7

Sophie came home with many drugs. Aldactone, Amoxicillin, Aspirin, Atrovent, Diuril, Lovonox, Lasix, Magnesium Gluconate, Reglan, Plavix, Potassium, Prilosec, Sodium Chloride, Sotolol, and Viagra. The Lovonox, an outpatient version of heparin, had incredibly precise dosing – exactly 0.19 milliliters every twelve hours. It also had to be injected under Sophie’s skin, a new procedure that she despised. We gave her 37 doses of medicine a day.

I slept on the floor of Sophie’s room so I could give her breathing treatments at 11pm and 5am, medicine at midnight, change her milk at 2am, and get up with her around 6am. Jana slept in our room with Paige, waking every three hours to feed her and comfort her. During the day, I tried to take Sophie out for a neighborhood walk, letting her enjoy the birds and giving me some fresh air so I did not fall asleep in the house. Dealing with a newborn and a sick toddler at the same time was all consuming, but I did not care. We were a family of four all living under one roof. It felt like victory, at least to me. Jana didn't think it felt like such a victory, however, and she collapsed into bed whenever she didn't have an immediate task. Fearing another too-happy-versus-too-sad confrontation, I let her be.

Although Sophie was no longer living at Stanford, we visited almost daily for one appointment or another. Our Thursday cardiology appointment included an echo, which showed some increase in the blood pressure gradient over her mitral valve. I was worried, but the staff told me it wasn’t too bad, and sent us home with orders of vigilance.

In the late afternoon, I took Sophie for a short hike in the hills above the Stanford campus. There were sweeping views, a cooling breeze, and some of Sophie's favorite wildlife, too. “Bir! Bir!” she cried, pointing at birds cruising a thermal. At the top of the hill, I hugged her close for warmth. Together, we admired the dusk-time view as the first few lights flickered up from Silicon Valley, and in the other direction the sun set over the Pacific.

Gail had become concerned about the elevated mitral valve pressure gradient, and asked us to come in for another echo first thing in the morning. The mood in our house turned instantly dark.

I drove Sophie down for her follow-up echo with a bag packed, just in case. Jana cried as she waved us goodbye. Sophie was coughing more despite her breathing treatments, but had been happily playing with her new doll Paige. It was hard to make her leave, knowing she might not be back that night.

Sophie was so used to the echo procedure that she regularly slept through them. It was almost Pavlovian. I asked the technician the pressure gradient across Sophie’s mitral valve. While echo technicians weren't allowed to interpret or explain their pictures for us, that being the cardiologist's job, they could give us basic factual information if we asked for it. In a normal person there was no mitral valve gradient, because the blood pressure above and below the valve were the same. 0. As the pressure difference got larger, it indicated an issue with the mitral valve. Bigger numbers meant bigger problems. At ten, they labeled it severe.

“23.”

I slumped, defeated. I knew exactly what would come next. An hour later, Sophie was back in an ICU bed, and a cardiologist was giving me a speech about the risks associated with tPA. As Sophie played with a nurse she knew all too well, I went to the car to fetch her things.

*

Over 48 hours, Sophie’s third round of tPA successfully unclogged her mitral valve. Sitting in the ICU, back on a heparin drip, she was safe. Nonetheless it was clear a change of plan was needed. The mechanical valve had to go. But there was no animal tissue mitral valve small enough for Sophie’s heart. We had exhausted all the textbook approaches.

One option was to bunker down in the ICU, keep the heparin flowing, and list Sophie for heart transplant. But nobody could yet accept that defeat – Sophie’s heart worked beautifully when her valves were clear. Another option was to try a second mechanical valve, thinking there may have been an issue with the first one. Everyone at Stanford thought this was just throwing good money after bad. Thinking outside the box, Dr Hanley had a third idea. Porcine pulmonary valves, like the conduit Sophie had already, came in smaller sizes than porcine mitral valves. If he could cut away the conduit part, leaving just a cuff to sew the valve to the surrounding tissue, he could use the pulmonary valve as a mitral valve. It would not last a lifetime, but it could give Sophie a year or so, at which point she would be big enough for a proper porcine mitral valve. This, he thought, had never been done before.

