Really Silly Verses

There once was a room by the highway
With views of the New Jersey skyway.
It came with double doors
And was up seven floors
In a bustling hospital hallway.

And those two doors were there for a reason
Having to do with the season.
'Cause in winter, you see
One might catch RSV
And end up with coughing and wheezin'.

Which fact is the point of this story
Without getting overly gory,
There are bugs in my nose
But that's just how life goes,
And I don't want my breathheads to worry.

I'm not happy, it goes without saying,
About how these cards have been laying.
I try to understand
This is part of the plan
Of this wonderful game we're all playing.

There are drugs, there are fears, there are times when
It feels like you simply cannot win.
But for now there are lights
From New York City nights
And tomorrow we're blessed to try again.

Of Bugs and Blessings

Okay, seriously, I think something out there is mocking me for complaining about not really having much to write about on the CF/transplant front lately. And when I say "mocking," what I really mean is "trying to drive me insane." Because there has been A LOT going on here in the past couple of days. And while it's mostly not what I would call particularly good news, it has served as a much-needed reminder of all I have to be grateful for, and everything I've gained since June. Which is, you know, never such a bad thing to be reminded of when you get right down to it. On the other hand, I think I've learned my lesson now.

Can we go back to boring, please? I promise I'll be good and not complain. Honest.

The first thing that happened, as you may or may not know from the blog, is that I had a bronch and got a little sick. That, in and of itself, is neither uncommon nor a particularly bad sign. Most likely it was more a reaction to the sedation anyway, and not any sort of indicator of overall health. Because the fact of the matter is I feel more or less fine, and I certainly felt great going into the bronch. If anything I've been living life a little too much lately (wait, is that even possible? But I know you guys understand what I mean). It just feels beyond amazing to be able to breathe, to have the freedom and the energy and the time to go where I want and do what I want and come home and not be run ragged. I can't even explain it. And that is definitely blessing number 1, since we're counting and all.

But anyway, I got a little sick (okay, fine, it was a lot sick - only it was for like 2 days instead of weeks, which is the big difference between now and before if you ask me), and then I got better. And then we found out that I had pseudo and aspergillus growing in my lungs - most likely stowaways from my CF lungs that had been hanging out in my sinuses and main airway, both of which still have CF of course - and we started treating those with IVs and nebs and orals, just to be safe. Which, of course, was all well and good especially for those of us in the "better safe than sorry because I no longer have an immune system to speak of because I (heart) my lungs and want to keep them" camp. Basically I was grateful to have treatment options, grateful to be able to do things from home instead of in the hospital, and grateful to have had 3 weeks off of IVs from Sept until October. All was right with the world.

A couple of days ago, my doctor called to tell me I had a new pathogen in my lungs - one not there (to any of our knowledge) before my surgery. This new bug is called Steno Malt, and interestingly enough another of my CF friends had recently cultured it as well. (Interjection: not only is this bug not passed person-to-person, but this friend lives out of state and I haven't seen her since my transplant, nor have we exchanged anything by mail. So unless the bug jumped out at me through her FB page, I'm guessing this isn't a cross-contamination issue. If bugs are spread via FB, on the other hand, the CF community is, in a word, screwed. Talk about giving new meaning to the term computer "virus.") Okay so fine, I have Steno Malt. The good news is that with my new lungs not having CF nothing is likely to colonize in there. So we discussed treatment plans, added some antibiotics, and I went on my way. No used crying over, um...spilled (Steno) Malt?

Fast forward to today, when I got yet another call from my doctor. Yeah, that's right, here we go again. Only this time it was a little weirder and little more creepy, to be honest. Apparently I have a mycobacterium in my lungs. No word yet as to which - only that it is NOT tuberculosis and NOT mycobacterium avium complex (MAC). I guess that means it may be abcesses, but again - we're just not sure. I am going to take the cautious route and have a CT to check for any possible nodules, which can be a byproduct of these bacterium and can, so I understand, cause all sorts of problems. Depending on the results of that, and the culture when it finally grows out, we'll devise a treatment plan (if any). In the meantime, I figure I can only make the situation worse by freaking out. Many of these myco strains aren't even really issues, and again, it's unlikely to colonize at this point from my understanding. Also, I can't stress enough that I don't appear to have an active infection right now. I'm feeling pretty good (tired from the drugs, but good), and my PFTs are totally stable. I'm so so grateful for that - and for the fact that my new lungs are still holding up beautifully despite this onslaught. Sorry, CF, you're gonna have to take your reign of terror elsewhere, because I'm not buying it. Welcome to blessing number 2.

I kind of feel peaceful about the whole thing, or as peaceful as it's possible to feel while still being (admittedly) a little freaked out. I have an entire team of great doctors who seem to be all over this - I even ran into my CF doctor yesterday and she's being consulted as well. Nothing makes me feel more secure than knowing that they have a plan (even if that plan is to do some tests and see...or even just wait) and that they're communicating that plan to me. The fact of the matter is that I trust them. A lot. And I feel very well taken care of, much as I may joke about them being hyper-vigilant. So right there: blessing number 3.

And finally, I realized at about 12:30 am this morning that yesterday, October 12th, was my 4 month transplant anniversary. Has it really been that long? And at the same time, has it really ONLY been 4 months? Wow. At first I felt a little sad that I had let the day pass without a celebration, to be honest. I mean, not that I need a reason to put on a fun dress and indulge in some really yummy food, but it's always exciting to have a special occasion, right? And then I realized that I hadn't noticed because I was too, well...busy? Happy? Not sitting around thinking about being "sick," even in the midst of all this madness? And that, friends, gave me more cause fr celebration than anything. I actually laughed out loud. To actually have a transplant anniversary is an amazing gift. To have a life so fun that you can actually forget about that amazing gift, if only for a short while, is, in a word: indescribable. Not that I would ever want to stop acknowledging and being grateful, but to have the chance to live so hard that I don't even think about my lungs? I honestly never thought I would type those words. Blessing number 4, and 5, and infinity. That one counts for everything.

Oh, and thank you, donor Bob. We make an exceptional team. (PS: Sorry about the pseudo, and the steno, and the myco. Wish I could make these silly bugs leave OUR lungs alone!)

So yes, I've learned my lesson. I'm back to counting my blessings out loud, which always seems to help when I feel a little overwhelmed, and to be honest I keep coming out ahead in my tally. I know how lucky I am to be able to look at this as a "rough patch" rather than the rest of my life. I know how precious and fragile this gift is - how much it is meant to be loved and enjoyed and cherished for exactly what it is...not a cure or a rebirth so much as a beautiful awakening. I know that God has given me this peace for a reason and that I should trust. Mostly I just know that I'm happy and busy right now, and so far it doesn't seem like the pseudo or the myco or anything else has been able to take that away from me.

I also know that CF sucks. You know, just sayin'.

Hopefully I'm well on my way to getting back to boring. In the meantime, I'll just try and enjoy the ride as much as possible. And hopefully I'll keep on forgetting what day it is, even as I try to always remember to be grateful for each and every one.

