Constance & Seeds of Hope

From the wind it was carried across the miles and brought to rest upon chance,  atop a place it could be inspired to grow, a place it could dance - 

A place the tiniest seed of hope only dare to take root, a place only hope dare know. 

Its truth seemed insurmountable and its potential off-written. The wild world seemed wrought with unchangeable circumstances and an unfavorable position. 

The week has found me within the walls of a room on 7B in preparation for another ERCP. This time, replacing the previously placed temporary stent within my bile duct with a more permanent one. After three rounds of chemo and having to be put under anesthesia again, my pulmonary team wanted to hit these lungs with some potent antibiotics to help them rebound and weather whatever may come. Gratefully, I will be headed home by the end of the day. 

That tiny seed of hope embracing all its odds left no match unmet - 

determined to weather every storm and be grateful for every sunset. 

Today she is known as “Constance”, she’s not grand but

she’s not small. 

She is made of patience and peace - her leaves unfurled

waiting for the sun to call. 

             I sit beneath a “Constance” set between a tower of                 reddish-brown brick 

shielded from the warmth and radiance of the sun hearing an invisible clock tick.

Shadows shift, dance, and dissolve as the wind moves and evolves.

Her leaves a muse to the season’s touch, light pouring through her canopy painting patterns of contrast by nature’s brush. 

I look up and see how she shows signs of the changing season found. 

The earliest of leaves caught just right by the wind gently find their way to the ground. 

Leaves left behind cling gently to the branch - 

fluttering by way of synchronicity as if they are just waiting on chance.

The wind is warm and welcomed. It’s persistent in its presence, yet feels strangely comforting as I tuck my short new bob behind my ears for what feels like the hundredth time. My tingly numb fingers quickly reach the end of my hair and I feel a familiar sting - a reminder of this reality. One that feels both familiar, yet so foreign. 

It is mid September so the sun hangs low in the sky. Pools of amber light pour across concrete canvases and the world has for a moment, an embraceable warmth. A gentleness. I close my eyes and do my best to hold this moment within my hands - grateful to be immersed in such beauty wishing for time to simply stop. For any progression to simply stop.


But, this is the truth I’ve always known, even if I haven’t always embraced it. My life has been built upon trying to stop time - to stop the progression of CF. And now, to stop the progression of pancreatic cancer. So, why then does it feel like I am a stranger in my own life’s story? 

    Because I never imagined to know such a season. 

The shadows cast by Constance’s leaves cannot dance upon the pavement without the contrast created by the sun’s radiance. We cannot know the power of the light if we do not know the dark. We cannot know the beauty of this life and its priceless meaning without knowing all we have to lose. We cannot survive this life without even the tiniest seed of hope. 

From the wind it was carried across the miles and brought to rest upon chance, atop a place it could be inspired to grow - a place it could dance. 

A tiny seed of hope only dare to take root - only hope dare know. 

Love to you all.

Simply Surviving : PC & CF

 “You’re so brave.” A phrase over the years that has been graciously poured upon me like a midsummer’s late afternoon rain. There's peace within such a downpour. Puddles ripple with a gentle invitation that beg my inner five-year-old to never look back while jumping feet first into the unknown depths of such uncharted waters. 

But, what if I told you that I am not brave? 

I am simply doing the only thing I can. 

Surviving. It’s all I have ever known. 

Cystic fibrosis has been an ever-changing storm raging within my life for over thirty-seven years. It seems ironic that for most of my life, thirty-seven was the median life-expectancy for someone born with cystic fibrosis - a vast improvement from eighteen when I was born. You can imagine that this past December’s birthday of turning thirty-seven was filled with many different emotions. (Feel free to read my birthday reflections here at “Lucia & Life Expectancy”.) 

“You’re so inspiring.” Another phrase that has been generously gifted to me by so many of you. Your words instill a radiance that illuminates an impenetrable hope and gives light to the most melancholy and dreary of days. Such warmth renews the spirit and reminds the heart that one cannot blossom without a little rain. One cannot bloom without a little hope. 

But, what if I told you I am not inspiring. 

I have simply been trying to hold onto this one wild and beautiful life.

In all honesty, I have simply been trying to survive.

“You’re so strong”. A phrase that has swirled around and through me like the wind across the painted prairie. It embraces every part of me being, and at times, holds me up when I'm sure that I don’t have the courage to stand on my own. 

But, what if I told you that I am not strong.

