Realizing Hope : The Making of Me (Day 4)

We are beautifully set within a framework of unique cells composed of unique experience and circumstance. They are a part of what makes us truly who we are and have accompanied us all of our days.  Through the best of moments and the most difficult, they have silently helped  us to become the person we are today. Our truth reflected in every single one. 

The truth is, these faulty cells of mine are reflected in every beautiful part of my life. While I am not defined by CF, it undoubtedly is a thread that has helped weave the most beautiful tapestry. I’ve learned over the years that even the most difficult of moments boast a unique and transformative beauty that has led me to know the most meaningful existence - one wrought with grateful purpose and passionate people. This terrible thing that has caused so much hurt, suffering, and tears in my life has also been a stable, yet unpredictable presence throughout the course of my existence which has shaped me into the person I am today. It’s the only way in which I truly know this body, even if CF is its ruthless and irremovable tenant. The chaos of CF is a part of my framework. I only know my body gripped with suspicion, mistrust, and compromise - all which have  influenced how I live to seek, see, and embrace the beauty in every breath I am gifted. 

While CF is an unforgiving and destructive force that will always be woven within my framework, I can’t help but be grateful for these faulty cells and the beauty they’ve brought to my life. It's taken me many years to no longer try and run from who I am and the very cells that compose my entirety. On Saturday, when I held that first dose of Trikafta in my hands, a drug that aims to modify my existence at a cellular level, my mind raced with so many different thoughts and my heart churned in a tide of emotion. 

A deep part of me couldn’t help but wonder:

“will I still be “me”?” 

“What if I’m not, and everything I love and find so breathtakingly beautiful is lost?” 

“What if I feel like a foreigner within my own body?” 

“Will it be worth it if these cells modified to give me life, in fact, make me feel as if I have lost it”? 

To be continued...

Feeling its Effects

- Day 3 -

I’m grateful beyond words for this life-changing opportunity and the potential to know more of life’s beautiful possibility. It’s one that is constantly on my heart and one in which forever be a part of the voice I vow to give to CF, no matter what my cells may look like. Most of all, I think constantly of the people and families who yearn so desperately for such a life-changing opportunity. And for that, I will never take such a thing for granted, but instead will breathe bravely and embrace every breath as it is given while holding and fighting for all to be worn with such emotion and possibility derived from such a life-changing opportunity. 

As I sat down to our table, opening up my laptop and setting my coffee within reach as I do every morning to work, I took an unassuming breath through my nose as I typed away on an email. That breath hit my senses differently than ever I can recall. The smell was fragrant, like the beautiful smell of spring blooming in quiet beauty. I looked over at the flowers my dear friend, Spencer, had brought me days ago, and I smiled.  I could suddenly smell them. And, it was a magnificent thing.

Mark made dinner for us and the smell was incredible, but it was the flavor that blew me away. Now, Mark is a great cook, but tonight everything tasted different. It was divine. Everything tasted so alive and flavorful. I remember having a similar sensation after I had sinus surgery years ago which clearly disappeared as my sinuses became impacted again with inflammation and infection over time.

Realizing Hope : The Heartache of Hoping (Day 3)

Every beautiful and difficult moment of our lives is rooted in a seed of hope. Each hope born out of circumstance or experience, sought after or merely unexpectedly given. Hope is always evolving and blooming into life anew, just like the very breath in which we take and give back to the world. Sometimes, hope is a beautiful realization of all our heart could ever possibly dream. It’s breathtakingly beautiful.

But hope also holds the potential to culminate in a way in which shatters our heart. A devastation that grips every breath, forcing us to grow in a way we never thought possible or silence us from ever hoping again. But within even the greatest devastation are embers of hope, waiting to be set ablaze within our souls. It’s what we do in that devastation that makes us truly who we are and allows us to fully live our truth. For that is where the greatest possibility lies. 

CF has broken my heart and my spirit before, and I’ve learned to guard myself against the hope I freely give to it. After all, the higher the hope the greater the risk of losing myself to its heartache. And that hope, or the loss of it, never falls upon just oneself. I hide my tempered hopes beneath an air of bravery and gratitude. It’s to protect not only my own heart, but the hearts of those I love so dearly.