**PS: Please take a second if you haven't already to check out the links on CF/transplant over on my sidebar. I recently added two amazing blogs (A Breath of Fresh Air and Seven Stars) by two wonderful women with CF who also happen to be personal friends and role models of mine. Everyone else on there is great too, and please send me an email if you'd like your blog on the list. I'd love to check it out.**

Perfect (Kind Of)

Wow. Okay, so where to begin? For lack of a better intro, I'm just going to start with the important stuff:

I've been really, really sick.

Seriously, when I say "sick" this time, I really mean sick. As in, unbelievable-grossness-meets-super-sedated type sick. Or like...well, like CF sick, really, so I'm sure a lot of you out there know what I'm talking about. The high fevers, vomiting, lack of appetite, high heartrate, low O2, would-get-scared-but-any-emotion-just-serves-to-make-my-headache-worse kind of sick that no one ever really wants to go through. And I have to be honest, it wasn't particularly pretty. And of course it was compounded by the ever-amusing (except when they're not, in which case they are really, really NOT -- trust me on this one) effects of sedation. Wow. Good times to say the least. I think the only thing more fun than being hunched over the toilet while running a fever is being in that position and then looking around and not knowing exactly where you are or how you got there. Yeah, it was just that amazing.

That said, I survived. Thanks to the help of some very wonderful friends willing to put up with my randomly spouting nonsense due to the drugs and, of course, the passing of our good friend time, I was pretty much back to "normal" within 24-48 hours. Of course, the definition of "normal" right now for me includes both an aspergillus (fungal) infection and a pseudomonas (bacterial) infection. So no change to the plan, as I'm still doing the IVs, but now I get three drugs instead of one. Oh well. I'm kinda of the mindset that once you're on it really doesn't much matter -- might as well load the suckers up and wipe out everything at once, right? And the best news I got today (which is also, come to think of it, pretty much the best news possible...ever) is that the results from my biopsy are back and there is NO rejection. Question: is it weird that I feel an odd sense of victory over my own immune system? Not sure if that makes me the winner or the loser, honestly, but it means I get to keep breathing, so I'll take it.

As we used to say in college: major score, baby. (Which statement, by the way, has the unfortunate side effect of making my college friends and I look bad. We weren't normally this uncool. I promise. Kind of.)

Okay, so all that aside: the fact remains that for the past two nights there has been a sick presence in my apartment. On Monday night, without question, I took the grand prize. On Tuesday night, however, things got a little murkier.

The short story is that Sampson got the hiccups. I'm not entirely sure how it happened, though I think it was brought on by literally inhaling two organic mini milk bones. I'm pretty sure those things are just expensive crack for dogs, because Sam goes at them like a deprived junkie every time I head toward the treat closet. Which, to be fair to me, is less a "treat closet" than a "treat shelf" -- my dog is not spoiled enough to have his own closet, though I readily admit that the distinction is a fine one.

The larger point here is that, having acquired the hiccups, Sampson went what could maybe be politely termed "crazy." There are less polite terms, but I'm not going to mention them here, because they'll make my dog look like some sort of Dr. Jekyll and Mr. (Raw)Hyde. Suffice it to say that this was not good. In his panic, Sampson began by jerking around in a pretty solid imitation of a seizure, or an electroshock therapy patient -- by which I mean all-out, on-the-floor style convulsions. And, to be perfectly honest, he does this sometimes. (Yes, I'm aware that I am indicting myself for possible puppy neglect here, but it has, as a matter of fact happened before. My mom has even seen it. In my defense, the vet has declared him seizure free.) So I sort of wait the convulsions out, at which point he starts tearing around the apartment like a madman. And that's impressive, since the entire apartment is only three rooms and has hardwood floors. I swear that dog hit so many walls that if he wasn't prone to seizures before, he may be now. After which mad dash he ended up in the bathroom, he grabbed his bone for some hardcore gnawing, then ended up in my bathroom "digging" his way through the bathmat, and generally acting distressed. His grand finale included throwing himself on my bed and whining while trying to snuggle closer and closer to my body, and that continued until the hiccups subsided -- about 20 minutes later.

My emotions during this display ran the gamut from amusement, to concern, to near-panic, to cornering the dog and determining that yes, he did in fact have the hiccups and probably wasn't dying, to second-guessing that, to annoyance, and back to amusement mixed with a lot of relief when it finally did turn out to be nothing.

Okay so sick shorkie + recovering Piper = kind of funny, kind of crazy blog post. Case closed, right?

Well, maybe. And then again, not so much. Because the interesting thing about watching this whole performance was the realization that when faced with the hiccups, Sampson turned into, um...well...kinda...me?

Yeah, you read that right. What I mean to say here is that apparently, when sick, there is something deeper and gutteral that tends to take over, especially when we don't understand what's going on with our bodies. (Try as I might, I was unable to fully explain to Sam the nature of hiccups. I actually had him drink some water and "promised" him that it would make him feel better. It didn't. Then I remembered how much I hate it when people make promises about my health that don't pan out. Shame on me.) At any rate, the reaction Sammy had, though probably more physical and overt than what I would have done in a similar situation, was something I could definitely relate to on some level. I've FELT that need to run around and try to escape whatever is chasing me -- I've actually acted on it, though with lung disease that one is hard. I've FELT that need to grab on to something -- anything -- familiar and distracting. To retreat somewhere dark and lonely where I can go crazy in peace. And finally, of course, to whine and seek comfort. That one in particular made perfect sense to me. Right, mom?

Granted, Sam was suffering from a case of the doggy hiccups. I'm not going to sugarcoat things here: transplant is not mild indigestion. Far from it. But having so recently experienced my own first "real" illness post-transplant, and acknowledging that I really do have no clue how to manage this "new" body of mine, I couldn't help but empathize with my scared little puppy. Not that I'm claiming this is some sort of mind-boggling revelation or anything, but I really do find some comfort in knowing that maybe I'm not alone in my reactions to feeling out of control sometimes. Maybe we all need a little retreat into the bathroom to try and dig our way to freedom from the tiles. And once we're done with that, it's nice to have someone to curl up with -- preferably someone willing to listen to you whine.

The moral of this story (if there is one, I guess), is that I've now had my first bout of random sickness post-transplant, and Sampson has suffered the effects of his own gluttony, and believe it or not we've both survived. Not that it was easy, or fun for that matter. It wasn't. We both had our moments of being scared, of not understanding, and of flat-out wishing we could run away. (If it hadn't been for my migraine, believe me, I might well have tried to dig my way out of the bathroom.) As it was, we were both about as lucky and as blessed as anyone can be in this crazy life. We got better. Which fact leaves us with the chance to start it all (again) tomorrow.

Perfect. Kind of. Maybe? For now.

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.

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.

