An Inartistic, Overdue Update

August 14th - I had my third outpatient surgery to remove the toothpaste-consistency phlegm from the sinuses behind and around my eyes and above my eyebrows. The surgery went extremely well, despite the initial bleeding and pain which lasted 24 hours.

September 4th - I went to the CF Clinic to see Dr. Simon. My lung function was lower than usual, but not alarmingly so; I went on oral antibiotics and was scheduled to return in a month. My prescription for Zoloft was also doubled from 25mg to 50 mg, and I have found that my anxiety has since increased in frequency but decreased in severity.

September 29th - After my sixth straight week on oral antibiotics (I was already on different ones before the September 4th appointment) with no sign of improvement, I went to the ER and was admitted to the hospital. I had lost 7 pounds and had loud crackling in my left lung, so I was probably pretty close to developing pneumonia again, which would have been disheartening, considering I just had severe pneumonia in June.

October 5th - I was discharged from the hospital on one IV antibiotic and two oral antibiotics. That night, I got an awful headache and took my temperature to find that I had a low-grade fever. For the rest of the work week, my cough, headache, fatigue, and joint pain worsened.

October 9th - Deciding that I couldn't take the pain anymore, I contacted Dr. Simon's office online, and they advised me to return to the hospital. I did so.

October 16th - I was discharged once again from the hospital, this time returning to my home in Saint Joseph instead of my apartment in Ann Arbor. I want to be with my family while I finish healing, and I plan on going back to AA on Tuesday. My course of IVs will end on Sunday (tomorrow) and I will deaccess my port then. I'm struggling with the pressures of school and the feeling that my body seems to be deteriorating faster than it used to, but I have a greater support system than I could reasonably ask for. Thank you to my parents, Vinny, and the close friends who have taken the time to check on me. I'm not sure what's coming next, but I'll do my best to take it in stride.

Since Coming to College . . .

. . . I've learned not to place my happiness on the shoulders of those around me.

It took hours of sleeplessness, tears, and phone calls to my mother, but I have finally realized that putting the entirety of my emotional health into someone else's hands ends up being detrimental to both parties. This habit is one which I developed halfway through high school.  The silver lining? The fact that I developed it at all was because there was a very special person in my life at the time who was willing to carry me through the peaks and troughs of 16, 17, 18, and 19. But, as Chinua Achebe wrote, "things fall apart." During that age of heavy maturing, I realized that my happiness was at the mercy of someone who wasn't me, and why would that ever be something to desire? Now, as long as I have myself, I'm okay - and I'm very thankful to the person who taught me that, however painful it was for both of us.

. . . "cystic fibrosis" is no longer abstract.

Since the fifth grade, I've been admitted to the hospital two times a year. That was my routine, and as much as recurring lung infection can "work" for a person, it worked for me. Now, after watching my health decline more significantly and more quickly during these past 11 months than it ever has before, I've come to understand why the median life expectancy of someone as mutated as me is 37-years-old. Never in my life have I been able to imagine myself as an old lady, but I've never been able to imagine myself dying young, either. When I pondered the demise of my existence, it was always fuzzy. At the hand of my freshman year of college, with its loneliness and excitement and independence and busyness, there is a port permanently in my chest. Health insurance companies are knocking on my parents' door every day to collect bills I never used to acquire. I can no longer run up a flight of stairs (though I'm working on it). My health is now at a very tricky stage, and I'm forced to face that if I let myself go, today, I'd plummet. If I ignored my pills and physical therapy, I'd plummet. If I stopped exercising, I'd plummet. If I chose to stop fighting for my life, right now, there is a very big chance that I could begin to die. This is something I'm thinking about for the first time, but now that I am, I'm choosing to win. Now I CAN imagine myself dying young, but that's the only place where that scene will play: in my imagination.

. . . sandwiches have replaced cereal as my official "lazy meal."

Milk expires quickly. I don't have time to keep buying milk.

. . . I've stopped waiting.

