6

Jana and I arrived at Stanford before Sophie woke on August 12th, our fourth wedding anniversary. She had been in hospital getting tuned up for surgery since we made up our minds to stay at Stanford. We dressed her and took her for a dawn ride around the halls. Stanford felt more like a luxury hotel than a hospital, with its green internal courtyard, light-filled corridors, and rooftop garden. Sophie had grown used to riding around here just as she had at Michigan, and had favorite sights she would point at expectantly as we neared, like the little aquarium in a waiting room that had toddler-height windows to the main hallway. "Fish! Fish!"

Soon, it came time for the familiar routine of taking her to pre-op and saying goodbye. The row of sterile bed spaces, at once futuristic and reminiscent of the 1950s. The other inpatient kids, some deathly, some manic. The subdued parents. The waiting around that feels like forever, then the final hug that is over in an instant.

This morning we had a new element in our pre-op routine: meet the surgeon and tell him what to do. We hadn't had a chance to tell Dr Hanley our decision to let him do it his way, with the larger mechanical mitral valve if needed and the mechanical pulmonary valve, but only if the mitral valve was also replaced. If he saved Sophie’s native mitral, then we wanted an animal valve on the other side. The volunteer retiree at the OR spoke in hushed tones and with great reverence about the Great Surgeon we were meeting. We smiled sheepishly and shrugged.

Seven hours of wandering and napping in the public areas of the hospital later, we got word to talk with Dr Hanley again. Sophie was out of the OR, bound for the ICU. Her mitral valve could not be saved, and he replaced it with a larger mechanical valve that he squeezed into its proper spot. She also had a mechanical pulmonary valve. To cap it off, initial testing already showed big improvements in her blood pressures.

Was the operation a success? Dr Hanley certainly thought so, but then he only ever thought saving Sophie’s valve was an outside chance. We were pleased she was safe and really happy about the lower blood pressure, but had a nagging regret she was already dependent on a metal part to survive.

A big challenge for people with artificial valves was setting up the blood-thinning medicine. People who had too much blood-thinning medicine could start spontaneously bleeding, sometimes fatally. The blood-thinner Coumadin was even sold as a rat poison, where deliberately huge overdoses made the rats bleed out internally. But people who have not enough Coumadin could get blood clots, also potentially fatal. It took trial and error to get the right dose and the right blood thickness, measured with a test called the INR.

Coumadin was enigmatic. Its effect could even change if a person altered their diet. Sophie's diet, of course, was entirely consistent from one day to the next. But her INR numbers flew all over the place, seemingly with no relationship to how much Coumadin she had. Without changing doses, it would jump from too thick to dangerously thin, then back down again, and back up again. Changes in medication sometimes had the intended effect on her INR, sometimes the opposite. Gail and the other cardiologists stared at her wild INR charts for more than a month, confused about what they were doing wrong.

Sophie also felt bad because we couldn't give her any nutrition by mouth, not even water, as it set off her gag reflex and she threw it all up. Mouths usually hydrate via liquid going through, so Sophie’s mouth felt dry even when her intestines were constantly supplied with milk. While we couldn't give her a glass of water, we were allowed to dip a washcloth in water and let her suck it out. The sucking regulated her intake just enough.

Sophie would ask for thirty or more rounds of wet washcloth each day, and any time she saw a glass of water she would beg for that instead. The sadism of having to deny even a glass of water to her daughter really ate at Jana. How could the universe have conspired to turn a mother against her child like this? At an intellectual level, we both understood why it was worse for Sophie to have a cup of water than a washcloth, and if ever she did start to retch we both felt awful watching on helplessly. For Jana, the sight of her daughter pleading - incessantly and without success - for something the world declared a human right was almost too much to bear. Around and around the cycle went, with Sophie diving on a second-best damp washcloth, sucking out all the moisture with every bit of strength her little mouth could summon, finding it was dry within a few seconds while her thirst remained unquenched, and then pleading with her parents for more. Witnessing this scene made Jana feel like a bad Mom, regardless of any reason or science.

While sitting in the ICU that month, Sophie got a pair of infections, one so serious we had to avoid touching her for days on end, wearing rubber gloves and surgical masks to stop the infection from spreading. There was something deeply awful about stroking my daughter’s her hair through a rubber glove.

