Gutsy Surgery: Six Months Later

I think medical time moves differently than regular time. It has split personalities, perhaps. When you have young children, the weeks and months seem to fly by somehow (“Can you believe he’s going to be FOUR?! How is he almost four years old?!!!”) but in many ways, even this last big surgery already seems like a lifetime ago.

A friend asked me recently, “So, was the surgery a success?” She’d asked me something similar before surgery – how will you know if it works? – and I had fumbled through an explanation about how there may never be a clear answer to that question, in much the same way that there’s never really a finish line for us; there’s no real “cure” with L, it’s always an ongoing process of management, and varying degrees of better and worse.

Today though, just six months out, I can say without hesitation that our October surgery was a resounding success. Even to write that makes my breath catch just a little – how gutsy of me, to make such a bold proclamation, and surely perhaps a little naive? In our house, we don’t even say “at least bedtime should be easy tonight” because that’s the surest way to guarantee it will take hours; we’ve spent so much time refusing to look directly at progress for fear it will slip away that to voice the words “resounding success” feels downright dangerous. As certain as I am that this surgery was a success, I am equally certain I will live the rest of my life waiting for the other shoe to drop.

And yet – his appetite came in with a roar less than two weeks post-op, and there’s been a hungry lion living inside him ever since. He eats seven or eight times a day, and not just grazing; he’s eating full meals, and my picky eater’s short list of “L-approved” foods has expanded from roughly a dozen fruits, carbs and cheeses (but only in very particular shapes and forms) to an entire world of food groups and variety. Meats! Fish! Nuts! GREEN VEGETABLES!

He’s eating, and tolerating nearly unrestricted liquid intake, and his gut is working so incredibly much better than we ever dared to hope before. He’s having beautiful poops (Look, mama! Let’s take a picture!), and only 2-3 times each day as opposed to the so many we were seeing before. His belly is smaller – it would get horribly swollen before surgery, due to the dilated sections and bacterial overgrowth – and he very clearly just feels better all around. The cause and effect of all these things seems a bit chicken-or-egg, but they all play off each other to keep the rest moving in the right direction.

He was able to come off of TPN (IV nutrition) about six weeks after surgery, at which point we switched to overnight g-tube feeds (that means we’re running a medical version of Pediasure through the feeding tube in his belly, which goes directly into his stomach as if he were drinking it, only in a slow, steady drip that’s more easily absorbed). Over the last three months, we’ve cut the volume of that overnight Pediasure in half, and he’s been able to maintain weight, stay hydrated and stay on his growth curve; we’re hoping to keep forging ahead and sooner or later ditch the overnight feeding pumps altogether.

The biggest remaining hurdle is vitamin deficiencies, which aren’t going away anytime soon and can cause some serious health problems over time if left untreated. But even there, it’s an ongoing process of management, not a discouraging setback – and we have some exciting prospects on the horizon in the form of patches and pills.  

We’ll never be completely free and clear. L’s gut could re-dilate. He could develop a new stricture or obstruction or perforation – I still suppress a dull panic every time he complains of a stomachache. He could experience an illness that unexpectedly throws us into a long downward spiral. And as he grows, he may go through stretches where his body can’t keep up with his nutritional needs, and we may need to supplement in one form or another.

But for now, he is thriving. He is healthy. He is line-free. And we are making up for lost bathtime.

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Tales of a Line-Free Kid: The Pinch

Being line-free has more advantages than I can quickly catalog here, but it also has one definitive drawback: needles.

Malabsorption necessitates frequent blood draws to monitor vitamin levels, hydration, liver enzymes and a plethora of other things. Depending on what else is going on with his health, L has had monthly, biweekly, weekly or occasionally even daily labs since he was born. However, with the exception of a brief stretch in the summer of 2014, L has also had a line of some sort his entire life. That means that we have had the good fortune (can we really call it that?) to have been able to draw his labs through his line, needle- and pain-free. Because we were, erm, between doctors for several of those line-free weeks in 2014, he has had a grand total of one traditional blood draw in his entire life, at around 15 months old when he was too young to remember.

Until today.

I was nervous, heading into our appointment today. I have friends with kids – small children, far too young to be so brave – who are so accustomed to needles that they chatter and giggle through labs. There are others, though, many others, who have been so traumatized by extensive poking and prodding that they require sedation for even the simplest blood draw. L does well when we explain to him well ahead of time what’s coming, and for a 3.5-year-old he is very receptive to being reasoned with, so I hoped I could employ some of the tactics we have long used to talk him through dressing changes; but, thanks to regular dressing changes, he also panics when we so much as pull tape off his skin. I felt as though an awful lot of weight was riding on how I managed this first needle.

In the empty waiting room, we made a game of squeezing together into each of the extra wide chairs. As the panicked screams of another poor little soul rose ever-louder from the back, I amplified my laughter in a futile attempt to mask what awaited him.

Finally, it was our turn. “I’m scared,” he whispered.

“I know, buddy. But remember what we talked about? I’ll be with you the whole time, and it’ll just be a little pinch and then it will be all done.”

The phlebotomist led us to the back, and L climbed up into her giant chair unprompted. He was immediately taken with the perpetual motion toys on her desk (Dory! A pterodactyl!), and perked right up when I offered the holy grail of distraction: YouTube toy review videos (curse you, Ryan’s mom, but in times like these I am grateful for every shrill unboxing. And by times like these I mean blood draws, 5 am, and when I just want to pee in peace for once, dammit).