We sent word of Sophie’s new issues to Boston and Michigan, along with Stanford’s suggestion. Michigan replied first, having had yet another conference about Sophie. They agreed the current valve had to go, and that transplant should be a last resort. Despite some misgivings in the Michigan team, Carly and Dr Bove both thought that an animal valve was the best option, and liked Dr Hanley’s cut-down-conduit plan. After receiving this advice, we shared it with Gail.

“We have two centers in actual agreement,” said Jana “, I can't believe it.”

Neither could Gail. “I don’t know what to do with myself.”

“Don't worry,” I said with a wry smile, “I'll just call Boston.”

On cue, Boston had a different recommendation. It was also for an animal tissue valve, but the particular arrangement was quite different. Instead of cutting away the conduit on a porcine pulmonary valve, they recommended inserting a bovine melody valve, complete with a full stent housing, in the mitral position. They said the advantage of this was you could put in a too-big valve initially, and only partly expand the stent. The valve would still work. Then as Sophie grew the stent would be further expanded in the cath lab, saving her poor sternum at least one date with the saw. Boston’s leader in this area said he had done it six times previously, but admitted only one was in the mitral position and that child had a transplant shortly afterwards.

Melody valve

Dr Hanley was very down on this idea, and on any valve with depth. He quoted studies showing these Top Hat valves, named because the valve ring and housing look kind of like an upside down top hat, had a bleak history of, of all things, clots. The struts were also prone to metal fatigue, with a risk of tiny shards of metal being ejected into Sophie’s bloodstream. If one reached her brain, that would be the end.

I emailed the doctor at Boston to pass on Dr Hanley’s concerns. He emailed back professing “no idea what Frank’s top hat literature refers to” and giving glowing statistics about his stent mounted valves. I sent this email to Gail and Dr Hanley, and the last I heard of it the two surgeons were emailing directly, I assumed to electronically wave research studies at each other.

The ICU doctors at Stanford knew about our options. We asked some for their opinions. They all agreed with their boss, and the more junior they got, the more uncritical the agreement became. Some of the senior cardiologists were, however, more fulsome in their thoughts. One, who had worked at Boston previously, was especially forthright.

“You know, the thing about the guys at Boston is… Well, they're really cowboys.”

“Yeah,” I replied, “I had heard. But a cowboy is just what we need now, right? Playing it safe isn’t going to get us far.”

“Well, there are cowboys and there are cowboys, you know.” The cardiologist fixed me with a stare, eyebrows raised.


Rob Salmond
Surgery probably on Friday
Dear everyone

Sophie will have further heart surgery to replace her mitral valve, tentatively scheduled for this Friday. While her mitral valve did de-clot over the weekend and remains clear this morning, it has now clotted on multiple occasions even in the face of lots of anticoagulation treatment and Sophie’s medical team has decided that mechanical valves are not safe for her at this point.

We will be implanting a porcine valve in its place.

There are plenty of people walking around with porcine mitral valves, but those people are all substantially bigger than Sophie. Porcine prosthetic mitral valves simply aren’t made in her size. To get around this, the surgeon is going to jury rig a mitral valve by taking a commonly-used porcine pulmonary valved-conduit (which comes in smaller sizes) and removing the conduit part to leave only a valve, which he will then try to fit into the mitral position. Sophie will, to the surgeon’s knowledge, be the first kid to receive this particular form of bioprosthesis. There was one other option on the table proposed by Boston, involving a bovine valve mounted inside a stent in the mitral position. The N on that treatment currently stands at one, and that kid got a transplant two months later so we have no evidence on how that solution works beyond two months, either. We asked Stanford and also Michigan to consider both options, and both strongly preferred the valved-conduit. So that is what we are going to try.

