34 : Living in the Wait

 

So much of life and beautiful time we waste by being distracted by the notion of waiting. We fail to realize that living and waiting are always present within each other. That waiting is not a suspension of time, but a fueling of hope. It’s often within the season of waiting we find the truest meaning of living. It’s within the waiting we find an unmatchable peace that can enliven our senses and inspire our spirit. It’s in the waiting that we have the opportunity to make every beautiful breath extraordinary. And if we are brave enough to look, it’s within that quieted season of waiting we find ourselves.

Thirty-Four

I realize that I’ve spent a great number of breaths over the last thirty-four years gripped in a perpetual season of waiting. Holding my breath and silently pleading with a life in which I was waiting to begin. Hoping that the circumstances of my truth would free me to not only live by hope but live within its realization. Waiting for answers to which I didn’t even know the questions, only a discontented restlessness within myself. For a long time, a restlessness that I thought could be drowned out by the chaos of the world around me as I felt time slipping through my fingers - desperate for hope to rescue me. I used the world’s chaos to distract me from living within the difficulty and unknowns of the present - projecting a contentment into an unguaranteed future. But as I look back upon the last thirty-four years of this gifted life, it’s in those lost moments of waiting that I can clearly see I found not only what it meant to truly live, but in fact, found myself. 

This birthday casts itself as a beacon of hope amidst such a seemingly shadowed season of waiting. For as long as I can remember I’d wish for more. More beautiful breaths, more years with those I love, and simply more time. While that wish will always be on my heart, it’s during this season of waiting that such a wish is overshadowed by a weight of gratitude. A gratitude born out of this quieted season of isolation. Have tears frequently stained my face over the last ten months? Does my heart deeply ache from missing my people? Yes, but this gratitude that consumes my heart today is born out of a stillness that can only be found from being alone amidst a season of waiting. It is a gratitude that silences the chaos and unearths what matters most - allowing that which truly fills me with life and gives meaning to every breath to illuminate a hope that perpetually fuels my life. A hope that frees me to live and love more deeply no matter the circumstance. 

Living within Hope

This season of waiting you feel you’re in right now? The one that you can’t wait to get out of? Put your hand on your chest. Close your eyes. Take a breath. You are filled with beautiful life. You're not being held back from living the life you want, but instead you're being encouraged to open your eyes to your life that is filled with authentic meaning no matter the circumstance. This season can be a gift in which you learn to live within the wait. Out of such a season we can learn that every breath not only can be but is extraordinary in its own way - a way in which challenges us to be truly present. Lastly, that restlessness you feel? It’s the hope that fuels your life and gives gratitude to every breath you’re given. 

It is my greatest hope that when I take my last breath that I will be able to look back on the entirety of my life and know that I was never lost within waiting. That instead my life was found lived within a hope veiled in an entire season of waiting which gave me the strength to dare to dream, to love, and embrace this life one beautifully gifted breath at a time. Love to you all.

"I Cried" - from the tears of a 33-year-old with an underlying respiratory disease amidst a pandemic

I cried tonight. I muffled my sobs within the palms of my hands as streams from the shower above raced my tears to the drain. A rolling steam engulfed me as I felt myself within the grips of disbelief - grasping for a different truth.

I cried for the life I feel we have just gotten the chance to finally live and dream about and could lose at such an unfair cost. I cried at the thought of one of us getting sick and being separated during these tough moments.  I cried at hearing the panic in your voice and reading it within your texts as we realized the scenario we feared was becoming more and more real by the hour. I cried recalling the desperate look within the oceans of your eyes - unsettled within the unknowns and the honest inability to protect me. I cried because I am unable to protect you. 

I cried for the nonrefundable trip we have to cancel after waiting two years to take. I cried at the thought of not tasting the salty air - watching our favorite sunsets cast an unmatchable glow across the gulf skies as my hand would silently finds itself laced within yours. 

I cried for the people who have lost someone to this devastation. I cried for those who are oceans away and those within my city who see the same horizon. I cried for those on the frontlines of saving someone’s loved one - tired, torn, and exposed. I cried for those facing moral dilemmas of care and compassion. I cried for the uncertainty that faces us all. I cried for a crisis that is dividing and devastating our world. I cried for the days ahead in which I know will be even more difficult. I cried for my compromised community that most likely will face loss because of such a virulent virus. 

