Age at diagnosis of sickle cell disease in a developing country

Area of Science:

• Pediatrics
• Hematology
• Public Health

Background:

• Sickle cell disease (SCD) is a significant inherited blood disorder.
• Early diagnosis of SCD is crucial for timely intervention and improved outcomes.
• Understanding factors influencing age at diagnosis can inform public health strategies.

Purpose of the Study:

• To determine the median age at diagnosis for sickle cell disease in children.
• To identify factors influencing the age at diagnosis at University College Hospital Ibadan.

Main Methods:

• A retrospective study was conducted reviewing case notes of 457 children with SCD.
• Data collected included hemoglobin phenotype, socioeconomic class, and history of dactylitis.
• Statistical analysis was performed to identify significant factors.

Main Results:

• The median age at diagnosis was 2.0 years (range: 2.5 months - 14.0 years).
• Children with Hb SS were diagnosed earlier than those with Hb SC (p=0.01).
• Higher socioeconomic class (p=0.003) and a history of dactylitis (p=0.000) were associated with earlier diagnosis.

Conclusions:

• Late diagnosis of sickle cell disease remains a challenge in Ibadan.
• Neonatal screening programs are recommended to facilitate early detection and improve survival rates.