Drugs and Money: Kalydeco

Kalydeco. It's not a cure, but it's a treatment that is a daily cure.

On January 31, 2012, a year and a half before Crosby was born, the FDA approved a drug for CF patients (ages 6 years and older) who have the specific G551D mutation in the Cystic Fibrosis Transmembrane Regulator (CFTR) gene.

About 4 percent of those with CF, or roughly 1,200 people, are believed to have the G551D mutation. Crosby is not in that 4%. {As I have stated on here before, Lance and I have decided not to disclose what Crosby's mutation's are for privacy reasons. We believe that Crosby's genes are personal to him and any information regarding those specific genes needs to be kept private, unless he decides to share later on in life.}

The CF Foundation partnered with a small drug company and contributed more that $100 million for the research and creation of Kalydeco.

For you chemistry nerds out there, here is the chemical structure of Kalydeco (Ivacaftor)

Kalydeco is a pill that is taken two times a day with fat-containing food. It helps the protein made by the CFTR gene function better and as a result, improves lung function and other aspects of CF such as increasing weight gain.

Kalydeco is the first available treatment that targets the defective CFTR protein, which is the underlying cause of cystic fibrosis. Kalydeco was a breakthrough for CF patients because other treatments only combat the symptoms. Kalydeco is so effective, that CF patients on the pill are able to pass sweat tests (test that confirms CF) and their lung function can go up to 100% without any other daily treatments such as the vest or nebulizer being necessary. It's not considered a cure, since the pill must be taken every day, but it is a daily cure.

Taking Kalydeco results in significant and sustained improvement in lung function. I've read other stories and reports where adult CF patients, who have suffered their entire lives, after a week of being on Kalydeco suddenly exclaim, "so this is what it feels like to really breathe!!"

Kalydeco is effective only in patients with CF who have the G551D mutation, so 96% of the CF population is still waiting for their daily cure. And Crosby is still waiting for his.

There was a really interesting article in the New York Times today about the expansion of CF research. I encourage everyone to read it here: http://www.nytimes.com/2014/11/19/business/for-cystic-fibrosis-foundation-venture-yields-windfall-in-hope-and-cash.html

The CF Foundation presser explaining the receipt of $3.3 billion (BILLION!!) can be read here: http://www.cff.org/aboutCFFoundation/NewsEvents/11-19-Expansion-of-Research-and-Programs.cfm

Do you pray? I do and have for as long as I can remember. I'm sure most of you who do pray can remember a time that you prayed to God and said, "If you just give me this one thing, I will never ask for anything ever again.”  Maybe you were 8 and were wanting a new bike. Maybe you were 19 and wanted that boy or girl to ask you out. Maybe you prayed for a job or for a specific outcome on a test, or approval on a home loan.

I've prayed that prayer before for 100 superficial, inconsequential things. But have you ever prayed that prayer for something so important to you that you really would give up everything else?

My prayer is always the same. I would give anything up, never ask for anything again, please just let my baby breathe and live and grow.  Please just ease his suffering and allow him to have the life he deserves. Please don’t make me attend my child’s funeral. Please let there be a cure for cystic fibrosis. 

Thanksgiving is approaching. If I am thankful for one thing this year, it's the Cystic Fibrosis Foundation. Because of the CFF, one day my prayer for Crosby just may be answered and his Kalydeco may be just around the corner!

Momma Bear and her Man Cub.

Love,

Carmen

Veteran's Day

I often think about our veterans and what they do. 
I think about the sacrifices our military makes.
I think about that kind of courage.
To all the veterans on this Veteran's Day: thank you for freedom you provide me and those I love.

I love watching military reunion videos. The families, the kids, the babies, the dogs!
Nothing will brighten your day like those videos.

I tear up every time I watch one.
Because that's when I think about their families.
The ones left behind.
The ones waiting daily in anxious anticipation for the return of their own personal hero.

My dad was a veteran, but I never had to endure long term separation for active duty because he was out of the Army before I was born.

So on this Veteran's Day, I send an extra heartfelt thank you to military families.
Thank you for enduring what I don’t think I could.
Thank you for being strong.
For us.

These videos get me every time.
Grab a tissue. Or a box.

{Tech note, if you're ready this on email or feeder, you'll have to actually go to my blog website to view the videos.}

The dog ones are especially sweet too:

Love,

Carmen

Halloween, 2014

This Halloween was not Crosby's first, but we really didn't celebrate the holiday last year, so it was unofficially his first!

We had fun going over to our friends' house, Jeff and Carey. I felt a tradition in the making!

The pics speak for themselves...

In case you haven't figured it out by now, we were Forest Gump, Jenny and Lieutenant Dan.

At the party, we did roll Lieutenant Dan's pants up so that he could walk around!

For Lieutenant Dan's hair, I cut up a wig and glued the strips on the inside of the bandanna. Crosby didn't mind the "hair" at all. I think he is so use to my hair being in his face that it just didn't bother him. He didn't tug at it or try to take it off all night! 

Homer, Marge and Maggie (they have enough kids to have had a Bart and Lisa, but the older two didn't want to participate in the family theme!)

Elsa and Ana were fighting over who got to hold Maggie.

Fred and Wilma

We went on a really fun hay ride through Jeff and Carey's neighborhood so that the kids could Trick-or-Treat. Crosby loved being out and watching all the action.

My little bumblebee has had this costume since her first Halloween, 7 years ago! She just wanted it documented that despite her larger belly, it still fits!!

And sometimes, even military officer's need their paci!!

We had a great Halloween. Until next year....

