Thursday, March 5, 2015

Long Way Home

It was late afternoon and I was sitting in the hospital cafeteria, along with about 20 of my fellow hopeful lung-transplant candidates. I remember it being hot, an Indian Summer in the concrete jungle, and the constant whir of oxygen concentrators filled the space with an added sense of the surreal. We were here, together, sick, and ready to learn about the science fictionesque process by which one of our most vital organs would soon be replaced. We were excited. We were scared. We were confused. And man, were we ever ready to put this chapter of our lives behind us as the speaker for that day made her way to the front of the room.

The talk was titled "medical compliance post-transplant", and it focused on the pills, treatments, and various routines post-transplant patients are required to incorporate into their daily lives after the surgery. The nurse brought out each pill in turn, explained its purpose and use post-tx, and then noted how long patients would generally spend on any given medication. Not surprisingly, her answer for most of them was "for life." Post-tx patients can never go off anti-rejection meds, of course, because our bodies are preprogrammed to attack any foreign object, and the lungs in my chest will always contain my beloved donor's DNA. But because of anti-rejection pills themselves carry with them a whole slew of other side effects (lowered ability to fight off infection, low calcium levels and impaired bone density, partial resistance to insulin, and many others) we often are forced to take lifelong pills to help cancel out the other pills. Sound a little confusing? Well, it can be. The miracle of modern science is that we are literally able to save each other as our final gift of life. The downside is that we haven't quite caught up yet to the point where that gift can be fully accepted by the body as part of ourselves. And that, my friends, is why transplant research continues to be so important -- for the donors, for the patients, and for the world.

As we listened to the nurse explain all of this, I could see the eyes of some of my fellow hopefuls grow wide and fill with anxiety. Eventually one of them, a man of about 55 years old who was there with his wife and daughter as his caretakers, rose slightly out of his seat and said the words so many of us were thinking:

"Wait a minute, I thought I was getting a transplant to CURE my lungs? Why is it so much work taking care of them after?"

The nurse didn't miss a beat as she answered:

"For many of you, transplant will be a godsend. It will save your life, literally and figuratively -- it will allow you to keep breathing, but it will often also allow you to rediscover your self. The one who loves baseball, the one who jogs on the beach every morning, the one who can't wait to book the next plane ticket. Those people you were will come back into your life. Welcome them home.

But to call transplant a 'cure' is perhaps a bit misleading," she continued. "Transplant is a trade: giving up a disease that is killing you for another disease that carries with it medication and risks. Post-tx your disease becomes your immunosuppression. You are left vulnerable (not weak) and these rules are the best advice we have right now to keep you safe. For those of you with CF, as you know, you will also still have CF throughout your body, just not in your lungs. No one said transplant would be easy. I will tell you that everyone I have ever asked has said it was worth it."

The room was silent but for the hiss of oxygen. Her words, so raw and honest and compassionate and TRUE, echoed in all our heads throughout the rest of her talk. It was then, as I was standing to go and gathering up the tubing from my own O2 concentrator, that I made a promise to myself.

Whatever this "cure" threw at me, I was going to take it with as much grace as I could muster. Whatever the risks, I was in this for the long haul. Whatever came from this transplant, I would welcome it home. 

Flash forward 6 years, two transplants, and countless little (and not so little!) "bumps in the road" and I am here to tell you without hesitation: it has been worth it. I thank my donors with every breath for the experiences they have given me, from my marriage to watching my nephew take his very first breaths. I carry those moments, and so many more, with me always, so that when my "new" disease of immunosuppression and all that comes with it rears its ugly head, I can remember why I chose this road in the first place.

This past Wednesday, one week ago, I had a small surgical procedure to remove some high-risk cells from my body. Unfortunately, cancer is one of those not-so-fun risks that gets higher when a patient is chronically immunosuppressed, so any possible sources have to be caught early and treated aggressively. In my case, doctors were able to use a combination laser and excision treatment, and the good news is they are extremely optimistic that the surgery might be the only treatment needed. And as strange as it is to know I live in a world where "possible" cancer treated with a surgery could be considered "easy" -- the fact is that I remain incredibly lucky. To have lived long enough to experience these side effects, to be healthy enough to face them and recover, to have so many people by my side who are there for me and doctors who care enough to listen and know enough to get the job done. To have this life, with all its crazy side effects and twists and turns and miracles -- well, that is the gift that truly brings me back to myself, over and over.

