The Break Up

My love affair with my lungs began just over twenty-six years ago. November 1981, to be exact about the whole thing. Like so many other relationships, this one started out nearly perfect. Oh sure, there were the usual getting to know you bumps in the road—the awkward first few moments as a couple when neither of us knew exactly what to do with ourselves—but then I opened my mouth for that all-important first wail and the honeymoon began. From then on, my lungs and I were, quite literally, inseparable.

As often happens, though, I think my parents knew there were going to be problems down the road long before I did. My love of my lungs and the life they gave me was too all-consuming to allow for any doubts or questions. And so, even after the pneumonia that led to my diagnosis with cystic fibrosis at six weeks of age, I remained deeply and utterly committed to making things work with my lungs. Of course, there were bad moments. Moments when I sat on the floor of my bedroom, the nebulizer mask on my face smothering me with its salty mist, and thought wistfully of what it might be like to have lungs that weren’t quite so demanding—that showed some interest in the activities I so much enjoyed, like hiking, horseback riding, or downhill skiing—but I never seriously considered ending the relationship. How could I? After all, my lungs and I were a pair. For better or for worse. In sickness and in health.

And so, year after year, I shouldered the burden of meeting my lungs’ increasingly controlling and manipulative cries for attention. I spent countless dollars on expensive machines to make them happy, soothed them with special medicines and salt water therapies that left the rest of my body screaming in indignation, consulted some of the best minds in the country for advice on how to put the spark back into our relationship, and spent more than a few sleepless nights tearfully begging them to put in just a little more effort. And then, sometime around the twenty-fifth year, the truth finally dawned on me:

I was in an abusive relationship.

So it came that, a year later, I am finally ready to begin the process of getting out of what has clearly become a bad situation. I’ve already negotiated the terms of the divorce for myself in a way that only a lawyer could. I’ll keep my metaport and my ineffective pancreas if my lungs are willing to take the mucus and the scarred airways. I’d like to keep my doctor but am only too happy to relinquish all custody rights to the pseudomonas (although I worry that those little critters might insist on some form of visitation). For all I care my old lungs can take all the fancy medicines and machines I bought for them when they leave. And, yes, my lungs will be moving out. This whole living together thing isn’t really working so well anymore.

Of course, I have my doubts. I worry that I might never be able to find another pair of lungs that I can love like these. That I might leave one bad relationship only to find myself struggling against an even worse one somewhere down the line. I worry that I’m not strong enough, that my neediness after the separation from my lungs will become a burden for my friends and family, or that I might not be up for the challenge in some crucial way. Most of all, like all newly single girls starting over yet again, I fear the overwhelming possibility of rejection.

But ultimately, I know that this is something I at least have to try. I have to trust the advice of all those people who love me well and who insist that I can do better. I have to trust myself that this is right and that I deserve a chance at life with new lungs. In short, I have to go back to believing in fairy tales…or at the very least in happy endings.

So here I am. Twenty-six years old and just starting out on what is likely to be a very long journey to free myself from the lungs I once loved. And still love, to be honest. Despite all the fights and the power struggles and the petty revenge, my lungs and I have always been there for each other. That sort of bond doesn’t break easily. I know I will be sad to lose them, but I will not be sad to see them go.

Breaking up really is hard to do.

Maybe

Today was a pretty normal day. I got up, did treatments, went to work, worked excessively long hours (as first year attorneys at NY firms typically do), and then came home with just enough time to relax for an hour or so and then get in all my treatments before midnight so I could go back to bed. Not that every day is like that - promise I do have a social life - but that's a pretty typical weekday schedule for me.

But now tonight, with all my treatments for the day behind me, while I'm feeling good after finishing IVs, after having walked home from work on a lovely city night that felt more like September than muggy August, and with my 43% still lingering in the air around me like a protective aura...tonight I'm finding myself sitting around thinking about the maybes in my life right now. Not the typical CF maybes - the ones we all live with day to day: maybe I'll get sick, maybe I shouldn't go out again tonight, maybe I won't be able to make it through the night without a coughing fit - nope, none of that. These are the really, really good kind.

Maybe this new Aztreonam will keep working so well for me.
Maybe I'll be able to go another 3 month (3 whole months!!) without doing more IVs.
Maybe 43% is my new baseline.
Maybe I've finally hit a point again where I can have some control over my health.
Maybe I'll make it to 30 without a transplant.
Maybe I'll meet the love of my life and he won't mind that I have this deadly disease, sleep with O2 most nights, and have semi-failing organs.
Maybe my friends and family will be able to stop worrying.
Maybe I'll live to be 40...even 50...even older...

