Cystic Fibrosis Kryptonite: The Common Cold

Originally Published on www.healthcomu.com on December 2, 2014

A couple of months ago ushered in the symbolic beginning of fall in our house. The transition from health to sickness is sudden and relentless. It was a beautiful day and everything seemed so right in my world. I was in the kitchen when I heard a muffled sound from the bedroom, a cough. My heart stopped. My mind immediately rolls through the countless possibilities. Second and third coughs ensue and the conclusion is clear—the dreaded cold going around school unfairly settled in my little one. I watch her closely, my ears unable to hear conversations around me, my only focus is now that small noise that so many don’t even hear. Within moments I could deduce that it was the start of countless worry filled nights.

Cystic fibrosis (CF) is a sneaky savage, robbing years and stealing dreams. An individual with CF can harbor a single bacterium for months or even years without a pulmonary exacerbation, controlling it with respiratory therapy and intermittent antibiotics. Then, like a thief in the night, a simple respiratory virus is introduced, and a war begins. The body is a humbling machine but it can only do so much. The multisyllabic devil that has taken residence in the body is like a guest that has overstayed his or her welcome. The body becomes used to it and turns its back to fight the new invader. Herein lies the problem, as the real enemy has just been left unattended to wreak complete havoc on my daughter’s body. So, while a cold is unfortunate for all children, to my daughter it is the beginning of hours of treatments, an array of antibiotics that threaten her body in countless ways, and the unnerving reality of the control CF has over our lives and our future.

The options are limited. She is unable to fight cystic fibrosis and a common cold at the same time without ammunition of some sort. Phone calls are made; emails flood her practitioner’s inbox. There is a window of time when you can fight the infection with the hope of maintaining lung function; however, there is always the possibility of permanent, unforgiving damage. I cannot live with that. So, the week of hell begins. Every treatment that seems to steal our day is doubled and even tripled when her body demands it to be. The hope to avoid a hospital stay gives power to charge through sleepless nights and coughing fits that seem impossible to live through. Then the questions begin to surface about whether our efforts are enough. And then just like the thief came into the night, he leaves. The coughs reside and the smile comes back. We won…this time.

 

What I wish I Could Say

Originally Posted on www.brfcf.org on October 7, 2014

My mind halts the words that fill my heart. How does one explain the motivation that drives me, in a hope to drive others? Where do you draw the line between truth and self pity, reality and fear that resides in one’s soul. With each breath, I feel an unfair mix of gratefulness swirled with unrivaled guilt. Grateful that I am given the gift of each breath, guilty that my daughter was not. So, I will take a moment to look at CF, not to evoke a feeling of pity, but to reveal the truth. I struggle with each fundraising video, each drama invoking Facebook post that is vying for attention and money. However, they are exhaustingly accurate, it is just the accuracy I loathe. So, for this moment, I will be strikingly honest about a day in the life.

I awake each morning with that startle that something is wrong, something is looming, a forgotten hardship in my blissful sleep. Like unsurmountable debt or a looming termination, except it is a person, and she is a living breathing part of me that lies peacefully upstairs. As consciousness parts like a cloudy day I am reminded of the noose that slowly constricts my peaceful state. My daughter is a warrior, she is my hero and she is racing against time.

We awake an hour and a half earlier than all the other children in order to clear the viscous demons out of her lungs. We are like a well oiled machine of breathe, blow….. huff, huff, huff. While this time is a reminder of the control of CF, it is time that my little girl and I bond over the disease that often runs our lives. We read stories, we giggle, we are just a mom and a daughter. CF is a part of our lives, truthfully a huge part, but it really only jolts me into reality on a number of occasions. That moment when you can no longer pretend that reality exists, you can no longer block out the images of possibility, that is when I become fragile. The Facebook posts from broken parents who lost their children who were never given the opportunity to have a sweet sixteen party or have a first kiss, some were not even afforded the opportunity to go to middle school, they found their path to heaven all too early. This is a reality that I try to justify with access to care or healthy choices, but I know this is not accurate- this is life with CF, there are no guarantees for a tomorrow. While that is everyone’s slogan, it hurts more when you actually have a daily reminder of that mortality. I have this moment, just being a mom playing tag when I hear a catch in her breath as I race past, or she awakes from a restful sleep coughing, my heart drops, a sign that life is not fair and despite dedication and relentless care, CF mocks us as we have given into the elusion that we have some ounce of control. I walk with heavy feet to the pharmacy to pick up the latest drug to add to the arsenal that we have filled cupboards with, it is the infinite reminder that we are needing more and more to fight everyday and she is only eight.

