Today, I had yet another doctor's appointment. A few days ago, I went in to get my blood drawn so I can stay on a new medication that attacks the root of the Cystic Fibrosis problem. The doctors are inching towards a cure, and while they put me on these pills that are designed to change how my cells function in my body, they have to monitor me closely for health and research reasons. 

Cystic Fibrosis, as many now know, is a progressive disease. Its progression cannot be completely stopped yet, but it can be slowed significantly. However, the new pill I've been on since March is showing promising signs in both me and almost every CFer on it. Yet, CF is still progressing. While I've received great news today, there are some places of concern. 

I'll start with the bad. 

One of CF's main targets is my pancreas. I can't digest my food without medication, and while my pancreas produces insulin, it doesn't do it well. As I get older, the scarring on my pancreas caused by CF gets worse. While I've made significant diet changes in hopes of taking a lot of stress off my pancreas, CF related Diabetes (CFRD) is inevitable. However, CFRD isn't like the diabetes people are used to hearing about. In fact, CFRD is different in everyone, just like how CF is unique to everyone who has it. There's ways to avoid the needles associated with diabetes for the most part, so I'll be looking into those in the near future, and the symptoms in me are minimal. Even with my organic wild game, fruits, and vegetables diet, that lacks almost all processed junk foods, not even I can stop the progression of CF or the development of CFRD. However, I'm on a medication that is showing promising results with everything including my pancreas, and there is another medication that is much more effective in trials, so I'm not exactly worried. 

Now, on with the good stuff. 

There's a test I do every time I go to the doctor's called a Pulmonary Function Test (PFT). Basically, I blow into a machine that measures how well my lungs work. A 100% PFT is considered that of an average person. Anything higher is above average, and anything above 150% is considered significant. The higher the PFTs, the better the lungs. For the last two years, I've been stuck between 85% on my worst days, and 93% on my best days, and that was with another medication called Albuterol, which is an inhaler commonly used by people with Asthma to open up the airways. Those PFTs were also taken when the air wasn't smokey and the pollen wasn't a problem. 

So, naturally my doctors were ecstatic when I started blowing PFTs between 98-102% without Albuterol, or any other treatment for that matter except for what I do in the mornings. With this, they did a small exercise tolerance test, and I did very well with that too. I'm certainly a lot healthier than the vast majority of CFers my age, older, and younger, and my doctors said that's something to celebrate. They told me if I'm going to stay healthy and fit, I must push myself whenever I can, and they also recommended I threw out my PoS office chair in exchange for a yoga ball, so I can work out a few muscles while I'm just sitting down. 

While I haven't grown Pseudomonas in the last several months, my doctors still want me to go on at least one antibiotic every other month for a year, just to deter any possible chance of developing Pseudomonas again. This I'm not happy about. The inhaled antibiotic they want me to do dries up my insides and sticks to everything it touches. It's like breathing in watery maple syrup for 20 minutes, twice a day. Only, it tastes like the stuff I sprayed on my taxidermy so my dog wouldn't eat it (I only know this because the spray can wasn't exactly great and it sprayed most of the stuff in the air), which is very bitter and unpleasant. So, it looks like I'll be doing that six months out of the year, even though I haven't tested positive for Pseudomonas in a pretty long time. 

In order to understand the significance of this, you have to understand what Cystic Fibrosis usually means when someone is diagnosed with it. Cystic Fibrosis means death in a lot of ways. Most CFers my age are dead or close to death. Most CFers younger than me are dead or close to death. And only 5% of the world's CFers, which is only a few thousand people worldwide, are older than 40 years old, which is the current life expectancy for those with CF. My great uncle died as a toddler from the same exact gene of CF that I have, and I've met people who've lost loved ones to the disease. I've become a hero to a lot of those who've been affected by CF, since I don't give up and don't back down. 

People with CF, including me, have medication lists that are often a few pages long, and spend a lot of time doing treatments as well. I spend about an hour in the mornings treating my CF. This includes using a machine called the vest, which is an inflatable vest I wear that attaches to a machine, and that machine pumps air into the vest to shake my lungs. The shaking breaks up the gross stuff in my lungs so I can cough it out easier. 

