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Sickle Cell Disease



Abstract

This research paper focuses on the investigation of sickle cell disease, its pathophysiological mechanisms, structural characteristics, and potential health threats. It also examines the issues of its emergence, development and long-term consequences. The paper researches the questions, related to the diagnostics of the disease, the examination of laboratory tests data and the discussion of the possible nursing interventions as well as the evidence-based management practices. The investigation of the problem applies the scientific methods of literary review, content analysis, synthesis, and generalization. The results of the research reveal the complex problem of sickle cell disease, approaches to its diagnosis and effective medical interventions.

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The findings show that this disorder is a serious blood disease, which causes modifications and irrevocable changes in the form and structure of the red blood cells. The available diagnostic tools and laboratory tests allow examining the disease and defining the effective ways of treatment and looking after the patient. Since the disease may cause different symptoms, the nursing interventions may considerably vary. Overall, the sickle cell disease is a blood disorder, which calls for a complex system of interventions and evidence-based management practices, determined by the laboratory analyses and tests.

Keywords: sickle cell disease, hemoglobin S, deoxygenation, polymerization, nursing interventions.

Research Paper Introduction

Sickle cell disease or sickle cell anemia is the disease, which affects the functioning of the red blood cells and leads to the irrevocable changes in their internal and external structures. The emergence of the disease is connected with the abnormal levels of chemical substances within the blood cells. To be more precise, the researchers connect this disease with the changes in the level of concentration of hemoglobin S, potassium, and oxygen in the red blood cells (Yazdanbakhsh, Ware, & Noizat-Pirenne, 2012). The sickle cell disease changes the shape of blood cells under the influence of dehydration, deoxygenation, and overwhelming polymerization.

As a result, the disease constitutes serious threats to the general functioning of the cardiovascular system. The sickle cell disease may be diagnosed with a variety of methods, which help to indicate the degree of damages and define the most appropriate nursing interventions and treatment programs. This paper analyzes the cellular pathophysiology of sickle cell anemia, defines the diagnostic methods and laboratory data correlations, and discusses effective nursing interventions and evidence-based implications for nursing practice.

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Pathophysiology of Sickle Cell Disease on the Cellular Level

Sickle cell disease or sickle cell anemia is the result of malfunctioning of blood cells, caused by the abnormal levels of hemoglobin S and insufficient oxygenation (Yazdanbakhsh, Ware, & Noizat-Pirenne, 2012). As a result, the blood cells undergo some significant modifications and changes. To start with, the process of deoxygenation leads to the insufficient circulation of the hemoglobin S, in the blood flow. As a result, the lack of oxygen changes the structure of the hemoglobin protein and makes it interact with other substances and chemicals (Nur et al., 2011).

The researches specifically show that deoxygenated hemoglobin tends to establish connections with the hydrophobic residues of the globin, rather than hydrophobic valine residues (Yazdanbakhsh, Ware, & Noizat-Pirenne, 2012). Consequently, such modifications change the very structure of the fibers of the blood cells, which acquire the shape of sickles. Actually, this resemblance has determined the name of the disease.

The emergence of the long, sickle-shaped fibers activates other abnormal mutations of the blood cells, triggering the pathophysiological mechanisms of the diseases. For example, the appearance of the long fibers itself ruins the homeostatic stability within the blood cells, for the account of activation of several channels, responsible for the regulation of chemical content of the cells (Nur et al., 2011). As a result, the launching of new kinds of ion channels leads to the reduction of potassium and increases cellular dehydration. In its turn, the dehydration results in the overwhelming polymerization within the blood cells. This process does not favor the health of the patients since it creates additional load on the functioning of the cardiovascular system in general, and blood circulation, in particular (Yazdanbakhsh, Ware, & Noizat-Pirenne, 2012).

The polymerization leads to the concentration of hemoglobin and some other proteins on the external membranes of the cells. Obviously, such a situation violates the natural microenvironment of the cells and causes the appearance of new forms of connections and links between different groups of chemicals (Yazdanbakhsh, Ware, & Noizat-Pirenne, 2012). It causes the formation of anionic phosphatidylserines and membrane phospholipids. In the long run, such modifications lead to the generation of micro-particles, stored on the external membranes of blood cells.

Another reason, explaining the pathophysiology of the sickle cell disease, is the reduction of cells’ elasticity (Nur et al., 2011). Normally, the blood cells possess high flexibility and elasticity, which allows them to adapt to different conditions and preserve their internal stability. Besides, the elasticity is the vital quality, which allows red blood cells passing through the capillaries without losing their essential properties and forms. The insufficient oxygenation reduces the elasticity of blood cells and damages their membranes, as well. Frequently, the cells are unable to recover their primary forms, even if the oxygen tension returns to the normal index. Thereby, the less elastic blood cells tend to cause vessel occlusion and anemia, as they pass through the small capillaries (Nur et al., 2011).

