For The Rest of Us

I'm sure by now most of you have heard the news.

For those who don't yet know, however, today was a watershed day for the CF community. For a long time now, CF research has been aimed at what Dr. Beall, the President and CEO of The Cystic Fibrosis Foundation, likes to describe as "long shots on goal." Now I'm no sports junkie by any stretch, but apparently this means taking a chance and focusing your energy on getting things done, even if it requires a little faith and courage to get you there. And today, with a little assist from the FDA (like that, guys?), it seems the CFF and the entire CF/medical/research world scored a huge goal, touchdown, home run, or whatever the heck you call it when you strike a MAJOR point against a seemingly undefeatable opponent. Because as of today, January 31st, 2012, the FDA in the United States has officially approved the use of Kalydeco (aka VX-770) for the treatment of cystic fibrosis in patients with the G551D mutation.

Wowza.

If you don't know what Kalydeco is, let me explain briefly. This is a drug -- the first of its kind -- that actually targets and corrects the underlying defect that causes CF. Simply put from a non-scientist, it opens up a chloride channel that is typically blocked in CF patients with a specific mutation. Granted, not all CFers have this mutation. In fact, it represents only about 4% of our population. Which means, given that there are about 70,000 cases of CF worldwide, that this drug ONLY offers the opportunity to SAVE THE LIVES of about 2,800 people. It may work on others as well, but as of now it will ONLY allow 2,800 beautiful souls to grow up, live life, marry a loved one, start a family, have a career. It will ONLY save about 2,800 families and communities worldwide the pain of losing a child or a young adult. And this approval ONLY means that about 2,800 pairs of eyes, and the eyes of all their families, friends, and loved ones wept with joy this morning when the FDA made its announcement. Only that.

As for me, I don't have the G551D mutation. I know people who do, wonderful people whose lives have in some cases already been completely changed by this new development, but I also know a lot of people, like myself, who don't. It remains to be seen whether this drug alone or in combination with other medications might work even part of the miracle it promises for that 4% on other members of the CF community. Right now that question, like so many others in CF, remains unanswered. And while this drug represents the raw hope and truth that the CFF has now PROVEN that this disease can and WILL be conquered someday, it is fair to say that this morning's sweet announcement also carried with it the bitter reality that some of us, without question, will still die waiting for our chance.

So what does this mean for the rest of us, then? Because honestly folks, I've been there. When I was first referred for pre-transplant testing in 2008, this new class of drugs was really just a glimmer on the CF horizon. I remember so clearly the thoughts I had back then: that maybe if I could move somewhere with one of the study trials my lung function would miraculously make the leap and allow me to participate; that maybe if I could just hold out one more year we'd make it to a miracle and I could wiggle my way in on compassionate care or extended access; that maybe if we could just speed it up, even by a month or two, this would all be MY chance and I could stop watching from the sidelines and start living this wonderful new reality that seemed so darn close I could have reached out and touched it if I'd only been given the opportunity.

Yeah, I remember that.

Obviously, you guys know the way that story played out. I got tested, was eventually listed, and became one of the lucky ones fortunate enough to walk the precipice all the way to a double-lung transplant, at which point my life was saved by a completely different type of miracle. I was, and am, beyond blessed in this reality, but I have to admit that it didn't always feel that way to me in the moment. The simple truth is that it's hard when breakthroughs that represent so much hope and that deserve such intense celebration don't quite mean for you what they might mean for others. It's hard, as much as it is wonderful, to watch as other people receive a "cure" for your disease -- just as I can only imagine how hard it is for the parents or spouses of my friends who didn't make it to transplant to read this blog. In some ways, the bitter truth is that nothing hurts more than the miracle that comes too late.

I can honestly say for myself, though, that the only way out from the sidelines was to throw myself back into the game, and this time to try and be a stronger player than ever. What I realized through my transplant is that there are a multitude of ways to save a life, and it is always a team effort. There's nothing quite like being on a waiting list for lungs with some of your favorite people in the world, or knowing that in order for you to live another beautiful soul will have to leave this world, to make you think twice about what it means to take care of each other -- to have an assist when deep down you're also waiting for your own "long shot" at the goal, at glory, or even at life. And oddly enough, I know now that had I gotten my wish and had that chance at a miracle shot, I would most likely never have been healthy enough to fight as hard as I did for the goal we just made.

