Freedom Is Just Another Word

Well, as of tonight, I am officially, 100%, totally free.

Yep. I officially have no more port access, no more IVs to infuse, no more home healthcare company to deal with, and no more ridiculous medication schedule to adhere to. OK, fine, so my medication schedule is still a *tiny bit* ridiculous, but no more so than any other normal, healthy, fabulous cystic!

And it feels absolutely amazing.

I didn't think this whole "IVs for 6 weeks" plan would be too bad, and honestly I guess I have to admit that it really wasn't. Aside for the whole gravity drip snafu, the frustrations of dealing with the home health nurses, and the bum deal of being on an ineffective drug for nearly half the time, it could have been a lot worse. But let's face it, that's not saying a whole lot. By the end of this thing (i.e., tonight) I was literally going crazy! And to all those amazing CFers out there who have endured months or even years of constant IV abx, you are all my new heroes. Seriously, well done to you!

Unrelated sidenote: I've started jotting down my meds, exercise routine, communications with my doctor, and recorded HR/O2 levels in a little notebook I bought. Sort of like a daily medical journal, and I also make a note of how many hours I slept and any other pertinent information (e.g., worked until 10 pm, just as a random example). Yes, I do realize that is totally OCD and crazy on my part, but the thing is, I've been getting sick lately a LOT and I wanted to just try tracking everything health related for a little while to see if I can notice any patterns. At the very least, it holds me accountable in a weird way, because if I decide to skip exercise or a neb treatment one day for any reason, or totally blow off sleeping, then I have to write it down and actually fess up to it later. But mostly I'm not doing this to scold myself (or even to reward myself, though that would be more fun) - it's really just so that if I get sick again less than a month out like I have the last couple of times I can look back and try to see when it started and if there's anything that might have triggered it. And maybe in 6 months I'll have enough information to be able to make some important adjustments and stop this crappy cycle.

Or I'll just have a really creepy health journal. Either way.

Secrets of an IV Junkie

If you've been following this blog for any amount of time, you might know that I'm a super huge fan of my port, which happens to be in my upper left arm. I've had this sucker for 9 years (since I was 18) and I absolutely LOVE it. I love pretty much everything about it: how I can cover it so easily just with long sleeves, how I can vest without any discomfort or inconvenience even when it's accessed, how even in tank tops it's basically invisible when it's not accessed, and how it doesn't, um, get in the way. Ever ;)

The only things I don't like about it are: 1) I can't access it by myself, and 2) it moves more than a chest port (so I've been told) and kinda freaks out some of the nurses who aren't as familiar with the arm placement. But other than that I love it. Well, today I found a third negative, and while it still doesn't convince me that I want my next port to be in my chest, it's still annoying as hell.

Basically, the fact that I can't access the thing by myself means that I am at the mercy of the homecare nurses for pretty much anything, including monthly flushes and dressing changes. This has honestly never bothered me too much b/c the monthly flushes are no big deal and the dressing changes just happen when they come to draw blood labs. So no big problem, right? Right...well, most of the time. This time, though, I'm on IVs for 6 weeks. That's double the time I normally stay on these things, and it's far and away my longest course ever. So because I can't access myself, I just rely on the nurses like normal. And because I have to rely on the nurses, the skin around my port is ALWAYS covered by the dressing and tegaderm. It only gets to "breathe" for like 30 seconds when they pull the old needle, alcohol the area, and then put the new needle in and the new tegaderm on. No time to shower or wash the area, no time to let the skin relax, basically no time to do anything at all. It's normally not an issue, but this time around it's not particularly pleasant.