As she pulled out the needle, I was irrationally taken aback that there was no warm up, no sleight of hand. All of a sudden, there was a big shiny needle just staring at us. “All right, here we go,” she said. “Just a little pinch.”

I braced myself to hold him down, as I’ve had to do far too many times before, but he gave only the slightest whimper when the needle went in, and another small whimper when it came out. He watched like a hawk as she wrapped his arm in coban – red, because she had heard him say red was his favorite color as we watched the blood flow into the syringe.

He turned to me, and I held my breath, waiting for his words.

“Mama.”

His little voice was very stern.

“That pinch made a HOLE.”

“Yes, buddy, it did. I suppose I should have said it would be a poke instead of a pinch. But it wasn’t as bad as you thought it would be, was it?”

“Yes, it was! It was exactly as bad as I thought it would be –”

My mind raced to come up with exactly the right words to steer him back in the right direction. But there was no need:

“Can I have more stickers?!”

Line free is the way to be….I think….probably….Yes, okay, we’re definitely maybe ready for this. I think.

This morning we got a belated Christmas gift:

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I hadn’t wanted to say too much until it was actually done, because we have already delayed the line removal procedure once due to a cold – and we have been close enough to this enough times to know that even when things seem to be going stupendously, that can disappear in the blink of an eye (remember what happens when we get cocky enough to look progress directly in the face? Poof! Liquid shit.)

It’s funny, thinking back over my evolution as a short gut parent. This is the fourth time we have stopped TPN, and the second time we have pronounced ourselves line-free. The first time, a lifetime ago, when we were still with our old GI and before I had begun to make peace with the NOT KNOWING, we only stopped TPN because I pushed for it – and oh how I chafed when we were required to keep his line for a whole month before pulling it, just to be sure he could gain weight without it! And when we finally did achieve that golden pinnacle of line-free-ness, I threw a party, and I fought like hell when we began to move toward replacing a line and going back on TPN.

The second time we stopped TPN, I felt seasoned. I accepted the waiting period, or so I thought, because I knew exactly what kind of hell it had been the previous summer when L’s nutrition slowly failed. But I still went to every appointment with eager anticipation that this time would be the day GI would give the approval to pull the line, or maybe this time, or surely now this one – and I made myself crazy, as seven months slipped by, with me always thinking this week would be the week, until a series of mild illnesses proved that L was not ready to be off of TPN after all.

The third time we stopped TPN, just about nine months ago, I was resigned to the process. I fully expected months on end of weight checks and labs and waiting just a little longer, and then a little longer still. I didn’t ask when we could pull his line (or at the very least, I didn’t ask relentlessly at every opportunity). And four months later, when TPN returned and we began discussing surgery options, I was not surprised. I didn’t fight. I knew he needed it.

And now, having just stopped TPN for the fourth time, I have completed my 180 degree swing. Last month, just two weeks after our last night of TPN, GI began talking about pulling his line by Christmas.

Are you sure? Shouldn’t we wait just a bit longer?!

He can do it, she said. He’s ready.

And so, just in time for the new year, we head into what I supposed you could call Phase 2 – although this is really more like Phase 2 version 4.0, and in reality, like always, we will just keep chugging along and hoping this doesn’t turn out to be Phase 2 version Samsung Note 7.

Although, you know, now that Ivy is gone, if Phase 2 begins to catch fire we can just toss L in a pool. He’s been asking to go swimming….

Look out, 2017. Next year is the first year of the rest of L’s life.

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Gutsy Surgery: A Timeline

For anyone who is interested, here is a breakdown of the first two weeks after intestinal tapering (and yes, there is poop talk. With descriptions. I mean, you are reading a blog about guts, after all).

Even though the surgeon ended up only tapering and opening up a stricture rather than tapering and lengthening, we were told this would be fairly similar to a STEP or Bianchi recovery since he still had a long staple line in his gut, but with less risk of complication that could delay progress. Given the difficulties we endured in his first year or so after his first three big surgeries, we were prepared for all of the worst scenarios, but L hit the best case scenario on basically every mark and came in right at what I had estimated would be our shortest possible stay (10 days). Here’s an overview of what it looked like:

Sunday, T-1 day: Admitted to the hospital for a bowel prep

  • We were told to be there at 11am, but since L didn’t need an IV or an NG tube placed we just hung out in the playroom until about 5pm.
  • We followed standard procedure for a bowel prep – NuLytely at 462ml/hour – and we were shocked that it didn’t rush through him. I was incredibly skeptical, but he actually required an extra hour of the bowel prep solution (run as a continuous feed through his g-tube) before he produced an substantial BMs. In all, he pooped a total of six times in about 15 hours.
  • On clear fluids only until midnight, when he became NPO. To our surprise, he did not get upset about that.

Monday: Surgery

  • Surgery took about three hours, most of which was working through adhesions from previous surgeries (but he didn’t end up doing the lengthening procedure which would have taken longer, just did tapering instead).
  • By 10am when we were in pre-op, L knew something was up and was getting upset about not eating/drinking.
  • Epidural went in at the beginning of surgery, after he was out.
  • At 3.5 months old he had 35cm of small intestine; now at almost 3.5 years old, that has grown to 105cm.
  • Tapering and lengthening turned into tapering and opening up a stricture, as detailed here.
  • Post-op: Slept for most of the next 24 hours. G-tube draining to gravity; 24 hours TPN; NPO. Asked for a drink once, but was not upset when I told him no.
  • High fever (103F) and elevated heart rate, which is apparently a normal response to gut bacteria that inevitably get out into the abdominal cavity.
  • Some pain control issues overnight, but doses of narcotics came quickly when we needed them.

