Not so fast

It's been one week and a day since James arrived at the hospital for his Fontan, and it seems he will be here a while longer still.

The complications and "rough start" after the Fontan are slowly getting better, but he's not done with them yet... and that delay is starting to be an issue in itself. Immobility and swelling are not easy on the body.

The fluid leakage (pleural effusions) in his chest are still lingering around, and the leaking actually has picked up pace lately. Even worse, he's leaking fat - a condition called chylothorax. Basically, he started eating over the weekend, but his body is still so waterlogged that the pressures in his body aren't allowing his body to drain properly, so fat is entering his lung cavity and making it more difficult for him to breathe and recover. This is not good, and it means another week or so in the hospital at minimum, and he's back to a no-food regimen with IV instead.

He's been starting therapy to sit, stand, and walk but we didn't get far. Sitting went ok, standing was attempted but he didn't actually put weight on his feet, and so walking clearly is not going to happen yet. He barely moves his hands still, and even his favorite activity (the ipad!) is not inspiring him.

He is covered in bruises and scabs, and the amount of swelling makes it hard to get IVs and needles in him as needed, so he's been poked and stuck all over the place. The swelling also makes it uncomfortable to move, but without moving the swelling doesn't go away. No food for 48 hours won't help him want to exercise either.

We had hoped for an uncomplicated Fontan, but that's not what we got. The first couple days had his doctors scared. The pleural effusions are turning out to be a big complication, which could potentially mean weeks of recovery still needed. Next steps (and hope) are to see if 48 hours without food fixes the chylothorax. Best case scenario, this approach works and his fluid keeps draining and 1 more week in the ICU will be enough.

He is fully awake now, no longer in a fog, and he's clearly scared - but he is also so brave about all this crazy activity around him. He appreciates all the love sent his way!

Baby Steps

Today, James finally got out of bed and into a chair! :)

Sitting up was his first step toward taking actual steps, and at the end of the day we put him on his feet. Still too unsteady to stand, but tomorrow we'll see if we can get him to walk a few steps, maybe across the room.

James is completely off IV drips, getting just IV injections for things he can't take orally, and all of his central arterial and venous lines have been taken out. No pumps, no machines, just James and his new blood pathway system. He even drank some bottle and ate some jello and chicken soup broth.

Still not much in the way of talking, James tended to do more croaking today, but just after the doctor on call removed his last central line and was about to walk away, James uttered his first full phrase since the surgery: "thank you, doctor." :) It was a bit garbled and hard to hear or understand, but a great sign James is taking some great baby steps to recovery.

Tomorrow: walking, more eating, and maybe some more speaking. 

Dinner and a Movie

One, Two, Three, Four....

I declare a thumb war! :)

James was up every hour last night, falling back to sleep fast each time.

Then at about 4 am, he woke and asked for water and his iPad. He drank a little, and held his iPad, then fell asleep watching a lullaby video.

By this morning, they had weaned him off almost all his meds, and reduced his CPAP oxygen to normal air, with just a little nitric to keep his lungs open; they're weaning him off the nitric today, and when they're done they'll start weaning him off oxygen and forced air to take his CPAP off, though that could be a few days.

And then, one by one, the other tubes, wires, and IVs started coming out. His main chest drainage tube came first, followed immediately by his cardiac pacing leads, and then his Foley catheter. He's got a low regimen of meds for pain, but he was sedated so long that his body is still, overall, fairly numb, and he is still really out of it, so he's only had a few low doses of morphine, and otherwise has been taking tylenol and ibuprofen.

Anyway, James has a long way to go, but he is a trooper and is doing an awesome job with recovery.

Thank you all for your happy thoughts and support as our family goes through this. We couldn't do this without each and every one of you.

Busy End to a Quiet Day

So, after a long pause, we extubated today! :)

The breathing tube is out and James is now on a slightly more complex version of a CPAP device: forced air with oxygen. Fluid is steadily coming off, which is good, and they plan to try reducing his different meds and oxygen concentrations overnight.

His Fontan circulation is now fully operational.  

