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,
pool of blood,
leaking stomach acid,
puppy dog pajamas,
lives in our hands,
A full life We moved to Boston when Max was 7
months old, where Mark Puder and Kathy Gura saved his liver and life
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,
go go go,
hippos, and a
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
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
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
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
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
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
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
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
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
The idea of Beijing in August was starting to feel foolhardy. We had
committed to give talks there during our
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
Baba's came in July. Pancreatic cancer. Mine came in October. Breast
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.