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:

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:

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.

Follow @ThisGutsyLife on Twitter

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.

Follow @ThisGutsyLife on Twitter

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.

.

.

.

.

.

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.

Follow @ThisGutsyLife on Twitter

A reader’s introduction to the adventures of Frankentestine: Part 2.

During the 131 days we were in the NICU, I thought we would be there until L was fixed and then we would go home and start the regular babyhood I had imagined, just on a delay.

In many ways, the hard part didn’t really start until we went home.

This post is not intended to catalogue exactly how and why things were difficult, or how deeply we felt those difficulties. That can come later, and gradually. I have, however, realized over time that many people – even people close to us – don’t really understand the basics of what it takes for L to stay healthy. I was floored when an uncle asked me how L didn’t get dehydrated with all the pooping, for example, when so much of our world was wrapped up in the IV fluids we administer every night. And just last weekend I realized that L’s great-grandmother didn’t even know he had a central line, let alone what that meant.

So here are the basics.

L’s gut doesn’t work well. He only has about 20% of the small intestine he should have, and what he does have doesn’t function as well as it should. Remember our old buddy Frankentestine? All those sewn together bits mean a whole lot of scar tissue, and scar tissue doesn’t absorb liquid or nutrients. It also means pieces of tissue that shouldn’t really be next to each other have to learn to communicate and work together, so motility (the ability of the intestine to move food and liquid from stomach to bum) was really, really poor at first – and then when it started to pick up, boy did it ever pick up. In the first few months home from the hospital, he went from regularly vomiting huge amounts of dark green bile because his gut couldn’t move it through to pooping pure liquid 15 times a day because he didn’t have enough gut to slow it down.  

All of this means that he has needed help to stay hydrated and nourished. A lot of help.

Since his NICU days, before he was really eating or drinking anything at all, he has had two surgically-placed devices that allow us to hydrate and nourish him despite his Frankentestine’s best efforts to make that impossible:

Central Line: Also known as CVL, CVC, central venous catheter. Broviac and PICC are two types (L has had both). It’s not a port, but it’s kind of like that. This is a semi-permanent IV line that goes straight into a major vein. It requires surgery to place or replace (which we have done six times now).

L’s central line allows us to deliver TPN (intravenous nutrition), which gives him more or less everything he needs to stay nourished and hydrated. We were able to gradually cut the number of hours per day he needed to be hooked up to TPN by more than half, but for most of his life L has received TPN nightly, at home, with Z and I serving as his nurses.

We have a love-hate relationship with L’s line, in a big way. It has saved his life; he could not have survived without it. We even personified it, way back at the beginning when we had never known L without an IV pole tagging along. Her name is Ivy (see what we did there?).

But at the same time, it has been the bane of our existence. Ivy landed us back in the hospital 4 times in six weeks shortly after we finally, finally brought him home from the NICU. And we’ve been back in so many times since then that I’ve lost count.

Because a central line goes directly into a major vein, it’s a huge infection risk – and small children can go septic FAST. This means that as long as he has a central line, every time he has a fever we have to spend 48 hours in germ-infested medical prison trying to convince L there are more fun things to do than repeatedly flush the toilet on isolation at the hospital waiting on blood cultures to rule out line infection. Every. Single. Time. We have been admitted for ear infections; we have been admitted for colds; once we were admitted for what the unit pediatrician concluded was probably teething.

Having a central line means that he has to keep the insertion site covered with a sterile dressing. We do sterile dressing changes once a week, in our home. These were no picnic with a squirmy baby, and they are decidedly worse with an opinionated toddler. Because the dressing needs to stay intact, his chest can’t get wet, so L has never been swimming, never played in a sprinkler or splash pad, and only very rarely gets a real bathtub bath, when we have time to waterproof his chest and then change the dressing immediately after.

It also meant we couldn’t access childcare for a long time, until we found a center founded by nurses that was equipped to take care of him with a line. He’s in a regular room, but they have RNs right down the hall who can be there at a moment’s notice. Regular daycare centers wouldn’t have been safe for him because they aren’t trained to handle line care, and they wouldn’t take him even if we were comfortable with it; because of his line, he’s a liability.

G-Tube: Also known as Mic-Key button, g-button, gastric tube, feeding tube. This is a tube that goes directly into his stomach. It looks like a beach ball valve and is held in place with a little balloon on the internal end that we fill with water once it’s inserted. It can get wet. It doesn’t have to stay sterile. It doesn’t require any ER visits or hospital admissions unless a rare malfunction/misplacement arises. If it falls out, we just pop it back in. It’s much, much, much easier to manage than a central line.

