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.
About Me
- Piper
- I am a 33-year-old wife, sister, daughter, friend, law school graduate, CFer, lifelong student of public service, blog writer, patient, Sagittarius, reader, Top chef fan, double-lung transplant recipient (twice!), and dog owner living in Colorado's beautiful Mile High City. I love all things colorful, funny, inspiring, or needlessly sarcastic. I share my city with about 2,500,000 other remarkable people, share my disease with 70,000 other beautiful souls, share my life with some unbelievable family and friends, and share my apartment with one very handsome guy and one really fat mutt with a kick-butt personality. We make it work.
Sunday, August 30, 2009
Friday, August 28, 2009
(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.
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.
Wednesday, August 26, 2009
Break it Down
Literally.
Okay, so last night I had a bit of what one might term a "meltdown" (or, you know, a "total and complete freak out" would probably work too). There wasn't a whole lot going on to trigger it, aside from, well, everything that's been going on and building up and swirling around for the past few months, plus the fact that despite a serious try with oral antibiotics I'm back to running fevers and have a disgusting cough, which means I'm going back on IVs tonight. And then there was the difficult conversation I had with my transplant coordinator earlier in the afternoon, highlighting some of the reasons it can be frustrating to be listed at a wonderful, but very large and busy, center. And it was late, and I was tired.
In other words, yeah, it was a pretty well justified breakdown, if I do say so myself.
It's funny to me that after a lifetime of studying various types of expression (I was an English major in college, followed by 3 years learning how to be argumentative and unpleasant -- um, I mean law school), I've only very recently discovered my innate talent for the breakdown. I think this is partly due to the fact that, given the choice, I always try to be funny or stoic or generally not overly dramatic, especially when it comes to the details of my personal life. Sure, I'll bawl my eyes out at a movie or even some of the more sappy Mastercard commercials (the one with the lost dog KILLED me), but I don't particularly love to cry in front of people or have tough conversations if I'm the subject. I find that most of the time it just causes worry and pity, neither of which I like at all.
But you know what? Last night I had a breakdown, in living color and high definition, right there on my living room couch, in full view of my mother and with my father on the telephone long distance from Colorado. I had a breakdown and I managed to sob out every single question and fear and doubt in my mind about CF and lungs and transplant and even this silly virus that I can't seem to shake. I finally said something (well, I said everything really) to my parents about what it's like to be listed for transplant with a lung function still in the upper 30s/low 40s. I told them how scared I am that my tendency towards infection won't go away with the transplant, that instead I might just have fewer resources to fight them off. I told them how frustrating it is to work so hard for some improvement in your lungs or to keep an infection at bay, only to find yourself BACK in the hospital with yet another needle jutting out of your poor, overused port. I told them about my dreams lately, about worries for them, about . . . well, you name it, we talked about it.
In hindsight, I really should have seen this coming. It snuck up on me with about the same amount of stealth as a MAC truck, to be honest. Between the increased frustration of feeling sick for the past couple of days and the pressure of all the transplant stuff, I was showing some pretty clear signs of being on a one-way stroll down meltdown street. Take, for instance, the fact that I'd been waking up in ridiculous positions (head at the foot of the bed, feet dangling off a side) for the past week or so. Definitely not a sign of restful sleep.
So I had a breakdown.
And then, after it was all over, my mom and I actually made popcorn and sat on the couch until 1:30 am with my puppy curled at our feet, which kind of made me feel like I was in a Lifetime made-for-TV movie, but also made me feel really happy and relaxed. Happier and more relaxed, in fact, than I've felt in a really long time. My mom and dad, for their parts, were amazing, because while they did express their own fears and worries (rightly so), what they didn't do is shower me with pity. Instead of saying "I'm so sorry" when I talked about all this, for the most part they focused on the fact that they support me, that they think my questions are valid, that they've seen how hard I've been fighting and they're proud of me for it, and that they agree: the process is scary and frustrating and sometimes just warrants a little breakdown, just as long as it's the right kind of breakdown.
Yeah, you know the one. It's that breakdown that feels totally all-encompassing, that leaves you completely spent and suddenly feeling a little lighter and a lot less crazy. It's the one that comes right before you look around, pick up your thoughts that are scattered all over the floor, discard any truly irrational fears, give a great big sigh, and move on to the next thing in life, which is hopefully a little more upbeat and a little less exhausting. Breakdown accomplished.
So no, I'm not entirely proud of the fact that I spent over an hour last night sobbing about CF and transplant and all these things that on a daily basis I try not to let get the best of me. I'm certainly not bragging about keeping my parents up late and forcing us all to spend one more evening with CF as the centerpiece of our thoughts and lives. I'm not proud, but I am here to tell you that last night, for the first time in weeks, I slept soundly, in one position. And I woke up this morning with my head on the pillow.
Thank goodness for breakdowns.
Okay, so last night I had a bit of what one might term a "meltdown" (or, you know, a "total and complete freak out" would probably work too). There wasn't a whole lot going on to trigger it, aside from, well, everything that's been going on and building up and swirling around for the past few months, plus the fact that despite a serious try with oral antibiotics I'm back to running fevers and have a disgusting cough, which means I'm going back on IVs tonight. And then there was the difficult conversation I had with my transplant coordinator earlier in the afternoon, highlighting some of the reasons it can be frustrating to be listed at a wonderful, but very large and busy, center. And it was late, and I was tired.
In other words, yeah, it was a pretty well justified breakdown, if I do say so myself.
It's funny to me that after a lifetime of studying various types of expression (I was an English major in college, followed by 3 years learning how to be argumentative and unpleasant -- um, I mean law school), I've only very recently discovered my innate talent for the breakdown. I think this is partly due to the fact that, given the choice, I always try to be funny or stoic or generally not overly dramatic, especially when it comes to the details of my personal life. Sure, I'll bawl my eyes out at a movie or even some of the more sappy Mastercard commercials (the one with the lost dog KILLED me), but I don't particularly love to cry in front of people or have tough conversations if I'm the subject. I find that most of the time it just causes worry and pity, neither of which I like at all.
But you know what? Last night I had a breakdown, in living color and high definition, right there on my living room couch, in full view of my mother and with my father on the telephone long distance from Colorado. I had a breakdown and I managed to sob out every single question and fear and doubt in my mind about CF and lungs and transplant and even this silly virus that I can't seem to shake. I finally said something (well, I said everything really) to my parents about what it's like to be listed for transplant with a lung function still in the upper 30s/low 40s. I told them how scared I am that my tendency towards infection won't go away with the transplant, that instead I might just have fewer resources to fight them off. I told them how frustrating it is to work so hard for some improvement in your lungs or to keep an infection at bay, only to find yourself BACK in the hospital with yet another needle jutting out of your poor, overused port. I told them about my dreams lately, about worries for them, about . . . well, you name it, we talked about it.
In hindsight, I really should have seen this coming. It snuck up on me with about the same amount of stealth as a MAC truck, to be honest. Between the increased frustration of feeling sick for the past couple of days and the pressure of all the transplant stuff, I was showing some pretty clear signs of being on a one-way stroll down meltdown street. Take, for instance, the fact that I'd been waking up in ridiculous positions (head at the foot of the bed, feet dangling off a side) for the past week or so. Definitely not a sign of restful sleep.
So I had a breakdown.
And then, after it was all over, my mom and I actually made popcorn and sat on the couch until 1:30 am with my puppy curled at our feet, which kind of made me feel like I was in a Lifetime made-for-TV movie, but also made me feel really happy and relaxed. Happier and more relaxed, in fact, than I've felt in a really long time. My mom and dad, for their parts, were amazing, because while they did express their own fears and worries (rightly so), what they didn't do is shower me with pity. Instead of saying "I'm so sorry" when I talked about all this, for the most part they focused on the fact that they support me, that they think my questions are valid, that they've seen how hard I've been fighting and they're proud of me for it, and that they agree: the process is scary and frustrating and sometimes just warrants a little breakdown, just as long as it's the right kind of breakdown.
