The Little Things

This is a picture of my thermometer.


I acquired this little yellow doom-stick in a total isolation room on the seventh floor of my hospital. It tried to "stick" it to me (terrible pun totally intended) by registering a fever on the day of my planned discharge and royally freaking out my doctors. After I had weaseled my way out of the situation with the help of my good friend Tylenol and all the argumentative force of a very expensive legal education, I figured the little troublemaker owed me a favor or two, so I pocketed it. (Note to readers: this is 100% legal in the hospital with disposable thermometers -- you've already paid for them anyway.) Unfortunately, in my glee at "free" new medical equipment, I forgot and left my beautiful blanket behind on my hospital bed. Hey, you win some, you lose some, right?

And what I "won" in this case was a judgmental piece of plastic that lately has been spoiling my plans way too often, despite a hefty dose of immuno-suppressants and a polymyxin/aztreonam IV cocktail. To say that me and "Thermy" here are not close friends would be an understatement on par with calling the cast of Bravo's Real Housewives, "just a little bit annoying." Still, most of the time the two of us are able to bury the hatchet and get along for the 10 seconds or so that it takes Mr. Killjoy to do his job and register a fever.

Most of the time, that is, until last Friday.

The events in question started out pretty much like always in that I was super cold and shivering under about 15 blankets when it suddenly dawned on me that maybe I should actually take my temperature rather than blaming the 60 degree weather outside. So I fished the little monster out of my medicine cabinet and popped it in my mouth. As I did so, I also made my patented cross-eyed contortionist face, which allows me to see when the little "F" on the thermometer stops blinking, and then I know the reading is done. It's a fun little party trick, only this time my friend, who was standing about 10 feet away from me, happened to pull a confused face of her own.

"Hey Pipe, why are you still holding that thermometer in your mouth? It's been beeping for a while now."

Um . . . pardon my language here, but shit. Turns out the thermometer that I've had for 4 months -- the one I thought was annoyingly silent -- is, in point of fact, anything but. The darn thing beeps, apparently with some repetition, and it has been making this beep at me every day at least twice a day, week after week, month after month, from a distance of about 6 inches from my ear. And I have never heard it. Not even once.

Okay, so let me just pause for a second here to acknowledge that this is not the end of the world. I have known for over a year now that I have permanent hearing loss at the high frequency level from a lifetime's worth of high-dose tobramycin use. And, weirdly enough, I'm okay with that, especially because my particular case is very mild by hearing loss standards. Even on the day I failed my hearing test I didn't really think much of it. I was, more than anything, disappointed by the loss of yet another helpful drug to the side-effect gods and my life more or less continued on as normal -- I just sort of accepted that I wouldn't be able to understand people very well if there was a lot of background noise and then I moved on. There wasn't anything I could do about it, anyway.

On the other hand, though, the incident this past week really threw me for a bit of a loop, and I have to admit that I'm still a little confused as to why. I mean, not to sound dramatic or anything, but I wake up every morning and pop a handful of pills designed to make my body as vulnerable as possible to any and all infectious agents, with the hope that such vulnerability will also help protect the foreign organs that currently reside in my chest. I follow this up with shots to ensure my blood doesn't clot and (more often than not for the past few years) several hours spent plugged into high-dose antibiotics that make me walk like a drunken sailor and lose much of the sensation in my face and hands. I'm completely used to checking my blood sugar and giving myself insulin shots at the dinner table by now, and I'm surprisingly cavalier about waltzing around New York City with a needle sticking visibly out of my chest. And given all of this (not to mention the slew of other stuff people with chronic illness face on a daily basis), you'd really think it should take more than a silly beeping thermometer to ruin my day. Right?

Wrong.

That insignificant piece of plastic and its inaudible beep really, really, really pisses me off.

People like to tell us that we should focus on the beautiful little things in life, and also that we shouldn't sweat the small stuff. But I'm the first to admit that, more often than not, I seem to get it the other way around. Lung failure and subsequent open-chest surgery, constant IVs, and a medication schedule that would confuse most pharmacists seems, well, kind of run-of-the-mill to me now. Not to sound blase or anything, but I've been through it, I've seen friends go through it, and I've even learned to laugh at some of the morbid stuff. I had to. But yet I still get angry and defensive when my family (lovingly) teases me about my less-than-perfect performance as a patient in the ICU. I still get mad when my housekeeper rearranges my medicine cabinet (do.not.touch.the.drugs.), and I'm kind of ashamed to even admit how pissed off I get when I can't find a cab in the rain. And yes, even I am aware that the last one on that list is possibly the single whiniest complaint ever. I'm still 100% guilty as charged.

So, yes, it's true that I can ignore a rainbow or a kind smile from a stranger, and still get my day "ruined" by something that I know is, in the grander scheme of things, probably not even that big of a deal. And for the record I'm not saying that hearing loss of any kind at 29 is acceptable, but I am admitting that I'm not quite sure why I choose to focus so much energy on that, rather than on the multitude of other things that are going right (or wrong) at any given moment in my life. I'm not sure why I can handle talking about an infection that nearly killed me, but not about the ICU that saved my life. And I'm not sure why some things seem so much more intense in the moment than they do, say, a couple days down the road -- or even to the person standing 2 feet to the right -- while other things can seem like no big deal at the time, until I work myself into an angry frenzy two days later. I think that for me personally it all comes down to the way I see myself (whether that be as someone who can laugh at a little thermometer or as the world's best ICU patient), but it could just as easily be about trying to put forward an image of the person I wish I could be.

I do know for certain that it's not for me to judge what's big or small in another person's life, especially when I can't even get it completely sorted out in my own. I've also accepted the simple fact that it isn't up to me, at least on the very gut level, what sort of things will leave me chuckling at my own ridiculousness and what will make me cringe every time I hear the story. What is up to me, however, is the way I choose to react to something once it's already happened: how and when I choose to let out my frustration, the conversations I might need to have to solve the problem, and what I might do to minimize my discomfort in the future. I can't necessarily promise that I'll never be caught sweating out the small(er) stuff, but I can choose to ask the question of why something is really bothering me and what the best way is to deal with the problem. And if I'm really strong I might even choose to listen to the answer.

Provided, of course, that I can even hear it.

Serum Sickness

If you have CF, you're most likely a little bit of a druggie. Or at least, here's hoping you are, because drugs and pills and medicines and pharmacies are, let's face it, just a way of life for most of us. Our pill organizers and neb cups runneth over, to borrow a phrase, and for the most part that's a very, very good thing. After all, that life expectancy doesn't just keep increasing itself -- it's all thanks to developments and breakthroughs in the therapies and treatments we use to stay as healthy as we can for as long as we can, God and that pesky pseudomonas willing.

But what do you do when the "cure" becomes, well, not so curative? What happens when the options available to help keep you healthy suddenly turn on you, and you find yourself getting ill from the very weapons you use to combat the illness?

Fasten your seatbelts, ladies and gentlemen, because you're about to get on board that delightful little ride we call serum sickness. And just to warn you, things might get a little turbulent in here before it's over.

Serum sickness, for the blissfully uninitiated, is what happens when your body becomes ill in response to a drug used to treat an underlying condition. It's similar to a classic allergic reaction, only the symptoms are more immune-system based (so think fevers, feeling ill, swollen lymph nodes, nausea -- all the good stuff). And the treatment, as well, is similar to a classic allergic reaction, only you're dealing more with corticosteroids (hello, prednisone!) rather than, say, a quick dose of benedryl. In other words, it is not. fun. at. all.

For some reason or another I seem prone to serum sickness reactions, especially when it comes time for IV antibiotics. I used to only use tobra and cephalosporins whenever I needed a tune-up, and I noticed that I would often get fevers, vomiting, and general malaise within a few days of starting the drugs. This probably should have been enough to tip me off that something was wrong, especially when the fevers started creeping up toward the 103 range and the vomiting became a near constant issue, but in addition to my CF I seem to have a self-diagnosed case of "tough it out syndrome." Meaning that, since everyone knows IV antibiotics make you feel like you've been hit by a MAC truck for the first few days, and since I can only assume someone who had just been hit such a truck might experience fevers and difficulty holding down food, I decided pretty much unilaterally that what I was experiencing was "normal." So, while I never hid the symptoms from my doctor at the time, I also never really expressed just how serious these flu-like bouts of illness really were. And because I would finish the IVs and immediately start feeling better -- no more vomiting AND no more stupid CF infection! -- I just assumed we had to be doing something right. All of which combined to make it several years (and a fever of over 104) before I actually voiced the somewhat perplexed opinion that something maybe wasn't so normal about all of these issues, at which point I was immediately removed from the cephalosporins and began discovering that medicine could actually make you feel better -- without first making you feel like you were dying. Call it a revelation, call it an epiphany, call it whatever you like. I just called it a major relief.

