How CFTR Modulators Changed My Reality

Many people with cystic fibrosis are counting on cystic fibrosis transmembrane conductance regulator modulators to improve their overall health. I'm learning that they have effects I hadn't counted on. 

| 7 min read
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Elaine K. Malik
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One of the most memorable and enduring scenes from “The Matrix” is the one in which Morpheus offers Neo two pills … one blue and one red:

“You take the blue pill -- the story ends, you wake up in your bed, and believe whatever you want to believe. You take the red pill -- you stay in Wonderland, and I show you how deep the rabbit hole goes.”

Neo has to decide whether he wants to embrace this new reality. It's an uncomfortable reality, but it's the true one.

He can stay in the world he always believed was the real one up until that point … where he's comfortable and he knows what to expect.

Or he can choose to fulfill his true potential and take the leap into the unknown.

Cystic fibrosis transmembrane conductance regulator (CFTR) modulators have that same ability to reshape my reality in ways I didn't imagine.

The Yellow Pill and the Blue Pill
Last summer I was offered two pills -- one yellow and one blue -- tezacaftor/ivacaftor (Symdeko®). But rather than choose one or the other, I had to choose both to get to Wonderland and see what reality looked like on the other side.

This double-combination therapy treats the root cause of my CF -- two different faults in the way the cells in my body create and process the CFTR protein. My DNA told my cells to do it this way. A mutated gene, identified as F508delta, told my body to do something different than your garden-variety human.

What is CFTR?
The CF Foundation says, “The CFTR protein helps to maintain the balance of salt and water on many surfaces in the body, such as the surface of the lung.”

This mutation causes my body to create CFTR proteins in a deformed shape. They fold in such a way that they can't do their job. Plus, that chloride channel that they travel through is closed. Even if the proteins were made in the right shape, they still couldn't get through the door.

Two defects, two pills prescribed to correct those differences. Yellow pill. Blue pill.

Unlike Neo's red pill, my pills haven't taken me to a completely alternate reality quite yet, but they have helped me glimpse my future truth.

The body I walk around in every day has been obstructed from its full potential for my entire life. Now I'm getting a peek at the truth of my body as it would be without this disruptive and fatal mutation.

I'm getting a sneak peek of the future that could arrive as soon as next year, when the third pill finally arrives.

A Glimpse at Wonderland
There is so much promise out there. This promise exists not just for me, but for thousands of us. For adults like me who were already blessed with the gift of reaching mid-life, this means that now we might have to think about heretofore foreign concepts like menopause and retirement plans.

Young adults may feel more driven to pursue the careers that weren't accessible to them before -- careers that have demanding hours and rely on healthy respiratory systems.

The youngest children may never even see a progression to severe lung disease at all.

It's the most exciting thing that's ever happened to me. And yet, if I'm being honest … it also intimidates me. Because based on my limited look at this new reality, there are some things I'm going to have to unlearn and relearn about my own body.

Exploring the New Reality
Tezacaftor/ivacaftor hasn't made a big impact on my lung function. But I'm noticing that I gain weight a LOT more easily than I used to. The “CF diet” I've learned to think of as normal is starting to manifest in my body like it would in a person with a regular human metabolism. For someone who's struggled their entire life to gain weight (and keep it on), this is a pretty dramatic shift in my body and in my understanding of what it means to take care of it.

For the first time in our 16 years of marriage, my husband and I can eat the same thing for dinner. This is huge. This is a quality-of-life metric that my care team can't measure.

The Body in the Mirror
At the same time, I've had a dawning realization that the body I'm seeing in the mirror is not the one I'm used to seeing. I want to feel like I can embrace this new body as I get used to seeing myself in the mirror, and on camera, in my new body.

To be honest, I get nervous now when I post pictures of myself on social media, because I know they don't match as closely with what the outside world (and especially the internet) tells women is “beautiful.”

I love this new body and this new fat in all these places that makes me feel warmer and more graciously feminine. But it's hard to reconcile that against the judgment of the world.

It may not look like much of a difference now, but when triple-combination CFTR modulators arrive, it's likely to bring about major change.

What “Healthy” Does (Not) Look Like
The picture that we see as “healthy” on fitness magazine covers and Instagram feeds is not really healthy at all. No matter how strong you are, a physique chiseled to the degree that you can see individual muscle fibers is not healthy.

That picture of “health” that we keep in our heads is actually what an underfed, undernourished body looks like. That's also what my body used to look like.

Navigating the New Normal
My new body has more gentle curves where there used to be lines of visible muscle. More prominent padding in places. I'm learning to embrace the curves. Based on what I already know, I expect that I will have to put some effort into relearning what a healthy diet looks like for me, in my new “Wonderland” body. I'm learning to navigate the new normal, in more ways than one.

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It also means navigating the changing fluid dynamics of my airways, and the impacts that has on my singing technique and my career as a singer. Adjusting the exercise-work-life balance for whatever new energy or stamina I achieve. Monitoring my blood sugars to see impacts on my CF-related diabetes.

I'm already discovering that things that I have always assumed just “are the way they are,” might not be what I thought they were, after all.

It's a little daunting to think about, relearning the world as I know it. But this is the challenge I've been waiting for all my life.

I'm ready for the red pill. Let's see how deep the rabbit hole goes.

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Topics
CFTR Modulators | Emotional Wellness
Katie-Malik-Headshot

An opera singer, choreographer, voice actor, and freelance writer based in Seattle, Katie's travels have taken her around the country and the globe. She has been on solo tours in Europe and Asia, and on multiple trips to Sweden -- where her inspirational story captured the country's heart on the top-rated, international, Emmy-winning TV series, Allt för Sverige. Also a registered yoga teacher, Katie created a video series for Pacster.com in collaboration with the Cystic Fibrosis Trust, “Yoga for Cystic Fibrosis.” She holds a BA in vocal performance and music theory from Seattle Pacific University, where she received the Philip J. Mack music scholarship and graduated magna cum laude. You can connect with Katie on FacebookInstagramTwitter, and LinkedIn, or visit her website, katiemalik.com.

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