Management of pregnancy in sickle cell syndromes

We have seen a marked decrease in maternal and perinatal morbidity and mortality among pregnant patients with sickle cell disease. This has been the result of coordinated efforts with the obstetric and hematologic teams. Patients are counseled prior to pregnancy regarding the risks and are given the opportunity to modify their life style to prepare for the additional metabolic burden of gestation. Once pregnant, they are instructed in the techniques to recognize and avoid complications. They are observed frequently for the appearance of pain crisis and other medical and obstetric complications. If complications are identified, they should be treated aggressively. Transfusion therapy is important in the management of patients; however, prophylactic transfusion does not change outcome. Although significant laboratory techniques aid in fetal and maternal supervision, the universal fundamentals of good clinical perinatal care provided through the combined efforts of the obstetrician and hematologist contribute to the framework for the modern management and successful outcome of patients with sickle cell disease during pregnancy.