Comparative Study Of Electrolyte Patterns In Sickle Cell Disease Patients

Comparative Study Of Electrolyte Patterns In Sickle Cell Disease Patients
Abstract
A study on the comparative study of electrolyte patterns in sickle cell disease patients in Owerri was carried out. Thirty sickle cell disease patients and twenty normal individuals as controls are included in the study. 11 patients on hydroxyurea therapy and 19 are nonhydroxyurea therapy. The serum levels of sodium, potassium, chloride, calcium, magnesium and phosphate were estimated in all the study subjects. There is significant difference observed among Total Bilirubin, Urea, and Creatinine and chloride levels among Sickle disease patients compared with normal individuals. No much significant results were observed among patients on hydroxyurea therapy and non- hydroxy urea therapy except for Total bilirubin and Sodium levels. The study concluded that electrolytes plays crucial role in the path physiology of sickle cell disease, but hydroxyurea therapy does not seem to alter the electrolyte levels in patients.
Table Of Contents
Preliminary Page(s)
- Title
- Declaration
- Approval
- Dedication
- Acknowledgement
- Abstract
- Table of Content
Chapter One
Introduction
- 1.1 Background of the study
- 1.2 Statement of the problem
- 1.3 Objectives of the study
- 1.4 Justification
Chapter Two
Literature Review
- 2.1 Overview of Sickle Cell Diseases
- 2.1.1 History
- 2.1.2 Epidemiology of sickle cell disease
- 2.1.3 Pathophysiology of sickle cell anaemia
- 2.2 Clinical features of sickle cell disease
- 2.2.1 General Features
- 2.2.2 Painful Crises
- 2.2.3 Infections
- 2.3 Sickle cell anaemia and nutrition
- 2.3.1 Sickle cell anemia and undernutrition
- 2.4 Health Care Needs for Sickle Cell Disease Patients
Chapter Three
Materials Methods
- 3.1 Research design
- 3.2 Population and Sampling
- 3.3 Inclusion Criteria
- 3.4 Exclusion Criteria
- 3.5 Instrumentation
- 3.6 Reliability
- 3.7 Validity
- 3.8 Sample Collection
- 3.9 Statistical analysis
- 3.10 Ethical Committee
Chapter Four
Results And Discussion
- 4.1 Results
- 4.2 Discussion
Chapter Five
Conclusion And Recommendations
- 5.1 Conclusion
- 5.2 Limitations
- 5.3 Recommendations
- Bibliography
Chapter One
Introduction
1.1 Background of the study
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). Inheritance of sickle cell disease predisposes to four main types of crises: vaso-occlusive, splenic sequestration, aplastic and hemolytic. The morbidity and mortality from these events may be manifested as pain, acute chest syndrome (intrapulmonary sickling), pulmonary hypertension, cardiac abnormalities, cerebrovascular hemorrhage/infarct, splenic or hepatic sequestration, autosplenism, renal disease, liver disease, avascular necrosis of the femoral head, priapism, and life-threatening acute hemolytic or aplastic anemia. Triggers for an acute crisis include hypoxemia, dehydration, acidosis, stress, infection, trauma, hypothermia or in some cases, no identifiable predisposing risk factors (Aldrich and Nagel, 2008).
Electrolytes are substances that become ions in solution and acquire the capacity to conduct electricity. The balance of electrolytes in the body is essential for the normal functioning of the cells and organs. Electrolytes measured by blood testing includes sodium (Na+), Potassium (K+), Chloride (Cl-) and bicarbonate (HCO3-). Electrolytes have normal range values, and complications may arise, if any of the electrolytes are higher or lower than the normal range values. Sodium (Na+) is the major positive ion (cation) in the fluid outside of the cells. In combination with chloride, it forms a salt (NaCl). Sodium (Na+) regulates the amount of total body water and plays a critical role in electrical communication especially in the brain, nervous systems and muscles.
Biochemical abnormalities have been associated with sickle cell disease; however there is paucity of information on the roles of these ions in the pathogenesis and management of sickle cell disease (Oladipo et al., 2005). In sickle cells, an abnormal activation of potassium chloride (K+Cl-) co-transport system was proposed to be involved in cell potassium (K+) loss and dehydration (Vitoux et al., 1989). Deoxygenation of sickle cell is known to increase cation permeability of sodium (Na+), Potassium (K+) and calcium (Ca2+) (Rhoda et al., 1990), At lower Hydrogen ion concentration (pH), urea was able to stimulate potassium – chloride (K-Cl-) loss from sickle cells, leading to cellular dehydration, even in regions of low pulmonary oxygen tension (PO2). Potassium – Chloride (K+ Cl-) co-transport is abnormally active in erythrocytes containing positive charged haemoglobins such as haemoglobin (S) (HbS) On deoxygenation, haemoglobin(S) (HbS) cells exhibit a distinctive solute permeability pathway, P sickle, activated stochastically and partially inhibited by 4,4- disothiocyano 2 – 2 disulfostibene (DIDS) and dipyridamole. It is often referred to as a cation channel although its permeability characteristics remains vague and its molecular identity is unknown (Browning et al., 2007).
