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Okpala et al (2002) suggested that methods of pain relief in SCD may depend on the type of pain presented: acute, chronic or a mixture of the two types, and if the patient is opioid-naive or opioid tolerant. Therefore, each chromosome in a pair controls half the total globin chain production. The essay sheds light on the process of production, transaction, and consumption of acids within the human body. People who have sickle cell disease frequently experience painful attacks known as sickle cell crises, which can result in death. Hemolytic anemia is present by the age of 6 months, although vascular occlusion symptoms may not appear until later. The gene therapy used was an erythroid-specific gathering of anti-sickling proteins that would make up more than half of hemoglobin and almost all of the red blood cells in the body. And as already highlighted, chronic pain management for patients with SCD is still insufficient (Kutlar, 2005). A male preponderance has been reported in Africa and the United States, which is poorly understood (Serjeant et al., 2005). These are vasoocclusive, aplastic, splenic sequestration, and hyperhemolytic. Genetically speaking, its made up of 2 alpha and 2 beta chains. Multiple studies now demonstrate that the degree of anemia does not correlate with fetal outcome. In someone who has SCD, the red blood cells become hard and sticky and look like a C-shaped farm tool called a “sickle” . IV fluids, which are delivered via a vein, may be administered as well. Europpean Journal of Scientific Research Vol.32 No.4 (2009); pp.528-532 59National Heart, Lung, and Blood Institute: Evidence-Based Management of Sickle Cell Disease. Advances in treatment for patients with SCD already resulted to lengthened lifespan but patients still experience pain, lower quality of life, depression and mortality at young ages still remain for some patients. In a study conducted by Smith and colleagues (2008) validating the biopsychosocial model for management of adults with SCD pain and their health care use, found that pain among SCD patients were a daily phenomenon and many of the patients struggled with their pain at home than coming to the hospital or ER. This will be limited to available records of SCD patients. Lowering amount of circulating leukocytes, and reticulocytes. Alters. Not consenting or withdrawing consent, may adversely affect certain features and functions. If the damage cannot be arrested through medications, the spleen shrinks in size and ceases to function. The findings of this study will add to the knowledge about available options, rationale for preferences by both patients and health care providers, and provision of best practices towards the treatment and control of pain among SCD patients. Each effect will be marked by the nurses depending on their observation of positive or negative occurrences. Deficiency of folate or vitamin B12 inhibits purine and thymidylate syntheses. People who are older are more likely to develop complications such as chronic lung disease and painful leg ulcers as they age. Each globin chain gene guides the RBC to make a specific globin chain; the globin chains match with a heme and then assemble into a complete Hb molecule. Review of literature Sickle Cell Disease Also called sickle-cell anemia, SCD is a disorder ad inherited abnormalities of the. There are various methods and medicines used to treat or reduce pain among SCD patients. IN TRAININGS.pptx SICKELE CELL DISEASE MODULE 3 SEJOJO.pptx TO BE PRESENTED. The American Society of Hematology is developing guidelines for sickle cell disease pain management, which will be released in 2020. Painful crises are treated symptomatically with analgesics.
Using this form of gene therapy is still experimental and needs further testing before it can become a viable option for those with SCD. Delayed growth and sexual maturation appear to be related to nutritional factors, although the exact mechanism is still not clear. This leads to anemia, and as a consequence to hepatitis and other infectious diseases. Opioid is an alternative for uncontrolled pain and the amount may be increased in small increments such as diamorphine at 2-3 mg to prevent the risk of central nervous system depression (Okpala et al, 2002). GP Chi tren hay l? kho c?n xem nhi?u.pdf 1. GP Chi tren hay l? kho c?n xem nhi?u.pdf Sickle cell disease 1. It was hypothesized that because of the impact that it has on the body it must also play a role in something as important as proper sleep. Transfusion and hydroxyurea are the mainstays of current treatment; additional therapies are in development and await demonstration of efficacy in the clinic. If a patient exhibits signs of resistance to standard treatments, he or she should be referred to an appropriate specialist. Some people may suffer from what is called Sickle Chest Syndrome. In other types of sickle cell disease, one copy of HbS and a copy of a different abnormal ?