Tuesday, October 29, 2013

'Tis the Season

I originally posted this as a status update on my personal Facebook page as a way to respectfully chime in to one of my favorite seasonal traditions: the great flu shot debate. Two hours, 22 shares, and 16 comments later, I realized this was an issue that people need to be talking about, and so I decided to take it to some of the coolest and smartest folks I know -- all of you. Please be aware, of course, that this is my own highly personal perspective as a person, as a CFer, as a transplant recipient, and as a a strong believer in community health. Because what we do matters. I promise.

Every year around this time I watch for the first flurries. I sit in wait for those swirling flakes of debate that go hand in hand with the coldest season of the year and the inevitable question that, sooner or later, we all have to face. Should I get a flu shot?

Honestly, I can't answer that question for you. No one can, except maybe your doctor or your child's school depending on its vaccination rules. I can't tell you whether it's worth the so-called risks of the shot itself, or playing the bad guy and bundling your kids off to the doctor again. I haven't read every piece of medical literature out there on the subject, nor could I even if I made it my full-time job. I'm not here to discuss conspiracies or WHO statistics or the devastating spread of preventable disease in countries where vaccinations are not available. I'm not here to police your decision at all, in fact. I'm here to share mine.

I don't have a choice in the whole vaccine thing because when I got my lung transplant they made me sign a paper promising that I would stay current on all vaccines for the rest of my life (except live vaccines, which are dangerous for me). So every year, while other folks debate, I simply trudge out to my doctor's office, endure the poke, and keep my promise to myself, my healthcare team, and my donor. That said, even my doc admits that vaccines might not work on me very well simply because I don't have much of an immune system to react to the dead virus and produce the necessary antibodies. Because of this, everyone who lives with me or shares close contact with me on a regular basis is also required to get their shot to help keep me (and my precious lungs) safe. That means my boyfriend is getting his shot for pretty much the first time ever this year. Bear in mind that he's not so super into needles, that one, but he's doing it for me anyway. Love is never having to say "I'm sorry my genetic disease requires me to purposefully destroy my immune system so that you have to get a shot."

Here's the point, though: if he didn't know me, he would most likely be just fine without the flu shot. True, over 40% of the children who died of the flu in recent years were apparently not high risk patients, but even so -- most normal, healthy folks will be just fine, even if they do get the virus. But the fact is that he does know me, and chances are good that you, too, know someone who is deathly afraid of catching the flu for a really good reason. Chances are you know someone, even if you aren't aware of it, who has a lower immune system capability, or an underlying disease, or is elderly, or is pregnant, or . . . well, the list is pretty extensive, actually. These people are afraid of the flu because, to us, this virus is way more than an inconvenience or a few missed days at the office. And we care about the flu shot -- yes, even your choice to get the flu shot -- not because we want your arm to be sore or because we get a kick out of watching healthy folks facing their fear of needles. We care about it because this issue, for us, is about more than your right to build up your 7-year-old's immune system "the natural way." It's about our right to go outside of our houses between the months of October-April without risking our very lives because that 7-year-old (or his parent, classmate, teacher, or someone else who caught the flu after him) is in front of us in the grocery line.

Vaccination is a personal choice, right? But spreading germs once you catch them is not. Even with great hygiene and the best of intentions, those pesky little things have a way of making themselves at home on classmates, coworkers, friends, loved ones, public surfaces, and all sorts of other shared spaces. And maybe that's not a huge problem for you or for your child. In fact, maybe it's a risk you are willing, able, and even happy to take. That's fair. But before we make these so-called personal choices about preventative healthcare, maybe we should all look to our right and look to our left and then ask ourselves honestly: are we truly willing to risk the health of our neighbors (friends, family, strangers on the street) for the sake of this one "individual" decision?

Because that's the kicker with community health, guys. It's never as personal as we think it is.

So if you choose not to get your flu shot this year, please also consider staying home if you or a close family member is ill. Please consider the fact that most people shed virus for at least a day before they even show symptoms of a virus like influenza, and please consider educating your children about proper hygiene habits like coughing into their upper arm, washing their hands frequently, and sneezing into a tissue whenever possible. Most of all, please consider how your choices may impact the people around you and their overall health and well being as well, and once you've considered all of that, please make your personal, individual choices wisely.

Our health depends on it.

Thursday, October 24, 2013

The S Word

I believe in the power of words.

To me -- as a lawyer, as a blogger, as a speaker, certainly as an empowered patient, and even just as a person -- words have always been important. How we choose to express ourselves colors our perceptions and those of everyone around us, just as the words others choose impact our own understandings. As a kid I remember hearing that the Inuit and other American Eskimo tribes have something like 50 words for "snow" (FYI: read this article from The Washington Post if you're interested in learning a little more) and being fascinated by the idea. Mostly I remember thinking that, as a Colorado girl, I should probably have at least 25 of my own words for snow, and also that clearly this language thing was more complex than I thought it was. More specifically, I was intrigued by the notion of having multiple words to describe a single phenomenon, and the way that choosing one word over another might change the whole darn thing. And then I went back to coloring or multiplication tables or whatever it was that I happened to be doing at that time. Because, you know, I was a kid.

Fast forward to last week somewhere in a major research hospital in the Southern United States.

I was sitting in yet another waiting room awaiting yet another exam for yet another lung transplant and feeling an extreme sense of been there, done that syndrome. After all, I have been "here" (as in the physical state of end-stage lung disease) before, and I have done that insofar as I've already come through this entire process once. I was wearing what might as well have been the same hospital gown and lugging what might as well have been the same O2 tank and sitting in what might as well have been the same uncomfortable chair thinking what might as well have been the same slightly scattered thoughts while trying to push aside what might as well have been the same ugly fears and series of "what ifs..." from my brain. In short, I was frustrated and I was tired and I was ready for the test to be done, so I decided to distract myself by looking through my "transplant folder" at some of test summaries and other medical documents I had either brought with me for  or accumulated during the evaluation. I opened it up, grabbed one at random, and began reading the "notes" section.

"Ms. Beatty is a 31-year-old female who suffers from cystic fibrosis and advanced rejection of transplanted lung..."

And that's as far as I got.

I've seen the words "suffers from cystic fibrosis" so many times that the phrase has almost lost its meaning. Almost. But seeing it there, in black and white on a piece of paper by a very well meaning physician not-so-almost took my (very limited) breath away. Sitting there in that waiting room in the scratchy gown and the uncomfortable chair, I sucked my oxygen and gave a furtive glance around me at the nurses, doctors, other patients, and support people nearby. Then I asked myself honestly: were we suffering? Or, more to the point, I guess, was I suffering? One minute ago I had just been "bored" and maybe "tired" or "annoyed." Was I now supposed to suffer on top of all that?

Did they get it wrong? Or does the medical community just have 50 different words for snow...er, make that "suffering"?

Look, cystic fibrosis isn't pretty. Chronic rejection isn't pretty either. Neither of them are afflictions I would wish on, well, frankly I wouldn't wish them on my worst enemy to tell you the truth. Most people I know with CF feel this way, even those who claim it as a blessing in their lives because they feel it's made them stronger, more grateful, or whatever other moral high ground illness might give us a right to claim. I don't disagree with their perspective (who am I to do so anyway?), but for me having cystic fibrosis has never been about the blessing or the curse. It's something I have, plain and simple. It's something I fight. It's something I'd like to see eradicated and cured altogether so that no one else ever has to go through it, because I don't believe you need to experience CF to have a happy life. And I believe that given the choice, most of us would choose not to have it in our lives or in our children's lives. Because it is painful, it is frustrating, it is hard, and it can be deadly. It can be just about the most despicable little monster on the planet.

So does that mean I suffer?

