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.

Liftoff

When I was about 12 my mother, who at the time was the dean of a graduate school in Colorado, had a faculty member who also happened to be an astronaut in his spare time. (I guess you could call it moonlighting....ba, dum, bah!) Anyway, bad puns aside, I thought this was pretty cool because 1) I had never met an actual astronaut before and was forever intrigued by the idea of freeze-dried ice cream, and 2) her association with this particular astronaut meant that my family was invited down to Florida to watch the shuttle launch when he was sent up to the International Space Station. A trip to Florida to watch the shuttle launch, by the way, gets you waaaay more street cred in elementary school than your run-of-the-mill visit to Mickey. This was the big time, and in my head it was going to be replete with visits to the "cockpit" (do shuttles have cockpits?) and time spent in one of those zero gravity spinning things you used to see on ads for Space Camp. I was, to put it mildly, super stoked at the prospect.

The launch was a night launch, which is both rare and spectacularly beautiful. We huddled on bleachers in the middle of some very uninhabited strip of land/dust, with the lights of the launching station spotlighting the shuttle in the distance. We weren't close by any stretch -- at least a mile away, I would guess, if not more -- and we had to get there early, so for the first hour or so all I really remember was a sense of being uncomfortable and bored. I was excited, sure, but I was also a pre-teen sitting with my parents in the Florida wilderness before the age of texting and cell phones and with nary a spinning zero-gravity ride in sight. I was, truth be told, kind of over it.

Over it, that is, until the countdown clock hit about 5 minutes to liftoff. At that point I swear I felt a tingle go through my entire body. I sat up straighter, I looked off into the distance at the lights surrounding the shuttle. I imagined the astronauts -- a crew of 5, including two women I had seen the day before at a panel -- strapping themselves into their chairs and straining their eyes for a last look at the family and loved ones seated with us in the distance. I even considered the name of the shuttle, The Atlantis, and its implications of worlds lost and the constant quest for hidden treasures. I stayed lost in these thoughts for about 4 minutes and 30 seconds, and then I joined the crowd in counting down toward the release of that vessel into the universe, full of the hope, joy, fear, light, and sheer anticipation of our collective human desire to live and to learn and to discover new wonders.

It is now T minus 11 hours before I, having finally finished my last round of inhaled and oral treatment for RSV, will be released back into my own little bustling universe of New York City. And though I can't claim a goal quite as lofty as those of the astronauts I watched so long ago, I have to admit that my skin is once again tingling with anticipation at the simple thought of making my way through the night and the snow that falls like so many stars to my family, my friends, my puppy, and my life.

I am so beyond grateful.

The nurses, staff, and doctors here are too amazing for words. I felt so cared for and looked after, which (while I may occasionally complain about all the interruptions) is something I am unbelievably thankful for. The care I get at my hospital is aggressive, compassionate, and even occasionally mixed in with a healthy dose of good fun and humor. I truly hope never to take that for granted.

That said, I am ready for liftoff. The past 8 days in here have felt somewhat dreamlike both in their intensity and in their timeless, floaty quality. The drugs they've had me on in here mess with my senses, to the extent that I can't even really feel my own body (case in point: this morning I bit my tongue due to numbness, and it's hard to type because my fingers can't feel the keys). For the past 5 days I've also been behind double doors, removed from the normal sounds off the hospital and unable to see my visitors, who must enter my room looking frankly ridiculous in paper gowns, orange masks (sometimes with plastic eye visors on them), and latex gloves. When I checked in here I was texting back and forth with a friend who I will never text with again, and that is the most unreal part of all. But somehow or another, like floating through space, time still managed to pass, and now I'm just a short while away from my own night launch. And happy as I am that I have such a wonderful hospital and dedicated team, that moment honestly can't come soon enough. Once again I am over the boring part and ready to skip ahead to the action, please.

So I'm officially commencing the countdown -- and I hope to see you all out there among the stars.

Really Silly Verses

There once was a room by the highway
With views of the New Jersey skyway.
It came with double doors
And was up seven floors
In a bustling hospital hallway.

And those two doors were there for a reason
Having to do with the season.
'Cause in winter, you see
One might catch RSV
And end up with coughing and wheezin'.

Which fact is the point of this story
Without getting overly gory,
There are bugs in my nose
But that's just how life goes,
And I don't want my breathheads to worry.

I'm not happy, it goes without saying,
About how these cards have been laying.
I try to understand
This is part of the plan
Of this wonderful game we're all playing.

There are drugs, there are fears, there are times when
It feels like you simply cannot win.
But for now there are lights
From New York City nights
And tomorrow we're blessed to try again.

Balance It Out

Well, hello there, beautiful breathheads:

I'm writing this afternoon from my room on the 7th floor transplant ward of my hospital. My view is pretty darn cool, if I do say so myself, as my window looks right out over the Hudson River, The Empire State Building, and all those beautiful lights that make New York City's skyline so breathtaking. Oh wait, I'm sorry, where are my manners? Would you like to see it, too?


Amazing, right?

Unfortunately, though, my other view is slightly less...appetizing? I can, for example, see my body, which is currently sporting not one, not two, but...wait for it...three separate site for IV access: two peripherals and my port. I can also see the number of IV bags hanging from my pole, and I can honestly say that never have I seen so many in one place feeding into my body at the same time other than my time in the ICU immediately post-transplant. I can see the many doctors who keep walking through my door, and I can see the clock on my wall counting down the minutes until my next dose of cephapine, to which I am allergic. Oh, yeah, and I can see this:



I don't care what anyone says, peas and carrots are not supposed to be neon.

Aside from the food and the lock-up situation, though, things are going pretty smoothly over here. I was admitted directly from my bronch this past Tuesday after my doctor found continued secretions in my gorgeous new lungs. He's the "better safe than sorry" type (good find in a doctor, by the way), so he decided to pull me in here for a good old-fashioned dose of in-house medicine. This was actually tossed around as an option last week and I had time to prepare. I'm not pleased about it, to say the least, but I am accepting of it, which is a big first step. And after all, I want to do right by these beautiful breathers -- they (and I) deserve nothing less. The plan right now is to do a course of one very strong IV antibiotic called polymyxin B; do a normal course of IV levaquin, mycofungin, and oral minocycline; desensitize me to another option for use now and in the future as needed (heeeello cephalosporins! Oh, how I missed you and your thrilling scent of cat pee!); and check out if there's any rejection since I've had a couple of episodes since my surgery. Oh, and we're gonna throw in some sinus exams/consults and vascular treatment, just for laughs. We're funny like that, I guess.

So I'm in here, and I'm looking out at my twinkling city and I am, of course, super bored because that's how things roll at the hospital, and I find myself sort of leaning into the view -- as if my body just wants to leap out the window and fly down through that mass of steel and water and sky and humanity. Because it does. I suddenly realize that I am aching to get out of here. I am literally tingling with the thought of being allowed something as simple as the chance to walk down my silly little street in the West Village again. I am, in a word, desperate. It's not an uncommon feeling for me because I absolutely hate being "sick" and will do anything to avoid places that make me feel that way, but in this case I'm on a drug that they just won't let me start at home. I feel fine, all things considered, but I'm not allowed to leave.

Polymyxin as a drug is well-known for its evil side effect profile, one of the big ones being that it is nuero-toxic. Patients can experience this effect in different and diverse ways, but for me it's always been a tingly, numb-like feeling in my face and hands and a more or less destroyed sense of balance. And as those who know or have ever met me in person can attest, I am not particularly graceful to begin with, so any loss in this department is a huge step down in my ability to perform such complicated feats as walking or, well, writing this blog. And right now, that stuff is hard. It's really, really hard, as a matter of fact.

When I lose my sense of balance, I feel unsteady, awkward, and unproductive. I feel confined right now, quite literally insofar as I am in a specific ward on a specific floor of a specific hospital and can't leave -- I can't even take a long walk for fear of falling into some poor kidney recipient or whatnot. I can't shake these infections either, even post-transplant, and that makes me feel even more off-kilter. And so I find myself here, leaning into my window, staring out at the city and the life I adore from behind the glass of the life that keeps me going, but threatens to drive me crazy in the process. It's a familiar sensation to me, as I remember doing the exact same thing from Denver Children's Hospital when I was 15. I can't seem to check into this place, either mentally or physically, without feeling a sense of loss and desire. I just want to be done with this part of the production and on to whatever comes next.

But I can't be totally free, obviously, and I can't wish away CF or transplant or any of their nasty little siblings. I can't change what's already happened and I can't (or rather, I don't want to) sit around and worry and squander the moments when I could be out showing these lungs a good time. I can't bear the thought of cheating myself out of any more time. Period.

And so I have to choose balance, by necessity. For me it means knowing when to push my doctors for more in the life/freedom department and when to back off and trust their judgment in the health/lung part of the deal. Truth be told, I want both a long and a happy life, and I'm not willing to compromise much on either. So I have to learn how to walk this tightrope, even if it does sometimes land me with my nose against a frosty windowsill. The alternative on either side to me would mean giving in to my disease: either by being stubborn and allowing it to take my health and good judgment from me or by being too scared and allowing it to take my spirit. I can't (and I won't) let either of those things happen if I have any say about it.