It's all kinda overwhelming. To be honest I'm not used to thinking about all this because I tend to take life as it comes and to focus really hard on doing whatever I can in the moment. That's why it's so important to me to work, even though "maybe" I could be healthier longer if I slowed it down. I would rather live my life now, the way I love it, than hedge my bets too much and be overly cautious in the hopes of sustaining myself into a lukewarm future. And "maybe" that's morbid or immature, but it's true.

Still I'm not beyond a few daydreams, even at midnight on a random Wednesday night. So here's to the maybe, here and now.

Evaluation: Day 2

Okay, so back to the eval...

Day 2 could have been renamed "x-ray day" b/c that's pretty much it. Started off with a regular chest x-ray, the highlight of which was when the probably 18 year old x-ray tech, after confirming that I had CF, asked me if I had ever had a chest x-ray done. Hmmm...guess they don't mention CF much in x-ray tech school. I came very close to responding that I was probably doing chest x-rays before he was even born, but he was kinda cute and I wasn't wearing a shirt (hospital gowns don't count!) so I decided it probably wasn't the time to get too snarky. Next came the CT scan. Again, total yawn. A transplant evaluation can seem a lot like a typical day to a CFer.

The next text was called, I believe, a "lung perfusion scan." The doctor administering this caught my attention when he pulled me aside and asked me whether I had any contact with pregnant women or infants on a regular basis. Um, no, but what does this have to do with a lung transplant? Turns out that this particular test involves, wait for it...inhaling radioactive gas. Wow. So there I was, in my hospital gown, hanging out in the waiting room in an NYC hospital and clutching a piece of paper that explained to anyone willing to listen that I was, in fact, radioactive. Apparently the paper is useful if you happen to try to go through airport security within a couple of days of the testing. Anyway, this scan was not pleasant. First, the breathing of the gas was done through something resembling a bi-pap mask, designed so no room air could get in. It felt a little suffocating, honestly. Second, I had to lie on my back and try not to cough for about 5 mins. Did I mention I have CF? Not gonna happen, lady. Third (and this is the wimpy part), the x-ray machine looked like a gigantic heavy cube, which they then lowered by mechanical arm down over my chest until it was about 2 inches from my face. I thought I was going to be crushed. So on Day 2 we learned that claustrophobia + suffocation + lying flat on your back = super p*ssed off cystic. Valuable lesson. We also learned something about my lungs, I think, but I was a little sketchy on those details.

The second half of Day 2 was all about the social aspects of tx. I met with the social worker, nutritionist, and psychiatrist for the tx team. The first two actually overlap with the CF center at Columbia Pres, so I knew them just fine. I was stunned to learn that they recommend a low salt diet post-tx. Are you kidding me? I think if they make me do that I will end up licking my arm just to flavor my food! I seriously live on salt. But yeah, that was (sad) news to me. And then the psychiatrist basically told me about pre- and post-tx depression, which made a lot of sense. Right now I'm lucky enough not to suffer from depression (knock on wood), but I totally understand how chronic illness or major life change could bring it on, so we're going to keep an eye on that in general.

I finished Day 2 by going to Wendys and adding extra salt to my fries. Because apparently I need to get it while I can.

On a totally separate note (kinda), I finished my IVs today after only 2 weeks! And to top that, my FEV1 was at 43%! I almost cried, I was so happy. Honestly, it made up for all the lung perfusion scans in the world!

Stay tuned for Day 3 aka "The Day of 1,000 Exercise Tests" ....Okay fine, so it was more like 2, but whatever.

Pics, ports, and ponderings

Wow, awesome responses to the interactive blog. I'll be compiling some of the responses I received from people I know (sent the Q&A out by email) and people from the blog into a new post soon. I think we'll have some really good info, and hopefully it will give all us "pre-txers" a chance to get some answers from all the wise post-op people out there!

In the meantime, I promised I would answer my own questions, so here goes:

Group 2: Pre-Tx CFers/Patients
1) Age?
26
2) What stage are you at in the tx process (i.e., evaluated, listed, etc)?
Evaluated but not listed - my chest is still 100% natural, baby.
3) Why did you decide to have a tx?
Lack of a better option I guess. I'm totally freaked out by the prospect, but also willing to do whatever it takes to stick around here as long as I can. And I'm excited about getting some lung capacity back, but I'm not so naive as to think it will solve all my CF problems.
4) What are you most excited about for post-tx life? Most scared of?
I'm from CO and I used to love to hike in the mountains. I can still exercise pretty well, but not so much at high altitudes. Stories I've heard from post-tx CFers make me super excited that I'll be able to breathe again on top of Pikes Peak someday! As for being scared, I'm kinda freaked out by surgery in general. Been lucky for a CFer, never having any sinus surgeries or anything besides a random appendectomy earlier this year. So such a big surgery looming in my future is a big deal to me.
5) What is one question you would like to have answered about tx?
Well, considering I made up all these questions, a lot of them have already been answered. But I would be curious to know what the process of "reinflating" or expanding the lungs was like for people. I hear about this a lot, but I don't really get what it means.