Which is why, when I heard about Kalydeco's approval, I cried too. I cried for my friends who have this mutation and what it might mean for their lives and their health. I cried for the many, many workers and volunteers and researchers whose efforts have made this possible. I cried, of course, for the families who will benefit, and especially for the younger generation who might never need to experience any of the flat-out bullshit that comes along with this disease. I cried for my fellow lung transplantees and the lungs or the lives that might have been saved. And I cried for all the amazing CFers and families out there who assisted in this shot, who are heroes -- every one of them -- in their own right, and who are still waiting for the moment when they, too, will get the chance to score. I cried, as it were, for all of my teammates, past and present and future, and for the knowledge that this goal represents a huge score (and a promise of victory) for each and every one of us.

Congratulations, teammates.

What Are You Reading?

Because I've been reading a LOT of really great stuff lately. Stuff like, oh, this for example:

Not Just a Cheerleader: Foundation Helped Drive Cystic-Fibrosis Research

and this:

Vertex Submits Application for Priority Review

or even this:

New Drug Targets Cystic Fibrosis

Talk about a fun mid-semester reading list, right?

As most of you already know, Vertex Pharmaceuticals announced a couple of days ago that it was submitting its new drug Kalydeco (formally known as VX-770, though I still think they should have gone with Em Schaller's and my suggestion of "Blue Lightening") to the FDA for priority review and approval in the treatment of cystic fibrosis patients with the G551D mutation. In English, this means that Vertex, a large pharma company that has been partnering with the CFF for several years on the development of this and other CF drugs, has asked the FDA to approve a new drug that targets the actual defect of this disease for about 5% of CF patients. The 5% in question all have copies of a specific gene mutation that causes a channel blockage in the CFTR process. This drug opens the channel, and that is awesome.

What's even more awesome, though, is that this drug might actually have a wider application than 5%. Right now we KNOW from clinical trials that Kalydeco is effective at helping folks with this specific mutation. What we don't know yet, and what the CFF and Vertex are now working tirelessly to find out, is whether the drug will work for other patients with similar mutations (commonly referred to as "class 3" mutations), whether the drug may be useful in the treatment of so-called "milder" CFTR mutations ("class 4" and "class 5" mutations), and whether the drug might be able to be used in combination with other drugs to treat more common mutations (including DF508, the most common CFTR defect and one that is officially considered a "class 2" mutation). We don't know this stuff yet, but the folks behind this new drug are already on it, and new trials are launching to figure out the answers.

So, you know, if you have CF and feel like being part of something truly magical (and maybe giving your lung function a serious boost in the process!), check out the CFF website to see if there are any relevant trials near your clinic: Find a Trial.

Because this stuff can't happen without us, guys. I promise.

I have to say that it's fascinating to me whenever "my" disease starts appearing everywhere in the media. As a CFer, I've gotten pretty used to being somewhat under the radar when it comes to common medical knowledge. I never tell anyone I have CF without having my explanation of the disease ready, and I'm certainly not prepared to tell someone and have them respond with "ooooh, yeah, I read about that the other day in 'The Cape Cod Times.'" It's a new experience for me and, I'm not going to lie, at least a little unsettling. But it's also revolutionary, in many ways, because what this means is that the world is taking notice of us: of a (relatively) tiny little orphan genetic disease that affects about 70,000 people worldwide, many of whom will die fairly young. And the only reason people would do that, guys, is that we are creating miracles. We are giving them something to believe in, to hope for, and to strive toward. Or, to put it another way, we are giving them answers to questions they never even knew they had.

Take this article for example: Smoker's Lungs Similar to Those of Cystic Fibrosis Patients. This made its way around FB the other day and I snatched it off my friend Amy's profile. The interesting thing here isn't really that smoking makes your lungs suck (we knew that, right?), but more that smoking makes your lungs suck and CF research can help. Recently CF has been all over the place because the stuff we're doing -- the money we raise and the research it supports and the science that creates -- has implications for all sorts of disease out there. Genetic ones, yes, of course, because NO ONE has EVER cured a genetic disease before, and we are so so so close to being the first. But other diseases as well, because just as I now know several non-CFers who inhale Tobi and use the Vest on a regular basis, it's clear to all of us at this point that pushing the envelope on CF treatment to the extent we are all doing, every single day, is going to change the way people think about medical research. Period. And that, quite frankly, is the sort of news I absolutely love to read, and the sort of thing that makes me -- dare I even say it? -- super proud to have CF and to be a part of this community.

