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On Sophie’s first night home, she could not sleep. Jana wanted rest, but Sophie’s cries prevented that. So Jana sang for her. It was an original nursery rhyme, to the tune of a cat food commercial.

Sophie, Sophie, Sophie, Sophie.
Sophie, Sophie, Sophie, Sophie.
[Repeat.]

Jana’s sister was staying with us, helping us adjust. She cracked up in her room. And I cracked up in our room. And although Sophie kept crying and Jana was not asleep, some of the tension evaporated out of the house. But some remained.

Being at home with a sick child had challenges. Medication was a big one. Fifteen doses across eight different times of the day and night. 12, 3, 8am, and 12, 2, 4, 8pm. We had little plastic cups containing oral syringes filled with multicolored meds. We replenished them daily. Sometimes Sophie spat up just after we gave her a med. We peered at the spit to see what color it was, so we would know what to give her again. We rarely missed a dose, but felt guilty and nervous when we did. I was sure I missed more doses than Jana, and sometimes I didn't tell her when I did, fearing the angst I would cause even more than the look of disappointment she would give me.

Other challenges were the battery of appointments at the hospital, the stream of medical visitors, and constant vigilance for signs of cardiac distress. And then there are all the other things that happen with a normal baby. Jana had some parental leave but I was still at work. We coped as best we could.

Within six weeks of leaving the hospital we were back, this time to get Sophie’s intestines fixed. Dr Mychaliska had done this surgery many times. He would make a small incision in Sophie’s abdomen, take out her appendix while he was there, unpack her malrotated intestines, scrape the intestinal cavity walls so they would inflame, scar, and adhere, then re-pack her intestines back into the now sticky cavity, this time rotated properly. He was done within two hours, and Sophie was home within four days.

For the next three months, Sophie was close to normal. She learned to roll over and play with toys and annoy the cat. She smiled and waved frantically at things. We had family visitors come to stay, who were aghast at all the medication but loved playing with her all the same.

Caring for Sophie still took considerable time and energy, and there was little left over for Jana and I to connect with each other, even less than with a typical newborn. So we didn't really talk. Jana was scared to tell me her nightmares about Sophie, worried I would dismiss them as transitory post-partum depression. And I did not share my dreams for my daughter, as I did not want them stomped on.

In the early summer, we took Sophie out to California to meet the rest of Jana’s family, where she was subjected to typical baby injustices like being bathed in a hotel sink and being bossed around by older cousins. She still had to have fortified breast milk delivered by bottle, which was an awful inconvenience for Jana, who pumped eight times each day. There were times when Sophie did not want to eat at all, and we struggled to get her growing. For a few days we had to feed her milk three milliliters at a time, forced into the side of her cheek through an oral syringe. This was massively frustrating, especially for Jana who was the lead feeder during the day, but it caused no mortal fear, at least for me.

Children’s heart surgeons have a very different job from adult heart surgeons. With adults, two basic things happen to hearts: pipes get clogged; or valves and muscles wear out. The solution is to bypass the blocked pipe, or to replace the broken valve, or to transplant the heart. The range of surgical procedures is relatively small.

In young children, the variety of conditions is much wider. The heart is a complex structure. There are four muscle-lined chambers, a central wall dividing the red blood from the blue, four valves, two great arteries, two coronary arteries, a set of major veins, other pulmonary veins, a network of electrical connections, and more. Any element of this system can be too big, too small, in the wrong place, doubled up, or missing altogether. Solutions involve changing the basic structure of the malformed heart.

Adult heart surgeons give well-designed hearts a tune up. Children’s heart surgeons make badly designed hearts viable.

In Sophie’s case, the surgeons had two basic options. The first route was to make Sophie’s heart operate much like a normal heart. Dr Bove would construct the missing central heart wall, making it an unusual shape to accommodate her mislocated aorta. He would split her large central heart valve into two smaller ones, one on each side of the wall. He would create a new pipe leading from the right side of her heart out to her lungs, replacing the function of her BT shunt (which Dr Bove would take down). He would also create new miniature pipes inside her heart to take blood initially dumped in the wrong place to instead go somewhere useful.

This “biventricular” or “2V” repair was a highly complex operation, quite new in kids like Sophie. Splitting an internal heart valve in two is exceptionally difficult, as is creating little internal pathways for relocating blood in the heart. There was more danger in the OR down this path. But the benefit further on was a heart that looked and functioned much like normal. Sophie could more easily be an ordinary kid, and adult, if she had this repair.