I haven't yet found the perfect combination, and I seriously doubt I ever will get it exactly right. After all, I am alive, and that makes me prone to mistakes. But tonight, in my cozy, lonely, familiar hospital room looking out over my huge, crowded, exciting city I know that I am the consummate balancing act between these two worlds. And it doesn't feel bad at all.

Well, aside from the food, of course.

xoxo,
Piper

Wearing My Heart on My Sleeve (Or on My Blog, Anyway)

First things first: I didn't get admitted to the hospital.

Okay, now that we've gotten that out of the way, I think I left a few details out of the last post, which is probably to be expected from an attempt to post a blog immediately after returning from the ER. So, backing up a bit, let me explain why I was in the ER in the first place. I think I mentioned that I had a resting HR of 225 bpm for over an hour before I headed in, which probably sounds completely insane, but for me is, well, only mildly nuts. I also know that I mentioned SVT, which stands for supraventricular tachycardia. In my case, this is caused by something called an "accessory pathway" in my heart. When triggered, this accessory pathway kicks in shortens my heartbeat circuit. So think of it this way: an electrical impulse travels around your heart by a predetermined pathway in order to cause a heartbeat. I have an extra pathway that cuts off some of this normal circuit, and when that pathway is switched on it causes my HR to speed way up, because obviously it takes less time for the electrical impulse to travel the shorter circuit. It's kind of like a heartrate shortcut, I guess. And when this happens, my HR jumps from its normal rate of about 100 all the way up to 225 in literally and instant. And when it stops, it drops back down just as quickly.

The good news is, SVT isn't usually a major issue. It can cause problems, and some cases can even be life-threatening, but I've been lucky enough to always be able to break the attack and switch "off" the accessory pathway by myself, at home, without medical intervention. It normally takes 10-20 minutes of lying down and performing things called vagal maneuvers. These are a series of progressively more and more embarrassing tricks to try and slow my heartrate by stimulating the vagus nerve. And the really cool part is that it's best to have someone with you as you perform these circus acts (examples of which include bearing down like you're trying to make a bowel movement, forced gagging, and submerging your head into ice water), because if you pass out at any point or feel dizzy it's probably time to head into the ER. Fun stuff.

I didn't pass out, but I wasn't able to stop it either. So after an hour and 15 minutes of groaning on my mom's bed with ice packs over my eyes, I finally gave up the ghost and made my way toward a downtown hospital (read: not my CF center), with strict instructions to call or page my CF doc with any developments.

Upon arriving at said nameless downtown ER, I checked in at registration and headed to triage, where I used my pulseox to try and convince the triage nurse that my HR wasn't playing around. No go. She told me I would have to wait "at least an hour" but that in the meantime she had orders to give me a quick EKG. I counted that as a partial victory and headed back with yet another nurse, who, upon running the EKG immediately paged several doctors and led me straight back through the waiting room and into the patient area waving my EKG and tapping any doctor we passed on the shoulder to whisper "SVT!" in a hurried voice. I may or may not have smiled smugly as I was led past the triage nurse who demanded I wait, although I'm the first to admit that it's hard to look smug when your heart is beating faster than a marathon runner's. I like to think I pulled it off though, because I'm just an overachiever like that.

Once back in the patient area I was treated to a bevy of concerned professionals assisting me with everything from changing into my hospital gown to covering me with those annoying heart monitor things, all while asking questions like "why are you on oxygen?", "are you short of breath?", and my personal favorite "oh wow, is that cough normal for you?" To be fair, they didn't have a chart on me since they were working extremely quickly, and I was only able to explain about the whole CF/transplant thing in between other questions about the real issue: my heart. And when they asked about drug allergies and I gave them my list the nurse responded "well good, those are all antibiotics and we won't need any of those." Definitely an odd statement to the ears of a CFer.

I have to hand it to the staff of the unnamed downtown ER, because they got the SVT stopped incredibly quickly by an advanced technique known as . . . taking my blood pressure? Um, yeah, you read that correctly. They put the BP cuff on to take vitals before beginning treatment, which was going to be a quick dose of heart meds via a peripheral IV that had already been placed, and low and behold the squeezing acted as some sort of off switch and suddenly my HR bounced right back down to 110 bpm. And let me tell you, there is no better feeling than that moment. Imagine having your PICC pulled after 6 months, and then double the relief. No joke.

So, moral of the story: SVT sucks, but not as badly as CF, because after all that they decided that due to my cough they might like to keep me in house. And obviously this made no sense to me, seeing as 1) I wasn't at my CF center, 2) I had the cough pre-SVT, and 3) I had already spoken to my CF doc earlier that day and knew I was scheduled for clinic and to start IV abx in the morning. I told them as much, and luckily they agreed with the plan of attack, though I was told to "make sure [my CF doc] gets everything checked out" (as if that were ever in question anyway). And then I left, about 2 1/2 hours after arriving, making it one of the shortest ER visits of my life and, thanks to a drunk man celebrating his birthday, also one of the more entertaining.

I'm not going to drag you through all the CF stuff that went down the next day at clinic, but I will say this: I ended up on IV merrem and levaquin, with no residual symptoms from the previous nights' episode aside from a general feeling of exhaustion, and -- the most important detail -- back at home. Which kind of brings this post full circle.

So there's the back story that explains the madness. I know I should count myself lucky that my only ER visit since October has been for an unrelated and fairly benign heart issue, and I definitely do. But at the same time I'd be lying if I tried to say that this latest experience didn't shake me up a bit, because as long as I've known about my SVT I've always been able to control it myself. I would slip into it suddenly (ironically coughing and PFTs are major triggers for me, since both involve sudden, forceful movements), and then know that aside from the humiliation of "bearing down" in front of whichever poor soul happened to be closest to me at the time, I was going to emerge unscathed and not much worse for the wear within a half hour or so. And I know, of course, that one episode of uncontrolled SVT that was eventually stopped without meds anyway isn't a big deal. I know that, and I'm treating it as such. It's just that on top of everything else, I really wish I didn't have to worry about the now very real possibility that a racing heart might send me to the ER with no warning.

So you know, there's that.

But the good part about all of this is that I'm here with my Sampsonbear, currently SVT-free (a great way to be), and hoping that these IVs kick in soon and help me feel better so that I can get back to the business of more fun activities, like my mother's upcoming birthday. And in the meantime, trust me, I'm not looking for any more shortcuts.

Just Another Manic . . . Tuesday?

Seriously.

So today started off easy enough. Got up, did treatments, began negotiations with my doctor about starting IVs tmw, and headed out into the NYC Summer heat (yep, it's finally here) with my parents to look at some property. Nothing too surprising -- even the IVs seemed semi workable, and I set up a clinic appt for tmw to help decide the age-old inpatient vs. home question. Add in a French cafe for lunch and thugs were looking, if not perfect, then at least within the realm of normal.

And then along comes SVT.

Yep, that's right -- this post isn't entirely about CF. SVT is another set of letters that makes my life complicated, and today it was on in full force. For over an hour my HR was around 225 bpm, before I ended up at the nearest NYC ER. Luckily the issue was resolved pretty quickly, and I was simply made to wait for a couple hours to make sure everything was stable. I was also given strict instructions to get fully checked out tmw at clinic, which was obviously never in question.

The best part about this experience? Well, it might have been the drunk man who told the cop who brought him in that she had "a thick ass" and was "a sneaky doughnut eater." To her credit, she won best retort of the evening with the line "sir, that is One thing I definitely do NOT have, is a thick ass!" turns out she had recently qualified by time alone for the Florida marathon. Nice.

Or it might have been that my dad was the victim of street fraud, when a man on the sidewalk literally threw fried rice on him and then demanded $12 because dad had "ruined his dinner."

But I'd say the winner hands down is that, for now, I'm home with the sammybear, camped on my couch and savoring a few hours of peace before clinic tmw and almost certain admission. True, between the SVT and the CF I'm more than a little fed up with the abbreviations in my life right now, but for now I choose to focus on that one, universal truth:

There is, truly, no place like home.


- Posted using BlogPress from my iPhone

Another Day

For those of you who are wondering, it's just another day here in New York City.

Just another day that started out here . . .





. . . and finished up here.




It's another day filled with IVs and nebulizers and oxygen and chest PT and enzymes and antibiotics and dermatology students (not kidding) and discharges that actually happened ON TIME (nope, still not kidding) and doctors wearing "droplet precaution isolation" masks in my room even though I'm officially negative for swine flu just because I still had the sign on my door because I'd really prefer everyone mask/glove/gown up anyway.

Another day of downtown taxi rides and puppy reunions and homecooked (non-hospital) meals and comfy (non-hospital) beds and views from my (non-hospital) window and TV watched from my comfy (non-hospital) couch and maybe even some mild (non-hospital) meditation.

