Sickle cell anemia in the female patient

Seventy-two thousand Americans are homozygous for the sickle cell gene and 2 million are carriers. The gene offers protection against malaria but can be a cause of chronic pain and early death. Life expectancy is 48 years for females. Some people with sickle cell anemia live into their 60s and beyond. The purpose of this article is to review and summarize evidence from clinical, translational, and epidemiologic studies that have examined the clinically relevant aspects of sickle cell anemia as it relates to the female patient. Studies were identified through a MEDLINE search for articles in English between the years 1966 and 2005. References from identified reports were also used to identify additional articles. Women with sickle cell disease experience multiple complications. These complications can affect each and every organ system and are often worse in pregnant women. Progestins, hydroxyurea, and bone marrow transplant appear to ameliorate sickle cell anemia. Other therapies being evaluated include those that increase fetal hemoglobin concentration and prevent dehydration of the sickle red blood cells. More than one third of pregnancies in women with sickle syndromes terminate in abortion, stillbirth, or neonatal death. Recently, a number of genes modifying the clinical severity of sickle cell anemia have been identified. Sickle anemia is associated with immense suffering and multisystemic complications. In addition to the now-established therapy with hydroxyurea and bone marrow transplants, there are multiple investigational treatments that offer the hope of extending life expectancy while diminishing associated morbidities. Whether any of these new agents are safe in pregnancy has yet to be determined.