Health & Great Strides

I haven't posted about my physical health in a while, so I figure I may as well update you. The good news is that I haven't been on antibiotics since mid-September, which means it's been 6 & 1/2 months!! Bad news (or...not so fun news) is that the streak ends now. Over the last few days, I've had a sore throat and stuffy nose. I was hoping it was possibly just allergies, but today the frequency and consistency of my cough changed which told me it was time to bring in the 'big guns' - aka: Cipro.

I tried being proactive and got in contact with my doctor yesterday in case my allergy symptoms changed and I needed to begin Cipro over the weekend when she's out of the office. Of course, that couldn't go as smoothly as planned....I didn't get to the pharmacy until this afternoon to pick it up (because I wanted to start tonight after having crappy lung day today) and they told me they had no record of it - grrr!! So I called and texted my doctor who was as equally frustrated, but she kindly sent in the script again. Now I'm finishing up my treatments and plan to call Walgreens when I'm done to make sure it has arrived and has been filled before I go to the pharmacy this time.

Weight-wise, I think I'm hanging around the same weight as before I got pregnant with the boys. I know after I had them, I lost a few pounds, but the way clothes are fitting recently, it seems I've gained it back. I do not own a scale because otherwise I'd obsess over my weight, so we'll just see at my next clinic appointment.


Finally, I wanted to share about my Great Strides team this year. Colleen's Friends & Family will be walking at two walk sites this year - DeKalb and Bloomington. Every year our friends and family join us to walk and show their support for all people with CF and the CF Foundation. I'd guess we've raised close to $10,000 over the last five-six years together. The CF Foundation puts 90 cents of every dollar donated towards educating families, new programs and research for new drugs. Just this week, the CF Foundation release information on a Phase 3 Vertex study drug combined with Kalydeco has had very positive results!! This is where your donations go!!! This is why donations (and awareness) are so important! They're actually going somewhere and accomplishing things!

It's so exciting to hear of new drugs coming down the pipeline to hopefully be available soon for more and more CF patients. Unfortunately, all of these wonderful new drugs are still NOT a cure! I still have to do all of my treatments and take the same amount of medications as I did before I began Kalydeco (plus the two K pills daily). But the Kalydeco has helped keep my lung function stable for three and a half years, and helped me get to and maintain a healthy weight.

Anyway, my friends and family and I will be participating in Great Strides this year to help raise money for the CFF and more clinical trials! We'd love for you to join us! If you're unable to walk with us, and would still like to donate, all information can be found using the links below. :)

DeKalb info: http://fightcf.cff.org/goto/dekalb17

Bloomington info: http://fightcf.cff.org/goto/bloomington17

*All donations are 100% tax-deductible and truly, EVERY DOLLAR MAKES A DIFFERENCE!

Thankful November, Week 3

Each day of November, I'm going to take some time to recognize at least one thing I'm thankful for, no matter how big or small, then I'll post them at the end of the week. Go here for the first week, and here for the second week.

9) I'm thankful for understanding friends. Today, we spent the day with our friend, Tony because he lives about five minutes from where we're doing our CLIMB tomorrow. This evening, another one of our friends, Chris, came up to hang out with us, too. The guys decided they wanted to go out to an arcade bar, and I decided I was going to call it a night - and I didn't get any crap from them this time! They understood that I wasn't feeling well and wanted to catch up on my sleep, especially before a big day and I just really appreciate that! I also love that Tony was asking me all about how I've been doing and everything I've been taking, like my Kalydeco. He works as an athletic trainer at a high school and is very health-conscious (he's completed TWO Iron Man events!). We compared digestive enzymes (mine are way more powerful!) and he asked lots of questions about how K has been helping me. I love educating others more about my CF, and I appreciate that he cares enough to ask. And thank you, Chris, for letting me sleep! :)

10) "I'm alive and well." Today, Tim and I completed the CF CLIMB! And although I'm in the middle of a nasty cold/sickness/infection - whatever it is - I was reminded that I'm still doing well. I'm still well enough to complete a physical challenge such as climbing 58 flights of stairs. I'm very thankful to be as healthy as I am today. Today was also a huge reminder for how much exercise is good for my health. I'm really hoping I can get back into an exercising routine to make myself even healthier. :)
I heard this song on the radio a week or so ago and I can't get enough of it! Here's my favorite part:

"But not me, I'm alive

And today you know that's good enough for me

Breathin' in and out's a blessin' can't you see

Today's the first day of the rest of my life

And I'm alive, and well.