It's another day of gorgeous fall weather and chilly breeze and possible new developments on the transplant front but who wants to think about that kind of stuff anyway because today is just another blessed, wonderful, fantastic, flu-free day that is all about going HOME!

And home is where I plan to stay.

Keep on Keepin' on Like a Bird That Flew . . .

Random Sidebar/Fact of the Day: LOVE Bob Dylan. Love the above-mentioned lyrics. Love listening to my ipod while gazing out the window at the Hudson river and the George Washington Bridge. Love having nothing really better to do than sit around on 5 liters of O2 because I can't leave my room due to hardcore isolation protocol . . .

Whoops, took that one a little too far.

Okay, so the past couple of days have been, um, "eventful", and not so much in a good way. After my super fun day at the races (literally) on Sunday, I was all set to get the H1N1 vaccine and then start IVs on Monday for a quick tune-up. My lungs sounded better than they do with hardcore infections, and my PFTs were still stable, but my increased cough, lung goo (sorry, it had to be mentioned), and the recent growths in my cultures convinced us that we should act early rather than wait. So I started the IVs on Monday evening and settled in for what everyone expected would be a fairly easy and uncomplicated round of drugs.

Then again, if CF has taught me anything, it's to expect the unexpected.

Monday night I ran some medium-grade fevers (around 101) and was a little achey, so I called my doc and we all chalked it up to most likely a vaccine/IV drug combo, especially since it's not uncommon for me to run some fevers at the start of IVs. This theory seemed to be confirmed by a lack of fevers all day Tuesday, although I was tired and a little achey. Tuesday night, though, my fever spiked up to 103.5 and simply wouldn't BUDGE for about 4 hours. Call the doc again, start TamiFlu immediately, check in again in the morning. Wednesday morning, O2 is down, but fever is also only around 100.5. Still no sign of a sore throat, additional cough, or headache -- just achey and feverish. By Wednesday afternoon, when my fever went back up to 103.5, however, I knew I was destined for an all-expenses paid vacay at my favorite "Club Med" -- and sure enough here I am.

Surprisingly though, I haven't run any real fevers since coming here. They brought down my initial fever, were able to hydrate me thoroughly through the IV, and since then I've felt SO much better. My O2 levels are still low, but that's why God invented nasal canulas, right? And they're holding steadily above 95 on the increased dose, which means the old fighters are still working, if a little compromised right now. I have faith that those numbers will bounce back -- they always seem to for me, thankfully.

Now for the REALLY good (if only preliminary) news: early results show that this is NOT swine flu! We're still waiting on the more advanced, more accurate test, but if that comes back negative tonight and I have no fevers through the night or tomorrow morning, I should be free to leave and go home to Mr. Sampson by tomorrow afternoon. Totally visualizing that negative flu result now!

They do think these fevers might have been drug-related after all. My amazing team here includes my wonderful CF doc, as well as two fabulous doctors from infectious disease and vascular surgery, so I'm pretty confident that whether this is drug, virus, or clot related they'll figure it out eventually. At this point, though, I'm just happy to be seemingly on the mend.

I don't mind sharing that this whole flu thing had me pretty freaked out. I'm so sick (no pun intended) of seeing people with CF struggle with this virus and other complications. I hate knowing that life is so fragile that it can hang in the balance of a cough not covered, or a hand not washed, or any other of a number of factors. I also know that this is true for people regardless of whether or not they have CF -- I guess it's all part of what makes life precious at the same time. Still, it's never easy watching your friends get sicker, whatever the cause. I feel blessed to have so many wonderful cystics in my life -- the friendships are really amazing and I wouldn't trade them for anything -- but it still hurts to have to say it: breathe easy, Lauren. You'll be missed.

I have a lot more news to share on the transplant front, and hopefully will be able to update soon when things are a little more certain. And for now I'm just going to keep on keepin' on, and hopefully be home in time for dinner tomorrow.

Third Time's the Charm

Good news: the catheter is OUT!

Yep, afer 2 1/2 months of weird fevers, blood cultures every few days, over 6 CT scans of various parts of my body, countless x-rays, 4 hospital stays, and continuous use of one IV or another (aside from a brief 2 week break in there), the probable culprit is out of my body for good! I cannot even tell you how excited I am.

Of course, in true Piper style, I didn't make it easy on anyone. They were scared to put me under general, so the whole thing was under conscious sedation (apparently I told the nurses all about my sister's awesome fashion line -- how great of a little sister am I?). They cut open my arm in multiple places, clamped off a vein, removed the scar tissue, and then ended up having to literally "lasso" the catheter as it started slipping away into my heart. Wow. They thought the surgery would take about 20 mins to an hour -- it took over 2! But I have an awesome team, they got the job done, they did it safely, and I'm scheduled to finally go home tomorrow. Hopefully minus one very pesky blood infection.

I'll still be on lung IVs for a while, but hopefully not too long. This is week 3 and I normally do 3-4, so here's hoping I get to call it all quits after that. We're going to leave in my PICC for now, in part because it seems silly to get a new port when they'll have to remove it during transplant anyway. The other reason is that it took them 3 hours to place this PICC in interventional radiology (no joke) because my veins simply "do not do PICCs" anymore, as the radiologist said to me afterward. So it might be wise to leave this one in just in case I end up back on IVs in the next month or so. The other option is a new port, and while I'm not opposed, I am a little gun-shy right now. I think it's justified.

By the way, this is totally not to say CFers should avoid ports. Totally not. I LOVED mine. And it was great, for 9 whole years. It saved me countless PICCs (off the top of my head I can say that I've had at least 25 rounds of IVs in that 9 years, which is ridiculous -- most of them in the last 3), and a lot of pain. What happened to me with the stuck catheter was totally weird and very rare, so please don't take it as anti-port propaganda.

Okay, so other news.

1) I am now "officially" on O2 24/7. I say "officially" because I am actually still stating a little above 90 most of the time, but I desat with minimal activity (like walking around the apt), so my doctor placed me on 2 liters with instructions to use my judgment and be careful. It's a new development to have the script for the 24/7 O2, but it doesn't change much, since I was being pretty careful to use it with any real exertion anyway.

2) I have a Frequencer! This is a new airway clearance device that I asked my doc to look into and she, being amazing, got me a demo and then a free trial. I love it! It works like chest CPT only using sound waves that go through your body. You don't feel squeezing or beating, but then when you speak your voice vibrates just like the vest! You hold the little speaker over each part of the lung for 2 mins, just like CPT (front only -- it travels through muscle and gets both sides of the lobe), and it works wonders. I cough up tons every time I use it. Highly recommended to try out, especially for people who want a basically silent, tiny, and super effective alternative to the vest. I promise pics and a more in-depth post later.

3) Just an interesting tidbit: I just found out that my center is one of just a couple in the country currently experimenting with transplanting CFers directly off a type of artificial lung called ECMO -- basically a machine that takes blood out of your body, oxygenates it, and returns it. The idea is that the vent is problematic for CFers because you can't cough. So my center is experimenting with taking CFers who go into respiratory distress and weaning them off the vent and onto ECMO, and then actually taking them out from under sedation so that even while hooked up to ECMO they can cough and do some airway clearance. The idea is that the CFer will then be in better shape going into transplant and hopefully have better outcomes than off the vent transplant (though many of those turn out fine, it has to be said). I don't entirely get it, but it was offered to me as an option should things get that far (knock on wood). ECMO is sometimes used after transplant to help lungs that for some reason aren't functioning well in the first couple of days, but this is apparently a much more novel and experimental use. There has already been at least one success story here using the method. I haven't decided yet if I would go for this option or not, but I'm discussing it with my family now just in case. I just think it's crazy incredible what new things are developing in the world of CF lung transplants!

That's it. Thanks everyone for your thoughts and prayers -- they definitely came in handy to make this try a success!

Surgery Fun Tomorrow

Well, I've been home for exactly a week today, and I officially go back into the hospital tomorrow. Not for a tune up though (still on at-home daptomyacin for the blood infection and merrem for the lungs). This time it's for surgery.

Yep, fingers crossed, we're finally going to get rid of the rest of that blasted port.

Basically, I've been running low to mid-grade fevers since I got home, and while we're pretty sure part of that was from the lungs (which had to be off all anti-pseudomonals for a few days due to bad kidney functions after the polymyxin), we still think part of it is from that lingering port/blood issue. But having gone in twice prior and failed to remove the catheter -- once in his office with lidocaine and once in the OR with lido and "twilight" sedation -- because the darn thing is fused to my vein in the armpit/shoulder area, the surgeon is going to take a more aggressive approach this time.

The plan is to go in through my armpit (sidenote: not sure if this means through the actual "pit" itself, or if it really means a bit higher -- better shave just in case!) and clamp off the offending vein on either side of the scar tissue. Then he'll be able to cut in there with a tiny incision, dislodge the catheter, and then hopefully pull it out no problem (the port itself is already gone) and sew up the vein.

