8

I spent my last night in California alone at the Ronald McDonald House. Jana and Paige had already left for Michigan; my job was to chaperone Sophie in the medical jet. I woke around six and arrived at Stanford at the same time as the flight team, who arrived wearing stripy retro flight suits complete with embroidered wings.

Gail was on duty, and reported on Sophie’s status. We packed her things and got her strapped into her car seat-on-a-gurney for the day. It was not easy to do, with all her cords and tubes and medicine to keep straight.

It was hard to say goodbye to Gail. She knew Sophie’s issues better than anyone now, and Jana and I had both grown very fond of her. Gail had been an unwavering voice of sanity - sometimes the only one - as Sophie baffled us all, and as famous doctors fought with other famous doctors. Just as important, she had also gone out of her way to know us as more than just the parents of that interesting case down the hall. I gave her a hug that vastly understated how much she meant to us, and we wheeled Sophie to the waiting ambulance.

*

“The plane is only a few months old, and nobody has used its bathroom yet. We figure there’s no honor in being first. But it is back there if you need it.”

Sophie's second private jet flight

The nurse pointed dismissively to the rear of the jet, through a door jammed with emergency medical equipment. If there was a bathroom in there somewhere, I could not see it. I became determined to hold on. The flight was uneventful as Sophie’s blood pressures behaved nicely, although the dry air did make her need for wet washcloths even more pervasive than normal. We stopped to refuel in Wyoming. I ran for the bathroom. Landing in Pontiac, Michigan, the outside air hit us with a freezing winter blast, an unpleasant contrast with the balmy California winter. I ran for the bathroom again.

Sophie’s first stop at Michigan, as a precaution, was back at the ICU. She had not been in Michigan’s ICU since she was less than two weeks old, but little of the surroundings had changed. When the team gathered to discuss her case, however, I participated in the discussion as if I were one of the senior doctors. It was a far cry from our confused mute performances when Sophie was first in the unit. Through Sophie’s travels to Boston and Stanford we had become the repository for Sophie’s long medical history, and for earlier discussions across the three centers. What had we tried before? Why didn't it work? Who suggested this thing or that? Why? Did she do better with this drug or that drug? Why is this tube going in here, and not over there? What are our options? Our expertise had just crept up on us, as it did for most of our long-term ICU friends. I was proud of my ability to make a contribution to the medical discussions, though not as proud as I was of the way Jana and I had coaxed so many smiles and laughs out of Sophie through her almost endless, often grim periods in hospital.

Sophie had done well on the flight, and the next morning she was transferred out of the ICU and out to the floor. Michigan's infection control people decided that Sophie, with her long history of infections, needed rubber glove treatment until it was time for her to go home. No visits to the play area, no car rides, and no direct human touch until she was all better. We fought it, dreading the thought of Sophie never feeling our touch again. But we failed.

The standards demanded we all wear full gowns, masks, and surgical gloves in Sophie’s presence. Determined not to let Sophie become a leper, Jana and I only partly complied. I wore a mask, but always pulled it down under my chin to let Sophie see me smile, if only to remind her that smiles were possible. Jana also refused to wear gloves, wanting for Sophie whatever healing power her touch could provide. She looked after the hospital’s hygiene with very thorough hand-washing instead. Nobody stopped us or asked us to change.

Within two days, however, Sophie’s breathing became more labored. A pool of fluid had formed under her right lung again, pushing it up and in and making it harder to inflate. We moved her into moderate care for closer observation. Her weight increased by 15% in two days and she became weaker, needing more oxygen support just to keep breathing. There was excess fluid in all parts of her body. Four days after leaving Michigan’s ICU, Sophie was back, this time sedated and with a breathing tube.

We set up Sophie’s bed space just as we had in so many other new hospital rooms. Pictures of her family and her in happier times taped around the bed. Pictures of Team Sophie on the wall. A foam floor mat, toys strewn across it, ready for her next play time. An iPod and speaker for bedtime. Her favorite animals, usually kitty and always panda, waiting for a cuddle on the bed. And her bravery beads from Stanford, forming a necklace of hurdles already cleared much longer than Sophie, hanging on a bedpost.

Sophie needed to pee her excess fluid away. If we got rid of the fluid, then she could breathe again, and we could remove the tube. Every hour we measured, to the milliliter, how much liquid had gone into her body and how much had come out. Negative shifts were a cause for celebration; positive shifts lead to despondent alarm. The doctors racked their brains for the cause of this fluid gain, but without great results.

