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. 

Drawing Straws

The biggest endeavor in life is enacting the art of empathy: to show we care or understand something beyond ourselves. There is no greater feeling in life than being truly "heard" or feeling like you have been there for someone in both times of laughter or tears.

The Question

A frequent question and one I know is on the minds of many is "what does CF feel like?" or "Does it hurt?" 

The answer? I don't know any different. I take whatever my body has to give me every day and use it to its fullest potential.  I just imagine everyone has to think about breathing, that their body is stiff and aching in the morning, or that it takes time for everyone's lungs to "warm up" for the day.  I assume everyone has days where they feel great and days their body feels like a prison. The only way I can liken it to something you might understand is to the feeling of a chest cold, or when your body aches from being cold or having the flu, or that your lungs are like wet towels being wrung out.  This is my beautiful life though, and it's the only one I get.  There are moments my body lets me completely forget that I have CF. Those days are utterly blissful and I revel in them, pushing my body to the max knowing tomorrow may betray me and force me to feel every part of CF.  Each day may be different, but one thing remains the same: each is a gift filled with one more beautiful breath.

Challenge

While there is no way for anyone to know what it truly feels like to be another person, I have a CF challenge for you.  This idea was posted on the Cystic Fibrosis Foundation website. 

I had Mark try this challenge [he has lungs of steel] and the following is based on his observations: 

Warning: at any time you feel light headed or extreme discomfort, STOP!

1. Find a medium sized drinking straw [a flexible straw you buy in packs at the grocery store, not a McDonald's straw].

2. Plug your nose and breathe through the straw for 6O seconds. 

A few seconds in you might not think it's so bad, but then around 25 seconds you begin to consciously think about the air pouring into your lungs.  Then around 35 seconds you wonder if it's almost over.  By 6O seconds your lungs are beginning to panic a bit and scream to be released from such bondage.  But people with CF don't get to stop: it's why each breath seems so valuable.  Some days it feels like it takes everything just to breathe. Because it does. 

*If you are up for a challenge climb the stairs, walk up and down the aisles of the grocery store, or just put dishes away from the dishwasher while breathing through the straw. *

I want a life filled with breaths used to their fullest potential.  I want each of them to be encompassed by empathy for those around me: for people to know how much I care about them.  Think about your week.  Who made you feel loved and appreciated? Who listened? Thank you to all who listen, give endless love, and share yourselves selflessly with me.  Love to you all. 

Today is a brand new day.  How can you master the art of empathy?  

The Bull Fight

Transport yourself for a moment to someplace in Spain.

Madrid, Spain

You are attending one of Spain's iconic and magnificent Bull fights.  You find a good seat and wait for the Bull fight to begin.  There may be thousands of people in that arena, but there's only a single person fighting the bull.  All of the matador's experiences, training, and support from people he holds dear, has impacted him to be who he is in that very moment.  The matador has a mountain of support, encouragement, and cheers swirling about him, but he is the only one who can lead the fight.  Although he may be alone in the ring, even if he falls, stumbles, or wants to give up, there is a sea of people ready to help him stand and fight.

Impact

Take a moment and think about the amazing people who have influenced your life and the people that impact your every day: from your best friend to the cashier at the grocery store.  Has their relationship helped you through some tough times?  What sticks in your memory from years ago, or even today? Think of those people who have stuck with you through it all, those people who lifted you up when you thought you couldn't stand, or what about even that friendly smile from a stranger that somehow made your day more beautiful?  They all have shaped your life to make you who you are at this very given moment, but only you are in control of what you do with that knowledge, passion, and appreciation for life.

learning how to fight in Spain

The Battles of Life
Each of our lives contains some struggle or a battle we must fight: whether it be beneath the surface or unfolding before the world.  The battles we are facing may be different, but we are each bound together by the fight.  What have I learned through each struggle with CF?  I have learned while no one can fight the battle for me, I am not alone in the fight.  It is honestly one of the most incredible feelings to look at my life and see the amazing people who have impacted it so greatly, or that are still a huge influence today: friends, family, my CF team, teachers, mentors, or the check out woman who genuinely smiled at me.

