Organ Donor: Be one

We have a friend that is fighting for her life right now as she awaits a new pair of lungs.  This family is so very dear to us.  Their support during our own family sadness has been huge.  Cystic Fibrosis is being very beastly.  Hope is plentiful among this family and her many friends.

Last night I let Elena know what was going on with our friend, Joy.  She said she knew that things were not well.  We talked about lung transplants and what it means to be an organ donor.  Elena asked, "Why wouldn't everyone be an organ donor?" Great question.  Many people just do not know they can be. Then she was quiet for a moment followed by her saying "No one would want my CF lungs."  I said, "No, but they could use other things like your eyes, your heart..." which she said, "I have pretty eyes and an awesome heart."  YES, yes she does.

Cystic Fibrosis, oh what a beast you are.  You have taken so many people on roller coaster rides that were not the fun kind.  You have robbed people of loved ones.  You make people fearful for their future.  You take precious time from people each day as they work hard to defeat you.

Nope. Not in my house and on my watch.  We will take this roller coaster ride, we will embrace each moment we are given, we will not allow fear of the unknown, and we will never stop fighting to defeat you for our daughter Elena and her many friends with Cystic Fibrosis.  Life is short.  There is NO time to waste being angry, sad, lost, mad, scared, disappointed, or confused.  We are so thankful for the people in our life that recognize this and share this mentality.

Get the word out!  Be an organ donor.  Elena is asking you on behalf of all those who may be in need of more tomorrows.

Sizzlin' CF to a stand still

The Labor Day Big Peach Sizzler was today.  This race is organized by and benefits the Miles for Cystic Fibrosis organization.  The first year for this group was 2007.  The Goodrow Gang was there and there we have remained.  We love this group and all it stands for: Raising awareness of Cystic Fibrosis, collecting donations for vital research through the Cystic Fibrosis Foundation, and lending a helping hand to those in the CF community with financial needs through the Reaching Out Foundation ALL while promoting a healthy and active lifestyle.

 

This year we wanted to remind the Big Peach Sizzler 10k runners to think: #whyirunmiles4cf

 

The idea started 5 years ago when I saw a CF dietician running with her patient's names written on her arm to inspire her as she ran.  Her idea would grow into my finding 13 inspirations with Cystic Fibrosis so I could dedicate each mile of my half marathons to one of my CF friends.  The first mile is always the first person I met with CF following Elena's diagnosis (David Adkins...my mile 1 every time!)  The last mile is always my Elena.  She waits for me at the end of the race and I hug her before I finish that last .1 mile of the half.  My CF family has grown and as many of you may know, it takes a village to support our CF families.

 

The Sizzler race is 6.1 miles. 

So we have 6 fabulous CF BIG face signs that were held high at each mile marker. 

 

The runners enjoyed the noise, the costumes, the music, and the reminder of

#whyirunmiles4cf

 

Our mile marker was a muggle free zone.  We represented each house of Hogwarts.

 

Here you have Hufflepuff.

 

Ravenclaw

 

Slytherin

 and...

Gryffindor

 

We enjoyed having our Viking friends join us bright and early for the Mile 1 party palooza!

Mile 1 had a lot a lot a lot of cowbell.

 

The runners just kept coming!  We saw several CF supporting friends run by!

 

The highlight of the day was the Awards ceremony.  We finally met the face behind the magic of the Hogwarts Running Club (www.hogwartsrunningclub.org)

 and his lovely family.  Our CF family just grew a lot more!

 

Hogwarts Running Club was amazing. They surprised us by adding a TWO to the FRONT of our $5,000.00 check...

That's right, they donated $25,000.00 to Elena's team Rosebuds after they heard her story earlier this year.  It was such an amazing surprise that there were very few dry eyes in the group. 

 

THANK YOU HOGWARTS RUNNING CLUB!!!

 

I leave you with a reminder of what Cystic Fibrosis does to our loved ones.

 

CF hoards time due to treatments, steals freedom because of surprise illnesses suddenly sprouting up, thunders through days spent at doctor offices and hospitals, crashes down on family events, sucks dry bank accounts to pay for treatment & meds, and strikes fears into the hearts of CF parents that worry about one day...

 

CF may take away the life of their little one as it has done to so many others. 

Dear CF,

CF, I hope you have your boxing gloves on because this CF family is ready to fight. 

I know exactly #whyirunmiles4cf and you are gonna lose this fight.
Sincerely, a CF mom with a lot of strength and sass

 

CF does not slow this kid down at ALL

Week one of school is behind us.  I'm pretty sure this has been our best first week ever!

We have done a "First Day of School" video since Elena was in Kindergarten. 

Day 2 of school...why not go zip-lining and learn about nature on the hike to the tour start!
Yes, please!  This was AFTER Elena had 10 vials of blood taken for annual labs...how does she DO it?!?!

Day 3 was a home day where we enjoyed the cooler weather and Elena dove right into her school work. 

She is loving the flexibility of being at home and doing her school work in her new grown up bedroom.

 

We ended the day with a family science experiment. 

