Positive Thoughts Thursday: Health & Fall Fun

I've been totally slacking on blog posts lately, but honestly there hasn't been much going on. I'm glad that several of my other blogger friends do their "Thankful Thursday" posts each week because it reminds be to get back on here and appreciate the little (and big) things in life. :)

• Today I'm thankful for good health. I've been on Kalydeco now for six weeks. And just now, over the past couple days, I feel like I'm finally slowing down with my mucus movement. Over the last six weeks, I experienced some serious mucus movement in my lungs, especially deep down in there. Things started moving around the first few weeks I was on Kalydeco, then I got a cold which tightened my lungs for about a week. And then after that cold, I felt like I was constantly coughing my brains out! I'm pretty sure I've coughed up every color of the rainbow mucus (except maybe blue/purple...) and I've gotten a LOT of stuff out. Over the past couple days, I feel like I've finally been able to take a good, nice deep breath without coughing up a chunk of my lung. 
• Related to the above post - I'm really looking forward to my CF appointment coming up soon. I'm very anxious to know what my FEV1 is, especially now since I feel like my mucus production is slowing down. I'm also curious to see if my weight has gone up. Since starting Kalydeco, I've been having at least one, usually two, Ensure Plus's each day which is an extra 350 calories each. I hope K really helps me in the weight-gain department.
• Fall weather! I am not a fan of the heat. I enjoy sunny & 75 (Joe Nichols, anyone?). It's so much more comfortable to teach in the cooler weather. I love having the windows open all day and getting fresh air in the house. And of course, all the fall fun & fall food (aka: apple donuts!)! This weekend we're hanging out with Tim's aunt & uncle, and his sister, her husband and their two little guys! We're going to the pumpkin patch/apple orchard on Saturday -- always a good time! :)  
• Last weekend, Tim and I walked in (my third) our second Great Strides of the year. I was asked to speak at this one which I enjoy doing. I like to share my story with others, especially parents of little ones with CF to give them hope about their child's future. I was also able to meet one of my blogging cysters, Cheriz - who's now in the hospital for a tune-up- hope you start feeling better soon!! Here are some pictures from the event.

• Oh, and how could I forget?! Yesterday, Tim and I celebrated our third wedding anniversary! :-) Today marks our 7th anniversary of being together, total - seven years ago today, Tim asked me to be his girlfriend while eating lunch in the "Dog-Pound Deli". This year, not only were we husband and wife, but we also became "homeowners"! I'm looking forward to what this next year (and MANY more) has in store for us! I love you, Tim!!!! 

Feelin' Good

I've been feeling great the past few days! Sadly, my spring break is coming to an end and it's back to work tomorrow, but I'm craving that routine again. I've had a nice week off with several days to myself, read a couple books, hung out with some friends, got some more Great Strides stuff figured out - overall, it was a nice, relaxing break.

This is the shirt I wear every time I go running because it
gives me the motivation to keep going and Out Run CF!

I also really pushed my running this past week, but more so the past couple days. The route I've been running around my neighborhood is about 2.4 miles and I've always walked and ran it. Yesterday morning I did part of my treatments - my albuterol to loosen my mucus and open my airways - then I went running. I ran (well, jogged) my entire route around my neighborhood without stopping or walking and I think that last few blocks I had a huge grin on my face because I knew I was going to make it! It was so weird because I was breathing great and my legs weren't killing me - it felt so good! When I got back I finished my treatments with my hypertonic saline (HTS) to make me cough up whatever was way down deep in my airways. This morning, I had to do the same thing! I wanted to know if it was just a fluke, or if I could really do it again. And I did! I did the same thing with my treatments, albuterol first, run, and now HTS. This run was much harder on my legs, but I made myself push through it because I knew that if I could do it once, then I could do it again. My plan is to keep running the entire route for a couple more weeks and gradually add in another block at a time until I'm up to 3.1 miles which is the length of a 5K. I've got 68 more days to be comfortably running a 5K and if you would have asked me a week ago if I'd be ready, I would have thought it would have been a close call, but now I'm sure that I'll be able to do it! I never thought I'd be a good runner and I wouldn't consider myself one now, but I'm just so proud of how far I've come since the fall when I started all of this. Go me!

30 Days of Blogging: Day 20

Day 20 - The meaning behind your blog name

Well, the blog started out as "How I Live, Laugh and Love with CF" because I wanted to go off of one of my favorite quotes "Live, Laugh, Love". I wanted to show people out there that it's possible to live a 'normal' life despite having CF and how I make it work. I like to try to mention one of those words in every post to try to tie it in all the time, but it doesn't always work.
After I had the blog for about nine or ten months, I noticed there was a big focus on my CF in the blog (which is what I wanted), but I wanted the title to be something not blatantly "CF". So I played around with some things and came up with *Live*Laugh*Love*Breathe* because not only am I doing the first three all the time, but my other major focus in life is to breathe as deeply and best as I can - I just thought it was very fitting for me. I've grown to absolutely love this phrase and I wish they made things in stores that had "Breathe" tacked on the end of it, too...but Tim says we have enough "live, laugh, love" stuff in the house already! :-)
I always want it to be a constant reminder to myself (and him) that that's what life's about: living life to the fullest everyday, laughing things off and not taking them too seriously - basically, just relax!, loving each other through everything, and breathing deeply & being/staying healthy.

