[Sickle-cell anemia in the 1st 5 years of life]

A programme for antenatal diagnosis of sickle-cell anaemia (SA) and SC haemoglobinopathy (SC) was started in Cuba in 1983. The purpose of this paper is to report the clinical and haematological data of a group of children diagnosed before birth or in the first few months of life.

Between June 1986 and December 1992, 78 children under 5 years of age were studied at the Haemoglobinpathies Clinic of the Instituto de Hematología e Inmunologia in La Habana; of them, 53 had SA and 25 had SC, there were 41 girls and 37 boys. A comprehensive questionnaire was carried out during the first visit to the clinic, about the onset of the clinical manifestations, as well as a careful physical examination plus peripheral blood study and Hb A2 and Hb F assay. Parents were informed, and folate and prophylactic penicillin were given orally to the children.

The yearly average admissions to hospital were 1.6 +/- 3.8 for SA and 0.1 +/- 0.6 for SC. The average number of veno-occlusive crises was 0.2 +/- 0.6 in SA and 0.1 +/- 0.6 in SC (p < 0.04); the average hand-foot crises were 0.3 +/- 1.1. for SA and nil for SC. The average number of infections per year was 0.3 +/- 0.6 in SA and 0.1 +/-0.3 in SC, without any significant difference. Significant differences were found in the haemoglobin rates and reticulocyte counts between both haemoglobinopathies. Hb F rate was 7.80 +/- 4.78% in SA and 2.83 +/- 3.45% in SC.

Admission to hospital, hand-foot crises, infections and splenic sequestration are the commonest clinical events during the first 5 years of life. The number of infections episodes decreases with prophylactic oral penicillin, and overacute pneumococcal infection is suppressed. Parent education allows one to early treat sequestration crises, and partial splenectomy avoids repetition of this complication, while preserving the splenic function.