Three Simple Words

Everything is different. Three simple words that will live within me for the rest of my days and upon every breath that I am given. Tears still fill my eyes as those three words resonate in my mind. I can hear the tone in Mark’s voice as he said them, and I can trace the painful and loving honesty in which they were born to an uncontrollable truth that silently fills our life together. The uncontrollable truth called cystic fibrosis. Indeed, everything is different, but it was an honest difference I thought only I could see.

Why are those words so difficult to hear? Because I didn’t know the differences that existed intrinsically within me were noticeable to the outside world, most of all to those I love. I thought I had hidden them deep within the most cavernous parts of my being for no one to see or experience. But I was reminded that it’s not just me enduring the barrage of potent antibiotics and the effects of CF, but that their impact is much farther reaching. That their devastation silently touches all those present in my life. Suddenly, I felt exposed and like I had failed. I had failed at protecting those I love from me, from CF. Most of all, however, I felt as if I failed at protecting Mark.

The Difference Within

I don’t think Mark fully knew the extent of those three simple words and the depth of their meaning. Just how different is everything? How different am I?  Here’s an honest glimpse: 

For five weeks potent drugs saturated every ounce of my being – unknowingly permeating every part of my conscious and unconscious existence. Toxic drugs laden with a desperate unspoken hope silently infiltrated my body from within - making me slowly feel like a stranger within my own body. With every infiltration of antibiotics it seemingly gets harder to navigate this body that feels as if it’s becoming more and more foreign to me. I don’t recognize this body that has accompanied me for more than 29 years. As these drugs always seem to silently strip me of myself, I feel a bit of who I am slowly slip through my fingers. I desperately try to hold onto any familiar part of my existence. I push myself harder, constantly trying to prove to myself that I am in control - holding onto anything recognizable within myself. But there are times such powerful drugs and CF feel as if they are whitewashing every part of me – stealing my thoughts, focus, vibrancy, and self-trust.

I anxiously retreat away from the world, terrified that I am unrecognizable not only to myself but to those I love as well. Terrified that I will fail to be the person they need and lovingly know. I do my best to pretend all is fine. I do my best to pretend and prove that I am better than fine. Desperately trying to prove that no matter the merciless pounding from CF itself and the antibiotics I take, I am immovable, unshakable, and unstoppable – all in hopes of protecting those I love from the wake of CF. Even now, I feel a devastating (and knowingly ridiculous) disappointment in myself and my inability to hide such differences and protect those I love from myself and CF.

 

To Be Me

My heart turns back to Mark, thinking of what he’s silently endured and wondering the extent of such pain I have unknowingly caused him through the years. He has never shown any anger, disappointment, or remorse for this life with me - no matter how different everything at times may seem. He may never know the true impact of those three simple words, but I will always remember their deep and life-changing meaning that were born of the deepest love and honesty.

I know at the core of my being I can’t fully protect him or those I love from the brutal realities and effects of CF. It’s a truth itself just like CF. Yes, indeed, everything is different, but one thing will always remain the same: the endless gratitude that fills my very existence. My gratitude for every beautiful breath that I am given and the beautiful love that fills my life. Love to you all.

What has someone said to you that has left you changed?

Unbreakable

It had been five months since potent combinations of IV and oral antibiotics coursed through my veins - my body beginning to feel more like my own again and I was beginning to recognize the person I saw in the mirror. My mind was running at full speed again. Each day my mind shed a little of its doubt and mistrust of its own body – unassumingly instilling the belief within me that I was invincible.

But I am not invincible, and CF is ever-present. I’d be lying if I said I hadn’t been avoiding the signs of an exacerbation, desperate to give my body the chance to show me - show CF, that I was strong and unbreakable.

Reminded

How quickly I forgot the feeling of powerful poisons coursing through my body. For three weeks my body has been inundated with powerful IV infusions, oral antibiotics, and extra treatments. Its crippling presence evident in every aching joint and fatigued step. Every morning I can feel the sunken-ness of my own eyes deepening as I force myself to fall into my life’s usual expected momentum.  My body feels heavy and struggles to keep pace with my mind. I am reminded that my body is not my own. I am reminded that beneath the surface the fight against CF threatens to break me – trading a beating from antibiotics for the mere hope of getting one more day. One more beautiful breath.

This isn’t something new I’ve experienced, but this time it does seem different. As CF and its truth steadily chip away at my being, I can’t help but reflect over these past several years and the extended and frequent courses of powerful antibiotics. Is this how I’ve always felt? Has my body always felt this beaten during such courses? I think back to last year when for sixth months straight without break my body was inundated with different powerful drugs in hopes that something would combat the life-stealing force of CF. I remember those days being difficult but it is as if their honest paralyzing sting has been replaced or freed from my memory.

Mere Hope
It’s amazing how quickly the mind chooses to release and replace those excruciating past experiences with unwavering hope. Of course, those difficult moments live deep within us always, but we move on from them – choosing not to live in our brokenness but in the beautiful hope of the present and future. We live for those moments that the body proves its strength and mends the brokenness of that which comes in the wake of living with the realities CF, or whatever difficulty we each may face. I have no doubt that this trying course of antibiotics will soon be finished and these difficult moments too, will be overcome by the unbreakable hope that lives deep within.

Today, each beautiful breath is dedicated to all those that must live with and bare witness to the truth that is CF - parents, spouses, family, friends, medical teams, and the incredibly strong, tenacious, and hopeful individuals with CF. Love to you all.

Whatever difficulty you may be facing, remember there's an unbreakable hope that lives deep within you. 