Buried Hope

A few months ago, after the approval of Trikafta which came almost a half a year earlier than expected due to such promising clinical studies, I found myself in the grips of abundant hope after finding out I would, in fact, be eligible for this drug based upon the FDA’s wording and the luck of my genetics. My mind began racing with possibilities and hopes that I had thought been buried long ago. Suddenly, the “what ifs” of life I had mourned and silenced found a voice again. My mind racing and my heart bursting. That night, after sharing the news with close friends, I crawled into bed hours after Mark had been fast asleep, just as I’ve done for years. This time, however, tears drenched my pillow as I stared at the love of my life, hoping beyond all hope that we might grow old together. I laid there with my mind running wild and my heart remembering what it was like to dream - thinking about the other loves of my life: my dear friends, singing, our goddaughters, traveling, and so much more. It’s a hard thing when you’ve spent your entire life preparing yourself to no longer be a part of the people you love’s narrative to suddenly be engulfed in a flame of unbridled hope. Living simply in the beauty of every present breath is a beautiful thing, but it also has been a way to protect my heart when it comes to living with something so unrelenting and merciless as CF. Living within present hope and a humbled gratitude have been my solace and my means of survival. 

Guaranteed to bloom

The truth is, these lungs are damaged, this body is worn, and it’s riddled with over three decades of the effects of cystic fibrosis. These lungs have only known recurring infection, are adorned with bacterias resistant to antibiotics, have weathered countless adverse effects, and years of progressive scarring. There’s much about my life with CF that will forever be unchangeable, even with a drug like Trikafta. But regardless of what comes from knowing such possibility, I take the deepest breath I am gifted at this very moment and guard my hope and my heart with all the gratitude I have. For I don’t know if I can mourn the same hopes again. But, I can and will find the beauty in whatever this journey brings and the new hope it inspires.

There are never any guarantees in this life and that is merely why we must hope. It gives us life, regardless of how it is ever realized. Out of devastating hope something more beautiful than we could have ever imagined flourishes into existence - blooming into something greater beyond our dreams. As I begin this third day of Trikafta I’m filled with hope. A hope that this life will bring me to where I am meant to be and that it will be realized in my living truth - bringing beauty to the world through whatever voice is needed.  Love to you all.

Feeling its Effects 

- Day 2 - 

Each morning as I pop the first two Trikafta pills out of the blister pack for the day, I hold them tightly in the palm of my hand, take a thoughtful breath, and gratefully hold the memory of those with CF who have come before me and all those currently waiting to know such a possibility in the form of three small pills. I whisper beneath my breath, “Come on, little body. We can do this”. 

When I woke up the on morning of day 2, the first thing I noticed was that I was breathing differently through my nose. I can’t explain it exactly. I could feel air passing differently through my nasopharynx. The air felt somehow unrestricted. Don’t get me wrong, there’s still a lot of pressure in there, but it's the first time in a long time I didn’t feel as if I had to pull air into my body through my nose. Day 2 also brought me a new sense of smell I never knew I was missing. It may sound bizarre, but I buried my face into Kal’s fur (he is so fluffy right now!) and noticed he smelled differently. Not bad, just different. Like, I could smell the oil from his skin. When I hummed a song today, I also felt the vibration differently in my face. Or, I should say I actually felt it which hasn’t been the case in a long time.

My lungs also felt incredibly sore and my usual dry cough had some rattle to it. Most of all, I could feel things in my chest moving around. I imagine my lungs are like an expert level pinball machine with so much scarring and bronchiectasis making it difficult for any mucus finally moving around to find its way out. I’m also trying to be cautious as to not break any ribs or exacerbate the last ribs I broke a month ago. We knew going in to be cautiously optimistic with how these lungs might be affected. I have to remember that positive change doesn't always come in the form of a bigger number, but can come in so many other different ways. For now, I am patient with these lungs and giving them much grace and gratitude.

As for side effects, by the end of day 2 the horrid stomach pain I've been hearing about settled in. I had a similar pain years ago and the only way I can describe it is if you’ve ever had the wind knocked out of you or been punched in the gut in seventh grade by a soccer ball. But, as we know, CF doesn’t just affect the lungs, it affects every organ. Imagine 33 years of buildup in your body’s duct work is finally moving the way it should. “Come on, little body. We can do this”. 

*This is merely a reflection of my personal experience taking Trikafta. Please consult your care team if you have thoughts or questions about Trikafta.

Realizing Hope : Changing the Unchangeable (Day 2)

For thirty three years I have known nothing but a life with cystic fibrosis. A journey plagued with nothing but heartache and loss. Loss of dreams, loss of lung function, loss of freedom, and loss of life -  figuratively in the sense of my own and literally in the form of my brother and friends afflicted by the disease. I have seen and felt the devastation of CF. I’ve seen the burden it not only places upon those who live within its grip, but also the people whom love and cherish those that live such a circumstance. A circumstance that until now for many of us has been unchangeable. Whose course for decades had been similarly yet uniquely decided, given only the certainty of the present moment while clinging to hope that sustained us.