Yeah, you read right: sew up the vein. Vascular surgery is amazing.

Anyway, because I'm thin enough for the surgery to be rather close to the surface (thanks, CF!) and because I have crappy lungs (um, thanks again, CF?), we're going to do this whole thing under "twilight" sedation once again. So I won't be asleep, just very, very loopy. And normally that means that I would be able to just go home when it was all over, but my CF doc wants me in the hospital for one night just in case. I'm not making any protests there.

One night to (hopefully) set this whole thing in order and get rid of the mystery fevers of 2 months and counting once and for all sounds pretty damn good to me, thanks very much.

Not particularly excited about the whole process, obviously, but I feel blessed to have such a competent team, and I'm super happy about not having to risk the breathing tube at this point in my CF life. Avoiding the vent is key right now and I've been promised that if my O2 drops below expected levels they'll do a type of blowback O2 (where they literally blow it into your mouth with a machine) and try basically everything else before they do a tube. But I've had conscious sedation plenty of times and never needed more than my nasal cannula, so I'm just going to stay focused on that. It also apparently says all over my surgical instructions "no general!" and "no intubation!" -- or so the nurse told me on the phone.

Hopefully I'll be able to update tomorrow from Columbia and let you all know that things went smoothly and that the little bugger is finally OUT! Everyone seems confidant, and I trust this surgeon a lot, so I'm anticipating a good outcome for sure.

Thoughts, prayers, intentions, and good vibes are always welcome in the meantime, though!

Like Riding a Bike

Well, I'm home! Back to my apartment, my much-missed windows (currently coated with a not-so-much-missed rain, but what can I say -- even drizzle looks gorgeous against the New York City skyline), my favorite junkie snack foods, my bed, and, of course, my adorable Sampson. Best of all, I've been home for three days already and, while we did have one small scare due to a nasty fevers and nausea combo pack that sent us back up to Columbia Pres for a pow-wow with my amazing team and a possible admission, I dodged that bullet with only some blood draws and a promise to spend a lot of time putting hot compresses on my much-abused arms. So that means we're now facing my first weekend home from the hospital since . . . well, since it was actually summer.

And yes, we're all quite happy with the situation, thanks for asking.

My dad is here now because he's been making a very admirable effort to be out here as many weekends as possible, given that now both his daughters AND his wife more or less reside here full time. It's so awesome to have the whole family around, to be honest. When I'm sick, it just makes me feel safe and cozy to be surrounded by all these people taking care of me. And when I feel better, we get to enjoy the city as a family, walk the dog, or Erin and I go out and live our normal lives and leave my parents to get a little, um, alone time. After all, that does seem only fair when your spouse of over 35 years lives in another city halfway across the country.

So anyway, my dad's out, and he's actually brought with him a little toy: one of his old mountain bikes. Now, first let me explain that growing up in Colorado there were a couple of things my sister and I were doing from about age 2, and the top slots on this list were skiing and bike riding. It's just a Colorado rite of passage, I guess, kinda like turning 16 and immediately learning how to control your car in a white-out blizzard, or going to your first Broncos/Rockies/Nuggets game and learning what it means to unconditionally love something that will continue to break your heart. Repeatedly. It's just something you learn to live with.

So I wasn't really surprised when my dad turned up at the apartment with a lightweight, dirt-encrusted, spike-wheeled mountain bike to be the partner of the heavy-set, stylish city cruiser bike (with a basket for Sammy of course!) that my mother and I had purchased a couple of weeks before. Nope, I had expected that the day would come when my family would transplant at least a little of our Colorado-ness to the urban mountains of New York City, and a mountain bike seemed the perfect way to meet that expectation.

I just didn't expect to (nearly) fall off.

In my defense, I was riding the city cruiser, on a Manhattan sidewalk, WITH the (very nervous) dog in basket. I was definitely multi-tasking, simultaneously playing the roles of the apologetic rude pedestrian misusing the sidewalk, the overly protective dog mama comforting her rather stunned little guy, and enthusiastic Coloradoan anxious to feel the wind in her hair after a good four to five years without any reason or excuse to ride a bike at all. A strange combo, sure, and one that I can assure you nearly killed me, my puppy, and several unsuspecting New Yorkers out for a stroll, who were, perhaps, accustomed to dodging tourists in Times Square but were far less prepared to dodge wobbly bicycles on Canal St.

It was a beautiful, slightly horrifying moment. The good news being that I, my puppy, and my bike all made it home in tact and surprisingly un-traumatized.

All that was, of course, several weeks ago (back when it was actually summer, and pre-hospitalpalooza 2009), but it's been on my mind for the past couple of days, and for one very legitimate reason: for the first time in 9 years, I have a PICC line. And I have to re-learn how to ride it, so to speak.

To be honest, I thought it would be really easy, kinda like the mistake with bike. I remember PICCs as being relatively unobtrusive little buggers, something I chose to do away with more for the convenience of the port than because of any inherent flaw in the original product. And then my port finally went kaput after 9 years (and by "kaput" I mean "got infected and had to have two attempts at surgical removal, both of which were unsuccessful given that the thing has attached itself to my vein in my upper arm and flat-out refuses to leave") and I was left with no option but peripherals and PICCs. Needless to say, after the 14th infiltrated peripheral (leading to two infections), I was more than ready to give the PICC the old college re-try.

And so far it's been great. Really. I mean, forget about the fact that the supposedly 15-30 minute procedure with IR to get the thing in ended up taking three hours because my veins are particularly small and unwilling (they had a lot of these things in the pre-port years), because after that I've had no problems. And for that I count myself REALLY lucky, because apparently in the past 9 years I've become something of a PICC idiot.

Yesterday at clinic I was scolded for not wearing a proper "sleeve" over the PICC, although I had managed to secure it so that it wouldn't be pulled out, far more than I ever would have done with my port tubing. Nope, apparently that's not enough, I was told, and vague memories of some sort of mesh, tube-sock-like material came rushing back to me. Oh yeah. Next I was asked when the dressing was changed post-insertion, and I told my doctor that I was under the impression I had a week (standard dressing change time for a port access). Wrong again. The PICC, I'm told, requires a dressing change within 48 hours of insertion, after which one can resume a weekly schedule. Huh. Well, live and learn.

There's a few other weird features of this PICC, if you ask me. It definitely looks different from the old PICCs I remember as a teenager. It has no clamp, and I'm not sure if that's a new feature or just something I got used to with the port tubing, but it sure feels weird to me. Even the dressing used is different, with a sort of slit in edge of the tegaderm to allow the PICC to pass through. I don't know if any of this is standard, but I know it's all pretty new (or new again) to me.

I guess the next few weeks will tell if having a PICC is truly like riding a bike, but so far I have to admit that I see the similarities. You can grow up with it, hold onto it as long and as hard as you can, but there will probably come a time when you decide to move onto something else for a while, be it out of necessity or just plain choice. And then someday you might find yourself suddenly faced with the prospect of trying again, despite the fact that it's been years, and everyone (your CF doctors or the pedestrians on Canal St) is going to expect you to be able to handle the reunion, for your own sake and for theirs.

And you will handle it all, eventually. Because a few wobbles and even a sudden gasp or two are just fine, so long as everyone gets home safe.

All About Me

So things are moving right along towards transplant over here in Columbia-land. My score has increased quite a bit lately because of increased O2 needs, lower PFTs, poor weight gain, and a decrease in my 6 minute walk score. They actually think they may have lungs for me here in the next couple of months -- some people were even talking as if I might get transplanted during this hospital stay. Yowza. I'm definitely on board, and I have a huge amount of faith all of the sudden that this is right, things are falling into place to give me a second chance at life for which I will be forever grateful, and that God and the Universe and Columbia Presbyterian will bring me these lungs at the exact right moment for me. I'm definitely ready to breathe and be back to my old, energetic, happy, excited, optimistic, hyper, "normal" (haha) self. I'm just so grateful to even have the chance at that sort of opportunity.

In the meantime though, I'd like to go home. I mean, not to sound snippy or anything, but this whole hospital and failed surgery thing is getting old. Luckily my sats and energy levels are up, and my fever is down, so my doctor is on board with letting me go as soon as they place the PICC line. Unfortunately (there is always an "unfortunately" in the hospital -- have you noticed that?), the PICC and IR teams are out until Tuesday, so that's the earliest I'm gonna get it placed it looks like. Long weekends are so much fun for the outside world, but in the hospital it tends to just mean 3 days of not seeing your doctor and not being able to move forward, which is frustrating. My poor arm has blown through 8 peripheral IVs in three days and is swollen up like a basketball. Hopefully tonight they'll at least be able to get permission to use my left arm, which so far has just been recovering from surgery and resting. I think it's about time it started pulling its weight around here, although it points out that for the past 9 years it's handled all the IV work. What a complainer!

