Realizing Hope: Waiting to Exhale (Day 12)

I close my eyes tightly and brace myself, pulling as much air into my lungs before my gloved fingertips touch the door handle and I am consumed by the icy grip of South Dakota’s wintery breath. My body hesitates a moment longer before adrenaline forces me to find quick footing. The crisp still air stings my pursed lips as I hold onto the air for a moment longer. But, the held air in my lungs begins to burn, and I must give it back to the world in exchange for a breath filled with sharp icy daggers that set themselves fervently upon the air in which I seek to sustain me. But, as I feel the sting of winter move from my lips to my lungs I step out from the shadow of the peak of my house - the stunning January sun hanging against the bluest bright sky and reflecting off of the crisp hardened snow. Its radiating light reminding me that spring will come again. But, most of all, it reminds me to simply enjoy this very moment and its journey, as the coldest winter days in South Dakota most often give us the most beautiful blue skies. Ones that are breathtakingly beautiful in so many ways. 

I feel like since the first dose of Trikafta I’ve been waiting to exhale. You read that correctly. I’ve been waiting to exhale. A drug that seemingly begins to work within hours of the first dose has transformed the lives of some individuals with CF to feel like versions of themselves from decades bygone. I also know, just as CF manifests itself within everyone so differently, this drug, too, works differently within each of us. Every individual’s experience is going to be unique to them, and hope realized in different ways. But one thing is the same: all of us are filled with the same hope. 

To be honest, I’m still holding my breath and waiting to exhale. Truthfully, I’m not sure I’ll ever be able to fully exhale and trust this body enough to let it all go, even though within these short eleven days I’ve been given some priceless and unexpected gifts from being lucky enough to take this drug. They may be simple and seemingly small, but in the grand scope and expanse of this life they’re breathtakingly beautiful in and of themselves. For the simplest gift of laughter can shatter the iciest January air and bring life’s hope-filled season of Spring into every breath. Still, I’m cautious, I’m guarded, and I simply have lived my life until this point focused on the beauty in every present breath. It’s the way I’ve survived against a disease filled with many seasons - ones that have not always been the most beautiful. I know this Trikafta journey is not simply a single season, but is a compilation of seasons that will hopefully accompany me to the end of a breathtakingly beautiful life exhausted in years and the bluest January South Dakota skies. But no matter the season it will always be filled with hope and the deepest gratitude. Love to you all. 

Feeling Its Effects
- Day 11 - 

I'm always astonished when I think about how our bodies adjust to become our truth and how when that suddenly changes our association with our own truth must change as well. Things I've been eating for years suddenly have a different taste. I've eaten oatmeal squares all day, everyday for more than a year to help keep my blood sugar stable throughout the day. This week they taste different. I can taste the brown sugar and honey baked into every little square. 

I can smell my own hair for the first time in a very long time. I know that seems like not a real big thing, but it just proves to me how much I haven't been smelling these past years. Also, while having my blood drawn the other day I could smell my phlebotomist's gum. 

Realizing Hope: Joy, Laughter, & Trikafta - Written by Mark Bonnema (Day 11)

Things have been different around our house. No, it’s not Cooper (our goldendoodle), he still sleeps 23 hours a day and only wakes to be fed. It’s not Kalvin (our cockapoo), he still has the world’s most crooked teeth and demands to sit on your lap at all times. Its not the cold, drafty windows… who knows if we will ever get around to replacing those, after all, it’s fun writing messages and love notes in the frost that gathers on them daily in the winter. It’s not so much me (this is Mark writing, by the way, Ashley’s husband) – I’m still likely to be at work, in the kitchen, or at a local brewery hangout. You may also have noticed I do not use as many adjectives in my writing as Ashley.

The difference around our house is, of course, Ashley. Ashley is different. Ashley has been letting us all follow along with each passing day and each additional dose of Trikafta, chronicling in writing the changes manifesting in her body, thoughts, and emotions. I notice the subtle, wet crackle in her cough signaling the thinning and mobilization of her secretions. I hear her take an extra-long sniff of the aromas coming out of the kitchen as her sense of smell heightens. I also notice how quickly our peanut butter supply is dwindling, as she takes a scoop with each Trikafta dose to promote absorption of the medication.

