When asked about the day that changed their life, most women will say it was they day they graduated college, got married, became a mother or began their career. Those experiences change everyone who goes through them, and no doubt changed me as well. But there was one day that changed my life in more ways than those possibly could have.
In 2009, my miracle baby, Jeanaveve, was about to celebrate her first birthday. Because of a cold she couldn’t seem to shake, we took her to see her pediatrician, Dr. Kassas. He thought he heard a slight heart murmur while listening to her chest to rule out pneumonia. Many children have slight murmurs when they’re little, he told us, but most outgrow the murmurs. He asked if we’d be willing to take Jeanaveve for an echocardiogram just to rule out anything serious. About a week later, we were trying to keep our active little girl still long enough for the sonographer to get a good photo of her heart. Shortly after the echo, we went on vacation for a few days. When we returned, the answering machine was blinking at us urgently. They hadn’t been able to get a clear picture of our baby’s heart, but thought they saw something. The right side of her heart appeared enlarged. We were being referred to a pediatric cardiologist in Buffalo, NY.
When we first met Dr. Orie, our heads were spinning. We had just put Jeanaveve through another echo, this time in his office, and were trying to keep our little one still and quiet long enough to hear the news. Dr. Orie was patient, though, and while we could see he was very busy, he took his time explaining everything to us, and even drew us some diagrams so we could visualize what was wrong with our baby’s heart. Jeanaveve probably had Partial Anomalous Pulmonary Venous Return (PAPVR), which means that one or more of the veins that bring oxygen rich blood to the heart from the lungs connected to the right side of her heart, when they should all go to the left. This was the most likely reason for her enlarged right heart. We were lucky to have caught this early, Dr. Orie told us. Most children aren’t diagnosed until they’re seven years or older. Dr. Orie sent us home after telling us that he would take Jeanaveve’s case before a board of several pediatric cardiologists from all over upstate. The board is a brain trust, basically. Everyone weighs in on the case, so you’re getting multiple “second opinions” at once. We went home anxious, but confident in the fact that we hadn’t been told to limit our daughter’s activities. How bad can it be, if she’s still allowed to do whatever she wants? Nonetheless, I tried to read everything out there about PAPVR. During the waiting and researching phase of our Congenital Heart Defect (CHD)/PAPVR journey, I felt alone. There wasn’t much information out there about PAPVR. Most of what I found simply explained what the condition was, and how rare it was (less than 1% of CHD cases). Nothing made it seem too bad, but we still worried and I still wished I had someone else I could talk to about what we were going through, and what we could expect. There were a few charities listed online for CHD and a couple advocacy groups. Nothing near our tiny little town, though. It was like being stuck behind two way glass. I knew we weren’t the only family going through this, but I couldn’t see anyone else. Finally, we got the phone call. The pictures from the second echo weren’t detailed enough. We would need to take Jeanaveve to Women’s and Children’s Hospital of Buffalo on the day before her second birthday. They would put her to sleep, and try the echo again. A few days after the sedated echo, another call. This echo was better, but still not quite detailed enough.
On June 24, 2010, we would take our two year old to University of Rochester Medical Center/Golisano Children’s Hospital in Rochester, NY. Dr. Orie’s colleague, Dr. Leonard would put our princess to sleep and snake a catheter up through a vein in her leg to her heart, where they would inject some dye and take more pictures of her little heart in an effort to understand exactly how her blood was flowing and where. Most children with PAPVR have two anomalous veins and a hole between the chambers of their heart. This would be the best way for the doctors to see how much of that was going on in Jeanaveve’s chest. We were in awe that they could do this on a tiny little thing like our baby. Born at five and a half pounds, she wasn’t exactly a big kid; even at two years old, she weighed barely 20 pounds. What seemed like an eternity later, Dr. Leonard led us into a private room off of the waiting area, where he showed us the pictures. It was very clear that Jeanaveve had no hole in her heart. Excellent. It also appeared that only one of her veins was connected to the wrong side of her heart. This was good news! Right? Well, sort of. Dr. Leonard didn’t want to speculate, but all of her pictures would be taken back in front of the board, and they would decide if our baby would need surgery to correct the connection. She did. Our crazy, happy, active, intelligent, beautiful princess would have open heart surgery. We were to wait for the call. She would likely have surgery in Spring of 2011. Since hers was not an emergency case, we could wait till then and not have to make the drive to Rochester in the snow. We might also get bumped by an emergency case. Jeanaveve went to Early Head Start and played like usual. Her Dad and I tried not to talk about what was coming. I knew he was having a hard time dealing with this journey. He was 38 when she was born. He’d led a hard life. He often blamed himself for her defect. I had been through the same. I was a smoker. I had been to the bar before I knew I was pregnant. I blamed myself as well. If I’d stopped drinking or quit smoking earlier in my pregnancy, maybe this wouldn’t have happened. I knew, though, that none of that mattered. We couldn’t change anything, and our daughter would need us to be strong for her while she was hospitalized. I stayed strong. I explained her condition to everyone who asked. I memorized every fact I could scrounge up about CHDs in general, and hers in particular. I refused to allow myself to cry. I wouldn’t let her daddy hear worry in my voice. I would be the strong one, even if it killed me.