Not much other news. My blood infection is clearing up and I've had a couple of negative cultures. They're still worried that it will come back as soon as they stop the dapomyacin, but if that's the case then I can either: 1) have vascular surgery in my shoulder under "twilight" sedation to remove the rest of the catheter, or 2) go on oral abx to control the blood infection until I get the transplant, at which point the catheter will be removed anyway. I think we're probably looking at option 2, but I'm pretty fine with either at this point. It kind of creeps me out to have that catheter still in me without the port, but whatever. I guess it's not as though I have to see it, right? And it just adds to my awesome "sickgirl" powers, I'm sure.

Speaking of power, I'm really really trying to keep my strength up and regain what I lost. When this blood infection hit, it made me more or less catatonic for a couple of days, and when you're already on prednisone that will wipe out muscle mass pretty quick. I felt like I was dreaming when my doctor suggested that I spend "a little time every day" sitting up in a chair to keep up my strength. Sitting?! I asked if I could walk the hall and she seemed a little concerned but of course said yes, and even offered me a walker! Well, I'm proud to report that after several days of walking laps up and down the ward, today I used my pass and walked OUTSIDE, down the block, around the corner, and down through another building to get to the hospital courtyard. Granted, once there I promptly fell asleep and proceeded to nap sitting up for the next half hour or so, but then I got up and walked back, with a short detour to the vending machines. No, it wasn't the workout of my dreams, but it was something, and I got fresh air, and to be honest it was all I had in me for now. I've also been spending time sitting int he lounge, and I plan to walk some more laps this afternoon. It's not great, but it should hold me until I can get back to my treadmill on Tuesday!

The one great thing about the hospital on weekends is fewer people come into your room and but you for random reasons. I've had my CPT for the day, done most of my IVs, and seen allt eh residents covering for my doctors, so for the first time since I got here I think I can actually shut my door, get out my book, and be as antisocial as I please.

Which is exactly what I plan to do.

To My Darling Sampson

Hey Sammybear, it's your mama writing. I'm pretty sure you haven't learned how to read or use a computer in my absence (although if you have, MAJOR props go to grandma for her puppy-training skills), but I still just couldn't resist the chance to send you a message.

Now before you even say it, I know I'm in the doghouse (yes, pun intended) for my recent extended absence. I know that being gone for 5 days and then coming home for one ridiculous night before leaving AGAIN for another week is hardly good puppy-mom etiquette. And while I know you're enjoying having both your grandma and grandad in town for a while plus extended visits with Aunt Erin, I also realize that it just plain sucks when your best friend and playmate goes totally AWOL. You're such a sweet puppy, Sam (random rocket-science experiments aside), and I'm so sorry that I keep having to leave you.

The problem this time, just so you know, was the port. You know that thing in my arm that means the nurses have to come once a month and you get to root around in their bags for fun things to chew on? Yeah, well while you're off snacking on rubber gloves and stethoscopes, the nurse is busy taking care of my port-a-cath, a permanent IV line running from my arm to my chest which has now become infected. So on Tuesday when they sent me home because my lung infection seemed under control, they had no idea that I was going to crash very suddenly overnight because of a full-on systemic infection from the line. Neither did I, and of course, neither did you. You were just happy, like any good dog would be, to have your rightful human home and back where she belongs.

And speaking of back, Sammy, I really am. As in, back in the EXACT SAME ROOM. I wasn't even gone 24 hours, so they managed to put me right back where I came from, which is nice because it kind of feels a little more homey. Only not really homey because, of course, you're not here. (Note to self: speak to hospital about establishing designated "shorkie visiting hours.") But yeah, anyway, back to why I won't be home sooner . . .

See, once they decided my port was infected yesterday, they sent me to have it taken out. So I went down to the procedure room and got all numbed up with lidocaine and they opened up my arm and started to remove the port. Sounds pretty simple right? Pop out port, pull out catheter, sew up patient. Granted, it's not quite as easy as it sounds, I'm sure, but still, none of us were anticipating any problems.

But then again, it is your mama we're talking about, Sammy, so maybe we should have been better prepared for total ridiculousness.

Step 1 went fine - the port popped out no problem. Step 2, on the other hand, not so much. The surgeon tried to pull out the catheter and I told him I felt a pain in my back, so he stopped. When he stopped, the catheter literally sprang back into my vein, sort of like a vacuum cord when you hit the "automatic retract" button. Weird. Okay then, so he tried again. Same thing. Seriously, the thing was stuck. Solution? Tuck the port back into the arm and proceed directly to step 3 (sew up patient), and then schedule port removal in the actual OR for the following day. And I wasn't too upset about it. After all, if I'd been happily nesting in some place for 9 years, I probably wouldn't want to leave either.

So today I had surgery in the OR to get my port removed, sort of like when you had surgery to get your . . . well, you remember your surgery, Sampson. Anyway, unlike you, I was able to remain awake for my surgery, but I was given some fun sedative drugs that made me nice and talkative to the surgeons. This time the plan was a bit more complex: open up arm even farther, remove port, try again to remove catheter, if that fails, slide larger catheter up around current catheter in attempt to gently dislodge catheter from vein, sew up patient.

Well, they tried Sammybear, but it still didn't happen. Turns out this baby is stuck to my vein with some serious scar tissue way up in my shoulder, and the only way to remove it would be through full on surgery (sorta like what you had) only no one wants to do that to me because of the risks associated with putting a CFer on the vent. So I'm kinda stuck for now with an infected catheter in my system (they did, interestingly, remove the actual port -- just snipped it off and put something on the catheter to keep it from sliding into the vein). The good news is that the infection is strep, not staph or pseudo, so it's very treatable and I'm already responding to the additional antibiotic they've started me on to attack the bug. So the infection as it stands now isn't so much dangerous. The bad news is that they can only use this antibiotic for 4-6 weeks, and after that they'll take more blood cultures. Since the catheter is still in there, though, it's likely that the infection will reoccur, in which case we're either going to have to decide how to treat it (we could alternate antibiotics, maybe) or go ahead with the removal. If I can somehow deal with this until transplant then they should be able to just remove the catheter once they're already inside there. Fun, fun, huh Sam?

Anyway, in the meantime I'm getting meds through a lame-old peripheral IV line in my hand. I've already blown one, and my left arm is out of commission until it heals from surgery in a couple of days, so let's hope they can find enough veins in my right arm! You'd have fun with me, Sammy, I have tubes everywhere! On Tuesday, once they're sure the infection is under control for now, they're going to give me an old-fashioned PICC line so that I can keep doing these IVs without continuing to blow veins left and right. And the good news is, once the PICC is placed then I can FINALLY get home to you! I know you can barely contain your little puppy self with all the excitement!

As for the future of the port (and the fun nurses with their magic bags of chew toys), well, I may or may not get a new one. They don't want to put a new one in if the old one is going to be a continued source of infection because it would just infect the new port (sort of like why they do double-lung transplants in CFers). So if in 6 weeks or so after I'm done with all the IVs the blood cultures come back clear and they think the old catheter is clear of infection, I can get a new line placed pre-tx. Otherwise, no dice. And they also seem to think I'll be transplanted sooner rather than later at this point, so it may not even be a major issue, since all ports would have to come out during transplant anyway.

So that's the story, Sammybear. I miss you madly, but hopefully now that we finally have this a little more figured out I'll be able to come home to you a much happier, healthier, and spunkier Piper.

And I'm sure you're already plotting some creative ways to keep me busy.

A Room with a View

When I moved into my current apartment I was really excited about a lot of things: extra space, hardwood floors, a nice kitchen, and, most of all, honest-to-goodness floor-to-ceiling windows along an entire wall of every single room in the place (excluding, of course, the bathrooms). Being a recent graduate of student housing with a spectacular view of the Harlem projects, from which I subsequently moved on to a 6th story Upper West Side place that had a view of a grafitti-laden brick wall (as a bonus prize, a number of the grafitti markers were offensive racial slurs and/or curse words), you can imagine how excited I was to move into this mecca of lower Manhattan. I'm not kidding when I say that from my perch on the base of the island I can see all the way up to the Chrysler Building, and all the gorgeous sparkling lights in between. Given this excitement, I made the move without too much thought about those little things called "window treatments." Blinds, curtains, drapes, shades, whatever you want to call them, I rejected the entire concept in creating my decorating scheme. I was determined not to let anything get between me and my view, even if I put it there voluntarily.

It wasn't until I'd actually lived in the apartment for several months that winter turned to spring and spring turned to summer -- funny how that always seems to happen -- and then I began to realize the possible stupidity of the no-blinds choice. I mean sure, I love waking up to a bedroom full of bright sunlight and the delicious warmth of a summer day. I just wish it didn't happen at 6 in the morning. Turns out that floor-to-ceiling windows are more than just cool decorative perks -- they also make awesome alarm clocks.