While I'm doing the vest, I've also got a nebulizer, which just vaporizes liquid medication for me to breathe in. I have a few different medications in that including saline, which moisturizes my lungs to make breathing easier, Pulmozyme, which breaks down any and all infections in my lungs, and the Pseudomonas killing stuff (which is just called Tobi) that coats my lungs in a thick layer of antibiotic that kills mainly Pseudomonas infections, and prevents further infections. Only that makes breathing kinda hard and gross while I'm on it. 

These don't even scratch the surface. I take another 50 pills a day, which 20 of those are digestive medication so I can digest my food, two of those are the pills that attack the root of the CF issue, and the rest of them are vitamins and supplements. A few of those pills are chewable, which makes things a little bit easier, but not a lot. 

People often ask me how I keep track of everything. I honestly don't know. I just do. I hardly remember the names of my pills, but know them when I see them. Most of the time I just know my pills by the look. I memorize their colors, their shapes, their functions, and whether or not they have a taste. I especially remember them if they have some sort of effect, such as what happens if I forget to take them, or what happens when I take them.

A lot of people would think it would be catastrophic if I ran out of some of my pills, or went on a trip without them. Truth is, it's really not. I mean, it sucks sometimes, but it's not the end of everything. One time, when I was a little girl, my dad didn't bring enough enzymes (the digestive pills I take) to Minnesota. Luckily, the Mayo clinic is the best hospital as far as treating Cystic Fibrosis. Most major CF breakthroughs can be attributed to doctors at the Mayo clinic. So, dad just had to call my doctor, and my doctor called another doctor at the Mayo clinic who knew me, and a package with enough enzymes to last me a month arrived on my grandparents' doorstep a few hours later. 

However, some things aren't so easily replaced, such as my vest and nebulizer. 

It's already a huge pain in the ass to take those things on trips, especially airplane trips. First of all, the vest and nebulizer have a combined weight of 50 pounds. The TSA isn't too fond of medical equipment. Either they rummage through everything if I check it, or they rummage through everything with me standing next to it if I carry it on. But what happens if I carry those things on with me for two flights to London, England? Well, customs also rummaged through my stuff just to check it out. We made it to our hotel just fine, which was two blocks away from the Tower Bridge. We knew about how different the outlets are there, but in our sleep-deprived minds, we didn't realize just how it was different. 

A foundation called Make-A-Wish made the London trip possible. I kinda wished it on a whim since they called us out of the blue one day. My only best friend I ever had was really into Harry Potter, and while I've never liked Harry Potter, I wanted to fulfill my friend's wish to go to that museum while also fulfilling my wish to travel overseas. Plus, I was lazy and didn't really want to take the time learning some things in a new language. So, London it was. We had a blast except for the first day, when I blew up both my vest and nebulizer in the hotel room. 

My mom had the outlet converters right, but they weren't set to reduce the voltage. I was standing directly in front of my vest machine with my back to it, and I kept hearing a sharp ringing noise, but since no one else acknowledged it, I assumed it was just me. Suddenly, there was a pop that startled all of us, and smoke puffed out of the vent in the back of my vest machine. We all panicked in different ways! I rushed to force open the window, my best friend was already halfway down the hallway, my best friend's mom ran to the bathroom to start filling up the bathtub, and my mom was the brave one. She picked up my vest and threw it into the tub, which actually made everything wet except for the tub. 

And if that wasn't enough, my nebulizer, which was plugged into the same converter, also exploded a few moments later, and that also took a bath. I found and dragged my friend back into the room by her arm, after finding her standing in the lobby trying to act as natural as possible. My mom decided the best thing to do was send me with my friend and her mom out onto the morning streets of London, while she had some quiet time to figure out a plan.

While I was out, my mom called a few people, got a British nebulizer delivered to our room, and decided to just pound my back for ten minutes a night to simulate the vest. All in all, we were all good, nothing was allowed to catch on fire in the chaos, and I was given a temporary replacement vest at home while they fixed the one that blew up.

More recently, as of June 2018, my mom took me to Boston, Massachusetts for a leadership conference run by a friend she met a few years ago. I didn't like the city, but I loved the leadership conference and the people I met. We had purposefully left my vest at home, since it would only be a few days and I'd be moving around a lot in Boston. However, as soon as we made it to our hotel for the three day trip, my mom realized she actually forgot something very important! The medication I have that attacks the root of the CF issue had been left at home, along with my sinus rinses and my anxiety pills!

