Monday, December 24, 2012

The Night Before A Beatty Christmas

'Twas the night before Christmas
And all around here
Not a creature was stirring
Not even Sam Bear.

The meds were all taken
T-cells were suppressed
In SUNO pajamas
The whole fam was dressed.

The TOBI was over
The meal was done
And we were all dreaming
Of holiday fun

But before all the presents
And spreading of wealth
Let us say merry Christmas,
Peace to all, and good health!

With love, beautiful people.

Piper, Sampson, and the Beatty Family

Sunday, December 16, 2012

On Swimming Through Fires

It's been kind of a rough week in CF land. And in the interest of full disclosure, please read this blog in its entirety before sharing with any young CFers or other children. It's not obscene or inappropriate, but it does deal with the harsher parts of cystic fibrosis.

One of the most painful realities of CF is that the more involved you are with the community -- the more you take the time to share with, learn from, and work alongside of other people who share your disease -- the more likely you are to come face to face with the super hard fact of how rotten this little monster really is. Or, to put it bluntly, the more likely you are to know people who will die. Of your disease. Of your complications. Of parts of your illness that you may or may not experience yourself, and may or may not survive if you do.

And let me tell you guys right now: it sucks. All of it.

Whether it's watching desperately from Facebook as a friend dies awaiting lung transplant and knowing that you were lucky enough to be given that gift, or watching from across the hallway as one of your friends struggles with severe complications FROM lung transplant and knowing you haven't necessarily escaped unscathed after all (and you certainly can't let your guard down NOW, if ever) -- well, it's all pretty much equally sucky, and it's all very much part of day-to-day reality of cystic fibrosis. And just because it's a side of CF that most of us don't like to talk about all that much, that doesn't make it any less true. For any of us.

I sometimes think about the lines from Mary Oliver's beautiful poem Dogfish:

And nobody gets out of it, having to
swim through the fires to stay in
this world.

And I know that there is truth in what she writes here, that nobody gets out of it.  I know that CFers aren't alone or unique in facing struggles -- even when it seems like we are, or maybe like we should be. I know that even a disease where surgeries become "no big deal" and the only hope for realistic, long-term survival is either to replace your vital organs or somehow find a way to modify your very genetic makeup, the things that make you what you are, is still not the be-all-end-all of human suffering. I know that when we start to think like that about CF it becomes isolating, paralyzing, and even self-desrtuctive. I know all that, and I know that every single one of us, everyone and everywhere, will have a moment when we are "swimming through the fires", and that mine are no more or less important than anyone else's. And nobody gets out of it, after all.

I know all that. Of course I do. But I just wish I didn't have to know it so damn well.

My heart goes out this holiday season to all of us who are swimming through fire, and especially to those who swim in my particular river. To my friend who celebrates the life of his sister taken far too soon, my beautiful girl who lies fighting on life support for her 1-year-old lungs so she can marry her fiancé next summer, my super smart friend whose heart is now too weak (barring a miracle) for new lungs to come, and all the rest of our wonderful underground who need help, love, positivity, or just a simple breath of air. You all are so much my current, my river, and my healing water that this fire, hot as it is, will never stand a chance.

And I will swim with you for as long as it takes.

Wednesday, December 12, 2012

Webinar: Providing Best Care for Individuals with Cystic Fibrosis

Today I had the pleasure of "attending" a webinar as part of the American Thoracic Society's Cycstic Fibrosis Week 2012, a joint product of the ATS and the Cystic Fibrosis Foundation. The topic of today's discussion was "Providing Best Care for Individuals with Cystic Fibrosis: A Partnership with Patients and Families." It featured an informative lecture by Dr. Michael Boyle of the CF Center at Johns Hopkins University and a Q&A period featuring some great contributions by CF patients, parents, and care partners. Special kudos for this event go to a woman I am proud to call a friend and fellow CF patient, JD, and Emory grad -- as well a phenomenal patient advocate and manager of the CF Legal Hotline -- Beth Sufian.

I encourage all of you to check out the taped version of the webinar yourself, which I'm told will be available at the ATS cystic fibrosis week website (link provided above). I also encourage you to get involved in CFF-related educational programs and webinars in the future, which are often provided through the CFF and through programs like the CFRI's CF Discovery Series. For now, though, here are some of my main takeaway points from this discussion. I should emphasize from the outset that these are MY observations and have not been discussed with Dr. Boyle, the CFF, or the ATS. Nothing below is meant to serve as anything other than my personal understandings and opinions.

Got it? Awesome. 

Takeaway #1: Yes, Compliance, Care, and Medication DO Matter!

Dr. Boyle spoke about a study on CF homozygous twins (meaning identical twins with the exact same genetic makeup) that showed a strong correlation betweens genetics and lung function in CF patients. But he also spoke about a surprising curve ball with this study, which was that identical twins with CF showed a far more significant disparity in lung function AFTER they separated and moved into separate homes. This indicates that non-genetic factors like compliance, care (wether self-care or care-center driven treatments), environment, and medications DO actually matter in determining outcomes for people with CF. And yeah, I know what you're thinking, which is probably a great big "duh, Piper, my mom told me that when I was, like, 5 years old!" kind of moment, but really: knowing and seeing actual evidence that the choices we make in life and in health make a difference to our overall well being even beyond the genetic card we happen to be dealt is always worth repeating.

Bottom line: when it comes to our bodies we are neither fully in the driver's seat nor fully passive, and the decisions we make are always going to have an impact. Most importantly, we can choose to be honest (yeah, really honest) with ourselves, our care partners, and our healthcare teams in addressing these issues.

Takeaway #2: Good Care Centers Are Active Care Centers

Dr. Boyle spoke about a care-center study that involved observation of CF care centers recognized for having some of the best therapeutic outcomes across the country. The results of this study were too numerous to really discuss each one in full here on the blog, but for me a couple of big findings were that 1) good care centers take time before the patient arrives to discuss each case and come to an agreement on what issues need to be addressed during the relatively short patient visit (optimized patient care) and 2) Dr. Boyle's statement that good care centers are empathetic, compassionate, and caring without necessarily being too nice. What he meant by this, apparently, is that good care centers are compassionate in their care but refuse to be "nice" when it comes to aggressively treating infections or drops in PFTs, nutritional issues, or the need for patients to be proactive about their own healthcare. Wow, what a cool distinction. 

What WE, as patients and families, can do in this issue is be involved (joining or forming a patient/parent advisory board at your clinic, for example, is a great first step), talk to our doctors about these sorts of findings and about ways to improve our personalized care, and basically lead by example. If we want our doctors to maximize the value of our time in the office, for instance, we can have our own "pre-visit meeting" with our families, our partners, or even just ourselves and a journal and consider questions we need answered, prescriptions we need filled, issues we need addressed, and concerns we want acknowledged. This is a great way to ensure we actually get what we pay for in terms of clinic visits, and bonus points if you can really impress your doc and make him/her take notice of your extra effort!

(Dr. Boyle also spoke at length about the fact that good care providers TALK TO PATIENTS. Duh moment number 2, I know. But what I took away from this is that your docs should be discussing your test results, x-rays, cultures, CT scans, etc WITH you, not just making decisions based on undisclosed results. If they don't do this, ASK. It's your body, for goodness sake, and you deserve to know what's inside of it as much as they do!)

Take Away #3: Transition is Tough, but Not Transitioning is Even Tougher

My personal favorite moment of the webinar came when Dr. Boyle showed a joke slide of two parents pushing a full-grown man in a stroller and joked that he thought maybe this family was on their way to CF clinic. Haha, right? But seriously, guys: HALF OF THE CF POPULATION IN 2012 ARE CF ADULTS. You, or your child, are/is going to GROW UP. It's up to families and patients alike to transition this care in a workable and meaningful way, and preferably BEFORE the patient his 18, 19, 20, or whatever and leaves the family compound.

The simple truth is that YES, you/your CFer can typically go to college, and YES, you/your CFer can typically live alone, and YES, you/your CFer had BETTER get used to talking directly to doctors about your/his/her body early, because that stuff matters. Dr. Boyle suggested that the most successful transitions start early (he noted that "transition begins at birth") but obviously parents and patients need to recognize their individuality in crafting specific timelines. Suffice it to say that if -- barring special circumstances, of course -- no 18-year-old CFer should be unable to list his/her medications, speak to a doctor, understand this disease, or prepare his/her own neb treatment. You wouldn't wait until someone was 16, hand her a driver's license, and expect her to be able to navigate a freeway -- and nor would you want her dependent on rides for the rest of her life -- so why would you do any less training with something as important as healthcare??

We're not in Never-Never Land anymore.

Finally, I just have to mention Dr. Boyle's awesome point that the goal is not perfection: the goal is LIFE.  This echoes my personal best practices advice for anyone dealing with this little monster: don't beat yourself up for little mistakes or bumps along the road, and also never, EVER doubt have the power inside of you to make this work. You just do. Sorry, cystic fibrosis -- you'll never be as strong as we are.

