Back into the fray

It looks like surgery is going to happen. We have a meeting Monday to discuss the specifics of the procedure (his team is weighing several options), but he already has an appointment scheduled in the OR on Tuesday. 

More surgery: not what anyone wants, but here we are...

James walked across 3 units today though, easily the longest walk he's taken since this ordeal began. And he requested food today, after a rough week of not tolerating even a bite, so we are happy to see him resume some semblance of normal activity.

There are 6 weeks between a rock and a hard place

We've been discussing more immediate treatment options for awhile now, and are still playing the waiting game for one major reason: how recent his last surgery was. The Fontan requires about 6 weeks of recovery (only a week of which is usually in the hospital!) and our doctors were adamant that signing him up for another surgery before the 6-week mark is absolutely to be avoided... especially since all surgical options are "shots in the dark", bringing about new risks with at best a 50/50 chance of success.

As of yesterday, it's been 6 weeks.

This is the soonest they would even consider cutting into him again, and even then... no one is eager to rush into the OR again. So we'll keep waiting, but the next-level, more intensive options are unfortunately officially on the table now. His fluid output had decreased over the last month but has jumped back up significantly again recently, so the chance of needing another intervention is looking higher than ever.

James' general demeanor is improving though as he gets used to the ICU and gradually accepts his new routines. We have finally accepted that he won't be home and starting school on time, so we're making plans to give him preschool lessons in the ICU, bring more of his activities to him, take him outside more often, and make the coming weeks or more feel as normal and fun as possible.

Gatsby's Long Haul

Did you know that one of F. Scott Fitzgerald's draft names for Jay Gatsby was James (Gatz)? I was never a huge fan of The Great Gatsby, but every now and again I can't help but notice my son embodying a meme-like stance of decadence and defiance as he sits and waits, sits and waits, sits and waits. He doesn't have the Long Island Sound to sit and stare at, but our view out the window at Weill Cornell ain't a bad place to wait: we get to see Roosevelt Island, morning NYC sunrises (weather permitting), and all the boat traffic scooting by on the East River. And no, these images are not James taking up smoking; that's a fruit snack. 

Meanwhile, as we wait, we find more and more ways to defy the system while seated. Even if we can't go home, we found a way to get him outside: it's as simple as a wagon ride. Of course, to achieve this we also needed to complain about being cooped up for over five weeks, add a little scientific argument about vitamins and sunshine, and remind them daily how much they take going outside for granted by asking them to tell us about the weather during rounds.

And what magical, mystical, blessed sciences does rounds reveal?

Nothing. Or little to nothing. Or virtually nothing.

They (doctors and nurses) are still confounded. They monitor bloodwork for reduced this's and that's, elevated odds and ends (yesterday, elecrolytes; today, albumin and IgA; "wait and see" for tomorrow's menu...); they still tally his daily pleural chest tube output (seems to be getting a little higher, not a little lower, which everyone says is strange, and means sooner or later they'll start talking about ligations and catheterizations and such, but "wait and see"...); and they still watch James stare them down each and every time they enter the room from behind the curtain (did the Wizard of Oz once say, "wait and see"?...).

This is, sort of, a standard protocol for chylothorax, "wait and see," because there is no steadfast cure, no proven way to expedite recovery, and almost no mathematical or scientific way to determine how long it will take (means, modes, standard deviations can't help us here). And while James seems to be defying even the norm here, letting doctors see virtually nothing as we all "wait and see" the days fly by (today is.... Day forty-one going on forty-two? but who's counting?... Everyone. James has surpassed the majority of patients most hospitals have seen in terms of duration of hospital stay for chylothorax, and he's on track to exceed them all...), it seems we need to embrace the big ideas for any long haul -- patience, steadiness, perseverance. With maybe an occasional dose of decadence, and a healthy batch of defiance.

Lazy Sunday

What a perfect day for a nap in the summer sun! After 34 days, he finally got to escape for a few minutes outside. Being wheeled around like a little prince sure is tiring...

Has it really been so long?

Our last post was a week ago, and it seems that the days since have flown by in a haze of hospital beeping, interrupted nights, and bad coffee.
 
