Thirty five weeks

 

I sat in the nursery rocking Emmarie, her 17-month old legs spilling out over my belly as her head nestled against her sister sleeping in my womb. I thought back to our 33-week appointment. No one actually said the words “Clara is going to die.” I wish they had. I would rather hear those words than know them. They had never proven true; they had always cracked open, leaving a glimmer of hope.

I was 21-weeks pregnant with Clara the first time I heard them, their syllables foreign to my heart. “Trisomy 16 is incompatible with life,” our primary Maternal Fetal Medicine (MFM) doctor explained, his voice gentle and broken from the other side of the phone. I crumpled against our upstairs wall, body sagging into the floor as I relayed to David the words I never imagined saying.

“Our daughter isn’t going to make it,” my mouth fumbled out, voice shaking, tears cloaking my vision as David looked back at me. I couldn’t say the word “die” aloud. Doing so might make it true.

“What? What do you mean?” David placed Emmarie in her crib as he moved towards me, his red shirt all I could see against the graying world. We knew Clara had a complicated heart. But, this?

“David. She isn’t going to make it.” No one ever told me you don’t get to pick the moment you learn your child is going to die. Emmarie wailed from her bedroom as she watched her parents huddled, sobbing on the floor.

The doctors were wrong, that time. Clara’s actual diagnosis—Trisomy 16 mosaicism—is so astonishingly rare that when they saw an extra 16th chromosome in her genetic mapping, they assumed every cell in her body followed suit. Full Trisomy 16.

Almost as quickly as she was diagnosed, holes began appearing. Trisomy 16 is so incompatible with life that babies do not typically make it past the first trimester. Clara was already 21 weeks. Then she was 22 weeks. Then she was 23 weeks. Then she was 24 weeks and moving, kicking, and falling into normal sleep-wake patterns. During our 25-week appointment, under the dim lights of the ultrasound room, we read her amniocenteses report like a book, cover to cover, and learned what we all should have known from the start—Clara had a primarily normal cell configuration. Only some of her cells had an extra 16th chromosome. Only a fraction of her precious body had a different genotype.

Our hearts began stirring with the smallest twinge of hope. Of the very few documented cases of Trisomy 16 mosaicism, over three quarters were living a full and complete life. Our hearts jolted still. The remaining quarter with poor outcomes, those who did not make it beyond childhood, were those born with complicated hearts or whose affected cells corresponded with nervous system development.

Clara fell into the first category. She had a complicated single ventricle heart, a truth we had known since our 18-week appointment. Mitral valve atresia. Double outlet right ventricle. Transposition of the greater vessels. These major CHDs, along with a smattering of other minor CHDs, led to a heart in which Clara’s left ventricle had not developed. Or, if it had developed, it was so small it was not visible on an echocardiogram. She would need a series of open heart surgeries to survive, and even these were considered palliative. These surgeries would not fix Clara’s heart; they would add beautiful, precious days to her life. She might live to be five, maybe 20. If things went well, she might, with a heart transplant, live to be in her 40s. In a world where we are growing cartilage and printing three-dimensional everything, I had to keep shoving these truths into my brain. We believe doctors can fix anything. We believe what we see. And we see the unexpected miracles, the ones garnering thousands of likes and shares on social media. The deaths of children don’t attract nearly the same public attention.

If it wasn’t medically possible to fix Clara’s heart, we would take anything they could give us. One year or fifty years. To have a decent chance of surviving her first open heart surgery, though, the surgeon wanted Clara to be as big as possible. Trisomy 16 mosaicism causes intrauterine growth restriction (IUGR), resulting in babies who are born early and well under four pounds. Clara’s ultrasound weight composite report read, “below the first percentile.” She was barely treading water at two pounds. I ate ice cream every day, sometimes twice. It never helped.

Given Clara’s complicated heart and extreme growth restriction, our MFM prepared us for what was still the most likely scenario at our 25-week appointment where we learned about her mosaicism—losing Clara in the womb. “Count her kicks, feel her movement, love her well, talk to her,” he said. He let us look at her through ultrasound, bringing black and white images to three-dimensional life.

As we waited, as we prayed, as we celebrated every day I woke up feeling her movement beneath the crushing fear encroaching on our minds, we tried to love Clara well. We told her how beautiful she was, how God had made her perfectly. We took her on boat rides, letting her feel the jostling of the waves. We visited the ocean, explaining how the saltwater smells as the wind whips sand through your hair. We told her about how Jesus came to die for our sins so that we could one day be made whole and reunited with Him. We told her about the great God she was going to meet. We sang her songs. We laughed when she kicked furiously as her sister cried. I began working in the yard, telling her how the flowers smelled as their tiny buds bloomed into magical, mysterious petals of beauty. David talked to her through my stomach. I rocked her as I cradled Emmarie in my arms every night before bed with tears streaming down my face. We tried to fill Clara’s days with a lifetime of memories, just in case. There was never enough time. There were always words left unsaid.

