Maxwell Charles Munakata
A short gut, a long road, a full life.


A short gut
Max was born with 21 cm of functioning small intestine (less than 10% of the typical length) as a result of a 1 in 5 million condition, extreme long-segment Hirschsprung's Disease. Before he was diagnosed, a neonatologist told us that we would be "screwed" if Max had Hirschsprung's. Max spent his first 130 days of life across 4 hospitals.
See: The diagnosis. The decisive surgery. The stoma resection.

A long road
Max is dependent on IV nutrition pumped through a central line to his heart and continuous feeds pumped through a tube into his stomach. We don't know whether he will ever come off of these. We lived with a suitcase in our car while we waited for the call to fly him to Pittsburgh for an intestinal transplant, a relatively new procedure. We were racing against time while Max's liver failed from the IV nutrition.
See: Stopped breathing, neon vomit, pool of blood, oral aversion, blood infection, detached lifeline, leaking stomach acid, food allergies, electrolyte imbalance, blinders, puppy dog pajamas, lives in our hands, exhaustion.

A full life
We moved to Boston when Max was 7 months old, where Mark Puder and Kathy Gura saved his liver and life with Omegaven, an omega-3 based lipid. We now have the luxury of time to wait and see how his intestine adapts, and to watch the latest transplant developments come in. Max is thriving. We have 50,000 people to thank. We have never known such happiness.
See: New normal, sharing, giggling, crawling, Paris, go go go, dolphins, hippos, and a mouse, intruding, healing, rocking, reassuring, family awards, conferencing, conjugating, eating, more giggling, praising, driving, graduating.

The neonatologist was wrong. This is Max's story.

(Newest entries are at the bottom -- dates can be clicked on the left.)

Tuesday Dec 30, 2014

We didn't plan to spend Christmas Day in the hospital. Or Christmas week. Or going into the week of New Year's. But we can imagine worse scenarios.

We can also imagine better ones. Like lounging around with old friends, watching our kids play together on the beach. We'd been anticipating this trip for months, after a demanding semester and a flu that hit everyone in our family from Thanksgiving through Christmas. It took down Naoko and Mike (who were staying with us), and led to our kids not seeing baba and jiji at all during their extended visit to Colorado, because we didn't want to expose them to our nasty bug. (Baba and jiji enjoyed their Thanksgiving Day turkey, ham, and desserts with Junko's family at their house, while the rest of us ate the green beans, broccoli, stuffing, yams, and cranberry sauce that we had planned to bring.)

We did get two days of our dream trip to St. Thomas. But Max's flu symptoms weren't resolving. We knew this had been an extended bug for everyone. He had had all the same symptoms as the rest of us, which led us to think that this was the flu, and not something more serious like a line infection. But now he was starting to look jaundiced, and he was developing extreme pain in his shoulder.

It turns out we were probably right about the diagnosis, but wrong about the seriousness.

We called United early in the morning on Sunday, Dec 21st, and got new flights going home that day. The plan was to land in Denver that night, with Randy taking Max to the hospital and me and Kai taking a shuttle home. But after flight delays in DC, we ended up not getting home until 1:30 in the morning on Monday (4:30 AM St. Thomas time). Shuttles weren't running, and Max's temperature was normal. He seemed tired but fine, as he has through much of this process. We came home and slept, trying not to kick ourselves for flying home unnecessarily.

Randy took Max to Kaiser on Monday, while Kai and I shopped for groceries for the week. Kaiser sent Max and Randy down to Children's Hospital -- they could check for the flu much faster, and for an infection in Max's line. This meant he would have to stay through 48 hours of negative cultures for bacteria -- a wait we had not faced in almost 3 years. (We haven't been facing much of anything medical for years. Max has been thriving, staying out of the hospital, and enjoying school, mountain-biking, friends and parties, traveling, and Legos and trains, still.) We hoped this wait would be as short as possible, so Max could come home Wednesday, Christmas eve.

Instead, the next 24 hours brought a bewildering series of procedures, test results, and associated possible outcomes and treatments. We've never really felt like "seasoned" hospital-goers, but especially not in the face of the things that were coming at us now, like the possible need for heart surgery, or the risk of an embolism in Max's brain, or the likelihood of bacterial infection in Max's bones.

First Max's cultures came back positive, for staph aureus. Staph is the most likely source of bone infections, which would explain Max's joint pain, and his red blood cell levels (which turned out to be quite low), because a bone infection can shut down the production of blood cells in the bone marrow. Two powerful antibiotics (rifampin and nafcillin) got started based on their ability to work best on this specific bug. But we know this is a sticky bug -- Max had it 7 years ago, and it couldn't be killed off in his line. We prepare for the possibility of surgery to replace Max's line, and a long hospital stay -- several days to try to kill off the bug, then removal of the line if these attempts fail, then 2 days to wait for negative cultures with the line removed, then inserting of the new line.

This test result brings more than the risks associated with having an infection in the blood. It brings overwhelming guilt at the thought that we somehow caused this infection, and feelings of helplessness given that we thought we'd been so careful with our handling of Max's TPN and central line.

Then Randy talks with an infectious disease doctor, who explains that the most likely cause of Max's trajectory of symptoms is not a line infection that we introduced from the outside. Instead, he probably started with the flu (his first symptoms), which turned into bacterial pneumonia, which led to the staph aureus spreading throughout his blood. This is a common trajectory, whereas starting with a line infection and ending up with respiratory infection in the lungs (which showed up on a chest x-ray) is unlikely. The trajectory of flu to pneumonia to staph in the blood doesn't normally lead to jaundice, unless there is bacterial infection of the liver or gall bladder, but Max's abdominal ultrasound is normal. The jaundice may reflect the vulnerability of Max's liver given his long history.

