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.
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Allergic bronchopulmonary aspergillosis is a hypersensitivity lung disease that results from exposure to Aspergillus fumigatus and occurs primarily in people with asthma or with cystic fibrosis. These guidelines were developed via a consensus conference of experts in 2003.
Stevens DA, Moss RB, Kurup VP, Knutsen AP, Greenberger P, Judson MA, Denning DW, Crameri R, Brody AS, Light M, Skov M, Maish W, Mastella G, and participants in the Cystic Fibrosis Foundation Consensus Conference. Allergic Bronchopulmonary Aspergillosis in Cystic Fibrosis-State of the Art: Cystic Fibrosis Foundation Consensus Conference. Clin Infect Dis. 2003 Oct; 37 (Suppl 3), S225-64.PMID: 12975753
The 2003 recommendations addressed diagnostic criteria, screening, imaging, and treatment recommendations. Since 2003, there has been advancement in diagnostic ability via serology and immunologic testing. Most advancement has occurred in the treatment options. There has been more clarity in steroid dosing, but additional treatment options, including intravenous (IV) pulse steroids, systemic and inhaled antifungals, and anti-immunoglobulin E (IgE) therapy, have been explored. Future directions include the need for improved animal models as well as controlled treatment studies.
Allergic bronchopulmonary aspergillosis (ABPA), a lung disease of hypersensitivity to Aspergillus fumigatus (A. fumigatus) that primarily occurs in people with asthma or with cystic fibrosis, is seen in 2 to 19 percent of people with CF. People with CF may be predisposed to ABPA because of abnormal airway surface liquid and CF mucus. The disease is characterized by a variety of clinical and immunologic responses to A. fumigatus antigens. Clinical manifestations include:
The diagnosis of ABPA in people with CF is difficult and often delayed because of shared clinical features. ABPA has been associated with accelerated deterioration in lung disease, so timely diagnosis and treatment are imperative.
An expert panel was convened and a consensus conference held in 2001. The panel used the United States Preventive Services Task Force, 1989 grading methodology.
Acute or subacute clinical deterioration (cough, wheeze, exercise intolerance, exercise-induced asthma, decline in pulmonary function, increased sputum) not attributable to another etiology.
Serum total IgE concentration of >1000 IU per mL unless patient is receiving systemic corticosteroids (if so, retest when steroid treatment is discontinued).
Immediate cutaneous reactivity to Aspergillus (prick skin test wheal of ≥3 mm in diameter with surrounding erythema, while the patient is not being treated with systemic antihistamines) or in vitro presence of serum IgE antibody to A. fumigatus.
Precipitating antibodies to A. fumigatus or serum IgG antibody to A. fumigatus by an in vitro test.
New or recent abnormalities on chest radiography (infiltrates or mucus plugging) or chest CT (bronchiectasis) that have not cleared with antibiotics and standard physiotherapy.
Acute or subacute clinical deterioration (cough, wheeze, exercise intolerance, exercise-induced asthma, change in pulmonary function, or increased sputum production) not attributed to another etiology.
Total serum IgE concentration of >500 IU per mL. If ABPA is suspected and the total IgE level is 200-500 IU per mL, repeat testing in one to three months is recommended. If patient is taking steroids, repeat when steroid treatment is discontinued.
Immediate cutaneous reactivity to Aspergillus (prick test wheal >3 mm with surrounding erythema, off systemic antihistamines) or in vitro demonstration of IgE antibody to A. fumigatus.
One of the following:
Maintain a high level of suspicion for ABPA in patients >6 years of age.
Determine the total serum IgE concentration annually. If the total serum IgE concentration is >500 IU per mL, determine immediate cutaneous reactivity to A. fumigatus or use an in vitro test for IgE antibody to A. fumigatus. If results are positive, consider diagnosis on the basis of minimal criteria.
If the total serum IgE concentration is 200-500 IU per mL, repeat the measurement if there is increased suspicion for ABPA, such as by a disease exacerbation, and perform further diagnostic tests (immediate skin test reactivity to A. fumigatus, in vitro test for IgE antibody to A. fumigatus, A. fumigatus precipitins, or serum IgG antibody to A. fumigatus and chest radiography).
First line: systemic steroids -- prednisone 0.5-2.0 mg per kg per day, one to two weeks -- then attempt to taper within two to three months.
Second line: antifungal -- oral itraconazole 5 mg per kg per day (max 400 mg per day) for three to six months (if slow or poor response to steroids, relapse, or steroid toxicity).
Bronchodilators, inhaled steroids, antiasthma drugs only if indicated for asthma.
New animal models are needed to better represent ABPA in CF. For example, Urb et al. developed a promising murine model for chronic Aspergillus airway infection (Infect Immun 2015, 83, 3590).
New and/or validated ways to better classify and distinguish Aspergillus lung phenotypes (i.e., colonization, infection/bronchitis, sensitization, and ABPA) are needed. For example, Baxter et al. added sputum Aspergillus PCR and galactomannan to standard serologic assays (J Allergy Clin Immunol 2013, 132, 560-6.e10). Gernez et al. showed diagnostic accuracy of basophil activation test (Eur Resp J 2016, 47, 177).
There is continued need for randomized placebo-controlled trials testing the efficacy of antifungals and Th2-directed biologics in people with ABPA CF.
Relevant manuscripts published after the original guidelines are listed below. These manuscripts have not been reviewed or endorsed by the guidelines committee.
Caroline Okorie, M.D., MPH and Richard B. Moss, M.D. (Stanford University)
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