Unfortunately as my has CF progressed, so has the list of drugs that cause these types of reactions. It now includes zosyn (antoher previous go-to drug) and, in what has to be one of the most ironic twists of my disease so far, even merrem. I say ironic because I actually participated in a study at one point at my pediatric hospital to see if IV merrem was effective with CF infections. I was lucky enough to get the drug at that point rather than the placebo, and my PFTs skyrocketed from a baseline of about 85% to over 100% for the first time since I was a young child. My doctor immediately declared merrem our "ace in the hole" and from then on I had visions of merrem as a miracle drug, ever at the ready to swoop down and kick some serious PA (pseudomonas ass). More recently, though, my knight in shining IV armor has started letting me down, and although I still call him into battle every so often, I've started having to seriously monitor his behavior with -- yep, you guessed it -- more prednisone. It's a tricky situation for sure, and one which constantly challenges both my doctor and me to walk the line between controlling my CF infections and controlling the reactions that require more drugs that are, themselves, not so good for the body.

But hey, nobody ever said CF was easy, right?

I guess all of this is a really long way of explaining some of what happened yesterday at my transplant clinic appointment. Unfortunately, we got some startling news regarding my position on the list -- news that reminded us all, once again, that this is a delicate, frustrating, ongoing, and most of all unpredictable process. And there's no way for anyone to change that fact.

I know transplant is the right option for me given my disease progression and my personal belief system and philosophy. I also know that getting new lungs, like living with CF, is never easy. No one ever promised it would be -- quite the opposite, actually, as we were told over and over again by friends, doctors, and just about everyone else who's ever "been there" that the journey would be, at times, grueling -- and maybe it shouldn't be anyway. Maybe it's necessary to walk through some fire in order to truly appreciate just how amazing this gift from a stranger willing to rise from the ashes of personal tragedy and share his/her life with another really is. Maybe you need a little fire and brimstone to really grasp the miracle that is transplant. Maybe.

Or maybe not.

Because even though no one promised it would be easy, the two words that popped into my mind yesterday as I packed up to leave my center were simple and all too familiar to me as a CF patient: serum sickness. By which I mean that we have reached a point in this transplant odyssey where the process itself has become draining, leaving all of us feeling just a little bit sick and wondering if what we're going through is, in fact, "normal." Only this time there is no prednisone, there is no other antibiotic waiting in the wings, and there is no way to simply check into the hospital for a quick desensitization. This time we're left to find our own way off the ride and back on track to the healing process, and let me just say that the path is definitely not a clear one.

But you know what, that's okay. I'm actually okay with all of this right now, maybe even more okay than I was a few days ago. Because just like that moment when I finally realized that IV antibiotics don't have to come with a side of spiking fevers and uncontrollable nausea, I feel as though yesterday was a bit of an awakening for me. An awakening into a world where it's okay to talk about the fact that transplant isn't easy; okay to have long, drawn-out conversations with my CF doctor about what's truly going on in my life and my lungs; and okay to recognize that there is, in fact, a balance between being grateful for the "cure" and being aware that sometimes even the best things we can do for our bodies have unpleasant side effects.

Call it an unasked for lesson, but it's still, once again, a huge relief.

The Truth about Waiting

(Or; Why I'm Not in the Fortune-Telling Business)

First off: okay, um, wow? Can I just say that the response for t-shirts has been AMAZING? Who knew so many people were in the market for a new set of lungs (or, you know, a picture of them at least)? Anyway, here's the deal:

*The shirts are officially no cost. I know we all have enough to deal with in terms of payments and financial worries, and I don't want anyone to miss out on the opportunity to give my blog free publicity -- er, I mean to own a beautiful and timeless piece of fashion history -- because of cost. So no charge for shirts.
*If, however, you would like to make a donation toward the cost of printing and shipping, I would be thrilled to accept it. I'm working on setting up a paypal account for that purpose, or simply e-mail me and I'll send my address along for cash/check donations. Just as an FYI, the cost is about $10/shirt, so please limit your donation to that.
*Some people have asked about toddler/child sized t-shirts. As of right now I have two people interested in receiving shirts for their little guys. I'm working on finding a provider willing to print youth sizes with no minimum order, so if you're interested in showing off your kiddo in style, please let me know.
*OFFICIAL DEADLINE for shirt orders is Friday, May 28th (good catch, Jenny!). Sorry about the short notice, but I want to get these printed and sent out ASAP.

Okay, so we're cool on the shirts.

It's been about a month since my last official call, and I'm definitely starting to get restless. Don't get me wrong, there's a part of me that's grateful for the extra time to get everything in order, but you can only repack your hospital bag a certain number of times before you start just wanting to get it over with, already. It's crazy to think that headed into April (with two dry runs from March already behind me), I was absolutely certain that I would have new lungs within the month. Well, okay, so that's not entirely true -- I haven't gotten so cocky yet that I believe I can predict the future, but I was pretty sure the "real" call was coming soon. And now to suddenly find myself at the end of May and still with my CF lungs, well, let's just say I won't be setting up shop as a fortune teller anytime soon. It's humbling, when you think about it, to realize just how uncertain this whole process is. One month you're getting 1-2 calls every week, and then a whole month passes with no lungs available for you. Frustrating, for sure, and also a reminder of why it helps to be on the healthier side of the "transplant window" going into the waiting experience. Lungs don't always come when you expect them, or when you want them . . . there's more to all of this than I will ever understand, but I know I'm grateful to have time to wait, for now at least.

Anyway, after weeks of trying to stay relatively close to home and obsessively checking my phone every five seconds in case I somehow missed a call, I finally woke up and decided that's not the way this relationship is going to go down. After 4 canceled dates and now no calls for several weeks, I'm pretty sure I could do better. No, I'm not switching centers, if that's what you're thinking. I'm still happy overall with my choice and love my doctors, so that's not in the cards anytime soon. Instead, I've decided (again) that it's up to me to actually live my life as best I can during this period, despite the uncertainty of it all. I say "again" because you may remember that I already had this brilliant revelation months ago, but it's all too easy to forget your vow to keep living when the call seems imminent.

So yeah, all of this is a long way of saying that I'm now daring lungs to come and ruin my plans. I've been planning meals out with friends, trips to the Cirque du Soleil, parties at my apartment, and even a couple of day trips. On Monday my mom and I took the train out to New Haven, CT to see my wonderful friend graduate with his (second) master's degree from Yale, and then yesterday we rented a car to travel out to Woodbury Commons -- a truly amazing upscale designer outlet center -- for some shopping and a little fun outside the City. True, we brought enough O2 with us to cover if we got the call along the way, and we're still staying within a couple of hours of my center, but it's still nice to be able to actually make plans and follow through with them.

I think it all goes back to the concept of "readjustment" that I wrote about a few posts ago. For me, at least, there was this huge adjustment when I finally accepted that transplant was on the horizon for me, and then again when I actually went on the list and realized that lungs could technically come for me any day. As my father put it "we went from desperately hoping for a cure to desperately hoping for a fresh set of disease-free lungs" within the span of a couple of years (although obviously we're still hoping for a cure -- we will beat this monster even though to do so wouldn't "save" my lungs at this point). Talk about a major shift in expectations. But I still don't think it compared to the adjustment that took place after my first dry run, when we all suddenly woke up to the hard reality that this could, in fact, happen at any moment, and whether we were prepared or not wasn't going to make any difference to the lungs when they finally arrived. So we went into intense transplant mode, which continued hardcore until the "damp run," at which point we kind of shrugged our shoulders and decided that we had, in fact, seen it all and were literally as prepared as any family could possibly be. So we waited, and waited, and then the adjustments began again as it began to dawn on us all that 4 dry runs does not a transplant make. In other words, each day is a new day, with as much or as little of a chance of a matching donor as the day before it, and expectations don't mean much when the whole thing is out of your hands.

And so I decided to readjust one more time: back to life, back to being grateful for every new day with the lungs God gave me, and back to focusing my energy on the things I can control, rather than spending all my time thinking about something that is going to come when it comes regardless of what I think about it. I figure if time flies when you're having fun, the least I can do is try to speed up this waiting a little bit.

And, in the meantime, if any lungs want to come along and crash my party, that's just fine by me.

Lessons from the Westside Highway

I had an epiphany today.

Like many great moments in history, this one happened in the backseat of a Manhattan taxi cab. (As a sidenote, I'm convinced that cabs breed brilliance b/c of the "near death experience" nature of most intercity cab rides.) So anyway, I was sitting there -- hoping to avoid a collision and thankful that, just in case, I had already registered to become and organ and tissue donor -- and I had my epiphany. Because suddenly, in the midst of all the honking and the lane changing and the jaywalking pedestrians, I had this single, crystal clear thought:

Life would be so much easier if I could just stay seated all the time.

Seriously, that's what I thought. And no, don't worry, that wasn't the brilliant epiphany. Because immediately after having that one, singular thought, I started to remember how absurd, wrong, and well, just "un-Piperlike" that thought really was.