There's something magical about saying "When I grow up . . . " with stars in our eyes. It elicits the idea that the future is a place of more possibility, luck, and motivation. Operating with the future in mind, we - naturally, as humans - become complacent. There's always tomorrow. There's always tomorrow, tomorrow, tomorrow. But what if I don't want to grow up? What if my best friend revealed that my most sunshine-y quality is the fact that I am childlike? If I never grow up, where is my magical future where dreams are possible and motivation floods my blood? Well, I know I'm still (barely) a teenager, but I think it's here. I want to read and write and make videos and help people and spread awareness for cystic fibrosis, and I want to do those things seriously. Professionally. I'm never growing up, so I'm never going to receive a message from the universe that reads "Do it now." Why would I wait until graduation day to begin chasing my passions? To begin helping people? I wouldn't, so I'm not. And let me tell you, there are big, big things on the horizon.

I'm Back

My little feet are exhausted and I'll tell you why!
1. I've been running.
Your eyes are not deceiving you! I finished up my three-week round of IV antibiotics on Monday, and my home care nurse came that day to deaccess my port (this means she removed the needle, but the port itself will remain under my skin for the next time I get sick). Later that night, I went on my first run since the winter. I won't lie and say I went very fast, but I did go far - 40 minutes worth of jogging/walking! My lungs were hurting, as were my legs, but at one point during an inspirational song I honestly almost cried out of happiness. Kinda lame, I know, but I just felt so proud of myself. It's been scary to see my health decline over the past year, so choosing to fight for my life has renewed my sense of purpose in the world. I've gone on two runs since the first, and I think they're already starting to get easier.

2. Class is back in session.
I haven't stopped taking classes since I first moved to Ann Arbor in the fall. Although the spring term ended two weeks ago, I had to take two "Incompletes" and I am still making up assignments. But I think Zoloft (my anxiety drug) has started to work, because I haven't yet had any panic attacks about catching up; fingers crossed that it stays that way! Summer term started last week, and I'm really happy about my decision to drop one of the classes I was going to take. Just having my Introduction to Literature class on Tuesdays and Thursdays is giving me a lot of time to do extra treatments and rest, so hopefully my lungs will get back to where they need to be within the next few months.

3. I went back to work.
Tonight was my first shift back at the coffee shop since I got sick, and I was ridiculously nervous to close by myself. I was only on my second week of the job when I had to go to the hospital, so I was unsure about my ability to remember all of the drinks. It ended up going really smoothly, and I found a little surprise in the tip jar at the end of the night. :)

My lungs have been pretty junky, these post-IV days . . . I'm having trouble going to sleep and staying asleep because I'm coughing more than I usually do. Additionally, I'm waking up super early with tons of thick mucus and chest pains, which isn't the most fun. I had a clinic appointment yesterday which my mom was able to come up for, and my lung functions weren't what I wanted them to be: only 63%. That number was a bit disheartening to see after JUST having gotten out of the hospital, but it just goes to show how sick I was in the first place. I feel really dumb for waiting so long to get treatment, but it was an awful time for me to be sick and I was hoping to just get over it. Unfortunately I set myself back pretty far, so it'll take a while to regain that lung function. But that's just even more motivation for me to stick with my running! I love this life and I want more of it.

Dr. Simon said that I can start taking Orkambi! He estimated that it will take 6-8 weeks for insurance companies to figure out how much of it they're going to cover, which better be 100%, because the drug costs $250,000 a year. I can't wait for pharmacies to start stocking it so I can see if it does my lungs any good.

Here's my most recent vlog - my mom and I took the camera to the CF clinic. Subscribe, if you'd like!

Best Day EVER!

I've been in a funk - a very funky funk. But today, the first genetic therapy drug for my mutation of cystic fibrosis was approved, and I think I've broken free.

Never before have I experienced such nauseating, debilitating anxiety as I have these past two weeks. I cannot name the root of my nerves, and believe me, I wish I could. It feels impossible to fix yourself when you don't know why you're broken. But today, I felt as if I was slapped in the face by the mane of a unicorn made out of gummy bears. 

Guys . . . Orkambi is here.