The short story? Nothing to report. Output is still high and we are exploring options for long-term treatment since nothing seems to be making this go anywhere anytime soon. Today marks 1 month in the ICU, though technically James has been here 32 days, and with no end in sight, we could have weeks or months to go.

The longer story: James is now on a no/low-fat diet, so we've been testing out food this week. He had a great day eating yesterday, but couldn't keep anything down today. (Solution: add a new med.) He has barely been sleeping. (Solution: add another new med.) His heart rate has been high ever since his atrial flutter episode last weekend, and docs are trying to slow it down (by adding a new med). Each med has its own side effects, including nausea, insomnia, and elevated heart rate, which circles us back to the beginning of this paragraph...

As a result of much of the above, folks here think James may be suffering from delirium, a common psychological condition that affects people in long-term hospital care. It would explain the poor sleep and his increasing agitation. (Mommy and daddy are pretty sure they're also beginning to suffer from delirium at this point, and they're only being partly facetious...) We've already started advocating for home care, or moving to a clinic closer to home, because we all know that James' crazy cornucopia of conditions will clear up faster in a familiar, happier environment...

Still, many mini-positives to note: James is still improving on the walking, and has made it all the way around the PICU now, albeit with many tears and complaints. He's been cranky a lot lately, because, of course, he's tired, but we've also caught him singing to himself and singing along with his playlists and videos. He has been coloring and using stickers a ton and is apparently ambidextrous now after having spent so long with his right hand out of commission. He is even starting to help the nurses, lifting his arms and offering his i.v.'s for temperature and blood pressure and other checks.

We had lots of toys and activities prepared to occupy him while stuck in bed, but we hadn't anticipated a month and more, so we're starting to cycle and recycle through things at home. Of course, we're starting to think about what else we can do now because kiddo is starting to get bored!, so if any of our faithful and creative readers have any brilliant ideas, please share away.

We are all so ready to go home...

Compounding Complicated Complicating Complications

Day 26, Boatswain's Log from the SS James, hull leak is staying about the same, we're still adrift at sea with no sails or engines, and miraculously we're catching more fish for food, but high seas are making the remaining crew nauseous and mutinous, this ship is taking a beating, and still no land in sight...

A drawling, quiet week until Thursday, during which there was not much to report.

Thursday, however, we began a series of very eventful days, with one thing leading to another, and another, and another, and another . . .

Thursday morning, James went for a walk. Physical therapists gave him the cutest little walker, looked like a little old man walking down the halls and out to see the fish tank in the family room. Cried the whole way there, the whole way back, and at every point in between, but he did it all on his own.


Thursday, afternoon, Cardiology brought him in for a post-op cardiac catheterization. They wanted to check and make sure his chylothorax wasn't being exacerbated by a new Fontan connection that left too much pressure in his lungs. Kirsten and I weren't fans of this idea -- we knew high pressures were the most likely cause of the chyle leaking from his lymph system -- but the team presented a good argument for it, and we couldn't ignore the logic. It was very frustrating, as you can imagine, when they brought him out, confirmed his pressures were high, and then said there was nothing they could identify that would help: previous to the procedure, they mentioned ballooning, stenting, and coiling, but none of these options would have done anything, they said, to help. So, they performed a collage of minimally invasive procedures that will keep James knocked out for a few days, for what seemed/seems like nothing, reminding us what we all already know: James simply needs time for his body to adapt.

Friday, when he was finally awake after his restless night after the cath and long day-time nap, James suffered from another complication: atrial flutter. This is essentially one of his heart's atria running a sprint while the rest of his heart jogs to catch up, making all of his heart run fast, but his atria run super fast. While no one can say for sure that it was a direct result of the dyes or fluids or methods they used during the cath, because this does happen to healthy people without direct cause, this was one of the things they warned us could happen as a result of the cath, and required another procedure to get his heart rhythm's back into sync: cardioversion, or simply a nice little shock treatment.

Of course, James has a Fontan heart-lung system, with chylous effusions, so at this point almost everything that can happen to him puts him at substantially higher risk of clotting, and atrial flutter left to it's own defenses (most normal people would get back to normal rhythm without intervention) makes even more possible a clot in the heart. So, they did the cardioversion quick and fast Friday evening, shocking him back to normal rhythm, and putting him on digoxin to help maintain his normal heart rhythm.