So what do you guys think? Because no matter how many webinars or webcasts or whatever we attend (or don't) the real takeaway message here is that we've got to join the conversation. Our bodies, our disease, our little monster. Have at it, guys.

Thanks to Beth, the ATS, Dr. Boyle, the CFF who made this webinar a truly great educational experience.

Monday, December 10, 2012

Catching Up

Oh, hello again, beautiful people.

First of all, I want to thank everyone for the super sweet messages asking where I've been since my last post. I also want to apologize to anyone who might have been worried. When I started this blog, way back in 2008, I decided I wanted to write about "Life, Lung Transplant, and Cystic Fibrosis. And Everything In Between." At the time I was 26, working as an indentured servant -- um, I mean as an "associate" -- in a New York City law firm, and had just been evaluated for a double-lung transplant, for which I was at that point inactively listed. I was on a never-ending cycle of IVs, was using O2 to get around most of the time, and was pretty constantly in and out of the hospital. What I didn't realize at the time was that my life back then WAS more or less about lung transplant and cystic fibrosis. What was in between was a whole lot of work -- working to keep myself "healthy" enough to stay out of the hospital and inactive on the list, working to stay ahead of the curve at a job I loved even though I was barely able to keep myself breathing, working to fit in all my treatments and exercise and appointments and IVs and whatever else I had going on at the time. Working, more or less, to keep my head above water. You know, where the real air is.

In the past two and a half years, I've had what some people would consider a pretty wild ride post-transplant. There have been some bumps in the road. I spent most of the first year after my surgery STILL on IVs, for example, and I've been in and out of the hospital a few times even just in the last couple of months. I've had port infections and cath infections and lung infections and "normal people" infections and even some just really strange infections, although I'm told that nothing is really all that strange in the twilight zone that is lung transplant living. But the simple fact is that even with all this stuff, I have still experienced way more of LIFE in the past 2.5 years than I had for a long time prior to receiving my new lungs. And sure, Donor Bob and I have now been through a couple of ports and a couple of rough patches and a couple of rounds of photopheresis (with more to come on that score, lucky us!), but we've also been through a couple of birthdays I wouldn't otherwise have seen, had a couple of trips back to my gorgeous home state of Colorado (which I couldn't even visit anymore with my CF lungs), and shared more than a couple truly wonderful experiences and opportunities that I could never have imagined without the gift he gave me. And better than just having all those things together, we've LIVED them. Which is to say that I've been able to live again because of his gift, and I hope a little piece of his spirit lives on through me somehow. I know I carry his memory with me always, as well as his lungs.

So here's my latest update, lovely friends, which is that Donor Bob and I have been busy catching up since our last post.

We've been catching up with lung transplant with our continued determination against this silly rejection and the placement of our brand new Vortex port for photopheresis (scheduled for hopefully next week -- yay!).

We've been catching up with cystic fibrosis through the incredible opportunity to participate on a new project with the fabulous folks at the Cystic Fibrosis Foundation and through more and more chances to meet and be inspired by so many of you all -- our indomitable "cystic underground", for which we are forever grateful.

Best of all, though, we've been catching up on life by turning 31 together. Take that, CF! And, of course, through a thousand little miracles each and every day and the countless breaths that allow us to share in them together.

But don't worry, beautiful breathheads, we'll still be here to catch up with you all via this blog as often as we can. In the meantime though, we wish you all a very beautiful holiday season, the happiest of Hanukkahs, the merriest of Christmases, and the most joyous of new years full of hope, health, and as much of this wonderful life as y'all can possibly handle.

And Everything In Between. 

Piper, Donor Bob, and Sampson

Wednesday, November 14, 2012

This Little Light of Mine

Today was day 1 of my photopheresis odyssey. Well, okay, so actually it was day 1 of my photopheresis TREATMENT, since the "odyssey" really began several weeks ago when I started exploring new hospitals, conquering new catheters, and just generally raising hell with both the hospital scheduling gods and my long-suffering doctors. But really, I digress. Suffice it to say that this morning at 8:30 am found me sitting in my hospital's admissions dept, sipping bad coffee and ready to get this party started.

Turns out, photo is kind of neat in a very vampirish way. For those readers who might be squeamish about blood: STOP READING NOW! (Also, you may want to try a non-health blog next time.) For those of you who are totally cool with the three CF monsters of needles and doctors and blood (oh, my!), I present to you a Matter of Life and Breath original creation:

"Photopheresis, in Photos"

Quick refresher: I am receiving photopheresis treatment for recurrent rejection of the lungs, in my case characterized by three bronchs all showing some degree of ACUTE rejection coupled with somewhat wavering lung function. The rejection is, we suspect, somewhat related to my body's decision that it will not absorb prograf for weeks on end, until it suddenly changes its mind and absorbs ALL the prograf it possibly can. These sporadic bouts of high prograf apparently make me more susceptible to infection, while the more consistent low prograf levels make me a fun target for rejection. Don't ask about cyclosporin (the common alternative to prograf) because it's not on the table for me right now.

Anyway, to stop this cycle we've decided to take a different approach. Hence, the photopheresis. This is a photopheresis machine:

Kinda big and cumbersome and, turns out, also kind of tough to crowd into a single-person hospital room that is also populated by a visitor's chair, a table full of snacks (hey, I have CF, it's totally necessary!), a vitals machine and stand-alone thermometer on a pole, an IV pole, and a nurse's station complete with computer and nurse to monitor me throughout the 4-hour treatment. Wowza.

The treatment, by the way, is done (for now, at least) through this tiny little nothing of a catheter. Helloooo, sexy.

The treatment is called photo because of what it does. You've heard of a blood transfusion, right? Well, this is more like a blood transFORMATION. Basically the machine collects blood from my body and spins it to separate the plasma/"buffy coat" (WBCs, for simplified purposes -- "buffy coat" sounds to me like a nail polish color I would prefer to avoid) from the red blood cells. The red blood cells are then stored in a bag and returned to me with saline immediately. The plasma remains in a little cylinder within the machine and is saved until 4 cycles of blood draws are complete. Then the plasma is treated with a medicine to make it photosensitive and run through a tanning bed -- er, make that "UV-light tunnel" -- to create the desired change in my WBC make up. Check this out, guys. 

Top of the photo machine, complete with blood tubes (that's the red stuff, fyi) and the cylinder, which spins the blood to separate it and stores the plasma for treatment:

"Bags of blood" sounds like a bad 90s rock band. Instead, it means these suckers:

Tanning beds are a big no-no post-transplant. Unless, of course, they're for your blood:

Cool. So once the blood is separated, it actually runs through those tubes of UV light you see in the tanning bed. The initial collection takes about 2-2.5 hours (depending, because the machine slows down or speeds up to adjust to your blood flow somehow). The separation takes like 20 mins, but is done concurrently with the pulling -- so you end up with 4 cycles of pulling/separation lasting 2.5 hours total. The RBCs are also being returned to your body after they're separated. Crazy town, right? I wonder if they're lonely.

Meanwhile, the machine keeps you updated with a handy little screen that will say stuff like "collecting 1" (meaning collection cycle #1) and "returning 2." Oh, and remember those bags? Well, they start to look a little different. 

Those are actually just RBCs chilling, waiting to be returned to my body. 

Once the whole collection of the WBCs/returning of the RBCs is over, the machine treats the WBCs and then beeps to let the nurse know its work is done and she'd better take over. That's when the nurse removes the blood store and empties it into a bag, which she hangs and reinfuses via gravity drip -- far and away the most low-tech part of the process. This is what the blood cylinder looks like when it's outside of the machine:

Probably not going to enter that one in many "most beautiful picture" contests anytime soon. Yuckola.

I didn't take any photos of the treated WBCs being reinfused back into my body, probably because my sugar bottomed out at 50 before the thing was over. Not cool, photopheresis and/or hospital lunch tray. Not cool AT ALL. Other than that, my stay thus far has been uneventful. I'm actually managing ALL my own meds this time around because it's really not worth ordering a full regimen of CF and lung transplant drugs up from the hospital pharmacy when I'd probably be close to discharge by the time they got the order right. This makes me happy because I know I have the right drugs in the right doses at the right time already at my disposal. Love my hospital to the moon and back, of course, but what can I say? 

CFers just do it better.

Closing pic for this photo photo post is of the view from my hospital room window at night. The picture doesn't do it justice, actually, but a lot of that black stuff is river. The lights in the distance are New Jersey. Hard to imagine that just about two weeks ago that river was flooded over and those lights were dark from the storm. Hard to imagine that just about two years ago my body was flooded with infection and my own light was growing dimmer. Hard to imagine that this hospital has been a constant space of healing throughout both those disasters -- my city's and my own. Hard to imagine just how much a tiny bit of light can heal.

Love and light, beautiful people. 