I'm alive, and well." 

~I'm Alive by Kenny Chesney & Dave Matthews~

11) Today I'm thankful for motivation, especially from my CF friends! After posting about our CLIMB, I got a lot of awesome feedback, and it made me want to do more. I miss the feeling of setting a fitness goal and the best part - the feeling I get after I accomplish it. So today I set a new goal and that's to run another 5K. But not just any 5K - this one is the Rock CF River (half marathon or) 5K in Michigan, put on by a great motivator, and fellow CFer, named Emily Schaller. Today I learned that several CFers that I've 'met' online from around the country are all coming out to either run the Half or 5K, and I'd love to join them. So my motivation for this winter is to get my butt back in shape for this race at the end of March! :-)

12) I'm thankful for the Cystic Fibrosis Foundation and anyone and everyone who has ever donated to them! Without your monetary support, I would not have many of the medications that I take today. Nearly 90 cents of every dollar donated to the CFF goes directly to CF research and education. That research (which is extremely expensive) is for all the new medications that go to years of clinical trials before becoming available to patients. Read this article to learn much more about the CFF and how far they've come - it's a great read! THANK YOU, THANK YOU, THANK YOU!!!! You're helping to keep the CF community alive! :-)

13) I'm thankful for a HUSKIE victory tonight!!! GO NIU!!

14) I'm thankful for the roof over my head! I love our new house and we're very lucky to be able to own our own home. It's an awesome feeling to finally not be renting anymore, and to be able to do whatever we want to our house.

15) Tonight, I'm thankful for YOU, that's right, you, reading this blog right now! Without you (the readers), there'd be absolutely no point in blogging. I love knowing that people care to read what I have to say, whether it's about my CF, teaching, or just life in general. I hope that with this blog, someone somewhere is connecting with what I have to say and not feeling like they're the only one out there. I hope that someone somewhere is learning about CF through this blog. I hope that someone has benefited/gotten some advice from any of my posts. Just like the CF Living videos, I do this blog not only to raise awareness about what CF is and how I deal with it, but also to hopefully be that resource that someone needs. I was there once and I was looking for something like this - to tell me I'm not going through this crazy thing called cystic fibrosis alone - and I hope that I can be that for someone else. :)

16) Today, I'm thankful for weekends. Tim and I are enjoying a nice relaxing day filled with errands this morning, a late lunch, and a lovely nap on the couch! It's really nice not having anything planned this weekend, finally, and to be able to spend some relaxing, stress-free time with my husband. :-)

Kalydeco: Day 3

Today I haven't felt much of a difference than I usually feel from day to day. I have noticed that I'm still coughing, but it's not all day long (and it hasn't been since I've started K). The coughing comes in spurts throughout the day (I've noticed it the most a couple hours after I do my morning treatment) and when it comes, it's lots of thick, sticky and green gobs coming out. There was only one gob that came out today that had tiny specks of red, so I'm glad that's getting better. I'll go back to doing my full HTS neb tomorrow with my morning treatment.
Today's evening dose posed a new problem thing to keep in the back of my mind all the time because I've never had to take a regular evening medication (aside from Cipro). Tim and I hopped in the car around 6:15 to head to Menards and as we turned down the first block after our house, I said to Tim, "Oh no, we better be home by 7:00 because I have to take my Kalydeco then." Well, we both knew that wasn't going to happen because Menards is about 15 minutes away and that would leave us 20 minutes to shop - no way! So I decided to just go back around the block to head home and grab it. On the way back to our house I asked Tim to check my purse to see if I already had a protein bar in there, or if I should grab that out of the house, too. He found one in my purse and told me it was 12 grams of fat - perfect! When we got back home, I ran in and grabbed the bottle of Kalydeco and back off to Mendards we went. I felt a little weird eating a protein bar while walking through the store, but whatever. I think I'm going to have to keep my purse well-stocked with protein bars and/or peanuts. And also a cyster recommended keeping two extra pills in my purse for occasions just like this - great idea!

**And finally, I wanted to share a link to this great article from Discover magazine that the Cystic Fibrosis Foundation sent me the link to - it's an awesome, informative read! It discusses how/when scientists discovered the CFTR gene in the 1980s and how cystic fibrosis research has progressed since then. It's amazing to learn how much they've been working on creating medications like Kalydeco and how it's all been funded throughout the years. It makes me appreciate the jobs of researches & scientists, and everyone's fundraising efforts even more!!!

