Thursday, February 26, 2009

My CF Statistics

I've noticed a new trend with cystics posting their "CF Stats" on their blogs. 1) I hate to be left out of a trend, 2) it looks like fun, 3) they're great to read, and 4) did I mention I hate being left out of trends? ;) Anyway, here are mine:
  • My name is Piper
  • I turned 27 in November
  • I was diagnosed at 6 weeks with borderline failure to thrive and bacterial pneumonia that had spread to menengitis. Yikes.
  • Like most CFers, I got my enzymes in applesauce growing up. And, like all of them, I won't touch the stuff now. I wonder if the person who came up with that brilliant idea knew how much he was hurting the applesauce industry long term?
  • I have done CPT as long as I can remember. I used to have an ironing board like table that tilted down, and I could climb on it with my head pointed toward the floor while my parents beat me up. The only way I got break is if I promised to run laps around out yard.
  • I never went to CF camp and I never had a make-a-wish. I remember being asked if I wanted to go to CF camp (before they closed), but I said no. I went to "regular" camp instead.
  • I had one picc growing up until I hit 15. After that I was hospitalized at least once a year.
  • No sinus surgeries. Take that, CF.
  • I rode horses for years competitively as a showjumper. I owned several horses in middle school through high school. My doctor once told me cepacia was more common in rural areas to try and get me to stop. He also made me wear a mask in the indoor arena. I refused to stop and rarely wore the mask, but I was super healthy.
  • I had my appendix out in 2008. Because of my CF they weren't sure what was going on and made me wait for three days in the hospital on IVs before they gave me surgey. The night I had it uptown there was another CFer who had her appendix out downtown, and our surgeons communicated on how to do it b/c they were both freaked out.
  • I was told I should think about transplant in January 2008 and I was done with the evaluation 8 months later. I'm told this is fast.
  • I got my port when I was 18. The radiology guy who placed it wanted to put it in my chest, but I was REALLY not down with that for some reason. I wanted an arm port. But the guy told me right before giving me the happy drugs (I was awake the whole time) that if he couldn't make it work in the arm he would put it in the chest, so no promises. 9 years later I still have my fully functioning arm port and I love it!
  • I have one DF508 mutation (normal) and one complete deletion of the CFTR gene (definitely not normal). This means I inherited no CFTR at all from one of my parents. The official mutation name is "CFTRdele1-24" but this isn't tested for even on the full panel. Only the deletion panel revealed my second mutation, so for years we had no clue but knew my sweat test was well over 100.
  • 27 and still no CFRD (knock on wood). Again, take that, CF!
  • I refused O2 even for night until my doctor was literally not going to take no for an answer. For me, O2 was a BIG hurdle.
  • The sickest I have ever been in my life was in Australia for New Years 2007. I can't even think about that trip without wanting to cry. It was bad, and it was my own fault b/c I was too scared to go to the doctor before we left for fear she would make me cancel. She would have, and I should have, but I didn't. I went, but couldn't really enjoy it AND wound up making my family miserable. It's still one of my deepest regrets. Ever.
  • I'm the only cystic in my family. My older sister is a carrier, but CF free.
  • I was born next door to a little girl two months older than me. When I was 6 weeks old I was diagnosed and her parents felt terrible for my parents. A year and a half later, my parents talked to her parents about getting tested for CF b/c she had similar symptoms. She was tested, and had CF, and the two of us (and our weird story) became part of the reason that Colorado was the first state to institute newborn screening. We're still friends, and her younger sister also has CF.
  • When I was diagnosed the doctor told my parents to "take her home and love her for as long as you can, because she won't be around long." I'm still here. I have no clue if that doctor still is ;)
  • I was a poster child and keynote speaker for the CF foundation for about 20 years. I appeared at events in CO, CA, and GA. Mainly I covered an event called the American Airlines Celebrity Ski, which is held each year in March and has raised several million dollars for CF research. I am grateful everyday for the chance to have been a part of that.
  • My parents won the local chapter "Breath of Life" award for Colorado in 2007. I am so proud of them.
  • Between my parents, my unbelievable big sister, and my friends, I have what my doctor has called "the best support she's ever seen." I am so lucky.
That's all I've got. If you read this and have CF or have a loved one with CF (haha, as if anyone reading this isn't connected to CF!), consider yourself "tagged." Let's hear those stories, people!

