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.