The nurse just came to change the dressing and my poor arm looks like it wandered through a particularly brutal war zone. The skin is red and irritated - it has little bumps from the tegaderm and the area is totally raw from the sticky adhesive. Yuck. To top it off the port needle has this little place where the needle joins the tubing, and the nurse last time failed to put the usual gauze under that area, so now I have a nice little HOLE in my arm from that thing digging in. Double yuck. Thankfully it's far enough from the actual injection site that I'm not too concerned about infection (and it's under the tegaderm, so clearly the area is sterile), but still. Not fun. I didn't even feel it, most likely because the constant pressure just sort of dulled the whole sensation. Again, more yuck.

Don't get me wrong, I'm thankful for IVs and for all the medical miracles that help CFers and blah blah blah. But truth be told, next Wednesday cannot come fast enough. I am soooo over these IVs and ready to get this damn access pulled already! If not for my sanity's sake, then definitely for my poor arm!!

Bye, Bye, Gravity Drip

Yep, you read that right - the dreaded gravity drip is HISTORY! Woo-hoo! Honestly, I think I'm a little too excited about this, but the freedom of not having to deal with that thing, especially at the office, is totally overwhelming right now. And for those wondering, I did in fact infuse via gravity drip in my law firm, thanks to my private office, a clothes hanger, and some mad macgyver-like skills on my part. And proud as I was of the whole arrangement (think tubing hanging down over my keyboard from the bookshelf above my desk) I'll still be only too happy to throw it all away tomorrow.

The reason for this change is my awesome, amazing, and basically unbelievable doctor. She and I decided at my appointment today (5 weeks down!) that I should switch back to Merrem for the final week. Apparently my recent cultures showed some insensitivity to Aztreonam, which probably explains why I haven't been feeling 100% for the past few weeks. I have to say, it's kind of a relief to learn that my cultures just aren't sensitive to the abx combo I've been using, because while that certainly sucks, it's a whole lot better than having PA so strong I can manage to get flare-ups even while on the correct drugs. And it also means that there's a good chance I'll feel a lot better by the time they pull my access next week, which is exciting. I was doing great on the Merrem during the first few weeks of this IV course, so I have high hopes.

My doctor and I did talk about transplant. My FEV1 this time around was 35%, which is about where I typically stand when I'm healthy, but not yet up to baseline. My baseline is more like low 40s. So there's still room for improvement, of course, but things weren't too bad. I think both she and I were a little disappointed that this is where I stand after 5 weeks of IVs (even considering the Aztreonam wasn't doing the full trick, between that AND the Tobra IV I still should have had a little more improvement I think), but there's not a whole lot we aren't trying at this point. She noted that they don't really take into account infection frequency in calculating lung allocation scores. I told her I was still interested in delaying transplant as long as possible. I think the mark of a really good doctor is that even with things not being where we would both like them, still left the appt feeling SO MUCH better, and with sort of a renewed commitment to fighting this thing for as long as I possibly can. She's not giving up on me right now, and she doesn't think I'm ready to be listed, and frankly that's good enough for me. I can still do a lot of what I want to do, and while I am really worried about what's going to come of work, for right now I'm going to keep trying to be a lawyer and a CF patient. So watch out crappy CF lungs, because I'm waaaay over this little comedy of errors we've been acting in together for the past few months. And this time it's personal.

But for tonight, Sammy and I had some fun in the dog run, I had a great St. Patrick's Day dinner with good wine and a good friend, and now me and the boy are watching a very funny re-run of The Office . . . without the gravity drip!

Wishing you all a very happy St. Patrick's Day!

May the road rise up to meet you,
May the wind be at your back,
May good friends be there to greet you
And your table never lack.

May your life be filled with laughter,
and your heart be filled with song.
May God shine His light upon you,
As you live your whole life long.

Still Here

So for the past few weeks I've been working like crazy, at least for me. The weird thing is I probably haven't been working as many hours as most people in my law firm, but I still feel exhausted. I've been putting in extra hours during the nights and weekends, something I know I shouldn't be doing, but I've had a case that was just starting out and needed a lot of attention to make all the deadlines. And honestly, I really thought I'd be okay with the whole 6 weeks of IVs thing going for me. I mean, who gets sick when they've been on heavy duty abx for 4 weeks, especially when they're STILL doing them? It doesn't make sense, right?