An MRI reveals that the bacteria has not spread to Max's bones. This is consistent with an orthopedic doctor's exam.

But the bacteria has settled on the left mitral valve of Max's heart. They have always given him an echocardiogram with prior line infections, but we have never given it a second thought. Now we understand what they are testing for. There is a small colony of blood cells glued up with the bacteria there. This is causing a mild back flow through the valve, which is not of concern at this point.

The most serious risk is that some of this tissue could break off and flow through the blood stream, causing an embolism somewhere in Max's brain. It seems like there is nothing the doctors can do about this proactively. They can only monitor and detect after the fact that it has happened. This is maddening. Also, if some tissue remains on the valve after the antibiotics do their work, heart surgery would be required.

We've never had to think about Max's heart, or his brain. And now, after establishing that there is nothing we can do other than monitor him along with his medical team, we try not to think more beyond that. Our understanding is that these are low probabilities, though we'd be more comfortable if someone could provide actual numbers. They say it depends too much on individual situations. I wonder if this is their way of trying to shield us from the truth.

Max's temperature is still elevated on Tuesday, the day after starting antibiotics. He has been going through procedures all night and day. He doesn't feel good. He has rapid breathing Tuesday night (likely caused by his low red blood cell count), and gets a blood transfusion Wednesday morning.

Gradually, some good news starts coming in. Max's blood cultures from Wednesday morning are negative 12 hours out, then 24 hours on Christmas morning. The best present of all. And Max is feeling good. The hospital allows Kai (and all siblings) to visit the wing as a special treat on Christmas Day. Siblings aren't otherwise allowed in during flu season, so this is the first time the kids have been together in three days. They jump right into talking and playing together, while Randy and I prep the Christmas dinner I've brought from home. Our celebration together as a family feels like the shortest few hours ever. Kai and I drive back home through the snowstorm we were trying to beat.

Friday brings us to 48 hours of negative cultures. This is a huge, unexpected relief. It also seems consistent with the idea that the infection was not introduced via the line, in which case it might have been harder to clear. This good news would normally mean time to go home. In this case, they want to keep monitoring Max's other issues.

The echocardiogram team arrives in the morning. The preliminary report from the infectious disease team indicates that there is still some "vegetation" present on the valve. They say it typically takes a while to go away, and the expectation is for it be gone after 6 weeks of antibiotics. Max looks much better than most of their patients with this vegetation (which they see a lot of). Most patients have little red lines and spots in their hands and feet, where the smallest embolisms land and block the small peripheral arteries. Max has had none of these, which is a very good sign. But they emphasize that the cardiology team will make the final call, and that it can be pretty hard to see a small growth on the valve, so cardio might be reluctant to say anything definitive.

We wait all day for the final report from the cardio team: The heart growth has decreased in size and does not look like anything that will require surgery. It is not of much concern to the cardio team, so they indicate that the GI and infectious disease teams can determine when to discharge Max. These teams decide that Max should stay for 3 more days, until he finishes the rifampin. We can run the other IV antibiotic from home every 6 hours, run weekly labs, visit the infectious disease outpatient clinic a few times, and follow up with echocardiograms.

Things clearly could have been much worse. We are so glad that we flew home when we did. We are grateful that Max's infection is resolving like it is. We are relieved to think that our line control procedures are most likely sound, since we fixed one issue with them almost 3 years ago.

We are not relieved to know about brand new risk factors that we had been largely unaware of. But it is good for us to know these, and to know that they are low-probability risks. It is somehow reassuring that aside from the jaundice, these would be the same risks faced by any kid with pneumonia and associated staph infection. To help us face them in the future, we will look into pneumonia vaccines (we had all already taken the flu vaccine) and be prepared to draw blood for cultures more quickly.

Our friends kindly referred to their remaining time in St. Thomas without us as their scouting trip, to find the best places for us to experience when we can all go back there together. We will dream about that possibility. And we will appreciate how lucky we are to be home.

Sunday Sep 6, 2015

I was holding my breath during our week in China. We want to be brave with Max's medical condition. We don't want to be foolhardy.

Max is in the hospital again, for the fourth time in 9 months. When he was born, this was the life we were told to expect. But we haven't experienced it until now.

The first hospitalization was in December -- my last post. The next one was in February -- a translocation of gut bugs from Max's intestine into his bloodstream. That hadn't happened since he was a baby. That should have been a clue. Instead, we focused on other possibilities: Max had finished his antibiotics from his last hospitalization a week before. We had held off on flushing his distal intestine during the uncertainty of his last hospitalization. Maybe these things had offset the balance in his system, leading the bad bugs to overtake the good. And he had been shivering on the ski slopes the day before the admission -- the cold might have meant reduced blood flow to the intestine, and greater vulnerability.

We received an unexpected gift between the two hospitalizations. In follow-up appointments to check on the bacterial growth on his heart from the first infection, a team of doctors concluded that there was nothing there, and in fact, that nothing had been there in the first place! Just an imaging artifact that made a bright spot in a particular view.