Tuesday, post-op Day 1: Epidural and narcotics for pain; began stirring midday; out of bed in the afternoon; catheter removed; g-tube draining to gravity; NPO.

  • 24 hours out: first real smile, in response to tech’s Lightning McQueen lanyard. Still speaking less than usual, and mostly in whispers.
  • Fever down to 99-100.
  • Pre-op we were told he would need to stay in bed while he had the epidural, but they actually wanted us to get him out of bed at least three times a day. Tuesday afternoon he sat up on the couch for a while to play – I carried him over very gently and with full support, and he leaned against me, not sitting up on his own. He still had feeling and movement in his legs, just too sore and unstable to move much.
  • Asked for a snack and a drink, but did not get upset when told no. This is a child who has had RAGE at previous stretches of being NPO for any significant length of time.

wagonrideWednesday, post-op Day 2: more of the same.

  • Fever gone.
  • Wagon ride! And up and playing on the couch again. Much more personality back today – even some major giggles.
  • Still requiring full support to move (not using abdominal muscles at all) – and afraid of being moved, even if it didn’t end up hurting.
  • Without the catheter, he is not peeing without great concentration, and then only in small amounts. Increased fluids, but it seemed to be an epidural issue because he returned to normal urination as soon as the epidural was gone (Thursday night).

Thursday, post-op Day 3: more of the same.

  • Good day in terms of pain management. More willing to move.
  • Right at bedtime on Thursday, his epidural began occluding. Pain team could not salvage it, and we spent Thursday night without it and pulled it first thing in the morning on Friday. (Lasted around 80 hours.) Some pain control issues overnight.
  • More than 600ml out through the g-tube (that’s a lot considering nothing was going in); still dark green and opaque.

Friday, post-op Day 4: Upper GI follow-through; g-tube capped during imaging but returned to gravity afterward; still NPO.

  • Imaging took 5 hours; with the epidural gone, we were allowed to go to the play room in between. No leaks or other issues visible in imaging.
  • First post-op steps (in the playroom, of course!)
  • Did just fine with g-tube capped, but poured out several hundred mls immediately as soon as we let it drain again. Still dark green, and too much to leave capped; returned to draining.

Saturday, post-op Day 5: Approved for clears; drank a total of 1oz

  • Made it through the night without epidural or narcotics, but needed morphine before bed and upon wakeup.
  • At bedtime and at wakeup – panicking from the pain.
  • POOP! Five times in about three hours in the late morning, dark and loose, then no more for the rest of the day.
  • Very little drained through the tube overnight; some out in the morning on Saturday, but it was less volume and also less green, and after seeing poop they were content to cap it off and see how he did.
  • Allowed sips of clears, but did not take in much.

Sunday, post-op Day 6: Clears only; drank 9oz; tolerated.

  • Significant discomfort, but we suspect it was from holding in poops – he was afraid to go because he was afraid it would hurt.
  • 9oz total clear fluids; no vomiting.
  • Did not poop all day, until late evening when he squeezed out a tiny bit because he really really really wanted that damned Paw Patrol train set we’d been keeping as an ace up our sleeves.
  • Paw Patrol train set convinced him to play on all fours and reach across with apparently no discomfort.
  • Surgical resident approved crackers just before bedtime; L ate two. Apparently resident did not consult with GI because the next morning we were on soft foods only (no crunchy carbs, much to our pretzel-lover’s chagrin).

Monday, post-op Day 7: Soft foods; pain under control.

  • Approved for full diet of soft foods. Ate a few bites banana, peaches, cheese.
  • FORMED POOP. Formed. Solid. Pieces. Let’s take a moment to marvel at those little pale, smushy, miraculous tootsie rolls. 
  • Pain under control as long as he has fun distractions. Once he was tired and the play room was closed for the day, back to sad and uncomfortable. Until he remembers the Paw Patrol train.

Tuesday, post-op Day 8: Home.

  • Discharged on 20 hours TPN.
  • Our stay was a total of 10 days.

Wednesday, post-op Day 9: Appetite!

  • Significant afternoon discomfort – but may have been hunger pains?!
  • Very few bites at breakfast and lunch, then dinner: ⅓ mac and cheese, whole peach cup, slice of deli ham. Sips of liquid throughout the day.
  • TOLERATED ALL OF THAT!
  • 2 poops

Thursday, post-op Day 10: More of the same; still on 20hrs TPN.

  • Still hungry, not at all thirsty
  • 2 poops. Formed poops. We’re talking Play-Doh poops, guys!
  • Only discomfort is when tired; easily distracted by fun toys.

Friday, post-op Day 11: GI appointment. Cut to 12 hours TPN (to begin Saturday night). Still on soft diet.

  • Thrilled with weight gain and amount of intake he’s tolerating; cut TPN to 12 hours – he was on 10 hours before surgery.
  • Restricted to a soft diet for at least another week.

Saturday, post-op Day 12: More of the same; last day of 20 hours TPN.