Saturday, February 21, 2009

Borderline Saturday

Do any of you remember when NPR's All Things Considered ran its segment on a young woman named Laura Rothenberg and her double-lung transplant story? The show was called "Breathing for a Living" and it ran while I was in college b/c I vaguely remember my parents calling me up and telling me that I needed to listen to it. Of course, I was in college and had better things to do than listen to NPR (or rather, I THOUGHT I had better things to do -- turns out frat parties aren't nearly as important as I probably thought they were at that point), and beyond that I frankly wasn't all that interested. What did this 22-year-old woman with a lung tx up at Brown have anything to do with my life as a college student in the south with 80% lung function? I kind of figured that it made about as much sense for me to listen to her story as it did for me to listen to stories about people with AIDs. Interesting maybe, sad definitely, but not my disease. Not in any realistic sense anyway.

Okay so I'll spare you all the flash forward and the dramatic retelling of how fast CF can creep up when you're not paying much attention. You know the story anyway. Suffice it just to say that I've had a little bit of a reality check in the past few years. And that reality check has definitely left me eager, in a way I would never have imagined, for the stories of other cystics. Seriously, I have a need bordering on compulsive just to hear or read more about what other people with my disease are going through, or what they have been through, or even what they might go through in the future. I can't really explain it, and honestly I don't question it much. I think it's normal to want to connect with others like you, even if your experiences are different, and really I'm just kind of sad that it took my 25 years or so to figure that out. Also, I love what this newfound interest has given me: it's what led me to the online CF communities, opened up a whole wealth of stories of people living successfully both with CF lungs and post-tx, and helped me connect with a bunch of other CFers who are all dealing with the same or similar issues that I am right now (um, job issues, anyone?). So yeah, basically now I'm a bottomless pit of all things CF-related, and I'm okay with that, as long as I remember to leave enough time in my life for other stuff too.

Luckily for me, cystics DOMINATE the world of lung-transplant memoirs. Seriously, go to Amazon and check it out. It's basically CF, CF, emphysema, CF, random other lung disease, CF, CF. I'm not sure why this is, maybe because we tend to be younger and have more urge to write after transplant? Maybe we have more interesting medical histories than the average transplant patient so our stories are more interesting? Who knows. The point is, we totally own this genre. And among the books that I've read lately in this category is Laura Rothenberg's own memoir, which is the backstory behind that old radio program I refused to listen to six years ago.

It isn't beautifully written, in many ways. It reads more like a diary than a real narrative and reading it felt a little voyeouristic to me, because it's as though she was writing for herself -- she references people and happenings without explaining them, which makes the whole book read like a personal recording of events not intended for a wider audience. But it is amazingly honest.

She writes about feeling left behind by her friends, about sitting alone in a hospital wondering why more people don't call, or thinking about how weird it is that life still goes on as normal outside her little room. And that's sort of how I've felt lately, even though I'm lucky enough to not be confined to a hospital bed, b/c even as I go through the motions of getting up and going to work and going out with friends, I still feel as though I'm existing somewhat in a "sick world" that other people just don't live in. Sure, life goes on as normal, and I understand that, but at the same time I sometimes marvel that there are little things happening (people are buying groceries, a guy is talking on his cell phone, secretaries at my firm are typing memos and lawyers are racing to the next meeting) and all the while I'm juggling that normalcy with a whole other side of my life that deals with transplants and FEV1s and oxygen tanks. I dunno, sometimes it's weird trying to reconcile the two.

I'm sure sometime in the future I'll cross more fully into the sick world. And hopefully at some point, maybe post-tx, I'll be able to re-enter to some degree the healthy world -- where people can make plans with friends for a week or two weeks or even a month out and not have to worry about being forced to cancel last minute or whether they'll have to lug their IV antibiotics along. But for now I'm living on the border between the two, still grateful for the time I get to spend doing all my favorite normal things, but always aware of the threats of illness. I guess we cystics are border people by nature, although at times the lines seem more blurred than others.

