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.

Thin Ice

There's a hesitation within my step.  My body tenses as I suddenly become aware of every cell within  my body: from my fingertips down to the soles of my feet.   I take a deep breath and gasp as I feel the pungent crisp air pass between my lips and fill my lungs.  For a moment I hold my breath and close my eyes, fearing not only to take the first step, but the journey across the uncertain ground.

Do I delicately place each step, aware of every movement I make hoping the fragile thin ice below my feet will support me?  Or do I run, seeing how far I can possibly reach before I feel or hear the ground give way?

It's time to take that first step, to set out upon the frozen sheet of icy glass and see if it will hold me.  The ground below me has been unsteady these past months, but each step I've taken has been coupled with an army of people and a barrage of potent antibiotics and medications hoping to stabilize and firm the ground beneath me.  It has been an emotional, frustrating, and exhausting endeavor to come this far, but I am amazed and so very thankful to have weathered the journey: to be able to breathe. 

Prepared to Swim

The last 161 days have consisted of two PICC lines, a sinus surgery and bronchoscopy, two visits inpatient to the hospital,  a multitude of oral medications and IV antibiotics, Influenza A, endless hours of treatments, a few drug reactions, and countless visits to my doctors.  But I have finally reached a point in which I have shown enough stability to stop IV antibiotics. It is time to see what my body will do on its own.  I'd be lying if I said I am not nervous or terrified.  It feels as if I am stepping out onto a sheet of thin ice: wondering how far I can get before the ice starts to abruptly crack beneath me, plunging me into the depths of the icy water.   How little trust I have in this body after it has betrayed me so vehemently these past months.  It is not "if" my body will betray me again, but "when."

But I must do it.  I must take a deep breath, step out onto the ice and have faith that it will hold me.  And if not, I surely can swim.

Without Fear

Wherever my steps are leading and however uncertain my path ahead may be, the journey is still most amazing. Let the brisk air pass between my lips and touch the depths of my lungs,  reminding me that I am alive . Let me not only walk without fear upon the uncertain ground, but skate across the glistening thin ice, always being a witness to the beautiful life I've been given.

Thank you to my wonderful CF team, the depth of my gratitude for each of you is immeasurable.  Thank you to my amazing friends and family who have steadfastly supported, loved, and shown me grace through this entire journey: it means more than you will ever know.  I only hope to share as much goodness as I have so graciously been shown and given.  Love to you all.

Are you walking on thin ice and prepared to swim?

Tenacity & Strength

The true strength and tenacity within ourselves are the sole make up of the people who hold us up, believe in us, and forbid to give up the fight when we are in need of saving.

As I sit across from one of my doctors at my appointment this last week, I was overcome with the emotional realization that I am not in this alone.  As I made light-hearted jokes about the future and talked about the tentative plans ahead, I knew the only reason I got to where I am today wasn't from me fighting CF alone, but from the multitude of doctors, nurses, pharmacists, friends, family members, and complete strangers holding me up, believing in every step of my journey, and always passionately fighting with me for another breath.   What an incredibly humbling thing to realize.  I wouldn't be here if it weren't for their relentless encouragement, steadfast support, and their commitment to doing everything possible to give me another tomorrow.

To Breathe
The last few months have been filled with endless IV antibiotics, countless pills, doctor visits, hospital stays, tests, blood draws, and the need for unyielding tenacity.  The last few months have held some of the most beautiful of moments and some of the most difficult.  At times, I thought I'd never feel like me again.  That with every setback came the panic and desperation to breathe, just for another day, month, or year: to merely live one more day in the life I love with the people I love.

I still feel that desperation to live, but today it is coupled with celebration.  At my appointment last week I had a FEV1(lung function) of 50%: almost double since this past November.  I have never worked harder and desperately wished more for that number.  To be able to breathe is simply amazing.  This is most likely where things will level off, but I will never stop fighting for more.

