Monday, May 30, 2011

The Challenge of a LIFETIME

Some things in life just don't come along often.

For example, it is extremely rare for me to start off a thought, sentence, or blog post with the sentiment, "One of the reasons that I'm happy I have CF is . . ." That's not to say that there aren't certain elements of having this disease -- and sharing this experience -- that I feel grateful for, but I am gonna go ahead and admit that I am not, in any real sense of the word, all that grateful to have been born with a fatal genetic disease. But, like any rule, there are exceptions, and this is one of them.

One of the reasons that I am happy I have CF is, I get to meet some awesome, incredible, inspiring, amazing, and downright fabulous people. People, for example, like all of you.

Or people like Mike Freemantle, whom I had the total honor of meeting a couple of weeks ago. And though Mike doesn't have cystic fibrosis himself, I still have to give credit where credit is due to CF for this meeting, because Mike is the kind of guy who . . . well, let's just say that Mike is a man of extreme energy. It's the kind of energy that leads people to push themselves, to challenge limitations, and to go beyond their own experience. In short, it's the kind of energy that connects everyone who has ever pressed against the outer realm of the extraordinary -- whether by illness or by choice or by anything in between.

Oh, yeah, and it's also the kind of energy that might lead someone to look at an ordinary road bike and think, "Hey, I think I'll hop on that sucker and take a leisurely 21 day coast-to-coast tour through 13 different states. And what the heck, maybe I'll raise about $50,000 for the Greater NY Chapter of the Cystic Fibrosis Foundation while I'm at it in honor of my friend (and fellow amazing rockstar) John. You know, just for kicks."


All of which leads us to yet another one of those amazing events that just flat-out doesn't come along often enough. Because this morning at the ridiculous hour of 6 AM, Mike and many of his supporters stood in the even more ridiculous sudden rain shower that swept across Times Square getting ready to launch what must have initially seemed to many people like the most ridiculous thing of all. One man, one bike, one big RV with the Great Strides/CFF logo proudly displayed, and more than a thousand miles between Mike and his final destination. But anyone who knows CF knows that the seemingly impossible is sometimes the most important, non-ridiculous goal of all. And anyone who knows Mike knows that the same is true about him. Because believe it or not he's done it before, and now he's decided to do it again.

Only this time, it's for all of us.

Below are a few pictures of the magic behind the madness (and the man behind the magic). Please, please, please check them out, then go to to join and support Mike on his cross-country bike ride for a cure. He'll be keeping track of his experiences, his route, his pictures (the man has a camera strapped to his handlebars, people!) and, of course, his fundraising.

Because things like this just don't come along often -- and when they do we should all be so lucky as to have the chance to go along for the ride.

Only 13 states -- because he's an underachiever

Flier used to raise awareness of the cause

The one and only FREEMATOUR BUS in Times Square

Gosh, that's a handsome . . . logo. Um, yeah. The logo steals the show.

1 Bike, 1 Bus, 1 Rainy Horizon

"What, it's so wet that even Times Square is completely empty? Whatever. I've seen worse."

Start Your Engines

One small pedal push for Mike. One awesome push for CF awareness

Mike, you've got the wind of 30,000 beautiful people at your back, dude. Best of luck, safe travels, and, above all, thank you. For the chance -- and the challenge -- of a lifetime.

Sunday, May 22, 2011

Because of You

Dear Cystic Fibrosis:

I know it's been a while since I've written you, though I honestly can't say I'm sorry. It's true that you are not my favorite penpal, but in my defense, you've been kind of busy -- that whole "wreaking havoc on innocent, beautiful lives" day job of yours seems to take up a lot of time, plus I have yet to see you take a vacation. It must be rough knowing that so much is riding on your every move, that you literally hold the health and happiness of over 30,000 people just in the US alone in your hands. I'm not sure I would want that kind of pressure, to be perfectly honest, though I like to imagine that if I did have such power I would use it for good rather than evil. But hey, to each his own, right?

Anyway, I thought it was probably about time for you and me to get back in touch. In part this is because I think it's only fair to let you know that you are, once again, being a very royal pain in my butt. Not that you ever stop, obviously, but it feels like sometimes -- every once in a very blue moon or so -- you seem to back off just enough to give me a false sense of security. You lie low for a few days and I feel better, and my friends seem healthier, and no one is dying or sick or stuck in the hospital, and for just a few brief moments I remember that there was a time when I thought you maybe didn't matter quite so much. That maybe, if I kept my eyes shut and wished hard enough for long enough and with enough faith in the power of miracles, that you would just disappear. And yes, I feel weird writing that, even to you. It seems strange to have to tell someone (or something, rather) that you hate them. But I do, CF. I really, really, really do.