I cried for those who live in fear - paralyzed by realities they cannot fully control. I cried for those who mock the encompassing unknowns of this virus and the people trying to protect others and themselves. I cried for us all. 

I cried tonight. I muffled my sobs within the palms of my hands as streams from the shower above raced my tears to the drain. A rolling steam engulfed me as I felt myself within the grips of disbelief - grasping for a different truth.

*Please, dear friends. These are not moments in which we need to be cavalier or prove our self strength. Sometimes the strongest thing we can do is take a step back, take a deep breath, and do what is best for one another: social distancing, isolation if necessary and precautionary, good and rigorous hygiene practices, and patience. We can come out of this together, but we need every one of us to act in the best interest of our most vulnerable. 

Realizing Hope : A Silence Between Two Hearts (Final Entry)

When the future seems anything but promised you simply stop dreaming and start merely living - immersed and grateful in the present moment you’ve been given for the heartache of unknown tomorrows becomes too much to bare. The dreams that once permeated your life have dissolved into a silent yet palpable hope - becoming the only stability in which your life owns. And, truthfully, those dreams don’t seem to matter anymore. We mourn them, and they silently live embedded within our hearts forever as we set our eyes upon the present and simply find the most happiness amidst the act itself of simply becoming. We dare not spend our life dreaming to become, but instead living to become who are meant to be with whatever time we are given. 

Dissolving Dreams

In the last several years it has been increasingly painful to think about the future. Dreams that once fueled my future were met only with CF’s sharp realities - pushing me to become immersed within the moment and embrace the mere act of living. The single breath that bound me to this moment had become my only given grace and was the only part of this life with CF I could trust. I stopped dreaming as I could see my own painful unrealized hopes reflected in the eyes of those I loved. I felt I had lost my place a part of their future because of a disease that would forever be a part of my present. I stopped dreaming to save myself. 

Namely, I stopped dreaming about a future with Mark and a story I feared I’d never be a part of. I stopped dreaming about growing old together, about places we’d visit, the adventures that would find us together, and, most of all, the simple moments in which the future would become our best of days - days reflected in a life and love woven together through who we had not only become as individuals but had become together through everything. I stopped dreaming of a future in which CF wouldn’t write the story for us and the dreams that entangled our hearts would be the life giving hope behind every breath. I simply stopped dreaming because it was too painful when I looked at Mark to mourn the life I so wanted us to have. I stopped dreaming about the future not only to save myself but to protect Mark from the perceived possibility of living a disappointed life. For almost a decade, with every breath I’d give back to the world a piece of those dreams would follow, leaving a growing silence between our hearts. But, as much as I’d withdraw from life and press myself into the safety of the present moment, Mark would turn and meet me there - never allowing me to live alone in such a place of hopelessness no matter our reality. For dreams no longer mattered, only this very moment in which we were guaranteed.

A New Chapter

And then my life was given an immeasurable gift. A gift with no promises but instead a possibility born of realized hope. A gift called, Trikafta, - a gift in the form of a new chapter I never dreamed of writing. In the last month I’ve done something I haven’t dared do for a great number of years. Something that I didn’t realize just how much had suppressed my spirit and had withdrawn me from my own life. I began to dream again. And with every dwindling cough and day in which my body fights to find this lasting trust within itself, a dream finds itself in the form of a new hope born upon every breath. It sends a gripping pain through my chest and turns my stomach upon itself into knots. For I know how hard it was to mourn the dreams I learned to let go, and I don’t know if I am strong enough to mourn them twice. Because in truth, these dreams will most likely still be stolen and held captive by the realities of CF, Trikafta or not. 

Filling the Pages

But, if I’ve learned anything this past month it’s when the dust settles upon an unpromised future and hope is all that remains, dreams begin to fill the silence left upon the pages that I’ve been too scared to write. This time, however, dreaming is different. My heart is different. I am different. Am I terrified? Of course. But every dream ever born of my heart will forever be reflected in the beauty of learning to live within this very moment and embracing not only who I shall become regardless of that dream, but living within the hope itself that gives it life. 

Mark’s steadfast presence through every difficult and beautiful breath is a reminder that our life together will never be about the dream itself, but will always be about how we continue to live and grow within each moment we were given. That is what will define our story. For it is those collective moments of simply becoming that will fill our pages and make the most beautiful masterpiece. A masterpiece forever written in a hope born from a courage to simply become. Love to you all. 