Friday, November 9, 2012

On Changes and Catheters

This is me:

This is me with Sampson Bear, just because:

And this is my new dialysis-style tunneled catheter, courtesy of yesterday's visit with my old friend the vascular surgeon:

For some of you (even those of you with working kidneys), this probably looks pretty familiar. It vaguely resembles a double lumen central line, with the main difference being that this is a tunneled cath with one line (the red one) running into my artery and the other line (the blue one) running into my vein. The purpose of this catheter style is remove blood from one side so that in can be treated -- in my case with photopheresis (aka, UV light treatment to prevent chronic rejection) -- and placed back in my body via the second line. The line is different from a port access because, well, there is no port. The lines themselves tunnel into my body and can't be deaccessed unless the entire line is pulled. There's no needle involved and the entire apparatus can stay in place for several months or a year or whatever. It's basically a long-term blood treatment line.

Except when it's not, that is.

In my case, a dialysis-style cath was never really the goal, and it (hopefully) won't be staying in my body long-term. The original plan was to place a specific kind of port-a-cath called a Vortex port, which would allow photopheresis treatment through a more traditional port-style catheter (i.e., one that sits under the skin and can be deaccessed when not in use). My surgeon implanted the current cath in an attempt to "stretch out" my veins to allow for better access for the Vortex port. From my perspective, I went to sleep thinking I was getting a port and woke up with a dialysis cath and, quite frankly, wasn't a very happy camper. I understand why it was done, but I'm not excited about having this new piece of permanent hardware in my body -- even for a week. I'd be lying if I said otherwise. At this point, though, the new goal is to get a full session of the photopheresis treatment through the current cath early next week and then proceed with removal of this crazy line and a replacement with the originally planned vortex port.

All in all this will take me to a total of three "regular" ports, one dialysis cath, and one Vortex port before this whole cycle is over -- a process of "permanent" catheters that began when I was 18, which was also the year my body decided to give up on PICC lines. I'm grateful to have the option of ports and catheters and central lines (oh, my!) but I'm still hoping this next Vortex port might be enough to last me a while. If nothing else, I think "Vortex Port" sounds pretty badass, especially when you consider that my last port was called a SlimPort. We're definitely moving on up in the world of tough guy terminology over here.

But for now, at least, I get to start my photopheresis treatment this upcoming Monday or Tuesday. I also get to meet my new attending transplant doc over at my photopheresis hospital, who will be overseeing my treatment in collaboration with my primary lung transplant team at my regular hospital. Another day, another doctor. Another procedure, another port placement. Another chance, another catheter.

All of which I know will be worth it, in the end, for another beautiful breath.

Monday, October 22, 2012

Hey CF, The 1980s Called...

They want their diagnosis back.

For those of you who don't know yet, I'm 30 (nearly 31!) and I was diagnosed with CF at six weeks old -- way back in 1981. It's a diagnosis that I share with about 29,999 other people in this country, people who are rich and poor, young and middle-aged (yay!), religious and atheist and everywhere in between, and people who are conservative and liberal alike. CF doesn't discriminate and it doesn't pick sides. That means when it comes to fighting this deadly disease, we're kind of stuck with a mixed bag of people -- heck, even our awesome Congressional CF Caucus is a bipartisan effort. And you know what? I wouldn't have it any other way.

Having CF and living with it for 30 years does not in ANY way, shape, or form make me a spokesperson for the CF community. That said, the experience of doing so -- especially when combined with the "fun" of attending law school -- has, I think, made me pretty aware of some of the problems with the healthcare system in this country. The views in this and every blogpost authored by me are mine and mine alone. They are my OPINIONS. And before I even begin this discussion, let me say that I know for a fact that Gov. Romney is a friend to the CF community. Don't believe it? Feel free to check out this link, just as an example. Now, enough with the disclaimers already.

If you watched the debate, you may have seen Gov. Romney's statement that we don't "absolutely need" healthcare reform (slightly out of context there -- what I believe he meant was that we don't "need" all aspects of the recently passed health care bill). You might also have heard him say that there are states doing a fabulous job of administering medicaid, states like, oh, I don't know...Arizona?

And that's when I literally spit water into my lap.

I don't want this blog to be political, but I do want it to be honest. And my honest opinion, folks, is that calling out Arizona for successful management of medicaid for disabled and indigent citizens is, to put it mildly: a load. of. crap. There, I said.

In October of 2010, Arizona decided to stop funding lung transplants for ANY of the approximately 100 people on their medicaid rolls who desperately needed them (see, e.g., this article). None. Zero. Nada. Among the affected people was at least one young woman with cystic fibrosis who had, in fact, worked as a basketball coach prior to her slide into lung failure. The 27-year-old woman, Tiffany, was waiting on lungs for end-stage CF when the change took hold, cutting off her ability to pay for the admittedly pricey surgery. You can read her story here because she was brave enough to share it.

The state of Arizona justified this change by saying lung transplant was too risky; basically, the survival rate as compared to those who did not get the surgery was too low to justify the cost. (They also denied liver transplant coverage for Hep C patients because of a claim that the disease had a 100% recurrence rate.) Doctors and later lawmakers claimed the state was acting on faulty, outdated information. Regardless, the situation continued unchecked for several months, despite the controversy. Several months, guys. And as someone who has been through the lung transplant process, I can tell you that each day on the list feels like a year even when you know you CAN afford the surgery. Not cool, AZ. Not cool.

For the record, both lawmakers and lay people alike from both parties stepped up to rectify the situation, and funding was restored for transplant in April of 2011. Ms. Tate did receive her lungs.

Look, I get that Medicare and Medicaid are tough subjects, and often sore spots amongst the CF crowd. I get it because I've lived it. I've actually been personally struggling to come to grips with the fact that Medicare refuses to cover my admittedly necessary and LIFE-SAVING transplant drugs just because I used private insurance to cover my transplant, and thus saved the state hundreds of thousands of dollars. That doesn't make sense to me, either. But then again, that policy isn't being held out to the world as a "good example" of healthcare policy genius. The fact that Arizona's handling of Medicaid is...well, that just infuriates me, regardless of party.

You don't have to love the healthcare bill just because you have CF, any more than you have to vote for Romney just because you love dressage horses (as a personal aside: I fit into both categories). What I would ask is this: that as responsible people, citizens, and lifelong users of the healthcare system, that we do OUR OWN research into the issues that most affect our daily lives. Pay attention to what states are getting it "right" (whatever that means to you) and what policies you want to see put into action. It's almost impossible to know your candidate's position on every little issue out there in the political universe right now, but you should always know his/her position on the ones that matter most to you personally. And whether it's bayonets or lung transplant funding, never settle for less than the full story.

Happy voting, y'all.

Thursday, October 18, 2012

If You're Feeling Sinister

There are lots of words that most post-transplant patients aren't very fond of hearing. "Prednisone", for example, or "infection", "bronch", "flu-season" (one word for the purposes of being super scary), "IVs", and my personal least favorite: "hospital." But all of those words, scary as they may seem, are downright joyful compared to the one word that all organ transplant patients dread beyond all others. Yep, you guessed it:


Y'all might remember that a few weeks ago I was hospitalized (bad word #1) for IVs (bad word #2) because of a bronch (bad word #3) that had shown some sinus infection (bad word #4) and some prednisone-resistant (REALLY bad word #5) rejection (horrid, terrible word above all other bad words). As I explained then, the plan was to hit the remaining rejection with the big guns and wipe it all out in one very effective -- if somewhat uncomfortable -- shot so that life could continue more or less as normal for Donor Bob and me. So I checked into the hospital, I got the IV treatment, I took antibiotics for the infection, took the rabbit serum for the rejection, and then was released back on the world with the understanding that we would repeat the bronch in a couple of weeks to ensure that the rejection had indeed been, well, rejected. 

I had my repeat bronch last Thursday and honestly, it went fine. My lungs, according to my doctor, looked pretty good. My infection seems to have cleared up at least enough for me to be breathing a little more through my nose, and my O2 saturations have climbed back into the decent, if not terribly awesome range. As far as I was concerned, all systems were go and the results were bound to be good. Sadly, this is why I choose a career in law rather than fortune telling. Because my bronch results apparently are still showing some mild rejection, even after steroids and rATG, and that, in a word, is just plain uncool.

So what do you do when the big guns fail? Well, apparently you go nuclear. This is all starting to sound a little sinister to me.

My doctor and I have discussed our options and are leaning toward a process known as "photopheresis." Now JDs might be know-it-alls, but most of us are in NO way MDs, so bear with me on this explanation. My understanding is that photopheresis is a process through which blood is taken out of the body, separated into red and white blood cells, and then the WBCs are treated under UV light to cause some sort of small mutation. The mutation, I believe, then somehow acts to inhibit the body from producing or using t-cells to attack the lungs. That's the complicated part that I don't understand. What I do understand, though, is that this treatment involves removing blood from my body, treating it, and returning it, and that I will need to undergo those treatments for a while, at least. I'm currently working in true Piper style to make sure that this process is 1) medically effective (this part comes from being engaged in the process and trusting my docs) and 2) personally acceptable (this part comes from being engaged in the process and trusting myself). I'm hopeful that between the awesomeness of my very astute and capable medical team, the awesomeness of my wonderful family and friends, and the awesomeness of me that I can actually drag out every once in a while, that this is going to end in one truly awesome result.

