Wednesday, March 31, 2010

This is Hard

I'm not quite sure how to even sit down and write this post. Emotional roller coaster doesn't even begin to describe the events of last night, but I guess when in doubt, go with the cliche. And I guess in some ways, a roller coaster image is fitting: the highs and lows, the feeling of racing along a track over which you have no control, the slow motion of the moment contrasted with the whirlwind feeling you get when the ride is finally over, that whole "did that even just happen?" feeling of stepping out of the car.

This is hard.

Yes, this is Piper writing again. No, I'm not the world's most amazing recovery artist. Yes, that means I didn't get the lungs. The right lung, once again, wasn't perfect, and in this game you don't settle for any maybes. I'm so grateful to Columbia for being discriminating, for searching for lungs that are absolutely right for me, and for having the courage to pull the plug, even after 9 hours of build up, when the right lung just doesn't look...right.

Still, this is hard.

My right wrist is bruised beyond recognition from the arterial line -- a yellowish purple battle scar to remind me of the physical pain that is just a part of this process. My heart is bruised too, in a way that it wasn't after last week's dry run -- an invisible yet poignant scar to remind me of the emotional pain, the loss, and the tragedy that is also a part of this process. And, after two calls in less than two weeks, my hope is higher than ever that MY lungs are close behind all these rehearsals -- a beautiful light to hold onto to remind me of the beauty that is, of course, just a part of this process. It is all just a part of transplant -- such a miraculous, cruel, amazing, delicate, unpredictable science.

And it is hard.

So today I remind myself that hard doesn't always mean not worth it, and that for every "dry run" I've had someone else has been given a gift beyond measure. I remind myself that people are donating, that calls are coming, and that human kindness is flourishing and that strangers are constantly changing each others' lives through the simple choice to give not only of their money or time, but of themselves. I remind myself that it is not just my desire for new breath that controls this process, but a much wiser, more loving plan. I remind myself of all of this, and I promise to hold it all in my heart, to remember.

But, truth be told, it is hard.

Here We Go Again...

So after a day of IVs, shorkie time, working out, and random blog musings on the waiting game, it looks like it's time to try again for new lungs. I got the call last night at 11 pm and it's not 1:20. I'm checked in, have a hospital bed in a single room (after much confusion stemming from the fact that only doubles were available and I am, of course, on contact isolation), have just gotten a lovely IV in my right hand (literally in between typing that last comment and this one), and am dressed in a stylish-beyond-all-reason hospital gown. Fantastic.

This is what I know so far: 1) the donor is neither high risk nor cardiac death, 2) the donor lungs are CMV positive -- since I am CMV negative this means I will be on anti-virals (IV and then oral) for a period after surgery), 3) there is, as always, a back up family here in need of a single lung. The resident is on her way up to talk to me about other details, but I'm guessing that if all goes well the surgery will be scheduled for the early morning hours of Wednesday, March 31. All good vibes, prayers, thoughts, and anything else positive you can send our way are of course always welcome.

I just want to say again how thankful I am for all of you. I love you all and I'll be blogging again on the flip side. No sleep 'til new lungs!

Much love and peaceful, prayerful moments,

Tuesday, March 30, 2010

This Unfolding

Rain courses down the living room window next to where I sit, typing these words by the grey light of a cloudy city day. I've always loved the rain, for some reason -- never complained about the afternoon thundershowers that so dominated my childhood summers in Colorado, even as the flashing lightening and deep booming echos startled my horses and put my swimming lessons on hold for the hour. And even today, with the mountains replaced by towering buildings and the frightened horses giving way to honking taxi cabs, I still find rain more beautiful than irritating. It's something about the peaceful rhythm of its fall from the heavens, or the eerie magic of a mist-filled skyline that does it to me, I guess. Or maybe it's just the confirmation of yet another cycle: water from the sky to the earth, the changing of the seasons, April (or late March) showers drifting slowly into the promise of May flowers...or so I'm told, anyway. There's just something about rain that makes me want to savor the moment.

