Wednesday, July 29, 2015

Swimming Lessons

I learned about undertow the hard way.

When I was young, probably around 5 or 6, my parents took my sister and I on vacation to one of those all-inclusive beach resorts called Kona Village. It was a childhood paradise -- the type of place where kids could run (relatively) free within the safe confines of the resort's borders, and fun in the sun with endless sand castles was just about all I had in mind. My sophisticated and daring 8-year-old sister, on the other hand, had bigger plans. And those plans involved something strange and new to my land-locked state vocabulary: the boogie board. 

For those of you who might also have grown up in Colorado (or one of the other interior "Square States"), a boogie board is the shorter, fatter version of its more sophisticated cousin, the surf board. The purpose of a boogie board (at least when you're a small child) is basically to dog paddle your way far enough into the ocean to catch one of the small, non-threatening waves and "ride" it back onto shore by lying on your belly and holding onto the board for dear life. And yes, it really is almost as much fun as it sounds. 

And so, after my father had dutifully fitted us into damp and uncomfortable life jackets and the guy at the equipment stand had demonstrated the dos and don'ts of boogie board etiquette (don't stand up on the board, do keep the board attached to your wrist at all times, don't use the board to hit your younger sister when no one's looking), we grabbed our boards, marched past the several life guards on duty, and headed bravely toward the surf. 

My dad did the first run, demonstrating beautifully how to ride a tiny wave, which would have been graceful had he not been 6'2" and trying to hang 10 in approximately 2 feet of water. Needless to say, he promptly washed up head first onto the beach, and I made a mental note: too close to the shore equals sand in the face. Lesson learned. I was ready.

Buoyed by determination and my life jacket, I launched myself into the surf and swam for the waves. When I saw one that looked suitably ginormous (a towering wall of water almost a foot and a half high!) I went for it. My board caught water, picked up speed, and rushed toward the shoreline 10 feet away. I was on top of the world! I grinned! I laughed! I raised my fist in pure, sweet, childhood victory!

I fell.

Thirty years later, I'm still not sure what happened. Most likely the wave broke, which I was not expecting, and I got tossed into water that was about 2-3 feet deep at its lowest point but that felt like the deepest, darkest ocean. My board was still attached to my wrist but now something was suddenly sucking me backward, away from where I knew the shore should be. The water pulled me toward it, dragging me under, throwing another wave into my face. My eyes burned and my mouth seemed full of salty panic when suddenly, as if from nowhere, I felt hands lifting me up. My dad removed me from the undertow, righted my board and set me back on my feet right where I had been thrashing: in shallow water, just steps from the shore. The entire ordeal had lasted less than 30 seconds; it felt (still feels, as I remember it) like hours. 

And we walked out of the ocean, together.


Childhood water sports not withstanding, I've always considered myself extremely lucky. If cystic fibrosis is my ocean, well then I think it's fair to say that I've become a master at riding the waves. I always strive to have the right equipment with me and I try to pay attention to the examples set by others. But still, every so often, it's hard not to venture too far off the shoreline, to find a wave so large you're scared to ride it, to get knocked off your board unexpectedly. These things are, in fact, a part of every life, CF or no -- all of us will at some point or another find ourselves out to sea, scared, or even flailing in the water. And some of us might also, through no fault of our own, sometimes get sucked down into the undertow of anxiety and depression. (Both of which, by the way, are common in people living with chronic or life-threatening illnesses, including CF. So much so that studies are now being done specifically focused on these issues in people with CF, and hopefully that means more discussion around a serious, and not at all shameful, topic.)

Look guys, I've struggled with anxiety and I know how tough it can be to talk about. I know the feeling of sitting in a doctor's office discussing lung function and liver enzymes and failure to thrive and thinking "what is this doctor going to think if I suddenly bring up anxiety attacks or sad thoughts in the middle of all these 'real' issues? Will I still be eligible for transplant? Will they stop listening to my complaints if they think I'm just anxious or depressed all the time? Will they think I'm crazy? AM I crazy?! I suck it up and deal with all the pills, treatments, coughing, nausea, oxygen, and [insert therapy or symptom of choice here]. Shouldn't I just be able to suck it up and deal with this depression and/or anxiety BS too?" I've been there, I promise, but I also know that depression and anxiety aren't just real -- they're completely normal. And take it from a girl who knows: if you get to the point where you're anxious about asking for help for your anxiety -- it's definitely time to reach out. 

When I capsized my board, I was wearing a life jacket and attached to a floating piece of plastic. I was surrounded by loved ones and trained professionals who knew how to help me. And yet, in that moment I felt terrified -- unable to separate the shallow water beneath me from the great depths of my imagination and certain that what had once seemed fun and manageable was suddenly dangerous and out of control. I needed help, and thankfully I was able to accept it when I saw it, in the form of a loved one's outstretched hand. He helped me stand back up, on my own two feet, because once I knew where I was and saw where I was going, I knew I could get there. I just had to take that first step. 