Because Sophie was so used to all the sedatives, we also had to give her drugs that paralyzed her to prevent her from ripping out the breathing tube. Even though she couldn't move her arms and legs, there were times when her narcotic cocktail wore off enough for her to keep her oversized, bloodshot eyes open, sometimes alone in a world of masks and rubber gloves. It was horrible cruelty.

After a few days we found a way to keep Sophie from ripping her breathing tube without paralyzing her. We turned off the paralytics and immobilized her arms instead, first with rigid sleeves to prevent her elbows from bending, but when she got too smart for those we had to tie her arms down to the crib instead. It was still awful and depressing to have to tie Sophie down, but it was a step up on making her body a living tomb. We kept her entertained as best we could when she woke up, giving her things to hold and untying her arms when we were there to supervise. And we kept pumping in the diuretics, hoping she could pee her way to health.

Hematology was also continuing to search for the cause of Sophie's very unusual blood clots. They ran test after test, and found nothing. Nobody knew what kind of problem she had, let alone how to fix it.

It took Sophie almost two weeks to pee off enough fluid to get her breathing tube out, but she still needed lots of extra oxygen to breathe. All the time spent with a crowded throat had ruined Sophie’s vocal cords, at least for a while. We would have to wait for her to heal before she could talk again.

Extubated for the last time

Sophie’s spirits rose after her breathing tube came out in early February, but she was still very tired. I would get to the hospital between eight and nine, the noise of my arrival often waking Sophie for the day. In the morning an ocupational therapist came to see us, which gave Sophie the chance to get out of her crib and into her play area. Together we worked on her muscle tone and coordination, both of which were poor following two months of infections and sedation. At one point she even managed a couple of assisted steps to get from the therapist to me. But they were not joyful steps. Her only motivation was to get across to me so I would pick her up and the ordeal would be over. After the therapist left, Sophie would sit with me in an armchair while we read books or watched videos. After a short while, she would fell asleep on me, often for more than an hour. I enjoyed these naps, having Sophie nestled with me as much as the machines would allow. It reminded me of happier times in earlier hospital stays. At noon I would wake her and transfer her back to the crib, where we would play a little before she fell asleep until around two. And by the time Jana and Paige came in to relieve me at four, Sophie was again tired.

Every now and then, there were glimpses of the old Sophie. One afternoon I arrived with Paige to relieve Jana, our schedules temporarily reversed. Sophie not only smiled when I arrived, but almost immediately pointed to Jana and waved her bye-bye. Jana’s form of caring was more gentle, mine more rambunctious, and Jana thought Sophie was saying to her: “My playmate is here. Time for you to go!”

On February 12, I had one of the proudest moments a Dad can have: I saw my daughter learn to cheat. Sophie had a toy school bus with holes in the sides of different shapes, and a set of plastic things to put through the holes. But no matter what the shape of plastic piece I gave to Sophie, it would fit through the square hole if she turned it sideways. Hexagon, circle, triangle all went in. Through all the meds and her pain, I detected joy as she positively snatched whatever I gave her, turned it sideways, and shoved it through the same hole in her bus.

*

The next day, Sophie’s 334th day in a hospital, my phone rang at 5:30. Sophie’s oxygen needs had gone up in the night, and they were intubating her. That was not unexpected. I grunted and went back to sleep.

My phone rang again at six. It was the same nurse.

“We need you to come in. Sophie is arresting…”

I did not hear what she said after that. Jana was out of bed faster than me. We were dressed and out the door, Paige in arms, within three minutes. As we were throwing clothes on, Jana looked at me.

“She’s going to die.”

I did not know whether it was a statement or a question.

“She may do.”

There was fresh snow on Ann Arbor’s deserted Sunday morning streets. I had to drive carefully, and consciously slowed down to make sure we got there in one piece. The worst thing would be to miss her because of an accident.

“Our job is to keep calm and let everyone do their job. We can only help Sophie if we are keeping ourselves together.” I tried to steel Jana as I drove. She stared out the front windscreen.

The phone rang again. “How far away are you?”

We swung into the parking garage the wrong way, parking illegally in the empty valet lot. We hurried through the corridors, ignored all the signs to call before entering the PCTU, ignored the hand sanitizing station, and ran for Sophie’s room. A nurse swept in and took Paige from us as we got close.

