Introduction
Sickle cell disease is a deadly disease. Sickle cell anemia is considered to be the most common kind of sickle cell disease. It is a critical disorder which causes the body to produce red blood cells that are sickle- shaped. The red blood cells are meant to transport blood and oxygen to various parts of the body. This is made possible through the hemoglobin (that transmit oxygen from the lungs to the other parts of the body). In case of sickle cell, there is abnormal hemoglobin which causes the cells to have a sickle shape affecting their proper functioning (Platt, Thorington and Brambilla et al, 1991). This piece of work looks at the disorder of sickle cell anemia and the various aspects associated with it.
Pathophysiology/ Causes of Sickle Cell Anemia
Sickle cell anemia is a disorder that is brought about by a mutation in the beta-globin chain in the hemoglobin. As stated earlier, rather than having normal hemoglobin, an individual suffering from sickle cell anemia has sickle hemoglobin that is characterized by polymerization when it gets deoxygenated. The process of interruption of normal tissue perfusion caused by the abnormal sickle cells is however complex and difficult to understand. All patients suffering from sickle cell anemia are not affected equally by this disease despite the fact that there is genetic identity at site of the sickle hemoglobin mutation. For this reason, the central components of the pathophysiology of sickle cell anemia may be based on secondary genetic determinants as well as the acquired erythrocyte and vascular damage as observed in an individual. Another vital focus of pathophysiology of sickle cell anemia is the loss of red blood cell elasticity. While normal red blood cells are reasonably elastic thus allowing the cells to pass easily through the capillaries, in sickle cell disorder, the red blood cells have low elasticity since low oxygen tension fosters the cells’ sickling which in turn damages the cell membrane thus reducing the elasticity (Bunn, 1997).
Diagnosis of Sickle Cell Anemia
Different diseases are diagnosed differently. Sickle cell anemia is diagnosed through blood count which is usually slightly lower as compared to other kinds of sickle cell diseases. Another way through which sickle cell anemia could be diagnosed is through the use of sickle solubility test which reveals the presence of sickle hemoglobin. This test involves the use of a mixture of hemoglobin S and a reducing solution and the results are observed. In case of sickle cell anemia, the solution is usually turbid while it is clear in case of normal hemoglobin. Hemoglomblin electrophoresis is also a diagnosis method. It involves use of a gel through which different kinds of hemoglobin are subjected to and expected to move at different speeds. Genetic testing is also used though to a very small extent due to lack of specificity in the results attained (Platt, Thorington and Brambilla et al, 1991).
Medical Treatment of Sickle Cell Anemia
There are various treatments that have been designed to fight the disease of sickle cell anemia. The main goal of treatment of sickle cell anemia is usually to manage and control the disease’s symptoms and more so to reduce the number of crises experienced by the patients. The medical treatment should be continuous, for instance, the taking of folic acid supplements to help make red blood cells.
Some of the common forms of treatments include provision of pain medicines for instance hydroxyurea, abundance consumption of fluids, as well as blood transfusions. Antibiotics are also advocated for to prevent bacterial infections particularly among children suffering from sickle cell anemia. Sickle cell anemia is associated with a variety of complications which ought to be treated to avoid further effects that can easily lead to death of a patient. Some of the treatments include; hip replacement incase of avascular necrosis in the hip, gall bladder removal incase one suffers from gallstone disease, kidney dialysis or transplant for those with a kidney disease, surgery of the eye incase of problems, wound care, treatments of persistent and painful erections as well as drug rehabilitation and provision of counseling programs in case of psychological complications.
It is believed that bone marrow or stem cell transplants cures to sickle cell anemia. However, this treatment is not usually common to many patients due to the fact that it is relatively difficult for a sickle cell anemia patient to get a well matching donor for the transplant. Despite this, it is evident that currently there are better ways of handling the disease and research studies are still underway in an effort to come up with better treatment options and thus save more lives. For instance, in the recent past, most sickle cell patients often died as early as 20years to 40years particularly due to organ failure but today, patients can live up to more than 50 years (Bunn, 1997).
Clinical Manifestation
Sickle cell anemia manifests itself in different ways. Various complications are experienced, for instance, Chronic Hemolysis that includes anemia, jaundice, delayed growth, pigment gallstones, and aplastic episodes. There is also Vaso-occlusive sickle cell crisis that accounts for most of the severe complications that are suffered by sickle cell anemia patients. The vaso-occlusion occurs in all places where blood flows and the crisis experienced are extremely painful. Some of the complications associated with vaso-occlusive include stroke, leg ulcers, chronic nephropathy, priapism, pain syndrome, papillary necrosis, acute chest pain and avascular hip necrosis among others. This shows how serious and painful sickle cell anemia is and hence the need to take appropriate preventive and curative measures (Dampier, Setty and Eggleston et al., 2004).
Nursing Interventions
Based on the manifestations of sickle cell anemia and the pain patients go through, there is dire need for nursing intervention aimed at not only prolonging life but also making living of the patients bearable, away from too much pain. It is found necessary for nurses to have adequate skills and knowledge in regard to handling the various issues that surrounds sickle cell anemia especially the painful crises. One area in which the nurses ought to be well educated is the physiologic process of pain. In addition, the crisis’ unchained factors should be well known and taken care of by the nurses. In a nut shell, there should be an appropriate way of dealing with the unchaining factors, an orientation and education program aimed at enlightening the patients, a way of focusing on the origin of the pain experiences as well as an appropriate application of the desirable interventions for each particular situation. This will greatly help in dealing with the various issues associated with sickle cell anemia and thus save the patients from any unnecessary pain and suffering (Platt, Thorington and Brambilla et al, 1991).
Nursing Assessment
Nursing assessment is deemed to be an extremely crucial aspect when it comes to management of sickle cell anemia among patients. Nursing assessment for instance play a significant role in prevention of sickle cell anemia and as we all know, prevention is usually better and easier than cure. Medically, sickle cell anemia can only occur when two individuals who have sickle cell traits bear a child together. For this reason, following nurse’s assessment, there could be some genetic counseling carried out to all individuals who have the sickle cell traits. Sickle cell anemia could also be detected during pregnancy and appropriate measures taken. For instance, the change in red blood cells shape could be prevented through certain measures such as treating of infections as soon as they are detected, getting adequate oxygen and getting of enough fluids. Nursing assessment is therefore a critical step that allows for better intervention measures to be taken (Bunn, 1997).
Conclusion
From the above discussion it is evident that sickle cell disease and sickle cell anemia to be specific is a critical disorder that not only claims a lot of lives but makes those who suffer from it go through a lot of pain and discomforts. Although there are treatment measures that have been put in place, there ought to be more effective preventive and curative actions among the medical practitioners in an effort to minimize the cases of sickle cell anemia and help in enhancing the lives of the patients in the best way possible.
Reference List
Bunn, H. F. (1997). Pathogenesis and treatment of sickle cell disease. The New England Journal of Medicine, 337:762.
Dampier, C., Setty B.N, and Eggleston B, et al. (2004). Vaso-occlusion in children with sickle cell disease: clinical characteristics and biologic correlates. J Pediatr Hematol Oncol 26:785.
Platt, O. S., Thorington, B.D., and Brambilla, D. J, et al. (1991). Pain in sickle cell disease. Rates and risk factors. The New England Journal of Medicine, 325:11.