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!!!

Day 30 already?!

Wow, I guess today marks a month since I've been on Kalydeco. Wow. It does not seem like it has been that long! I am happy (and proud of myself) to say that I have not skipped even a single dose since I started! Yay! Lol. :)

I wished I had some new updates to post, but everything is about the same. There are only little things that I have noticed, but I can't be sure that it's not stemming from something besides the medicine.

I haven't exercised since the mile on the treadmill. I did something to my back and really hurt it! It hurt for almost 3 days straight. Then, I started to have pains in my left side- between my ribs and my hip. It hurts when I put pressure on the inside. Like when I squat down or bend over or things like that. It's not as bad today as it has been, though. It hurts when I move around at night, like to change sleeping positions. The night before last, I woke up in the middle of the night just drenched with sweat! I'm not sure what the deal was there, since I was in shorts and a tshirt and had my air conditioner on. But, I haven't done that again, so it was just a one time thing.

I have been feeling tired since... Wednesday I think. But- that is the day that I found for sure that I was able to graduate. I think that all the stress and tension I had about school just kind of went "whoooosh" and left. I think now I am tired from NOT stressing or worrying for once! I know it sounds crazy, but I honestly think that my body was used to being stressed out and now that I feel like a huge burden has been lifted, I am feeling just worn out! I feel like I could sleep for hours and hours on end!

The good news is that I am not really feeling CF bad. Which is something that I hardly ever say. Yes, I do have times where I am short of breath or am coughing, but my lungs don't feel bad. There are times when I just know that something is going on in my lungs that's not supposed to, and there are times that I feel great and then find out that something's not right. This being said, I have no idea how the doctor's appointment is going to go on Tuesday. I can see myself getting a great and awesome report and coming home for 3 months, but I can also see some unexpected infection pop up in there and need some "tuning up" for a couple of weeks. I don't know.

I am honestly nervous about how this appointment will go. More so than normal. Not because of the chance of a hospital stay, but because of the results of the PFTs and X-rays after a month on Kalydeco. I know that I feel different -better different- and I want so desperately for it to show on paper!! I just want to see some improvements! Oh, I want that so very badly... I guess we will just see. What happens, happens. Whatever will be, will be. That's become our motto.

So until Tuesday, I will be going ahead and preparing for a hospital stay, just in case. And I will update on Tuesday sometime after the visit to let you guys know what's going on...

Day 15- booooo

What the heck. I am still not feeling good. It's so aggravating. I don't know what's going on.

I don't feel congested, although I feel out of breath- still. I'm up to 4 treatments a day, and inhaler sometimes in between. I'm considering calling the doctor. I know a lot of you are probably going (you should have already gone, yadda yadda) but my doctor/hospital is almost 3 hours away, so I can't just take off and go on a moment's notice. Plus- gas prices don't help either. If I do have to have a tune-up, I can't do home IVs because it's so far away, and have to spend the WHOLE time at the hospital. So it's not as "easy" as you think to decide when to see the doctor. If I am not better by Monday, I'm calling to see if I need to make a trip. This would be majorly inconveniencing, as I am still doing homework and interning. But, what good is it to choose anything over my health?

My symptoms are:
Shortness of breath
Hard to catch my breath
Major headaches (almost migraines)
Fatigue
Wheezing
Feeling feverish

I have a scheduled appointment on May 8th...

Overwhelmed.

I'm feeling a little better this morning, and can say that I believe I'm on the uphill climb of getting better. I slept pretty good last night, although I do still feel sleepy this morning. As of right now (halfway through a treatment) I don't feel near as tight chested or wheezy as I have the past few days. I'm thinking that maybe I'm still tired because my immune system was fighting whatever was making me feel bad, and now it's recovering? Maybe? I don't know.

I was asked yesterday if I felt any different, and just how different. And I did not hesitate to say that I am breathing better. There is absolutely no doubt that I feel better in my lungs. Even if I go back to the doctor and there's no significant change in X-rays or PFTs, I KNOW that something is different. Period. No one can tell me anymore that Kalydeco won't make me feel better, because it 100% absolutely positively has. I am so thankful that I got to at least try it because I'm sure that it has changed me- even if it's just made me cough less strenuously. That alone was completely exhausting.

I am trying to finish my homework before graduation. If I don't get good grades, I won't get to graduate. And I'm afraid if I don't graduate now.... Well, I'm just afraid. I hate that because of CF, I had to take so much time off from school. I hate that! Now I am more than a month behind on assignments and they are due within the next week. It's so overwhelming. If I hadn't had to go in the hospital, I would be on track right now. It's stupid. I am working as hard right now as I can, going from one subject to the next, doing past lessons while trying to work on current ones, while also trying to finish my internship... It's frustrating and stressful to say the least. My poor little body is so stressed out! I am trying to get everything done, but it makes me so tired! My brain, my body, my everything. My muscles are sore from sitting at the computer and writing in notebooks. I'm just frustrated that this seems to happen right when I don't need it to. It really stinks that no matter how hard I seem to get to catch up, I'm still so far behind. It really does a number on my confidence.

Well since I can't actually write while doing my treatment, this post took up treatment time. Now that treatment time is done, I am going to back to work. Sigh. I so wished that I never would have had to go in the hospital again. I don't know hospital stays can't wait until nothing important is happening. But CF won't wait, and neither will graduation, so I'm off to get as much done today as I can. Sigh.

One week down, three to go.

It's been a week tonight since I started Kalydeco, and although I have felt better than I "normally" would, today wasn't such a nice day. It's actually the first time in the week that I haven't felt better than the day before. In fact, I feel worse- but I'm not back to feeling "normal" bad.

This morning at 7, I did a treatment and then ate breakfast, and just doing that I was tired. Not sleepy but not exhausted either. It's tough to really explain. I wasn't feeling good, but I wasn't feeling bad either. I just wasn't feeling like I had been, like maybe a few steps under. So after I did all the morning stuff, I decided to go lay back in bed for an hour or so. I got up close to noon because I was so tight chested and was wheezing so loudly! I did an albuterol breathing treatment, hoping that would help. It didn't really, and by 1 I was laying back down and resting. I wasn't really hungry when we sat down to eat because my stomach was hurting a little bit too. I still felt short of breath and almost felt like I could puke if I wanted to. By 5, I went to do another albuterol treatment/Vest and fell asleep after that until 7 something. I didn't feel like eating much with the 2nd dose of Kalydeco, but I ate a peanut butter sandwich and drank some juice.

It's 9:30pm, and I am doing another treatment right now, and if I still feel tight, I guess I will do more throughout the night. I will say that I feel like it is my asthma- not any kind of congestion or anything. I knew I had asthma, but it was hard to distinguish between what was an asthma wheeze and what is a CF wheeze. Especially when all I focused on was CF symptoms. I can tell that I don't feel as congested or crackly as I did a week ago, but I am feeling the symptoms of asthma.

One good thing is- I started allergy medicine today, so maybe that will give me some kind of relief. I may also start on an antibiotic, because I have been running a fever and my nasal discharge is a yucky color. That's about the only update I have today... Maybe this feeling will go away soon, and I will start feeling good again ASAP.

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!

Here we go!

Kalydeco got to me approximately at 6:50 this evening! I have already taken one dose with a spoonful of peanut butter (since you have to take it with fat containing foods).

We shall see how soon I feel any different- good or bad. I am nervous about all the coughing I have heard that it makes you do. I am looking forward to getting some of that old junk out, though!!

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!!