Clinical management of sickle cell hemoglobinopathies during pregnancy

Abstract

Although the maternal and perinatal morbidity as well as mortality in pregnant patients with sickle cell disease is significantly higher than those parturients with hemoglobin A, marked amelioration has been obtained by comprehensive health care utilizing expert perinatal teams and by providing careful attention to preventive measures for infection and crisis. Through these consistent efforts, the life span for the mother/fetus/newborn has improved considerably over that observed in the past. Although there is no present cure for sickle cell disease and no effective and safe physiologic treatment in a preventive sense, our experience suggests that adherence to careful management principles, offered in this article, will increase the likelihood that significant vaso-occlusive crisis and infection can be reduced, thus resulting in a healthier pregnancy outcome. In addition, the future is bright for the development of pharmacologic agents to prevent sickle cell crisis and for advances in techniques designed to induce endogenous production of normal hemoglobin.