RECOMMENDATIONS

Document Overview

Design and created by Guideline Central in participation with the Cystic Fibrosis Foundation.

Chronic Medications for Maintenance of Lung Health

Cystic Fibrosis Foundation

Publication Date: Jan 3, 2013

Page Last Updated: May 5, 2026

Strength of Recommendations

Strength of Recommendation
Category/Grade	Definition
A	The committee strongly recommends that clinicians routinely provide this therapy. There is high certainty that the net benefit is substantial.
B	The committee recommends that clinicians routinely provide this therapy. There is high certainty that the net benefit is moderate, or there is moderate certainty that the net benefit is moderate to substantial.
C	The committee recommends that clinicians consider providing this therapy to selected patients depending on individual circumstances. However, for most individuals without signs or symptoms there is likely to be only a small benefit from this service.
D	The committee recommends against the therapy. There is moderate or high certainty that the service has no net benefit or that the harms outweigh the benefits. Clinicians should discourage the use of this service.
I	The committee concludes that the current evidence is insufficient to assess the balance of benefits and harms of the service. Evidence is lacking, of poor quality, or conflicting, and the balance of benefits and harms cannot be determined.

Document Overview

Document Title: Chronic Medications for Maintenance of Lung Health

Authoring Society: Cystic Fibrosis Foundation

Document Publication Date: Jan 3, 2013

Page Last Reviewed/Updated: May 5, 2026

Document Type: Guideline

Country of Publication: United States

Full Text Freely Available: Yes

Full Text Guideline: www.atsjournals.org/doi/full/10.1164/rccm.201207-1160OE

Document Scope, Criteria, and Use Cases

Scope: Management, Treatment

Diseases/Conditions (MeSH)

D003550 - Cystic Fibrosis

Keywords: CFTR, antibiotics, cystic fibrosis

Inclusion Criteria: Female, Male, Adolescent, Adult, Child, Infant, Older Adult

Health Care Settings: Ambulatory, Hospital, Outpatient

Intended Users: Nurse, Nurse Practitioner, Physician, Physician Assistant, Respiratory Therapist

Recommendation Development Processes & Methodology

Supplemental Methodology Resource: Data Supplement

Number of Source Documents: 90

Includes peer/external review process?: Yes

Includes public comment process?: No

Methodologist involvement?: Yes

Patient involvement?: No

Includes multi-disciplinary group?: Yes

Includes systematic review?: Yes

Grades quality of strength of evidence?: Yes

Grades quality of strength of recommendation?: Yes

Discloses conflicts of interest?: No

Includes benefits/harms analysis with recommendations?: No

Chronic Medications for Maintenance of Lung Health

RECOMMENDATIONS

UNCHANGED FROM 2007

Inhaled tobramycin—moderate to severe disease

Inhaled tobramycin—mild disease

Dornase alfa—moderate to severe disease

Dornase alfa—mild disease

Inhaled hypertonic saline

Azithromycin withP. aeruginosa

Oral antistaphylococcal antibiotics, prophylactic use

Inhaled corticosteroids

Oral corticosteroids

Other inhaled antibiotics

Oral antipseudomonal antibiotics

Leukotriene modifiers

Inhaled or oral N-acetylcysteine, or inhaled glutathione

Inhaled anticholinergics

NEW AND MODIFIED RECOMMENDATIONS (2012)

Ivacaftor

Inhaled aztreonam—moderate to severe disease^†

Inhaled aztreonam—mild disease^†

Chronic use of ibuprofen (age < 18 yr)

Chronic use of ibuprofen (age ≥18 yr)

Azithromycin withoutP. aeruginosa

Chronic inhaled β₂-adrenergic receptor agonists

Oral antistaphylococcal antibiotics, chronic use

Strength of Recommendations

Document Overview

Document Scope, Criteria, and Use Cases

Recommendation Development Processes & Methodology

Chronic Medications for Maintenance of Lung Health

RECOMMENDATIONS

UNCHANGED FROM 2007

Inhaled tobramycin—moderate to severe disease

Inhaled tobramycin—mild disease

Dornase alfa—moderate to severe disease

Dornase alfa—mild disease

Inhaled hypertonic saline

Azithromycin withP. aeruginosa

Oral antistaphylococcal antibiotics, prophylactic use

Inhaled corticosteroids

Oral corticosteroids

Other inhaled antibiotics

Oral antipseudomonal antibiotics

Leukotriene modifiers

Inhaled or oral N-acetylcysteine, or inhaled glutathione

Inhaled anticholinergics

NEW AND MODIFIED RECOMMENDATIONS (2012)

Ivacaftor

Inhaled aztreonam—moderate to severe disease†

Inhaled aztreonam—mild disease†

Chronic use of ibuprofen (age < 18 yr)

Chronic use of ibuprofen (age ≥18 yr)

Azithromycin withoutP. aeruginosa

Chronic inhaled β2-adrenergic receptor agonists

Oral antistaphylococcal antibiotics, chronic use

Strength of Recommendations

Document Overview

Document Scope, Criteria, and Use Cases

Recommendation Development Processes & Methodology

Inhaled aztreonam—moderate to severe disease^†

Inhaled aztreonam—mild disease^†

Chronic inhaled β₂-adrenergic receptor agonists