One rubber-gloved afternoon, I came to Sophie’s room to find her upset, her heart rate at 190. I scooped her up and calmed her down. Despite being happy again, her heart rate stayed high. I asked the nurse, who said it would come down. It climbed. She asked a cardiology fellow, who tried some stretches that can lower heart rates. Not this time. He called in a cardiologist who called for an EKG, a test that measures the electrical activity in the heart. When the EKG team arrived, it came with another two cardiologists and hangers on, and what looked like a tire balancing machine. Sophie now had the full medical gaggle crowded around her bed, all donning yellow coats, rubber gloves, and masks just in case they got called on. It looked like the decontamination scene from ET, except that Dr Hanley had joined the melee, an island of tweed in a sea of yellow plastic coats.

“Do you want the crash cart?” said a voice.

“Yeah, let’s have it available.”

To run the EKG, someone had to put 12 stickers on Sophie’s body. She hated that. Sticky going on meant sticky coming off. Her heart rate hit 220.

“That little valve is holding up pretty well to this,” said Dr Hanley to nobody-in-particular. “You know, those mechanical valves aren't designed for much more than 180.”

Sophie’s rate hit 233.

Some drugs arrived. Most were backups in case the first drug, adenosine, failed. The team thought Sophie had developed a temporary short circuit in her heart called a super ventricular tachycardia or SVT, causing the upper chambers in her heart to contract too often. Adenosine would stop this in seconds by temporarily dampening the flow of electricity around the heart. If it did not work, we had plans B, C, and D on deck.

First one fellow squeezed the adenosine into an IV line, then a second fellow sent in a flush to make sure it reached the heart immediately. The timing mattered because adenosine had a half-life in the body of less than ten seconds. We all looked up at the monitor, which read 220. Two or three beats went by, and the rate was still 220. My spirit sank. But on the fourth beat, the rate dropped straight away to 140. Sophie, although upset at all the attention, was safe again.

The gaggle quickly dispersed, leaving their gloves and masks, a crash cart, and many doses of exotic drugs behind. I started cleaning up; Sophie dozed off.

About a month after her surgery the infection issues had subsided, the antibiotics had run their course, there were no more SVT episodes, and we were left only with the issue of anticoagulation. Sophie’s INR readings had never really settled down, and often showed her blood was too thin, tipping the scales too far against clotting and in favor of bleeding. Despite that, Gail said we could move to Stanford’s step-down unit instead of the ICU. Feeling really happy with this idea, I left for lunch. I came back waving and smiling at Sophie, who was looking glum.

“Sophie’s G-J just came out.”

Whenever we were doing well, the universe threw shit at us. The hole where Sophie’s G-J tube used to be was slowly oozing gastric juices. We weren't heading for the step-down unit after all. A few days later, after scouring local hospitals to find the right sized replacement G-J tube, and then waiting a couple of days for someone to actually put it back in, Sophie was once again getting nutrition straight into her intestines.

Shortly after, my parents came to visit from New Zealand. I wanted to show off Sophie’s new heart valves to my Dad, himself medically trained. Sophie’s valves had a distinctive “click” through the stethoscope as the metal leaflets struck the surrounding ring on closing. Giving Sophie another wet washcloth to pacify her for a moment, I lined up the listening piece and passed the earpiece to Dad. He looked puzzled.

“There’s a bit of a ‘woof’ in there, too.”

Silly old Dad. Sophie’s valves clicked, they didn’t woof.

The next morning, Gail said that Sophie’s new valves, both of them, sounded different. Another cardiologist agreed. They woofed. An urgent appointment was made for Sophie at the cath lab.

When Gail came fretfully striding around the corner we knew the news was bad.

“Both valves are stuck. On the mitral side, one leaflet is stuck semi-open, the other moves fine. Both pulmonary leaflets are stuck fully open, too. This is very serious. I am so sorry.”


Rob Salmond
Further complications
Dear Friends

Sophie's heart has encountered another problem. Both of her new mechanical valves are now leaking. Her pulmonary valve is locked in the open position, allowing blood to flow freely in both directions rather than one, and her mitral valve has only one properly functioning leaflet instead of two.

Loyal readers may recall that the big benefit of these mechanical valves is that they don’t leak. It seems we just can’t catch a break.

Sophie had a heart catheterization this morning to figure out what was going on with her valves. She is sedated today as we try to loosen up her valves with some aggressive anticoagulation medication – the thought being that there has been some thrombosis (clotting) that is gumming up the works. We’ll know whether that is working by tomorrow morning. Our surgeon is cautiously optimistic about this experiment; our cardiologist is more pessimistic.

If the valves are still functioning poorly in the morning, then we will likely have to send Sophie to the OR tomorrow to get both valves replaced once more. Exactly what kind of valves we would replace them with is unclear at this point.

