New Game Plan?

I went into today's clinic appointment full of anxiety and apprehension. I felt this way for multiple reasons. The biggest was the fact that I have had multiple trips to Vandy that didn't show desirable results. My lung function has been on the decline despite all of my efforts with breathing treatments and trips to the gym. Honestly, I was beginning to believe that I had reached the limit of my lungs and that I would only lose lung function in the future. It has truly been just as much an emotional struggle as it was a physical struggle to keep all of the hard work up. I just kept telling myself that even if I wasn't seeing what I was hoping for, I was feeling pretty good.

On top of that, I have been dealing with some pretty random and very painful joint pains. They hit a joint for a day or two making my trainer have to rethink my workouts to avoid the effected area. It has been frustrating to say the least. It has affected my ability to push myself in my workouts which could definitely have a negative effect on my lung function.

The cherry on top of my worries was the weather. Temperatures in Tennessee have been on quite a roller coaster. We saw highs in the 50's and 60's last week and then had a 30 degree drop this week. When there is a drop like that, my lungs seem to close up. They become tight, and it makes it much more difficult to breathe.

So that brings us to today. I went in expecting my lung function to be the same or lower than last time, but I was in for a surprise. I did my PFT three times like usual. My third time was my highest, and while it definitely was not the highest I have ever had, it was higher than October's. I went from 42% on October 21 to 44% today! I am ecstatic! To add to that, I also gained a few pounds which always puts a smile on my doctors' faces. The only negative from the visit is that I still don't have any answers about the joint pain.

What's the game plan now? In the words of Dory, "Just keep swimming! Just keep swimming!" I'm going to keep pushing through and taking things one day at a time. I'm on day 771 of being compliant with my treatments, and I'm working out with my trainer four to five times a week. Fingers crossed that I keep seeing an upward trend, but if not, at least I'm feeling good and enjoying my life!

2016 Goals

Normally, I write a New Year's post January 1st. It's a post that focuses on how the previous year went and the goals for the new year. The problem is that I've been struggling to figure out my feeling about 2015 and where I want to go from here.

2015 was difficult for me. The year started off strong, and I finally started to see my lung function improve a little. I continued with my treatment compliance and my workouts. About halfway through the year, things started to change. I had my first lung bleed, had my wisdom teeth taken out, and my lungs just had troubles dealing. My lung function dropped, and has been lower than I would like ever since. I have worked incredibly hard, and my lungs have fallen into a pattern. They feel great for a couple weeks, and then they will hurt and feel terrible for a week or so. I have felt like I've been getting nowhere with them and with my health. To top it all off, I started 2016 by getting a respiratory virus... yay me...

The second half of the year wasn't all bad though. I never stopped keeping up with my treatments, and December 31st marked two years of being compliant! I didn't give up on working out either. We have been working on my lungs in almost every workout, and I hit 210 pounds on dead lifts! I also started taking Orkambi, but the jury is still out on if I have seen benefits from it.

So that brings us to my 2016 goals. What am I going to work towards this year? What am I going to do to reach those goals?

1. Make it to three years of 100% compliance with breathing treatments.
2. Stick to my workouts (continue to get stronger and work harder).
3. Work on my nutrition (find a balance between eating healthy and keeping weight on).
4. Stay positive (no matter what, I will keep pushing and fighting. When times get tough, I'm going to keep going).

Here's to a new year, new opportunities, and new goals. Happy 2016!

Small Hiccups Are Part of the Battle

Today was my second attempt at a clinic appointment, since September 16th didn't quite work out and ended up with my car being smashed and me not actually making it to Vanderbilt. I went into today's appointment knowing that my lung function would not be as high as I would like it to be. I have been feeling more congested the past few days and really the last couple weeks.

We made it there in one piece this time. I went into the pulmonary function test expecting the worst. Even though I blew with all that I had, I just couldn't muster what I usually can. My FEV1 was 42% all three attempts. That's down from 47% in June. My concerns were confirmed. My lungs are struggling right now. The good news is that my weight remained virtually the same which indicates that I'm not sick sick... just a little low in my numbers.

My doctor and I discussed how I have been feeling the past few weeks, and she was not overly concerned with the drop in lung function. She felt it could be from allergies or just a small flare up from the MRSA in my lungs. We decided that the best course of action would be to do a round of some strong oral antibiotics paired with steroids to hopefully give me a little kick start so that I can start feeling like myself again.

She was very complimentary of my commitment to compliance with my treatments. She was very pleased with my overall health. So even though my numbers aren't where I want them to be, I'm not letting myself feel defeated. We have a plan, and I am going to push through this small hiccup like all of the others.

I appreciate all the prayers and well wishes. You all help me stay motivated to be the best version of myself every day! So thank you for that!

Month 1 on Orkambi

Back in July, this new drug, Orkambi was approved by the FDA. Orkambi is the second drug that has come out that actually treats the underlying cause of cystic fibrosis. I wrote a post about it, and you can read that here. After jumping through hoops for my insurance company to cover the quarter of a million dollar yearly price tag, I finally started taking Orkambi on August 14.

 I was told by many others who had already started taking it that it would cause quite a few side effects that would not be very pleasant. One day into taking it, I already started to feel what others had been calling "the purge." My lungs became tight, and I started coughing up a ton of junk. The chest tightness lasted for a few days, and as soon as it started to lessen, my nose turned into a constantly running faucet. They say that the first few weeks is filled with your body getting used to the new medicine and the medicine causing all of the deep down, old mucus and junk to be moved up and out of the lungs.

During the first couple of weeks, I felt pretty terrible, but I just pushed through it. The coughing slowly became less, and my nose slowed down with the running. Throughout all of it, I continued with my treatments and with my workouts which were much more difficult while my chest was tight.