I grew up in Colorado. I spent my childhood hiking, biking, and skiing, when I wasn't busy swimming, riding my horse, and "galloping" around my backyard over hurdles in make-believe horse shows with my friends. Later on, in college, I volunteered my time at a day shelter for homeless youth, at which my primary job was to chase five year old children around and around the center's playground. In short, while I may never have been a super athlete, I have always been extremely active. And I certainly never in my life thought it would be "fun" to sit still.

For me, the hardest part of this whole process has been the feeling that I am slowly but surely "losing" parts of myself and my personality, if only temporarily. I no longer have the physical energy to do many of the things I love to do -- even small, silly stuff like dancing around my apartment or chasing the puppy. And I no longer have the mental energy to commit to certain other activities -- long conversations are sometimes tiring, and I find myself less likely to expend effort on being funny or outgoing. It's not like I'm not me anymore -- I definitely am. But I sometimes feel like a painting that's been left out in the sun too long. The picture's still there, with the artist's unique flair, but the colors are maybe just a little bit muted.

But, I promised you guys and epiphany, so here it is:

No matter how much CF takes from me right now, I will never allow it to cause me to lose sight of myself.

Okay, fine, I know that sounds a little bit cheesy, but you'd be surprised at how hard it is sometimes to say to yourself "okay, today I might only have the energy for the necessary things -- the treatments and the exercises and the appointments and the breathing -- but tonight I will make some time, even just a minute, to remember what it was like before those daily tasks took up all I had for the day, and to dream about plan the time when they won't again." It's not easy. In fact, sometimes it's painful to acknowledge that things are changing at all, but my revelation today was that in the acknowledgment -- in the understanding that while certain parts of me will always be present, others have necessarily taken a backseat lately to the simple task of staying alive and breathing -- there also comes a realization that this is not the only way for things to be. I don't have to accept these changes as permanent or even as a guaranteed part of my life right now. I can acknowledge them and then consciously choose when it's worth getting out of breath to do that silly nighttime rompus with Sampson. I can give myself the okay to focus my energy on the important stuff while still remembering that inside it all is a funny girl with a sarcastic sense of humor. I can give myself the freedom to take care of myself now, while still reserving just a little bit of precious energy to fight for the woman I was, am, and will be again.

And that, my friends, was an epiphany worthy of even the most terrifying taxi ride.

New Post

I feel like I need a new post. Today is Easter -- a celebration of New Life and spring and grace and love (or at least that's how I see it) -- and if there were ever a day deserving of a new post, I think this is it.

Today was lovely.

Okay, so I don't mean to romanticize it. Today was not my most relaxing Easter, to say the least -- my throat is sore, my brain is still a bit confused and addled, my body has multiple holes in it that I keep discovering, and my oxygen is low. I'm tired, and drained, and a little loopy and beat up (but hey, you should see the other guy!). And to top it all off, my father went out yesterday AFTER the "damp run" and decided to blow off steam with a bike ride around Manhattan. All well and good until someone drives a van out in front of you and you end up in yet ANOTHER New York City ER getting stitches in your lip. And would you believe the man had the decency not even to call those of us back home trying to sleep off the ICU? He just got his stitches and rode his bike back to the apt. Needless to say we were all a bit befuddled when he sat down at the table with a busted lip and a sprained wrist. (On the bright side, it takes some of the attention away from me and my battle wounds!)

But today, of all days, truly was lovely.

Today I took my puppy on two walks, balancing a leash, a gimpy father, and portable O2.

Today I ate an amazing Easter dinner with my mom, my dad, and my amazing sister, with my puppy at my feet.

Today we finally talked about what happened, shared the funny stories about half-awake conversations and waiting room drama -- and were actually able to giggle at (almost) everything that went down.

Today I took a really, really hot bath.

Today was 75 degrees in New York, and I spent time outside with my puppy in the dog run, watching him play (or not play) with all his friends and neighbors.

Today my parents presented me with an Easter basket for the first time in years.

Today I breathed, perhaps not as deeply as I thought I would be breathing, but with lungs that still work and with air that means I am still alive.

Today I spoke with my wonderful doctor, again.

Today I did exercises in the apartment until I could feel my muscles starting to respond, and felt the fogginess sort of melting away.

Today I watched The Blind Side with my family. I loved it.

Today I learned that rebirth doesn't just come from transplant, or from great events, or from even that Ultimate Sacrifice that God made for us. Today I learned that sometimes new life just means waking up and seeing things a little differently -- knowing that even when things go wrong, even when we're a little beat up and a lot disappointed, we can still keep breathing.

Today was a really, truly, honestly, lovely day.

Positive Charge: Blogger Challenge

Okay, so apparently once I get on a roll I don't like to stop. I'm feeling good (well, feeling better) and it's translating into a sort of...blogtasticness? Um, yeah, anyway. You'll have to forgive if these posts are maybe a bit more random than usual. There's just so much bouncing around in my head right now, and I feel like it's as good as any a time to get it all out there. So with all that said, I have a new topic and I would totally like your input, sweet readers:

What does "positivity" mean to you in the context of CF, or life in general?

I feel like "positive attitude," "positivity," and other words get thrown around a lot by really amazing and well-meaning people, but I'm not sure everyone's version of what it means to be "be positive" is really the same. I mean, I highly doubt many people see themselves as having a "negative attitude" for example -- they may see themselves as being "realistic" or even "honest," and in turn they may think that others are unduly cheerful or even "in denial." And I guess this all got me thinking, because is one person's positive thinking another person's denial? Or is my realism your negativity?

And even more vexing, is our "hope" or "positivity" threatened by stories that don't fit into our paradigm? Or is our sense of self and worth degraded when others do better than we have with the same disease?

Honestly, I'm not trying to start a turf war here. I'm asking these questions because they seem to come up more often than I might expect, and now that I've hit transplant stage it's like they're screaming at me from the page, whether I like it or not. To use an example of two blogs I very much admire by two women who inspire me daily but who are dealing with two VERY different cases of CF: where does "I have CF, So What?" end and "Not so Bright and Shiny" begin? Because each of these are very real, honest, and open depictions of cystic fibrosis, and I don't think anyone in their right mind could argue with the notion that both of these women embrace life and live it to the fullest with grace, wisdom, and style. And more than that, they're both working very hard to stay positive in the face of some pretty daunting obstacles -- but do their versions of "positivity" differ, to some degree? Well, maybe. Probably.

To put this in a personal context, I have to admit that I often feel the need to "champion" the transplant CF community (which is, of course, not to say that I am the best or even an adequate representative for my fellow cystics in waiting -- it's just that I happen to be involved in a lot of discussions and therefore have the chance to speak up). I want people to understand that transplant is an option, that it's not the end of the world, that it's okay if you get sick and you're honestly doing the best you can, that there's no guilt in having cystic fibrosis or being overwhelmed once in a while. And more than that I want to emphasize that there is no one approach to CF -- I have known so many people who blow me away with their own unique brand of grace and strength in the face of this disease, having nothing to do with lung function or life expectancy or whatever other number you might be tempted to zoom in on. And when I first was told that I needed a transplant, I quite frankly considered it my mission to let everyone know that sometimes, darn it, you just need new lungs.

Fast forward almost two years from my initial meeting with the transplant team (sidenote: holy WOW, are you kidding me?? TWO YEARS??): not a whole lot has changed except that 1) I'm now actively listed for transplant, and 2) I've toned it down a bit. And, no, the latter is not because I no longer believe that people should know more about transplant and that transplanted CFers should be visible inside the community. It's more because I've become aware that it's not my job to force my view of CF on other people. Because if I'm really, totally, brutally honest I have to admit that my speaking up was largely for me -- I wanted people to know that I was trying, was living, was exercising and doing treatments, and still needed a transplant. And yet, somehow, as I've become a little more secure in both my disease progression and my own role in managing my CF, I've felt less and less need to justify my position (although obviously the impulse isn't totally dead, as proved by this blog). So now -- while I still find myself asserting transplant as a possibility, and a positive step for people with severe CF -- I also like to think I've mellowed out to the point where I can read statements like "you CAN control this disease" and not feel somehow attacked. I realize the person is saying that there are things you can do to improve your chances, and my response has moved from "must. assert. self." to more of a "here's to trying!"

But it's still confusing sometimes. How do we, as a community, strike a balance between honoring all CFers in their fights against this disease without judgment and encouraging people to believe they can actually have an impact when it comes to their personal health? Where is that line between guilt and empowerment? How do we showcase hope without pushing the sickest among us (those who need support more than ever) to the sidelines? And, to get back to the original train of thought for this post, where does denial end and healthy positivity begin?

Can we get to a point where we can say "yes, I got off the transplant list and I'm darn proud, but I understand that others might not, and that doesn't mean they're doing something wrong"? Can we honestly shrug our shoulders and say "hey, I need a transplant and I know in my heart that I did the best I could, but that doesn't mean I don't admire the other guy who managed to get off the list"?

And since we're all in this together against a disease that seems to attack each one of us differently, is there a way to reconcile our individual stories with respect and acknowledgment for the stories of others, even when they seem to contradict our own experience?

