Last updated March 15, 2022

Chronic Medications for Maintenance of Lung Health

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

Publication Month/Year

January 3, 2013

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