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.
Finding a way to reduce lung inflammation in cystic fibrosis has challenged researchers, but we have made important advances in our understanding of this CF complication in just the last few years. Several drugs also are in development to fight another complication -- the inability to clear mucus from the lungs.
Katherine Tuggle, Ph.D.
October 19, 2018
NACFC Session Examines the Importance of Partnerships in CF Care
Fighting Infections in the Age of CFTR Modulators
A colleague once explained the complexity of inflammation by comparing it to a bowl of spaghetti. How do you find the one noodle (or pathway) that can reduce inflammation when there are so many?
The reality is, it's exceptionally difficult. Researchers have worked for years to find a drug that can dampen the exaggerated immune response in people with CF without affecting the body's natural defenses against infection or causing serious side effects. Inflammation may not get as much attention as other complications of cystic fibrosis, but it destroys lung tissue and can eventually lead to respiratory failure.
Significant progress has been made since 2015 when the Cystic Fibrosis Foundation convened a group of CF physicians and researchers to outline a path forward to tackle inflammation. This progress was the focus of today's Plenary 2, Anti-Inflammatories and Mucociliary Clearance Therapies in the Age of CFTR Modulators, at the 32nd annual North American Cystic Fibrosis Conference (NACFC) in Denver.
Improving treatments for complications of CF, like inflammation, was chosen as the theme for plenaries 1 and 2 because complications have a detrimental impact on the lives of people with CF. The Foundation expects that a large percentage of people with CF will still need these treatments in 20 years, despite the availability of highly effective cystic fibrosis transmembrane conductance regulator (CFTR) modulators. With this in mind, in 2017, the CF Foundation spent more money on research into CF complications than on any other area.
Janeil Whitworth, who has CF and takes a CFTR modulator, is battling chronic inflammation. In a video shown during the plenary, she talked about how happy she was to participate in one of the trials for a potential anti-inflammatory drug.
Janeil has been taking high doses of ibuprofen to reduce inflammation in her lungs, but she has to stop taking it when she goes on antibiotics for a pulmonary exacerbation. Life would be better, she said, if she could continue taking an anti-inflammatory while she received IV antibiotics.
What is exciting is that in the next few years, we are going to know whether Janeil's clinical trial, as well as the trials for three other potential anti-inflammatories are successful, said Felix Ratjen, M.D., Ph.D., who delivered the second plenary. Dr. Ratjen is an expert on inflammation and head of the division of respiratory medicine at The Hospital for Sick Children at the University of Toronto.
The results of these trials will hopefully lead to the approval of a novel therapy or inform a new way to address inflammation.
Dr. Ratjen said that inflammation is caused by dysfunctional CFTR protein, infections, and impaired mucus clearance in the lungs. He also explained how research has shown that neutrophils, a type of white blood cell, play a key role in CF lung inflammation.
In people with CF, neutrophils release massive quantities of enzymes that overwhelm signals to stop the immune system's attack on infection, leading to lung damage. Researchers have been looking into potential ways to inhibit these enzymes.
Dr. Ratjen talked about the positive Phase 2 trial results of acebilustat, a drug that would help resolve inflammation caused by neutrophils. He also discussed the Phase 2 trial results of lenabasum, which would increase the production of anti-inflammatory molecules while reducing the production of pro-inflammatory molecules. The results indicate that lenabasum can trigger the resolution of inflammation without suppressing the immune system.
In addition to funding anti-inflammatory research, the Foundation is supporting the development of therapies to aid in clearing the excessive mucus that builds up in the lungs of people with CF. This is an important area to address because the thick CF mucus in the lungs creates a fertile breeding ground for infections and makes it difficult to breathe. Although mucus clearance improves with the use of modulators, we need to make more progress in this area especially for those people who are not on modulators.
Currently, the Foundation is supporting multiple drug development programs focused on clearing mucus by improving airway hydration and thinning the CF mucus.
Although recent advances in CF care and drug development have led to significant improvements in the lives of people with CF, there is still a need for more effective mucus clearance therapies, anti-inflammatories, and anti-infectives.
People like Janeil need better drugs.
“When I think about the fact that my chronic inflammation can be controlled with a new treatment, I feel excited,” she said at the end of the plenary video. We see her walking hand-in-hand with her husband, Peter, and their 1-year-old son Fenn.
“It's really important that we continue to invest time and money in developing new anti-inflammatories so that we can have better quality of life and reach the milestones that we want to reach.”
Watch the full plenary below.
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Katherine Tuggle, Ph.D.
Director of Research, Cystic Fibrosis Foundation
Katherine has been involved with CF research since 2009 as both a graduate student and postdoctoral fellow at the University of Alabama at Birmingham. In 2014, she joined the Foundation to oversee its basic science research programs, including the 11 basic science research centers around the country. She is also involved in a number of Foundation initiatives focused on developing and validating model systems that may be used for personalized medicine. In her free time, Katherine enjoys traveling with her husband and spending time with her German shepherd.
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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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