Kalydeco Update

The Cystic Fibrosis Foundation posted this update on their website today:

FDA Grants Six-Month Priority Review of Kalydeco (VX-770) — First Potential Drug to Target Underlying Cause of Cystic Fibrosis
December 15, 2011
Vertex Pharmaceuticals, Inc., announced today that the U.S. Food and Drug Administration (FDA) has granted a request for a six-month priority review of a potential new CF therapy, Kalydeco™ (VX-770).

The company is seeking approval of the drug for people ages 6 and older with the G551D mutation of CF.

The expedited review sets a target date of April 18, 2012, for the FDA’s approval decision, four months earlier than the standard review time of 10 months.

If approved, Kalydeco (kuh-LYE-deh-koh) will be the first drug available that targets the underlying cause of CF. The FDA grants priority review for several reasons, including situations where a potential drug offers a major advance in treatment.

Kalydeco was discovered in a collaboration between Vertex and the Cystic Fibrosis Foundation, which provided substantial scientific, financial and clinical support throughout the development process.

Vertex’s application for approval of Kalydeco, submitted to the FDA in October 2011, included results from Phase 3 clinical trials of the drug in people ages 6 and older with the G551D mutation of CF. The results 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 the placebo.


This is fantastic news! I'm not sure exactly when it'll be released/sold to patients, and how much it'll even cost, but it's moving along quicker than expected - can't complain about that.

I LOVE MUSIC!!!

I don't know what I would do if I didn't have music in my life daily. It absolutely can turn my bad day around, it's an ultimate pick-me-up, can motivate me (especially while running), and sometimes it's good to just calm me down. I really enjoying learning song lyrics too. Not only is music fun to dance and sing along to, but I love when songs have great messages/lyrics to just soak up and listen to. I could make a list 100 songs long that I could call my 'favorites' and still feel like I haven't captured them all. Here's a list of some of my favorite songs: (if you click on the title of the song, it'll bring you to a youtube video so you can hear it! :-) )

• Michael Bublé: Sway; I love this song because it makes me want to learn how to swing dance! I absolutely love Michael Bublé's voice, I could listen to it any day, anytime - makes me so happy.
• Rascal Flatts is my all time favorite group and I pretty much love all of their songs!! Here are a few of my absolute favorites of theirs: Day Before You; Unstoppable; I Won't Let Go; Fallin' Upside Down; The first three have very special, moving lyrics to me - the fourth one is just fun!
• Taylor Swift is another one of my favorites! I'd Lie; The Best Day; Long Live - I could listen to her ALL day long!
• Keith Urban just has a beautiful voice: Somebody Like You; This is definitely my favorite song by him!
• Jason Mraz: We Sing. We Dance. We Steal Things.; This is probably one of my favorite whole albums - I think it's the most played in my iTunes, yes, even over Rascal Flatts. I could listen to this album entirely without skipping any song and loving each one - highly recommend it!
• Jack Johnson has a very soothing and comforting voice. He's great to just listen to when you want to relax. I really like Banana Pancakes, Good People and Better Together - that was our wedding song. :-)
• 'N sync/Justin Timberlake: 'N sync was the first group that I was obsessed with - I had all of their CDs (still do!) and I saw them in concert in seventh grade! I also really enjoy Justin Timberlake's more recent solo stuff. I Drive Myself Crazy; LoveStoned/I Think She Knows
• The past few years I've really come to love country music. It has amazing lyrics and really fun songs - here are some of my favorite country people and one of their songs that I enjoy: Carrie Underwood (Mama's Song), The Band Perry (If I Die Young), Zac Brown Band (Whatever It Is), Josh Turner (Would You Go With Me), Lady Antebellum (American Honey)
• Lucky Boys Confusion: Hey Driver; This is a local band from the Chicagoland area. Tim and I and some of our friends have seen them a couple times play at a bar in town. They really put on a good show - and it's not country!
• Maroon 5: I believe I've previously expressed my thoughts in a different post on Adam Levine and his sexy voice, but this is my favorite song from them, by far! The Way You Look Tonight
• Savage Garden: Truly, Madly, Deeply; Heard this song in 4th grade and have loved it since :-)
• Dave Matthews Band: Ants Marching; I'm not a huge Dave Matthews fan, but I really like this song.
• Pentatonix: This is the group that won season 3 of the Sing-Off. I really enjoyed their interesting style of acapella singing! Video Killed the Radio Star
• Steve Miller Band (and random classic rock/oldies): The Joker; Some Kinda Wonderful - adorable lyrics (Grand Funk Railroad), Ballroom Blitz (Sweet)
• Aretha Franklin: RESPECT; This is my jam!! Just ask anyone who attended our wedding!

This seems like a lot, but I could keep going on, and on, and on -- this is so hard to stop! I absolutely love music, I cannot go a day without it! I hope you enjoy the playlist I provided for you! :-)

Christmas Spirit

I hope all of you had a wonderful Thanksgiving and weekend! We did - it was great spending time with all of our family and to be able to relax and enjoy this nice, long weekend! This year, I've really been looking forward to Christmas and I don't know why. I don't really have anything on my Christmas list this year, but I've been in 'Christmas mode' for a while, especially this weekend.

We avoided Black Friday this year because ever since working retail, it hasn't really appealed to me. I worked in a big-box hardware store for three years and saw a lot of crazy people shopping the day after Thanksgiving, it kind of turned me off. I did take advantage of some black Friday deals online though, and I ordered our Christmas cards (which I'll post once I get them) and a gift for someone in the family, so I feel like I did accomplish something Christmas-y that day. Instead of hitting the stores, this year for black Friday & Saturday, Tim and I painted (and Tim re-did some drywall) in his aunt and uncle's two bathrooms in their house. They've been out of town visiting some of their family and will be back tomorrow to see it all completed - I hope they're happy with it. It was a lot of work, but I'm really glad we were able to do it for them. :-)

Sorting through all of our Christmas stuff!

