The Unsettled Sea of Stability

Within me lies a ceaseless current that fiercely drifts between the unpredictable seas of desperate contentment and self-resolve. It’s a tumultuous tide that finds a constant tension between passionately wanting and expecting more of myself and gratefully embracing all that I have. A strong current is rooted in a reality that lies just beyond sight at water’s edge – a devastating cliff silently existing beyond my view. I am sailing amidst the dark in unknown and unpredictable waters, always cautious of becoming too comfortable with life, as I know the course of life can quickly change.

A Restless Current

The salty seas of CF are steady right now. This past Friday at my CF appointment I blew a FEV1 of 50% (lung function). My last three lung function tests in the last five months have all been within a percent or so of each other. The resounding theme of such a number being the word “stability.” I’d be lying if there wasn’t an underlying current of disappointment. I know, it’s unfounded and ridiculous. So, you must be wondering the reason for such a restless current of disappointment? Shouldn’t I be relishing in the waves of life’s present stability? I should, and there is an undeniable part of myself that truly does cherish these beautiful moments of stability.

But, I crave progress and gain as I feel it distances me from the realities of CF. It makes the existence of CF in my life less harsh and painful. In every quiet moment of stability I feel as if I’m further being pushed out into open water – more exposed and vulnerable. Maybe it’s because I have seen and felt how quickly the unforgiving storms of life can rage - engulfing me in its crippling powerful wake. Maybe it’s because I’ve tirelessly fought for every breath of stability - always consumed not with the question of “if” but “when” another storm will overwhelm me. Maybe it’s because I’ve seen the price I and those I love have had to pay and the changes we’ve all had to endure just to cling to stability - always seeing the reflection of my own disappointment in their faces when I am not able to tell them of any gain. Maybe it’s because within this time of stability I realize more and more what can all be lost. 

Along this journey, harbored deep within me is also a constant guilt. I recognize how incredibly lucky I am - always knowing I could be sailing a very different sea of CF right now. How can I be so wholly grateful for every beautiful breath while still wanting more - desperate for any sort of positive progress? I know there are so many people with CF that would do anything for such stability and 50%. I want calm seas and more beautiful breaths for all of us.

Change

The past year has come with great self-reflection and many life changes. An ever-present desperation to live fully in every moment was working against me. For most of my life I ignored my tired body, constantly pushing harder to try and distance myself from the progressing undeniable realities of CF in my life. As a storm of CF would rage, I’d batten down the hatch and sail myself unknowingly further into the storm, thinking ultimately I would sail through the tumult of CF. But I never fully sailed through, I just steadied the boat – always knowing the seas below me were waiting for the right winds to catch me off guard. And with unforgiving force they did, threatening to quickly capsize me and plummet me into the powerful stormy seas of CF.

But I continued sailing – my fervent sight always set upon the horizon and calm waters. Some difficult decisions were made that January of 2015 and little did I know more would only follow. Those months were incredibly difficult but these days of stability are filled with their own unique struggles. Even within this stability are brutal reminders of the progressive and unrelenting disease of CF within my body. But the most difficult thing that challenges me? Protecting me from myself. I’m constantly trying to restrain myself from easily falling back into the deep seeded belief that I am invincible and unbreakable. I’m constantly striving to keep this ship balanced upon the unfair waters of CF that lie below. To get to this point of stability, it has taken great work and dedication. I know it’s this cautious self-awareness and this strict self-discipline that have allowed these steady tides of stability. I know I will never out-sail CF, but I can do my best to weather each current that I’m graciously given.

Stay the Course

I’m learning to embrace this stability while always charting my journey upon an endless hope and contentment. I must not misinterpret lifeless stagnancy for stability. After all, my life is anything but lifeless. Each beautiful breath is filled with more life than ever. Today I will graciously cling to the stability of 50% all while tirelessly fighting to keep it. Love to you all.



Keep your eyes on the horizon and enjoy today’s steady waters.

The Great Escape

I think we have all had that feeling of wanting to pack a bag and set out on the open road, never to look back.  We are filled with a great hope that whatever we are running from won't find us wherever we are going.  We are trying to escape the weight of our world in pursuit of a place [whether physical or mental] of peace, contentment, and fulfillment. Or we are merely searching for a place to help us forget.

The Getaway

This past week Mark and I took the baby camper out for its maiden voyage of the summer.  For the most part the renovations had been finished and it was ready to take on the open road.  I don't think there was a more perfect week to be in South Dakota: encompassed by a perfect breeze, warm skies, the unwavering beauty of the cottonwood trees, and the chorus of the Missouri River waves lapping against the shoreline.  For four days I felt as if I had somehow escaped the reality of my world: my world of doctors appointments, PICC lines, IV antibiotics, pharmacy visits, phone calls, treatments, school, restlessness, the chaos of my life, decisions, disappointments, etc.  For some reason I felt as if none of it existed.  Everything was right in the world.  Sure, I still had to do treatments, take my cocktail of pills, do IV meds, study for a midterm, homework, etc, but it still seemed like I had escaped my other world for just a few moments.  Just merely existing, taking in what was around me, appreciating the mere beauty of it all, and just breathing were all that really mattered.  

As Thursday morning approached and our time to pack up the baby camper became a reality, I began to find myself getting crabby, restless, and nauseous.  I didn't want to think about my world so patiently waiting for me back in Sioux Falls: the appointments, the treatments, the renewed strength and stamina I needed to somehow find to continue to fight and win this current battle with my lungs, teaching, and class.  Following class, Thursday night I would drive straight to Minneapolis for a morning doctors appointment with my MN CF team: thrown so quickly back into the realities that are my world.  

