Early-life clinical and hematological profiles: a comparative study of children with and without sickle cell disease in the first three years of life

Abstract

Sickle cell disease (SCD) has been associated with significant morbidity and mortality, particularly in early childhood. Understanding the hematological and clinical outcomes during the early years of life is crucial for guiding interventions to improve care for affected populations. This study investigates the relationship between fetal hemoglobin (HbF) levels, hematological profiles, and clinical outcomes in children with SCD, from birth to three years of age. By comparing children with and without SCD, the study aimed to identify trends in disease progression and highlight the need for early screening and intervention. This longitudinal study recruited and followed children aged 0-3 years. Clinical complaints and hematological parameters were recorded at each visit. Statistical analyses explored associations between HbF levels, hematological parameters, and clinical outcomes in children with SCD. 414 children were recruited, 35.5% with SCD and 64.5% without SCD. Children with SCD experienced a higher frequency of clinical complaints. The hospital admission rate was higher among children with SCD (19.6%) compared to those without (1.7%) (p < 0.007), with severe anemia being the leading cause of admission (56.3%). At birth, children with SCD had higher HbF levels (72.87%) that declined over time. The rate of decline correlated with an increased frequency of clinical events in children with SCD. Additionally, children with SCD had lower red cell indices, and higher reticulocyte count as well as white blood cell count. The results emphasize the need for early intervention and continuous monitoring to manage the disease effectively. Further research is recommended to explore the impact of different interventions on improving the quality of life and survival rates in children with SCD.