Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2025
Pregnancies in women with cystic fibrosis in the era of CFTR modulators: Current challenges and future perspectives. Insights from the Italian cystic fibrosis registry.
СтатьяПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод пока не подготовлен
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Salvatore Marco, Campagna Giuseppe, Amato Annalisa, Pierannunzio Daniela, Ripani Pietro, Taccetti Giovanni, Fabrizzi Benedetta, Majo Fabio, Padoan Rita
Аннотация
BACKGROUND: The prognosis for people with Cystic Fibrosis (CF) has significantly improved due to the introduction of CF Transmembrane Regulator (CFTR) modulators. This study aims to analyze pregnancies and related outcomes in Italian women with CF while also identifying current and potential future challenges.
METHODS: This retrospective study utilized data from the Italian cystic fibrosis Registry (ICFR), focusing on pregnancies occurring between 2016 and 2023. Key clinical variables assessed included genotype, age at conception, body mass index, percent predicted forced respiratory volume (ppFEV₁), presence of CF-related diabetes (CFRD), and CFTR modulator therapy. The outcomes analyzed were pulmonary function and BMI as a surrogate of nutritional status before and after pregnancy, as well as preterm birth rate, birthweight, and mode of delivery.
RESULTS: Between 2016 and 2023, the ICFR reported 172 pregnancies, of which 148 (86.0 %) were carried to term. The median age at conception was 31.8 years (range: 17.0-43.9), median BMI 21.6 kg/m² (range: 16.5-38.9), and median ppFEV₁ 79.7 (range: 29.0-120.4). CFRD was present in 32 women (21.6 %), and 55 (37.2 %) were receiving CFTR modulator therapy. Better clinical status at the beginning of pregnancy was observed in 2016-23 period, with a higher mean ppFEV1, in the mean age at conception and in the mean BMI value. In the present study, only a limited number of women were in severe clinical condition (<5 % had a severe ppFEV1 value or a BMI showing a malnutrition status). The overall preterm birth (<37 weeks gestation) was 24.1 % (34 cases), higher in women with CFRD (40.6 %) and lowest in those receiving CFTRm (22.7 %). Median birthweight was 2,990 g (range: 800-4,155 g). The overall cesarean section rate was 54.9 %, peaking at 81.2 % in women with CFRD.
CONCLUSION: Understanding national pregnancy trends and outcomes is essential for informing effective public health strategies and improving maternal-infant wellbeing. An increasing number of women with CF are achieving successful pregnancies and delivering healthy babies. It is essential to provide women treated with CFTR modulators with up-to-date information regarding the safety of these therapies during pregnancy and breastfeeding.
METHODS: This retrospective study utilized data from the Italian cystic fibrosis Registry (ICFR), focusing on pregnancies occurring between 2016 and 2023. Key clinical variables assessed included genotype, age at conception, body mass index, percent predicted forced respiratory volume (ppFEV₁), presence of CF-related diabetes (CFRD), and CFTR modulator therapy. The outcomes analyzed were pulmonary function and BMI as a surrogate of nutritional status before and after pregnancy, as well as preterm birth rate, birthweight, and mode of delivery.
RESULTS: Between 2016 and 2023, the ICFR reported 172 pregnancies, of which 148 (86.0 %) were carried to term. The median age at conception was 31.8 years (range: 17.0-43.9), median BMI 21.6 kg/m² (range: 16.5-38.9), and median ppFEV₁ 79.7 (range: 29.0-120.4). CFRD was present in 32 women (21.6 %), and 55 (37.2 %) were receiving CFTR modulator therapy. Better clinical status at the beginning of pregnancy was observed in 2016-23 period, with a higher mean ppFEV1, in the mean age at conception and in the mean BMI value. In the present study, only a limited number of women were in severe clinical condition (<5 % had a severe ppFEV1 value or a BMI showing a malnutrition status). The overall preterm birth (<37 weeks gestation) was 24.1 % (34 cases), higher in women with CFRD (40.6 %) and lowest in those receiving CFTRm (22.7 %). Median birthweight was 2,990 g (range: 800-4,155 g). The overall cesarean section rate was 54.9 %, peaking at 81.2 % in women with CFRD.
CONCLUSION: Understanding national pregnancy trends and outcomes is essential for informing effective public health strategies and improving maternal-infant wellbeing. An increasing number of women with CF are achieving successful pregnancies and delivering healthy babies. It is essential to provide women treated with CFTR modulators with up-to-date information regarding the safety of these therapies during pregnancy and breastfeeding.