Click
here to close Hello! We notice that
you are using Internet Explorer, which is not supported by Echinobase
and may cause the site to display incorrectly. We suggest using a
current version of Chrome,
FireFox,
or Safari.
J Cyst Fibros
2017 Nov 01;166:687-694. doi: 10.1016/j.jcf.2017.01.008.
Show Gene links
Show Anatomy links
Pregnancy among cystic fibrosis women in the era of CFTR modulators.
Heltshe SL
,
Godfrey EM
,
Josephy T
,
Aitken ML
,
Taylor-Cousar JL
.
Abstract
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 15-44years, 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-years 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.
Accurso,
Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation.
2010, Pubmed
Accurso,
Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation.
2010,
Pubmed
Ahluwalia,
Cystic fibrosis and pregnancy in the modern era: a case control study.
2014,
Pubmed
Boyle,
A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: a phase 2 randomised controlled trial.
2014,
Pubmed
Centers for Disease Control and Prevention (CDC),
U S. Medical Eligibility Criteria for Contraceptive Use, 2010.
2010,
Pubmed
Gilljam,
Pregnancy in cystic fibrosis. Fetal and maternal outcome.
2000,
Pubmed
Goss,
The effect of pregnancy on survival in women with cystic fibrosis.
2003,
Pubmed
Goss,
CFTR modulators and pregnancy: Our work has only just begun.
2016,
Pubmed
Herzog,
Contraceptive practices of women with epilepsy: Findings of the epilepsy birth control registry.
2016,
Pubmed
Jones,
Potential impact on fertility of new systemic therapies for cystic fibrosis.
2015,
Pubmed
Kaminski,
A successful uncomplicated CF pregnancy while remaining on Ivacaftor.
2016,
Pubmed
Knapp,
The Cystic Fibrosis Foundation Patient Registry. Design and Methods of a National Observational Disease Registry.
2016,
Pubmed
Plant,
Contraceptive practices in women with cystic fibrosis.
2008,
Pubmed
Quanjer,
Multi-ethnic reference values for spirometry for the 3-95-yr age range: the global lung function 2012 equations.
2012,
Pubmed
Ramsey,
A CFTR potentiator in patients with cystic fibrosis and the G551D mutation.
2011,
Pubmed
Roe,
Contraception in women with cystic fibrosis: a systematic review of the literature.
2016,
Pubmed
Rowe,
Cystic fibrosis.
2005,
Pubmed
Sawicki,
Rate of Uptake of Ivacaftor Use after U.S. Food and Drug Administration Approval among Patients Enrolled in the U.S. Cystic Fibrosis Foundation Patient Registry.
2015,
Pubmed
Simcox,
Decision making about reproduction and pregnancy by women with cystic fibrosis.
2009,
Pubmed
Thorpe-Beeston,
Contraception and pregnancy in cystic fibrosis.
2009,
Pubmed
Tonelli,
Pregnancy in cystic fibrosis.
2007,
Pubmed
Ventura,
Estimated pregnancy rates and rates of pregnancy outcomes for the United States, 1990-2008.
2012,
Pubmed
Wainwright,
Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR.
2015,
Pubmed
Zielenski,
Cystic fibrosis: genotypic and phenotypic variations.
1995,
Pubmed