Yeah, you know the one. It's that breakdown that feels totally all-encompassing, that leaves you completely spent and suddenly feeling a little lighter and a lot less crazy. It's the one that comes right before you look around, pick up your thoughts that are scattered all over the floor, discard any truly irrational fears, give a great big sigh, and move on to the next thing in life, which is hopefully a little more upbeat and a little less exhausting. Breakdown accomplished.
So no, I'm not entirely proud of the fact that I spent over an hour last night sobbing about CF and transplant and all these things that on a daily basis I try not to let get the best of me. I'm certainly not bragging about keeping my parents up late and forcing us all to spend one more evening with CF as the centerpiece of our thoughts and lives. I'm not proud, but I am here to tell you that last night, for the first time in weeks, I slept soundly, in one position. And I woke up this morning with my head on the pillow.
Thank goodness for breakdowns.
Thursday, August 20, 2009
Logistically Speaking
Have you ever noticed how sometimes in life things just work out beautifully with absolutely no advance planning?
Like that Sunday 10 months ago when I was pulled from a nap by my darling sister's phone call and the simple phrase: "Get up and get dressed because I think I've found your future mate." Not always the most welcome words in the world when you're lying there with smeared mascara and bedhead, but as it turns out, 30 minutes later I was standing before a 4-month-old Sampson, who was deftly attempting to demonstrate his unbelievable cuteness by chewing on my sister's fingers. Okay, fine, so maybe biting isn't a preferred human method of flirting (although maybe it should be?) but it definitely worked on me -- hook, line, and life-changing sinker. And while I never expected to bring home a puppy that afternoon (and in fact had vowed never to have anything resembling a shih-tzu after a rather traumatizing run in with my childhood nanny's blind, deaf, and very snarky "puppy"), I couldn't be happier with the long-term results. No planning, no advance warning, and possibly the best outcome ever.
That moment aside, though, I've learned that there are certain experiences in life that DO require preparation. I'll be the first to admit that I've sometimes had to learn this lesson the hard way -- such as the time I failed to prepare at all for a science test in 9th grade and became quite possibly the first cystic in history to get a nearly failing grade on questions about the "biology of human organs" (oh, the irony!). Or that decision I made to wait until the very last night of the very last day of my college experience to even try and pack up my rather large bedroom and everything else I'd accumulated over two years of living with the best housemates ever. Good housemates, yes. Useful assistant packers in a crises, not so much. In other words, I've had a few examples of poor planning in my lifetime, and I hope I've learned from them.
And then along came transplant.
Okay, I am a lot of things, but (lousy grade on 9th grade Bio test notwithstanding), I am generally NOT stupid. So it's not as though I was expecting transplant to be something I could waltz right into with zero prep work or planning and wake up 8-12 hours later with new lungs, new breath, and everything totally organized to start my new life. And any delusions of simplicity I actually might have had in the beginning were quickly (and rightfully) shattered by my wonderful but very straight-talking transplant team. I was told I would need to attend educational seminars, schedule and complete all "routine maintenance" health appointments (eye doctors, dentists, etc) and immunizations AND receive from each doctor a letter of clearance, organize my support team and living situation to be ready for post-transplant living, and of course work on gaining weight and maintaining my exercise routine to be as healthy as possible for the actual surgery. This, of course, in addition to the three full days of outpatient testing I completed in August 2008, many of which I have subsequently repeated just so that everything is as up to date as possible.
And when you think about it, that's a lot of advance planning for a surgery that you can't really plan AROUND at all. In other words, I can't plan when the call will come. I can't plan which of the three amazing surgeons at my center will be on-call the day/night I get lungs. I can't plan to be back on my feet or back at work on any specific date. I can't tell my housekeeper the dates when she'll have to take Sampson in advance, and in fact I can't even tell her how long she'll have to keep my beautiful boy, since I have no way to plan how long I'll be in the hospital. It's as though the lack of ability to plan has mysteriously morphed into a compulsive need to double-plan just so everyone's as prepared for unplanned chaos as possible.
Um, yeah. It's really, really fun and exciting over at my house right now, let me tell you. Fun, exciting, and relaxing.
But okay, fine, I can live with a little chaos. Just not too much, which means that, having finished the first list of "planning to-dos" (pause for a major pat on the back here) given to me by my transplant team, I'm moving on to my own personal list. And I'm suddenly discovering that there's still a LOT to be done. Over the past week, in the frenzy of being listed, I've had to come up with ways to notify old friends -- some of whom I'm not really in touch with much anymore but know would want to be updated about something this major -- about the transplant, put together a ready-made listserve of email address for people (family, close friends, etc) who want to be notified of things as they happen once the call comes, and set up a place (probably a website of sorts) for people who just want the more occasional updates to be able to find and share information during the surgery and recovery. I've also had to consider what I want to bring with me to the hospital (for my stay that could be anywhere from 8 days to, well, as long as it takes), and what my family might want/need in the waiting room. I need to plan how I can best stay active and engaged and social while on the list without wearing myself out and over extending, and I have to make sure everyone knows that I might have to drop these commitments at the drop of a hat to go get the transplant. I've most recently moved on to planning out tough conversations, including finishing up my advance directive and other things that, as a lawyer, I just can't not think about. And finally I'm working on a personal goal of being able to walk 2.5 miles at 4 MPH without stopping (or dying) at the time I get called for surgery. Because a wise friend of mine once said "if you walk into a transplant, you'll walk out of it." And I figure she ought to know, since she walked upright into the O.R. before her double-lung and liver transplant. Yowza.
I gotta admit, I kinda wish finding new lungs could be a little more like finding my dog. I mean, how awesome would it be to be peacefully dozing on a fall weekend and have my sister call up with an adorable (and feisty!) pair of perfect new lungs for me? But I have a strange feeling that's not how it's gonna work out this time -- and if I do get that call, I think I might have to commit my wonderful sister, so that wouldn't be good either.
But if I'm really truly honest -- and when am I not? -- I have to admit that there's a part of this process that is actually kind of fun and exciting (in a very "man, I wish I didn't have to do this" kind of way). I'm learning a lot about myself and my family, I'm reconnecting with some really old friends, I'm getting to see first hand just how wonderful all these people in my life who have come out of the wordwork -- in fact, sometimes out of the pretty distant past -- to support me truly are, and I'm pushing myself in ways I didn't even know I could be pushed.
In other words, I think I'll have to wait, and plan, and deal with the uncertainty of not being able to control everything when it comes to the biology of lung disease and human organ transplant -- and hope that at least this time around I can earn a passing grade!
Like that Sunday 10 months ago when I was pulled from a nap by my darling sister's phone call and the simple phrase: "Get up and get dressed because I think I've found your future mate." Not always the most welcome words in the world when you're lying there with smeared mascara and bedhead, but as it turns out, 30 minutes later I was standing before a 4-month-old Sampson, who was deftly attempting to demonstrate his unbelievable cuteness by chewing on my sister's fingers. Okay, fine, so maybe biting isn't a preferred human method of flirting (although maybe it should be?) but it definitely worked on me -- hook, line, and life-changing sinker. And while I never expected to bring home a puppy that afternoon (and in fact had vowed never to have anything resembling a shih-tzu after a rather traumatizing run in with my childhood nanny's blind, deaf, and very snarky "puppy"), I couldn't be happier with the long-term results. No planning, no advance warning, and possibly the best outcome ever.