Well, apparently the answer to that question is a big resounding I DO, because, well, I did. Not really bad sick, obviously, but definitely a little more short of breath, tired, achey, and coughy kind of sick. I think I've finally managed to come through it: my case settled and I finally wised up and (with the exception of the obligatory social Friday night) made the smart choice to stay in and really give myself a chance to recuperate this weekend. So I've done a lot of sleeping, some working out, walking my dog, and as much eating as I can handle in between the rest of those activites. And I haven't done a single minute of work since Friday. I'm actually pretty proud of myself.

The thing is, I'm NOT okay with this. I'm not okay with the fact that even though I've been religiously doing IVs, adding extra treatments, and pretty much trying everything I know how to do (excepting of course, for cutting my hours, which I KNOW I should be doing), but yet I still get sick! I get sick ON IVS! WTF?! Sorry, I don't mean to sound like a whiner here, but it scares the living shit out of me to think about the fact that I am four weeks into IVs and still getting short of breath just playing with my dog. It's not okay! It makes me feel like I might never ever get back to where I was, and I'm not even sure what I even mean by that anymore. I just have this mental image of myself as really happy, really energetic and excitable and up for anything, even downright hyper. And I still feel those things, inside, but when I try to act on any of them I feel totally stunted. I look at my puppy and all I want to do, basically in the whole world, is run wild with him in the dog run, or walk briskly down the street with him at my heels, out to enjoy a saturday in the park and have a little fun. Instead I find myself sitting lamely on a bench, watching him as he once again amuses himself with the other dogs and their more energetic owners. And then I get up, I try to make it all happen, but I only last about 5 minutes before I'm panting, coughing like crazy, and watching as the other owners go scattering in all directions in search of somewhere less contagious. It's really getting pathetic, seriously.

I don't understand it. I think the worst part is, I honestly feel like I'm failing. Failing myself, failing my family, failing my doctor. I do so much and it's not enough, and I'm too scared to take the only step I feel like there is left for me to take, which is probably just bucking it up and leaving my job. My job that I worked so hard for. My job that, random and lame as it might sound, I honestly love. My job without which I HAVE NO IDEA WHAT I WILL DO WITH MYSELF. But maybe it's that or my lungs. Maybe the writing's on the wall and I'm just too stubborn to admit it right now.

I know so many people with CF. So many people with lung function right around where mine is. And for some reason none of them have been on IVs for all but two and a half weeks of 2009. I mean really, we're in MID MARCH and I've literally been on IVs more this year than I've been off. That's not okay. There's something wrong and I'm so so sad to have to admit that.

I'm scared of what will happen if I leave my job and nothing changes. I'm scared out of my mind about what this means for my chances of living with this disease. I'm scared because they don't take frequency of infection into account when calculating your score for transplant and I know that a really big one right now could really do some damage. I'm so scared that one of these times I'm going to find myself unable to recover, and I have no idea how to tell any of this to anyone in my life because, honestly, I don't want to drag anyone else down. I can't win right now against this CF, and I feel like the only way I can keep fighting is to just stay positive and keep working, keep pretending that I'm totally fine with all of this, or at least that I've reached a place where I can be strong about it. And I guess 90% of the time I do feel that way, and I am grateful for the things I can still do, but for the other 10% of the time I feel totally out of control. And if I'm totally honest I would also say that I am sometimes unbearably sad about this disease and what it's taken from me so far. I just desperately want to hang on to whatever's left.

There's much more than just this going on in my life, I promise (and most of it fun!), but for tonight that's all I've got.

True Confessions

Alright, serious confession time. There is something I've been keeping from all of you on this blog, and it's not something I'm necessarily proud of. Actually, it's pretty embarrassing, to be totally honest. But clearly I can't keep it a secret forever, so here goes nothing . . .