But what I notice the most, the biggest change around our house since Ashley began taking Trikafta, is the return of Ashley’s laugh. A full bodied, drawn out, whole-hearted laugh that can fill the main level of our house. It’s the laugh I grew to love and craved in high school when we began dating. It’s the laugh she would unleash when she had no cares in the world and was living a life of blissful denial in the face of CF.

I know many of you have heard Ashley laugh. It is an unspeakable and incredible feat of perseverance that, despite all life has put in her way, Ashley has never lost her sense of humor and her ability to laugh. But through the years, CF had slowly eroded some of the vibrancy and fullness of Ashley’s laugh. Perhaps she was afraid it would spur a coughing spell. Perhaps the air just was not there to support a marvelous belly laugh. But since Trikafta, it’s back, and its unbridled! I hear it while she is talking on the phone call to her people (you know who you are). I hear it while she is scrolling through a Facebook feed. I hear it while she teaches lessons to her students.

A laugh can give you a glimpse of the joy radiating from one’s soul. Trikafta has ignited the joy in Ashley’s soul. Ashley is hopeful, filled with life and happiness, and her soul is radiating with laughter. Laughter is contagious, and I feel Ashley’s laughter working on me… coaxing me to dare to dream, to dare to hope for the future – our future, and to fully reconnect with the joy in my soul. Trikafta is not a miracle drug instilling joy into people’s soul, but it has brought hope and joy back into Ashley’s life and into our home. Take time to cherish today and share a joy-filled laugh with someone you love.

Realizing Hope: Looking into Love (Day 10)

There have been more moments than not this past year that I’ve held my breath. For I feared letting it go would mean this very moment is passing me by. That I’m losing this battle to hold on to all that I love. I’d silently sit with the world somehow quieted for a brief moment, with Mark next to me and our dogs (the boys) curled up between us. It was as if we were living in the middle of life’s exhale - that brief moment of stillness and calm before our body begins to vigorously pull air back into our lungs and our body is overtaken by a poetic chaos that gives us life. It’s in that brief moment I think, “these are the best of our days”. It would take everything I had in that moment to hold in the tears that wanted so desperately to overtake each breath. I’d hold my breath in hopes to make it all last a little bit longer. As if I owned the moment - forbidding to let it go.

Years ago, Mark and I learned to rarely talk definitively of the future for its realities knowingly were too painful for both of us to hear spoken aloud. We’ve learned to put on a brave face for one another, learning most of all to cherish the present and live wholly in the only thing we are guaranteed - this very moment. We live this life from still frame to still frame - holding our breath while silently pleading with a disease we know can destroy this life we both know and love so much. In those still moments, I close my eyes as I hold my breath to keep from meeting Mark’s gaze. Because in those moments I feel less than brave and that I will become undone. And, I must be brave. Reflected in his eyes are the dreams that lay dormant within our hearts and that we dare not let escape our lips. Instead, we live in a silent hope that seemingly sustains a gifted love rooted in the present. 

When the news of Trikafta first was released back in October Mark and I didn’t say much about the matter other than in light passing comments. Those comments knowingly were rooted in something deeper but that neither of us wanted to embrace. But, they were heard nonetheless. We were being protective of each other and our hearts. While out to dinner just a few days after the FDA’s approval, a couple whose foreheads and eyes were kissed with the gift of years and their hair dusted white walked into the restaurant past our booth. Mark looked at me and said, “that is going to be us someday.” My breath quickly caught on the years of built up emotion trying to escape upon hearing the sound of such a hope we for years had held silent. My mind stirred with the silent plea that such a sentence may not only be a hope, but become a living truth. That night, As it always does, my hand found Mark’s as we walked towards the door to go home, but not before I looked back towards the couple to paint a forever picture in my mind - something to hold onto along with my hope.