That moment aside, though, I've learned that there are certain experiences in life that DO require preparation. I'll be the first to admit that I've sometimes had to learn this lesson the hard way -- such as the time I failed to prepare at all for a science test in 9th grade and became quite possibly the first cystic in history to get a nearly failing grade on questions about the "biology of human organs" (oh, the irony!). Or that decision I made to wait until the very last night of the very last day of my college experience to even try and pack up my rather large bedroom and everything else I'd accumulated over two years of living with the best housemates ever. Good housemates, yes. Useful assistant packers in a crises, not so much. In other words, I've had a few examples of poor planning in my lifetime, and I hope I've learned from them.
And then along came transplant.
Okay, I am a lot of things, but (lousy grade on 9th grade Bio test notwithstanding), I am generally NOT stupid. So it's not as though I was expecting transplant to be something I could waltz right into with zero prep work or planning and wake up 8-12 hours later with new lungs, new breath, and everything totally organized to start my new life. And any delusions of simplicity I actually might have had in the beginning were quickly (and rightfully) shattered by my wonderful but very straight-talking transplant team. I was told I would need to attend educational seminars, schedule and complete all "routine maintenance" health appointments (eye doctors, dentists, etc) and immunizations AND receive from each doctor a letter of clearance, organize my support team and living situation to be ready for post-transplant living, and of course work on gaining weight and maintaining my exercise routine to be as healthy as possible for the actual surgery. This, of course, in addition to the three full days of outpatient testing I completed in August 2008, many of which I have subsequently repeated just so that everything is as up to date as possible.
And when you think about it, that's a lot of advance planning for a surgery that you can't really plan AROUND at all. In other words, I can't plan when the call will come. I can't plan which of the three amazing surgeons at my center will be on-call the day/night I get lungs. I can't plan to be back on my feet or back at work on any specific date. I can't tell my housekeeper the dates when she'll have to take Sampson in advance, and in fact I can't even tell her how long she'll have to keep my beautiful boy, since I have no way to plan how long I'll be in the hospital. It's as though the lack of ability to plan has mysteriously morphed into a compulsive need to double-plan just so everyone's as prepared for unplanned chaos as possible.
Um, yeah. It's really, really fun and exciting over at my house right now, let me tell you. Fun, exciting, and relaxing.
But okay, fine, I can live with a little chaos. Just not too much, which means that, having finished the first list of "planning to-dos" (pause for a major pat on the back here) given to me by my transplant team, I'm moving on to my own personal list. And I'm suddenly discovering that there's still a LOT to be done. Over the past week, in the frenzy of being listed, I've had to come up with ways to notify old friends -- some of whom I'm not really in touch with much anymore but know would want to be updated about something this major -- about the transplant, put together a ready-made listserve of email address for people (family, close friends, etc) who want to be notified of things as they happen once the call comes, and set up a place (probably a website of sorts) for people who just want the more occasional updates to be able to find and share information during the surgery and recovery. I've also had to consider what I want to bring with me to the hospital (for my stay that could be anywhere from 8 days to, well, as long as it takes), and what my family might want/need in the waiting room. I need to plan how I can best stay active and engaged and social while on the list without wearing myself out and over extending, and I have to make sure everyone knows that I might have to drop these commitments at the drop of a hat to go get the transplant. I've most recently moved on to planning out tough conversations, including finishing up my advance directive and other things that, as a lawyer, I just can't not think about. And finally I'm working on a personal goal of being able to walk 2.5 miles at 4 MPH without stopping (or dying) at the time I get called for surgery. Because a wise friend of mine once said "if you walk into a transplant, you'll walk out of it." And I figure she ought to know, since she walked upright into the O.R. before her double-lung and liver transplant. Yowza.
I gotta admit, I kinda wish finding new lungs could be a little more like finding my dog. I mean, how awesome would it be to be peacefully dozing on a fall weekend and have my sister call up with an adorable (and feisty!) pair of perfect new lungs for me? But I have a strange feeling that's not how it's gonna work out this time -- and if I do get that call, I think I might have to commit my wonderful sister, so that wouldn't be good either.
But if I'm really truly honest -- and when am I not? -- I have to admit that there's a part of this process that is actually kind of fun and exciting (in a very "man, I wish I didn't have to do this" kind of way). I'm learning a lot about myself and my family, I'm reconnecting with some really old friends, I'm getting to see first hand just how wonderful all these people in my life who have come out of the wordwork -- in fact, sometimes out of the pretty distant past -- to support me truly are, and I'm pushing myself in ways I didn't even know I could be pushed.
In other words, I think I'll have to wait, and plan, and deal with the uncertainty of not being able to control everything when it comes to the biology of lung disease and human organ transplant -- and hope that at least this time around I can earn a passing grade!
Doctors, Doctors Everywhere
. . . and not a drop to drink?
Well, actually, scratch that, because I think I might actually need a drink after the fiasco I just endured over on Madison Avenue. And to be totally honest, you might want to grab a drink too, awesome readers, because this is a heartbreaking saga of sheer ridiculousness -- you know, the kind of ridiculous that only I can seem to really pull off for some reason. And, like all truly epic stories, it begins in a dentist's office.
Okay, not really, because it truly began in midtown Manhattan traffic, which is, I assure you, probably the only place on earth I would LESS like to be than in the dentist's office. And this particular cab ride up Madison Avenue was made all the more pleasant by the 90-some-odd degree, incredibly humid day outside our dirty and grafitti-laden cab windows. And yet, I felt oddly calm, like a woman with purpose, a woman on a mission . . . in other words, like a woman who is listed for double-lung transplant and needs a root canal.
Yeah, that kind of woman.
As a root-canal virgin, I wasn't entirely sure what to expect, so I brought along my mother for moral support. This was frustrating for both of us, because I've been really trying to attend more of my transplant center's "lung group" educational/support meetings lately, and unfortunately the only time available for the crucial root canal also happened to overlap with a particularly interesting looking group meeting on post-transplant meds. I had hoped to at least send my mother up to take notes, but kinda chickened out at the last minute and decided I needed/wanted her to be with me (you know, in the waiting room doing nothing, where she'd be most useful in my warped perspective). So here we both were, in the cab, on our way to what we knew wouldn't be the most pleasant of experiences, and silently kicking ourselves for missing something educational and informative up at the hospital. Oh yeah, AND we were hot, did I mention that? Because it was really, really hot.
Anyway, we arrived at the dentist and I gave my name and quickly got called back to go sit in the dreaded root-canal chair, which I'm convinced is somewhat on display so you can serve as a cautionary tale to other patrons and especially young children ("look, little Timmy, see that woman over there squirming in pain and trying not to cough? That's what happens when you don't floss every night!"). So I sit. And I sit. And I wait. I sit. I wait. I look around at all the posters of radiant smiles and wonder how they really get their teeth that white in just two simple treatments. And I wait.
Finally a nice young dental hygienist comes in and says that, due to my medical record and the drugs I'm on (admittedly a scary number of antibiotics for anyone whose calling card doesn't read "professional drug tester"), the dentist cannot treat me without prior medical clearance. Which, of course, had never been mentioned to me before because, after all, I'm just the patient and the only link between my doctors and my dentist. So you know, why bother telling me this?
In other words, the situation as it was explained to me is pretty simple: Because I'm listed for transplant, I need prior MEDICAL clearance in order to get a dental procedure that I need to get in order to get DENTAL clearance for the transplant listing.
Yep, makes sense to me.
It made even more sense when I learned that the medical clearance has to come from my prescribing doctor (i.e., my CF doctor - NOT my transplant team) and then the dental clearance needs to go to the transplant team (NOT my CF doctor). So doctor A must give clearance to dentist B to work on my teeth so that he can give further clearance to doctor C, so that doctor C's partner, surgeon D, can eventually remove my old, nasty CF lungs (cared for by doctor A) and replace them with new, pretty, healthy lungs to be cared for exclusively by doctor C.