During the second hospitalization, the doctors gave us the go-ahead to travel a month later to Amsterdam, Bruges, and Leiden. They reassured us that if something bad were going to happen as a result of finishing the antibiotics, it would happen before we were scheduled to go. Nothing bad happened. We went. We gave talks, saw old friends, and gobbled frites. Max wrote poetry about cobblestones and steppy roofs and Flemish art. He sent it to his teacher, who shared it with his class during their poetry unit.

As spring passed, Randy and I started talking hopefully about the hospitalizations as a weird blip, rather than a new phase we had entered. Then Max had another translocation and hospitalization in June, just after celebrating his 9th birthday. None of our theories explained it. He hadn't just come off of antibiotics. We hadn't held off on flushing his distal intestine. He hadn't been cold.

We saw Christian's family on the East Coast later that month. I told his mom about Max's hospitalizations, and how we didn't know why they were happening. My voice trailed off. Was this really the end of my story? We had no idea what was going on? It felt unreal. I had to confirm with Randy later that this was truly our situation.

We had another scare in July, on the West Coast with family. Kai came down with a high fever. Max said he wasn't feeling well. But he never got a fever, so we escaped having to check him into an unfamiliar hospital. We came back to Colorado uneventfully. Max went in for an endoscopy of his intestine to try to determine why he was translocating.

The idea of Beijing in August was starting to feel foolhardy. We had committed to give talks there during our "weird-blip-rather-than-new-phase" optimism.

Someone on Max's team knew a doctor there. We received reassurances that we should have good care if we needed it. I was re-doing my Pimsleur audio course in Mandarin, which I had taken in preparation for a trip with family to Taiwan last March. But it was terrifying to imagine having to check Max into a Chinese hospital for his typical week-long stay. My Mandarin was suited for laughing with cab drivers over stilted conversations, not something to rely on in an emergency.

Why were we facing this uncertainty, after years of smooth sailing?

Then it hit Randy. This hadn't happened since Max was a baby. Since we started him on Omegaven, the omega 3 lipid that saved him from the liver damage caused by intralipid, the omega 6 lipid.

But last January (2014), we started Max back up on intralipid. More and more doctors seem to be acknowledging that intralipid is associated with liver damage and death, while Omegaven prevents it. But they also emphasize the importance of a balance of fatty acids. Intralipid by itself, especially in babies, can be problematic, but it may be beneficial in more balanced doses in older children. This is the message Randy came away with, from his work as a parent representative on an FDA workshop on TPN-associated liver disease.

It took many heated discussions for us to agree to start Max on intralipid again. We started at a 1/4 dose. We went up to a 1/2 dose. (His other 1/2 dose of lipids comes from the Omegaven.) We watched Max and his liver numbers closely. For months. We saw no bad signs. We saw many good ones. Max grew. His seemed less thirsty. His skin seemed less dry. (Omega 6's help fluid transport.) He seemed better able to fight off illnesses than the rest of us. Other parents commented on how good he looked.

None of us had thought of intralipid to explain Max's mysterious February and June translocations, because so much time had passed. But lags in the system can be long. Once Randy realized that intralipid might be the culprit, we cut it immediately. Max's doctor agreed that the inflammation caused by omega 6 could have resulted in the translocations. Our plan was to avoid it until we made it back from China, or even longer.

But Max started to look gaunt. We re-introduced occasional intralipid. We flew to Beijing. I know it seems crazy. I know because Randy and I have the same conversation every time we contemplate a trip. Yuko: Going to China is crazy. Randy: You say that for every trip. And I convince you every time to go. And all of us are always glad we went. Yuko: That is true.

Our biggest struggle in China ended up being freezing and refrigeration. Hotels elsewhere (including Japan and Taiwan) have stored our TPN in their restaurant refrigerator (if we couldn’t fit it in a room fridge) and stored our ice packs in their hotel freezer. We use these to keep Max’s formula cold during the day and overnight, and to keep his TPN cold while we are in transit.

Our first hotel stored our supplies, but they could never seem to find them, and the ice packs never got hard. We supplemented with a frozen dumpling package. Our next two hotels refused to store the ice packs. The first one was during our conference, so our local host stored it for us in his lab. The next hotel brought us a small cup of ice, and said they could not give any more. I figured out how to say that Max’s medicine needed ice. They brought two more cups. By far my most useful Mandarin of the trip.

We returned to Colorado filled with noodles and dumplings, awe for the Great Wall and Terracotta Warriors, and dreams of future travels. (Max convinced us to make the 12-hour last-minute round trip journey by train to see the warriors, based on what he had learned about the Qin dynasty during a unit on China in 2nd grade. Very persuasive.) We returned home breathing a little easier.

Until 18 days later, with this hospitalization.

Tuesday Sep 8, 2015

Max went in for a regular checkup at Children's Hospital last Thursday. Everything looked good, including the results from his endoscopy in July, we learned. So it wasn't clear what we could do to avoid future infections and hospitalizations. Now everything has changed.

On Friday morning, Max showed signs of bleeding from his intestine. This can happen occasionally without being cause for concern, and Max said he felt fine. He went to school. After school, he continued to show intestinal bleeding, while continuing to feel fine. We called his doctor and decided to bring him to Children's Hospital to get checked out. We emailed friends to cancel a poker game we were hosting that night. We thought Max might come home later that night. We suggested that we could reschedule our game for Saturday, or worst case, Sunday.