Lowering the WBC count in sickle cell anaemia is potentially therapeutic. Hydroxyurea is good inhibitor of ribo-nucleotide reductase, which has positive effect by increasing fetal haemoglobin in red blood cells and decreases the episodes of pain events; thereby enhancing the use of hydroxyurea is a promising approach to improve health outcomes among sickle cell disease patients. Hydroxyurea, is a prototype known to promote HbF production indirectly by perturbing the maturation of erythroid precursors, is administered orally daily once, without any adverse effects and is effective for patients with sickle cell disease. This study therefore compares the electrolyte patterns in sickle cell disease patients.
1.2 Statement of the problem
Sickling of red cells in sickle cell anaemia patients is caused due to polymerization of molecules of deoxygenated haemoglobin – S into rigid, rod-like polymers. Fetal haemoglobin lacks beta-globin chain which inhibits sickling in vitro by interfering with the polymerization of haemoglobin-S. Many Clinical trials are conducted till date, the observed results suggested that increased fetal haemoglobin concentrations may have desired benefits in sickle cell anaemia. Cytotoxic agents have a positive effect by stimulating the fetal haemoglobin. Among them, hydroxyurea proved to be effective for clinical trials, as it is easy to administer and relatively safe. Beyond the cytotoxic effects of hydroxyurea, it also reduces bone marrow production of neutrophils, reticulocytes and platelets which is an important mediator of inflammation. Studies involving sickle cell patients showed that there is increased loss of body fluids and electrolytes leading to dehydration and metabolic errors. Hence proper electrolyte balance is required for the normal functioning of cells in the human body. Electrolyte imbalance leads to complications i.e. if any electrolytes values are higher or lower than the normal. The present study was carried out to evaluate and determine the relationship between electrolytes values in Sickle cell anaemia patients and observe the effects of hydroxyurea on serum electrolytes that are going through hydroxyurea therapy and non-hydroxyurea therapy. The serum electrolytes focused in this study are sodium, potassium, chloride, magnesium, phosphate and calcium.
1.3 Objectives of the study
The main objective of the study is to carry out a comparison of electrolyte patterns in sickle cell disease patients.
Specifically, the study sought:
- To determine the level of the sodium, potassium, bicarbonate and chloride electrolyte in patients with sickle cell disease.
- To compare the sodium, potassium, bicarbonate and chloride electrolyte level of patients with sickle cell disease with those of normal individuals.
1.4 Justification
Sickle cell disease is a public health problem and accounts for a lot of morbidity and mortality in the society. Sickle cell disease is much more prevalent in Africa and Mediterranean crescent. Although the effects of sickle cell disease (SCD) on general morbidity and mortality have been studied but not much work has been done on the comparative study of the electrolyte pattern in sickle cell disease patients. There is therefore the need to bridge this gap. Therefore, this work is done to justify the electrolyte pattern in sickle cell patient in Owerri metropolis.
Chapter Five
Conclusion And Recommendations
5.1 Conclusion
This study was carried out on the comparative study of electrolyte patterns in sickle cell disease patients. Serum electrolytes play a vital role in the patho – physiology of sickle cell disease and their complications. Dehydration induces sickling. From the current study, it was evident that the serum levels of most of the electrolytes vary significantly between sickle cell disease patients and controls but among the sickle cell patients with hydroxyurea and without hydroxyurea here is no statistical significance found.
5.2 Limitations
The sample size of the study was small, hence cannot be projected on whole population. Follow up cases can give better insights into the serum electrolytes values and other variables in this study.
5.3 Recommendations
Barriers to care and to compliance with treatment of sickle cell disease should be studied, including economic factors such as cost of care and insurance coverage.
Patient education on importance of compliance with drug management strategies, availability, and accessibility to medication should be improved.
The positive impact of alleviating micronutrient malnutrition on physical activity, education and productivity, and hence on national economies suggests that there is also an urgent need for increased effort to demonstrate the cost of these deficiencies, as well as the benefits of addressing them, especially compared with other health and nutrition interventions.
Assessment of quality of life (QOL) is necessary to fully understand the needs of adults and children with sickle cell disease; and health care professionals should be able to use effective measures to evaluate QOL in the management of sickle cell disease.


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