Okay, fine. I get it, huge hospital medical bureaucracy, you're just trying to prove a point. Show off a little. Well, bring it, because I was not to be defeated. Instead I promptly insisted that despite my inability to get a root canal (and I resisted calling the dentist a "wimp" for being too afraid to work on a CFer), I wanted to see the dentist anyway and get him to give me a full mouth exam. In other words, confirm that the root canal is in fact all that needs to be done. And once he had done that, I gave his lovely hygienist the number for my transplant coordinator and instructed her to tell them that I had been fully examined and that I had rescheduled my root canal for the following week because of a need for medical clearance. Finally, I called my transplant team myself to confirm that this information from my dentist would be enough to serve as "dental clearance." Thankfully, it was.
So in other words, I need medical clearance sometime before next week and then I can get my tooth fixed and life will go on as normal. My transplant team, in the meantime, is satisfied, and I feel like I actually turned a bad situation around at least a little to get a somewhat okay result. As for the medical clearance, in all fairness this wasn't something that was on my CF doctor's radar at all, since I never told her I was going to the dentist (although I did tell the transplant team, multiple times). And since I hadn't been told at all by anyone that I needed such a clearance, I guess I really can't blame any one particular doctor for this mess either. To be honest, everyone was really nice about it all, if a little confusing.
The saddest part of the tale is that I still missed my transplant meeting, but I guess it was worth it just for the minor victory of getting the dental clearance through to my coordinator. Then again, on the bright side, now I have a whole new week to be excited about the root canal all over again.
Thank goodness for happy endings, right?
Well, actually, scratch that, because I think I might actually need a drink after the fiasco I just endured over on Madison Avenue. And to be totally honest, you might want to grab a drink too, awesome readers, because this is a heartbreaking saga of sheer ridiculousness -- you know, the kind of ridiculous that only I can seem to really pull off for some reason. And, like all truly epic stories, it begins in a dentist's office.
Okay, not really, because it truly began in midtown Manhattan traffic, which is, I assure you, probably the only place on earth I would LESS like to be than in the dentist's office. And this particular cab ride up Madison Avenue was made all the more pleasant by the 90-some-odd degree, incredibly humid day outside our dirty and grafitti-laden cab windows. And yet, I felt oddly calm, like a woman with purpose, a woman on a mission . . . in other words, like a woman who is listed for double-lung transplant and needs a root canal.
Yeah, that kind of woman.
As a root-canal virgin, I wasn't entirely sure what to expect, so I brought along my mother for moral support. This was frustrating for both of us, because I've been really trying to attend more of my transplant center's "lung group" educational/support meetings lately, and unfortunately the only time available for the crucial root canal also happened to overlap with a particularly interesting looking group meeting on post-transplant meds. I had hoped to at least send my mother up to take notes, but kinda chickened out at the last minute and decided I needed/wanted her to be with me (you know, in the waiting room doing nothing, where she'd be most useful in my warped perspective). So here we both were, in the cab, on our way to what we knew wouldn't be the most pleasant of experiences, and silently kicking ourselves for missing something educational and informative up at the hospital. Oh yeah, AND we were hot, did I mention that? Because it was really, really hot.
Anyway, we arrived at the dentist and I gave my name and quickly got called back to go sit in the dreaded root-canal chair, which I'm convinced is somewhat on display so you can serve as a cautionary tale to other patrons and especially young children ("look, little Timmy, see that woman over there squirming in pain and trying not to cough? That's what happens when you don't floss every night!"). So I sit. And I sit. And I wait. I sit. I wait. I look around at all the posters of radiant smiles and wonder how they really get their teeth that white in just two simple treatments. And I wait.
Finally a nice young dental hygienist comes in and says that, due to my medical record and the drugs I'm on (admittedly a scary number of antibiotics for anyone whose calling card doesn't read "professional drug tester"), the dentist cannot treat me without prior medical clearance. Which, of course, had never been mentioned to me before because, after all, I'm just the patient and the only link between my doctors and my dentist. So you know, why bother telling me this?
In other words, the situation as it was explained to me is pretty simple: Because I'm listed for transplant, I need prior MEDICAL clearance in order to get a dental procedure that I need to get in order to get DENTAL clearance for the transplant listing.
Yep, makes sense to me.
It made even more sense when I learned that the medical clearance has to come from my prescribing doctor (i.e., my CF doctor - NOT my transplant team) and then the dental clearance needs to go to the transplant team (NOT my CF doctor). So doctor A must give clearance to dentist B to work on my teeth so that he can give further clearance to doctor C, so that doctor C's partner, surgeon D, can eventually remove my old, nasty CF lungs (cared for by doctor A) and replace them with new, pretty, healthy lungs to be cared for exclusively by doctor C.
Okay, fine. I get it, huge hospital medical bureaucracy, you're just trying to prove a point. Show off a little. Well, bring it, because I was not to be defeated. Instead I promptly insisted that despite my inability to get a root canal (and I resisted calling the dentist a "wimp" for being too afraid to work on a CFer), I wanted to see the dentist anyway and get him to give me a full mouth exam. In other words, confirm that the root canal is in fact all that needs to be done. And once he had done that, I gave his lovely hygienist the number for my transplant coordinator and instructed her to tell them that I had been fully examined and that I had rescheduled my root canal for the following week because of a need for medical clearance. Finally, I called my transplant team myself to confirm that this information from my dentist would be enough to serve as "dental clearance." Thankfully, it was.
So in other words, I need medical clearance sometime before next week and then I can get my tooth fixed and life will go on as normal. My transplant team, in the meantime, is satisfied, and I feel like I actually turned a bad situation around at least a little to get a somewhat okay result. As for the medical clearance, in all fairness this wasn't something that was on my CF doctor's radar at all, since I never told her I was going to the dentist (although I did tell the transplant team, multiple times). And since I hadn't been told at all by anyone that I needed such a clearance, I guess I really can't blame any one particular doctor for this mess either. To be honest, everyone was really nice about it all, if a little confusing.
The saddest part of the tale is that I still missed my transplant meeting, but I guess it was worth it just for the minor victory of getting the dental clearance through to my coordinator. Then again, on the bright side, now I have a whole new week to be excited about the root canal all over again.
Thank goodness for happy endings, right?
Tuesday, August 18, 2009
Long Overdue and Not Nearly Enough
Okay, so I think it's time we here at A Matter of Life and Breath took a second to recognize all those crazy, wonderful, brave, funny, smart, interesting, and, um, well, frankly amazing people who make our lives as cystics possible.
In other words, this post is going to be about my family.
I feel like I need to do this for a couple of reasons, the first being that my mom is currently living with me in NYC (more on that later) and just really deserves a shout out. The second reason is simpler: although my family does not, at least for the most part and to the best of my knowledge, read this blog, they are a HUGE part of who I am and where I come from. So I feel like I can't explain much more about my life and my CF and this whole crazy transplant journey thing without explaining who they are, too.
Yeah, I know, when most people think "lawyer" they probably have visions of men in pinstriped suits springing fully formed out of a crack in the middle of Wall St. (because surely if there's a gateway to the underworld it must be somewhere in lower Manhattan), but surprisingly there's not a lot of truth to that rumor. Not in my case anyway. I actually sprung fairly under-formed (or, at the very least, underweight) from my nest on the day after Thanksgiving, and into the arms of a very cool group of individuals consisting of my mom, my dad, and my older sister, not to mention a rather snarky English Sheepdog named Amanda. I was born, it was love at first sight -- from my perspective anyway, although the look on my sister's face when my dad snapped a picture of her "meeting" me for the first time tells a slightly different story -- and 6 weeks later we suddenly all had new identities.
We became a CF family.
