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.