Figures
- FIGURE 1
Number of pregnancies reported in the US Cystic Fibrosis Foundation (CFF) Patient Registry in women aged 14–45 years. Reproduced from [2] with permission. Source of data: cystic fibrosis patients under care at CFF-accredited care centres in the USA, who consented to have their data entered.
Tables
- TABLE 1
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.
- TABLE 2
Common antibiotics used for treatment of a pulmonary exacerbation in people with CF
Medication/class Route of administration Target pathogen Observations/considerations Use in pregnancy Use in lactation Tobramycin
Aztreonam
Levofloxacin
ColistimethateInhaled Pseudomonas aeruginosa Little to no systemic absorption with inhaled route Yes Yes Fluoroquinolones Oral or i.v. Pseudomonas aeruginosa Low risk of fetal cartilage damage and generally avoided during pregnancy Avoid Yes Aminoglycosides i.v. Pseudomonas aeruginosa Generally avoided and used if severe infection or critically ill given eight cranial nerve toxicity with some aminoglycosides Avoid Yes Piperacillin/tazobactam i.v. Pseudomonas aeruginosa Considered low risk in pregnancy and lactation Yes Yes Cephalosporins i.v. Pseudomonas aeruginosa/MSSA Considered low risk in pregnancy and lactation Yes Yes Carbapenems i.v. Pseudomonas aeruginosa/MSSA CF women have been shown to be more likely to develop severe pre-eclampsia, and carbapenems lower the seizure threshold Yes Yes Penicillins Oral or i.v. MSSA Considered low risk in pregnancy and lactation Yes Yes Trimethoprim-sulfamethoxazole Oral or i.v. MRSA Trimethoprim may impair folic acid metabolism;
sulfamethoxazole has been associated with fetal haemolytic anaemia and neonatal hyperbilirubinaemiaAvoid Yes Vancomycin i.v. MRSA Limited human data during first trimester but no evidence of teratogenesis Yes Yes Linezolid Oral or i.v. MRSA Infants exposed during lactation should be monitored for diarrhoea or vomiting Yes Yes