I always think about the story my parents tell about my diagnosis. I was in the hospital, sick with pneumonia, meningitis, and failure to thrive and my parents were, understandably, scared out of their minds. They were sitting in my room feeling tired and helpless when a little girl pushed her IV pole up to the door and started chatting with them. My parents think she was about five years old or so, and the main thing they remember about her, besides her bright smile and the way she cheered them up with her visit, is that she told them she had a disease called cystic fibrosis. So my parents, being diligent nerds (ahem, I mean scholars -- but at least I come by it honest!), immediately went home that night and looked up CF in a medical dictionary. And then my father wrapped his arms around my mom and said to her, "well, at least our daughter doesn't have to go through something like THAT." We got our diagnosis the very next day.
It's crazy to me to think that from that conversation and the bombshell that was dropped on them the next day my parents evolved into full-scale CF warlords. I'm not kidding. Those two are fighting this disease and they are out for BLOOD, and after 28 years of it they don't show any signs of slowing down. If I can brag for a second, my parents have organized fundraisers, sat on boards of directors, advocated in politics, and won a well-deserved Breath of Life award in Colorado a couple of years back for all their CF dedication. Which is all pretty impressive, obviously, but it pales in comparison to the awards they should have received for putting up with the world's most stubborn CF child. Because, no joke, CF has been a battle for me since day 1 as well, but for me it was always about being "not sick" and avoiding at all costs anything that made me look, feel, or act "sick" compared to my peers. So while I was perfectly willing to push myself to excel in pretty much any other activity, let's just say treatment time was never my finest hour. I don't remember anymore whether my parents had to forcibly restrain me to sit with my nebs or tie me down to our CPT table, but I do know that they kept me healthy throughout my childhood and well into my teens even up against some very challenging obstacles. And when they do finally institute the Nobel Prize for hand-to-hand combat in CPT, I'm pretty sure my parents are going to be on the short list, for which I can now (finally) say without any irony that I am COMPLETELY grateful.
As for my sister, I have no idea what it's like to grow up with a "sick" little sister, but I'm guessing her perception of it is that it's not too different from growing up with a not-so-sick sister, because she always faithfully stuck by her conviction that I was not to be treated any differently from any other younger sibling/crash test dummy/scapegoat/servant. (On a sidenote, my sister is the one person who DOES occasionally read this blog, and I threw in that last bit entirely out of love . . . and honesty . . . but mostly love.) She's basically as stubborn as I am but tends to use it in more productive ways, and her main message to me growing up was that if I could look to her for guidance but still act like me (and not, for example, try to steal her clothes or CDs at every chance), I'd be doing something right. As usual, she was pretty dead on with that point.
So after 28 years of ups and downs and misplaced PICC lines and exciting new drug developments (and some drug letdowns, let's be honest) and fundraising and enzymes in applesauce and transitions from cpt to percussor to autogenic to flutter to vest and back to flutter and to IPV and back to vest (with some occasional CPT and IPV thrown in for good measure); after one extremely dramatic port placement during which I insisted that there was no way that thing was going anywhere NEAR my chest (I totally won that battle) and one clinic that just didn't work out and four scary years of trusting me away at college and countless home IVs and multiple hospitalizations and one epic fail of a right-heart cath; after sleepless nights and coughing fits and tears and really really uncomfortable hospital beds; and after promising me that I could take on the world if I wanted to, that CF was no reason to stop living or dreaming, that college and all my dreams after it were totally within reach, and that they would always be behind me no matter what . . . my family is still here, not so much behind me as beside me.
My mom is living in NY to help me through transplant. My sister holds a full time job as an incredible fashion designer for a successful new company and still manages to make it over to my apt several times a week for Top Chef or dinner, and she recently nursed me through some fevers that were quite frankly not for the faint of heart. My dad (another lawyer, just in case you thought that CF is the only genetic defect that runs in our family) is still holding down the fort in Denver, but rearranging his schedule so that he can be here as often as possible. Even my godmother has flown in from Colorado to help me through some of this pre-transplant/infection bonanza madness. And while that sometimes makes for a very crowded apartment, it also makes for a lot of peace of mind. Between my amazing family and my indescribable friends, I was once told by a member of the Columbia team that I had "the best support system" she had ever seen, and I didn't know what to say aside from "thank you."
So while they may never read this, with the possible exception of Erin, I guess it's time to extend that compliment to the people who really deserve it. I know that my diagnosis has always been and will always be, just like the genes that caused it, shared by my entire family, and I could never express how lucky I feel to have that help in shouldering the load.
In other words, this post is going to be about my family.
I feel like I need to do this for a couple of reasons, the first being that my mom is currently living with me in NYC (more on that later) and just really deserves a shout out. The second reason is simpler: although my family does not, at least for the most part and to the best of my knowledge, read this blog, they are a HUGE part of who I am and where I come from. So I feel like I can't explain much more about my life and my CF and this whole crazy transplant journey thing without explaining who they are, too.
Yeah, I know, when most people think "lawyer" they probably have visions of men in pinstriped suits springing fully formed out of a crack in the middle of Wall St. (because surely if there's a gateway to the underworld it must be somewhere in lower Manhattan), but surprisingly there's not a lot of truth to that rumor. Not in my case anyway. I actually sprung fairly under-formed (or, at the very least, underweight) from my nest on the day after Thanksgiving, and into the arms of a very cool group of individuals consisting of my mom, my dad, and my older sister, not to mention a rather snarky English Sheepdog named Amanda. I was born, it was love at first sight -- from my perspective anyway, although the look on my sister's face when my dad snapped a picture of her "meeting" me for the first time tells a slightly different story -- and 6 weeks later we suddenly all had new identities.
We became a CF family.
I always think about the story my parents tell about my diagnosis. I was in the hospital, sick with pneumonia, meningitis, and failure to thrive and my parents were, understandably, scared out of their minds. They were sitting in my room feeling tired and helpless when a little girl pushed her IV pole up to the door and started chatting with them. My parents think she was about five years old or so, and the main thing they remember about her, besides her bright smile and the way she cheered them up with her visit, is that she told them she had a disease called cystic fibrosis. So my parents, being diligent nerds (ahem, I mean scholars -- but at least I come by it honest!), immediately went home that night and looked up CF in a medical dictionary. And then my father wrapped his arms around my mom and said to her, "well, at least our daughter doesn't have to go through something like THAT." We got our diagnosis the very next day.
It's crazy to me to think that from that conversation and the bombshell that was dropped on them the next day my parents evolved into full-scale CF warlords. I'm not kidding. Those two are fighting this disease and they are out for BLOOD, and after 28 years of it they don't show any signs of slowing down. If I can brag for a second, my parents have organized fundraisers, sat on boards of directors, advocated in politics, and won a well-deserved Breath of Life award in Colorado a couple of years back for all their CF dedication. Which is all pretty impressive, obviously, but it pales in comparison to the awards they should have received for putting up with the world's most stubborn CF child. Because, no joke, CF has been a battle for me since day 1 as well, but for me it was always about being "not sick" and avoiding at all costs anything that made me look, feel, or act "sick" compared to my peers. So while I was perfectly willing to push myself to excel in pretty much any other activity, let's just say treatment time was never my finest hour. I don't remember anymore whether my parents had to forcibly restrain me to sit with my nebs or tie me down to our CPT table, but I do know that they kept me healthy throughout my childhood and well into my teens even up against some very challenging obstacles. And when they do finally institute the Nobel Prize for hand-to-hand combat in CPT, I'm pretty sure my parents are going to be on the short list, for which I can now (finally) say without any irony that I am COMPLETELY grateful.
As for my sister, I have no idea what it's like to grow up with a "sick" little sister, but I'm guessing her perception of it is that it's not too different from growing up with a not-so-sick sister, because she always faithfully stuck by her conviction that I was not to be treated any differently from any other younger sibling/crash test dummy/scapegoat/servant. (On a sidenote, my sister is the one person who DOES occasionally read this blog, and I threw in that last bit entirely out of love . . . and honesty . . . but mostly love.) She's basically as stubborn as I am but tends to use it in more productive ways, and her main message to me growing up was that if I could look to her for guidance but still act like me (and not, for example, try to steal her clothes or CDs at every chance), I'd be doing something right. As usual, she was pretty dead on with that point.
