Management is essentially identical to that for women with cardiac disease (Chap. 49, p. 953). Women should be kept comfortable, but not oversedated. Conduction analgesia is ideal (Camous, 2008). Compatible blood should be available. If a diicult vaginal or cesarean delivery is contemplated, and the hematocrit is <20 volumes percent, then packed erythrocyte transfusions are administered. There is no categorical contraindication to vaginal delivery, and cesarean delivery is reserved for obstetrical indications (Rogers, 2010).
Many clinicians do not recommend combination hormonal contraception because of potential adverse vascular and thrombotic efects. In their systematic review, however, Haddad and coworkers (2012) found that complication rates were not higher with their use in women with sickle-cell syndromes. he Centers for Disease Control and Prevention categorizes combination hormonal contraception, intrauterine devices, implants, and progestin-only contraception as having no risk or as having advantages that generally outweigh theoretical or proven risks (Curtis, 2016).
The frequency of sickle-cell trait among African-Americans averages 8 percent. Carriers have occasional hematuria, renal papillary necrosis, and hyposthenuria, which is urine of low specific gravity (Tsaras, 2009). And although controversial, sickle-cell trait does not appear to be associated with increased rates of abortion, perinatal mortality, low birthweight, or pregnancy-induced hypertension (Pritchard, 1973; Tita, 2007; Tuck, 1983). One unquestioned relationship is the twofold increased incidence of asymptomatic bacteriuria and urinary infection. Sickle-cell trait should not be considered a deterrent to pregnancy or to hormonal contraception.
Inheritance is a concern for the fetus of a mother with sickle-cell trait whenever the father carries a gene for abnormal hemoglobins that include S, C, and D or for 3-thalassemia trait. Prenatal diagnosis is discussed in Chapter 14 (p. 290).
Approximately 2 percent of African-Americans are heterozygous for hemoglobin C, but even if homozygous, hemoglobin C is innocuous (Nagel, 2003). Only when coinherited with sickle-cell trait to yield hemoglobin SC is the trait problematic. Pregnancy in women with homozygous hemoglobin CC disease or C-3-thalassemia carries relatively benign associations. Table 56-3 shows our experiences from Parkland Hospital (Maberry, 1990). Other than mild-to-moderate anemia, pregnancy outcomes were not abnormal. Supplementation with folic acid and iron is indicated.
Data from Maberry, 1990.