First Steps

Almost two years ago, we watched with joy and amazement as James took his first steps - at the park and into Daddy's arms.

I had that same experience again yesterday. James has gotten so much stronger this week, and is such a trooper about pushing himself. He cries throughout each standing session, and his little legs shake and buckle beneath his weight. Yet as soon as he sits, I ask if he wants to try again, and he says YES. Yesterday he crossed a hurdle: he stood entirely on his own, only lightly holding my arm for balance, and straightened his legs. Legs that went unused for almost 2 weeks supported his entire weight for over 10 seconds.

Just imagine, if you can, lying sedated with your legs bent for so long, with barely any food, then trying to unfold them and stand up. It was an awesome moment to see him straighten up, feel him let go of my arm, and stand on his own.

He tried to take a step, and even though he needed help, he initiated the motion all on his own. He has a "this is hard, but let's go for it" attitude. He says he wants to walk "to the car". One mountain conquered, and the next already underway :)

His pleural effusions are still leaking pretty consistently, though yesterday showed some improvement. Doctors have emphasized that this is a long, slow process, but James' heart is healing wonderfully so they are no longer worried... He will eventually kick this too, and we will go home.

Building Endurance

At every surgery, James has some setbacks.

After the first surgery, the day he was born, he had to gain back the one-third of his weight he dropped during surgery, he had to learn to eat because, unlike normal babies, he never was given a chance to breastfeed when he arrived, and he had to learn to breathe on his own again.

After the second surgery, again we had some of the same issues as above, mostly about eating, having to put him on a naso-gastric tube to ensure he'd get enough calories, but also cognitive, psychological, and physical development. James needed to learn to be an infant: talking, walking, playing are foreign things, and his second surgery set him back to just about newborn status.

No small feats to overcome, these setbacks, even for healthy babies.

This time around, James awoke from sedation hungry, but silent, and immobile. He wanted to eat, he wanted to drink, like a newborn, but he couldn't (or wouldn't) speak, and he could barely lift his arms.

In the last week, James has begun to come back. He is talking more, playing more, moving more, and still very, very hungry. Still no classic smiles from James, but he is showing levels of determination, perseverance, and grit that I don't know I have ever seen in grown-ups. He has been, and continues to be, one of my greatest heroes.

We still have a long way to go medically before James can come home -- weeks, possibly months, before he's stable enough to take his pleural drainage tubes out -- but he has already come back to us in spirit, and I am positive he will continue to amaze us all as the days continue to fly by.

Split family

Elise hit the 6 month mark on July 14, the same day James had his Fontan. This milestone got lost amidst the activity, but not forgotten: I took a series of photos of her the day before to commemorate her little 6 month old size and personality.

Given the extended hospital stay and uncertain timeline, we finally decided to send our baby to stay with Grandma and family for awhile. She's going to have a great time, but we miss our little ladybug, and hate that we have to be split apart right now.

So tonight I'm looking at photos of my smiling, giggling, crawling, jumping, teething, chewing, grabbing, arm-waving, growing girl.

Weekend Wrap Up

We had a long and determined conversation with our doctors on Friday about leaving James alone. Despite all their talk about how chylothorax takes time and he needs rest, the ICU is not restful and the constant regimen changes and status checks and blood work were wearing him out. People were in and out of his room nonstop, and he was terrified, and his blood pressure and heart rate and other stats were unable to stabilize.

We said: STOP, remember he is 3 years old, your bedside manner needs serious work, and quit scaring the daylights out of him!

So, he got a quiet, restful weekend. His team is being quiet around him and limiting interactions. He got some calming medication to help him adjust. He needs to have the energy and drive to fight this, and he's getting that back. He spoke a ton today! Thomas, bye bye, grandpa, funny, water, marker, yes, bumblebee, yellow, and more. He read The Very Quiet Cricket and said his favorite part, "not a sound". He had several visitors and took another wagon ride and sat up for a long time.

No walking yet, but his left leg has been very swollen so the IV came out today (there's a new one in his hand, but you win some, you lose some). Hopefully that will let his leg improve enough to try some standing tomorrow. Walking later this week?

This condition can take weeks to heal so we're hoping for sooner rather than later... and praying for no further complications.

Deep Breath

James had a second chest tube inserted last night (Thursday) to drain his left side. Over a liter came out, most of it almost immediately. His chest X-rays looked much better today, which is great: all that fluid needed to come out. However, he's otherwise unchanged: still leaking at the same rate, stats all still the same, oxygen has been hovering around 90-92 (when it should be more like 98). After a day of reintroducing food, he's back to a no-food diet with TPN feedings, and they have been discussing reinserting a central line. The more they poke at him, the more stressed he gets, but stress increases the leakage and directly contributes to this condition. It could be a week, or several weeks, or even longer of hospitalization... it could be months of return visits... it could mean other things that we don't fully understand yet.