And I will welcome her home. No matter what. 

Thursday, December 25, 2014

Noel

When I started this blog back in 2008, I had no idea what I was getting myself into. 

I was 26 at the time -- one year out of law school and knee deep in my first "real" job as an associate in one of New York City's major law firms. I was young, happy, excited about my career, and just on the cusp of multiplying my life by the sum of one very active and playful little furball/puppy. You could practically hear the themes song for the Mary Tyler Moore Show swelling around me as I tossed my proverbial beret high into the Manhattan skyline. Oh, and I had just been told by my doctor that after 26 years of living with cystic fibrosis, it was time for me to start the evaluation process for a double-lung transplant.

To quote Mr. Dickens: it was the best of times, it was the worst of times. 

I don't really know what I expected out of this blog, but I can tell you pretty honestly what I didn't expect. I didn't expect the following that you guys have given me -- the beautiful comments or the thought-provoking questions. I didn't expect the relief of sharing my story across the wires -- how crucial to my sanity and truly cathartic this online journal of sorts would become as my journey progressed. I didn't expect the joy or the fear that comes alone with opening one's heart (and lungs) up to anyone with a keyboard and the desire to read along for a few pages -- the excitement of logging on to a blank page and the thrill of anticipation when I finally hit that "submit post" button. I didn't expect that I would write my way right through that first transplant and on to the craziness of chronic rejection. Heck, I didn't expect to ever be writing the words "first transplant." I certainly didn't expect to be here -- 2,000 miles, 6 years, 2 new sets of lungs, and one wedding ring later -- trying desperately to find the words to restart the story.

Yet here I am. And on Christmas Eve no less.

I think it's fitting that the eve of my second first transplant anniversary falls on a night in which we celebrate a birth. It comes at a time full of childhood magic, and it brings with it the wonder of a night that -- through the light of a single star -- brought with it a whole new dawn. It is a night that changes as we grow, moving from the candy-striped glitter of reindeer hooves and elf-made toys to the hope and prayers for new life, for peace, for all those intangible gifts that we never once thought to ask for from Santa. It is a night that reminds us all how one life can change the entire world. That the story as we know it might be constantly changing, but it is far from over. 

So as I lie here tonight, still at last after the sickness and the chaos of the past year, the breath within my chest an even and soothing rhythm, overwhelmed with gratitude and surrounded by grace, I am struck by one thought to get me through this first Christmas Eve with my beloved Donor Nick:

All is calm, 
All is bright.

And the story is just beginning. 

Monday, January 27, 2014

Leaky

Hi there, lovely readers.

I've had a lot of you ask via the blog or FB or messages how I'm feeling lately, and I guess the best answer I can give you right now is that I'm feeling a little, well…

I'm feeling a little leaky.

As of right now, I'm still in the hospital with two chest tubes: one "regular" tube that has been in since my transplant and one "pigtail" tube that was placed last week as a way to combat something called a "pleural effusion" (layman's translation: fluid in chest cavity around the lungs). Both tubes have been working on draining fluid, and the regular tube has also been hooked up to suction (exactly what it sounds like -- just a tube that sucks out air) to remove subcutaneous air from under my skin. That would be air that leaked in through -- you guessed it -- the chest tube. Confused yet? Good, because I am too.

Basically, the story is that I'm leaking some fluid, and I'm leaking some air, and both of these things need to be resolved before I can really think about leaving, which is way more fun than leaking any day. All of these things have also left me feeling a little "leaky" emotionally, meaning I've just been a little drained, a little defeated, a little not-so-Piper-like lately. And that just means that this 30+ day hospital stay, as necessary and wonderful as is it is, is getting a little old. There, I said it. And you know what? I'm not even sorry.

In fact, I even told my docs as much the other day, pointing out that my mental state recently has been a little harder to control in terms of keeping my head in the game and staying positive. Don't get me wrong, I'm not flying off the handle (yet). I'm not depressed (yet) or overly anxious (yet), but I believe in being proactive and I believe there's more to life with illness than pretending this stuff is all sunshine and rainbows all of the time. So I asked my team what we could do to make some progress over here, and they came up with a reasonable, medically appropriate answer.