  • AM: Best poop we’ve ever seen. Who wants to see a picture?! Because I definitely took a picture of it.  
  • Pain under control with no medication at all during the day; single dose before bed Friday and Saturday.
  • Drank almost nothing, because still running TPN 20 hours/day.

Sunday, post-op Day 13: First day of 12 hours TPN; hungry and thirsty; tolerating increased quantities; first day of no pain meds.

  • Breakfast: 2 cheese sticks, whole slice deli ham, ⅓ banana!
  • Drank around 16oz of Drip Drop and milk; tolerated it brilliantly.
  • Only 1 poop, right at wakeup.
  • Confident enough to go down the slide at the park by himself for the first time post-op.
  • Did not take a dose of pain meds before bed, and had discomfort throughout the night – possibly also related to going all day and all night without pooping.
  • Still not a single vomit so far.

bandaidsIn the days after surgery, L put Band-Aids on all of his cars and trucks and on each Paw Patrol pup. Yesterday, he took them off, because “they’re feeling all better now.” I think that is not insignificant.

We are keeping him home this week to make sure he continues to tolerate increased fluids, because we’ve really only just started that, and he is still on soft foods at least through this week (back to GI on Halloween), but since he’s down to 12 hours on TPN and seems to be flying through this recovery, he will most likely be ready to go back to preschool next week – just three weeks out from surgery. He is excited to see his friends!

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What to expect when you’re expecting surgery

I set out to write an honest post about what it’s like to be drawing close to a big surgery that could change our lives dramatically without knowing whether that change will be dramatically good or dramatically bad. I think that the best picture I can give of what that feels like is to say that right now, I’m not at a point where I can dive into all that.

Let’s just say there are feels. Lots of feels. So many that I kind of need to ignore the feels and stick with facts for now.

So here are some facts and tips from our first three experiences with big surgeries, along with some additions from other minor surgeries we’ve had along the way. L was only 3.5 months old at his last big surgery, so my facts and tips regarding those kinds of procedures are focused on tiny bodies and very early stages of development, and I don’t have advice for preparing older children to understand what’s about to happen – although I would love to hear yours, if you have some.

Here’s what I do have:

Ask questions. Early and often, and don’t stop until you’re satisfied with the answers. Write down a list of questions and bring it to appointments with you to make sure you get them all answered. Ask them to explain again if you don’t understand. Ask questions even if you don’t really know what you need to ask.

A good place to start may be to ask what kinds of things you should be asking, especially if you can connect with a knowledgeable and objective third party – a parent who’s gone through the same procedure; a specialist or surgeon who is familiar with the diagnosis or procedure but is not involved in your child’s care (i.e. before we met with the surgeon who’s going to be operating on L next month, I had emailed a surgeon at Boston Children’s and had inquired with specialists at two other hospitals in other states via friends, just asking what we should ask the surgeon and if they had any advice to help us make our decision).

And at L’s second surgery, our surgeon had expressed concern about keeping L under anesthesia for any longer than he had to after the two-hour mark, but we were expecting a minimum of four hours in the OR; in our pre-op meeting with the team, I simply turned to the anesthesiologist and said, “Make me feel better about this.” And you know what? She did.

img_3911You can probably accompany your child further than you think. If they don’t offer, ask. For L’s last line placement, I scrubbed up and carried him right into the operating table, then stayed with him and held his hand and talked to him while they put the gas mask on to start sedation. Plus then you get a cool paper outfit and neat hat and stuff that you’ll definitely want to keep for the scrapbook.

Do not do this if you’re going to freak the flip out once you get in there. Seeing the OR could be scary. Your child might struggle with being handed over, or laid on a table, or with getting the mask, or a number of other things. It’s a horrible feeling to watch your child’s eyes glaze over and drift away. If any of these things are going to make you lose it, don’t go in. You’re not taking the easy way out; you’re doing what’s best for your child. The surgical team needs to be focused on taking care of your kid, not on calming you down.

Anesthesia is scary – but in most cases, it’s maybe less scary than it seems. The anesthesiologist that I mentioned told me that there were people in the OR whose sole job was to monitor vitals and make sure that if there were any signs of adverse reactions, the surgeon knew to wrap it up immediately. She also told me that while extended anesthesia in babies and young children has been linked with developmental disorders and delays, it’s likely that correlation does not equal causation because children who undergo extensive anesthesia have a reason for needing multiple and/or lengthy surgeries, and those reasons are more likely to be the cause of those correlations than the anesthesia itself (i.e. L had gross motor delays, but he had his abdomen cut wide open three times and spent a good deal of his first few months flat on his back, for pete’s sake).

Ask for updates. Most hospitals probably have some kind of update system in place – we could see on a screen what phase of the procedure they were in based on a color coding system, for example. But ask what else they might be able to do. During L’s big surgeries, we had a nurse call my cell phone right from the ER to give updates on how things were progressing. This not only was a huge reassurance, but it also meant we didn’t have to sit in the heavy hush of the waiting room for 6+ hours. Which brings me to my next point:

Don’t just sit in the waiting room the whole time. Get up. Walk around. Eat something. Go outside. Bring a laptop and find somewhere comfy to watch something stupid on netflix. Maybe even laugh, if you can. You’re allowed to feel things besides fear and anxiety and dread.