Well, obviously I don't have the answer, which means it's time to pass the buck. And of course I thought I'd put it to you guys, since you're basically the best wealth of information any girl could ever ask for. Post your thoughts here in the comments or write your own blog and let us know where we can find it. In other words:

Let's get real.
Let's be honest.
Let's start a conversation.
And, of course, let's be positive.

Quality Control

Recently, a CF friend of mine posed what I think is a really thought-provoking question on one of the CF forums. As a little bit of background, this awesome woman is working with the CFF to help generate ideas on how CFers can best help themselves improve their own quality of life, as well as ways that CF care teams and the Foundation can assist CF patients in this goal. It stems from the notion that the more informed, educated, and active a CFer is in his/her own care, the better the overall result from a holistic perspective (i.e., physical health, but also quality of life and other aspects). Anyway, we all know that every CFer -- um, make that every person -- is different, so she took the time to see what other people had to say on the subject.

Did I mention she's kind of awesome?

I've been thinking a lot about the phrase "quality of life" lately -- in part because it's such a major issue when facing transplant. I've heard it said again and again that the numbers aren't the whole picture (I've even been the one saying it in more than a couple of instances); you have to look at the bigger picture of how you feel and how able you are to live the life you want to live. And, on a personal level, I know that sometimes the actual story behind the numbers is far more important than what you can read on any graph or percentage point or PFT printout. But the question remains: how do you measure something as subjective as "quality of life," especially with a chronic disease like cystic fibrosis?

I have been blessed with an incredible quality of life. Honestly, I'm not exaggerating when I say I have no idea what I did to deserve it. But I do think I know (at least in part) why it has been as amazing as it has, and I can sum it up in one simple, beautiful word:

Empowerment.

Let me be frank here: when I say "empowerment" I don't mean "sense of invincibility." I was fortunate enough to be born into a family who believed (and told me every day) that I could do whatever I wanted to do with my life, CF or no CF. I was equally lucky, however, to have a family that understood the severity of my disease, the importance of fundraising and active involvement in the community, the need for great CF healthcare, and the need for emotional support. And because I was lucky in that respect, I was able to experience a good fortune domino effect that has led me to some of the best CF centers in the country, with some of the most competent, caring, and talented doctors. All of whom believed beyond a shadow of a doubt that I needed first and foremost to have a life, and then after that we could worry about how long that life might last.

Because no one told me I couldn't, I played soccer, skied, biked, traveled, participated in after-school activities, and had sleep overs throughout my childhood. (And okay, fine, my CF doctor did try to tell me that I couldn't ride horses, but my mom quickly told him that I'd be doing it anyway if that's what kept me active and healthy. Thanks, mom...seriously!) Because my parents always stressed school and education, I had no doubt that I would be going to college, and because they never told me it wasn't an option, I decided to move across the country to do it. Because they playfully argued over whether I would be more likely to get a law degree or become a professor, I always understood that I could go to grad school if I chose. And because they were always 100% willing to drop everything and stay with me in the hospital, or come help me through a rough patch, or ease me through a transition in clinics, I knew that they still expected me to acknowledge my disease and make it a part of my life, not a hindrance or a defining quality.

And now I suddenly find myself facing transplant. To be perfectly blunt, I have to admit that I've lost a little bit of my "quality of life." In the process, though, I've learned that maybe "quality" isn't defined as narrowly as I once believed.

I can't work right now -- but I still have my degree, my experience, and my knowledge. I can't live alone at this moment -- but I have lived alone in two major US cities and abroad, and I know that I will again. I can't travel while I wait for new lungs -- but I have memories of 5 continents and a wanderlust that I know will return full-force after transplant. I might not be able to go skiing -- but I have friends who gathered in Vail to send prayers and light to me from across the country until I can be back on the slopes with them next year. I'm on IVs every 2-3 weeks like clockwork -- but I know that who I am when I'm in that hospital bed or curled up on my couch is not who I am all the time, will not be who I am forever.

In short, I've learned that this stage of my life is harder because of CF -- but it does not change the fact that I have lived and loved and experienced life. And more importantly, it does not change who I am.

If you've been reading this blog at all lately, you've probably noticed that I write a lot about how much other CFers inspire me. This is 100% true. What I really hope, though, is that all these amazing CFers, and CF parents, who I know are out there (um, yeah, I'm looking at you) are also busy inspiring themselves.

Let me lay it out here:

This is not about having the best lung function numbers. This is not about earning the highest degree or getting the best job. This is not about running harder or faster than anyone else. This is not about avoiding transplant or even about surviving until we're all old and grey and sitting around in rocking chairs reminiscing about the "old days." All those things are fantastic and wonderful goals, but they're not what true "quality of life" is about, or at least not in the opinion of this old-school CFer turned hyperactive schoolchild turned horseback rider turned English major turned lawyer turned transplant patient turned friend turned blog writer.

What this is about, at least to me, is the notion that life is not defined just by the milestones we reach, but by the path we take to get there. "Empowerment" means choosing to grab whatever life throws at you by the horns and make it work for you. It doesn't mean that you never get sick, or sad, or angry, or overwhelmed -- it just means that you understand that you are more than just those bad moments. And maybe even that you believe in your ability to make what my dad calls a happy ending, regardless of the outcome.

And that, I believe, is an outstanding quality.

A Little Bit of Perspective

If you've been following this blog at all lately, you've probably caught on by now that I am slightly obsessed with gyrotonics. Okay, maybe make that "not-so-slightly", because let's face it, I find that if you're going to obsess, you might as well take it all the way. And all the way for me lately has meant 3 sessions a week, for an hour each time, with a private trainer. I love the excuse this gives me to get out of the house even when I don't feel awesome. I love the cozy feel of the studio -- all warm and lovely and filled with people moving and flexing and playing on the towers or the jumping board or the mats on the floor. I love the movements, and the empowerment, and the fact that I can actually feel myself getting stronger and more flexible from week to week. I love all of it.

Except, of course, for the stretching.

Let me explain: each gyrotonics session begins and ends with some stretches and movements designed to open up your body and warm your muscles. Sounds pretty standard, right? Yeah, I thought so too, until I experienced it for myself. I swear sometimes it feels as though the world's gyrotonics instructors huddled together in some dark room one night and hatched a plot to torture unsuspecting fools like me through the use of roller balls and resistance bands. And sure, I know somewhere deep down in my heart of hearts that this is (*gasp*) good for my body, but in the moment it tends to feel more like one of those "enhanced interrogation techniques" we've all read about in the news. Seriously, I'd sing like a canary if it meant that I never again had to endure the pain involved in "rolling out" my thighs.

I distinctly remember one session early on in my gyro career when I was literally gasping in pain by the final stretching period. My instructor was carefully guiding my movements, ensuring that I wouldn't overextend and hurt myself, but also pushing me to break through that wall of comfort, basically forcing me to ask my muscles to go a little further, and open just a little more than what felt easy. And the result was pain. Pure, unadulterated, evil pain. So I lay there, on my back with one leg extended skyward, my muscles shaking with effort and my body clenched against the suffering, when my instructor looked down at me and smiled. I thought for sure she was going to release me and let me go slink off into a corner to lick my wounds, because she could obviously see that I was in distress, but she didn't. Instead she just looked at me calmly for a second and said simply "you need to go there," as she guided my leg into an even more excruciating stretch. Just five little words to tell me how much my body needed this, and I believed her. Kind of.

So needless to say my general attitude since then has been to go to gyro, get through the first part, really enjoy the main bulk of the hour, and then endure the painful stretching and opening at the end with resolute and resigned silence. That's what "you need to go there" meant for me, after all: do and get through it, and who cares if you enjoy the process, right?

Well, maybe. The other day, as I was slipping out of my shoes and coat, I took a second to wonder why I was dreading this stretching part so much. And immediately the words "you need to go there" popped back into my mind, because, to be honest, they never really left it in the first place. So there I was, coat half off and contemplating what it means for the body to really "need" something, and then, all of the sudden and without really meaning to, I made a personal decision that the stretching for that day simply would not hurt. My decision was based on the logic that if my body needed to stretch, then doing so was not harmful; in fact, quite the opposite. Pain is the body's way of telling you when you are doing something wrong, like sticking your hand into a fire, for example. Holding your hand in a flame is supposed to be hard -- your body makes it hard on purpose -- because it isn't a particularly good thing to do. Stretching, on the other hand, is good for you. Exercise is good for you. There may be sensations that accompany these actions, but those sensations aren't really pain: they are the feelings of muscle growing, or opening out to greater flexibility, or connecting with other muscles to form a stronger, more balanced movement. I wasn't sure changing the label would change the experience, but I was bound and determined to try.

So are you ready for a miracle?

Well, don't be. I've had three sessions since that day and the stretching still isn't easy or comfortable, necessarily. But it honestly isn't painful somehow. On the first day I tried out my new perspective, I noticed that I was breathing into the stretches more deeply, accepting on some level that these were good and that I really did "need to go there." I don't dread these parts of the session anymore either, in fact I find that I kind of like them in a weird way because I see them as a vital part of the workout -- not just something to get through and get over with. It's not so much that anything about the stretches themselves has changed; it's just that I'm opening my mind now instead of just my muscles and joints. And that seems to be making a difference on some level.

