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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Poop, There It Is

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:

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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.

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Shit Doesn’t Always Just Happen

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….

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