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.

The Nature of the Beast

There are times when I just sit and reflect about how life is going, and today is one of those days. As most of you know, I have been focusing on my health more so than ever before. I have been 100% compliant with my breathing treatments for 467 days now, and I have been working out hard 4 to 5 days a week. I am so proud of my accomplishments with both my treatments and my fitness. I have finally figured out how to balance my health, work, and personal life, and I am happier now than I have ever been. Sounds great, right?

Unfortunately, the weather has been changing, pollen has been high, and my cystic fibrosis lungs have not been happy. Last weekend, I woke up on Saturday, and it felt like my lungs were in a vice grip. By Monday, I had absolutely no voice, and I got to hear, "Mrs. English, what's wrong with your voice?" all day from my students. I've been continuing with my treatments and workouts, and I am slowly starting to feel better again. It's been a week, and I am still not back to normal yet.

That's the nature of the beast though. Cystic fibrosis is a disease that robs you of your breath no matter what you are doing to fight it. There are good days, and there are a lot of bad days. The real test comes when you are having those bad days. What will you do? Will you keep fighting? Will you push on? Will you give up?

My choice is to keep fighting. I have to believe that all of the hard work I am putting in is making a difference even if I don't feel it right now. So I will keep up with the compliance, the workouts, and the research to figure out ways to push myself even further.

Because of CF, there is only so much I can do. The rest of the fight comes from the research that provides me with new treatments and medications... and hopefully, one day, a cure. That's where you come in. You can help add tomorrows for those of us who live and breathe with cystic fibrosis everyday. It's easy to do. Just click on the link below, and then push donate. There is no such thing as a donation that is too small, because every little bit adds up to make a huge difference.

http://fightcf.cff.org/goto/kaylaskrusaders2015

I am also selling Kayla's Krusaders t-shirts for $20 each. After paying for the cost of the shirts, the rest of the money will go straight to the Cystic Fibrosis Foundation. If you are interested in ordering one, let me know.

Thank you all for you support! 

Why Great Strides is important to me

This is something I wrote last year before the Great Strides walk, and it still rings true. It is so very important for us a CF patients and families to step up and raise money. I'm starting my fundraising process on social media, and if you are interested in making a contribution, all you have to do is click on the picture below. 

As Great Strides has been creeping up on me, I've been thinking a lot about how far we have come even in the last 20 years. When I was diagnosed with cystic fibrosis in 1993, I was 6 years old. That in itself shows you where we were and how different things are now. Newborns are all screened for CF now. It is very rare for someone to slip through the cracks like I did. The life expectancy for those of us with the disease was around 15 years when my parents found out. They had never even heard of CF, but they knew that they would be doing everything in their power to fight this horrible disease.

When I was 10 years old, The Vest was a brand new thing. I still remember the day that I got it. I was so excited (a feeling I don't really have about it anymore). It was a huge deal because up until that moment, I had spent every morning and evening lying on a declined board while my parents pounded on my back and sides. I just remember hating every minute of it, and I know my parents felt horrible doing it.

Fast forward to a few years later, I was a freshman in high school in my honors biology class. We had a substitute, and there was a small excerpt in my textbook that we were reading out of about cystic fibrosis. At first, I was pretty excited to see CF mentioned in something... That excitement quickly faded when I began reading. I read that day that my life expectancy was 16 years. Keep in mind that I was 15 years old at the time, and my parents had never mentioned life expectancy before. They wanted me to live my life to the fullest, and that meant not worrying about how much longer I had to live. I was devastated, and it took a little time to work through that and to realize that a number in a book had no effect on me.

I used to spend an extra 20 minutes twice a day doing my TOBI treatments, and that is now in a podhaler form that takes no time at all. Kalydeco, the first drug to treat the underlying cause of cystic fibrosis, was approved in 2012. Clinical trials are ongoing for Kalydeco and VX-809 for those of us with deltaF508. There is an inhaled form of Vancomycin (an antibiotic used to combat MRSA which I grow in my lungs) in trials right now. 

There have been so many advances in the cystic fibrosis world, and it is mind blowing to think that children with CF now have so much more hope for the future than I had when I received my diagnosis. The life expectancy has gone up drastically from 15 to close to 40 years! What an amazing thing! That's only possible thanks to all of the money that has been raised over the years to help fund the research and advancements it has led to. That is why I work so hard to raise money for the Cystic Fibrosis Foundation. Some of the new things may not help my lung function get much higher than it is right now at 48%, but they could definitely keep the lung functions of those children from dropping so quickly to where mine are.

I urge you all to stand up for your loved one with CF. With so few people living with cystic fibrosis, if we don't fight it, who will? We are the biggest hope for the future in the battle against cystic fibrosis.

How does CF really affect my life?

