Abstract
BACKGROUND: Reproductive life planning is key, now that people with cystic fibrosis (pwCF) may live into their 60s. This study explores contraceptive use, pregnancy trends, and whether concomitant cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy reduces contraceptive effectiveness. METHODS: Females with CF aged 18-45 years from 10 U.S. CF centers completed a self-administered web-based questionnaire. Pregnancy rates were calculated by linear-mixed models with a logit link detected associations with contraception and modulator use. RESULTS: A total of 561 pwCF (median age of 29 years [IQR 24.9-35.8] years) completed the survey. Most participants (n = 499, 89%) used modulators, and almost all (n = 555, 99%) used contraception. Condoms (n = 448, 80%) and oral contraceptive pills (n = 363, 65%) were the most prevalent methods used. One-third (n = 189, 34%) reported ever being pregnant. Of those reporting pregnancies (n = 319), about half (n = 151, 48%) were unintended. Pregnancy was significantly associated with age (20-29 years or 30-39 years), partner cohabitation (aOR 21.5, 95% CI 5.1 to 91.1), and non-hormonal contraceptive use (aOR 5.1, 95% CI 1.1 to23.0). Among pwCF cohabitating with a partner, modulator use was positively associated with pregnancy (OR 1.8, 95% CI 1.3 to 2.6) (p = 0.0008). CONCLUSIONS: Despite almost universal contraceptive use, unintended pregnancy among pwCF is common. Likelihood of pregnancy is increased among CFTR modulator users who are partnered, although CFTR modulators themselves do not appear to decrease hormonal contraceptive effectiveness. Patient education about contraception is an increasingly critical aspect of CF care.