Finally, I've debated a lot about this next thing because I REALLY don't do the "sick pictures" thing, but I know given some recent sh*t that's gone down a lot of people are understandably skeptical about internet blogs in the CF community. I for one have been super impressed with how well most of us have kept up faith in others throughout all the madness, but since I know this blog deals with some really sensitive issues, and because I want people to feel totally comfortable sharing and taking advice in this space, I've decided to post a couple of quick pictures of myself and my gorgeous IV line I have in right now. And even better, this will give some of you probably your first glimpse of the rare and ellusive "arm port" - in which I am a HUGE believer.

(Ignore the fact that I'm laughing. Do you have any idea how hard it is to take pictures of yourself with bothyour face and your entire upper arm showing?)






Shout out to Apple, bc these pics were also taken with my brand new iphone. I'm SUCH a fan.

Interactive Blogging: CF and TX (Please Share!)

So when I started this blog I wanted it to be about tx. In part this is because it's the biggest thing going on in my own life right now, but it was also in part because I know that leading up to my decision to get evaluated I was ALL ABOUT cruising the web to learn from others. And CFers have SO much to share in this regard...seriously, the people I have "met" online have been SUCH an amazing resource for me. Anyway, that gave me the idea that I might be able to be somewhat "interactive" with this blog. I'm basically a social person, and I get a little sick of always writing about myself, so I thought maybe I could give other people a chance to share.

Here's the deal: answer as few or as many of the following questions for your "group" as you feel comfortable with. You can either do it in the comments section here or email me directly at beatty_112@yahoo.com (with or without your name) and I'll post the info on the site anonymously. I've also enabled anonymous comments so feel free to share without identifying yourself. If we don't get answers from people who have "been there, done that" then I will try and get some answers from people I know who have experience with tx. Tell your friends and get them to share too! Just keep in mind that you're helping your fellow CFers (or people with other lung diseases) and making this blog more interesting :)

Group 1: Pre-Eval or Non-Tx CFers/Patients
1) Age?
2) Do you foresee tx as being something you might need in the future?
3) If so, would you consider tx?
4) What factors might influence that decision?
5) What is one question you would most like to have answered about tx?

Group 2: Pre-Tx CFers/Patients
1) Age?
2) What stage are you at in the tx process (i.e., evaluated, listed, etc)?
3) Why did you decide to have a tx?
4) What are you most excited about for post-tx life? Most scared of?
5) What is one question you would like to have answered about tx?

Group 3: Post-Tx CFers/Patients
1) Age?
2) When and where did you receive your tx?
3) What was the best part of your tx experience? Worst?
4) Name 1 change post-tx that you DIDN'T expect or weren't fully prepared for?
5) What is one piece of advice you would share with others about tx?

Group 4: Caregivers
1) Relationship to CFer/patient?
2) Do or did you feel involved in the tx process? How so?
3) What is/was the hardest part for you of helping a loved one through tx?
4) Any questions or advice you would like to have answered/share?
(BTW, caregivers are awesome. Big shout out to all of you!)

Okay, here's hoping we get some responses. Come on guys...I'm not nearly exciting enough on my own!

(PS: Yes, I promise I will answer the questions too!)

Evaluation: Initial Consult and Testing Day 1

Okay, I've been promising this for a while, but I finally feel up to sharing about the eval process so here's goes. And before I start, let me just remind anyone reading that this is one person's experience at one center. It may be very different somewhere else...I just have nothing to compare it to so all I can do is recount what happened to me. And, to be honest, it wasn't NEARLY as scary or emotional as I thought it would be. It was long though - spanning three full days of medical tests - so I think it's easiest to break it down by day and go from there.