I’ve found myself struggling with that hope these last couple of months as Trikafta has become more and more of a possible reality, as I’ve heard incredible stories that by most would be defined as miracles. I’ve done my best to guard my heart and my mind against running wild. To stay grounded in the reality of this body - a reality that has been rooted for years in a silent resilient hope that has helped me to survive. It has helped us survive.

Having the opportunity to take a drug like Trikafta is such a gift, but one wrought with so many emotions. As I’ve struggled the last few weeks with the weight of this gift, Mark’s voice has been a steady chorus of encouragement laden in something so much deeper than hope. There was something different about his voice and the look in his eyes when there’d be talk about Trikafta. Again, I’d avoid meeting his eyes or letting the hopeful timbre of his voice crack my composed façade. When I got the message on New Year’s eve that I had been approved for Trikafta I showed Mark, and I will never forget the look in his eyes. It was a decades worth of hopes that had been buried for years suddenly finding life in his eyes. Reflected within them I saw not only myself in his present but a part of his future as well.

I realize all those dreams may still be just that - dreams. That CF may not seemingly disappear from my life and transform it into something unrecognizable. But, if I am gifted simple stability in which I can live just as I am right now for longer, a life filled with those moments in which we are merely together and I can silently think to myself, “these are the best of days”-  that will be my miracle. The only one I truly need. Love to you all today and always.



Feeling its Effects

- Day 9 -

My intention with sharing my journey through these posts was not only to write about the amazing changes I'd hopefully witness within myself and come to know as a new truth to my life and the immense gratitude I have for such an opportunity, but also to share the complex emotional journey and the knowingly not-so-good days.

This weekend my body just couldn't find a new rhythm. My stomach and joints were less than happy with me and made for long exhausting days. Being doubled over is tiring, especially when there seemingly is nothing I can do to alleviate the pain. Thankfully, my stomach has calmed down. I can only hope with the continued passing of days that this body finds its new normal - one in which these joints are tolerable. We simply press on taking all the difficult with the good.

For those of you who know me well, you know I rarely sleep. Insomnia seems to be a placeholder in my life that forbids to eradicate itself. Trikafta seems to have made my insomnia worse this last week, no matter how I try and counter it. Insomnia has been such a difficult thing in my life with CF as I know how much this body needs rest, but it simply just won't give into it. This body of mine has always had a mind of its own. Even with less sleep than usual, my mind seemingly is working more efficiently and effectively. I'm feeling less like I'm living in the middle of a daydream during the day and an intense fog. It's hard to imagine what I'd feel like if I had more than just a few hours of sleep. 

But even with those less than enjoyable additions to the weekend, there are a few things I couldn't help but be grateful for that were seemingly different. With every weather shift and front that comes through my sinuses (along with my joints) can always tell long before we see or feel them outside. Knowing a front was coming through my sinuses were not overly swollen and inflamed like they usually are. They were still a bit "stuffy" in comparison to the last week, but nothing like I'd usually experience. There was no pounding sinus headache, pressure, or pain in my teeth. And for those of you whom experience sinus issues you know how wonderful being free of such a things is. 

Realizing Hope: The Voice of a People (Day 9)

Today is a beautiful and extraordinary day. My heart is contented in quieted peace as any words that dare escape me only want to pour themselves out through grateful tears. I get to share the screen with over a dozen miraculous human beings from all over the world whom together we share something beautifully rare. We all have a love of singing, but that’s not the rarity that connects us. We all share a common bond of genetic mutation - one that has shaped our lives in the form of a diagnosis called cystic fibrosis. One that has brought us together for this rare and beautiful moment. One that has allowed us to come together to be a part of the sINgSPIRE Virtual Choir.

Seeing these incredible women across the screen is truly life-giving. They are what feed my soul and give me strength - knowing that I am never alone in this journey with CF. That there truly are individuals who understand this complicated disease and the life it demands of us. We each have such diverse stories, but it’s such a breathtakingly beautiful thing when we come together as one voice. 

