Maintenance CF therapies used during pregnancy and lactation
| Medication | Route of administration | Observations/considerations | Use in pregnancy | Use in lactation |
| Mucolytics | ||||
| Dornase alfa | Inhaled | Little to no systemic absorption | Yes | Yes |
| Hypertonic saline | Inhaled | Little to no systemic absorption | Yes | Yes |
| Inhaled antibiotics | ||||
| Tobramycin | Inhaled | Little to no systemic absorption | Yes | Yes |
| Aztreonam | Inhaled | Little to no systemic absorption | Yes | Yes |
| Levofloxacin | Inhaled | Little to no systemic absorption | Yes | Yes |
| Colistimethate | Inhaled | Little to no systemic absorption | Yes | Yes |
| Macrolide antibiotic | ||||
| Azithromycin | Oral | Data have shown no risk to low risk to the fetus and/or infant | Yes | Yes |
| Nutritional and digestive supplements | ||||
| Fat-soluble vitamins (A, D, E, K) | Oral | Doses of >25 000 IU per day of vitamin A may be potentially teratogenic | Yes | Yes |
| Pancreatic enzymes | Oral | Compatible with pregnancy and lactation | Yes | Yes |
| CFTR modulators | ||||
| Ivacaftor | Oral | Well-tolerated in case series | Likely safe | Possibly safe# |
| Lumacaftor/ivacaftor | Oral | Well-tolerated in case series | Likely safe | Possibly safe# |
| Tezacaftor/ivacaftor | Oral | Well-tolerated in case series | Likely safe | Possibly safe# |
| Elexacaftor/tezacaftor/ ivacaftor | Oral | Well-tolerated in case series | Likely safe | Possibly safe# |
#: juvenile rats exposed to ivacaftor developed cataracts leading to a label recommendation for cataract evaluation in children exposed to ivacaftor or ivacaftor containing products; no formal evaluation of liver function testing has been performed in infants exposed to CFTR modulators in utero [35]. Reproduced and modified from [3] with permission.