Also, just a reminder, I started considering eval in January. I then scheduled the initial consult, but with insurance approvals, work schedules, and whatnot I didn't make that happen until May 9th. So I had some time to process, which I think was helpful. Also, my mom and dad flew in from Denver and my sis lives in NYC, so they were all present for the initial meeting with the tx doctor. I can't even begin to tell you how helpful this was to have the support. Basically, we all met in an exam room with the tx doctor who will be taking over my pulmonary care from my CF doc (I will still see my CF doc for other aspects of CF). He was very nice and surprisingly able to handle my family's very sarcastic sense of humor. We asked him nearly every question in the book, from "how long is your wait once a patient gets listed" to "how long will I be on the vent after tx" to "what is your policy on communicating with the family during the surgery?" He was super helpful - even when he couldn't give a simple answer, he gave as honest of an opinion as he could. It was great, and really laid a foundation of trust. What was helpful to me was just having a list of questions to ask on hand. I literally walked in with three pages of questions and checked them off as we went. My sister "stole" some of my questions and I gave her crap not thinking up her own!

Then, July 31 (again, the delay was mostly scheduling snafus), I had my first "testing" day. We decided ahead of time to do all the tests except the right heart catherization - my doctor said there was no use doing this test since I didn't plan to be listed right away and we would have to redo it later. So no scary heart needle test for me. But everything else I had pretty much done before. They sent me a schedule ahead of time so I knew what was coming and, again, my mom came out to the city for the process. (Yes, I know, my parents are saints, but they also REQUIRE you to have some support person there - they are evaluating your support as well!) Day 1 was basically an echocardiogram (like an ultrasound of your heart, with a "bubble," which was weird), followed by tons of PFTs (the kind in the box), followed by an arterial blood gas test, and then outpatient blood draws and an EKG. Piece of cake.

For me, the biggest hurdle for day 1 was just hearing people say "lung transplant candidate" and having to hand over requisitions that all said "transplant evaluation" on them in big letters. Talk about a bit of a shock. I even had one nurse at the echocardiogram unit ask me when I was scheduled for surgery. Um....hopefully not for a LONG time. But still, it wasn't so bad. Mostly just tiring and annoying, because we all know that hospitals aren't the most efficient places ever. Also annoying was the fact that they all call me "Betty" at the hospital. I'm guessing this is because my last name is "Beatty" and they think "Piper" is way too weird to be a first name, but still...Betty? At least the doctors can get my name right!

I think the best thing about my experience was actually the lag in time between the initial conversation and the initial consult, and then between the consult and the actual testing. Yes, at the time it felt like it was just drawing out the already painful reality, but looking back it was such a lifesaver to have time to process and accept that I might need a tx before diving into the testing. Because honestly, you don't want to be any more stressed than you have to be for this process. It was also helpful that I felt pretty good at the time I was getting evaluated (although two weeks later I'm on IVs!) because I would NOT have handled it well had I been too sick. So I guess that's a good reason to get tested early...I cannot imagine doing the evaluation if you actually needed a tx right then. Too much to handle when you're already scared or really ill, in my opinion.

So that's day one. More fun with days 2 and 3 coming soon!

CF = Completely Fake?

I wasn't going to post anything about this at all because I didn't want to add attention to a nasty situation, but now I can't sleep and I figure I may as well just get it off my chest.

Quick update on me: I'm back on IVs because things were just getting a little too junky. It's annoying because my PFTs were basically at baseline during my tx eval (which I promise to write about soon!), but I was feeling like crap every night and more or less couldn't breathe right before treatment times. Too much nasty gunk. So the abx are helping and I finally feel clear again. Yay! And if it weren't for the itching, which I think is a reaction to the Zosyn, I'd probably be asleep right now.

But I'm not, and besides the itching thing I think part of what is making me lose sleep right now is actually the internet. Um, no, not because I'm so in love with this blog that I just had to post before I could sleep, more because some things have taken place on the internet lately that have really disturbed me. If you're aware of the whole "Gina" issue than great; if not, long story very short is that a well-known website dedicated to a young woman with CF who was apparently trying to survive a transplant despite unbelievable setbacks, was just recently exposed as a fraud. Only when I say this woman's problems were "unbelievable" I should probably add that we all believed her. Thousands of people, most of whom likely had some knowledge of CF, were completely fooled by this woman. And I was definitely one of them.