While these moments are heavy and burdensome I must relay that we still revel in the first snow, seeing buds on trees when spring finally arrives in Montana, hot chocolate still tastes just as amazing and halloween is still the most exciting day of the year. These moments are as sweet as they would have been without CF being a part of our lives, maybe even more so, because we never know when the moments are going to end. Until you face the mortality of someone that you love more than yourself, you really don’t fear death, truly fear it. It is only once you love with all of your heart that you understand what true loss is. Until then fighting is simply an action, not a part of who you are. I am a fighter, I fight for my daughter.

In the Woeful Place that I Sometimes Wallow

Originally Published on www.brfcf.org on March 18, 2014

 

The snow falls swiftly, with purpose. The clouds blanket the town, coldness envelopes the world…or maybe just mine. Words have been uttered that are feared, thoughts are being considered that do not yet belong in our lives. How can one small defect wreak such havoc on a small person? A person so young, so full of life and spirit. There is nothing in my life that can seep into my thoughts except- how do I fix this? The answer that makes me weep, is that I cannot. I cannot cure her. I cannot help my daughter.

There are no promises I can make; no fancy plans I can propose that will make CF any less of a demon than it is. So, I sit here, alone in the dark wondering what my purpose is.

I come to two conclusions. I am here to realize the power of this moment–I am here to be taught by her. My sweet daughter, the girl whom holds the strength that I seem to be lacking, is now my mentor. I am no longer the teacher in this story. Unbeknownst to her, she is teaching me about appreciating the gifts of life, the gift of breath. I am here to make sure that my daughter knows her potential, that she does not give way to any misguided ideas that she has an expiration date already decided for her.

Days ebb and flow through a cyclical pattern of what is known, what is expected and inevitable change. In that change is fear, sadness and overwhelm as CF deems it to be. I go through this pattern of disheartening acceptance, which seems unbelievably unfair at 34. I weep for the unknown, for the future which is potentially not mine to dream. I almost begin to accept the words and then this maternal feeling of power consumes me and I decide to fight this beast with everything I have, because she deserves to be here even more so than I do. And that is when my heart stops, my head remembers that we are already doing everything…there is nothing left to do but have faith in science, medicine and God. Faith that something more powerful than me knows how to move forward in this frightening place that I sometimes allow myself to reside.

Each passing acquaintance begs the same information. With a tilt of the head and a sympathetic look they ask the question that must be asked. Intentions are good- the reminder is not. My daughter is in a fragile state each and everyday. Her health while good today, may not be tomorrow. Most days I hold hope for what her future entails, but on the days when overwhelms seeps into my thoughts I retreat, emotions are too great to respond. The older she gets the more fear that clouds the hope that fights for control. I do not articulate these thoughts for sympathy, in fact, quite the opposite. I know how fragile life is, sometimes a simple meeting or gathering is more than I can muster and I have to be okay with that. I will ask you to be okay with that.

While my thoughts may seem grim right now, it is in effort to be transparent. While CF dictates so much of our lives, we are much more than cystic fibrosis and I do not want her to feel defined by her diagnosis. We are strong and happy in this moment and that is the only moment any of us are guaranteed- so we will live it with hope and faith in the future that is left waiting to be seized.

The Gift of Homeschooling

Originally Published on www.brfcf.org on October 25, 2012

The alarm clock rings, it is a distant sound in my vivid dream. I begin to rise into consciousness. It seems unfathomable that it is time to get up…I just went to sleep. The fall and winter months are kryptonite for CF. No matter how sanitized and isolated you reluctantly allow your child to become, colds are inevitable. Those simple colds become coughing fits for the child and night time panic attacks for the parents. Three AM treatments are commonplace during the cold season — they make the cough settle but the mind race. How can we maintain all the balance during the day when our nights are so unbalanced and full of stress?

