Clinical Features and Outcome of Sickle Cell Patients in Pediatric Emergency Departments in Lubumbashi, Democratic Republic of Congo

Aims: To describe the epidemiology clinical outcome, and therapeutic aspects of sickle cell patients admitted to pediatric emergency departments, and to identify the risk factors associated with the clinical outcome.

Study Design: Descriptive cross-sectional study.

Place and Duration of Study: University of Lubumbashi teaching hospital, pediatric emergency departments and Jason Sendwe Referral Hospital from 10 February 2020 to 30 April 2022.

Methodology: 105 children with sickle cell disease aged 6 months to 16 years were admitted and recruited in the study. The Kaplan-Meier method was used to analyze survival to D22, the log-rank test to compare survival curves, and Cox regression to identify mortality risk factors, at a significance level of p ˂ 0.05.

Results: The main reasons for admission were infection (83.8%), hyperalgesic vasoocclusive crisis (73.3%) and severe anemia (36.2%). According to the Adegoke severity score, 60% of the children had a severe clinical profile, 32.4% were moderate and 7.6% were mild. Median survival was 5.9 days. Survival decreased from 80% on day 2 to 67.6% on day 22. Survival was significantly shorter for moderate and severe clinical profiles (P = 0.001), transfer from a peripheral hospital (P = 0.006), and diagnosis of an infectious syndrome (P = 0.002). The critical period was the first 2 days of hospitalization, with a mortality rate at 20% compared with an all-cause mortality rate at 12.4%. In the adjusted multivariate analyses, death risk factors were transfer (P=0.04), severe clinical profile (P=0.033), hospital stay >2 days (P=0.04), infectious syndrome (P=0.01) and suspected hepatocellular failure (P=0.009).

Conclusion: Sickle cell morbidity and mortality in Lubumbashi are high and associated with mostly controllable risk factors. The prognosis for sickle cell disease can be improved by training health workers in sickle cell disease and by better organizing specific care at all levels of the health pyramid.