If there is surgery tomorrow, it will be more moderately more dangerous than the surgery she had five weeks ago, because all the anticoagulation medicine will make any bleeding from the procedure harder to control.

We’ll try to keep you up to date tomorrow with some news. In the mean time, thanks for all the support you have given Sophie so far, and please keep her in your thoughts.

Cheers,
Jana and Rob
September 17, 2010 at 3:24pm

We did not understand, at all, what had just happened. We had fed so many anticoagulants into Sophie, risking spontaneous bleeds as we went. A lot of the measurements suggested we had given her too much. But here we were, clotted.

If the other leaflet on Sophie’s mitral valve also got stuck, she would die straight away. Things were not quite as severe on the pulmonary side – Sophie was basically back to having the pipe arrangement she had been stuck with before the latest operation.

The initial treatment for Sophie’s stuck valves was to put a clot-busting medicine called tissue plasminogen activator, or tPA, into her blood. The tPA would quickly break down the congealed blood on the valves, like an acid, freeing them up to move again. But the risk with tPA was that it could also break down clots elsewhere in her bloodstream, opening up old wounds, and even causing spontaneous new bleeding in areas with delicate blood vessels like the gut and the brain.

The team started the tPA treatment, sedating Sophie so she could not risk bleeds by thrashing around in her crib. We knew the tPA was doing its thing because Sophie started bleeding out of the catheter entry incisions from earlier in the day. She oozed constantly from her neck and inner thigh. Parents and doctors and nurses all took shifts holding pressure over the wounds to minimize the blood loss. The next morning’s echo showed both valves were moving again. We stopped the dangerous tPA infusion, immediate crisis averted, and resumed her Coumadin treatment on even bigger doses. The team also turned to the big puzzle. How did she clot her valves with such a high INR score? It just didn't seem possible.

But before we could answer the big question, we were back in crisis mode. Within a few days, Sophie’s mitral valve was again stuck, and the next day it was working again but her pulmonary valve was fully stuck once more. As parents, we had become used to being confused about Sophie’s medical issues. But now absolutely everybody in one of the best specialist units in the world was baffled, too.

Back on the tPA she went. Sophie’s catheter wounds leaked more blood. Again we drugged Sophie to keep her sedate. After 12 hours, we saw no progress. And 24. One cardiology fellow took such a long nighttime shift holding pressure on her catheter wounds that she fell asleep, right on top of Sophie’s thigh. In a desperate attempt to avoid emergency heart surgery with compromised blood, we significantly increased the tPA dose for six hours, taking a calculated risk of catastrophe. At the end of the six hours, an echo showed both valves clicking away nicely, looking as if nothing had happened. We had averted disaster again.

But Dr Hanley wanted us to keep the tPA flowing. He thought our earlier problem had been in turning off the tPA on first movement, rather than waiting until all the clots had entirely melted. He quoted studies of children surviving tPA runs of up to 72 hours, and asked our permission to “really clean those valves off properly this time.”

At the end of the 72 hours, Dr Hanley wanted still more tPA. Another 12 hours, he said. We were really uneasy with this, especially as Dr Hanley seemed to be basically guessing about the tPA, beyond the evidence and outside his specialty, with Sophie’s life on the line. The team talked to hematology. “Highly uncomfortable” came the reply.

A junior cardiologist, who was distractingly attractive, like a 19 year old soccer star dressed up as Elliot Reid from Scrubs, came to talk with me about Dr Hanley’s request. Was it OK with us?

I fretted about even more risks of bleeding in the brain or in the gut, each of which could kill Sophie right there. She told me, gum in mouth, that balancing risks was always tricky.

I asked how much extra valve cleaning we would actually get between hours 72 and 84. I was told that nobody really knew, but Dr Hanley was really good at this, and the ICU team agreed with his recommendation.

I asked why they agreed. She parroted the same things Dr Hanley had said to us earlier. I wondered was this independent agreement, or just subservience? I pressed further.

“The issue we’re really dealing with is a hematology issue, right?” Yes.

“And the risks and benefits are all about hematology rather than cardiology. Risk of bleeds versus benefits of anticoagulation, right?” Yes.

“And our hematology expert thinks this is a bad idea. She’s opposed. The big boss in cardiac surgery thinks it is a good idea. And the team seems to be falling right in line with the surgeon.” Again yes.

“So why are you siding with status over expertise?”

The doctor stared at me for a moment, said little more of substance, and scurried away. Maybe I had been too accusatory; too aggressive. Would I have been brave enough to make that kind of accusation to a senior cardiologist like Gail? Was I talking down at her like a student although, despite her youthful looks, she knew far more about this than I did? I felt both guilty and aggrieved.

