Holistic care of patients with sickle cell anaemia (HbSS) was carried out in a dedicated support group and clinic in Lagos. This paper examines the outcome of this initiative using mortality, hospital admission and blood transfusion rates from inception in April 1988 to December 1995. Patients with sickle cell disorder and their families were admitted to the Sickle Cell Club and its associated Sickle Cell Clinic. All patients and parents were counselled on recruitment and were regularly followed up within an interactive family friendly environment. Other measures included preventive health and nutritional education, prompt treatment of illness and free supplies of vitamin supplements, malarial prophylactic and other necessary medication. The records of consecutive patients with HbSS were reviewed for this study. Over the study period, the number of subjects increased from 290 in 1988 to 1223 in 1995. The mortality rate fell from 20.6% in 1988 to 0.6% in 1995 (P < 0.0001); the number of hospital admissions fell from 350 (119%) in 1988 to 30 (4%) in 1995 (P < 0.0001); the number of patients transfused with blood fell from 260 (90%) in 1988 to 25 (2%) in 1995 (P < 0.00001). We conclude that the provision of well-organized holistic care can significantly reduce illness and deaths and improve the quality of lives of people living with HbSS in developing countries.

The pattern of acute illness was determined in 102 adolescents and adults with sickle cell anaemia who presented to the emergency unit of a Lagos hospital. The patients had a mean age of 20.5 years (SD 13.1) and a male-female ratio of 1.5. The symptoms included fever (72%), fatigue and weakness (59%), anorexia (59%) and pain (57.5%) while major clinical signs were pallor (100%), jaundice (71%) and hepatomegaly (68%). Sixty-eight per cent of patients had sickle cell crises, including one with hemiplegic stroke, 10% with combined anaemia and pain crises, 33% with anaemia crises only and 23.5% with pain crises only. Sixty-three per cent had infection which was malaria in 24.5%, bacterial in 17% and viral in 6%. Of 16 patients with pyrexia of unknown origin, seven responded to treatment with chloroquine and eight to antibiotics. Infection was detected in 50% of the patients with sickle cell crises. The association between anaemia crises and malaria was significant (P < 0.05). Of the eight deaths, seven (88%) had anaemia crises. In contrast to studies conducted two decades ago in the same hospital, the prevalence of anaemia crises now exceeds that of pain crises and malaria now exceeds that of bacterial infection. Severe symptomatic anaemia (anaemia crisis) was more frequently associated with infection (mostly malaria) than was bone pain crisis. The Girdle pain crisis more frequently resulted in a fatal outcome than the uncomplicated bone pain crisis.

No abstract

No abstract