Wednesday, April 3, 2013

Blood is Thicker than Water

...and MY blood, apparently, is thicker than it should be.

So I visited my doctor yesterday for a quick clinic appointment, only instead of the normal chest x-ray/bloodwork/PFT routine this visit started with a CT scan, complete with IV contrast. And for those of you who need the cheat sheet version of why this additional test was necessary, let me quickly bring everyone up to speed:

In late January I visited clinic only to find that my PFTs had taken a rather severe plunge from where they'd been in December after all my photopheresis/rejection and port-placement "fun." The drop was concerning enough that my doctor ordered a bronch, which showed no acute rejection but did come back positive for a virus that can cause pneumonia. He also ordered a regular old CT scan (no contrast) and that showed some diffuse patches of what appeared to be infection. The end result of all this was IVs, rest, and waiting, with routine follow-ups scheduled to monitor my PFTs.

Since then, my PFTs have been falling. I'd say that it's been slow and steady, only it hasn't been slow; it's been terrifyingly fast. Since the start of the year I have lost well over 30% of my lung function. The weird thing, though, is that this hasn't presented like typical chronic rejection (or BOS). What I mean by that is the numbers are following a strange pattern, and the CT scans and other tests we've run haven't indicated that this is traditional BOS. And yet my lung function has been clearly falling, so much so that my doctor suggested we might want to consider an open-lung surgical biopsy -- and that I begin the process of updating my testing should I continue my free-fall right up to the point of retransplant.

Um, yeah. Scary.

So this past Tuesday I had my CT with contrast and then I headed up to the PFT lab, where I blew my little lungs out only to come up with numbers that were pretty similar to my last visit. I was trying to console myself with the fact that at least they hadn't gone down drastically, but to be honest, I was in a bad space. More to the point, I was super confused. I felt very strongly, in my heart of hearts, that this wasn't supposed to be the end of my journey with my Delightful Donor Bob. And, you know, I was scared. And sad. And, well, a little pissed off, to tell you the truth.

And then imagine my delight when my doctor called me into his exam room and told me that this latest CT had found -- drumroll please -- blood clots. In my lungs. And some dead lung tissue up behind the clotted area that is most likely what we were seeing on the original CT scans. And my heart, that little clotting trickster that it is, literally leapt for joy.

Only in CF-land could blood clots in the lungs be considered good news, right?

Blood clots are treatable, even if there may be some form of permanent damage from the clots. Blood clots are a known enemy -- which means we know what weapons we have to use against them and we know what strategies generally work to get things back on track. Blood clots do not require open-lung biopsies or testing, both of which were cancelled before I skipped out the door from clinic. Most importantly, though: blood clots are not chronic rejection. My lungs are not failing, nor is there any reason at this point to believe that they will.

Honestly, I need to take a second to let that one sink in.

I woke up this morning still clotted, but feeling infinitely more free. It's appropriate that just the other day I wrote about my gratitude for my donor, because this morning it was reaffirmed to me 1,000 fold. I have so much living left to do, and it looks like these lungs are going to have to stick around and come with me while I do it. For that, I know that I am beyond blessed.

So there you have it, beautiful people: all the (lung) news that's fit to print. And while I still have some tests to get through and some treatment to sort out before I can call this latest chapter "over", I can also delight in the knowledge that there are many more chapters to be written.

And I hope you'll stick around to share them.


  1. This I was totally freaking out but such scary good news. I am so happy they found it and I Hope they can get everything cleared up super soon.

  2. This brought me to happy tears. Many many more chapters to be written.... and it will be a pleasure to continue to read them.

  3. Happy happy day! (Never thought I'd say that about blood clots!) Go get 'em, Piper. You are a true beauty inside and out. Praying for many more "chapters" in your story yet to come!

  4. Your amazing Piper. Your commitment to CF and the way you never give up is inspiring to me. I can get down sometimes about CF and all the things I can't do anymore and your blog always makes me pick myself up. As long as we are alive there is still things to do and be happy for. I'm still a long way from lung transplant but when I need one I hope you will still be here with this blog to cheer me on.

  5. Dearest P, SOOOOOOOO Happy and relieved with this news!! Lit candles for you in Vancouver Cathedral, not only for chapters, but for volumes to come!! xoxox, Your Godmother

  6. Heidi Karn BarkerApril 5, 2013 at 3:11 PM

    Ugh, chronic rejection - these are words which, as a fellow transplantee, bring the wrong kinds of butterflies to my stomach. So happy this is not the case and that the clots are treatable. Sending good thoughts your way!