Testing the Waters
The direction and nature CF takes isn't clearly understood, nor can its future be clearly articulated.  What we do know is that with every ruthless infection and hit my body takes, it is harder to put the broken and shattered pieces of my health back together.  This shell of a body may have been left weaker and its resilience taking a brutal beating, but my determination and will are stronger than ever.  For the last 22 weeks (and counting), my body has been inundated continually with powerful cocktails of IV and oral medications in hopes of beating down the relentless drug resistant bacteria and fungus that are trying to steal my vitality, my strength, and my very future.  With that said, the time is coming in which the boundaries and limitations of my body without all these drugs needs to be tested.  I am filled with such a cacophony of emotions:
Gratefulness, for getting to this point in which I almost feel like me again.
Anxiety, for knowing the future is bound by the destruction of CF and the pain it will cause so many.
Infinite joy, for the relationships that have fed me and brought so much beauty to my life.
Nervousness, in wondering how long it will be before my body will betray me again.
Love, for the endless grace and kindness I have been shown.  
Hope, for the possibility of tomorrow.

I am so very grateful for those who have held me up through the hardest of moments, those who have celebrated each victory, those who continue to believe in my journey, and for those that have fought with me for every breath.

I am learning not to allow the past events to fill me with fear, or the unknown of the future to fill me with trepidation, but to live for the beauty that is present in this very moment.  CF may be progressively present in my life, but it will not rule by fear.  I will breathe bravely: continually renewed by the strength and tenacity that live within, knowing I am never alone in this fight.  Love to you all.

Each of our lives is filled with people who pour themselves into us, giving us strength and tenacity to endure whatever our journey may have in store.  

Thank them today.

Celebrate

twinfallsfireworks.org

There are few things as miraculous and beautiful as looking up into the night sky and seeing a burst of color dance across the sky: an amazing celebration for every onlooker.  I remember as a child sprawled out on a blanket in the middle of the outfield of the neighboring town's baseball diamond, looking up at the sky and waiting in anticipation for the light show that was going to be painted across the sky.

There is something so special about the moment when the first firework unfolds its glory into the night sky for all to see.  We know we are about to witness an entire fanfare of timeless and awe striking beauty. The sound of each firework being lit, hearing it soar to the sky, and the anticipation that stirs within as we wait to see the magnificent burst of color against the black canvas.   I think we have all whispered to ourselves "that kind is my favorite."

twinfallsfireworks.org

Effortlessly, the miraculous explosions of color dance against the night sky like a choreographed dance before our eyes.  At the beginning, one firework goes up, then another, not impeding on one another's moment.  Then suddenly, there are multiple fireworks awakened at the same moment, creating a mesmerizing and continuous explosion of color in the sky.  A flourish of fireworks continues to build in intensity until suddenly it's over.  All that is left is a sea of smoke and the smell of sulfur.  Once again, the sky is returned to its black canvas: the celebration of endless color has ended.

Celebrate
What makes a celebration so special?  The word alone gives excitement.  We wait in excited anticipation for each celebration in our lives, each event worthy of fireworks.  The day and event finally arrives. We are encompassed in joy, but then the day is over.  The celebration is done.  All that is left of the day are memories and the joys we experienced that will forever live within us.

But why can't every day contain that level of excitement?  Why can't we anxiously and excitedly look to "tomorrow": the day after the celebration?

Each day is a celebration in and of itself. There is always something to celebrate, great and small. This day has been given to you, celebrate it.  Does it require a show stopping fireworks display? No, but each day deserves a celebration all its own.  What am I going to celebrate today? I celebrate the beauty that greeted me as I walked outside this morning. I celebrate the opportunities the day has in store for me. I celebrate that I have the most wonderful people in my life. I celebrate each moment that makes me smile.  I celebrate each breath.  Love to you all.

What are you celebrating today?

Stormy Skies

A crack of lightning streams across the sky while the strong roar of thunder reverberates through the earth like the deep bass of a pipe organ.   A chorus of voices sing out with each drop of rain that resounds against the window pane.  The sounds and sights of summer have returned.

Perfect Night

This past week has been filled with the most beautiful and gentle of rains.  It has also been filled with some harsh summer storms that seemed relentless and uncompromising, leaving a path of destruction behind.  What also filled this past week? Some of the most beautiful and still evenings I can remember.  The sun was beginning to set and everything seemed to glow of a beautiful pink and gold hue.  For a moment it seemed as if life was standing still, that I was holding it lightly in the palm of my hand.  I could almost hear the shifting of the sky as the stars began to replace the sun bathed sky.   For a moment, everything seemed absolutely right in the world.    

