Top (CF) Chef

Just about everyone who knows me will vouch for three major things about my personality:

1) I rarely, if ever, cook anything edible other than, say, the occasional bowl of cereal or, when I'm feeling super gourmet, some sort of random stir-fry/5th-grade-science-fair experiment hybrid involving pretty much every single ingredient that happens to be in my kitchen at that moment;

2) That the aforementioned lack of culinary skills does not in any way, shape, or form dissuade me from religiously watching just about every cooking show known to man, often DVRing the episodes and re-watching them later (as if the results of the show might have changed?), plate of take-out Chinese food in hand; and

3) That I have been, at times, known to be just a little, tiny, teensy bit competitive . . . although this one is somewhat up for debate. (And I WILL win that debate, I promise.)

All of which combined led me to literally squeal with excitement when I recently received an email from our good friends over at Abbott Pharmaceuticals. Turns out that the makers of Creon (you know, the drug that allows so many of us to eat all that yummy food in the first place) are sponsoring a new program called CFChef. And in my opinion, not a moment too soon.

CFChef is designed to help people with CF meet the intense and sometimes challenging dietary requirements of living with this disease. According to Abbott, the program (which can be found at www.Chef4CF.com) is there to serve both as an educational resource for patients and families as well as a sounding board for the sharing of information and recipes. Awesome.

So where's the fun part?

Turns out CFChef is celebrating its new launch by hosting a CF recipes contest. Now I want you all to close your eyes. Imagine your favorite Top Chef/Iron Chef/Food Network Star. Imagine s/he was cooking a meal for a CF audience and needed your help. You have 30 minutes and a mystery basket filled with dried pasta, brazil nuts, gas-station beef jerky, and ScandiShake powder (chocolate OR vanilla, just because I'm feeling generous). Your time starts...NOW!

Okay, maybe not.

But the contest DOES start now, and the rules are relatively simple. Just go to www.Chef4CF.com and enter your favorite CF recipe before Sept. 28, 2011. The top four winning recipes will be selected by a panel of experts including:

-Suzanne Michel, CF dietitian
-Boomer Esiason, Former NFL Quarterback, TV Personality, and CF Dad
-Michael Symon, Food Network "Iron Chef" (ohmigod, ohmigod, ohmigod!)
-Ali Christensen, CF Patient and "America's Got Talent" Contestant (Not to mention all-around sweetheart, as I had the honor of meeting her and her equally talented sister this year at the CFF's Volunteer Leadership Conference)

Tell me you're not super excited already. Go ahead, try and say it with a straight face.

Personally, I'm not much of a chef (and my recipe for "Piper's Special Stir-Fry Surprise" is just waaay too top secret to leak out onto the internet), but you can bet that I'll be encouraging my friends and family to enter! I'm also super excited to see (and to try!) some of the recipes that I know all my brilliant, beautiful Breathheads are going to submit. And if sharing and helping out the CF community while gaining personal glory isn't enough motivation for you, ask yourself when else in your life you're likely to have a chance to submit a recipe to a healthcare professional, a sport's star, a singer, AND AN IRON CHEF all at the same time? Yeah, that's what I thought.

Seriously though guys, this is an awesome chance to help out your fellow CFers, strut your stuff, AND have some fun in a program sponsored by one of the CFF's major corporate partners. What more could you honestly ask for?

So here's to community, cooking, creativity, calories, and, above all, to curing CF. Happy cooking, y'all!

**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.

Our First Dance

It's funny how often I get asked about my life post-transplant as some sort of comparison to my past. In the past year or so I've had people ask if having new lungs means I'm "cured", had people wonder aloud whether I feel "better than you have in years", and even had a small subset of people worry that transplant (or at least my so far kind of up and down experience with it, I guess) might not be worth it at all -- might, in fact, be "worse than it was when you were 'just' dealing with 'plain old CF.'" And, yeah, pretty much all of those are direct quotes from various blog readers, CF friends, other friends, family members, and just curious new acquaintances.

I always do my best to answer this sort of stuff accurately, but most of the time I just find myself sort of stammering around trying to find the right words to explain the inexplicable. And then, this weekend, I was lucky enough to have one of the many experiences that helps put this sort of thing entirely into perspective. So from now on, folks, here's my answer.

My cousin got married last Saturday.