Which, to bring it all back full circle, is another reason I'm so glad that I've become such a CF junky: it's nice just to know that there are other people out there straddling these two worlds right there with me.

Tuesday, February 17, 2009

Mush, Mush, and More Mush

So okay, I know I haven't tackled this thing in over a week, but in my defense that's been mostly to spare whoever reads this blog from slipping into a boredom-induced coma. My brain has been pretty much mush since starting IVs last Wednesday and it just didn't seem fair to drag everyone else down with me. Because let's face it, most of us have enough to worry about without adding Irritable Blog Syndrome to the list (and for anyone who cares, yes, I did in fact fight with myself over that terrible pun, but what can I corny side is WAAY stronger than the rest of me). And then I realized that if any group of readers is likely to forgive my abx haze, it's probably you guys. In other words, bring on the mush.

A couple of updates from last time:

1) As predicted, I'm back on the needle and under the gun. Basically I was feeling like utter shite and getting short of breath doing those basic life things that we all take for granted 90% of the time, so I knew it was time. Amazing how IVs start to look real good real fast when you can't make it to the bathroom without stopping for air. So anyway, we're doing a longer course this time -- probably around 6 weeks -- to hopefully wipe out whatever has been causing these constant infections. At the very least I'm looking forward to 6 weeks when I absolutely cannot feel as bad as I have been in between IV rounds. It's so nice to be able to breathe! Or at least, sort of breathe anyway.

2) That last sentence kind of brings me to the next point, which is that I'm on the verge of becoming a full (or near full) time oxygen junky. My sats are just too low most of the time, staying in the very low 90s even when I'm healthy and resting. At my last tx appt my tx pulm told me I should be using the O2 "liberally with any activity", which I choose to interpret as whenever I exercise plus anytime beyond that when I both need it and it makes logical sense to have it on. In other words, I'll be bringing it to work to use in my office, but probably not to any meetings with partners anytime soon. And if I start to really need it 24/7, then I'll reevaluate when the time comes. But I have all sorts of fun ways to use the stuff now since my insurance paid for a home concentrator with fill system, three different sized tanks (think large, small, and extra small), and a portable Sequal concentrator for travel/office use. I know what a battle home O2 is for some insurance COs and believe me, I'm counting my blessings on this one.

3) I'm angry at CF right now. Not an all the time, pervasive, "can't see how much I have b/c I'm so focused on the negative" type of anger. And not a cry myself to sleep kind of anger either. It's just that I'm so frustrated right now b/c I can't stay healthy, even though I'm trying, and I haven't yet found a way to reconcile the way I want to live to my life with some of the choices that I'm having to make, even though I'm trying there too. It makes me mad to wake up in the morning already feeling tired, and then to drag myself out of bed only to find that it takes me an extra half-hour to get ready b/c I have to keep stopping just to catch my breath. It makes me upset to be trying to work and instead find my mind consumed with whether or not I can breathe, or what my resting heartrate is at just that moment. And I know a large part of this is me, and not really just the disease -- I know other people who have issues just the same or worse or even just different than me who still manage to get everything done -- but right now it's just hard.

Hopefully that last point doesn't come off as too whiney b/c that's not really the point. It's just that lately I find myself feeling these moments of just heartbreaking sadness or confusion or loss or frustration, and then everyone tells me how well I'm doing with it all and I want to just respond with "yeah, but it's HARD. It's so damn hard and there are so many of us -- 30,000 patients and countless more family and lovers and friends -- who are all trying to live with this sadness, trying to survive the bad moments and get back to the business of being human, and it's HARD. So thanks for the sentiment and the observation that you think I'm amazing, but honestly I'd trade all that inspirational BS just to be able to get up, walk my dog, eat a meal without thinking about the calories or the fat content, and get on with my day." And I guess maybe that's the rawest, most honest reason that I finally decided I had to write, mush or no mush...because in the long run the only people who can really get that need are others who feel it, and I'm so grateful to have people like that available to read these rants of mine.

