It is a surreal time to be living with CF. To say there are a multitude of emotions I feel within a given day would be an understatement. Maybe it is the grateful vantage point I get to hold because of the dedicated work of Breathe Bravely - immersed in a community as vast as the night sky yet connected by a prescribed constellation called CF. Much of our lives have been held within a hope, a wish, that our live’s storied truth might be changeable. That our course might be charted along a different set of stars other than the one set within our genetic code.
And for many of us that course has changed, evolved, and transformed beyond the perceived limits set by unchangeable circumstances. After all, I am writing this very message because of a hope, a wish, and most of all, a commitment to action by a greater community to never give up. Over the years there’s been much to celebrate. Advancements, breakthroughs, and a tenacious spirit that has given more todays to so many of us. But by sheer definition a celebration fails to hold such value without knowing the weight of loss, heartache, and hopelessness.
How does a person make sense of all of these emotions within themselves, let alone try and make sense out of it for a greater community? After all, I believe I can speak as a person living with CF that we simply want to be the good news, to be the living hope so that we might universally live in the unchained euphoric ethos of celebration - a place where no individual, no family, no community might know the exhaustive devastation that can come from cystic fibrosis.
While there are so many variables when it comes to CF, there’s a single absolute that I’ve come to know alongside this disease. It’s that alongside progress, advancements and milestones it becomes more difficult to tell the encompassing story of CF. From a single diagnosis comes 90,000 different stories, circumstances, and evolving truths. Disease, diagnosis, genetic mutations, manifestation of disease, accessibility, support systems, and sometimes simple luck are what manifest our realities. The unknowns are our only constant.
LIVING MY TRUTH
I spent many decades in my life running from CF and keeping any signs of it hidden the best that I could. I knew this disease only as something that brought immense pain and heartache. And then ten years ago on April 1st, 2014 because of the increasing unavoidable realities of this disease paired with unrelenting voices of support I opened up my life and began to share my truth. Little did I know that in doing so such truth would bring about some of the greatest meaning. Over these last ten years alone, CF has played quite the role within my life. If I allow myself to reflect long enough on the time dedicated to this aspect of my story there’s an unavoidable and overwhelming sense of sadness. A quiet reality that can be measured in months of IV antibiotics, hospital stays, test tubes of blood, airway clearance treatments, hemoptysis (coughing up blood), medical debt, tears, pills, antibiotic resistant “bugs”, and altered plans. Despite these unchangeable circumstances there has been so much beauty. While I’d trade the pain this disease has caused the people I love in a heartbeat, for myself I wouldn’t change a moment if it meant never knowing the depth of love and abundance of good this life has to give. And within this last decade there have been moments of realized hope. Moments worthy of unparalleled celebration, gratitude, and unbelievable awe.
I also recognize how difficult it is to witness a life lived with CF and how much strength and endurance it takes as someone breathing beside us. That is not lost on me. I see how desperately the people who love us want us to know a life of peace, healing, and freedom. It’s one thing to endure unchangeable circumstances but it’s another to see how it affects the people you love. At times, I feel that’s the worst part of it all. It can feel suffocating with the amount of hope one feels they need to hold in order to somehow protect the people close to them. “Chronic” and “progressive” are words that have accompanied my life for well over three decades, but just because they’re familiar doesn’t mean they’re understood. I’ve found at times it’s just easiest to hide behind silence and a smile.
WAITING ON A MIRACLE
As many of you know, on October 21, 2019 a quintessential drug called “Trikafta” was approved for the most widely common mutation of CF. Individuals had to have a single Fdel508 mutation to qualify for eligibility. 90% of individuals with CF have at least one copy of this mutation. I, gratefully, fell within the cohort of individuals who have a single copy of Fdel508. I remember that day so vividly. The phone calls and uncontainable feelings of joy. To tell people that you love that their hope has been realized is unlike anything else. To tell them that the tomorrows deserved by so many are not only a dream but a true possibility. It is unimaginable. It was a beautiful moment filled with so much opportunity and charted a course of possibility within all of science. One that has changed so many lives.
Have I witnessed such a drug transform the lives of friends, students, and community members? Yes. Is it a beautiful gift to see hope realized and people you love within a community become who and what they never thought possible? It’s hard to find the words that adequately convey the impact of such advancement. There may be 90,000 different CF stories but one thing is true amidst them all - we all hope for a future world without CF, not simply one where it is “invisible”.