So after 28 years of ups and downs and misplaced PICC lines and exciting new drug developments (and some drug letdowns, let's be honest) and fundraising and enzymes in applesauce and transitions from cpt to percussor to autogenic to flutter to vest and back to flutter and to IPV and back to vest (with some occasional CPT and IPV thrown in for good measure); after one extremely dramatic port placement during which I insisted that there was no way that thing was going anywhere NEAR my chest (I totally won that battle) and one clinic that just didn't work out and four scary years of trusting me away at college and countless home IVs and multiple hospitalizations and one epic fail of a right-heart cath; after sleepless nights and coughing fits and tears and really really uncomfortable hospital beds; and after promising me that I could take on the world if I wanted to, that CF was no reason to stop living or dreaming, that college and all my dreams after it were totally within reach, and that they would always be behind me no matter what . . . my family is still here, not so much behind me as beside me.
My mom is living in NY to help me through transplant. My sister holds a full time job as an incredible fashion designer for a successful new company and still manages to make it over to my apt several times a week for Top Chef or dinner, and she recently nursed me through some fevers that were quite frankly not for the faint of heart. My dad (another lawyer, just in case you thought that CF is the only genetic defect that runs in our family) is still holding down the fort in Denver, but rearranging his schedule so that he can be here as often as possible. Even my godmother has flown in from Colorado to help me through some of this pre-transplant/infection bonanza madness. And while that sometimes makes for a very crowded apartment, it also makes for a lot of peace of mind. Between my amazing family and my indescribable friends, I was once told by a member of the Columbia team that I had "the best support system" she had ever seen, and I didn't know what to say aside from "thank you."
So while they may never read this, with the possible exception of Erin, I guess it's time to extend that compliment to the people who really deserve it. I know that my diagnosis has always been and will always be, just like the genes that caused it, shared by my entire family, and I could never express how lucky I feel to have that help in shouldering the load.
Sunday, August 16, 2009
Rocket Man
My household currently consists of three inhabitants: me (obviously), my shih-tzu/yorkie mix (aka "shorkie") puppy Sampson, and my amazing mother, who has basically abandoned her life in CO (and my long-suffering father) to come live and stay with me in New York before, during, and immediately after the transplant. All this so that I didn't have to choose between my doctors here, who I love, and the option of relocating my life temporarily back to Denver in order to have full-time familial care post-transplant. Seriously, how lucky am I?
(And yes, for those of you wondering, my father gets out here as often as he can, and my sister lives here already, so we're quickly becoming a cross-country family. On the plus side, I am REALLY looking forward to some of the incredible trips we're going to be able to take post-transplant recovery using all those frequent flyer miles!)
Anyway, back to the point of this post, which is that while there are three inhabitants of my apartment right now, I am, to the best of my personal knowledge, the only one of those three who actually has cystic fibrosis. I can say this with relative certainty because my mother has actually had genetic testing (she's a carrier, obviously), and my puppy . . . well, let's just say he hasn't demonstrated any sort of poor growth issues yet. Seriously, let's face the facts: he's a 20 lbs shih-tzu/yorkie -- there's just NO WAY that dog is pancreatic insufficient. That, when coupled with his complete lack of a cough (I'll admit I'm discounting that time he swallowed an entire "turkey disk" dog treat whole and had to cough it back up -- twice. I just really don't think that counts as a "productive" cough by the CF definition) leads me to the conclusion that my puppy most likely does NOT have CF. And as a CF-free dog, he has no need for digestive enzymes, the vest, nebulizer cups, or Xopenex inhalers. Or at least, you know, that's my humble opinion anyway.
Yeah, try telling him that.
Not long after I got Sammy I noticed he had a slight obsession with my treatment time. I was frankly expecting the sight/sound of a mechanical vest and noisy little pari ultra compressor to freak out my little guy, who at that point had already demonstrated his complete aversion to vacuums, thunderstorms, and even the terrifying sound of my tiny New York style dishwasher. So imagine my surprise when, night one, this puppy of mine comes right up to my vibrating vest, sniffs around a bit, and immediately curls up right next to it, head resting on the "box" throughout my entire treatment. This developed into a routine that I found straight up adorable . . . until the day when it evolved from cute little puppy massage bed into a "let's chew the power cord" game. Seriously? Thankfully I caught the change early, and the problem was solved with a little bit of black electrical tape.
My dog's obsession with CF paraphernalia has, unfortunately, proved much harder to fix. In the past year, Sammy has -- despite my most diligent efforts -- chewed up three neb cups, one O2 "connector" for extension tubing, and (perhaps most disturbingly) an entire box of rubber gloves he found in the bag of a naively unsuspecting homecare nurse. Brilliant work, puppy of mine.
More recently I reported to some of my friends that I had pried open my dog's mouth to find inside . . . a Pancrease MT-20. Apparently, my puppy had gotten ahold of an entire bottle of enzymes, opened them, and found them to be at least enough to his liking that there were several dismantled enzymes and tiny balls scattered across the floor. The remaining, uneaten enzymes were unceremoniously deposited nearby, molded tightly into a little ball formed by dog spit and half-dissolved enzyme coating. It was, in a word, disgusting. (Another word might be "messy.") A quick call to the vet, however, revealed that they do in fact make doggy enzymes that are not so different from our own, and that the beloved shorkie would be just fine. And he was fine -- his stools were the pride of the dog run for several days due to their intense firmness and easy clean up -- except for his still uncured obsession.
Flash forward a couple of months to tonight. I'm relaxing in the living room, my mother has gone down to Whole Foods market to get us some yummy ingredients for dinner, and the evil (Um, I mean sweet and adorable) shorkie was, I believed, resting quietly in his bed. His bed, by the way, also happens to be in my room. So I'm happily reading my book, minding my own non-trouble-making business, when suddenly I hear what sounds like a rocket ship taking off from my room. Granted, there was no doubt that this must have been a small rocket ship, but I'm not kidding when I say there was at least 30 seconds worth of ignition, turbo charge, and blast off. And, seeing as how there are NO rocket ships of any size in my room (or elsewhere in my apartment, for that matter) and the only things I have that even might RESEMBLE rocket ships involve tanks filled with highly combustible gas, I was probably justified in being a little freaked out.
My fears were magnified by what can only be described as the fastest shorkie on the face of the planet making a direct beeline OUT of my room, where he promptly took a flying leap onto my lap. Let me just say, having a terrified puppy come storming out of your room, from which NASA appears to be running mini-scale flight tests, is really just NOT a comforting sign. True, it's better than having an injured puppy, or a puppy riding aboard an out-of-control oxygen tank (both of these seemed like viable alternatives in the moment), but it's still just. not. good.
So after a perplexing visit into my apparently still-intact and pretty much untouched room, I decided to let the only eyewitness show me exactly what happened. I coaxed the shivering Sammy back into the room, only to notice that he immediately: 1) sniffed the air (which even I could tell had an odd smell to it), and 2) ran quickly to the edge of the bed, peered under it, and then beat a hasty retreat back behind my legs, as any good little mama's boy should. So I looked under the bed, and what did I find?
A mini rocket ship.
Sort of.
Well, okay, not at all. But I get how it happened. Want a hint? Because apparently pressurized inhalers can, when properly punctured, actually travel a pretty good distance. At least to halfway under a queen-sized bed. And let me (and Sammy) tell you, they sound darn impressive while doing it.
Exhibit 1: My Xopenex inhaler
Exhibit 2: Extreme close up of the puncture hole now in the inhaler, shaped (suspiciously enough) much like a shorkie tooth mark.