Sophie’s room had all its doors slid open, and was teeming with staff in yellow gowns. Sophie lay sideways and unconscious across her bed. On one side of the bed was a woman wearing a helicopter flight uniform, standing on a step stool and giving compressions with two fingers. It was Sophie’s first Code Blue.

The senior on-duty cardiologist, Gabe, motioned us to come in and to hold hands with Sophie. No latex gloves for us today. We told her it was going to be OK, and how well she was doing. Sophie didn't react. After a short while, Gabe suggested we might go and talk.

“Sophie was fine until about four, and then it all started to go downhill. We don't know the cause, but I think it is that damned mitral valve again.” He looked at us with a face almost of exasperation– we had all worked so hard to keep that valve moving.

I felt utterly deflated.

“We tried to get an emergency echo while giving compressions. It was next to impossible to get good pictures, but the images we did get suggest her mitral annulus is down to 4mm.”

The valve opening had been three times as wide only three days prior.

“What happens now?”

“Have you heard of ECMO?”

I had. Jana wanted to know more.

ECMO is a longer term version of the heart lung machine. It pumps blood around the body, performing the heart function, and puts oxygen in the blood, covering for the lungs. It is a cumbersome, complex machine that requires two full-time nurses to operate. It is an emergency option – not quite a Hail Mary play, but close. Fewer than half the people who go on ECMO ever leave the hospital alive, and Sophie’s history made her a more complicated ECMO patient than many others.

“ECMO gives us an opportunity to regroup, and figure out what to do next. It buys us some time.”

“Time for what?” I asked. “We were at the bottom of the barrel last time, with people recommending last-ditch experiments. And now here we are again.”

We talked about Sophie’s options. I tried to be calm and analytical. Gabe left us for a minute to check on Sophie.

Jana called our friend Sarah in Maryland, who had been Team Sophie’s online captain throughout her journey. She asked if Sarah could come to Ann Arbor. Sarah was out the door, heading for BWI, within minutes. We talked about calling family, but it was 5am in California, 2am in New Zealand. What could they do? We let them be.

“Well the good news is we have re-established some native circulation. She’s at about 150 beats a minute, under her own steam, but it is pretty weak, pretty irregular.”

I felt relief but not joy. I thought Sophie’s time had come. Putting her on ECMO looked to me like a ticket to watching her through a bank of machines for a day or two before she finally succumbed. I didn't have any hope about finding a solution for her problems. My last whisp of hope had vanished when I saw Sophie getting chest compressions that morning. We still didn't know what her blood condition was; let alone how to manage it.

As we talked, I never said that I thought we should let Sophie die. It was too hard to make the words. But the questions I asked made it increasingly obvious that I wanted to let her go, to hold her one last time and say goodbye nicely.

Jana agreed, and she drew up the courage to verbalize it before me:

“I think she is done. Done.” she had her determination-fighting-through-the-tears face on. But she wanted some reassurance:

"If this was your child, Gabe, what would you do?" Medical people often don't like to answer questions like this, because they substitute medical judgment for parental judgment. I interrupted, with a different tack on the same question:

“Is there anything about the decision we look like we are about to make…” – I still couldn’t say “let her die” – “…is there any part of that decision that you think is unreasonable? Hasty?”

Gabe looked at the floor and shook his head. “No.” He was quietly emphatic, letting us know, I thought, that he would have done the same thing. He went to tell the team.

We sat stunned in the windowless consultation room. Jana started to cry. I didn’t. I made a little speech for her about letting Sophie feel our love, not our fear, when we held her. She stared at the wall, tears streaming down her face.

Sophie was back to lying properly in her bed. The helicopter lady was gone, but the room was still full. A cardiology fellow was at the foot of the bed calling the shots. Two nurses were on one side of the bed administering drugs, monitoring Sophie’s pulse, and drawing blood. Other folk came and went. Respiratory therapists, other fellows, senior cardiologists.

Every couple of minutes, the fellow would order very large doses of drugs to support Sophie’s blood pressure through the weak heartbeat. It was very matter of fact; a well-honed drill.

“How often do people recover from an episode like this?”

She paused but didn't look at me. “Not that often.”