While we are taking on some risk with this experimental surgery, we think it has a reasonable chance of working for her, maybe for a year or two (at which point we can potentially upsize the valve). Also, if this treatment proves unsuccessful, the team’s best estimate is that the valve would likely fail slowly, allowing us all time to regroup and come up with a new plan. Having heard a sobering presentation about the problems often associated with heart transplants in kids like Sophie, we are more willing than ever to take some risks in order to avoid that life for her.

We will try to keep you up to date as we learn how the surgery goes. Please keep Sophie in your thoughts on Friday. She’s a tough little girl, but she could use all the positive vibes we can muster. Thanks so much for your support and love for Sophie.

Cheers,
Jana and Rob
November 17, 2010 at 4:03pm

We decided to again trust Dr Hanley’s judgment, and opt for his cut-down conduit experiment. Our first attempt for the surgery failed because Sophie had spiked a fever the night before. When Dr Hanley learned of the delay, his disappointment was obvious. He was looking forward to the challenge.

Our second attempt was the day before Thanksgiving. Sophie was due in the OR at 7am, so we got up at 5:30, delivering Paige to my visiting Mum on the way out the door. For the fifth day in a row, Sophie was in a wonderful mood. She laughed excitedly when her TV played the Dora theme, and was over the moon when we took her for a dawn ride. Sophie’s demeanor made it even harder to send her to the OR. Couldn’t we just let her enjoy herself for a few days? Gail said this was the best, safest time to take a child into surgery. A good mood signals a healthy body, which means better outcomes.

Anesthesia did not come until after eight, and when they arrived there was an issue with Sophie’s medical chart - Sophie’s file was so large it had crashed anesthesia’s computer. This was not a new issue - at rounds each morning the team had to start loading Sophie’s file one or two kids before coming to see her. The anesthesiologist called the hospital IT helpdesk, who not-very-helpfully suggested deleting parts of Sophie’s file to make it fit. Choosing which parts of a very sick child’s medical file to delete was apparently not a concern for the IT person - maybe he would have looked at the multiple valve replacement surgeries and deleted all but the last one, or kept a random sample of the hundred or so echo reports.

We of course ignored IT and figured out something else instead. Jana and I waved goodbye to a Sophie high as a kite on ketamine, smiling and playfully pulling on a doctor’s shirt as the OR elevator doors closed.

Later, we met Dr Hanley on his way to the OR, just as we had the month before. Almost three hours passed between saying goodbye to Sophie and saying hello to Dr Hanley. Prepping her for surgery was growing ever more difficult.

I had last-minute concerns about his plan. If these other porcine valves all had struts to keep them stable, wouldn't this one - which had no struts at all - be really unstable? Dr Hanley said not to worry, he was leaving some extra fabric on the valve, which he would sew to Sophie's heart to provide stability. This made me more nervous, because the more Dr Hanley told us, the more this valve sounded like one of these Top Hat valves he so disliked earlier. But I bit my tongue, as there were no other options.

“Well, it was arduous,” said Dr Hanley when we met him again outside Stanford’s ICU. Sophie had been in the OR for almost twelve hours, Dr Hanley for nearly nine. He had installed the cut-down porcine conduit valve, as planned, and fixed a few other smaller problems he had found "while poking around in there." After her long surgery, Sophie was again very puffy, much like after her 2V repair in Boston. Her tongue again spilled out of her mouth, and she had mountains of fluid accumulated throughout her body. She had to pee this excess fluid out over upcoming days, and we gave her diuretic medicine to help. She also had an air buildup in her chest that was partly crushing one lung, and was experiencing some heart rhythm problems, too.