I found myself in Maylie’s school meetings, trying to explain the hurdles that we have to jump through as everyone else sleeps soundly and nighttime disturbances are just the beginning.

Once the alarm clock brings me to the highest level of consciousness that I will be obtaining on such little sleep, I run downstairs to lay out the morning vitamins and antibiotics. I put the compressor on the stool, grab the latest Junie B. Jones book, the fuzzy blanket, hypersal, sputum cup, acapella, and cayston. I run back upstairs wake Maylie from the first peaceful sleep that she had all night and shuffle her downstairs in the dark to start her day. The struggle begins. Treatment is difficult, compliance is low and “Mom” is not the hero in this story. We finish treatment to move on to our next battle–breakfast. Food does not sound good after the respiratory marathon that May just endured. I accept a deal of drinking a glass of half and half–my choices are slim at this point. I have the 5% BMI cloud hanging over my head. The girls grab their scooters as I throw May’s lunch in her bag and we run to school. I hear the bell ringing a block away. I give May a quick kiss goodbye as she runs in the door. I am left to wonder how I could make it more smooth tomorrow.

I walk home feeling the sadness of the morning overtake me. Why is everything always a rush? Why do we not have more moments to really relax and enjoy each other? I spent the only hour and a half that I have with my daughter before school being her nurse, dietician, and respiratory therapist. I just want to be her mom.

I try to imagine what it would be like to homeschool. I always imagined homeschooling my kids, it seemed connected and involved, the way a parent/child relationship should be — the part I often feel like I miss out on being a CF mom. As soon as May was diagnosed at 3, I felt like it was verboten to even consider homeschooling. I would be the “bubble mom” and I would be asking my daughter(s) to be social outcasts. While none of this is true, we all have to admit that, in the CF community, homeschooling has a negative connotation. It does not hold the same meaning and justification as it does for kids without CF. It is simply one more attempt toward complete isolation from viruses or bacteria.

I bring the stroller when I pick Maylie up from school. It is her “on” month for Cayston and we walk downtown while she does her antibiotics. We stop by the park on the way home — airway clearance in disguise. It is almost five o’clock when we arrive home. Time to start dinner and treatment. She sets up all of her colors and coloring books on the counter as I set up her nebs and PEP. We spend the next 45 minutes working together to “take deep breaths,” and “huff,” over and over and over. It is a script that is so heavily embedded in my head that I don’t even think about it anymore, I am like Pavlov’s dog. We finish nebs just in time for dinner. We finish dinner just in time for Cayston and we finish Cayston just in time for bed. We give our hugs and our kisses and I go to bed with the same heavy heart I did last night and the night before. I ache for our time to be more valuable, more connected. I can count moments on my hand per day that our interactions were not CF related. I want more than that. So, despite the taboo, I start to revisit the idea of homeschooling.

The first week of homeschooling I questioned myself endlessly. Did I do this for the right reasons? Did I take on yet another battle to fight with her– treatment, pills, feeding tube and now school? Will she suffer socially? These concerns, while all valid were moot by the end of the week.

Last year, Maylie missed 76 days of school. Kindergarten was Maylie’s first “consistent” school experience and she had the most unbelievably supportive classroom. I was in awe of how understanding and forgiving they were of her frequent inability to control her GI system as well as her lack of awareness of the how “uncouth” it is to spit mucus on the playground. I never once saw a child make fun the many hurdles that CF places in front of our children, I saw a genuine spirit of sweet acceptance. Leaving Bozeman and this school was a difficult choice and had we stayed my consideration for homeschooling may have turned out differently.