Ultimately we agreed to Dr Hanley’s plan, not because we thought it was a good idea, but because we did not want to refuse the team permission to do its job. Despite hematology’s concerns and our own nervousness, the ICU doctors were the real experts in Sophieology.

“We’re giving permission, but we don't agree.”

Gail had a hunch that the problem for Sophie wasn’t her blood; it was the INR test. We knew the INR number was wrong – the clots proved that. So when Sophie finished her record-setting tPA run she started on another anticoagulation medication called heparin that worked differently and was measured differently.

Two weeks after Sophie’s last clot, we again left the ICU. Sophie’s heart valves sounded crisp, and the rest of her heart functioned very well when the valves were moving. Her other issues had subsided, and she was a happy, smiley girl, especially washcloth in hand. Even in the ICU we had been allowed to take her, with her nurse, for trips in her car around the unit, outside to the private ICU patio, and sometimes even out and around the public areas of the ICU level. When we got to the step-down unit her horizons expanded further to include unaccompanied trips to the roof garden, the fountain, the aquarium, and the model train set. It was a day for goodbyes and celebration.

The next day, Sophie’s pulmonary valve jammed open.

As soon as we heard the valves were again jammed, we knew that clearing these clots with medicine would not work. We also concluded, as did Dr Hanley, that the mechanical pulmonary valve experiment had failed. Sophie needed an animal tissue pulmonary valve.

Two days after leaving the ICU, Sophie was again in the OR under Dr Hanley’s knife.

Because we had to make fast, high stakes decisions about Sophie in that day or so, there wasn't any room for us to react emotionally to the news. I suspected that Jana was genuinely numb from this whole turn of events, rather than bottling up rage and anguish, and for my part I allowed myself only a few seconds of lip-pursing and bewildered-staring before asking "OK, what do we do now?"

Sophie had been away from the OR for around eight weeks, which was the worst possible time for re-sawing her sternum. It had partly but not fully healed, meaning that sawing through it was more like chipping away at old tile than like sawing through a piece of wood. It took Dr Hanley a long time to get in. At the end of the surgery, eight hours after he had gone in, he told us that Sophie’s mitral valve looked entirely pristine, so he had left it there rather than replace it with another identical valve. She now had two of her own valves, one pig valve, and one metal-and-plastic valve.

Gail and Dr Hanley then asked to do something else unusual with Sophie’s anticoagulation. We knew that both the usual measures had been lying to us, saying she was anticoagulated when she clearly was not. So they wanted to figure out her anticoagulation using more rustic methods. They would start with high doses of heparin and raise it higher and higher until Sophie bled spontaneously out her nose. Then we would take a little off that “too much” dose and call that the dose she needed.

“This would almost be malpractice under normal circumstances. But we don't have many other tricks available.”

Three days later, Sophie oozed blood from her nose. The team took all kinds of measurements of this baseline, and dialed down the heparin just a touch. The bleeding died away, and we all crossed our fingers.

When she wasn’t in the OR or on tPA, Sophie was the darling of the ICU. Well enough to be mobile, young enough to be cute, and familiar to almost everybody, she drew affection wherever she went. One of the nurse practioners made the mistake of showing Sophie a duck-themed ringtone on her iPhone. Whenever Sophie saw her after that, she demanded ducks, gesticulating and yelling, and woman became known as “duck.” Later, she even bought Sophie some ducks for them to play with together. Staff did action songs for Sophie on command, even out in the corridors. An act as simple as wearing a folded newspaper hat while out for a ride brought doctors and nurses streaming into the corridor, camera phones ready. ICU environments did not play host to happy little girls often.

As the team’s trust in Jana and me grew, Sophie’s horizons expanded. Even while resident in the ICU, we were allowed to take Sophie, unaccompanied, anywhere in the children’s hospital, so long as she stayed connected up to a monitor beaming her health information back to the ICU desk. Sophie and I often headed for the roof garden where she could play with flowers and look for helicopters, and to the ground floor train set where two trains raced each other around a mockup of Monterey, complete with cannery row and underwater whale viewing. All of these treats made Sophie almost giddy with delight, and the look of surprised delight her face when she heard the distant rotors of an approaching chopper could brighten any room.

Jana and Sophie often hit the cafeteria instead. Jana would position Sophie so she was looking at the people coming through the checkout, and Sophie would stare intently at each person in turn. Those who smiled or waved got a smile and a wave back, but if someone ignored Sophie her stare would slowly harden into a stink-eye until the next customer came along. Most people smiled and waved, because Sophie’s enthusiasm was contagious. This was just another of the ways she found, almost effortlessly, the little pieces of joy scattered through her hospital-bound existence.

