Maternal sickle cell disease and twin pregnancy: a case series and review of the literature

Abstract

Objectives: Maternal sickle cell disease (SCD) and multiple gestations are well known separately as causes of high-risk pregnancies, however, there is sparse information available on maternal and perinatal outcome when both conditions occur together. This case series describes the outcomes of women with maternal SCD and twin pregnancy in the largest single-center case series to date. Methods: Retrospective identification of all twin pregnancies in maternal SCD patients between 2006 and 2016 at Guy's and St. Thomas' Hospital, United Kingdom Results: Eight women were included: seven with HbSS and one with HbSC. Our cohort experienced common SCD-related and pregnancy-related complications such as painful vasoocclusive crises (VOC), acute chest syndrome (ACS), and pre-eclampsia and less common complications such as peri-partum cardiomyopathy and delayed hemolytic transfusion reaction. Only two out of eight women had relatively uncomplicated pregnancies. Seven out of eight women required transfusion antenatally and there was no maternal or perinatal mortality. A review of the available literature highlighted the lack of available information on this uncommon cohort. It was evident that outcomes have improved over the years, where historical studies demonstrate higher rates of maternal and perinatal mortality. Discussion: The antenatal and postnatal complications described in our study and literature review highlights the significant morbidity and mortality associated with these high-risk pregnancies. Conclusion: Our case series highlights the advantage of closer monitoring and comprehensive multidisciplinary care in delivering improved clinical outcomes.

Homozygous Deletion Alfa-Thalassemia and Hereditary Persistence of Fetal Hemoglobin, Two Genetic Factors Predictive the Reduction of Morbidity and Mortality During Pregnancy in Sickle Cell Patients . A Report From Democratic Republic of Congo

“…During pregnancy, several authors report a high frequency of complications such as acute thoracic syndrome and pre-eclampsia 23,24. Their frequencies (9 to 16%) vary from one series to another 20,23,24. In our series, the frequencies of these two complications are similar to those reported by other authors on the one hand and similar between the three genotypes on the other hand.…”

“…VOC episodes were the leading cause of morbidity during pregnancy in SCD, as also reported by other authors 23. These VOC increase with gestational age and are more frequent and severe in the perinatal period.…”

“…In our series, VOC seizures were more common in the HbSS group. During pregnancy, several authors report a high frequency of complications such as acute thoracic syndrome and pre-eclampsia 23,24. Their frequencies (9 to 16%) vary from one series to another 20,23,24.…”

Fetomaternal Outcomes among Patients with Sickle Cell Disease: A Retrospective Study

“…Several published reports suggest that twin pregnancy with SCD is associated with poor outcomes. 14 Out of 32 patients, only 1 patient was booked with the hospital and received proper antenatal care, including thromboprophylaxis and blood transfusions, and was timely managed when she presented with acute chest syndrome and painful crisis, also investigated for any existing renal dysfunction and PH. The remaining 31 patients were unbooked and admitted for labor pains or associated complications.…”

Sickle Cell Anemia in Pregnancy

“…In the study, all the women were required to be admitted to a level II or higher care ward in the postnatal period. 42 Since severe complications of SCD and acute crisis can develop in puerperium, the same level of care and vigilance should be sustained as has been termed for antenatal care. Progestogencontaining contraceptives such as the progesterone-only pill (Cerazette®), injectable contraceptives (Depo-Provera®), and the levonorgestrel intrauterine system (Mirena®) are safe and effective in SCD.…”