Tune-Ups, Adjustments, and Other Technicalities

Okay, since I'm pretty sure my post of a few days ago constitutes a renewal of my vows to my blog, I guess it's time to do my part and start sharing. And, just so everyone know up front, this isn't going to be the most of uplifting of posts. Nor is it going to be an angry rant. It's not going to be a litany of complaints (I ended my pity party last week -- sorry for those who never scored an invite) and it's not going to be a philosophical exploration of my latest illness-induced existential crisis. It's not even going to be very funny (gasp!), which for the 10% of people out there who actually think I'm funny most . . . um, well, make that some . . . okay fine, any of the time, might be a major disappointment.

This post is just going to be about my life. It's going to be about my CF. It's really just going to be, plain and simple.

The fact of the matter is that things are kind of feeling off lately. By which I mean . . . well, I guess it's kind of hard to explain what I mean. I think I mean that I've been sick, which in turn has led to more antibiotics and less energy, and also an increase in some of my other drugs that have, shall we say, less than awesome side effects. And as a consequence of this infection-ridden and drug-overloaded state, my body feels kind of, um, well I guess weird would be a good word to describe it. I feel exhausted a lot of the time, and I don't mean just the sleepy kind of exhaustion (although that's definitely part of it). It's kind of like my limbs are just heavier, and one activity is usually enough to make me want to lay low for the rest of the day. I have some other symptoms -- like the fact that I'm still coughing, have a high HR, and ran a low grade fever for part of the day today -- but generally speaking I don't feel bad, per se. I just don't feel quite right, either. I'm not sure what it means, but it's definitely affecting my ability to stay upbeat and push my way through this most recent bout of infection. Whether it's the lung stuff or the drug stuff or some other sort of stuff remains to be discovered (and, as we all know with CF, it might never be fully explained), but I'm hoping things are going to even out here over the next week.

And, of course, I'm well into week 3 now on IVs. This is the 5th course of 2010 already. Suffice it to say, I'm over the IVs. There's a reason they're called "tune-ups" -- you're just not supposed to need them this often. Period.

Uncool, CF. Really uncool.

The other side of the coin is the emotional stuff, and dealing with the fact that I seem to have hit a little bit of a transplant road block. Throughout the end of March and all of April I was getting calls pretty constantly (sometimes up to two a week), but lately I haven't been receiving, well, any calls at all. I know of course that transplants ebb and flow and that there can't be a suitable donor for me every week -- I'm certainly not blaming anyone or complaining about my center -- but it's still frustrating to go through that many dry runs and then hear nothing for such a long stretch. And it's also hard because my mother and I started living a little differently when we were getting all those calls. How could we not, after all? We started staying a little closer to home, planning our days a little differently, and even -- in a veritable fit of optimism -- making some plans for the future. Crazy dreamers that we are, we could actually imagine a time when the phone might not govern our lives, and distance from the transplant center might not be our number one criteria when planning a weekend. Meanwhile my sister has been putting off things like necessary business trips because she certainly doesn't want to be in Africa while her little sis is getting new lungs. In other words, going from thinking transplant was "so close we could taste it" (or, in some cases, so close we had actually said our "see you laters" and were in our respective OR/waiting room positions) to thinking once again that it "could be 2 days, could be 2 weeks, could be 2 months" is really a huge adjustment. Not one we can't handle, of course, but it's been kind of tough at times, not gonna lie.

Of course, there's a lot of good stuff happening right now as well. "Team Piper" (yep, I'm just that creative) is going to walk for the Cystic Fibrosis Foundation this next Sunday and has already raised over $4,000 for a cure. I am super blessed to have a ton of friends and family coming to walk with me -- my Godmother is even flying out from CO! My daddy came into town this weekend and he, my mom, and I walked from TriBeCa to upper SoHo today (call it practice for next weekend) where I promptly bought myself a very cool new present. And I've decided that as long as I'm not getting a call, I should get busy doing other things, so I've planned a couple of cool NY outings for next week and am hoping to start completing some projects I've had floating around in my head for a while now. To top it all off my dog walker is currently vacationing in Europe (NYC dog walking is clearly a lucrative professional choice), which means that I foresee a lot of outdoor bonding for the shorkie and me over the next week. Not a bad thing, for sure.

See, told you it wouldn't be totally depressing, right?

So there you have it: the physical, the mental, and the, um, other stuff. I think the long and the short of it is that I'm still waiting, and any transplant patient (or hardcore fan of 80s rock) will tell you that the waiting is the hardest part. I'm pretty sure I'm holding my own with it (most of the time), but that doesn't make it easy, and it doesn't make it all that fun. Luckily I'm also pretty sure that last one is something I can change with a little effort -- and maybe another step down on my Prednisone taper.

And here's to trying, beautiful people.

It's My Party

So have you ever backed yourself into one of those corners where you haven't done something in a while and then you want to get back to it but you want to make a return worthy of such a long absence and then you can't quite get inspired to really do something special so you just put if off even longer and longer and longer, thus perpetuating the whole cycle?

Um, yeah, me neither. Obviously.

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

I spared you the "little dry run that wasn't #1" where I never left my apartment because the lungs turned out to be high-risk and I am (thankfully) not yet at that point.

And then I spared you the "little dry run that wasn't #2" where I again never left my apartment because my body decided that it would be more fun to spike fevers and play jokes like a resting heartrate of 145+ instead of going into the hospital for yet another try at a transplant.

And then I spared you the inevitable starting of IVs that came after #2, because clearly if you're so sick they're refusing to give you new lungs it's time to call in the big guns.

And then, fast forward nearly a month, and I suddenly realized that I had pretty much spared you guys right out of my life.

Because truth be told life does go on, even when CF rears its ugly head. Since April 23rd I've not only had a few missed dry runs (none of the lungs were ever used, by the way, as if that's supposed to make it any better) -- I've also had a lot of other, non-sickness stuff happen too. I've had my wonderful father come back out from Denver, I've taken my fantastic mom to mother's day brunch, and I've even been able to enjoy some of the surprisingly non-gross weather we've been having in NYC lately (today, obviously, being the major exception -- it is truly gross out there).

So are you ready to thank me yet? Yeah, I didn't really think so.

I guess the simple fact is that if anyone can handle all my CF ups and downs, it's probably you guys, because by and large you've all been there too. And that, of course, is what makes the CF community so invaluable -- like any strong relationship, we're here for each other for better or for worse, in sickness and in health (and then, more often than not, in sickness yet again). We listen to the whining, empathize with the ranting, and even muddle our way through all the Prednisone madness, all while somehow managing to be grateful for the moments of pure, well, life that show up in the middle of all that other stuff. I'm just sorry that it took me about 20 days to remember how special that is.

So this may not be the monumental, witty, and healthy return I originally envisioned, but at least it is a return. And sure, I may still be on IVs -- and I may even have added a new med to the mix just this afternoon -- but I'm still here, still waiting, still breathing, and sometimes, even despite it all, still blogging.

And between all of that, I'd say this pity party is officially over.