We'll skip over the complicated scientific explanation of what Orkambi will do for roughly 8,500 Americans (those with the double delta F508 mutation who are over the age of 12) and get right to the good stuff. In clinical trials, patients taking Orkambi gained 3% lung function over time. This may not seem like a lot, but it is important to understand that cystic fibrosis is a progressive disease. This means that it only gets worse. Any improvement at all is, quite literally, a contradiction of the disease itself! And while some patients did not see any lung improvement, they saw a significantly slower decline. On top of that, patients in the trial had an easier time keeping weight on, which significantly aids to lung function as well.

Right now, people with CF are skydiving without parachutes - once the disease hits its breaking point (and this happens at a different age for everybody), our plummets are fast and unavoidable. Orkambi is our parachute. Orkambi can slow us down. Orkambi can save us.

Now, I won't pretend that Orkambi has worked/will work for everyone. Different people react . . . well, differently. But the results are there. The science is there. And where there is hope, there is life. Dum spiro spero. 

I just want to take this moment to thank everyone for their support. I want to thank you (yes, you!), the human who is reading this sentence. Together, we bought this drug. When the government refused to help us, we payed the scientists to do their jobs. We gritted our teeth and went door-to-door. We sold t-shirts, baked goods, and purple ribbons. We walked mile after mile, year after year, shouting to anyone who would listen that someday, CF would stand for "Cure Found." And no, this is not a cure. But it's the closest thing to a cure that any of us has ever seen, and that is something worth celebrating. 

My Anxiety: Part One

I frequently worry, and I have for a long time. The physical manifestation of these worries can be observed by those who choose to be observant. Just a few weeks ago, a bus driver in Chicago locked onto my eyes as I hesitantly disembarked - Don't be nervous, honey, she said to me. But how couldn't I be?

• I was travelling alone. I had a train to catch. I was running low on money.
• My stop had either come and gone or had yet to come at all, but I wasn't sure which.
• People were staring at my clothes. People were staring at my luggage. People were.

Of course I was nervous, and of course she could tell. When the world around me crumbles, idiosyncratic breadcrumbs fall behind my feet. Visually, it is quickened breath, teary eyes, bouncing knees, a clenched jaw, and hands that fly from fists to jazz hands and back again. Metaphorically, it is a plea for help.

Since puberty, panic has been Plan A. Though I strive to present myself as a rather self-sufficient young adult, the word "overwhelmed" is one I use often when on the phone with my mother. For years, my life has grown more and more into a puzzle that I cannot put together, and when I can't find a piece, I cry. I pull my hair, I hyperventilate, and I shout to whomever can hear me that I need them. 

I saw a counselor from the end of 9th grade to the beginning of 12th. My grandpa's battle with Alzheimer's had been growing more gory, as had mine with cystic fibrosis. Like any other heterosexual girl of my age, I was in love with a boy who didn't seem to notice me, and a very close friend of mine was diagnosed with severe anorexia. I was unhappy. That same close friend recommended her counselor to me, and I went, spending over $100 of my parents' paycheck every week to snottily sob on a beige loveseat.

Growing up with a life-threatening illness, I was (and am) a faithful believer in medicine. After more than two years, I was diagnosed with nothing, and since I wasn't any better and my meltdowns were only growing more frequent, I stopped going.

The triggers of my anxiety are patterned; I break when I feel lost, unloved, or judged. Unarguably, however, the most debilitating panic attacks stem from my feelings of failure. This is the anxiety that rips into my relationships. This is the anxiety that one social worker noticed. This is the anxiety that diagnosed me with Anxiety.

Tonight, I begin the healing process. Tonight, I swallow a little green pill before bed and lose myself in a packet entitled "Free/Low-Cost Counseling Services in Ann Arbor." But then again, I hate to be lost.

Sixty-Five Roses

            I didn’t know that was going to be the last day. I swear I didn’t know. If I had known, I would’ve baked oatmeal raisin cookies instead of buying chocolate chip. I would’ve worn the earrings she liked—the ones that looked like little turtles. I would’ve brought a game she actually enjoyed playing. But I didn’t know, and I didn’t bake or wear or bring, and that was the day Rose died.

