She's Not Heavy, She's My Cyster

One of my favorite things about this blog is getting lots of emails from readers from all walks of life: from fellow CFers to parents to siblings to medical professionals to people with other lung diseases to just really supportive strangers. The people are all different, but one thing many of them have in common is the question: "why are you so open about your disease and does it ever scare you to put yourself out there like that?"

There are about a million different answers as to why I choose to share my life with CF and transplant publicly. I could credit my parents, who taught me to be open about CF at a young age and who got me involved with the CFF basically from the word go after my diagnosis at 6 weeks old. I could credit the world for blessing me with amazing people who are all so understanding about CF and my health and who have never made me feel bad about myself just because I'm a little bit different. I could credit all of these people and more and I definitely wouldn't be lying -- it's amazing what a few good people can do for the soul in terms of keeping me honest.

But most of all, I credit you guys.

The CF community is, in a word, exceptional. My friends and "CF family" have truly stood by me "in sickness and in health", never afraid to laugh, cry, commiserate, or celebrate right along with me throughout the journey. In my most arrogant moments I sometimes fancy myself a "writer", but when it comes to expressing how grateful I am for everything you all have given me through the years, well -- words fail me. Honest. You guys are everything.

And this article, from today's NY Daily News, just proves it.

Not only am I lucky enough to call these lovely ladies my friends, I am also blown away by their courageous strength in sharing their stories. From Lyndsey's recent stint on NY Med, Katy's appearance on Say Yes to the Dress, Kristy's dedication to speaking to nursing students about CF and organ donation, Allison's appearance on cooking shows to promote healthy eating for CFers and diabetics, and Kelley's recent work signing up organ donors at NY's Fashion Night Out, these women are great representatives of the spirit and energy found throughout the CF community.

So how do I do it? Easy. I just remember that 30,000 heads are always better than one.

And then I follow your lead.

Reality Bites

One of my favorite parts of any visit to the hospital is leaving. Seriously, it's right up there with feeling better as one of the most pleasant aspects of an admission. Not that my hospital isn't amazing, of course -- because it totally is. I'm lucky enough to be seen at a hospital that has both an incredible CF team and a caring, smart, and proactive transplant team. Everyone -- from the nurses on the transplant ward to the PFT techs to the pulmonologists to the slew of other doctors/staff members I have on my team (infectious disease, endocrinology, ENT, thoracic and vascular surgery, allergy . . .  the list is pretty impressive) -- works hard every time I pass through those doors to make me happier, healthier, and just basically more Piper-esque. I'm incredibly grateful.

And yet, I still love leaving. I love packing my stuff and actually putting on street clothes. I love signing my discharge papers. I love cutting off my hospital ID and allergy wrist bands. I love walking outside into the fresh New York City air (ha, ha). I love looking back over my shoulder and waving goodbye at that wonderful team and everyone else still hard at work healing or being healed in that wonderful, awful, miraculous place. I love coming home to my puppy, my friends, my food, my bed, and a hot shower. I love, love, love, love, LOVE the freedom to wake up the next morning and know that I am free and I am alive and I am able to make happen anything I darn well want because I'm a rockstar and I can do it all by myself, thank you very much. I love it.

Well, okay, so maybe it's more accurate to say that I love most of it, because that final part -- the "making things happen" part -- is almost always easier said than done for me after a few days spent living like a sick girl in the hospital time warp. By the time I actually check out of the hospital and head back into the real world, it seems like I always find myself floundering for a couple of days before I manage to regain my footing. Even after 30 years I'm still not quite sure why this happens so regularly or why I never seem to learn my lesson and find a way to avoid it, but it does and I don't. Without fail the first few days of "freedom" end up being about 30% actual life and about 70% of me standing around looking bewildered and making countless but unhelpful "to do" lists about how to get back on track while I simultaneously agree to every single request made of me so as to prove just how "normal" and "NOT SICK" I really am.

Or, as one of my wonderful friends recently put it in an email:

"It's cool, Pipe. We wouldn't expect anything less from you."

Yeah, me neither.

I know I can't be alone in this. As a group, most CFers I know tend to be pretty intense people. As CF adults, we've lived with illness and hospitals and doctors for a long time, and many of us are masters of acting as nonchalant as possible in the face of what most people would consider some really serious health issues. I watch a lot of my CF friends make the exact same comments I've made my entire life ("Oh, the hospital? You mean that two weeks of IVs/rabbit blood/coughing up blood/bowel obstruction/pain/poison/whatever? That little thing? I got out YESTERDAY, man! I'm totally FINE now! Life is AMAZING!") and I always wonder: are they really feeling that great after all that, or are they just really darn good at faking it? Or is it maybe just a little bit of both? To be honest, I don't really know the answer.

