SICKLE-CELL DISEASE AND PREGNANCY

Sickle cell disease refers to a group of genetic disorders characteris ed by the predominance of hemoglobin S. This includes sickle cell anem ia (SS) sickle hemoglobin C disease (SC), sickle beta thalassemia plus (S beta(+)Thal), sickle beta thalassemia zero (beta(0)Thal), sickle w ith alpha thalassemia (SS alpha Thal) and rare combinations of sickle hemoglobin with Hb D, Hb O, etc. While pregnancy does carry risk for t he woman with sickle cell disease (SCD) and for the fetus, pregnancy c an be well tolerated by the major genotypes. Infants born from these p regnancies may tend to be small for gestational age and undergo premat ure delivery. While complications for both sickle-related events and f or pregnancy are seen, data to date state that women are able to compl ete their pregnancies successfully. Counselling, regular prenatal visi ts and aggressive treatment of acute events are always indicated. Ther e is no proof that prophylactic transfusion alters the outcome of preg nancy. 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 surgic al intervention. Thus, blood transfusion will continue to have a role in management of obstetrical and medical. indications accompanied by m eticulous prenatal care and early detection of complications. In addit ion, newborn screening should be recommended for the early detection o f infants with disease.