***

            “Took you long enough!” she yelled. I rubbed my hands with sanitizer upon entering room 223, trying not smirk at the way her voice cracked when she was irritated. Florescent overhead lighting mimicked noon in the worst possible way.

            “Oh, be quiet. It’s eight in the morning, you brat.”  A shiver seized the hair on the back of my neck as I adjusted the thermostat. There was no point in asking if she wanted a blanket—she didn’t. My eyes brushed her bones, knowing that drum-tight, moon-colored skin was the normality of her condition. Her blue lips fused into a firm line as she adjusted her oxygen cannula, then her floppy sunhat, which she insisted on wearing year-round.

“What’d you bring me?” she asked.

"I'll tell you what I should've brought you—nothing.” Rose laughed in reaction, mucus bubbling in the back of her throat. “Hey, can you even see with that thing on?”

            "Obviously. Not that there’s much to see here, anyway," she said. And she was right. With rounded furniture in varying shades of gray and a third story view of sidewalk cracks and bus station smokers, blindness seemed like a tumor worth trading for. Out came the cookies and the deck of cards.

            "Seriously? We played Uno yesterday," Rose said, not at all trying to conceal her disappointment. I constantly eyed the growing dullness of her corn stalk hair and wondered whether she was actually swallowing the pills I placed next to her milk. But when it came to what she was feeling, there was no guesswork involved. Methodically, I took off my coat, tossed it onto the empty recliner, and sat at the foot of the bed.

            "Well, Uno is a classic. And the classics are classics for a reason,” I replied, shuffling.

            “That’s what my dad says about his music.” I paused for a moment to watch her. Her freckles hid in response to January snow, clashing with the perennial power of her eyes. Her port dressing was coming loose, barely visible above the neckline of her t-shirt.

            “When was the last time you talked to your dad, Rose?” Now it was her turn to pause.

            “Last night.”

            “And what did he say?”

            “Nothing. Just the usual stuff.” She was growing more and more annoyed, her gaze refusing to set from my fingers and the cards.

            “Is he coming to see you, soon?”

            “Deb, does it matter?” Her eyebrows blew together and furrowed. “He’s too busy with work. Besides, you’re way more fun. And you’re a nurse. If I was dying, you would know how to save me.” She pushed her bangs behind her ears and I could see tiny dewdrops shimmering on her forehead.

            “Why would you say something like that?” I asked.

            “Because it’s true.” A cough rattled from within her ribs, proof that disease had rooted itself in a place neither of us could reach.

            “Here—we’ll start with more cards this time. It’s harder, but it’s also more fun.” Rose’s nostrils flared as I passed her seven Uno cards and straightened the deck between us. She froze, staring at her purpling fingernails.

            “You won’t stop coming, will you, Deb?”
            “Well, considering how often you tease me, I should.” I dug a finger into her side and a single, scratchy laugh was uprooted from behind her teeth. “But, no. I’ll always come. Life would be far too boring without you.”

            “Ugh. Stop mushing out on me and play.” She picked up a chocolate chip cookie, bit into it, and grimaced. “You know,” she said, chewing, “these taste like shit.”

***

            I often look back on that day and consider the things I could’ve done to give myself more time with her. I ask myself if the meticulous lip-liner was worth it—if my sedan really needed to be washed. They called it “Rose’s Celebration of Life,” but that’s not at all what it was. Or at least that’s not what it turned out to be. I withdrew into my skin, safe and unchanged, and peeked: thirty people in a dim room they could barely afford, listening to a man preach about a god they didn’t believe in, weeping over the loss of a girl they never paid attention to.