Additionally, the sickle cell disease occurs in the case of the prevalence of hemoglobin F over the hemoglobin S. As it has been already mentioned, the hemoglobin S shows great capacities for interaction with other groups of chemicals (Yazdanbakhsh, Ware, & Noizat-Pirenne, 2012; (Nur et al., 2011). However, its reduction under the circumstances of deoxygenation increases the amount of hemoglobin F. The latter causes the rigidity of cells’ membranes and activates the polymerization of the red blood cells. When long, sickle-shaped fibers appear, the cells lose their capacities of adjusting to the microenvironment and overcoming the negative outcomes of the enormous polymerization.

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The rate of development of the vascular crisis and sickle cell disease depends primarily on three factors. These are the level of deoxygenation, the presence or absence of hemoglobin F and the degree of hemoglobin S concentration. Depending on these variables, the structure of the red blood cells may either rapidly change or preserve its homeostasis during a continuous period. Moreover, the polymerization usually does not occur in the case of microcirculation of blood, which passes through small capillaries in a short period.

Although the density of hemoglobin intracellular concentration and rates of polymerization do not visually differ from those in the normal red blood cells, these factors lead to the changes in the density distribution of the damaged cells. The researches show that the increase of thickness and polymerization depends on the high level of reticulocytes together with a relatively decreased concentration of hemoglobin S (Nur et al., 2011). The very presence of the dense cells leads to the combination of dehydration and the damages of the intracellular membranes in the process of external polymerization. The last stage of this irrevocable process is the formation of long sickle-shaped cells with the defected membranes and decreased elasticity, which cannot be improved even by the normal supplies of oxygen.

The dehydration of red blood cells highly depends on the amount of potassium in the microenvironment. The concentration of potassium is responsible for the regulation of blood circulations as well as the preservation of homeostatic stability. However, the changes in the amount of potassium seem to influence the dehydration of cells and create difficulties with the normal circulation of red blood cells (Nur et al., 2011). Therefore, the pathophysiology of the sickle cell disease highly depends on the rate of potassium and its interconnections with the transporting mechanisms of the cells.

Overall, it is possible to conclude that the pathophysiology of the sickle cell disease depends on such factors, as the degree of hemoglobin S, concentration of potassium, indexes of dehydration and deoxygenation, the spread of intracellular polymerization, and the level of elasticity. The malfunctioning of these elements and their modifications lead to the irrevocable changes in the structure and shape of the red blood cells. As a result, the sickle cell disease indicates such conditions of the red blood cells as the inability to restore flexibility and acquire a sufficient amount of oxygen under the influence of overwhelming concentration of hemoglobin S and polymerization of external membranes.

Diagnostics of the Disease and Laboratory Data Correlations

Sickle cell disease can be diagnosed with a variety of methods and tools. One of the most frequent methods of diagnostics includes the utilization of blood film. This measure helps to discover the structure of the blood cells and determine any modifications and changes, if existent. Normally, the red blood cells should have a level of hemoglobin S in the range of 6-8 g/dl (Fonseca et al., 2012). If the level of this protein remains higher than usual, it can indicate the sickle cell crisis. Another evidence of the sickle cell disease is the low level of a reticulocyte count. The latter is responsible for the generation of red blood cells. If the level of reticulocyte count remains below normal, it signals about the damages of red blood cells.

Another way of laboratory diagnostics of the sickle cell disease is the usage of sodium metabisulfite (Fonseca et al., 2012). This method helps to define the concentration of chemicals within the blood cells and compare them with the normal indexes. Moreover, this diagnostic measure assists in determining any modifications of the internal and external structure of red blood cells. In the same way, the “sickle solubility test” defines the level of hemoglobin S in the blood microenvironment (Fonseca et al., 2012).

The abnormal rates of hemoglobin S can be also observed with the help of hemoglobin electrophoresis and gel electrophoresis (Fonseca et al., 2012). These laboratory analyses focus on the investigation of the red blood cells’ speed and the amount of hemoglobin in them. Frequently the genetic testing may be used to identify sickle cell disease. In fact, this is one of the most important and reliable methods, since the sickle cell crisis is a genetic disease, which can be transmitted from parents to children. Thus, the examination of the genetic nature and characteristics may strongly assist in the diagnosis of sickle cell anemia.