When Max arrived in the ER, they gave him IV hydration fluids. His initial blood cell counts looked good, but they explained that these numbers can look fine at first, because the concentration of everything that remains is still normal after blood loss. It is only when you replace the fluid loss that you see the drops in concentration. Sure enough, his subsequent blood counts dropped, and as they were preparing to move Max to a room on the regular floor, his heart rate dropped. So they kept him in the ER until 2 AM to be monitored. Then they moved him to the PICU (pediatric intensive care unit) for further monitoring overnight.

On Saturday, they took him to the OR for an intestinal endoscopy. His last one had been less than 2 months ago, but they wanted to take another look while something was clearly wrong. His blood cell counts dropped further, so he received a blood transfusion Sunday. This guaranteed he would stay another day. He at least moved from the PICU to the regular floor Sunday afternoon.

This scope revealed ulcers in Max's intestine. They start around 15 cm up from the lower end of it, and keep going to about 25 cm up -- the doctor looked all the way up to 35 cm up, with about 5 cm left to go. (So his intestine has grown a bit from the 21 cm he started with, as expected.) The part of the intestine with ulcers wasn't checked during his last endoscopy, because it was difficult to access, and there was no cause for extensive probing before this bleeding. One ulcer looks like the prime suspect for the recent bleeding. The biopsies show eosinophilic inflammation, with no indication of bacterial or viral causes. The treatment is an oral steroid, budesonide.

Max has been on this before. We loved it at the start (December 2012), because he suddenly started absorbing more of his formula and growing -- for the first time in 1.5 years. But we stopped it after 8 months because he showed some behavioral problems that could be related to absorbing some of the steroid. We have since observed Kai exhibiting similar behavior at a similar age, so we hope that it was a developmental issue rather than a medical one.

We arrived home Monday evening, excited. We have an explanation -- not only for Max's recent bleeding, but also for his rough year. His intestine is inflamed (possibly exacerbated by the omega 6 lipid), which makes it vulnerable to ulcers and to translocation. We have a treatment plan -- to reduce the omega 6 lipid to 3x per week instead of daily, and to administer the steroid, which has been good for his intestine before, and which we can insert via the ostomy (instead of the g-tube) to have more direct contact with the area of inflammation and perhaps reduce its absorption systemically -- it is supposed to act on the surface of the intestinal membrane. This hospitalization feels like cause for celebration.

Max is certainly happy. Often when a medical team stops by his hospital room on their rounds, one doctor will sympathetically ask him, "Are you ready to go home?" Max will enthusiastically reply, NO! The residents will start curiously popping their heads into the room. Often one will exclaim, "I've heard about you!" The kid who is happy to be in the hospital (at least after the first 2 days of feeling crummy in the case of translocations). Maybe he knows he gets better there. He definitely knows he gets Lego there. During this stay, he kept busy building a camper van given by a kind nurse, and an Imperial Starship from Daddy.

People ask how we manage. Max's happiness helps. I don't think we rely on false hope. We know this treatment plan won't necessarily work. Just like we knew we wouldn't necessarily be home in time for poker. But we kept trying to reschedule it for a little later in the weekend, until it became definitive that we could not. We know so well that things may not go as planned. We prepare for the worst. We try to anticipate the best.

Tuesday Feb 2, 2016

Max's surgeon wasn't sure if he would recognize us. He performed Max's second, third, and fourth intestinal surgeries at Children's Hospital Colorado. They were all done in Max's first 14 months of life, so almost 9 years have passed.

I knew we would recognize Dr. Partrick. But I was surprised by how relieved I felt to see him. Sometimes reminders from that era take my legs out from under me. Handing our baby over to the anesthesiologist never got easier. Nor did our struggles to manage Max's pain afterward.

But Dr. Partrick has made all the difference in Max's life. We hug. We are hoping he can do it again.

Max has been hospitalized 5 times from December 2014 to December 2015. The latest episode meant another Christmas in the hospital. A gut bug (klebsiella) had made its way into Max's bloodstream again -- the same cause as 3 of his 5 hospitalizations. We had enjoyed 4 days in the mountains with my family, when Max spiked a fever. We packed up and drove the 5 hours to Children's Hospital in the middle of the night, and got Max started on antibiotics the morning of Christmas Eve. We opened presents the next day in the hospital cafeteria, since Kai had a cold and was not allowed in the in-patient wing. "Good timing," declared Randy, about our second Christmas in a row here. He was serious. We had to cut our Snowmass vacation short by only 7 hours. And we brought Max home two days after Christmas, just in time to fly to LA for sand castles and Disneyland and Star Wars and feasting with both of our families.

Good timing. Randy's outlook on life really is this positive. But he is realistic too. We need to get these infections under control. During Max's recent hospitalization, the doctors pointed to his non-functioning intestine as the likely culprit. Twenty centimeters of it remained after Max's third surgery. It has probably grown over the years since. No neurons in this intestine means no peristalsis, so bacteria can easily accumulate and then translocate into the blood stream. So now we are looking at intestinal surgery number 5, to remove most of this remaining intestine.

Getting rid of bad intestine might seem like a no-brainer. Randy is eager to get it done. Dr. Partrick and the rest of Max's Denver team is ready. Dr. Puder in Boston is on board. We are just waiting to hear back from the Pittsburgh team, to ensure that this surgery won't jeopardize any future options for intestinal transplant. (We still hope for the possibility of a stem cell transplant down the road -- amazing advances continue to be made.)

Max is blase. Our hospital bills come along with a substantial investment in Lego sets to help him pass the time. This investment seems worth Max's apparent lack of fear or dread.