So we might be a super small group of people with a super weird set of genes that is super easy for the rest of the world to ignore, most of the time. But I'm happy to say that we're also super dedicated, because we get stuff done. We're super persistent, because we don't take no for an answer -- whether that no comes from a set of doctors who said we'd never live to graduate high school or a set of pharma companies who said we'd never be profitable. We're super loud, because we know that it's not so much the size of your crowd as it is the strength of your voice and the truth of your message. And we're super close, because let me tell you guys: it's working. And if you ask me how I know that now, well, I've got a seriously good answer.

I read it in the paper.

Ask Not What a Cure Can Do For You

I like to come on here every once and a while and brag about my family. This is, of course, partly because my entire family is pretty much awesome, and I love them.

The other reason I like to brag, though, is a little more selfish. You see, I happen to have a family that is 100% committed to combating, controlling, and yes, to eventually curing cystic fibrosis. Not only do these wonderful folks donate money any time they can to this cause and support me through thick and thin with the disease, but they also serve as volunteers with the CFF, they get out and walk to raise awareness, they spread hope and prayers and love to just about everyone in their communities (but also to families dealing with CF), they serve on boards and chair committees, and perhaps most importantly: they continue to ask.

My father likes to say that "making the ask" is the most important part of curing this disease. Recently I heard him speak at the CFF's Greater New York Chapter Bi-Annual Scientific Update. This was a wonderful event put on by the amazing staff at the GNY Chapter to help answer questions about the sometimes crazy weird science that accompanies this disease we all consider to be a part of daily life. The program featured CFF Vice-President for Clinical Affairs, Dr. Bruce Marshall, and Emily Schaller (founder of the awesome Rock CF Foundation), both of whom gave testimony to the amazing new class of drugs out there that will treat the underlying defect of cystic fibrosis -- that amazing triple-punch combo of Ataluran (nonsense mutations), VX-770 (currently for G551D, though may have wider use), and VX-809 (DF508); and the new drugs like VX-661 that are just entering development.

These are awesome new drugs guys, and they work. We know that. We know that they do at least some of what they are supposed to do. We know that we are closer than ever to figuring how they work best, who they work for, and what might work better down the line. To that end, the CFF continues to do studies, pledge money, and move forward in solving this equation that we worked so hard to figure out in the first place.

I say "we" there because this really has been a collective effort. 56 years ago when a group of CF parents and loved ones came together to say they'd had enough of their children's fatal disease being ignored or unfunded, one might have said that it was them against the world. As recently as the early 1980s, the CFF had a working budget of about $1 million dollars to wipe out one of the most deadly and most common genetic illnesses in the US. The numbers have grown quite a bit since then (and, not coincidentally, so has the science and the treatments and the life expectancy), but one thing hasn't changed at all: this is still a community-based effort -- OUR effort, guys -- that relies 100% on donor funding.

So WE are super close, but WE'RE not really there yet. There's a lot of stuff to be done, including studies that take a lot of money and a lot of help. And that guys, is where WE get to come in again. Because something my dad said in his speech the other night really stuck with me, and I think it's worth sharing here:

"We are no longer looking to invent the lightbulb or to find the light at the end of the tunnel. We've done that. All we need now is money to fuel our lamp. And we have to realize that in our future either one of two things is going to happen:

Either we will have to end each day knowing that someone out there is saying goodnight and goodbye to a child, a sibling, a loved one, or a friend with this disease;

Or together, WE can say goodnight, goodbye, and GOOD RIDDANCE to cystic fibrosis."

I think right then was the moment I decided to stop asking what a cure could do for me. Instead, to paraphrase our late President John F. Kennedy, I think it's time we start seriously asking ourselves what WE can do for a cure. And hey, while you're asking yourself, why not ask a couple of friends as well? And why not ask them to ask a couple of others? Because I'm pretty sure most of you guys out there have friends and loved ones, and I'm pretty sure most of them do too. And I'm sure you get where I'm going with this, because while no gift is ever too small to accept, it is equally true that no gift should ever be big enough to make us stop asking until this disease is cured.

So what I'm asking you guys to do is this:

Get involved in your own health and your own cure. Different people are going to do this in different ways, obviously, but I think the main message is that we should not wait to see what other people, other groups, and other treatments are going to do for us. WE need to be the ones out there spreading this awareness. WE need to take the information about our disease to the next level if it's not good enough yet (and here's a hint, it's not). WE need to seek out and participate in these studies as much as possible. WE need to keep on it and get out there. WE need to give whatever we can in the form of time, energy, money, space, whatever to get this job done. There's no one specific action that I'm asking you guys to take here. I know circumstances vary. But I am asking -- pleading, beseeching, begging -- that each and every one of you take a second (or maybe even a blog?) and try to figure out a way that you personally can be more involved in your own community and in the fight(s) for the cause(s) you believe in.