Today, after three months of working on our latest project, I get to share with these wonderful singers the culmination of our latest project in its finalized masterpiece. You see, because it is recommended that two individuals with cystic fibrosis not share a space due to risk and outcomes of cross-infection, it makes it impossible for us all to come together as one in the same space to share our voices. These women are defying a diagnosis. Their individual voices are a miraculous thing, but when they join them together the passion and strength behind the sound is more powerful than CF ever will be. And it’s because of each of them, I never stop fighting for more beautiful breaths for all of us. It’s because of each of them my hope lies rooted in simply having the strength, voice, and breath to share many more beautiful moments with each of them. My greatest hope, however? It’s that all of them will have the opportunity to live in the realization of such hope. 

Please join us on the Breathe Bravely Facebook page Tuesday, January 14 at 8pm CST for the world premiere of “We Give Voice”, or find it on our website www.breathebravely.org/wegivevoice

Here’s a teaser into our latest project: 

Visit the Breathe Bravely Facebook page and help us share the word about our upcoming project! Our voices are a powerful thing in and of themselves, but when we join them together to give voice to cystic fibrosis they are a beautiful and unstoppable force of hope.

Realizing Hope : Surviving on the Unspoken (Day 8)

Gentle snowflakes lightly swirl outside my window. I sit within their silence that is composed of unique and unmatchable wonderment. As I watch them dance outside my window, they all look similar and are defined by the same unassuming beauty. But, in truth, they are each unique to themselves - differing in size, shape, and possibility. Each falling from the sky with the same intention, but none knowing if they’ll ever make it to the ground or where exactly they will settle. Some fall heavily with great intention and determination and others seemingly enjoy the journey and never make it to the ground. As I watch the peaceful falling snow descend from the cool sky I see hope reflected in every beautiful flake that dances upon winter’s crisp breath. 

There are hopes that easily escape from our lips and blanket the world with their determined spirit. And then there are those we keep deeply embedded within the safe confines of ourselves, never daring to whisper them to the world for simply doing so leaves us breathless. They are the ones that we can’t bare to hear ourselves say so they find release in the form  of tears that unexpectedly overwhelm our eyes and stain our cheeks.

Our days are defined by hopes of each magnitude, seen and unseen. They swirl about us and within us as we pursue each day unknowingly seeking to see them find their fruition. Each simple hope realized in a gifted beauty we take for granted much of the time - just like the very air that fills our lungs. For as long as I can remember, I’ve survived on unspoken hope. It silently burns within me and melts away the cold, harsh, and gripping realities of CF. They’re composed of hopes married with dreams in which my heart wishes more than anything  to hold within my hands. But, even the most determined of snowflakes melt at some point when we try to hold them within our grasp. So, I keep them tucked away for fear that they’ll never reach the ground, or that they will melt before my very eyes at the hands and horrors of CF. 

As I step out into the crisp bitter South Dakota air this morning, an indelible hope escapes upon the breath I just gave back to the world - the warm air escaping my lips and kissed by tiny ice crystals dancing upon morning’s light set to remind me that as long as I’m breathing, hope will always be present within me and around me. No matter if they live silently within my soul or are shared on the breath of today’s wintery spirit. Just because each unique hope may not fall in the form of a beautiful, gentle snow, and it may instead come in the form of rain, I must remember both are renewing and life-giving in their own unique and beautiful way. All that matters is that there is hope and it’s ever-present. Love to you all. 

Feeling Its Effects

- Day 7 - 

If you know me well, you know I struggle with the cold. It takes the pain in my joints and body and seemingly magnifies it leaving me stiff and consumed in an icy pain. My hands are usually a pale white and feel icy cold to the touch no matter how I try and warm them. As I craw into bed at night my feet hurt because they're so cold. I usually try and get Cooper to lay on my feet to give them some warmth. But, something has been different the last few days. As the temperatures here in South Dakota dip into the single digits and winter gives its best blustery show, my hands aren't cold to the touch. I've noticed it on several occasions where my hands would be normally freezing with pain and they actually feel warm. How is this possible you might ask? When your oxygen level is low or your lungs don't work effectively or efficiently, your body spends a great deal of extra work keeping your organs functioning and working, sacrificing blood flow to the outer extremities. Better blood flow has to be good, right? :) 