Luckily for the past few days I've been avoiding the situation altogether by hiding out in a dark, dismal dungeon (um, whoops, I meant "cool, cozy corner") of Columbia Presbyterian Hospital. Otherwise known as a "respiratory isolation room", this little piece of . . . um . . . "heaven" comes complete with its own double-door separation shield from the outside world (by which I mean the hallway) and a separate air-filtration system. As a (small) concession to those fools who actually prefer to be able to see beyond the four walls enclosing them into their own private leper colony, the room does, in fact, contain a window. Unfortunately, as mentioned in the last post, this window appears to look out into some sort of eerie hospital graveyard -- a place where chairs, cots, and other miscellaneous furniture come to die. The effect is far from comforting, although it does provide one with adequate darkness to sleep until noon, so who am I to complain?

Another unadvertised perk of the "the hole?" Well, for someone not, in fact, actually on respiratory isolation, and therefore not restricted from leaving her room, there is a real opportunity for shock value. Simply grab your IV pole and head out for a romp in the halls, keeping a close eye out for the looks of horror you receive as other patients realize you emerged from "that room." Trust me, this is 100 times more exciting than coughing at the gym and just hoping that someone is paranoid enough to think you have swine flu, because this time they don't just THINK you have it: THEY HAVE PROOF. Or, you know, they think they do, because you did, after all, come out of "the hole." Who cares that the nurses smile and wave as you nonchalantly pass the station? You came out of the hole, and the hole is clearly marked as "strict isolation." Case closed, counselor, so you can take all your fancy mumbo-jumbo about "CF vs. tuberculosis and the different types of isolation" and shove it. (Only they won't, of course, be saying any of this to your face, because they'd sooner lock themselves in their own holes than come within 5 feet of you.) Add to this the slightly drunk-looking walk of a patient on IV polymyxin, and you've got yourself a recipe for some relatively exciting jaunts around the ward. Seriously, what's not to love here?

Well, sadly enough, yesterday I was moved out of "the hole." I am now in a normal room, with a normal window, that just happens to look East and thus brings me comfortingly close to the alarm-clock-like effect of my windows at home. And word on the street is that I might actually BE home by tomorrow night or Tuesday morning, because my sats are way up, my cough is subsiding, I haven't had a fever in several days, and I seem to be tolerating the polymyxin about as well as anyone could have predicted. True, I had a tiny bit of hemo this morning, but it was less than a tablespoon and no one seems overly concerned considering the beating my airways have taken from all the coughing/infection lately. They also took some samples from that strange rash on my legs today, so they should be able to confirm that it is, in fact, not serious. And then I can almost definitely go home, yay!

I did volunteer to do a study to help determine proper dosing rates for the use of IV polymyxin in CFers and transplant patients. I figure since I can't help out with many of the CFF's studies, I can at least help the medical community understand the drugs that are already approved a little bit better. So that may keep me here until Tuesday morning, but really that's the only reason anyone sees now for me to stick around. Here's hoping it stays that way and that I can do my part and then get the heck out of dodge and back down to my puppy.

And my many, many windows.

(Poly)myxin it Up

Alright, we've had the break DOWN, so now let's mix (or maybe in this case, "myx") it UP, shall we?

Here the situation: yesterday, after starting IVs the day before, I realized that the combination of fevers, chills, being exhausted, and coughing up tons of goo probably didn't bode too well for my ability to effectively manage and stay on top of my CF care at home. I knew I needed a little extra umph, and my doc agreed, so I went ahead and checked into my beloved Columbia Presbyterian and ended up here -- about 10 miles and the length of an entire (rainy) island away from my puppy and my apartment.

It's been non-stop ever since.

Okay, I really hate to complain, but let me get this one off my chest (and yes, bad pun intended, deal with it). I am in, quite possible, the most ridiculous hospital room ever. Seriously. It's an isolation room, but not just contact isolation like they use for most CFers. Oh no, this is full-on, hardcore, "wow, she must have swine flu AND tuberculosis to warrant that kind of lock-up" style room. There's not even a window to the outside in here, as if I might accidentally infect the boats on the Hudson were I allowed to see them. Although, to be fair, the room does *technically* have a window. It's just too bad that said window a) is covered by a large cabinet of sorts, and b) looks out into a weird hallway that appears to serve as some sort of storage area for unwanted hospital chairs. Weird.

Alright, so I'm admitted, and there's NO WAY that I'm infecting ANYONE within 50 miles of this hospital with Pseudo (at least not yet -- apparently I'm first in line for a room change as soon as one becomes available, for which I am totally grateful). Sounds good, right?

Well, sort of. See yesterday they made the somewhat difficult, but probably necessary, decision to actually STOP the antibiotics. They stopped the drugs, waited for my fever to spike, and then drew blood cultures that were free of anti-infectives and should show exactly what's going on just in case this isn't a classic lung infection. I think, although I'm not entirely sure, that they did this mostly because of the weird, still-unexplained fevers I had been running a few weeks ago, and the off chance that this is some sort of relapse of that problem rather than what it likely is, which is yet another fun CF exacerbation. So the downside of all this was that last night I had to go through more fevers, and I coughed all night, and I had to spend a night in the hospital without receiving any treatment, which is always frustrating. But at least if those cultures are negative (and they most likely -- finger's crossed -- will be) then there won't be any more talk of removing my port.

So today is a new day, and they just now restarted my antibiotics. I am now on tobra and a new drug: polymyxin. Here's the Antibiotics for Dummies version: the polymyxin family is most commonly known to CFers because of Colistin, which a lot of use as an inhaled antibiotic. I'm not on IV Colistin, but a slightly different form, known as Polymyxin B. Consider it for all intents and purposes the same thing. Basically, this drug (as I understand it) is pretty much the ultimate in last-resort drugs to treat PA. "Ultimate" because it's normally very effective. "Last resort" because it's also known to be pretty neurotoxic and nephrotoxic. The nephrotoxicity doesn't tend to be a problem with patients who have normal kidney function to begin with, I'm told (and thankfully I do), but the neurotoxicity can cause pretty unpleasant side effects in some patients, ranging from tingling and numbness to confusion and hallucination. Yikes. The good news? Well, first there's the fact that I'm in the hospital, so I have people here to monitor and help me. And second, well, the effects (both neuro and nephro) tend to go away upon discontinuation of the drug, which has a pretty short half life (a few hours). And finally, well, the only reason we're forced into this drug is my allergies, and not insensitivity on the part of my PA, so if the side effects are too uncomfortable after a dose or two, I'll simply stop this IV and get desensitized to zosyn or ceftaz, which I'm told is a pretty straight-forward process.

I'm not going to lie, I'm a little nervous. My infectious disease doctor ordered the dos (which is normally once a day) broken down into two doses to try and avoid any reactions. That's a good thing, except for the fact that each dose lasts 4 hours because he's also ordered it diluted in a TON of dextrose. But if I tolerate it well then yes, I can go home on it, and while it would likely be a pain to be on an IV that long, it's better than the hospital.

Not everyone has side effects so hopefully I'll be lucky. And hopefully this drug will do the trick and I'll be able to go home on Monday or Tuesday. Weekends around here are always pretty boring, but finger's crossed that I'll at least have a window sometime soon, so I can at least watch the river during my 4-hour IV drip.

Everything You Ever Wanted to Know about My Lungs (But Were Afraid to Ask)

So instead of random poetry and cryptic messages tonight, I thought I'd give you a general update on me and explain why my blogging etiquette may be a little wishy-washy for a little while.

If you've been following this blog, you'll know I had a lot of issues pre-Turkey, some of which were likely attributable to low potassium levels and some of which were really mysterious. My PFTs were down a bit from baseline and my cough was up, so we did a course of IVs, ordered just about every "just in case" script you could possibly need for a trip oversees (TamiFlu, Cipro, Levaquin . . . ) and even made sure I had the name of CF doctor in Istanbul. Then off I went.

The trip was phenomenal, despite the fact that I began running fevers and having severe soreness in my arms and legs about halfway through. Because I had started the cipro, though, I had managed to get a bit of sun rash even with SPF 75 (no joke), so I attributed the fevers/aches to a mild case of sun poisoning. I had pretty great energy aside from the pain though, and I spent the days sea kayaking, swimming, hiking, and generally tromping around the country, using O2 as needed. Basically I felt "better," despite the night fevers, sweats, and aches, than I had in a while.

Fast forward to coming home, where I more or less tanked. I was coughing up TONS of mucus despite being strictly compliant on my trip and was exhausted and really achey. Weirdly, though, my PFTs were up at 38%, which is very decent for me, but obviously a number isn't everything, so we started IVs, ran blood cultures, and tested for a virus called CMV, as well as flu and swine flu just to be safe.

Two days later I'm negative on all the viruses, I still have fevers of 101-102 daily, and I'm sleeping ALL day and sore as can be whenever I'm awake. At that point we noticed the low potassium levels so I got permission to just take advil round the clock and get the potassium back up to normal, in the hopes that this was a virus and would resolve as my body got healthy enough to fight it off.

