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

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

Whoops, took that one a little too far.

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

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

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

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

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

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

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

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

Third Time's the Charm

Good news: the catheter is OUT!

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

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

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

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

Okay, so other news.

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

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

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

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

Great Strides #1

How many cystics does it take to walk two complete circles around the Philadelphia zoo (and actually LAP a few of their non-CF teammates in the process)?

Give up?

Well, apparently the answer to that all-important question is two, just so long as those two are as unbelievably awesome as Amy and me! (Okay, so maybe we're believably awesome, but still awesome nonetheless.)

In case you haven't already guessed, I took the train into PA this morning -- REALLY f-ing early in the morning, I should add -- to join Amy's "Blue Crew" Great Strides team in walking laps around the animals at what is I guess the nation's first zoo. It was actually my first time in Philadelphia at all, aside from passing through on the train between DC and NY, which I'm fairly certain doesn't qualify as a real visit even by fast-paced NYC standards. Basically I came away from it all with the following observations about the city of brotherly love:

1) It's really, really, REALLY hot

2) If the various team t-shirts for the walk are any indication, the people of Philadelphia have a mild obsession with the color blue.

3) It has hills. Even in the zoo.

4) It is largely if not entirely populated by Amy's friends and co-workers, who came out in pretty much record numbers to support her and to fight CF (but then, we all understand why Amy attracts that sort of admiration, right?).

5) Oh yeah, and it's home to some rather ridiculous but very adorable monkeys.

Very fun day. Amy was sweet enough to chauffeur me around for the day in her stylishly decorated jeep, which I can guarantee is a LOT cooler than the cabs I'm going to use to cart her around NY next week. Six years in NY and I have yet to see a cab with any bumper stickers at all, much less one as great as those in Amy's collection. We also got to compare pulse-ox readings after completing the aforementioned hill climbs. Believe it or not she kicked my butt -- mine was just consistently crappy in the mid to upper 80s, but Amy is apparently a woman of far more exciting extremes, so her's was both higher and lower than mine at various points. Show off.

I'm super excited to host her in return next week and do the Manhattan walk. Last time I checked Battery Park City didn't have any flamingos, zebras, or large organgutanges, but we do have some really pretty views of the harbor. And either way we're raising money to help fight CF, which always makes for a wonderful day.

Too Late or Too Early?

It's really, really late. Or else it's really, really early - just depends on your viewpoint. Either way, I probably shouldn't still be up, but my IV schedule coupled with a poorly pressurized intermate that took double the time to infuse, on top of an already somewhat late night spent at a fabulous restaurant in NYC enjoying some of my new-found (re-found?) health, all add up to a late night. A night that will, unfortunately, probably give way to an early morning b/c I still have to infuse the imipenem every 6 hours. But it's okay, I'm not complaining.

Life is worth it, right?

Being out with friends is worth it, going to see the band play last night was worth it, walking all the way from my TriBeCa apartment to the dogrun in Chelsea this afternoon just because it's cooler and I thought my puppy would have more fun was worth it. Not bothering to check my O2 levels during said walk because it was 70 degrees and sunny and I was out with my puppy along the river and just didn't want to think about CF for a couple of hours was with worth it too. Totally.

So I promised an update after Thursday's clinic appt, and I know it's a little late in coming. There was, to be honest, a lot to digest, and I'll probably be taking a little break from health blogging for a while as I just kind of sort through it all. But for right now I'll give you the bare facts as I know them:

• FEV1 back up somewhat, at least to a level that didn't scare me completely
• IVs until next Friday
• Off work for a while -- have to recover and give my body a chance to get back where it needs to be and . . .
• My doctors, both CF and transplant, feel it's time for me to go active on the transplant list

Yeah, that last one was a little bit of a shock to me too, especially that particular day. I just wasn't expecting it at all, which is a little strange considering a couple of months ago, in January, I was sort of questioning why exactly we weren't moving forward. But right now, I dunno . . . maybe it's because I'm on IVs right now and feeling pretty healthy, but it just kind of came out of nowhere for me.

No wait, scratch that. Out of nowhere would probably imply that I don't get my doctor's reasoning here, which isn't true. I get it. I get that I've been on prednisone at fairly high doses for several months without any sign of being able to successfully wean off it. I get that my BMI is way too low and I'm not really gaining weight. I get that my O2 levels are getting progressively lower, and that the last couple of infections have sent them plummeting into the mid-to-low 80s. I get that a week ago I was in the hospital sating 89 on 5 liters of oxygen. I get that in the past 6 months I've stopped responding to some of my "best" IVs. I get that I had 5 rounds of IVs in 2008. Most of all I get that this is already my third round of IVs in 2009, one of which lasted for 6 weeks, and it's only April. Believe me, dear God believe me . . . I GET IT! I am one sick little cystic, and nothing I do seems to be changing that fact.