Realizing Hope - Certainty in Change (Day 7)

We wake each day seeking certainty and stability. We look towards the east as our eyes search for any glimpse of the rising sun - seeking a silent certainty as we step out into a cacophony of chaos beyond our doorstep. The gentle brightness of the morning light infuses our spirit with an empowering hope that inspires us with a strength for whatever uncertainties may lie beyond that moment that we will be brave enough to face each just as we are. Then, nightfall dares to descend upon the day. The sun breathes its assuring beauty across the sky - kissing our eyes with its certainty of another day’s promise. 

We look to the sky to remind us that some things will never change. But in all actuality we are witnessing just the opposite. For the sun itself does not rise and fall in the same place in the course of a given year, and neither do we. Each day is new. It is brilliant with possibility rooted in a certainty only born through change. 

Change is the only certainty we have in life. It permeates our very being and is the mere definition of living. We fear change because we believe it lacks certainty, but it’s the very basis of any certainty we come to know. So why are we so fearful and paralyzed by the very change we are seeking for the sake of realized certainty? 

Because it’s always filled with so many unknowns in which we cannot control. 

In a life lived with cystic fibrosis, change and certainty have a complicated relationship. Our bodies are vying to destroy us - changing without our permission and we cling to any certainty we possibly can. It’s a brutal balance. One in which we know we can’t live without. Because in every new treatment to save us lies the certainty of change - it just might not be realized in the hope our hearts had set upon it. But there will be change. Sometimes that change comes like the mere rising and setting of the sun, easily seen and understood, becoming an assured certainty to the landscape of our life. Sometimes that change lies within the hidden certainty of mere minutes in the form of growing daylight - embraced by the small quieted certainties of progress. 

But certainty and change are impossible without hope. Every known and unknown certainty in our life is born from an unspoken hope that breathed life into its possibility. A drug like Trikafta will change many people’s lives, including my own. That change will be realized uniquely within every individual who is lucky enough to know such an opportunity. There will be change. That is for certain. The uncertainty lies within how that hope will be realized within each of us. The uncertainty comes from not knowing what kind of change that will exactly be and how it will manifest itself within our lives. But, all we can do to dispel those uncertainties and unknowns is bravely hope. With the break of a new day and the grateful setting of the sun, I will hope. And, in that hope alone comes certainty of change. Love to you all. 

Feeling its Effects

- Day 6 - 

The miracle that is this drug is not lost on me. I know there are changes happening within my body that I cannot even begin to fathom, nor can I readily feel or see. But, nonetheless change is happening - both positive and challenging. 

Yesterday came with continued challenges in the form of joint and body pain. I seemed to be able to stay on top of the worst of it and keep the edge off for most of the day with strategic timing and dosage of nsaids. Pain is something with CF I’ve learned to live with, but this intensity is pretty brutal and exhausting. With the good comes the trying, and I’m hopeful that this is just a phase as my body continues to adjust. 

Tremors and neuropathy are a frustrating combo. They’re something I’ve struggled with over the course of my years and progressive usage of antibiotics. I woke up yesterday morning with my hands tremoring and neuropathy in my left arm. I know with my own journey with tremors and neuropathy there are tougher days when they appear and other days when they seemingly non existent. I rise just ready to take on the day with whatever this body and life has given me.

There are still so many incredible things that my body is rediscovering. I’m in awe of the little things that have surrounded me that I haven’t been able to know. You know when you open a carbonated drink and take the first sip and it bubbles up into your nose? It hurts, right? I felt that for the first time in years yesterday. I forgot that even existed. I can’t help but smile. 

I asked Mark last night if my hands and fingers looked different to him at all. He said, “yes, they don’t look blue”. I was shocked. Maybe because I live looking at my hands all day that I have never thought them to be discolored or blue. Clearly that is not the case. 

