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Hemoglobin S-C disease and fetal well-being: A case report

Most women with sickle-cell (S-C) disease now live long enough to have children. However, the pregnancies of these women are associated with an increase of maternal morbidity and a high rate of perinatal mortality and morbidity [1–3]. The high perinatal mortality rate [4], ranging from 58 to 134/ 1000, is attributed to chronic anemia or to altered conductance through the intervillous space by the sickling cell and vaso-occlusion that may lead to placental insufficiency; the same mechanisms are also proposed to explain the increased incidence of fetal growth restriction, prematurity and spontaneous miscarriage [1–3]. Furthermore, the ideal approach to blood transfusion of pregnant women with S-C disease is not clear. We describe a case of resolution of fetal heart rate (FHR) decelerations and normalisation of Doppler indices in umbilical artery, middle cerebral artery and ductus venosus in a pregnant woman with hemoglobin S-C disease, after blood transfusion. A 17-year-old, woman from Mali, gravida 1 para 0, was admitted to our unit at 25 weeks and 5 days gestation for abdominal pain. At that date, her medical history was uneventful. On admission, physical examination was unremarkable; especially, neither hypertension nor proteinuria were noted; hemoglobin level was 9.4 g/dL and platelet count was at 146 giga/L; furthermore, moderate liver cytolysis (aspartate and alanine aminotrasferase levels at 150 and 125 IU/L, respectively) was found but no diagnostic criteria for hemolysis (lactate deshydrogenase levels within normal ranges and absence of schistocytes on peripheral blood smear) was noted, excluding complete hemolysis elevated liver enzymes low platelets syndrome. However, hemoglobin S-C disease was diagnosed (HbS, 51.4%, HbC, 48.6%). All other laboratory data were normal, particularly the tests for hepatitis (A, B and C antigen) were negative. Finally, FHR was initially normal. Continuous intravenous hydration, oxygen therapy and screening for infections were performed. Two 12 mg doses of betamethasone were given at 24 h interval. Two days later, the patient reported painful crisis; at that moment, fetal monitoring showed recurrent moderate-to-severe variable decelerations [Figure 1(a)]; ultrasonography found a small for gestational age fetus whose estimated weight was 650 g; Doppler velocimetry revealed first absent enddiastolic velocity in umbilical artery [Figure 2(a)], second a decreased middle cerebral artery pulsatility index at 0.96 with a middle cerebral artery peak systolic velocity at 38.7 cm/s (1.15 multiple of median) and third a low atrial waveform velocity in ductus venosus at 7 cm/s. A blood transfusion of 3 units was administered resulting in an increase of hemoglobin level to 11.3 g/dL, and percentage of HbA1 to 30.1%. During follow-up, painful crisis was relieved by symptomatic treatment and antibiotic therapy; at this date, a moderate pre-eclampsia was diagnosed. Immediately after transfusion, no anomaly was noted in repeat FHR tracings [Figure 1(b)]. At 27 weeks and 6 days gestation, ultrasonography demonstrated significant fetal growth with an estimated fetal weight of 850 g; Doppler analysis showed normal pulsatility indices in umbilical artery [Figure 2(b)] and middle cerebral artery (1.20 and 1.21, respectively); furthermore, middle cerebral artery peak systolic velocity was 53.5 cm/s (1.44 multiples of median), and atrial