Sitting there in that waiting room, I had the overwhelming urge to grab my pen, cross out that line, and rewrite the sentence to read "Ms. Beatty struggles with cystic fibrosis and advanced rejection of the transplanted lung." Because you know what? I do struggle with it. Every single day. I struggle to breathe now, but even before that I struggled to find time to do all my treatments with CF lungs, to find balance between life and an illness that just wouldn't go away. Sometimes I struggle with my doctors, or with myself, or with my care-partners (my preferred word for support people), or I struggle to deal with some of the side effects from medications. Sometimes I just struggle to keep it together in a crowded waiting room, and hey, who hasn't been there before? I struggle against cystic fibrosis every time I go out with the mission to raise money for a cure. I struggle against it when I watch other people fighting this fight, or watch my family watch me. I struggle against it because I don't want to surrender and I refuse to suffer. So, yes, I struggle sometimes with cystic fibrosis/lung disease, and you know what? I'm okay with admitting that.

Cystic fibrosis is not optional. I was born with it, I have it, and I have to figure out how to live with it every day. In a similar vein, I know that "rejection of the transplanted lung" in my case was not anything that I (or my body) did "wrong." Because believe me, if there were some huge, looming mistake that I made I would have found it by now -- God knows I've been searching. So I also know that being where I am right now was not optional either. Neither of these were choices. But I know that, for me at least, and at this stage of my disease, suffering would be.

Maybe that's just the linguistics nerd in me again. Maybe it's because no matter how much I struggle with CF or chronic rejection or SVT or any of the other ridiculous health issues that seem to rear their ugly heads at inopportune moments, I still consider the rest of my life to be pretty effing amazing when you really stop and think about it. Maybe it's because I have a multitude of different words to describe how people deal with the pains and realities of chronic illness, and to me suffering seems like a last resort. Maybe that's because I've been lucky -- and believe me, don't I know it. Maybe it's just because I'm too darn stubborn to admit I'm wrong. And maybe, just maybe, that little bit of stubbornness is enough to keep me alive.

Or maybe it's all just semantics anyway. But I, for one, still struggle with it.

Sunday, October 13, 2013

Just Breathe

A few years ago I had a minor, unsedated procedure to remove an infected port-a-cath. At the time I remember lying on an elevated platform in my vascular surgeon's office with a bunch of sterile dressing draped over my head and neck area, with a bunch of men hunched over my pretty much naked chest. And yes, it was every bit as much fun as that sounds. But I digress.

At some point during the process, my surgeon needed to numb up the area enough to cut my skin and vein open and pull the evil cath-of-death out. This involved sticking me repeatedly with lidocaine, in my neck and chest, while I lay there sick, semi-undressed, basically blind, and breathing through sterile sheeting. And because he was a good surgeon and recognized that I might not be entirely comfortable with the situation as it was, he instructed me carefully that the numbing step was about to happen, that it would hurt, and that he wanted me to try and breathe through the discomfort -- physical and mental -- because it would only be temporary and the result was important. Breathe through it, he said. Interesting choice of words, doc.

Breathe through it. 

Later on, when my lungs failed, a pulmonary rehab trainer told me that during times or activities of high stress, most people tend to breathe erratically. Or, to put it another way, to stop breathing altogether. When lifting heavy objects, for example, the natural tendency is often to hold our breath until the burden is removed. When faced with a scary situation we breathe in rapidly -- and then we hold it until we believe the danger has passed. Heck, according to my trainer many of us go so far as to exhale completely before attempting even unremarkable tasks like climbing a staircase, which means we do so without the full use of our breath. And she must be right, because to this day I can remember hearing my mom's Jazzercize instructor crying out "don't forget to BREATHE" to an entire class full of women with tightly wound perms and colored leotards. In fact, to hear how often we as humans subconsciously choose to stop or just stunt our breathing, one might get the ridiculous idea that oxygen is an optional life source.

Guys, trust me on this one: it's not.

So where does that leave me now? Well, recently my lung function has taken a bit of a nosedive, leaving me with a whopping 22% FEV1 (the amount of air one can exhale in one second). And this is, sadly, what we might call NOT a great number. All of which means that after much consideration, time, and effort on the part of my amazing team(s) of doctors and specialists, I have decided to actively pursue a second lung transplant. At this point in the game with chronic rejection, it's become clear to all of us that retransplant is the next step if I am going to try for that long and heathy life I truly believe is possible. My days with Donor Bob, amazing as they have been, are coming to an end one way or another.

And I will breathe through it.

I will breathe through the discomfort and remind myself that this is only temporary. All of it. I will breathe into the hard stuff, remember to breathe when it gets scary, remember that there is no box so heavy that it can't be moved. I will breathe, and I will not forget. And I will take that 22% and use it for all it's worth -- I promise you that, beautiful people -- because that choice is and will always be mine alone. Not CF's, not chronic rejection, not any of the other little monsters hanging out in the shadows. I will breathe, because that is MY breath.

And there will be discomfort.

I hate this disease more than anything for what it does to my family. I hate having to go through this again because I feel like the rest of my body is so beholden to this one very temperamental part. And I hate watching as life gets smaller and smaller traveling down the medical rabbit hole -- again. I want my loved ones and myself to have more time to enjoy the things that make up our lives when we're not overwhelmed with this "sick stuff", want to go back to the days when my health was A factor in my day-to-day plans and not THE factor. I want all of you to be able to read this blog and smile and know I'm okay (I AM okay, by the way). But then again, I know the reality is that this discomfort, is only temporary -- and the result is important.

So I'm asking my loved ones, my family, and all of you to do me one huge favor:

Please breathe through it with me.

And together, we can blow them all away. 

Tuesday, September 17, 2013

An Honest Reflection

Well hello there friends, loved ones, and beautiful strangers:

I am writing this post because I looked in a mirror today. Okay, to be honest, I look in the mirror every day (fine, sometimes several times -- I'm a girl, after all), but today was a little bit different. Today was different because today I was caught off guard. And again, to be fair, I probably should have expected to run into a mirror or two when I walked into Bed, Bath, and Beyond. It seems like the kind of store where mirrors would be plentiful -- bedroom mirrors, bathroom mirrors, and, well, beyond. So, yeah, I probably should have been expecting to turn a corner and get hit with a random mirror attack, but I wasn't. I was surprised.

The first thing I noticed when I saw myself today, besides the fact that my jeans were somewhat dirty (to be expected after a day of trying to help unpack my new apartment), was my new scar -- the one from my melanoma removal last week, which also happens to be the one that currently is full of stitches until tomorrow afternoon. And this was probably noticeable at least in part because this 2.5 inch beauty is, in fact, on my face. Or, rather, on my chin to be exact. Oh, yeah, and did I mention it still has stitches? Also, that said stitches are surrounded by a bruise that makes it look like I slammed my chin repeatedly into some immovable object? Because yeah, that would all be true.

And this might also be the time to mention that I'm recovering from a cold, and that my lung function is such right now that walking to the store without O2 is enough to make me breathless. Because that's all the truth also -- for right now, at least -- and that's what I came face-to-face with this afternoon on aisle 4, right next to the rotating fan I was planning to purchase.

One word, guys: Ouch.

So, dear friends and strangers, let me say one thing in response to the mirror in aisle 4. Let me be the first to tell you that even when I'm standing there tired and breathless and sporting war wounds on my face, there is one thing I want more than anything else in the world.

Please look past all that. Please see me, instead.

Please see a woman who is smart, adventurous, playful, and even, on occasion, kind of funny. Please see the person who is stronger than facial scars and damaged lungs -- the kind of gal who pushes boundaries, who fights back as hard as she knows how, who just in the past year has come through some pretty tough stuff and has done it with as much patience and humor as she knows how. Please see someone, if you can muster the imagination, who has completed road races and raised literally hundreds of thousands for charities, who has a randomly silly laugh and a puppy who still thinks she hung the moon (shhh, don't disillusion the poor guy!). See someone who is loved, who is happy, who is not afraid of the future (not really, anyway) because she has faith in herself and in God and in all the wonderful people who surround her -- like you, for example. See someone who is a lot like you, actually, because she laughs and cries and gets overly excited about little details and votes and likes ice cream and she, too, wishes she could be on a beach somewhere instead of standing by the mirror in aisle 4. See a woman who could tell you all about living life for 3 years with breath and energy and health, because she had that and she knows deep down that she will have it again soon.