A Season of Waiting

[Following Blog post written by Mark Bonnema]

A sincere thanks to all who have offered their thoughts, prayers, and love these last few days. Ashley continues to be hospitalized at the University of Minnesota Fairview Hospital with an exacerbation of a pulmonary infection. Her care team is uncertain if the problematic culprit is an antibiotic resistant bacterial infection, or an intractable fungal infection.  Regardless the cause, Ashley's lungs continue to feel and act like a wet sponge... so inflamed and full of fluid and mucous that they repel oxygen rich air. Her lung function continues to fall, reaching new lows for her. Shortness of breath, headaches, and extreme fatigue are the current norm. Ashley is beginning to wonder if she will ever be able to walk down the hall or up a single flight of stairs again without feeling like she will pass out.

Most hospitalizations for Ashley start out with the same routine... Ashley politely declines to wear a hospital gown in favor of her own clothes, she tells the hospital staff she has not traveled out of the country or been in contact with anyone displaying ebola symptoms, and... she provides a sputum sample. The hospital lab then takes the sputum (i.e. phlegm or lung mucous) sample and cultures it, allowing any bacteria or fungus present to grow on a nutrient rich petri dish. Any bacteria or fungi that grow are identified, and then a sensitivity  test is conducted, where the microbes are exposed to different antibiotic and anti fungal medications to see if they are resistant or susceptible to each medication. Ashley has progressed deep enough into her journey with CF that the bacteria that she cultures in her sputum are resistant to almost all available antibiotics. She has had to use the common antibiotics too often, and the bacteria have learned to survive in the presence of such antibiotics. (Not to worry, these bacteria are usually harmless to the general public with intact immune systems, hence I won't hesitate to steal a kiss from her without worry of becoming infected or developing pneumonia myself).

Currently, the only antibiotic that the bacteria in Ashley's lungs show any slight sensitivity, or potential to be affected by antibiotics, is a drug called piperacillin-tazobactam, which is related to a drug Ashley had an allergic reaction to when she was a child (augmentin). This is a problem. Her immune system, while failing to eradicate the bacteria in her lungs, over-responds and causes an allergic response in the presence of some antibiotic medications. The care team takes this situation seriously, and has a strict protocol in place that requires admittance to the intensive care unit (ICU) for close observation if a drug that has previously caused an allergic retain in a person is to be utilized. Ideally, we would not have to consider using this antibiotic. But in reality, it is one of the only options that remains. We have been waiting since Friday for a room to open up in the ICU so Ashley can undergo a desensitization test for the antibiotic that may be the key to overcoming her current lung infection. The ICU has been full without reprieve since Friday. While we are glad Ashley does not have imminent need to be in the ICU, we also would like to start the piperacillin, which may provide relief from her lung infection... relief from labored breathing, headaches, and fatigue, relief from the feeling that her condition is worsening and stealing her vitality and life.

CF, as a chronic disease, is often characterized by a slow decline in health and lung function and an even slower improvement. When a lung infection is at its worst and lung function has reached its lowest, it can seem an impossible task to wait for the treatments and antibiotics to do their work, all the while wondering if they will even work at all. Days and weeks may pass with little or no change.

See Ashley "Enjoying the ride amidst the wait."

Today was the first day of Advent - a season of waiting in the Christian faith. Ashley and I have passed through the spiritual and mental process that this holy season requires many times. It is usually with joy and anticipation that we enter this season of waiting upon the birth of Jesus Christ. But never before have we been forced to practice and experience waiting like this. It is no longer a mental or spiritual exercise when each and every one of Ashley's labored breaths yearns and pines for relief. Waiting for breath is difficult, it is painful and disheartening, and agonizingly real.

Ashley and I are immersed in a season of waiting. When will we be able to begin the new antibiotics? When will Ashley's lungs begin to clear? When will her breath return? When will she feel like herself again? When will she be able to make music and sing? None of the physicians or members of the care team are able to answer this for us. We must wait. Time will tell. This Advent will be unlike any we have ever experienced before. We will experience true waiting.

Your continued love, prayers, and support help us endure the wait. Thank you.

Wait in joy, heartache, and hope for what tomorrow may bring. 

Priceless

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

 Cost of equipment and other therapies

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

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

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

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

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

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

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

What are you willing to pay to live another day?

      

      

Rest & Renewal

Sunday, it's known as being the day set aside for rest, relaxation, and renewal.  But what happens when that rest and renewal are so hard to come by?

The complications associated with Cystic Fibrosis can cause severe insomnia and fatigue, making physical and mental renewal even more difficult.

What contributes to CF related insomnia?

 - Decreased lung function

 - Inflammation in the lungs, airways, sinuses   making breathing difficult, let alone sleeping

- Decreased Blood Oxygen levels

- Side effects of medications

- Cough

- Pain

For someone with CF, the basic tasks of everyday life such as mere breathing take significantly more energy, making restorative rest even more crucial.  Exhaustion and fatigue compromise an already weak immune system and the ability to fight infection.

Hello, my name is Ashley, and I am a CF insomniac, and I would do anything for a night of blissful sleep.  I dread the night, playing host to a struggle of wills between my exhausted mind and body.   By 3 a.m. I am already trying to prepare myself mentally for the next day: knowing the exhaustion just continues to compound with every waking moment.   What do I do when I find myself exhausted and becoming more and more so?  Push even harder.  I am renewed by the magnificent people in my life, the opportunity to live a life with no regrets, and each beautiful breath that passes through my lips.

What renews you?

I am so thankful for every waking moment, and I have a lot of them!  Love to you all!