As I sat reminiscing about those seven weeks of hopeful uncertainty, rocking my girls in the nursery, breathing in the scent of baby shampoo and milk dried lips, I thought to myself, yes, I would rather hear the words “Clara is going to die” than know them. The tears, the uncertainty, the preparing to say goodbye, the agonizing expectation of “when”—all of it, was nothing like knowing the truth is coming at you like a tsunami you can’t escape.

And, the truth was Clara had Trisomy 16 mosaicism. She was below the first percentile in weight. She had a complicated single ventricle heart. Although she was defying every medical odd, her pediatric cardiologists had discovered she had one of the worst additional complications to pair with a single ventricle heart during her anticipated 33-week appointment.

Clara’s pulmonary veins, which should have connected to her left atrium, transporting oxygen rich blood from her lungs to her heart, instead ran down through her diaphragm and connected in to her inferior veni cava (known as subdiaphragmatic TAPVR). Once her PDA closed at birth—the temporary hole that closes in every newborns’ heart—each breath she took would cause her diaphragm to compress these veins. Blood would back up into her lungs. Congestive heart failure.

Clara would need immediate surgery—the we’re-reserving-an-operating-room-for-24-hours around her scheduled birth kind of immediacy. This surgery is already extremely challenging on an otherwise healthy heart. Clara’s heart was not otherwise healthy. Compounding the issue, the needle required to fix her pulmonary veins was currently the same size, if not bigger, than her veins due to her growth restriction. If by some miracle they could operate on Clara at birth and she did make it through that first surgery, she would be on heart-lung bypass (ECMO) for weeks, maybe months to let her heart and lungs recover. ECMO is designed to be a temporary solution. A day or two tops. Daily blood transfusions, clots, strokes, infections. The list of associated risks with extended stays on ECMO is endless. Most children never come off it.

As if our beloved daughter kicking in my womb didn’t have enough stacked against her, Clara would then still need to go through her staged open-heart surgeries. With her TAPVR and compromised heart, her lung and heart function would never qualify her for surgery. No matter what medical pathway we took now or how hard Clara fought, the outcome was always the same. Clara would need a heart-lung transplant, but would never meet the criteria to receive one.

My stomach churned at the horror of it all as I sat in the nursery. I was too numb to have any logical emotional response when the doctors first explained Clara’s TAPVR. I wanted to go back to that place, that no-feeling, what-are-you-saying place. Instead, the moment sitting around a white circle table with the warm sun peaking through a small window as a panel of doctors kindly, gently explained to me how my daughter would likely die, was frozen in my mind like David’s face as I walked down the aisle on our wedding day. Only it was a nightmare I kept reliving. It played in my mind in the quiet of the house and in the chaos of the grocery store. It continued beneath every normal conversation. I understood why even the chief pediatric cardiologist hadn’t been able to suppress his tears as he walked us through Clara’s diagnosis.

Yes, we could elect to operate on Clara’s heart at birth. As one cardiologist said in a soft, but firm voice during a follow-up meeting, doing so would be akin to child abuse. We would be putting Clara through endless pain for the selfish gain of a few extra hours or days on a heart-lung bypass with no hope for a long-term solution. I was offended by his words. Wouldn’t operating be a mom fighting for her child when he or she can’t? Did he not see how hard Clara was fighting? We couldn’t give up.

The cardiologist was right. In the words he said and in those he never said, he was right. I wanted Clara here because I wanted her. I wanted my life with her, every waking moment. A mother’s job is to fight for what’s best for her daughter, not what’s best for her own life—even if that means letting her daughter go.

I looked around the nursery. How do you let your child go? It was going to be their room. Emmarie and Clara. The walls a light yellow with gray and coral accents. Clara’s crib would go across from Emmarie’s, her matching coral blanket draped off its side. It would be their place. A place for sisters, best friends only 18 months apart. A place for my girls. A place where they would toddle and walk, crawl out of cribs and sneak into beds. A place where they would trade evening bedtime stories for midnight talks about boys. A place where we would quietly open the door long after whispers had stilled every night to see what they had been doing. How could I let it all go?

How could I let a part of my body and soul go? How could I let the child who I had loved from the moment she was conceived go? How could I make the decision? How could I make the rationally right decision and the emotionally wrong decision? Nothing about this made sense. We had spent hours on our knees contending before the Lord. We prayed with expectation, with the hope that He would intervene. It was easy to say Clara’s fighting spirit meant His answer to her life on earth was yes, “Yes she will live.” It felt impossible to accept that He had created her fearfully and wonderfully, with a fighting spirit and inconceivably rare genetic and heart makeup for a purpose that would transcend her life here on earth.