So okay, my dog might not have CF. And he might have a very strange (and seriously uncool) sense of what makes for a good chew toy. But he just might have a future career in NASA, and how many puppies can say that?
(And yes, for those of you wondering, my father gets out here as often as he can, and my sister lives here already, so we're quickly becoming a cross-country family. On the plus side, I am REALLY looking forward to some of the incredible trips we're going to be able to take post-transplant recovery using all those frequent flyer miles!)
Anyway, back to the point of this post, which is that while there are three inhabitants of my apartment right now, I am, to the best of my personal knowledge, the only one of those three who actually has cystic fibrosis. I can say this with relative certainty because my mother has actually had genetic testing (she's a carrier, obviously), and my puppy . . . well, let's just say he hasn't demonstrated any sort of poor growth issues yet. Seriously, let's face the facts: he's a 20 lbs shih-tzu/yorkie -- there's just NO WAY that dog is pancreatic insufficient. That, when coupled with his complete lack of a cough (I'll admit I'm discounting that time he swallowed an entire "turkey disk" dog treat whole and had to cough it back up -- twice. I just really don't think that counts as a "productive" cough by the CF definition) leads me to the conclusion that my puppy most likely does NOT have CF. And as a CF-free dog, he has no need for digestive enzymes, the vest, nebulizer cups, or Xopenex inhalers. Or at least, you know, that's my humble opinion anyway.
Yeah, try telling him that.
Not long after I got Sammy I noticed he had a slight obsession with my treatment time. I was frankly expecting the sight/sound of a mechanical vest and noisy little pari ultra compressor to freak out my little guy, who at that point had already demonstrated his complete aversion to vacuums, thunderstorms, and even the terrifying sound of my tiny New York style dishwasher. So imagine my surprise when, night one, this puppy of mine comes right up to my vibrating vest, sniffs around a bit, and immediately curls up right next to it, head resting on the "box" throughout my entire treatment. This developed into a routine that I found straight up adorable . . . until the day when it evolved from cute little puppy massage bed into a "let's chew the power cord" game. Seriously? Thankfully I caught the change early, and the problem was solved with a little bit of black electrical tape.
My dog's obsession with CF paraphernalia has, unfortunately, proved much harder to fix. In the past year, Sammy has -- despite my most diligent efforts -- chewed up three neb cups, one O2 "connector" for extension tubing, and (perhaps most disturbingly) an entire box of rubber gloves he found in the bag of a naively unsuspecting homecare nurse. Brilliant work, puppy of mine.
More recently I reported to some of my friends that I had pried open my dog's mouth to find inside . . . a Pancrease MT-20. Apparently, my puppy had gotten ahold of an entire bottle of enzymes, opened them, and found them to be at least enough to his liking that there were several dismantled enzymes and tiny balls scattered across the floor. The remaining, uneaten enzymes were unceremoniously deposited nearby, molded tightly into a little ball formed by dog spit and half-dissolved enzyme coating. It was, in a word, disgusting. (Another word might be "messy.") A quick call to the vet, however, revealed that they do in fact make doggy enzymes that are not so different from our own, and that the beloved shorkie would be just fine. And he was fine -- his stools were the pride of the dog run for several days due to their intense firmness and easy clean up -- except for his still uncured obsession.
Flash forward a couple of months to tonight. I'm relaxing in the living room, my mother has gone down to Whole Foods market to get us some yummy ingredients for dinner, and the evil (Um, I mean sweet and adorable) shorkie was, I believed, resting quietly in his bed. His bed, by the way, also happens to be in my room. So I'm happily reading my book, minding my own non-trouble-making business, when suddenly I hear what sounds like a rocket ship taking off from my room. Granted, there was no doubt that this must have been a small rocket ship, but I'm not kidding when I say there was at least 30 seconds worth of ignition, turbo charge, and blast off. And, seeing as how there are NO rocket ships of any size in my room (or elsewhere in my apartment, for that matter) and the only things I have that even might RESEMBLE rocket ships involve tanks filled with highly combustible gas, I was probably justified in being a little freaked out.
My fears were magnified by what can only be described as the fastest shorkie on the face of the planet making a direct beeline OUT of my room, where he promptly took a flying leap onto my lap. Let me just say, having a terrified puppy come storming out of your room, from which NASA appears to be running mini-scale flight tests, is really just NOT a comforting sign. True, it's better than having an injured puppy, or a puppy riding aboard an out-of-control oxygen tank (both of these seemed like viable alternatives in the moment), but it's still just. not. good.
So after a perplexing visit into my apparently still-intact and pretty much untouched room, I decided to let the only eyewitness show me exactly what happened. I coaxed the shivering Sammy back into the room, only to notice that he immediately: 1) sniffed the air (which even I could tell had an odd smell to it), and 2) ran quickly to the edge of the bed, peered under it, and then beat a hasty retreat back behind my legs, as any good little mama's boy should. So I looked under the bed, and what did I find?
A mini rocket ship.
Sort of.
Well, okay, not at all. But I get how it happened. Want a hint? Because apparently pressurized inhalers can, when properly punctured, actually travel a pretty good distance. At least to halfway under a queen-sized bed. And let me (and Sammy) tell you, they sound darn impressive while doing it.
Exhibit 1: My Xopenex inhaler
Exhibit 2: Extreme close up of the puncture hole now in the inhaler, shaped (suspiciously enough) much like a shorkie tooth mark.
So okay, my dog might not have CF. And he might have a very strange (and seriously uncool) sense of what makes for a good chew toy. But he just might have a future career in NASA, and how many puppies can say that?
Saturday, August 15, 2009
Call me Crazy
Powerful drug,
Regretfully necessary. An
"Evil little miracle,"
Drowning sanity (and sleep) as you wash away inflammation.
No, I don't like you;
I both love you and I hate you. And I'm
Sorry for the indecision, it's
Only that I'm feeling a little flustered, mixed-up,
Not quite myself, which might have something to do with you, or
Everything.
(PS: On a more personal note, thanks so much for all the fantastic comments to my last post. You guys are so amazing!)
Regretfully necessary. An
"Evil little miracle,"
Drowning sanity (and sleep) as you wash away inflammation.
No, I don't like you;
I both love you and I hate you. And I'm
Sorry for the indecision, it's
Only that I'm feeling a little flustered, mixed-up,
Not quite myself, which might have something to do with you, or
Everything.
(PS: On a more personal note, thanks so much for all the fantastic comments to my last post. You guys are so amazing!)
Thursday, August 13, 2009
Notice of Separation
Dear Cystic Fibrosis:
It's hard to believe that it was just a little over 28 years ago when you and I took our vows together. Of course, I'd be lying if I said I remember any part of the ceremony, but I imagine I looked small and beautiful (if a little sick and underweight) in my infant hospital gown. You, I'm sure, looked much the same as ever, hidden as you were in my tiny little genes -- 28 years and you haven't changed a bit, though I wonder if that's much of a compliment for a couple of fixed mutations. Either way: you were there and I was there, and at 6 weeks old I took a vow to live with you as best as I could, through all your wild mood swings and reckless flare-ups, despite your unpredictable nature and frankly very bad temper (you could totally use some anger management classes, just fyi); for better or for worse, in sickness and in health.
I guess I didn't know at that time that you'd been courting me in secret for quite some time -- 9 months and 6 weeks to be exact about it all. We were young then, and even the experienced adults around us couldn't recognize the signs of our burgeoning relationship. Isn't that always how it goes though? You don't know how deep you're in it until your hands and feet are stuck in the tar and no amount of prying or tearing or punching will tear you loose -- you're just stuck with that tar baby and that's the end of it. And then I guess we're all just left to do the only thing we ever know HOW to do, which is to make a life out of it all.