Reality

We all try to escape, whether it is in a day dream, looking up to the sky to take in its beauty, losing ourself in a conversation with a friend, or taking off with bags packed on a quick getaway.  The one thing that still remains though, is our world is still waiting for us wherever our great escape may lead us.  We may be able to escape our life for a bit, but we cannot outrun it.  It's the only life we have been given, and it is ours to live fully. Even if it is filled with doctors appointments, PICC lines, IV antibiotics, and extra treatments: this life is all mine.  I am grateful for the little escapes, but know they cannot last forever: it's always back to life, back to reality. 

There will always be an open road in which you yearn to set out upon, leaving all the weight of your world behind.  But this is the life you have been given, you are strong enough to live it, and no one can live it like you can.  Sure, we all need an escape every once in awhile, but your true, beautiful life will always be waiting.

Are you living your life or trying to make an escape?

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?

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 Journey

Salzburg, Austria, 2OO9

Have you ever read something that has changed you?  

Have you had words from a page stir endlessly within you after drinking them off the page?  

St. Rèmy, France, 2OO9

My dear friend, Maren, gave me this poem several months back.  The poem's stark words resonated so deeply within me that I find myself rereading it and thinking of it often.  Thank you, beautiful friend for sharing it with me: it has changed me.  

"The Journey" by Mary Oliver

One day you finally knew

what you had to do, and began, though the voices around you kept shouting their bad advice ---though the whole housebegan to tremble and you felt the old tug at your ankles. ‘Mend my life!’each voice cried.

But you didn’t stop.

You knew what you had to do,

though the wind pried with its stiff fingers at the very foundations though their melancholy was terrible. It was already late enough, and a wild night, and the road full of fallen branches and stones.

But little by little,

as you left their voices behind,the stars began to burn through the sheets of clouds, and there was a new voice, which you slowly recognized as your own, that kept you company as you strode deeper and deeper into the world, determined to do the only thing you could do – – –

determined to save the only life you could save.

France

My Journey

Living life is a beautiful journey.  Yes, its path can be laden with stones, branches, and gusty winds, but they all give meaning to the journey.  It is not a journey if there are no points to look back upon or move forward towards.  Our journey encompasses moments of euphoria and moments of struggle: together they make us realize just how beautiful and delicate life is.  This is the only life we are given, and we are influenced and changed by all parts of our journey.  Our journey makes us who we are. 

France

Why?

I don't ask "why" I have CF.  Instead, I am grateful for each day, each trial, each experience, and most of all, each relationship: it's all just a part of my journey.  Some days the path on my journey feel impassable. Storm clouds seal the sky. The heavy rain makes it hard to see the path: where I am going or where I have been.  The wind has no mercy.  The path has been littered with broken branches, leaves, and has been flooded from the downpour.  I am paralyzed.  But then?  The clouds begin to break and the light of the stars reflect upon my face.  Suddenly, the path is illuminated from the bright moon: I can see from where I came and where it leads.  Living life can be a beautiful storm, but it makes the stars seem that much brighter.  

Austria

I have CF, and it may cause some storms along my path, but it does not define my journey.  What does define my journey? Every beautiful breath.  Love to you all. 

Take today and appreciate your journey: where you have been and the path still waiting to be explored.

Beautiful Mountains & Deep Valleys

Take a moment and imagine a breathtaking mountain scape: blue skies, snow capped peaks, and rushing brooks.

A photo from my time in Bavaria.

Imagine yourself picking up a stone along your walking path.  Imagine the trees next to you; the smell of pine.  Feel the sun streaming through the trees and the sound of the birds singing their songs.  Imagine that moment when you come upon a "scenic lookout." Whatever the altitude, there is beauty.  

When looking back at the last year alone my PFTs represent a great mountain scape.  Today, I had an appointment with part of my team and added another "scenic lookout" to my mountain vista.

My Mountain Scape

What are PFTs?

Pulmonary Function Tests are used to test the health of the lungs.  They compare the lung function of a person with CF to the predicted values of a person similar in weight, age, and gender who has healthy lungs. There are several specific things a PFT measures [FEV1, FVC, FEF25-75],  but the "scenic outlooks" I always look at are the FVC and FEV1.

FVC [L] - the Forced Vital Capacity is the total amount of air you can take in and blow out.

FEV1 [L]- the Forced Expired Volume is the amount of air you can blow out in 1 second. 

What is an example of normal?

FEV1 - 8O% and higher

Decreased lung function is a primary cause of CF.  A sudden decline in PFTs can be a sign of infection and progressing lung damage.  PFTs are done to help my team and I know the needed course of treatment.  As I have seen in the last year and a half, lung function is easy to lose, terribly hard to regain, and more importantly maintain as my CF continues to assert its presence. 

Today's Scenic Lookout - Beautiful

My FEV1 was at 5O%. Meaning my lungs today are functioning at half of what a female my age's should.  The lowest my FEV1 has ever been is 36%.  Who knew numbers could carry so much meaning? 

5O% has never been a more beautiful number.  It has been a long time since I have seen that number, and I have fought tirelessly for every percent of it.

  

Each beautiful element is necessary to create the entire magnificent landscape.  The smallest pebble helps build the strongest mountains, and the deepest valleys make the mountains soar even taller. 

I WILL continue to climb this mountain: valuing each extra ounce of breath as a true gift, always aware of the valley below.  Love to you all.

Take the time to reflect today on the beauty of the mountain range that is your life.

What "scenic lookouts"influence your mountain scape?