So that's the update. The good news being that I feel so much better, and I actually had a great weekend with lots of friends and family and even an Opera at the Met (I know, super spoiled huh?). But it's undeniable that this disease has been affecting my life in totally news ways these past years, and I guess it would be unrealistic for me NOT to react to that, at least a little. Maybe even unhealthy. So I'll take the good news, and deal with the bad, and in the meantime I guess I'll just try and fight the inevitable mushiness that comes with middle ground. At least for the next five weeks or so.

Monday, February 9, 2009

Enough for Now

Okay, so I have lots of pictures. The inauguration, my sister's 30th bday, some random funny shots of my dog...I'll be uploading at least a few from each in the next few days or so. I know this blog is (mostly) about CF, but we all need a little break sometimes, right?

In other news, I'm sick again. Blah. I emailed my doctor about it today and we're going to touch base on Wednesday, but the plan is most likely going to include a 4-6 week course of IVs. We really, really need to find a way to stem the tide of these infections a little, and she thinks this might do the trick. No guarantees obviously -- this is CF we're talking about, after all -- but it's worth a shot. At this point I'll try pretty much anything. I just want to feel good and stay that way for a while!

Anyway, my deep thoughts for the moment are as follows:

1. It could be a LOT worse - I'm honestly feeling pretty lucky right now that I have access to this sort of care and have such a great doctor who I trust more than I can say. CF sucks either way, but knowing you have an amazing team behind you and the resources to get the care you need goes such a long way.
2. I get to do this all as an outpatient (I think). Yay!
3. I am SOOOO excited to feel better. Enough said.
4. By the time I get off IVs it should be just about time to break out the shorter sleeves and lighter-weight jackets. This timing is perfect for an arm-port girl like myself ;)
5. My mother is actually in town this week to attend the Westminster Dog Show (a friend of hers has a dog showing) and then to take my sister and I to the Opera. This is either a coincidence or part of a master plan that's greater than my own, but either way I couldn't be more grateful!

I honestly can't wait to devote a little space on this blog to talking about something other than pseudomonas. I can't wait to walk my dog and not have to count the steps back to my apartment. I can't wait to start exercising again. I can't wait to go out with friends and have energy at the end of the evening. I can't wait to go to work and be able to stride down the halls with my "confident lawyer walk" again instead of just concentrating on looking normal enough to pass as semi-healthy. I can't wait to actually be excited about meals. And most of all, I can't wait for the end of winter and some fun weather to enjoy the new health that I *know* I'm going to have after all this hard work.

On another personal note, my mom and I are taking my sister to Canyon Ranch in Massachusetts later this year as part of her bday gift. My sister and I (and maybe a girlfriend of mine, depending on how it works out) are also planning on a trip to Japan in the upcoming year. Both of these sound unbelievable to me...thinking that I might be able to do these trips and feel healthy enough to truly savor them actually makes me want to cry. I want pictures of me enjoying myself that don't make me wince later remembering how hard it was to breathe behind the smile.

So yeah, the IV part kind of sucks. But I have a lot of hope for this right now. And for now, despite everything else, that feels like enough.

Saturday, February 7, 2009

Fly Away Little Bird

Just a note to for those who knew her, or those who never had the pleasure...

Jenn, a fellow CFer waiting for a double-lung and liver transplant up in Mass., passed away yesterday morning after an unexpected decline. She was a very cool spirit whose kindness and generosity is demonstrated in her final wish to have her own body donated to CF research. I think it says so much that a young woman in her late 20s would be so gracious as to think of others when planning her last requests. Reading Jenn's story, and knowing her these past months, I can only say that she was an inspiration to all of us considering transplant -- she fought through many complications and uncertainties without ever allowing that struggle to eclipse her own light. So fly away, rest peacefully, and breathe easy friend. And whatever comes next, I hope your journey is a good one.

Please keep her family and loved ones in your thoughts right now.

