Prolonged, But Not Saved

I don't know if any of you out there frequent or even glance at the online news source known as The Huffington Post. It's not my sole or even favorite place to go for news, personally, but I do follow it on Twitter (you can find me over there at @piperbeatty, by the way) and I even drop by the website every so often. And last night, as I was waging yet another (unsuccessful) battle for sleep against the twin terrors of prednisone and prograf, I noticed the following article:

Girl With Disability Allegedly Denied Kidney Transplant

Okay, let me just start out by saying that I have done exactly zero outside research on this particular story. I am not, in any way, shape, or form, posting the above link as a statement of fact or as a condemnation of CHOP (the hospital referenced in the article). I don't have any rights to this story and I don't make any representations as to the article or its content. Everyone straight on those facts? Okay, cool.

All disclaimers aside, what I did do is read the article. And while I was doing so, I found the following statement, attributed to a pediatrician and geneticist, that the young subject of the article should probably

"...use a 'living designated donor' who would understand that 'Amelia's life would be prolonged, but not saved, by the transplant.'"

And that, guys, is where I had to stop reading, look around, and then read the whole piece again from the beginning. Because, quite honestly, I was baffled.

What, exactly, is the difference between "prolonging" a life and "saving" it? I mean, heck, we all die eventually, right? If I throw myself in front of a speeding bullet headed straight toward an innocent child, I haven't really saved that child in the sense that she will never die, have I? What I've really done, really and truly, is prevented the child from dying that day, that minute, that instant, and from that bullet. Assuming that my attempt is successful (I don't know that when I make the decision to leap, after all), then the child will still die somewhere down the road, though I think we can all agree that it would hopefully be a long way down the road and not just around the next bend. But even if it is sooner rather than later, my act of jumping in front of the bullet is still considered "saving her life", right? I mean, if the kid dies in 6 years, no one is going to look back at my action and shake their heads sadly, muttering "well, that's a darn shame. She only prolonged little Suzie's life, when she thought she was saving it!"

On some level, I read the doctor's attributed statement as reasonable, I guess. It seems to be saying that the donor should know in advance that the kidney transplant will not cure the child's disease or other disability -- that she will likely still suffer complications down the road and possibly reject the kidney or damage it, or maybe die of other causes. And I get that, seriously. As someone who was blessed to receive new organs, I know the sad truth that these suckers are scarce (sidenote: go here and sign up if you agree with me and want to fix that!). I understand that not everyone can receive new organs and that doctors have to make extremely hard calls about who can and cannot be placed on the transplant list. All of that makes sense to me, even if it does sometimes strike me as incredibly sad.

But as to informing donors that their gifts are "prolonging" rather than "saving" lives? Well, that, to me, is where things get murky. I'm 100% for information and informed consent, but I also don't know how or why that information is really valid. What sounds more reasonable (and maybe this is more along the lines of what the doctor had in mind in the first place) is to tell donors and recipients alike that transplant is not always (or maybe ever) a total cure. Transplant comes with risks, it comes with side effects, and sometimes it comes with unexpected results. What it never comes with, unfortunately, are guarantees. And as someone with a disease that was not cured by my transplant (I still have CF in my other organs and in my sinuses), I also take it as somewhat of a given that transplant does not have to be perfect to still be a wonderful, joyous, and very much worth it miracle. I have no doubt whatsoever that the doctor quoted in this article would agree with me on that as well.

What scares me is the idea that potential donors out there might think their gift is any less valuable if it simply "prolongs" a life rather than "saves" it. That scares me because I have spoken to transplant patients who have told me that "even just having one deep breath with these new lungs would have been a miracle big enough to make everything worth it." It scares me because I know myself, as a patient, how valuable an organ is to anyone who is lucky enough to receive it, and also to their family and friends. It scares me because the real value of a life is not measured by the number of days it continues, but by the passion and the strength and the goodness with which it unfolds, even if that unfolding ends far too soon. It scares me because some of my favorite stories are short ones, and I want to make sure that those stories are cherished just as much as the longest novel.

Most of all, though, it scares me because it ignores the fact that all donors, all gifts, and all recipients are, at the end of the day, equal. The beauty of organ donation for me lies in the personal connection it represents: one soul reaching across the void or the fear to offer another soul a second chance, because we are, after all, made up of the same stuff. And the best thing about second chances is that they are always a testament to faith and spirit -- even if they don't always last forever.

