Patho-Case Study of Sickle Cell Paper

Patho-Case Study of Sickle Cell Paper

Pathophysiology and Symptoms of Sickle Cell Disease

Sickle cell disease results from the hemoglobin molecule, beta-globin mutation. Polymerization of mutant hemoglobin S takes place when a single amino acid is substituted in the β-globin chain. This causes the impairment of the erythrocyte survival and rheology. Sickle cell disease causes abnormalities observed through the development of microvascular vaso-occlusion cycles and hemolytic anemia (Sundd, Gladwin & Novelli, 2019). This eventually causes infarction and injury of the end-organ ischemia-reperfusion. Redox instability and inflammations are also caused by intravascular hemolysis and vaso-occlusive events which consequently influence the occurrence of progressive small- and large-vessel vasculopathy. The symptoms of this disease include pain crisis, anemia and fatigue, leg ulcers, dactylitis and arthritis, liver congestion and sudden pooling of blood, heart and lung injury and bacterial infections among others. Patho-Case Study of Sickle Cell Paper

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Diagnostic Texting

Sickle cell anemia is diagnosed using special blood tests that check for hemoglobin S. The tests employed for screening of sickle cell disease in the contemporary society include deoxyribonucleic acid (DNA) testing, high performance liquid chromatography (HPLC) texting and hemoglobin electrophoresis testing. These are routine tests for screening of newborns even though adults and children can also be tested. The SickleDex test is considered inappropriate for screening of hemoglobin due to its inability to distinguish sickle cell disease from sickle cell traits as well as detection of hemoglobinopathy. Additional tests include blood tests that detect the low red blood cell count and disease complications. Differential diagnoses include Septic arthritis, gout, avascular necrosis, connective tissue diseases, acute abdomen, perthes disease, trauma and osteomyelitis.

Evidence-based Treatment Modalities

The treatment for sickle cell disease involves bone marrow transplant. This is the only evidence-based potential treatment that guarantees complete cure of the patient. This intervention is applicable in individuals of 18 years and below since it increases risks in the older population (Hoppe & Neumayr, 2019). However, it is limited by the availability of donors which makes it increasingly difficult to achieve. On the other hand, the operation has presents heightened risks that include loss of lives. As a result, treatment modalities focus on symptom management and prevention of complications. Notably, pharmacological approaches available for sickle cell disease patients include antibiotics such as penicillin, pain killers and hydroxyurea (Totten et al.,, 2019). Prevention through vaccination is also crucial for children as well as blood transfusions that increase the number of blood cells in circulation. Patho-Case Study of Sickle Cell Paper

The information in this case study is relevant in nursing practice. For instance, it informs practitioners on the pathophysiology of sickle cell disease and diagnostic testing which promotes accurate screening and formulation of appropriate treatment plans. Moreover, it informs nurses of other disorder with closely related symptoms that helps separate them through differential diagnosis. Additionally, the case provides evidence-based treatment modalities which guide the nurse in management of the sickle cell disease both in adults and children.

Education sessions for patients with sickle cell anemia are significant in enhancing acceptance and initiating management and self-care. A master’s prepared nurse can use the knowledge acquired in this case to select the unique needs of individual patients and be able to customize the education strategies according to those needs. The knowledge also guides the nurse on the effective approaches to employ to patients depending on their age and the appropriate treatment considering their literacy levels and the complication levels of the infection.

The most important information presented in this case is the pathophysiology part of sickle cell disease. This makes up the foundation of diagnosis, treatment and prevention of the illness. This section presents the changes that occurs in the patient’s body and therefore, is essential in conducting of targeted clinical trials to create effective medication for treatment of the underlying effects rather than the physical symptoms. It also helps in understanding the cause of the symptoms manifested by the client and suggests the potential underlying impacts. Furthermore, this information is important in the formulation of diagnostic and treatment approaches. Therefore, it presents a basic and significant part of management of sickle cell disease.

The most confusing or challenging information presented in this case is the treatment section. The availability of limited treatment approaches for this infection challenges the nursing practice. Bone marrow is presented as the only effective method of treatment of sickle cell patient. However, the associated risks are fatal and the intervention is only applicable in patients of 16 years of age. As such, further research is essential in developing more modalities of treatment since sickle cell disease prevalence is increasing with time. It will also help in getting rid of confusions associated with the best approaches necessary for treatment. Patho-Case Study of Sickle Cell Paper

Sickle cell disease is associated with adverse patient safety issue including the severe complications. Patients experience delayed growth, secondary bacterial infections, pain crisis and pain episodes. The treatment also increases safety concerns since the disease causes pulmonary hypertension, gallstones, lung crises and leg ulcers as well as eye damage that do not heal. More so, blood transfusions increase the risk of contracting other infections such as HIV and AIDS in the event of inaccurate screening. Therefore, regular medical care and early diagnosis prevents the occurrence of complications, facilitates prevention and supports management of the symptoms associated with the illness.

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References

Hoppe, C., & Neumayr, L. (2019). Sickle Cell Disease: Monitoring, Current Treatment, and Therapeutics Under Development. Hematology/Oncology Clinics.

Sundd, P., Gladwin, M. T., & Novelli, E. M. (2019). Pathophysiology of sickle cell disease. Annual Review of Pathology: Mechanisms of Disease14, 263-292.

Totten A. M., Hansen R. N., Wagner J., Stillman L., Ivlev I., Davis-O’Reilly C., Towle C, Erickson J. M., Erten-Lyons D., Fu R, Fann J., Babigumira J. B., Palm-Cruz KJ, Avery M., McDonagh M.S. (2019).Telehealth for Acute and Chronic Care Consultations. Comparative Effectiveness Review No. 216. Retrieved from  https://doi.org/10.23970/AHRQEPCCER216  on May 9, 2019

Patho-Case Study of Sickle Cell Paper

 

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