Love,

Carmen

Cold and Flu Season

Dear Family, Friends and Fans of The Crosby Show:

November 1st marks the start of cold and flu season here in the South. Last year, with a newborn diagnosed with a severe, life-threatening lung disease, we were advised to hunker down for the next 5 months, not take Crosby anywhere and not allow sick people or any children in daycare or school to be around Crosby. We were told that viruses can lead to hard-to-get-rid-of infections, and infections in tiny baby CF lungs could have life-long damage. In an effort to keep him as healthy as possible, we followed those recommendations and by the grace of God, we kept Crosby healthy.

Now here we are, a couple of days into a new cold and flu season and I am once again paranoid about germs. Lance and I were at a CF fundraiser this weekend and Crosby's CF doctor from ACH was in attendance. We love him. He asked about Crosby and warned me that they are seeing A LOT of respiratory illness in the ER and on the floor already. He advised to keep him away from sick people and keep him out of public places. At the end of the evening, as our beloved doc waived bye to Lance, he shouted, "keep Crosby away from sick kids!"

The fact that it has been reiterated to us that there is NO such thing as "JUST A COLD" for CF patients, I thought I would write about why it is so, SO important that people not be around Crosby (or Lance and I, or my mom) when they are sick. 

This has been an adjustment for Lance and I. We are social people and hate asking all the time if folks are sick, asking folks to stay away if they are sick, canceling plans if someone has the sniffles, or coming off as complete germophobes. It can be very awkward for us.

I realize that colds are not a big deal for most people. Colds use to not be a big deal to me. Over the past year, Lance and I have had to force ourselves to think in detail about germs and that doesn't necessarily come naturally. However, I will say that I have gotten good at being cautious, after all, I have someone super special to protect and I will do ANYTHING to help him stay healthy and stave off lung damage for Crosby.

For you and I, a cold is just a little cold. We may sneeze, cough and feel achy, but it only lasts a couple of days and we're back to  normal. You and I are able to carry on with life as normal, without missing a beat. Most people even go to work with a cold. However, the mucous in our respiratory systems is the consistency of water. The cold virus in our bodies causes us to produce more mucous than normal, but our lungs are healthy and able to fight off that cold and rid the virus from our bodies within 7 to 10 days. 

Crosby's mucous as a CFer is the consistency of honey. The cold virus in the respiratory system of a CFer causes their bodies to produce more of that sticky, honey-like mucous than normal. Thick mucous hanging around in CF lungs attracts more viruses, lots of bacteria and even fungus. The honey-like mucous traps all those nasties there and they multiply. For many CF patients, catching "just a cold" means spending two or three weeks on IVs to fight off the after-effects. After all that infection, there is also the very real possibility that a CFer may become so sick that their lung capacity is permanently impacted. Colds for CF patients can mean IV antibiotics, extra lung treatments and hospitalization.

Because so many of us have the attitude that "a little cold" shouldn't keep us from going to work or sending kids to school, it is very easy for all kinds of germs to spread. I am beginning to realize just how selfish those actions can be for those who have compromised immune systems or diseases like CF. For those people, a "little cold" could have life-altering consequences. I am also aware that unfortunately, way too many people have bosses or teachers who are not understanding or lenient when it comes to calling in sick. If you find yourself forced to go into work or school while sick, while maybe awkward, wearing a mask is a great way to keep from passing the germs on. As for other things like church, parties, get-together, even going to the grocery store... I do not believe there is any other obligation that is worth spreading germs to kids like Crosby or their care-givers. Please be considerate.

Did you know that a cold is actually most contagious at the beginning, when the sneezing first starts and the nose is running clear? Later, when you still feel bad but you're blowing green goop out of your nose or your child has that green goop oozing out of their nose, you're actually much less contagious than before.  Obviously there are some exceptions to this—some folks have perpetually-runny noses during allergy season, and a runny nose that stays clear and lasts a long time with no other symptoms is probably just allergies. 

So please, please PLEASE be conscientious as we go into cold and flu season. Lance and I love all our friends and we love all our family, but as the Holidays are fast approaching, if you have any symptoms of a cold or the flu and we are going to be in the same place, please let us know. We don't expect everyone to alter plans because of us, but we do expect you to let us know that kind of information so that WE can make an informed decision on where we go and where we take Crosby. It is OUR decision, please let us make it by telling us if someone who is sick will be in attendance. We would certainly never shame or embarrass anyone for a cold, we will simply just choose to stay home. 

• If you are sick, please do not come to our home. 

• If you are sick and are going to be at a get-together where we will be, please let us or the host know. 

• If you come to our home, please immediately wash your hands and use hand-sanitizer. 

• Do not bring kids who are in daycare or school around Crosby during cold/flu season. 

• Do not kiss Crosby or get in his face, whether or not you have any symptoms. 

• And please, GET YOUR FLU SHOT.

We ask that everyone who knows and loves us to PLEASE be sensitive to the other people around you who might have compromised immune systems or have family members who do. 

Your willingness to help keep CFers germ-free might be the only thing standing between them and the hospital this winter.

Love,

Carmen

TSO package for sale, proceeds go to CFF

TRANSIBERIAN ORCHESTRA

I have a Transiberian Orchestra package for sale and all proceeds go to the Cystic Fibrosis Foundation. 

TSO will be in Little Rock on December 6. The package includes a private suite at Verizon Arena that accommodates up to 18 people. You also get:

- Food, Drinks and service

- 15 concert tickets!!!

The package is valued at $3500. I will accept the top offer, minimum bid is $1250. If interested, please email me at sanderscarmen@hotmail.com and I will accept bids until 5:00 pm on Friday, November 7th.

This would make a great holiday office party or Christmas family outing. The package is tax deductible and helps add tomorrows for those suffering from Cystic Fibrosis.