And we walked out of the ocean, together. 

Thursday, July 2, 2015

On Vertex and Victories

I woke up this morning just like most days. Bleary-eyed and exhausted after a fun night of fighting transplant insomnia, I half walked, half stumbled my way into the kitchen to grab a cup of water and a few crackers before making my way toward my medical-supply shelf. Blood sugar safely checked and vital signs good, I reached for a handful of pills, gulped them down, and began the morning ritual of planning my next high-calorie breakfast. And, in the meantime, I reached over next to me and picked up my phone.

And just like that, my day got better. 

There's nothing quite like waking up to a slew of texts that the newest CF drug -- the next source of hope, another chance at health, at growing older, at LIFE -- has just been approved by the FDA. Even if the drug can't help you specifically (this current drug is not yet being marketed towards those with my specific mutation combination, nor has it been tested on folks who are post-transplant), just knowing that there's a new option out there for people like me, people I know, and people I call my community is enough to bring tears of joy and gratitude into my eyes. Because I know that somewhere out there in the world, at least some of the 70,000 other beautiful people who "breathe like me" are waking up to the news that their world may be about to change. That they, too, may soon be offered the chance to reap the benefits of this newest little miracle. That, if nothing else, they have a chance now -- a chance to see if this could be their time, their magic, and THEIR moment. These are my brothers and sisters in arms, after all, and who am I not to delight in their success?

But then again, that's not entirely true. I have my selfish motivations as well.

You see, today, July 2nd, 2015, is a HUGE day for the CF community as a whole. This is not because Orkambi itself is a "cure" (it isn't), or because we've finally achieved the goal of having a corrector option for ALL people with CF (we haven't, and we will not rest until we do), and it's not even because this could mean longer, healthier, and fuller lives for the people who CAN benefit from this new drug (okay, that one is totally true). Today is amazing simply because it represents yet another victory of our incredible community over this deadly little monster called cystic fibrosis. This approval, in other words, represents the strength we have in numbers (however small we might seem) and the breathtaking tenacity, courage, and commitment of our team against what sometimes can feel like an unstoppable enemy. It represents US, beautiful people. Because believe it or not, WE made this happen.

Without participants for the study, this could never have happened.

Without fundraisers, walkers, climbers, bikers, and hikers for CF, this could never have happened.

Without the tireless efforts of our determined advocates, who lobbied so hard for access to clinical studies for those with orphan diseases and for evaluation standards by the FDA, this could never have happened. 

Without our community's willingness to stand alongside the Cystic Fibrosis Foundation as it took a HUGE risk in developing new and innovative approaches to rare-disease research (approaches now being reproduced by others for their own success), this could never have happened.

Without our incredible showing at the FDA committee meeting in May, where our patient representatives spoke bravely about the impact Orkambi has had on their lives and a professional blogger for the investment news group TheStreet exclaimed that he had never before had so many actual community members tune in to his live blog of the meeting, this might never have happened. 

Without our refusal to take no for an answer, this could never have happened.

But it did happened. It's happened twice now. And that means, quite simply, that it can happen again. And again. And as many times as it takes, until WE make cystic fibrosis a thing of the past. 

Happy Independence Day, beautiful people. You certainly earned it.

Thursday, May 21, 2015

What I Know Now

Here we go again.

In so many ways it feels as though the last year or so has been a total whirlwind. We've now gone through a full set of seasons since I officially left Durham, North Carolina and brought my new pal Donor Nick back home with me to Colorful Colorado. And while I wouldn't exactly say it's been easy ("easy" is not a word I tend to associate with either cystic fibrosis or lung transplant), I can say that it has most definitely, without a doubt, been worth it.

As a little bit of background, let me just say that this second double-lung transplant was, no question, the single most difficult thing I have ever experienced in my 33 years of living with a chronic and deadly illness. Seriously, guys, this one not only took the cake in that contest, it took the whole darn dessert tray. To say that the process of retransplantation left me feeling somewhat stunned, shaken, and well, just kind of "un-Piper-like" would be a little bit of an understatement.

Now, before I go any further here, I really want to emphasize that this is definitely NOT the case for all second lung-transplant recipients out there. Doctors do tend to warn retransplants, as we're called, that recovery is often tougher because the surgery itself is more complicated. For one thing, by the time you get called in for your second set of donor lungs, you've already had at least one surgery in the exact same area, meaning the doctors are much more likely to encounter scar tissue in there. For another, years of immunosuppression and other pretty hardcore therapies leave the body somewhat vulnerable, and recovery is always easier when you're in the best shape possible going into the surgery. But even with both those factors (and a whole lot of other complicated medical issues) in play, some people breeze right through their second transplants. I even have several friends who found the second surgery to be easier than the first in terms of recovery time and post-surgical complications. And to those lucky, wonderful people, I can only offer this: congratulations, guys, because as far as I'm concerned, you all are clearly superhuman.