No such luck. Two Sundays ago, in a last-ditch effort, I switched from Merrem to Imipenem, but by that Monday, after two weeks of fevers peaking at around 102.5ish, I went into the hospital, where I had every test under the sun and was found to have: 1) an enlarged spleen, 2) anemia, 3) low potassium (knew that), 4) elevated liver enzymes, 5) some residual pneumonia (remember, this is 2 weeks into IVs), 6) a collapsed upper left lobe (and okay, that last one is no big deal for me because it seems to happen intermittently and resolve itself), and 7) a fever of 102.6. Surprisingly though, after the initial day in the hospital, my fever never spiked above 99.5 again. My pain started to resolve and things were looking up. All blood cultures were negative and all virus/parasite tests came back clear. Infectious disease wanted to pull all my abx to get a blood culture without any drugs in the system (which might mask the problem), but my CF doc wasn't comfortable with that so I remained on tobra and imi. I also had an echocardiogram to rule out a heart valve infection. This was, of course, in addition to the two CT scans, abdominal ultrasound, and various other tests (mostly blood draws). They also tested my sputum for pretty much every weird bacteria under the sun, and so far no dice (except that I did learn from infectious disease that I have "dozens" of pseudo strains in addition to my staph and achromobacter -- lovely).

Okay, so no fevers in hospital = jailbreak, right? YAY! I got out last Thursday, went home, had some peanut butter, took a nap, and woke up with a fever of 100.5. No joke. Plus I had some weird rash all over my legs, so of course I called my doctor. The end result: benadryl for the rash, keep an eye on the fevers.

I ended up back in my clinic today after running daily fevers of 100+. Here's the deal, the fevers appear to be getting milder. The red splotches all over my skin aren't too concerning. My spleen, potassium, and liver are back to normal. We think this might be FINALLY resolving, EXCEPT: I still have fevers, I'm still sleeping all day, and I still ache like nobody's business. We ran more blood cultures, she wanted to admit me, I asked if it was really necessary since I have someone living with me right now (shout out to my awesome godmother, who flew in from CO to take care of me!), and she said it was fine to stay at home where I'm more comfy.

Here's the plan: IV abx through wednesday and I can take tylenol to break the fevers. Starting inhaled colistin. Discontinuing all oral abx including zithro. This way, IF the fevers persist past Wednesday and the stop of the abx, we can draw more blood cultures without the risk that the abx in my bloodstream are hiding the infection. And then possibly my port will need to come out.

The good news? We've eliminated literally everything they know of that might be super serious. If it is a blood infection (please pray it's not), then it hasn't spread to my heart valves, which is fantastic. It's entirely possible this is just a virus they don't really know much about, similar to mono, and it may even BE mono except that I had it years ago so there's no way to definitively test me for that now.

The bad news: if the fevers aren't gone by Friday, viral or not, I need to go back into the hospital and wait this out. And if they truly can't find any cause they may remove my port just to be safe, which is annoying as hell. Then again, seeing as I've had the thing for 9 years there's actually a good chance it's part of the problem -- they rarely last that long without some sort of issues. I'm really hoping if they remove it they'll allow me to get it back in my arm; my doctor promised to work on that.

The awesome news: I'm still at home with my puppy and typically only having one fever per day. Once it breaks, which it does with rest, gatorade, and tylenol, I've been cleared to do some light walking to keep my strength up and regain some of my lost workout time. Nothing serious here, obviously, but honestly I never knew walking a shorkie down a crowded lower-manhattan street could be so much fun. And tomorrow I'm going to meet my friend at the best pizza bar on Wall St. for lunch, assuming I'm up for it, so life is slowly, slowly regaining SOME sense of normalcy. AND, despite the collapsed lung that hurts like the devil everytime I breathe in (you get used to it, believe me) I suddenly have O2 sats that actually reached 97 on room air! They're hovering around 94 now all the time, which is unbelievable, amazing, wonderful, and beyond all words. I can't wait to see what my PFTs are when this is all over, considering they started at 38%.

Alright, sorry for the symptom dump. I just thought I'd fill everyone in just in case I go AWOL again, or on the off chance any of you were torn to pieces by my sudden lack of fun updates. I'm pretty sure there's more fun to be had though, even if it does have to wait it's turn through all the madness.

Pip's Tips: Lessons on How to Survive the Hospital

Lesson 1) BYOE (Bring Your Own Everything/Essentials/Enzymes)

Hey, life in lock up is rough, we all know that. Why make it worse by not eating? Today (day 3 of hospital admission, although to be fair only my second FULL day), my nurse did something surprising and unexpected: she gave me enzymes with my meal! Granted, the enzymes have been ordered since day 1, and I've asked for them on a daily (well, make that 3x per day) basis, but today they suddenly showed up. At breakfast. And dinner. Not so much lunch, though, and definitely no extra for snacks. And yet, here I sit, fully fed and not on the toilet.

Nope, I'm not superwoman (ahem, well, I mean, not for that reason anyway). I just know from experience that self-medication is often necessary in the joint, and like a good boy scout, I've learned to be prepared. For me this means having "back-up" supplies of any med that I might be uncomfortable without or which might be time sensitive. My personal "big three"? Enzymes, xopenex inhaler (for those days when
morning" nebs come around 1 pm), and most recently ativan.

Lesson 2) If the peripheral IV that the nurse is inserting is for a CT scan, make sure s/he KNOWS it's for a CT scan

This one might seem a little strange. After all, why wouldn't the nurse know what the peripheral IV was for, since you clearly already have a port/PICC/whatever else delivering your antibiotics? And why should it matter WHAT the IV is for anyway? An IV is an IV is an IV, right?

Sorry, cyster, and not so fast, fibro. Turns out, CT scan IVs require a larger-gauge needle, perhaps because the contrast dye doesn't go pass through the smaller gauges well. I'm unclear on the technical details here, probably because everything was feeling a little fuzzy after the IV nurse inserted (and subsequently blew) no less than 4 peripheral IVs in my arm in less than 15 minutes. He then properly inserted and did NOT blow a fifth IV, which fit snugly into a cute little vein in my hand. Alas, it was not to be, because as he left the room he commented on my uneaten lunch and I explained I was NPO before the CT scan. He immediately turned around, got a really pissed-off look on his face, and removed the hard-won IV from my hand, explaining that this particular IV would not work for a CT scan and no one had informed HIM of the need for a large-gauge needle. Whoops.

I felt for the guy. I really did. He inserted (and blew) one more IV before giving up entirely and calling in another IV nurse, who managed to outfit the inside of my right wrist (right under the thumb) with the least-convenient IV ever. But I'm not complaining. Never. Not my style.

Lesson 3) Become Master of Your Own Discharge

My doctor told me this morning that I could leave tomorrow. Since then, I've been telling everyone who will listen (and some who clearly didn't want to) that I'm out of here as soon as possible tomorrow, preferably by early afternoon. And no, this is not because I'm some sort of evil, demanding, devil child who will do anything to force her way out of here and back to a working shower. Not at all. It's just that I've done the discharge dance enough times to know that things get scheduled for the day of your discharge, and then you have to wait. So why not tell the CPT guy in advance that you're scheduled to leave so he doesn't leave you for last? Why not inform the nursing station so they can make sure to get you your morning nebs extra early? In short, why not (politely, of course) bring everyone onto the same page, so you don't get stuck waiting on some last minute test or x-ray that just didn't get on the schedule in time? So far tomorrow I know I'm in for an echocardiogram before I get to break out, but my nurse has promised me it should be early in the morning. And of course if it isn't I'll wait patiently (thanks, Ativan!), but wouldn't it be great not to have to?

Lesson 4: Neb Cup Sanitation -- Piper Style

Don't know about you guys, but at our hospital we have a one neb cup per med per 24 hours rule. That basically means, fr things like HTS or xopenex, that they get used 3-4 times before they get switched, and in between that time they're sitting in the hospital room, exposed to hospital air.

Or are they?

Step 1: Receive and label neb cup (this one's for DNAse)

Step 2: Remove glove from box of sterile gloves that should be near the door of EVERY CFer (so nurses, etc, don't spread germs from patient to patient)

Step 3: Place glove securely over mouthpiece and top of neb cup, thus minimizing exposure of neb mouthpiece to hospital air, and ensuring that nothing will get into the cup of the neb through the top openings

Voila. Okay fine, it's not how your mama used to sanitize your nebs, and it's certainly not foolproof, but ask your nurse for a clean basin lined with plastic to place these babies in once they're covered and you can be relatively secure in your neb-cup safety. For 24 hours, at least.

And there you have it, tips for surviving life in the leper colony. And hopefully something to make you smile while you wait for those discharge papers to be written up.

Welcome to the Hotel Presbyterian

Such a lovely place, although I'm hoping I can both check out AND leave -- preferably by the end of the week.

