NOT KNOWING

In my last post I described a moment in which I allowed myself to think, “Things are really going well for us.” This sentence was significant because I formed it very deliberately, feeling out each word in my head against my strongest instincts to not let myself get too hopeful.

There’s a second reason why this thought was significant, though, and that reason didn’t even occur to me until several minutes after the thought came into existence, which just amplifies the reason why it’s so very significant:

I had this thought while L was still on TPN.

If you had asked me a year ago – a few months ago, even – I would’ve told you I couldn’t feel like things were good while L still had a central line. And there I was, gushing to myself about how great my life was going without even registering that L still had his line.

I’ve already outlined why his line is the bane of our existence. That hasn’t changed. But somewhere along the way, it’s become so normal for us that it doesn’t feel horrible anymore. We’ve adapted.

For a very long time I clung to the thought that someday L would be fixed and we could move on. It took me a very, very long time to wrap my head around the fact that we might never reach a point where we could say things were finally, definitively good. I think in the first weeks and months my brain might have refused to entertain that notion out of self-preservation – if I had really comprehended from the start that we would be living in this state of NOT KNOWING for the rest of our lives I’m pretty sure I would’ve short circuited right then and there.

So I spent L’s entire first year devoting all my energy to trying to get rid of his central line, and thinking that once it was gone we would finally be in the clear to move on with our lives. I threw a party when he became line-free. And I explained away the signs that he was slowly declining, even though in retrospect it is obvious that he still needed TPN. That summer was The Bad Summer, and it was hell – but GI was trying to let him succeed without TPN, and I refused to admit to myself that we might have to take a giant step backward until his labs suddenly plummeted and we wound up in the hospital for an emergency blood transfusion. Even then, I fought tooth and nail for anything that might keep us from having to re-place a central line; I raged, I cried, I yelled at residents; but he needed it back, and back it came.

After our first round of trying to shed L’s line failed miserably and I realized how much better things were for us after we took that big step back, I thought that I had become more okay with NOT KNOWING. But I hadn’t, not really. I had realized I probably needed to be more okay with dwelling in the in-between space – progress! – but I still spent all my energy trying to claw back out of it. We spent seven months last year living month-to-month, thinking all the while that maybe this next appointment will be the one where she says we can pull it, or maybe just one more month, and now just one more month again. Only he didn’t gain weight, and at every appointment he showed one deficiency or another or several. But going without TPN wasn’t failing either; he didn’t lose a significant amount of weight and his deficiencies weren’t that severe. And so we waited, in medical purgatory. I thought that I had grown stronger, but I still had a long way to go.

There is not a single point at which I suddenly became okay with that pervasive NOT KNOWING. But there have been several small moments that made me realize how far I’ve come. That day in the car was one of them – for a brief moment, the fact that I could forget about TPN took my breath away. Another came last winter when a fellow intern and I were commiserating over job search woes; neither of us knew what was coming next, and I recognized in her the same kind of overwhelming anxiety-riddled impatience with NOT KNOWING that a few years ago would have kept me up at night and tied my stomach in knots, and realized that instead I was strangely calm in the face of this particular round of NOT KNOWING.  

Don’t get me wrong on this – I have not transcended to a higher plane of zen acceptance of uncertainty. I had not found some noble way to be at peace with the fact that the final weeks of my already-pitiful intern income were looming and if I didn’t land a job we would be plunged into exponential debt almost immediately, andplusalso I might have made a really bad gamble with signing on to be a 30-year-old intern to begin with and my whole entire future was hanging in the balance and could come crashing down at any minute. No, I can’t say I was really at peace with that. If I were to guess, I’d say the parts of my brain that should’ve been going up in flames about all that are just burnt out. They have been overworked to the point that they have packed it in and are waving a permanent white flag.

But you know, if those fried neurons allow me to have moments of calm where there used to be moments of rage and panic, I’m okay with being a little burnt out.

I promised I wouldn’t spit shine things that ought to stay ugly. I would never, never, promise someone going through hell that things will get better, because sometimes they don’t. But honest doesn’t mean devoid of hope. So here is an honest message of hope for anyone who might find these words at a point when you think you can’t keep going:  

Sometimes it doesn’t get better. But you get better at it.

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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:

AnatomyofaLucas

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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