And that gets us to Saturday.

Today, James was cranky, nauseous, irritable, and refused to sleep. After being woken every 30 minutes last night to check his heart, or give him meds, or take his blood pressure and temperature, or change his diaper, or give him another med, or do another EKG, James has decided he won't sleep. Beyond him becoming a vampire, he is now becoming an insomniac. He wouldn't stand, cuz the digoxin makes him dizzy and nauseous, and he wouldn't lie down, cuz the digoxin and the shock and the stickers and wires make him edgy, and he wouldn't sleep, cuz he's again wary of anyone in the room with him, including mommy and daddy.

But, he spent the morning watching videos, eating ice, playing ipad games, and generally wearing himself out with restlessness, so he finally went down for a nap -- eyes partly open just in case. And, tonight we got him to do some light standing, stomping, stretching, and a few unsteady, but purposeful, steps -- to get his ipad :)

3 week Fontanniversary

The Fontan was 3 weeks ago today, and we're still here, and nothing has really changed in the last week.

Our team of Doctors, who did an amazing job with the Fontan and have made some great efforts in getting him closer to going home, have admitted that no one knows much about chylothorax, why it happens, or how to fix it. The next step will be a catheterization later this week. It's a "just checking" move, which is unlikely to resolve anything, so we aren't expecting to see much progress or have much news to share.

James received a couple of care packages today, which we greatly appreciate. His "room" is now decorated with cards and pictures and there are Thomas stickers everywhere. And mommy and daddy have snacks! :)

The IV in his right hand forces him to draw with his left. He is right-handed, so it's frustrating, yet he keeps asking to color anyways. It's pretty amazing how well he manages :)

ugh, it's Monday

Many people share the sentiment: ugh, it's Monday.

Today, it's not a return to the work week that's dragging me down but disappointing numbers from James. The TPN and medication seemed to be starting to slow the leaking, but yesterday it ramped back up again. Total fluid output more than doubled.

It's hard to stay confident when the leaking has gotten worse.

Unless a miracle happens tonight, we're anticipating a serious discussion about other options.

Ugh.

At least James is happy today!

Tug of War

Yesterday we did standing and walking, and more standing and walking, and more and more standing and walking. Yesterday was a long day. This past week has been a long week. And at the end of a long day, a long week, what better is there than a long, hard, peaceful rest?

James slept, without meds, for a full 9 hours last night, from 7 to 4 am. He has been awake ever since, and is looking happier and more James-like every day. We even got a James-original smile yesterday!

Medically, though, we are still playing tug-of-war. 

Tug-of-war was always one of my favorite family reunion and summer camp games. Two teams vying for victory over each other through a balance of weight, strength, and strategy. I think I liked it most, however, because who won wasn't always about the strongest or heaviest or most strategic: sometimes luck, or sheer will, or environment had a silly way of turning the tides. 

Despite the improvement often seen (~60%) through the conservative three-part treatment plan James is on -- TPN (i.v. nutrition), Octreotide, a medication used to slow the flow of lymphatic fluid, and no food or drink, all for up to 2 weeks -- James' condition still has yet to improve in the last week. There was a marked improvement in the first few days of this plan -- last Thursday, Friday, and Saturday -- down from about 800 cc's per side per day to about 150 cc's per side per day.  But then not much happened, so they took him off Octreotide to see if he just needed time to rest, recover, and start walking.

After a week and a few days, we're averaging about 200 cc's total, or a cup, per day for the last 3 or 4, but it has been fluctuating up and down, up and down. Progress, but progress has slowed, and sometimes reversed, so docs are gonna put James back on Octreotide. It should only take a few days for this treatment to do something noticeable, and we're hoping both that this plan will help and, more importantly, that James will be patient enough to get through Tuesday without eating or drinking anything.

This past week, James has been continuing a very long and hard game of tug-of-war -- he pulls a little, his condition pulls back a little. He's not happy about being here instead of home, being forced not to eat, or having all his i.v. tethers and these hospital walls keeping his freedom of movement limited, but every day he keeps playing this game of tug-of-war.