(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.

The Joy of Cooking

What do you get when you take one cystic, add two months of fevers and acheyness, throw in two courses of IV antibiotics, add a dash of hospital stay and some of the best doctors in the country, mix it up with two very fun and awesome weeks in Turkey, sprinkle on a dash of pretty much every single medical test ever imagined and then let bake (at a pretty much constant temperature of 101 degrees or higher) until covered in a very creepy, weird cluster of reddish-brown rashes?

Apparently, actually, you get a perfectly healthy Piper.

Yeah, I know, I don't get it either. Then again, I've never been much for cooking, although I will admit to a slight addiction to Top Chef. But I AM a lawyer (recent "retirement* from active practice aside -- once a lawyer, always a lawyer), and that should make me a pretty logical person, or at least one used to getting surprising results from odd facts, and for the life of me I still can't figure this one out. What I DO know is this:

*I had one additional 101+ fever on Wednesday night, after writing my last blog
*After said fever, which of course occurred at night and was surprisingly difficult to break, I emailed my doctor and basically said "enough! I surrender!"
*My extremely wise doctor decided to wait a couple of days, order a few more necessary tests including blood cultures while off the antibiotics, and in the meantime allow me to remain in the comfort of my own home.
*Two days later (and two days fever free), my tests were all negative, once again indicating that it was some sort of virus, and my abdominal CT scan showed only two abnormalities: a slightly enlarged spleen, and a missing appendix.

I have to admit, I'm relieved about the appendix. As someone who got the chicken pox TWICE, once caught the flu twice in the SAME SEASON, and may well have gotten mono TWICE if the past two months are any indication, I really wouldn't have been too shocked if my body had decided to grow another one of those suckers just to treat me to the joys of yet another appendectomy. Seriously. That's just how I roll.

Whew.

Yeah, so anyway, the spleen is still ever-so-slightly enlarged, but the fevers seem to have gone the way of the appendix at last, and not a moment too soon if you ask me. Which, of course, nobody did, but you are reading my blog, after all.

Today I spent the day enjoying what I would call "mild reality." I left the house several times, twice to take short walks with my puppy, once to walk to a neighborhood restaurant for a yummy lunch, and once to visit the very famous FAO Schwartz toy store to pick up some board games, puzzles, and cards. Yeah, that's right. I figure if I might be stuck inside some days/nights/hospitalizations during the wait for these new lungs, I may as well have some way to make staying in a social occasion. You know, as prepared as any 27 year-old who just blew over $100 at a toy store can be.

Um, yeah.

So tomorrow is going to be a slightly less "mild" dose of real life, involving actual brunch plans with friends (*gasp*), some relaxing sunbathing with a couple of girlfriends on my roofdeck, more dog walking, and a trip to the gym during which I solemnly vow not to expect too much of myself (but still to make genuine effort on the treadmill for at least 20 minutes).

In other words, what do you get when you stir all those things (and more) up and let the whole mess sit for the better part of Summer, 2009? Well, for better or for worse, I guess the answer is life, although I'm sure most of you cystics out there probably already knew that.

Maybe I'm not such a lousy cook after all.

I Believe in Miracles

Since you came along, you sexy things, you.

Alright, seriously, which one of my readers is secretly some sort of magical long-distance healer? I really want to know, because I've got some major jobs out there for you, whoever you are . . .

Honestly and completely without kidding, my fevers ended the night I posted my all-encompassing symptom dump. And no, lest you worry that my body is any less dramatic and attention-hoggish than the rest of me, they did not just fizzle out and go away. Oh no. In true Piper style these suckers decided to go out with a bang -- a 103.5 degrees at 3 am bang, to be precise, complete with coughing fit and, well, that thing that happens to CFers when they cough too much and drank three Boost plusses the night before. Um, yeah. Thanks so much for that friendly goodbye, fevers from hell. Love you too.

But, since that night I've been fever free. Which is not to say completely healed, but definitely on the mend! The really amazing part is that my doctor called yesterday and my white blood cells are back to normal and inflammation indications in my blood labs have also decreased to the point where they feel comfortable letting me STOP the IVs. That's the fifth time I've said those words in 2009 and let me tell you, it never stops feeling awesome.

My main complaint (because, hey, we can't be too cheerful over here . . . or can we?), is basically that I'm still pretty sore. I have an ultrasound this afternoon to see if my spleen has gone back down to normal, but I think my body's just been through the ringer so much lately that it's a little beaten down, much as I hate to admit it. I'm trying to silence my inner drill sergeant for a few more days and keep to mild walks (shouldn't be too hard considering I'm still sleeping several times a day), but the big problem is that my vest HURTS. I mean seriously guys. I'm not a fan of pain meds because I hate how they make me feel (same with anti-nausea, this should be interesting post-tx . . . ), but this is bad. I don't think it's the lung collapse, because frankly that happens all the time and I don't have too much trouble powering through it. This, on the other hand, is all over, achey, evil pain. I'm still doing the vest 3x a day for 30 mins (lungs, you owe me AT LEAST a month of health for this one!), but I'm having to do it at a way lower frequency than normal, and even then it's just barely tolerable. Can anyone PLEASE offer some suggestions about ways to make the vest a little easier to handle during serious viral pain? I tried layering soft clothing, but that only made me hot, sweaty, and itchy. Gross.

Okay, so now thaat I've given you enough nasty visuals to ruin even a cystic's appetite (why should I be NPO alone?), I'd like to just end by letting you all know how seriously grateful I am for the good thoughts, healing vibes, prayers, intentions, and sweet comments from all of you. I seriously feel tons better without those nasty fevers hanging around, and though I know it's baby steps back to where I want to be, I can't even express how amazing it will feel to be IV free, fever free, and hopefully soon, pain free. I'm going to be riding my new bike all over Manhattan and catching up on all the summer sun I've missed, big time.

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.

sick cystic, dozing dog

say that 10 times fast anyone?

still feverish, still waiting for answers, and now stuck inside on a rainy day. (and by "stuck," i mean cuddled up with my puppy on my sofa watching him dream and staring out at the cloudy skyline -- is New York the only city that looks gorgeous when grey?) anyway, i did learn this morning that it is NOT a heart valve infection, and the resulting deep void of answers led me here. enjoy.

without answer

what if this is just another normal,

another winding road without a name,

and what if i just simply journey forward,

reflecting on the path from which i came?

what if instead of cursing at the darkness,

i look around to watch the fireflies,

and turn my glances upward for a moment

to see the starlight dancing in the skies?

what if instead of waiting for disaster,

or some too-easy rescue that won't come,

i simply trust my feet to carry onward

and pray that in the end they lead me home?

and if i must accept the path is changing,

sure footing turned to dirt and wet like clay,

at least i can be grateful for the walking

and reach out towards the hands that guide my way.

and if in fact two roads meet in the forest,

and both are known to lead to the same plight,

then maybe this road too has been well-traveled:

one hundred thousand tries to get it right.


(and ps: for those of you out there who prefer the [relatively] funny piper, she's still here i promise. good vibes for a mellowed-out fever would totally be appreciated, though. she tends to prefer to do her turn in the body when the weather is a bit cooler in here!)

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 ;)