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!

Why Me?

These are the questions I will sometimes get when people find out that I have a child with a genetic disease. 

• Are you mad? 
• Do you feel you have been burdened with a giant heavy weight in life? 
• Do you ever think, "This isn't fair."? 
• My personal favorite is that people assume I walk around saying, "Why me?"

My oldest when she was only a few days old.

My first thought to all these questions is...why are you asking ME?  You should talk to the person that HAS the genetic disease.  SHE is the one with all the "burdens" you are referring to.   I can honestly say that from the scary moment when our oldest child was born sick, through the numerous surgeries, and then the Cystic Fibrosis diagnosis; I didn't waste time wallowing.  My boot straps were pulled up and boxing gloves were on snug.

My oldest all grown up and wearing my clothes...that's just crazy.

However, here are my responses to these questions.

Am I mad?
Mad at what, whom, why?  I have nothing to be mad about.  I have a husband that loves me and our 4 beautiful children, one who happens to have a life shortening genetic disease.  What is there to be mad about?  I embrace each day and look for the good.  My sister has always said I am a Pollyanna; always looking for and finding the good in things/situations/people.  Guilty as charged; It's true.  Why see bad things when you could see good things?  It's simple; good things make you happy and bad things do not.  I want to be happy.  Do I still see the bad stuff?  EVERYDAY the bad stuff is there.  Bad stuff will swallow you up if you allow it.  Yet, there is always a sparkle of happiness.  I see it and you can, too.  ALLOW yourself to see the good in others and eventually they may choose to return the favor.  Life is full of tough situations and difficult relationships.  There are plenty of opportunities where the wrong words are spoken and the feeling of love and respect seems lost.  YET, goodness is still there.  Once I talk through my sadness on life's tough moments and feelings of loss...the sparkle of good shines again.  Go ahead, be Pollyanna...you know you want to:)

Good friends by your side will make the "Pollyanna Effect" easy to maintain.

Do I feel I have been burdened with a giant heavy weight in life?
Cystic Fibrosis, breast cancer scares, surgeries, lost relationships, lack of forgiveness, miscommunications, car accidents, health issues...how could I pick just one heavy weight?  Instead, I have chosen to not carry any weights with me.  I choose to carry around smiles, giggles, adventures, and joy. 

There are times when I can't find those things just like my car keys the dog has run off with or my phone that I can't seem to keep track of unless it is strapped to my forehead. 

Then one of my kids will run over and kiss my cheek saying I am the best Mommy ever or my husband will send me a text from work saying he appreciates me so much.  Ahhhh, yes, there is the joy and giggles that were lost for a moment and then found again.  Choose to look for and carry around that happy and drop the sad like it was hot. You owe that to yourself and your family.

Do I ever think, "This isn't fair." ?
Why would I ever waste my time thinking this?  Friends have sickness, other genetic diseases, cancers, and major health problems they juggle everyday. There are people all over the world starving to death, without homes, and without fresh water. There are friends out there that have lost loved ones too soon; parents, siblings, or children in utero, at birth, as young toddlers, children, teens, and adults. They hold onto their memories and I get to hold onto my husband, my kids, and my parents. Yet, I am going to complain my daughter has a genetic disease that MAY take her too soon?  I don't think so.  She has access to amazing medicines and doctors at any moment we need them to help her with her complicated health needs.  My life is nothing to complain about...ever.

Do I ever ask, "Why me?"
I have not and will not ever ask this question of myself.  Do I wish my child did not have this health issue to complicate her life?  Sure!  No one would wish heartache and health issues on someone they love.  I do not think I was given a child with special needs because I could handle it.  I think ANYONE can do ANYTHING for their children when they have love in their hearts.  Some will do better and/or more gracefully than others and we will all think that we are failing in some way, but parents are always going to try their darn hardest for their children. 

You should see the trend here.
(If not, my English professors told me that a journalist/author I am not.)

A very wise and beautiful Aunt told me, "Live with NO regrets." 
Life is simple yet we chose to make things complicated. 
K.I.S.S (Keep It Simple Stupid)
Show love to people around you. 
Find joy in as many things as you can during your day.  Make it a game.
Don't carry the weight of sad/bad/mad when life is so short. 

Yes, life is difficult, yet you are still alive...marvel at that and be thankful for it.  You still have time to love on the people around you.  Yes, you can mope; but while moping, be searching for that sparkle of good while taking the moment to squeeze your family tight. You have this moment right now and once it passes, it is gone and you can't take it back.

Sooooooooooo........Why me?  Because I am worth it.  I am worth fighting for.  I owe it to my family to take care of myself so I can take care of them.  I deserve joy and loads of it so I can spread that goodness around. YOU DO TOO!

P.S.  Elena's health is doing very well as you may have guessed from her pictures above.

This Post is Brought to You by the Letter "E"

E is for:

 

Example: she leads by example with her siblings and all who she come in contact with.

 

 

Explorer: She is the first to create or join in an adventure.

 

Energetic: She can make the little things exciting! (oh, there is another E word!)

 

Extraordinary:  Give her a little idea and she will RUN with it and it will be completed in a way that makes you go...WOW!

 

Elaborate: NOTHING is simple with this one.  Her stories, plays, skits, shows, explanations...all are EXTREMELY Elaborate and we love it. (unless we are in a hurry, then we ask for the condensed version)

 

Elegant:  All she does is with grace and style.  I hope to be like her when I "grow up". 

 

Emotional: Things are super tough for her sometimes.  However, she can handle the difficult times with ease.  Usually, she handles her health difficulties much better than her mama and I'm ok with that!

 

Eccentric: Theater and Acting is her JAM!

 

Efficient: We are a homeschool family on the go a lot and she does her best to be compliant in her treatments.

 

Exuberant:  She will use her positive "force"

on you when you are near her.  Watch out!

 Edgy: She wanted a pet tarantula so she could have her own pet that people would think was weird...she met both goals on this one.

 

Extreme: She will ride roller coasters one after the other until her Mom's face is purple and Mom is saying "Uncle."

 Extraordinary:  She teaches her self more than I could ever teach her.  Life experience has matured her so much and she is only 11!  She graduated from Elementary school this year...middle school... really?!? 

 

Effervescent:  She is spirited, bubbly,  and full of the essence of life on so many levels.

 

Empty is what my life is NOT with Elena,

the three little E's, and my husband around;

EVEN with the hardships of Cystic Fibrosis.

 

Elena

 

There are many reasons why we

walk, run, volunteer, donate, fight, persist...

to End Cystic Fibrosis and make it stand for Cure Found so Elena's Exemplary life can go on and on till she is a 100 plus!

There is still time to donate towards our team at: https://www.makeyeshappen.com/Athlete/Profile/7433