This has been confusing, and scary, and I haven't processed it all yet so I'm not going to write anything else about it right now.

Instead, I'll share that James is getting stronger about sitting! He had another chair-and-Ipad session today. He took a ride in a wagon and looked completely adorable. He practiced standing. He still didn't really manage it, but he put weight on his feet for a few whole seconds. He has new headphones, and they seem to help him relax and tune out all the doctor babble over his bedside :)

The Irony of Eating

A couple of days ago, James was diagnosed with chylothorax, a life-threatening complication from his Fontan procedure, where fat and lymphatic fluid from the lymph system drains into the lung cavity, making it difficult and eventually impossible to breathe. They discovered this from the fluid he's been draining from his right pleural cavity. And when they found out, docs were scrambling around all day, arguing about best courses and steps to take, and all the while they kept James awake and annoyed, further annoying him by trying to find new places to put IV lines.

Apparently, the only way Docs could agree on to cure his complication was to take him off food and water (i.e., starve James) and put him on IV nutrition for the next 24-28 hours, then transition him to a low-fat diet. It's a bit ironic and silly that, before the surgery, James was on a high-calorie, high-fat diet, doctors said, to help his body be better prepared for recovery, and now that he is recovering, the thing his body needs is the exact opposite.


So, they've had him on IV nutrition for about 36 hours, and today, his pleural fluid is looking clearer, which is good, but it still has a high lymph count and the quantity hasn't lowered, which aren't good. He also is showing increased fluid in his left side, which is also not good. This means James might have another procedure tomorrow, inserting another chest tube in his left side, so both sides can drain freely.

Medically, James is staying the same: critical, but stable, with a long way to go before he can come home.

Psychologically, James is coming back! This morning, he sat up in a chair, played with his iPad and stickers, drew with crayons, and said some words. He's much less guarded about people coming into the room, and he's occasionally smiling, even giving some of his nurses and doctors high-fives.  

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...

Fontan Is A Go

James, mommy, and I arrived at Weill Cornell very early this morning for James' Fontan procedure.

I'm not sure mommy and daddy are ready, but James is ready, Dr Chai is ready, and all the folks here at the hospital are super ready for James.

Mommy looks great, cuddling with James:

Daddy has his late-night sleepless-eye bags on:

And James has got his too-cool-for-school tiger scrubs on:

We'll keep you all posted on progress. Send your happy thoughts, hugs, and prayers c/o James :)

Waiting for surgery...

In case you missed the update a few weeks ago, James' surgery got bumped to next week. The new date is July 14.

In the meantime, we're enjoying some family time at home, taking James to the park, getting his new room set up, and having lots of cuddles. He is reading books a lot and singing to himself frequently. He loves to snuggle with his blanket on his very own couch.

Elise is about to hit her own mini milestone: she'll be 6 months old on the same day that James heads to the hospital.

3 years old!

James turned 3 last week, and we had a small celebration with ice cream cake and gifts. The cake was for our enjoyment, of course, because he doesn't like cake or ice cream. He got some great gifts though, which he already is tearing into and mastering new skills:

- Spelling: he got a set of books with an electronic read-along pad that has prerecorded words and spelling activities. By the next day he had already figured out the spelling portion, and not just learned but memorized several words. He can spell sun, moon, art, baby, dog, cat, and cow without even a prompt now!

- Music: Between his new piano and his triangle (plus other instruments, but those are the favorites) his room has been emanating a constant cacophony of noise. He loves it. Fortunately Elise is a deep sleeper.

- Books: The flip flop flaps and alliterative silly sounds of his new pop-up book have him tackling some tough pronunciation like a champ!

- Stickers: These have already been plastered all over his face.

- Food: This is more of a birthday gift to us, but he's been enjoying cocoa puffs and spaghetti lately, even to the point that he will walk to his chair, sit down, and request them. Quantities are small, but requesting food is always a big moment for this kid.

- Baby toys: wait, oops, these aren't his. But the new apartment means they're out on display for Elise, so he is thrilled to rediscover old loves.

Baby nostalgia aside, reading and spelling are fun activities for him lately, and he chooses those all on his own so we are thrilled he is so enamored with learning to read. And thank you to everyone who helped him celebrate!

Some video of James lately: