CF Awareness: "but you don't look sick"

When people discover that I have Cystic Fibrosis it goes one of two ways. 

One: they get real quiet, begin slightly nodding along as I talk, then quickly dismiss themselves at the sound of the first cough that passes my lips, running away in blind fear of something they know nothing about. 

Or two: the reveal is followed by wide eyes, a dropped jaw, and a chorus of "by golly, I would've never known" all tied together pretty with a "but you don't look sick."

 

me and my little sis, Natalie on Christmas 2014

 

Meanwhile, I'm praising the Lord that more often than not, the latter response is what occurs. Then I pull the file from my memory, labeled "CF 101" and dazzle them in all my infinite wisdom, dashed with a little wit, and we both walk away at ease, in complete understanding, to live happily ever after! Amen! 

Noooooooot! I usually answer the few questions they're bold enough to ask (they'll go home and Google the rest) leaving them in a state of confusion the size of Texas. 

So first things first, what is Cystic Fibrosis?:

Cystic Fibrosis is a life-threatening, genetic (so no, you can't "catch the CF") disease that affects many parts of the body, but mainly the lungs and digestive system. 

People with CF inherit (there it is again, still not contagious) a defective gene, from both parents, that causes their body to produce a lot of thick, sticky mucus in the lungs, pancreas, and other organs. 

This mucus clogs the airways of the lungs making it extremely difficult to breathe. Go grab a straw. Got it? Now put it in your mouth and hold your nose. Breathe in and out through the straw for a bit. Is it uncomfortable? Feeling a little woozy? Imagine every breath feeling this way. It would make a lot of things tougher, wouldn't it? This is how people with CF feel daily, with each breath. It's tough to breathe through such a barrier. 

Ever wonder why I always have that persistent cough that I just-can't-seem-to-kick? This is why! Coughing is sort of like my body's natural defense mechanism against all the mucus threatening to completely block my airways. Each cough rattles my airways, dislodging and moving that mucus around and out of my body. This air way clearance aids in preventing lung infections that lead to permanent lung damage. While coughing is usually a universal sign of sick for you, it is actually a (frustratingly annoying) help for me. 

Not only does the thick mucus restrict airways, it also traps bacteria in the lungs, resulting in reoccurring infection and inflammation. These infections lead to severe lung damage and, inevitably, to respiratory failure. Yes, you read that correctly: failure. While there are treatments and prevention methods there is no cure for Cystic Fibrosis.  

On the left you will see a typical chest x-ray, while on the right you will see the typical chest x-ray of a person fighting Cystic Fibrosis. All the black is air, as you can see there is considerably less black in the x-ray on the right. Instead you can see all the airways blocked with mucus, inflammation, and infection.

 

While respiratory issues are the most common symptom battled by people fighting CF, it is not the only one. The pancreas is also greatly affected by the buildup of thick, sticky mucus. It prevents the release of digestive enzymes that help break down food and absorb nutrients, causing malnutrition and poor growth (which explains the ever popular 'compliment:' "you're tiny").

You may have noticed me taking medicine each time I eat. These are pancreatic enzymes that will break down my food for digestion, just like the ones in your body do for you. Without these my body can not absorb any nutrients from the food I eat. Even with enzymes it is hard for my body to absorb certain nutrients, causing issues with the small intestine, like extreme bloating (makes six pack abs nothing more than a distant dream), excessive gas and severe abdominal pain. 

The mucus in the pancreas also affects the release of insulin, causing many adults with CF to develop Cystic Fibrosis Related Diabetes (CFRD). It is not Type 1. It is not Type 2. It is in a league all of its own. 

CF adult care is still developing, because until recently people with CF didn't live to or through adulthood. Which means more facets of the disease are being discovered as people grow older with CF. CFRD is one of these discoveries. Right now, my understanding is that the pancreas is blocked with too much sludge causing it to quit producing insulin. Therefore, this type of diabetes can not be managed by pills or diet changes, just the injection of insulin. 
 

Chronic sinusitis also goes hand-in-hand with Cystic Fibrosis. It's basically an all inclusive membership to the stuffy, sneezy, snotty, sinus infection club for life! *and the crowd goes wild* because of this, thick mucus fills each of the sinus cavities, blocking airways and trapping infection, causing: nasal polyps, head aches, migraines, loads of pressure, and occasional vision and hearing loss. 

Cystic Fibrosis is a super patient specific disease-meaning it affects every single person differently. While there are similar symptoms, severity of each symptom varies depending on the person + their specific gene mutation. Natalie and I have the exact same gene mutation: Double Delta F508. However, it looks a lot different on me than it does on her. I, personally, consider sinus infections my biggest feat with CF, while some with CF don't have a single sinus issue. The majority of my lung infections start with a sinus infection..that turns to an upper respiratory infection, that then so kindly settles into my lungs as a full blown "CF Exacerbation" (we'll talk more details on that later).

CF also affects random parts of the body, like skin. You might've seen the new "salty girls" campaign that buzzfeed recently published. It's pictures of girls confidently displaying the reality of living with Cystic Fibrosis. Sooooo why are they called "salty?" Malfunctioning sweat glands, which cause perspiration to contain excessive salt. Basically, we sweat salt. With this being said, please suppress all desires to lick me in the middle of a workout-or ever, at all. 

Working out at CrossFit Alabaster

 

This was a huge issue for me when I began crossfit. I was sweating out all of my salt stores, causing extreme fatigue and light headedness. I was sentenced to at least three gatorades a day + had to cut my intake of h2o; because I was flushing all the salt out of my body. This is a habit you'll probably see me pick back up as the Alabama summer heat rolls in. Purple drank for the win! 

  

Cystic Fibrosis also affects your liver, makes you more prone to osteoporosis, the medicines affect your kidneys, it affects your energy level, relationships, daily schedule, school, work, and the list goes on. 

So, no, I don't look sick, but what you can't see is my body constantly fighting a battle with each breath, just to keep breathing. 

 

 

May: Cystic Fibrosis Awareness Month

May is Cystic Fibrosis Awareness month! This May I've decided to take a little leap out of my comfort zone and get a little uncomfortably close with everyone on the internet! *cue the Jaws theme music*

My little sis, Natalie, and I were both diagnosed with Cystic Fibrosis (CF) at a young age. Growing up fighting this disease has been quite the roller coaster ride for us, our family, + our friends. However, it was recently brought to my attention that even those closest to me don't know a lot about how CF affects our lives, because to you we "don't look sick." And for that we are immensely thankful! Because who would want to walk around under a "sick little girl" cloud?! Not me!

Natalie and I enjoying a day at Noccalula Falls

 

 

Through a series of blogs I hope to help my friends, family, and the general public understand more about the life threatening disease that we battle every day. It is my desire to shed light on some of the questions you have (googled), but are too afraid (or polite) to ask. 

With that being said, there is nothing I would love more than to be able to answer those questions for you. So please, I'm begging you to, please ask away! In the comments, via text, email, carrier pigeon, or when you see me. 

Here's to raising awareness! The first step in making "CF" stand for "Cure Found!"

Fight for your soul

[Preface: I'm seven days out of, what I hope to be, the last set of sinus surgeries of my life. Twenty-two (ish) should be just enough for this girl! Second surgery coming up quick- in three days. And I feel like a balloon of sorts, at least from the neck up. You'd be amazed at the things they can not only stuff IN your nose, but then leave there for NINE entire days! However, I'll spare you the nitty gritty.]

My head hurts-  really bad right now. My body is in a constant state of discomfort-my lips are irritatingly chapped and my mind is fixated on a particular person, for some strange reason. My nose feels like the pressure behind it could blow it (+my teeth) right off of my face at any given moment. Yet, all around me, everything is still + quiet. 

It's times like these..

The ones where you just want to stop and catch your breath, but you can't-because of this, because of that- because of life! Ok!.. You just can't. 

Then for just a moment you realize you're the only one spinning out..- almost out of control-but you aren't alone. 

Because God is near. 

And tonight He reminded me of His sweet promises to us, written in Isaiah 41:10, "fear not, for I am with you; be not dismayed, for I am your God; I will strengthen you, I will help you, I will uphold you with my righteous right hand."

It's in these times when such sweet words penetrate my heart, ease my mind, and calm my restless soul. And give me hope, as they remind me that I am deeply loved. 

It's times like these that I see the importance of fighting- for my life, because of my soul. 

Praise the Lord, He is already the victor! Therefore our souls have been won! But that doesn't mean the war is over. There's still life to live here, on this Earth and because our souls have been won we must fight for our joy in this life.  

Constantly being on guard against the devil's sneaky attempts to distract us from the truth. The truth that God is good + in control, even when we do not understand. The truth that God cares deeply for his children, even when we do not "feel" it. 

Now the devil's attacks are never so straight forward, but sneak in through the feelings of loneliness + distance produced from trying to recover from surgery. Then when not addressed with truth, will undoubted snowball into a huge mess of hurt + frustration. 

So yes, God sees you here..- sitting in your parents bathroom floor, fighting to bear the pain of constant discomfort + the physical exhaustion of, yet another, sleepless night and he is NEAR. 

And in this moment he is calling you close-reminding you that, because your soul is His, He promises to sustain you.  (Isaiah 46:4)

Believing that God is true to His character and faithful to His word, I'm fighting against the enemy's attack to wreak havoc + rob me of my joy in this life by asking that God produce in me steadfastness for this storm, which He allows and promises to carry me through. 

Please bring glory to You, that this fight might not be in vain, amen. 

There's a fight for your soul-don't be a by stander.