Trikafta is here.

If you haven’t heard, Trikafta, aka The Triple Combo, was approved by the FDA last Monday (October 21, 2019). This is a breakthrough medication that directly targets one of the main Cystic Fibrosis genes. Those of us who have at least one copy of the Delta F508 mutation, which is about 90% of the CF population, will be eligible to try it. 

The results thus far have been hugely promising as many trial recipients have seen an increase in lung capacity, decreased mucus production and lung infections, and in many cases it has drastically improved overall quality of life. All of that, however, is not a guarantee that it will have the same effects for everyone and it also doesn’t mean all our bodies will be able to tolerate it. We each have to do our best to determine if and how it can benefit us. 

To someone not stuck in a loop of sickness, recovery and managing symptoms, it can seem like the math is easy: Sick + new treatment = better. 

But better is relative to where you are starting from and each person’s particular case. Therefore, managing expectations is a major component to this equation. 

For some of us better would the ability to make through a trip to the grocery store without having a coughing attack. Or another worthwhile improvement would be to not need supplemental oxygen, which would allow us to move about more freely without cords, tanks and looking so sick. Better could also mean less hospital admissions and/or a lessening of anxiety induced hyper-vigilance in coping with our disease progression. And some of us will hopefully find ourselves being able to return to work or school, start a family, and/or fulfill some big life goals. 

It’s important to understand however that new treatments are often packaged within language like,  Maybe, Hopefully, Possibly, and Soon. When these words are coupled with other alluring terms such as, More, Better, and Longer, the imagination can’t help but to start entertaining stories of what could be. “Maybe” the new drug could help me feel “better.” “Hopefully” it will enable me to do “more.” “Maybe” it could “possibly” even allow me to put off transplant “longer,” or at least add some years to my life. When can I get my hands on this drug? Soon. When could I start to see results? Also soon.

If you’ll notice, nothing that I just said painted an exact picture of anything. Like a good PR campaign, all the words are subjective, abstract, and opaque. At the most they give us permission to move our imagination toward positive outcomes rather than negative ones. Ultimately though, these words act as more of a Rorschach test than as a definitive picture of one’s future life. Because, just like a Rorschach, we each see what we want to see. It doesn’t describe anything of physical substance beyond our own subconscious biases.

We cannot know if or what Trikafta will allow us to do prior to starting it. All of us, no matter our health, have to learn to navigate hope and statistics with fear and opening ourselves up to possible disappointment. The small number of cases that don’t respond to the treatment, have horrible side effects, or that absolutely cannot take it, is part of the math that we have to do. Are we willing to risk hope, and our bodies for an unknown better

For many of us the answer is an unequivocal YES. But it’s personal. We all have our own path to walk with this disease. Therefore if you are someone supporting a person with CF who can take Trikafta, please don’t just hope for better and only ask, “is it working?” Instead, step back and ask about how they are holding hope and what they hope better can mean for them. Give them space to tell you all the ways this news is exciting, frustrating, scary and/or guilt inducing. This medication, like all the ones before and after it, is not a beginning or end, but rather part of their journey. We can’t let our imaginations distract us from staying current to what is happening in real time, today, in our bodies, hearts and emotions as we decide to say yes, again, to hope. 

How I confronted my fear of CF Clinic

I had my regular CF check up today, which happens every 3 months. I used to dread these appointments to the point where I would feel physically ill. I thought I would share how I finally confronted that anxiety and learned to advocate for the kind of holistic care I needed.

If you find these videos helpful I’d love it if you shared them! Help raise #cysticfibrosisawareness and that #mentalhealthmatters

#beanadvocate #speakup #askforhelp #youarenotalone

My contribution to Cystic Fibrosis Awareness month

May is CF awareness month and I wanted to do something to contribute to the conversation in a way that felt true to me.

So I’ve decided I wanted to try my hand at doing a video series! I will be taking you through conversations that having Cystic Fibrosis has both prompted and required me to have with myself, others and God.

My hope is that by giving you a glimpse into how I process my life with illness it opens up the possibility for richer and more connecting conversations with you and the world around me.

Here is my first one. Enjoy!

A poem for 2018

In this season of hesitation
I am learning bravery, 
and to ask,
why do I pause?
where is the fear taking me?

I remember that God is good
all the time,
and especially on Mondays,
when movement is easier.

I say goodbye to unhelpful questions
that keep me paralyzed and afraid of failure.
I say hello to all that is before me: 
awkward moments, uncertain hours, 
 a racing heart -
the feel of YES. 

Free Write

Writing without stopping. As fast as I can. I don’t know quite what it will be, I never do. It’s unnerving, every day to face this question of what I will write. I agonize all day long, wandering and wondering… why do I avoid it? What am I afraid will come out?

I just hold my breath until I can’t anymore, holding and holding, over and over again. Why can’t I just breathe in and out, write and rest, write and rest?

Sleep, think, write, walk, run, breathe, hold, release, hold, release. Catch, find, keep, release. I have the tension wrong, the purpose is off, the rhythm is bad. It’s unsustainable, ridiculous, holding and holding, fainting for lack of oxygen. Holding, agonizing, wringing my hands, avoiding the chair, looking at anything but the page.

For whatever reason my excuse is that I don’’t know what I will write, or what I need to work on. When the question is rather something that should be asked after the fact: What is this that I have written? What need is next? Not, what should I write? I’ve tried, its always doomed from the beginning. It’s like asking, what shall I do with this life? All the while forgetting Life is happening, in motion, some of it gone.

Hold tight but not too tight, you’ll hurt something, break apart when you should have been bent, or shatter when you were meant to conform. Breaking is hard, too much for the body. It must bend and twist and move, and glide and sing. Pull and push, invite and give. Not break, and hold and disappear. It is a conundrum. A truth. To work the movement. To bend the will, to listen to the voice, to say yes when all is screaming no.

The no is valid, it is the backdrop. It is the blank slate that every yes is put upon. It holds the tension, the edges, the lines, the glimmer. Without it the yes would have no texture, no feel or look. It would never be right without it’s opposing wrong, it’s blank space, with unending light. Light with no dark, dark with no light. Fall without summer.

To be here, to write, to think, to put it all down. Ten, nine, eight, seven…slipping into the hole of Alice in Wonderland, to find what needs to be found. You do not ask, why, what or how. Just do it: become, transform, right foot, left foot. Around and around. Step, flick, swish.


Journal entry. Date: January 2018

Schedule

A work schedule

full of a subtle tension

made their way

seeking a fresh start

This morphed into an unexpected decision about the future

due diligence

is more important than ever

After breakfast

just drive

Around noon

sprawl introspectively

The next day

Write over the base line

In September extract seven of the 11 of her songs

beats may be too on the nose

Before Christmas

Ask why

Tell the story

Use your voice

Poetry Found by Elizabeth Honan Amber May 2018