Sickle cell anaemia in adults at the Kenyatta National Hospital, Nairobi Kenya

Abstract

The clinical, haematological, radiological and other data were collected from 38 adults of both sexes and analysed with a view to describing the natural history of homozygous sickle cell disease at the Kenyatta National Hospital. The report demonstrates marked variability in the clinical features of sickle cell anaemia in the adult population. The improved standard of living and the availability of health facilities have had favourable influence on the clinical course of sickle cell anaemia at the Kenyatta National Hospital. In the steady state the chronic haemolytic anaemia was characterized by significant reticutocytosis. The other bone marrow functional elements were similarly active as evidenced by the neutrophil leucocytosis. Persistently low mean corpuscular volume (MeV) was found in 20% of the subjects who also had the microcytic hypochromic features on the peripheral blood smear consistent with iron deficiency. The dietary history confirmed nutritional iron lack, but it is planned to scree.n the family members for the S-beta~thalassaemia trait in a future study. The clinical spectrum of sickle cell anaemia in these adults is characterized by the vascular occlusive crises and multi-organ pathological complications both of which constitute an important reason for-hospital admission. Avascular femoral head necrosis, chronic osteomyelitis, leg ulceration and cholelithiasis contributed significant morbidity. Severe chronic haemolytic anaemia on the other hand was the cause of fatal intractable heart failure in one case and most likely was contributory to the second death reported in the present series. It is hoped that this report will stimulate more interest in the clinical aspects of sickle cell anaemia in East Africa. Now that the biochemical studies have made such great strides it is only fitting that more attention should be directed towards the practical application of these technological advances.