Right now, we're conducting an experiment. We plugged one of the chest tubs 24 hours ago, and so far the fluid accumulation and air leaks have not gotten worse with that tube out of commission. Should that trend continue for another 24 hours -- a full 48 in total -- then we may just pull that tube and see what happens with only one tube left. It's a good experiment because sometimes the body just needs a little urging to start doing its job and picking up the slack from the leaky fluid and air, and it looks like that might even be true in my case. Believe me, all fingers are crossed and all our thoughts are dry and air-free over here.

So that's the latest and greatest from over here in our tubular, leaky little world. My doctors have also "prescribed" lots of walking and some time outdoors as often as possible, for which the North Carolina weather is a blessed help right now. I'm happy that the team is taking my restlessness seriously while continuing to balance my physical health needs -- it shows concern for me as their patient on both human and clinical levels. All in all, that's probably the best indicator I have that things are looking less leaky around here -- for the tubes and the tears.

And here's to all of it drying up!

Thursday, January 23, 2014

On Looking Forward, Looking Back

**Some reader discretion advised -- tough CF topics ahead.**

It happened about a week ago.

I was just sitting here in my snug little hospital room, minding my own business and catching up on some emails when all of the sudden my eyes fell upon a message addressed to this blog's mailbox. I reached up excitedly to click open the email (have I told you all lately how much I love hearing from you? Seriously.) and there it was: the question I knew was coming. The one I was never really sure how I would answer, but that I knew I would have to eventually -- as much for myself as for anyone else, really.

Hey Piper, it's [name withheld] and I had a lung transplant several years ago. My doctors tell me now that I am most likely in chronic rejection. We are working to stop the progression of this terrible disease, but if not I may be forced to consider retransplant. I always said I didn't want another set of lungs; now I'm not so sure. How did you make this decision, and what was the hardest part of it for you? Sorry if this is too personal. I just really need some advice on the issue. Thanks.

Nope, thank you.

As soon as I read these words I flashed back to the period just after my first transplant, during the long (and somewhat complicated) "recovery" period. I was excited to finally be doing well with my new lungs, by which I mean still on IVs but breathing deeply and generally beginning to feel like Piper again. Best of all, I was feeling like Piper the Sequel -- still me, but with the added ability to do amazing things like, say, walk down the block without coughing or even (gasp!) jog a few feet. Pretty impressive, if I do say so myself.

So anyway, I was feeling pretty good, but I was still fresh off the table in a sense, and I distinctly remember telling my parents that I didn't think a second transplant would ever really be in the cards for me. Why not, asked my astonished and somewhat shell-shocked mother (note to self: right after transplant surgery is probably not the best time to bring up a second transplant surgery, especially to the mother of the 28-year-old recipient). And my answer was pretty simple, maybe even brave.

I calmly explained that I only wanted to die twice. I had been to the edge and back with my first transplant, literally experiencing the pain and torment of watching my entire body shut down one small part at a time. Although I spent most of my time focused on the positive aspects of what I could still do even with my very limited lung function, new issues, seemingly small but adding up to much more in their totality, crept up almost daily to remind me of how sick I truly was. Nightly baths eased some of the pain, but they could hardly wash off the truth that my body, despite my strong spirit and my will to live, was dying slowly and very painfully. Suffocation is a terrible fate to wish upon anyone, as is the advanced infections that so often accompany end-stage CF. And as positive as I am that we will someday beat this disease, the truth of the matter is that people -- young people, vibrant people, strong people -- still fall prey to its evil ways every single day.

Thankfully, and because of the gift of my first Beautiful Stranger, Donor Bob, I wasn't one of them. But I came close. And from that time on I decided that I would only die twice -- the second time would be my final goodbye to this phase of existence. I was not yet 30, but I was at peace with that decision for myself.

Fast forward 3 years, and things were…different.

