Thursday, February 25, 2010

Day in the (Transplant) Life

Disclaimer: With a couple of exceptions for Sampson and the view from my apt window, most of the below were taken at my hospital, under the unflattering light of an exam room, using an iphone. Suffice it to say these are slightly less than "glamor shot" conditions -- consider yourself warned.

So today my mother:

and me:

left this little guy behind:

and headed away from this view:

(all the while dragging this* with us):

until we finally reached this view, and all that comes with it

In other words, I had transplant clinic.

*For those of you wondering, this machine is my Sequal Eclipse 2 portable O2 concentrator. This is one of my all-time favorite pieces of equipment. It's (relatively) lightweight, it's on wheels, it has either pulse or continuous flow, and if it starts to run out all you have to do is plug it into the nearest outlet or switch out the battery. I have portable tanks too, esp. for working out or things that involve a lot of outdoor walking, but this machine is FANTASTIC for overnight trips, airplanes (FAA approved), and marathon appointment days at the hospital.

Not a whole lot to report. I apparently still need new lungs, and they are apparently still working on making that happen, hopefully sooner rather than later. I am starting IVs again today, but only one since I'm on inhaled TOBI already and we're going to hold off on IV tobra for a while. I did another 6MW test and dropped a couple hundred feet to around 1600, but I guess that's still not all that bad, especially considering that I'm in the middle of yet another exacerbation.

Um, yeah, this is my 3rd round of IVs for 2010, in case you're keeping score. And you know I hate useless comparisons, but it looks like 2010 is on track to be every bit as ridiculous IV wise as 2009, if not more so. Clearly I'm an IV junkie at this point and should be in some sort of 12-step program -- just so long as step 1 involves new lungs!

Anyway, IV madness aside, today really wasn't too overwhelming. I'm feeling more and more secure in my transplant team, and they've been very forthcoming and honest about where I stand on the list and what my options are. I know at this point in the game it's all about patience and just trying to live as much as possible until those perfect lungs come along. And I'm really hoping that the IVs I'm starting today will help with that too. Because let's be honest, it's a hell of a lot easier to be patient when you're not choking on your own breath half the time. Or maybe that's just my personal opinion.

Then again, coming home to a (snow-soaked) shorkie makes it a little easier too:

Much love for a wonderful weekend, beautiful people.

Wednesday, February 24, 2010

Not a Clever Blog Post

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

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

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

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

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

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

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

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

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

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

Saturday, February 20, 2010

An Open Letter to My Entire CF Family

Dear fellow cystics and all those who love them:

Well, I guess the time has come for me to write this letter. To be honest, I've been putting it off, I think in part because I know it's going to be sappy. And I don't always love sappy. But I do love the truth, and I know that sometimes, tough as it is to admit, it takes a little sap to get us there. So, friends, here is my sap, my heart, and my truth:

I admire every. single. one. of. you.


I'm not kidding. I really do, although some in different ways than others, of course. If there is ONE thing that my time as a member of the CF community (both in real life and online) has taught me, it's that there is no ONE way to handle this disease. There is no foolproof approach, and there is no cystic (or CF family) who I have met that is not trying to live in the best way they know how. And for me, that is the very definition of admirable. So let me break this down for you just a little more:

To the 8 year old boy who just celebrated his 1 year lung transplant anniversary (and his phenomenal mother), you are amazing.

To the 32 year old woman who just got listed for transplant but still managed to find time this past year to get married, and to continue at her job despite huge obstacles, you are amazing.

To the 25 year old woman who works full time and is pursuing a masters in bioethics while balancing a rewarding family/social life and asking some really tough questions about CF, you are amazing.

To the 29 year old poet who seems sometimes to put my own soul into words, and the other 29 year old woman who just took a huge leap of faith in moving several states to pursue her own sense of adventure, you guys are not only neighbors now, but you're also both amazing.

To the 30 year old man who improved his own lung function by leaps and bounds (or should I say strides) through his dedication to exercise, and who is now using his gift to inspire other CFers, and who found time amongst it all to get engaged, you are amazing.

To the woman with CF on the transplant list in FL, who is moving to follow her dreams of new lungs, and who took the time to fold cranes for each of her fellow cystics on the waiting list, you are truly amazing.

To the young man who has severe cystic fibrosis coupled with another genetic disease, and to his mother who is unafraid to write honestly about their lives (the good and the bad), who clearly moves mountains to help her son drink in and enjoy every drop of life, you guys are nothing short of amazing.

To my friends who are "square pegs" and had to fight for their own CF diagnoses, who came to terms with CF at an age when the rest of us were already well-versed in its ups and downs, and who advocate for recognition and better treatment of "their CF," you guys are pretty darn amazing.

To my friends who are raising families, running 5ks, fighting their clinics to get the care they know they need, searching for love, getting married, starting new jobs, taking disability to focus on their health, trying out new treatments, going the extra mile (or 2), starting blogs to educate other CFers, and generally out there living, you are guessed it: amazing.

