In early October 2009, we loaded up our car and drove to Boston. Our employers at the University of Michigan had given us the fall semester away from teaching obligations, on full pay, to care for Sophie. We were far luckier in this regard than most parents of heart kids.

Sophie’s oxygen saturation was now dangerously low, as her undersized shunt struggled to get enough blood flowing through her lungs. It was around 60% of normal. In the car, she needed concentrated oxygen constantly wafting across her face. We were loaded down with our own stuff, a crib and toys for Sophie before and after the hospital stay, and bicycles for getting to and from the hospital without braving Boston parking. Arriving into town a few days before her surgery, we settled into our short-term apartment and took Sophie walking around Boston and Cambridge, visiting friends.

Pack-and-playing, October 6, 2009

The day before Sophie’s surgery, we went with Sophie to Boston for a series of pre-operative checkups. Sophie was measured and weighed and tested and imaged, and we met with administrators, anesthesia people, and others. We were also scheduled to met with Dr del Nido, but before we could meet the surgeon himself we met with one of his surgical assistants, in the final stages of his children’s heart surgery training.

The surgical assistant spoke in overly glowing terms about his boss, almost exuding a cult-of-personality. He had earlier trained at Michigan, and insisted on calling Sophie’s surgeons by their first names, “Pedro” and “Ed,” just to prove they were on first name terms. He was the puppyish, egotistical jock of the surgical stereotype. We liked him less than he did.

Through all Sophie’s treatment, only a handful of her caregivers rubbed us the wrong way. There was only one real jock. Only a couple of times did the staff’s professional frustrations lead them to snap at us. “No, no. You have to listen to me.” On a couple of occasions, someone betrayed a default attitude that the parents are idiots. “You see, your daughter’s heart is very sick…” But out of thousands of often high-stress interactions with nurses, doctors, and others, the rate of personality clash was incredibly low.

Then we met with Dr del Nido. He did not appear like a cult-of-personality figure at all. He was gentle towards both Sophie and us. He made sure we understood that we could still do the Fontan surgery in Boston if we preferred. It wasn’t too late to change our minds, and it would be perfectly OK with him. But we were unbending.

“We came to Boston for the 2V, so that is what we want.”

Early the next morning, the pre-op waiting room for people just arriving at the hospital was half-full. Many of the kids were playful, admiring the fish in the aquarium and enjoying being out of the house in their pajamas. The parents, however, were more reserved.

Once Sophie was weighed and measured again, and we had again confirmed that we knew what was going to happen today, we were shown through to the pre-op holding room, a series of small cubicles snaking this way and that around various corners, each with a bed, a curtain around it, a couple of machines, and very little room for anything else. After half an hour, Vincent the anesthesiologist came to take Sophie away. He gave her an oral sedative to take the edge off before they injected her with the more powerful anesthetics later, and it came time for us to say our goodbyes.

Jana held Sophie first. I had no idea whether to join them in a group hug or to give her a moment with Sophie alone. So I just stood there feeling awkward, not knowing what - if anything - to do with my arms. Finally Jana passed Sophie over to me and I cradled her, looking down at her smiling back up at me. I brought her close to my head and we cuddled cheek to cheek for a moment. Just before I gave her to Vincent, I kissed her forehead and whispered to her, channeling what I thought my own Dad might say at a moment like this:

“Be good today.”

Vincent turned with Sophie and left. Jana and I were alone with an empty stroller.

We waited out the day at home. We packed away Sophie’s crib and toys. Jana settled down to read articles about heterotaxy. I watched Jason Bourne movies instead.

Nine hours after Sophie went into the OR, Dr del Nido met with us in the mostly deserted post-operative consultation area. The other families had already left. He seemed relaxed, bordering on casual as he greeted us. It was just another day in his extraordinary office. Sophie was doing fine, and was on her way up to the ICU to get stabilized. We could see her later this evening.

“She was pretty much as advertised,” he said, referring to the structure of Sophie’s heart, “and we were able to carry out the repair as planned.”

Jana von Stein
Out of the OR
Sophie is out of the OR and her 4-chamber heart is beating on its own. It was a very long operation (about 9 hours), but she did very well. The surgical team completed the repair as they had planned, and there have been no significant complications so far.

The next several days are going to be really crucial. She will remain anesthetized for a few days, as they have to wait for the swelling to die down before they can close her up, then wait for the heart to relax into its new function, before they let her body wake up and move around.

