Monday, January 27, 2020

Realizing Hope: Waiting for a Miracle (Day 24)

We sit within a silent hope 
Every breath begging to know another day

We are waiting for a miracle

We hope within within these weakened walls 
A tenacious spirit giving light to its darkness

We are waiting for a miracle

We fight to withstand the unforgiving storm of CF
A courage set strong within our sails

We are waiting for a miracle

We survive on blind bravery
Clinging to an unseeable hope visible only within one’s truth

We are waiting for a miracle

We wake with an undeniable determination
A strength reflected in a life we dare dream to know

We are waiting for a miracle

We give voice to the beauty alive within every breath
Reflected in ourselves the truest of wonder for which needs no seeking

We are waiting for a miracle We are a living miracle



Sunday, January 26, 2020

Realizing Hope: The Lives of Those I Love (Day 23)


Little do we ever realize just how much we want to live until our life seems to no longer be ours to do so. At times, a disease like cystic fibrosis has the ability to make an individual feel like the life they love so much is no longer their own. It progressively permeates every moment of our lives, and its realities find themselves silently reflected within the most beautiful and bitter moments. Some of those moments I carry with me every day, and they’ve defined the ever-changing narrative of my life.

The truth is, in every beautiful and meaningful moment, the thought of CF can’t help but permeate my consciousness. There’s a heaviness within every moment that causes me to want to clutch my chest in an overwhelming gratitude and silently plead to know a future filled with such beauty and the people in which I share those very moments. My fear of CF has never been about the mercilessness of the disease and its unforgiving realities, but has always been about missing out on the lives of those I love. I become breathless as every muscle within my chest becomes tight with emotion. In those moments I plead with this body for more time while drawing in every ounce of beauty I can fit within every breath this body will give me. 

Prayerful Tears of Hope
This weekend I sat across from one of my best friends as we stayed up late talking and laughing for hours as we’ve done many times. I couldn’t help but think of how grateful I was to have the gift to be a part of her life and see the person she has become. Over the years I've learned that one of the most beautiful gifts is watching those you love become the people they are meant to be. And, most of all, it’s such a gift to be a part of that journey with them. As I sat across from her last night, I couldn’t help but think about how just a few years ago we had sat together in a similar way holding back tears in a hospital room - filled with a heartache of an unknown and unfair future. She had left her thirteen-month-old daughter, whom is also my goddaughter, at home as she came to be with me for the day. A day shrouded in an impenetrable reality of CF and time we suddenly felt was truly finite. A hope, similar to the one that lives within my heart still today, found itself in the form of prayerful tears upon my cheeks. A hope that I might see my goddaughters grow up and change the world. A hope that I might be given the gift of watching my friends become the people they are meant to be and grow through every season of life, including becoming parents. It’s one of the most beautiful gifts  - to watch those you love live a beautiful life. But, it’s even more beautiful when you get to be a part of it. 

The Beautiful Season of Today
Ruby, my goddaughter, is six years old now. I feared I’d never see her within this beautiful season of her life, nor did I ever expect the opportunity to love her little sister, Paige, just as much. As I sat across from my dear Kendra this weekend and all those years ago, I couldn’t help but feel immersed in a breathtaking gratitude to be a part of her life and again be given such a beautiful moment.

As I stood in the kitchen this weekend surrounded by the girls and my dear Kendra, popping my morning dose of Trikafta out of its packet into my hand,  I couldn’t help but be filled with a hope that I might know this same beautiful moment through every season of the lives of those I love. In that moment I was filled with a humbled awe born only of a realized hope. A realized hope that for the very first time has allowed me to feel that the life in which I’ve always wished to live is indeed my very own. Cystic fibrosis will forever be reflected in the most beautiful and bitter of moments in my life, but I can only hope those moments inspire a long lifetime of gratitude reflected in the lives of those I love. Love to you all.



Feeling its Effects
- Day 22 - 
I spent the weekend laughing more than I have done collectively in years. There was a freedom that lived each laugh that I only can remember feeling more than a decade ago. It permeates every cell of my being and makes me feel more alive than I have in a long time.  