There still may be no certainty to this life or this body of mine that has been riddled and beaten by CF for over three decades, but for the first time in my entirety, there’s a hope realized in possibility. And that possibility comes in the form of three small pills taken every day for the rest of the days I am gifted. 

What is Trikafta? 

It’s a culmination of brilliance, years of tenacious research, trial and error, and most of all, resilient hope. It’s the fourth drug of its kind for CF that targets the underlying cause of cystic fibrosis at the cellular level, but is the first approved for individuals with just a single Fdel508 mutation. There are over 1,800 mutations that cause cystic fibrosis - manifesting so differently in every unique individual and causing their own set of progressive and fatal complications. Clinical studies in Trikafta showed great promise with an average lung function increase in participants of 14%. While there are no guarantees that all or any of us who begin this journey may know such extraordinary gain, and by no means is this a cure, each of us cannot help but live within the unbridled hope that our lives may know simple stability and the ability to dream beyond today. 

As tears continue to find their place upon my cheeks throughout this emotional journey of gratitude, I remind myself as I start this new day that any and every breath I am given is a beautiful one, no matter the number that fill these lungs. The hope that lives upon every breath of yesterday, today, and tomorrow is because of the life-changing people that have poured themselves into giving every one of us that lives with CF another beautiful breath, and who continue to do so. For each of you I am grateful beyond measure. You are daring to change that in which was dared to think unchangeable and will not stop until we ALL may know such a possibility. Love to you all.

Feeling its Effects - First 24 Hours - It was so special to share the moment of taking my first dose of Trikafta with someone who has been such a significant part of my CF journey and so much more. It's a moment I will hold in my heart forever. Within an hour of taking my first dose, my usually overly dry mouth was anything but dry. For the first time in years I had an abundance of saliva - to the point I felt as if I was going to start drooling. Also, my usually dry eyes which make me feel every blink suddenly were moist with renewing tears. It may seem like a simple thing, but it's pretty crazy to feel the effects of how my cells are changing in real time - for the first time in my life, for the better.




*To learn more about Trikafta visit https://www.cff.org/Life-With-CF/Treatments-and-Therapies/Trikafta/. And to support further research efforts so that all may know such possibility donate to the Cystic Fibrosis Foundation at cff.org

Realizing HOPE - Trikafta

Hope. It’s where we live to survive. It’s where we’ve turned our mournful tears into something that, in turn, becomes what sustains us instead of destroying us. We heal, we repress, we move forward - not forgetting but simply motivated to fix our eyes upon the present so that we may have the strength to endure the future. Not only for ourselves, but for those we love. We are nothing more and nothing less than the hope that courses through our veins. Our tired spirits revived by a courage inspired by the people in which we fight to embrace for another day - drenched in a hope that connects the certainty of this very moment to a future that pleads to be known in every beautiful breath. 

I have uncontrollably shed more tears in the last few days than I have in years. It’s the realization of hope born to a reality I never truly dared to think possible - one that I have honestly protected myself from thinking about. Today I take my very first dose of Trikafta, a drug that has the potential to transform this life and truth of cystic fibrosis and the very hope that has sustained me into something I cannot even begin to fathom or begin to find the right words. There are only tears of overwhelming gratitude.

The transformation of hope into a living dream is a terrifying and emotional leap - one filled with no guarantees. The only guarantee is this very moment and the hope it will always hold. Join me here for daily posts as I share the first 30 days of my hopefully lifelong Trikafta journey. 

As I begin this Trikafta journey, I can’t help but carry on my heart the people who are still waiting for their own hope to be realized in such a way. The tears I shed overwhelm my eyes and drench my cheeks - rooted in a deep heartache for those who are waiting to have their own hope born of new priceless possibility. Please keep them on your hearts. 

Love to you all. 

Forgetting CF

There isn’t a day that goes by that I don’t think about all that is life. About how different tomorrow could possibly be, or how different even today could have been. I think about yesterday, am grateful, but silently wish that I had done more. Such ruminations usually find me as early as the moment my feet find themselves beneath my body and my lungs turn to face the day. I brace myself to cough - getting the first true sense of the day while pushing through the breath-gripping stiffness left by the night. Like the bud of a rose tightly wound in upon itself on a cool morning, these lungs, too, aren’t ready to open for the day. I do my best to breathe life into these lungs so that their tight embrace upon themselves might open to let each breath fully, yet unsuspectingly bloom.

I do my best to shed the weight left by life’s heavy questions - breathing bravely as I set out to give the day all I am able to give and release myself of CF’s defining grip. My mind constantly at odds with itself - trying to positively define myself in the wake of what it means to live with CF, yet coexist in a world in which most people never have to live with it, let alone think about it.