So probably it is true, after all, that most transplants are more of a "life prolonging" surgery than a "life saving" one. But maybe the deeper truth is that if we could all be so lucky as to prolong the life of another, then we just might end up saving our own.

**Blogger Challenge: Personal Disease Perspectives**

I recently got asked a question by a CFer who was worried about her upcoming evaluation for lung transplant. She wrote me a very thoughtful and articulate email about the subject in which she noted that she did her vest 2-3 times every single day, took every enzyme and pill exactly as prescribed, and worked out 3-4 days a week on average despite her steadily dropping lung function and ever more frequent lung infections. She also noted that she sees her doctor at least monthly and goes on IVs every 3 weeks or so, during which time she carefully schedules her infusions to be exactly 8 hours apart and makes sure to follow any additional treatment instructions (such as extra rest or spacing out her calcium from her cipro) that her doctor might recommend. She follows a detailed dietary supplement plan worked out in consultation with her nutritionist and, in her own words, she "tries very hard not to let [her] mind make excuses for any lapses in [her] responsibilities to [her] body." She told me all of this, and then she posed a simple question: "Should I be worried that the lung transplant team at my hospital will decide I'm not a good enough candidate for lung transplant?"

Wow.

Faced with this sort of question, my first reaction was exactly what I hope all of yours was too, meaning that I basically just sat there staring at the email in shock and then started to re-read it to figure out what part of the puzzle I must be missing. "Good enough candidate" was her exact term, and for the life of me I couldn't understand why she was questioning herself like that. After all, the entire message leading up to that point was basically a textbook description of the perfect patient, someone both mentally and physically committed to fighting her disease and maximizing her chances for survival even in the face of some pretty tough obstacles. I thought surely she must have some other underlying health issue or random skeleton in her closet (did she smoke? was she secretly selling her antibiotics on the black market?) that she wasn't telling me about. Otherwise why would she possibly be worried that her transplant team would reject her? What would drive a diligent, motivated, and obviously smart young woman into a crises of confidence so severe that she was, essentially, wondering whether a panel of doctors would deem her "good enough" for a life-saving procedure?

So I asked her.

Turns out this young lady was worried not because of her compliance record or her past medical history or anything else having to do with her behavior either as a person or as a patient. She was worried precisely because, as she put it, "I keep reading stories about how people saved themselves through exercise, compliance, and changing behavior. I feel like I've tried everything, but my FEV1 is in the toilet and it keeps sinking lower even when I do everything I'm supposed to do. How do I know the doctors even believe me at this point that I'm trying my hardest to make things better? How do I even know I'm not missing something?"

Okay, I'm gonna say it again: wow.

The moment I read those words, I started crying. I feel like this young woman summed up perfectly the frustration, self-doubt, and fear that a lot of CFers unfortunately feel when they suddenly start to lose control over their health. CF is a crazy disease in that it is controllable, to a point, through proactive care, self-responsibility, and treatment. But that whole "to a point" caveat is where things start to get tricky, because for some people the "point" seems to be around 90% control, whereas for others it seems to hover much lower. We're all pretty used to hearing that each CF case is different and that genetics, compliance, environment, and lots of other factors all play into our personal progression with the disease. But what about the fact that even two people with the exact same mutations, same FEV1, same bacteria, and same exercise program can still experience different results? How do we account for that in a medical system that, understandably enough, has to quantify things like Lung Allocation Scores and the risk of non-compliance after transplant based on general assumptions like "people with a 45% FEV1 are less sick than people with 29%" or "these treatments work, therefore patients who take them appropriately and as directed should see improvement"?

I think this email also struck a personal chord with me because of my own recent experience post-transplant. It's weird, I feel like I've been relatively healthy, but I'm also not blind: I can see that most people at my center don't seem to spend nearly as much time on IVs or fighting weirdly yo-yo like PFTs as I do. And if I'm 100% honest, hand on heart, I'd also have to admit that I've had a few "what the heck am I doing wrong?!" moments -- times when I have literally freaked out at myself for the perceived "sin" of getting another infection, or having a low prograf level, or not blowing hard enough during the testing. I actually had one moment when a PFT tech asked me if I understood the proper technique for the test and I very nearly forgot that I've had close to 30 years experience with this stuff before I stammered out a meek little "um, I'm pretty sure I know how to do it."