While dealing with Sophie’s post-op concerns, we also learned about her potential as a transplant candidate, just in case. The doctors did not paint a pretty picture. There were many more people in need of hearts than hearts available. The wait could be brutally long, and many candidates died waiting for a heart. Even if a heart was found, there would be a regimen of medications and tests much more taxing than the one Sophie had endured already, along with the constant fear of her body rejecting the donor heart. Most transplanted hearts got rejected eventually. If Sophie had a transplant, chances are she would need multiple further hearts in order to live a full life.

There were four big factors that doctors considered when deciding how high to place a person on the transplant list. Three of them were no problem for Sophie. But the fourth issue was about Sophie’s immune system, and was a problem. Some people’s immune systems are especially sensitive to foreign tissue, and two things that usually make a person more sensitive are previous blood transfusions and earlier implantation of foreign objects. Sophie had long histories on both scores, and some blood test results showed her immune system was much more sensitive than most people's to foreign tissue. If Sophie ever did get a heart, there was a really high chance her body would reject it.

Gail asked whether we wanted Sophie on the list. We had an awful conversation. Putting Sophie on the list meant probably many months in hospital waiting for a heart that may never come. Even if it did, Sophie’s future with a new heart sounded bleak, not cheery. But it was maybe Sophie’s last chance. Ultimately Jana and I agreed not to list Sophie for transplant. We thought it would not be fair to subject her to much more pain and suffering, especially given how short-lived the transplant was likely to be. We agreed, even though the decision was severe. Either she would make it with the heart she had, or she would not make it.

Awake with breathing tube

More than two weeks after her surgery, we finally removed Sophie’s breathing tube. She had been awake for days, impervious to the sedatives, sternly watching TV over the top of the breathing apparatus. But even when the tube came out Sophie was not herself. Her movements were labored and effortful, she wasn’t as pleased to see us, and was less interested in playing. My routine with her involved an extra nap, because she was more easily exhausted. When I took her outside for rides, she was passive. Helicopters would fly overhead, and she would not look up as she had before. I showed her flowers, and she just stared at them blankly. We set up a play area for her in her room, and installed an extra long oxygen tube so it would reach. Her use of the toys was the deliberate, determined action of someone completing a task, not the joyful play of a toddler. We forced smiles on the outside as we helped her, but inside Jana and I wept. Where had our little girl gone? Would she ever be back?

Our official time as visiting scholars at Stanford was coming to an end, and so did our house rental. So we moved into the local Ronald McDonald house. Twice a day, on my trip between Ronald McDonald and the ICU, I walked through the Stanford Shopping Center, where the Palo Alto branch of the world’s elite had gathered to buy each other Christmas gifts. Their mood was celebratory as they forked over small fortunes at the Apple Store and Burberry Kids. While I walked with them, sharing their air, I did not share their state of mind.

One day a preacher reminded the shoppers about what he thought was the meaning of Christmas – repenting sin. He made me wonder: How did religion cope with dead children? They were too young to have accepted Jesus, and too young to be responsible for their sins. I had no idea which consideration was more important, and neither did Jana. Morbidly interested, over the next few days I read about dead infants when Sophie slept.

Christianity’s attitude to dead children had changed over time. Initially, Catholics took a stern view – unsaved people went to hell, sinners or otherwise. But as time wore on, the church became more permissive. Some came to believe, quoting support in scripture, that children who die before they know right from wrong, and before they had a chance to know the gospel, go straight to heaven. Not all Christians took this new view, but most probably did.

In Islam, there was no debate. Children who die under the age of 14 go to paradise, while anyone older is judged according to their actions.

The idea that, if Sophie were to die and if one of the religions was right, she would not automatically go to hell was comforting even though I was not religious. It showed leading religions did not damn my daughter just for being born sick.

Through December, Sophie got one infection after another, something sadly common among long term patients in ICUs the world over. She got a version of e.coli throughout her body, requiring antibiotics and the mask-and-glove treatment. Then she got another infection called VRE that was resistant to most of the available antibiotics, meaning even stricter precautions against touching her, isolation, and new, harsher antibiotics. These depressing episodes were doubly dangerous for Sophie. As well as all the usual risks with infections, there was also the risk that the infection would cling on to her artificial valves. If her valves became infected, we would have to replace them in the OR straight away. Our vigilance increased still further.

