Smooth Sailing

Back for a cardiac catheterization Thursday. 

The short version: 

We’re home, safe and sound, with a decent bill of health from the cardio team, and James is grumpy because he can’t run and play in the snow and has to lie around watching movies all day 🤪

The longer version:

We’ve been able to hold off from procedures for over 2 years, which has been a nice relief, but it was time to do a little poking around to see how James's body has adapted to the Fontan over the last 2.5 years: a fluid test, a nitrous test, and some closer looks at collaterals and valves. Pressures haven't changed, which is good, and means the pulmonary hypertension drugs are working; fluid dynamics show he's not the best at managing fluids, but no interventions needed this time; and they coiled up one more collateral to mitigate future pressure issues. Overall, they tell us James’s Fontan looks clean and about as normal as it did when they did the last cath; so it’s good to know he’s growing into this well, with one Glenn arterial valve pocket to keep an eye on for clotting risks as he continues to grow. So, for the foreseeable future we’ll just be doing regular checkups (EKGs and BP and O2 sats) and imaging (ultrasounds and X-rays) to monitor the usual trouble spots. 

And, as always, it’s not the procedure but the post-op that's the difficult part. James hates being still, and a cardiac cath post-op requires a 6 hour session of laying pretty much absolutely still so the femoral artery and vein can close back up. Of course, I don’t know anyone what would be ok laying still for 6 hours straight, and the nurses totally understand, but it means multiple versions of sedation and extended naps, to the point that docs and nurses get a little flustered, extra docs and meds are called in to help, and we almost ended up staying overnight. Grumpier and grumpier. But, we got home late Thursday, with our grumpy 7 year old, and all is well with the world 😁

As an added bonus, we’ve been given the green light to fly! Now we get to debate whether it’s Florida or Seattle first.

Thanks for all of your happy thoughts!

Pilgrims' Progress

Happy Friday everyone!

An update is well past due.

First and foremost, we've moved to Pittsburgh! This explains, somewhat, our lack of posts in the last year or so. Moving is busy, and hard, and busy.

Kirsten and I have brought the kiddos to Steel City, City of Bridges, turned them into tasty Burghers, made Stihllers out of them, and, so far, they are loving it! Kiddos love their new schools and friends, they thoroughly enjoy having a yard to play in, and they are growing almost too fast for our tiny house to handle. 

Kirsten loves being back in her old hometown (she's had many, but this is her favorite), has an insatiable appetite for home improvement ideas, and, most days, loves being back at work and working on developing her career.  

And, though I truly miss my life and career in NYC, I am loving the change of pace in this new city, the challenges my new career is bringing me, and I couldn't be happier about my new Job Title(s): Work-On- and -At-Home Dad. And I even get to begin creating a never before seen title: writer-in-residence-amidst-renovations-progress! Get it? Get it?! Ha! Ba-doom! :) It's a cheesy title in progress, and I'll be sure to update this as I move forward and grow into this title. 

Anyway, now for some medical updates. 

As those of you who have been following James's progress over the last few years know, his pleural effusions have been his biggest challenge and difficulty. After his Fontan procedure when he was 4 to finish up the single-ventricle open-heart surgery barrage, to go along with everything else he was given, he was granted the sad diagnosis of chylothorax, a form of pleural effusions caused by and associated with some single-ventricle surgeries. 

Brief recap lesson: Chylothorax is a pleural effusion where lymph fluid drains into the lung cavity due to high pulmonary blood pressure. Essentially, though the Fontan was supposed to help route blood to his crazy heart-lung system, it increased the pressure so much that it caused his lymph system to go haywire and start dumping fluid into the envelope around his lungs...

They tried chest tubes to drain it, which didn't work, diuretics to dry it out, which only helped a teeny bit, and put us on PDE5 inhibitors (aka Viagra) to reduce the pulmonary pressure, which seemed to work, but only with diuretics. That was 3 years ago. 

Since then, we've seen a lymph specialist to talk about a lymphangiectomy, switched from viagra to cialis to simplify his medical schedule (we were giving him meds 5x a day, from 6 am to 11pm... its a wonder he didn't develop a sleeping disorder from that!), and tried on 3 separate occasions to reduce the amount of diuretics James takes to help reduce his medical regimen and any negative side effects, like PLE, liver and kidney failure, and basic dehydration.

To compound it all, our move has brought us into UPMC and Highmark's feud over inappropriate capitalistic dominance in the healthcare industry in this region, which has made us have to be waaaaay too aggressive in our advocacy stance for simple life-saving care... Healthcare shouldn't have to be this complicated. But that's for another post...

Today's medical news: We seem to have successfully weaned James off diuretics! He went in for some follow up imaging yesterday and his X-ray is clear! We go back in late August for another round of imaging to confirm, and possibly schedule another cardiac catheterization to evaluate pressures while on the PDE5 inhibitors, but I'm not a fan of another procedure, and neither is mommy, so we'll see... Either way, no more waking James up at 6 am and 11 pm for meds, no more diuretics that make for too frequent bathroom visits for our little guy, we get to hold off on the lymphangiectomy for another season, and heart failure is now no longer an imminent threat!

To celebrate, Make-A-Wish is also granting James his wish soon, maybe even on or by his birthday! He really wants a playset, and has been asking for years, so now that we have a yard, and the yard is more and more ready to accommodate his wish (long story, which I'll save for another post, but briefly: for plumbing and necessary home renovations, we've had to do some massive amounts of landscaping to get to this point). Make-A-Wish has scheduled delivery of the playset parts, and now we're just scrambling to get the earth level and ready for construction. Here's hoping we get this thing up before James' birthday!  

I'll add some before and after pics of the yard and playset to either this post or another post when I have time. Meanwhile, here are today's morning magical pics from about when the story behind this post began and today 😊

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.