For those of us who might be dubbed "crazy control freaks" by others (I personally prefer the term "highly motivated, results-oriented individuals who might admittedly be somewhat crazy"), accepting that there are parts of our bodies that might not be 100% cooperative is challenging, to say the least. We have faith in the power of individual action, and when it fails we feel judged and inadequate. For others who are good at grasping the uncertainty of life with a progressive, chronic illness, the challenge might instead lie in keeping a sense of personal responsibility in the face of what seems like an invincible enemy. There's judgment there also (probably as much by the control group as anything), as well as a sense of powerlessness. Oddly enough, both of these approaches can eventually lead to the same point: a deep-set fear that our disease is in charge, and that nothing we do will ever be "good enough" to stop it. In the worst case scenario, both approaches might even cause actual non-compliance, with patients adopting the mindset that nothing we can do as individuals actually matters when it comes to fighting CF. (And yes, I know this from personal experience. I spent a good part of my teenage years convinced that if I couldn't fully control every single aspect of my CF then I was failing and should more or less just stop trying. I'm embarrassed to admit how long it took me to snap out of that trap.)

This is somewhat of an oversimplification, of course. As lifelong patients, most of us will fall into both camps at some point or another, switching sides seamlessly as we get older, or sicker, or go through different life phases -- or even adopting different views for different issues (those who might be hardcore when it comes to doing treatments, for example, but nonchalant about the power of exercise). It's more of a spectrum than a true dichotomy, in that sense, with a whole lot of middle ground in between the two extremes.

So since I couldn't really answer the question (well, sort of -- I did write that I would personally go down there and tell off her transplant team if they dared decide that she isn't "good enough" for new lungs), I've decided to put it to a panel of experts in an official "Matter of Life and Breath Blogger Challenge: Personal Disease Perspectives Edition." Here are the rules of the game:

1. Write a blog explaining your personal thoughts and experiences in dealing with CF control and progression. This could include your views on whether CF is in fact a "controllable" disease, your personal definition of compliance, your thoughts on whether (or how) someone with CF should be judged in terms of "good enough" self-care (what makes you feel judged? do you think those fears are justified? is judgment ever useful in this context?), your own struggles with control vs. unpredictability, and how you keep motivated in the face of so many questions. Or, you know, whatever you want to write about really. It's your blog.

2. Comment below with a link to your blog so that all of us can read your response. YOU DO NOT NEED TO LINK TO MY BLOG IN YOUR ANSWER. If you'd like to do so, please feel free, but this is about starting a discussion, not publicity.

3. Encourage your own readers to get in on the conversation by posting the same instructions on your blog. Remember, the more responses, the better the conversation. Let's see if we can get this one going as much as with past challenges.

4. If you don't have a personal blog (or just don't feel like going through steps 1-3), feel free to still make yourself heard by simply leaving a comment with your thoughts below.

5. Non-CFers are 100% welcome to participate, either by pulling from their own experiences or simply by offering their perspective as people, friends, and loved ones.

Much love, light, and healthy debate to all of you, beautiful people.

Inquiring Minds

Hello, beautiful blog readers.

I'm sorry I haven't written in a while. After I get out of the hospital there's always a sort of "regrouping" that occurs -- time when I just kind of struggle to get back into the swing of normal (ahem, well, normal for me anyway) life and all that entails. I'm sure many of you out there know the drill as well as I do: there are new med schedules to synch up with whatever routine you already had, new prescriptions to fill and doctors to visit, new additions to your day like extra blood draws or physical therapy, weight to gain (yay for no more hospital food!), friends to call, emails from frantic relatives/coworkers/teachers/friends/friends-of-friends/cousins-of-friends/mailmen/etc to answer, and relationships to be renewed after your short "mini-vacation." Honestly, I sometimes think the time directly post-hospital is more difficult for me mentally (and sometimes physically) than the time spent actually IN the joint. Granted this is probably because I tend to be pretty harsh on myself and focus on whatever I'm NOT accomplishing rather than the things I AM, but still. The fact remains that life post-lockup is sometimes, shall we say, somewhat less than relaxing.