-globin subunit are inherited. The mechanism is sickling and sludging of blood in the corpora cavernosa, with failure of detumescence. Fetal haemoglobin consist of ?2?2 the Gamma globin chain binds HbS, and prevents. Sickle cell disease SCD is a serious inherited condition affecting the blood and various organs in the body. The majority of people with SCD prefer to manage mild or moderate painful episodes at home. Early diagnosis and regular medical care to prevent complications also contribute to improved well-being. Healthy red blood cells are round and they move through small blood vessels carrying oxygen to all parts of the body. This can be done with over-the-counter medications, as well as prescription painkillers. Sickle RBC adherence to endothelium is the sine qua non of this interaction and is mediated by a number of cell surface receptors on the RBC and on the endothelial cell. In the DH, individualization of care uses the basic practices of “rapid assessment, early treatment, frequent reassessment and aggressive titration to relief,” (Benjamin, 2008, P 7). However clinical results gotten are poor, with about 40% of patients progressing to total. Pulmonary insufficiency and pulmonary hypertension can occur. This is the process that makes SCD useful in certain cases of malaria, because the parasites are unable to attach to these red blood cells. Alpha thalassemia is most often due to a deletion of one or more of the two alpha globin alleles on chromosome 16, but over 20 additional genetic defects producing alpha thalassemia have been described. The protoporphyrin in heme is broken down into bilirubin, which the liver excretes into the gallbladder as bile. Because it prevents a protein that helps sickle cells move through narrow blood vessels, they are easier to move through. People who are older are more likely to develop complications such as chronic lung disease and painful leg ulcers as they age. Accessed from Pelehach, L. (1995). Understanding sickle cell disease. This decreases the number of crisis seen in patients but does not offer a permanent solution(Hilliard et al. 2018). Thus, replacement of urinary losses requires continuous intake of fluids in order to prevent RBC dehydration and its exacerbation of the sickling phenomenon. The part about sickle cell trait inheritance was interesting to read.
Gene therapy remains an achievable goal, and much progress has been made in recent years. Consult the Centre for Transplant at Kokilaben Dhirubhai Ambani Hospital for assistance on Bone Marrow Transplant. In the aplastic crisis, there is a temporary suppression of the RBC precursor cells in the marrow, usually by a viral infection. However, there are various techniques to reduce the severity of SCD or to manage the pain from SCD. The first stage is assessment where self-appraisal of attitude and readiness in determining the type of the SCD, nature of the pain and the assessment of its importance for the patient. Many of the SS mice were assumed to have pain while sleeping. Use of the medicine Hydroxyurea has been forwarded (HemOnc Today, 2010). Such haemoglobinopathies mainly thalassaemias and sickle-cell anaemia are globally widespread. This mutation makes it so that decreased oxygen levels are always traveling the body. These two examples of end-organ damage are related to extreme environmental stress. SCD pain was also described as complex and past, present and anticipated pain experiences has impacted on the individual’s’ response to life and pain events (Max et al, 2006). People from malaria-endemic regions (Africa, India) are at risk. Abnormal hemoglobin is stiff, sticky and distorts the red blood cell into a sickled shape. Sickle cell disease SCD is a potentially devastating condition that is caused by an autosomal recessive inherited hemoglobinopathy which results in the hallmark clinical sequelae of vaso- occlusive phenomena and hemolysis. Thus, the prevalence of sickle trait (Hb AS) increased in each successive generation. Sickle cell disease is a group of inherited red blood cell disorders. The patient reported malaise, fatigue, weakness that started 3 days before. This process is applicable to intermittent injections or oral administration of opioids but not to PCA. These blockages cause repeated episodes of severe pain, organ damage, serious infections, or even stroke. This review aims to provide an evidence based update on how to manage patients with this disease in the community. Routhieaux, J., S. Sarcone and K. Stegenga. (2005). Neurocognitive Sequelae of Sickle Cell Disease: Current Issues and Future Directions. Pulmonary insufficiency and pulmonary hypertension can occur. Various degrees of facial pain expression in children equivalent to this rating scale has been used in pediatric clinics. Vaso-Occlusive Crisis (VOC) is defined as pain resulting from tissue ischemia caused by vaso-. They become rigid or sticky and get stuck in narrow blood vessels, thereby reducing the blood flow or oxygen transport within the body. The body of an affected person does not have enough normal-functioning red blood cells to transport oxygen through the body tissues. Hyposthenuria is related to the high concentration of solute in the medulla of the kidney, which accentuates the sickling process locally and can damage the kidney concentrating mechanism. This results in hemolytic anemia and recurrent vaso-occlusion in the microvasculature due to increased red blood cell adhesion and retention. Since access to health care is seen as one of the main problems for SCD patients, it becomes important to identify ways to mitigate its most immediate or important effect on patient which is pain described as chronic, acute or both. However clinical results gotten are poor, with about 40% of patients progressing to total.