I stood by Sophie’s side, holding her hand. Jana had climbed through the tubes and wires and taken up position at the head of the bed, stroking Sophie’s hair.

“Can she even hear us?”

A nurse said she could still probably hear us even though she was in a coma. People who had woken from comas sometimes reported with scary accuracy some things said while they were unconscious. They could not react, but they could hear.

A blood report came back. Sophie’s pH was down around 7.0, massively more acidic than normal, and hugely dangerous. The fellow said it had been down around that level for a couple of hours, and that likely had caused irreparable organ damage. It was just another of many last straws.

The nurses rearranged the furniture for us. There would be no bed, just two rocking chairs and the machines. When they were done, there was only Sophie’s regular nurse Denise left in the room. The sliding doors were closed again, the curtains drawn. Other parents passing by would not have to witness Sophie’s living funeral, even though the whole unit no doubt knew what was going on in bed 13. Instead they could only stare at the curtain, as we had stared at so many others before.

After pumping some milk for Paige, Jana settled into a rocking chair and held Sophie first. She looked asleep, but not comfortably asleep. A lot of her color was gone. Jana talked quietly to Sophie about how much she loved her, and how there wasn’t going to be any more pain. We sang Sophie her favorite action song, the itsy bitsy spider. She used to do her own stripped-down version of the actions along with us, but this morning she just lay there.

Then it was my turn to hold her. Jana lay Sophie down in the crook of my arm. She had spent so much time there in recent months. It felt comfortable and familiar. I rocked the chair like normal, resting my head on the top of Sophie’s more for my own reassurance than for her. Her hair felt nice on my cheek. We sang for her again.

Denise was over in the corner of the room, staring at her computer and trying to melt into the background as best she could. But every now and then she came to the chair to check Sophie’s pressure and support it with more drugs. Her heartbeat was getting a little weaker.

It came time for me to give Sophie back to Jana.

“Are you sure you want me to hold her?” she asked. We both had a sense that this would be the last swap.

“Yes.” I said. “This is one of the privileges of being a Mom. It is what Moms get to do.” In retrospect it was hard to say why I called it a privilege to hold your kid while she dies. But Jana knew what I was getting at.

Before I let go of Sophie, I said to her: “I love you and I am proud of you, my little Sophie. My Little Sophie. You will always be my friend, Sophie. You will always be my friend.”

We swapped, and I pulled my chair as close as I could to Jana and Sophie. I wanted us to be a tight little unit, and I started to cry.

After a couple of minutes there was a knock at the door. Who would want to be part of this scene? Who would want to disturb us now?

It was Carly, Sophie’s primary cardiologist since 20 weeks gestation. She had come in on her day off to see us, and to say goodbye to her patient. It was nice to see Carly, despite the grim circumstances. Along with Gail at Stanford, she was our closest doctoring confidente and a good friend to Jana and me as well. We talked a little about what had happened. Carly already knew. We were out of questions, out of plans. Carly listened briefly to Sophie’s heart before she left.

“I think she is ready.”

Once Jana had Sophie tucked in her arm again, I asked Denise to stop giving Sophie drugs. If it was Sophie’s time, I didn't want to prolong the grisly vigil. Her heartbeat got weaker, but it was still there on her monitor, beating at about 150.

Jana talked to Sophie, saying she didn't have to fight any more. “It’s all done, baby. You did such a good job.”

A few minutes later we called in the respiratory therapist and asked her to extubate Sophie. For some reason, I wanted Sophie’s last few breaths to be her own. She was very gentle, making sure not to damage Sophie’s skin as she removed all the tape from her face. I’m sure that was more for our benefit than for Sophie’s – making sure we didn’t have to witness one final insult.

After making sure we were ready, she pulled the tube out.

Sophie took a few small breaths. Maybe five. They seemed weak and desperate, and she had no strength to keep it up for more than a few seconds. She stopped taking in air, but her heart kept beating. 150 at first, then dropping down quickly. I could hear it slowing on the monitor. Down to 120, 110, 100. Denise had taken up a position near the monitor, and she was talking us through it.

“Really slowing down now.”

I looked up at the monitor, and saw Sophie’s heart beating at 27 beats per minute. I looked back to her, nestled in Jana’s arms, Jana’s forehead resting on hers. Nobody said anything, and there was no longer any sound from the ventilator. The only sound was the heart monitor.

And then it, too, fell silent.