Waves of Generosity - ALS

There is a mass movement happening.  Have you been swept up in its powerful current?  Have you dumped a bucket of ice water over your head?  Donated to the cause?  It's changing the face of the fellow orphan disease called ALS.  It's giving hope to those who suffer, a voice to all those who have been silenced because of the horrific disease, and celebrating the memory of those who have been lost.

Look how one small action has created a mass movement of good.  Look at what people are capable of doing, the fires of change they can ignite.  The generosity and kindness of people are truly amazing.  That single spark can create a better life for all those who suffer, maybe even a cure.

Sea of Support

Living a life bound by the high and low tides of an orphan disease [CF], I understand how incredible and humbling it can be to have family, friends, and complete strangers fight for your cause.  I think it is so incredible what the power of human compassion, empathy, and caring can do: what it can accomplish and the lasting impact it can have on an individual.  Speaking from experience, it is honestly life changing.  The buckets of ice may start to slow, but the lasting effects of people's giving spirit and generous support will last forever.  It's not the dollar amount I remember when I look out at the sea of support I have behind me, it's every single face, personal note, and act of kindness.  As for now, ride this wave, and keep the ice coming!  

Just like CF, ALS steals many mothers, sisters, uncles, dads, and best friends far too soon.   It steals dreams.  It steals hope.  It steals a future.  This is why it means so much to me to show my support to other causes besides the one that directly impacts my life.  The war can only be won if we fight together, no matter the disease.  In the end, regardless of the cause you choose to support, do it with your whole heart. Whether it is CF, ALS, Breast Cancer, or Heart disease, we are all in this fight together: a fight for tomorrow. Your support and voice gives each person impacted a hope for tomorrow and the strength to fight each battle, no matter the disease.  Generosity and kindness are powerful forces: share it unconditionally.  Love to you all. 

I challenge each of you to donate $25 to the cause of your choice.  Remember, we are all in this together. 

Generosity

How do you show gratitude? Do you send a card? Flowers? Give a gift? A hug?

Today I am struggling. Struggling to find the words and actions to show how truly grateful I am.  How do you adequately thank someone for literally giving you another breath? For giving you the gift of life?  Not just watching from the sidelines, but actually fighting in the game?  

This morning was the Great Strides Walk in Sioux Falls.  It took all I had this morning not to be a complete emotional hot mess.


Emotion
I still am on the verge of being overcome with emotion. I still feel that lump in my throat and my eyes at a single thought of this

morning start to well with tears.  What amazing people I have in my life, and it all started with a single step several months ago by my dear friends.  I could have never imagined the emotion, the people, the support, and love that has led up to today.  How can I possibly ever thank everyone? How can I show all of you how much you truly mean to me?  I think I will go my entire life searching for the right words and suitable actions to show my gratitude.  Nothing will ever be enough.

The Act of Kindness
Today's walk was truly beautiful. Team Ashley for Sioux Falls raised over $6,OOO. Wow. This leaves me absolutely speechless.  The generosity of people has been overwhelming. All I can offer is thank you and my love to each of you.  Your generosity, love, and, support mean more than you will ever know. The event itself raised over $156,OOO.  That is absolutely amazing.  That could be the cure.  That could be one more beautiful breath. That could be another year with friends and family.  That could mean dreams.  That means hope.

There is so much good happening in this beautiful life. Thank you, from the depths of every brave breath for walking, donating, and showing endless love. All the love I possess to each of you.

How do you show your gratitude?

Priceless

How far would you be willing to go for a chance at one more breath?  To ensure you had one more chance at sharing laughter, tears, and making beautiful memories?  Would you spare no expense if it meant staying alive?

I am so truly privileged to have good health insurance and for the specialty CF programs that allow me to get the medications needed to fight CF and grant me every extra beautiful breath.  I am so grateful for my CF team that ensures I have the capability and access to what I need to fight CF: they spend countless hours dealing with my insurance company, enrolling me in or finding out information about specialty programs, and managing and adjusting my prescriptions.   But CF still comes at a high cost.  I am so very lucky to have Mark who never questions the financial burden of CF, but sees every therapy and treatment as another day together. 