Kai and I have that emotional space covered. In theory, this surgery will be straightforward. Max should recover for 3-5 days in the hospital, and then take several weeks to get back to his full cops-and-robbers recesses. I understand the theory. But I would like to have more evidence. The published literature focuses on elderly patients with colon cancer, which may not be relevant for Max's outcome. We have reached out to our network of short gut families, but with a 1 in 5 million condition, and with every kid being unique in anatomy, gut function, etc., we haven't found a single other person who has had this procedure.

Kai keeps asking when the surgery will happen. This question is surprisingly difficult to answer. March 3 was one potential date. We next considered March 17, so that Max could recover over spring break -- he doesn't want to miss school. Then suddenly we were talking about Feb 4. That is now the day after tomorrow. We'll see whether we hear back from Pittsburgh in time.

That would be good timing. We hope that this surgery will make that phrase mean something less bizarre during future Christmases.

Thursday Feb 4, 2016

"Max is in surgery," I tell Kai as we leave our house to walk to school, just before 8 AM. "He did a great job."

"He's done?" Kai asks excitedly. I realize I need to choose my words more carefully. "No, he just went in," I clarify. "But he was really calm beforehand. And he fell asleep smiling, because the medicine they gave him is called laughing gas, and it makes people laugh."

"That's funny. What are they doing now?"

"Well, they are making an incision in Max's belly."

"What do you mean?"

"A cut. They are making a cut. But he won't feel it at all, because he is asleep and is on drugs that make him not feel it."

"He'll probably feel it a little bit."

"Maybe. But remember the story about how much pain I was in when Max was ready to be born? And then they gave me the epidural, and I started telling everyone how beautiful they were?"


"These drugs really can make you feel nothing. So they will reach through the cut, and they will pull out Max's non-working intestine. They will cut most of that out and close it up."

Our conversation ends. We spend the rest of the walk trying not to slip on the icy roads -- not holding hands, because Kai is more stable than me and doesn't want me to take him down -- and we walk single file down the strip of sidewalk shoveled clear of snow.

I walk home and pack -- Randy's backpack (which he forgot in the 4 AM confusion of taking Max to the hospital for a 5:30 check-in), a glass container to warm up leftovers in, Max's fleece jacket (just in case the surgery leaves him more willing to wear one when cold), snacks.

On my drive down, I think about how amazing it would be if everything went just as a colleague wished: a perfect surgery, a quick, Lego-filled recovery, and many years of infection free fun. I know I need to brace for worse. I think about how I will be strong no matter what.

In the hospital parking lot, I see a message from Randy. The surgery has already not gone as hoped. There are too many adhesions (scarring connective tissue) to do the surgery laproscopically (via a small incision using a lens connected to a videocamera system). So the surgeon has to make a bigger incision. This may mean more pain later and a longer recovery. I realize that I will not always be strong no matter what.

At 10:30, we get the call that they are finished and closing up Max's incision. Actually, two incisions, we learn later. Two cuts. They had thought that the surgery might be straightforward enough to not need an epidural for later pain management. But now they have decided to start one.

At 11:30, Dr. Partrick comes out to the waiting area. I nudge Randy awake. Dr. Partrick explains that the first incision was 6 cm, the second one was 4 cm. The first cut allowed him to access and follow the non-functioning intestine down to the second cut, where they pulled out 20 cm, removed it (using a special device that cuts while sealing off the blood supply), and stapled the remaining 6 cm closed. The removed intestine looked unusually vascularized and inflamed -- good to get it out. The larger-than-expected cut meant that Dr. Partrick could easily access this intestine without any risk to the functioning intestine, which was not disturbed at all.

We get taken back to see Max around noon. He looks pale. His surgical bleeding was minimal -- the main loss came from blood collection that the GI team wanted for regular tests. He is not acting pale though. Two staff members are trying to restrain him as he groggily pulls at wires and tubes on his body. He especially wants his urinary catheter out -- but it must stay in while he has the epidural. I hold his hand.

I was anticipating trying to help see him through his pain. But Max is trying to get up, and we are instead urging him to rest. Max has had "emergence agitation" when waking from anesthesia before. We discovered with his endoscopy in September that it is well-controlled by precedex, which he has on board now. He does rest for a bit, as he falls back asleep, wakes, falls asleep again, wakes.

He tries to get up again. This time, he feels some pain, and rests without needing encouragement. We explain the button next to him. He can push it whenever he feels pain, and more medicine will be delivered in his epidural. He can press it as often as he wants. (It will deliver only small doses, but his presses will be recorded so that his team will know when his pain needs are not being covered.)

Max does not push the button.

He also does not seem to mind having this conversation with me every time he wakes:

"Are you comfortable?"


"Are you in any pain?"


Max's vital signs look good, as they did throughout the procedure. The terrifying situations we faced with him after surgeries as a baby are less of an issue with older kids. A room for Max becomes available on the surgical recovery floor, and we get taken there around 1:00.

Max still complains about the catheter when he wakes. But his conversations transition toward Lego and cartoons, even if he falls back asleep before even getting started.

Randy asks me about measures that a student and I are developing for a grant application due next week. I tell him that a colleague's kids have blue belts in karate, so that he can inform Max when he wakes -- he and Kai were wondering about this during our dinner conversation last night, when I told them about an amusing post from this colleague asking if anyone had a spare steering wheel for a school project. We eat lunch, appreciating the ability to talk about mundane things, while Max sleeps and recovers. He is loving when he wakes.

Max does not push the button. Medicine is amazing. Things will go as they go. We are just as strong as we are.