We will keep you posted, but for now we have to just wait and see how she adjusts.

We cannot thank you all enough for your well wishes. They helped our spirits today immensely.

Jana & Rob
October 7, 2009 at 5:46pm

We all shared a moment of nervous laughter and joy. It had worked! Dr del Nido went on to give us some of the details of the surgery, many of which passed us entirely by in the emotion of it all. Sophie was sitting upstairs with a four-chamber heart that was beating along all by itself. He did say that the valve repair was one of the trickier parts of the operation, and that down the line Sophie may need some more work in that area, but for now we could all celebrate a great outcome.

An hour later, the receptionist gave us directions to Sophie’s bed space and buzzed us into Boston’s cardiac ICU, which was much bigger, newer, and shinier than Michigan's.

Sophie looked awful.

She appeared far worse off than the two other times we had seen her fresh from surgery. Her face was all puffed up, and her tongue was so inflamed it spilled out of her mouth, which was stuck permanently open. Her arms looked like those of an overweight chain smoker or the Michelin Man, they were so balloon-like and pale. Her eyes were closed tight, no movement at all, with her ultra-puffy eyelids seeming to envelop her eyeballs so she could not physically open her eyes even if she wanted to.

The heart-lung machine tends to do this to kids and Sophie had been on it, her own heart stopped entirely, for over four hours. Around her were all the machines we were familiar with from her surgery at Michigan, only more of them, delivering breaths and drugs, monitoring pressures and heart rates.

The reality hit us hard that evening. Sophie may have enjoyed a surgical victory today, but the process was extremely hard on her, and she was now very sick. Jana expressed her horror immediately, telling me how terrible our little girl looked. I could already see that, so I stood quietly, taking in the surreal, grisly scene without saying much except asking nurses factual questions about this tube or that wire. Jana slumped on the side of Sophie's crib, forlorn.

Sophie lay unconscious for several days while we all watched her heart’s progress. The “squeeze” in her undersized left ventricle was remarkably good, and gave us a lot of hope looking ahead. But some of her blood pressure readings were concerning, especially the ones coming from inside the heart itself. They were too high, were staying too high for too long after the surgery, and could damage her heart and lungs if left untreated. Dr del Nido ordered a catheterization, which showed instead that part of the fabric wall he had created in the OR had torn away from the surrounding heart muscle, creating a channel between her two ventricles. This channel caused some blood to flow the wrong way, backing up the whole system and causing the blood pressure to rise.

We got this news at 5pm on the 16th, nine days after her 2V repair, and by 6pm they were wheeling Sophie into the OR for open-heart surgery again. Our goodbyes were hurried and harried, with both Jana and I adopting an almost military tone of gaining information, checking for dissent, and signing papers as quickly as we could. Every hour they waited exposed her delicate lungs and heart to higher pressures than they should cope with, increasing the risk of irreparable damage. Already she had potentially dangerous fluid build-up inside her lungs.

By 9pm we were back in the ICU getting debriefed by Dr del Nido, who had found the tear in the wall, fixed it, and reinforced it. In a few weeks native heart muscle would grow over the stitches and material, making this a short-term issue only. But over the next few days we would need to watch and see whether this repair indeed fixed Sophie’s pressure problems.

Dr del Nido didn't say why the tear occurred, and certainly didn't concede any fault. “Sometimes these things happen.” In later months I would wonder whether I was told everything in that debrief. Surgeons needed a certain ability to forget past bad outcomes and concentrate only on getting today’s case right. And all doctors had a psychological and financial incentive to conceal blame when they could, which was especially easy in a complex field when talking with lay-parents. Maybe I thought too much and too suspiciously about that day. I will never know.

A week later, with her blood pressures trending nicely downwards, we started to peel off the medications paralyzing Sophie and keeping her asleep. The wall repair seemed to have worked. After such large surgeries, and so long spent mainly unconscious, however, the process of waking Sophie up again was slow and painstaking.

Sophie was a high maintenance patient during that period, and there were times the staff would ask us to wait outside for an hour or two while they worked on her. We waited in a little room for families, sharing it with other parents, siblings, and support people. One mother was trying to look out for her son without the benefit of much formal education. When the surgeon told her he had stopped the boy’s heart during an operation, a routine step in open-heart surgery, she mistook that as a failed attempt to kill her son. She told us she had lost all trust in the doctors, and was withdrawing consent for treatments involving anesthesia like trips to the cath lab. Her misunderstanding put her son at grave risk, and put the doctors in a dreadful moral position. They could not talk her down directly, because in her eyes they were now the enemy. How far could they ethically bend the truth to get a parent to do obviously the right thing? At these moments we were even more grateful for our own educations.

