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.
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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.
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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.
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The Cystic Fibrosis Foundation provides standard care guidelines based on the latest research, medical evidence, and consultation with experts on best practices.
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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.
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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.
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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)
The guidelines were published in October 2003, they were reviewed in April 2019 and it was determined that no update is needed at this time.
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