For me, the second transplant was a rough ride almost from the get go. I didn't actually feel as bad in the months leading up to it as I had with my CF lungs before my first transplant -- my numbers were dropping, but my spirits were remarkably high (note to self: falling in love is pretty good medicine), I had a lot of faith in the treatment options open to me, and I was still able to function more or less as I had been doing before the rejection hit. Even after we tried a drug called Campath to stop the rejection and it failed to do so, I still felt fairly confident. As it became more and more clear that retransplant was my only viable option, I readied myself for that possibility as best I could by continuing to stay active (with oxygen), using supplements to hopefully increase my weight, and talking with my doctor about which transplant centers might be right for me. I knew I wanted to relist at my original center, but by then I was also halfway across the country from them, plus I knew I needed to be able to get lungs FAST in case my PFTs continued to slide rapidly. So I chose a new center, flew out there, got evaluated, was accepted to the program, took all the necessary steps to double list with my original center, and was well on my way. Which is right about when I learned I had cancer.

Yeah, you read that right.

Thankfully what I faced was "only" what is known as Melanoma in situ (aka, Melanoma stage zero). Basically, this meant that after two facial surgeries to remove all the cancer cells and a clear PET scan, I could most likely move forward with lung transplant. Also at around this time they discovered that I had multiple pulmonary emboli due to a failure of my anticoagulant medication. By the time those two issues were addressed, I had come in for a routine clinic visit and discovered that my lung function was sitting below 20%. It was definitely time to move.

To be honest, so much of what happened after that initial flight to Durham and introduction to the transplant program there is a hazy blur in my mind. Even now I have huge gaps in my memory from that period (we later found out I was retaining dangerous amounts of CO2, which is probably enough to make anything a little hazy), although I do recall some of the basics. I know I had a dry run in which I flew back to my original center in hopes of receiving lungs, but it didn't work out and I got sent back down to North Carolina and spent nearly 48 hours completely asleep. I know my oxygen levels started dropping so low at night that my husband and parents decided to forgo sleep in favor of keeping an eye on me so they could wake me up if anything scary happened. I know I stopped eating entirely and for the first time in my life had to be fed exclusively via a g-tube that was inserted in my stomach, and even then I was below the acceptable weight range for listing. I know that walking around a flat track in pulmonary rehab with oxygen had become more strenuous for me than a hike up a 14,000ft mountain would have been in my healthier days. I know that a friend came to visit for Thanksgiving and my birthday and left somewhat convinced he might never see me again. I know I blew a PFT so low that I didn't blow again until I was transplanted, over two weeks later. And I know that at some point in that whole mess I ended up in the hospital, on a bipap machine, having multiple anxiety attacks every day and literally begging my team to find me lungs.

And, of course, I know that they did.

I knew the second the phone rang at 8am on Christmas morning that this was it. There was no question in my mind as I listened to Patrick talk to the nurse from behind my bipap mask. I was calm, I was ready, and I knew this was probably my very last chance. So I instructed my mom to bring the bipap with us, switched over to my oxygen machine for the car ride, sat down in my wheelchair to say goodbye to Sampson Bear, and away we went with the lights from the tree winking their goodbyes. I wouldn't see that apartment again for 40 days and 40 nights, exactly, and it was without a doubt a passover for my entire family. That was the day we all said goodbye and thank you to my beloved Donor Bob and welcomed Donor Nick into our family. It was also the day I started what has -- and will likely continue to be -- the long process of "recovery" from the life-saving, life-changing, and 100% life-affirming journey of lung transplantation.

But there are also other things that I know and remember out of our time down south. I know the doctors, nurses, and staff were incredibly kind to us and gave me the best possible medical care. I know that despite having a relatively complicated case, my team did everything they could to get me lungs. I know that I met some absolutely amazing people at the rehab center while walking that dreaded track -- people who could make me smile even when my lungs were screaming and who were remarkable in their own strength and resilience throughout the process. I know my family dropped everything to be with me, and believe me when I say that I know how lucky that makes me. I know someone chose to donate life, to make possible new beginnings out of their own worst moment, and for that I know I will be forever grateful. I know that however long or rocky that initial road out of the hospital was for me, it was doable, because I know lung disease is not for the weak of spirit. I know I laughed sometimes, I know I smiled, I know I cried tears of raw and real happiness and gratitude. I know God listened. I know I read each and every card I received. I know I'm still breathing. And I know that now, for perhaps the first time in over a year, I can type these words and mean them:

I know that I am still here -- and that I will continue to heal, month by month and year by year.

For a lifetime.

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.