Always Ready to Fight
Even though there are so many amazing people standing next to me, ready to fight, I know first and foremost I have to be the one to lead .  No one can do it for me.  I am the only one in control of my life and making sure to see the beauty in each breath.  You each have given me love, strength, and motivation to fight.  You each have so selflessly given all you can to me and I am so truly grateful.  At times I wonder who is in control, CF or me?  I am the only one who can decide if CF will define my life, and I have promised myself it never will.  CF will not win the battle.  I am strong, I am fiercely motivated, and I am taking in life one brave breath at a time. 

Think of the power you hold in shaping some one's life, even if it is something as simple as a genuine smile.  Your kindness may give them what they need to fight a battle: seen or unseen. You each are fighting your own battles, and I am with you every step of the way.  I only hope that I have given you the love, support, and confidence in yourself to know that you can fight.  I will be there to help you stand, when all you think you can do is fall.   Thank you to each of you for fighting this battle with me; you have shaped my life more than you will ever know.  Love to you all. 

No one can fight the battle for you, but they will fight with you.  Are you ready to fight?

Extraordinary

The last four days I have found myself taking in all that the beautiful city and people of Chicago will offer.  The sights and sounds of the city always fill me with renewed life: the fast paced diverse world buzzing all around so full of life.

Morning Train Ride

One of my favorite things in a big city is taking the train, metro, or subway system.  I love figuring out where I need to go, how things connect, and emerging from below the streets into a new world.   What I love most is seeing all the people coming and going in their day.

Where are these people going? Who are they with, and do they realize how significant they are in their life?  I see a woman read a message on her phone and reply with a text. I see her smile.  She is filled with happiness.  I see a woman who looks like she has been crying. She is alone and upset.  I see a man buried in a book, dressed beautifully. He is lost in thought in another world it seems. Then there is the couple holding hands with love in their eyes.  Life seems limitless to them.

Chicago

Deafening Silence

The train is filled with deafening chaotic silence.  The sounds of verbal conversation, laughter, or chatter are very minimal, yet the air around me seems filled with a symphony of untold stories, dreams, and passionate ideas.  The lives we have been given are unique to us, but we are all bound together by the same passion to live and the stories that fill our lives. 

Each of our lives is filled with moments of laughter, moments of uncertainty, and moments of pure bliss.  There are so many stories in each of us: extraordinary stories of our lives to be shared and witnessed. We just have to look, listen, and join the conversation. 

Whether on the train, in an elevator to go to the 94th floor of the Hancock building, or standing at a corner waiting to cross Michigan Avenue, it feels like the air around me is heavy and swollen with stories waiting to be told.  The air is waiting to be set ablaze with the inner voices each beautiful person so desperately tries to suppress. The deafening silence around me these past few days has made me think about the silent battles we each fight.  We each have things we struggle with: one not more or less important than the next.  If they affect us, they matter.  My silent battle? CF.  No longer am I diligently trying to hide CF from my story.  CF is part of my extraordinary life.   

Sharing Goodness

From the top of the John Hancock Building in Chicago

Amidst the chaos of life these past few days, I witnessed so many genuine acts of kindness.  Whether it be someone going out of their way to pick up a water bottle I dropped, an unsuspecting person several feet ahead of me reaching into their pocket to drop change in the cup of a homeless person, or someone just giving a gentle smile as I passed, they each added such beauty to life.  Each act of kindness makes the struggles of life that much more bearable.  We are all part of this life together.  I often wonder how can I make life more beautiful for someone else, whether it be a friend or stranger.  The meaning I get from life? The beautiful relationships I have with you, listening to the stories of your life,  knowing your struggles, and letting you know how much you mean to my life.  If I only do one thing worth anything in life, I hope it is that I made your life more beautiful in some way: you each have certainly made my life extraordinarily beautiful. 

The "Cloud Gate"

You have a great effect on the world around you.  Your life story has a beautiful influence on the world.  Speak up and tell it. Your life is extraordinary. Thank you all for being such a beautiful part of my life. Love to you all.

Speak up. I am listening. I want to hear your extraordinary story.

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. 

Oh, the Places You Will Go

Time Square, NYC

Monaco

I love to travel. I love to experience new places, cultures, and people.  I love the excitement of planning and packing for a new adventure.  I love discovering the world beyond myself.