What I do know, though, is that when most "normal" people come back from the hospital, they're expected to be tired, to rest, to require a little downtime. When I come back to the hospital, people apparently expect me to be extra intense. This little pattern I have of being manic right after an admission is, I've recently discovered, NOT normal. Apparently I'm NOT the status quo. And even more surprisingly, it seems like most "healthy" people are able to actually allow themselves to be sick and then allow themselves ample time to HEAL and FEEL BETTER without immediately feeling the need to prove themselves as superhuman the second they get discharged. Wowza, guys. Who knew?

As for me, I'm not sure "normal" is really in the cards anytime soon. Personally, I'd settle for a little more rest, a little less crazy, and a lifetime's worth of lessons on how to make that happen.

Mission: Possible

Captain's Log:  September 20, 2012. Day 4 of mission, 7th floor, 12:10pm.

Since arriving in the foreign world of "Planet Hospital" four days ago, our crew has been busy trying to repair the lung mechanism and conditions continue to improve. I have successfully made contact with that strange yet somehow wonderful species known throughout this place as "doctors" and they, in turn, have called in some sort of rabbit army to aid me in my quest. Though the rabbits are somewhat unpredictable (they seem to bring with them a whole slew of annoyances, from fever to general stomach discomfort), they are, as best I can tell, doing their job. Reports indicate that the invading t-cells are being effectively subdued, and one of the doctor leaders has suggested that I might be able to withdraw from stage 1 of the mission (that frustrating stage better known as "operation inpatient") as early as tomorrow evening.

My fingers are certainly crossed.

The inhabitants of Hospital are truly a unique bunch -- many of them seem able to run around continuously for days on end with little, if any, observed sleeping habits. The world is inhabited by a host of both daytime and nocturnal creatures and therefore remains at full activity at all times, which makes normal human sleeping patterns difficult to sustain. Also strange is that these energetic creatures apparently do not eat, as so far I have observed absolutely NO sign of edible food stuffs. This scarcity has been partially relieved by messengers from the distant realm of New York, of whom I am lucky to have several. These messengers not only provide nourishment, but also much needed company and the occasional picture of "King Sampson Bear" from back home. For them, I am grateful.

Yesterday morning one of the doctor leaders had scheduled me for an interesting activity known to his kind as the "gastric emptying study." (Doctors as a species, I should add, are certainly a curious bunch -- it's not uncommon for them to want to perform several of these types of study activities in a single day.) The first part of this mission was simple: I was told to refrain from eating the already inedible food stuffs served to humans on this planet. Done and done. Next they demanded that I eat radioactive oatmeal in place of a regular breakfast and lie down on a table under what they call an "x-ray" arm. They kept me in that position, immobile, for an hour -- a condition that normally would have annoyed me but which I found surprisingly pleasant, as it was the first quiet hour of actual sleep I've had since arrival. This morning, the doctor leader informed me that the study showed delayed emptying (par for the course in my particular brand of humans, by the way) but not to an excessive degree. After a short peace talk, he and I agreed to wait and see before trying out any new weapons on this latest enemy.

And so we sit. And so we wait.

If all goes according to plan, we should be launching the final two rabbit army assaults on the t-cells tonight and tomorrow, after which (please, please, please) I will be able to return home to King Sammy and restart most of my normal activities back in TransplantLand. I have been warned that the effects of the rabbits may remain with me for several days, but I am, as always, optimistic. If I can handle slaying the CF monster, after all, I think I can handle some bunnies. The continued plan for the remainder of the mission includes stabilizing the effects of that dreaded rejection monster through a few minor weapons adjustments and, sometime in the near future, a possible frontal attack on the sinus region to once again annihilate the Pseudo bugs. All in a day's work for someone with 30 years of wartime experience.

I'll sign off now, with the final thought that this mission, while scarier than previous assignments, has already renewed my faith in the treacherous exploration we call lung transplant. Though ultimately I hope more and more of our species will find such travels unnecessary for their particular journeys, I am grateful to have had the chance to wander this new terrain with such amazing support from my fellow travelers, the wonderful doctor leaders, and everyone else out there that makes up this world so strange -- and so beautiful.  

Until Next Time: Piper

Don't Think Twice, It's Alright.

It's been approximately 36 hours since I first checked into the hospital for this stay, not that anyone's counting. So far in that time I've received 2 doses of 500ml IV solu-medrol (the steroid, and for perspective my maintenance dose is 10mgs), 1 dose of IV ganciclover as a CMV prophylactic (with another to follow later tonight), 2 doses of oral prophylactic antifungals, 2 doses of oral antibiotics for my pseudomonas, and 2 doses of 100ml rATG (aka, "rabbit juice") infused over approximately 8-12 hours to soften the blow of the side effects.