Key: Blue is blue blood, red is red blood, purple is mixed blood. Green pipes are surgically implanted. Black stripes are other new structures, like closed off pipes or new walls or reconfigured valves.

The main alternative approach to biventricular repair is the "Fontan procedure." The Fontan is a solution for a wide range of complex heart problems, and the idea is to make do with the way the heart has developed naturally. If the heart has only one large pumping chamber, which Sophie’s did because of the lack of a central heart wall, then the body should make do with one pump. The way to achieve that is to cut off all the connections between pipes and chambers carrying blue blood and pipes and chambers containing red blood. With some new connections, blue blood would make its way to the lungs without the aid of a second pump, pushed along only buy the weight of the blood coming in behind it. Major veins would be connected directly to pulmonary arteries. Red blood going out to the body, however, would enjoy the services of the large, single heart pump. This shift in anatomy is too much for a child to bear all at once, so the Fontan usually takes place over two stages.

The Fontan is safer than the 2V over the short term, mainly because there is less complex fiddling around in the depths of the heart itself. But over the long term, Fontan kids do not appear to survive as long, or have as high a quality of life. The tradeoff was simple: short term risk vs long term risk. But how much of each kind of risk Sophie would face was anyone’s guess.

Dr Bove thought that on balance Sophie would do better with the Fontan circulation, also known as a one ventricle or 1V solution. But he said she was a pretty borderline case. Sophie’s left ventricle, which pumps blood to the body, would be smaller than usual in a 2V repair, because of the crookedness of Sophie’s incomplete central heart wall. Would it be strong enough to carry the load? Dr Bove wasn’t convinced. He was also worried about splitting her central heart valve in two, especially because it had already started to leak.

We had suspected this would be his recommendation, but it made us both sad nonetheless. Any time you settle for a second-best anatomy, it is a kind of defeat. In Dr Bove's office, Jana adopted my "pretend professional" disposition of stern attention to the facts, although inside I think she was wanting to let out a desperate scream for Sophie to get the other surgery. Dr Bove clearly sensed our hope for 2V, telling us that he was normally pretty aggressive about pursuing a 2V track whenever he could, but that in his experience 1V can work very well, too.

We agreed to his plan, of course. He was a world-leading surgeon, and this was our first time.

*

Sophie’s first operation towards the Fontan, a Hemi Fontan, was set for the end of August. Depending on the complexity of the case, she could be in the OR for six hours or more. Most of the work in the operation, however, took place outside the heart itself, unlike the biventricular repair approach, which would help with Sophie’s recovery. We found blog after blog on the internet dedicated to kids walking around with Fontan anatomy. These kids were alive, no matter the complications they detailed in their writing. With each passing day, we each grew more comfortable with Dr Bove’s decision. Soon Sophie’s biggest surgery would be behind her – the Hemi Fontan being a much larger undertaking than the later Fontan Completion.

Then two weeks before the surgery, we received the second opinion we had sought from Boston Children’s Hospital, another world-leading center:

Thank you for your patience. Dr. del Nido reviewed the case with one of the echocardiographers from our Department. We believe that she is a candidate for a biventricular repair based on the anatomy. I will try to contact your pediatric cardiologist in the next several days to discuss.

Please forgive our delay in responding.

D. Fulton

Two top hospitals had contradictory advice on what to do for our daughter.

We immediately sent for a tie-breaking third opinion, this time from Dr Richard Jonas at National Children’s Hospital in Washington DC. Jana read everything she could find on MEDLINE about the two approaches, because the surgeons had both said it would ultimately be our call. She passed on the stuff she thought was most interesting to me.

We tried valiantly to understand the medical journals, but while we had statistical training we were not medical doctors, and I am sure we came up short several times. Based on my amateur reading, at one point I even tried to convince Dr Bove to consider a different surgery again for Sophie, the Nokaidoh, because I thought it sounded good in a journal. He was polite but firm in his dismissal, saying:

“Even Dr Nokaidoh would not recommend the Nokaidoh in Sophie’s case.”

Meanwhile the cardiologists at Michigan and Boston were talking among themselves, and with each other, about Sophie. Carly told us that the strong consensus was that there was no wrong decision here. Sophie really was borderline. Despite some misgivings about Boston’s plan, the Michigan team did update its own plan after hearing it. Michigan now recommended a second, upsized BT shunt now, to again tide Sophie over so she could grow. Then in a year they would try the 2V repair, figuring it would be easier to do the difficult valve work when the valve itself was bigger. Going for the 2V one way or another all sounded really positive to Jana and I, until Carly said:

“The flip side, though, is that she is more likely to die in the OR.”