When I decided to pursue a second transplant, it was largely because my life, in the time between my first surgery and my diagnosis of chronic rejection, had changed. For one thing, I was in a new relationship, and I knew I wanted to stick around to see where this one went. For another, I was increasingly comfortable in my own skin as an adult and felt more at ease with the idea that I was not in control of every little aspect of my health, nor did I necessarily need to be. The idea of dying didn't scare me as much as it once had. And, of course, I felt the very natural, very human resistance to the notion of "giving up." A part of me, I acknowledged, had been training my entire life to fight against illness, and it wasn't about to stop now. And so I began the process of trying to halt my chronic rejection, but also began mentally and physically preparing for the day when I, too, might have to face the choice to relist, again, for the precious Gift of Life. And I knew that, should that time come, I would ask God once again to bless me with new lungs, and then I would work my butt off for them, even if it mean dying a whole other time over. The rest, I knew, was somewhat out of my personal control.

Look, guys, transplant: it's not for wimps. Neither is life with CF, for that matter. I've honestly never known a group of patients who, for their entire lives, are asked the way we are to endure pokings and proddings, hospitalizations and "procedures", invasions of privacy and personal space, treatments and medications and daily battles just to keep all the rest of it at bay -- and that's just for some of our "healthier" patients. But at the end of the day, most of us don't have much of choice besides being strong. What others call "inspirational" isn't even an option for us; we act that way because we have no other choice if we want to survive.

And so I took that second leap of faith. And miracle of miracles, after a brutal battle, it paid off. I can't even bring myself to write yet about the road I took to get to my second transplant, but I can tell you this: in retrospect, it was worth it, every step.

When it comes to life -- or death -- with this disease, there are no "right" answers to the tough questions like "should I get a second transplant?" What there are, however, are answers that are "right" for me, or for you, or for anyone else who might be struggling with the "what ifs" and the "what fors" of decisions most people will never even have to make in their lives. It's not an easy position to be in, and my heart goes out to all of you in that same boat for whatever choice might lie ahead, but my answer is always going to be the same: it doesn't matter what I chose or why, because this time, guys, it's all up to you.

And I wish you nothing but peace, love, and light along your journey.

Tuesday, January 21, 2014

On Waiting

Well hello again, beautiful people.

So as most of you probably know by now, I received a very special gift this Christmas -- December 25, 2013 -- in the form of sparkling new lungs from my newest partner in crime, Donor Nick. And despite my declaration at age 11 that I would never ever love a Christmas gift as much as I loved my new pink Huffy bicycle, I'm pretty sure this one takes the cake for the best.Christmas.present.EVER. To be honest all I really remember is the vague sound of the telephone ringing, the bumps in the early morning darkness as my boyfriend sprang up to get it, the thumbs up he gave me from the bedroom doorway, and then an overwhelming sense of peace that this was it, this was right, this was going to happen. True to his saintly name, my newest donor had arrived just in the "nick" of time.

He arrived in the nick of time because I was dying, literally. I had been placed on bipap for high CO2 and plummeting O2 saturations, but even that was barely working anymore, and I knew the next step was a ventilator. I was struggling just to keep my head above water in rehab, much less gain any real ground toward my strength or weight goals pre-tx, and my family had taken to staying up in shifts throughout the night to watch me breathe -- a brutal exercise in unconditional, heartbreaking love that I think any CF or lung disease family can relate to on some level or another. I knew deep down that there wasn't much time left, and they knew with me, and yet we all still hoped, wished, and prayed for a miracle.

And then it happened.

We had been teasing and joking about new lungs for Christmas for a couple of weeks -- who wouldn't have? It was the perfect image -- Santa packing a living, breathing set into his bag and stuffing them down the hospital's chimney on that not-so-white Carolina Christmas morning. And yet the reality is that it wasn't a jolly fat man from the North Pole who saved my life that day. It was a beautiful stranger and his amazing family who suffered the ultimate tragedy of losing a loved one and a family member on that holiest of holidays, on the day of birth and celebration, and who chose to turn around and change that grief into hope, into love, into a gift from one human to another that is beyond all measure -- the Gift of Life. It is to them, not to some dude in a red suit, that I will always be thankful. It is to the miracle of organ donation, that brings life from death and rebirth out of worry and tears and so much hardship, that I will forever owe my life. And I promise to use this gift as it was offered: in faith, in compassion for others, and most of all in love. I promise to celebrate the life inside of me every single day.

