A successful uncomplicated CF pregnancy while remaining on Ivacaftor

Kaminski, R.M.; Nazareth, D

doi:10.1016/j.jcf.2015.11.013

Cited by 34 publications

(31 citation statements)

References 1 publication

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“…Ivacaftor is classified as a category B pregnancy risk by the FDA, a classification that is given when there are no data to support its potential harm during pregnancy, nor human studies that evaluate the safety profile during pregnancy (14). A single case of a complication-free pregnancy and normal full term infant in a CF woman taking ivacaftor has been reported (15); however a corresponding editorial highlighted the pressing need for more data to support the safety or risk to infants born to women taking CFTR modulators (6). Phase 2 data of CFTR modulators show that sweat chloride, a measure of CFTR function, rapidly returns to baseline after cessation of CFTR modulators (21, 22), therefore discontinuation of a modulator throughout pregnancy may compromise maternal health.…”

Section: Discussionmentioning

confidence: 99%

“…Currently, the information included in the package inserts for the approved modulators suggests that physicians counsel women with CF on modulator therapy to use an alternative to hormonal contraception if they wish to avoid pregnancy (13, 14); however there are no data evaluating contraceptive effectiveness during CFTR modulator use. Similarly, there are limited data on the effects of modulators on the fetus, although a recent case report suggests that the modulator, ivacaftor, did not adversely affect the pregnancy nor the newborn infant (15). It is unknown how many women with CF might choose to stay on modulators throughout their pregnancies.…”

Section: Introductionmentioning

confidence: 99%

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Pregnancy among cystic fibrosis women in the era of CFTR modulators

Heltshe

Godfrey

Josephy

et al. 2017

Journal of Cystic Fibrosis

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BACKGROUND Little is known about how new therapies that partially correct the basic cystic fibrosis (CF) defect (ivacaftor and lumacaftor) might alter hormonal contraceptive effectiveness, impact pregnancy outcomes, or affect pregnancy timing. Examination of pregnancy rates among CF women during periods of CFTR modulator therapy initiation will provide foundation for further research in this area. METHODS The Cystic Fibrosis Foundation Patient Registry was used to examine pregnancy rates and outcomes by genotype class before, during, and after the introduction of CFTR modulator therapies between 2005 and 2014. RESULTS Among women with CF, ages 15–44 years, there was a slight downward trend in annual pregnancy rates from 2005 to 2014 (2% reduction per year, p=0.041) Among women with G551D, pregnancy rates during phase 3 ivacaftor trial years was 14.4/1000 women/year compared to 34.0/1000 prior to the trial period (relative risk [RR]=0.65; 95% CI=0.43–0.96; p=0.011) and 38.4/1000 after drug approval in June 2012 (RR=1.52 post approval compared to trial period; 95% CI=1.26, 1.83; p<0.001). Pregnancy outcomes did not significantly change between 2005 and 2014 for any genotype class. CONCLUSION Evidence of significantly increased numbers of pregnancies among women taking approved CFTR modulators is important because of the unknown risk to pregnancy and fetal outcomes. Increases may be temporary following pregnancy prevention during controlled clinical trials, or from altered perceptions about maternal survival with new approved treatments. As more women with CF become eligible to receive modulators, the CF community must study their effect on contraceptive efficacy and safety during pregnancy. With increased health and survival due to modulation, family planning topics will become more common in CF.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Pregnancy among cystic fibrosis women in the era of CFTR modulators

Heltshe

Godfrey

Josephy

et al. 2017

Journal of Cystic Fibrosis

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“…With the recent introduction of ivacaftor and lumacaftor as CFTR modulators, there have been a number of reports of these agents being used during pregnancy and during breastfeeding [56,58,130,131]. To date, there have been no reports of abnormalities associated with these agents, but the relative benefits of remaining on CFTR modulator therapy before, during and after pregnancy would need to be balanced against any theoretical risks.…”

Section: Cftr Modulatorsmentioning

confidence: 99%

ERS/TSANZ Task Force Statement on the management of reproduction and pregnancy in women with airways diseases

et al. 2019

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This European Respiratory Society/Thoracic Society of Australia and New Zealand statement outlines a review of the literature and expert opinion concerning the management of reproduction and pregnancy in women with airways diseases: asthma, cystic fibrosis (CF) and non-CF bronchiectasis. Many women with these diseases are now living into reproductive age, with some developing moderate-to-severe impairment of lung function in early adulthood. The statement covers aspects of fertility, management during pregnancy, effects of drugs, issues during delivery and the post-partum period, and patients’ views about family planning, pregnancy and parenthood. The statement summarises current knowledge and proposes topics for future research, but does not make specific clinical recommendations.

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“…Rather, these decisions reflect the potential for harm to a fetus or newborn for a therapy for which overall benefit has yet to be demonstrated. Postmarketing case reports in the peerreviewed literature (which typically do not result in label amendments) can provide important additional information for safety assessment associated with pregnancy and/ or breastfeeding (28,29). Importantly, not all safety-based exclusions are merely pragmatic.…”

Section: Reasons For Inclusion Versus Exclusion Of Subpopulations In mentioning

confidence: 99%

Potential for Therapeutic Benefit among Cystic Fibrosis Populations Excluded from Clinical Trials or Labeling of Marketed Therapies

2016

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Remarkable advances in the management of individuals born with cystic fibrosis (CF) would not have been realized without empiric trial and error by CF clinicians with treatments developed and available for other purposes. As the testing and registration of CF-specific treatments have increased, so too have associated health care costs, particularly those of chronic medications. The transition of CF from a lethal pediatric disease to a life-shortening one with an adult majority, concurrent with sharp increases in chronic medication costs, has placed many CF treatments under increased scrutiny by third-party payers, particularly when prescribed to individuals from CF subpopulations that may not have been included in registration trials. Despite overall health improvements in the CF cohort and the increasing availability of CF-specific therapies, many physicians remain tasked with managing the health of patients from subpopulations that may be too young, too sick, or too complicated to have been included in clinical trials. An understanding of why particular CF subpopulations may have been excluded from registration trials, as well as consideration of a treatment's described mechanism of action, can support assessment for the potential for benefit (and risk) in these populations and help physicians advocate for patient access to treatments.Keywords: cystic fibrosis; excluded subjects; off-label; risk and benefit

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A successful uncomplicated CF pregnancy while remaining on Ivacaftor

Cited by 34 publications

References 1 publication

Pregnancy among cystic fibrosis women in the era of CFTR modulators

Pregnancy among cystic fibrosis women in the era of CFTR modulators

ERS/TSANZ Task Force Statement on the management of reproduction and pregnancy in women with airways diseases

Potential for Therapeutic Benefit among Cystic Fibrosis Populations Excluded from Clinical Trials or Labeling of Marketed Therapies

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