5

Gail Wright, a children’s cardiologist at Stanford, took the last steps in her 13-year postgraduate training at Michigan. That is how she knew Carly. And because she knew Carly, she came to know Sophie.

We needed Gail for two reasons: first to keep an eye on Sophie's heart for a few months; and second to help us give her a feeding tube direct into her abdomen. Nobody had figured out why Sophie was continuing to vomit up most of her food, despite multiple x-ray studies and even a study at Michigan where a endoscope was shoved down her esophagus. Maybe, we thought, the irritation from the feeding tube snaking down from her nose to her stomach was causing a problem. The solution, then, would be to surgically insert a port that goes directly to her belly, meaning we could spare her the nose tube.

There were, as usual, multiple options, and disagreement about which was the best. A “G-tube” that goes to Sophie’s stomach? Or a “G-J tube” that does that and goes direct to her intestines, too? An add-on “Nissen” option to make is physically hard to vomit? Yes? "Tight" or "floppy?"

We hit the books once more, again flying essentially blind, and opted for a G-tube with no Nissen. It if worked, we had mucked with her system as little as possible; if it didn't work, we could always do more later.

“Now, our anesthesia team is excellent. One of the best. But there is always a slim chance that things go wrong...”

Gail was delivering a speech we had heard many times before. Our first meeting had gone well, we had a good rapport with Gail, and she had declared Sophie to be in good shape.

“...so if something were to happen, would you want us to revive her? You could decide she has already suffered enough.”

We had not expected that part.

No doctor had given us the option to let Sophie die since her first few echocardiograms while still in the womb. Was this Gail’s personality talking, or a change in Sophie? We told ourselves, hopefully, that it was Gail.

We had become pregnant with our second child, another girl, soon after Sophie came home from the hospital at Michigan. We called her dos in utero and were terrified about her heart. Would dos have the same issues as Sophie? If so, we knew we couldn't handle doing it all again. We would terminate the pregnancy in that case. The tests we had done at Stanford showed dos' heart looked fine, but she failed a screen for neural tube defects. What was wrong with us? Were we just incapable of having a healthy baby? Should we keep it? After three very stressful days, we found the only thing we were a victim of was a 94% false positive rate on the screening test. Little dos was declared OK. But Jana wouldn't believe success until she held it in her arms.

The first time we tried for Sophie’s G-tube surgery, we got all the way into the pre-op room before the anesthesiologist canceled it. The low potassium level in her blood could be dangerous under anesthesia, so we were sent home with potassium supplements.

Our second attempt two weeks later was interrupted by her pre-operative checkup, which showed fluid buildup on her lungs. We checked immediately into hospital to dry her lungs out in preparation for the surgery. We didn't dry her out in time, so we went home.

Why did Sophie have fluid buildup on her lungs? Earlier buildups had made us worry about Sophie’s heart, her newly functional left ventricle and newly-split mitral valve especially. Gail looked, and found nothing.

On our third attempt, we checked in pre-emptively to make sure Sophie’s heart was OK, lungs were dry, and potassium was up. She was ready. But on the morning there was a delay. Then another. Then the surgeon wanted to talk to us. She had changed her mind, and now thought a GJ-tube was the best option. We listened, and agreed. But she didn't do GJ-tubes, interventional radiology did those. So we went home.

The fourth time we tried for a feeding tube placement, six weeks after the initial attempt, was a nervous time. Gail was concerned about Sophie’s heart, and anesthesia was worried about keeping her safe in the OR. The procedure went off without a hitch, and we could throw Sophie’s nose tubes away. We resisted the urge to have a ceremonial nose tube bonfire.

We would start her on intestinal feeds to get her weight up, because it isn't possible to vomit from the intestines all the way up. We would try our luck with stomach feeds later.

*

As Sophie recovered from her GJ tube surgery, Gail took an echo of her heart. It showed considerable mitral valve damage. The valve did not open as far as it should, making blood flow more difficult; and it did not close tightly, either, meaning some blood went back the wrong way when the ventricle fired below. This was why Sophie’s lungs had been wet.

