Newborn screening for sickle cell disease in the Republic of Benin.

AIMS: To develop a strategy for neonatal screening of sickle cell disease (SCD) and effective enrollment of affected neonates in a comprehensive follow-up programme adapted to the socioeconomic conditions, health structures and cultural background of an African setting. METHODS: The strategy implemented at the two largest maternity services of Cotonou, the economic capital of the Republic of Benin, involves a team of specifically trained midwives, first to identify pregnant women at risk, and second to provide active and repeated information and sensitisation to these women to encourage voluntary demand for newborn screening and enrollment in the follow-up programme. RESULTS: Among the consecutive pregnant women studied (about 3000), 79.5% of the informed women at risk for fetal SCD asked for testing of their offspring, 85.2% of the newborns who tested positive were enrolled in the programme, and more than 80% were still being followed up after 5 years. The under-five mortality rate in this series was 15.5 per 10,000, a figure that is 10 times lower than the general rate recorded in the Republic of Benin. CONCLUSIONS: The results demonstrate that this specifically tailored strategy is relevant to this setting, given the unique conditions of this African country.

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

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. (Blood. 2000;96:1685-1689)

Outpatient management of fever in children with sickle cell disease (SCD) in an African setting.

Because hospitalization and intravenous antibiotics for treatment of a potentially fatal bacterial infection in febrile children with sickle cell disease (SCD) are difficult to apply, outpatient treatment has been considered in developed countries for selected patients. Eligibility criteria and procedures may differ in developing countries because of unique economic and social conditions. After clinical evaluation within 36 hr of the onset of a fever exceeding 38.5 degrees C, children with SCD who are being closely followed as a part of a SCD cohort in Cotonou (West Africa), were treated as outpatients. The antibiotic regimen consisted of intramuscular injection of ceftriaxone 50 mg/kg/day for 2 days followed by amoxicillin 25 mg/kg x 3/day x 4 days and oral hyper-hydration. Patients were observed for 6 hr and thereafter discharged with a medical control at day 2, day 8 + day 15. All 60 children included completed their treatment, and none were lost to follow-up. A definite or a presumed bacterial infection was the cause of the febrile episode in 76.7% of cases. An appreciable decrease in fever was observed from day 2 and only 2 patients were hospitalized at day 3, one for abdominal painful crisis and one other for persistent fever without documented infection. No severe bacterial infections, recurrence of febrile episode, nor death were encountered during the follow-up. The cost of this outpatient approach is US $30 per patient as compared to US $140 per patient if the patient had been hospitalized. Outpatient management of febrile episode in children with SCD is feasible and cost-effective in Sub-Saharan African. It requires, however, improved medical education on SCD and immediate medical attention after the onset of fever.