What is CF?

So on my About page, I said that I have Cystic Fibrosis. I’d be willing to bet that you have never heard of it.  It affects about 30,00 people in the United States and 70,000 worldwide.  It is a genetic disease that is passed down from both parents.  Cystic Fibrosis, or CF for short, causes thick sticky mucus throughout the entire body.  This causes tons of problems, but it is known as a lung disease.  The mucus in the lungs traps bacteria, making the lungs a breeding ground for infection.  I am constantly being hospitalized for pneumonia.   In the past, I was hospitalized for pneumonia and other complications eight times.  And those stays were anywhere from two days to three weeks.

But aside from the constant lung infections, there are other problems.  The biggest one I have to deal with is my pancreas not working properly. It does not release the digestive enzymes like it is supposed to. Therefore, my body is not able to get all of the benefits of the food that I take in.  Of course, I take supplemental enzymes every time I eat.  Six pills per meal, to be exact.  Even with those, my body does not get everything it needs.  On top of all of that, my body needs about 6,000 calories a day to function.  I have such a high metabolism because my body is constantly working overtime using all of the energy it gets to focus on breathing.  You read that right.  I need three times the amount of calories as a normal person just to breathe.

But remember, enzymes are not the only thing that your pancreas produces.  It also produces the insulin your body needs to process the sugars that are put into it.  The CF prevents the insulin from leaving the pancreas and getting into my system.  So I have          Type III Diabetes too.

My medical history, you could say, is a walk in the park.  If, instead of a park, it was hell.  Across Lego bricks barefoot.  My first surgery was when I was four; it was a sinus scrape, as well as adenoids removal to treat my snoring.  When I was 11, I had a case of Intussusception.  What that is, is when your intestine decides it wants to telescope into itself. Mine was my small intestine because a blockage had formed.  After drinking the solution they use to clean you out for colonoscopies, they decided that the only thing they could do to treat it was emergency surgery.  They took out six inches of my intestine, and my appendix just because they were in there and didn’t want problems later.  Luckily, the doctor that was looking at my ct scan was able to identify it because he had learned about it in medical school.  The hospital had to fly in a specialist from Alabama children’s hospital to do the procedure.  My case was special because like I stated earlier, I was 11.  It is typically diagnosed in infants up to 18 months old; it also is more common in boys.  It is the cause of intestinal blockages in less than 1% of adults.  It also only occurs in 1% of patients with Cystic Fibrosis.

In the past couple of years, my health had decreased drastically. While I was in high school, the CF never stopped me from doing anything.  I was in every student organization possible, including the robotics team and the marching band.  I marched while playing the instrument that takes the most air, the piccolo.  I even did a short stint spinning flag; even though I was only four feet and change, I spun six-foot flags with ease and was one of the best on the squad, even making the weapons line before things went south with scheduling and the robotics team.  But as time went on, things got harder.  Started to take a toll on my body.  By the end of my senior year, I couldn’t keep up with everyone else.  I still went off to college like wanted to, but I had to leave at the very beginning of the second semester.

My lung function was way down, and I was missing too much school to keep up with.  I had a port placed while I was off at school, and of course with my luck, there were complications.  The catheter tube inside that ran over my heart was cut too long, and it caused a blood clot to form.  Originally it was diagnosed as an Atrial Mixoma, which is a benign tumor in the upper left or right chamber in the heart.  I decided I needed to leave school then.  When I got home and got a second opinion, I found out that it was a blood clot and not a tumor, and that it was a common thing that the doctor sees often with his CF patients.   Unfortunatly, the cardiologist decided that the port needed to come out and did a procedure meant to suck the blood clot out. The pulmonologist, or lung doctor, was not consulted in this decision and did not approve of this procedure.  He wanted the port to stay in and me to just take a blood thinner.  Not only that, but the procedure was not even successful in getting the blood clot out.  And the doctor let me lose a ton of blood before deciding that I needed a transfusion.  After that, I was in and out of the hospital for what seemed to be every six weeks.  I just could not shake the infections.  I aquired some dangerous bugs that I cannot get rid of, and could prevent me from getting a lung transplant in the future.  Luckily, the hospital that I go to decided that I still qualified, that I wasn’t sick enough.  That April, I had been treated with a new antibiotic.  The next month I was in the hospital for almost a month.  The infection did not clear, and the infection was in my blood as well.  We couldn’t figure out what was causing the infection, adn why I was consistently having 102 and 103 degree fevers for over a week.  I went through a cycle of having chills and being sick to my stomach, to the fever breaking with me sweating it all out and being ravenous, to going back to another fever a few hours later.  I was exceeding my daily limit for tyelnol safe for my liver.  The doctors took extreme precautions by giving me three heavy hitting antibiotics at once.  Luckily once those had been in my body for a few days, the infection finally started to break.

That episode in the hospital took a huge toll on my body.  I was down to about 70 pounds.  My blood sugars had been extremely out of control.  The doctor decided that something had to be done.  So I got a feeding tube placed, another port in my left arm, and talk of a lung transplant arose.  Since I was there, they decided to do the workup at that time. There were tons of tests and exams, as well as being educated about the whole process.  When  I left the hospital, I had two goals: gain weight, and stay out of the hospital for a reasonable amount of time.  I was told when I did that, I would be considered for transplant.  Armed with my new equipment (my new feeding tube, a port for at-home antibiotics, and oxygen for at home and portable use), I set out to get ready for the biggest and scariest event of my life- getting new lungs.  Having the port again made it easier to stay out of the hospital and prevent me from picking up even more infection from the hospital. But with the antibiotics, and nightly tube feeds, my blood sugars only got worse.  At one point, it was over 600.  That’s close to going into a diabetic coma.  I should have died.

I had been trying to get an insulin pump forabout a year, but I still didn’t have it.  In August, my new Omnipod came in, as well as a Dexcom G6.  With these tools, I have been able to control my diabetes so much better.  The pump prevents me from having to get 6-10 shots a day, and the dexcom allows me to constantly moniter my blood sugar without pricking my fingers.  It uses an implanted sensor to give a blood sugar reading every 5 minutes.  Having my sugars controlled caused my health to improve almost instantly.  I gained 15 pounds in a month and a half.  I stayed out of the hospital enough for the doctors to approve my listing for a double lung transplant.  It took almost two months for the insurance claimes to go through, but it was official December 20. Now its a waiting game.  I could get the call at any time to get new lungs.  I’d high tail it to Ochner’s in New Orleans, to be on the operating table eight hours later.

So with all of this, I am sure you can tell that Cystic Fibrosis is nothing to mess around with.  It is a chronic, terminal illness.  That means I will always have it, and one day it will kill me.  But that doesn’t mean I’ve let it stop me from living my life.  I just need someone to carry me up the stairs, and I can’t go out partying and drinking like my friends do.  But Cystic Fibrosis has never defined my life, and that won’t start now.  If anything, it has helped me to appreciate every minute of my life I can get.

Articles that were written about me and my fundraiser:

http://www.katc.com/story/33632916/pokmon-event-to-raise-funds-for-double-lung-transplant-patient#.WBzT07qoZa8.facebook

http://www.theadvertiser.com/story/news/local/2016/11/04/pokmon-go-may-help-me-breathe-get-new-life/93297742/

Where you can get more information about Cystic Fibrosis:

https://www.cff.org/

The song “I Lived” by One Republic was written about a boy with CF and with CF in mind.  I highly reccomend you check out the music video.  It’s great and brings to light our lives

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