Saturday, January 25, 2020

Realizing Hope: All That Matters Now (Day 22)

Continued from previous post....

This is hope realized in its own unique way. It may not be the way I imagined, but it's beautiful in its own realized way. This is three weeks of hope born of a life-giving opportunity called, "Trikafta."

This body, breath, and voice feel foreign to me. My lungs feel hollow and my support strangely exhausted as if it doesn't have the strength to sustain the voice these lungs once carried. I'm reminded during this journey that we are never done growing into ourselves. And it's in those moments of trying growth we find the beauty we seek living within every breath. I will hold hope-filled gratitude within these lungs while learning again how to set that beautiful breath to the song that lives upon my heart. All that matters now is where I go from here. Love to you all.               



"All That Matters Now" from Finding Neverland

Friday, January 24, 2020

Realizing Hope: A Breath of Change (Day 21)


Continued from previous post...


There’s a moment of unmatched euphoria when the presence of the breath from which I dwell effortlessly inspires a song that pours from my entire being  - carried by a truth that suspends reality for a brief moment and renews the hope that echoes through the darkest places of my soul. In those moments, time stands still and all that is present is an unbreakable connection between my breath, body, and voice. In that moment, I am truly alive. In that perfect moment there is no cystic fibrosis. There is no pain. In that moment, the constant ticking of time is silenced and replaced by the life and breath of music reverberating within my soul to its own life-giving beat. In that moment, I am reminded that I am strong enough for whatever this life with CF may bring. In that moment, I am reminded why I fight for every beautiful breath. 


But, I know that very moment and the music in which is married to my only promised breath, is destined to find its end. And all that matters is where I go from there - hoping someday that I may again be given this sweet moment of reprise. 


Just Enough
For three weeks now I have started my day with two tiny orange pills that are accompanied by a soul gripping gratitude I never dreamed possible along with a spoonful of peanut butter. I’ve closed each of those days with a single tiny blue pill composed of priceless possibility rooted in my heart’s deepest hope of feeling forbidden air pour into these lungs. A hope born of a future of growing old alongside the ones I love and simply knowing just enough breath to simply sing two full measures again. I’ve been hesitant to talk about my lungs these past few weeks as it’s something I have always had a hard time sharing - a topic rooted in a reality I find at times hard to face. I simply live within the confines of what these lungs will give me today and do all that I can with the breath I have been given. But, if I could just for a moment know the feeling of having just enough...

These Lungs: 
Being, Breathing, Singing
So, after three weeks how do these lungs feel? By no means do I feel like I’ve suddenly regained years of lost lung function or that CF no longer grips these lungs, but I had tempered my expectations of such things from the start of this journey due to the nature of my own disease progression. It doesn’t mean it’s not something I’d hoped would miraculous become a truth I’d experience, but I feel my Trikafta journey will be one of longevity and stability in my life with CF - realized in different miraculous ways other than within the amount of air that fills these lungs. And, maybe these lungs just need more time to settle in with Trikafta before they embrace change?

But, my lungs do feel different. When I take in a breath, I feel the air hit the roof of my mouth and pour down the back of my throat differently than just a few weeks ago. The air tastes different and drenches my soft palate in a cool fluid breath. It doesn’t feel as if I’m trying to draw a breath into my body through a thickly woven towel. My breath feels light upon my lips, but strangely heavy within my lungs - making them feel a bit hollow. Most of all, singing it hard. I don't recognize this breath or this voice. For the last three weeks it’s as if I’m learning how to sing all over again. My air feels heavy and like my diaphragm can’t fully support its weight the way I’ve always known it to. After singing for just 20 minutes my body feels shaky and my support exhausted. I feel lightheaded, like too much oxygen is escaping my body and it can't be replenished quick enough. I feel like I’m pouring too much air through a single note in which my voice can’t find solid footing. I’ve spent years conditioning my respiratory system to work with these lungs and their barriers, where now I don’t recognize these lungs in which I enact the same muscle memory and engrained technique used just weeks ago. A muscle memory that once used every last ounce of capacity to not even make two full measures. 

