Project Abstract

Abstract
Sickle cell disease (SCD) is a genetic blood disorder that affects millions of individuals worldwide. Patients with SCD often experience a variety of complications, including electrolyte imbalances. Serum sodium concentration is an important electrolyte that plays a crucial role in maintaining the body's fluid balance and is essential for proper nerve and muscle function. This study aimed to investigate the serum sodium concentration in sickle cell patients and its correlation with disease severity and clinical outcomes. A comprehensive literature review was conducted to identify relevant studies on serum sodium concentration in sickle cell patients. The search included databases such as PubMed, Scopus, and Google Scholar, using keywords related to sickle cell disease, serum sodium, electrolyte imbalance, and clinical outcomes. Studies that reported on serum sodium levels in sickle cell patients and their association with disease severity, complications, and mortality were included in the review. The findings from the literature review revealed that sickle cell patients often exhibit lower serum sodium levels compared to individuals without the disease. Several factors were identified that could contribute to the dysregulation of sodium balance in SCD, including dehydration, vaso-occlusive crises, and renal dysfunction. Lower serum sodium levels were associated with increased disease severity, higher rates of hospitalizations, and a greater risk of complications such as acute chest syndrome and stroke. Furthermore, the review highlighted the importance of monitoring serum sodium levels in sickle cell patients as part of routine clinical care. Regular assessment of electrolyte imbalances, including sodium, can help healthcare providers identify patients at risk of complications and implement appropriate interventions to maintain electrolyte balance and improve clinical outcomes. In conclusion, serum sodium concentration is a critical parameter that warrants attention in the management of sickle cell disease. Monitoring and maintaining optimal sodium levels in these patients may help reduce the risk of complications and improve overall health outcomes. Further research is needed to better understand the mechanisms underlying sodium dysregulation in sickle cell disease and to develop targeted interventions to address this issue effectively.

Project Overview

INTRODUCTION

Sickle cell disease (SCD) is a group of inherited disorders of the beta-hemoglobin chain. Normal hemoglobin has 3 different types of hemoglobin – hemoglobin A, A2, and F. Hemoglobin S in sickle cell disease contains an abnormal beta globin chain encoded by a substitution of valine for glutamic acid on chromosome 11 (Bunn,2007). This is an autosomal recessive disorder. Sickle cell disease refers to a specific genotype in which a person inherits one copy of the HbS gene and another gene coding for a qualitatively or quantitatively abnormal beta globin chain. Sickle cell anemia (HbSS) refers to patients who are homozygous for the HbS gene, while heterozygous forms may pair HbS with genes coding for other types of abnormal hemoglobin such as hemoglobin C, an autosomal recessive mutation which substitutes lysine for glutamic acid. In addition, persons can inherit a combination of HbS and β-thalassemia. The β-thalassemias represent an autosomal recessive disorder with reduced production or absence of β-globin chains resulting in anemia. Other genotype pairs include HbSD, HbSO-Arab and HbSE (Meremiku, 2008).

Sickle hemoglobin in these disorders cause affected red blood cells to polymerize under conditions of low oxygen tension resulting in the characteristic sickle shape. Normal red cells live about 120 days in the blood stream but sickled red cells die after about 10 – 20 days. Because they cannot be replaced fast enough, the blood is chronically short of red blood cells, a condition called anaemia. Aggregation of  sickle cells in the microcirculation from inflammation, endothelial abnormalities, and  thrombophilia lead to ischemia in end organs and tissues distal to the blockage (Hayes, 2004).