PS: This is my 100th post - I think this is the perfect topic to sum it all up so far! :)

KALYDECO!!!!!

From the Cystic Fibrosis Foundation website: 

Vertex Submits Application to FDA for Approval of VX-770 – First Potential Drug to Target Underlying Cause of Cystic Fibrosis

October 19, 2011

Vertex Pharmaceuticals, Inc., announced today it has submitted an application to the U.S. Food and Drug Administration for a potential new CF therapy, VX-770 — under its new proposed trade name, KALYDECO™.

If approved, it will be the first drug on the market that targets the underlying cause of cystic fibrosis. Therapies available to people with CF to date only treat symptoms of the disease.

The company is seeking approval for the drug in people with cystic fibrosis age 6 and older who carry at least one copy of the G551D mutation of cystic fibrosis. (THAT'S ME!!!)

KALYDECO (kuh-LYE-deh-koh) was discovered in a collaboration between Vertex and the Cystic Fibrosis Foundation, which provided substantial scientific, financial and clinical support throughout the development process.

“The CF Foundation is thrilled that KALYDECO is on track for possible FDA approval in 2012,” said Robert J. Beall, Ph.D., President and CEO of the CF Foundation. “This is a significant step forward in our collaboration with Vertex and is further validation of the CF Foundation’s drug development strategy. We remain committed to accelerating the development of similar targeted medicines that will benefit all people with cystic fibrosis.”

Vertex has asked the FDA for priority review of the potential drug, which, if granted, could shorten the review from 10 to 6 months. The FDA grants priority review status for several reasons, including in situations where a potential drug is considered a major treatment advance. (YAY!!!!!)

Results released earlier this year from Phase 3 clinical trials of KALYDECO in people with the G551D mutation of CF showed that those receiving the drug had remarkable and sustained improvements in lung function and other key symptoms of the disease, compared with those on placebo. (YAY AGAIN!!!)

As FDA review of the potential drug gets underway, Vertex has set up a program to provide KALYDECO to people age 6 and older with the G551D mutation who are in critical medical need and could benefit from the treatment prior to potential approval. (Not me, but great idea for those who could benefit!)

The expanded access program is designed for people with CF who have highly limited lung function and meet other criteria. (Information about the program is available at CF Foundation-accredited care centers.)

KALYDECO is currently being evaluated in combination with another oral drug in development, VX-809, in people with the most common mutation of CF, Delta F508. 

Vertex plans to begin the second part of the Phase 2 KALYDECO and VX-809 clinical trial this month and will evaluate the two drugs over a longer period of time.

Frequently asked questions about Kalydeco (this one was my favorite):

What were the results of the VX-770 Phase 3 clinical trial in adults?

The Phase 3 clinical trial tested VX-770 in patients age 12 and older who carry at least one copy of the G551D mutation of the CF gene. Patients who received VX-770, compared to those on placebo, showed a marked improvement in lung function (FEV₁).

Those who received the drug gained 10.6 percentage points more on a lung function test after 24 weeks than those getting a placebo, a difference that is statistically highly significant. Patients continued to take either drug or placebo for another 24 weeks and the improvement was sustained. Lung function, the primary endpoint of the trial, was measured by how much a person could exhale in one second, a standard test.

In addition, patients receiving VX-770 gained nearly seven pounds, on average, over the course of the trial. People with CF have a hard time gaining and maintaining weight because the buildup of mucus in the pancreas limits the body’s ability to absorb essential nutrients and vitamins.

Patients also showed improvement in other secondary endpoints of the study, including reduced likelihood of pulmonary exacerbation and decreased respiratory symptoms.

In addition, average sweat chloride dropped toward normal levels in patients on VX-770, compared to those on placebo. Excessive sweat chloride is a key clinical indicator of the disease.

The overall findings are profound because they demonstrate that a chemical compound can improve multiple clinical measures of CF by targeting the basic defect. CF therapies currently on the market address the symptoms of the disease, not the underlying cause.

This is such amazing news for me and everyone in the CF world!! Especially for me and the other 4% of the the CF population with the G551D mutation!!! The results of the clinical trail were outstanding!!! The progress of this drug gives me so much hope for the future. Yes, it may take another year or two before it is available for CF patients, but hey, I've been waiting 23 years for something like this, so what's another two?! I am very excited to share this news with my readers, friends and family. I am also thankful for your love and support over the years. Because of your donations, drugs like this are making significant progress and significant changes in peoples' lives. THANK YOU to anyone/everyone who has donated to the Cystic Fibrosis Foundation because you are making it possible for me to live a wonderful, happy, and healthy life.

I believe that one day this drug will help me achieve my ultimate goal: 

To LIVE, LAUGH, LOVE and BREATHE for a long time!!!!