Of course, I was pissed! Back at home, I wanted to stuff those things in my backpack, but mom told me not to worry about it. So, we had a bit of a fight over it, which ultimately ended with my mom saying, "It's only a few days, you will be fine. Let's go get some dinner, and you can pick." 

My mom was right. I was fine, and I was also happy to eat. I didn't realize just how hungry I was until I devoured my Boston clam chowder and fish 'n chips in under 10 minutes, and I almost forgot what I was angry about. The pills still occasionally popped up in conversations with my mom, but she'd always say, "Well you're not dying, so I think you'll be fine." or something along those lines. And she was right.

There's humor in everything. A lot of those things weren't funny then, but they're hilarious now! Of course, I don't know of really anyone who can relate to my situations, whether it's planning in an extra 20 minutes to bicker with the TSA, or blowing up important medical equipment in a really nice hotel in a different country, but I bet everyone can imagine what they might do in situations like that. I'm sure I've had more mishaps like that, but chances are they're blocked memories. 

Physical activity is extremely important for those with CF, even though the majority of CFers avoid rigorous activity. I'm not one of those CFers. I participate in the hardest and most dangerous sports in the world, just to stress out my lungs and heart as much as possible. I'm adventurous, so I need action. I don't like just running, hiking, or biking. I like a purpose, and I like adrenaline. 

Paintball is a game that makes me run and gives me a few very good reasons on why I need to be running. In paintball, you've gotta be faster than a paintball traveling 300 feet per second! Sounds impossible, but it's possible. I always wear knee and elbow guards since I dive and skid behind bunkers to avoid getting shot. I'll also wear hoodies, long sleeve motocross jerseys, gloves, a backwards cap, and a bandanna under my paintball mask, since getting shot on bare skin sucks! But, even though it hurts, I still play and it's probably one of the best things I can do for myself. Paintball is a lot of hiding, but it's also a lot of running, and to play it well you need to use your head. 

My most memorable game was almost two years ago now, and I was the last person defending the fort in the Alamo scenario. There were around 40 people on each side at the beginning of the game, but 20 minutes in, everyone except me was attacking the Alamo, and the refs were pointing me out to my enemies. One ref even joined in on the fun, so I was about to get "lit" by 80 people, which in paintball, getting lit in the game isn't supposed to be fun. Getting lit means I was gonna get at least ten people shooting me from 5 feet away, and 70 others shooting at me from a distance. Paintballs hurt so much, that players are encouraged to surrender if the shooter is less than 15 feet away, and I was encouraged more and more by a ref to surrender as my attackers crept closer. 

I had 250 paintballs loaded and ready to go, and just as the barrel of the first enemy's paintball gun came into view, I started wildly shooting! I got about five people out before another ten came in from all sides and all shot at me at once. I begged for them to stop, and when they did, I was soaked in green and pink paint. I wasn't in pain. I was actually laughing, and I've since fell in love with paintball. However, I couldn't sit down properly for about a week. 

I'm probably as healthy as I am, because unlike what parents of CFers are typically advised to do, my parents never held me back. In fact, they encouraged me to play and be athletic, and as I got older, they kept me active in the hardest sports available, including Tae Kwon Do, horseback riding, motocross, paintball, and hunting. I was active in swimming until the water tried to kill me, but even then, I still play in the ocean as much as I can on vacation. 

The activity brings up the gross stuff out of my lungs and body, and keeps me strong and fit. Of course, this is great, but it really brings up the grocery bill even higher than it already usually is. It's not unusual for me to raid the fridge after a long day, and devour two pounds of burger and a whole batch of bananas. My mom will sometimes give me "the look", but she's never angry about it. She just can't believe my 125 pound skinny ass can eat all that and still be a stick! And sometimes, neither can I. 