To my newfound friends at my own transplant center, who keep me laughing and smiling and who generally make it all bearable, not only do I owe you all a huge thank you, but wow, you're amazing!

And to my "transplant idols," whether you're there for me on the phone when I go through dry runs, or have a blog that I just can't get enough of, or showed me that it is indeed possible to run a half-marathon with transplanted lungs, well, I don't even think I can begin to say how amazing you guys are.

In short, I'm amazed by my fellow CFers. And I just want to say to all of you that I embrace your stories, your triumphs, your tragedies, and your lust for life. I will celebrate with you, cry with you, and proclaim your awesomeness from the rooftops if I have to, because I think you are what makes this disease worth fighting. You are the truth of this disease, the living proof of its blessings and its cruelty, and I am beyond grateful for the lesson.

It may be sappy, but that doesn't mean it's not real. Because the reality is that we're all just out there trying to find our own path through life, and breath.

With deepest admiration and respect,

Friday, February 12, 2010

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?

Thursday, February 11, 2010

Stream of Consciousness Post #2

last day of ivs and first day of freedom. hot showers, extra hours, maybe time to stop and smell the flowers.

not roses though. i'm sick of those.

having trouble accepting some of the little things that i'm supposed to take without question. i worry about stuff that doesn't seem to bother anyone else, like what i'll do when i'm suddenly not seeing my cf doctor anymore. feeling kind of like some overgrown adolescent (but then again, who doesn't, really?) because of all this change and my reaction to it. it's almost physical, or i'm sure it would be if there were energy left to have a physical reaction. pages and pages and pages of journal filled with . . . thoughts? questions? whining? i'm not even sure where the deep musings end and the complaining begins anymore. and in the end maybe that's the kind of insight illness give us: the freedom to take ourselves way too seriously.

people all around telling me to just be patient. i want to tell them that i'm the ultimate patient -- 28 years and counting. patience is a virtue, i guess, but lately it seems like patients are just a burden. and i'm so sick of all the waiting: waiting rooms, waiting lists, waiting around, waiting to exhale. i've become a cliche or a sleight of the tongue -- a sick patient who is sick of being patient.

and i know i'm one of the lucky ones.

i'm so lucky to be alive, lucky to have friends and family who support me, lucky to have healthcare, lucky to love my doctor, lucky to have lungs that can choose to work when they feel like it, lucky to have drugs to keep the monsters at bay -- my own personal three-week cootie shot. so very, very lucky to have time left to wait.

it's kind of a thin line between lucky and sucky, when you think about it.

and so tonight i'll keep my vigil, count my blessings, and fall asleep to the whoosh of the oxygen concentrator once again. and tomorrow i'll do more of the same, only different. because each dawn is a new day, and patient's is an underrated state of mind.

Sunday, February 7, 2010

Leave Me Breathless

Okay, guys, it's time for yet another true confession over here on a Matter of Life and Breath, but I'm warning you: this one is not for the faint of heart (or faint of lung, for that matter). That's right, it's time for you all to learn about my secret fear, the stuff of nightmares, truly the type of thing that keeps me up at night, or out of certain of my friends apartments.

Time to take a really, really deep breath, CFers . . .

Okay, have I freaked you out yet?

Yes, I'm ashamed to admit that I truly, deeply fear stairs in all shapes and sizes. For all my honestly held "exercise is good" beliefs, I just can't deal with these monsters. But, for the sake of my health and my ego, I'll sometimes try to push myself up a flight, or maybe two, or, you know, some other reasonable number.

Like, say, 50.

You know, just as an example.

No, I didn't climb 50 flights of stairs this weekend because, frankly, I'm not that amazing. Apparently those genes (the super-awesome ones) in my family all went to my gorgeous cousin, her fabulous husband, and their four amazing kids, all of whom put on their climbing gear this Saturday and hiked their way to the top of a hotel in order to help find a cure for cystic fibrosis.

I don't know about you, but it leaves me breathless. Literally.

With the MN Vikings Cheerleaders, of course

No, you're not misreading. It really does say "Floor 26 -- Halfway to the top"

Um, I'd need ice cream and snacks too

With a sign for Nina, another CFer from out in Gloria's home state of WA
(and a new friend of mine on CysticLife)

Gloria, Steve, Isaac, Lola, Sam, and Caleb (who, as a toddler, is a little bit young to climb, but no doubts here about his future potential!), you guys are all, in a word, unbelievable. I feel blessed beyond words to have you as my cousins, and I know I speak for the entire CF community when I say a very heartfelt thank you. Believe me, you have 30,000 + hearts (and lungs!) beating in gratitude for you guys right now. And to everyone else who has ever donated or raised a penny for this cause, a huge shout out and thank you to you also.

I may have nightmares about stairs, but I have some pretty amazing dreams as well. And you guys are making them possible.

Friday, February 5, 2010

Get What You Need

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

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


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

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

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

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

The rest of my body.

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

It really hasn't been the greatest of weeks.

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

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

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