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.
People with cystic fibrosis have thick, sticky mucus that blocks passages in their lungs, making it difficult for them to breathe and predisposing them to infections. New drugs are under development that will help break up and hydrate mucus in the lungs to make it easier to clear.
Mucus is essential in the lungs, where it traps germs and pollutants that we inhale. Tiny hairs on the outside of cells, called cilia, propel the mucus out of the lungs and into the throat where the mucus can be swallowed or coughed out.
In people with cystic fibrosis, mucus is dehydrated, becoming so thick and sticky that the cilia are unable to propel mucus out of the lungs. As a result, the mucus clogs the airways, making it difficult to breathe. Because the mucus in people with CF is also abnormal in other ways, it is less able to kill germs than the mucus in healthy people, creating a fertile breeding ground for infections.
In people with CF, mucus becomes thick and sticky because of problems with the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The CFTR protein normally forms a channel to transport chloride -- a component of salt -- through the membranes of cells lining many surfaces in the body, including the surface of the lung. When the protein is not working correctly or is not present at the cell surface, chloride becomes trapped in cells and cannot attract the fluids necessary to hydrate the cell surface. Without the fluids, mucus becomes dehydrated and takes on a thick, sticky consistency.
This animation depicts how mucus builds up in the lungs of people with cystic fibrosis.
Researchers believe another protein channel on the surface of the cell adds to this hydration problem. This channel, known as the epithelial sodium channel (ENaC), absorbs sodium (another component of salt) into the cell, which also helps to maintain the correct balance of salt and fluids on the cell surface. In people with CF, researchers think the sodium channel is hyperactive and takes in too much salt. Thus, this overactive channel contributes to the dehydration problem in the airway.
To advance the development of new treatments, scientists are studying how mucus is secreted normally to identify how to restore its proper consistency and germ-fighting qualities in people with CF. They are researching the following:
Scientists also are conducting research on how much liquid covers the cells lining the airways and what factors contribute to airway hydration. In addition to the chloride and sodium channels, researchers are examining the role that calcium-activated chloride channels (CaCC) have in hydrating mucus and whether these channels could be stimulated to improve the flow of fluids in the airways.
Researchers are evaluating drugs for their effectiveness in preventing, thinning, and breaking up the mucus in the airways. These drugs include CFTR modulators, ENaC inhibitors, CaCC activators, and mucolytics (mucus thinners).
By restoring function to the CFTR protein, CFTR modulators help restore the flow of chloride out of the cell and prevent mucus from becoming dehydrated, making it easier for people with CF to clear their lungs. Early clinical trial results have indicated the triple-combination modulators will likely be more effective than current modulators and could potentially treat a much larger population of people with CF.
Researchers also are working on drugs that are mutation-agnostic, which means they can help all people with CF regardless of their mutations. One such drug would prevent mucus from becoming dehydrated, inhibiting the hyperactive ENaC to enable more salt on the cell surface. The salt would then attract fluids, hydrating the mucus. Several ENaC inhibitors are under development in clinical trials.
In this video, Scott H. Donaldson, M.D., associate professor of medicine and director of the adult CF center at the University of North Carolina, talks about ENaC inhibitors and other approaches being explored to improve mucociliary clearance.
For more information on current treatments, visit Medications.
Follow Us On
Drug Development Pipeline
We're attacking the disease from every angle. The Foundation is propelling every drug in our pipeline forward and will not stop until there is a cure.
About the Drug Development Pipeline
The Cystic Fibrosis Foundation facilitates the development of promising new drugs, and the drug development pipeline enables you to track the progress of these potential therapies.
How Drugs Get on the Pipeline
The Drug Development Pipeline features an extensive list of drugs that are in development or approved for treating cystic fibrosis. For a drug or program to be shown on the pipeline, it must meet certain conditions.
How an Experimental Drug Becomes an Approved Therapy
Ever wondered what it takes for a potential cystic fibrosis drug to become approved by the U.S. Food and Drug Administration? Here's an explanation of the four phases of clinical research.
Help us blaze a trail to better treatments and a cure for CF. Find a clinical trial that may be right for you.
Cystic Fibrosis Foundation
4550 Montgomery Ave.
Suite 1100 N
Bethesda, MD 20814
800-344-4823 (toll free)
Sign up for our emails