Friday Feb 5, 2016

The day after the surgery is great on paper. Max's functioning intestine starts to wake up. The urinary catheter that he so hates is removed. The fever of 101 that he wakes with goes away. (Infection is always a risk with surgery, but Randy discovers in our records that Max has always had a fever after surgeries and it has always gone away on its own after a day. Apparently the body can react with a fever if the lungs are too relaxed.) Max gets a sponge bath. He completes the Lego Hogwarts Express locomotive. His pain is generally manageable -- he hits the button only a couple of times.

But this day is tough to experience. Max's team wants him to start moving around, to get him breathing, get his intestine working again, and help get the epidural meds to flow down more to his lower incision, where he is having some pain -- the epidural is targeted more on the larger upper incision. All morning, Max doesn't want to move. He feels crummy. He is spitting up. He wants both Randy and me to not leave, to stay with him overnight. When we say that there is only room for one person to sleep on the couch, he points out that Daddy fell asleep in the chair earlier. He asks why someone else can't take care of Kai.

After lunch, Max says he wants to walk from his bed to the couch -- 10 feet or so. Randy and I assist him, moving very slowly. He sits for a few minutes, then walks to the chair, where he works on his Legos. He starts to feel itchy, likely from the epidural, so he gets anti-itch meds (nubain) to counter it.

The anesthesiologist comes by and says that itchiness from the epidural is normally focused around the chest and face, not the torso and legs -- so that Max's itchiness might be a result of the alcohol wash that happens before surgery. It gets washed off thoroughly but can pool around the back.

We move on to the sponge bath. The hospital regulation is for kids with central lines to get daily baths of some form. And it seems like a good idea given that Max's itchiness might have been related to stuff on his skin. I mention that Max had been itchy, and get reassured that the foam soap we are given is hypoallergenic. We give the sponge bath, rinse Max off, and walk him back to bed.

He suddenly becomes violently itchy. He scratches his thighs raw in the seconds before we can restrain him. While struggling against us, he locks eyes with me. He says he would rather be dead. We give him benadryl. I kick myself for not having looked more carefully at the soap ingredients, and start googling "calophyllum tacamahaca seed oil." But the bottle does say hypoallergenic right on it, and the hospital is very good about such things. The nurse explains that the itchiness is likely from the epidural meds -- walking around caused it to move down and concentrate in Max's legs. This was probably true the first time too, and Max was standing even longer this time. He gets his next round of nubain. Gradually, his itching calms.

Max says that he wishes he never had this surgery.

Randy and I end up debating through dinner about who gets to stay with Max. Randy wins. He does not want to drive home and then have to drive back to the hospital Sunday (when I need to be home because of our Monday schedule). He does not want to face that commute, or the despair of returning to sleep in the hospital after having slept back at home.

I suggest that we update our records with the details from today -- both the on-paper positives and the experienced negatives. This may be my way of trying to get some sense of control, since this information can help us to prepare for future surgeries, epidurals, etc. Randy one-ups me with his sense of control. He declares that we will not need the information from today again.

Sunday Feb 7, 2016

The weekend is good on paper and in our experience. On Saturday, Max sits upright during the morning, gets out of bed, plays with his Legos, and goes for a walk around the wing. He gets a less itchy pain med in his epidural. On Sunday, he gets the epidural pulled so he can easily leave the wing (where siblings are not allowed during flu season), so Kai and I drive down to enjoy a nice long lunch and play session together in the cafeteria.

Max moves slowly, and walks with a hunch. But he is clearly recovering. He sits and builds a K'nex rollercoaster.

At one point, Randy swoops Kai up and carries him upside-down. Kai squeals and drops his Wolfy stuffed animal. When he gets free, he starts searching under the chairs and tables for Wolfy. He looks all around, puzzled. Randy is puzzled too. I seem to be the only one who witnessed Max make a beeline for Wolfy, scurry back to his seat, and hide Wolfy behind him. No one suspects Max. He locks eyes with me. We smile.

Monday Feb 8, 2016

When I wake Kai for school this morning, he exclaims, "IS MAX HERE?!" He later explains that he had just gotten up to go to the bathroom, fell back asleep, and had a 1-minute dream that Max was home.

I inform him that Max is still in the hospital. But he is continuing to do well on over-the-counter oral pain meds. He comes home this afternoon.

We are deliriously happy.

We also know that this transition can be surprisingly stressful, as we get resettled and find our new routines after medical upheaval and too little sleep. In this case, we find that Kai can't stop hurting Max. Max wants to roughhouse, and Kai seems eager to please. Randy and I step in to say no. Max needs to heal.

But then Kai starts to make his stuffed animal Wolfy say random things -- like how his favorite team, the Cowboys, won the Superbowl yesterday. (Max and Randy rooted for the Broncos on the small tv in their hospital room, where Kai was not allowed. Kai and I drove back from the hospital so he could root for the Panthers on our home screen, while I cooked a hopeful welcome-home meal.) Wolfy has a delusional history of believing the Cowboys are making their way to the championship game.

Max is laughing, but begging Kai to stop. He explains that his stomach hurts when he laughs, coughs, or yawns. Wolfy says that he is on a Storm Trooper Diet. None of us know what that means, which makes us laugh even harder. Kai proclaims, "I am Elon Musk," which somehow, when one is trying hard not to laugh, is hilarious. I tell Max to do mental multiplication to distract himself from Kai.