Winter 2010. End of Winter 2011. Spring 2011. No phone call. Finally, I called the hospital. They called the cardiologist. The date was set. September 15, 2011. With little time to plan, I did all I could. I scrimped on groceries to overpay bills. I asked my parents to take care of my son. I asked complete strangers to pray for my daughter. (A few extra voices mentioning her to the Big Guy couldn’t hurt, right?) I struggled with a feeling of guilt, added to my worry. Guilt that we were asking for so many prayers and well wishes while so many other CHD children fought much more serious conditions. Many of them losing that battle. I joined the Mended Little Hearts of Greater Rochester. I bought my baby a special teddy bear who wore a zipper on his chest with a felt heart behind it. The heart bore white stitches. I used him to explain to my three year old that soon, some doctors would put her to sleep and unzip her. Just like Teddy’s doctor’s did to him. They would fix her heart and zip her back up, and she had to be brave, just like Teddy had been. I wrote a letter to my daughter that she may never read. I packed our bags. I loaded the address into the GPS in the car. I stayed strong for my baby and her daddy. Even though I wanted desperately to fall apart. I wanted to cry, I wanted to punch the walls, I wanted to scream “WHY?!” And then I felt guilty again. Because in the grand scheme of things, we were already so lucky.
And then there we were. Waiting for the anesthesiologist to come take our precious girl. Dr. Gensini, one of the pediatric cardiac surgeons at URMC/GCH came to talk to us and explain what would happen during surgery. They would put Jeanaveve to sleep, hook her up to a heart/lung machine and stop her heart. A small “baffle” (picture a Chinese finger trap) would be made and inserted in her superior vena cava to re-route the oxygenated blood to the left side of the heart. They would make it just big enough to accommodate blood flow through it, and around it. Then they would restart her heart and “zip” her back up. She would probably not need another surgery. Ever. By the time Dr. Gensini was finished speaking, Jean was in his lap, talking with him and playing with his ID badge. It’s impossible to be “at ease” when your child is about to undergo open heart surgery, but seeing how much Jean trusted this man she just met helped. Daddy and I waited numbly and mostly silently for almost 6 hours. Finally they wheeled Jean past the waiting room to her room in the Cardiac PICU. We were allowed back to see her as they were removing the breathing tube. There is nothing that can make you feel more helpless than seeing your baby lay in pain and knowing you can’t kiss it away. The cry that escapes a recently extubated child is the most pitiful, sad, scary thing you will ever hear. The first few hours were the worst. Jeanaveve was extremely thirsty after surgery, but drinking wasn’t allowed until her belly woke up from the anesthesia. She was offered small sponges on sticks dipped in water. She eagerly chewed at them and asked for more. She even sat bolt upright amid her pain to demand that a passing doctor, “Gimme that cup!” On the second day post-op, we were to encourage her to walk the halls to aid the fluid drainage from her chest. we had to carry a box that held the drainage with us wherever we went, and carry it carefully, since it was attached to our baby, entering her little body just above her belly button. We were in the PICU for six days, staying just a floor above our baby when we needed sleep, thanks to the Ronald McDonald House. When they said we could go home, we were excited, but apprehensive, too. It had only been six days, after all. Not even a week. But off we went. Jean healed so well and so quickly that she was able to start Head Start with her friends on the first day back. Being a “heart mom” changed me in so many ways. I see my daughter as a hero, not just a five year old learning to read in Kindergarten. I do as much as I can to raise CHD awareness. I wrote letters to State Senate and House members to try and get Pulse Ox testing (a painless test for newborns to screen for CHDs) made mandatory in New York State. The law passed in 2013, and will be implemented statewide soon. I have joined forces with Mended Little Hearts of Greater Rochester to offer help to other families in my area, so they won’t have to drive two hours to get the support I missed when we were starting our journey. I urge my friends and family to donate to CHD related charities instead of sending me gifts for birthdays and holidays. I stayed strong for my family throughout our CHD journey, but my daughter showed me what strong really is, and for that I’ll forever be grateful. For that I’ll forever, from this point on, be an activist, an advocate, a supporter, as well as an incredibly lucky, blessed mom.