Not a Clever Blog Post

Okay, so once again I find myself without any clever stories or deep thoughts, but with a nagging need to update this blog just to give you all some sense that I am still breathing, still surviving, still living (it's not the same thing), and still...well, waiting.

I am somewhat under the weather (which is rainy and gross), slightly under stimulated (because of the weather and the fact of being under the weather), and fully under the covers (at this moment, not all the time).

I am definitely over being sick (who isn't?), occasionally overwhelmed with the process (I'm told this is "normal"), and completely overjoyed for my friend Kelley (she received her new lungs Monday morning and is doing well).

I have emails to reply to, but I promise I still love you all.

I have phone calls that have gone unanswered due to coughing spells and breathless moments, but you will hear from me.

And no, the irony that I am keeping people waiting is not lost on me, in case you were wondering.

But, here's the deal: today I am going for a walk, I am going to do several treatments, I am going to rest and nap when I need to, I am going to pet my dog and I will let him win at tug of war, and then I will maybe read or write or work a crossword puzzle and chat with my mom and maybe a couple of friends. And then tonight, I am going to try and go out for dinner, just so long as the weather holds up and I'm not too much under it.

And then tomorrow I am going to go to my appointment, and rock my 6-minute walk test because I always do. The doctor will tell me that I need new lungs and I will agree with him. He will probably also tell me that I need to see my CF doc soon to get more IVs and I will agree with that as well. We will both be agreeable. And then I will go home and I will be exhausted and maybe coughing so hard again that my 30-minute frequencer session will end up taking over an hour (like it did this morning) and my mom will say (like she did this morning) that "at least you weren't up in the middle of the night vomiting" and I will say "yep, at least there's that." Because it's true. There's always something to feel good about, after all.

So the world will keep on turning every single day until I get this transplant, and then every single day after that, and who knows what will happen except that I will get up every morning try to remember that I am not surviving, I am living.

And as for today, I will go on a walk.

Get What You Need

I don't even know where to begin with getting back on track with this blog. I feel bad worrying people with lack of updates, but of course I'd imagined being able to come back with a bang -- some witty commentary on life or disease (or, even better, life WITH disease) that would leave everyone laughing, crying, or just shaking their heads at the wonder of it all. I had it all planned out in my mind, except, of course, for the actual words, which I figured would just flow like water as soon as I sat down and put pen to paper (er, finger to keyboard?). And let me just tell you all, readers, it was going to be great. I mean seriously, we're talking Pulitzer Prize for Best Writer of a CF Blog with a Not-So-Clever Pun in the Title -- they have a category for that, right?

But, to quote the Rolling Stones, "you can't always get what you want." So, instead, you're gonna get the truth.

Sorry.

See, the truth is that the past couple of weeks have been a little ridiculous. Oh wait, sorry, the real truth is that the past couple of weeks have been a LOT ridiculous. First of all because I had a CF exacerbation, as mentioned in the last post, and then because I went on IVs. Two IVs, to be exact: tobramyacin and merropenem.

If you have CF and are over the age of zero-to-very-young, most likely you've experienced antibiotics in some form, which means that you probably know that sometimes it's a toss up as to which is the lesser of two evils: the disease or the "cure." Because let's face it, a lot of the drugs we use to fight these infections are, well, evil. Brilliant, yes, but evil, and antibiotics (esp. in their hardcore, CF-dose IV form) are no exception to this rule. Hence the fact that the first few days of IVs are often likened to the feeling of, oh, say getting hit by a truck, or faceplanting into cement, or any number of other fun experiences that involve collision contact with really, really hard surfaces. In other words, IVs may be really helpful, but they're also really annoying.

I know what you're thinking: does she think I don't already know this stuff, or is she just going for the Pulitzer Prize for Best Writer of a really, really obvious CF Blog with a Not-So-Clever Pun in the Title?

The answer to that is neither (though if you know anyone offering out the latter prize, I'll be happy to accept!). See, turns out that while all IVs are annoying, they can sometimes be, well, a little more than that. And lately that's been the case more often than not for me, because unfortunately at my stage of CF and with my particular and individual presentation of this disease, I need to be on IVs pretty much more often than not. This is the arrangement that my pseudomonas/achrombacter has basically forced us into, and it seems to be working out except for one very minor detail:

The rest of my body.

Yeah, you know, the non-lung parts (also known as "all that other stuff" by us dedicated lungo-centrics). Apparently they have feelings too -- who knew? And those feelings are hurt by things like low potassium, vomiting, tachycardia, inflammation, and, well, you get the picture. When I say "hurt," by the way, I mean it literally. As I'm quickly learning, there is such a thing as serious pain associated with having severe-stage CF -- when people say that it's not BS, and they're not just whining.

It really hasn't been the greatest of weeks.

Obviously, I'm in pretty constant contact with my amazing doctor, and we're treating things as they come up. We've also switched my antibiotics around now because it seems pretty clear that at least some of the problems were stemming from a reaction to one of the drugs I was using -- it's not an allergy in the strict sense, and I think is probably just due to overuse. Like I mentioned, "all that other stuff" in my body is kind of acting on overdrive right now, because it's having to deal with the systemic effects of both chronic infection AND chronic aggressive treatment. Poor sucker doesn't stand a chance.

So it's day two of a new plan that now involves three IV antibiotics (but minus one particularly tough one for me) and some hardcore supplemental potassium. I already feel tons better, just after a full day, and I think things are definitely looking up. Which means that hopefully sometime in the near future I can get back to writing you all the more funny/entertaining/insightful posts that you want.

In the meantime, though, maybe we should all just try and get what we need.

Personal Health Update

Well, back on IVs starting tomorrow. I'm not feeling absolutely terrible, just coughy and tired and run-down. In other words, bad enough to want to feel better, and to know that I can't get there by myself. So I'm calling in some help, and hopefully sometime soon I can get back to feeling like this:


Yeah, yeah, I know. NO ONE looks at that photo and thinks "aww, doesn't that person look adorable!" It's okay, I'm used to playing second fiddle to this little guy.

Anyway, just wanted to give the brief health update in case I'm not around as much in the coming weeks. Assuming the antibiotics don't entirely kill what little creativity and/or brain power I have left I'll definitely try and make at least a few posts. In the meantime I'm going to focus on getting healthy and staying as in shape as possible until those new lungs of mine decide to join the party.

And I'm going to go take a really long, hot, needle-free shower.

On a more personal note, I have some amazing cousins who live in MN (um, sorry about the Vikings, Glo. If it's any consolation, I guess both our home teams lost today.). Last year Glo and her son Isaac climbed stairs to help find a cure for CF, and this year the whole family is getting in on the act. So much love and thanks in advance to Glo, Steve, Isaac, Sam, Lola, and Caleb. I'm sure I don't have to tell a blog full of CF readers how impressive it is that my family is willing to climb stairs to help make CF stand for "Cure Found." I mean seriously, talk about dedication! It makes me feel unbelievably loved and supported to know that even when this disease can feel so overwhelming, there are just amazing people out there standing with us in this fight. To the Beatty-Ruff family, and to anyone who has ever donated or walked or climbed or skiied for CF -- you are my personal heroes. (Even more so if you're also an organ donor!!)