In the meantime, a part of me can't resist the fun timing of being hooked up to a vampire machine just in time for halloween. (Well, okay, I could totally resist it if they gave me the option, but since they haven't I'm just gonna run with it.) I've come to the hard-won conclusion that this treatment might be all about sucking my blood, but it is in no way going to suck my spirit or my life -- at least not so far as I can help it. My mission is to trick this rejection into giving up, while treating myself to as much normalcy as possible, and if that means I have to fake it 'til I make it for a little while at the outset, well, I've always been good at costumes. And as for you, CF, I have just four little words of advice this Halloween season:

Be afraid, little monster.

Because I'm not.

Wednesday, October 17, 2012

Show and Tell: The School Debate

Every time CF makes national headlines (much less an appearance on The Today Show!) everyone in the community seems to sit up and take notice. Most of the time this is an exciting moment: there are articles on new medical breakthroughs, spotlights on outstanding or inspirational community members, and the occasional awareness piece that makes us all super grateful at the amazing people we have in our corner.

And then, sometimes, it's not.

Today several news sources featured a version of this article, about a young man who was released from school after administrators learned that he "carried the genetic marker" for cystic fibrosis. From my own personal reading of this and related stories, the story seems to revolve around a boy who was asked to leave a northern California middle school after it was discovered in his medical records that he has some version of genetic CFTR mutation. The school apparently acted out of an abundance of caution because there is another student at the school who indisputably has CF, and the school was concerned about the potential for cross contamination. What the article DOES NOT make clear (from my readings, at least) is whether the boy in question is a carrier (i.e., has one CFTR mutation but not the two required to cause cystic fibrosis) or whether he has abnormal mutations that his parents and doctors believe are non-disease-causing. The article above refers to him as a carrier but also mentions that he has a "combination" of genetic markers and that his family and doctors are "monitoring him closely" to make sure he does not actually have the disease. In other words, I'm not sure what the boy's exact medical status is -- nor should I be, necessarily. Those facts are generally between a patient and his/her doctor (and the parents in the case of a minor) and part of the issue with this particular story might be whether such privacy rights were violated in the first place.

Personally, I have several friends with CF, both pre and post-transplant. It's my choice to do so, and I like to think I'm careful when it comes to cross-infection concerns and keeping myself healthy. I want to emphasize that people with CF can have relationships (both in person and through other mediums, like Facebook or CysticLife) and that these relationships -- far from simply being risky and dangerous and scary -- can actually be incredibly helpful psychologically. Our relationships with others who share our disease help remind us that we are not alone, help inspire us to keep going and to do great things with our lives, help drive home the importance of medical compliance and self-accountability, and help make us feel part of something larger and stronger than we are as individuals. In other words, people with CF are important to each other because they give us a community of others like us -- not to the exclusion of others but to the benefit of ourselves (and our sanity).

That said, I understand the debate. I understand the fears. And, you know, I'm not in middle school, so I totally get that parents of middle school children might be more cautious about this particular issue than I am as a full grown adult with adult friends. So for anyone who might need a refresher, here's a good place to start, courtesy of the Cystic Fibrosis Foundation:

Living with CF at School: Guides for parents and teachers

Stopping the Spread of Germs: Handbook on staying healthy with CF (specifically addresses interactions between CFers and the notorious "3 Foot Rule").

At the end of the day, whatever the outcome of the case in question, one of the Cruel Facts about CF is that patients, caregivers, and parents are constantly having to make choices about their priorities in health and in life. There will always be moments of panic surrounding "should my child have this playdate?" or "should I study abroad and/or take this fast-paced job in my dream career?" or "ack! I've been invited to a party and I know it's going to be crowded and I think there might be cigarette smoke going on somewhere but I really want to go because all my friends will be there and so will that guy I like and I have to be normal sometimes and I really really REALLY hate this disease!" (Yes, trust me, it WILL happen.) I personally think it's up to us as individuals to educate ourselves (hint: the CFF and the BEF both have GREAT resources available) and our communities so that we're acting out of real understanding of ourselves, our priorities, and our disease. We can also help out by being open and honest, and by helping other people know more about our disease. Because knowing, after all, is half the battle.

Happy learning, everyone.

Sunday, October 14, 2012

On Speeches and Sampson

I took a long, wonderful walk around the city today. I have to admit to loving this Indian Summer weather, and I think Sampson is right there with me. He posed for this very regal looking shot on the Charles St. Pier over the Hudson River -- nose to the wind and eyes on the boats bobbing along through the current.

It was nice to be out walking in the sunshine, especially after last night's fantastic fundraiser for the Cystic Fibrosis Foundation. Most of you know that the CFF is a cause very near and dear to my heart (and even nearER and dearER to my lungs). Last night the fantastic Young Professional's Leadership Committee for our very own Greater NY Chapter held it's annual "Prom to Remember" event, and I was honored to be able to say a few words on behalf of myself, the Foundation, and all of the amazing, wonderful, blow-your-mind-style awesome 30,000 people living and breathing every day with CF. It was, in a word, epic.

It never gets old, speaking on behalf of and next to and together with all of you guys. It never feels stagnant or unimportant or run of the mill. The experience of being able to proclaim to an audience how great the CF community is and how far we've come in the last 60 years or so since the CFF's founding in 1955 is one that seems forever humbling for me, and I mean that in the best way possible. To know the CF community is to love it; to know the work of the CFF is to admire it; to know the beauty of coming together for a cause is to long for it. This is not a disease that requires any sort of "hard sell" to the audience. I simply open my mouth and share OUR stories, and the wonderful part just sort of takes care of itself.

But last night was important to me in a different way somehow and it wasn't just because some of my best friends in this city had a part in planning the event, or because I got to wear a costume that actually involved a wedding gown AND a tiara. Last night was different for me, I guess, because last night was one of the first times I've spoken about CF since my transplant when I felt truly vulnerable in terms of my own health. Last night was one of the few moments since receiving these perfect new lungs when I felt, well, I guess the right word is probably "uncertain," or maybe even "scared." Because right now I do feel a little bit scared, if I'm honest, and it's not a pleasant feeling to remember -- especially when it comes to breathing.

Before anyone gets too worried, though, let me say this: I am fine. I am dealing with some rejection and infection and drug level issues that are not the end of the world, but that are persistent and frustrating for me and my doctor. This makes them scary. It doesn't make them dire, by any stretch, but it does make them scary. There. I admitted it.

So the other night when I took the stage to talk about CF, I felt particularly vulnerable -- and that made me particularly grateful. I am particularly grateful right now for my kind doctor and wonderful medical team, who all seem to know the right balance between caution and courage. I am particularly grateful for my family and friends who organize, attend, and support events like the YPLC Prom, and who share my life whether I am feeling strong or weak or anywhere in between. And I am particularly grateful for the CF community as a whole for giving me 30,000 plus reasons NOT to feel alone in this journey through cystic fibrosis. Or, in other words, I am particularly grateful to each and every one of you. Everyday.

And if all else fails, I can always take a lesson from my wonderful dog: keep your face to the wind, follow the current, and always remember who's holding your leash.

Friday, October 12, 2012

Life in the Balance

Most of us will make a lot of promises throughout our lifetimes. From our first whispered promises of friendship to a new classmate or neighbor to professional oaths to committing our lives to another or pursuing the very best for our children, promises are important not just because they make public our intentions, but because they force us each to put into words our goals, aspirations, and -- hopefully -- our desire for actual action and follow through. They are important because, among other things, they give us the chance to examine ourselves.

I made one of my biggest promises two years and four months ago today, as I was being wheeled into the OR for my transplant surgery. Some of you might know that I had already had some not so fun experiences with last minute dry runs, and so I very distinctly remember the process of being wheeled off and wondering if this was finally the real deal. And what I remember thinking in my head, word for word, was this:

"Please God and Donor and Donor Family: if you just let this work this time, if you can just let this be it and these lungs be perfect, then I promise to do everything I can to keep these lungs healthy and to live to make you proud."

Not super eloquent, maybe, but at least it was honest. I made that promise with every last breath I had in my body at that moment. At the time I had yet to "meet" Donor Bob or his precious, amazing gift -- I had yet to experience the raw joy that is so-called "normal" breathing -- but I knew in my heart that this was a union worth praying for, committing myself to completely, and celebrating. Two years and change later, I wouldn't have asked for it to play out any other way.