Also in that time frame, the elderly gentlemen across the hall from me have shown no signs of learning how to properly secure their hospital gowns in the rear end area. Not that I'm looking or anything.

I woke up this morning feeling kinda crappy. The first reason for this is that "woke up" is a bit of a misnomer; it would be more accurate to say "after remaining awake for the entire night, I felt pretty crappy in the morning when OTHER PEOPLE were waking up." Shocker, I know. IV steroids seem to do that to me. Add onto that the combination of general malaise from the drug itself and people coming in and out of my room all night to monitor my side effects, and you'll probably get the general idea of why it can be so hard to sleep in this place.

The second reason for the crappy morning is equally shocking: turns out human bodies aren't so hot on rabbit blood. Crazy, right?

But it ain't no use to sit and wonder why, babe. We just do what we do here to keep me breathing, and quite frankly I'm okay with that.

It's weird to think of poison keeping me alive, but in all fairness that seems to be what's happening. Treatments like this remind me that my body is essentially maintained in a state of toxicity for its own good. Transplant is like a sticky kind of compromise I reached with cystic fibrosis and my natural desire to live: I gave up my lungs and CF gave up part of its hold on me, and in return I got a beautiful life full of love and laughing and walks with my dog and talks with my friends and the sight of the Colorado mountains or the New York City skyline at night. And CF and its cronies got a kind of "get out of jail free" pass for most of my immune system function and now they get to play fun little games with rabbit blood. Sick little bastards, aren't they?

But don't think twice, it's alright.

When I think of this disease it's hard for me to see it as anything other than a monster, even if my life is in someways "better" for it. Meaning, I guess, that my life is better for pretty much every experience I've been lucky enough to survive and learn from, even if it can be tough to notice in the moment. I won't say I'm grateful to CF (it's taken too many of my friends for me to EVER say that), but I am grateful for the challenge. And if I can't say that I've defeated it yet, I can at least say that I've won a few well fought battles -- and come out the other side as someone I hope I can be proud of. Just as I know I'll get out of here soon even if 5-7 days seems like an eternity right now, I also know that my walk with CF and transplant won't be over for a while. I haven't shaken this little monster, but at least I was strong enough to change the game.  So if I can't say goodbye until the moment when we all can say it together, well, I know that's alright, too. As a matter of fact, I'm looking forward to it.

So I'm walking down that long lonesome road, babe
where I'm bound I can't tell.
But goodbye is too good a word, gal
so I'll just say fare thee well.
And I ain't saying you treated me unkind,
You could've done better,
But I don't mind.
You just sorta wasted my precious time.

But don't think twice, it's alright.

On Rabbits and Rejection

H is for hate this. I'm sorry, it's true.
O is for "oh gosh, food here tastes like poo."
S is for sitting and waiting all day.
P is for Piper does not want to stay.
I is for icky, rejection that's you.
T is for all the things I'd rather do.
A is for at least my docs are the best.
L is for lungs that will soon pass the test.

All right, so now that we've got that one out of the way...

I checked into the hospital this morning for what appears to be a rather stubborn case of acute inflammatory rejection in my lungs and airways. This is, by the way, the same rejection (and the same pseudomonas infection) that my doctor caught at my bronch a few weeks ago and that we treated with high dose prednisone for a couple of weeks. I took the steroids, took some antibiotics, did a few days of IVs, and just like that I started to feel as much like new as a gal living with "gently used" organs ever can. Then this past Thursday my doc did did a routine follow-up bronch and discovered that not only is the rejection still there, but it's gotten slightly worse. Cue the dramatic music.

Although, actually, don't cue anything, because word on the hospital street is that this really isn't the end of the world. Apparently steroids are the first line of defense against rejection, but when they fail to do the trick (and sometimes they do), there are other, more potent drugs that they can use to nip the problem in the bud before it escalates into something more serious and less treatable. Drugs like, for example, the one I'm currently infusing as I type:

"Antithymocyte Globulin Rabbit."

The antithymocyte means it is a lean, mean, t-cell killing machine. The word globulin means, I believe, proteins found in the blood stream. And the word rabbit means, well, rabbit. Yeah, I know, although to be fair this is hardly my first walk down the medical/animal ethics path. Digestive enzymes, for example, are made of pig parts. Pulmozyme, no joke, is derived from Chinese hamster ovaries. Put that in your neb and smoke it.

And this drug, it appears, is made of rabbit-produced antibodies designed to attack and kill human t-cells in order to protect the organs of yet another human. This is starting to feel kinda crowded.

The protocol for this drug is "at least" five doses running for "at least" five days in the hospital. When I told my doctor that I wasn't a huge fan of the words "at least" in the medical context, he chuckled. Not a great sign. In addition, while I'm in here, I'll also be receiving IV ganciclovir (an IV antiviral), oral levaquin, and high-dose steroids in the form of IV solumedrol. All of that stuff is pretty par for the course on any CF/transplant admission (plus it gives me a plausible excuse if I'm ever feeling grumpy), so I'm pretty much fine with it.