I have an addiction . . .

. . . to TV infomercials.

There, I said it. And it's true. Really sadly, pathetically true actually. I love the damn things, to the point where I will often just sit there mesmorized, convinced that the product on my screen is the answer to pretty much every problem in the universe. "Wow!" I think to myself, "If I had that special slicer/dicer/whatever thing, I would totally julianne my own potatoes!" Who cares that I don't honestly know what it means to julianne a vegetable? And honestly, does it really matter that I haven't purchased potatoes for myself in several years? Clearly buying a miracle slicer would change all that!

No, I don't normally actually BUY the stuff. I pretty much just sit around in a trance imagining what I could do with whatever the greasy looking man and overtanned woman on TV happens to be selling. Although before you totally write off the whole thing, I should say that I once did buy a blender like thing called the "Magic Bullet" which is honestly the best thing I've ever purchased. I never made any shakes in my old blender because it was a pain to clean, but this thing lets you blend stuff right into a cup. I put some Boost Plus, vanilla ice cream, and chocolate syrup in the magic bullet and let it do its thing. The Boost Plus is pretty much the only thing I'll drink in the high-cal supplement category, but I actually really like these shakes. I normally use chocolate, but vanilla works too.

Anyway, this has nothing to do with CF, beyond the fact that I may have developed this obsession through bored hospital channel surfing. Right now I'm up waiting for my tobra IV to finish infusing, and I just stumbled across a GREAT informercial for some sort of miracle space saving hanger (it bends). I have plenty of hangers, and I'm pretty sure this invention won't help me gain weight like the blender, so I probably won't order it, but I'm pretty sure it could do wonders for my dress closet!

On a personal health note: things are fine. I'm still on IVs (into week 4) so that's nothing new. No more issues with the gravity drip, thankfully. Had a great weekend with some friends and some really amazing NY food and weather. My parents called after the CF Ski event this weekend in Vail, which raised over $450,000 for CF research in three days. Wow, good going guys!

Also, if you have a second, please take a little time to visit The Finding Jenn Project website. This was started by a great CF friend and Cyster of mine in memory of a wonderful knitter who fought an amazing battle with CF. For all you knitters out there, this is a great way to strut your creative stuff AND help out CF patients!

Tonight's Top Ten List

So I think I finally have this gravity drip thing figured out, although it took a while. So far today I have successfully maintained a 25-40 minute infusion rate for 2 out of 3 treatments. Woo-hoo! So as much as I now hate home healthcare, I can at last proudly proclaim that I am in fact smarter than a five foot pole and 6 feet worth of tubing. Thanks to you guys, that is.

On an entirely separate note, I recruited someone to be an organ donor today. Actually, to be fair, he said he had wanted to be a donor for a while now, but he hadn't signed the necessary forms in NY. So I encouraged him to get cracking, and he registered! Double woo-hoo! But I guess that brings me to my next question for everyone out there, which is this: how do you guys typically bring up the subject of organ donation when dealing with non-CFers?

Most of my "cysticly-challenged" friends probably aren't donors. I don't think this is because they don't want to be, necessarily, but more due to the fact that NY has a pretty annoying registration process. It's not just a sign your license and go sort of thing. And once you've registered, you're pretty well locked into it here. Your family can't override your wishes and you have to jump through a couple hoops to get yourself off the registry (as I understand it). Some people I've chatted with find this system scary, and others -- like my friend today -- just "haven't gotten around to it." Now, granted, most of my friends are in their 20s and 30s, so they hopefully have plenty of years left in which to register as organ donors. They also mostly don't have wills or healthcare proxies written either, so I think a good part of the non-donor thing is just an unwillingness to plan for anything involving end of life stuff. Which I totally get, btw. I've been faced with the possibility of my own mortality basically since I was born, and I didn't even draft a will until last year. It's just not the sort of thing anyone really wants to think about, obviously.

