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.