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.

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. 

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. 

Your Reflection

This post is dedicated to my brother, Nathan, who I catch a glimpse of at times staring back at me through my own reflection.

Nathan was 6 years older than me.   After more than 4.5 years of tests and continuous unanswered questions, he was diagnosed with Cystic Fibrosis.  At that time the life expectancy for a child diagnosed with CF was about 14 years old.

Nathan spent the majority of his life surrounded by nurses, respiratory therapists, and doctors who all became like family.  Home became the hospital.  It was where everyone knew his name and treated us like family.  I am so grateful for those kind nurses, RT, and doctors: many of which I still know and love dearly.

From as early as I can remember, my days revolved around going to the hospital to visit him: whether it was Iowa City, Omaha, or Sioux Falls.  When he would come home, it would be for short periods of time and usually on home IVs.  Inevitably, the infection would always get worse and he'd have to return to the hospital.   As the disease progressed, so did his fight for every breath.  He was so small.  He wasn't taller than 5 ft and never weighed more than 85 lbs.  Walking 15 feet was an exhausting task: bound by CF to watch the world race past unaware of just how lucky they were.  There are moments I want nothing more than to ask him questions, talk to him about his life, tell him how sorry I am for not understanding until now, and express my guilt for being healthier than he was.

Nathan died at the age of 17 from complications due to Cystic Fibrosis.

Memories.

It seems like an entirely different life when I look back now.   Because there were 6 years between us and I was still the little sister,  I have trouble recalling a lot of specific memories.  Maybe this is the cause of time, or maybe a response to what I saw happening before me as child?

What I do remember?

His laugh.  The way he said my name.   His love for instant pudding.   The make-a--wish trip we took to Disney World.   The color of his eyes.   Him instilling the fear I have of swimming in lakes or oceans.  His walk.  His baseball card collection [he gave me a few of his Hologram cards when I was little].  Him burying me in the sand.   His smile.  Us jumping on our parent's bed.  His hands.  The words of his last goodbye to me: he gave me his dog.  The day he died.

Who do I see when I stare at my reflection?  

My brother.  I catch glimpses of him when I smile a certain way, when my face is extra puffy from all the medication, and most often in my tired eyes staring back at me.     


Thank you to all the nurses, respiratory therapists, and doctors who fight CF everyday: you have given me the gift of every breath.  Love to you all.

 Who do you see in your reflection?