Sensing trouble, Gail ordered a visit to the cath lab. It showed high pressure throughout the left side of Sophie’s heart. Left alone, it would kill her, causing wear and tear on the heart muscles, fluid buildup in the lungs and a lack of oxygen in the blood, and permanent stiffening of the lungs themselves. It needed to be fixed within days to weeks, and we were half a continent away from the surgeons who knew Sophie the best. In Boston Dr del Nido had mentioned the valve repair being tricky and the possibility of future work on the valves. Technically, he was right, although he must have hoped the valves he created would last more than eight months.

After the echo and the cath we set about drying Sophie’s lungs out so she could go home. The initial estimate was two days. It took two weeks. Gail sent the echo and cath information to her own chief surgeon at Stanford, Frank Hanley, and also to Michigan and Boston.

We met first with Dr Hanley at Stanford. The first thing we noticed was he did not dress like other surgeons. He wore his scrubs all the time, and rejected the standard white doctoring coat in favor of a brown tweed sports coat. He looked like a homeless medical student.

Dr Hanley said that if Sophie’s mitral valve were either only leaky or only narrowed, fixing it would be very possible. He used the analogy of a plug and a plughole. If a plug is leaking around the sides, you can make the plughole smaller to fix it. If the plughole is too small, you can cut the plug a bit to fit it in the hole, even if it risks a little leaking. But Sophie’s valve was both leaky and narrowed, meaning the normal techniques were much less likely to work. He wanted to fix the valve, though, and gave himself a “nominal 20%” chance of doing it. Otherwise, he would have to replace the valve.

Sophie had another valve issue, too. Her new pulmonary valve, which Dr del Nido had inserted in Boston, didn't really work any more. It was basically just an open pipe, running from her right ventricle to her lungs. It was not as big of a deal as the mitral valve, but if Sophie was going to be in the OR anyway, Dr Hanley suggested they may as well fix it, too.

But what kind of valve replacements, if necessary, should Sophie have? Again there were options, each with their pros and cons. There were metal and plastic mechanical valves, human tissue homograft valves, and pig or cow tissue heterograft valves. The mechanical valves lasted the longest, but required blood-thinning medication for life that made it easier for people to bleed. The homograft valves didn't require the blood-thinners, but were too weak for use in the high-pressure mitral position. And the heterograft valves were strong enough to use as a mitral, and didn't require the medicine, but didn't last as long as mechanical valves, and didn't come in sizes small enough for Sophie. Just as before, Jana and I were lost.

Dr Hanley suggested a mechanical mitral valve, the textbook option. For the pulmonary valve, he had a non-textbook suggestion. Another mechanical valve. These valves were generally not put there because valve strength wasn’t quite so important on the pulmonary side, and most doctors judged the risk from a weaker valve there as lower than the risks associated with blood thinners. But if Sophie were to have a mechanical mitral valve and need anticoagulation medicine anyway, why not just use a mechanical valve in the pulmonary position, too? There weren’t a lot of studies on this, because it was rarely done. But the little evidence Dr Hanley had seen encouraged him.

We felt comfortable with Dr Hanley, and he had clearly done his homework on Sophie’s case. When we asked, he told us he would have recommended a 1V for her the previous year. “But,” he said, noticing our crestfallen looks, “hindsight is easy.”

We heard next from Carly at Michigan. She had consluted with Dr Bove, who was even more pessimistic about saving Sophie’s mitral valve than Dr Hanley. “Very low chance.” Michigan agreed with Dr Hanley’s idea of a mechanical mitral valve, but not with a mechanical pulmonary valve. Theirs was the textbook recommendation for both valves.

We also arranged to talk with Dr del Nido in Boston, fitting in around his OR schedule. As it happened, he called while we were treating a friend from New Zealand to an all-American tradition – lunchtime breakfast food at IHOP. Fortunately, it was nearly deserted, so while Sophie and our friend played with bacon and sausage, Jana and I crowded around a cell phone in an IHOP waiting area to talk with Harvard’s chief children’s heart surgeon.

Dr del Nido started by saying he felt good about saving Sophie’s native mitral valve. That was a pleasant surprise.

“Dr Hanley said it was 20% chance, and Dr Bove said ‘very low.’ What chance to you give it?”

“Oh,” he said with a chuckle, “I’m a bit more optimistic than that. I think we have a better than 50/50 chance of saving the valve.”

