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My daughter Sophie is dead. She died a week before her second birthday, from the effects of a complex set of heart defects. But she did not die of a broken heart. She was neither wretched nor alone. She was loving and she was loved. Her everyday life was together brutal and inspiring. She was, and is, a tragic hero.

The first Jana and I heard about Sophie’s heart was in a darkened ultrasound room. It was Jana’s routine 20-week scan. Everything had been fine, and we felt good. Almost carefree. But then the sonographer fell silent and concentrated hard on her images for many minutes.

“You look intent. Is everything OK?”

A pregnant pause.

“I think I see a hole in the heart.”

Externally, Jana and I were calm. In our heads, we panicked. Did our unborn daughter’s heart look like a donut? I had only ever heard of holes in the heart that Go All The Way To China. Would she even make it outside the womb? We had infinite questions; there were no immediate answers.

We had to wait a week for a full diagnosis. Jana fretted that the baby would spontaneously abort, just like her first pregnancy a year before. Were we incapable of having kids? This fear consumed her through the week. I thought it was premature to draw conclusions from one miscarriage and one sonogram, so I didn’t obsess over the baby’s heart while waiting for the expert scan. Jana would cry and I would tell her it was too soon for tears. I thought my stoicism was uplifting. She experienced only cold.

We sat in another darkened ultrasound room, this time in the pediatric cardiology department at the University of Michigan. It was our local hospital, but it also happened to be one of the top five centers in the US for treating kids with malformed hearts. We had three appointments there, filled with sentences like:

“I’m sorry for the delay. We deal with complex anatomy every day, but this is really complex anatomy.”

Each time we went, they found new things to worry about. Carly Fifer, a specialist children’s cardiologist, finally told us that our daughter had a condition called heterotaxy with right isomerism.

With this condition, both sides of Sophie’s torso believed they were the right hand side, meaning that some right-sided structures in her body developed twice, while other left-sided structures were malformed or not formed at all. That doesn't really matter for kidneys, and isn't too threatening for lungs. But she had no spleen, her intestines were twisted into her abdomen in a dangerous way, and the syndrome had wreaked havoc with her heart.

The central heart wall separating oxygen-rich red blood from oxygen-poor blue blood was crooked and mainly missing. The rest of us have two valves in the middle of our hearts; Sophie had one. The rest of us have two pipes bringing back blue blood from the body; Sophie had three, including two going to the wrong place. The two pipes out of her heart were lined up wrong, one of them also too narrow to do its job. And more. Without treatment, she would die within a week of her birth.

Heterotaxy is so rare that researchers don't agree on exactly how rare, but when it does present it has a signature pattern of defects. Sophie’s was a typical case of right-sided heterotaxy. Carly even described it once as “garden variety heterotaxy,” because she had the signature pattern of defects. This was true in the same sense that Hurricane Katrina was a “garden variety category five storm.”

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Sophie Salmond came into the world in February 2009. She breathed on her own and cried and wriggled almost like normal, arriving the old fashioned way for good measure. But Jana did not even get to hold Sophie before she was whisked away to her new home in one of Michigan’s intensive care units, the Pediatric Cardio-Thoracic Unit.

The PCTU in Michigan’s since-abandoned Mott hospital building felt like a rabbit warren. A U-shaped corridor had a teeming medical workroom on one side; darkened, curtained patient rooms on the other; and medical equipment stacked almost haphazardly along one wall. Sophie was in an open bay, with two other patients. She lay in a tiny incubator bed with a heat lamp. She wore a stripy hospital hat, and was swaddled in a blanket that came up around her cheeks. Only her face poked out, eyes closed, making her look like a cross between a tiny mummy and a Russian doll.

The blanket fell away when I picked Sophie up, revealing a set of wired stickers with hearts printed on them, a board tied to one of her arms to steady an IV, a clamp over the remnants of her umbilical cord, and an oxygen saturation probe around one of her feet. A large monitor displayed her heart rate and other vital signs. Beside the bed, various syringe pumps delivered vital drugs with names I did not understand.

Later than evening, when I wheeled Jana up to see her, Jana cried on sight. I didn't cry, wanting to show strength for both Jana and Sophie. So I stood beside my crying wife with a "there, there" hand awkwardly on her shoulder, staring almost blankly at my first child, wired tight into a hospital bed.

At rounds the following morning, doctors talked in code.

