Thursday, April 24, 2014

Our 10th Wedding Anniversary

What a weird feeling to be celebrating 10 years of marriage today!  

April 24, 2004 - Photo by Kevin Beasley
I feel like I just graduated college and met Brian. 

Spring 2004 - Picture taken by our friend Lane.
But, how in the world am I suddenly a mom of three children and already celebrating ten years of marriage to my best friend?? 

Fall 2013 - Picture taken by Khimberly Caton from Snaptastic Shots.
Brian and I met at the University of Missouri.  He was on staff with our campus church.  I had just graduated college and was starting my first job as a TV reporter at the CBS affiliate, KRCG-TV.

We started dating in the Fall and it didn't take long before I knew he was the one with whom I wanted to live the rest of my life.  I found Brian to be thoughtful, loving, capable, committed and Christ-centered.  I am thankful to say, ten years later, he is still all of those things and much more.

I won't lie.  We've worked hard for these ten years of marriage.  Our first fight as a married couple was on our honeymoon.  Our last fight was just last night.

But the beautiful part of marriage, we have found, is our fights always end is reconciliation and a deep commitment that no matter how tough life gets, we're doing it together.

We knew when Bennett was diagnosed with cystic fibrosis, we wanted to particularly pay close attention to our marriage.  So, we have made marriage counseling a healthy part of our relationship. We also have continued, through much of our marriage, weekly date nights so we can get away and connect.

There have been times when I have loved the heck out of my sweet husband.  There have been times when I have not.  But I am incredibly thankful for the person that Brian is.  He is a fabulous father, a loving husband, an incredibly insightful and smart man and the person whose opinion I most value and respect in life.

Spring 2012 - Photo by Khimberly Caton from Snaptastic Shots
Brian and I have lived in three states, had three children, owned two homes, cared for one dog and enjoyed 10 years together so far.  Cheers to ten more, Brian!  Happy Anniversary!

Sunday, April 20, 2014

Wednesday, April 16, 2014

VLC 2014: People I'm Privileged To Work Beside

This is my last blog post about the CF Foundation's 2014 Volunteer Leadership Conference...but it might just be my favorite one.

The following pictures are of just some of people I met at VLC and I am privileged to work beside in our quest for a cure for Bennett and all those with cystic fibrosis...

Bob Beall (CEO of the CF Foundation), myself, Brian and Dr. Preston Campbell (CFF Executive Vice President of Medical Affairs)

Paige (my co-chair of the Waco Great Strides CF Walk), Amy (our CFF Northeast Texas Executive Director) and me

Joe Flacco (you know, from the Baltimore Ravens, who has a 3 year old niece with Cystic Fibrosis) and Erin (CF mom of 4; one with CF)

Kathy (a CF mom to a sweet little 6 year old girl with CF), me and the band "Branch and Dean" -  Steve Branch (left) and Dean Scallon (right) performed at VLC and did an amazing job.  See their hit "The Dash" (written about Steve Branch's son who died of CF last year):

Catherine (another CF mom whom I originally met on Facebook); She's pretty nifty because she has a salt room at her house for her son with CF

Mary Weiss, a CF mother who is known for her son coining the phrase "65 roses," his way of saying "cystic fibrosis."  Unfortunately, her son died this past year from CF.

Love love these girls!!  Angie and Mary (both CF moms)
Angie was one of this year's VLC co-chairs and has two daughters with CF.
Mary is mama to two little boys with CF.  Read Mary's blog here:

Rebecca and Brock (CF mom and dad to a son with CF); See Rebecca's blog here:

Rebecca (CF mom to a little girl with CF); see Rebecca's blog here:

Erin and Martin (CF mom and dad); See Erin's blog here:

Margarete and Mark Cassalina (CF mom and dad of two children with CF, one of whom is living.  Margarete is the author of the book "Barely Breathing")

Cory and Jennifer (CF dad and mom to two little ones with CF)
I was super excited to meet Cory and Jennifer again.  I had originally met them while at a CF function in Fort Worth.  But, then, our connection grew stronger when we realized we share several mutual friends in Texas.  Big state, Small world.

Of course, my favorite person I'm privileged to work beside: my husband and Bennett's daddy.  I feel like the luckiest girl.

Monday, April 14, 2014

VLC 2014: Foundation Strategic Plan Update

Outside of the "State of the Science" Session at the annual Volunteer Leadership Conference, each year, I look very forward to hearing the CF Foundation's"Strategic Plan Update".  

I take very seriously my responsibility of fundraising to find my son a cure.  So, I regularly want to make sure the money being raised is being used in the most effective way possible.  It is during this session each year that we volunteers get an update from the Foundation and learn how money is being used.

