Prevalence, Pattern of Disease and Outcome of Children with Sickle Cell Disease Admitted in a Private Health Facility in Southern Nigeria

Introduction: Sickle cell disease, an autosomal recessive genetic disorder is characterized by chronic haemolytic anaemia often leading to life-threatening events triggered by acute sickling of red blood cells and microvascular occlusion resulting in frequent admissions.

Aim: To determine the prevalence, pattern of disease and outcome of children with sickle cell disease admitted at a private health facility in southern Nigeria.

Study Design: A retrospective study.

Place and Duration of Study: Study was carried out at a private paediatric hospital in Port Harcourt, Rivers State over a 1-year period.

Methodology: Data was retrieved from the hospitals’ Health Management System. Information obtained were age, sex, diagnosis, indication for transfusion if any, number and types of transfusions, duration of admission, mode of payment of bills and outcome.

Results: Out of 1597 admissions, 59 were children with sickle cell disease giving a prevalence of 3.7%. Males predominated with a Male:Female ratio of 1.7:1, majority were £ 5 years (50.8%) and had insurance (86.4%). Most were admitted during the rainy season (61.0%) and had duration of stay of 1 – 5 days (74.6%). Commonest diseases among these children were sepsis (69.5%), vaso-occlusive crises (52.5%), malaria (52.5%) and severe anaemia/hyper-haemolytic crisis (30.5%). Of the 35.6% who received blood transfusion, majority received sedimented cells (81.0%), were transfused once (71.4%) with majorly blood group O Rhesus D Positive blood (44.8%). One child died with mortality rate of 1.7%. Vaso-occlusive crisis and tonsillitis were significantly observed more in older children ³ 12 years whereas there was no statistically significant association between gender and duration of stay with the pattern of disease.

Conclusion: Sickle cell disease accounted for 3.7% of total admissions with a mortality rate of 1.7%. Sepsis, vaso-occlusive crisis, malaria and severe anaemia/hyper-haemolytic crisis were the commonest diagnosis. Neonatal screening, parental education, patient compliance, vaccinations, regular follow up, and comprehensive health insurance will significantly reduce morbidity and mortality in persons with Sickle cell disease.