I hate you for what you do to my friends, for the lives you've taken, for the joy you seem to take in hurting families, worrying loving parents, leaving behind dedicated spouses, and doling out way more pain than any young child should ever really have to endure. I get it; I understand that you are part of life and that, like anything, you have your beauty. I see how strong you make people like my friends, who are facing sickness or transplants or second transplants or new additions to their families when they themselves can't breathe. I see the light that you spark in them and it is almost enough to make me think you have something to offer -- but then I remember that each of these people are strong DESPITE you, not because of you. And then I hate you all over again, and I realize that I simply cannot wait until the day when their collective light is allowed to burn even brighter without your darkness. Because let me tell you dude, it is going to be one hell of a sparkle show when that moment finally happens.

Which brings me to my second point, CF:

You don't stand a chance.

Nope, sorry, not kidding. I absolutely mean it. And as proof I've spent the last couple of weekends at events that have raised literally hundreds of thousands of dollars to eradicate your presence. I've stood next to friends and family members who have lost a loved one and who are still so dedicated to kicking your butt. I've watched in awe as one small child took her place in the center of a huge group photograph -- her future as bright as the smiles on her team members' faces. I've had the chance to be personally inspired by Mike Freemantle, who is literally riding his bike across the country to raise money for the Greater NY Chapter of the Cystic Fibrosis Foundation. I've seen people walk in the rain, people walking with strollers, people dressed in costume, and people coming together in mutual love, hope, and celebration.

And all of this, CF, is not because of you at all. It is despite you. It is not because you make us sick, but that we are strong despite your efforts. It is not because you rip apart our community, but the way we rally despite these great losses. It is not because we have no faith, but that we are hopeful despite your nasty tricks. And it is not because you weaken our spirit. It is that we will always be the bigger, faster, and more determined team -- despite it all.

So I don't mean to add to your workload, little monster, but if I were you I would consider taking up running -- and pretty fast too. Because if the heart, soul, and dedication that I've seen these past few weeks alone is any indication, you're in way over your head with this one.

And unlike the rest of us, CF, you are running alone.


Tuesday, May 17, 2011

Our Little Monster

Got my biopsy results today.

I have what my transplant clinic described as "minor inflammation," which could be caused either by "true" rejection or just kind of brought on by the other part of the news, which is that my goo culture from the other day grew out our old friend pseudomonas. Talk about a party crasher. Anyway, the combined effects of these results is more IVs (polymyxin and aztreonam) for 3 weeks and a 2 week taper of prednisone until I get back to my maintenance dose of 10mg.

Luckily, the pseudo is just hanging out in my airway -- a sign that once again this is all about the sinuses and much less so about the lungs. Unfortunately, the added rejection/inflammation (it's been labeled as A1 rejection) means that I have to postpone my sinus surgery for 4-6 weeks to give my system some time to reset itself and avoid extre infection risk. And I also have to get another bronch in 3 weeks, which obviously isn't the best news in the entire world, though honestly they feel like old hat now. I think a part of me feels weird whenever I'm not on versed after the past year.

My general sense right now is that things, quite honestly, could be worse. Not sure if that's just because I have so many friends who actually are doing worse than I am, or whether it has more to do with the fact that actually facing up to the past year would involve going somewhere I just don't want to go right now. I think it's the former. Weirdly, the one emotion I don't feel right now is fear. I feel anxious to get to the other side of this bridge, impatient, frustrated, hungry (thanks, prednisone!), kinda whiny, and a little overwhelmed. But I don't feel scared. Somehow or another the last couple of years of living in constant infection have coupled with 29 1/2 years of life with cystic fibrosis to make me much more prone to sarcasm and humor in the face of illness than tears or nervous worrying. And I still haven't figured out if this is a good thing (because it keeps me sane and productive) or a bad thing (because it inhibits me from taking things seriously), but I can tell you without question that it is, for better or for worse, a Piper thing. I have the feeling it might be kinda rampant in the CF community in general, to tell you the truth.

At any rate I'm still at home vs. the hospital, I'm still confident that my doctor knows what he's doing, and I'm still at least somewhat on this side of the whole "not crazy" spectrum -- though that last one might well change after a couple days on high dose steroids, believe me. And for all of those things I remain, for now and for always, extremely grateful.

For all those other things, though, I'm not grateful at all, which is why I wrote a simple (and uncharacteristically "R" rated for my normal internet chatter) status update on Facebook this afternoon:

dear CF: fuck off, you little monster. thanks.

That one little sentiment got more "likes" more quickly than anything else I've posted on there. Ever. Which means that to anyone who has ever wanted to scream, cry, kick CF where the sun don't shine, and then run away laughing: don't worry. You have no idea just how not alone in that you are.

Much love and happy hunting to you all, my beautiful fellow monster fighters.