We given many gifts in life to help us live it. Some of us are given more or less at different times, but all of us are givers, and all of us are receivers. The balance for those of us who have received our gifts in the form of new life is to learn how to protect those gifts without compromising our abilities to live with them. I don't know if I've always struck the perfect balance, frankly, and I'm honestly not sure if I'll ever manage to get the scales exactly right. But I do know that I have done my damndest to keep my promise(s) to myself, to Bob, and to all the many, many people who have loved or cared for both of us.

And so, tonight, I think it's important to send a little shout out to all the others out there who part of our collective balancing act. If you've ever asked yourself "where exactly does LIFE fit into all this medical mumbo jumbo?" or "how can I pay it forward for all the kindness that's been shown to me throughout this struggle?" -- well, then this post is for you. I may not have all the answers, but I do know that Donor Bob and I will be right there alongside y'all as we continue to hunt for them.

We promise.

Friday, October 5, 2012

Positively Nuts

I get lots of fun emails through this blog and from the CF community in general.

Yesterday, for example, I got an email asking why I chose to get a transplant. Actually, what the email really said was closer to "why on earth would you willingly let them rip your lungs out of your body, replace them with a dead guy's, and then destroy your immune system to make the whole thing work? And then to top it all off you're now letting them inject you with rabbit guts? This sounds scary. Seriously, lady, you must be positively INSANE!!"

So I wrote back, and the gist of my answer was this: Good question, dude.

And then today I was called (via FB, at least) "intimidatingly positive," which I found particularly funny because most of my friends would probably consider that crazy talk. Because the fact of the matter is that I don't always have a super positive, go getter, CF warrior attitude when it comes to the stuff that is, as the email above correctly pointed out, kinda sorta definitely super scary. I wish I did because I think life in general is probably easier on optimists, but the real truth is that sometimes my own optimism falters, fails me, or just plain isn't around in the first place and I have to make do with the reality of the situation and a not-so-positive attitude. I tend to refer to these moments as "fear spirals" because that's what they feel like -- strong currents of scary circumstances that twist me around and force me into reactive mode, where I feel like I'm being flung in circles and don't really know how to break out of it and get back on track to something better. These moments are, thankfully, far fewer and further between now that I have my new lungs (initial scariness of receiving them notwithstanding, of course), but they still happen. And when they do, I've discovered that the best way to combat them is with what I call "retrospective forward thinking."

See, I told you I was crazy.

Retrospective forward thinking refers to the act of remembering all the goals I set for myself back when I was dying, and all the many reasons I came up with then for making the scary choice to let a doctors replace my vital organs. See, when you're fighting for every single breath and spending most of your time in a hospital bed or on the couch or hooked up to IVs/machines/oxygen, you have a lot of "free" time for stuff like personal reflection. And for me, most of that time was spent thinking about all the many awesome and wonderful things I would do once I got my new lungs. I passed hours and hours thinking, writing, praying, or dreaming about the goals I had for myself and the world I could create if I just kept pushing through all the bad crap, and believe it or not the list I came up with was actually a pretty good guide for what matters (to me, personally) in life. Looking back on it after my transplant I was surprised to see that it wasn't just sickly ramblings -- it actually makes a lot of sense. As a few examples:

  • Give back to people who have given so much to me, both personally and on a larger scale. Find ways to make life meaningful that aren't just about spoiling myself.
  • Spend time building relationships and seeking out connections. Look for people to learn from, to grow with, to laugh with, and to make each other better.
  • WALK MY DOG!!!!
  • Treat my lungs and my body as a gift to be cherished and as a vehicle to help me accomplish my goals. Seek out exercise that makes me happy, stick with it, and do whatever routines are necessary to keep me healthy, breathing, and able to be me.
Pretty simple, I know, but also really valuable. I find myself rereading these and my other goals over and over sometimes -- whether its because I'm in the hospital and receiving a scary new rejection treatment; whether its because my prograf level won't stay stable and I start to wonder if I'm REALLY doing all I can (Should I find a new drug? Am I eating wrong? Maybe I should try taking the drugs with water, at 11:11, while facing East and standing on one leg??); or whether I simply need a good kick in the butt to jump start my motivation and get back to it. To me these goals are like a contract I made between my mind, my body, and my amazing Donor Bob. I feel obligated to keep moving toward what I know is truly important (even if that changes down the road or from time to time) not just because I was given something wonderful, but because these are the things I chose to cling to when I needed it most. 

Life. Purpose. Connection. Health. These are the real reasons I went through transplant, far more so than fear of dying or an abstract desire to stick around for a few more years (though those were part of it, not gonna lie). Those four core goals were and are what keeps me going, helps me through the scary stuff, and makes even the spirals seem like bad weather on an otherwise clear horizon. They are the main reasons I manage even the inadequate amount of positivity I can actually muster sometimes through all this crap that is CF. 

And they are also, for better or for worse, what keeps me Piper -- and all the positively crazy stuff that goes along with it.

Sunday, September 30, 2012

She's Not Heavy, She's My Cyster

One of my favorite things about this blog is getting lots of emails from readers from all walks of life: from fellow CFers to parents to siblings to medical professionals to people with other lung diseases to just really supportive strangers. The people are all different, but one thing many of them have in common is the question: "why are you so open about your disease and does it ever scare you to put yourself out there like that?"

There are about a million different answers as to why I choose to share my life with CF and transplant publicly. I could credit my parents, who taught me to be open about CF at a young age and who got me involved with the CFF basically from the word go after my diagnosis at 6 weeks old. I could credit the world for blessing me with amazing people who are all so understanding about CF and my health and who have never made me feel bad about myself just because I'm a little bit different. I could credit all of these people and more and I definitely wouldn't be lying -- it's amazing what a few good people can do for the soul in terms of keeping me honest.

But most of all, I credit you guys.

The CF community is, in a word, exceptional. My friends and "CF family" have truly stood by me "in sickness and in health", never afraid to laugh, cry, commiserate, or celebrate right along with me throughout the journey. In my most arrogant moments I sometimes fancy myself a "writer", but when it comes to expressing how grateful I am for everything you all have given me through the years, well -- words fail me. Honest. You guys are everything.

And this article, from today's NY Daily News, just proves it.

Not only am I lucky enough to call these lovely ladies my friends, I am also blown away by their courageous strength in sharing their stories. From Lyndsey's recent stint on NY Med, Katy's appearance on Say Yes to the Dress, Kristy's dedication to speaking to nursing students about CF and organ donation, Allison's appearance on cooking shows to promote healthy eating for CFers and diabetics, and Kelley's recent work signing up organ donors at NY's Fashion Night Out, these women are great representatives of the spirit and energy found throughout the CF community.

So how do I do it? Easy. I just remember that 30,000 heads are always better than one.

And then I follow your lead.

Tuesday, September 25, 2012

Reality Bites

One of my favorite parts of any visit to the hospital is leaving. Seriously, it's right up there with feeling better as one of the most pleasant aspects of an admission. Not that my hospital isn't amazing, of course -- because it totally is. I'm lucky enough to be seen at a hospital that has both an incredible CF team and a caring, smart, and proactive transplant team. Everyone -- from the nurses on the transplant ward to the PFT techs to the pulmonologists to the slew of other doctors/staff members I have on my team (infectious disease, endocrinology, ENT, thoracic and vascular surgery, allergy . . .  the list is pretty impressive) -- works hard every time I pass through those doors to make me happier, healthier, and just basically more Piper-esque. I'm incredibly grateful.

And yet, I still love leaving. I love packing my stuff and actually putting on street clothes. I love signing my discharge papers. I love cutting off my hospital ID and allergy wrist bands. I love walking outside into the fresh New York City air (ha, ha). I love looking back over my shoulder and waving goodbye at that wonderful team and everyone else still hard at work healing or being healed in that wonderful, awful, miraculous place. I love coming home to my puppy, my friends, my food, my bed, and a hot shower. I love, love, love, love, LOVE the freedom to wake up the next morning and know that I am free and I am alive and I am able to make happen anything I darn well want because I'm a rockstar and I can do it all by myself, thank you very much. I love it.

Well, okay, so maybe it's more accurate to say that I love most of it, because that final part -- the "making things happen" part -- is almost always easier said than done for me after a few days spent living like a sick girl in the hospital time warp. By the time I actually check out of the hospital and head back into the real world, it seems like I always find myself floundering for a couple of days before I manage to regain my footing. Even after 30 years I'm still not quite sure why this happens so regularly or why I never seem to learn my lesson and find a way to avoid it, but it does and I don't. Without fail the first few days of "freedom" end up being about 30% actual life and about 70% of me standing around looking bewildered and making countless but unhelpful "to do" lists about how to get back on track while I simultaneously agree to every single request made of me so as to prove just how "normal" and "NOT SICK" I really am.

Or, as one of my wonderful friends recently put it in an email:

"It's cool, Pipe. We wouldn't expect anything less from you."

Yeah, me neither.