So far, all I can really say about this admission is that's it's going to last for "at least" this upcoming week and is probably going to be somewhat unpleasant -- between the steroids and the rabbit poison (um, I mean "treatment") designed to essentially destroy my body's natural defenses, I'm not betting on a barrel of laughs. Then again, the guy in the room across the hall from me apparently missed the memo that hospital gowns actually have ties in the back to keep them closed, so maybe this will be kinda amusing after all. At any rate I am 100% optimistic that it will be effective -- and right now that's just about the only thing that really matters.

Now let's hop to it.

PS: On a personal side note, a friend from the CF community got her call for transplant tonight at this very hospital. Knowing I was here she sent her very lovely mom to come find me and tell me the wonderful news. A few hours ago I got the AWESOME update that she is, indeed, in the OR right now receiving her new life. Please keep her family and that of her donor in your thoughts, prayers, and intentions tonight as they make the leap of faith that is lung transplant. I'm so happy for her I could, um, let's just say "jump" for joy right now. Thanks in advance, beautiful people.

In Case of Emergency...

Earlier today, I posted a comment on FB about the scary obesity rates in our country (namely that obesity-related issues have recently surpassed smoking as the number one cause of preventable disease, and that Texas alone spends approximately $10 million in treatment of these disorders each year). I recently learned these facts from a combination of my post-tx exercise trainer and my current obsession with workout shows like The Biggest Loser (yeah, I know), and they startled me, so I posted.

This might seem a little odd after my rant on here about gaining weight, but I also intended my post to be helpful to the CF community. Because, let's face it, as anyone who grows up in a CF household can tell you, taking care of a child (or a sibling) with a chronic illness is TOUGH. It demands a lot of time, energy, and money. Add to that the fact that CFers typically need more calories than the average child, and you have a recipe for overweight caretakers, family members, and siblings. When I posted the statistics, I had in mind those little safety videos you see in airplanes -- you know, the ones that remind you to be sure to secure your own oxygen mask before helping your child? Hearing these scary facts made me think "oh, gosh, I really hope all these truly awesome and devoted CF family members out there are remembering to take care of their own health too!" Because trust me, gang, we need these wonderful people, both in the CF community and in the world at large.

Anyway, I posted these facts with a quick statement of what I hoped was encouragement for my friends -- CF community or not -- and asked them to please remember to stay healthy and take care of themselves. For CFers (and some other people), this might mean gaining weight. For other people (and some CFers), it might mean losing. And for all of us, it probably means eating better and going to the gym/pulmonary rehab more, not to mention staying on top of important stuff like medical testing, self-exams, sun protection, and responsible alcohol or substance use.

Hey, it's important.

There's a lot of debate out there within the CF community over personal health responsibility. Exercise is a great example, where some people swear by it and others swear it hasn't done much for them, especially in terms of numbers and lung function. I personally don't find this dichotomy all that shocking: of course exercise is going to have different effects on different people, just like having the disease we collectively call "cystic fibrosis" means different symptoms and severity for different individuals. Of course we all know that exercise, as a general rule, is good for you. But it all gets a bit murkier when it comes to forcing yourself out of bed with yet ANOTHER fever and 25% lung function to go walk on a treadmill and cough up goo until you vomit. That's a tough pill to swallow sometimes, even for a CFer. On the other hand, a group of wonderful CF women I correspond with regularly on these types of issues recently pointed out to me that part of feeling mentally healthy as a CFer is the knowledge that you're doing all you can to prevent your disease from dragging you down. And that, according to most doctors and patients alike, includes stuff like nutrition and exercise. As well it probably should.

I don't have any easy answers on this one. I'd like to offer my personal perspective as someone who's lived through end-stage CF, which is that doing little things that offered me some "control" (even theoretical control) was a HUGE comfort to me right before my transplant. Making space for exercise in my own life was important because it left me feeling empowered and less helpless -- and I'll take empowered with low lung function over helpless and STILL with low lung function any day of the week.  But again, that was only my personal experience.

I think the point is here that we need to be asking ourselves what we can be doing to make space for health in our own lives, whether we live each day with CF directly or not. We need to be securing our own oxygen masks, and then maybe we'll have the energy to look around and help others. Maybe it means getting to the gym more, or maybe it means finding another activity in your life so that it's NOT all about CF. Maybe it's as simple as coffee with friends or some time alone with a great book or finally taking control of that debt that's been harassing you or being part of your own cure by fundraising. At any rate, it's empowering. It's an anti-victim, no excuses stance. Most of all, it's about doing for yourself what you would like to someday do for others. 

And that makes it a lifesaver.