Much love and goodnight, beautiful people.

(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.

Breathing Underwater

Anyone who knows me knows that I have a couple of weaknesses. (Well, okay, so maybe more than a "couple," but really, who's counting?) The first, if I'm being completely honest, is probably candy, but the second, and far more important one for the purposes of this post, is most definitely anything bright, shiny, and colorful. In college I had a friend who used to joke about my "inner raccoon" because of my tendency to be mesmerized by things that glitter, sparkle, or glow in any way. More than one trip to Vegas has included a story about me wandering off from my friends, only to be found staring at the slot machines (not playing, mind you -- staring). I sometimes think I chose to move to New York City not so much because I wanted to go to law school here, but because it has some absolutely amazing views of lights at night. And, of course, more recently I flat-out insisted that the Christmas tree in my apartment (where my whole fabulous family is gathering for the holiday, because they love me that much!) have multi-colored lights instead of plain white. It's not that I don't love elegant -- it's just that I prefer my tree to look like a rainbow that just happened to land in my corner, if possible.

My third weakness, believe it or not, is fish.

Yes, I have a thing about fish. I have no idea when it started, or how I got so hooked on those little swimmy creatures, but I have to admit that I am, in fact, an addict. I love watching them, love going to aquariums, and tend to get super over-excited whenever I spot them out in open water. And yes, I am completely aware that this is an odd little quirk of mine (particularly given the fact that I a) have been bit by a dolphin, and b) once had a seagull poop in my hair while visiting SeaWorld, either one of which you'd think would have turned me off to aquatic animals altogether), but frankly I just can't help myself. I mean sure, they're slimy and they smell bad, but they're also graceful and beautiful and, well, colorful.

So I guess given these combined obsessions, it's no wonder why I pushed so hard for my family to spend New Year's 2007 at the Great Barrier Reef in Australia. It's still a bit of a mystery to me where I picked up the original idea, but I do know for certain that my main plan for the trip involved me, a snorkel, warm summer waters (Southern Hemisphere), and a whole lot of gorgeous, wildly colorful sea life. Think all the spectacle and glitz of Vegas lights multiplied by 100, but without the cheesyness and theme-based restaurants, and you'll probably have a pretty good idea of what I had in mind.

What I did not have in mind, however, was CF.

I'm not going to go into the events that led me to get as sick as I did before that trip, but suffice it to say that I should never have gone. I was flailing, literally, and everyone around me could see it a whole lot better than I could at the time. All I knew was that this was my trip, my idea, my family vacation, and I was not going to miss out on it for any reason -- even a really, really good one. So I went, despite everyone's pleas and warnings and despite my own best interest, and trekked around Sydney despite mind-blowing pain, and I ate kangaroo despite having no appetite whatsoever, and I snorkeled, of course, despite not being able to breathe.

Well, sort of.

It would be more accurate to say that I floated, supported by multiple "noodles" because I was too tired to really swim and because I knew that at any moment I might have to yank my head out of the water and take out my mouthpiece to get a good breath. And in between these panicked gulps for air, I peered down at the magical world floating just below me and tried to make out the colors through the tears accumulating in my facemask. I made my sister stay right next to me the entire time, smart enough at least to know that if you're going to snorkel in the ocean while sick, it's best to use the buddy system. And I tried as best I could to recapture some of the excitement and wonder of previous snorkeling expeditions, knowing that I had risked too much not to at least savor the moment. Unfortunately, I guess I had to learn the hard way that it's hard to savor anything when you're feeling strangled.

Trust me when I say it's not a coincidence that no pictures from that family vacation grace the walls or bookshelves or nightstands of any of my family's homes. Honestly, it's painful even to write about, because it makes me feel silly and selfish and ashamed all over again. And yet, at the same time, I'm actually thankful in a weird way for the whole experience. I had definitely pushed myself too hard, let illness go too long, not been in proper control of my CF to the best of my ability, before "Australia-Gate 2007," but never before had I been so forced to face the consequences. For better or for worse, I can honestly say that I have never since put off contacting my doctor or those dreaded IVs, and remarkably I've found that most of the time this works to my advantage. True, I have to face the fact that something planned long in advance might have to be canceled last minute due to CF -- and that royally sucks sometimes, no doubt about it -- but at least I'm not likely to find myself half a world away from my doctor and drowning. It's an experience I'd prefer not to relive, I guess.

Or at least not relive entirely, because there are definitely aspects of that trip that still make me swoon. Namely, my family, the color, the beauty, and, yes, the fish. So the other day when I sat down to make a list of the many things I plan to do with my new lungs, is it any wonder that "snorkel somewhere beautiful" came in pretty close to the top of the list? And as I sat there writing that goal out on paper for the very first time since January of 2007, I was struck by one simple, yet amazing, fact: achieving it is entirely possible for me. Even likely. And this time I promise you that I will savor every second.

Because breathing is a miracle. Especially underwater.

Ode to Prednisone: A Late-Night Performance

Prednisone, oh Prednisone
Wherefore art thou . . .
so. freaking. annoying????

It's 1 am. I'm tired. Really. Honestly. Actually, "exhausted" might be a better word for it. I feel as though I could literally just let go and sleep for a day.

Well, I feel as though I could sleep for a day, that is, if I weren't on 40 mg of prednisone daily as part of a short "burst" up from my maintenance dose of 10mg.

Seriously, what is it about this drug that makes it both so effective and so totally, completely, beyond-a-doubt, and over-the-top, just plain evil?! I mean, don't get me wrong, I'm beyond grateful for the decrease in inflammation and increase in general breathing ability, plus gotta love the fact that I won't be blindsided by the steroids post-tx since I'm already way too familiar with them anyway, but still. Have I mentioned yet that it's 1 am?

This. Is. Ridiculous.

Bright side: thanks to my dear friend Mr. Prednisone (yes, prednisone is male, and with "friends" like these you definitely don't need enemies), I'm less likely to wake up coughing and needing airway clearance at 4 am, which has been my usual pattern as of late. So maybe I'll actually be getting more sleep? Hmm . . . seems unlikely, but I'm willing to grab ahold of it as a possible "silver lining" at this point. Okay, so I'm grasping at straws here people, but then again what did you expect?

I'm on prednisone, remember?

It's not as if all this extra energy doesn't have some advantages though, I guess. I mean, I enjoyed some serious quality time out with my parents tonight over a fabulous dinner and still had it in me to walk over to Times Square and pretend to be a tourist for a while. And of course this was after everything else today: after filming and playtime with Sampson and bonding with my dad over our shared sense of hypercompetiveness (let's just leave it at the fact that we got our game on in several different activities, and I unfortunately lost at most of them -- whatever happened to fathers letting their daughters win??). So you can see why my body is exhausted.

Yeah, it's just too bad my mind is too hopped up on drugs to follow suit.

