Sickle Cell Disease Blood Disorder Library Resources

NHLBI estimates that sickle cell disease (SCD) affects 70,000 to 100,000 people, mainly African Americans, in the U.S. (NHLBI, 2009). The disease occurs in about 1 out of every 500 African American births and 1 out of every 36,000 Hispanic American births (ibid). Sickle cell disease is an inherited blood disorder characterized by defective hemoglobin, a protein in red blood cells that carries oxygen to the tissues of the body. Although individuals with sickle cell disease now have better survival rates, the disease remains a major public health problem. Millions worldwide suffer complications from this disease. It is estimated that over 2 million Americans are carriers of a sickle cell trait (SCT) with a carrier rate of 1 in 12 for Blacks and 1 in 100 for Hispanics. 

An example of a reproductive life stage concern for women with SCD and women with sickle cell trait is childbearing. Women with SCD are known to have high-risk pregnancies mainly because of fetal risks.  These women also face serious maternal risks, such as an increased risk of experiencing both medical complications (infections and thromboembolic events) and pregnancy-related complications (preeclampsia, eclampsia, preterm labor, placental abruption, and fetal growth restriction). The risk for experiencing these complications is significantly higher than in other women. Women with SCD also have higher rates of cesarean deliveries. SCD complicates 0.1% of pregnancies but accounts for 1% of all maternal deaths (Villers MS, et al, 2008). Not only women with SCD but also women with SCT are at risk of having a child with SCD if their partner also carries sickle cell or thalassemia trait. Women with SCT are known to be at higher risk of urinary tract infections during pregnancy and perhaps other infections; they have double the risk of blood clots, further increasing the risk of thromboembolic events associated with pregnancy itself. 

Another area of concern for women with SCD is menstruation. Research has indicated that menstrual cycles can contribute to painful crises in some women with sickle cell disease. One of the most important concerns cited by women and girls with SCD and SCT was the pain they experienced during menstruation (SCDAA annual meeting, September 2010, input from attendees to the FWGBD presentations). Additionally, recent studies show low usage of hormonal contraceptives and lack of access to family planning resources within this population group (O'Brien S, et al, 2011).

Acknowledging the severity of menstrual pain and appropriately managing this pain in women and girls with sickle cell disease/trait is essential to improving the care of sickle cell patients. Providing preventive care to the pregnant woman with sickle cell disease/trait is essential in warding off severe or life-threatening complications. Healthcare professionals should be educated about the range of morbidity associated with this disease in menses and in pregnancy. Also, a more specialized network of treatment centers is needed to reach reproductive-age women with sickle cell disease/trait, especially within the African- and Hispanic-American population groups. A stronger network of clinics providing comprehensive coordinated care can dramatically reduce complications, hospitalization rates, and medical costs. Education and research can help determine the most effective model for identifying and caring for women with this disease.