"Sophie is 14 hours old. Heterotaxy right. Unbalanced AV canal. DORV. Common AV valve. D-malposed arteries. Pulmonary stenosis. TAPVR. Bilateral SVC. Left IVC. Asplenia."

"She's on prostans?"

"Yep. Straight away."

“1V or 2V?”

“We don’t know yet. Shunt this week for sure.”

Jana and I stood by the bed, mute. We hadn't the faintest idea what the doctors were saying, and had read somewhere that parents should not interrupt during rounds. Someone would return later to answer our many questions.

Sophie's first operation, the “shunt” was designed simply to make sure she survived more than a week. It was not a long-term fix. In the womb, all babies have a connection between the red blood and blue blood circulation systems, because their lungs do not work when the body is surrounded by liquid rather than air. This is the ductus arteriosis. In normal babies, this connection closes up around a week after birth, because outside the womb it causes inefficient circulation. But in a kid like Sophie the ductus is what keeps them alive, giving enough blood flow to the lungs to enable OK-but-not-great oxygen delivery elsewhere. The surgery, more formally a Blalock-Tausig Shunt, creates an artificial ductus. It is inefficient in the short term because red and blue blood still mix together inside the heart. And it is unsustainable in the long term, as it does not grow bigger with the child. But it would allow Sophie to get big enough so that more complex operations can be reliably performed a few months later.

On the morning of the surgery, the nurses tied a bow around her head for good luck. We held her for half an hour before the whisked her away for five. The surgery was straightforward for Dr Ed Bove, one of the most well respected children’s heart surgeons in the world. He had done literally thousands of these before. What seemed almost fantasy to us laypeople was nothing more than a warm up for him and his team.

When we approached Sophie’s bed space after the surgery, some staff turned to follow our movements. They knew the first-time shock. Sophie was immobile, flat on her back. There was tape over both her lips and a plastic contraption hovering above her head, all holding a breathing tube in place. A bank of automated syringe pumps stood stacked six or seven high next to a ventilator, all blinking and dosing. Multiple syringes stood connected to Sophie’s bloodstream, ready to take samples at a moment’s notice. She had a large bandage vertically down the middle of her chest, two sets of wires exiting her chest but not connected to anything, multiple IV entry wounds, a urinary catheter, and various other monitors stuck to her, their wires coiling away toward the overhead screen.

I gasped a little and felt my legs wobble. Jana cried. I didn't.

It felt like the end of Sophie’s innocence. Most kids get more innocence than four days.

The sight of kids in post-operative distress was just one aspect of hospital life that we were becoming accustomed to. Over the next few days, as the machines were turned down and then off and Sophie was allowed to awaken, we learned of others. Some were scary, like the sound of the Code Blue alarm and the gang-rush of semi-panicked medics to a child in mortal danger. Others were not scary at all. One morning, Jana discovered something buzzing and moving in Sophie’s bed. A bee? Surely not in an ICU. It was, in fact, a vibrator, complete with phallic shape and racy box.

“Why is there a dildo in my daughter’s bed?”

Vibration helps babies relax and sleep, but nobody had designed a special one for kids, so the hospital kept a supply of the adult ones instead. At some stage, that decision must have involved an awkward conversation with purchasing.

Sitting in an ICU, you sometimes get to know the parents around you. In Sophie’s bay there was a teenage Mom who kept to herself while dealing, all alone, with a very sick six month old, and there was Dan and Sara from Missouri, staying with their other kids in a cramped hotel room, teleconsulting into a job in order to keep health insurance, and caring for a two week old boy even more complex than Sophie. We liked them and talked with them often and invited them for dinner. They canceled on the day because of a “complication.” We never saw them or their boy again.

A week after her surgery, Sophie left the ICU, bound for convalescence in the step-down ward. “The floor” was a welcome change. There was talk of going home, and education on what to do when we got there. There was a playroom. Parents dared to look ahead with hope, rather than look for monsters under the bed.

In her car seat, homeward bound

On the floor, we learned how to treat Sophie outside a hospital, with no machines or call buttons at our disposal. A day before she was due to leave, we turned off all her monitors to simulate home. After two weeks of beeps, the silence was deafening. With experience, we would learn to treat the child rather than treat the numbers. At that time, however, it seemed reckless and dangerous.

While Sophie convalesced on the floor, the doctors looked at her intestines. They were malrotated, as often happens with “garden variety heterotaxy.” The fix is straightforward, but it is surgical. When we left the hospital, our next surgery date was already booked. Our farewells were more “see you soon” than “goodbye.”