Realizing Hope: Moving Mountains (Day 28)

Continued from previous post….

 Living with cystic fibrosis feels as if I’m desperately clinging to the side of a mountain’s ragged and crumbling edge, my face pressed against its side as I hold my breath with arms outstretched wide - my hands desperate to find something that will hold my tired grip. I can feel my toes tightly curled within my shoes trying to anchor myself to the thin rocky ledge which isn't wide enough to fit both of my feet. I feel the wind daring to pulling against my body as I try and steady myself. If I lose my focus to the rocky terrain that surrounds me, and I catch a glimpse of the world below. I feel myself begin to sway unsteadily as my body becomes disoriented - losing any shred of balance as I feel my own body being pulled from beneath my tight grip. 

Then, this drug called, “Trikafta” came into my realm of possibility. And I dared to cautiously dream - to not only release my grip from the side of this crumbling mountain called CF, but to reach out, grab hold of a new ledge, and begin to climb. That I might look out at the beautiful vista before me and deeply breathe in a beauty I never thought possible. But, I also knew that these lungs are filled with decades of irreversible scarring and bronchiectasis that stubbornly have become a part of who I am. This craggy landscape that lives within the walls of this chest simply cannot be transformed within day. Or, possibly ever. But, I know the progression of CF. I’ve watched it take its merciless toll on those I love and a community for which I will be forever connected. I knew, with no guarantees, Trikafta was my only chance at slowing the unforgiving progression of CF within myself. 

Changing the Unchangeable

So, I took my first dose of Trikafta 28 days ago and cautiously hoped - guarding my heart and my mind against exaggerated expectation while staying rooted in grateful, yet pragmatic possibility. Woven within the freefall of life-defining numbers etched within the walls of my being. Is the unchangeable changeable? 

I had a lung function test yesterday in which I blew an FEV1 of 56%. This is an 8% increase since my PFT in November and since beginning Trikafta. I haven’t seen over 50% in a year, and a value even close to 56% in over five years. I have been unable to keep it anywhere near there as it has always found its way back into the 40s for which I’ve always fought to maintain. I’m speechless and my heart is overwhelmed with gratitude at this opportunity to see such a number again. It’s as if in that very moment I felt my body relax a bit, and I could merely enjoy the view from where I am at. That I could literally and figuratively breathe. There are still no guarantees in this life with CF, but for this moment, this is hope realized in a simple set of numbers - numbers with which I hope these lungs will continue to wholly embrace.

While I may feel like CF itself is a mountain, I actually haven’t seen the mountains in many years because of these lungs. I can only dream that with such realized hope that I may see such a beautiful perspective born of possibility again while fighting for more tomorrows for everyone with CF so they also may enjoy such a breathtakingly beautiful view. Love to you all.  

Realizing Hope: Defined by Numbers (Day 27)

From the moment we breathe our first breath numbers assign themselves to our existence. Our lives forever in tandem with a changing yet unmovable system that defines our days and silently seems to set a value upon our lives. Some remain a marker for our lives - bound by a memorable and meaningful moment reflected in a set of digits for all our days, while others are like a steady ripple in which cannot be contained to a single moment.  

A Disease in Numbers

My life with CF has been bound and defined by numbers. The very basis of cystic fibrosis realized within an abnormal sweat chloride level which diagnosed me at just one month old. I reflect back upon the years and realize how much of my life has been and continues to be influenced by numbers: test results, lung function scores, baselines, trending outcomes, life expectancies, dosages, weight, and time that seemingly feels as if is merely slipping through my fingers. Every number a reflection of a disease that I feel I cannot control. A disease that makes me feel as if I’m clinging to the side of a mountain that just keeps getting higher and the terrain more steeply unsteady - unforgiving beneath my feet. 

While there are many numbers associated with the progression of CF and its stability, lung function is one that is closely monitored throughout one’s life with CF. It’s one that can make you feel as if the very life that fills your lungs is being unfairly stolen by your own body. That simple number can silently say so very much - whispering words of depravity formed from a lie that this life you live isn’t your own, but instead belongs to an unchangeable circumstance called cystic fibrosis. 