Afternoon Storm

The next morning I awoke to an intense rain only to be followed by beautiful beams of sunlight that poured out over everything.  How very quickly things changed.  One moment the trees were soaking in the warm rays of the sun, and the next, tornado sirens were proclaiming their heed of warning.  In a matter of what seemed like just moments the sky opened up and let out a great fanfare: hail, rain, thunder, lightning.  The chorus grew louder as the drops of rain and hail poured out from the sky with more power.  We watched helplessly from inside as mother nature seemed to quickly devastate the world outside the stucco walls of our home.  

Eventually the clouds lightened and began to break apart, the rain ceased, and the sound of thunder was only a subtle rumble in the distance.  Soon, the radiance of the sun painted everything it could reach.  The evening turned into another one of pure beauty and perfection. 

The Storm & CF

What does any of this have to do with CF?  The raging storms of CF have been quite merciless lately.  It seems the atmosphere is just right for the makings of harsh storm.  My PICC line is still adorning my arm and a new course of treatment is underway.  In just 3 weeks my lung function went from 5O% to 37%, all while on IV and oral antibiotic therapy.  We've changed courses and plans in hopes that something will alter the direction of this storm.  

Hail

Like the weather, the conditions of CF can change so quickly.  In just one day CF can turn the sun kissed skies into an unforgiving storm.  

Renewal

Life comes with many storms and days filled with rain, but each one of them is simply beautiful.  As the warm rains of summer pour out from the sky, the earth is renewed and replenished.  Without those storms, the sun filled days would seem less bright, and we wouldn't appreciate the true beauty in those still and beautiful nights.  We can never be sure when a storm may come, foresee how strong it will be, or how much rain will fall, but we can be certain that the sun will shine again. We can be certain there will be days of endless sunshine and beautiful still nights.  The more rain that falls just means bigger puddles to splash in, and if the stormy days seem to never end, we can always dance in the rain.  

Another Beautiful Evening

This CF storm in my life shall too pass.  The destruction caused by this storm may have lasting effects, but that doesn't mean I won't fight to rebuild what has been lost or destroyed.  There will be days with light rain, days of sunshine, and days with fierce storms, but for each day I am truly grateful.  The beauty of life is impossible without the renewing rains from a storm.  I leave you with these words from a dear friend: "Anyone who says sunshine brings happiness has never danced in the rain." Love to you all.  

What storm are you weathering?

In Good Company

Think of the number of people you encounter in your day, your week, or lifetime.  Look at the people who surround your life.  What brings you together?  Is it because you're family, went to school together, or because you share similar interests?  Another element that brings people together is often experience:  going through the same heartaches and joys.  

What if you couldn't be around those people?  What if you put their life in jeopardy by being near them, or that they were harmful to your health?  You have experienced similar pains and joys of life, but you can never share yourself wholeheartedly with that person. You cannot show that person empathy or compassion through a hug, a quick visit over coffee, or just the touch on the arm.  You can't even be in the same room, or building. 

This is CF.  

Because the bacteria fostered in the lungs of people with CF is so life threatening, we are a great danger to each other.  I may be growing a bacteria that someone else has not yet been subjected to, and vice versa [MRSA, pseudomonas aeruginosa, NTM]. New and more bacteria means more rampant infections, more scarring in the lungs, worsening lung function, and respiratory failure.  Per guidelines of the CF Foundation "Only one person with CF is allowed at foundation sponsored indoor events, offices, or meetings. If it is an outdoor public event people with CF should maintain at least 6 feet from each other." Great precautionary measures are taken in the CF clinic as well: gown and gloves for all who enter.  Even though the bacteria that wreaks havoc on my lungs won't affect you, you can still be a carrier that leads to cross-infection. 

Until the last year or so, I never realized how isolating CF was.  I think of my last hospitalization, about the floor of the hospital and how many of us had CF. We were locked in our rooms, strategically maneuvered from one location to the next, and shut off from the only people who could truly understand.  I could hear them coughing, or would catch a glimpse of them as they walked the halls, but never could sit on the edge of their bed and talk about life.  Sure, I had my incredible friends and family, but I just wanted someone to really "get it." What do you do when all you want is talk to someone to justify that you aren't utterly crazy?  That someone else feels and think the same things as me?  

