Learn about cystic fibrosis, a genetic disorder that affects the lungs, pancreas, and other organs, and how to treat and live with this chronic disease.
CF is a rare genetic disease found in about 30,000 people in the U.S. If you have CF or are considering testing for it, knowing about the role of genetics in CF can help you make informed decisions about your health care.
If you or your child has just been diagnosed with cystic fibrosis, or your doctor has recommended testing for CF, you may have many questions.
Diagnosing CF is a multistep process. A complete diagnostic evaluation should include a newborn screening, a sweat chloride test, a genetic or carrier test, and a clinical evaluation at a CF Foundation-accredited care center.
Raising a child with cystic fibrosis can bring up many questions because CF affects many aspects of your child’s life. Here you’ll find resources to help you manage your child’s daily needs and find the best possible CF care.
Living with cystic fibrosis comes with many challenges, including medical, social, and financial. By learning more about how you can manage your disease every day, you can ultimately help find a balance between your busy lifestyle and your CF care.
People with CF are living longer, healthier lives than ever before. As an adult with CF, you may reach key milestones you might not have considered. Planning for these life events requires careful thought as you make decisions that may impact your life.
People with cystic fibrosis are living longer and more fulfilling lives, thanks in part to specialized CF care and a range of treatment options.
Cystic Fibrosis Foundation-accredited care centers provide expert care and specialized disease management to people living with cystic fibrosis.
We provide funding for and accredit more than 120 care centers and 53 affiliate programs nationwide. The high quality of specialized care available throughout the care center network has led to the improved length and quality of life for people with CF.
The Cystic Fibrosis Foundation provides standard care guidelines based on the latest research, medical evidence, and consultation with experts on best practices.
As a clinician, you’re critical in helping people with CF maintain their quality of life. We’re committed to helping you partner with patients and their families by providing resources you can use to improve and continue to provide high-quality care.
As part of the Cystic Fibrosis Foundation's mission to help improve the lives of people living with cystic fibrosis, the PSDC initiative taps the CF community to inform key efforts to support the management of daily care.
Your cystic fibrosis care team includes a group of CF health care professionals who partner with you to provide specialized, comprehensive CF care.
Many people living with cystic fibrosis and their families face complicated issues related to getting the care they need. Cystic Fibrosis Foundation Compass makes sure that no one has to do it alone.
CF Foundation Compass is a service that helps people with CF and their families with navigating insurance options, connecting to legal information and experts, finding available financial resources, and tackling other life issues.
CF care team members are paramount in providing highly specialized care to people living with CF. CF Foundation Compass can help by serving as a strategic ally for care teams, so team members can focus on their patients’ care.
CF Foundation Compass can help you navigate insurance, financial, legal, and other issues you are facing. Use this online form to start your conversation with a Compass case manager today.
The Cystic Fibrosis Foundation is the world’s leader in the search for a cure for CF and supports a broad range of research initiatives to tackle the disease from all angles.
The CF Foundation offers a number of resources for learning about clinical trials and treatments that are being developed to improve the treatment of cystic fibrosis.
Our understanding of CF continues to evolve as scientists study what causes the disease and how it affects the body. These insights drive the development of new and better treatments and bring us one step closer to a cure.
Researchers, supported by the CF Foundation, have made tremendous advances to improve the health and quality of life of people with CF. We are committed to providing the tools and resources you need to continuously build upon this work.
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.
Elaine K. Malik
February 11, 2019
Redefining the Definition of Wellness
How I Use the CF Patient Registry to Inform My Care
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.
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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Elaine K. Malik
Adult with CF
Elaine, better known as Katie, is an opera singer, choreographer, voice actor, and freelance writer based in Seattle. Katie's travels have taken her around the country and the globe, 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. She is also a registered yoga teacher and creator of a “Yoga for Cystic Fibrosis” video series for Pactster.com
in collaboration with the Cystic Fibrosis Trust. Katie is the founder of CF Yogi and a recipient of the Foundation's 2018 Impact Grant. 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 Facebook, Instagram, Twitter, and LinkedIn, or visit her website, katiemalik.com.
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This site contains general information about cystic fibrosis, as well as personal insight from the CF community. Opinions and experiences shared by members of our community, including but not limited to people with CF and their families, belong solely to the blog post author and do not represent those of the Cystic Fibrosis Foundation, unless explicitly stated. In addition, the site is not intended as a substitute for treatment advice from a medical professional. Consult your doctor before making any changes to your treatment.
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