L’s g-tube was placed at his last big NICU surgery. At first we only used it to vent his belly, which means it allowed us to let out some of the goop that wasn’t able to pass through before it built up enough to make him vomit. A lot of kids will transition from TPN to continuous tube feeds, which means hooking a pump to the feeding tube that runs a slow, continuous trickle of nutritious liquid (breastmilk, formula, PediaSure, liquified whole foods, etc.) directly into the stomach. In theory, this is easier to absorb than drinking a large amount all at once. For L, it inexplicably made him vomit, even when he was drinking much larger volumes than we were running through the tube. So we’ve been using it for vitamins and medicine and small amounts of certain pureed foods that help us micromanage what’s moving through him. And to be honest, it’s pretty nice to never have to make a toddler take medicine.

Here’s a very clinical diagram of L’s essential medical anatomy:

The end goal is to get rid of both of these things. That’s priority #2. (And yes, you can go ahead and assume that it’s deliberate any time I say #2.) The line should go long before the tube, but eventually they should both be able to go.

Priority #1, though, is keeping him healthy, and he still needs these things to keep him healthy. Now that he tolerates most foods and he drinks a normal amount of liquids each day, we constantly micromanage his intake in order to micromanage what comes out the other end. But he still needs a boost from Ivy and his g-tube.

We’re working on it, though. And things are happening. More on that next time.

Follow @ThisGutsyLife on Twitter

A reader’s introduction to the adventures of Frankentestine: Part 1.

My son didn’t poop until he was 4 months old, and that first little doody cost upwards of $3 million.

This is the story of why.

L was born with gastroschisis, which means he had a hole in his abdominal wall. The cause(s) of gastroschisis are not known, which as you might imagine has been various degrees of crazy-making for me. But that’s a story for another day.

Gastroschisis can leave any or all of the abdominal organs forming outside the body. For L, it was just his small intestine, but the hole in his abdomen was so small that it allowed very little blood flow to that intestinal tissue, so it didn’t form properly, and it didn’t work.

We went through a series of three major surgeries in an attempt to reconstruct enough small intestine for him to get by on. The first was performed when he was just two hours old; that first night, our surgeon returned the intestinal tissue back into his belly and created a jejunostomy. I imagine most of you have heard of a colostomy, in which the colon is diverted out through a surgically-created opening in the abdomen and empties into a colostomy bag – a jejunostomy is similar, only much, much higher in the intestinal tract (in the jejunum).

L had only 10cm of small intestine from stomach to ostomy. That meant he didn’t have time to absorb anything before it exited, and too much milk going in could actually dehydrate him as it rushed on out. So when he started getting tiny little bottles when he was about a week old, he was limited to about a teaspoon of milk at a time, a handful of times a day.

Because he couldn’t eat or absorb nutrients through his gut, the NICU placed a PICC line (a more heavy-duty IV line) through which he received total parenteral nutrition (TPN) and lipids around the clock. TPN is a complex mixture of all the basic components of nutrition that is delivered straight to the bloodstream. In the haze of those early days, I was miffed for a short while that they would call it “parental nutrition” when really parental nutrition ought to be what was leaking through my shirt every few hours, thank you very much – but it turns out that parenteral means it bypasses the gut.

TPN saved L’s life. He would’ve starved without it. But it can also cause significant liver damage. We have been lucky; L’s liver seems to be unaffected by TPN so far. But I feel like I owe it to the TPN-dependent community to add this information, because it’s not uncommon for people already facing substantial health issues to wind up needing a liver transplant because TPN wreaks havoc on their system. This is infinitely frustrating in light of the fact that there’s an alternative version of the lipids component of TPN called Omegaven that could spare many of these livers, but it’s often unavailable to patients because insurance companies frequently refuse to cover it. /end rant. For now.

His second surgery, at two months old, was meant to salvage what tissue could be salvaged and then reconnect his intestinal tract. It turned out that salvaging meant spending 6.5 hours piecing together 15 tiny segments of tissue, each of which averaged only a little over a centimeter long. We lovingly dubbed this patchwork gut his “Frankentestine”; our surgeon told us L had just enough.

With so many connection points the risk of internal leaking was high (and before long that risk proved to be reality), and so the ostomy remained until a third surgery six weeks later. At that point L’s ostomy was taken down, his gut was reconnected and the real adventure began. We spent another month in the NICU before he was discharged to continue the very long, very slow process of rehabilitating his bowel at home.

To be continued….

Follow @ThisGutsyLife on Twitter