Various forms of hemopoietic cell transplantation are emerging as "cures" for sicle-cell syndromes and severe thalassemias (Hsieh, 2009). Oringanje and coworkers (20l3) performed a Cochrane review and found that only observational studies have been reported. Bone marrow transplantation has 5-year survival rates that exceed 90 percent (DaIle, 20l3). Cord-blood stem-cell transplantation from related donors also shows great promise (Shenoy, 2013). Finally, successful gene therapy has been accomplished by lentiviral vector-mediated addition of a beta globin gene into stem cells (Ribeil, 2017).
only after all other possible causes have been excluded. Pain
TABLE 56-2. Pregnancy Morbidity with Hemoglobin SS with sickle-cell syndromes is caused by intense sequestration of sickled erythrocytes and infarction in various organs, especially
Odds Ratios Oukome Hb.SS Hb.SC bone marrow. These episodes may develop acutely, especially
Preeclampsia 2-3.1 late in pregnancy, during labor and delivery, and early in the 2.0 puerperium.
Stillbirth 6.5 3.2
Preterm delivery 2-2.7 1.5
Growth restriction 2.8-3.9 1.5
Data from metaanalyses by Boafor, 201n6; Oteng-Ntim, 2015.
Pregnancy is a serious burden to women with any of the major sickle hemoglobinopathies, particularly those with hemoglobin SS disease. Several large studies have defined this relationship. Villers and colleagues (2008) studied 17,952 births in women with sickle-cell syndromes. Chakravarty and associates (2008) studied 4352 pregnancies. A more recent cohort study of 1526 women was reported by Boulet and coworkers (2013). Last, a cohort study taken from more than 2 million women compared those with sickle-cell disease to normal controls (Kuo, 2016). Common obstetrical and medical complications and their relative risks from a composite of most of these studies are shown in Table 56-2.
Maternal morbidity common in pregnancy includes ischemic necrosis of multiple organs, especially bone marrow that causes episodes of severe pain. Pyelonephritis, pneumonia, and pulmonary complications are frequent. Although the maternal mortality rate has improved, perinatal morbidity and mortality rates remain formidable (Boga, 2016; Lesage, 2015; Yu, 2009). Perinatal outcomes include increased risks for preterm birth, fetal-growth restriction, and perinatal mortality.
In nonpregnant women, morbidity and mortality rates from SC disease are appreciably lower than those from sickle-cell anemia. Indeed, fewer than half of these women have symptoms before pregnancy. In our experiences, afected gravidas sufer attacks of severe bone pain and episodes of pulmonary infarction and embolization more commonly than when they are not pregnant (Cunningham, 1983). Some adverse pregnancy outcomes are shown in Table 56-2.
Women with sickle-cell hemoglobinopathies require close prenatal observation. Any factor that impairs erythropoiesis or increases red cell destruction aggravates the anemia. Prenatal folic acid supplementation with 4 mg daily is needed to support rapid red blood cell turnover.
One danger is that a symptomatic woman may categorically be considered to be sufering from a "sickle-cell crisis." As a result, serious obstetrical or medical problems that cause pain, anemia, or both may be overlooked. Examples are ectopic pregnancy, placental abruption, pyelonephritis, or appendicitis. Thus, a diagnosis of sickle-cell crisis should be applied