The whole thing is really upsetting to me, for I think pretty obvious reasons. I did not ever claim to "know" Gina, although I checked her blog frequently - I did so out of sympathy and curiousity, rather than friendship. I wasn't burned nearly as badly as some of the others in the CF community. But I was reading this site as I was going through the tx eval process, I was filing away Gina's "experiences" as realisitic possibilities. In short, her story both gave me hope (that someone near death and on ECMO could receive a very last minute tx despite massive infection) and scared the sh*t out of me (that such horrible results could come as a result of tx). I know parts of her story really could happen; they just didn't happen to her. But I am left trying to reconcile the fact that I know her story was fake with the fact that I have been incorporating her experiences into the formation of my own view of tx. It's hard to just discard all that info now - I honestly don't know whether some of my feelings are related to Gina or not. I have to assume her "story" colored my viewpoints on the subject, but I wouldn't know where or how to begin subtracting that color from the picture I have painted now.

The online CF community has lost of lot of trust in the past few days. The sad thing is that online support is pretty much the only way groups of Cfers can "meet" without risking serious cross-infection. I have been debating pulling out of the forums and groups for the past couple of days...I just don't want to risk more breaches of trust and serious misinformation. On the other hand, the forums are so helpful (example: tonight I returned for the first time since the whole mess started to ask for advice on this stupid itching). There are some genuinely wonderful people there. But they are people whose trust was violated, and who are in turn capable (sad as this is to admit) of violating the trust of others.

I guess we found yet another potential side-effect of living with this disease: Cystic Fibrosis Related DRAMA.

The News...

Okay, so back in January my CF doc suggested that it might be time to start looking into being evaluated for transplant. Her reasoning was good: the evaluation process can take a long time, many people find it hard to get evaluated once they become super sick, and it's emotionally draining so better to get it out of the way earlier and have more time to process the whole ordeal. All very solid points and very logical. Basically the exact kind of thinking that normally makes me jump right on board. But this time...not so much. The news hit me like a ton of bricks. Sign up for four days of intense medical testing all so some doctors I've never met before can determine if I'm qualified to have my lungs pulled out of my body and replaced with those of a stranger? Was she crazy? My actual reply was that I would need some time to think about it and talk it over with my family, but my internal response was more along the lines of "get me OUT of here!"

Deep down I knew she was right. Since early 2007 I have been on and off IV antibiotics about every couple of months because of serious lung infections. I'm now on supplemental O2 at night to help my body rest. And, probably the most serious indicator, my FEV1 hovers between 38 and 44% when I'm healthy. I don't even want to go into my numbers when I feel sick. Factor in that just about 5 years ago my baseline was FEV1 85%, and it doesn't take a genius to figure out that my CF has progressed extremely rapidly....this was definitely the time to be considering all of the options. But still, lung transplant? Wasn't that for people who can't work or leave their apartments? I just kept going over and over the reasons why lung transplant didn't make sense in my life: I worked, I exercised, I could go out with friends until the early hours of the morning! It all just felt way too surreal to be true.

I'll admit it took me a few days to get past that initial phase of feeling overwhelmed. Actually, that was 7 months ago, and I still feel a little overwhelmed to be honest. But basically I went home, called my family, and then started the (very) long process of determining whether transplant was the right road for me. I gave myself some time to grief the thought of losing my current lungs (even at their worst they've still always been there for me); I spent hours online and on the phone getting stories of lung transplant from other cystics, reading up on the procedure, and generally gathering information; and I did a LOT of thinking. What I discovered was that I definitely wanted the option of lung transplant, and that therefore I needed to be evaluated. I also finally came around to the thinking that it was better to do this now, while I'm relatively healthy and have a fairly normal life to fall back on, than to wait until everything falls apart and try to brave the testing process then. CF is unpredicatble, I know that, and anything I can do to stay two steps ahead of the game is definitely worth the effort.

Introductions

As a first time blogger I have to admit that I'm not entirely sure what to do with this space now that I've created it. I' guess this is the part where I tell everyone that not very big on the whole computer thing but that I wanted a place where I could keep my thoughts and experiences about this newest stage of my life. But rather than bore you all with that, maybe I'll just introduce myself so we can get on with it...

My name is Piper and I'm a 26-year-old living with cystic fibrosis in Manhattan, NYC. I'm also a lawyer, a sister, a daughter, a friend, a former competitive showjumper, an insatiable reader of all great literature and some great beach reads, and a whole lot of other things. I'm sarcastic, have a slightly wicked sense of humor (just ask any of my nurses), have as much fun with my life as possible, and am constantly exploring New York and the rest of the world with my wonderful friends and family. Long story somewhat shorter, I started this blog because I recently underwent an evaluation for a double-lung transplant at Columbia Presbyterian Hospital, and I wanted somewhere to keep track of the whole process. But more than that I also just thought it might be a fun way to let people know what's going in my life, and maybe even to learn from others who might have been through similar experiences.


So that's about it for now. Promise to write more soon.