Never give up and always have hope!

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!!!

Feeling down today...

Still not feeling very good.. But, I have heard that this is to be expected within this period of the medicine schedule. Feeling worse for a while is worth feeling better for a longer time, I guess. Hopefully anywhere between a 3-10 day span, I will get as much nasty, bad, yucky CF junk out so that I can feel better than I have been in a long time. I just want to get back and do what I was enjoying doing within the first week (like getting out of the house, and going to work, and shopping, and crafting). But- I just have to feel worse before I can feel better right now. Although it's hard not to get discouraged, I still have faith that I can feel better, and that it will happen. Hopefully sometime soon.

Most of the things I have been feeling (sick-wise) are being short of breath, lack of energy, sleepy, and just all around tired. I will say that am not back to breathing and coughing like I was before starting. I can absolutely tell a change in how I am breathing, how deep I am breathing, and how I don't have my breath catch because of a cough or crackle. And while all of this is how I am now, I shake my head at how different things could have been.

I can feel how bad my lungs are now, though. Like when I try to take a deep breath, yes it is deeper than it used to be, but- I still feel like I could breathe in more. Deeper. Kind of like I used to could be able to breathe years ago. It seems like so long ago, but then again it doesn't. I get really sad when I think about it. And mad. And frustrated with myself. And life. And CF. Then I think of how unfair CF really is, and that makes me even more sad, and mad, and frustrated. I think of how just 5 short/long years ago, I could run and have no problems at all. I could play a full basketball game and not even feel short of breath. I could keep up with everyone else and I could breathe, and I had muscle, and I could do difficult things with no problems!

And then within the span of a month, I am where I am now. I can't even think about running because I get tired. I can barely SIT and WATCH a basketball game without feeling like I can't breathe well. I am back down to the lowest weight I have been since before high school. I can't do simple things like doing laundry without being so short of breath and having to sit down. It's stupid and it's unfair and it's frustrating and it's aggravating and it's.... just not fair. Yeah, I know "life isn't fair"... yeah I kind of got that. But until something like this happens to you, you just can't tell me that. Because obviously I know.

CF is stupid and I hate it. Not because of how it effects me now, but how it completely just.... broke me. I'm broken. I don't know if I can ever be fixed like I was, either.


And it's not fair.

I just want to feel better. Like I was. I am praying for that. I am so ready to say I feel good again. Not "okay" or "fine" or "eh" or "hanging in there". I want to be able to say "good" for once.

Day 2

Today is the 2nd day, and I don't really feel any different. I will say that after last night's dose, I felt a little dizzy about an hour after I took it. It wasn't unbearable though. It's like the dizziness that Diflucan (fluconazole) gives me when I am on it. So- not a big deal to me. When I woke up this morning, I had a bit of a sore throat. Again, it wasn't terrible or unbearable. I can't be 100% sure that it was from the Kalydeco though. Like I said in one of the previous posts- I have been battling a cold or something and it could have been from "mouth-breathing" (I am a nose breather when I'm not stuffy) through out the night, or even some post nasal drip. After getting something cool to drink and blowing my nose, it went away. When I went to do my morning treatment, I didn't cough more or less than usual. It was all the same. I have noticed that I have to blow my nose quite a bit more though. I got a LOT of junk out from the time I woke up to the time I finished the treatment. Don't know if that was the medicine or not, either, so please don't think I am trying to say it is or it isn't. It's just what happened.

I took my 2nd dose around 8:30am, and think that I will stick to an 8:30am/pm schedule. That way I can take it before I leave for work, and then at night before a night treatment.

I haven't had any headaches, fever, or pain (yet) so as of right now, I don't even feel like I have taken anything. From some of my other Cysters' experience with it, they said that they didn't feel a full effect from the medicine until the 3 or so dose. So..... I guess we will see how I feel tomorrow after tonight's dose! I am feeling a little tired, but that really is nothing new. I am always, always, always tired. I am hopeful that Kalydeco will give me just a teensy bit of energy to do things I need to, and so I won't get tired so quickly doing small things.

I have to catch up on all my school homework, so I am going to do that. I will try not to forget to take notice and post if there are any kind of changes at all. Okay, as I finished that last sentence-

I just started coughing, very productive and wet. I coughed up approximately 1/4 c of mucus at one time. Whoa. I also just blew my nose and got out a bunch of snot/mucus too. It wasn't too yucky looking, about the same as when I just regularly cough, so I'm thinking that is just the top of the pile down there. I want to start exercising more- like running or something fairly... I can't think of the word... just more. But I don't have the energy yet, or the breath yet. Maybe by Monday I can start doing that!

I also want to keep track to see if my weight has gone up, so I weighed this morning. If I tell you, don't judge, remember?! It was 69.4. Okay, okay, pick your jaw up and stop shaking your head. I can't help it. Remember this is my page, and if you want to judge or criticize, then get off. I am posting this as a way to help me know where I started and where I end up. I will try to weigh every 3 days or so, and see if there will be any noticeable changes. I guess that is all the updates I have for now, and maybe I will have something good to post about next time!

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:

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!!