Sunday, May 15, 2011

The News

In case you haven't noticed, there's been a lot going on around here lately. And believe me, it's fine if you really haven't noticed, because I've been a little absent and I realize that my last post was a little, um, cryptic? But the point is that whether it's been obvious on this blog or not, there has been (and continues to be) a heck of lot going on in My So-Called Cystic Life -- some of it good and some of it bad. So I thought I'd take a second to catch my beautiful breathheads up a bit on all the news in my life.

The bad news is that I remain on and off IVs every 2-4 weeks like clockwork, and have since my transplant almost a full year ago (before that I was just "on" though, so maybe this is better?). I'm always a little tormented about what (if anything) to say about that, but the simple fact is that I never promised this blog would be easy. I never promised it would be 100% positive. I never promised it would always be fun. What I did promise, however, is that it would be honest, so I figure I should just bite the bullet and type the truth. For the past few months my docs have been hardcore pushing a new drug cocktail on me, consisting of mostly polymyxin and aztreonam as opposed to my usual aztreonam/merrem/imi/cipro/cayston/levaquin/minocycline/whatever combo. It's been . . . um . . . well, let's just say that it's been interesting (and that it's involved enough walking into stationary objects to make The 3 Stooges extremely proud). It's also involved a couple of rounds of cefapime (neither of which lasted more than a couple of days before the drug was pulled due to allergies) and a whole host of other "fun" excitement from nausea to low WBC counts to weird pigmentation issues. (Seriously, if one more person asks where I got so "tan" they're going to get a squirt of polymyxin in the eye!) So, um, yeah . . . the past few months have been decidedly "not boring" from an infection standpoint -- although one has to wonder when the infection starts to just become "normal" and being healthy actually becomes the "weird and unsettling" part. I'm trying hard not to slip into the mindset, believe me.

The good news is, though, that while all of this has been pretty annoying, I really can say that it's been exactly that: annoying. As in, not life-threatening, not super scary, not mind-blowing, and not "oh my goodness get me out of here because I just don't know if I can take this anymore" style frustrating. Just annoying, plain and simple. My kidneys aren't failing due to the drugs, my lungs seem to be holding up just fine despite the onslaught, and my gut is . . . well, let's just acknowledge that they didn't give me a gut/pancreas transplant and leave it at that. (And to the CFers out there: don't pretend you don't all know exactly what I mean by that, by the way!) From a major transplant complication perspective, in fact, I really don't have much to report on here at all -- and for that I am beyond grateful.

The new news is that I did have a bronch last Friday, and after it was over I did in fact make it over to see my ENT in one of my hospital's outpost buildings way the heck across town. For those of you who may have already had the -- ahem -- pleasure of experiencing a bronch, you'll understand that going anywhere directly afterward defies all normal laws of logic, reason, and fentanyl. Nonetheless I was able to make it, thanks in no small part to my lovely sister, and while there I learned that my sinuses are pretty much completely blocked. As in, there was no black on my CT scan, in any of my sinuses. At all. So we went ahead and scheduled sinus surgery, which I'm really hoping can be done on an outpatient basis, and I think we're all hoping things get better after that.

As for the actual bronch, it was uneventful. Doctor found some slime in my upper airway (thanks again, sinuses!) and we're checking for rejection due to a very small dip in my more recent PFTs. The goo culture and the biopsy results should be in next week, but until then I'm choosing not to worry and to focus on things like CF awareness month and Great Strides instead. Not that I have anything against sinus slime, per se, but, well, it just doesn't make for a very pleasant weekend to dwell on that sort of stuff.

So that's the update -- 100% free from any crazy wordplay or other random piperisms. And I have to say that it really does feel amazing to write it out; to know that I have the sort of friends and community who is ready (and able) to receive this slimy, gooey, messy story of tangled IV tubing and annoying antibiotics and perpetual procedures without judgment and without shock or pity or total confusion; and, if i'm lucky, to maybe even earn the chance to let it go. And this should have been old news to me, I'm sure, because I don't know how many times you guys have taught me this lesson in the past, but old habits die hard and I think I will forever be the girl who would prefer to be cryptic than to be (almost) heartbreakingly honest.

Sorry guys, I had to do it.

So thank you, all of you, for teaching me honesty and for reading -- even when the going gets admittedly tough. I wish I had better words to say it. I wish that I had half the force as all of you together have. I wish that I could be as big of an inspiration for this community as it has been for me. And, above all, I wish you all lots and lots of news now and for a very long time into the future.

And all the friends you need to get you through it.

Monday, May 9, 2011


Well, here it is, beautiful readers. Spring has finally sprung in all its (almost) warm weather glory, the West Village is full of (almost) graduated seniors who are all-too-ready to revel in the (almost) finished school year, my shorkie is looking (almost) skinny in his new summer haircut, and I've (almost) hit another milestone in this crazy, winding, wonderful and (almost) mind-blowing journey that we call post transplant life. By which I mean, of course, that as of this week I am (almost) at my 11-month transplant anniversary.