Because WE as CFers, as people, as a world, simply cannot wait for the cures that will save us.

WE have to be them.

The Challenge of a LIFETIME

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

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

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

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

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

Right.

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

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

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

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

Only 13 states -- because he's an underachiever

Flier used to raise awareness of the cause

The one and only FREEMATOUR BUS in Times Square

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

1 Bike, 1 Bus, 1 Rainy Horizon

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

Start Your Engines

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

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

Because of You

Dear Cystic Fibrosis:

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

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

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

Which brings me to my second point, CF:

You don't stand a chance.

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

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

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

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

Sincerely,
Piper

Miracle Marathoners and Radical Runners

Okay first of all, I think it's time for me to swallow my pride and offer you guys a serious and very heartfelt apology. I know I probably qualify for worst blogger of the year award at this point, a title that I'm 100% ready to accept for myself given the complete lack of updates for the past month. What I am not willing to accept is the fact that I apparently worried some of you with my unannounced hiatus taken right after the announcement of some minor medical issues. And for that, I am truly sorry. Please rest assured that I am fine, have finally come off of the IVs and many of the oral antibiotics, antivirals, and antifungals I was using to combat these infections, and have simply been taking some time to 1) live my life, and 2) reassess the direction in which this blog is going to go from here on out. But I'm back, I promise, and I'm going to be around a whole lot more starting right about now (cue the scary music, right?). So please stay tuned for some pretty cool new stuff that is in the works.

Anyway, I just couldn't let this weekend pass without mentioning a few absolutely awesome people and flat-out fantastic accomplishments. These four individuals inspire me to no end pretty much every single time I get the pleasure of seeing or talking to any of them, and I thought it important to share their incredible feats with the rest of the CF community.

1) Remember Ben? Well, on October 10, 2010 (10/10/10, for those who like that sort of thing), Ben ran in the Chicago Marathon to benefit the Cystic Fibrosis Foundation. Despite ridiculously hot temperatures that left him dodging fellow runners who lost their gatorade in the middle of the run (um, ewww), Ben not only finished but also beat his 2009 time for the NYC marathon and raised several thousand dollars toward a cure for CF. As an aside, tomorrow is also Ben's birthday (and that of his twin brother, Nate). Congratulations to both of them, and a HUGE thank you to everyone who donated, to Dianna and George (Ben's amazing parents, who were also a big support throughout my transplant), and all the runners on the CFF team. Well done, guys.

2) Many of you have probably heard of Emily Schaller over at Rock CF and Mandy Sharpe at Run Sickboy Run and CysticLife -- both incredible women doing awesome things for the CF community and beyond. Well, they've done it again. These two are collaborating to bring you the Outrun CF Virtual Run event.

-What: A fantastic event where CFers and their loved ones/supporters from all over lace up their shoes and hit the tarmac/trail/track to show their commitment to fighting CF and staying healthy. Held on March 20, 2011 (the same day as the Rock CF Half Marathon), this event will allow you to participate without the pesky trip to Michigan (although people in the area should totally head out to the real deal!).

-How: Easy. Just register, track your training using all the helpful tools provided, and then on the big day head out to run as much or as little as you can to show CF who's boss.

-Where: Your town, your street. No travel required.

-Who: You, of course. And all your friends and family. Naturally. Because I happen to know all my readers are some seriously cool people, and you must have some seriously fun people in your life willing to take this challenge for themselves and for YOU.

I'll be running, and so will Donor Bob (or his lungs, anyway). Hope we'll "see" you all there!

3) Okay, this one blows.my.mind. No joke. Nearly a year ago I was blessed to make the acquaintance of a very special man with CF who was also awaiting transplant at my center. He had a tough wait and many dry runs (though I would ultimately beat him on that score, ha), but his commitment to exercise and his never-say-die attitude convinced me beyond a shadow of a doubt that he would get his lungs...and he did. Several months before me, in Nov. 2009, my friend received his perfect match -- and then blew us all away by being out of the hospital in a mere 6 days! And now, less than a year later...well, I can't even describe the awesomeness of what he's doing. Just go HERE and see for yourself. Trust me, you don't want to miss this.

Congratulations, Tim! We are all so proud of you!

(His wife, Beth, also runs a CF charity that deserves a shout out as well. Check out Breathe for a Cause.)