Of course, we weren't sure this was gonna happen at all today. James was off sedatives at 4 am, and it took him over 12 hours just to wake up. Slowly, he came out of his dream cloud, gave an occasional cough, shrugged his shoulders, and raised his braced arms to scratch his itchy brows.

Once awake, he was super bleary eyed and made very tiny movements, but he was awake enough to take out the tube. And as soon as it was out, so was James -- fast asleep again.

James has barely made a peep all evening, with super hushed "yesses" when asked if he wanted water, and only one itty bitty croak to indicate he wanted to move a little to be more comfortable. So, we moved him, gave him a wet sponge to chew on, a few minutes of a Thomas video to zone out on, and he promptly fell right back to sleep -- again :)

Since then, they've reduced his meds drastically already, lowered his forced air pressure, and removed just about all his IVs and drips from the bedside.

Tomorrow, we're expecting him to be a lot more lively, but after a very quiet start, James gave us a nice happy busy evening.

One more hurdle cleared.

Little Dreamer

James has made slow but steady progress in the last 24 hours. Big picture-wise, he's unchanged: fluid retention, low blood pressure, sedated and asleep. The pain meds keep his blood pressure too low, but easing those meds makes him agitated, so he's been somewhat at a standstill.

There have been lots of minor changes and improvements though: little by little, his numbers are improving, and each improvement allows a medication to change slightly, and then he improves a teeny bit more...

He also looks a bit less swollen today, but the big news, as usual, will be when the breathing tube comes out. It's still expected to come out tomorrow, which means breathing on his own. It also means stopping the sedation and letting him wake up.

The fun part is, even while semi-unconscious, and connected to a couple dozen different tubes and cables, and laden down with arm braces (so he doesn't pull those tubes and cables in his sleep), James is a mover. Last night, he went from parallel to perpendicular in his bed, bit by bit, shimmying his way across and down the bed.

As always, James determines his own directions, and we can't wait to see him conscious and tell him we love him - let's hope he's ready tomorrow!

Wait and see

James has been fairly steady over the last 12+ hours, which is to say that he's been steadily unsteady. He still has a lot to worry about and his team of doctors are watching him closely. He's on a lot of medication, and needs more meds that he's not stable enough to begin. They're going to leave him be today, but check him frequently, and as soon as anything improves they'll gradually lower some doses and start integrating the next wave of meds.

He has a lot of fluid retention which has leaked around his heart and lungs and keeps him swollen and his blood pressure low. But treating this would drop his blood pressure, and it's already too low to allow that...

He had a very rough time immediately after the surgery, and all afternoon yesterday we were unable to see him while his doctors worked to bring down his temperature and get him hydrated and stable. Although they didn't give us details until afterwards, he was in critical condition. He's much more stable now, but will not be out of the woods for awhile.

His big concerns today are raising his blood pressure and getting the excess fluid to start draining. He still has a fever on and off but it has stuck to manageable levels throughout the night.

The BIG next step is taking out the breathing tube. Ideally this would have come out yesterday, but James is no where near ready yet to breathe on his own. The tube will stay in until Friday, most likely, if his current trajectory holds.

 

James' Recovery Begins

James is a trooper. He began the Fontan this morning at nine AM. Five hours later, Dr Chai comes out to tell us James's surgery went well. Six hours later, the surgical team finishes up, getting him ready for the move to the PICU. Seven hours later, docs in the PICU tell us James is sedated heavily, sleeping peaceful in the PICU, while they put in lines of various fluids and meds to stabilize and hydrate him for the recovery ahead.

Eight hours in, they tell us he's still not stable and they're working hard to get him there. We haven't seen him yet as they try to get him to a happier place - "hopefully soon" has been the mantra for the last few hours. 

And James has a hearty recovery in store, probably won't be lightened off sedation or woken up until he's more stable, most likely will be here a week or so as his body adapts to the new plumbing, but they plan to make the first big step and take out his breathing tube by tomorrow evening, possibly as early as tonight.




Meanwhile, mommy and daddy wouldn't mind a walk and a nap :)


PS Weill Cornell, mommies and daddies might also not mind if non-medical folks here decided to treat parents who are here with and for their children less like criminals and vagabonds hitching along for the ride, but that's for another post another day...