First off, Cystic Fibrosis is a genetic disease in which the lungs, pancreas, digestive system, heart, and sinuses are affected. You get one mutation from mom, one from dad. The two mutations "make" CF. My mutations are DF508 and 1717-1G>A. Mutation talk is for another time though.
Here is the cff.org's answer for "What is Cystic Fibrosis":
Cystic fibrosis is a life-threatening genetic disease that causes mucus to build up and clog some of the organs in the body, particularly the lungs and pancreas. When mucus clogs the lungs, it can make breathing very difficult. The thick mucus also causes bacteria (or germs) to get stuck in the airways, which causes inflammation (or swelling) and infections that lead to lung damage. Mucus also can block the digestive tract and pancreas. The mucus stops digestive enzymes from getting to the intestines. The body needs these enzymes to break down food, which provides important nutrients to help people grow and stay healthy. People with cystic fibrosis often need to replace these enzymes with medicine they take with their meals and snacks, which helps them digest food and get proper nutrition.
Just how exactly does CF affect the lungs and make all that nasty sticky mucus, you may ask. Well,
Normally, the healthy CF gene makes a protein—known as CFTR (Cystic Fibrosis conductance Transmembrane Regulator)—that is found in the cells that line various organs, like the lungs and the pancreas. This protein controls the movement of electrically charged particles, like chloride and sodium (components of salt) in and out of these cells.
When the protein is defective, as in cystic fibrosis, the salt balance in the body is disturbed. Because there is too little salt and water on the outside of the cells, the thin layer of mucus that helps keep the lungs free of germs becomes very thick and difficult to move. And because it is so hard to cough out, this mucus clogs the airways and leads to infections that damage the lungs.
This is how the lungs get full of that yucky stuff. Germs like, no, LOVE to settle in the mucus that will not come out. Treatments consisting of inhaled medicines (bronchodilators, antibiotics, and mucus thinners) and airway clearance techniques (the Vest, CPT) helps control the amount of mucus that stays in the lungs. Coughing and spitting out this mucus is the only way to get it out of the lungs. I know it sounds nasty and crude, but you know what? I really don't care. If it's going to help keep me alive and not sick, you better believe I'm a walking, talking, coughing, snotty, spitting fool! There are other medicines that I have to take to stay healthy, and they have nothing to do with breathing. I have to take enzymes (like the ones mentioned above) every single time I eat, I have to take special vitamins because my pancreas won't allow me to absorb any other kind, I have to take extra vitamin C to keep my immune system in tip top shape because it's so easy to catch ANYTHING. I have to take minerals like selenium. Iron (I'm anemic), fish oil, calcium, ibuprofen (for inflammation and also arthritis), omeprazole, just to name a few more and I'm sure I have missed a couple somewhere. All in all, I have to take approximately 50 pills a day and I spend approximately 6 hours doing treatments a day (8 if I don't feel well). Let's do the math here for a second. I have to sleep at least 10 hours or I don't feel good the next day. So- 24 hours in a day- 10 hours of sleep- 6 to 8 hours of treatments- And that leaves me only 6 to 8 hours for just me and what I WANT to do. This doesn't include the eating, coughing, or resting that I have to do everyday as well.
Got off track there for a minute. Back to the medicine. I was approved by the doctor and by my insurance to try Kalydeco (a new CF medicine) for a month to see if there will be any benefits. Kalydeco works by targeting the problem in the cell- the CFTR. I am not sure of how things will go, but I am hoping that they will help me feel even a teeny bit better. For a week I have been fighting a cold/allergies/sinus issues, and I am hoping that it will alleviate some of the pain I have there as well as in my lungs. Unfortunately, I don't have a home spirometer, so I will have to go by how I feel. I go back to the doctor on May the 8th to see how much of an actual change there will be.
As of my last pulmonary function test, my numbers are:
FVC- 39%
FEV1- 28%
FEV1/FVC- 75%
PEF- 53%
FEF2575- 13%
These aren't the best numbers at all. I am hope hope hoping with all my might that I can get them up at LEAST a couple of numbers. That would be such a blessing!
Well.... I am waiting on the couch, right beside the window for the delivery truck to get here!!
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