Philly bound

August 22: Philadelphia

CHOP, here we come! James has an appointment on the 22nd for...?? We don't have many details yet, but when do we ever...? We know what CHOP (Children's Hospital of Philadelphia) can offer, so they'll assess him and admit/test/operate based on his condition at that time.

Until then, he is having a wonderful time at home with his sister. Since she had been staying with grandma, they weren't actually reunited until a few days ago. Lots of giggles are finally filling the apartment again, as James becomes more at ease and less guarded by day. We had two doctor visits already this week, and his effusion has not noticeably progressed. CHOP clearly thought this wasn't urgent, since they didn't schedule him immediately, and this made our local docs nervous - but "not urgent" is a-ok by us.

First follow up done!

It's been a wonderful yet rocky few days. We're thrilled to have James home, but he is clearly affected by his summer ordeal. He wandered around somewhat in shock on his first night, and continued to be stiff and scared yesterday. It took 24 hours to convince him to leave the apartment again (to go to the playground) and he's been having nightmares.

His doctors wanted him back by Wednesday to follow up. It was not easy to get him back to in the hospital today. He managed like a champion, but the emotional toll is significant.

Unfortunately, today's follow up was not encouraging. The effusion has continued to progress. We are waiting for CHOP/Philadelphia to give us an appointment once they review today's test results, but we expect they'll want to see and admit him sometime next week. 

He's now having fun with his evening to start to make up for the challenging day!

Homeward bound

We're not done, but we ain't staying here!

James' tube hasn't had output in over a week. His X-rays and ultrasounds show that there is still fluid, and it's not all old - so the leak still exists. This problem of pleural effusions isn't resolved.

But, we've exhausted all the standard treatment options. We could wait longer to allow more fluid to drain, but the tube isn't even draining anyways... His effusion doesn't seem to be accessible by chest tube. There is no point in giving it more time.

The good news is: most of the fluid has been removed over the past month, and the leak appears to be a slow leak, so it doesn't seem to be critical at this time. We're taking James home to enjoy his summer!

The bad news is: this is just a temporary reprieve. As soon as the fluid builds up again (next month? next week? this week?), we'll be road tripping to Philadelphia to check into Children's Hospital of Philadelphia (CHOP). Their Center for Lymphatic Imaging and Interventions offers innovative imaging technology, thus allowing more precise surgical interventions, which we're hoping will be able to locate and more definitively close his leak.

Also, super reaffirming: CHOP, the only east coast hospital that does this, has performed this procedure on 50 children!! I mean, hey, it's not rare at all!! 😳😳😳

In the meantime, here's a photo of James taking his zebra pilot for a plane ride 😄

Absolutely Fabulous

New room decorations and a happy boy!

Mommy's Back! or, Memoirs in Odd Progress

July 27, 2016

 Day 29, this time around...

.    .    .

Mommy's back! She came into town late last night and got to the hospital early this morning, about 6am, where she found James had already been awake since 4am playing iPad videos. He’s truly addicted now: #techaddiction…

Of course, getting up at 4am is tiring, so Mommy and James were both soon again asleep, and woke later in the morning for breakfast, rounds, and today’s activities.

Mommy and James had a nice morning walk to the lab in another wing of the hospital for today’s X-ray -- usually they just wheel a mobile X-ray device into the room, but today they wanted both standing frontal and standing side-view images to better visualize the problem -- and then they headed to the playroom for train and castle and kitchen and toy animal playtime.

Meanwhile, the question everyone wants answered is: is the condition getting better or not?

This is the odd progress part.

Since we’ve been here this time around, James’ chylous effusions got better, then worse, then better again, then worse again, and since the beginning and meanwhile what was there and what was leftover is/was what is called loculated, or congealed in layers, like a disgusting booger or pus cake inside the lung cavity, which is more common in cancers and pneumonia and other like infections (empyema is a term they’ve used to compare it to, but this isn't that), then that started clearing up, then wasn’t clearing up enough or fast enough, then it was, then we were waiting for CHOP to say something, then we weren’t, then we were, then…

Yeah, like that. Odd.

Just like last time, I think it’s important for folks to remember that, because there is so little known about this, the easiest way to describe this is to think about this as something like a cancer that has come back from remission, and now it’s just playing with us.

Unlike most cancers, however, the long-term solution here isn’t just about a fix to the effusion (like plugging a leak). The long-term solution requires we figure out why it recurred in the first place, so we can prevent it from coming back again. This is, after all, the same complication from last summer, so we have to figure out why it’s back.

There are dozens of doctors here at NYP, and more at CHOP, all working hard to fix the leak, prevent another future leak, and get James home, but still not many answers...

So, while there’s not much to report in simple terms beyond smiles and reunions, and even less to report in terms of James’ odd progress (did Kirsten or I mention yet that James has never yet “seemed” unhealthy to anyone? This life-threatening issue is purely an internal one), sometimes the smiles, the reunions, and the odd progress reports are progress enough.

Thanks again to our families and friends who have supported us best: by visiting and sending happy thoughts, prayers, and well wishes; by bringing and sending foodstuffs and cards and colorful things to plaster on these drab hospital walls; and by letting mommy and daddy decide how best to organize the crazy logistics needed while our family deals with this unexpected oddness.

Cutie

I

Another week begins

Day 20 update:

Nothing to report.