I simply don’t have a choice. 

I just want to survive. 

If you know me, you most likely know that I have a healthy sense of gallows humor. It’s how I’ve survived most of my life feeling as if I am running out of time. Pair that feeling alongside the immense feeling of guilt for a disease that I know causes people I love so much pain. Such feelings are deeply embedded within my being - a sense of responsibility that I must prepare those that I love for a world without me. Such a life approach may seem horrific to most people, but it’s all I’ve ever known. Dark humor is all I have at times to cope with such unchangeable circumstances. Not everyone appreciates my humor, but sometimes it’s all I have to survive my own life. Many of those people may have heard me say at some point, “if CF doesn’t kill me, then we’ve won the game.” Now that doesn’t mean I stand in the eye of a storm staring down a funnel cloud. I am grateful for this one beautiful life, and each of you that bravely embraces me with your own inspired strength. I am simply grateful to be alive. 

The ongoing battle between my life and CF’s realities has never been one that has been fought fairly, and while I would love nothing more than our relationship with each other to be temperate, I know CF too well. It never plays by my preconceived expectations or listens to my pleas. Why am I telling you this?

Many of you are wondering if my pancreatic cancer diagnosis has anything to do with cystic fibrosis.

Trying to bring some levity to the heaviness that engulfed my hospital room, I said to the members of my concerned care team, “Well, I always say if CF isn’t what kills me then we’ve won the game. Right?” I realize this might make you cringe, but again, gallows humor.

But, the reality is that my latest diagnosis of pancreatic cancer is caused by cystic fibrosis. 

What a low blow. Am I right? 

This is no cause for alarm to my fellow individuals living with CF, but people with cystic fibrosis are at greater risk of cancer due to ongoing complications derived from the CFTR protein dysfunction. More specifically, we are at the greatest risk of colorectal, pancreatic, and respiratory cancers. Here, a published article from the NIH and the National Library of Medicine explains it better:

“Cystic fibrosis (CF) is a genetic condition that affects the lungs, digestion, and other body systems. People with CF have a higher chance of developing certain types of cancer. The reason for this is related to a gene called CFTR, which is altered in CF patients. This gene normally helps regulate the movement of substances in and out of cells. When it does not work properly, it can lead to changes in cells that make them more likely to become cancerous. The cancers most commonly associated with CF are colorectal, pancreatic, and respiratory cancers.”
Read the Article HERE:  “Cystic Fibrosis and Cancer: Unraveling the Complex Role of CFTR Gene in Cancer Susceptibility”

You might also be wondering why this hasn’t been talked about much in the world of CF.

Remember when I talked about life expectancy and CF? Well, thanks to a multitude of advancements, therapies, and medications, to put it quite simply - we are living longer. With such incredible advancements come many things we have yet to learn about CF in an aging population. Like, how decades of non-functioning CFTR protein can lead to changes within a cell which can lead to cancer.  I see this as merely an opportunity to start a new conversation - one that we’ve never been able to have before. One that is only possible because of these gifted years. One that involves screenings, education, changeable circumstances, and positive outcomes. One that involves more birthdays. 

My dear friends, I alone am not brave. Bravery lives within the hand that reaches out to mine and doesn’t let go. That gives an extra squeeze followed by a silent smile.

My dear friends, I alone am not inspiring. Inspiration is born of one of those hugs that when you try to pull away the other person holds on a little bit longer - forbidding to let go. 

My dear friends, I alone am not strong. Strength is born from simply living authentically in your truth - reflected in those that stand beside you and with you through the best of days and the most merciless of storms.

We don’t get to decide when it rains and we certainly don’t get to decide the severity of a storm. The only decision we are given is how we endure the storm - hoping we might just get a glimpse of a rainbow. Hoping we might merely survive.  Love to you all.

**A special thanks to Jenny Humphries for sending me, as she said, "pancreas to punch". My shirt is compliments of my love, Mark. He knows me well. Thank you to all of you who have overwhelming sent your love, well wishes, and support. It means more than we can say. 

For those keeping track - 2 Rounds down. 10 to go. 

A Bird and Cancer

There once was a bird named Resilience. 

She loved to greet each new sunrise with a song and watch the setting skies turn into a cacophony of painted impressionism as stars pierced through a canvas sewn by fireflies. 