And how much of a gift that truly, truly is.

She could tell you about living your dreams and making a difference, about medical miracles and the selfless gift of organ donation. She could tell you about loving someone and feeling loved back, and why we should never take that for granted. She could take your hand and put them on her other scars -- the ones you can't see, the ones on her chest -- and let you feel what it's like to feel the breath of a beautiful stranger giving life to another. She could tell you about speaking in front of crowds of hundreds for a cause you believe in or she could make up stories and rhymes to make you smile, because that's what she's best at in the whole wide world. And she would ask you about your life, too, because hearing the stories of others is one of her favorite pastimes. She could make you believe in something, anything really, because we all have the power to share that with each other.

Or, if all of that is too messy (and believe me, it can get messy) then please just see a person, any person, who is trying to make the best of whatever life throws at them, and who is grateful, even for her scars. 

That would be enough, I promise. 

And mirror, mirror, on the shelf
Shine past our scars, and show our selves.

With love and light, beautiful people. 


Tuesday, September 10, 2013

The 10 Commandments of Life with CF (or Anything Else)

Well, this was quite a week. Beyond flying to NYC for transplant clinic and a meeting with my surgeon (both went well, by the way) and having the small and thankfully superficial melanoma removed from my face tmw (thank you, immunosuppression drugs!), I've been pushing myself pretty hard. Thankfully the push has not been without rewards -- getting to see my sister, for example, and learning that I am not yet at the stage where my transplant clinic wants to relist me immediately for transplant. And, of course, another reward that I think is always a hard won achievement in the world of CF and lung disease: a little insight into who I am personally and who I would like to be as I continue progressing through this crazy, beautiful journey we call living. 

In particular this weekend, I was inspired by the grace and style of some of the fabulous people I'm lucky enough to have in my life. People like my friend Ali, for example, who recently lost a beloved family member to illness after herself battling through a rough summer. Ali, you are the classic example of someone who always thinks of others, even when you have every right to be selfish or tired. Or my sister, Erin, who held steady through a truly crazy week and never once lost her confidence or grace under pressure. Erin, you remind me how important it is to be confident and proud. My boyfriend who encourages me to go with the flow and let it unfold, my folks who remind me of the importance of family, or any number of all of you, who never fail to set the bar for how much complete strangers can love and support each other through absolutely anything. And so, it is in honor of all of these people, and everyone else, that I offer my own insights into the amazing lessons you all have taught me. Or, in other words, "The 10 Commandments of Life with Cystic Fibrosis (or anything else)."

Thanks for the inspiration, beautiful people.

10. Thou shalt not compare thy life, thy coping skills, or thy appearance in the hospital to others, or to what thou hast witnessed on TV or on the Internet. Grey's anatomy is just a sitcom, and no one's hair or makeup looks like that in the ER. Honest.
9. Thou shalt not measure the day's success solely by activities completed, but also by smiles shared, quiet moments of reflection, and small kindnesses given and received. Some triumphs, after all, are small. 
8. Thou shalt honor thy mind as thy body; and thou shalt remember to appreciate both for what they can do, not to disparage them for what they cannot.
7. Thou shalt be kind to thyself and others whenever possible.
6. Thou shalt forgive thy mistakes and the mistakes of others, even if it takes a little time. This stuff is hard, and no one is perfect.
5. Thou shalt feel, even the hard stuff. No, really. That's okay. 
4. Thou shalt allow others to feel. Also okay.
3. Thou shalt strive for perspective. Thou shalt also know it's not always easy to keep. 
2. Thou shalt remember that thou wast made as thou wast made -- it is not thy job to apologize for thy thyself or thy body. It is a gift, not an inconvenience. I promise.

And finally...

1. Thou shalt love others as thyself, and thyself as others. Really.

And now, the age old question: what would you add? 

With love, light, and learning,


Thursday, August 22, 2013

On Tubes and Testing

So some of you beautiful breathheads have been asking about something I've mentioned a couple of times in recent posts, at least in passing. Namely, the impedance study. Many of you, it seems, have never heard of such a thing and have no idea what it's for or how it works.

Man, am I ever jealous.

An impedance study (or 24-hour probe) is a very uncomfortable form of torture -- um, make that "medical procedure" -- designed to test for acid reflux, or GERD. Reflux, as I'm sure many of you already know, is a pretty common issue for folks with CF, and many CFers take pills like nexium or Prilosec to help manage the symptoms of this uncomfortable but generally tolerable issue. Personally I've been pretty lucky on that front, all things considered. I didn't take any sort of medicine at all for reflux until after college, which is really the first time I ever experienced any symptoms. Since then, whatever reflux I have has been pretty well controlled with medication, and I've simply had to adjust to the extra pills and move on. 

Or so I thought, anyway.

Turns out the reflux, the annoying little bugger, is a pretty big deal in the world of lung transplant. In fact, many great doctors believe that reflux can play a significant role in chronic rejection, because of damage to the lungs caused by accidental aspiration of the acid. Lovely, right?

Because of this concern, I had a different test, known as the Bravo Study, done a little over a year after my tx. This test involved a trip to the endoscopy suite at my hospital, where a wonderful doctor inserted a tiny little chip into my esophagus and gifted me with a little beeper to wear, on which I could record GI "events" like heartburn or Indian food. After a day or so of this fun activity, I returned the beeper. I never saw the microchip again, though I'm told it left my body of its own accord. As medical tests go, that one was both painless and easy -- a definite win/win. The results showed more or less what everyone expected of an adult CF patient with manageable GERD symptoms: some reflux, well controlled with medication. My team decided it was mild enough not to be too worrisome from a transplant perspective. 

The impedance study, by contrast, was not placed via endoscopy. Rather, it involves a soft but not insignificant plastic tube inserted up the nose, down the esophagus, and into the stomach, then taped into position for 24 hours. It was inserted by a nurse in a regular old exam room, proceeded only by the snorting of some rather potent lidocaine solution. And now it is in my nose, sticking out like a little nostril antenna, until it gets removed tomorrow morning.

I can feel your envy, all of you.

In fairness, the tube doesn't actually hurt in the traditional sense. It's uncomfortable and makes me want to cough and/or just pull the darn thing out, but it isn't painful. The main sensation is just that there is something at the back of my throat (there is) that won't move (it won't). Not exactly pleasant, for sure, but not the end of the world either. In fact, the best/worst part of it is really the look of the whole thing -- "best" only because of the amusement value and the fact that they told me to just "go about my day" normally with this thing. Because really, who wouldn't want to just pop on in for some shopping or a nice stroll through town with a taped-up tube sticking out of their left nostril? I mean, seriously.

And what will we do with the results of this fun little style experiment? Well, I'm told this will help determine if I need further, possibly surgical treatment for reflux down the road. Yes, the very same treatment previously deemed unnecessary, which is why my team is hopeful that this new test may give new information. The surgery itself is also pretty common for post-tx people, and involves a simple procedure that most people tolerate well. I say, if it will help my lungs and doesn't involve any more tubes down the nose then bring it on.

And in the meantime, I'm ready to get back to a more normal look and all "normal" activities, which currently include moving into my new apartment and hopefully even being seen in public again minus the nose candy. Because I know that ultimately this new approach could help keep Donor Bob's wonderful lungs alive and kicking. I know it will be worth even the most uncomfortable of tubes and tests to get me where I'm going.

And this, my friends, is just a little impedance.