By February 1st I have usually reached my insurance's deductible and out of pocket max from prescription drug costs and doctor visits.  Every January I know instead of booking a nice European vacation, I will be buying a chance at one more breath.  

The cost of 3O days to fight CF [prescription drug & therapy costs]

      Cayston.......................$6,786

      TOBI...........................$8,O12

      Pulmozyme..................$2,843

      HyperSal......................$62

      Prednisone....................$2O

      Amicar.........................$85O

      Voriconazole................$7,O15

      Mephyton.....................$349

      Pantoprazole.................$55

      Albuterol.......................$5O

      ProAir...........................$15O

      Azithromycin................$186

      Atenelol.........................$1O

      Cipro.............................$3O

      Bactrim..........................$15

      Enzymes........................$3,2OO

      Mucinex D.....................$4O

      Zantac............................$25

      Vitamin D......................$15

      CF Vitamin....................$15

      Dulera............................$25O

*This does not include the cost of IV therapy or hospital visits/ stays

Kalydeco, a new and promising therapy that corrects the underlying cause of CF costs $3O,OOO for a 3O day supply. [This medication is only effective on 4% of people with a certain CF mutation]  What would you be willing to pay to cure CF, to never worry about CF stealing another day?

 Cost of equipment and other therapies

      VEST.............................$15,OOO-$2O,OOO

      Nebulizer........................$15O

      Acupuncture...................$65/time

      Neilmed...........................$15

Travels costs for doctors appointments in Minneapolis & Sioux Falls per year.

      Hotel, Gas, Food............$2,OOO

This is my life, and I will do everything I can for one more beautiful breath.  I am so thankful for my CF team, Mark, and my family who have always done everything possible to ensure I have access to the best medical care and therapies: I owe every breath to you. The cost of living with CF? Roughly $3O,OOO a month for just prescriptions.  The chance at living? Priceless.  Love to you all.  

What are you willing to pay to live another day?

      

      

Hope for Tomorrow

A lot can happen in the course of a day, a week, a month, a year, or a lifetime.  I often think about the things my grandparents have seen change in their lifetime: electricity, cars, farming, indoor plumbing, the advancements in medicines and vaccines, and means of communication.

The advancements in CF treatments have made it possible for me to still be breathing.  There is so much hope for the future.  Each day is a new chance at a life saving breakthrough. 

Yesterday's blog post was Part I of II entries by pharmacist, Stacy Peters, again to whom I am so grateful.  She is constantly researching new therapies and is on the forefront of CF drug development.  In Part I, she discussed the defective protein in CF and its complex genetic challenges.

The Future and Hope for a Cure

In 2O12 a breakthrough oral medication called Kalydeco was released by the FDA.  This ground breaking new drug targets the underlying cause of CF for people with the mutation G551D: only about 4% of people with CF are eligible to reap the benefits of Kalydeco.  Even though I do not have the right mutation for this miracle drug, it gives us all huge hope in the fight against CF and the future. There will come a day when CF no longer steals anyone's breath. 

Here is Part II written by Stacy Peters:

"A new class of medications referred to as CFTR “modulators” has been in development for the last several years.  CFTR “modulators” work by:  1) increasing function of the CFTR protein at the cell surface (i.e. Kalydeco), 2) transporting the CFTR protein to the cell surface (i.e. lumacaftor or VX-661 – currently in clinical trials), or 3)  help the body “overlook” errors in the DNA that make the CFTR protein (ataluren – currently in clinical trials).  Unfortunately, since there are different reasons for why the CFTR protein/gate doesn’t work, there isn’t a “one size fits all” medication for everyone with CF.  While not a cure, the advantage with this class of medications as a whole is that they target the underlying defect in CF, whereas other treatments such as Pulmozyme® and TOBI® all target the aftermath such as the thick mucus and bacteria in the airways.

