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. 

Realizing Hope: Patient Transformations (Day 16)

The falling flakes of snow, a fury of beautiful wonderment. Its mesmerizing cacophony the unmatched medium fit only for a canvas laden with irrepricable possibility. The world closes its eyes to the masterpiece finding rest outside our windows. When we wake, the world suddenly seems brighter as the snow lay heavy on the still earth - blanketing everything that was brave enough to kiss the open air in the purest of white. The beautiful canvas illuminated by the sun just strewn before our eyes, waiting for us to sculpt and paint our own possibilities within it. The expanse of glistening bright snow begging each of us to leave our unique signature within its crisp stark beauty. Within that snow we can build and imagine so very much, but what we don’t see is what lies beneath that blanket of snow and the beautiful transformation taking place before the unseen eye. One in which takes great patience.

What we don’t see today is that in many months when the sun warms the earth and that once crisp white canvas begins to disappear, the most beautiful green life will emerge from that once quieted and cold earth. True transformation and possibility lives within patience. It’s easy to forget that. Even the building of a beautiful white clean canvas takes time, trust, and the courage to not only see, but live in the quiet beauty of its transformation. But, the beauty of true transformation is lost if we merely expect to see it happen before our very eyes instead of where the most significant transformation happens - within us. It’s the place which takes true patience and trust. It also takes the most courage.

Comparing Journeys

I have now had the incredible life-giving opportunity called Trikafta a part of my life for a full two weeks. These last few weeks have been filled with a myriad of diverse emotions, great anticipation, and most of all, a multitude of hope. These weeks represent a tremendous gift, and I hope are only the beginning of a life I never truly thought possible. These two weeks, however, have reminded me that my own Trikafta experience may be one reflected in a transformation born of patience and longevity, not seen or experienced within hours and mere days like many others have experienced with cystic fibrosis and their journey with Trikafta. Had I hoped that within hours and days I’d feel air pouring into my lungs unlike anything I had ever imagined? Had I hoped never again to wake during the night coughing uncontrollably? Had I hoped that my body would suddenly have the energy to keep up with my mind? Yes, but that’s not my current truth. And, just because those hopes haven’t been realized in the ways in which I had silently dreamed or read about in others' reflections, doesn’t mean my canvas hasn’t been transformed in its own unique and beautiful way in just these two weeks. This Trikafta journey and its transformation is unique to each of us. It’s a beautiful canvas we get to paint our hope-filled lives upon, realized and reflected within the hearts and minds of each of us in its due time. 

Patience is Possibility

When I looked out my window this morning I could have been crushed by winter’s cold suffocating presence. But, I chose to see the beauty it left upon my window as the sun illuminated every beautiful crystal of snow into a brilliant display of winter’s unmatchable beauty. I chose to see the beauty that lies within an awaiting transformation, for this snowfall will make those first signs of spring and its breathtaking beauty all the sweeter. I am grateful to see the beauty in today's transformations while living in the possibility of hope-filled patience. Love to you all. 

Realizing Hope: A Simple Miracle (Day 15)

There’s a living miracle in every moment.

Invisible to the eye.

Indescribable to the touch.

Yet, this miracle unassumingly tastes like life.

It’s a miracle we each know.

One we take for granted.

One when lost we’d do anything to know again.

It’s a gift whose worth is never fully realized until it dares to be a memory.

That simple miracle is the foundation of who we are.

Centered within all the love we ever dare give.

Every memory we create.

Every hope we share.

It’s the very giver and sustainer of this life we have been gifted to live.

Take a breath. 

Take two

Take 24,000. 

You’re feeling a miracle living within this very moment.

A miracle alive within YOU. 

Realizing Hope: "How Are You Feeling"? (Day 14)

The very essence of who we are and become is created wholly in change. It is the very catalyst of what silently allows us to grow in mind, body, and spirit. The anxiety we may feel when we think of change doesn’t come from change itself, but comes from our inability to control it or a mistrust we assign to it. But, even within the most uncontrollable change we unknowingly learn to live within its embracing metamorphosis. For time, in and of itself, is always changing. 

Learning to Live in Trust

For over three decades my body has been conditioned to mistrust itself due to changes in my life over which I feel I have had no control. I’ve learned that every seemingly “easy” day with CF is matched only with another to remind me that CF is unrelentingly holding my body and mind captive at all times. I’ve been conditioned through experience that change just merely is, and is nothing more or less than something I must approach with pragmatic hope. For hoping too greatly has left my heart broken and expectations for myself unrealized - suffocated by the realities of CF. I’ve learned to cautiously hope. To be guarded against myself and my trust of this body. I’ve learned that what I may feel today may so easily be stolen tomorrow because of CF. So, I’ve survived by gratefully living in the present for fear of a changing future - one in which I’ve learned I cannot truly control. It’s safest for me to live in what I know as the truth of today and how this body is letting me live - grounded in gratitude for every beautiful breath as it is gifted to me in this moment. 

So, the question that I’ve been avoiding for the last two weeks as I’ve started this Trikafta journey and knowingly is the one I evade on most given days is: “How are you feeling?” It’s one if you know me well that I do my best to deflect. For I’ve learned for my own self preservation to try and not compare one day to the next. I simply live within every day the best I possibly can - knowing that tomorrow will be different. I will live tomorrow just as I did today: with a grateful heart and with all that I have. This still doesn’t answer your question, but it instead forces me to truly live within the fears of change. Because this body of betrayal that I’ve known for so long has made me cautious and hypersensitive against any change, my silence is my safety and existing in the present becomes my life's only stable truth. For I fear the abandonment of realized hope that lives in the possibility of change.