Which, so far, means a whole lot of hospital celebrations. I'm coming up on week 4 in here, which is not exactly rare for retransplants as they are "messier" jobs than the first. That seems to be the case with me as the only thing keeping me here is a single chest tube which 1) refuses to stop draining fluid, and 2) is now also necessary to drain some small air leaks that have caused what is known as "subcutaneous air pockets" underneath my skin and primarily in my face. Yes, it makes me look funny (think "Fat Albert" on steroids), but at least the chest tube seems o be doing its job in bringing it down.

So now, ironically, I wait. I received my gift, and I'm happy to say that everything went wonderfully during the surgery (though they had to cut poor Donor Bob -- gone but never forgotten -- out of my chest piece by piece given the sorry state of my lungs this time around). Donor Nick looks great and my first bronchi came back completely clear of infection and rejection, indicating that he is happy and healthy in his new home. For all of that I am grateful. For the tubes and suction and the solutions to the "bumps in the road" both big and small, I am grateful (if sometimes a little impatient). And for the chance to experience all of this -- for the moments, for the lifetime, for the breath -- I am forever indebted and forever grateful beyond measure.

And that, beautiful people, is worth the wait.

Monday, January 13, 2014

An Introduction

Dear Readers, Followers, Lurkers, Commentors, Visitors, and All-Around Beautiful People:

First of all, on behalf of Piper, Sampson Bear the Dog, Piper's Family, and everyone else who makes this blog possible, we want to apologize for the recent lack of postings. Please rest assured that, despite the gap, our current mission remains the same as always: to bring you the most up-to-date anecdotes, randomly charming musings, and (strangest of all) true stories of life with CF and lung transplant -- and everything in between! Please also know that the vote is in on blogs with the most amazing, loyal, and forgiving reader base and we are delighted to announce that you all have won the prize, hands down. Congratulations from Piper and all of us!

Unfortunately, as we begin this new year of blogging and being, we do so without the help of one of our crucial team members thus far. As some of you may know, our beloved Donor Bob, whose breath and spirit proved invaluable not just to this blog but to all the experiences, moments, and people behind it, has moved on to greener pastures. Donor Bob graced us with his presence for 3 1/2 years, during which time he enabled Piper to do "Amazing Things" such as return to school for a master's degree, watch some of her closest friends begin families, celebrate her parents' 40th anniversary, turn 30, and meet the love of her life. Now if that's not a gentleman, I don't know what is. Sadly, Donor Bob fell ill early in 2013 and simply never recovered, despite aggressive treatment, to the point where we finally said our goodbyes on Christmas, December 25, 2013. May we always remember him with love and gratitude.

And now for our happy news: Welcome Donor Nick!

We are excited to welcome Donor Nick to the team after his miraculous and perfectly timed entrance into our lives this Christmas Day. (Piper's side note: I actually went into surgery on Christmas around 6:30pm and got out about 12:30am on the 26th, which also happened to be my parents' 43rd wedding anniversary.) Donor Nick has bestowed upon our girl two beautiful new lungs that apparently were so large they had to be "stuffed" in there like a couple of Christmas hams. We'll take it. Donor Nick, thank you for joining our wonderful, chaotic, crazy, beautiful lives. We are, as we remain with your predecessor, eternally grateful for the gift of Life you brought us through your Christmas Day miracle.

It goes without saying that we are blessed beyond measure by this new addition -- and it shows. Piper is recovering well, though still in the hospital in a pulmonary "step down" unit that affords more personalized nursing care. The doctors say the only thing keeping her inpatient are the chest tubes, which are taking a longer time to "dry up" than they did after her first surgery. Much of this is par for the course on second transplants, which tend to be messier business because of scar tissue and other complications. Thankfully our faith in the medical team never waivers -- we know we are getting some of the best care in the world and feel very much at peace with where we are today. Although Piper reports that she would really like to be reunited with that shaggy little mutt of hers one of these days. In good time, Piper. In good time.

So there you have it, friends and loved ones: all the news that's fit to print. And As we gear up for a big year in 2014, please know that we would be honored and delighted if you would continue to share the ride.

