"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

On your mark, get set, cure CF!

Rain storms are apparently our kryptonite! We were rained out of our kids race on April 25th for Rosebuds.  However, we made sure to run as soon as the rain stopped. 

Cystic Fibrosis can be an overwhelming disease for the patient and their family.  Everyday isn't difficult, but there are many moments during the day that can crush a parent.  We are fortunate that our daughter, Elena, is a champ with her medicines (the whole cabinet full of them).  She is compliant with her treatments (All 1 and 1/2 hours of them).  "Can't" is not a word in her vocabulary.  She views herself as pretty, smart, and confident.  That is what you want in life for your child. Elena has all the support she needs because of the many amazing people in her life. Thank you for being a part of that!

Here are our Rosebuds running today during the brief sunshine!

On your Mark, get set,

GO!

Short legs unite!

And the winner is...

 

 We have encouraged all our team members to run when they can and share pictures.

Here are some of our other Rosebud CF Superheroes running their own virtual race.

Our top fundraisers were the Johnston's Crew pictured above and the Goodrow Gang! 

Way to go Rosebuds!

 

A special award went out to our top and most consistent supporters every year since the first Rosebuds race five years ago.  Thank you to the Goodrow Gang's Nana and Papa for being a huge support in so many ways!  Hope you enjoy our Miles for CF super soft and amazingly cozy team hoodies!

So far the Rosebuds have raised $2.925!  Want to help us break $3,000?  Donate here: https://www.makeyeshappen.com/Athlete/Profile/7433

That brings the overall total of fundraised dollars by Rosebuds CF Superheroes since 2010 up to $69,725.05!!! 

Thank you to all who support our family and our cause to help find a cure for Cystic Fibrosis. Our hearts are full to the brim because of your kindness and generosity!

For our daughter Elena and all her CF friends, we say THANK YOU!

Raining...I mean Running..we HOPE!

The Rosebuds CF Superheroes will be raining...I mean running this weekend! (Please don't rain). 

 11 years ago at this time we held our little gray baby that we thought was slowly slipping away from us because of Cystic Fibrosis. She had already undergone 3 surgeries and was having severe complications. Her kidneys were shutting down, a lung collapsed, her breathing was labored, her intestines were still blocked, and we were numb because if we were not, we would have been unable to stop tears.

Here we are 11 years later and her lung function is up higher than it has been in over 2 years!!!!! Donated dollars get us closer to cure for CF. Please consider donating towards our family goal; helping us be a kryptonite to CF! We are $415 away from our $1000 goal! Thank you friends and family for being there in your words, hugs, thoughts, and donations. I'm continuously humbled by the support that comes in ALL sorts of ways.

You can donate here: https://www.makeyeshappen.com/Athlete/Profile/7433

One week!

One week until Rosebuds race day!  We are getting closer to changing lives of those with Cystic Fibrosis and their families "one mile at a time". 

Elena enjoyed being totally in charge of her Rosebuds table. She sold 9 pairs of her homemade earrings of which profits go towards her CF fundraising efforts at a local race yesterday.

Elena is so brave, strong, inspiring, and energizing!

I am a proud parent as I see how Elena lives her life selflessly. She is being an excellent example for her 3 younger siblings of how to give of themselves to help others using their talents. As if Elena does not do enough for others, she is now taking on a new project to help others. She is working on her plan so I will wait until she finishes it to share with all of you. Let's just say, she is a Superhero for many reasons.

My greatest reward as a mom is seeing my kids show so much love and understanding to others. They are doing good in this world and changing lives not because they feel they have to or because Mom made them, but because they WANT to. 

We will be a part of finding a cure for CF so this big girl can continue to be the awesome big sister that she is.

Join our cause:

www.milesforcf.org

Our next race is April 25th in Lawrenceville at the Gwinnett Braves Stadium for ages 14 and younger. Distance is 1.2 miles and the cost is only $5!

Join us in "beating CF one mile at a time."

A Sea of CF Superstars!

Tonight was a wonderful date night with my oldest boy, Ethan. It began with his selection of my dress, jewelry, jacket, shoes, and even an additional spray of hot pink color on a section of my hair! Then I had specific directions on how to stand and what my face should be doing for our pre-date picture. My little handsome date was wearing his new tux. The kid looked sharp in his tux and Mickey Mouse Converse kicks. Lesson I learned from this; I am no longer in charge of my clothing choices. Ever.

It was a special night. We went to celebrate all the volunteers that do so much to help the Atlanta chapter of the Cystic Fibrosis Foundation be the amazing success that it is.  We were in a room filled with a sea of CF super stars!  It was like walking amongst celebrities. I knew all their names as well as the wonderful things they have done to provide funds that are pushing superfantastic new drugs down the pipeline that will help our Elena and many others like her!  Now I had finally bumped shoulders and shaken the hands of these amazing folks plus hugged many long time friends in my fabulous CF family!

My parents were among the rewarded.  Since Elena's birth and diagnosis, my parents have gone above and beyond to make things happen in the CF community.  To listen to the presentation for each volunteer and see my parents among the greatest of the great was an honor. Did I cry? You betcha! Ask my dear friends and they will tell you I can't make it through a CF event without many a tear shed.

Ethan is the next generation of CF support for people like his big sister Elena.  He knows it, too. 

While on the car ride home, Ethan was planning his first invention. The conversation went like this:

"It is going to be a broom like Harry Potter that flies for real. However, I should be the one to test it. Em and EV are too small, You are too heavy (thanks for that kiddo), and Elena already has CF and I don't want her to also have to deal with a broken bone or something. But until I can do that, I think I'll do a parody to a Bruno Mars song in my new tux. I'll sing about Cystic Fibrosis for my sister. It will be funny because I look like Bruno Mars and I dance good."

Yes, Ethan, if will be funny. Yes, you dance very well. Yes, you will make a difference in this world because you want to help other people.

Wow, I've got my own little sea of CF Super Stars in my home, with my parents, and among my friends.

Want to join the sea with me?

You can donate here: https://makeyeshappen.com/Athlete/Profile/7433

Register your child 14yrs and younger to run a FREE race on April 25th here: http://milesforcf.org/rosebuds/

Register yourself for a 10k on Labor Day here: http://milesforcf.org/sizzler/

Join us in the sea of CF super stars. It feels good; really good.