Some of the sedatives Sophie was on were powerful, addictive narcotics. Morphine. Ativan. Methadone. In addition to the other challenges of checking her heart’s function and keeping a lid on her blood pressures, we also had to help Sophie cope with serious drug withdrawal as we woke her up. There were times when she could hardly open an eye through her sedation, but when she did it was not her normal sparkly eye that emerged, it was the beleaguered, overtired, almost lifeless eyes of movies like Trainspotting or Requiem for a Dream, complete with the unmoving, unfocused thousand yard stare. Those eyes were doubly horrifying in a baby.

Jana and I had developed a routine where Jana came into the hospital early and stayed until after lunch, when I relieved her, staying until Sophie had gone to bed for the night. One night during Sophie’s long withdrawal phase, I got to the hospital around 2pm and did not leave until Jana got in the following morning, because Sophie stayed awake screaming, desperate for more drugs, almost all night. She did not respond at all to the nurses’ soothing tones, instead ripping at their stethoscopes and her IV lines, but she responded a little to me. So I hugged her grimly for several hours straight in the middle of the night, terrified of who she might become if I left her and unable to call in help from Jana, who was completely wrung out from doing the same duty during the day.

As we befriended the nurses and some of the cardiologists around the ICU, they opened up more to us, telling us about this doctor and that, good restaurants in the area, and other (unnamed) patients they had known. We learned that Dr Jonas, who had sent us the third opinion about Sophie that included direct criticism of Dr del Nido, used to be Dr del Nido’s boss at Boston, until a faculty-room coup saw del Nido take over and Jonas move to DC. I asked one of our favorite cardiologists what had happened:

“Oh, no. No no no no no! I’m not talking about that. I like my job.”

One afternoon there was a pall over the ICU. The nurses we did not know wore obviously forced smiles as we passed, trying to project normalcy. The ones we knew well did not bother to hide their sadness. There was a new admission, a teenage boy many of the staff had known for years. His friends and family were crowded into the family waiting area. Some of the teenagers were seeing this side of their friend’s existence, the machines and tenuousness and sterility, for the first time. They were lining up to say a probable goodbye. They milled around outside his room, one with a guitar, unsure what to say or do. The family, though, had seen this all before. Later in the evening, the teenagers had left the patient and his family alone. We could not help but overhear snippets from Sophie’s room next door.

“… and if that does not work, then we should give him his wish.”

His wish seemed to be to avoid further suffering.

The next morning the room was empty and spotlessly clean, ready for the next patient.

Sophie continued to be on hefty, but slowly tapering doses of narcotics, but she had become easier to manage, so after more than three weeks in the ICU, she was transferred down the hall to Boston’s step down unit. Again there was a playroom. Again there was talk of home. Now that Sophie was a little older, we would wheel around the hospital units in a wagon or it a little car. She and I both loved the figure eight loops we would do around the floor and, when I was feeling daring, the medically unauthorized trips in the elevator down to the ground floor to visit the cafeteria or to admire the oversized falling ball sculpture in Boston’s main lobby. Anything was better than the claustrophobic boredom of sitting in a shared double room all day trying to entertain an eight month old.

As soon as the nurse practitioner mentioned the words “discharge papers,” however, we were back in the ICU. Sophie had developed in inability to keep down most of her milk even though it was going directly to her stomach through a naso-gastric tube, and had also built up some fluid on her lungs despite pretty good blood pressures. The fluid buildup was quickly diagnosed as a common issue, and fixed within a week. The inability to keep milk down, however, was more of a mystery, as well as making Sophie tired and hungry and cranky.

We went on twelve-hour shifts to cope with Sophie's puking issues, me during the day, and Jana at night. The only time we saw each other, for almost a week, was when we relieved each other from duty in Sophie's room. This kind of existence - basically cut off from everyone who cared about us while dealing with the most stressful situation we had ever faced - was hugely depressing. I coped by eating fatty food from cheap restaurants a lot. Banana stuffed French Toast. Eggs Benedict with sausage patties. Cream soups. Crepes with double cheese. Three times a day. I never really asked Jana how she coped, but I expected it involved crying and reading about heterotaxy.