I believe in the public and private school systems, I love the feeling of community that is formed in a classroom, but I also saw the valuable moments that I had with May just slipping away when I sent her off to school. We are now a mere two months into our first year of homeschooling. We awake on May’s schedule when she is sick, allowing her body more time to heal. We have a peaceful and productive treatment and a relaxing breakfast, all of which foster a new relationship for us. We have circle time, language arts, science, math, music, arts & crafts, social studies and relevant fieldtrips. After seemingly endless research on home school programs, I chose a program that is through an accredited private school, so the syllabus, books, crafts, etcetera are all pre-developed for us, her assignments are sent to her teacher and she is graded on her projects monthly. Homeschooling has allowed us to have a lot of flexibility, but I know that she is also receiving a really reputable education here at home. While homeschooling adds more responsibility, the additions of time, memories and overall contentment for us as a family override those initial hesitations.

 

65 Roses, NACFC & Hope

Originally Posted on www.brfcf.org on October 19, 2012

New York in the fall is like seeing a real Monet instead of a bathroom print. My eyes struggle to incorporate the red, orange, and yellow hillsides as they roll along the Hudson River. The colors undoubtedly reminded me of the passing of time. Time for us is typically marked by quarterly CF visits and rotating months of Cayston – markers that seem to sneak behind us like unwelcome knocks at the door.

Last year’s NACFC opening session was a flood of excitement. Vertex 770 was poised to become CF’s first control drug. It would only serve three percent of population but, it presented “the proof of concept” and the energy surrounding it was contagious and undeniable. At this year’s conference, it was the potential of the combination of 809 and Kalydeco that spurred enthusiasm. The results have been fabulous, and I am so happy for those friends and families whose lives may soon experience a change in ways I cannot allow myself to imagine.

I vividly remember the stark, cold walls of the clinic room three years ago. I think I was there. Was it really my life? It was Maylie’s second CF clinic appointment, and my heart was still weighed from her diagnosis. I was overwhelmed by all of the new information, yet still felt ignorant about CF. I remember holding my two girls when the nurse walked through the door and told me about Ataluren. She told that it was doing well in the trials; she told me 2012 would be an amazing year for individuals and families with nonsense mutations. I remember feeling so lucky.

Now I sit on a train pointing towards New York City. Three days have passed since NACFC, but I’m still trying to make sense of my thoughts and emotions. I spent the weekend deciding if my knee-jerk reaction to some of the blogs from individuals who attended the conference was warranted, or if I was simply overreacting to the over inflation of hope that I saw spattering the pages of Facebook.

Here were my published thoughts:

I find that hope drives me in so many areas of my life- at times it is the only thing I can hold onto as I look at my sweet May. I see myself as someone who believes in the impossible and I want to share that hope, that faith with others. I do have to say that I have read blogs and posts from other parents from NACFC 2012 and I find myself a little scared, maybe even slightly irritated as hope IS what we all hold onto. There was hope at the conference, but the over inflation of hope is a disservice to our community. Please do your own research. Call your clinic or the CFF and ask for reliable overviews of the data that was presented.

Twenty twelve has come and mostly gone, and a control for nonsense mutations is not around the corner. I am not a pessimist. CF researchers are doing amazing work. If I have learned anything in the past three years, it’s that humans are complex organisms and cystic fibrosis is a complicated disease. It is no simple feat to manipulate defective proteins so they function properly in living cells. We are getting closer each day, but progress is incremental.

My aforementioned post was intended to state that there is much hope in the world of cystic fibrosis, but it is important to live beyond hope. As a member of the CF community, I feel the need to be protective of all of our hearts as the risk in our community is so great. Don’t get me wrong, I am filled with hope for Maylie and I eagerly anticipate Maylie’s version of Kalydeco. However, my hope for May goes beyond a “CF cure.” Will she have the contagious smile and dedication of Emily Schaller? Will she make everyone laugh and be an amazing friend like Josh Mogren? Will her voice echo on stage like Rose Longue, and inspire all those who hear it? To me, hope for CF is more than just a control for the symptoms or a means to fix the basic defect, it is the desire to raise my daughter in the likes of all the amazing adults who have grown strong because of the hurdles and fears that CF has thrown at them. Each one of them have enriched my life beyond words and made the CF journey more manageable.

So here’s to hope for the future in whatever it may hold, and to being alive and present in what we have today.