I know I can't be alone in this. As a group, most CFers I know tend to be pretty intense people. As CF adults, we've lived with illness and hospitals and doctors for a long time, and many of us are masters of acting as nonchalant as possible in the face of what most people would consider some really serious health issues. I watch a lot of my CF friends make the exact same comments I've made my entire life ("Oh, the hospital? You mean that two weeks of IVs/rabbit blood/coughing up blood/bowel obstruction/pain/poison/whatever? That little thing? I got out YESTERDAY, man! I'm totally FINE now! Life is AMAZING!") and I always wonder: are they really feeling that great after all that, or are they just really darn good at faking it? Or is it maybe just a little bit of both? To be honest, I don't really know the answer.

What I do know, though, is that when most "normal" people come back from the hospital, they're expected to be tired, to rest, to require a little downtime. When I come back to the hospital, people apparently expect me to be extra intense. This little pattern I have of being manic right after an admission is, I've recently discovered, NOT normal. Apparently I'm NOT the status quo. And even more surprisingly, it seems like most "healthy" people are able to actually allow themselves to be sick and then allow themselves ample time to HEAL and FEEL BETTER without immediately feeling the need to prove themselves as superhuman the second they get discharged. Wowza, guys. Who knew?

As for me, I'm not sure "normal" is really in the cards anytime soon. Personally, I'd settle for a little more rest, a little less crazy, and a lifetime's worth of lessons on how to make that happen.

Thursday, September 20, 2012

Mission: Possible

Captain's Log:  September 20, 2012. Day 4 of mission, 7th floor, 12:10pm.

Since arriving in the foreign world of "Planet Hospital" four days ago, our crew has been busy trying to repair the lung mechanism and conditions continue to improve. I have successfully made contact with that strange yet somehow wonderful species known throughout this place as "doctors" and they, in turn, have called in some sort of rabbit army to aid me in my quest. Though the rabbits are somewhat unpredictable (they seem to bring with them a whole slew of annoyances, from fever to general stomach discomfort), they are, as best I can tell, doing their job. Reports indicate that the invading t-cells are being effectively subdued, and one of the doctor leaders has suggested that I might be able to withdraw from stage 1 of the mission (that frustrating stage better known as "operation inpatient") as early as tomorrow evening.

My fingers are certainly crossed.

The inhabitants of Hospital are truly a unique bunch -- many of them seem able to run around continuously for days on end with little, if any, observed sleeping habits. The world is inhabited by a host of both daytime and nocturnal creatures and therefore remains at full activity at all times, which makes normal human sleeping patterns difficult to sustain. Also strange is that these energetic creatures apparently do not eat, as so far I have observed absolutely NO sign of edible food stuffs. This scarcity has been partially relieved by messengers from the distant realm of New York, of whom I am lucky to have several. These messengers not only provide nourishment, but also much needed company and the occasional picture of "King Sampson Bear" from back home. For them, I am grateful.

Yesterday morning one of the doctor leaders had scheduled me for an interesting activity known to his kind as the "gastric emptying study." (Doctors as a species, I should add, are certainly a curious bunch -- it's not uncommon for them to want to perform several of these types of study activities in a single day.) The first part of this mission was simple: I was told to refrain from eating the already inedible food stuffs served to humans on this planet. Done and done. Next they demanded that I eat radioactive oatmeal in place of a regular breakfast and lie down on a table under what they call an "x-ray" arm. They kept me in that position, immobile, for an hour -- a condition that normally would have annoyed me but which I found surprisingly pleasant, as it was the first quiet hour of actual sleep I've had since arrival. This morning, the doctor leader informed me that the study showed delayed emptying (par for the course in my particular brand of humans, by the way) but not to an excessive degree. After a short peace talk, he and I agreed to wait and see before trying out any new weapons on this latest enemy.

And so we sit. And so we wait.

If all goes according to plan, we should be launching the final two rabbit army assaults on the t-cells tonight and tomorrow, after which (please, please, please) I will be able to return home to King Sammy and restart most of my normal activities back in TransplantLand. I have been warned that the effects of the rabbits may remain with me for several days, but I am, as always, optimistic. If I can handle slaying the CF monster, after all, I think I can handle some bunnies. The continued plan for the remainder of the mission includes stabilizing the effects of that dreaded rejection monster through a few minor weapons adjustments and, sometime in the near future, a possible frontal attack on the sinus region to once again annihilate the Pseudo bugs. All in a day's work for someone with 30 years of wartime experience.

I'll sign off now, with the final thought that this mission, while scarier than previous assignments, has already renewed my faith in the treacherous exploration we call lung transplant. Though ultimately I hope more and more of our species will find such travels unnecessary for their particular journeys, I am grateful to have had the chance to wander this new terrain with such amazing support from my fellow travelers, the wonderful doctor leaders, and everyone else out there that makes up this world so strange -- and so beautiful.  

Until Next Time: Piper

Tuesday, September 18, 2012

Don't Think Twice, It's Alright.

It's been approximately 36 hours since I first checked into the hospital for this stay, not that anyone's counting. So far in that time I've received 2 doses of 500ml IV solu-medrol (the steroid, and for perspective my maintenance dose is 10mgs), 1 dose of IV ganciclover as a CMV prophylactic (with another to follow later tonight), 2 doses of oral prophylactic antifungals, 2 doses of oral antibiotics for my pseudomonas, and 2 doses of 100ml rATG (aka, "rabbit juice") infused over approximately 8-12 hours to soften the blow of the side effects.

Also in that time frame, the elderly gentlemen across the hall from me have shown no signs of learning how to properly secure their hospital gowns in the rear end area. Not that I'm looking or anything.

I woke up this morning feeling kinda crappy. The first reason for this is that "woke up" is a bit of a misnomer; it would be more accurate to say "after remaining awake for the entire night, I felt pretty crappy in the morning when OTHER PEOPLE were waking up." Shocker, I know. IV steroids seem to do that to me. Add onto that the combination of general malaise from the drug itself and people coming in and out of my room all night to monitor my side effects, and you'll probably get the general idea of why it can be so hard to sleep in this place.

The second reason for the crappy morning is equally shocking: turns out human bodies aren't so hot on rabbit blood. Crazy, right?

But it ain't no use to sit and wonder why, babe. We just do what we do here to keep me breathing, and quite frankly I'm okay with that.

It's weird to think of poison keeping me alive, but in all fairness that seems to be what's happening. Treatments like this remind me that my body is essentially maintained in a state of toxicity for its own good. Transplant is like a sticky kind of compromise I reached with cystic fibrosis and my natural desire to live: I gave up my lungs and CF gave up part of its hold on me, and in return I got a beautiful life full of love and laughing and walks with my dog and talks with my friends and the sight of the Colorado mountains or the New York City skyline at night. And CF and its cronies got a kind of "get out of jail free" pass for most of my immune system function and now they get to play fun little games with rabbit blood. Sick little bastards, aren't they?

But don't think twice, it's alright.

When I think of this disease it's hard for me to see it as anything other than a monster, even if my life is in someways "better" for it. Meaning, I guess, that my life is better for pretty much every experience I've been lucky enough to survive and learn from, even if it can be tough to notice in the moment. I won't say I'm grateful to CF (it's taken too many of my friends for me to EVER say that), but I am grateful for the challenge. And if I can't say that I've defeated it yet, I can at least say that I've won a few well fought battles -- and come out the other side as someone I hope I can be proud of. Just as I know I'll get out of here soon even if 5-7 days seems like an eternity right now, I also know that my walk with CF and transplant won't be over for a while. I haven't shaken this little monster, but at least I was strong enough to change the game.  So if I can't say goodbye until the moment when we all can say it together, well, I know that's alright, too. As a matter of fact, I'm looking forward to it.

So I'm walking down that long lonesome road, babe
where I'm bound I can't tell.
But goodbye is too good a word, gal
so I'll just say fare thee well.
And I ain't saying you treated me unkind,
You could've done better,
But I don't mind.
You just sorta wasted my precious time.

But don't think twice, it's alright.

On Rabbits and Rejection

H is for hate this. I'm sorry, it's true.
O is for "oh gosh, food here tastes like poo."
S is for sitting and waiting all day.
P is for Piper does not want to stay.
I is for icky, rejection that's you.
T is for all the things I'd rather do.
A is for at least my docs are the best.
L is for lungs that will soon pass the test.

All right, so now that we've got that one out of the way...

I checked into the hospital this morning for what appears to be a rather stubborn case of acute inflammatory rejection in my lungs and airways. This is, by the way, the same rejection (and the same pseudomonas infection) that my doctor caught at my bronch a few weeks ago and that we treated with high dose prednisone for a couple of weeks. I took the steroids, took some antibiotics, did a few days of IVs, and just like that I started to feel as much like new as a gal living with "gently used" organs ever can. Then this past Thursday my doc did did a routine follow-up bronch and discovered that not only is the rejection still there, but it's gotten slightly worse. Cue the dramatic music.