I ended my session today with the same stretches I used to hate, and found that I barely broke stride or focus going into the movements that just a week or so ago had me gritting my teeth and clenching my fists. I concentrated on my breathing, exhaling into the motions, and just let it be . . . well, not easy, but maybe "right" is the word I'm looking for. I let it be right -- for me, for my body, and for the moment.

So I lay there tonight, my leg extended above and my muscles still shaking with effort, and my gyrotonics instructor looked down at my relaxed fists and easy breathing. She smiled, and I thought for sure she was going to let me go this time, and then she simply said "yes, right."

And pushed me just a little bit further.

(Re)Discovering Myself

So this evening I was flipping through some of my usual suspects of CF/transplant blogs to see how everyone's doing, learn lessons, get inspired, whatever, and I came across something that literally stopped me in my tracks -- namely, an entry posted on my friend Leah's blog by her wonderful husband. I don't have permission to link it here or else I definitely would, but Leah is a beautiful post-tx CFer who received her new lungs 3 weeks ago at Stanford (and was home for Christmas in what had to be one of the most awesome recoveries of all time!). And today her husband posted a quick update about life 3 weeks after the gift of, well, life.

The thing that touched me the most was his sheer joy at watching his wife live with healthy, non-CF lungs. And he drove the point home with his observation that there are aspects of her personality that have been hidden behind her progressing disease and are now, to his obvious delight, reemerging. It literally brought tears to my eyes -- such a moving and well-put tribute to Leah's enduring spirit and her ability to heal, to find her way back to herself through the grace of a generous organ donor and her faith. To say the least, I was humbled and inspired by what I'm sure for them was just a run-of-the-mill blog entry. So thanks go out to Seth and Leah for once again giving me that awesome tingly feeling.

I have to say that Leah's blog also made me think, big time. It made me think about illness and its effects, not only on our bodies but also on the other, less tangible aspects of ourselves. It made me think, specifically, about my own personality and the ways that it has changed since becoming "sick[er]" with CF -- for the better or maybe for worse, but maybe just also in neutral ways. It made me think about how hard most of us with chronic illness work to make statements like "CF doesn't define me" and "I have CF; CF does not have me." Statements that we absolutely mean. But then I think about other statements I have heard from post-tx CFers about how transplant allowed them the chance to be the person they were always meant to be, or allowed them to recover a piece of themselves that they thought had gone missing. Statements that are also 100% true. Statements that, honestly, make me sooo excited for the future and my life with new lungs.

Let me say this: I have tried, to the best of my personal ability, to be one of those CFers who can proudly proclaim that I am NOT defined by CF. And I'm not, honestly, because there is just so much more to me than coughing fits or late night treatments or accessed ports or steroid pills. So much of me that quite frankly has nothing to do with any of that. And so I've gone to sometimes great (and occasionally stupid, not gonna lie) lengths to prove that I was and am somehow "stronger" than this disease, at least in mind and spirit if not always in body. And as I've gotten sicker, I've clung even more intensely to my knowledge that who I am cannot be worn down by this disease. No matter what happens, no matter if I end up getting a transplant or not, no matter what the ultimate outcome, I truly believe and know that I am and will always still be Piper -- I will always be more defined by the way I lived my life than by the obstacles that I faced while doing it.

All that said, I think it would be sugarcoating CF to pretend like it hasn't taken its toll on my personality, especially in the past few years. There, I said it. My name is Piper and I'm not 100% perfect -- go figure. Because as much as I try and believe and hope, as much as I know that I'm strong and I think that I'm pretty darn good at the whole "positive attitude even in the face of a whole wheelbarrow full of CF bull****" thing, I still know that, like many of my friends out there in real life and in cyberspace, there are in fact aspects of my personality that don't get to come through as often now that I'm dealing with being "sick" almost full time. It's not because I'm purposefully suppressing these parts of myself; it's just that so much of my physical energy now goes toward the necessary (the treatments, the exercise, the appointments, the resting), and so much of my mental energy goes toward the preparation, and the waiting, and the realities of being, well, really sick -- I can't honestly sit here and say (er, type) to you that I haven't had to let a few things slide to the wayside.

For the most part, I try to hold onto the parts of myself that I cherish. I try to take at least a little time every day to do something to remind myself that I'm human, and more than just a disease or a number on a waiting list. When I have extra energy, this is simple: a visit with friends or dinner out or a walk with my dog can make all the difference in the world. When my body is sapped from antibiotics and steroid-induced insomnia and coughing, it's a little tougher, but a good book or some time spent writing or painting, or even a game of scrabble and dinner in with my mom and sister will lift my spirits when I feel trapped in my apartment and tied to my O2. So that only leaves the days when I'm wracked with fevers or infection, and on those days I just try to remember that I'm surrounded by love. And I'm pretty sure that when John and Paul sat writing the lyrics "all you need is love" they had no idea how applicable the words would be to a bunch of crazy cystics all waiting on a transplant list. Just sayin'.

So I guess as much as I want to believe that my spirit and personality are stronger than cystic fibrosis -- as much as I tell myself that this disease can attack my body but will never touch who I am inside -- I have to admit that there's a part of me aching to see what aspects of myself "reemerge" in a sense after transplant. And I'm reminded of what my sister said as my family of four left the hospital after our initial meeting with the transplant team in May of 2008. As we huddled, overwhelmed and exhausted, in the corner of the crowded hospital elevator, Erin turned to the rest of us and demonstrated, once again, her unflagging optimism: "just think," she said, "ten years ago this might not even have been an option. And now we're going to get the chance to see Piper able to do things she hasn't been able to do in a long time!"

At the time, of course, we all thought she meant physically, but now I'm not so sure. What I do know, though, is that I couldn't be more excited to find out.

Facebook Faux Pas

Why, hello there soapbox, how are you today? Me? Oh, I'm fine, still getting by, doing IVs, all that fun stuff. What's that? You want me to step up on you? Well, really, I couldn't. I mean, sure, this is a personal blog and sometimes I do tend to go off on things, but I just don't think anyone really wants to hear me rant about . . . what? You say you'll be super sad all Christmas if I don't stand up on you right now and get this off my chest? Well, geez, soapbox, you and I have been friends forever and I would hate to see you upset. I guess I could get on for just a small second. You know, if it'll make you feel better and all. Just remember, I'm only doing this out of the goodness of my heart . . .

Dear Facebook Friends:

First of all, just let me start this off by saying thanks. Thanks for looking me up, for finding me even though some of you I haven't seen since I was in grade school, for "friending" me or accepting my request, for caring about how I've been doing, for wanting to connect, and for allowing me to share in your life, your favorite bands, your drunken party pics, and, of course, your status updates. I really appreciate it, often more than I can say. Let's face it, in the past Facebook was something random I logged into every couple of weeks or so, but lately it's seen me through more than one lazy hospital afternoon -- allowing me a much-needed sense of contact with the outside world -- and for that I'm totally grateful. Really.

But, well, I didn't climb up onto this thing just to thank you all. I actually have something to say and I think it's important. So please, if you could all just stop typing for one second, I'd like to try and get a quick point across.

Life, as a general rule, does NOT suck.

Okay, so take a second. Let that sink in a little before you go back and write your next status update. Because honestly it's true: life does not suck. It doesn't. I don't normally love across the board general statements, but I'm willing to make one here because, in case I haven't mentioned it yet, life does NOT suck. And I'm sorry to have to be the bearer of good news, but your lives, in particular, do not suck either. This is true, in fact, despite what you wrote in your status this morning.

Wait, wait, please don't jump on me on all at once and tell me how much I'm misunderstanding what you wrote. Because trust me, I know that you were just being melodramatic when you wrote "fuck my life" because the coffee maker at your office was broken this morning. And I know that you don't actually "want to go to sleep and never wake up" because you have a headcold. I even totally understand that when you wrote "This sucks. 2009 sucks. It all just fucking sucks." you didn't really mean that everything sucks, just that you feel hurt and angry and upset right now. I get that, really, honestly, I do.

But please don't ask me why I get so sensitive about these "little jokes" unless you want to hear my real answer. Because if I'm honest I'd have to admit that it deeply offends me when people, even friends who I know are joking, take life or granted. It offends me because I know too many people fighting to survive, and because I see people who have way bigger problems than the coffee maker who would never dream of writing "fuck my life." It offends me because I know people who have chosen to move on -- I've seen the kind of illness that drives people to make that call for themselves -- and it's never about a headcold. And it offends me because I know people whose 2009s were harder than you could ever imagine, and yet they never seem to be the ones complaining the loudest.

So let's see if we can't reach some middle ground here and agree on a few basic points:

1) Coffee in the morning is helpful, sometimes even necessary. And broken coffee makers are a pain.

