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.
I used the Cystic Fibrosis Foundation Patient Registry Annual Data Report to ask my care teams questions to more effectively manage my CF. You can, too.
February 7, 2019
How CFTR Modulators Changed My Reality
Coping With the Loss of My Brother to CF
Thanks to advancements in care and research, cystic fibrosis is slow-burning when we aren't experiencing crises like sepsis or in end-stage disease. Due to the slowing disease progression, I once viewed the occasional loss of a few lung function percentage points as inconsequential. I didn't see the big picture until I entered end-stage CF -- those lost points add up.
In high school, I argued that my doctor treated my infections too aggressively with IV antibiotics: “I only lost 2 percent of my forced expiratory volume in 1 second (FEV1) this month!” My doctor responded by printing a line graph showing my lung function in freefall over two years. “Oh. I never viewed my lung function that way before.” Suddenly, I didn't think through the lens of what I'd lost over the month, but what I'd lost over the past two years -- over 20 percent FEV1. From that day on, I took more seriously the treatments, diet, and exercise I'd neglected.
It's funny what a simple graphic of the “big picture” can do to one's mindset.
However, not all doctors think like mine. Actually, she's one of only two doctors in the legions I've met who showed me the big picture. Many doctors underestimate health data's value to patients, assuming it's useful only for researchers and specialists. The Cystic Fibrosis Foundation doesn't think along those lines, nor should you.
For more than 50 years, the Foundation has collected voluntary Patient Registry Data from CF Foundation-accredited care centers in the United States, and they make much of it available for the patient population to review. These data reveal trends that influence improvements in personal, center-wide, and nationwide CF care.
Using my Patient Summary Report, I can review graphs similar to what my doctor once showed me to analyze the downward or upward crawls of my weight and lung function. I can also check how often I've been hospitalized and on what days. The personalized report even displays the dates of my last positive bacterial cultures.
Knowing my detailed patient history empowers me to assess my past for patterns that could inspire a new path of treatment.
You can get all of this data, too, by asking members of your CF care team for your Patient Summary Report.
The Patient Registry Data also fuels a feature that allows patients to compare vital statistics between CF care centers. You can use this information to start a discussion between you and your care team to inform which care center is right for you. Many variables can raise or drag a center's statistics, but if you're moving to a new region with more than one CF center, comparing hospital statistics could give you confidence in your care selection.
My favorite product of the registry data is the 2017 Patient Registry Annual Data Report of nationwide CF statistics. You may think the document is nothing more than an interesting (or even boring) overview of the CF community, but I've found personal hopes and worries while reviewing the data, and as a result, I've rethought my care strategies.
Here are just a few ideas for how to use the Annual Data Report to influence your care and lifestyle:
Know which bugs to avoid: Pages 31-36 reveal which bacteria are common in the CF population, and the data even split into age groups on page 32. Using this information, we see that nontuberculous mycobacteria are spreading in our community. Have your doctors discussed how to avoid NTM? Ask them, because there are precautions you can take.
Know your mutation class risks: Did you know your mutation class puts you more or less at risk of certain symptoms? For example, I have Class II mutations. People with this class of mutations are more likely to have liver disease and CF-related diabetes (p. 66) than people whose mutations are in Classes IV-VI. This knowledge makes me prioritize taking precautions like drinking extra water and less alcohol, and getting regular blood sugar checks.
Know you're not alone: The mental health statistics on p. 63 are staggering: Above the age of 18, 20.6 percent of us report anxiety disorder and 26.8 percent of us report depression. Many people don't seek mental health diagnoses because they feel such problems alienate them. But, this data makes clear that many in our community have the same struggles -- I do, too. Please, find help if you need to.
Know there's hope: It's the rocket fuel for our fight. Look at our progress and derive hope from it: The number of people with CF who have Pseudomonas aeruginosa has steeply decreased (p. 31). More people are getting lung transplants and living longer with them (pgs. 73-74). Our median survival rate rose from 31 years old in 1993 to nearly 44 in 2017 (p. 6). The beauty of numbers is that they don't lie: We're getting much better at tackling this disease.
Ask questions: Bring the data into your next clinic visit. Ask your CF care team how they use the Registry data to decide what treatments to recommend or changes to a nutrition or fitness plan that may be worth trying.
Read the 2017 Patient Data Registry report to see what calls out to you. Empower yourself.
Having difficulty interpreting the data? Check out this helpful resource. Don't want to read the report? There's a graphic-heavy data highlights handout.
Join the conversation on Facebook.
Adult with CF
Brad lives in San Jose, Calif. as a freelance writer and editor. He graduated from the University of Hawaii with degrees in history and journalism. When not writing small business profiles and cystic fibrosis awareness pieces, Brad is serving as president of the Northern California Chapter of The Lung Transplant Foundation. He's also hiking and rock climbing to celebrate his recent lung transplant. Follow Brad at his blog, Adamantium Joy, or on his Facebook page.
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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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