anatomical scan, we got the news that his heart had some issues and were referred to a pediatric cardiologist. He had an echocardiogram at 19 weeks and they identified the complete AV canal defect–holes between both the atria and ventricles of his heart. At 23 weeks they found that his aorta was somewhat narrowed heading into the heart, and that could be consistent with hypoplastic left heart syndrome–where the left ventricle is too small to function and eventually the heart has to be rewired to function on one ventricle. This outcome is a scary one in the sense that single ventricle hearts often need to be transplanted in early adulthood, and mortality at a young age is higher.
At that point I had two very close family members advise me to terminate the pregnancy–in New York, abortion is legal until 24 weeks. I was told that Shane’s heart was “incompatible with life” and that I would spend the first 2 years of his life in the hospital and never see my other children. I was devastated by what these people said, especially one who I trust and think to be incredibly smart (and works in the medical field). But I decided instead to trust in God and that the baby in my belly, whom we had already named Shane (“gift from God”) and whose brothers were already in love with, was intended for big things.
Shane’s heart was observed closely for the rest of my pregnancy and it was always thought that he would need to have a surgery very early in his life to repair his aortic arch and open it up before he left the hospital. I was paranoid toward the end of pregnancy about losing him and by the grace of God and my mama intuition, he was seen more often and they caught lowered fluid and induced me at 36 weeks and 2 days. I didn’t have to have a C section and he came out in 3 quick pushes. They let me hold him for a few brief moments and then whisked him away to the NICU. They showed me my placenta after–it had completely broken in half except for one thin strand of facia holding it together.
Shane was in the NICU for 24 hours before he was moved to the pediatric cardiac ICU, where my husband and I alternated 48-hour shifts for 27 more days. He had the surgery to repair his aorta at 12 days and they placed a band in his pulmonary artery to help prevent the unoxygenated blood from flooding his lungs and giving him more time to grow before his next surgery, the plan for which was to do a complete bi-ventricular (two ventricle) repair of his heart.
He came home on a few meds but no oxygen, no feeding tube, and basically acted like a typical (and super healthy baby) for the next 16 months. He had a heart catheterization at 1 year to expand the band because his oxygen was dipping and they wanted more time. The goal was always to see if his left ventricle would grow in size and get big enough that a bivent repair would be possible. So looming over us for that first year and a half was the thought that maybe Shane would ultimately need a one-ventricle repair if his left heart didn’t grow enough.
Just after Christmas, his oxygen was dipping more and surgery was scheduled. They did an MRI to see a better picture of the heart and our surgeon felt confident that a bivent repair was going to happen. We spent a month holed up hoping he wouldn’t get sick, which would delay surgery more. We had waited 18 months and couldn’t wait for his heart to be fixed.
However, the morning of his surgery, we were told that the doctors had looked at the images again and were not as confident about the bivent repair and might need to do what’s called a “Glenn” procedure. I freaked out. I had only ever heard “Glenn” in the context of a one-ventricle repair. The surgeon assured me that he could perform a bidirectional Glenn, meaning it would leave open the possibility of either a two ventricle or a one ventricle repair in the future, but that it would be the safer option if they went in and the left ventricle or the valve on that side were too small. We accepted the recommendation and moved forward. And Shane ended up with the Glenn.
Shane is still recovering–he suffers from a post surgical complication called chylothorax, where a leak in his lymph system causes fluid to accumulate in the pleural space (around his lungs) and he needs to be on a low fat diet for six weeks. He has also been readmitted to the hospital twice for low oxygen and swelling. He and my husband are there as I type this, and I’m home with our two big boys. I can’t say this is easy.
But what is easy is seeing our boy make every nurse and doctor who walks into his room happy. He gives kisses, hugs, waves, high fives, and just oozes love and joy. He is full of life in every sense of the word.
My heart is heavy some days not knowing what is next for us–I’ve been connected with some moms with remarkably similar journeys to ours and I’m now learning about new and exciting medical procedures involving growing the left side of the heart. And I’ve never been alone in this journey because the Down Syndrome community, the Heart Moms community, and the special needs community as a whole are the most supportive and wonderful humans I have ever known. Our whole community is never going to give up fighting for and praying for Shane and believing that he will live a long and happy life. It may not be the road we expect, and sometimes not the road we want, but it’s a beautiful road filled with a new form of love and appreciation for this life.