Sincerely,

Piper, Sampson, and All of Us


Tuesday, October 29, 2013

'Tis the Season

I originally posted this as a status update on my personal Facebook page as a way to respectfully chime in to one of my favorite seasonal traditions: the great flu shot debate. Two hours, 22 shares, and 16 comments later, I realized this was an issue that people need to be talking about, and so I decided to take it to some of the coolest and smartest folks I know -- all of you. Please be aware, of course, that this is my own highly personal perspective as a person, as a CFer, as a transplant recipient, and as a a strong believer in community health. Because what we do matters. I promise.

Every year around this time I watch for the first flurries. I sit in wait for those swirling flakes of debate that go hand in hand with the coldest season of the year and the inevitable question that, sooner or later, we all have to face. Should I get a flu shot?

Honestly, I can't answer that question for you. No one can, except maybe your doctor or your child's school depending on its vaccination rules. I can't tell you whether it's worth the so-called risks of the shot itself, or playing the bad guy and bundling your kids off to the doctor again. I haven't read every piece of medical literature out there on the subject, nor could I even if I made it my full-time job. I'm not here to discuss conspiracies or WHO statistics or the devastating spread of preventable disease in countries where vaccinations are not available. I'm not here to police your decision at all, in fact. I'm here to share mine.

I don't have a choice in the whole vaccine thing because when I got my lung transplant they made me sign a paper promising that I would stay current on all vaccines for the rest of my life (except live vaccines, which are dangerous for me). So every year, while other folks debate, I simply trudge out to my doctor's office, endure the poke, and keep my promise to myself, my healthcare team, and my donor. That said, even my doc admits that vaccines might not work on me very well simply because I don't have much of an immune system to react to the dead virus and produce the necessary antibodies. Because of this, everyone who lives with me or shares close contact with me on a regular basis is also required to get their shot to help keep me (and my precious lungs) safe. That means my boyfriend is getting his shot for pretty much the first time ever this year. Bear in mind that he's not so super into needles, that one, but he's doing it for me anyway. Love is never having to say "I'm sorry my genetic disease requires me to purposefully destroy my immune system so that you have to get a shot."

Here's the point, though: if he didn't know me, he would most likely be just fine without the flu shot. True, over 40% of the children who died of the flu in recent years were apparently not high risk patients, but even so -- most normal, healthy folks will be just fine, even if they do get the virus. But the fact is that he does know me, and chances are good that you, too, know someone who is deathly afraid of catching the flu for a really good reason. Chances are you know someone, even if you aren't aware of it, who has a lower immune system capability, or an underlying disease, or is elderly, or is pregnant, or . . . well, the list is pretty extensive, actually. These people are afraid of the flu because, to us, this virus is way more than an inconvenience or a few missed days at the office. And we care about the flu shot -- yes, even your choice to get the flu shot -- not because we want your arm to be sore or because we get a kick out of watching healthy folks facing their fear of needles. We care about it because this issue, for us, is about more than your right to build up your 7-year-old's immune system "the natural way." It's about our right to go outside of our houses between the months of October-April without risking our very lives because that 7-year-old (or his parent, classmate, teacher, or someone else who caught the flu after him) is in front of us in the grocery line.

Vaccination is a personal choice, right? But spreading germs once you catch them is not. Even with great hygiene and the best of intentions, those pesky little things have a way of making themselves at home on classmates, coworkers, friends, loved ones, public surfaces, and all sorts of other shared spaces. And maybe that's not a huge problem for you or for your child. In fact, maybe it's a risk you are willing, able, and even happy to take. That's fair. But before we make these so-called personal choices about preventative healthcare, maybe we should all look to our right and look to our left and then ask ourselves honestly: are we truly willing to risk the health of our neighbors (friends, family, strangers on the street) for the sake of this one "individual" decision?

Because that's the kicker with community health, guys. It's never as personal as we think it is.

So if you choose not to get your flu shot this year, please also consider staying home if you or a close family member is ill. Please consider the fact that most people shed virus for at least a day before they even show symptoms of a virus like influenza, and please consider educating your children about proper hygiene habits like coughing into their upper arm, washing their hands frequently, and sneezing into a tissue whenever possible. Most of all, please consider how your choices may impact the people around you and their overall health and well being as well, and once you've considered all of that, please make your personal, individual choices wisely.

Our health depends on it.