Wednesday, August 14, 2013

On Seeing Things Clearly

When I was about 15 or 16, I was told I needed glasses for most distance seeing activities -- things like reading a blackboard, riding my horses, or driving a car. In the context of my daily life at that time, the decision meant that I was more or less required to wear those things any time I was awake. Not a super exciting prospect for a teenaged high school girl, let me tell you. For a little while I resisted the notion completely, but by the time I got my driver's license I had decided that convincing my parents I was capable of driving without, for example, ignoring the road signs which I couldn't really read without assistance was more important that whatever vanity propelled me to "forget" my glasses at home 90% of the time. I started carrying the darn things around with me, and even wearing them in the car, which eventually led to wearing them on my horse and, finally, in the classroom, where I was surprised to learn that blackboards had actual words on them and not just random scribble designed to confuse students like me. Life got a little easier, and I finally accepted that maybe, just maybe, wearing glasses was a better solution than stumbling around blindly, even if my vanity did have to take a little bit of a hit in the process.

Then time went on and I eventually found myself in New York City, in a place where driving was rare and blackboards were relatively uncommon. I still had my glasses, but I began to wear them less and less, and that little change made me pretty darn happy. Happy, that is, until I started coming back to Colorado on a more regular basis and realized, through a series of (ahem) let's call them "reminders" from my parents and my boyfriend, that the whole "no glasses" era was decidedly over if I ever planned to sit in front of a steering wheel or ski down a crowded ski slope again in my lifetime. I ended up purchasing a new pair of glasses -- one that I actually liked -- and this time, for some reason, it felt different. The big difference being that this time around I was old enough to recognize the value in a tool that, though it may not be something I necessarily enjoy using, helps me to participate fully in the life I choose to live and the activities I choose to fill it with. Though I may still have a bit of self-consciousness where my glasses are concerned, I am happy to wear them if it means that I can drive myself where I want to go, reach my destination, and have even more fun while I'm there. And the being safe thing? Well hey, that's just a bonus.

So what does all of this have to do with CF and lung transplant, you ask? Well, around 2006 -- when I was starting my 3L year in law school -- my CF doc decided that it was time for me to begin using oxygen at night. As with the glasses prescription, this was one tool I didn't want in my life, and I was angry that my disease was forcing me to use something as "ugly" and "annoying" as an oxygen concentrator, even if it was only at night while I was sleeping. I remember being a strange combination of angry, confused, sad, and relieved when the gigantic home concentrator machine was delivered to my apartment and wheeled carefully into my room to rest at the foot of my double bed. I remember poking it with my finger unnecessarily hard, even pushing it around a little with my foot, and sighing dramatically at the loud sounds it made when the delivery man showed me how to turn it on. I remember wondering -- as I did with the glasses -- what other people would think when they saw it, whether it would shock them, whether I should try and hide the strange little monster or whether that would make it look even more ridiculous than leaving it all out in the open. Most of all, I just remember being over it, and after a minute or two of looking and staring and wondering I simply left the machine where it was and walked out of my room. For years after that I used the O2 as prescribed at night, but I never turned it on and rarely even looked at it during daylight hours.

That is, I never did so until nighttime turned into "with exercise" and exercise turned into "with activity" and, finally, activity turned into "24/7." By then I had conquered many of my issues with the oxygen machine, of course, based largely on the fact that I knew I needed the silly beast just to live. It was actually a relief, in some ways, not to have to make decisions about it anymore. No longer could I decide, for example, not to take a walk simply in order to avoid the oxygen problem -- now I had to wear it whether I was walking or not, so I might as well just go for the stroll. But I still looked forward to getting off of the machines and the tanks altogether, and I was more than happy when that day finally arrived thanks to the miracle of modern medicine, human kindness, and organ donation.

My old portable concentrator, a Sequal Eclipse on wheels. It rolled like a 
suitcase -- perfect for a city that was mostly concrete.

And now, I've once again moved home to Colorful Colorado, where not only are there more chances to get behind the wheel, but there's also a whole lot more to deal with in terms of altitude than there was back in The City That Never Sleeps (or Drives). And, true to form, this realization initially scared me, because in the back of my mind I knew that higher altitude + lower lung function was a recipe for the thing I had long since bid a joyous goodbye: the oxygen concentrator. Yep, you guessed it, I'm back in my home state and back on the juice, at least when it comes to activity above a typical walk around town. I picked up my new Inogen One G3 portable unit today, and so far I'm very impressed with its lightweight portability, its 4 options for pulse flow settings, and its ability to fit into a backpack for easy use while exercising. But with all that said, it's still oxygen, and oxygen is always a little annoying.

Sampson Bear, looking somewhat disgruntled at being a
 point of size comparison for the new O2 machine, an Inogen One G3. 

The crazy thing though? This time around it doesn't seem quite so bad.

Kind of like the glasses, this time I was actually excited to get the new tool, because I knew that it would help me go further, be faster, and maybe even breathe some new life into these wonderful lungs through the miracle not just of modern medicine and organ sharing, but also just plain old cardio-aerobic activity. This new concentrator, I am positive, will allow me to share more and better outdoors time with my family, friends, puppy, and boyfriend, and it will allow me to stay in better shape for if and when I need to pursue a second transplant. This time around, for whatever it's worth, my primary emotion upon receiving this little helper gift was one of gratitude -- because I know I'm lucky. I know not everyone with failing lungs gets the chance to have a tool with this much promise, just like not every kid with bad eyesight gets the right pair of glasses to fix the problem.

This time around, it seems, I know where I'm heading. And crazy as the journey might be, I'm excited to get where I want to go, to reach my destination, and to have a whole lot of fun along the way.

And I can't wait to see what happens next.

Monday, August 12, 2013

The Blame Game

The other day, over a sunny outdoor brunch with my boyfriend, I did something I rarely allow myself to do, at least out loud. We were sitting on a patio, sipping coffee and trying to plan our day around our dual love for being outside and active and the harsh reality of my compromised lung function and current lack (though we know it's needed) of oxygen therapy. As we sat there making and discarding plans, I suddenly burst out with what most people with chronic illness will tell you is the sentiment of doom: "God, this is so frustrating! Last year at this time I could..." 

Fill in the blank with whatever high-intensity activity you want and you basically have a foolproof recipe for self-pity. Last year at this time I could hike a mountain; this year I can barely walk a short hill without stopping. Last year I could, despite some medical challenges, walk and talk at the same time, with the same breath; this year I wait until I have recovered to even try and speak. Last year people were constantly telling me to slow down; this year I get to watch them all from behind (ahem, not always a bad thing!). Last year -- well, you get the picture.

The thing (okay, one of the many things) I love about this guy of mine is that he didn't buy it for a second. He listened -- maybe because he recognized that into every life a little self-pity must fall -- but he didn't accept it. Instead, he jokingly announced that since he and I met almost exactly one year ago next month that I could, if needed, just blame him for the whole chronic rejection thing. Then he quickly moved on to calling a friend to plan a walk that we could do together, albeit slowly, and one with a fantastic view to boot. Any wonder why I keep this guy around?

What it got me thinking about, though, is the notion of blame. There's a lot of folks involved in the care of someone with CF and/or a new organ, and a lot of thought goes into assigning responsibility for the whole process. It's MY job as the patient to be honest with myself and others and to take my drugs exactly as prescribed. It's my doctor's job as a medical professional to listen and make objective, well-researched recommendations about my health. It's my family's job to support me in my responsibilities, and the clinic team's job to support the doctor in his/her care plan. It's my insurer's job to negotiate a workable price to get me the drugs I need to stay alive and healthy. And it's my body's job, on some level, to simply cooperate with the whole ordeal. Any one of these cogs breaks down and fails to do its job, and Houston, we have a serious problem. So it's only natural, to some extent, that when something goes wrong the first thing we search for is the broken cog.

What's frustrating is when we find none, which is the case in my particular situation right now with chronic rejection. Sitting there pondering the question of who I should/could "blame" for this new little bump in the road, I realized without question that I did what I could to keep Donor Bob alive and healthy. I kept my appointments, exercised diligently (seriously, guys, for a while there I was a machine -- and I'm proud of that), took my drugs every day, dotted every "i" and crossed every "t" I could reasonably think of, with a few minor missteps along the way. Did I ever annoy my doctor by doing something wrong? Absolutely, because I'm still human. My cog might have had a few little rust patches here and there, but it never stopped turning and trying to do its job. And neither, so far as I can tell, did anyone else. In a way that's kind of scary, right? Because look where we ended up anyway. 

