A Day in the Life

I was looking through my old blogs, and I realized that I have never done a post about what my day looks like with all of my medications and treatments. I feel like it is important to share that with everyone, because for someone who does not have CF and has not really been around someone who has it, Cystic Fibrosis can be a very abstract thing. The amount of work that goes into keeping a CF patient healthy is astronomical, and as you read through my day, you have to realize that there are a lot of people who have to do even more than I do. So, let's jump right in. This is a typical day for me:

7:00 am - 1st breathing treatment of the day :
                            5 blows on the Acapella

                            2 puffs of Albuterol
                            The Vest for 20 minutes

                            Hypertonic Saline about 15 minutes (inhaled saline solution..... makes me cough a lot)
                            Pulmozyme (inhaled medication) about 15 minutes
                            Tobi or Cayston depending on the month. I alternate those 2 every month. Right now I'm
                                    on Tobi. Tobi is inhaled and takes 10-15 minutes. Cayston is also inhaled
                                    but only takes about 5 minutes.
                            1 puff of Advair

          *Total of close to an hour if I'm on Tobi and 40 minutes if I'm on Cayston.

8:30 am - I take a Nexium, vitamin, and Azythromycin

8:40 am - leave for work

5:00 pm - get home from work
                If I'm on Cayston, I do my second does of it now.  5 minutes

6:00 pm - workout (an hour or more depending on the workout)

7:00 pm - 2nd vitamin

9:00 pm -  2nd full breathing treatment of the day:
                            5 blows on the Acapella
                            2 puffs of Albuterol
                            The Vest for 20 minutes
                            Hypertonic Saline 15 minutes
                            Tobi or Cayston - Tobi (10-15 minutes) Cayston (5 minutes)
                            1 puff of Advair
           *Total of close to an hour with Tobi and 40 minutes with Cayston

Before bed - 2 squirts of Flonase in each nostril

Every time I eat, I have to take digestive enzymes as well. I take 6 with meals and 4 with snacks.

 Again, I do not have the biggest and longest regimen of those who have CF. Mine is kind of in the middle, but there have definitely been times where I have struggled with spending all the time necessary to do the treatments. That is always a huge struggle for me, because, there is always something I'd rather be doing. I'm working on that though, and it is slowly starting to pay off as I saw last week at my clinic appointment.(FVC went up to 82% from 70%. FEV1 went from 45% to 54%.)  My goal for the next clinic appointment is to get my FEV1 up to 60%. How do I plan on doing that? 100% compliance with my medicines and treatments..... and keeping up my P90X workouts. I will keep you updated on my progress. :)

New Update

Today was my clinic day.... As most of you know, I have been working pretty hard at trying to get my lung function up. My biggest motivation for doing it is because one day, I want to have a child, and we have decided that we will only do that if I am healthy enough. I'm not even sure what "healthy enough" looks like, but I know that it is higher numbers than I have right now.

Anyways..... drum roll please....... My numbers are now:
FVC 82% and FEV1 54%

That is up from FVC 70% and FEV1 45%

I will take that improvement any day! My next clinic appointment is February 1st. My goal for that one is to have my FEV1 up to 60%. Is it possible? I really don't know, but we will sure find out! :) I've got a lot of hard work ahead of me, but I think I'm up for the challenge!

Top 5

For those of you who are not Facebook friends, I decided to post my top 5 Great Strides Team T-shirts on here. Please let me know which one you like best! I'm looking for all the feedback I can get, because coming up with a design and then picking the one I like the most is way difficult! Thanks in advance for you help! :)

Exciting Times

So, not that long ago, I posted an article about the new drug that they have been testing that actually treats the cause of CF. Now, that drug only works for about 4% of the CF population, but it is a huge step in the right direction! Today, we got the amazing news that it has been submitted to the FDA for approval!!!! Here is the article:


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.

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.

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.

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.

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.

This article is from the Cystic Fibrosis Foundation's website. You can find more information here:
Vertex Press Release
FAQ's about KALYDECO
FAQ's about the combination of the 2 medicines

Anyways, thought I would share the very exciting news with you all! And by the way, I have very high hopes for the KALYDECO combination with VX-809 because Delta F508 is the mutation that I have.... So let's keep our fingers crossed and our prayers ongoing! :)

Finally!

For years, I have been saying that I was going to design a t-shirt for my Great Strides team, but I have failed time and time again. Well.... 2012 is the year! I have already started designing the shirts, and I have changed my team name to something a little more catchy. We are now Kayla's Krusaders. Here is the first design idea for our shirts.

 

I even added a little advertisement for my blog. :) I chose lime green for one purpose.... it stands out. You notice a shirt that bright. The ribbon and the rose are purple because in the US, that is the color of the CF Awareness ribbon. The rose on the back has two meanings. It is for the nickname that has been given to CF which is Sixty-five Roses. That came about because Cystic Fibrosis is such a difficult thing to say for young children. The second is that Rose is my maiden name making roses a big symbol in my life! Let me know what you think. 

And now there is a new one in the mix that my husband just designed. It has a nice UT feel, and I'm kind of liking it a lot. Decisions, decisions, decisions.

Happy Friday everyone!

The Countdown Is On

As you all know, I have been working pretty hard to see some improvement in my lung function. Well, my next opportunity to see if there is any difference in my numbers is October 26th at 1:30. I'm nervous but excited to see how much progress, if any, I am making. That's 14 days from now.

So what is the game plan for the next 2 weeks? I am going to keep up with my workouts, (Plyometrics is tonight after I finish writing this post) and I am going to be 100% compliant with all of my treatments. It has been quite the growing experience committing to these crazy workouts, but hopefully, it will all pay off soon.

On a fun note, here is a fun video from Ellen. It is hilarious! Enjoy! I hope everyone is having a great day!

Upcoming CF Fundraisers

Here are a couple of fundraisers in Knoxville that are coming up soon. :) They sound like fun!

Wine on the Water

UT vs. Georgia Tailgate