2 years ago I wouldn't think twice about hopping in the car or on a plane and jetting off on an adventure.  I was a completely free spirit that loved to get lost in an unknown city, and lived to spend the day exploring and soaking up uncharted territory.  I continually dreamed about the next trip abroad, a road trip over a long weekend, or a spontaneous adventure.  I have experienced France, Germany, England, Italy, Austria, Spain, and several wonderful cities and states in the US.  Today, I still endlessly dream of those trips, but am filled with so much trepidation, hesitation, and guilt.

My first thoughts no longer are, "what are the sights I am going to see?" "How many things can I experience in a single day?" "How can I really become a part of the culture?"

Today's first thoughts are, "where is the closest hospital in case something happens?" "What if I begin coughing up large amounts of blood and can't stop?" "How much can my body handle?" "Is the change in my environment going to make me sick or cause problems?"


Baggage

Madrid, Spain

No longer can I just jet away with minimal "baggage."  No longer can I trust my body to be stable long enough through a vacation with minimal treatments. The single backpack I used to use for my excursions is now in addition to at least 3 other bags.  My VEST alone is 31 lbs and luckily it comes with its own rolling bag.  I then have an entire bag for all my medications and neb supplies: aka the "bag o' drugs."  Also, some of my medications must remain refrigerated, so I have to have a small cooler and must think about accessibility to keeping it cold. Then comes my suitcase for clothes, and I am just as guilty as the next girl for overpacking.  How am I going to manage all of these heavy bags?  When we travel now, Mark so willingly takes care of carrying things to the car, into the hotel, back to the car, and up the stairs to our bedroom once we return home.  He never thinks twice about making extra trips or carrying extra bags.  I feel so guilty that our life has become so consumed by trying to keep me healthy, that we can never "get away" from CF.  When I shed tears of disappointment and anger towards my body, he takes my hand or holds me tight and says, "it's ok. This is OUR life."  

Madrid, Spain

Sitting or being immobile for extreme periods of time is hard on my body and lungs, but yet, after a day of pushing my body to its extreme it is drained and hurting.  I am constantly trying to find that happy balance in each day between pushing my body to the max and knowing its limits: I want to squeeze every ounce of life out of each day.  I can't waste a moment.  

Privilege
I have been so privileged to have had the opportunity to travel so much in my 27 years, but I want more.  I hunger for new uncharted adventures and spontaneous road trips.  I want to leave and not think about where the closest hospital is and how long it would take me to get there.  I don't want someone else to carry my bags.  I want to climb the stairs of the Eiffel Tower, go skiing on the Alps, and bike through charming cities.  

Switzerland

I have the greatest friends.  They changed our vacation plans just for me.  This summer we were excited to road trip out to Yellowstone National Park, but after talking about it with my doctors we decided it would not be a smart idea for me.  There isn't a hospital close by, and the change in altitude could make breathing utterly miserable for me.  Instead of sending me a postcard and bringing me a souvenir back, they completely changed their trip plans.  We are now road tripping to the Porcupine Mountains in Michigan, with stops along the way in MN and WI.  They changed their plans just for me.  There's nothing I hate more than being the center of one's focus, let alone the reason for changing someone's exciting vacation plans, but I am so grateful. They are the greatest friends. I love them so dearly and cannot wait to make wonderful memories on our "Random Road Trip 2O14."    

Our baby camper Palisades Park SD

More
I have tasted the world and I want more.  Even if CF has to make the trip with me, I will not be stopped from seeing the world. Mark and I made the trip to Chicago yesterday and will be taking it all in for the next few days. Thank you to everyone who has made my travels near and far so beautiful.  Love to you all. 

Florida

What places are you dreaming of going?

Sorrento, Italy

Paris, France

Road Tripping to Chicago

Madrid, Spain

Paris, France

Good ol' Yankton, SD

Target Field - Minneapolis, MN

Paris, France

Florida

Florida

Paris, France trip #3

 

Spring Break Paris, France 

Salzburg, Austria

Lausanne, Switzerland

Spicer, MN

Monaco

Cinque Terre, Italy

Southern France

Bavaria, Germany

Assisi, Italy

Rome, Italy

Munich, Germany

Rome, Italy