In another way, though, it's extremely liberating. The harsh truth is that illnesses like CF or chronic rejection are frankly not simple tasks to manage, and sometimes even the best-running machine can't handle every wrench these twin monsters throw into the process. What I can say wholeheartedly right now is that I'm glad I don't have to look back and wonder. I'm glad that the self-pity questions I'm asking (when I ask them at all) are mostly about "why did this happen?" and not "why didn't I listen?" I'm glad that if and when I have to part ways with my beloved Donor Bob -- whose beautiful gift I will never reject, no matter what my body decides to make of these lungs -- I will do so knowing I did his generous spirit proud. I'm equally grateful that I don't have to look so hard anymore for broken cogs. I'm ready, instead, to search for what is whole and what is beautiful. I'm ready to step into this next phase of the journey with confidence and trust instead of regret, with peace instead of pity, and with great expectations of the paths yet to come.

And I will get there eventually, beautiful people, even if the walk's a little slower this year.

Friday, August 2, 2013

Starting Over (Yet Again)

Okay guys, so where were we?

I feel like so much has changed in the slightly over a month or so since I last wrote, back when I was still in the hospital for my Campath infusion. For those who need a refresher, Campath is basically an atom bomb in an IV bag (you can read the more complete explanation here) -- it's a one-time infusion that knocks out the vast majority of a recipient's t-cells and thus hopefully stops the process of organ rejection. In my case, it was given after trying a variety of other treatments to slow/stop the progress of chronic lung rejection, which is a fancy way of saying it's a hard-core drug we brought in with the hopes of convincing Donor Bob to stick around for a little while longer. Because, quite frankly, I've gotten pretty attached to these lungs in the past 3 years, and I'm hoping to keep this relationship going for a long time to come.

I received the Campath back in June, and one of the things my doctors told me at that time was that we might have to wait several weeks to see any real results. So I did what any normal, self-respecting gal would do during that waiting period: I packed up and moved across the country.

I mean, come on, doesn't everyone do that?

So now I'm back in Colorado, which is my original home state, close to my family, people who love me, and the mountains I adore. I know I'm super lucky to be able to do this, thanks largely to the fact that my wonderful transplant team is willing to work with an equally amazing CF clinic out here to make sure I continue to get high-quality care for my beautiful lungs, and believe me: I am beyond grateful. Besides the obvious benefit of being out of the City during a time when I have little-to-no immune system to speak of, I'm also closer to my core support system, in a place where outdoor exercise is not only possible but almost mandatory (I'm just waiting for the day when hiking becomes a legal obligation in this state!), and able to test whether a change in venue and air quality might have a beneficial effect on my steadily declining lung function.

Because, sadly, that's where we are right now. And I'd be lying if I said that it wasn't absolutely terrifying.

My PFTs right now are hovering in the mid 30% range, which is not where I need them to be. In fact, to be perfectly honest, my current numbers look an awful lot like they did back when I started this blog in 2008. I'm hopeful that some of my ongoing "medical to-do list" tasks will help bump them up a bit (rehab and reflux and drugs, oh my!), but right now I'd be more than happy just to see the numbers stabilize. And believe me, I'm doing every single thing in my power to make that happen right now, from adding inhalers to pushing myself to stay active to simply believing that this is going to work out. Because it will. Because it has to.

Because I said so.

I'm not ready to throw in the towel on these lungs yet, though I am prepared to face the prospect of re-transplant when and if my wonderful team(s) of doctors decides that it's time. The idea of facing the entire process over again is daunting, sure, but that's a path that I've already decided to follow should the need arise. What I want more than anything right now -- aside from a magically high FEV1 at my next clinic visit -- is to walk down it knowing that I did all I could for these lungs, for Bob, for my family and friends, for Sampson, for my community, for myself. And I invite you to come along on this journey (yet again) because together, beautiful people, we have a way of making even the toughest roads a little less rocky. And because I know from experience that the muddiest crossings can make for the most beautiful miracles, and that, lovely friends, is exactly how I plan to make all this happen.


Friday, June 21, 2013

My Name is Piper...

Okay, guys, confession time:

I'm kind of a druggie.

Actually, I think the term my doctor prefers to use is "compliant patient," but come on -- let's call a spade a spade. On a typical day I take pills, shots, and a few drugs designed to be snorted directly up the nose. Supplement that with the occasional (nebulizer) pipe and at least a few binges of hardcore IV drugs a year, and it's tough to make the argument that I'm not completely dependent on the stuff. I'm more than a little grateful for the chance to have all these little treats in my arsenal against rejection, infection, and their old pal cystic fibrosis. And I think it's fair to say that it's come to a point where getting my daily fix is more than a habit -- it's literally a matter of life and, well, breath.

And I'm also pretty lucky, because I can tolerate almost any new drug my doctors decide to throw at me  in terms of allergies or side effects. Longtime readers may remember that right before my transplant I was reacting to literally everything under the sun, but in terms of relative severity to many of my fellow journeyers down the CF road, my "no deal" meds list is pretty small, and is entirely limited to antibiotics. Even drugs like the rabbit juice (um, make that "rATG") don't seem to impact me much, and beyond a slight sense of discomfort or irritation I'm generally good to go.

And then I met Campath.

Yesterday wasn't terrible, honestly. They premedicated me with a LOT of drugs, including IV solu-medrol (steroids), a small dose of oral pred, IV zofran (anti-nausea), tylenol, and benadryl. They also ran the drug pretty slowly, with what I understand was 30mgs infusing over a period of 2 hours after a dose of prophylactic IV antifungals. And the whole time I'm sitting there kind of giggling at the process, because I think there is NO WAY I'm going to need all these drugs because, really, I'm pretty darn stoic when it comes to medical stuff. So we start the drug and I upload an episode of Project Runway on my computer (let s/he who is without reality TV show sin cast the first stone), and there we go. Easy as pie.

Easy as rancid, gross, totally disgusting pie.

About 1 hour, or halfway, into the infusion I suddenly felt extremely tired and nauseas. Approximately 30 seconds later I had my head in a hospital basin and things were not looking pretty. My long-suffering mom ran to grab the chemo nurse while I contemplated the truly gross reality that is hospital food the second time around. Trust me when I say that I will never again claim that hospital food "looks like vomit." Been there, done that, ain't going back.

After a few minutes of that awesome activity, my body decided it was done trying to rid itself of IV poison via traditional methods, and decided instead to go into hibernation mode. They checked my vitals as I stared stupidly at the machine wondering what all those pretty numbers meant, and I emerged from complete burnout only long enough to insist I wanted to continue the infusion. Then I conked out for the entire rest of the treatment and then some, which I highly recommend to anyone thinking of receiving this drug. This has reaffirmed my long-held belief that the best way to combat problems you absolutely cannot solve is to go to sleep. Because when you wake up you'll probably see things differently, be refreshed, feel ready to tackle the rest of the day, and um . . . have some really cool looking hives?

Okay, maybe not so much that last one.

The hives didn't show up for a couple of hours after finishing the treatment, which apparently isn't that uncommon with this particular drug. It started with a distracting itch on my leg, and when I went to examine it I found a couple of small red bumps. I wasn't concerned, checked my stomach and scar area only to discover more hives, and chirpily called my nurse for some additional benadryl, which she promptly brought within 5-10 minutes or so of the request. By which time I was a raging ball of hives madness. And yes, I'm serious. It was like 0 to 6 million hives in 5 minutes flat.

I'm still pretty lucky, by the way. In the grand scheme of things, vomiting, aches, fatigue, and hives aren't much to complain about, especially as it seems I got off pretty easy compared to some people. There was no closing of the throat, no scary "oh my God I can't BREATHE!" style moments, and nothing particularly remarkable about the entire event -- unless you count my lifting my shirt right up to my scar line in the hallway to show a member of the medical team (some of) my spots. Classy. Thankfully that too was a relatively minor blip, as the very old man with the walker seemed completely oblivious to my very red, very bumpy midriff at that moment. Can't say that I blame him on that score, honestly.