Of course, it helps when that moment is warm and dry, filled with a cuddling shorkie after a hot bath and the anticipation of a late-night movie with my sister. It helps when that moment is a brief respite from the constant coughing, and comes in a hour where there are no treatments to be done, no more pills to swallow, no IVs to hook up until later tonight. It doesn't even hurt to have the moment fall in a rare space of "alone time" (sorry, Sampson, present company excluded) -- a place in time when my apartment is once again my own personal domain: my light, my shadows, my windows, my space.

My life.

It's weird for me sometimes to think of this space I'm moving through as a part of my life. There is so much language to the contrary: "my life is on hold," "waiting on the gift of life," "hoping to get my second chance," -- always waiting, hoping, wishing. There is a part of me that prefers to see myself as the person I was before all this started (wait, was there ever a "before" period? Or was it all just one seamless transition?), or the person I will perhaps become after this is all over. A part of me that would like to separate out this period of waiting and constant sickness as some sort of intermission -- a pause in between acts of the real show -- a time to visit the concession stand and stretch your legs until the main actress catches her breath and resumes the stage, her weaker, sicker understudy retired to the wings at last. There is definitely a part of me that would like to scrap this chapter -- just edit it all out until the page is gloriously, perfectly white again, and filled with the promise of any new story.

Yep, there is definitely that part of me.

But, weirdly enough, there is also a growing sense in my mind that this waiting is, in fact, not just a part of my life, but a MAJOR passage in my own personal journey. This is not an intermission, in other words, but perhaps a type of dramatic climax -- the moment when the plot comes together and the heroine finally comes to terms with something deeper than herself. Which sounds, of course, wonderfully dramatic and intriguing, but hardly suffices to tell the story of all the many moments that I have been living lately:

The moment when I was told I needed a transplant and I knew CF had won, in a sense, because it was taking my lungs from me.

The moment when I accepted that I needed a transplant and I knew CF had NOT won, in any sense, because it could never take my spirit from me.

The moment when I was told I could actually have a transplant and I knew gratitude and excitement and fear beyond any of my wildest emotional fantasies.

The moment(s) when I realized that no one has to start dying until they choose to stop living, and that one word doesn't have to define my entire existence, no matter how scary a concept it might represent.

The moment I was offered a chance at new breath from a stranger.

The moment I relinquished my chance at new breath into the chest of another stranger.

The moment I came back home to my shorkie and my family ordered (good) mexican food and my friends came over for an impromptu gathering that evening and I realized that I was okay, I was still breathing, and that I would live to see the next day and (hopefully) the next set of perfect lungs.

The moment I sat with my shorkie and watched the rain course down my living room window as I sat typing out a rambling message of what it's like to live in waiting, instead of waiting to live.

Recently I read a quote from a young psychologist who developed AIDS during the start of the epidemic. After learning his diagnosis, before he even told his friends, he wrote these words (taken from And the Band Played On, by Randy Shilts):

"It's important for me to keep a very close watch on this time. It would be so easy to think I'm not even going through this. It's an interesting time. I would not miss it for the world -- what it's like to go through this unfolding." -- Gary Walsh, 1983

Neither would I.

Monday, March 22, 2010

The Hardest Part

Okay, so now that I've had a little time to decompress after yesterday, I've come to a really somewhat startling conclusion:

Yesterday might have been one of the best days of my life.

Um, yeah, you read that right. And no, don't worry, I'm not suffering from post-traumatic stress delusion over here. Believe me, I never expected that I would ever write those words about a day filled with hospitals, blood draws, IVs, super stylish "gowns," and well, frankly, disappointment. I never thought that I would ever call spending 9 hours NPO being prepped for surgery a good time. And, to be honest, it really wasn't a "good time" in the traditional sense of the term, but I have since come to understand that it was a very "good time" in terms of learning a very valuable lesson.

Okay, let me explain.