MAX IS HERE. KAI IS HERE -- even if we finally have to send him out of the room with his antics.

Monday Feb 15, 2016

Randy asks Max whether he is going to admit how he spent today to his classmates tomorrow, when he will go back to school for the first time in two weeks. I'm not sure I'll be comfortable telling people.

Our original plan was to spend the long weekend in Houston. School was closed for the kids on Friday and today. Randy was invited to give a talk in Houston on Friday, and we often try to travel together to split Max's medical cares and make an adventure of it.

Then old friends in California let us know they would be coming to Colorado to ski. We decided to have Randy make a quick trip of Houston, and head to the mountains after that.

Then we learned about Max's surgery. We prepared to cancel our plans.

That seemed to make sense on Tuesday, the day after Max came home from the hospital. His temperature started to rise. He began spitting up. He was running only a degree high, but he was taking both ibuprofen and tylenol. It was hard to know how much they might be suppressing a fever. So we waited for them to clear out of his system. The evening brought the welcome news that his temperature was normal, and he was pain-free. No more meds.

On Wednesday, I couldn't find Max when I returned home from dropping Kai off at school. He was in the basement, riding our exercise bike. A friend of his came to visit after school. Max showed him how to climb on a railing and fall onto our new bean bag. Max explained to me later that the friend tried the forward and sideways falls that Max demonstrated, but skipped the backward fall. A 2-year-old came over in the evening, the son of an Australian colleague who came to visit my lab for a week, starting on the day of Max's surgery. This dinner ended up being our one chance to get together. Max and Kai initially protested hosting a "baby" who might mess with their stuff. But Thomas found a nerf football and engaged Max and Kai in a game of catch, with plenty of tussling between the big boys for the football. Max was still moving with a hunch, but otherwise seemed fine.

So on Thursday, Randy flew to Houston.

We started to think that maybe we could go up to the mountains afterward -- just to recuperate and see our friends.

We drove to Winter Park on Saturday, ate an early dinner, and went back to our hotel to relax. Max and Kai jumped from bed to bed for the next couple hours.

On Sunday morning, Randy and I worked while the kids did more "relaxing." We headed to the resort in the afternoon. Randy snowboarded while I worked and the kids got in some screen time -- Max playing games and Kai working on his novel. Then Randy and I switched places. After dinner, and a snowball fight, the kids did more "relaxing."

Today, given the countless hours of bed-jumping that have transpired, it seems reasonable to try a mellow ski outing. We meet our friends at Copper this afternoon. We agree to take it easy on green and blue runs. We hit a steep, bumpy patch on a blue run. Kai screams that he KNEW he would hate this plan. He hesitates. Then he points his skis down the mountain. He later explains to me that Powerline Glades (a black run, his first, that he tried over Christmas break with my family) has given him courage. He manages to keep up with Max, who is normally much faster than him. Then he passes Max. Max asks what the deal is. I explain Kai's newfound courage. I add that he is probably also inspired by Max, who has skied pretty assertively for a couple seasons.

That is the last I see of them. I wait to coordinate with our friends, while Max and Kai take off with Randy. They race down runs, with Max in the lead, then Kai, then Max, then Kai. They taunt each other and laugh. They ask if we can come back here over Spring Break.

It seems like anything is possible.

Saturday May 7, 2016

Our greatest hope was that we would not know until this Fall whether Max's surgery was successful. Only 3 months have passed since Dr. Partrick removed the non-functioning intestine. The infections that were landing Max in the hospital, which the surgery was meant to prevent, had been happening every 4 to 6 months. So we hoped that success would mean just waiting, infection-free, for this amount of time to pass.

We did make it back to Copper over Spring Break in March. Max and Kai moved their happy racing and taunting to big bump runs.

Then a last-minute change of plans on a Saturday morning in April took us to the Grand Canyon that evening. We had flown to Tucson to give talks that Friday and catch up with amazing colleagues. We planned to fly home on Sunday. But all United flights into and out of Denver were cancelled Saturday due to an impending snowstorm. United said they would offer only limited flights on Sunday, and they could not tell us anything about whether our flight would be cancelled. The next manageable flight would have gotten us home Tuesday. So we drove home over 3 days instead.

Max had a cold during both of those trips. They elevated his temperature and slowed him down a little -- but no more than any of the rest our colds this season.

Then came Max's routine checkup at the hospital. We typically receive a mix of good news and bad news and no news. Randy and I tend to focus on different aspects of this mix, leading to different levels of optimism and uncertainy. But we are in complete agreement with the assessment today.

Max's growth has been excellent, putting him into the double digits for height and weight percentiles for the first time in as long as we can remember. He looks strong.

Unexpectedly, his remaining intestine seems to be functioning much better since the surgery. The non-functioning intestine had been detached from his functioning intestine when he was a baby. So we had hoped that its removal would have no effect on the functioning intestine. Instead, taking out all the inflammed intestine seems to have made his whole system much happier.

Best of all is the liver biopsy taken during the surgery. After Max's first 3 months on TPN as a baby, his biopsy showed class 2-3 liver fibrosis. After 2 years of TPN -- 17 months of which included life-saving Omegaven -- his next biopsy showed that his liver damage had worsened to stage 4. His doctor published a paper stating that Omegaven did not help with liver damage. But many of us believed that Max's liver suffered most during the 7 months before we started Omegaven, and has been stable or improving since. His February biopsy, after almost 10 years of TPN, shows significant recovery: class 1-2 damage in one sample, and class 2-3 in another.

