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.
These guidelines were developed by consensus based on expert opinion and a medical literature review to provide evidence-based recommendations for chronic medication use for lung health maintenance.
Mogayzel PJ Jr, Naureckas ET, Robinson KA, Mueller G, Hadjiliadis D, Hoag JB, Lubsch L, Hazle L, Sabadosa K, Marshall B; Pulmonary Clinical Practice Guidelines Committee. Cystic Fibrosis Pulmonary Guidelines: Chronic Medications for Maintenance of Lung Health. Am J Respir Crit Care Med. 2013 Apr;187(7):680-9.
The treatment of cystic fibrosis has continued to evolve and become more complex with the development of a wide variety of medication options to improve and maintain lung health. At the same time, these therapies place a significant burden on patients with CF and their families, and their optimal use remains open to discussion.
To aid care providers in the use of these medications, the Cystic Fibrosis Foundation established the Pulmonary Clinical Practice Guidelines Committee to evaluate therapies and provide recommendations for their use to treating providers. In 2007, the committee developed guidelines based on the existing evidence for each medication and for specific patient populations.
The 2007 chronic medications guideline was updated in 2013. Studies of new medications approved for use in the U.S. after the publication of the 2007 guideline and additional data published on existing therapies were reviewed by a panel of experts. The guideline includes recommendations for aerosol therapy (bronchodilators, antibiotics, and mucoactive agents), oral antibiotics, anti-inflammatory drugs, and cystic fibrosis transmembrane conductance regulator (CFTR) modulators.
Since the publication of the guideline in 2013, additional CFTR modulators have been approved by the U.S. Food and Drug Administration (FDA). Important questions regarding how to best use these medications, long-term risks versus benefits, and other issues remain to be answered. The consensus guideline is intended to synthesize the available data to offer recommendations to providers on management strategies targeting their individual patients.
A multidisciplinary committee of 17 members reviewed the 2007 guidelines and developed a series of questions related to chronic drug therapies for CF. An evidence-based review was commissioned from Johns Hopkins University, with systematic reviews completed for each question. New reviews were conducted for each question, as some questions were new or revised, new medications and indications had been developed, and full systematic reviews were not completed for all questions during the development of the 2007 guidelines.
The review was limited to parallel, or cross-over, randomized controlled trials (RCTs). Members of the committee disclosed any potential conflicts of interest. If any perceived conflict was identified, members did not participate in any discussions or decisions on recommendations regarding that therapy.
Subcommittees were created to review the evidence summaries and draft recommendations for presentation to the entire committee. Final recommendations were graded using the U.S. Preventive Services Task Force (USPSTF) scheme, which encompasses an estimate of the magnitude of net benefit and certainty of net benefit. Find additional information about the U.S. Preventive Services Task Force (USPSTF) grading definitions.
B2 agonists: Evidence insufficient to recommend for or against chronic use
Certainty of benefit is low and cannot estimate magnitude of benefit
Anticholinergics: Evidence insufficient to recommend for or against chronic use
Tobramycin: Mild lung disease -- Recommend use
Certainty of moderate benefit is moderate
Certainty of substantial benefit is high
Aztreonam: Mild lung disease -- Recommend use
Aztreonam: Moderate to severe lung disease -- Strongly recommend use
Other inhaled antibiotics: Evidence insufficient to recommend for or against chronic use
Anti-Staphylococcus aureus agents for chronic use: Evidence insufficient to recommend for or against chronic use
Anti-Staphylococcus aureus agents for prophylactic use: Recommend against use
Certainty of benefit is moderate and benefit is negative
Anti-Pseudomonas aeruginosa agent: Evidence insufficient to recommend for or against chronic use
Dornase alfa: Mild lung disease -- Recommend use
Dornase alfa: Moderate to severe lung disease -- Strongly recommend use
Hypertonic saline: All lung disease regardless of severity -- Recommend use
Inhaled corticosteroids: Recommend against use for patients without asthma or allergic bronchopulmonary aspergillosis (ABPA)
Certainty of no benefit is high
Oral corticosteroids: Recommend against use for patients without asthma or ABPA
Certainty of harm is high
Ibuprofen: Patients under 18 years old with FEV1 >60% -- Recommend use (physicians should obtain and target peak plasma concentration between 50-100 μg/ml)
Ibuprofen: Patients over 18 years old -- Evidence insufficient to recommend for or against chronic use
Azithromycin: Patients with chronic P. aeruginosa infection -- Recommend use (patients should be screened for nontuberculous mycobacterium before initiation and at 6- or 12-month intervals and withheld in the presence of active nontuberculous mycobacterium infection)
Certainty of moderate benefit is high
Azithromycin: Patients not chronically infected with P. aeruginosa -- Recommend use
Certainty of small benefit is moderate
Leukotriene modifiers: Evidence insufficient to recommend for or against chronic use
N-acetyl cysteine: Evidence insufficient to recommend for or against chronic use
Inhaled glutathione: Evidence insufficient to recommend for or against chronic use
Certainty of benefit is low and cannot estimate magnitude of benefit.
Ivacaftor: Patients with at least one copy of the G551 CFTR mutation -- Strongly recommend use
Michelle Prickett, M.D., (Northwestern) and Edward T. Naureckas, M.D., (The University of Chicago Medicine)
The guidelines were published in April 2013, they were reviewed in April 2019 and it was determined that no update is needed at this time.
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