Sickle Cell Anaemia (SCA) management requires regular monitoring which is often challenging in resource-limited settings. This study explores the novel use of Platelet-Neutrophil Ratio (PNR) as a comprehensive monitoring tool in SCA. We present a case of a Jehovah’s Witness child with SCA, managed with hydroxyurea for elevated transcranial Doppler (TCD) velocities. PNR was retrospectively analysed against clinical parameters over 18 months. PNR showed significant correlations with Packed Cell Volume (r=0.588, p=0.002), Mean Corpuscular Volume (r=0.739, p<0.0001), and a negative correlation with TCD velocity (r=-0.53, p=0.115). A positive correlation with Haemoglobin F (r=0.461, p=0.434) was also observed. Notably, PNR fluctuations corresponded with clinical status; lower values coincided with a vaso-occlusive crisis, while higher values were associated with stable condition and optimised hydroxyurea therapy. This case highlights PNR’s potential as a valuable supplementary marker for routine SCA monitoring, particularly in resource-constrained environments. By providing a cost-effective means of assessing disease progression and predicting complications, PNR could significantly enhance patient outcomes. Further research is warranted to validate PNR’s efficacy and establish comprehensive guidelines for its clinical integration, potentially revolutionising SCA management in low- and middle-income countries.

Background: Sickle cell anaemia (SCA) is a prevalent genetic disorder in sub-Saharan Africa, characterised by frequent vaso-occlusive crises and other complications. Hydroxycarbamide (hydroxyurea) has proven effective in managing SCA but is underutilised in low- and middle-income countries (LMICs) due to cost and monitoring challenges. This study aims to ascertain the impact of a fixed low-dose hydroxycarbamide regimen on emergency room visits and treatment adherence for paediatric SCA patients in a resource-limited setting. Methods: An observational study was conducted over two years (January 2022 to December 2023) in a tertiary hospital in South East Nigeria. One hundred children aged 1 to 18 years with confirmed SCA (HbSS genotype) were administered a fixed low-dose hydroxycarbamide regimen (20 mg/kg/day, capped at 500 mg/day). The primary outcome was the number of emergency room visits due to SCA-related crises. Secondary outcomes included treatment adherence and factors influencing adherence. Patients experiencing breakthrough crises had their doses increased to 25 mg/kg/day or from 500 mg to 750 mg. Results: Over the study period, only one patient (0.01%) on the fixed low-dose regimen presented to the clinical emergency room with a vaso-occlusive crisis, necessitating a dose increase. Treatment adherence was high, with 84.2% of patients reporting consistent adherence throughout the study period. Younger patients were more likely to adhere to treatment (mean age of compliant patients: 8.49 ± 5.28 years vs. non-compliant: 11.62 ± 4.21 years, p=0.027). Father’s age was also associated with adherence, with non-compliant patients having older fathers (median age 49.0 vs. 44.0 years, p=0.02). No significant clinical side effects were observed. Conclusion: The fixed low-dose hydroxycarbamide regimen significantly reduced emergency room visits and demonstrated high adherence rates among paediatric SCA patients in a resource-limited setting. This approach appears to be a safe, effective, and feasible treatment strategy for managing SCA in LMICs, potentially improving accessibility and sustainability of care.

Background: Adherence to follow-up visits is crucial for optimizing clinical outcomes in paediatric patients with sickle cell disease (SCD). This study aimed to evaluate compliance with follow-up visits among paediatric SCD patients over a five-year period. Methodology: A retrospective analysis was conducted using data from the sickle cell disease clinic of Nnamdi Azikiwe University Teaching Hospital, spanning from January 2015 to December 2019. A total of 271 unique paediatric patients with SCD and 1117 follow-up visits were included. Compliance with follow-up visits, defined as attendance within 100 days of the previous visit, was assessed using Kaplan-Meier curves and Cox regression analysis. Results: During the study period, a total of 1117 follow-up visits were documented among 271 children diagnosed with sickle cell anaemia. The median age of the study cohort was 8 years (IQR: 4 to 12 years). Male patients constituted 57% of the study population. Fifty percent of subjects remained compliant with follow-up visits at 14.2 months (95% CI = 8.9-24.1 months) and this compliance decreased to 40% at 24 months (95% CI = 31-49 months). Males showed a lower likelihood to be compliant to follow-up visits than females (HR: 0.83; CI: 0.56-1.23). Following adjustments, patients who were sicker and young adolescents (10 to 14 years) had an increased likelihood of complying to follow up visits (HR: 1.05; CI: 0.71-1.57 vs HR: 1.15; CI: 0.682-1.940 respectively) but this was not statistically significant. Conclusion: The overall poor compliance rate to follow up visits underscores the urgent need for interventions aimed at improving adherence to follow-up visits in this vulnerable population. Counselling interventions and further exploration of factors influencing compliance are warranted to enhance the management and outcomes of children living with sickle cell anaemia.