A couple of my favorite CF transplant bloggers have recently been inspiring me with their words of wisdom about trust and acceptance. I'm always so grateful to read those messages because they remind me that even when things don't turn out as we would like them to, they still turn out as they should. And I guess I should preface all this by saying that I do, in fact, believe in the Greater Plan -- that God has a purpose for us, that we are all connected as part of creation, and that (as Sara so wonderfully reminded me) "the universe is unfolding as it should." But I'm also the first to admit that believing in that concept in the abstract is often a whole lot simpler than believing and actually trusting and rejoicing in that concept on a daily basis, especially when, well...shit happens.

Yesterday, though, the abstract became real for me in a way I don't think I could have ever anticipated, and in retrospect it seems like the best blessing I could have ever asked for.

I felt firsthand how one family's loss can, through their grace and generosity, become a miracle of hope for another family. I sat in a hospital room with my sister as we both shed tears for the family that was offering us a second chance -- that without even knowing us was willing to share the greatest gift we could even imagine -- and doing so unconditionally, without asking anything in return. And honestly, it was pretty indescribable to actually feel that miracle -- to know suddenly (rather than simply to think) that hope and life really can be born out of grief and death.

And then, later, the other side of that coin became equally real to me when I learned that my loss would be another family's joy and rebirth. It was just a flash -- a split second spent on a hospital transport gurney in the dimly lit OR holding area -- but I realized that just as I was so willing to accept that another family's loss for my gain was right, I also needed to trust just as fully in the fact that my loss was, in fact, exactly what should have happened. Which is not to say that it was easy, or that I was particularly thrilled when I got the news or when as I watched another man roll off to the OR where my surgery had been scheduled, but I guess it is to say that I was honestly at peace with it even in that very emotional moment. It wasn't "fun" for sure, but it was okay, and more than that -- it was right.

I really, truly believe that -- now more than ever. Just as I really, truly believe that perfect lungs will come for me, and for Beth and Jess and Jen and Rhi and Gina and Katie and Jerry and James and Andrew. I don't know that, of course, but I do think I know now that it will all be exactly as it should be, either way.

Does all this sound too passive for your taste? I promise I don't mean it to be. One thing I have learned through this entire process is that you have to fight and advocate for yourself, and you have to take active, positive steps toward making good things happen. But when things don't turn out exactly as I planned them, even after all my best efforts, I hope that I can also take from this entire experience the understanding that, well, that's okay too.

Tom Petty, by the way, was wrong: it's not so much the waiting as the trusting that is, in my opinion, the hardest part. And also the most beautiful.

Sunday, March 21, 2010


Wow, thank you so much everyone! I have honestly never felt so loved and lifted up in my life. I guess it's CAN be thankful for dry runs!

Speaking of which, I'm typing this from the OR holding area. The right lung was bad so the left lung went to the sweetest old man. In a weird twist, we were right next to each other in the holding area and his family came over to congratulate us and wish us luck (since we were the primary they assumed we would get the lungs). When the doctor came and told me the news, I actually got to look around the curtain and give them the good news. They all started crying -- it was so incredible, so bittersweet, and so miraculous! A moment to remember for the rest of my life.

I saw the family getting the heart as well.

Since I personally know firsthand the power you guys have to make a person feel held close, I'm asking that you please keep a grandfather, his wife of 46 years, and his son in your prayers today as they embark on their new journey of life and breath. At one point they had the 6 year old grandson on speaker phone to talk to his granddad before surgery. Wow.

And who wants a bad right lung anyway, right?

I can't thank you all enough for all the prayers and good thoughts. I know my lungs will come at the right time for me. Just a matter of trust at this point.

It's a Great Day for NEW LUNGS!

Att 5:30 this morning my phone rang, and I knew instantly that this was "THE Call." Honestly, who else would call at 5:30 am on a Sunday? Certainly none of my friends, anyway. So we grabbed our bags, said a quick "I love you" to the shorkie, and were out the door. And into...a half-marathon.