But all those things are really only part of the story. The other part is that I still feel happy (most of the time) and I still feel like ME (almost all of the time). I still run, I still leave my house everyday and do pretty much whatever I need to get done, and I still go out and have fun with friends. I don't feel like I can't do those things, at least not when I'm healthy -- which is, admittedly, not nearly often enough, but as soon as I start the IVs I tend to bounce right back. My baseline FEV1 after IVs is higher than the level at which most CFers are even referred for evaluation. Most of all, I'm just stubborn. I don't want to be here, at this point. I want to do something magical and miraculous and suddenly have the infections stop and my FEV1 shoot up to 50% and have everyone stand around laughing and saying "wow, Pipe, you really had us worried there for a second!"

Anyone know how to make that happen? Please?

I don't want to wait so long that one of these infections becomes life threatening. I also don't want to give up on my lungs -- which, despite their CF and their crappy mucus-filled airways, are still after all MY LUNGS -- before I have to. I totally understand that I'm not a black-and-white case, that for some reason my silly brand of CF has taken textbook progression and decided to see it as a challenge to beat, but still . . . I'm only human, and no human particularly wants to have to face these facts, right?

My doctor called me "stoic" about CF -- she said it was easy to get a false sense of security from looking at and talking to me, that you would never guess. She apparently hasn't read this blog ;)

I have some questions. I need some answers. And then I'll make a decision with the help of my amazing medical team (who I trust COMPLETELY) and that decision will be right for me right now. I don't care if that sounds sappy and trite, I'm still stubborn enough to believe that it's true.

Did I mention it's late? Or maybe it's early.

27 Years, 4 Months, 14 Days

. . . and counting.

Okay so I'm not going home today. The doctor (who happens to be my doctor's fraternal twin sister and another pulmonologist at this hospital) came to see me pretty early this morning. I had just woken up and had yet to do xopenex or my first airway clearance, so needless to say my O2 sats probably weren't as high as they could have been. I was really hoping to break out today, but honestly I guess one more day might actually be in my best interest (cringe).

I did get to leave my room for a walk today. Because of the whole CF = contact isolation thing, I'm not allowed to leave my room alone. But the PT came and actually had time to stay and take me for a walk after my CPT, so that was HUGE. Getting out of the room was totally key to my sanity, and getting back into some sort of exercise routine as fast as possible is definitely key to my O2 saturations coming back up. I begged the PT to get me an exercise bike if possible, but at the very least she left instructions with the nurse's station that I was to be allowed to take walks on the ward as long as I gowned first and took proper hand washing precautions. So I guess I'm about as free as anyone ever is on lock-up isolation.

So yeah, the walk. We did a solid 15 minutes at a nice brisk pace. I would have liked to go longer, but she had to leave (and she said SHE was tired, lol). I was just so damn happy to be moving I feel like I could have gone for miles! We did bring the portable O2 tank, and she started me out on 2 liters because we had just done CPT and so I was already a little "warmed up", so to speak. After a couple of laps, my O2 was at 90 and occasionally 89. So she cranked it up to 3.5 liters and we kept going. Well, I felt great, I was moving fast AND talking the whole time, but my O2 stayed exactly the same. Finally, it dropped to 88 after 6 laps or so and she insisted we stop for a sec to rest. So we did, and she looks down at the tank, and suddenly she covers her mouth with her hand and gasps. Turns out she had connected the tubing to the WRONG nozzle -- it was actually connected to the one they use to drain the tank. So I had been statting at 90-89 for 6 laps, while talking, at a pace she had already called "impressive" on room air! I was definitely pleased, and she made a big note of that on her little recording sheet. Then she turned it on for real at 1 liter and we went for another 5 laps with my O2 at 92. Woo-hoo!

She told me I could go out sans O2 for my afternoon walk(s) but that I had to promise to pace myself and stop after 5 laps max. She couldn't leave me a pulseox so obviously I'll play by the rules since I can't self-monitor. No sense pushing it too hard, and again - my new goal is to be aggressive AND know my limits. I'm naturally kind of a stubborn little brat (or another word starting with B . . .) so I think I need to start recognizing the difference between good pushing and bad pushing. And I appreciate the offers for a good kick in the ass from some of you, but you know, it's tough sometimes.

I miss my puppy. I want to go home. Above all, I am one DIRTY little cystic right now (yesterday I had salt crystals fall out of my hair from all the sweat coupled with the no showering rule). I still hate IVs and I still feel frustrated beyond belief that I got sick again so soon and that my sats are taking so long to bounce back. None of that has changed. But I guess you learn to see victories wherever they start to hide after a while. Kind of a "one small step for Piper, one gigantic step for Piper's lungs" type of thing.

It just so happens those steps are on the 9th floor of Columbia Presbyterian Hospital for now. Sigh.

Breathe for Me, Baby

Update: Day 3

• Number of IV antibiotics currently prescribed: 3
• Number of IVs discontinued today: 1 (bye bye, solumedrol!)
• Number of unfortunate talks with my doctor about abhorent feeding tube suggestion: 1
• Number of "Boost Plus" drinks subsequently consumed after said conversation: 6 (!!!)
  
• Number of hours sleep possible to obtain at a hospital: one of life's mysteries, but low
• Number of stupid questions (e.g., "how long have you had CF?"): 3 and counting
• Number of snarky answers (e.g., "27 years, 4 months, 13 days"): 3 and counting
• Number of blood draws: don't ask

and finally . . .