In addition, the method of stained RBC examination helps to distinguish between the normal, sickled-shaped and partially damaged cells. This tool is used to examine all types of blood cells and their modifications (Fonseca et al., 2012). In the case of the sickle cell disease, the stained RBC examination is effective in focusing on the structure of the red blood cells and defining any changes in their structures.

Furthermore, the sickle cell disease can be diagnosed with a variety of other universal tests and methods. For example, the total iron-binding capacity test (TIBC) shows whether the level of potassium in the blood cells remains normal or decreased (Yang et al., 2013). In case it is below normal, there is a probability of the appearance of the sickle cell crisis. Similarly to it, the serum potassium and uric acid test helps to examine the degree of vascular crisis, on account of measurements of potassium content (Fonseca et al., 2012).

The serum iron test shows the degree of hemoglobin S in the blood cells. It also helps to indicate the level of destruction of the cells’ membranes under the influence of the increased polymerization and hemoglobin storage (Yang et al., 2013). The erythrocyte fragility test assists in measuring the elasticity of the blood cells and determining the damages of the intracellular membranes (Fonseca et al., 2012). This diagnostic method provides data regarding the speed of circulation of red blood cells and the period needed for recovery from the negative influences, caused by the destruction of the homeostasis.

Commonly there are many diagnostic tests and laboratory data examinations, which help to define the sickle cell disease and inform about the rates of its development. Different tests and researches can show the levels of hemoglobin and potassium concentration as well as the degrees of polymerization and extracellular membranes’ damages. The diagnostic data and investigation results create a background for future nursing interventions and disease management.

Nursing Interventions and Evidence-Based Implications for Nursing Practice

Sickle cell disease may become apparent from a variety of symptoms. Depending on the symptoms, the nursing interventions should perform different functions to provide the most optimal care and support for the patients.

  1. First, the sickle cell disease may be reflected in the symptoms, similar to restlessness, tachycardia, cyanosis, confusion or dyspnea. This happens on account of deoxygenation or the reduction of oxygen supplies within the red blood cells. The other possible reasons for such symptoms may be attributed to the low oxygen tension in the cells, increased blood viscosity, and density (Nur et al., 2011). Such displays of the sickle cell disease call for the following nursing interventions. The nurses should monitor the respiratory activities of patients and take care of the functioning of accessory muscles.

Besides, it is essential to monitor the signs of fever, chest pain or fatigue. The nurses should teach patients to do respiratory and physical exercises, needed for the improvement of breathing and muscles’ activities. In addition, the nurses should monitor the heart’s activities of the patients and involve relaxation and visualization techniques for the sake of improvement of their well-being. It is also important to take care of fluid consumption, which should not be less than 2-3 l per day (Yawn et al., 2014). The other possible nursing interventions may include administration of supplemental humidified oxygen, chest physiotherapy, prescription of medicine and conducting of the required laboratory tests (Nur et al., 2011).

  1. Second, the sickle cell disease may occur in a form of acute pain, resulting in localized aches, commonly affecting the head, abdomen, bones, muscles, back, and joints. The pain may occur on account of deoxygenation of blood cells and deprivation of nutrients and essential chemicals within the cells’ microenvironment. In such a case, the nurses should be prepared to perform the following interventions. They should monitor the durability and area of pain localization as well as provide the essential methods of treatment, according to the doctor’s prescription.

They should also teach patients alternative pain relief techniques, such as relaxation exercises, meditation, guided imagery, and breathing techniques. The nurses may also need to provide massages of the affected areas and encourage physical exercises if the health conditions of the patients allow them. Additionally, it is essential to control the appropriate fluid and medicine consumption. The patients should apply warm compresses to avoid the cooling of the aching areas of the body. Finally, the nurses should organize the laboratory tests and measures to check the conditions of the patients (Yawn et al., 2014).

  1. Third, the sickle cell disease can be displayed in the form of skin injuries and problems. This happens due to the inappropriate circulation of blood, especially through small vessels and capillaries (Nur et al., 2011). As a result, nursing interventions should focus on the prevention of ischemic diseases, skin breakdowns, and open wounds. The interventions should include a set of care techniques, devised to avoid skin injuries and traumas. Under such conditions, the nurses should teach patients how to take care of personal health, even in sitting or lying positions. The nurses have to observe the skin of the patients and make the massages to prevent the emergence of any injuries and wounds (Yawn et al., 2014).

It is also important to keep patients in a dry, clean, and warm environment to avoid the overcooling of the body. The care of existed wounds and injuries should correlate with the available treatment techniques and supportive measures. In addition, nurses should provide the patients with essential information, regarding the self-help and behavior in emergency cases.