Rich Mattingly, the CFF's COO, gave this year's "Strategic Plan Update," as he usually does.  I really like Rich.  He is very good at what he does and seems to really appreciate the struggle that those with CF go through and the job we as parents do each day.  I went up to thank him for his work with the Foundation.  And he thanked me for mine.  :)

Rich shared this slide, which underlines the very important parts of the Foundation's new mission statement.  Rich explained that much thought and care had gone in to this statement.  

Rich shared how the Foundation has put a lot of effort towards a Strategic Planning process to help guide the Foundation in the coming years.

This is a list of the Foundation's new objectives going forward.  The Strategic Plan committee came up with these objectives.  I found it helpful to see what kind of focus the Foundation has right now.

This is a list of the parameters the CF Foundation have put on themselves going forward.  I thought the parameter stating no CFF funds will be used to pay directly for patient care was really interesting.  Rich explained that they "have found under no circumstance and under no model has it been beneficial to pay for medical care directly for patients of any disease."

These are 3 of the 6 strategies the Foundation is using to accomplish their mission statement and objectives.

This slide added #6 faster than I was able to capture it on my camera.  But #6 mentioned the inclusion and desire of help from the adult CF community. Rich said, "We will make sure that the voices of CF adults are heard."

This slide demonstrates the Foundation's research priorities.

These are the revenue priorities of the CF Foundation.  I hear there are some great new changes for the Great Strides program coming!!

One of the things that was spoken about a lot at the conference, including during this session was how to make the needs of the CF adults a priority.  Rich said, "We will make sure that the voices of CF adults are heard."

With that in mind, the CFF announced a new campaign they are working on called "My 21,000."  Twenty-one thousand breaths is the number of breaths per day.  The social media campaign will be spearheaded by CF adults in partnership with the CF Foundation in order to encourage fund raising and awareness for Cystic Fibrosis.  I'm really excited about this campaign.  I think the CFF is very plugged in to what the CF community needs in way of resources.  So, it is exciting to see them start this new campaign.

I was also glad to see how the CFF is responding to the CF adult community.  The CFF's new infection control policy does not allow CF patients to attend indoor CFF events.  This has been heartbreaking for our CF community.  So, I am glad to see the CFF work to find new ways to include them.

This year, the entire VLC program was live streamed so CF adults could "attend" online.  In addition, there was a Q/A panel of CF adults during one of the sessions that was really good.  2014 may be the first year with more than 50% of the CF population over 18 years old.  The CFF wants to reflect this change in CF population by making CF adults more present at CF events and activities such as VLC, which is fabulous!  I want there be a place for Bennett when he is an adult.  

The changes at the CFF demonstrate that we are slowly making progress with CF.  Cystic Fibrosis is becoming less and less of a childhood disease.  Each year, I can have more an more hope for Bennett.  I like these changes.

Saturday, April 12, 2014

VLC 2014: State of the Science Update

I was honored to be invited to attend the Cystic Fibrosis Foundation's Volunteer Leadership Conference in Bethesda, Maryland again this year.

I tried to capture as much of the information as possible to blog about for the benefit of other CF families.  So, sorry in advance for the crazy amount of slides! :)  

I have divided up my experience at VLC in three different blog posts.  The first one is from the "State of the Science Update."  Always, my favorite!!

Bob Beall, Ph. D., President and CEO of the Cystic Fibrosis Foundation addresses the crowd to welcome us to VLC 2014.

This room was full of people who are determined to do whatever it takes to find a cure for CF.

Michael Boyle, M.D., FFCP, Director, Adult CF Program/Associate Professor of Medicine @ John Hopkins Hospital (seated left), Bob Beall (middle) and Preston Campbell, M.D. is the CFF's Executive Vice President for Medical Affairs (seated right) gave the State of the Science Update.

This slide shows major projects of the CF Foundation over the last 50 years.  As the slide shows, it is in the last decade that the CF  Foundation has been focusing on therapeutic development (the development of medications).

If we break down the Therapeutic Era, we can see that it has been in the last 4 years, the CFF has worked on First Generation CF Modulators.  The top three arrows show the focus of the CF Foundation of developing therapies in the next 15+ years.

With regard to expanding Kalydeco to other mutations, the CF Foundation said "probably 15% of population will benefit, hopefully, by 2016."

This slide shows how effective Kalydeco has been versus some of the other therapies.  It is really amazing to see how much more effective Kalydeco is, compared to the medications many CF patients use daily such as TOBI, Azithromycin and Pulmozyme.