We did this experiment our own way for sure.

Day 4 we were back at our homeschool CO-OP where Elena taught her very own class!  She did so amazingly well.  I had the honor of being her assistant (although she needed NO help).  Elena is such a mature young lady.  Cystic Fibrosis is no piece of cake to manage.  Maturity helps her manage all she does with such grace.

 

Day 5 was all about exercise! Lungs stay strong when you exercise regularly. This is important for everyone, but especially for those with Cystic Fibrosis.  Elena and her siblings know this, understand this, and are always happy to help with this!

 

 

Days are hectic, but yet they don't feel as such when you are surrounded by so much happiness and great friendship...and coffee.

Mile 1 Dedication to our little grown up girl!

Mile 1 for Elena and all of her giant eyelashes!

This Labor Day we are doing things a little different with the Big Peach Sizzler 10k benefiting Miles for Cystic Fibrosis. Who doesn't  love to get up early and act goofy for a couple of hours? We will be up early on the morning of Labor Day with noise makers, costumes, wigs, cowbells (I've got a fever and the only prescription is more cowbell), and any shenanigans we can muster for a couple hours. Don't worry, we have a karaoke machine. The locals are gonna love us! Why are we doing this?

We want to be the loudest and craziest mile marker on the course! If you would like to be there in spirit, you can make a donation at the link below. My parents got our fundraising off to a great start by getting us only $500 away from reaching our goal (go Mom and Dad!)We have dedicated the first mile in honor of Elena. Our goal this year with the race is to have the runners see faces of those with CF at each mile along the 6.2 mile course so they recognize where their registration dollars are going. 

Link to donate is below:
https://www.razoo.com/story/Elena-S-Eyelashes-Will-Wow-You?referral_code=share

Show Love Under All Circumstances...even when CF weighs you down.

Elena has been making little videos.  Her latest was about her family.  She gets the importance of taking care of herself so she can take care of others.  She understands the value in showing love in all of her actions under all circumstances...yes, even when she is irritated by the daily hassle of her CF routine that doesn't seem to be improving her lung function month after month.

Elena is growing up so quickly and Cystic Fibrosis continues to be a challenge that she hits head on each morning with a smile on her face and her siblings by her side. 

That, my friends, is living life to its fullest. 

As our dog Flanders has taught us all, "Wag more, Bark less".  Trust me on this sound piece of advice.

Whatever...I'm getting cheese fries.

 

For a diet of a CFer, we can all share this sentiment.

As she orders them she requests more cheese...more...a little more...umm more...that's good.

 

Then she does stuff like this and can still hold it down.  THAT is talent, my friends.

 

Sing it sister!

 

 

 

"Ain't No Mountain High Enough"

24 hours; I needed 24 hours to think about what the FDA's approval of Orkambi REALLY means. (read more on the approval HERE).

"Ain't no Mountain High Enough..."

Remember when Kalydeco was approved?  Approval of Kalydeco seemed like a no brainer since the GIANT improvements in lung function were across the board for CF patients. 

What is KALYDECO (ivacaftor)? (as seen on www.kalydeco.com)

KALYDECO is a prescription medicine used for the treatment of cystic fibrosis (CF) in patients age 2 years and older who have one of the following mutations in their CF gene: G551D, G1244E, G1349D, G178R, G551S, S1251N, S1255P, S549N, or S549R.
KALYDECO is used for the treatment of CF in patients age 2 years and older who have an R117H mutation in their CF gene.
KALYDECO is not for use in people with CF due to other mutations in the CF gene. KALYDECO is not effective in patients with CF with two copies of the F508del mutation (F508del/F508del) in the CF gene.

Kalydeco will not benefit Elena because she has two copies of the F508del mutation (which by the way, she has an excellent Harry Potter/Cystic Fibrosis tattoo idea that goes along with her mutation...as seen below!) 


Kalydeco was a major break thru for CF research because it proved that CF could be "put on hold" and even help to improve the CF patients overall health!  It is not a cure, but it is bridge that will buy us time to reach that cure.

So what is Orkambi and where do they come up with these drug names?

Orkambi is meant for CF patients that have two copies of the F508del mutation like Elena!! There are printed studies that show very small improvements in lung function.  The HUGE improvements we see in Kalydeco are not seen with Orkambi.  Yet, this is still a drug that seems to "put on hold" any further destruction of lungs.  Orkambi appears to be another bridge to give us the extra time we need to fight, fund raise, and raise awareness of Cystic Fibrosis. 

We are in the process of checking Elena's eligibility to get a prescription for Orkambi.  Another game changer drug that has been passed down through the pipeline.  The other drug studies behind Kalydeco and Orkambi are mind blowing and more exciting (even MORE exciting is what drug names they will chose next...maybe Bambikalydecambi?  See what I did there?)

"Ain't no mountain high enough" to keep us from fighting for the cure...even this potential price tag.

Thank you to all who have supported our family in so many ways since we discovered Elena's diagnosis.  This is a big event in CF history and I'm thrilled to have you all share in the celebration with us.  Cheers!