We sent Sophie for the gut-doctors to look at using this study or that, but they couldn't find any reason for her to throw up. While we were searching for the answer to this riddle, however, Sophie’s symptoms dropped away a little. Not enough to say she was cured, but enough to send her to the floor.

Talk of home started almost immediately we were back out of the ICU. Not so much home as in "our house," but home as in "a transfer to the hospital at Michigan," so at least Jana and I could be at our house. There was no question of trusting us alone with her in the car for two days, but there was talk of flying her to Detroit. We assumed they were talking about a commercial flight for $500; they were actually talking about a medical charter plane for $25,000. We desperately wanted to go home and escape the downbeat rituals of shared medical family housing where people eat their individual microwave dinners silently in a shared dining room. But insurance said no. Sophie was already getting great care, Blue Cross said, so why pay the cost of a flight to get the same kind of care somewhere else?

We brainstormed and asked for advice, confident that we could talk Blue Cross around this time as we had in September. Even with the in-state discount that Blue Cross has somehow obtained for Sophie, Boston was a substantially more expensive hospital than Michigan, so after a few tense phone conversations, Blue Cross finally agreed to cover a medical flight for Sophie to Ann Arbor, not out of medical necessity or sympathy for us, but out of simple concern for minimizing their overall costs.

Sophie's first private jet flight

On November 19, six weeks after checking in at Children’s Hospital of Boston, Sophie left in an ambulance with Jana, bound for the airport. The University of Michigan’s survival plane was waiting for them.

I was bound in a different direction – to the parking lot, loaded down with a wagon full of Sophie’s gear, including big white insulated box. The only one we could find was labeled “Human Blood” on the outside, but it was actually filled with Jana’s leftover frozen breast milk. She had stopped pumping, and Boston had now put Sophie on a special synthetic formula to help with her chylous effusion and limit her vomiting as much as possible, but we wanted to keep the milk in case Sophie needed it later.

I was looking forward to the drive. Eight hundred miles where I could do literally nothing for Sophie. A night to myself. The driving would be boring, but boring sounded OK to me. When I was 90 miles out of Boston, Jana texted to say they were on their way.

“Wheels up.”

Through Massachusetts I was treated to the sights of the early winter forest in the afternoon light, with a few flares of red leaves among the brittle branches, and small cliffs rising above the road as it wound up the valley floors.

“Wheels down” said a text from Jana while I was still in Massachusetts. That was quick.

Albany traffic at dusk was heavy, but once the rush had died away I knew I could look forward to open roads all across upstate New York. My destination for the night was Niagara Falls, Canada, home of cheap hotels and all-round amusing tackiness.

The next morning, after a traditional Canadian breakfast of donuts at Tim Horton’s, I drove quickly across the plains of lower Ontario, bound for the American border. I arrived at around two, after a duty free stop for Canadian Cadbury chocolate, made properly in the UK rather than badly in Hershey, PA. When I got to the front of the line at the twelve-lane border crossing, there was a sudden flurry of activity three lanes away. An extra guard came up to a sedan waiting to cross. Immediately the trunk opened and a dazed man jumped out and ran in no particular direction. Border guards flooded the area. The woman driving the car got out, also to run in no particular direction. Neither got far.

After the commotion died down, I was called forward. Our bikes obscured the number plate on the rear of my car, so the border guard had to get out of her booth to get the number. As she went past the car to peer under the bikes, she looked in at the giant pile of stuff packed messily in the back seat and trunk.

“Where are you off to?”

“Ann Arbor. I live there.”

“And what were you doing in Canada?”

“I actually came from Boston. I’m just cutting through?”

“Uh huh. And where’s your kid?”

I paused. My kid? How did she know?

The empty car seat.

“Oh. She flew ahead. She’s already at the hospital in Ann Arbor.”

“The hospital?”

“Yes, she was in hospital in Boston, and they flew her by medical transport to the hospital in Michigan. They’re letting her finish her recovery closer to home.”

“Oh,” she said, taken aback a little “I’m sorry for her illness.” She had heard a lot of stories at the border, I guessed, but this one was probably not too common.

“And what is in the white box, sir?”

“Breast milk.”

“It says ‘Human Blood’ on the box, sir.”

I had forgotten. I didn’t know where her mind was going with this, but I was sure it involved suspicion. Awkwardness reigned until she finally relented and let me go, and I drove out onto the familiar litter-decorated highways of Michigan once more.