So today's post is a little bit of a cheat, but I think it's important. I wrote this as a guest post for a good friend's blog and realized I don't even have a good review on my OWN blog! As a last little salute to the end of May (which also happens to be Cystic Fibrosis Awareness Month), I wanted to take some time to really explain what CF is, some common, though very reasonable, misunderstandings about it, and what it means to those of us who "suffer" (though I hate using that word) from it. This isn't intended to be a means to start feeling sorry for CFers- most of us are not fans of pity, but rather just a way to understand the details of what makes our lives different. If you know someone with CF but don't really "get" it, this will attempt give you a better idea- hopefully in a straight forward, no biology degree required, way. For my next post, I'll go over my own personal experiences with CF- that is, the specific manifestations,or symptoms that I have, how my health has changed throughout my life, and what my specific treatment regimen is like, day to day. I hope you all enjoy!
Cystic Fibrosis (or CF for short) is a recessive inherited disease, meaning you are born with it and have to get it from both parents. Now, to avoid getting WAY too scientific about it, CF is essentially caused by faulty genes that then cause a certain protein throughout the body to not work correctly and leads to thick mucus, and really salty sweat. It is described as a respiratory disease that affects the digestive system as well. But just like SO many illnesses, there is so much more to it than that. I hope to give you a short glimpse of what CF means to a “cyster” or “fibro” (what we call girls and guys with CF).
One common misconception is that CF is a “childhood” disease. That, sadly, was absolutely true years ago- when I was a child, CFers were hopeful to live to 18 years. However, thanks to amazing researchers, the average life expectancy of a CF patient in the US is currently around 37 years. For us, that’s a whole LIFE longer than it was 20 years ago and there are now many adults with CF.
Another common misconception about CF is that it is a JUST respiratory disease that also has a digestive component. While these are absolutely the primary life threatening issues, CF is a disease that affects our cells and as a result, our whole body. In addition to frequent lung infections, CF causes pancreatic insufficiency which leads to malnutrition and difficulty maintaining a healthy weight. I know some girls out there may think, “Must be nice!” and I can’t TELL you how many times I’ve heard that. Sure, I can eat a lot more than the average person- but it doesn’t come free. EVERY time I eat I have to take a handful of pills that do the job that my pancreas can’t. What if I don’t? Bloating, pain, and constant trips to the bathroom- not fun. In addition, frequently getting sick requires energy. Having no extra fat on your body means that you get really sick really fast. While those are the obvious symptoms of CF, there are many additional problems that patients deal with. We often suffer from CF related Diabetes, CF associated arthritis, liver scarring, enlarged spleen, serious sinus issues, possible heart complications, infertility, osteoporosis, dangerously low vitamin levels, and I’m sure many more that I don’t even know about. One problem is that we are constantly dosing our body with antibiotics to extend our lives and stay healthy, but those could ultimately be causing other problems that can worsen over time.
We do inhaled nebulizer treatments and “airway clearance” (parents or significant others will pound on our chests or we can use other instruments that vibrate the lungs to loosen the mucus so we can cough it out) 2- 3 times a day which can take several hours, we give ourselves shots and IV treatments, and we take multiple handfuls of pills multiple times a day, every day. We often get over tired, and frequent respiratory infections and coughing often causes a lot more pain than you can imagine. Hospitalizations are commonplace and familiar (how many 20-somethings have favorite nurses in various hospital wings?? I do!). We get weird looks and are asked “do you need some water?” when we have coughing fits. Don’t worry, we aren’t contagious and thanks, but water won’t help. We worry about things most people don’t have to, like where the closest bathroom is at all times, whether a public place has a lot of sick people present, getting home from social engagements with enough time to do our hour plus of treatments and still get enough sleep, figuring out how to spend weeks in the hospital with as little impact on work or school as possible, or waiting for someone else to lose their life in order to get a second chance at ours with a new pair of lungs. We HAVE to work hard, not to necessarily get healthier, but to stay as healthy as possible and prevent getting worse.
But as awful as this disease is, it gives me and others a different perspective on life that I wouldn’t trade for the world. I don’t WORRY about getting old; I see every year as another milestone. I enjoy every breath that I take and any day that I can breathe, can move around pain free, and have normal blood sugars is a great one. I celebrate a 5% increase in lung functions (I generally function at 50% lung capacity). I am lucky enough to have found my passion and career early on and get to come home to a wonderful man who adores me, CF and all, and enjoy our amazing fur babies (two 5 month old kittens) together, every day. Sure, I get down in the dumps sometimes, but what good is it to spend my days feeling sorry for myself- especially if those days are limited?
In the words of the inspirational Eva Markvoort, a cyster who devoted her life to spreading the word about CF, and tragically lost her battle at the young age of 25, “Cystic Fibrosis means drowning on the inside, it means learning how to access my own med port at age 13, it means closer family bonds, as everyone pulls together to get through the tough times. It means making jokes about beating your children, and salty girls tasting better. It means living my life not for the number of breaths I take, but for the number of moments that take my breath away.” Right now, there are amazing drugs in development and getting FDA approval that bring us a thousand times closer to that big, amazingly scary exciting C word that has always seemed so impossibly far off in the distance for us. Every day, we fight our battle, individually and as a community, so that we can be one day closer to the famous day that CF stands for Cure Found.