Abstract
BackgroundAfrica has the highest burden of Sickle cell disease (SCD) but there are few large, systematic studies providing reliable descriptions of the disease spectrum. Tanzania, with 11,000 SCD births annually, established the Muhimbili Sickle Cell program aiming to improve understanding of SCD in Africa. We report the profile of SCD seen in the first 10 years at Muhimbili National Hospital (MNH).MethodsIndividuals seen at MNH known or suspected to have SCD were enrolled at clinic and laboratory testing for SCD, haematological and biochemical analyses done. Ethnicity was self-reported. Clinical and laboratory features of SCD were documented. Comparison was made with non-SCD population as well as within 3 different age groups (< 5, 5–17 and ≥ 18 years) within the SCD population.ResultsFrom 2004 to 2013, 6397 individuals, 3751 (58.6%) SCD patients, were enrolled, the majority (47.4%) in age group 5–17 years. There was variation in the geographical distribution of SCD. Individuals with SCD compared to non-SCD, had significantly lower blood pressure and peripheral oxygen saturation (SpO2). SCD patients had higher prevalence of severe anemia, jaundice and desaturation (SpO2 < 95%) as well as higher levels of reticulocytes, white blood cells, platelets and fetal hemoglobin. The main causes of hospitalization for SCD within a 12-month period preceding enrolment were pain (adults), and fever and severe anemia (children). When clinical and laboratory features were compared in SCD within 3 age groups, there was a progressive decrease in the prevalence of splenic enlargement and an increase in prevalence of jaundice. Furthermore, there were significant differences with monotonic trends across age groups in SpO2, hematological and biochemical parameters.ConclusionThis report confirms that the wide spectrum of clinical expression of SCD observed elsewhere is also present in Tanzania, with non-uniform geographical distribution across the country. Age-specific analysis is consistent with different disease-patterns across the lifespan.
Highlights
Africa has the highest burden of Sickle cell disease (SCD) but there are few large, systematic studies providing reliable descriptions of the disease spectrum
The Muhimbili Sickle cell (MSC) program was established through collaboration between Muhimbili University of Health and Allied Sciences (MUHAS), the oldest and largest biomedical university in Tanzania and Muhimbili National Hospital (MNH), the national referral hospital which is in Dar-es-Salaam on the Eastern coast
The median age at testing was higher in non-SCD than in SCD individuals (p < 0.001), while the sex ratio was similar between the two groups and there was no difference among those who were coming outside Dar compared to those coming from outside Dar es Salaam (Table 1)
Summary
Africa has the highest burden of Sickle cell disease (SCD) but there are few large, systematic studies providing reliable descriptions of the disease spectrum. The main goal was to conduct research that would delineate the spectrum of disease, identify the main causes of morbidity and mortality and provide evidence-based knowledge that would improve healthcare through the introduction of locally-appropriate interventions and policies. Preliminary information on this population, mortality rates [16], rates and risk factors for malaria [17, 18], bacterial infections [19] and other important clinical features [20,21,22,23] has been published. The MSC program has provided a platform to conduct studies to describe genetic determinants of clinical disease [24], successfully conducting one of the first genome-wide association studies in SCD in Africa [25]
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