*Oh yeah, and I forgot to add that I've had a slight headache today, off and on. I've also been having some stomach pains and bloating today - that could be due to the amount of not-the-healthiest food I've been eating yesterday and today, so we'll see how that goes tomorrow.

CF Awareness Month Blog #25 - CFF

I would not be where I am today without the Cystic Fibrosis Foundation. Since CF is not a federally funded disease, all research is completed off of the money received from donations. The CFF funds most research that is being done to help cure this disease. On their website, you can follow new drugs as they make their way through the pipeline, which for me, is very encouraging to actually see the progress being made! So many of the drugs that are "To Patients" are ones that I am currently on that are helping to keep me healthy so that I am able to live a normal life. Thank you to anyone who's ever donated to the CFF and thank you to those who are working at the CFF and/or doing the research to help keep me alive.

Positive Thoughts Thursday

Sometimes I feel like I use this blog to vent/complain about things going on in my life, and I don't want to continue with all of that negativity. So each week I'm compiling a list of positive, happy, lucky, fortunate things that are going on in my life, or anywhere in the world.

• HUGE update in the CF world this week! Vertex (the same company that created Kalydeco) is beginning phase 3 clinical trials to combine Kalydeco with VX-809 for CFers with the DDF508 mutations - this is about 50% of the CF population!
• Although I have to reset my compliance count tomorrow, I am very proud of myself for sticking to my treatments for the past year (minus a week)!
• My Great Strides team has raised $135 so far. We're about 6 weeks away from the first Great Strides!

Amazing, Fantastic, Wonderful CF Drug Advancement Today!!!

Chances are you've already heard/read about the wonderful thing I'm going to post about if you're on CysticLife or you're my Facebook friend, but that's okay! It's too spectacular to not read it again!

This is how I feel right now!!

The FDA approved Kalydeco, the new drug that targets the underlying cause of CF in patients with the G551D mutation (ME!) - about 4% of the CF population!!!!! I'm currently 'too healthy', according to my CF doctor, to receive the drug right now, but that is a-okay with me. :-)

Here's what the Cystic Fibrosis Foundation had to say about it. 

Here's what the FDA had to say about it.

Here's what Vertex (the company who is producing the drug) had to say about it.

It's such an amazing feeling to know that all of the clinical trials/studies have definitely paid off! But it is SO important to keep spreading awareness and raising money so that the other 96% of CF patients can see their new medication come through the pipeline and get FDA approval, too. There are several clinical trials going on right now for a combination drug for those with CF with the most common mutation, Delta F508.

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.

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!!!!

A Few Little Updates

How appropriate! Instead of Live, Laugh, Love
it's Live, Learn, Love! 

Well, I'm finally back to work as of Tuesday and it's been great being back. The kids don't start until next Tuesday, the 6th, so the past few days have just been with the staff getting things together. I am very happy to say that I will be working in Kindergarten again this year, half in the resource program and half in the special education program. This is kind of something new for me, so I'm definitely looking forward to it. I am going to be working with some wonderful people (staff) so I'm very excited about that, too. I've attended several workshops/seminars over the past few days that have gotten my 'educator brain' going again and it feels so great, I love it! I'm just looking forward to the kids starting and getting into the full swing of things again. Oh, and I'll definitely be keeping track of some awesome 'kinder quotes' again this year and posting them on here for your entertainment! :-)

As far as my clinical trial debate goes (see previous post), I finally emailed back Spring (the person in charge of the study) today. After talking to several people about my dilemma and getting lots of advice from people, I've decided that I really want to participate in the study, but I really don't want to take eight days off of work. It's not good for the kids, for the classroom teacher, or for me if I'm trying to get a job working in that school district. Someone suggested seeing if I could put off the study until next summer so that I wouldn't have to take any days off work - genius! So when I emailed Spring today, I asked if we could start the study in spring (haha), preferably around May. This way I'm not waiting an extra month til June when school is officially out, but by that time of the school year everyone's usually a little burnt out, so a day or two off before the end of the year isn't always a bad thing. I'm hoping that it's not too long of a wait to start because like I said, I really want to help out the CF community by doing this trial. I will update on here as soon as I hear something from Spring, so keep your fingers crossed that they are awesome enough to wait for me!