She was, just in case you were wondering, a beautiful bride. For anyone who knows her (or knows anything about my family, really) this really shouldn't be surprising -- the vast majority of my nearest and dearest are stunning both inside and out. She was also, and this is the awesome part, 100% the center of attention throughout her special day (and the many hectic -- but special -- days leading up to it). Exactly as it should be.

I watched her get married without interrupting the ceremony with my coughs or the hum of my O2 concentrator. I watched her and her beloved exchange heartfelt, handwritten vows and promises. When she asked me to do a short reading (SUCH an honor, by the way), I was delighted -- not scared or spending my time searching through medicine cabinets in the reception hall for a contraband bottle of cough syrup. I ate their fantastic wedding cake (with insulin, of course), I laughed easily at the toasts their many friends offered up to the radiant couple, I marveled at the way the rainy weather seemed to clear up just long enough for sun-dappled vows, I watched family movies of both of them that seemed to re-emphasize the joy that comes with growth and community, and then Donor Bob and I hit the dance floor for the first time together. I danced. Holy f-ing shit, guys, I DANCED!

Now, granted, I wouldn't say I danced the night away or anything. Let's not get carried away, shall we? My prograf levels have been ridiculously low lately -- even slipping to "undetectable" at one point, despite the fact that I do NOT screw around with the dosage of those drugs. As a result, I've started taking the pills and dumping them under my tongue three times a day to see if sublingual absorption and more frequent dosing can help bring all the pieces of the puzzle back together and stabilize my levels. Which means that every so often I stumbled in from some activity and sat amongst my extended family members carefully pouring the powder of several tiny little capsules under my tongue. And let me tell you all right now: for a gal who can swallow close to 20 pills without blinking an eye and with only a small sip of water, this method of time-consuming powder puffing was a little much. There's very little way to do it without attracting a good deal of attention in the process anyway. Chalk it up to family bonding, right?

I also spent a good deal of time and energy trying to make sure I didn't eat the wrong thing (thanks, immunosuppression!), or hang out too much in crowds, or bury my nose deep into the beautiful bouquets of fresh flowers my cousin and her wedding party had picked for the big day. I had to understand that I do, in fact, still get very tired very easily, that I don't have unlimited energy when I'm on a bunch of drugs, that I'm still coughing up some junk (spent a few hours of the weekend on the phone with various doctors to schedule surgeries and whatnot), and that I'm still combating a breathlessness that I hope is not associated with the lower prograf level. Yikes. I did my pills -- both prophylactic and treatment --, inhaled cayston 3x a day, was a little extra careful with my brand new port-a-cath site, and tried very hard to be a good transplant patient while still being a fun human being.

And then I danced.

Just imagine not seeing your extended family for several years (actually having cousins you have NEVER met in person) and then suddenly needing a lung transplant and calling on all of them to love you and support you. Imagine that they come through with flying colors -- going in every sense above and beyond the call of duty -- and that because of their love, their light, their prayers, their support, and their help (coupled, of course, with not just a little bit of kindness from strangers), you and your immediate family are still together, still one complete unit of a larger whole. Still, dare I say it, even relatively sane. Then imagine seeing all these people again -- all of those who have been worrying and loving and praying from afar -- and being able to walk into the room not just as yourself, but as someone who is there for another. As someone who, for one brief instant, is able to maybe pay back even just a tiny fraction of the love and attention that she received.

Kind of makes the whole prograf thing seem kind of insignificant, right?

So next time I get asked what life with new lungs is like, I think I'm going to dodge the question -- or at the very least punt it over to someone else to answer. Because I think my family would all agree that the woman who helped celebrate her cousin last weekend was not the little girl with CF that they knew as a child. And I promise you they wouldn't say that I was cured after witnessing my attempts to freebase immunosuppressants or eat enough gas-station junk to maintain my weight. They wouldn't call me a brand new person because, to them, I will always be Piper (and always the one who might be willing to throw the word "mawiage" into the poem somewhere, right guys?). And they would never say that it wasn't worth it, because, after all, I was there with them, celebrating her -- and of course they saw me dancing.

What I think they would say is that it is what it is. That the world is still turning. That beautiful, funny women are still marrying handsome, selfless men. That sunlight still occasionally makes a completely unexpected appearance at just the right time. And that thunder and rain are still equally a part of the process -- but that even those can sometimes bring just as many laughs as they do tears. Most of all I think they would say that the truest gift of all, sometimes, is the one that lets you be there as yourself, rather than simply as your disease. The one that lets you forget about your scars for one moment and focus instead on the miraculous joining of two very special hearts right in front of you.