This slide shows that scientists have seen significant change in psuedomonas for those who have been on Kalydeco.   This is super promising for all of those with Cystic Fibrosis!  We have typically thought that once a CF patient gets psuedomonas, they cannot get rid of it.  But now, with Kalydeco, there is real hope that even though one might acquire psuedomonas, they might be able to get rid of it.

This slide shows how Kalydeco has demonstrated to positively affect lung function, even after nearly three years!

What about the most common mutation, F508del?   Fifty percent of patients carry two copies of the F508del mutation and 90% of patients carry at least one copy.  We are looking for a combination therapy of VX-809 and Kalydeco (VX-770) to work.  At this time, it shows that although it does not work as effective as Kalydeco does for G511D, but the combination drug still shows very positive results!

This slide shows some of the Phase 2 clinical trial results for those taking VX-809 and Kalydeco.

At the conference, on our table, were some postcards with a distinct message from the CFF.  I thought I would share these messages here...

"Phase 3 Combination Trial Core Messages:
* We know there are high expectations in the CF community about the Phase 3 combination trials of ivacaftor (Kalydeco) and lumacaftor (VX-809), which are being studied in people with the most common mutation of CF, F508del.  However, drug development is a lengthy and unpredictable process.  The trials are still underway and it's too early to speculate about the results.

* The research process for a new drug (or combination of drugs) must follow the gold standard for how clinical trials are run, evaluated and reported to the medical community and the FDA.  This process is essential to ensuring that the new drugs are proven to be safe and effective when used in the broader population.

* This combination trial is only the first step in finding more therapies to treat the most common mutation of CF.  Based on what we already know from laboratory studies and earlier clinical research, we'll likely need to continue to improve the effectiveness of these therapies and perhaps use more than two therapies to achieve the full beneficial effect we all desire.

* The CF Foundation is fighting CF from ever angle and not relying on any one potential therapist to win the battle.  The Foundation is actively pursuing the development of other promising compounds with some of the best biotech and pharmaceutical companies in the world, including Pfizer, Genzyme and Vertex."

We hope to know the results of the Phase 3 VX-809 and Kalydeco clinic trials later this year.  The trial will last 6 months and includes more than 1000 patients.  There is hope that by this time next year, these two medications will be available for those with two F508del mutations.

What about patients that only have one copy of F508del?  This is where Bennett is and the place we are most interested in.  Phase 2 trials are underway with both VX-809 and VX-661.  There is hope that this will come along in 2016.

This is a great slide.  It shows all of the current pharmaceutical companies working towards finding a cure for CF.  When I first attended VLC 3 years ago, there were only a few of them.  Now, the list has grown.  That means we have even more reasons to be hopeful.  Others are seeing the financial potential and possibility of success.  For me, that means a cure is coming!!

This is the CFF lab in Boston.  See more about it here:

The CF Foundation is exploring new technology.  (I apologize.  My notes got lost on the way home from the conference so I don't have specifics on this slide.  But, when I find them, I'll post up more information here.)

While Kalydeco is able to help only 4% of the CF population right now, the CFF believes that Kalydeco in addition to a corrector medication will ultimately be able to help 95% of the CF population.  That will leave a remaining 3% of those with nonsense mutations and 2% without nonsense mutations.

So, what about nonsense (X) class 1 mutations?  The CFF is determined to leave no one behind.  Although Ataluren showed poor results in its last clinical trial, there is a belief that inhaled medications may have caused Ataluren to be less effective.  So, a new phase 3 trial is planned to start this year!

There are new screening programs for nonsense mutation therapies.  This is very hopeful news for those with CF whose genes end with an X.

The CFF wants to remove those X's (fix the genetic mutations) on the DNA.

Of course, even though the CFF is focusing on a cure, attention to other areas such as addressing CF symptoms and the unmet need of CF antibiotics has not been lost.  There is a need for antibiotics that treat some of CF's worst culprits: pseudomonas, B cepacia, MRSA and mycobacteria.

At the end of the session, someone made a comment to the presenters that they were disappointed by the update.  The person indicated they wanted someone to "wow" them with the news.

Dr. Campbell explained that there was news to be "wow"ed by, but he cautioned, "it would be irresponsible for us to promise success without bumps along the way...we are not making an Egg McMuffin here."

Bob Beall added, "This is not an easy problem.  We're curing a genetic disease!"

But, Dr. Boyle said, “It’s an exciting time. We realize there are challenges. We will get there; it’s just a question of how long.  The key to improving the lungs is fixing the underlying problem. Ivacaftor does that. It’s a wow!"