Why I Don't Write About Cystic Fibrosis

As someone who writes a lot, I am often prompted by friends to write about Cystic Fibrosis (CF), especially when I’m in a slump and asking for topic suggestions. I never really respond to that suggestion or write about it. But, deep down, I know they’re right. I mean the number one piece of advice known to even non-authors is write what you know, and since I have lived with CF for 46 years, it is definitely something I know. Yet still, I refused. Recently a friend asked why exactly I resist writing about it. I am generally not at a loss for words but that question left me in quiet contemplation. I now have a two part response. A short answer and a CF awareness plug. For those of you with CF, pun intended. ;)

The short answer is because I live it. I don’t want to give it life in my writing. I don’t want to give CF anymore life in my life than it already has. For example, when I was born in the 70’s, life expectancy was no greater than elementary school. I remember being told that I probably would not live past age 10. No, a doctor didn’t tell me that directly but he did say that to my mother when I was present. I guess he thought CF also affected my hearing (insert snarky face). Anyway, I never forgot it and the night before my 10th birthday I was terrified to go to sleep because I was worrying about dying. In fact I spent most of that year fighting bedtime until I expressed my fears. So many doctors put tags on my toes, so to speak. I finally stopped listening because I ran out of toes; but the thought of death has remained throughout my life. Don’t get me wrong, as a person my age with CF (which is not common), I am grateful and involved with helping - I volunteer for studies, have candid conversations with other CFers and their caregivers, share tips and tricks of managing the disease and I do so regularly through chat support groups and other means. But, writing is my escape. Writing is where I don’t have to think about my life in a day of CF. A day of hours of breathing treatments; of retching, hacking cough; of pain, struggling to breathe, more pain, respiratory therapy (see life vest looking thing in the video below. Yes it’s pink. No one said you can’t look cute during RT), exhaustion, medications, and did I mention pain? Managing CF is very much like a job. Think of it this way; even if you love your job, you still look forward to weekends and vacations. That’s the best analogy that I can come up with right now so I hope it’s relatable. I don’t like to complain and the older and less healthy I get, the larger the thought of death becomes. I spend so many hours of my life in a day of this illness that I need time away from it. Time to retreat and recharge both physically and emotionally. And that, my friends, is why I don’t write about Cystic Fibrosis. But, since I'm writing about why I don't write about, I might as well write about it. Right?

Roughly two hours a day are spent on inhaled meds. That's a lot of time just sitting attached to the umbilical cord that is the nebulizer tubing. I read, cough, catch up on shows, cough, play fetch with my dog, cough, and it helps pass the time. Cough.

My regimen (pictured above from left to right), is albuterol (to open airways), sodium chloride (to replace what my body does not pass to epithelial cells to break up mucus), Dornase Alpha (another mucus thinning mechanism), and Tobramycin, an antibiotic used every other month for 28 days to kill bacteria and help prevent infection. Math situation as follows: 2 hours/day breathing treatments + 2 hours a day vest therapy = But wait! There's more!

Pictured above is my daily cocktail; an assortment of vitamin & mineral supplements to, well, supplement what my body will not properly absorb or make on it's own. Staying hydrated is very important when you have a salt-loss issue (Note to self - buy stock in Powerade). Antacids are never far out of my reach. (Believe it or not, acid reflux can worsen or cause lung disease so don't ignore it. True story. Google it or, just keep reading because I'm going to add a link.) Speaking of acid reflux, the pills on the table (front row, far left), are digestive enzymes. Most CFers have pancreatic insufficiency, which means you don't digest food without help. I take roughly sixteen of these little beauties a day - four with each meal and two with snacks. Pancreatic insufficiency varies, so, again, some may take more or less, and there are some that don't need any. It all depends on the combination of genes you get when a mommy and daddy love each other very much (sarcasm and humor are my coping mechanisms, but that's another Hub). I take the rest of the prescriptions shown multiple times a day to treat or prevent various other illnesses and owies caused by CF.

This disease takes up enough of my life. I only allow it to have the time that is necessary. If I'm sick, I spend more time because, more meds, more hospital. I do whatever I can to stay as healthy as possible. Cystic Fibrosis does not define me. It lives in my life every day, but it doesn't have to live in my writing.

So, there you have it, the short story. And, in the grand tradition of Forest Gump, that's all I have to say about that.

Oh! except for the following CF awareness plug.

Much of the statistics about Cystic Fibrosis are available on the CF Foundation website which is https://www.cff.org/What-is-CF/About-Cystic-Fibrosis/ . But here is the gist of it; CF is a rare genetic disease (affecting roughly 70,000 people worldwide, 30,000 being in the US), that you are born with. Each parent must be a carrier of one defective gene (there are about 1700 CF gene mutations), and each must contribute a gene in order for you to have it. Since so many combinations of genes are possible there are different levels of severity, but most people are diagnosed by age 2, according to the CF Foundation. My extremely unscientific explanation of why CF causes severe lung impairment is that the defective genes are basically salt blockers. A regular salt/chloride level in the human body will generally retain fluids and help break up mucus (everyone produces mucus daily, not just when you’re ill). Since our CF bodies do not retain salt, which is why the test for CF is a sweat test (you could salt the rim of a margarita glass on my skin some days), the mucus becomes very thick and sticky and plugs airways - the perfect breeding ground for infection. Over time scar tissue from infection, chronic inflammation of lung tissue, and inability to move the mucus will lead to lung failure. Respiratory illness is the hallmark of CF, however mucus plugging also adversely affects the pancreas and absorption of fats, vitamins and minerals. In other words, I can eat like a sumo wrestler but the nutrition markers in my blood say I’m malnourished. If I had a set of lungs for every time I’ve heard, “you’re so lucky. I wish I could eat whatever I want and never get fat”, I’d be like Oprah - “You get lungs! And you get lungs! Everyone gets lungs!”

The CF Foundation website says the median age of life expectancy is 40, and that’s awesome, however, I’m assuming that pertains to those born within the last several years. Double lung transplants are relatively common (if you qualify), and increase life expectancy, but they are not a cure. The current transplant survival rate is 50% at five years. Organ rejection and complications from immunosuppressants are largely responsible for the 50% survival rate. While this stat is abysmal, there are 36% that hit the 10 year post transplant mark. Unfortunately, those that see that milestone also see instances of cancers such as lymphoma or lung cancer due to the immunosuppressants that are required for the rest of your life to keep your body from rejecting your lungs. There is oodles of info about Cystic Fibrosis on the CF Foundation website, please visit https://www.cff.org/What-is-CF/About-Cystic-Fibrosis/ to learn more. And remember, just because someone looks healthy enough to walk fast in Walmart, doesn’t mean they are. ;) Your kindness will help someone get through her life in a day of struggle.