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 😊