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Updated: Oct 19, 2025
A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Paediatric sickle cell disease at a tertiary hospital in Malawi: a retrospective cross-sectional study
Chikondi Sharon Chimbatata1, Master Ro Chisale2,3, Alfred Bornwell Kayira3
1Paediatrics, Mzuzu Central Hospital, Mzuzu, Northern Region, Malawi.
Insights
Sickle cell disease (SCD) in Malawian children is characterized by anemia, sepsis, and painful crises. Painful crises increase hospital stay, highlighting the need for prompt diagnosis and management of SCD.
Area of Science:
- Paediatric Hematology
- Communicable Diseases Epidemiology
- Public Health
Background:
- Sickle cell disease (SCD) is a significant cause of childhood mortality and morbidity in Malawi.
- Comprehensive literature on paediatric SCD in Malawi is scarce, necessitating further research.
Purpose of the Study:
- To describe the clinical features, management, and outcomes of paediatric sickle cell disease in Malawi.
- To identify factors associated with prolonged hospital stay and case fatality in children with SCD.
Main Methods:
- Retrospective review of clinical files for paediatric patients diagnosed with SCD.
- Descriptive statistics and logistic regression analyses were employed to analyze data.
- Statistical tests (χ², Fisher's exact, binary logistic regression) were used to identify significant associations.
Main Results:
- SCD accounted for 3.1% of paediatric hospitalizations, with 13.3% of cases newly diagnosed during the study period.
- Common clinical features included anemia (94.1%), sepsis (79.5%), and painful crisis (54.3%).
- Patients with painful crisis had a 1.7 times higher likelihood of prolonged hospital stay.
Conclusions:
- Anemia, sepsis, and painful crisis are prevalent in paediatric SCD patients in Malawi.
- Delayed diagnosis of SCD is a critical issue requiring urgent attention.
- While in-hospital mortality is relatively low, it may not reflect the overall disease burden due to potential under-reporting.
Introduction:
Sickle cell disease (SCD) remains a major cause of childhood mortality and morbidity in Malawi. However, literature to comprehensively describe the disease in the paediatric population is lacking.
Methods:
A retrospective review of clinical files of children with SCD was conducted. Descriptive statistics were performed to summarise the data. χ2 or Fisher's exact test was used to look for significant associations between predictor variables and outcome variables (case fatality and length of hospital stay). Predictor variables that were significantly associated with outcome variables (p≤0.05) in a χ2 or Fisher's exact test were carried forward for analysis in a binary logistic regression. A multivariable binary logistic regression was used to identify covariates that independently predicted length of hospital stay.
Results:
There were 16 333 paediatric hospitalisations during the study period. Of these, 512 were patients with SCD representing 3.1% (95% CI: 2.9%- 3.4%). Sixty-eight of the 512 children (13.3%; 95% CI: 10.5% - 16.5%) were newly diagnosed cases. Of these, only 13.2% (95% CI: 6.2% - 23.6%) were diagnosed in infancy. Anaemia (94.1%), sepsis (79.5%) and painful crisis (54.3%) were the most recorded clinical features. The mean values of haematological parameters were as follows: haemoglobin (g/dL) 6.4 (SD=1.9), platelets (×109/L) 358.8 (SD=200.9) while median value for white cell count (×109/L) was 23.5 (IQR: 18.0-31.2). Case fatality was 1.4% (95% CI: 0.6% - 2.8%)and 15.2% (95% CI: 12.2% -18.6%) of the children had a prolonged hospital stay (>5 days). Patients with painful crisis were 1.7 (95% CI: 1.02 - 2.86) times more likely to have prolonged hospital stay than those without the complication.
Conclusion:
Anaemia, sepsis and painful crisis were the most common clinical features paediatric patients with SCD presented with. Patients with painful crisis were more likely to have prolonged hospital stay. Delayed diagnosis of SCD is a problem that needs immediate attention in this setting. Although somewhat encouraging, the relatively low in-hospital mortality among SCD children may under-report the true mortality from the disease considering community deaths and deaths occurring before SCD diagnosis is made.