Anyway, I've decided to put the energy where it belongs: into a quick celebration of everything that is so right with my life right now, because even the worst case of 'roid rage isn't enough to cancel out the fact that I'm blessed beyond measure. Blessed with an amazing, compassionate, and wise doctor who only uses this drug in large doses when I need it but who never hesitates when I do. Blessed with a dad who pushes me enough to never just "let" me win (but who loves me even when I lose). Blessed with a family able to come into New York for the holidays. Blessed with meds that work and veins that are still holding on. Blessed to have a great guy to take me to dinner tomorrow and a lot of fun planned for the upcoming week. Blessed to be almost 28 years out with my original lungs and to have new ones on the way through a great center. I am blessed, and I hope that I can cherish every minute of it for myself and for people like Ronnie and Court and Eva and Kelley and Sara and Sam and Libby and Beth and Patti and all the other cystics out there who I could never name but who inspire me all the time. I'm trying so hard to hold onto this knowledge tonight in the face of a deep loss for the entire CF community, but seriously, blessed doesn't even begin to capture it.

Luckily for me though, I'm totally wired (1:30 am and counting), so I have plenty of time to savor every blessed little moment.

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.

Dear Random Guy

Dear Random Guy Sitting Next to Me at the Movie Theater,

First of all, hello and welcome to my blog. You don't know me, and that's totally okay (a lot of people reading this have never even seen me in real life, so you've got one up on them at least!), but since you're here and reading you'll probably learn some interesting things about someone you previously just thought of as "that annoying girl sitting next to me." Things like the fact that I have cystic fibrosis, a genetic and totally non-contagious lung disease that, unfortunately, causes me to cough occasionally. Or the fact that I'm currently on home IVs, which means that I sometimes run a tiny bit behind schedule, which in turn means that I have to rush down to my seat in a crowded movie theater and might, just as unfortunately, be just a little bit loud and breathless about the whole thing. You might also learn, because I'm saying it right now, that it is never my intention to disturb anyone by any of these relatively annoying byproducts of my disease. In fact, when I'm really sick and coughing up a storm, you can bet that I would make a point of staying home and avoiding quiet places like movie theaters. Not because I'm a danger to others, mind you, but simply because it's no fun to cough your way through what should be a silent experience.

Okay, so now that you know a little bit about me, I want to apologize. Yes, that's right, I would like to sincerely apologize to you and your girlfriend for what happened at this afternoon's matinee. See, when I rushed in after a CF-related delay, bounding up the stairs into the theater to take my seat before the start of the actual movie, I knew it would likely make me cough a little bit. And I knew, equally, that the film was just beginning and that people around me might not be entirely pleased. I did not know, of course, that anyone would take it upon themselves to voice the opinion, out loud, that "people who are sick should just stay f-ing home" (censored for the benefit of other readers, but then again we both know what was said), but I guess I could have been better prepared to give an appropriate response.

Instead, I snapped. I leaned over, as you know, and hissed in your face that my condition was genetic, akin to asthma, and nothing that you needed to worry about catching. And I'm ashamed to admit that I didn't say any of this politely; I didn't shrug it off and turn the other cheek, knowing that your comment was said in frustration and ignorance of the whole situation, nor did I calmly touch your shoulder and whisper that it wasn't contagious or that I would be happy to explain after the film. I didn't even pause to consider that perhaps you have your own valid reasons to fear germs, and were simply voicing a concern about being stuck next to someone with a possibly contagious illness. Nope, I did none of that, but I did make myself heard, and for that -- for the way I did so and the hostility in my voice -- I am very, very sorry.

You see, the thing that's hard to explain is that, for someone with chronic illness -- and especially a sometimes loud chronic illness -- the little comments and eyerolls and other most likely well-intentioned little gestures (cough drop, anyone?) can sometimes get a little overwhelming. Not that such an explanation explains or excuses my behavior because, quite frankly, it doesn't. After all, your comment was offensive to me precisely because it failed to take into account the totality of my situation, and yet my response was in fact no better. I responded to you without much thought for your feelings, your experiences, or even the best ways to spread CF awareness. Sadly, in doing so, I may not only have lowered myself to bad behavior, but I might also have missed a chance to truly educate someone about CF. Because this time, when the credits rolled and the house lights came back on, we both shuffled out without a word.

I am truly, deeply sorry.

So, Random Guy, I hope that you went home tonight and thought a little bit about that awkward interaction you had with the annoying woman at the movie theater, because it's been on my mind all night. True, I'm not proud of the way I acted, but I can hope that I've learned at least a small lesson about taking things in stride and not responding in anger, especially to strangers whose stories I don't know. And perhaps if you take the same lesson away from this all, then our meeting might not have been wasted after all.

And we can both be a little bit more proud of ourselves next time when the house lights come back on.

Sincerely,
Coughing Girl

(Poly)myxin it Up

Alright, we've had the break DOWN, so now let's mix (or maybe in this case, "myx") it UP, shall we?

Here the situation: yesterday, after starting IVs the day before, I realized that the combination of fevers, chills, being exhausted, and coughing up tons of goo probably didn't bode too well for my ability to effectively manage and stay on top of my CF care at home. I knew I needed a little extra umph, and my doc agreed, so I went ahead and checked into my beloved Columbia Presbyterian and ended up here -- about 10 miles and the length of an entire (rainy) island away from my puppy and my apartment.

It's been non-stop ever since.

Okay, I really hate to complain, but let me get this one off my chest (and yes, bad pun intended, deal with it). I am in, quite possible, the most ridiculous hospital room ever. Seriously. It's an isolation room, but not just contact isolation like they use for most CFers. Oh no, this is full-on, hardcore, "wow, she must have swine flu AND tuberculosis to warrant that kind of lock-up" style room. There's not even a window to the outside in here, as if I might accidentally infect the boats on the Hudson were I allowed to see them. Although, to be fair, the room does *technically* have a window. It's just too bad that said window a) is covered by a large cabinet of sorts, and b) looks out into a weird hallway that appears to serve as some sort of storage area for unwanted hospital chairs. Weird.

Alright, so I'm admitted, and there's NO WAY that I'm infecting ANYONE within 50 miles of this hospital with Pseudo (at least not yet -- apparently I'm first in line for a room change as soon as one becomes available, for which I am totally grateful). Sounds good, right?

Well, sort of. See yesterday they made the somewhat difficult, but probably necessary, decision to actually STOP the antibiotics. They stopped the drugs, waited for my fever to spike, and then drew blood cultures that were free of anti-infectives and should show exactly what's going on just in case this isn't a classic lung infection. I think, although I'm not entirely sure, that they did this mostly because of the weird, still-unexplained fevers I had been running a few weeks ago, and the off chance that this is some sort of relapse of that problem rather than what it likely is, which is yet another fun CF exacerbation. So the downside of all this was that last night I had to go through more fevers, and I coughed all night, and I had to spend a night in the hospital without receiving any treatment, which is always frustrating. But at least if those cultures are negative (and they most likely -- finger's crossed -- will be) then there won't be any more talk of removing my port.