On the roof garden with Sophie's cousins.

In October, Stanford’s ICU team threw Jana a surprise baby shower. They set up cakes and presents on the outside terrace, and I coaxed Jana out under pretense of taking Sophie for a ride. Sophie loved opening the gifts even more than Jana loved receiving them, and I even managed to sneak Sophie some Sprite on her washcloth to give her taste buds a workout. The mood was light and jovial, a really pleasant change of scene.

The mood was anything but that inside the unit. A case had come in by helicopter, bound for the room next to Sophie’s. “It’s going to be a train wreck,” said Sophie’s nurse to me in a whisper. Once the boy arrived, the staff gaggle outside his room was large and permanent. As we left for our party, there was an air of concern around the unit, but no drama.

But when I ducked back inside to get a sweater, there was human silence through the unit, people standing around in groups of two or three, all watching the screened off room. The only noise was the Code Blue alarm. Some people, including Gail, were desperately trying to work on the boy.

Half an hour later, Gail came out to join the party. She didn't know I had seen her working on a dying patient, and somehow she was able to join in the celebration, hiding her feelings of sadness and frustration. She had to – we were a different family. I had absolutely no idea how medical people managed that trick.

That evening, the room next to Sophie’s stood empty, sterile, and spotless.

*

We had an eventful Halloween in 2010. Two days before Halloween, the children’s hospital at Stanford threw a party for the patients. There was candy, stickers, and other treats for the kids all handed out by beaming, costumed staff. Many patients wore costumes, too. There were ghouls and skeletons and wonder women galore. Sophie’s ICU neighbor, a 16 year old waiting for a new heart, dressed up as a Geisha – we decided not to tell her the job description.

We dressed Sophie up as Dr Hanley.

As soon as I put Sophie in her uniform of scrubs and tweed, complete with personalized “Dr Sophie” embroidery, a steady stream of ICU staff came through to look. All left holding their sides. Apparently two residents had pulled this stunt before – donning sport coats and barking orders for a day – but never a patient.

Sophie was resplendent as she tootled around the hallways dressed as a surgeon and collecting treats, nurses hospital-wide cracking up as she passed. Late in the day Dr Hanley came by, on the command of his staff, and posed for a paparazzi session with Dr Sophie. He did not betray any sense it was parody. Perhaps he thought we had done the surgical equivalent of dressing Sophie like superman. I never asked.

On Halloween itself, a Sunday, I could only take Sophie for a short ride in her car, because across the hall Jana was giving birth to our second child.

Paige Salmond was born at 2pm on Halloween 2010. Paige spent a few hours under observation in the intermediate neonatal ICU, but by bedtime she was snugly in Jana’s arms in a maternity room. I snuck Sophie in for an evening visit, then also smuggled some sushi and beer from a local restaurant so Jana could celebrate freedom from her pregnancy diet.

Jana and Paige went home the following day, and Sophie left the ICU bound for the floor. Her new valves had been clear for three weeks, her heart function excellent, and we were making steady progress on her anticoagulation. We had even started talking again about experimenting with stomach feeds instead of intestinal nutrition. On the floor, our big job was to build confidence in her anticoagulation.

It was hectic living, with a fresh-from-the-oven newborn and a hospitalized toddler to look after. Jana woke every three hours, day and night, to feed Paige. I spent most of the day riding with Sophie around the hospital and watching movies with her in her room. We had Finding Nemo, Cars, and Ratatouille on high rotation. I lived for her midday nap, when I could escape the hospital to read for an hour at a lunch bar. I alternated between sandwiches piled with richly-cured Italian meats, and burgers served with old-fashioned milkshakes with extra cream.

When we swapped off Sophie duty, Jana would take Paige to the hospital. Staff flocked to see her. Sophie was interested in Paige as a concept, making the baby rocking sign when she caught a glimpse. But when we actually went to put Paige in Sophie’s arms, she tried to throw Paige out of the bed. “No,” she said firmly. My afternoons were spent holding Paige, hanging out with Jana’s Mom, and preparing food as Jana tucked Sophie in for the night.

Five days after leaving the ICU, Gail came to see Sophie and me late on a Saturday afternoon, bearing test results and orders for large quantities of drugs. It was a moment we knew might have been coming, but we knew enough about hospitals not to be sure.

“So, do you want to take your daughter home?"