Actually, to be fair, thehalf-marathon hadn't actually started, but that doesn't mean it hadn't already worked a number on my planned hospital route. See, today is the annual RoadRunners Half-Marathon in NYC, and it actually ends across the street from my apartment. Needless to say it took us a little while to negotiate our way up to the hospital -- my personal favorite moment was when my mom leaned out the window (over me) and started trying to explain to a NYC cop that we were on our way for "emergency surgery." He was sympathetic, but not much he could do.

Anyway, we made it up safe and in one piece without mowing down any runners on the way. I'm slightly overwhelmed right now (um, make that EXTREMELY overwhelmed) and have just been kind of in a daze all morning. But I'm checked into my room, I have an IV firmly in place in my wrist, I've had my EKG and they've taken about half my total blood supply, and I'm dressed to impress in a lovely hospital gown.

Surgery is tenatively scheduled for 2 pm, assuming this is a "go." I have a good feeling about this, and my sister will update as she is able. Thoughts, prayers, and of course all the love you can spare are always welcome.

Catch you guys on the flip side. I love you all :)

Saturday, March 20, 2010

Because Inquiring Minds Want to Know

Some Real Facts about CF and Transplant:
  • As of March 12, 2010 (the last published data update), there are 274 CFers on the national organ transplant waiting list. 248 of these CFers are awaiting double-lung transplants. 30 are awaiting liver transplants. (Source: Organ Procurement and Transplant Network Data)*
  • 153 of these CFers are in the 18-34 age group. Of the remaining 121 cystics-in-waiting, 72 are older than 34 and 49 are younger than 18. (Source: Organ Procurement and Transplant Network Data)*
  • According to the CF Patient Registry, nearly 1,600 people with CF have received lung transplants since 1991. (Source: CFF. Note: I have no idea what the end-point on this data is, although I know it to be at least 2003 or later.)
  • Living lobar lung transplants (where part of a lung is taken from each of two living donors and transplanted into the recipient) is still an option for some people with CF. In 2003, 17 people with CF received their miracle from living donors. That same year, 126 CFers were blessed with the gift of life from deceased donors. (Source: CFF)
  • As many as 90% of people with CF are alive 1 year after their double-lung transplants. (Source: CFF. Note: I'm again unsure of the date for this statistic, aside from the fact that it is 2003 or later. The average survival rate after transplant continues to increase as newer and more effective treatment options and transplantation techniques are developed.)
  • After transplant, the new lungs will never again "have CF." People with CF who receive transplants will still have CF in their pancreas, sinuses, intestines, liver, sweat glands, and reproductive tract. (Source: CFF)
  • Almost everyone can be an organ donor. There is no medical condition, age, or race that automatically excludes you from all potential organ donation (even HIV positive people can donate to others with HIV, for example). All major religions in the US support organ and tissue donation. (Source: Donate Life America Fact Sheet)
  • You can register to become an organ donor in your state here: Donate Life America
  • People with CF living after transplant can: run half-marathons, be professional athletes who have carried the Olympic Torch, follow their dreams as an actress, raise children, get married, become "Heroes of Hope," volunteer, have careers, finish school, write a book, make a movie, learn to surf or scuba dive, live for 20 years (and counting) after their surgery, make a difference, ski down or hike up some of the tallest mountains in the country, advocate for other patients with CF or chronic illness, or...well, you name it, we do it. (And yes, I honestly know -- or know of -- people in every single one of those categories.)
And, the coolest fact of the day by far...
  • One of my personal blogger friends with CF received her special gift of life last Wednesday night! So make that 273 people on the waiting list!
*Note: These statistics count the total number of candidates, meaning that individuals listed at multiple centers are counted only once. Also, I am including the table below due to issues with the source link for this information. All data can be found in its original form by conducting a search on the United Network for Organ Sharing website, and is compiled by the Organ Procurement and Transplant Network. The first column represents total numbers; the third column represents liver candidates; and the seventh column represents lung candidates. All other columns represent additional organs, including pancreas, kidney, heart, and intestine, as well as multi-organ transplants.