As an aside, Tim and I were lucky enough to share a surgeon to whom we will both be forever grateful, as we are to the entire team at our center.

Love and light, beautiful people.

Just Do It: Cure Edition

So I'm always going on and on around here about my awesome family, and sometimes I wonder if you all get sick of it. I mean, be honest, here. Are you tired of coming onto my blog all geared up to here (yet another) story about the life and times of a slightly crazy transplant patient and her very high-strung puppy, only to find yourself somewhat disappointed by the fact that all I ever seem to talk about anymore is my fabulously helpful mother, my fun-loving father, or my pretty much ridiculously talented sister? Yeah, I kind of figured as much. But fear not, lovely people, because I 100% promise to you all that this particular post that you are about to read will not be in any way shape or form about my fabulous family. No, really, I mean it this time.

It's gonna be about my equally fabulous friends.

Shocker. Bet y'all didn't see that one coming from a mile away, did you?

But, although I'm not kidding when I say that all of my friends definitely deserve to have a blog dedicated to their wonderfulness, this post is about one friend in particular. Remember this guy?

(Psst...he's the one on the right.)

Yep, that's Ben, who at around this time last year ran the 2009 NYC Marathon, and in the process raised about $4,000 for Team Boomer and the fight to cure cystic fibrosis. Um, yeah, did I mention that I have pretty fabulous friends? I wasn't kidding...

So this year, of course, Ben is at it again. Believe me, beautiful readers, I have tried (and tried, and tried, and tried again) to get this guy to slow down and stand still more often. And frankly, it just flat-out doesn't work. He pretty much makes me look lazy (actually, he would probably make the Tazmanian Devil look like a couch potato if the two ever went up head-to-head, though it's possible that our own "super fibros" Ronnie and/or Jerry Cahill could outrun him, not to mention Rock CF's incredible Emily. Just sayin', Ben.) Given that simple fact, though, I've decided instead to focus my energy on something a lot more doable than forcing Ben to stop moving. Something relatively easy like, say, finding a cure and saving the lives of over 70,000 children and adults around the world. Right. Because we all know that together we can totally do that.

Which is why, on 10/10/10, Ben is going to be running the Chicago Marathon in support of the Cystic Fibrosis Foundation. And, as we all know, the CFF is an amazing charity, with over 90 cents of every single dollar raised going directly towards life-saving research and drug development. In the immortal words of Dr. Bob Beall: money equals research and research equals science and science equals LIFE.

But, of course, none of you need me to tell you that, right?

PLEASE show your support for this amazing guy and this amazing cause. There are so many ways to do it, and they're all super easy.

1) Obviously, this is a fundraiser, the primary goal of which is to, well, raise funds. If you feel like helping out Ben's marathon awesomeness, please do so here.

2) If you live near the Chicago area, please go out and support Ben and all the runners on Sunday, October 10th. I'm not allowed to travel for a while post-tx, or I would totally be there. And, of course, cheer especially loud for all the wonderful people running for the CFF.

3) Feel free to leave Ben a message in the comments of this blog to let him know who he's fighting for and how much we all appreciate it. Believe me, this sort of encouragement will probably come in handy around mile 25 or so!

Honestly, I know I joke around quite a bit on the blog, but on a serious note: I'm so beyond proud and grateful to have amazing people in my life. And to everyone who continues to -- or has in the past, or ever will in the future -- put so much time, effort, and energy into the race to cure this crazy disease, please know that all the blogs in the world could never even begin to touch on how awesome you are. To you it might mean 26 miles (and 385 yards) of sweat and effort, but to 70,000 people around the world, it means everything.

12,500

Today was our Great Strides walk in lower Manhattan. I was SUPER proud of my team -- we raised about $12,500 (preliminary total) for a cure! That's money that will go straight to the Cystic Fibrosis Foundation, and about 90 cents of every dollar donated directly funds CF research and educational programs. And when you think about the new drugs in the pipeline and how freakin' close we are to FINALLY breaking through to target the actual defect that causes this disease, well, all I can say is wow -- Great Strides, indeed.

Thank you to all my wonderful walkers for showing up, for smiling even at 9:30 am on a Sunday morning, and for looking so amazingly HOT in my team t-shirts! I was especially proud of our two furry mascots, particularly "Tug" for rockin' the shirt like a champ. Also thanks to my CF friends Graves, Joni, and Gabby -- awesome to see you all!