All of which is just to explain why I haven't managed yet to sit down and write a real, honest to goodness blog post in the past week or so. And, quite frankly, why I'm still not quite able to do that today. Yep, sorry guys. I don't have any health updates (see my doctor again tmw, actually) or news to share right now, so I decided to take the cheater's way out and do something I should have done a long time ago.

Answer your questions.

See, if you glance over to your left, you'll see a contact info section on the sidebar of this blog. A surprising number of you have not only found that little hidden treasure, but also used it, for which I am both grateful and (gotta be honest here) totally shocked. The number of you who want to talk to me about everything from your wonderful lives to, well, your breath (or lack thereof) is both humbling and really exciting, because I get to see for the first time the depth and diversity out there within our little CF community. So cool. Anyway, more to the point, many of you also include in your emails questions about me, my life, my health, and my "everything in between." I try my best to respond to everyone personally, but I also get a lot of repeat questions and some that are just too unique and creative not to share with the whole. So, without further ado, I bring you the first ever edition of Everything You Ever Wanted to Know About Me (And Weren't Afraid to Ask!). Enjoy!

Q: Alright poser, you talk a lot about being "from Colorado," but I've also heard you mention a whole bunch of other places that you also say you've lived. Where were you born, anyway? And did your moves have anything to do with your CF?
A: The simple answer is that I was born in Colorado Springs, CO (a city renowned for its beautiful views, Olympic Training Center, and a whole lot of military bases). My parents both have roots in TX and OK, though, so when I was young we did move to Houston for a short time, then moved back almost immediately. The return to CO was, so far as I know, the only move that was primarily driven by my health needs. Beyond that I have lived in Boston, Denver, Atlanta, and New York City. Through it all I was blessed to have great care primarily out of Children's Hospital of Denver.

Q: Your dog is super cute. What the heck is a "shorkie" anyway?
A: Shorkie is a fancy name for "mutt" in that it denotes a shih-tzu (sh) and yorkie (orki) mixed breed. Sampson was sold to me (yes, I admit that I bought my dog. I am not, it turns out, the most socially responsible person on the planet) as a shorkie. In retrospect, I think this is untrue. He looks waaaaay too much like a Lhasa Apso for me to be satisfied with the "shorkie" designation.

Q: If you had your whole transplant journey to do over, is there one thing you would change?
A: Wowza. Um, yeah. I mean, I think so. It's actually hard to say, because the result was so amazing and I'm one of those people who truly believes that events flow out of each other, so I'm not sure I would "change" anything for fear of disrupting the final outcome, if that makes any sense.

I will say this: my family had a very dark time right before my actual call for transplant. There's a blog post about it somewhere (look around June 9th or 10th in the archive), but basically we were confused and thought that we had actually been knocked down on the list. We went out to dinner and had this tearful convo where everyone kind of let loose, and it suddenly became very clear that we were all really struggling to hold it together. I wish, in retrospect, that we could have been more honest about all that before the night in question. I wish we hadn't lost the faith, even for those few hours. Because getting the call from that dinner table was hard, and I was beyond shaken up by it all. I really wanted to be more peaceful as I was wheeled into surgery -- as it was the whole experience (from the fight at dinner to the disorganized chaos at the hospital upon arrival) was rattling. Not so fun, and I wish my last memories of my old lungs were a little sweeter.

Q: What are your top three things to have in the hospital?
A: Easy: 1) my own pillow/blanket, 2) something that connects to the outside world (preferably computer, but phone works), and 3) my own snacks. Obviously this assumes you would bring your own comfy clothes as well, but if not sub out #1 for clothes. I hate sleeping on hospital bedding, but I'd rather do that than wear a gown all the time!

Q: What are you finding to be the hardest thing about post-tx life?
A: Right after surgery EVERYTHING was hard. When I first came home I couldn't walk much, couldn't focus attention on anything, and couldn't really muster up much enthusiasm even for things I really wanted to do. I hurt and was tired, and I cried A LOT (weird for me, for sure). I actually wondered if I had made such an awesome choice. All that changed about 4-5 weeks post-surgery, and then I felt great. Seriously, it was a total "welcome to the world" moment...I just woke up one morning ready to kick some proverbial butt. And yes, granted, it was more of a transition than that in real life (little accomplishments every day added up), but it really felt kind of like flicking a switch when I finally crossed that line. Now I feel very much like me, only better!