Be prepared for post-op. Ask what to expect, and be prepared for it to not go 100% smoothly. This will vary greatly based on the type of procedure. If you’re expecting invasive neonatal or infant surgery here are some things I was completely and totally unprepared for:

  • Swelling. Preemies and infants are tiny. Surgeries involve an awful lot of retained fluids, and in an 8-lb person there’s not a lot of space for all that fluid to go. They cut open L’s belly, but the swelling extended all the way up to his face and right down to the tips of his toes. L was almost unrecognizable for a while after his big surgeries. That was hard, and made harder by how unprepared we were to see him like that.
  • Intubation. I knew they would intubate him. I was not prepared for the way it felt to see my child crying silently around a vent tube.
  • Pain management. It took a little while to find exactly the right combination of meds to keep him comfortable. And in the second week, when I was asked to weigh in on giving him another dose of narcotics or not, I was not prepared for the way it felt to see him slowly glaze over and drift away again and know that I had made that choice.
  • Unexpected outcomes. L’s jejunostomy was a difficult one, to say the least, and we thought it would be gone after his second surgery. The surgery was more complicated than we’d expected, and he kept that ostomy for another 6 weeks. It had not occurred to us that they wouldn’t be able to take down the ostomy at the second surgery, and so we felt a little defeated even though the news from the surgery was generally very good. If you’re up to it, hash out every possible outcome and complication with your surgeon before surgery so you can go into post-op as informed and prepared as possible.
  • Backward steps. Recovery = progress = moving forward. But sometimes, it’s not a straight line. Sometimes your kid isn’t ready for what you’d like them to be ready for. Sometimes there isn’t a clear finish line. Sometimes steps back are necessary for progress.

Accept help. People will probably offer. You will probably be inclined to turn them down. If you need something, don’t be afraid to say yes, and specify what you need.

Be gentle with yourself. Whatever you’re feeling? It’s okay. You might not react the way you expect. You and your partner might react differently. That’s okay too. Z tended to get emotional at all the appropriate times, like when we found out good news and bad news, and I tended to sit stone-faced through post-op debriefs feeling like an a**hole because I wasn’t properly emotional, but then cry at all the inappropriate times, like in the checkout line at the grocery store or driving 70 mph down the highway (that last one might be less okay).

If you find yourself needing more support, whether from family and friends or medical staff or a therapist – seek it. You’ve been through a lot, and you’ve spent most of that time taking care of someone else. Remember to take care of yourself too.

I’ve done this a few times, but I don’t claim to be an expert. This is the kind of thing that’s never quite the same, and always hard. If you have more tips or advice, please leave a comment!

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Adventures of Frankentestine: The Next STEP

A while back I mentioned L was having some imaging done, and then I casually dropped the fact that we may be facing another big surgery into a nice little feel-good story that turned out to be a not-so-subtle mention since everyone and their mother ended up reading it! So, now that we have some concrete plans on the table, I figured it was time to circle back and dig in to what exactly that means.

L’s gut is dilated. It’s far bigger around than it should be, and it has gotten significantly worse over the last couple years. That means that instead of pushing its contents along like you would expect, when it squeezes the stuff in the middle just kind of sits there, or some of it might actually go backward instead of forward, and that also allows normal gut bacteria to build up into not-at-all-normal amounts. Because of all this his gut (which is already at a disadvantage because such a big part of it is missing) definitely doesn’t digest the way it should.

We’ve tried a few things to push his nutrition in the right direction, and we haven’t seen the results we needed. So he’s back on TPN, and we’ve been talking about surgery to correct the dilation, and now that surgery is scheduled for four weeks from today.

There are two options: STEP and Bianchi. Both of them narrow the dilated gut to a more typical circumference, and at the same time increase the length of the gut, with the goal of dramatically improving bowel function.

The Serial Transverse Enteroplasty (STEP) procedure was pioneered at Boston Children’s Hospital, and as they helpfully explain, “the STEP procedure relies on the simple anatomic principle that the blood supply to the small bowel travels from the mesentery and traverses the bowel perpendicular to its long axis. In the STEP operation, special devices are used to simultaneously cut and staple the bowel in a direction parallel to this plane.” Got that? It’s simple anatomic principles.

Basically what that means is that in the much-wider-than-it-should-be tube of the dilated small intestine, they make a series of small cuts along each side that, when sealed with staples, make a narrower zig-zag tube out of the formerly wide straight tube, like so:

step-illustration-2

The Bianchi Procedure is a slightly older procedure that essentially takes the dilated tube of the bowel and cuts it lengthwise (if you can dig back to elementary school art class, that would be hot-dog style) and makes two narrower tubes out of the one wider tube, then sews them together end to end so you wind up with about half the width and twice the length. For your viewing pleasure:

bianchi-illustration

The STEP seems to be increasingly popular, but for a number of reasons that we are completely in agreement with, our surgeon prefers the Bianchi for L if possible. It doesn’t lend itself to punny blog titles quite as well, but the Bianchi does have a slightly lower risk of complications, and if it’s not successful, it’s much easier to do a follow-up STEP after a Bianchi than to do a Bianchi after a STEP. But either could be tremendously successful for us, and that will ultimately be a game-time decision in the OR.

The neat thing about these surgeries is that, if all goes well, he will not only end up with a much narrower and more efficiently-functioning gut, but also with as much as (nearly) double the length in his small intestine.

