A ten year review of the sickle cell program in Muhimbili National Hospital, Tanzania

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.

Cerebral Infarcts and Vasculopathy in Tanzanian Children With Sickle Cell Anemia

“…Full blood counts were obtained at the time of recruitment. For those who had been enrolled in a previous study, 21 we obtained from that database available daytime oxygen saturation (SpO 2 ) measured in clinic by pulse oximetry, hemoglobin level, reticulocyte count, and indirect bilirubin. Where daytime oxygen saturation and hemoglobin were available on the same day, arterial oxygen content (CaO 2 ) was calculated as:…”

A qualitative study on aspects of consent for genomic research in communities with low literacy

“…We recruited research participants enrolled in an urban hospital-based study aiming to understand genetic determinants of clinical heterogeneity of sickle cell disease (SCD) in Tanzania [18,19]. At the time of recruitment, the study consisted of 3751 individuals with SCD, 1779 were between 5 and 17 years of age with almost a 50/50 gender distribution [20]. Participants for the study reported here, were recruited through adult and paediatric clinics at Muhimbili National Hospital.…”

Higher oxygen saturation with hydroxyurea in paediatric sickle cell disease

“…Furthermore, when we performed the analysis excluding three patients who had adenotonsillectomy for OSA after starting HU (in these children improvements of SpO 2 could depend on relief of upper airway obstruction rather than HU), differences in nocturnal SpO 2 remained significant. Regarding other potential confounders, over the study period, the centres involved did not apply major changes in the standards of care for SCD patients (apart from increasing use of HU), and the SpO 2 would have been expected to decrease, rather than increase, by time 5 21 34. Therefore, it is unlikely that variables not taken into account had a relevant influence on changes in oxygen saturation in patients on HU.…”