I gratefully had the opportunity to begin taking Trikafta January of 2020. I tempered my excitement and expectations alongside a quiet pleading hope set behind a smile - a hope that my own experience with Trikafta would be what my loved ones needed it to be. Maybe, just maybe, unchangeable circumstances and trepidation for the future could be transformed into a tapestry of tomorrows where the moon feels within reach. Or, at least be a hope-filled reminder of progress, evolution, and possibility. To simply never give up hope.
THE ART OF SILENT CHANGE
Did such a drug change the course of my life? Absolutely. Has it written pages within my story that I thought were impossible? That is undeniable.
To be present for your own life. To wake without drowning. To actually feel an embrace from a loved one for the joy it gives rather than one that is rooted in worry. The kind I’ve known for so long in which you can feel fingertips desperate and silently pleading - holding on to dear life.
To meet the eyes of a friend and not worry your gaze will give you away. To have CF truly “look” invisible to the naked eye. I, too, almost let myself believe it. After all, I’ve had decades of practice doing so. And life did change, for a while. For a while the wake of CF had calmed. I had hoped such tumultuous tides would forever be a memory. It's amazing how today everything can be so familiar yet different. Whether it’s because of time, experience, endurance, or a drug… in many ways I have changed and this journey with CF will continue to change.
Over the last four years it’s been easy to slide back into a place of silence when it comes to sharing my life about CF. After all, during such a time we all needed something to believe in. We needed those glimmers of hope and stories of redemptive truth. For many of us during that time, hope is merely all we had. I realize just how much of a privilege that is.
To be honest, I have found silence to be a place of comfort rather than fielding the weight of disappointment. A disappointment and loss that I feel like I am responsible for, as ridiculous as I know that sounds. And maybe silence is easier than trying to find the right words to describe the changes I've endured. Will people think I'm crazy? That I want to be sick? What if I am crazy and I'm really just fine? I don't want CF to be my life again. My mind is a noisy place no matter the stoicism of silence. Recent conversations with loved ones have led me to realize that this comfortable place of silence is actually doing no one any good. While my story is different, my story is also connected to so many other people I love within this community. Their story is my story, and mine theirs. Silence doesn’t promote progress, nor does it protect the people I love. Invisibility can simply be a false sense of realized hope.
So, here’s my truth. Over the course of the last six months many realities I knew prior to Trikafta have returned: large episodes of hemoptysis that have required embolizations, weeks in the hospital at the University of Minnesota, months of IV antibiotics, mind numbing side effects, treatment upon treatment upon treatment, insurance headaches, never ending medical bills, and many altered plans. And, it’s pretty clear that this will continue to be my reality. The truth is that I still have CF. That no matter the stability and moments of “quiet”I have been afforded over the years thanks to CFTR modulators (Trikafta, Vanzacaftor, etc) and other medical advancements, this body is still riddled with the unforgiving circumstance called “cystic fibrosis”, and it always will be. So, why do I share this now? To be honest, I’d much rather live within the comfort of silence, and avoid being the reason for any pity, worry, or disappointment. Because my life is beautiful, it’s abundant, and there have been changes that have made me better. I share this with you because silence doesn’t save anyone. Silence doesn't change my own truth; the facts. While the sky may look different when you look up during different times of the year, the stars themselves are always present. CF continues to be a devastating reality within the lives of so many of us. Within the lives of my closest friends. My community. Your community. It’s anything but invisible to those who daily feel its presence hidden within an unspoken hope weighted within every breath. Whether it’s because of age, disease damage, accessibility and eligibility, and our mere genetic makeup, CF continues to demand our exhausted attention and unwavering strength - all while holding onto hope.
THE CONVERSATION
I ask you to keep fighting alongside us - for us. The conversation isn’t over nor is CF something of the “past”. Whether it’s a renewed commitment to the cause or merely being a part of sharing its diverse story, I ask you to join me in fighting for possibility.
If you’d like and are in the Sioux Falls area, The Cystic Fibrosis Foundation’s Great Strides is taking place Saturday, June 8 at Riverdale Park. Or, simply give a donation to the Cystic Fibrosis Foundation in honor of hope. While, yes, our ultimate goal is to find a cure for cystic fibrosis, there are thousands of people living with CF holding out hope for medical advancements that can combat the continuing damage of this progressive disease. We walk not only in pursuit of a cure, the research and formulation of new antibiotics, phage therapies, and gene editing, but also for the hope that fuels the possibility of more tomorrows. Tomorrows for EVERY unique person and EVERY unique STORY in EVERY CIRCUMSTANCE.
Join Team Ashley or donate to CFF
Love to you all.
