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 cystic fibrosis patients and families face complicated issues related to getting the care they need. But CF Foundation Compass makes sure that no one has to do it alone.
For many people with cystic fibrosis, dealing with insurance is as much a part of living with the disease as nebulizers and vests. Many people with CF and their families face issues related to getting the care they need, but no one has to do it alone.
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.
Our goal is to educate policy makers about the needs of people with cystic fibrosis so that they make smart decisions about CF-related research, treatment, and access to care.
We recognize the value of tapping into the expertise that only people with CF and their families have. We invite you to share insights to help improve and develop programs and services that support the daily lives of people with CF.
Our mission is to find a cure for cystic fibrosis and improve the quality of life for those living with the disease. We can't do it alone. Help us add tomorrows by giving today.
In addition to working for a cure, the CF Foundation supports programs and policies to improve the lives of people with CF. Help us by raising awareness of CF, participating in a fundraising event, or volunteering with your local chapter.
Airway clearance therapies improve the efficacy of the mucociliary escalator and elicit a cough. These guidelines reviewed and compared the various techniques and devices.
Flume PA, Robinson KA, O'Sullivan BP, Finder JD, Vender RL, Willey-Courand DB, White TB, Marshall BC; Clinical Practice Guidelines for Pulmonary Therapies Committee. Cystic Fibrosis Pulmonary Guidelines: Airway Clearance Therapies. Respir Care. 2009;54(4):522-537.PMID: 19327189
Airway clearance therapy (ACT), the process of improving the efficacy of the mucociliary escalator as well as eliciting a cough, is at the center of chronic therapies necessary to maintain lung health in individuals affected by cystic fibrosis. Several different types of airway clearance devices and device-independent airway clearance techniques have been developed in recent decades. The purpose of the 2009 Cystic Fibrosis Foundation clinical practice guideline was to review and compare these devices and techniques, focusing on outcome measures such as sputum production and lung function. Airway clearance therapies that were evaluated included:
Overall, the committee recommended that all people with CF should perform airway clearance to maintain lung function and improve quality of life (level of evidence, fair; net benefit, moderate). After review of the available literature, no form of ACT was shown to be superior to another form of ACT. The committee went on to recommend that the choice of ACT be individualized to the patient, as patient-specific factors may make one form of ACT superior to another for the individual patient. Finally, the committee recommended aerobic exercise as an adjunctive therapy for airway clearance, as it has additional overall health benefits.
In CF, mucociliary clearance is impaired due to dehydration of the aqueous layer of the airway surface liquid as a direct result of the dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Clinically, CFTR dysfunction results in decreased mucociliary clearance, airway obstruction, recurrent respiratory infections with neutrophil migration into airways, and an exuberant inflammatory response leading to damage to lung parenchyma over time.
ACT uses patient maneuvers or devices to improve mucociliary clearance and augment the cough. Many different forms of ACT have been advocated, and the purpose of this Cystic Fibrosis Foundation clinical practice guideline was to evaluate and compare ACTs to provide support for clinical decision making regarding specific therapies.
The committee initially met to identify ACTs that were believed to be most commonly used in clinical practice and for which there would be peer-reviewed literature available for evaluation. The two primary questions used for ACT assessment were:
The following outcome measures were included in analysis:
A systemic literature review was commissioned from Johns Hopkins University to identify all relevant literature. When possible, the recommendations were graded using the U.S. Preventative Services Task Force (USPSTF) grading definitions.
Level of evidence: Fair
Net benefit: Moderate
Level of evidence: Fair
Level of evidence: Fair
Grade of recommendation, consensus recommendation: B
Level of evidence: Fair
Net benefit: Moderate
Since the development of the original guidelines in 2009, several manuscripts relevant to ACT have been published. These manuscripts have not been reviewed or endorsed by the guidelines committee.
Airway clearance therapy represents a long-standing and important part of CF care. In the 2009 guidelines, several questions were identified as needing further research:
Relevant manuscripts published after the original guidelines are listed below. These manuscripts have not been reviewed or endorsed by the guidelines committee.
Michael Glenn O'Connor, M.D. (Monroe Carell Jr. Children's Hospital at Vanderbilt) and Brian P. O'Sullivan, M.D. (Geisel School of Medicine at Dartmouth)
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