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Updated: Apr 30, 2026
A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Verbal autopsy as a tool for identifying children dying of sickle cell disease: a validation study conducted in
Carolyne Ndila1, Evasius Bauni, Vysaul Nyirongo
1Kenya Medical Research Institute (KEMRI)/Wellcome Trust Programme, Centre for Geographic Medicine Research-Coast, P,O Box 230, Kilifi, Kenya. cndila@kemri-wellcome.org.
Insights
Verbal autopsy (VA) can help identify sickle cell disease (SCD) as a cause of childhood death in sub-Saharan Africa. This study shows VA accurately attributes SCD deaths, improving mortality data.
Area of Science:
- Public Health
- Epidemiology
- Medical Diagnostics
Background:
- Sickle cell disease (SCD) is a significant cause of mortality in children across sub-Saharan Africa (SSA).
- High childhood mortality from SCD often goes unrecognized due to a lack of newborn screening and accurate death recording.
- Verbal autopsy (VA) is explored as a method to identify SCD as an underlying cause of death (COD) in children.
Purpose of the Study:
- To evaluate the utility of verbal autopsy (VA) tools in attributing sickle cell disease (SCD) as a cause of death (COD) in children in rural Kenya.
- To assess the accuracy of two VA coding methods (physician-coded verbal autopsy [PCVA] and InterVA-4 model) against gold standards.
Main Methods:
- Utilized the 2007 WHO Sample Vital Registration with Verbal Autopsy (SAVVY) tool for child deaths in the Kilifi Health and Demographic Surveillance System (KHDSS), Kenya (2008-2011).
- Coded VAs using physician review (PCVA) and the InterVA-4 probabilistic model, referencing ICD-10.
- Validated VA COD assignments against hospital physician diagnoses and laboratory-confirmed SCD status.
Main Results:
- PCVA attributed 6% and InterVA-4 attributed 5% of deaths to SCD.
- High agreement coefficients (AC1) were observed between VA methods and hospital diagnoses (95.5% for PCVA, 96.9% for InterVA-4).
- Agreement between PCVA and laboratory-confirmed SCD status was 87.5%.
Conclusions:
- Modified WHO VA instruments, including specific SCD questions and codes, enhance its utility for quantifying SCD's contribution to childhood mortality.
- VA shows promise as a valuable tool for assessing SCD-related mortality in rural African communities.
- Further research is needed to confirm the generalizability of these findings.
Background:
Sickle cell disease (SCD) is common in many parts of sub-Saharan Africa (SSA), where it is associated with high early mortality. In the absence of newborn screening, most deaths among children with SCD go unrecognized and unrecorded. As a result, SCD does not receive the attention it deserves as a leading cause of death among children in SSA. In the current study, we explored the potential utility of verbal autopsy (VA) as a tool for attributing underlying cause of death (COD) in children to SCD.
Methods:
We used the 2007 WHO Sample Vital Registration with Verbal Autopsy (SAVVY) VA tool to determine COD among child residents of the Kilifi Health and Demographic Surveillance System (KHDSS), Kenya, who died between January 2008 and April 2011. VAs were coded both by physician review (physician coded verbal autopsy, PCVA) using COD categories based on the WHO International Classification of Diseases 10th Edition (ICD-10) and by using the InterVA-4 probabilistic model after extracting data according to the 2012 WHO VA standard. Both of these methods were validated against one of two gold standards: hospital ICD-10 physician-assigned COD for children who died in Kilifi District Hospital (KDH) and, where available, laboratory confirmed SCD status for those who died in the community.
Results:
Overall, 6% and 5% of deaths were attributed to SCD on the basis of PCVA and the InterVA-4 model, respectively. Of the total deaths, 22% occurred in hospital, where the agreement coefficient (AC1) for SCD between PCVA and hospital physician diagnosis was 95.5%, and agreement between InterVA-4 and hospital physician diagnosis was 96.9%. Confirmatory laboratory evidence of SCD status was available for 15% of deaths, in which the AC1 against PCVA was 87.5%.
Conclusions:
Other recent studies and provisional data from this study, outlining the importance of SCD as a cause of death in children in many parts of the developing world, contributed to the inclusion of specific SCD questions in the 2012 version of the WHO VA instruments, and a specific code for SCD has now been included in the WHO and InterVA-4 COD listings. With these modifications, VA may provide a useful approach to quantifying the contribution of SCD to childhood mortality in rural African communities. Further studies will be needed to evaluate the generalizability of our findings beyond our local context.
