Sickle cell disease in Sierra Leone: a neglected problem

  • GT Roberts
  • AE Gabba
  • TL Roberts
  • G Deen
  • I Wurie
Keywords: Sickle cell disease, Sierra Leone, survival, anaemia, haemoglobinopathy

Abstract

Background: Sickle cell disease (SCD) is common in Sierra Leone although its exact prevalence, incidence and clinical spectrum are unknown.
Methods: Using a statistical package, StatsDirect (Altrincham, United Kingdom) we analyzed the demographic characteristics, presentations, acute events, treatments and clinical outcomes in a cohort of SCD patients attending sickle cell clinics in Freetown, Sierra Leone between February 2007 and August 2010.
Results: There were 446 patients, median age of 13 years. Of these, 435 were homozygotes (HbSS), median age 13 years also. There were 248 females, median age 12.5 and 198 males, median age 14, resulting in a male:female ratio of 0.79. Eleven (2.4%) were Sickle Cell-HbC disease, median age 14 years. Patients demonstrated many of the typical features of SCD. The most common reason for hospital admission was bone pain crisis associated with an infection, followed by severe anemia. Aseptic necrosis of the femoral head, leg ulcers, septic osteomyelitis and gallstones were seen in 0.22% of patients, but strokes and acute chest syndrome were not observed. The death rate was 2.51/100 patient years observation with an estimated mean survival of 3.6 years (CI 3.2-3.7). Severe anemia was implicated in the death of 8 patients (50%), whereas only 2 deaths (12.5%) were attributable to bone pain crisis. One death (6.25%) was associated with pregnancy complicated by severe anemia and another with an adverse blood transfusion event.
Conclusion: The clinical outcomes in this series highlight the need for a more comprehensive provision of care for SCD patients in Sierra Leone.

Keywords: Sickle cell disease, Sierra Leone, survival, anaemia, haemoglobinopathy

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print ISSN: 0016-9560