And a VERY special thank you to everyone who donated to our team through this blog. You guys have no idea how amazing the response was after my post for a cure! I was truly overwhelmed with gratitude and excitement. Not that I ever for a second doubted that I had the best readers a girl could ask for, of course, but you didn't have to go to such amazing lengths to prove it! Please know that you not only made my day, you also made a HUGE difference in the lives of everyone living and breathing with CF throughout the world.

Here are a couple of pictures from the big day:

Team Piper Official Portrait

T-Shirt Front

T-Shirt Back

My Lovely Sister (and designer of the shirts!), My Father, and Me at the Walk

Okay, here's the fun part:

Are you an official "Breathhead"? Are you interested in having one of these fabulous lung t-shirts -- designed by a famous designer featured in the June issue of Vogue, no less -- for your very own? Would you like to spend the night of my transplant cruising my blog, reading updates from my talented sister, and sporting your own piece of beautiful Matter of Life and Breath apparel?

If you answered yes to any of the above questions, consider contacting a therapist for special help. Just kidding, contact me instead! That's right, we're thinking of ordering another batch of these beautiful shirts for all my wonderful supporters, and would like nothing more than to send YOU your very own. So let me know at matteroflifeandbreath(at)gmail(dot)com and please include info as to your preferred size and shipping address. All addresses will be deleted upon shipping.

Thanks guys, and thank you to everyone who donated, walked, or volunteered for the many Great Strides walks all over the country. You guys are, as ever, an inspiration.

An Open Letter in Support of a Cure

Dear Would-Be Donors, Volunteers, New Friends, and Supporters:

I am writing you today to let you know about an issue that is very close to my heart. In fact, it's directly over, next to, and surrounding my heart: in my lungs. And it's also in the lungs of many of my friends, in the lungs of young children, in the lungs of approx. 30,000 people in the United States alone. That's right, today I am writing you to let you know about cystic fibrosis (CF), a deadly disease that I and many other amazing, wonderful, and inspiring people live with everyday. It's a disease that directly affects the daily lives of so many, and I write on behalf of every single one of those remarkable individuals to ask for your help.

We need a cure.

When you have a genetic disease, science means everything. The Cystic Fibrosis Foundation (CFF) partners with drug companies and other groups to fund research that has led to major developments in the treatment of CF. In fact, since I was born in 1981 the median life expectancy for a person with CF has risen from only to 18 to slightly over 37 years. Much of this is due to the new drugs that have reached the market during that time period, none of which would have been developed without the generous support of donors. To say this is amazing progress for a genetic disease is a huge understatement -- especially when you consider that alongside the increase in life expectancy has also come a dramatic shift in the quality of life for most people with CF. There are now adult CFers holding down jobs, raising families, and giving back in the same way that so many have given to us through the years. And we are, all of us, so grateful for that opportunity.

But let me tell you why it's not enough. The simple fact is that while there have been amazing improvements in CF care since the early 80s, the focus of that care is still on treating the symptoms of the disease, rather than the underlying defect. We have drugs that help thin and hydrate the sticky, dry mucus that collects and pools in our lungs; drugs that help us compensate for our ineffective pancreases by feeding our bodies synthetic enzymes to digest food; we have drugs that reduce inflammation in the airways and allow us to take deeper, fuller breaths; and we have drugs that attack the many different strains of bacteria that inevitably find their way into our diseased lungs, causing recurrent infections that in turn lead to irreversible scarring; and, for a lucky few, we have the final option of transplant -- trading in our CF lungs for the lungs of a generous stranger in the hopes of increasing the time we have to savor this life.

Unfortunately, few of these amazing, life-enhancing drugs come without consequence. Overuse of steroids to treat inflammation can lead to osteoporosis, diabetes, and adrenal disease. Pancreatic enzymes can cause scarring to the digestive track and lead to further issues down the road. Antibiotics used to treat infections often become ineffective over the years, forcing the doctors to resort to increasingly strong drugs that can themselves cause kidney failure, hearing loss, joint pain, nausea, or allergic reactions. Eventually, the infections often progress to the point where antibiotic treatment is no longer enough, and transplant becomes the only option. While this is a remarkable second chance for many CFers, it comes with a lifetime guarantee of immuno-suppression as well as many common secondary conditions such as high blood pressure, diabetes, osteoporosis, and an increased risk of certain cancers.