The less neat thing about these surgeries is that there is a 5-10% chance of a number of complications that adds up to about a 20-25% chance of things not going 100% smoothly. This is not comprehensive, but potential complications include:

  • Because blood flow has been rerouted in his gut after all the previous surgeries, some blood flow could be severed which would result in a lost section or sections of bowel.
    • Although unlikely, it’s possible that this could result in the return of our old 2013 foe, The Ostomy.
  • Because of all the cutting and stapling and sewing and attaching, there’s risk of a number of different versions of bowel leakage (like fistulas and abscesses). If these occur and don’t resolve themselves, they could require further surgery and result in more lost bowel.

The bright side to both of these possibilities (although what’s not bright about abscesses and ostomies, amiright?) is that even if he ends up losing more gut, it should still work better. In theory, we’re working from a post-op ideal outcome of double the gut, so losing half of that would put us at the same amount of length but much narrower, and so much more functional. In theory.

The other fairly obvious complication, if you can call an expected outcome a complication, is that his gut will have to re-learn some things. We’ll be essentially starting over with feeds, moving to 100% TPN for the week or so after surgery and then slowly adding in oral and enteral (tube) feeds. The goal will be to gradually shift the ratio of calories he’s receiving from TPN to oral/enteral, and eventually come off of TPN altogether.

He may bounce back relatively quickly, but it could also be a very slow process. This is a wait and see kind of thing. Hello again, NOT KNOWING.

It’s scary. Recovery will probably be frustrating. It’s possible this will leave us in a worse spot than we are in now, and it’s difficult to choose something that has a chance of a worse outcome, especially since L feels great since he’s been back on TPN. But the reality is that if we do nothing, he will continue to have immense nutritional struggles and difficulty growing, and sooner or later he would wind up with liver damage from long-term TPN and then might need small bowel transplant or liver transplant or both, neither of which are likely to be as successful as the STEP or Bianchi are likely to be. And there’s a 75-80% chance that he will fly through all this with absolutely no significant complications at all.

This is our next step. It’s a very big step, and it’s very likely to be a step in the right direction. It could be huge for L, and for all of us. This surgery is scary, and difficult, and overwhelming, and it’s hope.

Original diagrams courtesy of University of Michigan Children’s Intestinal Rehabilitation Program, with helpful labels added by ThisGutsyLife.

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A Bit of VyGyvering: Using curly tubing for enteral feeds

When we switched from TPN to enteral feeds in March, we were supposed to go from the lovely, stretchy, old-school-phone-cord Vygon “curly” tubing we’d been using for TPN to the short, not-stretchy, not-curly, not-lovely, anxiety-inducing-because-who-knows-what-it-will-get-wrapped-around-in-the-middle-of-the-night regular tubing that comes attached to enteral feed bags.

That would not do.

So, after a bit of quick googling that turned up some ideas but no methods or results from trial runs, we decided to try our hands at MacGyvering the Vygon IV tubing so we could use it for enteral feeds. We simply hooked up the Vygon tubing to the G-tube extension on one end, inserted the end of the feed bag tubing on the other end, taped the crap out of it and hoped for the best. And — it has now been approximately 115 nights, if my count is correct, and we have fed the bed exactly once.

For anyone out there who’s interested in trying it, here’s a quick tutorial:

1. Widen this end of the Vygon tubing just a smidge by inserting the tip of closed scissors and spinning the scissors a few times. Bonus points if your scissors coordinate with the color of your feed connectors. Z is stylish that way.

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2. Insert the connector of the feed bag tubing that looks like a geometric purple Christmas tree into this end of the Vygon tubing that looks kind of like one of the Easter Island dudes with giant Dumbo ears. (As you can see, these are very technical instructions.)

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3. Insert the other end of the Vygon tubing into the small port on the G-tube extension.

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4. Tape the crap out of it. Wrap around the connection at the small port, keeping downward pressure on the Vygon tubing toward the g-tube extender, then keep wrapping around all the elbows of the port end of the extension in a way that allows you to come back around the connection point in both directions – so, if your first pass around it is clockwise, make sure you come back around counter-clockwise before you’re done so there’s pressure on it from all directions to keep it in place.

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When you’re finished it will look something like this:

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5. Tape the crap out of the other part too. Take a moment to make a weirdo face while you think about the prospect of being able to sleep better at night, if only your dear sweet child would actually sleep for once….

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6. Proceed with priming the tubing and hooking up as usual. Ta Da!

If you try this out, or if you have other solutions, we’d love to hear about it in the comments!

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Why nothing is simple: Bellyaching about bellyaches

Recently L has been talking a lot about his belly hurting. He has been vomiting only occasionally, but he tells me frequently he needs to puke. There’s nothing super worrisome going on, but his belly has been swollen and he has spells on a lot of days where he very clearly doesn’t feel good in between playing happily.

Here is a glimpse into why, for us, a bellyache is awfully complicated.

