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!