Thursday, February 5, 2009

Keep On Keeping On

So today was my first follow-up with the transplant team since the end of my evaluation in August 2008. I wasn't sure what to expect, but I followed their instructions to the letter: I invited a support person to come with me, arrived 30 minutes ahead of time to complete any paperwork, and wrote down all my questions so I would remember to ask. Amazing how much mental prep goes into these "routine" visits. Anyway, I was pretty on the ball, but even so I was definitely nervous. It's funny - I've been in and out of this hospital every month at least for a few years, but ever since I started visiting with the tx team it's just taken on a whole new meaning. I don't even really know what I mean by that. I guess just that this is now the place where I've cast my lot - I am choosing to make this hospital part of the biggest medical experience of my life. I didn't have to do that -- there were plenty of other places I could have gone -- but I chose Columbia Pres. and now that is truly, more than ever, "my hospital." I think I would feel very strange leaving it now. So yeah, walking in there today was both comforting and a little overwhelming.

The meeting itself was pretty much what I expected. I filled out a form to update the team on any changes or hospitalizations since my last visit. Then I went back and met with the tx coordinator. She and I discussed what tests had been done, what still needs to be done, and what the listing process will look like. At one point she had to leave the room to take a page and she handed me my eval sheet and told me I could look at it. So funny - it was a narrative of my complete medical history, including comments from doctors and other random facts. It talked about my gene mutations, my hospitalization history, my compliance with meds. Parts of it were funny (like the quote "she denies any pregnancies" - as though I might have hidden the truth), and parts of it were more sad ("she had a relatively healthy childhood with minimal CF-related infection until age 14, at which point she began progressive decline"). Parts were just interesting. As a whole, I was pretty fascinated both that the document existed and that they let me see it.

Moving on, the tx coordinator told me a few really good things. First, she told me that my case had been presented and accepted, meaning that if I complete the testing she would be authorized to list me tomorrow, if needed. That was awesome news, and something that just sort of took a weight off to hear. Something about the whole "acceptance" process seems weird to me - I'm glad to know I "passed" I guess. Anyway, she also said that I should start coming to the patient education group, and gave me a schedule for that. It's presentations by the social workers, nutritionists, surgeons, and other members of the team on pre- and post-tx living, as well as on the surgery itself. So I guess I should start making some time for that in my schedule, since I have SO much free time already (haha).

My tx pulmonologist came next, and he and I chatted about my recent infection history, which has to say the least. Basically he said that even though my PFTs are still in the high range for listing, it would be worth completing everything. He seemed generally nervous given how much I've been sick lately. I don't blame him - I'm nervous about that too. He is also unbelievably kind and wonderful to talk to. I am so blessed to have incredible doctors. But the plan as of now is to wait, but to keep a closer eye on things. I have to see him again in three months, and I get the feeling that if we can't say at that point that the recent infection blitz was just a one-time fluke, we may move forward. I dunno - speculation on my part there. But it was pretty much what I expected.

Finally I met with the tx surgeon, who gave me the whole rundown on what to expect from tx - the complications, the possible outcomes, the statistics, etc. It wasn't news to me for the most part, so I was able to stay pretty calm. I do think that there's a part of this whole process that is kind of in your face (for lack of a better term) on purpose - they tell you the worst possible outcomes b/c they want to weed out the patients who can't handle those possibilities. And maybe that makes sense, but of course it's always scary having people talk about tx and suddenly realizing that those are YOUR lungs they're talking about removing, YOUR chest that might sit open for 6-8 hours, YOUR life that will hang in the balance. It's oddly clinical in the moment, but sitting here processing it now I kind of get shivers. And frankly I think that's good, because I think it means that I'm feeling this as real and not just removing myself from the process. Or at least that's what I tell myself now.

I will say that the events of this week made this appt all the more surreal. Knowing that a few states away Jenn struggles to hold on to life, while down south Garran takes another breath with his new lungs - it was all very intense. They are both so brave, and such role models. Both of them have fought so hard to live, to get the new life they both deserve so much. And it saddens me to no end that one of them might never get that chance, but it also makes me all the more determined to pursue this process. I don't know when tx will come for me -- I hope it's not for a while yet even despite some of the signs -- but I do know I WANT it. I want it and I will fight for it. And I hope that if I'm lucky enough to get it, I will be wise enough to cherish it.

All good thoughts and positive energy to those who are fighting tonight. Your courage and strength is beyond this tired New Yorker's humble understanding.