I like to tell people that I don't let cystic fibrosis determine what I can and cannot do with my life. The truth of the matter is that CF does affect my life. It makes things more difficult, it is time consuming, and it tries to limit my physical ability. When I decide I want to do something, I have to fight for it, and I put everything into it. There is no doing things halfway. In the interest of bringing some awareness to what life is like living with cystic fibrosis, I wanted to shed some light on my daily life.

My days are filled with medications and breathing treatments to help maintain my health. Those are the basic necessities for those of us living with the chronic disease. I do two breathing treatments a day which include using hypertonic saline (a salty solution inhaled to loosen mucus and irritate the lungs to get said mucus out), pulmozyme (an inhaled medication that is supposed to thin the mucus), and the vest (a literal vest that shakes in order to shake and loosen the mucus in the lungs). My day starts with one of my treatments at 5:00 in the morning and ends with my second treatment before I go to bed. The morning treatment takes about 30 minutes while the evening treatment takes around 45 minutes.

I take numerous medications throughout each day as well. Those include digestive enzymes which supplement the enzymes my body is unable to produce. I take six with every meal and four with any snacks during the day. I also take anti-inflammatories and numerous vitamins to help my body stay healthy.

Those are the regular things I do everyday. If I am sick, my doctors add to my daily regimen. Usually, I will end up on oral antibiotics and sometimes steroids. If those don't kick whatever is going on in my lungs, I end up with a picc line which is for at home IV antibiotics. My husband just loves when I have have to have a picc, because I am such a big baby. :) Having an IV in your arm definitely complicates everyday life.

Daily medications have always been the easy part of maintaining my health, but the breathing treatments have been a whole different story. Many of you have been following my resolution to be 100% compliant with my treatments. I made it 365 days in 2014 and am now 24 days into 2015 with 100% compliance.

Now that I've mastered the whole compliance issue, I had to figure out what else I could do to push myself even further. I workout four or five days a week. My workouts include a little bit of everything including my favorite, lifting heavy weights. I'm up to 155 pound dead lifts and 100 pound front squats. What??!! :) I'm so proud of those numbers especially since I only weigh about 115. I have also started walking on the treadmill while doing my morning treatments. That has proven to be difficult due to the multitasking involved, but I'm hopeful that it will help in improving my lung function.

As if that weren't enough, I also have to make sure to get plenty of rest and eat plenty of food to maintain my weight. On top of that, I am a fourth grade teacher which takes a huge amount of time. I work all day and take work home with me almost every night, and I write lesson plans on weekends.

I don't really have any free time, but it is all worth it. I am living my life to the fullest following my dreams and doing things I didn't even know I wanted to do. I am incredibly thankful for all of the medications and treatments available to those of us living with cystic fibrosis.

 

Great End and Even Better Beginning

Everyone seems to be giving a little recap of their 2014, so I thought I would join suit. 2014 was a fantastic year, because it was the year that I finally took control of my health. I made choices that are positively affecting every aspect of my cystic fibrosis life. I made a resolution last year to be 100% compliant with my breathing treatments in 2014. Honestly, I wasn't sure I would be able to do it since I have struggled with compliance for quite some time. In fact, during college, I rarely did my treatments... how I wish I could go back in time and knock some sense into my younger self.

 I also started working out with a trainer who has been creating workouts for me that are specific to my body's needs. I have been doing things that I never thought I would be doing. I can dead lift 135 pounds! That's 21 pounds more than I currently weigh!

Another big change in 2014 was my vest. I invested in the Afflovest, and I love it! I can really tell a difference in how productive my cough is during my treatments. I'm so grateful to have the opportunity to use this new piece of technology. The fact that it has a battery makes it even better because I have had the opportunity to use it in the car and at work a few times!



Now for the best part of 2014/2015. As many of you know, my last clinic appointment did not go quite as well as I had planned. My lung function had dropped from 47% to 43%. After discussing it with my doctor, she decided to put me on Zyvox which is a pretty intense oral antibiotic that is used to combat the MRSA that I have growing in my lungs. She also wanted me to do a follow-up PFT in 3 weeks to make sure I was doing better. If there was little or no improvement in my numbers, we were going to try a PICC line with Vancomycin.

After the first week of antibiotics, I was feeling much worse than I had been when I was at the doctors. I called, and they decided to add yet another antibiotic along with steroids to help my body jumpstart the healing process. That seemed to do the trick, because I began feeling better after just a few days.

And that brings us to today. I went for my follow-up PFT, and I was pretty nervous. I was feeling great, but that doesn't always translate into the numbers. My lung function was at 49% today! Not only did I make it back to where I was in August, but I surpassed that and almost made it to 50%! I could not be more excited and relieved with my numbers today! It just continues to show me how much my hard work is paying off, and it encourages me to push even harder!

So as of right now, I don't have any specific resolutions for 2015, but I will say this: I will continue being compliant with my treatments, and I will continue pushing as hard as I can in my workouts. There is not a doubt in my mind that those two things are the reason my lung function is the highest it's been since June 2013. I feel amazing, and I can't wait to see what this year brings!