Perinatal outcomes in women with sickle cell disease: a matched cohort study from London, UK

Abstract

There are limited data on contemporary outcomes for women with sickle cell disease (SCD) in pregnancy. We conducted a single-site matched cohort study, comparing 131 pregnancies to women with SCD between 2007 and 2017 to a comparison group of 1310 pregnancies unaffected by SCD. Restricting our analysis to singleton pregnancies that reached 24 weeks of gestation, we used conditional Poisson regression to estimate adjusted risk ratios (aRRs) for perinatal outcomes. Infants born to mothers with SCD were more likely to be small for gestational age [aRR 1Á69, 95% confidence interval (CI) 1Á13-2Á48], preterm (aRR 2Á62, 95% CI 1Á82-3Á78) and require Neonatal Unit (NNU) admission (aRR 3Á59, 95% CI 2Á18-5Á90). Pregnant women with SCD were at higher risk of pre-eclampsia/ eclampsia (aRR 3Á53, 95% CI 2Á00-6Á24), more likely to receive induction of labour (aRR 2Á50, 95% CI 1Á82-1Á76) and caesarean birth (aRR 1Á44, 95% CI 1Á18-1Á76). In analysis stratified by genotype, the risk of adverse outcomes was highest in haemoglobin SS (HbSS) pregnancies (n = 80). There was no strong evidence that haemoglobin SC (HbSC) pregnancies (n = 46) were at higher risk of preterm birth, caesarean delivery, or NNU admission. Pre-eclampsia/eclampsia was more frequently observed in HbSC pregnancies. Despite improvements in the care of pregnant women with SCD, the increased risk of adverse perinatal outcomes remains.

Pregnancy outcomes in women with sickle cell disease in California

“…Multiple studies show increased maternal morbidity in SCD, 11,12,62 yet a few have investigated the timing of these peripartum complications to aid with appropriate monitoring or interventions. 44 We found that in Black women with SCD, sepsis risk steadily increased throughout pregnancy into the postpartum period.…”

Complicações materno-fetais em gestantes portadoras de doença falciforme

“…(Cardoso et al 2014) Resultados como esse, de piores desfechos no genótipo considerado mais grave (HbSS), foram encontrados em diversos materiais. (Oakley, et al 2022;Silva Pinto, et al 2014;Costa, et al 2015) Além disso, na pesquisa de Cardoso et al (2014), a síndrome torácica aguda foi mais prevalente no grupo de genótipo menos grave (HbSC) quando comparado ao HbSS, sendo considerada a principal causa de morte materna no estudo.…”

“…)Proske et al (2021) avaliaram 53 gestações com diferentes genótipos de DF. Nas pacientes com genótipos HbS/C, as complicações incluíram: crises vaso-oclusivas/crises de dor aguda (43% / 14%) demanda de transfusão (14%) e ITUs (14%).Nos outros tipos de genótipos avaliados (HbSS, HbS/β-talassemia e doença HbS/O-Arab) as gestações foram complicadas por Em outro estudo,Oakley et al (2022) avaliaram 131 gestações de mulheres com DF (diferentes genótipos) e 1.310 gestações não afetadas por DF. Na pesquisa verificaram-se que bebês nascidos de mães com DF eram mais propensos a serem PIG (RR 1,69), prematuros (RR 2,62) e requerem admissão na Unidade Neonatal (RR 3,59).…”

Infertility and treatment‐seeking practices among females and males with sickle cell disease in the Sickle Cell Disease Implementation Consortium registry

“…Of note, there is some evidence of the protective nature of transfusions on sperm in the setting of pre‐pubertal hydroxyurea use 7,40 . The meaning of our findings relative to hydroxyurea use is unclear given the variable used to measure hydroxyurea use was self‐reported use “ever during their lifetime.” Some studies have associated the SCD genotype, specifically HbSS, with adverse birth outcomes 26,41 ; however, to our knowledge, there are no other studies that have examined genotype differences in those with infertility. Our data did not uncover an association between infertility and genotype; however, further verification is needed.…”