Abstract
Hemoglobinopathies, including sickle cell disease and thalassemia, present unique health care challenges during pregnancy. Women with sickle cell disease generally tolerate pregnancy well, with low maternal and perinatal mortality, when provided access to coordinated high-risk obstetrical and hematological care. Pregnancy may be complicated by pre-term labor, intrauterine growth retardation, small-for-gestational-age births and increased sickle cell pain crises and complications. Transfusion support does not improve maternal or fetal complications, even if used to correct severe anemia, but does lessen the incidence of sickle cell events, and should be reserved for women with severe anemia (hemoglobin < 6.0 gm/dl), frequent severe pain crisis or other sickle cell complications. Women with sickle cell trait have an increased incidence of bacturia during pregnancy, but do not experience manifestations of sickle cell disease. Other hemobinopathies, including hemoglobin EE and hemoglobin CC disease, do not significantly affect pregnancy. Few pregnancies have been reported in women with Cooleys anemia (β°thalassemia) due to infertility; these women and some with hemoglobin H disease (severe α-thalassemia) require transfusion support during pregnancy. The presence of high-affinity hemoglobins has not been associated with adverse pregnancy outcomes. Recognition and accurate diagnosis of a maternal hemoglobinopathy is imperative for appropriate management and genetic counseling.
Keywords: Pregnancy, thalassemia, sickle cell disease, high-affinity hemoglobin, hemoglobin E, hemoglobin C
36