Chronic Medications for Maintenance of Lung Health
Publication Date: January 3, 2013
Last Updated: March 14, 2022
RECOMMENDATIONS
UNCHANGED FROM 2007
Inhaled tobramycin—moderate to severe disease
For individuals with CF 6 years of age and older, with moderate to severe lung disease and Pseudomonas aeruginosa persistently present in cultures of the airways, the CF Foundation strongly recommends the chronic use of inhaled tobramycin to improve lung function and quality of life, and reduce exacerbations. (A)
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Inhaled tobramycin—mild disease
For individuals with CF 6 years of age and older, with mild lung disease and P. aeruginosa persistently present in cultures of the airways, the CF Foundation recommends the chronic use of inhaled tobramycin to reduce exacerbations. (B)
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Dornase alfa—moderate to severe disease
For individuals with CF 6 years of age and older, with moderate to severe lung disease, the CF Foundation strongly recommends the chronic use of dornase alfa to improve lung function, improve the quality of life, and reduce exacerbations. (A)
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Dornase alfa—mild disease
For individuals with CF 6 years of age and older, with asymptomatic or mild lung disease, the CF Foundation recommends the chronic use of dornase alfa to improve lung function and reduce exacerbations. (B)
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Inhaled hypertonic saline
For individuals with CF 6 years of age and older, the CF Foundation recommends the chronic use of inhaled hypertonic saline to improve lung function and quality of life and reduce exacerbations. (B)
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Azithromycin with P. aeruginosa
For individuals with CF 6 years of age and older, with P. aeruginosa persistently present in cultures of the airways, the CF Foundation recommends the chronic use of azithromycin to improve lung function and reduce exacerbations. (B)
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Oral antistaphylococcal antibiotics, prophylactic use
For individuals with CF, the CF Foundation recommends against the prophylactic use of oral antistaphylococcal antibiotics to improve lung function and quality of life or reduce exacerbations. (D)
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Inhaled corticosteroids
For individuals with CF 6 years of age and older, without asthma or allergic bronchopulmonary aspergillosis, the CF Foundation recommends against the routine use of inhaled corticosteroids to improve lung function or quality of life and reduce pulmonary exacerbations. (D)
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Oral corticosteroids
For individuals with CF 6 years of age and older, without asthma or allergic bronchopulmonary aspergillosis, the CF Foundation recommends against the chronic use of oral corticosteroids to improve lung function, quality of life or reduce exacerbations. (D)
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Other inhaled antibiotics
For individuals with CF 6 years of age and older, with P. aeruginosa persistently present in cultures of the airways, the CF Foundation concludes that the evidence is insufficient to recommend for or against the chronic use of other inhaled antibiotics (i.e., carbenicillin, ceftazidime, colistin, gentamicin) to improve lung function and quality of life or reduce exacerbations. (I)
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Oral antipseudomonal antibiotics
For individuals with CF 6 years of age and older, with P. aeruginosa persistently present in cultures of the airways, the CF Foundation concludes that the evidence is insufficient to recommend for or against the routine use of chronic oral antipseudomonal antibiotics to improve lung function and quality of life or reduce exacerbations. (I)
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Leukotriene modifiers
For individuals with CF 6 years of age and older, the CF Foundation concludes that the evidence is insufficient to recommend for or against the routine chronic use of leukotriene modifiers to improve lung function and quality of life or reduce exacerbations. (I)
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Inhaled or oral N-acetylcysteine, or inhaled glutathione
For individuals with CF 6 years of age and older, the CF Foundation concludes that the evidence is insufficient to recommend for or against the chronic use of inhaled or oral N-acetylcysteine or inhaled glutathione to improve lung function and quality of life or reduce exacerbations. (I)
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Inhaled anticholinergics
For individuals with CF 6 years of age and older, the CF Foundation concludes that the evidence is insufficient to recommend for or against the chronic use of inhaled anticholinergic bronchodilators to improve lung function and quality of life or reduce exacerbations (I)
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Severity of lung disease is defined by FEV1% predicted as follows: normal, >90% predicted; mildly impaired, 70–89% predicted; moderately impaired, 40–69% predicted; and severely impaired, <40% predicted.
NEW AND MODIFIED RECOMMENDATIONS (2012)
Ivacaftor
For individuals with CF 6 years of age and older, with at least one G551D CFTR mutation, the Pulmonary Clinical Practice Guidelines Committee strongly recommends the chronic use of ivacaftor to improve lung function and quality of life and reduce exacerbations. (A)
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Inhaled aztreonam—moderate to severe disease†
For individuals with CF 6 years of age and older, with moderate to severe lung disease and Pseudomonas aeruginosa persistently present in cultures of the airways, the CF Foundation strongly recommends the chronic use of inhaled aztreonam to improve lung function and quality of life. (A)
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Inhaled aztreonam—mild disease†
For individuals with CF 6 years of age and older, with mild lung disease and P. aeruginosa persistently present in cultures of the airways, the CF Foundation recommends the chronic use of inhaled aztreonam to improve lung function and quality of life. (B)
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Chronic use of ibuprofen (age < 18 yr)
For individuals with CF between 6 and 17 years of age, with an FEV1 ≥60% predicted, the CF Foundation recommends the chronic use of oral ibuprofen, at a peak plasma concentration of 50–100 μg/ml, to slow the loss of lung function. (B)
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Chronic use of ibuprofen (age ≥18 yr)
For individuals with CF 18 years of age and older, the CF Foundation concludes that the evidence is insufficient to recommend for or against the chronic use of oral ibuprofen to slow the loss of lung function or reduce exacerbations (I)
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Azithromycin without P. aeruginosa
For individuals with CF 6 years of age and older, without P. aeruginosa persistently present in cultures of the airways, the CF Foundation recommends the chronic use of azithromycin should be considered to reduce exacerbations. (C)
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Chronic inhaled β2-adrenergic receptor agonists
For individuals with CF, 6 years of age and older, the CF Foundation concludes that the evidence is insufficient to recommend for or against chronic use of inhaled β2-adrenergic receptor agonists to improve lung function and quality of life or reduce exacerbations. (I)
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Oral antistaphylococcal antibiotics, chronic use
For individuals with CF 6 years of age and older, with Staphylococcus aureus persistently present in cultures of the airways, the CF Foundation concludes that the evidence is insufficient to recommend for or against the chronic use of oral antistaphylococcal antibiotics to improve lung function and quality of life or reduce exacerbations. (I)
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*CF Foundation personnel did not participate in any activity related to ivacaftor.
†Severity of lung disease is defined by FEV1% predicted as follows: normal, >90% predicted; mildly impaired, 70–89% predicted; moderately impaired, 40–69% predicted; and severely impaired, <40% predicted.
Recommendation Grading
Overview
Title
Chronic Medications for Maintenance of Lung Health
Authoring Organization
Cystic Fibrosis Foundation
Publication Month/Year
January 3, 2013
Last Updated Month/Year
January 9, 2024
Document Type
Guideline
External Publication Status
Published
Country of Publication
US
Inclusion Criteria
Female, Male, Adolescent, Adult, Child, Infant, Older adult
Health Care Settings
Ambulatory, Hospital, Outpatient
Intended Users
Respiratory therapist, nurse, nurse practitioner, physician, physician assistant
Scope
Management, Treatment
Diseases/Conditions (MeSH)
D003550 - Cystic Fibrosis
Keywords
cystic fibrosis, antibiotics, CFTR