TABLE 1

Maintenance CF therapies used during pregnancy and lactation

MedicationRoute of administrationObservations/considerationsUse in pregnancyUse in lactation
Mucolytics
 Dornase alfaInhaledLittle to no systemic absorptionYesYes
 Hypertonic salineInhaledLittle to no systemic absorptionYesYes
Inhaled antibiotics
 TobramycinInhaledLittle to no systemic absorptionYesYes
 AztreonamInhaledLittle to no systemic absorptionYesYes
 LevofloxacinInhaledLittle to no systemic absorptionYesYes
 ColistimethateInhaledLittle to no systemic absorptionYesYes
Macrolide antibiotic
 AzithromycinOralData have shown no risk to low risk to the fetus and/or infantYesYes
Nutritional and digestive supplements
 Fat-soluble vitamins (A, D, E, K)OralDoses of >25 000 IU per day of vitamin A may be potentially teratogenicYesYes
 Pancreatic enzymesOralCompatible with pregnancy and lactationYesYes
CFTR modulators
 IvacaftorOralWell-tolerated in case seriesLikely safePossibly safe#
 Lumacaftor/ivacaftorOralWell-tolerated in case seriesLikely safePossibly safe#
 Tezacaftor/ivacaftorOralWell-tolerated in case seriesLikely safePossibly safe#
 Elexacaftor/tezacaftor/  ivacaftorOralWell-tolerated in case seriesLikely safePossibly 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.