- There is CF.

For most of my life I’ve worked hard so that you'd forget about CF. To give you the gift of not worrying about this disease and its weight that is ever-present within every breath - striving to depict CF in a light of constant strength, perseverance, and unwavering hope. I do this not only for you, but for myself. It's a way to focus on the beauty of these 65 roses rather than feel the deep unforgiving thorns that leave their countless scars deeply embedded in my being. It’s always been a means of coping for me. It’s been a way to not feel defined by a disease but instead be merely bettered by an unchangeable diagnosis. It’s a way to try and forget the difficult and unforgiving side of CF and its irrevocable presence in my life. Most of all, it’s a subconscious way in which I try and protect those I love. And, I can do so because of how invisible CF can be to the unsuspecting eye - easily forgotten or excused away when not readily seen or felt.

But, the thing is, I can’t forget about CF. Why? Because in every cough, in every breath that catches just a bit, in every wave of fearful panic and sharp pain rooted in hemoptysis, in every VEST treatment, pill swallowed, IV antibiotic, port access, visit to the pharmacy, and unpaid medical bill -  there is CF. In every contemplation of my past, reflection of my present, and aspiration of a future - there is CF. In all of the good and beauty that permeate my life - there is CF. In moments of heartache and uncontrollable tears - there is CF. In every note I sing - there is CF. In every relationship I am grateful to know, realized or not - there is CF.

Carrying It Alone

And sometimes, that can be a big burden to carry on your own. CF can be an incredibly lonely disease. Not just for myself, but the 70,000 other individuals that live with CF as well. But, it’s not just them. It’s their families. It’s their closest friends. We cannot forget CF. It’s woven intricately into our lives and inspires each of our unique stories. While I am not defined by this disease, it does undoubtedly influence not only every breath that I am gifted, but the eyes in which I see the world, the heart in which I yearn to share, and the beauty I am determined to give back. Part of that beauty will always be inspired by a disease that one day, I long for us all to be able to forget - together. A day in which the only roses that may leave scars upon our lives are those that we put in a vase for all to marvel at because we simply must share such unforgettable breathtaking beauty. Love to you all.

What's your unforgettable?

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Together, let’s be the voice of CF.

This week is the final week of Cystic Fibrosis Awareness Month. A month devoted to giving extra voice to cystic fibrosis. I am asking that this week you take a moment to just breathe, to be wholly grateful, and, most of all, in that very beautiful breath remember cystic fibrosis and its unforgettable community of people. Together with hundreds of others whose lives have been impacted by the diagnosis of cystic fibrosis, this coming Saturday, June 1st, I will be walking as a part of the Great Strides for Cystic Fibrosis which raises money for the life-saving work of the Cystic Fibrosis Foundation.

Sign up to walk, be a virtual walker, or donate to Team Ashley. May we come together not defined by this disease, but as an unwavering voice of strength rooted in a capacity of meaningful compassion - fighting together for more beautiful tomorrows for all whom are impacted by cystic fibrosis.

Great Strides for Cystic Fibrosis

Saturday, June 1st, 2019

Riverdale Park, Sioux Falls, SD

Invisibly Seen: Ruminations on Five Feet Apart

I sat at the center of an empty theater with the essence of my life being reflected from my eyes - a moving image that seemed to have been pulled from the reels of my deepest unconscious being. It was epically surreal and constant feelings of deja vu reverberated through my body. I not only saw themes of my own life drawn out in technicolor before me, I felt them present within the very breath that poured into my body.

In-Visible

Most of all, sitting there I felt the echoes of diverse emotions permeate the weakened and patched fractures of my façade - daring to overwhelm me with a truth in which I internally battle to accept and fully embrace both physically and mentally. While what I saw isn’t an exact depiction of my personal journey with CF, there undoubtedly were many unmistakable moments of familiarity that dredged up a well of emotions. Ones in which I knowingly keep deeply suppressed and safely removed from the rawness of the world. Ones which silently accompany me through this life in which I’ve dedicated to living my truth - a truth founded upon CF that while may be visible to the world, can yet remain truly unseen.

Sitting there drenched in the theater’s darkness and embraced by its comforting anonymity, I felt for once in my life truly seen. While every individual with CF has a different living truth unique unto themselves, there is an unfathomable reality that much of the world has never been able to even begin to imagine. This movie in no way begins to depict every unique and complex story of CF. But, it does authentically capture CF’s unforgiving and unrelenting mental, emotional, and physical realities. It gives us the chance for CF not only to be visible, but for each of us and our unique stories to be truly seen and most of all, shared.