• Number (percentage) of O2 saturation on ROOM AIR tonight: 92% (!!!!!!)

My doctor made it very clear to me today that the only thing holding me here in lock up (and away from my puppy!) is my unstable O2 situation. She's going to be off tomorrow and Saturday but back on Sunday, so she tenatively wrote discharge orders for Saturday, BUT she put in the orders that if my O2 is consistently above 90 on room air by tomorrow afternoon, I can leave. And likewise if it is not by Saturday afternoon, I have to stay. Sounds like a reasonable compromise.

I am SO psyched about the possibility of early jailbreak. Sammy! Showers! My own bed! Sammy! Good food! Easter! Sammy! And yes, I admit I'm obsessed with my puppy. So sue me. No, really, please try it ;)

My friends have been awesome - I've had visitors constantly and a steady stream of non-crappy outside world food. Which is good because, as I mentioned above, my weight is about to become my new battlefield. I have to have a BMI of over 19 by the end of summer/early fall to avoid the dreaded tube. And considering I'm currently 18-18.5 on a really good day, that might be an interesting challenge. Although honestly, I respond really well to ridiculous goals, so here's hoping.

The other update is that I'm officially going to take some short term disability from work, but only for a few weeks, just to sort out this current infection and hopefully get things in a more stable position. And then I'm going to work out how I can stay on and be a productive member of the firm on terms that actually work with my health. I'm not worried at all about my law firm's role in this - they've proven themselves more than understanding and very wonderful in the face of a crappy situation - but the challenge is just going to be me. I need to set boundaries, I need to stick to them, and I need to start realizing that slowing down is not giving in to CF; it's another way of fighting it and NOT letting it take control completely. So I know I'm a bit thick-headed that it's taken me 27 years (and counting) to figure that out, but I swear I'm almost there. Almost.

I am hoping that if I'm not completely focused on work and health that I'll have more time/energy for the rest of my life and won't have to kill myself trying to pack that all into the weekends. So maybe this will be a way for me to take back part of my life, rather losing any more. Maybe.

But for tonight: 92% saturations on room air! Come on baby, breathe for me ;)

Really? Seriously? Honestly?!

Today may very well go down in history as the craziest, most ridiculous day of my entire life. I'll tell you all about it, but first I need to know: do you want the bad news, the crazy news, or the fucking unbelievable news?

The Bad News:

I'm sick again. Yuck. Just some gross viral crud that managed to worm its way into my freshly IV'd lungs despite my best efforts. So now I'm writing this from my room at Columbia Presbyterian Hospital, which I didn't make it to until 6:30 pm, because . . .

The Crazy News:

There was a fire in my apartment building. No joke. As in, some genius had a timed light (in an apartment . . . will someone explain that to me, please?) and it was too close to the drapes. So when it came on, the apartment owner wasn't home and the drapes got too hot and voila. Half the NYFD showed up to my apartment building, bells and whistles clanging, and I had to evacuate down 18 stories of STAIRS, without O2 (not in the habit of wearing O2 near open flames) while CARRYING my 20 lbs puppy. I had to carry the puppy because he's an apartment puppy and he doesn't "do" stairs. Brilliant.

The Really Fucking Unbelievable News:

So this genius with the timed light? Yeah, turns out he lived DIRECTLY above me. As in, one floor up, same floorplan, literally right on top of me. Which means that when his apartment caught fire, his sprinklers turned on, and when his sprinklers turned on (and he wasn't home), they created 4 inches of standing water on his floors. And that 4 inches of standing water had nowhere to go but down. Literally. Straight down, and into MY apartment.

My living room and both bedrooms had huge floods in them when I finally got back up to my apartment (suffice it to say I had to wait until the elevators were back on line to get up to the 18th floor). Huge flood. On my hardwood floors. And oh yeah, did I mention I'm sick? Like, 2.5 liters of O2 while resting, in the hospital style sick?

The Silver Lining:

Thankfully, I have an amazng woman who helps me out with all the little parts of my life that CF makes difficult, including cleaning and shopping one day a week. Well, that day just happens to be Tuesday, which means she was around today and immediately sprang into action. She was amazing - went around cleaning up all the water and treating the wood to minimize the damage. Luckily there is insurance to cover whatever work does need to be done to the wood floors, but right now I think it probably won't be that bad. Thanks ONLY to Bella, who I am now convinced is some sort of guardian angel. With her help I got packed, got out of the apartment, and she's staying with Sammy until someone else can get there. Wow.

Part II of the silver lining is that my doctor actually doesn't think I'll need to be in that long. She said she anticipated a short stay (maybe just a couple of days) and that this is probably just a virus gone awry.

So yes, it was a terrible day, although in retrospect it all seems more amusing than anything. And yes I am clearly going to have to make some big decisions sometime soon, but I think the first route is going to be trying some short term solutions while we work on longer term options. Which means I'm not quite as hysterical as I was earlier today.

And that's the best news of all.