2) Headcolds hurt. They do. I personally hate them.

3) Even perfectly healthy people have bad days/weeks/months/years sometimes. And they have every bit as much of a right to vent or complain about them, especially to friends, as anyone else. Illness doesn't make you noble, and being healthy certainly doesn't make you shallow.

and 4) Life does not suck.

There, was that so hard?

I guess all I'm asking for is a little bit of thought before you type out the most negative status message you can possibly muster. And if that's asking a little too much, well, I guess I understand that too, because who am I to stand between you and that tempting little "what's on your mind?" box? But just know that you're a whole lot likely to get sympathy from me if you keep it in perspective, and I promise I'll try my hardest to do the same.

xoxo beautiful people,

Piper and the Soapbox

Musings on a Birthday Week

If I had known earlier that at age 28 I would need a lung transplant . . .

I might have appreciated all those summer hikes and winter ski trips that my parents "dragged" me on in the Colorado Rockies throughout my childhood, instead of whining about sore feet or cold fingers;

I might have played a little harder, or laughed a little louder at the really silly, ridiculous, little things like that time that my friends and I dressed up as "fashion models" and took homemade glamour shots in my bedroom with my tiny little pink camera (and I might even have saved some of the pictures);

I might have been surprised to know that one of my fondest memories now, living life in the biggest city in the country, is of lying outside in tiny little Oxford, GA (after driving at 3am to get there), looking up at a total universe of stars, watching a meteor shower with two of my best friends, and wondering how it was even possible for there to be that much light and that much enjoyment even in the middle of the night;

I might not have fought so much with my sister over nothing, because that was a total. waste. of. breath. (in every sense of the word);

I might have sung along even louder at all of those Indigo Girls and Dave Matthews Band and U2 and Elton John with Billy Joel and Bob Dylan and Ben Harper concerts that I went to throughout college (and yes, even one embarrassing but very fun Backstreet Boys show);

I would have really, really savored that road trip from Berkeley to Denver, even the stop off in Vegas and the stay at (where else?) New York, New York Casino;

I totally wouldn't have changed a single thing about that trip to Greece after the NY Bar Exam, especially not that strawberry moonshine we drank on that final night in Athens, or the restaurant with the view of the Parthenon at sunset, or the donkey ride through the streets of Santorini, or even that stupid mountain I was "forced" to climb on Delos to get to that bunch of scattered rocks (whoops, I mean, "awesome ruins");

I would have fallen on my knees in gratitude for the greater plan when I met Sammy back in 2008, because who could handle waiting for lungs without a live-in best friend to keep you company?;

I would have rocked every pastel-colored bridesmaid dress I've ever been asked to wear (you know, more than I already did totally rock them, of course);

I would definitely have used that "Welcome to Graceland" keychain with the ridiculous picture of my sister and me with the fake Elvis on it (I'll give you two guesses as to which of us was sporting a full-on crop top -- hint: Erin has better taste than I do!) and I would have laughed every time I got in my car or opened my apartment door;

I might have realized earlier just how amazingly connected we all are, and I might have had the foresight to treat every stranger I met like someone who might save my life someday with an indescribable gift;

I would like to say that I would have cherished these lungs for all they've been worth to me for the past 28 years, but I know that I was too busy living to truly count each breath, so instead I'll just take a second now to say to these "old" twins: thank you, for all that you are and all that you helped me do. I forgive you for any tantrums you might ever have had, which were, after all, a part of me too;

I might not have changed much, but I sure would have noticed it all a whole lot more.

And now that I know, I can at least take the time to just that. Hopefully for a long, long time to come.

Life Lessons

It's no secret that I believe in the power of positivity. Honestly, if my blog does nothing else, I hope that it shares with my family and friends some insight into my view of the beauty of life (with or without CF) and the indescribable awesomeness of every. single. breath. I don't always have much to offer, and I've never claimed to be any sort of spiritual sage, but these past few years and months and weeks and days of living with CF and living in general have taught me that this gift of life is too amazing to spend suffering, regretting, or blaming. And of course my fellow cysters and fibros have added so much to that understanding -- all of us together, at all our various stages of progression and illness, health, life, and happiness -- stand in my mind as a sort of monument to the fact that overwhelming joy and the will to simply live are so much stronger than any disease or any set of mutated genes.

That said, there are CFers out there tonight who are not doing well. These people are kind, sweet, loving, and good-hearted individuals, with friends and family who cherish them and hate to see them fighting or in pain. And in the spirit of the upcoming week -- that of being grateful for all that we have, loving others, reunions and coming together -- I'm asking that each of you take a few minutes for our fellow journeyers in need of love, strength, peace, comfort, and light right now. Whether it's a moment of silent prayer, a shared blessing with your friends/family/congregation, the sending of good vibes, or just a second of your time to celebrate life in honor of these people, I'm sure that they, and their families, would deeply appreciate it.

For Eva, whose story has inspired and educated so many;
Natalia, who has an infant girl to come home to;
Courtney, who has touched my own life personally, as well as many others in this community;
Ginger, who has a devoted husband and young son;
For all the others, past and present, who show us what it means to LIVE . . .

Thank you for your strength in the face of obstacles, for your faith and love and optimism, for allowing us all to share in your stories -- through the good and the bad, for the money you've raised and the drugs you've helped to discover, for the companionship, the friendship, and the community that we've all shared. I am deeply humbled to be a part of it all, each and every day.

I truly believe that CFers together can move mountains. Hey, we defy expectations and conquer limitations each and every day, so what's a little miracle among friends, right? Please, please, please give a little of yourself and your time and your heart for those in our community who could use a little something extra tonight. And to everyone who is sick or healthy; living with CF at age 5 months or 50 years; breathing tonight alone or on oxygen or on the vent; caring for a loved one or being cared for by those you love; climbing mountains or climbing into your hospital bed -- thank you so much for the inspiration and the lessons.

W.W.P.D.?

So I have a HUGE couple of weeks coming up with the holidays approaching. I can't even begin to say how excited I am and how much I have to be grateful for (as always) this year. It literally blows my mind when I think that in the next TWO WEEKS I will:

• Enjoy out-of-town visits from my two best friends from college (one of whom was also my roommate for three years and is bringing along her boyfriend, who I've never met!), my father, my godmother, and my grandmother, along with a visit last night with a childhood friend I haven't seen since I was her bridesmaid several years ago.

• Film a CF awareness video on waiting for transplant for the CFF's American Airlines Celebrity Ski event -- which I've been blessed to be a part of for over 20 years.

• Attend a museum exhibit opening featuring some of my sister's fashion designs on display!

• Stop IVs!!

• Have a final follow-up appointment to hopefully end the "Not-Quite-So-Great PICC Clot Fiasco of 2009." Good riddance to bad rubbish.
  

• Celebrate my godmother and grandmother's birthdays with dinner and a Broadway show.

• Cook a Thanksgiving meal at home, to be enjoyed by friends and family in my own cozy little apartment, including my family's famous southern cornbread stuffing.

• Celebrate my 28th birthday -- first with a family dinner, then a few days later with friends.

Wow. Okay, I think I just got tired typing that all up. Honestly though, I'm sooo ready to just enjoy this special double holiday (birthdays + TG) with some of my absolute favorite people. And when I remember that people are flying in from as far as Colorado, Texas, and California to help make all of this happen and to accommodate MY schedule, well, it just kinda starts to seem unreal. I don't like to turn this blog into a contender for the cheeseball of the year award, but right now I'm feeling pretty warm and fuzzy -- and I wouldn't have it any other way.

Anyway, since absolutely none of that had anything to do with the title of this post (or so it would seem), you're probably all a little confused right now. See, as excited as I am about all of the above, and despite all the many warm fuzzies swirling around and filling my head with visions of mulled wine and amazing homemade stuffing, I'm also a little, well . . . scared of it all, to be perfectly honest.

I'm nervous because I know that my health depends on following my routine, and we all know that the first thing out the window during huge family gatherings -- no matter where they happen -- is predictable routine. I also know that my body needs more rest than it used to -- that as fun as it is to push myself until I drop, it's not always the wisest decision in the long run. I'm ready as can be for all the nice dinners and social events, until I pause for a moment to remember that this year I'll have to make decisions about when to wear oxygen when I'm out on the town. And finally, as psyched as I am about all the yummy calories coming my way (a CFer's favorite part of the holiday season, for sure!), I can't help but remind myself that it might be tough to make time in between all that eating and socializing to hit the gym.

So what's a gal to do? I think in the past I pretty much wrote off the holidays as a bit of a free for all. As long as I stayed on top of my treatments and didn't overwhelm myself to the point of total sickness, that was good enough for me. After all, it's only a couple of weeks, and CFers are nothing if not masters of "pushing through it."

But this year, I'm approaching the whole thing from a slightly different perspective. For starters, I don't know when the call for transplant might come, and I don't honestly have two weeks to slack off on important things like my exercise routine, because who knows if I'll have the time to make those up after the holidays are over? Perhaps more important, though, are the promises I've made myself over the past year, like the promise to really focus my energy on getting STRONGER every day, and of course the promise to always consider my health -- because if I'm going to accept this gift of life from someone then I'd damn well better be ready to make the most of it.