We were shocked. “Wow.”

“As far as I can see, the problems with Sophie’s valve are annular dilation and posterior leaflet immobility. The dilation means that the valve doesn’t seal properly, causing the leakage. The leaflet immobility causes the stenosis. I would fix the dilation by simply closing up the annular ring with sutures, making it a smaller circle and a snugger fit. And for the leaflet, I would split the anchor strings vertically to give them more flex and the leaflet more movement.”

I understood the first part about annular dilation. That was the plugs-and-plugholes Dr Hanley had mentioned. But the bit about anchor strings was new to us. Dr del Nido said we should think of them as rubber bands. Thick rubber bands were hard to stretch a long way, but if you cut carefully down the middle, you could get two thinner rubber bands with more flex in them. That was his plan for Sophie’s too-stiff anchoring strings.

On the pulmonary side, Dr del Nido disagreed with both Stanford and Michigan, although he did think Dr Hanley’s idea was “an interesting concept.” There was a new valve on the market called a melody valve, which was revolutionary because it could be replaced by catheter, saving the patient from surgery altogether. The valve folds like intricate origami into a tiny tube-shaped package, so that it can fit behind the tip of the catheter. Once the catheter is in the right spot in a pulmonary artery, some air inserted through the catheter tube deploys the valve and it expands into place. Sophie wasn’t exactly a candidate for a catheter-placed melody valve just yet, because she was so small. But Dr del Nido said that if we placed a melody valve this time in the OR, then next time we needed to replace it we could do it via catheter.

Yet again, we had consulted three of the best heart centers in North America, and received three different surgical plans. Once more, it was up to Jana and me to sift through their arguments and make the decision. We read articles and made spreadsheets and asked the doctors to comment on what the other doctors had said, all of which was tremendously stressful and confusing. Most evenings, Jana and I talked mainly about Sophie, as there were decisions to me made and hospital experiences to process. Jana, distinctly pregnant with dos, tired easily and was in bed only a couple of hours after getting home from the hospital. We didn't talk openly, as Jana was sick of being told to cheer up and I was sick of being told to be more sad and angry. Every now and then we had confrontations, with me demanding the immediate return of the "happy Jana" I remembered from years before, and her reminding me how much everything sucked. We were drifting further and further apart, Jana sobbing into her pillow while I looked on and did almost nothing to comfort her.

On August 9th we borrowed a group study room in the Redwood City Public Library, first to call Dr Hanley and get his reaction to the Michigan and Boston proposals, and then to figure out what to do. Dr Hanley was pretty dismissive of the melody valve option, saying that the benefit of a melody valve is not having to open the person’s chest up. If the chest was already open, there was no advantage. He also said that Dr del Nido's descriptions about dissecting the "rubber bands" inside Sophie's heart, which sounded almost like sci-fi fantasy to us, were the standard way any decent heart surgeon would try to save this valve. The difference between del Nido and the others wasn't that he could do things they couldn't, it was that he told us the details while the others didn't.

After talking with Dr Hanley, we tried to make up our minds. The surgeons all had the same view about wanting to save Sophie’s own mitral valve if they could, they just differed in their view of how likely it was. This was just a difference in bravado levels.

All the surgeons had recommended a mechanical mitral valve if replacement became necessary, but they had recommended different sizes. Michigan and Stanford wanted to go big to keep Sophie out of the OR for longer afterwards; Boston wanted to go small to make sure it fit properly. We hadn't the faintest idea who was right.

And then there were the three different options on the table on the pulmonary side. There is no way we were equipped to make judgments about the likely future performance of a mechanical valve in a lower pressure part of the heart. It was ridiculous to ask us to make those judgments. I had only passed physics in high school by cheating on a multiple choice exam.

We were lost. On the drive home, I asked Jana if she had a gut feeling about what we should do. She did. Did I? I did. It was clear that we were not going to be able to reason our way through this – there was just too much we did not know and could not learn in time. All the surgeons had told us that Sophie needed an operation very soon. We had tentative surgical bookings at Boston and Stanford within a week. We would have to decide now, and decide on instinct. Jana spoke first:

“I think we should stay here.”

“Me too.”