The effusions continue at the same rate. The tubes have been clogging so he had his chest tube replaced TWICE in the past 3 days. The fluid leaking now is red with blood because his body hasn't had time to heal. He is sore, cranky, and quite visibly sad.

The standard treatments haven't worked so we don't know what comes next: consulting today with other hospitals to come up with an idea.

No news is... news?

James is back in his room and resting. Ideally, we wanted to find a cause for his pleural effusions so we can know how to stop them. Today's cath did nothing -- ok, almost nothing -- to provide those answers.

It did rule one thing out: the pressures in his Fontan system are not out of whack. 

Given that the leaking increased in recent days, everyone expected to find that the Fontan was failing: we expected the refenestration to be inevitable.

Instead, his Fontan looks stable! 

(Current hypothetical speculation is now focused on pulmonary vasculature, pending analysis of mPAP, PVR, CO, SVR, RAP, etc...). 

And that's the news... 

Enough? 

How would you react and respond:

Realistic: No news means we haven't found anything news worthy; check back tomorrow...

Zen: There is no news; there is only what you make of it. 

Optimistic: No news is good news.

Pessimistic: No news? Seriously?

Relativist: No news is all the news there is, so it must be good enough.

Scientist: No news means our data is insufficient and our hypothesis has lacked clarity and focus; more lab testing needed!

Parentist: No news means no answers, but no bad news either, which is good, and we've avoided a big, drastic intervention for the time being, which is also good, so now we sleep this off and see about getting James to the hospital playroom and FaceTime with the family tomorrow 😘

133

133 days in the ICU, throughout his 4 years of life, so far...

This week has taken an unexpected turn. The effusions, minor when we arrived 2 weeks ago and basically gone last week, are back. Even though he's still in the ICU, still using all the easier treatment methods available - they are simply not working. The leak is back and it's gotten worse.

Thursday's plan is still the same.

But we are bummed... because this means the chance that Thursday's catheterization will be minor has just plummeted. He'll probably need the refenestration that we hoped to avoid. The side effects are severe, the medications to manage it are nasty, and it still may not be enough.

If this surgery doesn't fix the problem, next steps are even worse. There's no good option, only less-bad-than-doing-nothing options, with higher and higher costs to the rest of his body.

Mostly we just want our boy to be happy, and know he's loved, and enjoy his life for as long as possible, and not spend it stuck in a hospital bed. 

So we're frustrated, and sad, and lost, and angry.

And no, we don't want to answer questions. We do want lots of support for James as he hopefully gets through this latest bout with as minimal intervention, hospital time, and strain to his body as possible.

July 14 is the worst date

It's been a confusing week here in the PICU, because there's so much happening yet nothing is happening.

James' pleural effusions have pretty much stopped. He is already being weaned from the medications that helped dry up the leaking, and moving towards possible discharge this weekend if everything stays dry as he transitions off meds and back onto solid food.


But...
It's not as simple as "now it's here/now it's done". The pleural effusions last summer were a sign that his body wasn't yet adapted to the Fontan circulation which was the entire point of the surgery. Their return this summer means that his body STILL isn't working optimally with the Fontan system. The pleural effusions will be a recurring problem unless we make a change.

His team has therefore scheduled him for a cardiac catheterization next Thursday. Even if he is discharged before then, he'll be readmitted to the hospital on July 14. (Exactly one year from his initial Fontan! July 14 is officially my least favorite date.)

Best case scenario, they decide during the cath that everything looks good enough to simply try a lowfat diet as sole lifestyle change to keep the effusions from returning.

Any other case - really the only other option if things aren't totally great inside - is a refenestration of his Fontan. This means poking a hole, adding a shunt, opening a permanent connection; decreased oxygen circulation; and some nasty lifelong medications involving super sucky stuff like daily shots for the rest of his life.

We don't want this. James doesn't want this. Let's hope we can avoid it!

Super excited about Legos ❤️

What a view

A quick thunderstorm outside brought us this view from James' hospital bed 😊

Also, the results are in: the fluid is chylous pleural effusions again. His chylothorax is back to claim another summer. Hopefully it's a shorter fight to recover this time!

Didn't want to do this again

Yet here we are.

James had his regular checkup yesterday, and the echo showed some fluid accumulation. An X-ray and a sonogram later, we were sent straight to the Picu to check in and prepare for a chest tube.

He spent a reasonably peaceful night, unhappy to be here but adapting probably better than most 4 year olds would. Diuretics throughout the night were meant to see if a tube could be avoided, but they didn't reduce the fluid, so a chest tube goes in today.

We still don't know how much is leaking, from where, or what the fluid is. More testing today before we get answers about what we're dealing with here.

Not much changes in the Picu in a year:  all the same faces have been greeting him back.

How far they've come

How much these two have grown!

So long ago

That ICU stay that seemed to last forever now feels distant...hard to believe how quickly we move on! But amazing also to see the changes in those few months. We went from anticipating the Fontan, to wondering whether this nightmare would ever end, to finally realizing that this is all in the past.

CHD awareness week comes around in February each year, and this year we'll post new photos of our guy with several more scars than he had a year ago.

One little update: he wore a heart monitor in December to assess whether we could stop his digoxin medication, used to control and suppress the atrial flutter that showed up after surgery last August. Results were normal but not conclusive enough so the digoxin continues for now.

Wintertime

A little photo update of these kiddos in the snow and cold! They aren't a fan of the snow so much but they both love to be outdoors.

And siblings sharing nicely :)