In one of our few periods without gloves and masks, one effect of Sophie’s narcotic dependence wore off. She had been on morphine for weeks, making her constipated. While sitting on me, the constipation stopped. This event had a name in medical slang. “Code brown.” Sophie lost about 5% of her body weight in a few seconds as poop flowed like lava out of her her and on to her clothes, me, the chair we were on, the floor, and bits of her crib close to the chair. It took 25 minutes for the nurse and I to clean it up.

Then Sophie got a version of pneumonia, which gave her trouble breathing. A few days before Christmas, Sophie’s oxygen saturation plunged from near 100% to barely 60% of normal in an hour. The team tried to sedate her as best they could and put a breathing tube back in, which stayed in place until Christmas Eve.

Sophie didn't enjoy much of Christmas 2010, despite being newly free of her breathing tube. Charity workers came bearing huge sacks of gifts. We deeply appreciated the thought, but Sophie was scared of the strangers and scowled at the gifts. I ate Christmas dinner by myself in the hospital cafeteria. Then, as we were settling down for the night at Ronald McDonald, the ICU called. Sophie’s oxygen saturation had again plunged, and the breathing tube was back in.

The Christmas-time consumption I witnessed at the shopping mall also made me think about the money Sophie had consumed. When Jana and I got a hospital bill in the mail, we would play a “guess the total” game. We always guessed too low. Sophie’s bills already had amounted to over $6 million, and would jump to $10 million by the end. Even once all its discounts had been applied, the insurance company would ultimately pay out about $4 million for Sophie's care.

Were we doing something morally wrong in fighting so hard for Sophie? Surely we could have saved more lives if we had let Sophie die and used the money elsewhere. $4 million buys a lot of malaria-preventing mosquito nets or life saving immunizations. Of course, nobody could guarantee the money saved from letting Sophie die would actually spread to give healthcare to needy families. It could instead simply line the pockets of the insurance company’s shareholders or policyholders, maybe being spent at Apple and Burberry Kids. In that case, spending the money to help Sophie was a lot more worthwhile.

It was an uncomfortable thought – that maybe I was doing the world a disservice by fighting for my daughter. I comforted myself that there was real long-term value in doing hard, costly, risky things. Every cheap and common form of medicine was once expensive and exclusive. Trying to save Sophie may help the doctors save others later. But it seemed to me that we should only start paying for the expensive, risky ways of saving lives if we were already delivering everyone the cheap, easy ways to save lives. Most countries, including the US, were still a long way short of that standard.

*

After Christmas we also tackled another pressing issue: getting home to Michigan. Seventy undergraduates had signed up for my two winter classes. We had to transfer Sophie to Michigan so we could do our jobs, which meant another medical flight.

My girls

Gail and Carly had been in contact about this stunt, and felt comfortable that if Sophie was free from infection we could safely make the transfer. Insurance, again, had other ideas. Why should they pay for an $80,000 medical charter flight between two hospitals of the same level? There was no medical justification for that, and they only covered medically justifiable expenses.

I called the insurance company, who said the University, not them, handled all non-medical exemptions. So I called the University, who told me to talk to the insurance company. After a few rounds of this, I found a person at the University who could help, if she wanted to. Again, I made my pleadings based on the cost to the insurance firm of having Sophie at Stanford which, like Boston, was much more expensive than the hospital in Ann Arbor. Committee meeting followed committee meeting as the University considered my request to save them money and do my job. While we waited, my classes started without me.

Finally, the University agreed to cover the flight. After four further days of wrangling between various institutions about prices and authorization slips, a team from the University of Michigan’s Survival Flight service left Ann Arbor on January 11, tasked with picking Sophie up and taking her home.