At the same time, I really want my friends to at the VERY least consider organ donation, and preferably sooner rather than later. I think this means that I need to talk to more of them about it, but it's a tough topic to bring up. For one thing, it doesn't exactly fit well into our normal conversations and for another, well, it's just kinda awkward b/c I feel like people always think I'm PERSONALLY asking them for their organs. But honestly, let's face it, short of determining that I need a living-lobar transplant there is VERY little chance that I'm going to somehow end up with my best friend's lungs in my chest (knocks on every piece of wood in the apartment). So come on guys, this isn't about me. It's about giving someone -- or, in most cases, multiple someones -- a second chance at life just by agreeing to share what you no longer need! What could be difficult about that decision?

Anyway, here's my personal top ten list of reasons to be an organ donor. Feel free to use these on your friends/coworkers/eccentric family members/whoever else will listen:

1) Let's start with the obvious: Organ donation saves lives. Period.
2) You never had much of a use for that second kidney anyway.
3) You're so awesome, just think how much better the world would be if there were three or four people all with a little bit of you inside them!
4) Every time someone donates an organ, an angel gets his wings.
5) Despite what this movie would have you believe, you're probably not going to have too many chances to use your organs after you're dead.
6) Did I mention organ donation saves lives? Yes? Well good, because it does.
7) Signing up to be an organ donor takes less time and is more productive than any of the following activities: updating your outdated My Space profile to reflect that Britney Spears is not, in fact, still your favorite recording artist of all time; programing your DVR to record the entire season of Bravo's "The Real Housewives of Orange County"; or filling out that "25 random things about me" survey on facebook.
8) All the cool kids are doing it.
9) This puppy really, really wants you to be an organ donor:


10) No really, I'm serious: ORGAN DONATION SAVES LIVES!

Happy recruiting, everyone!

How (Not) to Use a Gravity Drip in 10 Easy Steps

Step 1: Call home health company and explain that you have neither the equipment nor the understanding necessary to infuse the random bags of medicine that they previously delivered to your house with absolutely no warning. Accept their assurance that they will correct both of the above problems, despite reasonable suspicion that they will actually correct neither.

Step 2: Return home from work to find pole and tubing, but no additional instructions nor call from the nurse.

Step 3: Call 24 hour line, get answer that no nurse will be coming because none was requested. Think back angrily at step 1 of the process and argue with man on phone. This will get you absolutely no where, of course, but do it anyway because arguing with people in the medical field is fun!

Step 4: Consider skipping yet another dose. Decide against it. Take some comfort in the fact that you're an educated, semi-intelligent woman (albeit one with aboslutely no clue what she's actually doing).

Step 5: Assemble pole and tubing. Squeeze udder-like tube thing on the top of the tubing. Do this largely because you've seen nurses do it in the hospital. Get excited when medicine enters said udder-like tube.

Step 7: Role ball-clamp to open tubing. Here's the fun part: Because you're an educated woman, make the logical decision that the clamp should be OPEN. And because you also happen to have no clue what you're doing, don't stop to consider the possibility that the ball clamp might actually control the flow of the medicine.

Step 8: Watch as medicine begins to drip out of tube. Success! (And yes, for those of you who know what's coming, it was dripping...it started off dripping because there was air in the tube.)

Step 9: Attach IV. Settle in to watch 30 minutes of TV while 30 minute IV infuses. Look up at IV med 10 minutes later. Notice it is basically empty. Disattach tubing from port and watch as a STEADY STREAM of meds flows out of the tubing. Freak out.

Step 10: Log onto website and receive wonderful information from fellow CFers on where you went wrong. Also receive assurance that you will not die from overdose. Resume breathing.

So yeah, I don't recommend actually following the above advice. Definitely makes for a stressful evening. On the other hand, if you prefer your aztreonam short, sweet, and highly concentrated, go for it.