The truly great news is that I woke up this morning with far fewer spots and hopefully at least a some fewer t-cells. I asked the other day how long it takes Campath to "work" in the body (i.e., how long before I have the desired effect of dramatically lowered t-cell counts) and was told that the drug works actually remains in your system and continues to kill t-cells (and b-cells, by the way) for a long time after you receive it. This is why it takes so long for t-cells to regenerate after this treatment. As for how fast my own counts are going down, that appears to vary some from patient to patient. In other words, I may be taking some of these suckers home with me after all, but they're not off the hook by any stretch.

So there you have it: the good, the bad, and the totally gross. Suffice it to say that I'm pretty sure this drug would have a very low street value if offered to the general public. It is NOT a fun ride, but I trust my team wholeheartedly and truly believe they are giving me the best chance at keeping Donor Bob around for a long, long time to come.

And that, beautiful people, is a habit I'd prefer never to break.

Thursday, June 20, 2013

On the War(Cam)path

So in case you haven't heard, I'm back in the hospital.

Before anyone freaks out, please know that I am not "sick," at least not in the traditional, infectious sense. I don't have a virus or a bacterial infection brewing in my lungs, no fever or strange symptoms, I'm not in pain or dealing with chills or nausea . . . at least not yet, anyway.

What I have right now is what most people want: an immune system that refuses to quit. Unfortunately, in a transplant patient, too much of an immune system is definitely too much of a good thing. And in my case in particular, it appears that we may be dealing with one (or both) of the following: recurrent acute rejection (recurring inflammation in the lung tissue and airways indicating an immune reaction, nonresponsive to traditional therapies like steroids) and chronic rejection (bronchiolitis obliterans syndrome, which is basically just persistent scarring and obstruction of the airways). Either way, the one thing that's clear is that my immune system and I have reached a standoff. It's been crampin' my style for awhile now, and there just ain't room in this body for the both of us.

So I'm officially evicting my t-cells from the premises, starting today.

T-cells are a subset of white blood cells that are part of the lymphocyte family. They fight infection by attacking intruders to the body, such as viruses, bacteria, or (in some cases) foreign organs. They are the border patrol of the metaphorical country of the human body. And mine have been misbehaving to an alarming degree, which we already knew. We've tried several methods to rein these guys in, but they just can't seem to help themselves. And just in case you think I'm kidding, here's a brief recap of the therapies we've tried thus far:

Increased steroids
Added immunosuppression drugs (aka, azithromyacin)
Antithymoglobulin Rabbit, or rATG (See this post: On Rabbits and Rejection)
Photophersis (See this post: This Little Light of Mine)

And now, please welcome to the stage (drumroll please) . . . Campath.

Campath is a brand name for the generic drug alemtuzumab, which is as crazy as it is unpronounceable. In very basic medical terms, it's an antibody infusion that targets t-cells and flags them for destruction by the body, leaving the person receiving the drug with little to no mechanism infection control. The good news is this can help stabilize and sometimes even improve declining lung function due to rejection. The bad news is, well, you lose your immune system. Like, entirely. For months or even years. Small price to pay for lungs that work, right?

To bring it back to the border patrol metaphor, using photopheresis for rejection is a little like starting a civil war to distract the patrol from attacking the friendly immigrants (like, say, a transplanted organ). Photopheresis changes some of your t-cells so that your t-cells don't recognize each other and therefore begin fighting amongst themselves. Kind of a dirty trick, but hey: all's fair in love and transplant.

Campath is more like a nuclear bomb -- instead of distracting the patrol, it just annihilates them and hopes that something else (aka, IV and oral antibiotics, antifungals, and antivirals) can pick up the slack to stop the bad immigrants like bacteria and viruses. It's generally considered a riskier option because -- like most bombs -- it can come with some added casualties and damage that no one really wants to deal with. In addition to leaving me ridiculously immunocompromised, for example, this drug can also increase the chance of things like skin cancer. Thankfully, regular visits to other docs like the dermatologist for preventative screening can help out with those additional risks.

Overall, I feel good about the plan, although I haven't actually received the drug yet. I agree with my doctor that right now we need to be aggressive in wiping out these pesky little patrolmen gone rogue, and as always I'm somewhat fascinated by the complexity of modern-day transplant care. I'm grateful that we have this option and so so hopeful that it will help my awesome Donor Bob feel a little more at home in his new(ish) surroundings. And trust me, between the option of keeping Bob around and keeping those rebellious t-cells alive and hopping, well, that's just a no brainer. I've enlisted some of the best doctors in the world to serve as generals in this crazy revolution, and I have every confidence that they will bring me through it with as little collateral damage as possible.

And that makes this a fight worth picking.

Wednesday, June 12, 2013


Today marks 3 years from the day my beloved Donor Bob gave me the gift of life in the form of his precious lungs. It marks 3 years of all sorts of wonderful, from turning 30 to watching my sister come into her own as a fabulous designer; from meeting the children of some of my best friends to meeting someone absolutely amazing in every way. It marks 3 years of breathing, celebrating, and LIVING (with all the awesome ups and downs that word entails) and it marks 3 years of continued involvement in the quest to find a CURE for this little monster -- for all of us. The past 3 years have been hectic, amazing, scary, irresistible, indescribable, and -- above all -- beautiful. Every breath is a reminder of the gift I've been given, of the power we all have as humans to make a positive difference, and of the fact that too many out there still struggle for air. It is a lesson I learn and relearn daily, all because of an unknown stranger who had the grace to teach it me as his last act on earth.

And I am grateful.

Today also marks about 6 months since my PFTs began declining. So far, despite multiple treatments, IVs, bronchs, CT scans, and other fun procedures, we have yet to stabilize the numbers to my doctor's liking. So today now marks 2 days since I met with the doctors at a different, affiliated hospital to (re)start photopheresis, a blood treatment that may help stabilize my numbers by confusing the heck out of my immune system, for lack of a better description. (For those of you who like details, check out this post on the ins and outs of photo -- with pictures! -- for more information.) In addition, we're going to add a fourth immunsuppressant drug to my daily routine (this in addition to the prograf, cellcept, and prednisone I'm currently taking), and run some additional tests for other causes of lung damage such as reflux. Pending results on those treatment options, we'll make some tough decisions, but the end goal is get Donor Bob to stick around for as long as possible. What can I say but that after 3 years, I've become pretty attached to him.

And still I am grateful.

I'm grateful because today I got to take a jog/walk down to my old apartment, the one I lived in right after I left the Upper West Side. This was the apartment I lived in when I was lawyer, when I was first told I needed a transplant, when I got evaluated, when I started this blog, and when I first met Mr. Sampson Bear. It was also an apartment I lived in when I was sick. Like really sick. I stood out front of it today, in my running clothes, and I breathed. I breathed because I still have breath to do it. I breathed because Donor Bob, however down, is not yet out. I breathed because together we are still standing, we are still moving forward. And I breathed because it never hurts to take a moment to look back on where you came from -- even when you're not sure where you're going.

And then I turned around and went home. Round trip, it was exactly 3 miles.


Monday, June 3, 2013

Community Faces: Julie Wagoner

One of the exciting things about the Community Faces Project is the number of people without CF who have written posts. Parents, siblings, spouses/partners, family members, and friends of people with CF (crazy fools) bring a totally unique perspective to the table -- and it's a viewpoint that I, for one, never tire of hearing (or of learning from).