So today is a new day, and they just now restarted my antibiotics. I am now on tobra and a new drug: polymyxin. Here's the Antibiotics for Dummies version: the polymyxin family is most commonly known to CFers because of Colistin, which a lot of use as an inhaled antibiotic. I'm not on IV Colistin, but a slightly different form, known as Polymyxin B. Consider it for all intents and purposes the same thing. Basically, this drug (as I understand it) is pretty much the ultimate in last-resort drugs to treat PA. "Ultimate" because it's normally very effective. "Last resort" because it's also known to be pretty neurotoxic and nephrotoxic. The nephrotoxicity doesn't tend to be a problem with patients who have normal kidney function to begin with, I'm told (and thankfully I do), but the neurotoxicity can cause pretty unpleasant side effects in some patients, ranging from tingling and numbness to confusion and hallucination. Yikes. The good news? Well, first there's the fact that I'm in the hospital, so I have people here to monitor and help me. And second, well, the effects (both neuro and nephro) tend to go away upon discontinuation of the drug, which has a pretty short half life (a few hours). And finally, well, the only reason we're forced into this drug is my allergies, and not insensitivity on the part of my PA, so if the side effects are too uncomfortable after a dose or two, I'll simply stop this IV and get desensitized to zosyn or ceftaz, which I'm told is a pretty straight-forward process.

I'm not going to lie, I'm a little nervous. My infectious disease doctor ordered the dos (which is normally once a day) broken down into two doses to try and avoid any reactions. That's a good thing, except for the fact that each dose lasts 4 hours because he's also ordered it diluted in a TON of dextrose. But if I tolerate it well then yes, I can go home on it, and while it would likely be a pain to be on an IV that long, it's better than the hospital.

Not everyone has side effects so hopefully I'll be lucky. And hopefully this drug will do the trick and I'll be able to go home on Monday or Tuesday. Weekends around here are always pretty boring, but finger's crossed that I'll at least have a window sometime soon, so I can at least watch the river during my 4-hour IV drip.

The Joy of Cooking

What do you get when you take one cystic, add two months of fevers and acheyness, throw in two courses of IV antibiotics, add a dash of hospital stay and some of the best doctors in the country, mix it up with two very fun and awesome weeks in Turkey, sprinkle on a dash of pretty much every single medical test ever imagined and then let bake (at a pretty much constant temperature of 101 degrees or higher) until covered in a very creepy, weird cluster of reddish-brown rashes?

Apparently, actually, you get a perfectly healthy Piper.

Yeah, I know, I don't get it either. Then again, I've never been much for cooking, although I will admit to a slight addiction to Top Chef. But I AM a lawyer (recent "retirement* from active practice aside -- once a lawyer, always a lawyer), and that should make me a pretty logical person, or at least one used to getting surprising results from odd facts, and for the life of me I still can't figure this one out. What I DO know is this:

*I had one additional 101+ fever on Wednesday night, after writing my last blog
*After said fever, which of course occurred at night and was surprisingly difficult to break, I emailed my doctor and basically said "enough! I surrender!"
*My extremely wise doctor decided to wait a couple of days, order a few more necessary tests including blood cultures while off the antibiotics, and in the meantime allow me to remain in the comfort of my own home.
*Two days later (and two days fever free), my tests were all negative, once again indicating that it was some sort of virus, and my abdominal CT scan showed only two abnormalities: a slightly enlarged spleen, and a missing appendix.

I have to admit, I'm relieved about the appendix. As someone who got the chicken pox TWICE, once caught the flu twice in the SAME SEASON, and may well have gotten mono TWICE if the past two months are any indication, I really wouldn't have been too shocked if my body had decided to grow another one of those suckers just to treat me to the joys of yet another appendectomy. Seriously. That's just how I roll.

Whew.

Yeah, so anyway, the spleen is still ever-so-slightly enlarged, but the fevers seem to have gone the way of the appendix at last, and not a moment too soon if you ask me. Which, of course, nobody did, but you are reading my blog, after all.

Today I spent the day enjoying what I would call "mild reality." I left the house several times, twice to take short walks with my puppy, once to walk to a neighborhood restaurant for a yummy lunch, and once to visit the very famous FAO Schwartz toy store to pick up some board games, puzzles, and cards. Yeah, that's right. I figure if I might be stuck inside some days/nights/hospitalizations during the wait for these new lungs, I may as well have some way to make staying in a social occasion. You know, as prepared as any 27 year-old who just blew over $100 at a toy store can be.

Um, yeah.

So tomorrow is going to be a slightly less "mild" dose of real life, involving actual brunch plans with friends (*gasp*), some relaxing sunbathing with a couple of girlfriends on my roofdeck, more dog walking, and a trip to the gym during which I solemnly vow not to expect too much of myself (but still to make genuine effort on the treadmill for at least 20 minutes).

In other words, what do you get when you stir all those things (and more) up and let the whole mess sit for the better part of Summer, 2009? Well, for better or for worse, I guess the answer is life, although I'm sure most of you cystics out there probably already knew that.

Maybe I'm not such a lousy cook after all.

Everything You Ever Wanted to Know about My Lungs (But Were Afraid to Ask)

So instead of random poetry and cryptic messages tonight, I thought I'd give you a general update on me and explain why my blogging etiquette may be a little wishy-washy for a little while.

If you've been following this blog, you'll know I had a lot of issues pre-Turkey, some of which were likely attributable to low potassium levels and some of which were really mysterious. My PFTs were down a bit from baseline and my cough was up, so we did a course of IVs, ordered just about every "just in case" script you could possibly need for a trip oversees (TamiFlu, Cipro, Levaquin . . . ) and even made sure I had the name of CF doctor in Istanbul. Then off I went.

The trip was phenomenal, despite the fact that I began running fevers and having severe soreness in my arms and legs about halfway through. Because I had started the cipro, though, I had managed to get a bit of sun rash even with SPF 75 (no joke), so I attributed the fevers/aches to a mild case of sun poisoning. I had pretty great energy aside from the pain though, and I spent the days sea kayaking, swimming, hiking, and generally tromping around the country, using O2 as needed. Basically I felt "better," despite the night fevers, sweats, and aches, than I had in a while.

Fast forward to coming home, where I more or less tanked. I was coughing up TONS of mucus despite being strictly compliant on my trip and was exhausted and really achey. Weirdly, though, my PFTs were up at 38%, which is very decent for me, but obviously a number isn't everything, so we started IVs, ran blood cultures, and tested for a virus called CMV, as well as flu and swine flu just to be safe.

Two days later I'm negative on all the viruses, I still have fevers of 101-102 daily, and I'm sleeping ALL day and sore as can be whenever I'm awake. At that point we noticed the low potassium levels so I got permission to just take advil round the clock and get the potassium back up to normal, in the hopes that this was a virus and would resolve as my body got healthy enough to fight it off.