Cystic Fibrosis All Ages

1-5 Years

6-10 Years

11-17 Years

18-34 Years

35-49 Years

50-64 Years

Wednesday, March 17, 2010

A Few Things

1) Thank you all SO MUCH for the comments and critiques on my "positivity" blog post. This was far and away the most successful "blogger challenge" we've had yet on A Matter of Life and Breath, and I have had SUCH a good time reading everyone's very informed, honest, and interesting perspectives. For those of you who would like to read more, here's a list of some of the blogs that featured discussions on the topic (sidenote: if your blog is not on this list and you have written or plan to write on the topic, please let me know and I will add it ASAP). Also, a HUGE thanks goes out to Ronnie and his always amazing Run Sickboy Run for bringing a lot of really great people to the table on this one. You guys continue to inspire and impress me, as always.

Welcome to Joshland
I Have CF So What
A Day in the Life of a CF Mom
Jamiebug's Cepacia Lung Transplant Blog
Lovin Lane
Breathing You In

(As I mentioned, I really hope to grow this list if people have more to say -- and if you're anything like me, there is ALWAYS more to say! -- so please keep them coming, and feel free to alert me to any blogs not yet on the list. I try my hardest to keep up with the CF community blogs, and if I'm missing one I always want to know!)

2) Transplant tidbit of the day:

Did you know that cystic fibrosis is the third most common reason for lung transplants in the US? It's behind Idiopathic Pulmonary Fibrosis and Emphysema, respectively. IPF just recently (sometime in the past few years) beat out Emphysema for the #1 slot. About 1600 lung transplants were performed last year in the US.

Okay, I don't know about you, but that makes me pretty excited. Don't get me wrong, I'd prefer NO ONE ever needed a lung transplant, obviously. But consider that the first successful double lung transplant wasn't even performed until 1986, and the first successful CF lung transplant wasn't until 1988. That means that in just about 20 years we've taken this medical technology from a whimsical pipedream to a medical reality for over 1,000 patients a year suffering from end-stage pulmonary disease. Um, wow.

And beyond just the "wow factor" there's also the "someday soon" factor -- as in, maybe someday soon they'll be able to "grow" lungs to perfectly match your body using your own stem cells and DNA. Or maybe someday soon they'll be able to combine bone marrow and lung transplants to successfully wean patients off of immuno-surpressive therapy post-transplant (a reality already for certain kidney and liver transplants). Not to mention new therapies in the works for lung transplant treatment such as inhaled cyclosporin, a nebulized version of a key immuno-surpression drug that allows the medicine to go straight to the source, bypassing other organs where its affect is not needed.

I do believe that it's entirely possible to imagine a world where CF is no longer the third most common cause for lung transplants -- as CF therapies grow and reach new heights that day seems closer and closer. In the meantime, however, I can't tell you how excited these new developments in "transplantland" make me. Honestly if I'd known medical science was this interesting, I might not have been an English major (freshman year biology grade notwithstanding, of course).

3) Speaking of transplant, please keep your hearts and minds open for a lovely and inspirational young woman with CF named Emily Haager. This surfer cyster/ambassador for the CFF has lived her life encouraging CFers to be active, hopeful, and happy, and she has now been in the ICU for over a month after a sudden and unexpected health issue. She is currently on the vent and hoping to recover enough to receive a lung and kidney transplant soon, but her family is requesting that all of her beloved CF community join them in prayer for this remarkable woman. If you are not already aware of her story, please visit her blog or her carepage.

And that's it. For those of you interested: I stopped IVs this past Friday and have been LOVING the beautiful NY spring ever since. Is it a coincidence that I feel better just as the temp hits 60? Um, I think not! And my recent walks down to the Statue of Liberty/Battery Park with my puppy prove the point.

Happy spring, beautiful people.