This is one of those stories. Written by an amazing CF mom, it captures that moment of recognition so many of us remember when we first understood the reality of cystic fibrosis. Perhaps not surprisingly, I relate to this story on an extremely personal level. I remember wandering into my grandmother's living room as a child, plonking myself down on the floor next to an end table that doubled as a bookshelf, and skimming the titles until my eye landed on the words "cystic fibrosis." The book was Frank Deford's memoir of his daughter's short but beautiful life, Alex: the Life of a Child. I snuck it back to my room and spent the next week or so turning its pages and shedding tears. Years later I would win the award from the National CFF that bears her name (that's Mr. Deford and myself on the right, holding the amazing painting I received for the award). She remains to this day one of the many CF spirit guides I carry with me on this journey.

CF was already a huge part of my life when I read about Alex's heartbreaking and inspiring life. But for many people who read the best-seller or watched the movie made of her life, that part of the picture would only unfold later. And this is Julie's wonderful story.

Community Faces: Sounds from the Underground

Name: Julie Wagoner
Relationship to CF: Mom to an almost 15 year-old CF superhero named Gage. DF508/N1303K, diagnosed at 23 months, upper right lobectomy at 7, two port placements, lots of bronchs and IVs, kidney stones, one sinus surgery, and a partridge in a pear tree.
CF Perspective: CF has been a struggle, but not one without rewards. The current reward is facing the dreaded teen years, which are absolutely kicking my butt! But, quite honestly, I wouldn't have it any other way.  

The year was 1986, the year I turned 14. I was a scrawny girl, burdened with prepubescent gangly limbs, stringy long hair, sporting sunburn and freckles, skinned knees and mosquito-bitten ankles scratched raw. It was summer. We were visiting family at the beach and I sat huddled against a door frame, my ear to the wall, peeking out from behind my cousin's bedroom door in order to see the television in the family room. My tears were hidden there, my throat sore from holding back sobs, embarrassed at the thought of being caught and teased, but unable to look away from the story unfolding on the grainy screen.
It was about a little girl, younger than myself, and she was dying. I'd seen plenty of movies before, about disease, about war, about monsters. I confess to being tender-hearted, though I hid it from most. But something about this story hurt in a way that was different, frightening. I grieved. I was grieving for a girl I had never, nor would ever, meet.
Nothing could be done for her? There was nothing? Surely that couldn't be right. Medicine could fix so much. How was it that this tiny young girl had no life ahead of her? I couldn't understand.
I watched to the inevitable end and stayed in my cousin's room that night, unsettled, sad, and confused. And while my vacation went on, as did my summer, she lingered in my thoughts. I found the book that the movie was based on at the library once we'd returned home, but I could not bring myself to actually check it out. It was too harsh, too cruel. Instead, I returned each week to find it, still on the shelf where I'd hidden it, and sat alone in a darkened corner, quietly reading the entire story, written in her father's hand, once more hiding my tears. No other piece of writing ever quite affected me the way that book did.
Had I known? Looking back I wonder if something deep inside me knew, even then, that the disease that stole this child from her parents lurked inside of me, hidden, silent, waiting. If some base instinct in my body foretold of what I would pass on to my son so many years later. If some part of myself had grieved, even as a child, not only for her, but for myself.
The movie was 'Alex: The Life of a Child', based on the book, by Frank Deford. It was about a girl named Alex, who died in 1980 at the age of 8 from cystic fibrosis.
My son, Gage, was born in 1998 and diagnosed with the same disease in July of 2000, 14 years to the month since I sat in that bedroom and watched the story of Alex. And because of her and her story, our lives are improved. Saved.  
I will always carry a piece of her in my heart.
Awareness. Pass it on.

Friday, May 31, 2013

On Playing the Tiles

It's the end of May, and also the end of National Cystic Fibrosis Awareness Month here in the states, but don't worry: we still have lots of Community Faces guest blogs to post. Because, after all, CF doesn't stop just because May is over -- and neither will we.

I was thinking the other day about what "awareness" means to me in and what it might mean to the other 69,999 people all around the world who struggle against this insidious little monster. Most people I meet on the streets today have some general idea of what I mean when I say I have CF, even if I do still get a lot of responses like "that's like asthma, right?" or even the occasional "oh, yeah, I think my my dad had that once." The visibility of our disease is increasing, especially with the buzz around new drugs like Kalydeco and the use of social media to help educate and inform more people about what CF is or why research is important. I have to admit, it's nice to see an option to donate to CF at the register of my local CVS or Party City, or to go into an ER these days and not have to be asked "when did you first catch cystic fibrosis?" by 75% of the staff. The proof's in the pudding, and we ARE making an impact. Awareness, it seems, is a beautiful thing.

Recently I read a book documenting the often hilarious underworld of competitive Scrabble. You know, the board game based on drawing tiles and forming words? Confession time: I'm kinda obsessed. In the book (which is called Word Freak and is a super fun read if you like journalistic nonfiction) the author notes that it's considered bad form at Scrabble tournaments to complain too much about the tiles you draw. Everyone has the same chances at drawing either a good or a bad rack, the author explains. The thing that separates great players from merely good ones is not what they draw, but how they play the letters once they have them.

I'm sure you see where I'm going with this.

Most people would probably assume that having CF means you drew a pretty shitty set of genetic tiles. In some ways, of course, they'd be right. I'm pretty sure that no one in their right mind would ever want to be born with a currently incurable disease that attacks two of the most basic and vital acts of survival: your ability to breathe and to digest food. I'm also fairly certain no one would willingly sign up for a disease that still kills children, that causes your body to literally drown itself in its own toxic goo, and that -- just when you think it's safe to let your guard down for just a single minute -- is constantly coming up with new and often super creative "secondary" issues to throw into its deadly mix. To be honest, when new parents reach into that letter bag, I doubt that any of them are hoping to pull out a "C" and an "F" for their children.

But sometimes it happens anyway, and then what? Then it's all about how you play the game.

CF awareness, to me, is about bringing other people in to play on our (winning) team. It's about reminding ourselves and the world that we are not beaten, not even broken down, and maybe just angry enough to push ourselves to play even harder. Seeing all the faces of kids and adults with CF filling my Facebook newsfeed during this month was a constant slap in the face about why I keep searching for newer and better moves to outsmart my opponent. Because what I am most aware of, personally, is that this is not a game we can afford to lose. These people -- myself, my friends, the kids I see at Great Strides and the parents I've comforted in waiting rooms -- are not "CF statistics." They are not the faces of this disease. They are the faces of themselves. Each and every one of the 70,000 of us worldwide is the face of a perfect creation, of a person with so much to offer the world besides just some vague notion of "awareness," of someone who is way more than just a CF patient.

That is the side of awareness I want to focus on. Forget the statistics for one second -- focus your eyes on a single face. Forget the monster in the darkness -- turn your face to see the amazing people standing in the light. These are the folks who learned long ago that they had better things to do than complain about their tiles. These are the people who are playing for their lives.

Turn your head. And help us win.

Wednesday, May 29, 2013

Community Faces: Amanda

Some of you might have guessed that I'm a big big super fan of the CF community.

Despite a lot of volunteer and advocacy work with The Cystic Fibrosis Foundation when I was growing up (a privilege offered me by my amazingly insightful parents, and for which I am beyond grateful),  I was not overly involved in what I have come to know and love as the Cystic Underground. For one thing, after all, there's only about 30,000 of us in the United States, and while two of these other 29,999 happened to live right next door to me growing up, I wasn't really on any missions to widen my CF circle in the way some CF kids of my generation did. I never attended CF camp, for example, nor did I seek out support groups or playdates even back when those were encouraged -- in the land before infection control. My parents had some friends with CF kids, and I met some through Great Strides or clinic or the hospital, but for the most part even up through college my contact with other CFers was limited to those I happened to run into either in the medical world or, occasionally, just through regular, everyday activities like, say, my church group in college.