Of course, I'm not planning to put a halt to any of the holiday fun. Health might come first right now, but it doesn't have to trump life (which is, after all, the reason we do all this stuff in the first place), right? So I've decided to make a simple change and ask one question that might make all the difference:

What Would Piper Do?

In other words, what would my body and spirit have me do over the long run? Sure, the turkey makes me tired in the moment, but that doesn't change the fact that Piper would still want to get in at least a walk. Much as I love to pack my days as full as possible, Piper has recently learned the value of resting and meditation to help de-stress and re-center. And yeah, sometimes I don't like the way the oxygen makes me look in public, but I'll bet Piper would rather wear the stuff and feel better so she can actually celebrate during all these birthdays and reunions. After all, despite my in-the-moment reservations, Piper is (hopefully) learning not to place so much emphasis on what other people might think, and maybe even to be proud of the cannula that shows the world that she can still get out of the house and have fun even while needing a little extra help. (Okay, so maybe not quite there yet, but making baby steps!)

I'm the first to admit that it's not a perfect approach. Piper, after all, is a lot of things, but she's definitely not perfect. I probably won't be able to market any "WWPD?" bracelets to the masses, and to be honest that's most likely a good thing. (Do we really need a bunch of Piper clones? Um, don't answer that!) But I am hoping that pausing for a second to ask myself this little question during the holidays will at least help me find that often delicate balance between life and all those other necessary things we CFers take on to manage our disease. Because when all is said and done, despite her many imperfections, Piper loves to savor things like holidays, food, friends, and family, and she also wants to be around to keep the fun going for as long as possible.

It's a tricky balance, sure, but this year I'm determined to try -- with a little help from Piper, of course.

Speculation

Tonight I got asked (indirectly) by a perfectly nice, normal person whether I thought being a lawyer had negatively impacted my health. No, she wasn't making a cute "attorneys are evil" joke -- although I was tempted to respond that my lungs were crappy even before I became a lawyer, but the loss of my soul took some getting used to. Ha, ha, ha, right? But no, she wasn't making a joke; she was actually quite serious and she was asking a question that probably others have wondered about as well.

The implication of her question (I think, as I said this woman was very nice and certainly didn't mean any offense) was that perhaps my job, back when I worked, was too stressful or took up too much of my time for me to really focus on my health. Or maybe she thought that I let my health slide because I was more focused on other things. Perhaps she was wondering if law school itself was too much of a strain, or if the 7 years in higher education was a good use of time for someone with a "fatal" lung disease. Maybe she just wondered whether it was "worth it" time wise for me to be so tied up in school when life is so precious. Maybe she didn't think through any of this and just asked the question because she was surprised to see someone with CF and a law degree -- especially someone waiting for transplant.

So in the interest of answering her question, and because I think it's an important topic given how important both my career and recent choice to take disability leave have been to this blog, I'm going to be totally honest here:

1) I can not now nor will I ever be able to say with 100% certainty that my career choice did not negatively impact my physical health; and
2) If I had it to do all over again tomorrow, I wouldn't change a single thing.

Let me tell you what I do know with 100% certainty. I know that I wanted to go to law school. Badly. It's what I wanted to do with my life, and completely independent of CF it had been my dream for a long time. I know that my health had begun to decline prior to entering law school, although my first couple of years there were not themselves extremely eventful CF-wise. I know that although my job was stressful and at times difficult, my compliance and time-management skills steadily improved throughout the time I was with my firm, actually increasing my chances of staying healthy in many ways. I know that I learned through my job to assert my needs as a CFer as well as a person, asking for days off when I needed them, learning to surrender to the hospital with grace instead of dread (okay, grace MIXED with dread!), and finally grasping how to ask for help when I needed it. I know that I equally learned how and when to push myself, what my limits really are, and how great it feels to meet a challenge everyone else said was impossible for me. I know that it was law school that brought me to New York, the city I love, just as I know that it was also law school that brought me to the doctors I trust with my life, whose advice, help, and wisdom I am so blessed to have experienced these past few years. I also know that when I finally did take disability leave, it had no apparent effect on the intensity, frequency, or general nature of my lung infections, but it did negatively affect my mental health and well being.

I know that I will never look back and think that CF held me back. Most of all, I know with 100% certainty that if I had to look a child with CF in the eye right now and say that it was worth it, I would do so without a second thought.

I don't believe everyone should go to law school, CF or no CF. That was my dream; it could just as easily be someone else's nightmare! But throughout my life there have always been people who have cocked their heads, pursed their lips, and wondered (sometimes out loud) whether I should be doing whatever activity it was that didn't "mesh" with their expectations for a CFer. And I do believe, very strongly, in defying those expectations and false limitations set on us by other people, or sometimes even by ourselves. Because the CFers I know are an amazing group of people -- and I really just refuse to believe that there's a spectrum of life that we cannot or should not participate in just because of our disease.

Maybe that makes me stubborn, but I'm 100% certain that it also makes me happy.

So would I still need a transplant if I had been, say, a sculptor instead of going to law school? Well, maybe, maybe not. I just can't say. In fact, I can't say how any single aspect of my life might have turned out differently had I made different choices along the way. It's not my job to know that, frankly, and neither is it anyone else's on this Earth. But what I can say is that if I had been a sculptor, well . . . I wouldn't have been a very good one, nor a very happy one. Trust me, my experience with the pottery wheel in Turkey when they tried to help me make a vase (end result: poorly constructed ashtray) was enough to teach me that much.

In case you're wondering, my real answer to the question was far more simple and to the point than this blog. I simply looked at this kind, well-meaning, and genuinely concerned woman and told her that I would never know why my health is what it is, but that I had made the decision to stay in the driver's seat of my own life for as long as I have it, and let my CF come along for the ride.

It may not be perfect, but that's definitely my final answer.

Another Day

For those of you who are wondering, it's just another day here in New York City.

Just another day that started out here . . .





. . . and finished up here.




It's another day filled with IVs and nebulizers and oxygen and chest PT and enzymes and antibiotics and dermatology students (not kidding) and discharges that actually happened ON TIME (nope, still not kidding) and doctors wearing "droplet precaution isolation" masks in my room even though I'm officially negative for swine flu just because I still had the sign on my door because I'd really prefer everyone mask/glove/gown up anyway.

Another day of downtown taxi rides and puppy reunions and homecooked (non-hospital) meals and comfy (non-hospital) beds and views from my (non-hospital) window and TV watched from my comfy (non-hospital) couch and maybe even some mild (non-hospital) meditation.

It's another day of gorgeous fall weather and chilly breeze and possible new developments on the transplant front but who wants to think about that kind of stuff anyway because today is just another blessed, wonderful, fantastic, flu-free day that is all about going HOME!

And home is where I plan to stay.

Diving In

Okay, let me start off this post by making one thing absolutely, perfectly clear: I believe in what is commonly termed "Western medicine." I have a huge amount of faith and trust in my doctors and I follow the routines they prescribe for me (not always without question, because I also believe strongly in understanding and advocating for your own health, but generally speaking if they say so, I do it). I believe some things in life are beyond our immediate control, and this includes our genes and whatever mutations might come with it. I believe that sometimes you can do everything right and still not always achieve a desired outcome. In short, I believe that diseases like cystic fibrosis are unpredictable and at times uncontrollable, and I am a traditionalist in the sense that I treat my CF with modern medicine, therapies, antibiotics and hospital stays.

I have also worked hard not to "blame" myself for the progression of my disease -- although I acknowledge that I haven't always made 100% perfect choices when it comes to CF management, I also know that I made the choices I thought to be best at the time, and that new opportunities and growth came from those choices that helped shape me as a person, so I can't be "angry" at myself for my choice to, for example, enter a tough and ridiculously stressful profession that at times left me choosing between work obligations, treatment, and sleep. Over the past few years I have realized one thing that I cling to: choices are made, and they have an effect on the future, but they can't be unmade later just because of an undesirable outcome. Instead you have to keep on choosing how to go forward, and for me it took a long time to choose to forgive myself, see the validity in the decisions I had made and how they had helped my life (even if they weren't perfect), and then move on with what I had to face next without a lot of defensiveness, or guilt, or anger. I'm still not perfect at this -- I have a type A personality so I always look for identifiable causes and "solutions" to problems, I'm not great at working within a spectrum of non-black and white answers -- but I'm learning. And I love that.

So where does all this self-reflection lead? Well, in a kind of uncharacteristic move for me, I'm starting to do what is called "energy work" (actually, it's called a lot of things by a lot of different people, from "healing" to "theraputic conditioning"). Call it what you will, it basically means that I'm lucky enough to have some amazing people in my life who are helping to guide me towards a new way of thinking about disease and health. I haven't delved too deeply into this yet, and I'm just starting to even become open to exploring this whole new world, but my understanding is that this is about mind/body alignment, and understanding how thoughts and memories and things we hold inside us affect our physical selves and our overall well being.