We arranged to talk with Dave Fulton, the cardiologist assigned to Sophie’s case at Boston, and Pedro del Nido, the chief surgeon there. They felt confident about their ability to fix Sophie’s valves now and felt good about the prospects for her left ventricle growing into its new role, especially if the surgery was done early. They pointed out Michigan's proposed re-shunt was a ticket to an extra year for Sophie with a leaky heart valve, which was not good for her.

We had two world-leading centers that had considered each other’s point of view and agreed to disagree. Michigan, initially hesitant about Sophie’s prospects with a 2V, wanted to wait and see, hoping maybe for a 2V repair in a year. Boston wanted to do the 2V now. The third opinion had not materialized, and we had to decide soon as Sophie was quickly outgrowing her tiny shunt, placed in her first week of life. The pressure on us, amateur newcomers in a world of exceptionally specialized and experienced professionals, was utterly unfair. It was nobody’s fault, of course, even if Jana sometimes cast fruitlessly around for reasons to blame herself. The unfairness came from the universe-at-large.

We felt we had three options: 1V at Michigan; Michigan’s wait-and-see plan; 2V at Boston. One day, we decided to just decide. We talked about pros and cons of the various options. Point met with counterpoint – none of them our own, but rather a series of partly, we hoped mainly, understood conversations with the two teams and rememberences from complicated journal articles. I got up and found two scraps of paper and some pens. We stared at each other for a moment, caught in a gaze almost like two deer in each others' headlights, and wrote our choices.

I preferred Boston’s 2V option, because it gave Sophie the best chance of a relatively normal life. 1V sounded like a path to a slow, lingering death to me – learning at school only for the sake of being there, not as preparation for later. And the re-shunt plan sounded half-baked, a worst-of-both-worlds compromise. I would rather roll the dice and give Sophie a chance than play it safe and consign her for sure to less of a life. I folded my paper in half, but held on to it.

“What did you write?” Jana asked.

“Are we swapping paper, or just reading out what we wrote?”

“You can just say.”

I told Jana that I voted for Boston’s plan. I didn't explain why. She knew why, just as I knew the reasons she could use to justify any of the three choices on offer. She looked at me for a moment, and put her scrap of paper on the table:

"2V @ Bos"

Boston scheduled us in for October 7, and we started to arrange the logistics of moving cities for weeks on end while Sophie recovered from the surgery. Then we got a letter from Blue Cross. It had denied our request to have the surgery in Boston.

It felt like another kick in the teeth.

Both Jana and I had a habit of meeting immediate adversity with resolve, not despair, no matter what we were thinking internally. We knew that an insurance company’s first instinct in tricky circumstances is usually to say no. It was now up to us to persuade them to say yes. We consulted a lawyer, and drafted an appeal that involved strong-arming Carly, the team at Boston, and Sophie’s everyday pediatrician to write letters declaring things that were not lies, but were not God’s Honest Truth, either. They all agreed and it came together remarkably fast, and then we waited.

Just three weeks before Sophie’s surgery date in Boston, Blue Cross rescinded its earlier denial and agreed to cover the surgery in full. We were at once elated, exhausted and scared, but I was pleased that I could put away the next phase of our efforts to get the surgery covered, which would have probably involved prostituting ourselves to the news media.

On September 16, a day after our insurance authorization came though, we emailed Dr Jonas in Washington to say we had decided for the 2V repair in Boston, and that his third opinion was no longer needed. Within hours he emailed his third opinion anyway:

My recommendation in conjunction with Dr Craig Sable from our echocardiography group is to follow the single ventricle track. There is an important risk of AV valve regurgitation and baffle pathway obstruction if a 2V approach is followed. Dr Del Nido is generally recognized as one of the most aggressive individuals in terms of following a 2V track in a number of situations which I would describe as controversial. However he is an excellent surgeon who I am sure will do his best for Sophie and your family.

Sincerely,

Richard Jonas

This was the least effective tiebreaker possible. Given all the movements since we had asked for the third opinion, Dr Jonas’ letter had actually reinstated a tie. And we were puzzled about why he would go out of his way to bad mouth Dr del Nido to a pair of complete strangers, too.

We did not have the strength to open up the 1V vs 2V discussion all over again. We basically ignored Dr Jonas.