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patient education

Sickle cell disease in pregnancy

key information

source: Women's Health and Education Center


Sickle cell disease refers to a group of autosomal recessive disorders involving abnormal hemoglobin (hemoglobin S). It is a devastating abnormality of red blood cells that results in circulatory impairment, tissue damage, infarctions, severe anemia, and life-threatening infections. Sickle cell disease affects between 70,000 to 100,000 Americans, mostly of African-descent, with a minority of Hispanic, southern European, Middle Eastern, and Asian Indian descent. Today, sickle cell disease is most often discovered during routine newborn screening.

Although sickle cell disease is associated with major morbidity, more than 90% of children with sickle cell disease in the United States survive into adulthood. Compared to the general population, however, their life-spans are 2 or 3 decades shorter and limited by both acute and chronic morbidity. Acute complications of sickle cell disease include ischemic, vaso-occlusive (pain) crises, acute chest syndrome (which most closely resembles pneumonia, but may also result from fat embolism from bone marrow, intrapulmonary aggregates of sickle cells, atelectasis, or pulmonary edema), stroke, splenic sequestration, acute renal failure, and cholecystitis.

The purpose of this document is to review sickle cell disease in pregnancy and to provide recommendations, screening and clinical management during prenatal and puerperium. Genetic screening can identify couples at risk for offspring with sickle cell disease and other hemoglobinopathies and allow them to make informed decisions regarding reproduction and prenatal diagnosis. Individuals with African, Southeast Asian, and Mediterranean ancestry are at a higher risk for being carriers of hemoglobinopathies and should be offered carrier screening.

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