We are the Cough Kids

Tonight, breathing is difficult, and this is a hurdle to which I am no stranger.
It's discouraging, truly, to feel that my more concentrated treatment efforts are met only with declining health. Having recently been focusing on medicinal compliance, hints of improvement have been on my mind. But now, even with my hard work, my chest feels tight.
The air in my throat cracks and bubbles.
My quickened breath is audible in any room.
I am tired.
Even as I finish that last sentence, my fingertips abandon the keyboard and travel to my lips. I am forced to physically catch my own disease, my body rejecting the mucus that invisibly drowns it.
I'll let you in on a little secret, though.
This doesn't completely suck.
No, no. Feeling crappy isn't always bad, especially for professional patients. You see, despite the fact that no one should be defined by singular objects, ailments, or significant others, to a certain extent, we are. We all are. We all have a thing.

-----------

You know that coworker who burps to herself every few minutes, problematically imagining that no one can smell it? How about that guy in lecture who lazily scrolls ESPN every Tuesday and Thursday? Well, they too are whole. Just like us, they have families and fears and favorites. Though we know them by stoic, unchanging names, they too are utterly whole.
To us, they are to "Belch Girl" and "Rugby Boy." To others, I am "Cough Kid."
For those of us blessed with seventh chromosomal mutations, this alias - while occasionally suffocating - is familiar. Pleasantly familiar. We've been the Cough Kids of the world since we first cleared our collective throat (most likely at 3-seconds-old). It's not the most glamorous nickname, but it fits. Cystic fibrosis is our ancient, fading, spit-soaked baby blanket.
So yeah, we hate getting sick. We chastise our decaying bodies for what they cannot do, but beneath salty skin and clubbing fingers, we rejoice: we are still alive. Our bones, while prone to premature osteoporosis, are still made of stars. Though we may not often admit it (because, hello, it sounds crazy), sickness remains an old friend. By nature, souls crave companions.
I am a CFer.
We are CFers.
And yes, we also dance, drum, write, snack, adventure, sing, and pursue the perfect winged eyeliner. These are pieces of our humanity, and calling attention to our disease does not discount our pieces. Accepting cystic fibrosis does not discount our pieces. Wearing proverbial stickers that read "Hello, my name is Cough Kid" does not discount our wholeness, and anyone who says otherwise is threatened by the raw confidence we have harvested since diagnosis.
I am sick.
We are sick.
And these aching, brittle, star-filled ribs define us in an incredible way.

Love,
Cough Kid #70,001

Solemn Gratitude

Right now, I find myself struggling with the concept that life offers no paved path. While those who know what they love receive little guidance on which forks to take, those who are anchored to nothing have an even harder time of knowing where to go. As I've said and thought so many times in the past few weeks, the future is unwritten. And while I have superficially accepted this fact, I am admittedly lost.

Somehow, living in a wide open world is sometimes more terrifying than living in one with no choices. It's very tempting to feel alone--the people who previously surrounded me like winter children on a recess playground have now dispersed in every imaginable direction. At the blast of adulthood's gun, we are sprinting.

My life one year ago was unimaginably different than the life I am living now, and that is a wonderful thing. (It means I am growing.) When I take time out of my day to consider this disparity, I feel lucky: genuine friendships and newfound independence have flooded my life. However, I've realized how horribly easy it is to remain unsatisfied. To think I have no friends and I can't do this alone and I'm just going through the motions.

Perhaps this thought process is one of society's ultimate plagues. Perhaps if I, or you, or anyone could consistently remain in a state of thanks, each day would uphold its supreme value in our collective mind. Perhaps if we revisited being winter children on a recess playground, spring on a college campus would reemerge as wildly sweet.

A Change of Attitude

"Life stops for no one."

I feel like I read that quote at least once a month on Tumblr or Twitter or blahblah or hooha and it never fails to stop me in my tracks. Simply, it's just so true. I think of everything I've experienced in my nineteen years on this planet that has made me undeniably wish for a second--just a second to breathe--and I realize how strong I must be to have made it here. We are all so strong for making it here.

Wouldn't it be nice? Wouldn't it be nice if we had control like that? If we could say "World, I can't miss a week of college classes to go to the hospital right now--please pause college. Thanks!" If we could say "World, I need to grieve" or "World, I need to think" or "World, I need to rest." But, unfortunately, life stops for no one.

Here's to digging our heels in and living in real time.