But you may have heard something about CF in the news lately -- say in a recent article published in The New Yorker. And if you did, you might have learned that the CFF is now helping to fund clinical trials for several drugs aimed at treating the actual genetic defect that causes cystic fibrosis. These drugs have the potential to change the entire approach to CF treatment, allowing perhaps for less use of the symptom-treating drugs that often lead to other, secondary ailments, and focusing instead on eliminating those issues at their source. These drugs have shown amazing promise in clinical trials so far and are currently in the late stages of testing before the final push to market.

And that, my friends, is where you come in.

It costs close to $800 million to bring a single drug to market, from research and development through testing and FDA approval. Because of this, it is ONLY through the support of people like yourselves that any of these drugs ever reaches the CF patient population. We are so close to potentially saving thousands of lives with these new therapies, but we simply cannot do it without your help. And, despite the seemingly astronomical sum required to make a difference, let me assure you that it's the little donations that add up to the bulk of this life-changing number. That change you found in your pocket when you were getting ready to do the laundry, the amount you would spend on your daily Starbucks latte, the cost of a night at the movies (or even the popcorn!) can be a miracle for all the many people whose every breath still depends on a cure.

May is National Cystic Fibrosis month and, across the country, people will be walking to support the CFF. Please add your name to the ever-growing list of amazing people behind this cause, whether it be through a gift of your time, your money, or even just a pledge to pass on the message of this disease to ten new people.

You can donate to my own walk by clicking this link and following the simple instructions.

30,000 people (and their 60,000 lungs) will thank you for it!

xoxox beautiful people,
Piper

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.

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.

Magic in the Making

Okay, I know I win worst blogger award...again. The fact of the matter is that there hasn't been a whole lot going on in my life lately to share, and believe me I'm not complaining. I was a little sick last week -- started back on IVs after my last post and then ended up having to add a high-dose steroid burst to help push things along -- but I feel amazing now. It's incredible what a few days, a few antibiotics, and a few (or, you know, six) prednisone every day will do for a girl! (And, on a random sidenote, is there any better feeling than being on high dose pred, having plenty of food in the house, and knowing that you're actually allowed -- um, make that encouraged -- to eat as much of it as you want? I've had some super yummy dinners these past few days, suffice it to say.)

But to be honest the truly incredible part of this weekend wasn't the steroids, or the IVs, or the ecstatic high that comes from feeling better after an infection, or even the non-stop foodfest. Nope, the really, truly, unbelievably amazing thing wasn't even going in New York, even though things were pretty great around here. As it turns out, though, everyone who's REALLY cool was hanging out not in the city, but on the mountain. Vail Mountain in Colorado, to be specific.

This past weekend was the 25th annual American Airlines Celebrity Ski for Cystic Fibrosis, which this year raised over $700,000 toward curing this disease in a single three-day event. Incredible? Absolutely. Heartwarming? Without a doubt. Magical? Well...decide for yourself:

Is it magic that a single event has been able to raise over $23 million to date for CF research, owing almost entirely to donations from individuals who return year after year -- who literally come for the fun and stay for the cause?

Is it magic that celebrities as diverse as sports heroes (Jim Lonborg, Matt Bahr, Jim Palmer, Billy Kidd), TV stars (Ian Ziering, Jim Sikking, Sandra Hess, MacKenzie Rosman, Kimberlin Brown, Wendie Malick, Grant Goodeve, Dennis Haskins), comedians (Jeff Dunham, Rosie O'Donnell), musicians (including members of Santana, Gavin DeGraw, and American Idol contestants), and so many, many more give their time and energy to support CF and raise awareness of this disease?

Is it magic that so many corporate sponsors, particularly American Airlines, have stuck with this cause since the 1980s, through good times and bad, through the discovery of the CF gene and up to now, when new drugs promise so much hope?

Most importantly, is it magic that one room full of about 1,000 people was able to raise well over half a million dollars for cystic fibrosis in the middle of a recession?

Speaking as someone who has been honored to be a part of this event almost since its conception, I'm here to tell you that it is, quite simply, magic. Every single year the people who come to this weekend blow me away with their generosity, their spirit, and their commitment to finding a cure. For over 20 years I have been with them on the ski slopes, raced along side of them on the slalom course as we also raced toward a cure, and shared in the joy, laughter, tears, and love that make this event so...well...magical.

I couldn't be there this year because I'm waiting for a little magic of my own, but I couldn't let the weekend pass without mentioning my wonderful second family and saying, once again, "thank you" for all that they do...for all of us. This is a group who took 30 seconds of silence last night to focus positive energy, prayers, and thoughts for me 2,000 miles away as I waited for new lungs -- coincidentally on the weekend when everything fell into place and I finally began to feel better.

