| Intervention | Indications/timing | Comments |
| CFTR modulator | - Most PwCF, depending on genotype and age.
| - Refer to UpToDate content and table on CFTR modulator therapy. The type of CFTR modulator and strength of recommendation depend on the genotype.
|
| Airway clearance therapies |
| Inhaled DNase | - DNase and/or hypertonic saline are recommended for:
- Age ≥12 years: For PwCF who are not taking HEMT and for those taking HEMT who have moderate to severe lung disease and continued sputum production.
- Age 6 to 11 years: All children.
- Age ≤5 years: All children with chronic respiratory symptoms or with more than rare pulmonary exacerbations.
- DNase is generally given once daily and hypertonic saline twice daily.
- Administer a short-acting bronchodilator before each treatment.
| - Expert opinion varies regarding thresholds for beginning or continuing these inhaled therapies. Suggestions from the UpToDate expert authors and editors differ from CFF guidelines[1-4] due to studies published subsequent to the most recent CFF guidelines[5-11].
- The regimen should be personalized, depending on the individual's preference and perceived treatment burden (cost, time).
|
| Inhaled hypertonic saline |
| Airway clearance physiotherapy (chest physiotherapy) | - All PwCF who produce sputum, twice daily.
| - No modality has been proven better than others.
|
| Prevention of infection |
| Vaccinations | For all PwCF: - Annual influenza vaccine.
- Pneumococcal vaccine, with PCV13 (or PCV15) and PPSV23, depending on age and prior immunization status[12].
- All other routine childhood vaccines.
- COVID-19 vaccines, following CDC guidelines.
| |
| Palivizumab | - We do not suggest prophylaxis with palivizumab for children with CF unless they have other indications.
| - Insufficient evidence for benefit[13-15].
|
| Infection control measures | - All PwCF in all health care settings.
| - Contact precautions, physical separation of PwCF, hand hygiene, and use of masks by PwCF[16].
|
| Bronchodilators |
| Inhaled beta-2 adrenergic receptor agonist | - All PwCF before each dose of inhaled antibiotics, DNase, hypertonic saline, or airway clearance therapy session.
- As a rescue medication for acute symptoms, in those with signs or symptoms of airway hyperresponsiveness.
| |
| Antiinflammatory therapy |
| Oral azithromycin | - All PwCF age ≥6 months, at time of acquisition of P. aeruginosa.
- Administer 3 times weekly.
- Do not give azithromycin to PwCF who are infected with nontuberculous mycobacteria.
| - Evidence is strongest for those ages ≥6 years who are infected with P. aeruginosa[2,16-19] but is also effective for younger children when begun at the time that they first become infected with this organism. Azithromycin is probably not effective for most of those who are not infected with P. aeruginosa.
- Benefits may be related to antiinflammatory and/or antibacterial properties.
|
| Ibuprofen (high-dose) | - Suggested for children 6 to 17 years with good lung function (FEV1 >60% predicted)[1,2].
- In clinical practice, this is not commonly used in the United States.
| - Not recommended for those with more severe lung function abnormalities or those who are older than 18 years of age. Requires pharmacokinetic studies to ensure proper dosing and monitoring for adverse effects (gastrointestinal bleeding, kidney function impairment).
|
| Inhaled glucocorticoids | - PwCF and definite signs and symptoms of asthma (including patients with asthmatic symptoms in the setting of ABPA)*.
| - Not recommended for those without these characteristics, due to little evidence for benefit and concerns about adverse effects on growth.
|
| Prevention of acute exacerbations |
| Inhaled tobramycin and/or aztreonam lysine | - All PwCF and persistent P. aeruginosa infection.
- Give twice daily for 28 days, alternating with 28 days off of treatment.
| - Refer to UpToDate content on antibiotic therapy for chronic pulmonary infection.
|
| Surveillance and early eradication of P. aeruginosa | - All PwCF without P. aeruginosa infection.
- Routine surveillance of sputum cultures or throat swabs, followed by eradication protocol if P. aeruginosa is detected.
| - Refer to UpToDate content on early eradication of P. aeruginosa.
|
| Neuraminidase inhibitor (eg, oseltamivir) | - Selected PwCF meeting criteria for postexposure prophylaxis or treatment.
| - Refer to UpToDate content on seasonal influenza in children.
|
| Treatment of acute exacerbations |
| Systemic antibiotics | - All PwCF and an acute exacerbation.
| - Selection of antibiotics is guided by cultures of respiratory secretions. Refer to UpToDate content on treatment of acute pulmonary exacerbations in CF.
|
| Intensify airway clearance therapies | - All PwCF and an acute exacerbation.
| |
| Systemic glucocorticoids | - Short course for PwCF and characteristics of an acute asthma episode. A prolonged course may be indicated for those with confirmed acute ABPA¶.
| - Although practice varies, UpToDate recommends against the use of systemic glucocorticoids, except as noted.
|
| Respiratory support | - As needed, guided by prognosis and individual preferences.
| - An individual's preferences should be established through discussions of advanced care directives, ideally occurring well before requiring the intervention.
|
September 2025