No such luck. Two Sundays ago, in a last-ditch effort, I switched from Merrem to Imipenem, but by that Monday, after two weeks of fevers peaking at around 102.5ish, I went into the hospital, where I had every test under the sun and was found to have: 1) an enlarged spleen, 2) anemia, 3) low potassium (knew that), 4) elevated liver enzymes, 5) some residual pneumonia (remember, this is 2 weeks into IVs), 6) a collapsed upper left lobe (and okay, that last one is no big deal for me because it seems to happen intermittently and resolve itself), and 7) a fever of 102.6. Surprisingly though, after the initial day in the hospital, my fever never spiked above 99.5 again. My pain started to resolve and things were looking up. All blood cultures were negative and all virus/parasite tests came back clear. Infectious disease wanted to pull all my abx to get a blood culture without any drugs in the system (which might mask the problem), but my CF doc wasn't comfortable with that so I remained on tobra and imi. I also had an echocardiogram to rule out a heart valve infection. This was, of course, in addition to the two CT scans, abdominal ultrasound, and various other tests (mostly blood draws). They also tested my sputum for pretty much every weird bacteria under the sun, and so far no dice (except that I did learn from infectious disease that I have "dozens" of pseudo strains in addition to my staph and achromobacter -- lovely).

Okay, so no fevers in hospital = jailbreak, right? YAY! I got out last Thursday, went home, had some peanut butter, took a nap, and woke up with a fever of 100.5. No joke. Plus I had some weird rash all over my legs, so of course I called my doctor. The end result: benadryl for the rash, keep an eye on the fevers.

I ended up back in my clinic today after running daily fevers of 100+. Here's the deal, the fevers appear to be getting milder. The red splotches all over my skin aren't too concerning. My spleen, potassium, and liver are back to normal. We think this might be FINALLY resolving, EXCEPT: I still have fevers, I'm still sleeping all day, and I still ache like nobody's business. We ran more blood cultures, she wanted to admit me, I asked if it was really necessary since I have someone living with me right now (shout out to my awesome godmother, who flew in from CO to take care of me!), and she said it was fine to stay at home where I'm more comfy.

Here's the plan: IV abx through wednesday and I can take tylenol to break the fevers. Starting inhaled colistin. Discontinuing all oral abx including zithro. This way, IF the fevers persist past Wednesday and the stop of the abx, we can draw more blood cultures without the risk that the abx in my bloodstream are hiding the infection. And then possibly my port will need to come out.

The good news? We've eliminated literally everything they know of that might be super serious. If it is a blood infection (please pray it's not), then it hasn't spread to my heart valves, which is fantastic. It's entirely possible this is just a virus they don't really know much about, similar to mono, and it may even BE mono except that I had it years ago so there's no way to definitively test me for that now.

The bad news: if the fevers aren't gone by Friday, viral or not, I need to go back into the hospital and wait this out. And if they truly can't find any cause they may remove my port just to be safe, which is annoying as hell. Then again, seeing as I've had the thing for 9 years there's actually a good chance it's part of the problem -- they rarely last that long without some sort of issues. I'm really hoping if they remove it they'll allow me to get it back in my arm; my doctor promised to work on that.

The awesome news: I'm still at home with my puppy and typically only having one fever per day. Once it breaks, which it does with rest, gatorade, and tylenol, I've been cleared to do some light walking to keep my strength up and regain some of my lost workout time. Nothing serious here, obviously, but honestly I never knew walking a shorkie down a crowded lower-manhattan street could be so much fun. And tomorrow I'm going to meet my friend at the best pizza bar on Wall St. for lunch, assuming I'm up for it, so life is slowly, slowly regaining SOME sense of normalcy. AND, despite the collapsed lung that hurts like the devil everytime I breathe in (you get used to it, believe me) I suddenly have O2 sats that actually reached 97 on room air! They're hovering around 94 now all the time, which is unbelievable, amazing, wonderful, and beyond all words. I can't wait to see what my PFTs are when this is all over, considering they started at 38%.

Alright, sorry for the symptom dump. I just thought I'd fill everyone in just in case I go AWOL again, or on the off chance any of you were torn to pieces by my sudden lack of fun updates. I'm pretty sure there's more fun to be had though, even if it does have to wait it's turn through all the madness.

sick cystic, dozing dog

say that 10 times fast anyone?

still feverish, still waiting for answers, and now stuck inside on a rainy day. (and by "stuck," i mean cuddled up with my puppy on my sofa watching him dream and staring out at the cloudy skyline -- is New York the only city that looks gorgeous when grey?) anyway, i did learn this morning that it is NOT a heart valve infection, and the resulting deep void of answers led me here. enjoy.

without answer

what if this is just another normal,

another winding road without a name,

and what if i just simply journey forward,

reflecting on the path from which i came?

what if instead of cursing at the darkness,

i look around to watch the fireflies,

and turn my glances upward for a moment

to see the starlight dancing in the skies?

what if instead of waiting for disaster,

or some too-easy rescue that won't come,

i simply trust my feet to carry onward

and pray that in the end they lead me home?

and if i must accept the path is changing,

sure footing turned to dirt and wet like clay,

at least i can be grateful for the walking

and reach out towards the hands that guide my way.

and if in fact two roads meet in the forest,

and both are known to lead to the same plight,

then maybe this road too has been well-traveled:

one hundred thousand tries to get it right.


(and ps: for those of you out there who prefer the [relatively] funny piper, she's still here i promise. good vibes for a mellowed-out fever would totally be appreciated, though. she tends to prefer to do her turn in the body when the weather is a bit cooler in here!)

Welcome to the Hotel Presbyterian

Such a lovely place, although I'm hoping I can both check out AND leave -- preferably by the end of the week.

Yeah, I landed back on my ass in 9 Hudson South (actually the best ward going over here, with a menu for ordering dinner, private rooms/bath, and guest internet). The twist is that we don't actually think this one's all about the lung infection, or at least 2 1/2 weeks of solid 101 degree fevers daily says this isn't your average pneumonia. We're thinking viral, but they're not ruling out an infection that we're just not hitting yet . . . including the ever-dreaded port infection. Fingers crossed it's not that, although other contenders include such fun little visitors as Lyme Disease or a relapse of Mononucleosis.

I'm just hoping that whatever it is, it starts to resolve itself FAST.

They've already switch my abx from Merrem to Imipenem, which they did over the weekend at home and may already be making somewhat a difference. Also my potassium levels are climbing, which is wonderful. So basically I'm in here, according to my doctor, "for a couple of days" (famous last words!) to get some tests run and hopefully figure this thing out for good. It's been bothering me for waaaay too long.

I didn't even put up my usual crazy stubborn (um, I mean, polite and well-articulated) fight to stay out of the joint this time. Sometimes you just know you're headed for those plastic sheets, and the best that you can do is just grit your teeth and bring your own comforter. As my blogger cyster Cystic Gal might quote, "you got to know when to hold 'em, know when to fold 'em". . . and hope to God that your doctors know when to let you walk away ;)