Thursday, July 28, 2011

**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?"


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.


  1. I have to say that this is such a struggle in our house and the MOST frustrating thing about this disease. We were told 'do your treatments, exercise, eat right, take your pills, see your doctor.' Aidan is only 6 and does ALL of these things 110% of the time. We do everything and anything in our power to beat this illness. But he feels sick all the time. In his case, it's not the lungs but the sinuses. This causes him to need IVs every 2-3 months,a port a cat, MRSA drugs on a frequent basis, surgeries every 2-3 months and he lives with agonizing pressure and headaches each day. His doctor says 'do your rinses'. He's been doing them since age 1, multiple times a day and he still gets sick all the time. He is a perfectionist and is good at just about anything he does. Yet the one thing he works SO hard at (his health) fails him time and time again....It is so defeating for him and he's only 6. I worry how this will affect him...He sees no end to this and cries how he can do this forever....I wonder sometimes how he will.....Megan

  2. Wow, I love this post Piper! It gives good insight into what it is like to fight this disease every day...especially good for those of us that are caregiver and won't ever really know how hard the struggle is to go against CF for our children. Thanks for sharinG!

  3. I love this post as well. I think I will be copying for my blog later today after my appointment.

    Also, I second your thoughts on your friend. If she's reading, there's no reason on this planet I can see why they'd say no to her. Short of the possibility that she's growing some evil bacteria that tx centers don't like. And even that wouldn't be her fault. It would just suck a huge nut.

    Livejournal is being a moron, so it won't let me sign in with that name. But this is Emily65Roses. :)

  4. What an excellent post. I feel so much for the young woman who emailed you, and hope she is plugged into the larger CF community where we can give her love and support.

    I'm pretty compulsive about my health, like many others. Never skip treatments, etc. So when I began culturing MRSA four years ago, my mind immediately went to the place where I tried to figure out where I must have dropped the ball. I know it's nonsense. I really think it is just human nature to want things figured out, and to play the charade that we are in control.

    Imagine my surprise to live to 57 w/CF only to start having joints replaced left and right due to an aggressive, erosive arthritis. Boy, did I not see that coming!

    For me personally, it's all a lesson in letting go. Letting go of the control freak-ism, perfectionism, judgmentalism and anxiety. My trust is in the Lord which gives me such a peace and joy. I'm just a pilgrim passing through here. I strongly believe the world is hungrily searching for examples of people who suffer well. My goal is to be one who does.

  5. LOVE this. My heart breaks for this girl. You knooowwww I've got lots to say on this one ;o) I'll try to condense it down from a mental manifesto into a blog post sometime this weekend. Looking forward to everyone else's perspectives!

    Fabulous and eloquent as always, beautiful you.

  6. Thanks Piper. As usual a lot to think about.

    We all work too hard at "doing everything right" that we won't have any regrets that our disease progressed faster than it needed to. It's both a driving force that keeps us going and a weight/responsibility that at times can seem awfully heavy. It is what it is....good with the bad. I try to let the pressure to "get everything right" be a positive's only when CF takes me out of the game for awhile that it gets kinda I fight getting benched with everything that I've got!

  7. Challenge Accepted :

    Though I do feel that I rambled more than answered... Oh well... :)

  8. Loving the post!!!

    Here is mine:

    I don't even know if I made any sense haha

  9. Joining into the discussion:

  10. And here's mine:

  11. I came over here from Stacey's blog and just have to say, this is a topic that has been on my mind a lot this month! I'm hoping to have the chance to take your blogging challenge this weekend. Thanks so much for writing such an insightful, articulate post on a topic that is VERY underrepresented!

  12. Here is mine:


  13. Thanks for getting me blogging again. Here's my link:


  14. Okay, why are none of the links working?

  15. I found this challenge on several other CF blogger pages and I wanted to find the source! Great idea and it sure sparked great discussion among the CF community. Here is mine if you would like to check it out!

  16. It took me awhile but I finally posted my thoughts.

  17. As others have said, this post is incredible! Here are (a few of) my thoughts:

  18. I wrote a few days back, but it wouldn't let m comment... Here are my thoughts

  19. thanks for the posts, everyone! (and feel free to keep them coming.) i'd like to encourage people to check out the other blogs if they haven't already, and to head over to "Welcome to Joshland" for another wonderful response that isn't posted on here yet.

    hope you guys have found this even half as thought provoking and inspiring as i have!

    thanks for everything you do, beautiful people.

  20. better late than never i hope! here's my blog entry:

    i'm absolutely blown away by the wisdom, insight and emotional poise of my fellow CFers.

  21. I'm so glad you gave this challange. It really made me sit and think about how I feel about my CF and my life. Here is mine!!

  22. Hi Piper/all, I have taken the challeng and written a post about in on my blog. Keep writing, I enoyt it :o)

  23. I found this through Stacey as well. Mine is at . I love reading your blog Piper! You continue to amaze me all the time.

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