The Landscape of These Lungs

Those numbers and scores mean something different to each of us, and in classic CF form, they feel different to each of us. I don’t love to talk about numbers as they don’t always clearly reflect how we feel, nor do they translate equally from person to person. There are so many variables when it comes to a person and how CF impacts them, and the mountains we must each climb which reveal their own unique obstacles. I simply do my best to be grateful for every breath I am given - seeking always to enjoy the beauty from within its gifted view.

I’ve been incredibly lucky that my life with CF has been, in part, pretty stable these last few years. Meaning, that over the course of the last 5 years, outside of some tough exacerbations and hemoptysis issues, my baseline for lung function has hovered between 45-50%. My lung function is something I’m so intrinsically in tune with as my very profession and passion are impacted by the unforgiving landscape of these lungs. I’ve desperately done all I can to continue to hold onto every breath I am given, while slowly feeling any hold I have upon my own body begin to slip within the last year. My tenacious grip losing its strength as I cling to unpromised stability. This body working harder - more unforgiving to the demands I continue to thrust upon it. Daily, CF reminding me of the life it thinks it owns. A life I wish to own every part of and live bravely beyond its perceived limits. 

To be continued….

Realizing Hope: Finding "Normal" (Day 26)

“....to just be normal”.

It’s a seemingly empty wish that has found itself upon my lips on numerous occasions - leaving echoes of its pleas reverberating through my mind. That set of words usually is married with tears that come in the form of an emotional release that have been building and can no longer be contained. These moments are most often a culmination of living at the mercy of the unexpected and unknown realities of CF. 

A New "Normal"

I woke up this morning allowing myself to realize that for the last week I haven’t spent my mornings dry heaving over the sink due to nonstop coughing - my lungs burning from the abrasive impact of tortuous muscles and forced air trying to move the suffocating thickness of mucus trapped within my lungs. Mornings that  would leave my body ravaged and exhausted before the day had even begun. Just less than week or so ago I would dread getting out of bed in the morning, lying still as the world nudged me to take part in its gift of another day. I’d lie still for a few moments garnering enough strength and willpower to push through the heaviness that found itself within my chest overnight, knowing what awaited me as I sat up and took the first true thoughtful breath of the day. Within those first moments, I knew whether or not the day was mine or if CF would make me work for every breath - setting that simple wish upon my lips of “I wish I were normal”.

After thirty-three years I’m not even sure what normal could possibly feel like. Is it not spending hours in a given morning coughing to the point of cracked ribs and exhaustion? Is it simply waking and not making deals with the day and the disease that forever has foretold its fruition? Was this very morning a glimpse of normal? Is this my own “normal”? Most of all, will I ever be able to fully trust its existence if it is such? Even within the realized constructs of this moment, I continue to hold my breath as this body has taught me never to trust itself, or namely trust the disease of cystic fibrosis that will forever define every cell, Trikafta or not. Most of all, I guard my heart from that trust being broken within myself. Whatever my personal definition of today’s normal may be, my body silently and fearfully pleads within itself that while I may never know true normalcy, that CF may continue to show me mercy and let me simply breathe in the beauty of possibility that was born to such a gifted morning as this.

To Just Be Me

The truth is we will never truly know what normal is because we’ve lived too much, we’ve felt too much, we’ve seen too much of a life defined within a different norm called cystic fibrosis. I think about that simple wish of normalcy and how for it to genuinely become an embodied reality the entirety of my history, my experiences, many of my relationships, and all the strength through heartache that has been realized would fail to be reflected in the landscape of my life. Truly knowing normalcy feels as if it would seemingly erase much of who I am and have become. I feel as if my eyes would suddenly no longer see the depth and the pricelessness present within every moment, and the entirety of my being would simply exist instead of emblazoned with a fiery passion to do all I can to simply live. For better and for worse, CF has conditioned me into the person I strive to be today: tenacious, compassionate, and wholly present in living each moment without regret. That simple wish may be set upon my heart for all of my days, but it is one that at its truth has helped make my life so incredibly beautiful. I wouldn’t trade a single breath if it meant not knowing the love that enlivens this life with meaning and purpose. My life with CF will never know such normalcy, but what matters most is that I instead embrace the wish to have the strength to simply just be “me”. Love to you all. 