Unspeakable Empathy

I left the theater that day struggling to comprehend what the general public would possibly feel during the course of those two hours and fifteen minutes. They simply couldn’t begin to know or understand what it was like to see your own truth depicted on a screen with such evident care and honesty. Mark and I had the theater completely to ourselves, so I will never know such an audience’s reaction. I couldn’t help but wonder if those who had seen the movie were experiencing the same raw emotion churning within them as I was experiencing. I wondered if they would walk back into the light of the world and see things a bit differently - if they would see CF a bit differently. Did they even know what CF was before this movie? Would they see not just a disease, but a real person? A population of people? Would I be able to see understanding in their eyes? An unspeakable empathy forged in action for fighting for more beautiful breaths? Could I see my own fight for every beautiful breath reflected in their their own eyes and a tenacity within them to fight for more?

Here’s to seeing the beauty in every breath - even those projected onto a screen. Here are some ways in which we can be a positive and life-giving part of this movie’s ripple:

1). If you are an individual with cystic fibrosis, live your truth and share your story! We are listening.

2). If you have not seen the movie, “Five Feet Apart”, I urge you to do so to get a small glimpse into the complexities and demands of CF. Remember, this is a fictional story, but the portrayal of CF and its realities are thoughtfully depicted in the movie. Thank you to Justin Baldoni and the cast of the movie for taking such care in the portrayal of CF and the overall representation of its community through the friendship and love shown through Claire Wineland.

3). TAKE ACTION. If you truly see us, join us in the fight in adding more tomorrows for each of us living with CF. Make a donation to www.cff.org. Your donation could literally be life-giving.

4). Watched the movie? Reach out to someone with CF and start a conversation. Ask questions, get involved, hear our diverse stories. Most of all, become part of our tenacious and strong community.

Love to you all.

*These are my personal opinions and thoughts in regards to the movie, "Five Feet Apart". Opinions and experiences will differ for every unique individual both living with CF and those who are hearing its name for the first time.

Building Castles : fighting for the opportunity to live

It can be a difficult place to live in the present when it feels like the sands are always shifting beneath your feet. There’s a perpetual anxiety that dwells within me that pulls me deeper into the quicksands of CF. An anxiety that doesn’t come from an inability to fight or finding the tenacity from within to give life your all, but an anxiety that comes from being denied the opportunity to fully live. I don’t think there’s a day that goes by that I don’t think about healthcare, about how the fight to live gets more difficult, more expensive, and comes at a cost to all of those I love. I see how so many have sacrificed so much for me while I and so many like myself fight with everything we have at the chance for another beautiful breath.

This week, positive preliminary results were released for the clinical trial of the VX-445 Triple-Combination therapy that targets the underlying cause of CF. This drug has the potential to significantly impact the lives of over 90% of individuals with CF that have at least one copy of the mutation F508del. This is what I and so many like myself have been waiting for. This is the living hope not only for a single tomorrow but for a set of many. But, paired with that hope is a bubbling anxiety that it will come at a significant cost. I think about the potential to be given a future never imagined and the potential for it to never come to fruition because that future costs too much, is a burden to those I love, or is untouchable because of ever eroding protections for people like myself with high medical needs.

I know from the outside it can seem simple to believe that we are too expensive, that it shouldn’t be the responsibility of others to shoulder the expensive burden of such a disease. I understand that. But, I live on the other side of that. The side in which I’ve watched my husband take a second job to pay my ever-increasing medical bills - knowing deep down they’re only going to get more expensive and complex as CF itself progresses and any and all life-extending therapies will come at a rising significant cost. To live with an anxiety every day that there are people entrusted with power who don’t believe your life lived with a pre-existing condition is worth protecting is a very disheartening and crushing reality. It’s also crushing to watch those you love be willing to sacrifice everything for you. It’s an anxiety that makes thinking of today, let-alone tomorrow unsettling at times. Especially, when you know such shifting sands aren’t best for building castles. Love to you all.

GIVING VOICE

Today, close to 150 CF advocates are taking part in the Cystic Fibrosis Foundation's March on the Hill in Washington D.C. where they are telling their personal stories to elected officials about the importance of adequate, affordable, and accessible healthcare for those not only with chronic illnesses such as cystic fibrosis, but all those living with pre-existing conditions. While family, friends, and passionate advocates are marching on The Hill today, we can help them as well in this movement by sharing our voices online for this impactful day of action. Please take a quick moment and visit the link below to send a message to your representative(s) : Online CFF March on the Hill