Finally, I forgot to mention this last week when I posted about my recent doctor's appointment. I asked my doctor about my gene mutations because I wanted to make sure I had the correct information; turns out I was thinking I had one mutation when I had a completely different one. So you're thinking, so what? Well, it actually makes a huge difference! 

To give a quick little background, to have CF you have to get a mutated CFTR gene from each parent (basically) to have CF. Some of those mutated CFTR genes have been identified and they're starting to do research to see if your gene mutation means that maybe you'll have a milder form of CF, or a more severe form, or more lung issues, or more digestive issues, etc. 

I found out last week that I have one of the less common mutations in the CF world when I was thinking that I had the most common one. So this makes a difference because of this post: One Step Closer! They are doing clinical trials for a drug called VX-770, but it is only for patients with the G551D mutation--which I have!!! I, for who knows what reason, was under the impression that I had a completely different mutation and that this drug wasn't for me at all....turns out I was completely wrong. I learned from the doctor last week that I was tested when I was about 3 for my two mutations (one from each parent) and they could only identify one - G551D. Then, when I transferred to the adult clinic when I was 18, my doctor tested me again, because they had made so much progress with CF reseatch, to see if they could identify my other mutation but they still couldn't. I was given this information after my doctor got the results five years ago, but I must not have written it down or something but I'm so glad I asked again. Of course, I didn't realize all of this until I got home from the doctors appointment, so I don't know what my doctor knows about the drug, but I'll definitely be asking the next time I go (in December)--kind of exciting for me to find all of this out. So, I'll definitely be keeping a closer eye on the progress of the VX-770 since I know that it's something that could work for me if/when it becomes available to CF patients!

Clinical Trial?

Yesterday while I was at my clinic appointment, my doctor mentioned something to me about possibly being eligible for a clinical trial. The study is to compare the antibiotic Tobi (which I'm already on) to a new inhaled antibiotic called Levaquin. Currently Levaquin is only available in IV form, but they're hoping to get it approved in the inhaled for because inhaled meds work much faster (according to the study lady, named Spring!).
I haven't signed up for anything yet, I'm just getting the information today and I'm processing it. Obviously it's something that I need to talk to Tim about because Spring said that we are not allowed to get pregnant while I am participating in the study. I think I can put that off for a good cause.
The study will be about six months long. I will be on either the Tobi or the Levaquin (the doctors will know what I am on, but I don't think I will) for 28 days, then I will be off for 28 days and that counts as one cycle. I will have to complete three cycles in the study. The part that I'm hesitant about is all of the clinic appointments. I will have to do an initial appointment to make sure I am eligible for the study while I'm off Tobi. Then if I'm eligible, about two weeks later they will start me on the drug. The first time I take the drug I will do it at the clinic to make sure that I don't have any negative side effects before they send me home to take it - so we're already up to two clinic visits, in other words, two days off work. Then every time I go off and go back on, I have to go to the clinic and get PFTs, blood work, fill out questionnaires and be evaluated by the doctor. In the end, it adds up to eight clinic visits over the 6 month period. I'm just afraid of taking eight days off work. All of the clinic visits will be paid for by the study people and I will get compensation for travel (and free parking!).
There are just several pros and cons, I'm not sure right now. Obviously, I have time to think about it, the lady was extremely nice on the phone today and told me that I can take as long as I need to think about it. I've always wanted to participate in a clinical trial; I think it's such a great opportunity for me to be trying out a new drug (maybe) that could help so many people!
I'd love to hear your input on what you think I should do, or if you've participated in a clinical trial before. This is all new to me so I'm just processing right now. Thanks for listening reading.

One Step Closer...!!!

Great news for CFers, and all of those who have donated to CFF, today!!! 

A new drug, VX-770, that is in Phase 3 of clinical trials is showing amazing results!! (More information in this New York Times article, too!) This drug directly works with the defective protein in patients with the G551D mutation, which only is about 4% of CF cases....BUT they're on the right track! Right behind this drug in clinical trials is another one called VX-809 which is going to be working with the most common mutation in CF patients (Delta F-508).

Each CF patient has 2 mutations of their CFTR gene (one from mom, one from dad) and doctors can perform tests to identify those mutations. I have one Delta F-508 mutation, and the other is unknown. So, the current VX-770 won't work for me, but it will help about 2,800 people world-wide! The next one coming up through drug trials, VX-809, might work for me to keep me living, laughing and loving!

This is what happens when all of YOU wonderful people make  

donations to the Cystic Fibrosis Foundation!!!!