But, for the first time in more than six months I’ve made it to the end of two measures. It’s hard, and these lungs aren’t giving it graciously, but it’s there. And today, within those two measures lives the most glorious song composed of possibility born solely from a hope called Trikafta. A song that makes me feel truly alive. It may be different tomorrow, but today I will continue to embrace these lungs for all they are. I am grateful for every beautiful breath that I am able to set to a song - rooted in a resounding truth that it will never matter the amount of air that fill one's lungs, but it's what you do with that air that truly matters. Love to you all. 

Thursday, January 23, 2020

Realizing Hope: The Rise and Fall of Just Enough (Day 20)


Our breath is like an endless tide -  rising and falling within ourselves and set upon the unassuming rhythm that gives us life. We stare out into the horizon and watch the waves swell. Very rarely do our eyes catch a wave and follow it fully to shore - watching each powerful current briefly embrace the sand only to be pulled back into an undertow to be reborn. The same thing happens within ourselves. Very rarely do we wake each morning, pulling air into our lungs only to follow its rise and fall as we give it back to the world to be re-inspired. 

Living within Each Breath
I wake each morning and close each night thinking about the same thing. I lie on my back and pull as much air into my lungs as possible as I feel my ribcage expand and my stomach rise. I feel the air pour into these rigid lungs - grateful for giving me another day while silently wishing I knew what it was like to breathe deeper. I set those wishes upon the whisper of life’s song as I purse my lips and feel every muscle in my torso work to set it free, only to pull those same wishes back into these lungs. For it’s this very breath that not only keeps me alive, but is in-part what helps me feel alive.

I live my life as a singer. I beg for every breath so that I might feel it find itself upon a song - wishing only that it could set sail upon unanchored wings. Wishing for some sort of miracle in which I would give me the freedom to choose when to be done singing, instead of these lungs forbidding to support the phrase I wish to finish. But, I also don’t know anything different than the support these lungs have given me all these years. To their credit, they have been reliable to me in that way. In these last few months, however, they’ve been giving me less and less support when I sing. Those two phrases I’ve been able to comfortably own for years within the limited air in my lungs, no longer seemed steady and trusting. No matter my technique, breath management, or focus these lungs would abruptly and unapologetically cut me off without giving a care to the words left empty and silent upon my lips. The very thing that made me feel truly alive was the very thing dependent on that in which fills me with life itself : breath. And, the reality is that I have cystic fibrosis - a disease that is vying to steal every last one. Deep down, however, I feel that is why I am in fact a singer - that my life simply depends on it. 



Artwork by Molly Noem Fulton
Breathing In Hope
When the possibility of Trikafta became a potential reality for me a few months ago my heart was submerged into an ocean wrought with hope filled emotion. At its very center, a hope rooted in a future reflected in the lives of those I love. One in which I had only ever dreamed about. But, deeper within me was not only a hope to be filled with life itself, but for that life-giving breath to fuel that in which makes me feel truly alive. That I may feel a breath pour into my body and know no limitations. I did my best to guard my hope and temper the possibility of such a thing. But, I couldn’t help but wonder as I lay there night after night and morning upon morning what it might feel like if hope were realized in the form of a deeper breath. If that hope were realized in the gift of just being able to sing two measures again without feeling betrayed by my own body. That I wouldn’t feel most days as if I’m losing that in which makes me feel most alive against my own will and determination. What if I felt free to sing the song I imagined?

During its clinical trials, Trikafta showed many significant and dramatic points of disease improvement in individuals with at least one copy of the Fdel508 mutation. One of these incredible improvements was on average a 14% increase in lung function, not to mention a significant increase in associated quality of life. Of course, this means there were individuals with a lung function increase greater than 14%, some with a slight increase, and those who saw no significant changes in lung function. Every individual with CF is so very different and it manifests itself uniquely to each of us. Some people within hours and mere days of taking their first dose of Trikafta could suddenly take a breath they’d only known years to decades before. And for others their experience has been different. While 14% would be simply amazing, and I can’t even begin to fathom what that would feel like, I also know the reality of these damaged bacteria laden lungs that reside within the walls of my chest and what the truth of their stubborn state may be. But, even within that reality it’s hard for my heart not to hope for just enough breath to give me back those two full measures. I promise I will use them for the most glorious song that lives upon my heart. 