Yeah, I landed back on my ass in 9 Hudson South (actually the best ward going over here, with a menu for ordering dinner, private rooms/bath, and guest internet). The twist is that we don't actually think this one's all about the lung infection, or at least 2 1/2 weeks of solid 101 degree fevers daily says this isn't your average pneumonia. We're thinking viral, but they're not ruling out an infection that we're just not hitting yet . . . including the ever-dreaded port infection. Fingers crossed it's not that, although other contenders include such fun little visitors as Lyme Disease or a relapse of Mononucleosis.

I'm just hoping that whatever it is, it starts to resolve itself FAST.

They've already switch my abx from Merrem to Imipenem, which they did over the weekend at home and may already be making somewhat a difference. Also my potassium levels are climbing, which is wonderful. So basically I'm in here, according to my doctor, "for a couple of days" (famous last words!) to get some tests run and hopefully figure this thing out for good. It's been bothering me for waaaay too long.

I didn't even put up my usual crazy stubborn (um, I mean, polite and well-articulated) fight to stay out of the joint this time. Sometimes you just know you're headed for those plastic sheets, and the best that you can do is just grit your teeth and bring your own comforter. As my blogger cyster Cystic Gal might quote, "you got to know when to hold 'em, know when to fold 'em". . . and hope to God that your doctors know when to let you walk away ;)

Be Still, My Heart

No, really, I mean it. Be still. Please? Pretty please? Just for a minute or something? Seriously Mr. Heart, I'll be your best friend!

(Disclaimer: the author of this blog would like to state for the record that the above is not actually a request for her heart to stop entirely. That would be a little "too still" for her taste. Instead she respectively requests that her heart simply stop its persistent new hobby of adding extra beats and/or skipping beats frequently. Because, much like Sampson's favorite passtime of sliding his bones under the couch and barking loudly until someone retrieves them for him, this new game is getting really old, really fast. Seriously.)

So yeah, I've got heart problems, and for once they don't involve one too many glasses of wine and a cute guy at some hip downtown bar. Although frankly the 8 million doctors appointments (note: actual number of doctors visited may vary) and two ER visits I've had in the past couple of weeks indicate that the wine/bar situation might actually be a less annoying kind of heartache. More dangerous, sure, but less annoying.

Long story short I have some unexplained arrhythmia in my heart that has been really persistent for the past week and a half. And finally, after approximately one hundred and two attempts at self-diagnosis ranging from "transplant anxiety" to "TOBI reaction" to "heart attack leading to imminent death" (with the last one being a personal fave), and the normal 2-4 day period of "toughing it out" that seems to be the unspoken code of all cystics everywhere, I actually managed to do something productive last night and take myself to the NYC ER for an EKG re: my CF. And let me just say: WTF?!

Seriously, if you've ever had a really strong urge to watch tons of people having the worst day of their lives, go to a New York City ER between the hours of 11 pm and 3:30 am. Yikes. I can honestly say I've seen funerals that were happier and more uplifting than that place, largely because most funerals I've been to have not been 90% populated by people coming down from drug highs and alcohol poisoning. And frankly I'm not a major fan of judging others, but the guy with the HIV who had just been discharged 1 day prior after a serious infection and then decided to do "just a line or two" of cocaine that morning to give himself a little "boost"? Um . . . yeah, he might need to experiment with some new coping methods. You know, just my opinion.

The good news is that nothing is seriously wrong with my heart. All the doctors agree that while this arrhythmia is oddly persistent and annoying, it's not really dangerous. My heart is actually quite healthy, in fact, unlike the rest of me. It's also quite strange and ridiculous, which fits right in with the rest of me, so at least we have that in common.

The saddest and most sobering part of the whole experience actually didn't involve me or my attention-hungry heart though. One of the residents came over to chat with me a little about CF right as I was being discharged, and she mentioned that she has a friend with a 6 year old daughter facing transplant from this stupid disease. As if that weren't tragedy enough, the little girl just contracted cepacia and has been removed from the list, apparently, so please keep them in your thoughts and send some good vibes out to them, wherever they are. In all seriousness, CF is a battle we can never stop fighting . . . not until every single child born with this disease has the chance to recognize his or her full potential. Every CFer I know is a fighter, in his or her own way, and my heart goes out to all of them. All of us.

And don't worry, that's a perfectly healthy offering on my part ;)

Shaken, Not Stirred

Okay first off, can I just say that wow, it's been an intense couple of days? Seriously, I don't know what I would have done if my mother hadn't flown in from CO to help me out for a while. Funny how sometimes you just need a mom around, right?

I got out of the hospital on Saturday right after CPT, which was awesome. The RT staff there is amazing - they always make sure to hit up all the CFers by noon at the latest, since in the words of one great RT, they know we need it to be comfortable. The bonus to this is that we get discharged earlier as well. So I was on my way home by noon, and not a moment too soon. The only bad thing being that the nurse or pharmacy or someone f-ed up and gave me parkinson's medication instead of my prednisone. I looked at the pills, which were round and slightly off white rather than round and white, and immediately questioned it. Thank god for my professional patient status, huh? Anyway, for future reference and to anyone who cares: 50 mg carbidopa does not equal 40 mg prednisone. Duh.

So I was discharged on a lovely cocktail of imipenem every 6 hours, aztreonam every 8 hours, and tobra every 24 hours. The aztreonam and tobra are via intermate, so no problems there. But the imipenem? You guessed it...gravity drip! And not only gravity drip, but the thing has to be mixed right before delivery. So my cocktail quite literally has to be "shaken" every 6 hours before I can serve it to my greedy little lungs. Bastards - they're always so demanding!

And some of you might remember the whole fire and flood post from Tuesday? (Sidenote: how biblical does that sound for holy week? Sheesh!) Well yeah, turns out there are still some major de-humidifier fans going full blast 24 hours a day in my apartment to help ensure that there's no mold growth. For those wondering, obviously I am not being complacent about this - I have inspectors set up to come multiple times and I will not rest until I'm positive there's no mold in my gorgeous new apartment. But in the meantime, Sammy and I have moved into the Millennium Hilton by the WTC site. Lovely. And of course my mom has as well, so that she can help out with the infusion schedule from hell. In other words, I'm living in a hotel four blooks from my apartment, with my puppy, and my mother, and my gravity drip. All of which is just a little overwhelming, to say the least. Thankfully: 1) the hotel was sweet enough to upgrade us to a king suite when we laughingly told them the whole story at check-in (gotta laugh, there's no other way to deal with this sort of ridiculousness), and 2) the insurance for the genius who started the fire is going to indemnify us the hotel room, as well as all other damage-related expenses.

And then, to top it all off, Sunday was obviously Easter, and of course my sister and I had invited several of our "orphan" NYer friends to Easter brunch at my place - "orphan" meaning their families live too far away to visit for a weekend. My mom, my sister, and I talked and decided we would prefer not to let fire, flood, and plague (told you it was biblical!) keep us from celebrating, so we ordered a honey baked ham and all the trimmings from Whole Foods, moved all the fans into the hallway, cleaned up the place (they cleaned, I rested), and hosted anyway. So. Much. Fun. No hospital, no nurses, no beeping: just family, friends, wine, good food, and the ever-present gravity drip ;) Afterwards we watched America's Next Top Model marathon and finished the wine until we had walk back through the city lights to our king suite. Tough life I have, right?

My only other news is that I officially took 1 month short-term disability from work. Obviously for these next 2 weeks with this IV schedule I can't be at the office, and afterwards my doctor and I both agree that I need time to rest, relax, and figure out exactly how I need to move forward. I have amazing benefits at work and am lucky enough in that department that this isn't a financial decision - and I spent a few weepy moments with my mom last night wondering exactly how I got lucky enough for that to be the case. For now I have a couple of projects I am going to (gently) work on for the next few weeks as I try to wrap my health and my mind around where things are headed.

Honestly I'm not happy about not being able to work, even temporarily. And I'm scared shitless about where my disease is heading. I'm also overwhelmed with how lucky I am to love my doctor, have family who drops everything and travels across the country to help me, friends who see me at my worst in the hospital and still show up to Easter dinner even as I lie drooping on the couch, and a job that will leave me with everything I need insurance and income wise regardless of whether I am physically able to work or not. I don't need to be told how blessed I am and how rare so much of that is. It's a weird balance, isn't it? I mean how much energy can you spend being sad when so much is being given to you? But by the same token, how much of a merry sunshine can anyone honestly be in the face of losing so much? And it is a loss, I'm not going to sugarcoat that side of it. I don't think I'd be doing myself or anyone else any favors by pretending I was thrilled with the situation.

So I'm out of the hospital, but on a bitch of an IV schedule. I can't live in my apartment, but I have a gorgeous hotel suite with people and puppies I love. I had a great Easter, but I felt sick. I have to take time off work, but I have a great support system and amazing benefits. I feel better, but I'm on my third round of IVs for 2009 alone. I have a lot of drive to get better and stay healthy, but I know doing so might mean giving up some things I love - plus I'm up against a killer disease.

I feel kinda shaken, just not entirely stirred.