I've been monitoring my PFT at home, and it did seem to drop some. At my last clinic appointment, my FEV1 was 47%. According to my at home reader, I've been running at 43% lately. A month into taking Orkambi, I was due to have my clinic appointment. This past Wednesday, I headed to Nashville for it, but unfortunately, I didn't actually ever make it to Vanderbilt. We got into a car accident in downtown Nashville... only about 10 minutes away from my destination.

My car ended up have some damage that made it impossible for me to drive it. My husband and I spent the rest of the day trying to figure out how to get back to Knoxville without a car. My insurance tried to get me a rental car, but sadly, there were none available in the correct price range. Thankfully, I have amazing friends and family, and we were able to bum rides back home.

Since I missed my appointment, I had to reschedule. I won't have my clinic appointment until October 21 now. So, we wont know for sure how Orkambi is effecting my lung function until then.

I'm just taking it all one day at a time, because it seems from reading others' stories that it takes awhile before you might see any positive lung function changes, and some don't see any increase. It's all just a waiting game right now. I'm still hopeful that I will see some of the benefits that this medicine can make happen. More updates on my progress to come...

Awareness and Hope

Just thought I would share the news story that the local news did about Orkambi here. They interviewed a CF doctor and a few CF patients including myself. I think he did a good job with the story, and we made sure to emphasize the fact that while Orkambi is a game changer, it is not a cure. I love having the opportunity to spread awareness!   Just click on the picture to watch the video. :)

Orkambi... HOPE

"Aaron Stocks, who participated in a trial for Orkambi, said that's certainly the case for him. The 30-year-old Maryland man said the drug gave him a noticeable improvement in lung function and helped him to gain weight, which is difficult for many cystic fibrosis patients. He recalled going on his normal run not long after starting the drug, only to be surprised to the point of tears when he realized how much easier he could breathe and how much farther he could go."

This quote from an article about today's announcement about Orkambi fills me with so much hope! The hope is welling up inside of me and is getting dangerously close to overflowing. I understand that not everyone on Orkambi has seen these same results, but I can't help but hope that I will see similar results. Actually, I have hope that I might even see a one percent increase in my lung function. To me, that would be amazing! Any gain in lung function is a huge victory for me since I fight every single day for every percentage of lung function I have. My lungs are tired, and my body is tired. I can only get them so far without this extra help. This could be the boost my lungs need to help me live a longer, healthier life. That last sentence in the quote, the one that talks about being "surprised to the point of tears" is something that I want to feel. I can't even imagine what that feels like. I just want the opportunity to feel it.

While it's a huge deal for me that Orkambi was approved today, it is an even bigger deal for all of the children who are fighting cystic fibrosis. With new medications like this being approved, their future is so much brighter than mine was at their age. There is so much hope for them to be able to live with their beautifully high lung function for longer.

Is Orkambi a cure? No. But I believe it is a huge step in the right direction. A cure is coming. I can feel it!

Does that mean we can slow down on fundraising or raising awareness? Of course not! It means that I am going to be doing everything in my power to raise more money and more awareness than I already was! There is so much more work to do! We are getting so close, and we can't slow down now.

If you would like to read more about today's announcement, check out these two articles:
Washington Post
Boston Globe

Blood... Oh my...

Thursday night was one of the scariest experiences for me with cystic fibrosis to date. This past week, I was at the beach with my husband's family thoroughly enjoying myself and every minute of the vacation. I was feeling great. My lungs felt clear as they usually do during my time spent at the beach, I had been going on long beach walks, playing games, swimming in the water (watching carefully for sharks :/ ), and completing my workouts provided by my wonderful trainer.

Everything was going better than I could have even hoped until that night. After finishing my breathing treatment for the night and finishing the chapter I was on in my book, I lied down to go to sleep. The feeling came on quickly, and it is something that I had never experienced before. There was a crackling in my lungs that told me I needed to cough which is common in my lungs, but it was followed by a gurgling feeling that truly felt like I was drowning. When I coughed to try to clear it out, my mouth filled with fluid which completely confused me.

I ran to the bathroom to spit it out in the sink... It was bright red. I quickly realized what it was. I was coughing up lots of blood. The sink began to look a bit like a murder scene with blood in the sink, on the mirror, on the faucet, on the counter, and on my face. It was terrifying. I have never been a fan of blood as it is, but when it is coming from my lungs, it is on another level. I won't say I panicked, but there were definitely tears in between spitting blood, and my husband was a trooper. While I had read a little bit about this happening in CFers, he had no clue it was a possibility. He did offer to call 911, but I reassured him that I didn't think it was necessary.

Normally, I would have called my CF doctor right away, but unfortunately, it was 2:00 am when this finished. I called them first thing Friday morning to see what they wanted me to do about this new issue. They decided to start me on an antibiotic, because many times, hemoptysis (bleeding from the lungs) can be caused by an infection. They also told me to lay off my Pulmozyme and hypertonic saline for 3 days to give my lungs a rest. (However, I am going to continue counting these days as being compliant with my treatments seeing as I am just following doctor's orders.) Since that night, I have coughed up more blood 3 more times, but I'm hopeful that it's finishing up since it's been 16 hours since my last episode.

It is just a reminder that living with cystic fibrosis is an never ending battle. No matter how well you are taking care of yourself, it can still rear its ugly head and remind you who is really in charge.

So how does this change my game plan? It doesn't minus these 3 days of no treatments and the antibiotics that I am on for 2 weeks. I will go back to being 100% compliant with my treatments, and I will work out as vigorously as I have been after a few days of rest. Life goes on. The fight continues. I have a lot to live for, so I will continue fighting to make that happen.