Sickle cell disease and pregnancy

Sickle cell disease refers to a group of genetic disorders characterised by the predominance of hemoglobin S. This includes sickle cell anemia (SS) sickle hemoglobin C disease (SC), sickle beta thalassemia plus (S beta + Thal), sickle beta thalassemia zero (beta zero Thal), sickle with alpha thalassemia (SS alpha Thal) and rare combinations of sickle hemoglobin with Hb D, Hb O, etc. While pregnancy does carry risk for the woman with sickle cell disease (SCD) and for the fetus, pregnancy can be well tolerated by the major genotypes. Infants born from these pregnancies may tend to be small for gestational age and undergo premature delivery. While complications for both sickle-related events and for pregnancy are seen, data to date state that women are able to complete their pregnancies successfully. Counselling, regular prenatal visits and aggressive treatment of acute events are always indicated. There is no proof that prophylactic transfusion alters the outcome of pregnancy. Transfusion therapy should be reserved for those patients with previous perinatal mortality, pre-eclampsia, acute chest syndrome, new onset neurological event, severe anemia and in preparation for surgical intervention. Thus, blood transfusion will continue to have a role in management of obstetrical and medical indications accompanied by meticulous prenatal care and early detection of complications. In addition, newborn screening should be recommended for the early detection of infants with disease.