And live we did! Hey, CF, we had some good times, right? More good than bad, actually, when you care to tally it all up. I mean sure, you tend to get all worked up for no apparent reason sometimes, and you do have a way of trying to make our lives all about you (like that little attention-hogging infection back in 2007? Um, yeah, SO uncalled for!), but by and large you and I have made a life together that far exceeded anyone's expectations. We've traveled, gone to school, pursued random dreams, had relationships, lost relationships, fought battles, and done pretty much everything we've set out to do over the years, to be perfectly honest. I wouldn't say you were always my favorite companion on that road, but I have to give credit where credit is due: you definitely don't have any problem with commitment.
Nope, CF, I don't hate you, honestly. I need to say that, and I'm willing to put it in writing. Granted, you might have trouble believing me, and probably for good reason. I admit I wouldn't be too hot to trust someone who spent hours plotting ways to raise money for a foundation working to wipe me off the face of the earth. It probably doesn't leave you with the best taste in your mouth, but then again, you're directly responsible for all that goo I coughed up this morning, and that didn't taste too good either. And anyway it's all true: I don't hate you, because you've pushed me to be more than I probably could have been without you, because you've brought my family closer together and forced us to realize what we mean to each other, because you've given me SUCH cool slang language (cystic, cyster, fibro), because you gave me countless funny hospital stories (no, I do not have fibrocystic disease of the breasts, thanks very much) and taught me more medical words than most residents know, because you've made me part of a fabulous community of people, and because you are, for all your flaws, a part of me now. You are literally all I know, and for that I find it difficult to let go.
Let go. Um yeah, kind of. See, the point is, CF, that while you haven't changed too much over these past 28 years, I have. I've lived and I've laughed and I've coughed and I've read some really good novels (and, let's face it, a whole lot of NOT so good novels) and, well, I've come to a kind of startling conclusion: I would be better off without you.
No, wait, before you freak out, I don't mean ALL of you (although if I could have that option I have to say I think I'd take it), but I do mean the biggest part of you. The part that has taken up residence in my lungs and insists on throwing raucous parties every few weeks or so, despite the fact that I keep telling you it's just not compatible with my lifestyle. Yep, that part. I'm sorry, but it, and you, has just got to go.
So I'm making plans to get a separation, and I hope it doesn't come as too much of a shock that I'm pretty far along in the process. I'd offer to find you good representation, but to be honest the judgment has already come down. You're out, I'm in, and hopefully some new lungs will help me move on with my life in a way I never could when you were with me. I'm sorry (sort of), but this is just the way things have to be.
If it makes you feel any better, I know I'll always bear with me the scars of our relationship: of all the times we fought and struggled and everything you taught me about myself. Physical scars, sure, but more than that also. And of course I know I won't be rid of you completely (though you can be sure I'm not going to give up trying to knock you out altogether -- no hard feelings, surely), but I'm hopeful that the part of you that remains will be less cumbersome and attention-hungry, and frankly just less annoying.
I know things might not be perfect when you're (sort of) gone. I accept that, and I'm okay with it. Right now, all I know is it's time for you and me to (kind of) part ways. So here's my official notice -- consider yourself served:
Listed for Transplant
NY Columbia Presbyterian Medical Center
August, 2009
Yours always (or at least until something better comes along),
Piper
It's hard to believe that it was just a little over 28 years ago when you and I took our vows together. Of course, I'd be lying if I said I remember any part of the ceremony, but I imagine I looked small and beautiful (if a little sick and underweight) in my infant hospital gown. You, I'm sure, looked much the same as ever, hidden as you were in my tiny little genes -- 28 years and you haven't changed a bit, though I wonder if that's much of a compliment for a couple of fixed mutations. Either way: you were there and I was there, and at 6 weeks old I took a vow to live with you as best as I could, through all your wild mood swings and reckless flare-ups, despite your unpredictable nature and frankly very bad temper (you could totally use some anger management classes, just fyi); for better or for worse, in sickness and in health.
I guess I didn't know at that time that you'd been courting me in secret for quite some time -- 9 months and 6 weeks to be exact about it all. We were young then, and even the experienced adults around us couldn't recognize the signs of our burgeoning relationship. Isn't that always how it goes though? You don't know how deep you're in it until your hands and feet are stuck in the tar and no amount of prying or tearing or punching will tear you loose -- you're just stuck with that tar baby and that's the end of it. And then I guess we're all just left to do the only thing we ever know HOW to do, which is to make a life out of it all.
And live we did! Hey, CF, we had some good times, right? More good than bad, actually, when you care to tally it all up. I mean sure, you tend to get all worked up for no apparent reason sometimes, and you do have a way of trying to make our lives all about you (like that little attention-hogging infection back in 2007? Um, yeah, SO uncalled for!), but by and large you and I have made a life together that far exceeded anyone's expectations. We've traveled, gone to school, pursued random dreams, had relationships, lost relationships, fought battles, and done pretty much everything we've set out to do over the years, to be perfectly honest. I wouldn't say you were always my favorite companion on that road, but I have to give credit where credit is due: you definitely don't have any problem with commitment.
Nope, CF, I don't hate you, honestly. I need to say that, and I'm willing to put it in writing. Granted, you might have trouble believing me, and probably for good reason. I admit I wouldn't be too hot to trust someone who spent hours plotting ways to raise money for a foundation working to wipe me off the face of the earth. It probably doesn't leave you with the best taste in your mouth, but then again, you're directly responsible for all that goo I coughed up this morning, and that didn't taste too good either. And anyway it's all true: I don't hate you, because you've pushed me to be more than I probably could have been without you, because you've brought my family closer together and forced us to realize what we mean to each other, because you've given me SUCH cool slang language (cystic, cyster, fibro), because you gave me countless funny hospital stories (no, I do not have fibrocystic disease of the breasts, thanks very much) and taught me more medical words than most residents know, because you've made me part of a fabulous community of people, and because you are, for all your flaws, a part of me now. You are literally all I know, and for that I find it difficult to let go.
Let go. Um yeah, kind of. See, the point is, CF, that while you haven't changed too much over these past 28 years, I have. I've lived and I've laughed and I've coughed and I've read some really good novels (and, let's face it, a whole lot of NOT so good novels) and, well, I've come to a kind of startling conclusion: I would be better off without you.
No, wait, before you freak out, I don't mean ALL of you (although if I could have that option I have to say I think I'd take it), but I do mean the biggest part of you. The part that has taken up residence in my lungs and insists on throwing raucous parties every few weeks or so, despite the fact that I keep telling you it's just not compatible with my lifestyle. Yep, that part. I'm sorry, but it, and you, has just got to go.
So I'm making plans to get a separation, and I hope it doesn't come as too much of a shock that I'm pretty far along in the process. I'd offer to find you good representation, but to be honest the judgment has already come down. You're out, I'm in, and hopefully some new lungs will help me move on with my life in a way I never could when you were with me. I'm sorry (sort of), but this is just the way things have to be.
If it makes you feel any better, I know I'll always bear with me the scars of our relationship: of all the times we fought and struggled and everything you taught me about myself. Physical scars, sure, but more than that also. And of course I know I won't be rid of you completely (though you can be sure I'm not going to give up trying to knock you out altogether -- no hard feelings, surely), but I'm hopeful that the part of you that remains will be less cumbersome and attention-hungry, and frankly just less annoying.
I know things might not be perfect when you're (sort of) gone. I accept that, and I'm okay with it. Right now, all I know is it's time for you and me to (kind of) part ways. So here's my official notice -- consider yourself served:
Listed for Transplant
NY Columbia Presbyterian Medical Center
August, 2009
Yours always (or at least until something better comes along),
Piper
Friday, August 7, 2009
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.
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.
Wednesday, August 5, 2009
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.
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.
Monday, August 3, 2009
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.
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.
Sunday, August 2, 2009
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!)
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!)
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