Realizing Hope : The Illumination of Belonging (Day 6)

We never belong just to ourselves. Each of us is wrapped within the hearts and lives of others just as the stars are wrapped within the night sky. The darker the sky the brighter the glow from each illuminated and suspended diamond. Each one individually beautiful and bright. Yet, it’s when they all shine together across the expansive canopy of the heavens that they make the night sky truly come alive. Their gentle, yet powerful glow enveloping each of us just the same - silently reminding us that we are all connected to one another, and most of all, that we are not alone. 

We are those stars. Illuminated by a hope that burns within each of us - silently radiating upon the lives of those we love. That hope is what connects us to each other. Your hope becomes my hope, and the light of my own hope silently finds a unique place within your own life. Hope is born of the most raw and deep emotion. A life with cystic fibrosis is survived only in hope, yet lived and fought in grounded reality - one that can be born of immense heartache, disappointment, and loss. There are a lot of diverse emotions that come along with living a life with CF, and those of us living with the disease watch our friends and family endlessly hope along with us. My hope doesn’t belong to me alone, it finds an echo within the wishful hearts of those whose love illuminates my life. For me, the most difficult part of living with this disease is not enduring its unrelenting and unfair wrath, but it is not being able to live in the realized hope of those who surround me.

A drug like Trikafta ignites so much possibility for hope to be realized. A drug like this carries so much weight within not only my life, but the lives of those that love each one of us affected by CF. Because what if that hope is not realized? Not only will it leave our own hope silently shattered, but even worse, we will watch the hope of those our hearts belong to lose their life-giving glow - extinguished by a suffocating disappointment. Regardless of how Trikafta unfolds in my life, I will be able to resiliently endure and find the beauty in every breath. But, what I am not strong enough to handle is how such unrealized hope is reflected in the eyes of those I love. Just as my hope is their hope, their pain is my pain.

No matter the moment or where exactly this Trikafta journey is meant to take me, it’s moments like this I must remember to simply look up. No matter if I can see them or not, the night sky is always filled with stars - reminding me that I belong to something so much greater than simply CF. That I am embraced just as I am, however I am, and that most of all, I am not alone in this journey no matter the hope we share. We shine brightest when each of our hopes is reflected in each other, no matter how they are realized. The only thing that matters is that hope is alive within each of us. Love to you all. 

Feeling its Effects 

 - Day 5 -

I’m grateful for the opportunity to feel all the sides and impact of this tiny powerful drug. CF manifests itself so differently within every individual, and a drug like Trikafta therefore will impact us each in its own unique way, and on its own timeline. There will be days that Trikafta will give me its best, and there will be days in which I am reminded that this body still and always will have CF. All days, however, will hold their own unique beauty. 

I can’t even begin to tell you how great it is to not pull my contacts off my eyes every single night, but instead they easily just pop out. Or, to have my pockets stashed with eyes drops to make it through the day. No longer does it feel as if my eyelids are passing over sandpaper when I blink. It’s pretty wonderful.

Because Trikafta is aiming to restore a faulty CFTR protein and the way it expresses itself, the chloride level in my body is altered. This affects EVERYTHING. Even the oil glands in my skin. My usually dry hair and scalp that can go at least 5 days without being washed suddenly is filled with oils that I am not used to. Mark said to me just the other day, “I don’t think you’ll be able to go 5 days anymore without washing your hair. Your hair looks oily”. That’s love. That was on day 3 hair. It may seem like nothing, but it’s everything. 

Day 5 was hard, I’m not going to lie. My body hurt. Every joint in my body on fire and no amount of ibuprofen would touch it. Even putting on my Monarch last night seemed suddenly crushing to my frame. Joint and body pain is not something new to my life, but such intensity and the inability to subdue in any way is. Usually, when I would wake up knowing my body was going to work against me for the day, I’d simply push through and busy my mind to overcome it. But yesterday, no matter how hard I pushed, my body pushed back. Today is a new day, and all we can do is take the best from it and be hopeful in tomorrow.

I know so many would do anything to endure the best and the worst of Trikafta. I know this opportunity, while knowingly will have its ups and downs, is an incredible and priceless gift that I do not take for granted.