Management of sickle cell disease during pregnancy: experience in a third-level hospital and future recommendations

To describe the outcomes of sickle-cell disease in pregnancy according to the different treatments adopted before and during pregnancy and to propose a systematic approach to treat sickle-cell disease (SCD) during pregnancy.

A retrospective descriptive study compared pregnancy outcomes among women with SCD who stopped hydroxyurea (HU) once pregnant (Group 1), were never treated before and during pregnancy (Group 2) or were treated by HU before conception who received prophylactic transfusion during pregnancy (Group 3). For each group we recorded the population's characteristics and the transfusion-related, obstetrical, perinatal and SCD complications.

We found 11 patients for group 1 (9/11 with at least 3 painful crises during the 12 months before conception), 4 for group 2 (3/4 with no sickle-cell complications during the year before pregnancy) and 2 for group 3 (one with previous multiorgan failure (MOF), one with previous stroke). No transfusion-related complication occurred. Group 1 and 2 developed SCD complications and a high number of acute transfusions and hospital admissions. Group 3 showed none of these complications, but one patient developed preeclampsia and preterm birth. Several obstetrical and perinatal complications occurred in group 1.

Not treating sickle-cell during pregnancy increases maternal and perinatal morbidity, even in mildly affected women. All sickle-cell pregnancies should be treated, according to the treatment adopted before but also to patient's SCD-history. We propose chronic transfusion to women with previous stroke or MOF or already under transfusion program, and HU for severely and mildly affected patients, respectively from the second and third trimesters. Additional prospective studies are needed to validate the results of the proposed protocol.