And, oh! readers, what a difference an (almost) year makes!

Because (almost) 11 months ago today I was still trying to recover from an (almost) transplant (aka the infamous "bad dry run") and was spending most of my time just struggling to breathe on (almost) destroyed lungs. The remaining hours were a toss up between the sleep that was my (almost) favorite activity during that period of my life, the treatments that took up (almost) 6 hours of every day, and the desperate attempts I made every so often to keep up with something that was (almost) like the life I had known before CF got the best of me. And, when I got really lucky, there were even one or two moments out of every day when I could (almost) forget what was happening to my body, thanks to the amazing community of friends, family, and yes, even doctors who (almost) always knew (almost) just the right things to make me feel (almost) better.

Or, to put it another way, life back then was (almost) all about the uncertain: those all-consuming goals that seem to lie almost within our reach, the destination that is almost around the next bend, and that pesky arch of pure color in the sky that promises a pot of gold if we can make it to that spot just almost right ahead of us. And it was, in a strange sense, an (almost) perfect way to live -- if only for the fact that it forced me to keep my heart in the moment and my eyes on the constant "almost" that was always just a little bit beyond my own horizon

Oh, yeah, and it was also (almost) 11 months ago when I sat down and wrote this:

In all seriousness though, I'm sorry for being such a bad blogger. I never meant to be gone for almost a month. I promise it started off innocently enough and with the best of intentions -- by which I of course mean that I got sick and decided to spare you all the invite to my personal pity party. Not to mention the fact that I also started high-dose prednisone to combat said sickness and, well, let's be honest: blogs written on steroids should probably come with their own special warning label. So instead of going through all that, I decided to take a little break. And gosh did I ever spare you guys a lot. Seriously, you can thank me later. ("It's My Party", posted May 12, 2010)

Wow. I guess sometimes 11 months can make (almost) no difference at all, huh?

Because the truth of the matter (or at least my truth, because that's an important distinction), is that while transplant has been (almost) unbelievable and sometimes feels like (almost) a cure, there are still times when I think that maybe almost just isn't good enough. There are times when I still --- even (almost) a year after receiving my beautiful, gorgeous, miraculous gift of life -- have difficulty writing on this blog. It's as if I'm (almost) afraid to share the fact that I've been back on IV polymyxin for (almost) an additional month now, or to admit that this means I've gone (almost) the entire time since my transplant still soaking up heavy-duty antibiotics like it's my job. And sure, I could mention the fact that my lung function right now is (almost) as good as I've ever seen it or that or that most days I wake up feeling (almost) "normal" (whatever that is, anyway). I could go on and on about how I'm (almost) able to feel like a dependable human being again because my 4ish-hour medical appointments are dwindling in number to an (almost) tolerable level where I (almost) don't leave in a murderous rage and am (almost) able to imagine that I have a life outside being a full-time transplant patient. Or, of course, I could let you in on the flip side of all that, which is that I (almost) always get home from even a few hours out exhausted because of all the side effects that come with my (almost) overflowing medication cabinet.

And believe me guys, I almost sat down to write about that all about once every single day for the past month. Almost.

I think the real truth of the matter is that there are always (almost always?) at least a couple of "almosts" in the mix when you're dealing with chronic illness -- or even with a chronic illness brought out as a sort of Hail Mary pass at a "cure" for the original chronic illness, as I'm now learning. There are always going to be issues -- some that are (almost) ignorable and others that are much more serious -- surrounding my own health and the health of those who share my disease. There are always going to be questions that don't have easy answers, or days when it all seems to come crashing down on top of us, or even those where we seem to come crashing down ourselves. There will be moments when we are overwhelmed by how much things have changed for us, and moments when we are equally as overwhelmed by how much they really haven't. And when it comes to all of that, I can say with absolute certainty that these are universal truths of human experience, every one of them. There's no almost about it.

And so tomorrow I will go to my transplant clinic, where I am (almost) sure that my doctor will stop my IVs and allow me to wait it out until my upcoming appointment for with my ENT -- which, by the way, is actually on the 12th and therefore explains why it would have been (almost) impossible for me to post this on my actual 11-month anniversary with my beloved Donor Bob. I am (almost) positive that the ENT appointment will lead to sinus surgery, itself another fun fact that (almost) everyone living with CF can look forward to at one point or another. And after that, if everything goes smoothly or at least (almost) according to plan, I am looking forward to maybe casting off this (almost) never-ending cycle of IVs and looking ahead to the next phase of this (almost) unbelievable experience of learning to live with (almost) perfect lungs in an (almost) never boring CF-meets-transplant-meets-Piper kind of life.

And I think I'm (almost) ready.