There are perks to having Cystic Fibrosis, such as the ability to eat whenever and whatever I want and not have to worry about getting fat. Of course, I do have to worry about getting malnourished, which is why I eat so much. Not many CFers have my appetite. In fact, most people with Cystic Fibrosis hardly have an appetite at all, and resort to G-tubes (tubes that pump nutrition directly into the stomach) to get most of their calories. Others are prescribed weed brownies, which keeps whoever eats them hungry. I'm glad I don't have to get surgery or get high to stay at a good weight. I just have to stay active, and then I'll come home to clean out the fridge for the day. 

But with CF, even eating is hard. Not only do I have to pack on the calories, but every meal requires 4 to 5 pills to digest it, and they're each a pretty good sized pill. I've learned how to take a handful of those pills all at once just fine, but occasionally one will go down wrong, and scrape the sides of my throat all the way down. So, if I'm ever eating and just suddenly pause and stop talking, that's why. Plus, I carry those pills around wherever I go, and they're made so you can open them up for little kids. Well, those pills like to open up and spill all over the inside of my wallet. Then I have to clear out all the crap I stash in my wallet to shake out the medication, because if it gets wet and it's in my wallet, it will literally digest anything less durable than a coin. It's happened before (Rest in peace 25$ Amazon gift card I never got to use). I've since learned my lesson, so all of my important stuff is in a plastic case inside my wallet, just in case my wallet gets wet. 

I have to watch my diet too. Anything that is too rich and/or too artificial doesn't work well with me. My pills only digest 50% of a meal, and if that meal is full of fat, carbs, dairy, sugar, etc, I will get sick for a few hours. So, I've learned that organic fruits and vegetables alongside a lean steak or two, is the best meal I could ever have. It gives me all that I need without the negative side effects. I'll have an occasional cheat day if my day will be relaxing, but I've been having less and less of those cheat days, especially as I prepare myself for hunting season. 

Honestly, this article merely scratches the surface of what I do to stay alive and well. There's a lot that I do that I struggle even more to describe. Fighting CF is more than just pills, exercise, and the right diet. It's more than just taking each day one breath at a time, and forgiving myself if I'm not quite as well or capable as I usually am. It's a lot of finding the good in things, even if it seems there's nothing good to find. It's definitely a lot of prayer and trusting God, since God has done miracles to get me out of inevitable situations. I can't thank Him enough for that.

I have to be pretty intelligent and brave to figure out the best path to go on, since I often find myself buried in scientific research and studies to find out what usually works and what usually doesn't. Sometimes, science betrays me, and I then have to completely go off of what does what to me by trial and error. Sometimes it takes weeks or months to figure out what works and what doesn't, since there aren't credible studies for everything, especially for Cystic Fibrosis. I'm just glad I have plenty of room to play around in. 

Sometimes my kitchen counter turns into some sort of redneck chemistry lab, and whatever I come up with goes into my body. My mom thinks is pretty sketchy since I barely made it past Chemistry in school, but I don't go off of formulas and diagrams. I go by what science says something does, and just check reliable resources online to make sure whatever new that goes into my body, won't negatively interact with something else I take. If I mess up, it shouldn't hurt me too bad, since it's all natural stuff. But still, I've had some incidents in the past. I've learned that washing my sinuses with Thyme is basically like snorting wasabi up there, and the pain doesn't go away for hours. I've also learned that a shot of eucalyptus oil doesn't do much when I drink it, except for it leaves behind a bitter taste that stays in the back of my throat all day. 

However, I've had some breakthroughs, which have worked to keep me as healthy as I am. I don't usually recommend my lifestyle to anyone else, since my body is much different than everyone else's. What works for one doesn't always work for another, so I just document what I'm doing since I know everyone is curious about what I do. That's why I took the time to write about my daily life in the first place. 

Just like anyone who comes across this, I'm intensely curious and like to learn. Only, most of my learning experiences come from experiments done on myself, by myself. People may say that's stupid and crazy, but I don't just blindly try something new. I must know it's almost entirely safe before I put something on or in my body. Sure, things are bound to go wrong sometimes, but that's just life. And, as far as I know, I can recover pretty quickly and even laugh about it. I'm not afraid of pain nearly as much as I'm afraid of illness and an early death. I'd much rather run around the house snorting like a bull for a few hours, than be curled up in some hospital bed in a lot worse pain than Thyme up the nose. 

(Finish with living with CF, not dying bc of it)