Friday, March 12, 2010

How You Met My Mother

Guest Post Stats:
Name: Kathleen Beatty (aka Piper's mom)
Age: Wouldn't you like to know.
Credentials: Political science professor. 31 years experience as a mom, 28 years bonus as a "CF mom." Primary caretaker (um, make that carePARTNER) in Piper's transplant journey. "Grandma" to Sampson the puppy. All-around superwoman. First time blogger.
Special Talents Include: Treatment enforcer, honorary pharmacist and nurse, CFF advocate.
Hometown: Denver, CO (currently Living in NYC)

Hi. I'm Kathleen Beatty, Piper's mom, and this is my first foray into blog writing. I have to confess being more than a little intimidated by my daughter's prose -- and humor -- but bear with me. I have something to say to the parents among you.

There is something truly surreal about being the parent of a hopeful lung-transplant recipient. I've thought about this a lot. How did I get from Point A -- living with a relatively healthy kid who had a very normal life (if one overlooks the 30+ pills a day, lugging ridiculous pieces of equipment into hotel rooms where neighbors wonder if a plane is taking off in the next room, living in the mist of nebulizers, and starting or disconnecting home IVs in every imaginable place) to Point B -- listening to a charming and funny doctor say things like "IF you make it past the first three months" and then later "IF you make it through the first five years, you WILL eventually experience chronic rejection." How did we arrive at a place where we can sit dry-eyed (most of the time) and hope that someday soon a young surgeon will rip open our child's chest, remove her rotting lungs, and sew in a new pair -- just like that?

I often think back to the milestones we've passed on our way to "end-stage cystic fibrosis" (and really, who decided to call it THAT??). I remember dreading those milestones -- the clubbing, then regular hospitalizations or home IVs, the colonization of dreaded bacteria, the central line, nighttime oxygen, feeding tubes (Piper has actually skipped that one), really frequent home IVs and hospitalizations, the bronchoscopies, CF-related Diabetes (she has so far dodged that bullet, too), osteoporosis or arthritis, and finally -- the monster dread -- the transplant. In each case, my husband and I would say quietly at night: "at least she's not on oxygen," or "at least she doesn't have XXX bacteria (the next dreaded bug)." When Piper was an infant and contracted pneumonia at four weeks, we even whispered, "at least she doesn't have cystic fibrosis" because, in the neighboring room a 5 year old was dying of CF. Only two weeks later we would learn that Piper, too, suffered from the disease.

In those weeks and months after Piper's diagnosis, I fell deeply into grief. Children are supposed to outlive their parents. They are meant to be happy and healthy -- to have "normal lives." I read every gruesome bit of literature I could find about the horrors of CF, and I spent lots of time driving around in my care, crying rivers for my daughter's "normal life."

But the truth is, Piper's actual normal life -- her own personal version -- really was lovely. She was and is so full of life, and she's happy. But of course that didn't mean that didn't mean that there weren't more milestones in our future.

A day came when the doctors we knew so well said "Piper is colonizing pseudomonas and needs to be hospitalized" or "Piper will need oxygen at night to avoid damaging her heart and to improve her sleep" and we would pack up our bags, go to that hospital, and watch for improvement, or we went to pick up the oxygen canisters and concentrators. And, while one little voice wants to say "no, it's not time yet -- not for my child," another part of us knows that it is time and that we have passed yet another milestone. And, somehow, we (as CF parents) not only find the strength to keep going, but to welcome the physical relief that new treatments may bring to our very normal kids.

This all builds up to transplant, the most dreaded of the dreaded it seems. Piper had described in her blog her feelings when her doctor explained that she was referring her for a transplant evaluation. I was there. I don't think either of us said one word on the way home from the appointment. No, it can't be time. She just finished law school. She's working at a major firm. She has big ideas about life. We can't be facing a lung transplant.

Since that day, we have looked at CT scans that show petrified swiss cheese where the lungs should be. We've had sleepless nights filled with more coughing than I thought possible (and for the parents of a CFer, that's saying a lot!). We've done treatments at 3 AM, 7 AM, midnight. She has coughed up unbelievable quantities of mucus, had terrible reactions to drugs, experienced chronic and frequent lung infections, and even lung collapses. We've even been to New York City emergency rooms -- not a happy experience. Through it all, though, Piper continues to live a perfectly normal life (her kind of normal and, yes, our kind of normal too).