Q: OMG, Piper, I've been reading your blog and I can't make sense of any of this. You got a transplant! You should be healthy! Why were you in the hospital? Are you going to get out soon? Come one, be honest. I can handle the hard truth. I just need to know one thing: ARE YOU OKAY?!
A: Yikes. Okay, first, take a second and just breathe. The last thing I need is any of my wonderful breathheads passing out from worry over ME (especially since I know we all have so much of our own stuff to deal with). So just relax, and then trust me when I say the following message, which I mean with all my heart and which is 100% the truth as I know it.

I. Am. Perfectly. Fine.

Yes, I have infections and yes, I had to go to the hospital. The thing is, though, I felt fine going in, and now that the drugs are done I feel fine again. I need to get my sinuses checked out because they may be dripping into my lungs. I need to get my weight up a bit. I need to increase my WBC count and I need to STAY HEALTHY. Other than that, though, I'm golden. The hospital stay mostly consisted of meds; meds that they don't like to do at home because of their side effects and/or special administration requirements. But for all intents and purposes I checked into the hospital to: 1) start polymyxin, 2) desensitize my body to cephalosporins, and 3) complete an in-hospital treatment for a virus. Easy peasy, right?

Q: What made you start blogging?
A: Basically I wanted a place to tell people (mostly CF friends who don't live in NYC) about my transplant eval experience. I realized pretty quickly that I didn't have time to fill everyone in on everything individually, and I started the blog to kind of post updates and thoughts that were too long or silly to repeat over and over in 50 separate emails. This blog is basically my response to the mass email, because I hate those.

As it grew, I expanded the blog a bit to include more day-to-day CF/tx stuff. My original thought was that this would be a chronicle of working life with with CF -- particularly in a fast-paced professional atmosphere. I soon realized that 1) while I was working I had very little time for blogging anyway, and 2) I was getting sick so much by the time I started this blog that my posts were less about balance and more about how to find a lifeline when you're drowning. I left work in April, 2009 -- about 9 months after starting this blog. I still believe that it is 100% possible to find a good, fulfilling, healthy balance between work and CF. I think I did balance work and CF effectively (not, however, perfectly) for several years. By the time I started this blog, however, it was clear that I needed to start scaling back and focusing on transplant. I did work full-time until about one year before my transplant, however, and I'm proud of that.

Q: How many nicknames does your dog have anyway?
A: A LOT. The most common names used on this blog are Sampson, Sam, Sammy, Sam-man (also written The Sam-man), Sammybear, Puppybear, Bear (also written The Bear), and Bearcub. Other people in my household call him Sammy-lito and Bearser, while my friend Julia calls him "The Muff" (short for muffin, maybe?). My sister, I should note, calls Sammy "Samuel P. Samuelson" (the "P" apparently is for "puppy"). This is decidedly NOT Sam's name, and I tell her that frequently.

Interestingly, none of these reflect the actual name on his papers which is, I kid you not, SAMPHSON. Yeah, I was horrified too.

Q: Who is this sister we keep hearing about? Is she older than you, or younger?
A: Erin Beatty is a wonderful woman best known for her grace and talent in performing 2 different (but equally thrilling) jobs: 1) Designer for SUNO clothing line, and 2) Sister to Piper Beatty, blog goddess extraordinaire. More to the point, Erin is kind, lovely, fun, smart, and incredibly poised under pressure. She has more than once kept my entire family sane and she has great fashion sense. She also once thought the Dire Straits were singing "Money for nothing and your checks for free," which I believe shows her many hidden talents as a lyricist and/or advertising jingle writer. She is slightly less than 3 years older than I am, and is the world's best sister. I am definitely the president of her (very large) fan club.

Oh, yeah, and it's also her birthday today. Happy birthday, Erin!!

And there you have it: more about me, my dog, and my family than you could ever want, need, or even imagine. On the other hand, if any of you DO still have questions (about anything), please feel free to send them over to the blog email (matteroflifeandbreath@gmail.com -- note that there is NO "a"). I don't promise to have all the answers, but I promise to give you my version of them, and that's the best I (or maybe any of us) can do.

Much love, beautiful people.