Although, actually, don't cue anything, because word on the hospital street is that this really isn't the end of the world. Apparently steroids are the first line of defense against rejection, but when they fail to do the trick (and sometimes they do), there are other, more potent drugs that they can use to nip the problem in the bud before it escalates into something more serious and less treatable. Drugs like, for example, the one I'm currently infusing as I type:

"Antithymocyte Globulin Rabbit."

The antithymocyte means it is a lean, mean, t-cell killing machine. The word globulin means, I believe, proteins found in the blood stream. And the word rabbit means, well, rabbit. Yeah, I know, although to be fair this is hardly my first walk down the medical/animal ethics path. Digestive enzymes, for example, are made of pig parts. Pulmozyme, no joke, is derived from Chinese hamster ovaries. Put that in your neb and smoke it.

And this drug, it appears, is made of rabbit-produced antibodies designed to attack and kill human t-cells in order to protect the organs of yet another human. This is starting to feel kinda crowded.

The protocol for this drug is "at least" five doses running for "at least" five days in the hospital. When I told my doctor that I wasn't a huge fan of the words "at least" in the medical context, he chuckled. Not a great sign. In addition, while I'm in here, I'll also be receiving IV ganciclovir (an IV antiviral), oral levaquin, and high-dose steroids in the form of IV solumedrol. All of that stuff is pretty par for the course on any CF/transplant admission (plus it gives me a plausible excuse if I'm ever feeling grumpy), so I'm pretty much fine with it.

So far, all I can really say about this admission is that's it's going to last for "at least" this upcoming week and is probably going to be somewhat unpleasant -- between the steroids and the rabbit poison (um, I mean "treatment") designed to essentially destroy my body's natural defenses, I'm not betting on a barrel of laughs. Then again, the guy in the room across the hall from me apparently missed the memo that hospital gowns actually have ties in the back to keep them closed, so maybe this will be kinda amusing after all. At any rate I am 100% optimistic that it will be effective -- and right now that's just about the only thing that really matters.

Now let's hop to it.

PS: On a personal side note, a friend from the CF community got her call for transplant tonight at this very hospital. Knowing I was here she sent her very lovely mom to come find me and tell me the wonderful news. A few hours ago I got the AWESOME update that she is, indeed, in the OR right now receiving her new life. Please keep her family and that of her donor in your thoughts, prayers, and intentions tonight as they make the leap of faith that is lung transplant. I'm so happy for her I could, um, let's just say "jump" for joy right now. Thanks in advance, beautiful people.

Monday, September 10, 2012

In Case of Emergency...

Earlier today, I posted a comment on FB about the scary obesity rates in our country (namely that obesity-related issues have recently surpassed smoking as the number one cause of preventable disease, and that Texas alone spends approximately $10 million in treatment of these disorders each year). I recently learned these facts from a combination of my post-tx exercise trainer and my current obsession with workout shows like The Biggest Loser (yeah, I know), and they startled me, so I posted.

This might seem a little odd after my rant on here about gaining weight, but I also intended my post to be helpful to the CF community. Because, let's face it, as anyone who grows up in a CF household can tell you, taking care of a child (or a sibling) with a chronic illness is TOUGH. It demands a lot of time, energy, and money. Add to that the fact that CFers typically need more calories than the average child, and you have a recipe for overweight caretakers, family members, and siblings. When I posted the statistics, I had in mind those little safety videos you see in airplanes -- you know, the ones that remind you to be sure to secure your own oxygen mask before helping your child? Hearing these scary facts made me think "oh, gosh, I really hope all these truly awesome and devoted CF family members out there are remembering to take care of their own health too!" Because trust me, gang, we need these wonderful people, both in the CF community and in the world at large.

Anyway, I posted these facts with a quick statement of what I hoped was encouragement for my friends -- CF community or not -- and asked them to please remember to stay healthy and take care of themselves. For CFers (and some other people), this might mean gaining weight. For other people (and some CFers), it might mean losing. And for all of us, it probably means eating better and going to the gym/pulmonary rehab more, not to mention staying on top of important stuff like medical testing, self-exams, sun protection, and responsible alcohol or substance use.

Hey, it's important.

There's a lot of debate out there within the CF community over personal health responsibility. Exercise is a great example, where some people swear by it and others swear it hasn't done much for them, especially in terms of numbers and lung function. I personally don't find this dichotomy all that shocking: of course exercise is going to have different effects on different people, just like having the disease we collectively call "cystic fibrosis" means different symptoms and severity for different individuals. Of course we all know that exercise, as a general rule, is good for you. But it all gets a bit murkier when it comes to forcing yourself out of bed with yet ANOTHER fever and 25% lung function to go walk on a treadmill and cough up goo until you vomit. That's a tough pill to swallow sometimes, even for a CFer. On the other hand, a group of wonderful CF women I correspond with regularly on these types of issues recently pointed out to me that part of feeling mentally healthy as a CFer is the knowledge that you're doing all you can to prevent your disease from dragging you down. And that, according to most doctors and patients alike, includes stuff like nutrition and exercise. As well it probably should.

I don't have any easy answers on this one. I'd like to offer my personal perspective as someone who's lived through end-stage CF, which is that doing little things that offered me some "control" (even theoretical control) was a HUGE comfort to me right before my transplant. Making space for exercise in my own life was important because it left me feeling empowered and less helpless -- and I'll take empowered with low lung function over helpless and STILL with low lung function any day of the week.  But again, that was only my personal experience.

I think the point is here that we need to be asking ourselves what we can be doing to make space for health in our own lives, whether we live each day with CF directly or not. We need to be securing our own oxygen masks, and then maybe we'll have the energy to look around and help others. Maybe it means getting to the gym more, or maybe it means finding another activity in your life so that it's NOT all about CF. Maybe it's as simple as coffee with friends or some time alone with a great book or finally taking control of that debt that's been harassing you or being part of your own cure by fundraising. At any rate, it's empowering. It's an anti-victim, no excuses stance. Most of all, it's about doing for yourself what you would like to someday do for others. 

And that makes it a lifesaver.

Monday, August 20, 2012

Weighing the Issues

Dear People Who Tell Me I Can "Have Some of Your Weight":

Great! Thank you! Despite having someone else's lungs inside my body, I guess I never realized how easy it was to share body parts! I will by all means take your excess body fat off your hands (or, in some cases, off your stomach). In return, you may have your choice of any one of the following:
  • My pseudomonas, because green is just SO not my color;
  • My sinuses, which are full of . . . um . . . let's just call it "CF gold";
  • My nose, but only because it's slightly pig-like (also, it's become quite attached to my sinuses);
  • One slightly mis-matched pair of mutated genes;
  • My pancreas, because I don't really use it anyway;
  • My hairstyle from the 80s, which I swear is gonna make a comeback
Please note that all items are non-returnable once worn.

Alternatively, we could all just sign up to be organ donors and leave the body-part musical chairs game to the experts. If you like that solution better, please visit the link below and share your gifts with someone who really needs them (when you're done with them, of course):

Either way, I know you meant well, and thanks for the offer.

Author's aside: I'm sure you all know that this post is intended in only the best of humor to make fun of the weight issues that many of us struggle with as a result of cystic fibrosis. I've long maintained that weight/body issues are actually common ground for most of us in lung disease and transplant (and probably also in life): whether you're trying to lose OR gain, this stuff is FRUSTRATING. And being on so many drugs that change our size, shape, or other aspects of our appearance (oh, hello there, prednisone!) probably doesn't help much. 

As for me, personally, I'm trying to gain. This past infection gave me a nice one-two punch to the gut (literally) and I've recently lost a few much-missed pounds. What's super annoying to me is that I've lost it despite being on a high dose of steroids, which typically helps people gain. I'm working on getting it back now by adding more small meals in the day and snacking constantly, but it's tough because, quite frankly, I already ate a lot to begin with! I'm also trying to do this without adding in 17 extra helpings of dessert each day. Prednisone + Diabetes + Extra Sugar = Comatose. And honestly, comatose just doesn't sound like very much fun to me right now. If any of you have any tips or suggestions, feel free to pass them along.

But whether you're sharing your stories, sharing your weight, or even just finally making the decision to share your organs -- happy sharing just the same, beautiful people.


Wednesday, August 15, 2012

Steroids, in 7 Verses

Steroids, oh steroids,
I hate you, you suck.
If you were a curse word,
You'd probably be f*ck.

But you keep me breathing
and help me survive.
It's worth a few mood swings
To keep me alive.

So strap on your big guns
'Cause Pseudo's in town,
With his old friend Rejection,
They can't keep us down.

We'll fight through insomnia!
Fight through sugar highs!
'Till both of these monsters
Have said their goodbyes.

And when it's all over,
And my face is all round,
At least there will always
Be some beauty found

In the wisdom of doctors,
The care of our friends,
The strength of our circles,
And faith without end.