Um, and just for the record (before you all abandon my blog in a fit of justified anger and frustration): I do not believe that any of this will CURE my CF. Not for a second, not an instant, and that's not even my goal. In fact, one of my new guides in this process (introduced to me by a fabulous woman whose positive energy is absolutely overwhelming) was herself a nurse practitioner for several years. She understands disease pathology, and she knows that CF isn't going away, and that in fact much of the damage to my lungs may well be flat-out irreversible. Her emphasis is on HEALING (as in, making one healthier), not on CURING (as in, altering one's genes -- at least in my case). The goal is increased health and positive change in mental energy, not "sayonara, CF, see you later" (awesome as that would be -- hint, hint CFF). For me personally, this most likely will mean an emphasis on strengthening on the body and preparing myself mentally for transplant, as well as developing techniques to hopefully be in the best shape possible for recovery. In other words, I think I need help preparing my "sick" body to be "healthy."

No, I won't be giving up my CPT and O2. If she tries to take away my xopenex and TOBI she just might find herself attacked by a very overprotective Shorkie (trust me, what he lacks in size he makes up for in other departments). And I just don't think either of us would be very happy with the results if she asked me to give up my enzymes. (Sidenote: eww. Enough said.) In fact, one of the principle tenants of her style is that the person must make CHOICES and then follow through with them. In my case, if I choose to make my body (and hopefully lungs) stronger, it then becomes my responsibility not only to focus on that goal and believe that I can do it, but also to take steps to make it possible. You can't, according to this philosophy, simply choose to have perfect PFTs and then sit around to wait for it to happen. Being positive and focusing on the goal are only a part of the formula, to make it all add up there also has to be action a willingness to dive headfirst into the fray to get yourself there. (For some reason, this really made me think of Ronnie.)

Okay so I've written this long, rambling post about all this stuff I plan to do. Scratch that, all the stuff I CHOOSE to do. Because I want to start this out with a public declaration, however silly and possibly even narcissistic it might be, that I choose to be open to a new way of approaching my health. I choose to get stronger and to reclaim my body for ME, not the numbers. I choose to be happy and I choose to trust that transplant will happen for me at the right time, with the perfect lungs. I choose to believe that my body will accept these lungs as the gift that they are. Most of all, I choose to believe that God has this worked out for me, that with faith and energy and work on my part everything will be as it is supposed to be. And I choose not to blame or punish myself for the choices that might not work out perfectly, because frankly I just don't need that in my life.

I guess I'm writing this in the hopes that I'll find some sunken treasure somewhere deep down in a sea of self-healing of which I've (truth be told) always been a bit wary, if not flat out distrustful. Moreover, I'm writing this because I've decided to dive in, with as open a mind as possible, and I figured this would be a great place to make the first splash. I don't expect much to change directly here on the blog, but I do plan to occasionally update you all on my project, so I figured you needed some background.

And anyone who cares to join me on my little swim, feel free to make some choices of your own -- write them down (either here, on your own blog, or somewhere you feel safe doing so) and start believing you can make them happen, then take steps toward making them possible. I can't promise results (I'm an experimenter here myself), but I promise good company along the way.

Light(s) at the End of the Tunnel

Some of you may remember my rant about going to the dentist and learning that the dentist needed medical approval from the tx team in order to examine me/clean my teeth. The transplant team then turned around and demanded medical approval from the CF team b/c they're not my "primary doctors." So then my CF team finally gave clearance, which allowed the dentist to finally, finally, finally give clearance to the transplant team. Keeping in mind that in between getting all the medical clearance and actually returning to the dentist for the examination I ended up in the hospital three times in just over two weeks and you can probably begin to understand why I'm so excited right now. Because . . . (cue drumroll) . . .

As of today, I have officially completed every one of my dentist's pre-transplant "recommendations." Seriously, this is a HUGE milestone.

See, my dentist thought that my teeth were in fairly good shape overall. He saw two fillings that he thought were loose and wanted to fix them preferably pre-tx b/c loose fillings can 1) fall out, and 2) become a good place for new bacteria to grow in the space between the filling and the tooth. And since they don't recommend having any dental work done until 6 months after tx, we decided now was the time to take care of those things (although he said if got THE Call I could still accept, as he thought the fillings would likely survive 6 months anyway). He also wanted to do a root canal on one other tooth, which I was kind of nervous about having never done one of those suckers before. So I managed to get the fillings re-filled (capped, actually -- we went with the "better safe than sorry" option since they were two small fillings in the same tooth), and then I was left with the root canal. Definitely not something to look forward to.

Well, for anyone considering a root canal in the near future, I have a slight suggestion: have risky and complicated vascular surgery to remove an infected port without general anesthesia three days before your scheduled dental appointment. This fool-proof method of making a root canal seem easy has a number of perks, including: 1) your left arm will be sore because it will have three large incisions healing on it (no pain meds were offered, and I didn't request any, but it honestly wasn't too bad) -- the pain will distract you from the root canal unpleasantness, 2) your right arm will be slightly sore because of the PICC line and residual phlebitis from the blown peripheral IVs -- again, more distraction, 3) you will, I promise, have sudden flashbacks to your time in the OR prep area every time you start to get nervous over the root canal, at which point your brain will basically sigh with relief because the comparative risks of a root canal are so totally not worth stressing out about. Believe me, it works, and thanks to the miracles of novacaine and advancements in dentistry, I can honestly say the root canal was no big deal at all. I think I was in the chair all of maybe 45 minutes, although that may have been because my dentist has a periodontist on staff who does nothing but root canals, so he's super good and efficient. Lucky me.

So yeah, I got the root canal a little over a week ago, and today they finished it by capping the tooth to once again guard against any future infection. And now I'm totally and completely done with the dentist (aside from, of course, regular cleanings and check-ups). In other words, check another thing off the pre-tx to-do list, please!

Anyway, now that that's all over, I also have a CF appt tomorrow, and then on Thursday I have a surgical consult to check out my wounds from port-fest 2009 and, um, to check out my other arm for the placement of -- you guessed it -- Piper's Arm Port II. I got word from my tx team that they are willing to leave arm ports in for CFers because you often end up needing IVs within the first year after tx anyway, plus everyone is on IV drugs for at least a little while immediately post surgery. It is clear that I'll need to get it taken out once it seems like my need for IVs is less (probably within or right after the first year), but since there's no guarantee of how soon I'll be transplanted and I don't want a PICC in my arm all year, not to mention the fact that I don't want to have to have a PICC placed every time I need IVs after tx as well, I've decided to go ahead and let my favorite vascular surgeon put another port in place. And yeah, because of my history it has to be done through surgery (again, no general anesthesia obviously) instead of IR. But the same amazing vascular surgeon who got me through the amazing port debacle has told me he'll be glad to handle my next placement. So I know I'm in great hands, and I'm actually pretty stoked to get another arm port, truth be told.

I also got my LTD from work approved, so I am officially no longer part of my Firm. Bittersweet, to be sure, but the right decision for now and I know I'm lucky in this economy to have this sort of option. I'll be applying for SSDI as well in the upcoming weeks in order to ensure I can get Medicare by the time my COBRA benefits run out. Definitely NOT the time to be caught without healthcare.

So that's the news. Next tx appt is on the 14th and I have to repeat PFTs and the 6 min walk. I'm almost positive my distance on the walk test will be lower than it was in May (I went 2200 feet at that time, which is apparently pretty good for someone on the tx list). The good news is that a slower walk would bump up my score, but I'll be bummed if it's too low anyway. And I know I'll have to wear O2 for it this time as well, so that's kind of a nasty milestone. This whole "24/7 O2 because my sats drop below 90 basically anytime I move more than 5 feet" has been kind of a rough pill to swallow. Since I'll be doing PFTs tomorrow at CF clinic I should have a pretty good sense ahead of time of what my score will be on that front. Hopefully I won't have dropped from my 33% a couple of weeks ago -- here's hoping I can even pull it up a notch or two! Again, it's kind of a double-edged sword with the LAS (lung allocation score, for the non-tx people), but I'd still rather be up than down.

The best part about all of this is that I've been feeling amazing lately. My aunt and uncle were in town this weekend from Texas and we took them all over, plus I've been enjoying dinners and lunches with friends, walks outside, and generally trying to make the most of the mild fall weather. I seriously love this time of year, so I'm so so grateful to be able to enjoy it. Just thinking back to where I was at this time last month -- honestly wondering if I would get out of the hospital at all pre-tx -- is enough to remind me to be grateful. Well, most of the time anyway.

Here is the picture of the week: gorgeous colored glass lights hanging from one of the stalls of the Grand Bazaar in Istanbul. I was honestly transfixed by all the color and light there and the picture doesn't do it justice -- it was like staring through the stained glass windows at the Saint Chapelle in Paris. I brought home a small one for my bedroom, but it's just not the same!