And if that isn't magic, then I don't know what is.

More Thoughts on Waiting

Note: the below text is a talk I gave last night at a wonderful CF fundraiser organized and chaired by a remarkable CF woman (and CF mom!) from one of the forums. She was kind enough to invite me to her event, and then asked that I share a few words about my story. I thought it fitting to repost here, but I have removed her name just to protect privacy.

L has given you all some great information about CF, but what I'd like to do now is tell you a story. And, like so many of my personal stories, this one begins in a doctor's office.

It was, I imagined, going to be a pretty routine clinic appointment. As I settled myself on the familiar exam table to await my doctor, I went over the specifics of this particular visit in my mind: PFTs down a point or two, might mean an oral antibiotic or some other slight tweak in the medication routine; my weight was too low, as always, so we might have to discuss the dreaded feeding tube issue once again; and otherwise nothing too remarkable. I swung my legs and fidgeted in the tiny clinic room, gearing up for what I knew was coming: the inevitable conversation about my increased need for IV antibiotics to fight infections in my lungs, and the implications of my decision to work full-time at a large law firm following my graduation from law school the previous May. It was now January of 2008, and while I sensed that things were changing with my CF, I had no idea just how much this seemingly run-of-the-mill clinic visit would change my life.

Transplant. It's a word most CFers are all too familiar with, at least as a concept if not as an actual reality. Since my teenage years, I had understood that lung transplant surgery was a last-resort option for people with advanced cystic fibrosis lung disease. I knew people who had undergone a transplant and had emerged smiling, full of life and healthy, vibrant breath supplied by their new lungs. But I was completely unprepared to hear the word applied to me and my disease, despite my personal understanding that my CF was progressing. And so, when my doctor took my hand and told me that she was recommending a referral for lung transplant evaluation, my first thought was, quite honestly, "for whom?" Because certainly she didn't mean me, the lawyer, or me, the daughter, sister, girlfriend, and friend. She couldn't mean me, the girl who loved to travel and be active and who adored her job. And then it hit me that she did, in fact, mean me, the CF patient.

It's funny how jarring news can sometimes work strange and wonderful things in my life, and it turned out that my referral for transplant was no exception to this rule. Immediately after learning the news, I began to seek out other CFers, figuring that I should get as many perspectives and as much support as possible during an otherwise confusing and difficult time. The issue, of course, was that CFers are rarely allowed to meet face-to-face because of the dangers of cross infection. A room full of CF patients might have offered me the support and help that I needed, but it would also be putting my health (and the health of others) in danger. But a chat room full of CF patients? Well, that's an entirely different story. And so it was that I stumbled into the online CF community, where I was lucky enough to meet L and others like her -- members of a unique collective of 30,000 + people in this country living with this disease, dependent on the medical advancements and new treatments made possible through the CFF and lung transplantation, and surviving on hope for a cure that suddenly seems not so far fetched thanks to cutting edge research and truly remarkable science. These were the people who helped coach me through my evaluation for lung transplant, who continue to support me as I've gotten sicker with CF, and who will celebrate with me when I finally receive the oh-so-precious gift of life.

So how could I resist the chance to come and meet one of these remarkable women in person? And when L asked me to share a little bit about transplant and my personal CF story, I was both humbled and excited. Which is fitting, I guess, because right now is a time that truly is both humbling and exciting for all of us out there fighting so hard against cystic fibrosis. There are new drugs on the horizon that show amazing promise in correcting the ion transport at the heart of the CF defect, potentially offering CFers with less advanced lung damage the possibility of life beyond this disease as we've always known it. And advancements in lung transplant offer those of us whose lungs have suffered more damage the hope that we too may soon breathe without our current limitations. Neither option is a cure, in the true sense of the word, but both paths seem to lead to a world where CF might have a new and different meaning, and all of it thanks to the continued dedication of the scientists, the doctors, the donors and the fundraisers, and the Cystic Fibrosis Foundation.

I guess the moral of the story is that all of us are, in a sense, waiting -- L and I, and the thousands of other CF patients and their families. We fight and we wait: for new lungs; for new treatments; for the chance to see our loved ones who have died of this disease once again; and, just as I waited that fateful day in the clinic office, for the news that will forever change our world.

I thought this was a fitting response to my own late-night rantings about CF and waiting, in a way. And I also understand that there are many people out there with CF who are walking an entirely different path -- those who will not receive transplants, or who are awaiting the next stage of a journey that is all their own. And I guess the question always remains:

What are we waiting for?