When he tells me his belly hurts or he needs to puke, it could be:

  • A stomach bug
  • His belly hurt earlier and he is three and he likes to repeat things
  • He ate something that would upset anyone’s stomach
  • He ate something that wouldn’t upset most people’s stomach, but it’s upsetting his
  • He wants to get my attention
  • His many supplements and vitamins are upsetting his stomach because he didn’t eat enough
  • He ate too much
  • He ate a normal amount but for whatever reason it’s moving through a little too slowly
  • He wants to watch a movie and I let him have extra screentime when he doesn’t feel good
  • He ate something that got lodged in his gut somewhere and is causing irritation (he once puked a strawberry he had eaten a full two weeks earlier)
  • He has an overgrowth of gut bacteria causing a buildup of gas, which can lead to swollen belly and vomiting (since bacteria feed on sugar and he lives on carbs, and overgrowth is common in guts with poor motility)
  • He recently learned what a “puke bowl” is and thinks it’s fun
  • He needs to poop
  • His belly feels a little off and the easiest way to tell me about that is to say he’s going to puke
  • He doesn’t actually feel bad but he doesn’t want me to touch his g-button right now
  • It’s bedtime and he wants to get out of bed
  • Overnight tube feeds are finally catching up to him and we need to give him a break from them
  • Other very unlikely but much worse possibilities that I try not to think about because very unlikely and also terrifying

Because we hardly ever know exactly which one (or combination) of these things it is, the ideal response could include:

  • Getting him to eat something
  • Not pushing him to eat because it will upset his stomach more
  • Skipping overnight tube feeds for a night
  • Giving him attention
  • Taking him to the potty
  • Letting him pretend to puke in a “puke bowl”
  • Waiting for him to actually puke to clear out whatever the problem is
  • Getting him to eat a balanced, low-carb diet (excuse me while I take a moment to double over in laughter)
  • Letting him have some screentime and teaching him he doesn’t have to fib about puking in order to watch a show
  • Treating stomach bug or bacterial overgrowth with antibiotics
    • This is complicated for reasons I’ll explain in a minute
  • Teach him not to cry wolf
  • Tell him it’s time for bed and he’s not getting up to pretend to puke or poop when he doesn’t really need to.
  • Let him get out of bed to poop or puke
  • Panicking about the potential for very unlikely but terrifying scenarios and rushing to the ER (for the record, we’ve never yet felt the need to do this)

Now, I’m no mathemagician, but if my calculations are correct that multiplies out to roughly 1.2 kajillion possible combinations of cause and response. And the thing is, at any given moment we are very unlikely to know the actual cause, so we have to guess at the appropriate response.

So it gets messy. (Literally.)

Potential consequences of choosing the wrong response:

  • Pushing him to eat when he shouldn’t might make him vomit, which would result in fewer net calories than just letting him not eat to start with. Over time, if this happens too often it will lead to weight loss and dehydration and he’ll have to go back on TPN
  • Not encouraging him to eat when he needs some food in his belly might make him feel worse, and if he misses out on too many calories over time he’ll start to lose weight and he’ll have to go back on TPN
  • We can’t decrease his vitamins, so if it turns out he’s miserable because the supplements upset his stomach, he’ll need IV vitamins and/or TPN
  • Trying to enforce a balanced, low-carb diet will mean he will never eat anything ever except maybe bacon
  • Letting him get out of bed to poop or puke when he is fibbing about it just to get out of bed will lead to neverending bedtimes every night
  • Not letting him to get out of bed when he needs to poop or puke will lead to a big mess and probably permanent psychological scarring from having a terribly mean mother
  • Trying to teach him not to cry wolf might make him feel like he shouldn’t tell me when he really does need to poop or puke. See above re: psychological scarring
  • Skipping overnight tube feeds might make him eat more, and one-night trials of this have gone well (yay!) but if he’s not absorbing what he’s eating he’ll start to feel worse and worse, eat less and less and end up back on TPN
  • Too much screentime will clearly turn him into a hyperactive slush-minded psycopath, because studies
  • Antibiotics wreak havoc on his gut so they are an absolute last resort, and also the usual suspects to treat overgrowth have not been successful for him. If he has significantly increased diarrhea/vomiting due to antibiotics, he will dehydrate and lose weight and end up back on TPN
  • Assuming that very unlikely, terrifying possibilities are not the cause could lead to the worst, or could lead to more surgeries and more lost bowel, which would mean ending up back on TPN, possibly indefinitely.

See any patterns there?

I think the pervasive state of acting within these unknowns is one of the hardest things to really understand for people looking in from the outside. If you’ve never dealt with complex chronic illness, it seems like there should be diagnosis → treatment → health, but for us it’s constant managing and balancing, and I want to really emphasize the constant part of that.

There’s a clear diagnosis, but that diagnosis comes with all sorts of secondary and tertiary issues that are not always so clear. Each issue, even if we can pinpoint the actual source of the problem, may have a different appropriate response for different people’s bodies, or on different days with the same person. Often the best option is choosing the lesser of two evils, which makes those choices extra hard – for example, we are constantly focused on weight gain and nutrition, so in a scenario where he doesn’t want to eat and I suspect pushing food will make him vomit, but it might actually make him feel better or stay the same, it’s hard for me to accept skipping those calories, because I’m supposed to be making him grow. For us, every facet of taking care of L is permeated with navigating those unknowns and balancing acts.

In any case, the vast majority of the time it’s nothing, or at least just the same old stuff. But because of all these unknowns, and because this has been going on long enough, we’re going in for some imaging next week to say hi to the old malrotated small bowel and see what it’s been up to in there. This might seem stressful, but it’s actually a pretty routine thing for someone with significant bowel issues, and I have spent enough time thinking about all the worst scenarios and wondering how to respond to the everyday scenarios that it should actually be a relief to have visual confirmation of what, if anything, is not quite right.

Say cheese, Frankentestine!

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Quick Update (you can look at this one)

I made the slightly unwise decision to start this blog right before we began a house-hunting adventure, which I thought would last a weekend and turned out to last for weeks, and across multiple minor illnesses. So I had planned a string of posts between the last one and this one, and now I have a very nice editorial calendar for May 2017….