Kalydeco (Ivacaftor) is currently the only CFTR “modulator” approved, it works for people with a mutation called G551D and other class 3 mutations (only ~4% of those with CF).  Kalydeco works by activating the CFTR channel or “gate” and helps normalize water and salt transport.  Since it only works by activating the “gate” on the cell surface in a very specific way, it doesn’t work for those who have other classes of mutations. 

There are several other CFTR modulators in clinical trials.  Some are using 2 drugs to attempt correcting the CFTR protein.  For example, in people who have delta F508, the most common mutation, there are 3 new medications being studied.  Lumacaftor in combination with Kalydeco, VX-661 in combination with Kalydeco, and N6022 which is in very early development.  The lumacaftor or VX-661 works by moving the CFTR protein to the cell surface, then Kalydeco will come in and open the gate. 

Ataluren is also in clinical studies for those with class 1 mutations.  It works by causing the cell to “overlook” the error in the mutated CFTR gene, allowing for the CFTR protein to be made. 

The goal of the CF Foundation is to ensure there is a CFTR modulator for EVERY mutation.  This will be quite a challenge given the variety of mutations out there.    

While CFTR modulators are all the rage in CF research, there are other very important medications and treatment approaches being evaluated.

·       New inhaled antibiotics to help suppress bacteria such as pseudomonas and MRSA.

·       New anti-inflammatories targeting inflammation in the airways and body.

·       New delivery devices that decrease the time it takes to nebulize medications.

·       Evaluation of existing therapies to determine if there are ideal combinations and treatment durations to maximize the effectiveness of the current approved medications.

·       For more information visit:  http://www.cff.org/research/

·       For more info on gene classes: http://www.cff.org/aboutCFFoundation/Publications/connections/archive/March2010/Targeting-Mutations-that-Cause-Cystic-Fibrosis.cfm

While there are no guarantees that medications in clinical trials will be proven effective, the rapid advance in technology and progression through clinical trials is promising."

New developments in treatments and the fight against CF are crucial, not only for the daily fight against CF, but for the discovery of a cure.  Treatments that have extended my life thus far are losing their effect: my CF is becoming resistant and less responsive to treatment.  The advancements in my lifetime alone have been truly amazing, and I cannot wait to see what the future holds.  Again, thank you to Stacy for sharing her amazing gifts making it possible for us all to breath.  I am so grateful to each of you who so passionately have fought and continue to fight to add tomorrows for everyone with CF.  I wouldn't be here without you.  Love to you all. 

What changes have you seen in your lifetime?

The First Step

"Sometimes the smallest step in the right direction ends up being the biggest step of your life.  

Family & friends at St. Peter Great Strides

Tip toe if you must, but take the first step. " - unknown

The fight against Cystic Fibrosis and the advancement in treatments all began with a single step.  That single step has affected thousands of people and lives giving hope to families, friends, and people with CF. 

I sang the national anthem before the walk.

ORPHAN

Cystic Fibrosis is an orphan disease which means funding for research is only possible through self raised funds for research.  There are no national or government funded programs for research and development for a cure.  All of the life saving treatments developed to fight CF have only been possible because of donations from people like you.  We must financially support our own endeavor to find a cure and new life extending treatments.  I owe my life and every breath to those who have given support, literally.  Every step I take, is because of the generosity of strangers, my family, and dear friends. 

Andy & me

St. Peter Great Strides

Yesterday was the Great Strides Walk in St. Peter.  It was an amazing sight to see so many people come together for CF.  Each of their lives had been touched by CF in some manner.  The morning was beautiful for a 5K walk, and I lived each glorious step from start to finish.  The St. Peter Great Strides raised over $45,OOO for the Cystic Fibrosis Foundation to go towards research and drug development.  People are amazing. You, are amazing.  That money donated might just save my life.