Community

For a long time I didn't want to read the stories of people with CF, I didn't want to read blogs, I didn't want to get newsletters about "what's happening." Why? It terrified me.  It was much easier for me to be in denial about the reality appearing before me. Maybe it was a way to shut out the destructive fait I witnessed for 17 years with my brother? Maybe it was me caring too much what everyone would think if they knew?  What changed?  Honestly, I felt alone and terrified.  I stumbled across a blog that so greatly impacted me: it is what gave me the strength to show the world the real me.  The blog's author is Caliegh Haber from CA. She is 23 years old and awaiting the call for a double lung transplant.  Because she is such an incredible individual tomorrow's post will be solely dedicated to her.  

Look at the people around you.  That hug you just gave? Cherish it.  That breath you just took? It's a gift. My gratitude and love for you all overflows.  Thank you so much for being my "community" and making each breath so incredibly beautiful.  Love to you all.

Don't just call a friend today, go have ice cream together. 

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?

Uniquely You

Purple Hair

There is no one like you.  Your genetic make up is unique only to you.  Your human genome is an intricate map that is the infrastructure to who you are: encoded within DNA sequences, or "genes," in 23 base pairs of chromosomes.

An amazing person and CF pharmacist by the name of Stacy Peters so graciously agreed to submit a posting for my blog.  I am so honored to share her passion for CF with you.  She is one of the most unique and wonderful people in my life: thank you for writing! This post will be Part I of II great submissions sharing her knowledge about CF. Here is Part I:

CFTR

"Cystic fibrosis is a genetic disorder that results in a dysfunctional protein called CFTR (cystic fibrosis transmembrane conductance regulator).  In people without CF, the CFTR protein works like a gate on the cell surface and regulates water and salt transport in cells lining the lungs, intestines, pancreas, etc.  In people with CF, this protein or “gate” does not work correctly.  When it isn’t working properly, changes such as thick mucus, pancreatic insufficiency, and various gastrointestinal issues occur.  However, not all people with CF are created equal; the type and degree of CFTR protein dysfunction varies depending on each person’s genetic mutations.  People with cystic fibrosis inherit 1 mutation from each parent, and you must have 2 mutations to have cystic fibrosis.  There are approximately 2000 different mutations of the CFTR gene.  Many have been classified into 5 different categories depending on what is wrong with the CFTR protein/gate. 

·       Class 1(protein formation defect):  The CFTR protein/gate is not made by the cell at all.

·       Class 2 (folding/trafficking defect – deltaF508):  The CFTR protein/gate is made, but it is stuck inside the cell instead of being on the cell surface where it needs to be to function.

·       Class 3 (gating defect):  The CFTR protein/gate is on the cell surface where it should be, but it doesn’t work.

·       Class 4 (narrow gate):  The CFTR protein/gate is on the cell surface but it’s too narrow so it doesn’t work as well as it should.

·       Class 5 (variable production):  The CFTR protein/gate is not made consistently but some active CFTR proteins make it to the cell surface so there is some function left.

·      Class 6 (rapid degradation):  The CFTR protein/gate is made and on the cell surface but breaks down too quickly.  (this class isn’t always included – some people lump it with class 5)

The CFTR gene is actually one of the longer genes  in the body, hence, it has more opportunity for errors to occur on it.  “It is estimated that about 2% of patients have large rearrangements, including deletions and duplications….”  Many gene alterations haven’t been “classified” yet."

My Mutations

What makes me, me?  My "special" defective CFTR genes are actually two different mutations.  One is a copy of the most common mutation: delta F5O8 and the other is... well very "unique."  My second mutation is rare and indecipherable, making it difficult to know what treatments are effective.  The make up of my genes and their uniqueness just makes it extra challenging to understand CF and its hold on my body. But with each advancement there is light and new hope for the future.

Uniquely Beautiful
You are one of a kind and so am I.  The very make up in which we exist makes us unique.  There is only one you, and it is beautifully unique.  Each of you makes the world so differently beautiful, and I am so thankful for your presence in my life.  A very special thank you to Stacy: I don't know what I would do without your knowledge, friendship, and drive to fight CF.  Love to you all.

Think of how your uniqueness makes the world so beautiful.