Study Describes Outcomes in Pregnant Women with Sickle Cell Disease

A new retrospective study has reported on maternal and perinatal outcomes in pregnant women with sickle cell disease (SCD).

The study also evaluated the impact of red blood cell (RBC) transfusions, describing outcomes related to maternal and fetal health.

“A better quality of care for individuals with SCD over the last two decades has improved the survival and quality of life and thus, there are increasing numbers of women reaching the reproductive age,” the study investigators acknowledged. As such, it becomes vital to continue to assess pregnancy-related outcomes.

A team from Santa Casa de Sao Paulo Medical School, led by Viviane Teixeira de Sousa, examined 55 medical records among 35 women with SCD. All patients were followed at the hospital’s outpatient unit for anemia and an SCD reference center between January 1, 2010-December 31, 2019.

The Study

De Sousa and team included those who had ≥1 pregnancy and SCD as diagnosed by hemoglobin electrophoresis and/or high-performance liquid chromatography.

“The on-demand RBC transfusion, defined as the transfusion instituted to treat complications, was indicated for pregnant patients if the hemoglobin (Hb) level fell below 8.0 g/dL or upon a 2-g/dL decrease in Hb in steady-state anemia (due to infection, increased hemolysis, renal disease, or multiorgan system failure) and in case of obstetric or acute SCD-related complications, such as stroke, acute chest syndrome, or acute splenic sequestration), regardless of the gestational age,” they wrote.

Patients with an established chronic transfusion protocol maintained their therapy, while those with a history of late miscarriage and/or neonatal death, intrauterine growth retardation, or twin pregnancy were initiated on chronic transfusion from the moment of pregnancy diagnosis or the first trimester of gestation.

Chronic transfusion protocol included simple or exchange transfusion every 3-4 weeks until delivery, with the target Hb level being between 9.0-10.0 g/dL in patients with HbSS and HbS levels below 50.0%.

The Findings

The investigators reported that mothers' mean age at delivery was 26.7 years, while the mean gestational age was 36.6 years.

Out of 29 newborns, 19 were full-term and 10 were pre-term. Furthermore, 24 and 5 caesarean and natural births, respectively, were observed. There were no maternal deaths.

The investigators also noted that pre-eclampsia and gestational diabetes were the main maternal obstetric complications. Vaso-occlusive crisis, urinary tract infection, and acute chest syndrome were the most commonly observed non-obstetric complications.

There was a total of 26 fetal deaths—24 of which were intrauterine fetal (14 early abortions, 10 late abortions, and 2 still birthirths).

In terms of RBC transfusions, 40 of the 55 pregnancies received transfusion.

“Pregnant women who received 6 or more transfusions throughout pregnancy had a significantly lower number of abortions, i.e., no cases of early abortion and only 1 case of late abortion, versus 14 and 9 cases in pregnancies with 0–5 transfusions, respectively,” De Sousa and colleagues reported.

Conclusion

The team concluded that their findings only confirmed previous literature showing high rates of maternal and fetal complications—even though no maternal deaths were observed during the course of pregnancy.

They also highlighted the association between number of transfusions and the lower risk of intrauterine fetal death.

“This observation leads us once more to question the likely beneficial role of transfusions during pregnancy in patients with SCD observed in many other studies, a period prone to an increase in the percentage of maternal and fetal complications,” they wrote.

Nonetheless, the investigators indicated a need for prospective, multicenter, randomized trials that could evaluate the benefits and risks of prophylactic transfusions.

The team, “Maternal and perinatal outcomes in pregnant women with sickle cell disease: an update,” was published online in in Hematology, Transfusion, and Cell Therapy.