Max is also absorbing fluids well. He does not get thirsty often, and when he does, drinking water hydrates him. This seems unusual given the other kids we know who are also missing the part of the intestine that would normally absorb fluid.

All of this good news means that we have decreased Max's TPN -- fewer fluids and calories since he seems to be absorbing more of these through his gut, and less time on the overnight pumps (9.5 hours instead of 11.5), since the reduced volume of TPN can be delivered during a shorter period. We have also increased his intralipids back to daily. We had cut them to half dose when we thought they might be contributing to Max's infections, but now people are confident that the removed intestine was the culprit.

The short pump time means that Max is basically only hooked up while sleeping. Great for life and for travel -- which brings us to the final piece of good news at the checkup. Max's team has been trying to coordinate with a team in Japan, to see if they can take on Max's medical care next year, when Randy and I will be on sabbatical and hope to spend time in Kyoto. Their initial response is positive.

Many other pieces will need to fall into place for this to happen. But given that our greatest hope for now was to be waiting for months more for signs that the surgery was a success, we are stunned to be hoping to go live in Japan.

Monday Dec 26, 2016

We thought that Max's health was the only thing we had to watch to know whether we could move to Kyoto for our sabbaticals.

We haven't actually paid much attention to it. Max biked and swam and adventured with us around Pittsburgh, France, Holland, Cleveland, and the Cascades this summer. He has been so energetic and healthy that the anxious wait I was anticipating to make it to this fall infection-free hasn't felt anxious at all, or even like a wait. Randy uncharacteristically held out for a later milestone of success -- our first Christmas in 3 years not spent in the hospital. But he was never too worried.

After 6 hospitalizations in 13 months, Max has had 0 hospitalizations in the last 11 months.

But in the space of 4 of those months, we received two distressing diagnoses.

Baba's came in July. Pancreatic cancer. Mine came in October. Breast cancer.

In both cases, disbelief transformed into fear and sadness. All bets for the future seemed off. We prepared to cancel all our plans.

But so far, we have cancelled nothing. Baba was one of the rare cases eligible for the long surgery to move most of her organs to access the pancreas, then remove the cancer, and finally put the organs back into place. Max encouraged her afterward to hit the button on her epidural 2 minutes before getting up and walking, like he did after his surgery in February. She has been strong through her recovery, gradually working back up to walking several miles each day. Her case looks as good as possible.

So a few days after we came home from her surgery, we flew to Washington state for Randy's cousin's wedding. I stood in a circle with Randy's aunts and one of his cousins -- three of them are breast cancer survivors. They offered their love and strength and understanding for my mom. It was such a powerful feeling. I had no idea how often I would be drawing upon it and support from friends and family in the months to come.

After a routine mammogram in October, we flew to visit baba and jiji over a long weekend. Then came a follow-up mammogram, an ultrasound, and a biopsy, with reassurances of a 95% chance that everything would be fine. It was not. Stage 0 breast cancer. Then came a follow-up MRI to check for spread elsewhere (none), consultations with surgeons, genetic counseling and testing (which came back negative), and a small surgery to check for spread to the lymph node (also negative). All good signs, but it seemed again like everything we had planned for the future would be off.

We were looking a month ahead, into December, for the main surgery to remove the cancer. I recovered day by day from the lymph node surgery. So a week afterward, we flew to La Jolla for a conference that we had been anticipating for over a year. The science was good. The chance to reconnect with old friends was amazing.

We flew to Japan a week later, for Thanksgiving break. We walked 27 miles around Kyoto in 3 days, taking in the spectacular fall colors for the first time, checking out a house to live in next year, and visiting the neighborhood elementary school. After much stilted Japanese-speaking and dictionary-searching and gesturing, a shy older student was brought in to help translate, and we learned that we can register the kids at the school after we get our residents' cards. We said that we understood, and turned to leave as the room full of staff members clapped. We met the doctor who agreed to become Max's overall managing doctor in Kyoto. Max and Kai got excited to learn Japanese, and got decidedly unexcited about visiting temples and shrines. We rode bikes on the scenic wide paths along the Kamo river. We caught up with two friends from college. We feasted on delicious yakiniku and udon and bread and ice cream.

We returned home to finish teaching, wrap up projects, and get the final go-ahead after pre-op appointments. I have been amazed at how much easier it is to be on this side of surgeries. I had been filled with dread thinking of Max's and baba's -- the difficult good-byes and hand-offs to anesthesiologists, the long waits for updates, the uncertainty and anxiety. After I got wheeled in for my small surgery, I looked up and noticed the large lights looming above me. I stirred and opened my eyes, wondering when the surgery would start, and was delighted to learn that it was long over.

The aftermath of the main surgery was rougher, with an evening of nausea, vomiting, dizziness, and pain -- but also comforting conversation with Randy, who described my speaking as remarkably cogent but hilariously slow. I came home the next day.

Max has been encouraging me to "hit the slopes" -- to heal quickly and go skiing, just like he and baba did after their surgeries. (He did this back in February. Baba did not do this since her surgery was in July, but I love that Max thinks she did.) Kai read to me from Diary of a Wimpy Kid, not worrying about how hard I needed to try not to laugh too much or it would hurt.

Randy has been heroic with Max's cares and everything else on top of that. I haven't come close to hitting the slopes, but my recovery is going well. We are envisioning our lives in Kyoto for 6 months next year. Baba may come to visit. We do not know everything we might want to watch for. We will see what comes.