Fast forward 25 years or so, however, and I realized that my CF was becoming more and more a part of my identity, rather than just a part of my lungs. For me, personally, this wasn't a bad realization -- it was simply an acknowledgement that my disease was looming somewhat larger in my life as an adult, and therefore required more of my day-to-day attention. As I entered the high-powered workforce, for example, I learned to reach out for advice from other CF folks who had been there, done that, and were in the midst of successful and fulfilling careers. As I moved out by myself in New York City, I sought support from fellow travelers who also had no one to bang on their backs or call the doc for them anytime they got sick, and I realized that CF adults can live safely and happily on our own. And as I faced the reality of lung transplant and struggled with how to react to my changing health status, I looked to my new friends in the CF community for guidance, support, stories, and survival tips. I don't think I'm exaggerating one bit when I say that you all helped see me through every step of the way, and continue to do so today.

All of which is just a small part of why today's Community Faces guest blog, from the founder of one of my favorite CF community hang outs, is super special. Huge thanks to Amanda for taking the time to write her story of faith, friendship, and so much more in the face of our shared little monster. I'm thankful for your friendship, your advice through the years, and that small little place you created "out of boredom" that has grown to have such a special place in all our hearts.

Community Faces: Sounds from Underground

Name: Amanda Berrie 
Relationship to CF: CF Patient -- DF508, R1162x
Facts about Amanda:  

I like painting, drawing and taking pictures.
I love my dachshund babies.
I can take or leave long walks on the beach, but the beach is fun.
Let's go fishing!

I don't really know how to write my story, so here we go. I was born seemingly healthy, happy and normal.  However I just wasn't growing like I should. A few doctors even started to accuse my mother of not taking good care of me. One day my mom saw a talk show, at a time she normally didn't watch tv, and they were talking about cystic fibrosis. A light went off in her head: "that's it!".  Finally a doctor listened and at 16 months I was diagnosed with cystic fibrosis.  And so the adventure began.

My parents, mom especially, instilled in me that although I have cf I am no different. My mother encouraged me in anything I wanted to do.  Whether it was playing with animals or being artsy, she always said "you can do it!", or "how can I help?".  She never let me use CF as an excuse, nor did she herself try to use it to stop me. I am forever grateful for that.

I'm a country girl, I grew up getting down and dirty and I still do to this day.  But I clean up real nice! :)   

I am married to my best friend. He is my leader, counselor, love, nurse, and protector.  God placed him in my life at the right time, and he saved me from the ugliness.  Although I had wonderful encouragement at home, at one point I let the ugliness of CF consume me.  That is the simplest way I know how to put it. Ugliness. Darkness. The Pit.  All consuming black hole.  I finally gave in and asked God to help me out of it, I couldn't do it alone or by myself. Yes I had my family, but something was just missing. This meant allowing God to help me and guide, the other part was having some one else along the way.  Enter my husband. Right time, right place.  Can you believe this guy even research cystic fibrosis on his own, and still wanted to date me!?!? With God, he pulled me out of that pit. While there is still ugliness, I now look for beauty and I see beauty everywhere. 

Through many hospital stays, PICC lines, ports, surgeries, home IVs, daily meds, treatments,  bad days, good days and all the quirks that come with cf;  I  learned quickly to enjoy the simple things in life.  The small things can be great and are what makes life so wonderful.  I was recently asked what is on my bucket list.  I couldn't answer. I enjoy everything as it comes.  My advice would be; don't be to busy trying to check things off a list and miss something that is right in front of you.

If you can't tell, I do have a strong faith in God.  ;) Above everything else, God guides me and leads me along this path, my foundation.  God is with me through everything.  This life is greater than myself, and if along the way I get to share my faith with someone else, that to me, is worth it.  If happiness, encouragement, God's love and hope is shared...worth it.

Almost 11 years ago I felt the need to reach out to the cf community.   I needed to talk to other cfers and at the same time I wanted to create a place for people to gather and get support, and build friendships.  So became a group called cf2chat created out of sheer boredom not thinking anyone would find it and if they did it wouldn't be many. To my surprise, it grew and grew quickly.   There have been many members come and go through out the years. Many life-long friendships made in the group for many.  There has also been great heart ache as members pass away.  Some times the pain is so great I would rather throw in the towel and hide away from the CF community all together.  That can not be done.  This group means so much to every one there.  This group has been something greatly needed for some.  People, whether they have CF or are a loved one, need this group and others like it.  The bonds that are made in groups like this are unique.  You connect with people who share in an experience exactly the same as yours, or at least similar enough you commiserate. You know what each other is feeling without having to express it. It is almost indescribable the support a person can receive, even if it is just a simple "We are sending you hugs."   

Since being involved in the CF community I have learned we are a unique bunch of mutants.  Strong, confident, appreciative, beautiful,  and supportive. Even if one doesn't feel that way,  they really are.  I feel honored to be part of it all. That may sound cheesy, but I like cheesy sometimes, and cheese....so cheese is good :)

To visit Amanda and more of the CF community online, please check out CF2Chat, a great support community for folks with CF and all those affected by this disease.

Wednesday, May 22, 2013

Community Faces: Jessi

People are always asking me whether I consider myself "sick."

The truth of the matter is that, most of the time anyway, I don't think of myself in terms of my illness. Through the years I've learned that it's far easier for me to go about my daily life if I'm not overly hung up on labels, diagnoses, medical records, or even the bronchoscopy report I saw yesterday that listed me as "class III: a patient with severe systemic disease." Thank you, grade assessment program.

When you get right down to it, though, I have a lot of medical codes after my name. Cystic fibrosis is my primary diagnosis, but it's followed by CF-related diabetes, lung transplant, complications of lung transplant and rejection, malnourishment (I can't even tell you how much I hate that one), immunosuppression, and a heart condition known as supra-ventircular tachycardia (SVT) -- among others. These are more than just numbers on a chart, of course, but when it comes to how I view myself they honestly don't make too much of a difference. Another day, another diagnosis, right?

That's part of why I love today's Community Faces post, written by a fellow CF lady who also happens to have a diagnosis of Epilepsy to add to her list of medical accomplishments. Special thanks to Jess for taking the time to write for the series and for sharing her story of life between the ailments. Happy reading, beautiful people.

Community Faces: Jessi Mollman

Name: Jessi
Age: 31

My name is Jess, I'm a 31 year old woman from Seattle, WA with CF and epilepsy.  My CF was diagnosed at about 6 weeks of age.  The epilepsy was diagnosed in 2010.

There is no known connection between CF and epilepsy, but epilepsy does make CF care more complicated.  There are some antibiotics, merropenum and immipenum are two, that increase the likelihood of seizures, especially in someone who has a history of them.  As a result of that risk those antibiotics have become a last resort for me.  Only if my bacteria become resistant to everything else will these meds be used for me. 

Sleep deprivation is a common trigger for seizures, and has triggered mine in the past.  This becomes a huge problem when I'm in the hospital or doing home IV antibiotics.  Its hard to sleep in the hospital because of the noise, the uncomfortable beds, nurses coming in to start meds in the middle of the night, among other things.  Even when I'm doing home IVs I have to adhear to a strict schedule and usually can't get a full 8 hours of sleep in a single stretch.  With my last round I was lucky to get 5 hours.  I know some people who adapt their medication schedule so they can get more sleep at night but I can't do this.  I have to keep the levels of meds in system as consistant as possible or I risk seizing.  I've actually had very small seizures when my antibiotic levels get too high.  I have to take every chance I get to nap or I risk having a seizure.

I was fortunate that the first anticonvulsant that my neurologist tried has been very effective for me and I don't have any side effects from it.  The only thing I don't like about it is that it interacts negatively with diflucan, an antifungal medication.  Antibiotics increase the chances of fungal infection because they kill the good bacteria in your body as well as the bad.  Diflucan is the easiest way to treat fungal infections because its just a pill.  However, since I can't take it I have to use creams or a mouth rinse when I get thrush in my mouth.  The creams and rinse don't work as fast as diflucan and the rinse tastes horrible.  Considering all that I have going on, is this really that big of a deal?  No, but anything that makes my life easier is a plus!

My epilepsy diagnosis was a total surprise, no one else in my family has it.  While it has made things a little more interesting for me I feel like I have adapted very well and continue to live my life.  Not much gets in the way of me having fun and taking care of business.