Effect of active prenatal management on pregnancy outcome in sickle cell disease in an African setting

Abstract

Sickle cell disease (SCD) is associated with an increased risk of medical complications during pregnancy. In sub-Saharan Africa, fetal and maternal mortality rates are particularly high. This study evaluated the effect of an active prenatal management program on pregnancy outcome in patients with SCD in an African setting. Pregnant women with SCD attending the National Teaching Hospital in Cotonou (The Republic of Benin, West Africa) were recruited before the 28th week of gestation. Management was based on providing information and education about SCD and improving nutritional status, malaria prevention, early detection of bacterial infections, and restricted use of blood transfusion. Maternal and fetal mortality rates and SCD-related morbidity were the principal variables assessed. One hundred and eight patients (42 SS and 66 SC) with 111 fetuses were included in the study. Thirteen fetal deaths (from 9 SS and 4 SC mothers) were recorded and 2 deaths of SC mothers. The maternal mortality rate of 1.8% was comparable with the overall maternal mortality rate for this maternity unit (1.2%). Few SCD-related events were recorded. Plasmodium falciparum malaria infection was the major cause of morbidity. Sixty-three patients (19 SS and 44 SC) successfully completed their pregnancy (58.3%) without requiring transfusion. Providing pregnant SCD patients with relevant medical care based on simple cost-effective approaches can have a positive impact on SCD-associated morbidity and mortality in an otherwise difficult setting in Africa.

Sickle cell disease: Reducing the global disease burden

“…For example, in Benin where neonatal screening and comprehensive health care management were practiced, the under-five mortality rate of SCD was 15.5 per 10 000, which is ten times lower than the overall under-five mortality rate. 20,21 These results are comparable with those in high-income countries, demonstrating the advantages of newborn screening and close follow-up of children in a comprehensive care approach. 22 Despite the overwhelming evidence of the efficacy of newborn screening for SCD, no country in SSA with 75% of the world's disease burden has a universal newborn screening program in place.…”

“…25 Educational programs regarding the timely recognition of signs of infection and prompt clinical care are best accomplished in the setting of neonatal screening programs. 21,25 The HbS gene mutation is included in a number of genetic alterations that have been shown to have a similar distribution to that of endemic malaria. Geographically, an association exists between the areas of the highest HbS allele frequencies and high malaria endemicity globally, as has been elegantly shown in sub-Saharan Africa, where the carrier state (sickle cell trait) is extremely common.…”

Prophylactic red blood cell exchange may be beneficial in the management of sickle cell disease in pregnancy

“…In addition, the odds of fetal death, preterm birth, low birthweight, and cesarean delivery were higher for females with SCD compared with those with no reported hemoglobinopathies after adjusting for maternal age, education, parity, plurality, insurance status at delivery, prenatal care utilization, smoking, and infant sex. [6][7][8][9] The maternal mortality rate was 1.6 per 1000 deliveries in patients, compared to 0.1 per 1000 in women without SCD. Pregnant women with SCD had a higher risk for preeclampsia, eclampsia, venous thromboembolism, cardiomyopathy, intrauterine fetal demise, and intrauterine growth restriction.…”

“…and treatment for individuals with SCD, coupled with advancements in neonatal care, have contributed to a decline in morbidity and mortality both for mother and fetus; however, the physiologic changes in pregnancy still carry important clinical risks for some patients with SCD. 8,9 Previous studies have reported that females with SCD had a higher prevalence of preeclampsia, lung disease, and heart disease during the antenatal, intrapartum, or postpartum periods compared with women without hemoglobinopathies. In addition, the odds of fetal death, preterm birth, low birthweight, and cesarean delivery were higher for females with SCD compared with those with no reported hemoglobinopathies after adjusting for maternal age, education, parity, plurality, insurance status at delivery, prenatal care utilization, smoking, and infant sex.…”

“…Low-and middle-income countries generally report increased maternal and perinatal morbidity and mortality in association with SCD. [9][10][11][12][13][14] In Turkey, maternal and fetal morbidity and mortality remain a major problem in pregnant patients with SCD. This retrospective study investigates the pregnancy outcomes of SCD patients who underwent prophylactic red blood cell exchange (RBCX) procedures from January 2000 to March 2013, and results were compared with a control group.…”