Realizing Hope: What Will You Do? - Written by Mark Bonnema (Day 25)

Health is a relative state. For some it's a gift, for others it is a blessing, and for some it's a burdensome trial. Some people are very intentional about maintaining and promoting their health, while others are more passive as time and their health fade. It is certain that health and its failings do not play fair. 

The gift of health, blessing of being healthy, and even the burden of illness do not reach people fairly or according to what they deserve.  Sometimes the way you live your life is not reflected in the health you have, and also, the life you have is not always a reflection of your health. Throughout history there are accounts of people in poor health accomplishing great things, and of people in great health have living a poor life.  What truly matters is what you do with the health you have.

Ashley and many others living with cystic fibrosis have been given a gift, a chance at health in the drug, Trikafta. All are deserving of this incredible new chance at health. But whether Trikafta brings a change in lung, GI, endocrine, and reproductive function it remains that you need to do, and live, and love, and excel and dream, and laugh, and grow, and dare, and be, and make the most of the chances at health and life that Trikafta potentially brings. Trikafta is just a drug (a really cool, awesome, life-changing drug), but what will it allow and inspire you to do? To be? 

Those of us not living with CF, whether or not we have your own illness or ailment, we are not off the hook! Being healthy is privileged and a wonderful state of being, but it is just that (and only that) until we do something with it! We have the same charge – what will you do with the health you have?

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

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.  

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

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. 

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…

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. 

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. 

Realizing Hope: Unique Voices of a Diverse Trikafta Journey (Day 17)

Cystic fibrosis affects over 30,000 individuals in the United States and 70,000 individuals worldwide. With over 1,800 different mutations, CF is a complex multi-system disease in which the body produces a thick sticky mucus causing a diversity of progressive complications throughout the body. Most people know CF namely because of its impact on the respiratory and digestive systems, but it impacts so much more. What makes cystic fibrosis so difficult to treat and define is not only its multi-organ impact, but that that it manifests itself so differently within each of us diagnosed with the disease. Even individuals sharing the same genetic mutations live vastly different lives and may face different complications or prognosis. Each voice of CF so different and so unique unto every individual. 

I admire and respect the remarkable individuals that make up our CF community. I think each of us can agree that we wish such circumstances didn’t exist for us to know one another, but since those are things we cannot change we can be rooted in gratitude that there are amazing individuals with incredible voices to be shared.  Today’s post is filled with those voices, particularly those who are on their own Trikafta journey. These are just a handful of stories and experiences reflecting all sides of this journey - each one set to its own unique voice. 

• Julie Desch, Somer Love, and Anne Governor share their Trikafta experiences. In this I Rok Interview we are talking about something that is super exiting for the CF Community. Trikafta! (video)

• What Does Better Mean on Trikafta? - CFF Blog by Elizabeth Amber

• The “New CF” - Building Habits for Life After Trikafta™ - Katie Malik

• Trikafta, the Pill of Broken Promises - Maddie Po

• Twice-a-day Life on Trikafta - Andy Lipman

• The Truth Behind Agony of Waiting - CF and the City65 Brittani Day

• No Longer Confined to the Sidelines - Lungs n' Roses by Jenny Livingston

• Trikafta Month 1 - Katie Fielding

• Walking Into A New Reality - Attain Health by Kat Quinn Porco

• An Unexpected Gift - Michelle Herpolsheimer

• Rosie Life with Grey - Canadian Perspective

• The Frey Life - A Friend's Perspective on Trikafta (Mary Frey) (vlog)

• My First Week on Trikafta - Cystic-Fibrosis.com blog by Kynsie Riedel

• The Golden Years - Cystic-Fibrosis.com blog by Joel Barlow

• Washington Post Trikafta

While each of our stories may be different, one thing is exactly the same: we fight CF together and for each other so that every single person living with CF has an opportunity at realized hope through life changing opportunities like Trikafta. Everyone’s Trikafta journey is different, just like their life with CF. And for some, Trikafta isn’t a possibility due to their mutations, or for some reason Trikafta is just not a fit for a person’s unique story. It’s important we hear everyone’s story though. From those whose hope is realized within amazing results of a modulator, to those whose hope remains steadfast in future advancements, and  everyone in between - everyone’s voice matters. And it’s for all the voices of CF we relentlessly fight for more beautiful tomorrows.