Okay, so the results of today's appointment:
I lost weight- but we think that is because I was so stressed out about graduation and finals. There wasn't much worry about that.
Xrays were good- in fact, there was one particular place on my lower lobe that was completely cleared out and that is something since it has been there forever it seems!
PFTs- From my previous appointment on February 21: FVC 34%, FEV1 22%, FEV25/75 8%, PEF 56%
PFTs- From today's appointment- FVC 47%, FEV1 32%, FEV25/75 11%, PEF 77%!!
Now, I did have a hospital admission and got released on March 11th, but my PFTs did not have any significant change from the admission PFTs from February 21st! My doctor was very pleasantly surprised to say the least. There has been no way I could get above 40% FVC on my own. We even looked back 6 months for more of a baseline, and this is even better than then (my FVC was 45%)! So- all in all, it was a VERY good appointment! I haven't been able to impress my doctor for a very long time!
I did 6 full PFTs and did not cough. I did 4 asthma tests and did not cough. It was awesome! I was so nervous after the PFTs because I "normally" hack my head off after the very first one. But this time, I went through 6 without any kind of coughing! I had to make myself cough just to get a sputum culture. Craziness. Pure craziness.
The doctor stopped my hypertonic saline. He stopped my Brovana. He stopped my Bactrim. He stopped my Tobi. Not even bi-monthly (or whatever) anymore. I have had 25 normal flora cultures, so the doctor completely took me off all antibiotics! Whaat?
I did lose 3 pounds, but... I think stress had a lot to do with that. Some people gain weight during stressful time, some lose. I am a loser. Lol! The doctor wasn't too concerned about it, and I am going to work to make it up for my August appointment!
Overall- I feel... wonderful. There is no way I want to even go back to how I was before. I can't begin to describe how much different I feel. Not only physically, but also mentally and emotionally. I know now that I can improve. That does wonders for my mind. I used to get so so discouraged when my lung function numbers would barely improve or not even move. I didn't think there was ever a time that I would get to see a 50% FVC anymore. Now I'm at 47%?! This just goes to show me that there is always hope. Always. I am just overwhelmed (in a good way) of how well today went. Really. There hasn't been that kind of improvement in my numbers in at least 4 years. I haven't felt this well in 4 years. 4 years. 4 years I have felt like there was no hope- that there wasn't any getting better, and I had to accept that I would continue to get worse, even though I didn't want to. Even by doing my medicines and treatments religiously, I could never just feel good. Now, I wake up feeling good. I get through the day feeling good. I go to bed feeling good. There is just no words for me to describe my happiness. My gratefulness...
I am so thankful to Rebecca, Mandy, Marci, Sandy (just to name a few) and others who had inspired me to push to get my doctor to write me a prescription. Even after he said no the first time, I continued with research, reading, and blog creeping enough to write him an email filled with articles and posts that finally convinced him to let me try Kalydeco. I am so glad that I didn't give up the first time. I just knew that I needed to try it- especially after I had read about similar mutations benefiting from it. I am just thankful....
. I think today proved a lot to me. The number one thing is to never give up. Even though I knew that deep down already. Never give up, and always have hope. I will continue with Kalydeco, and I have hopes that I will have improved even more in the upcoming months. I have hope!!!
Showing posts with label PFTs. Show all posts
Showing posts with label PFTs. Show all posts
Tuesday, May 8, 2012
Friday, April 6, 2012
The waiting game begins!
So today is the day that my medicine should be delivered! For those that aren't sure what I'm talking about, I'll give you a shortened version (if that's possible) of what is going on right now...
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:
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!!
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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