I've been bugging Tim for a while now to get our Christmas tree out of storage so that I could put it up this weekend, so that's what we did today. This is definitely the earliest I've ever put up a Christmas tree, but like I mentioned before, I've been in the Christmas spirit lately! I had the music going, all of the ornaments laid out and the lights on -- it was awesome! Now we've got dinner going in the crock-pot and it just feels like winter/Christmas is coming full force and I'm ready for it (until we start getting snow!). Today, while I was going through all of our Christmas stuff I this awesome ornament which I didn't even know we had! I was very excited when I found it, so of course I had to share it. :-)

In other news, I've been compliant with all of my treatments for 119 days now, been going to the gym twice a week still (oh, I went the gym on Black Friday, too!) and my next CF clinic appointment is December 22nd - a little less than a month away. I'm really looking forward to that appointment, hoping that my PFTs show how awesome I've been feeling lately. :-)  (And from the amount of smiley faces in this post, I'd say I'm feeling really good!)

What are you thankful for?

I'm thankful...

• to be healthy
• that I have a fun, supportive, awesome husband
• and lucky to have found someone who loves me for me, despite my chronic lung disease
• that I have some pretty great friends
• to have a job
• to have a job that's really fun and that I love
• to work with great, helpful co-workers
• to call some of my co-workers my friends
• to have a roof over my head
• to have a working vehicle
• to have money for all of my medication
• that I live in a place that allows access to the best medication and medical advancements
• for insurance (as much as I hate it)
• for all of my family - my side and my in-laws
• for the Cystic Fibrosis Foundation 
• for all of my doctors/nurses who take amazing care of me :-)
• to anyone who has ever donated to the CFF
• that I'm able to exercise without getting too short of breath
• to live across the street from a gym
• to have such a great relationship with my doctor and nurse
• to have learned so much about myself through writing this blog
• to have so many people reading this blog and taking time to provide their feedback
• to be able to live, laugh, love & breathe everyday!

Go Me!

I'm sorry, but I have to brag a little bit about my workout today. I don't think I've sweat this much since we decided to go hiking in over 100 degree heat index in July. :) I ran a whole mile straight for the first time since joining the gym - in about 12ish minutes, ten minutes on the elliptical, and then I went on the treadmill for another ten minutes which included jogging, a sprint, then cool down. I really think the sprint did wonders for my lungs (I'm still coughing!). I was already exhausted by then, but the person next to me was really encouraging and we did a sprint together (I would have never tried that on my own!). I'm going to try to add that into my gym routine each time, I could really feel it - even though it was only for about thirty seconds. I just feel so good, I'm breathing really well right now; I can't wait to see what my PFTs are in another month!

Things to Consider

I've always imagined that I'd grow up to get married, be a teacher, and a mommy. So far, I've got one of those goals accomplished, about half-way towards the teacher one, and not quite at the mommy stage yet. There are so many things to consider when having a child (more than I could even image as I was growing up): Do we have enough money? Do we have space? Will we be good parents? What will we do with the baby when I go back to work?...this list can go on and on, for a 'normal, healthy' couple. But add a mom with CF in the mix and the questions start looking like: Will our child have CF? How will my body handle a pregnancy? How will I keep myself and the baby healthy if he/she has CF? How far do we want to go to 'prevent' having a child with CF, or do we not mess with fate? Will I be able to go back to work after? Do we make enough money on one income if my health declines? Will our families and friends still be supportive if we have two CFers in the family?
It's a lot to think about. Numbers and probabilities start coming in to play such as 1:50, 1:602, or 1:2. Trying to make sense of it all, and trying to make the "right" decision, whatever that may be, is very difficult. I know I've posted about this before (here and here), but it's constantly on my mind and I wish there was an easy answer, but there's not - at least not right now. Isn't everything supposed to happen for a reason?? So, what's supposed to happen? What's the right choice??
By the way, I'm not looking for answers to my questions - I know that this is our choice, and only our choice but this is my way of 'thinking out loud'. 

100 and 5th

Today marks the 100th day in a row that I've done my vest (twice a day) and all of my nebulizers!!! Today also marks the fifth week that I've gone to the gym, two days a week! I am very proud of myself to reach these accomplishments, but I really look forward to continuing and making those numbers larger. I never thought the day would come where I'd say that I've done all of my treatments for over three months straight! I am also looking forward to my next CF clinic appointment which is December 23rd to see where my PFTs are at. My last clinic appointment was in August, and I was at 100% compliance for about three weeks at that point, so my PFTs weren't as great as I wanted them to be - even though they did go up a tad. I really hope that they have skyrocketed by the time I get tested in December.
A year ago, if you would have asked me how I would physically feel after being compliant with my vest/nebs for 100 days, I would have probably said something along the lines of having the best lung function I've had in a long time. Although I do feel great from doing all of my treatments, I don't think I felt much of a difference until I started going to the gym. I really feel like doing all of my treatments AND going to the gym has made me feel even better. I wish it hadn't taken me this long to figure this out!!! I've read it and heard it millions of times, but I was always too lazy to get up and exercise. I'm SO GLAD I finally pushed myself because I really feel like it's paying off with my lung function, and also my overall energy level.

So the moral of the story.... treatments + exercise = happy, healthy me!

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Questions for Tim

For those of you who have voted "yes" on the poll about Tim writing a post (so far there are 8!), can you please leave a comment about some topics you'd be interested in reading from him? I'm sure he's going to say there's nothing to write about so we must leave him with no excuses! :-) Thanks!