'Cause our time is coming.
You know that it's true.
Dear cystic fibrosis:
we're coming for YOU!

xo little monster,

Bright, Beautiful, and Full of Hard-Earned Life

For those of you who just finished watching NY Med, thanks. Thank you from the CF community, for being part of our never-ending awareness movement. Thank you from the transplant community for caring enough to tune in and to (hopefully) do your part by spreading the word about organ donation and the many beautiful lives it saves each year. And, of course, thank you from our small group of CF/transplantee friends (myself, Katy, Kristy, Allison, Kelley, and Jerry) for letting our amazing "cyster" Lyndsey into your lives for the past hour. I promise you that the best is yet to come from that girl.

As I mentioned in an earlier post, I don't normally like watching medical drama -- reality, fiction, or anything in between. For lack of a better way to describe it, medical stuff done to other people still gives me a pretty sever case of the heebie-jeebies. I hear doctors in Europe are working on a cure for those, but until they find one I think I'll just have to continue reading books right through "House"and "ER" and any of the many incarnations of "Grey's Anatomy." I made a good faith effort to watch the short-lived transplant (melo)drama "Three Rivers" while waiting for my lungs, but decided to quit after a woman awoke from her lung transplant in a quiet, peaceful hospital room with nary a vent nor overworked ICU nurse in sight. Something told me I wouldn't be lucky enough to emerge from my surgery with new lungs AND perfectly applied eyeshadow, so why even bother?

But NY Med is an exception to my rule, and not just because it features my hospital/doctors/nurses/friends. I actually like NY Med for an entirely different reason, and one that surprised me at first. I like it because it actually seems, well, like a hospital. It shows the controlled chaos that never seems to exist in ER, where every doctor is apparently well equipped to perform every procedure bedside and will do so at the drop of a hat as long as he gets to yell "STAT!!" at least twice. NY Med shows doctors as who they are -- people! coffee drinkers! daytime talk show hosts who got their start on Oprah! -- and patients as, well, more or less as themselves, which is to say almost exactly like the doctors. There's a lot of coffee drinking.

I had my transplant the year before NY Med began filming at Columbia. My lovely cysters Kristy and Lyndsey were both right on target with timing, though, and both of them got to savor the fun of being followed by cameras as their health slowly declined (Lyndsey received her transplant first, ending the filming). They enjoyed the cameras because the crews were sensitive, interested in learning about CF and transplant, and generally nice people who wanted to treat them like nice people. And I think that's the difference between the awareness raised by this show about CF and organ donation and, say, the episode of Grey's when a man was forced to choose between receiving a lung transplant and breaking up with his infection-ridden, scary, fellow CFer girlfriend. 1) Lyndsey's story makes sense, and 2) it made the story first and foremost about her, and only secondarily about people running around the hospital calling out dramatic commands.

Look, most of us have been in a hospital. In my case, I've even inside THAT hospital. And I think all of us can agree that it's tough, when you're in there, to get the sense that anyone has any focus on you and your life beyond the walls of hospital. It can be difficult to make heads or tails out of fact that you walk into those doors a whole and complete person, and you exit them a whole and complete person. For the time inside them, however, you become a complex system of blood, muscle, organs, veins, and nerve endings that are all different except for the two things they share: they are in you, and they need to be fixed. You become someone's job in that moment, which is a way different thing than becoming their friend or their lover or even their enemy. Because jobs are typically passive things to be acted upon and completed, and the best jobs try their hardest not to mess things up any worse than they were when you walked in here. I'm always a little worried when I meet a doctor who laughingly tells me that he used to love George Clooney on ER. I feel like I need to shake his hand and then politely remind him that I am not a prop, I do not wear makeup into surgery, and, no, nothing needs to be done "STAT!" Thank you.

So NY MED gives us the chance not only to spread awareness of diseases like CF, or of the importance of organ donation, or even of the fabulous humor of Dr. Oz. The great thing about this show is that it also offers us a chance to spread awareness of all the many wonderful patients behind these scary diseases and larger than life doctors. It gives the real world some perspective on the tricky relationships that emerge when doctors know patients as people as well as a medical mystery, and the highs and lows that come along with those decisions. I'm grateful my hospital has that, grateful that you got to watch it, and even more grateful that the wonderful soul you all got to meet tonight was my lovely friend. Lyndsey, darlin', if we ever design a patient awareness ribbon for the much-needed "Patient Appreciation Day", I think it should be colored just like you are: bright, beautiful, and full of hard-earned life.

This post is dedicated, with sincere gratitude, to the all the organ donors and their families who made this and so many other miracles possible.

Saturday, August 11, 2012

On Getting Over It

Ever have one of those days when you're just kinda over it?

I'm feeling like that right now. Yesterday I (re)started IVs after 11 months off. Honestly, I really wanted to make a year. I really wanted to get my port out. I really wanted to just be done with all this infection nonsense. And yes, of course I realize that 11 months is, in a word, awesome. I can still vividly remember a time (read: last year) when I would have given pretty much anything for 11 months. So yes, in that sense, I know that griping about 11 months is silly. When I think about my friends still fighting for their lives or struggling for breath I remember that I have zero right to complain at this point in my life, skipping along as I am with my gorgeous new lungs. But, darn it, I still really wanted to hit that year mark. For whatever ridiculous, petty, selfish, and deeply human reason, that goal was important to me. So I'm a little bummed out about being back on IVs, even if I should be grateful. So sue me -- that is, if you want to risk it.

I woke up a few days ago feeling, well, lousy. I had a sore throat, and I was coughing some. Nothing terrible, mind you, just kind of . . . off. I'd been having some pesky but relatively unconcerning symptoms for a few days -- mostly just a slightly elevated heart rate and some high blood sugars, and a couple of instances where I felt breathless. Then the other night I climbed the stairs in my apartment and starting feeling breathless and wheezy. I grabbed my pulseox more out of habit than concern and popped it on my finger.


Ridiculous, I thought. I haven't been below 96 or so since my transplant, even when sick, and I typically run at 100 these days. So I switched the batteries, put it back on and . . .


I almost laughed out loud at that point, just because it seemed so impossible. I walked into my living room, plugged in my old monster wall pulseox that came with my O2 concentrator (the same pulseox that I haven't used in 2 full years), and stuck my finger in the monitor.


And that's when I just got angry.

I did manage to get it back up about an hour or two later, after several sinus rinses and finally coughing up what looked like an old-fashioned CF mucus plug. And then the next day I put my normal skepticism aside and dutifully called my coordinator like a good little patient. I didn't tell her that I first recognized the problem while walking up the stairs after watching both "Toddlers and Tiaras" and "Here Comes Honey Boo Boo" on TLC (hey, there are some things even my transplant team doesn't need to know!), but I was pretty honest about the rest of it. The weird thing is that I don't have a fever, am not coughing heavily, and don't have a ton of other symptoms other than some yellowish mucus and a persistent sore throat (and I've lost some weight, but I tend to lose weight if I so much as look at a low fat muffin, so that's not much help). In fact, after two days on antibiotics I'm starting to feel like nothing was ever wrong in the first place.

Except for those pesky O2 sats, that is. They keep dipping and rising, apparently just for the fun of it. When I'm walking around they can go down to the low 90s, and when I'm sitting or lying down they tend to hang out around 97-98.  Other than that everything seems to be looking up, and my mucus and cough have already settled down to pretty much nothing. I'm not sure how the bronchoscopy I have scheduled for Monday is going to go. If I continue to clear things out with rinses and the cough continues to lessen, I'd guess that it won't really show anything at all, which of course is my preferred result.

I think what has me feeling "over it" right now is, more than anything, the fact that I just don't understand. I'd like to agree with my docs that this is/was a bacterial infection -- especially since I guess there has been some improvement with antibiotics -- but it sure doesn't feel like it used to when I'd have exacerbations. And I'd like to think that I'm just overreacting and it's absolutely nothing (aside from the obvious embarrassment of wasting my doc's time, which I HATE doing and is actually my number one hang-up as a patient), but then I'm not sure how to explain the dips in numbers. Here's hoping that I have some sort of weird energy field in my apartment right now that causes all pulseox machines to go crazy and spit out phony results, but I'm guessing the chances of that are kinda slim.

So for now I'm just trying to be a patient patient, and do my IVs and take my medicines, and not draw any crazy conclusions and certainly not look anything up on the internet. I'm trusting that my docs have this one covered and that I'm not just going crazy. And I'm trying to get over my own disappointment in myself for not making it a year off the super drugs because, after all, I'm still here and still breathing and I still feel relatively decent -- and at the end of the day that ought to at least count for something.

If any of you have any similar experiences you'd like to share, feel free to share them. I always strive in my head to be some sort of perfectly rational patient who KNOWS ALL THE THINGS and never over or under reacts to anything. But for right now, I have to admit, I'm feeling a little helpless and silly.

And hopefully I'll get over it soon.