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Management of pregnancy in cystic fibrosis

Kristina Montemayor, Elizabeth Tullis, Raksha Jain, Jennifer L. Taylor-Cousar
Breathe 2022 18: 220005; DOI: 10.1183/20734735.0005-2022
Kristina Montemayor
1Dept of Medicine, Division of Pulmonary and Critical Care Medicine, Johns Hopkins University, Baltimore, MD, USA
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Elizabeth Tullis
2Dept of Medicine, Division of Respirology, St. Michael's Hospital Unity Health, Toronto, ON, Canada
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Raksha Jain
3Dept of Internal Medicine, Division of Pulmonary and Critical Care, UT Southwestern, Dallas, TX, USA
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Jennifer L. Taylor-Cousar
4Dept of Medicine and Pediatrics, Division of Pulmonary Sciences and Critical Care Medicine and Pediatric Pulmonology, National Jewish Health, Denver, CO, USA
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  • ORCID record for Jennifer L. Taylor-Cousar
  • For correspondence: TaylorCousarJ@NJHealth.org
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    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

  • Figures
  • 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.

    • TABLE 2

      Common antibiotics used for treatment of a pulmonary exacerbation in people with CF

      Medication/classRoute of administrationTarget pathogenObservations/considerationsUse in pregnancyUse in lactation
      Tobramycin
      Aztreonam
      Levofloxacin
      Colistimethate
      InhaledPseudomonas aeruginosaLittle to no systemic absorption with inhaled routeYesYes
      FluoroquinolonesOral or i.v.Pseudomonas aeruginosaLow risk of fetal cartilage damage and generally avoided during pregnancyAvoidYes
      Aminoglycosidesi.v.Pseudomonas aeruginosaGenerally avoided and used if severe infection or critically ill given eight cranial nerve toxicity with some aminoglycosidesAvoidYes
      Piperacillin/tazobactami.v.Pseudomonas aeruginosaConsidered low risk in pregnancy and lactationYesYes
      Cephalosporinsi.v.Pseudomonas aeruginosa/MSSAConsidered low risk in pregnancy and lactationYesYes
      Carbapenemsi.v.Pseudomonas aeruginosa/MSSACF women have been shown to be more likely to develop severe pre-eclampsia, and carbapenems lower the seizure thresholdYesYes
      PenicillinsOral or i.v.MSSAConsidered low risk in pregnancy and lactationYesYes
      Trimethoprim-sulfamethoxazoleOral or i.v.MRSATrimethoprim may impair folic acid metabolism;
      sulfamethoxazole has been associated with fetal haemolytic anaemia and neonatal hyperbilirubinaemia
      AvoidYes
      Vancomycini.v.MRSALimited human data during first trimester but no evidence of teratogenesisYesYes
      LinezolidOral or i.v.MRSAInfants exposed during lactation should be monitored for diarrhoea or vomitingYesYes
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    Vol 18 Issue 2 Table of Contents
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    Management of pregnancy in cystic fibrosis
    Kristina Montemayor, Elizabeth Tullis, Raksha Jain, Jennifer L. Taylor-Cousar
    Breathe Jun 2022, 18 (2) 220005; DOI: 10.1183/20734735.0005-2022

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    Management of pregnancy in cystic fibrosis
    Kristina Montemayor, Elizabeth Tullis, Raksha Jain, Jennifer L. Taylor-Cousar
    Breathe Jun 2022, 18 (2) 220005; DOI: 10.1183/20734735.0005-2022
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    • Article
      • Abstract
      • Abstract
      • Introduction
      • Fertility in women with CF
      • Preconception considerations
      • Medication management during pregnancy
      • Pulmonary exacerbation treatment
      • Lactation
      • Management of pregnancy in individuals with CF following organ transplantation
      • Conclusion
      • Footnotes
      • References
    • Figures & Data
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    • PDF

    Subjects

    • CF and non-CF bronchiectasis
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    • Lessons from COVID-19 in the management of acute respiratory failure
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