To be continue…

Wednesday, January 22, 2020

Realizing Hope: Feeling Alive (Day 19)


Our lives may be reflected in different truths, but the very basis of our existence rises and falls upon the same breath that fills our lungs. That breath is what gives us life. But that breath, however, isn’t what makes us feel alive. I mean, truly alive. That very breath simply fuels a fire within each of us - inspiring our hearts and minds to seek that in which fills us with purpose and passion. It doesn’t matter the amount of air that fills our lungs, it’s what we do with it that makes us feel alive. 


Today, I’m asking you - What makes you feel alive?








Feeling its Effects
- Day 18 - 

There’s no denying this drug is powerful. It’s unlike anything our CF community, both patient and Care Team, have ever experienced. Things are changing day by day in the smallest and grandest of ways, while also transforming each of us uniquely in ways that have yet to be assumed. I’m sure there are many things happening even within myself that  I have not noticed because they’re happening at such a cellular level or so gracefully that they go about unnoticed. 

This week has been tough in comparison to the last two. I’ve had some exhaustion set in along with a sinus pressure I’ve known before. While my sinuses still feel different and far more open than just a few short weeks ago, I know the pain of angry sinuses well. The fatigue coupled with this relentless insomnia doesn’t prove to be the best combination as my body does its best to sort itself out and find its changing “normal”. I have to remind myself that my body is doing its very best, and I just need to give it some grace.

I’m grateful for my body and its endurance in trying to establish this new normal. For the most part, my digestive system has been overall happier than it has been in a long time, and for that I’m immensely grateful. I’m being cautious of what I’m eating and how much, as not doing so seems to be making my stomach most unhappy. It’s a learning curve I *believe* I am getting somewhat of a grasp on. It may be different tomorrow, but then we will just change our sails with the wind. I'm just grateful for the opportunity to sail on this journey. 

Tuesday, January 21, 2020

Realizing Hope: A Long Kiss Goodbye - Written by Mark Bonnema (Day 18)

She lay peacefully, her hair draping over the edge of the pillow. Her chest rises gently, easily against the comforter. A wry smile graces the corner of her mouth as her dreams take her to magical places. I approach silently in the darkness so as to not wake her. My hand gently embraces her shoulder as my lips caress her forehead…. Hey now! What kind of blog do you think this is!?!

But, alas, every word is true. Every morning when I rise, Ashley’s night is only a few hours young. She has a few precious hours of sleep to obtain yet, and I would not dream of waking her, as she usually has a big day ahead. I wake up, get ready for my day at the hospital or the farm quietly and in the dark. The dogs rouse, of course, and I let them out and feed them. Then, they are content to go right back to bed and keep Ashley warm until she is ready to greet the day. 

I try to quietly say goodbye to Ashley every morning with a loving whisper and a gentle kiss on the forehead. Sweet you say… but, perhaps, also a little salty? 

My good morning and good bye kiss is filled with love and affection, but through the years, it has also developed into a bit of a quick health assessment. You have a lot of nerve endings in your lips, and the skin on your lips is quite thin (don’t ask how I know this… its medical…). This makes your lips quite sensitive to things like fevers, night sweats, and excess salt. I sometimes can tell if Ashley is not feeling well, or having complications from her CF just from a simple, quick, gentle kiss in the morning.


But now Trikafta. Will this new medication stabilize Ashley’s health to the point that I can simply give a loving good bye in the morning? A kiss filled only with love and affection, free from the twinge of fear that lies deep in my being, the fear that each new day could bring a new fever/infection. A gentle whisper wishing her a good day ahead, free from the worry that a bout with hemoptysis could bring a drastic change to her day and to her health. A brush of my lips intended to merely connect two persons and instill life, love, energy, and affection rather than obtain quick health assessment data. 

I am hopeful. Trikafta is giving many people the space to be hopeful- persons living with CF, like Ashley, but also their families, spouses, friends, and communities. I am hopeful that I can open my heart and my mind to possibly in the place of fear and worry, and to the promise of a future where every morning begins with a simple kiss.