A Change In Me

I treasure the silent small things like tasting food again or waking up and being able to speak without having to clear out the mass amounts of mucus that have drown my lungs and throat during the night. I cling to laughing without fear, breathing through my nose, and not feeling like I’m living within the middle of a daydream all the time - nervous to interact with people because my mind isn’t effectively and efficiently working to let me easily form sentences or think of simple words. Most of all, however, I cling to the small, but life-giving change of these lungs simply letting me make it fully to the end of two measures of singing a song again without needing to breathe. I hesitate to set these words upon my lips as every fiber of my being has been conditioned to be skeptical and mistrusting. Because the truth is that at the end of the day, I still feel like I have CF. That has not changed, and the heartbreak of losing all of those little or big things and feeling lied to by my own body again leaves me utterly breathless. Not to mention seeing the excitement in the eyes of those I love and how badly they want things to be better, only to have their own realized hopes crushed. 

Beauty in Change

Life is changing all around us and within us. We may not be able to control that change, but we can decide how we allow our lives to be transformed within and by it. We simply must entrust our hearts to the mere act of change.  I knew this journey would not be easy, but whatever tomorrow may bring I must be brave enough to embrace this transformation. Things will most definitely be different tomorrow, and I can’t control whether or not they become my steady truth. The truth merely lies in a hope that can only be realized through the eyes of the one who sees such transforming beauty and chooses bravely to share it. And today, I choose to share that beauty. Love to you all.

Realizing Hope: Fingerprints (Day 13)

No one can live this life for us. It’s ours to live. But, whether it’s amidst the  loneliest and most difficult of times or the most vibrant and beautiful moments of our lives, we are never alone. We are beautifully composed of the fingerprints left on our lives by every person that has rippled across our existence. They are what silently hold us up as we feel we are about to fall, and they are the contented beauty we see reflected in ourselves in moments of pure joy and elation. Each of those unique fingerprints unknowingly inspiring every breath of our life. 

Even down to these faulty cells, my life has been transformed by the fingerprints of the people who have touched my life directly and indirectly. I can’t help but wake up each morning grateful for the people who have poured themselves into my life and have shaped me into the person I am today. I only hope that within this gifted life I get to live that I wholly reflect all of the goodness that each of those fingerprints has placed upon my life. Most of all, I hope my own fingerprint silently leaves a positive and meaningful impact on those whose lives I am lucky enough to know. 

Living with CF can be a lonely place to exist, but it’s because of these genes that my life is filled with some of the most incredible and influential fingerprints. Some in which I have not, nor ever will be able to meet face-to-face in real life. But, it’s our very fingerprints that connect us to one another in a way only possible because we share a life of diagnosed circumstance called cystic fibrosis. Each fingerprint of CF is unique unto its person, but is laden with the same saltiness due to CF - causing us to leave a lightly salted outline of our fingerprint on everything we touch. It’s those very fingerprints that remind me that I’m not alone in this life and give me strength to find the beauty in every breath.

The drug Trikafta aims to correct the underlying cause of cystic fibrosis at the cellular level. It influences the faulty CFTR protein that causes the body to produce an imbalance of chloride which leads to the complications that are manifested within cystic fibrosis. The chloride imbalance is what causes those with cystic fibrosis to sweat excess amounts of salt and is what makes our fingerprints extra salty. Trikafta aims to restore that chloride imbalance to a more normal level which, in hope, allows our bodies to slow the progression and damage caused by excess mucus caused by CF. 

But what happens when that fingerprint changes? That I no longer leave the same salty print I did just weeks ago? That I no longer can find my glass in a crowded room because I no longer can see my salty fingerprints scattered like an abstract frost along its sides? Seen or unseen, it’s those very fingerprints that will forever connect me to a most beautiful community of individuals whom will continue to inspire every breath that I am given. It’s that unbreakable connection with this incredible community that inspires me to give voice to CF, and fight even harder for a day when every person with CF has the chance to live within realized hope born of a life-changing opportunity like Trikafta. 

I think that’s the hardest part of this journey for me. It’s holding those pills in my hand and thinking of the amazing community of individuals with CF that have touched my life in such an impactful way, and how so many of them aren’t eligible for this priceless gift of hope just because of there genetics, or that they are being actively denied the opportunity I simply hold between my fingers.  And it’s those very fingerprints that lay heavy on my heart and send tears streaming down my face. I can’t help but look at my hands and not see the 70,000 other individuals with CF and the hope they each wish to hold within their own hands. CF is a part of each of our lives and no one can go through this journey for us. But, we certainly are not alone in it - nor will we ever be. It’s those salty fingerprints that inspire each of us to fight even harder for one another, for a day when we all can hold such hope within our hands. 

While Trikafta gives great opportunity to individuals with at least one copy of the Fdel508 mutation, that still leaves over 10% of individuals with cystic fibrosis that do not have such a life-changing treatment opportunity. Within such a life-changing treatment are thousands of fingerprints, including yours. It’s those very fingerprints that give us life and fight everyday to give us a chance at another beautiful breath. For that, I and so many are grateful beyond words, but I ask you to continue to help us in this fight until it’s done. For everyone. Love to you all. 

*If you feel it in your heart and want to leave your fingerprint I ask you to support the Cystic Fibrosis Foundation by sharing your voice, giving a donation, or participating in one of their incredible events. Visit their website to find out more and learn how research and development of new drugs is giving us all a chance at more beautiful tomorrows with those we love. Visit www.cff.org 

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.