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.
Trikafta has done wonders for my health but not my waistline. I was relieved to find out, however, that I wasn't alone in my concern about my sudden weight gain and that bringing the topic up to my care team didn't mean that I wasn't grateful for all Trikafta has done for me.
March 6, 2020
I’m Still Learning How to Talk About My CF With My Mom
Stepping Back as My Daughter Steps Forward
I remember the tears that started to well up in my eyes when I learned that Trikafta™ had been approved. My cystic fibrosis doctor had been telling me about how life-changing this drug was going to be and I was thrilled that it had received FDA approval.
I had heard about all the good things this drug was going to do -- improve lung function, enhance quality of life, increase energy and weight gain, and cause fewer CF exacerbations and less coughing. I was ready. I had been on Symdeko® for about two years and although it drastically improved my quality of life, Trikafta was supposed to be even better.
I started taking Trikafta and within 12 hours what I've heard called the “CF Purge” began -- bringing up a lot of mucus. Within a few days, that was finished, and the most noticeable change was my cough -- or lack thereof. I couldn't believe it. I remember asking my husband almost every night if he had heard me cough that day. It was so crazy. My lung function shot up -- 15 percent in just two weeks.
I had so much energy. I had been consistently going to the gym for the past four months and within the first couple weeks of Trikafta, I was doing something I hadn't done in more than seven years -- I ran an entire mile without stopping. This drug was everything I had hoped for.
Along with all the positive lung-related improvements, I also began to gain weight. My CF care team had said from the beginning that weight gain was experienced by people on Trikafta. I have been about the same weight within a few pounds for the past seven-to-eight years. My BMI was almost always in the healthy range but at the lower end. When I was younger, I had a G-tube to help me gain weight, but I had that removed when I was 19 and haven't had any trouble maintaining my weight for the last 10 years. I wasn't necessarily looking for weight gain. I knew the correlation between a higher BMI (in the healthy range) and improved lung function, so I wasn't opposed to gaining an extra pound or two if it meant keeping my lung function up.
What I wasn't expecting, however, was to gain 10 pounds in six weeks.
I am a college professor, so I was on winter break when I started seeing the weight gain, but it didn't really register with me about how much I had gained until I tried to get dressed for work and none of my work clothes fit. I have never been one to focus on the number on the scale; in fact, the only time I would ever check my weight was at clinic visits. Still, when I started to notice my clothes getting tighter, I decided to jump on the scale just to see if I had gained a few pounds. What I saw shocked me. How did I gain this much weight? How am I supposed to go to work when none of my clothes fit?
The question that I really struggled with the most was, “How could I be so ungrateful to be unhappy with something this drug has done?”
I love everything Trikafta had done for me, except this. Was that allowed? Could I be unhappy with one of the side effects and not seem like a vain individual? I knew there were people in the CF community for whom this drug didn't work or who were struggling to gain weight. How could I be struggling with this?
I knew I needed to talk to my CF care team about it. I am a very open person, and I don't like to keep things from them -- especially because I know their desire is always to help. I have a great relationship with my CF care team, especially my social worker, doctor, nurse, and dietitian. I knew I needed to talk to my doctor about my feelings, but I was worried. REALLY WORRIED. I was afraid he would dismiss my feelings, or worse (in my mind), tell me I was what I feared -- that I was being ungrateful.
I spoke with my social worker and in an hour-long cry session, explained how I was so happy and grateful for this drug, but I was really struggling with the weight gain. She reassured me that my feelings were valid and that I should talk to my doctor.
So, at my next clinic appointment I did just that. I shared my feelings, and prefaced it with my fear, “I need to talk to you about something that has been bothering me, but I'm afraid you're going to dismiss my feelings. I don't need you to fix it, I just need you to know this is how I'm feeling.” When I told my doctor, he listened. He immediately reassured me that this was expected and that I was not alone in my surprise. We talked about how the thing I was struggling with the most was not a number on a scale, because, honestly, I was happy with some of the weight gain, but how I was frustrated that none of my clothes fit. He explained that even though I thought my weight before Trikafta was good, what the weight gain really showed was that my body was actually becoming whole and healthy. For years, my body had been in a state of stress because of my severe lung disease, and now it could be whole. I asked if the weight gain would plateau, and he said it would. We also agreed to ditch the scale at home. And. I was told to go shopping for some new clothes (the dream of most girls, right?).
I encourage anyone with CF who may be struggling to share your feelings with members of your CF care team in a way that expresses your fears or concerns.
Don't shy away from sharing any of your issues even if you think they are not widely accepted. And for the CF care team members who may be reading this, I hope you will be a source of encouragement and provide a safe space for your patients to share their feelings whatever they may be.
Adult with CF
Meagan lives in southwest Virginia. She has a master’s degree in nutrition and a doctorate in Health Promotion and Disease Prevention Research, and she is a certified health education specialist. Currently, she is an assistant professor at a regional state university. Meagan serves the CF community as a member of the Ear, Nose, and Throat (ENT) Guidelines Committee and Teen Connections, and she actively participates in research trials at her CF care center. She is obsessed with her two mastiffs, Mya and Warren, and enjoys traveling with her husband, Ches.
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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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