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.

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.

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.

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.

Again.