The one that lets you finally hit the dance floor, despite your complications, in celebration of life, of love, and, most of all, of everyone who ever helped to get you through.

With love, light, and best wishes to Rachel and Chris, July 16, 2011.

The More Things Change...

. . . the more they stay just enough the same to drive you crazy.

December, 2009: Picture of dressing from port-a-cath #2.


July, 2011: Picture of dressing from port-a-cath #3 (in the exact same place!).


Happy belated 4th of July, beautiful people! As you can see, I've been a little busy, but I promise to be back soon.

xoxo,
Piper

Sleeping Lessons

It's 2:27 AM here in New York. My puppy is curled up peacefully next to me, dozing with his head resting lightly on my leg. He seems happy and comfortable, like there's nowhere else he'd rather be in the whole world. Most of all, he seems secure, which is amazing given the fact that just a little over a year ago he wouldn't sleep in my room at all unless I closed the door to lock him in. His preferred resting place back then was with my mom, not so much because he likes her better than me (though he does love his "grandma", no doubt about it), but because my labored breathing, humming O2 concentrator, frequent coughing fits, and restless shifting during the night made him visibly nervous. Loyal little guy that he is, he normally started out with me until I turned out my light to fall asleep, and then he was off to quieter, less scary pastures. And who can blame him? I would have run away from CF if I could have, too.

So it's nice to have him here with me again and to know that I've gotten to a point in my disease where I at least no longer scare small children and animals with my symptoms. Or, you know, at least not often.

See, as I already mentioned, it's about 2:30 in the morning my time. I was asleep until about 45 mins or so ago, resting after a long day that (once again) included a visit uptown to my favorite hospital, as well as a couple of other appointments, a PICC line dressing change, some prescription refills, and all the other fun activities that seems to go hand-in-hand with his disease. There's not a ton of crazy stuff going on right now, just inhaled and oral antibiotics for an infection found during my bronch last week, a little bit of scheduling for a new port placement and a much-needed sinus surgery, and a confusingly low prograf level. The last one bothers me a lot, mostly because I can't figure out how to make this drug stay consistent with my body. I take it absolutely everyday at the right time, and the level is still all over the place, which is -- as we all know -- one of the single most frustrating things for a patient (and probably for a doctor as well). But even with that little kink in the plan, there's still nothing really going on right now that I can't handle, or that I haven't handled about 9 billion times before, for that matter.

Only I just woke up crying. And when I did so, I noticed that, for the first time in almost a year, my dog wasn't with me. He wasn't even with someone else, since it's just him and me tonight in the apartment. He was, instead, out in my living room curled up in his bed -- the one that he sometimes uses as a last resort when he wants to sit somewhere and I'm up moving around. I was able to coax him back in here with me, of course, but I wouldn't say he was totally eager to give it another try. The good news is that he is, as of now, perfectly content right where he belongs.

And I know that I will be too, eventually. I feel relatively hopeful, at least, that things are going to get easier and that sooner or later I'll be free of infections and IV lines and nebulizers three times a day and constant appointments and procedures and whatever else. (Note to the universe: as far as that whole "sooner or later" thing goes, I'd really prefer sooner, if you don't mind.) And it's not even that those things are honestly all that terrible anyway -- after all, I did grow up with cystic fibrosis, so let's be honest: I'm used to it -- but it's just that right now I kind of wish that I could run away from it all again, and that's not exactly where I wanted to be more than a year out of transplant.

I think the strangest part of trying to manage all these expectations is that we have to have them because they give us goals, they give us drive, and they give us hope. But they also give us preconceived ideas of things that don't always work out exactly the way we plan them, and when that happens we can sometimes find ourselves awake in the middle of the night -- tired, cranky, and trying hard to convince our loved ones, friends, or even our dogs that we are actually just a little shell-shocked, not crazy.

As for me, though, I think I may need to take a cue from my darling Sampson (who, by the way, is now sleeping on his back with his tongue out, because that's how he rolls) and just do the best I can to regroup, resettle, and trust that things are going to seem less overwhelming and less scary in the morning.

Or, at the very least, that next time I wake up, he's going to be right there beside me.

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