It is also common knowledge that sickle cell disease may cause the dehydration of blood cells. Correspondingly, patients may suffer from the lack of required fluids in the blood flow. Actually, the symptoms of this problem are not evident, as they can be observed only on the cellular level. However, the nurses should be ready to deal with this risk factor and provide the required treatment and help. Under the conditions of dehydration, the possible nursing interventions should involve strict maintenance of fluid consumption during the day. Water consumption should correlate with the relevant diet and medicine prescription.

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  1. The nurses should evaluate such conditions of the patients’ health as the risk of fever, changes in the level of consciousness, the degree of skin turgor, and ask about the emergence of any kind of local aches (Yawn et al., 2014). Nurses have to assist in the procedure of blood transfusion and all the corresponding treatment techniques, prescribed by the doctor. Furthermore, the duties of nursing interventions include the analysis of laboratory tests and the administration of the needed procedures and exercises.

In addition, sickle cell disease may manifest in the form of ineffective tissue perfusion. It means that dehydration, deoxygenation, and polymerization may lead to inflammatory processes and cardiovascular problems. This outcome of the sickle cell disease may cause such symptoms as changes in the pulse and blood pressure indexes, angina, palpitations, visual disturbances, tingling in the extremities as well as bone and muscle aches. Therefore, the nursing evident-based management practices should include the monitoring of the patients’ well-being, checking the blood pressure and pulse, observing the existence of the inflammatory processes (Yawn et al., 2014).

It is also important to monitor the changes in the consciousness levels, the appearance of headaches and fevers, and the development of sensory and motor deficits. The nurses should also report about the changes in the localized aches, disturbances in vision, and coordination of movements. Moreover, the nursing interventions should also focus on the maintenance of appropriate room temperature, dryness, and cleanness. The appropriate nutrition and fluid consumption are vital, under such circumstances too.

  1. Finally, the researches and doctors admit that sickle cell disease may be followed by the problem of deficient knowledge (Yawn et al., 2014). It means that the patients are not aware of the seriousness of the disease, its characteristics, and its consequences. To improve the situation, they may ask different questions, request information, express the need in therapeutic interventions and sessions, participate in continued medical follow-ups and apply for a range of treatment programs (Yawn et al., 2014). Under such circumstances, it may be difficult to make the right diagnosis and define the causes of problems. That is why nursing interventions should combine the elements of therapeutic and counseling support in order to prevent any mental disorders and problems.

In this case, the nursing evident-based management techniques should start with the explanation of the disease and providing the necessary information about it to the patients. Secondly, the nursing interventions should promote a healthy way of life as well as discourage such habits, as alcohol consumption, smoking, chronic stresses, and irregular regime. It is also essential to provide instructions, regarding self-care and self-support in case of development of any problems, connected with the disease.

Finally, the nursing interventions should emphasize the importance of avoiding stressful situations, maintenance of the effective conditions of care and sufficient communication between the patients and their families. Generally, only a complex approach to the treatment of sickle cell anemia may guarantee a positive result.

Research Paper Conclusion

In conclusion, nursing interventions depend on the actual displays and symptoms of sickle cell anemia. Since this disease may appear in a variety of forms, the nursing interventions should change correspondingly. Sometimes the nursing monitoring and administration bear a universal character, typical for the treatment of other diseases. Therefore, the primary role in the treatment of sickle cell disease belongs to the effective diagnosis and examination of the problem.

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In addition, the sickle cell disease can be diagnosed with the help of such methods as hemoglobin electrophoresis, gel electrophoresis, blood film, stained RBC examination, total iron-binding capacity test, serum potassium and uric acid test, serum iron test, and others.

The pathophysiology of sickle cell disease is connected with the three main factors. These are the deoxygenation of blood cells, the level of hemoglobin S concentration and the degree of external polymerization. These factors lead to the structural changes of the red blood cells and make them less elastic and flexible. In addition, the amount of potassium, dehydration, and storage of hemoglobin F is also responsible for the emergence of the disease. The symptoms of the sickle cell disease are quite different, as they depend on every individual case of diagnostics.

However, they commonly appear in the form of:

  • skin injuries;
  • localized pains;
  • muscle, head and bone aches;
  • cardio-vascular disorders;
  • visual disturbances;
  • changes in the level of consciousness;
  • fatigue;
  • development of sensory and motor deficits;
  • ischemic problems.

The nursing interventions and evident-based management practices depend on the symptoms of the disease. Generally, they focus on the maintenance of comfortable conditions for the patients; controlling the compliance with the regime and medical prescriptions; administration of nutrition and fluid consumption; performance of breathing and physical exercises; providing the essential information and instructions; and management of the procedures and treatment processes.

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