Kendra & me

Endless Love & Gratitude

The steps you have each taken to fight CF, means hope for another tomorrow.  I cannot possibly thank you enough for the love and support.  Thank you to one of the dearest people in my life, Andy, who so passionately took on this cause and the St. Peter Great Strides Walk.  The greatest of love to you, Andy & Kendra, you are always there ready to take each step with me.  Thank you to my family and dear friends who came out to walk with us in St. Peter: it means more than you will ever know.  I am so grateful to each of you that donated.  Thank you to all the volunteers who made the event happen, and the people of the Cystic Fibrosis Foundation MN/Dakotas chapter who so enthusiastically fight for us every day. Your donations, love, and support are helping add tomorrows, and helping give breath to each life.  Love to you all. 

Kirby showing his support to add tomorrows.

Where is each step taking you today?

There is still time to donate to the St. Peter Great Strides, or there is another chance to walk with us May 31 in Sioux Falls! 
Click to sign up or donate here:
Donate to St. Peter Great Strides

Register to walk or donate to Sioux Falls Great Strides
Saturday, May 31 @ 1O a.m. Spencer Park

What's Right

This blog is dedicated to the health I do have and being so grateful.

CF has infiltrated my body, plaguing the natural function it so desperately longs to enact.  Despite CF leaving parts of my body ravaged, I am still so thankful for the body I do have that works so hard for me.  Why is my life so beautiful?  My eyes can see, my ears can hear, my heart beats for the people I love, and I am still breathing. 

We often get stuck focusing on what is wrong with ourselves, forgetting about all the things that are so right.  I am so thankful right now that my heart is strong, my kidneys and liver are working justly, and the muscles of my body and mind can do anything I set my mind to, even after all being repeatedly assaulted by CF. 

The Heart

Because of the lack of oxygen from complications associated with CF, there is extra stress placed on the heart and its responsibilities.  Medications can also affect the rhythm of the heart, causing an irregular heartbeat.  My blood pressure is monitored closely, and an EKG is done periodically to catch any abnormalities that might be happening.  My heart?  Beating strong and fiercely for life. 

The Liver

The nature of producing excess mucus because of CF extends to the condition of the liver.  Scarring as a result of inflammation and plugged ducts can make it difficult for the liver to function. A great concern is also the effects of some specific medications that are merciless on the liver's function.  LFT [Liver Function Tests] are done monthly to make sure that the Liver is not beginning to betray me.  My Liver?  Still holding its own and taking whatever is thrown at it like a champ. 

The Kidneys

As new treatments are developed for the fight against CF and people are living longer with a better quality life,  other complications not associated with the respiratory system are becoming more prevalent.  People with CF have a high chance of developing CFRD [Cystic Fibrosis Related Diabetes] and it becomes increasingly common the older one gets.  35% of people with CF ages 2O-29 and 43% of people over 3O years old have CFRD.  CFRD is unique to CF: it has components of both Type I and Type II Diabetes.  A shortage of insulin made is a result from scarring in the pancreas from CF, and/or the body developing insulin resistance.  

Kidneys can also be severely damaged by potent antibiotics used to fight CF.  A blood test is done to make sure the kidneys are working properly.  My kidneys? Free of CFRD [knock on wood] and conquering life.  I just have to remember to drink a lot of water to keep them working at their best. 

Right

CF may cause a lot of things to go awry in my body, but there are so many things that it does right: I take them for granted.  Even if my heart beats a little fast sometimes, it's still beating.  Life is coursing through my veins.  My eyes can see the beauty of the world around me.  I can smell and hear the rain.  Even though it may be a little, my lungs still bathe themselves in glorious air.  I am so thankful for everything that is "right."  Love to you all. 

Focus on what's "right" in your life today. 

The Battle

What does hope look like? 
Let me show you:

Hope is your genuine smiles, your compassion, your comments, your love and support.  It's your passion for each breath and living each day to its fullest potential.  It's telling somehow how much you care about them, appreciating them, and it's realizing how beautiful they make your life.  Hope is knowing that each breath is a true gift.

Thank you each for wearing purple!  I am so grateful for the pictures sent, messages shared, emails, FB posts, and text messages.  I was truly brought to tears by each one: you all are so very incredible.