When the ground beneath her feet seemed unsteady and foreign, Resilience knew the only option before her was to learn how to fly. The warmth of the sun kissed sky would sweep her up over every cumulus cloud - weightless with reckless abandon. It’s only at such heights she could see this was a gift that came in the form of a vista. Would she have ever had the courage to fly if it had not been for sinking sand? 

When the skies bore an unforgiving cry, Resilience closed her eyes and hoped the rolling soft waves beneath would catch her - cradling her like a softspun lullaby sung from the turning tides of seas below. Or, if the waves did not hold, she’d surely learn how to swim. If the skies had not fallen out of favor would she have ever dared to taste the saltiness of the sea?  

 

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In early July, while gratefully recovering at the University of Minnesota from compounding liver complications, it was discovered that I have Pancreatic Cancer. 

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There once was a bird named Resilience. She loved to sing and would dare find there was always something good to sing about. But lately, those songs have seemingly found a chorus of cries. A few tears shed for herself, but most set as an uncontrollable spillway for the abundant love in her life that stares back at her. Each tear representative of the heartache, pain, and loss she continues to cause by the content of her composition. 

As life has taught me over these thirty-seven years, I was where I needed to be when I needed to be during all of this. If it had not been for the sudden onset of Liver and Bile Duct complications, my diligent and caring research team at Sanford along with my attentive, unwavering teams at University of Minnesota, this latest diagnosis would have never been caught at such a stage. For each of them I am grateful beyond words and know I owe my life to them. A familiar yet humbling place to rest amidst a harsh unwanted reality.

I had wished for the harshness of such a reality to find its numb place within my resilient spirit as I tried to hold back tears while coordinating a new world of referrals, oncologist/ GI appointments, PET scans, labs, chemo schedules all while trying to find the words to tell the people I love. There are only a few times in my life that I have known such hard tears: saying goodbye to Mark when we moved apart during Covid, saying the final goodbye to our first dogs, and facing the harsh unfair realities of CF ten years ago. It’s a cataclysmic schism that tears your soul in two and leaves every cell of your being laden with a restless ache that never seems to recover. You can mend the halves back together but it will never be the same.

After a meeting between all the brilliant, caring, and tenacious minds that represent my CF team and Pancreatic Oncology Team at the University of Minnesota and the Oncology team at Sanford in Sioux Falls, the best case plan was composed and a date was set to start this new journey. All in hopes it would be timed to leave me feeling my best for preparations and performances for our Breathe Bravely Backyard Bash.

Well, sometimes life happens. Instead of starting chemo I would test positive for Covid and our best laid plans would be delayed. Finally, on Monday, August 5th I received my first round of Folfirinox - a hard-hitting chemotherapy combination used to treat Pancreatic Cancer. If this body proves its stability and strength, my second round of chemo will begin again on Monday, August 19 until Wednesday, August 21.

The plan: 12 treatments in 6 months. Or, one round every two weeks. This consists of three different drugs: fluorouracil, irinotecan hydrochloride, and oxaliplatin. Two of them are administered at the Oncology Infusion Center within a several hours and one of them is sent home via continuous IV Pump (an old friend with whom I am very familiar sleeping). Good thing I’m married to my favorite infusion nurse. ***Just kidding, OCC & Oncology nurses, I love all of you!

I know what you’re all wondering. “Ashley, what’s the prognosis?”

My dear friends, if CF has taught me anything over the last three decades it is that we take life breath by breath alongside a hefty shot of hope. We do the best we can with what we are given, however that is given. I wish I weren’t writing this post. I wish more than anything I wasn’t the source of tears in the eyes of my love, my friends, my family. But, there will always be unchangeable circumstances that come with unfair expectations. We take this latest journey one treatment, one scan, and one day at a time - doing our best to be grateful for every ounce of every moment that is gifted. 

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I used to think that “resilient” meant turning towards the fight and having the strength and stamina to endure all it had to give - collecting lessons, scraped knees, and survival skills along the way like badges I’d need my mom to sew onto my Girl Scout Junior sash.

But I’m beginning to believe Resilience wouldn’t fly into the storm. She’d turn, stretch out her wings and let the force of the storm’s raging winds and battering tides be her strength - carrying her across the landscapes of this beautiful life. 

Love to you all.

Invisible - the silent truth of CF

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. 

A BIT OF TRUTH

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

https://fightcf.cff.org/site/TR/GreatStrides/80_Minnesota_Minneapolis?px=1821624&pg=personal&fr_id=10485

Love to you all.