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Sickle Cell

Sickle Cell

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Etiology and Pathology

Sickle cell anemia/disease is a genetic blood disorder that is inherited from parents. It is caused by a defective cell called hemoglobin S which changes in shape from the normal disc shape to a sickle shape. The blood cells in healthy humans are always shaped like discs but when one has sickle cell disease, the cells bend into shapes that resemble sickles or crescent moon. The defective cells control the production of hemoglobin, the protein in blood responsible for carrying oxygen around the body. When there is a defect in the gene that produces hemoglobin, the hemoglobin S forms clumps in the red cells thus becoming sickle shape and blocks the blood vessel. This makes the red blood cells to deliver less oxygen consequently interrupting the flow of blood in the body. The disease is inherited by children from two carrier parents. If one parent has sickle cell, the child will only have one gene or sickle cell trait. This means that the child is only a carrier and does not have the disease. However, the child could still pass the gene to their children (Bryson et al., 2006).

Population Affected

There are certain populations that are more at risk of the sickle cell disease than others. These populations are of Middle Eastern, Asian, Caribbean, Southern Europe, African, Indian, and Pakistani origin. For instance, the Center for Disease Control (CDC) estimates that approximately 100, 000 Americans are living with the disease. A great percentage of these are blacks or African Americans. The statistics show that one out of every 500 African Americas has sickle cell. However, millions of people are affected throughout the world (CDC).


Symptoms

Patients with sickle cell anemia battle with lifelong problems which range from mild to severe symptoms which might require hospitalization. Some of the symptoms include fatigue, paleness, gallbladder infection, confusion, paleness, leg ulcers, painful joints, bone infection, breathing problems, jaundice, urinary tract infection, skin ulcers, eye problems or blindness, delayed growth, acute chest syndrome, anemia, stroke, and organ damage. The painful episodes are called crises (National Heart, Lung, and Blood Institute, 2008).

Co-morbidities/ Complications

There are several complications associated with sickle cell anemia including vaso-occlusion complications, hemolytic complications, and infectious complications. Vaso-occlusion complications include acute chest syndrome, liver disease, pulmonary hypertension, central nervous system disease which manifests as stroke, priapism, leg ulcers, retinopathy, osteonecrosis, renal effects/insufficiency, bacterium, and avascular necrosis. Infectious complications include E. coli sepsis and osteomyelitis. Finally, hemolytic complications include anemia and aplastic crisis (Neville & Panepinto, 2011).

Diagnostic Testing

Diagnostic testing of sickle cell is done by a simple blood test for both adults and children. In the United States, this test is a must for newborns before they are released from hospitals. The most common blood test is referred to as hemoglobin electrophoresis. The test measures the different types of hemoglobin in the blood in order to identify any abnormality that might be there. For those who have joint or bone abnormalities, a skeletal X-ray can be conducted (Rausch & Pollard, 1998).

Medications/Treatment

Currently, sickle cell has no cure but there are treatments available to provide relief from the painful crises, and precautions that patients can take to reduce their complications. The precautions include eating a proper diet, avoiding drugs and alcohol, and taking plenty of fluids. On the other hand, treatment options vary depending on the severity of the symptoms. The treatment options include taking folic supplements to increase red blood cells, taking pain medications or analgesics, hydration, oxygen therapy and blood transfusion. Hydroxyurea and bone marrow transplant can be done in case of severe complications (Steinberg, 2003).

Impact on Quality of Life and Implications for Family and Society

Sickle cell has a widespread impact not only on the patient but on family and society as a whole. Patients with sickle cell disease, especially the young ones experience ongoing stress which affects all aspects of their life. For instance students with the disease may perform poorly in their school work because they miss school more than their peers. Generally, there is lack of support in schools to ensure these students can catch up with their school work. Poor academic performance might impact negatively on their future employment prospects. Secondly, there is a general lack of understanding of the disease in the society leading to stigmatization and even discrimination considering that the disease mainly affect the black community. Children with sickle cell disease often end up having emotional and behavioral problems due to maladjustment. When it comes to families, they often feel burdened with the increase care demands and stress that come with the painful crises. Sometimes, caregivers miss work just to take care of the patient. It is important to have a support network within the community because lack of support can lead to feelings of frustrations and hopelessness for both the patient and care givers (Welcom, 2012).

Necessary Teaching and Nursing Interventions

Education is necessary for both the patient and their families or care givers. The teaching should include preventions and treatment strategies, coping skills, how to avoid infections or painful crises, what to avoid and the appropriate nutrition, when to seek treatment, and the regulation of patient’s medication. Nurses’ intervention is required with an aim of minimizing complications of the disease. The interventions include providing empathy and working with the patient as the expert, comprehensive assessment of patient’s needs, conduct physical assessment, ensure the patient and their care giver understand their condition, work with the patient to manage the painful crises, develop an effective self-management plan with the caregiver, and to address the social and psychological impact of their condition (Royal College of Nursing, 2011). Concept Map

References

Bryson., B.J., Byerly, K., Coolen, P., Mullin, K., and Niebrugge, D., et al. (2006). “Sickle cell disease: Critical elements of care.” The Center for Children with Special Health Needs Children’s Hospital and Regional Medical Center, Seattle, WA. CDC. (n.d). What you should know about sickle cell. Retrieved on July 25, 2013, http://www.cdc.gov/ncbddd/sicklecell/documents/scd-factsheet_what-is-scd.pdf

National Heart, Lung, and Blood Institute, (2008). Sickle cell anemia. Diseases and Conditions Index. Retrieved July 25, 2013. http://www.nhlbi.nih.gov/health/dci/Disease/Sca/SCA_WhoIsAtRisk.htlm

Neville, K.A. and Panepinto, J.A. (2011). Pharmacotherapy of sickle cell disease. Retrieved July 25, 2013 http://www.who.int/selection_medicines/committees/expert/18/applications/Sicklecell.pdf

Rausch M. and Pollard D. (1998). Management of the patient with sickle cell disease. Journal of Intravenous Nursing, 21(1): 27-39.

Royal College of Nursing (2011). Caring for people with sickle cell disease and thalassaemia syndromes: A framework for nursing staff. Retrieved July 25, 2013 http://www.rcn.org.uk/__data/assets/pdf_file/0004/372991/003874.pdf

Steinberg, M.H. (2003). Management of sickle cell disease. The New England Journal of Medicine, 340(13): 1021-1030 Welcom, J.S. (2012).

The impact of sickle cell disease on the family: An examination of the illness intrusive framework. Psychology Dissertation, Paper 91. Retrieved July 25, 2013 http://digitalarchive.gsu.edu/cgi/viewcontent.cgi?article=1094&context=psych_diss


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