The Delicate Balance of Infection

European Satchel Time

With progressing CF my lungs are always permeated with infection; the real battle is not in eradicating it, but in keeping it from completely taking over.   The bacteria becomes so complex and resistant that each infection becomes more and more difficult to combat.  I always have an infection, it just depends if it's running rampant or being held at bay.

 My lungs culture a type of bacteria called Pseudomonas aeruginosa and a fungus called Aspergillus.  A healthy body's natural defense system and working lungs can keep the bacteria and fungus from making a home and ruthlessly taking over, however, my sticky inflamed lungs are a perfect camp for bacteria and fungus to flourish.  Pseudomonas is an ever changing bacteria that becomes highly resistant to the ammunition being fired its way.   CF research and new drug development is so critical to the future of CF because of the high level of resistance to antibiotics the bacteria develops.  As CF progresses, antibiotics that once annihilated the enemy now seem powerless.  Pseudomonas is treated both with oral antibiotics, IV antibiotics, and inhaled antibiotics.  Aspergillus is treated with oral anti-fungals.  All come with the hoped benefit of minimizing the infection, but they also come with some wicked side effects.  Mentally, physically, and emotionally I can fight: hopeful for an extra breath of life.


How do we fight?  We fight physically, mentally, emotionally, and with hope.  We throw everything we've got against the prevalent infections: fighting them until they are more tired than we, trying everything we can to add a few breaths.  


PICC'd and Ready to Fight
My new PICC is in, my "European Satchel" is again my best friend, and I have an entire army behind me fighting.  Life is a beautiful battle for us all.  Within each battle there still lies hope.  Hope for the future, for dreams, for sweet memories, for blissful breathe easy days.  What is the most amazing part of fighting this beautiful battle? The people who stand next to you through it all.  Love to you all.


What hope lies within your battles?

Walk this Way

What if the next step you took added a tomorrow for someone?  How many steps would you take? What if those steps gave someone the chance at another breath of life?

Me, Andy, & Kendra

I am so grateful for the people taking steps to give me a tomorrow.  There are absolutely no words to describe how completely humbled and grateful I am to everyone who fights to give me a tomorrow.  I am left in complete awe of all the love and support from the army of people who are willing to fight for me.  

Great Strides

Some very dear people came to me a few months ago asking if they could raise money and awareness for CF through Great Strides.  I was so completely humbled and overcome with emotion.

Great Strides is a national fundraising event where teams walk to raise money for CF research and drug development in hopes of finding a cure. 

Will you walk with us? 

The first opportunity is in 

St. Peter, Minnesota.

Saturday, May 1O 

1O a.m. @ Minnesota Square Park

Jaci & me

Click here for more info and to register for our St. Peter Great Strides Team. 

Your second opportunity is in 

Sioux Falls, South Dakota. 

Saturday, May 31

1O a.m. @ Spencer Park

Click here for more info and to register for our Sioux Falls Great Strides Team.

Your generous love and support does not only give me a chance at another breath, but gives hope to someone's family and friends.  CF is hidden beneath the face of someone's best friend, someone's sister, someone's nephew, someone's wife, and someone's grandson: raise awareness, donate, or just come and walk with us!  

Endless Gratitude

There is no way I can ever possibly repay the generosity shown to me from my friends, family, and complete strangers.  Thank you to my dear Andy who asked me if his NHS chapter could start a team and raise money for Great Strides in St. Peter.  I am so humbled by the "silent' work that Andy has done to support not only me through everything, but also the work he has done in sharing his passion for CF with his NHS students and leading their involvement with Great Strides.  

I am also so grateful to my dear friend, Jaci who also came to me asking if she could start a team for Great Strides, but for Sioux Falls.  The hope that I see on Jaci's face and how much she believes in this cause brings me to tears.  Her selfless determination gives me hope.  

I am left with no words to offer that could adequately express my gratitude for each of them and what they are doing for me.  I can only offer tears of humility.  Love to you all. 

How many steps would you take to give someone another tomorrow?