This will be just a quick update, because I imagine some people have been wondering – how is the NOT KNOWING going?

L had an appointment this morning. It was his second since I last posted, because at the first one he had just come off of a very minor illness and had lost 2lb and had crummy labs (because no illness is really a minor illness when you don’t have much bowel). GI wanted him back in two weeks instead of a month, because he had slipped far enough fast enough that she didn’t want to let him keep going unchecked for longer than 2 weeks, if he couldn’t right the ship.

Today, he had gained 4oz since our appointment two weeks ago (good thing I bribed him to eat that banana on the way in!), which isn’t much, but it’s enough to say he’s not continuing to slip at an alarming rate, especially given that he’s had two massive pukes in the interim – likely due to bacterial overgrowth in his gut, which is common among short gut kids. GI recommended we keep working on getting him to eat a better diet, because apparently subsisting on cheez-its, pretzels and sliced bread is not an ideal balance (who knew?). His labs were mostly unremarkable, and we’ll wait until next time to check vitamin levels again because two weeks is too soon. If they keep heading downward, we may be back to IV vitamins. If not, we may talk about moving forward. For now, we are currently at sum total neutral, which used to drive me crazy but now just bumps up against those nerve endings that have gone numb and floats on.

And so we wait.

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Don’t look directly at this post.

About a month ago I was driving and thinking about all the recent happenings in our lives. I’m in a new job that I love and that makes us much more comfortable financially; L is doing well; we have opportunities opening up ahead of us. I thought to myself, “You know, things are really going well for us.”

Now, that’s not a terribly profound sentence, but allowing myself to actually, deliberately form that sentence in my head is significant. The last time I deliberately formed that sentence in my head was just a couple of weeks before L was born, and we all know how that went.

I’m not going to pretend like I had some premonition that L had an undiscovered medical problem – on the contrary, I was totally blindsided by that double-decker bus of information – but I knew enough about complications and pregnancy loss to have my share of anxiety that something would go wrong. By the seventh month, though, that was dropping away, and I allowed myself that thought, and then BAM! Things weren’t going so well anymore.

Since then, we have not allowed ourselves to look directly at progress because as soon as we do it vanishes. “I think his poops are slowing down!” Poof. Liquid shit. “He’s finally tolerating whole bottles!” Poof. Liquid shit. “He’s doing great without any TPN at all!” Poof. Emergency blood transfusion. And, liquid shit.

So Z and I do an awful lot of talking without saying any real sentences, and we’re not always great about updating people. I know some of our friends and family would like to know a little more about what’s going on with him sometimes, but, well, liquid shit. For the love of god, people, think of our furniture.

So here’s an update, but I’m prefacing it with all this because I need you to know that when I say things are going well it’s really more like “things are maybe kind of sort of going well but it could go south at any moment or we might think it’s going well when really it’s going horrendously and please don’t expect much and maybe also don’t talk about it or think about it or look at it.”

So stop what you’re doing and hold this post up in front of a mirror and read its reflection. Or have a complete stranger read it and give you a quick summary. At the very least, put on some sunglasses.

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Ready now?

Ok, here it is: We stopped TPN cold turkey 23 days ago. And it seems to be going well.

Sometime near the beginning of the year I didn’t feel like we were pushing forward very quickly, so I asked about trying enteral feeds to see if we could transition to that instead of TPN. Enteral feeds mean nutritious liquid is run through a feeding tube into the gastrointestinal tract (as opposed to the bloodstream with TPN) – what this boils down to for us is that if we could do tube feeds instead of TPN, we would be using the not-at-all-difficult g-tube instead of the bane-of-our-existence central line. And if it worked, we could get rid of his central line altogether.

GI had doubts, but I continued to pester her about it and at the beginning of March she agreed that a 1-night trial couldn’t hurt. So we hooked up the feed pump instead of the TPN pump and spent the night watching for vomiting, stooling out or distressed sleep (none!) and spent the next day watching for vomiting, changes in poop or changes in appetite (none!). To say the very least, GI was thrilled, and so were we – every time we’ve tried enteral feeds in the past it has inexplicably caused copious amounts of vomit.

So we’ve moved ahead with just enteral feeds and no TPN, replacing intravenous nutrition calorie-for-calorie with enteral nutrition. We’re still hooking him up to something overnight, but it’s easier to prep and it doesn’t have to stay sterile.

Now we wait. He has to prove that he can gain weight without TPN, and that he can maintain vitamin and mineral levels without TPN, and we need to keep his line for easy, needle-free access for frequent blood draws to check on these things for as long as it takes to prove he doesn’t need TPN, or to prove that he does.

We’re hopeful. No matter the end result, we can say with certainty that he is tolerating enteral feeds much better than he ever has before. Against my better judgement, I’ve already allowed myself to entertain the thought of summer swim lessons. But we’ve also tried ditching TPN twice before (without adding enteral feeds) – in 2014 he was off of TPN for 4 months, and last year we went a whole 7 months without TPN. The first trial period ended with an emergency blood transfusion and nearly two weeks in the hospital, and the second trial period ended with my two year old being admitted to the hospital because he got drunk from eating too much bread (yes, really – but that’s a story for another day). So we’re hopeful, but that hope is surrounded on all sides by this might not work.

And so we wait, for another month or another year. And we don’t look directly at it.

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