Something else has happened. A calm has settled over our Manhattan apartment. (Piper, however, would not agree that I or the rest of the family is "calm." During one of our "dry runs" the doctors informed her she should try to be at the hospital within a half hour, and I ignored my checklist and jumped straight into a nice hot shower. My husband continued his exercise class. My older daughter invited a friend over to calm her nerves. Piper was left fielding phones calls and wondering what in the heck happened to her well-organized support team -- needless to say she's whipped us all into shape since then.) But somehow we (and I mean all of us here, including you) know that we will find the strength to think a shiny new pair of lungs will be a great thing for our kids, when that time comes. We get better every day at living in the moment and at trusting that, as my husband said recently, "we don't know what will happen, but we do know that there will be a happy ending. That's our choice."

Wednesday, March 10, 2010

Positive Charge: Blogger Challenge

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

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

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

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

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

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

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

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

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

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

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

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

Tuesday, March 9, 2010

We Don't Need No Education

So today started out normally enough, I guess. Wake up, do treatments, eat lunch, go to appointment, go from appointment directly to mandatory lung education seminar/support group, learn about immunosuppression and post-transplant infection know, the usual. Certainly there was nothing this morning to suggest that this evening would be one of those "lessons in karma" kind of moments.

Which is, of course, the funny thing about karma, right? It always seems to sneak up on you.

But, I digress. Back to the story, which, as I mentioned above, begins with a fairly routine visit to my favorite hospital for an educational seminar on post-transplant living -- in this case, immunosuppression drugs. And, just to set the scene, this is the kind of seminar where CFers, COPDers, and all sorts of other lucky lungers young and old come together to learn useful facts like "DO NOT USE A LEAF BLOWER AFTER TRANSPLANT!!!" Which, to be honest, is probably not so useful for those of us living in Manhattan apartments (seeing as I have yet to encounter leaf debris in my 18th-floor hallway) but the point is: it's helpful to some people. As a CFer, though, I sometimes find that these talks are geared ever-so-slightly more towards the "rookies" of the lung-health world, and so I tend to zone out just a bit when the doctors start talking about the need to get rid of pet birds, or the emotional stress of taking tons of pills every day, or...and this is just a random example here...the many, many, many unpleasant side effects of prednisone.

Because I know everything about steroids, right?

At this point I'd like to pause and say that if you're a CFer and are unfamiliar with prednisone, congratulations. You're extremely lucky, and I'm completely jealous. In fact, I'm insanely jealous, but the insanity part is probably due to the fact that I am, in fact, on prednisone. I'm actually on prednisone all the time, but sometimes at higher doses than others -- and right now is one of those high-dose times. In other words, I'm a little bit cranky, a little bit unable to sleep, a little bit of an emotional nutjob, and a whole lot of hungry. And this last one is, of course, a good thing for a CFer, except for the fact that prednisone also tends to mess with blood sugars...which is what brings us to the next part of today's lesson.

Anyway, my mother and I sat through the very well-done and informative presentation, we listened and asked our questions, and then we started to go home. And as we walked out of the hospital, I turned to her and said what will now be known as (in)famous last words on the subject of steroids and side effects: "well, at least we know by now that prednisone never messes with MY blood sugars." And we smiled.

And literally 5 minutes later, my phone rang.

Um, yeah, I don't think I even have to finish this story. Suffice it to say that I actually laughed out loud when I was told that my blood sugar was too high -- not because it was funny, but just because it was so, um, shall we say predictable in its irony? And sure, I'll admit that laughter isn't really a normal reaction to this sort of news, but cut me some slack. After all, I am on prednisone.

Luckily it's not SUPER high, and the most likely fix is just to taper down on the drug as soon as possible, which we were probably going to do anyway, but it is a good lesson in not counting your side effects before they hatch. Not to mention a helpful reminder that no matter how much you know about CF, transplant, and life in general, you're never done learning.

And I'm pretty sure that's a good thing.

Monday, March 8, 2010

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:


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.

Sunday, March 7, 2010

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.