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.
Nontuberculous mycobacteria can cause chronic pulmonary infection or can reside in the lungs without causing progressive disease. Challenges include making decisions on when and how to initiate treatment.
Floto RA, Olivier KN, Saiman L, Daley CL, Herrmann JL, Nick JA, Noone PG, Bilton D, Corris P, Gibson RL, Hempstead SE, Koetz K, Sabadosa KA, Sermet-Gaudelus I, Smyth AR, van Ingen J, Wallace RJ, Winthrop KL, Marshall BC, Haworth CS, U.S. Cystic Fibrosis Foundation and European Cystic Fibrosis Society. U.S. Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus recommendations for the management of nontuberculous mycobacteria in individuals with cystic fibrosis. Thorax. 2016;71:i1-i22.
Nontuberculous mycobacteria (NTM) are ubiquitous environmental organisms that can cause chronic pulmonary infection, particularly in individuals with pre-existing lung disease, such as cystic fibrosis. NTM lung disease in CF is emerging as a significant threat to these individuals, although there is limited data on diagnosis and treatment.
To better provide guidance for this challenging disease, the U.S. Cystic Fibrosis Foundation and the European Cystic Fibrosis Society (ECFS) convened a committee of experts to address the specific issues surrounding NTM pulmonary disease (NTM-PD) in individuals with CF. This summary highlights a comprehensive list of questions associated with screening, microbiology, diagnosis, treatment, and issues regarding transplantation.
Nontuberculous mycobacteria are increasingly being isolated from the sputum of individuals with CF with estimates of prevalence increasing from 1.3 percent in 1984 to 12 percent in 2012. The most commonly isolated organism is Mycobacterium avium complex comprising 72 percent of NTM positive cultures, followed by M. abscessus.
NTM-PD is defined by microbiological, clinical, and radiographic features; although it is clear that NTM can reside in the lungs of individuals with CF without causing progressive disease, making decisions on when to initiate treatment is challenging. Other challenges exist in knowing how best to identify NTM, how to initiate treatment, and how NTM-PD affects lung transplantation consideration. The CF Foundation and ECFS created these consensus guidelines to help standardize the approach to NTM-PD.
The CF Foundation and ECFS convened a 19-member committee of adult and pediatric experts in CF and NTM-related lung disease. Using the PICO (population, intervention, comparison, and outcome) format, questions were developed and systematic literature searches were conducted. The committee drafted and voted on the recommendations accepting a recommendation if 80 percent of the committee agreed. The committee included 50 statements in the final consensus recommendations.
Cultures for NTM should be performed annually in spontaneously expectorating individuals with a stable clinical course.
Oropharyngeal swabs should not be used for NTM screening.
Cultures and smears for acid-fast bacilli (AFB) from sputum, induced sputum, bronchial wash, or broncho-alveolar lavage samples can be used to evaluate individuals with CF for NTM pulmonary disease.
Nonculture-based methods should not be used for detecting NTM in respiratory tract samples.
For M. avium complex, clarithromycin susceptibility testing should be performed on an isolate recovered prior to initiation of treatment.
NTM treatment should be considered for individuals with CF who have pulmonary disease as defined by the American Thoracic Society and the Infectious Diseases Society of America.
Individuals receiving azithromycin as part of their CF medical regimen who have a positive NTM culture should not continue azithromycin while evaluation for NTM disease is underway.
The continuation phase should include a daily oral macrolide (preferably azithromycin), and inhaled amikacin, in conjunction with 2-3 of the following additional oral antibiotics: minocycline, clofazimine, moxifloxacin, and linezolid, guided but not directed by susceptibility testing.
Monotherapy with a macrolide or other antimicrobial should never be used in the treatment of M. abscessus complex or M. avium complex pulmonary disease.
Clarithromycin-sensitive M. avium complex pulmonary disease should be treated with a daily oral antibiotic regimen containing a macrolide (preferably azithromycin), rifampin, and ethambutol.
An initial course of IV amikacin should be considered in the treatment of M. avium complex pulmonary disease in the presence of one or more of the following:
The presence of current or previous respiratory tract samples for positive NTM should not preclude individuals being considered for lung transplantation.
Individuals with CF who have NTM pulmonary disease and are being evaluated for transplantation should commence treatment prior to transplant listing.
The presence of persistent M. abscessus complex or M. avium complex infection despite optimal therapy is not an absolute contraindication to lung transplant.
Relevant manuscripts published after the original guidelines are listed below. These manuscripts have not been reviewed or endorsed by the guidelines committee.
Tara Lynn Barto (Baylor College of Medicine, Houston, Texas) and Kenneth N. Olivier (Cardiovascular and Pulmonary Branch, National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, Maryland)
The guidelines were published in January 2016, they were reviewed in April 2019 and it was determined that no update is needed at this time.
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