Sickle Cell Anemia: The Effects of a Childs Illness on the Family - Coursework Example

Sickle Cell Anemia: The Effects of a Child’s Illness on the Family Introduction & Background Gulbis et al, 2005 p.309, describes sickle cell disease as “a genetic disorder involving hemoglobin designated as hemoglobin S, an autosomic recessive hereditary disease. This inherited disease causes the normally round and flexible red blood cells to become stiff and shaped like a sickle. The stiff sickle shaped blood cells no longer flow through blood vessels easily, hampering blood supply to the tissues. The resultant inadequate blood supply is responsible for the pain and respiratory difficulties experienced by patients suffering from sickle cell disease, and it is also responsible for organ damage in sickle cell disease. These sickle shaped blood cells die much faster than normal red blood cells, which lead to anemia (sickle cell anemia) (Nestor et al, 2004). 2. Course of the Sickle Cell Anemia Fetal hemoglobin is not involved in the polymerization of hemoglobin and through that lowers the sickling of the red blood cells. Fetal hemoglobin levels in the blood start to reduce around the age of six months. The onset of the initial signs and symptoms occur within five to six months of birth and persist right through the life of the individual. The course of the disease is not the same for all individuals. It can remain asymptomatic, particularly in the case of newborns or can lead to intermittent crisis events that require hospitalization. Infections can give rise to sudden drop in hemoglobin, which is termed as aplastic crisis. However, crisis episodes of pain are characteristic of sickle cell disease and are the consequence of vaso-occlusion due to the blocking of the blood vessels by the misshapen red blood cells. These crisis events common to sickle cell disease can be brought on by dehydration, exposure to cold, infection and the environment in which there is low oxygen content, like high altitudes. The nature of the crisis events can be acute or chronic or both. These crisis events recur and cannot be predicted. A serious issue with sickle cell disease is the ischemic organ damage that can occur from the vaso-occlusion. The severe consequences of ischemic organ damage include stroke, pulmonary infarction or acute chest syndrome, priapism or prolonged painful erection, splenic infarcts or changes in the functioning of the spleen that result in enhanced susceptibility to infections, and damage to the kidneys and liver. Such organ damage causes quite frequently other conditions linked to the disease, like delayed growth, delay in puberty, and reduced lung function (Creary, Williamson & Kulkarni, 2007). 3. Stressful Effects of the Child’s Condition The socio-ecological theory regarding children with chronic diseases posits that the diseased child is in the center of several concentric rings with the family being the most proximal concentric ring to the child in the community. The effects of the disease have the greatest impact on the proximal ring of the family (Gustafson et al, 2006). The African-American population bears a very large proportion of the burden of sickle cell anemia. (Tashiro, 2005). The African-American community have several stress related characteristics that include single parent structure, low socio-economic status, consisting of poverty, lack of employment opportunities, reduced access to healthcare systems, and stress filled lives in urban communities (Telfair & Gardner, 1999). Stress experienced by families need to perceived from the from the three levels of cognitive comprising of education in terms of the illness and illness care, emotional responses to the illness and behavioural requirements in the management of the illness. Lack of knowledge about the how the child has got the condition, the impact of the disease on the child, and the care provisions requirements are part of the cognitive factors that enhance stress in the families. Evidence from studies suggest that seeing the child in pain and not knowing what is causing it and how to offer pain alleviation is a major emotional stressor for mothers. Emotional stress is also experienced at the time of the diagnosis from fear of what the disease could do to the child and the ability to cope with the health care needs of the stricken child, particularly when the socio-economic status is low. Added to this is the guilt of the parents of carrying the trait and their ignorance that has led to the condition of the child. The unpredictable nature of the disease adds to the stress of the care givers (Radcliffe, Barakat & Boyd, 2006). 4. Nursing Care Discharge Planning Nursing professionals are positioned ideally to play a lead role in facilitating health promotion and health maintenance required to lower the high levels of morbidity and mortality that is associated with the disease (Tanya, 2003). During an acute episodic event associated with sickle cell disease the nursing care plan includes admitting the individual to a hospital unit and restricting the patient to bed; evaluation of whether a hematology consult is required; evaluation of the pain experienced by the child with the assistance of an appropriate pain scale and administration of the prescribed narcotic; evaluation of oxygen saturation using pulse oximetry and administering oxygen when the levels are below 95% or as prescribed; finding out whether the folic acid intake regimen has been followed at home and administering folic acid as prescribed ; and evaluation of the food intake and output and use of IV fluids as prescribed (Pillitteri, 2009). Discharge planning of a patient with sickle cell anemia after an episodic event has to be viewed from the perspectives of planning related to specific episodes of vaso-occlusive crises, infections and surgical procedures; outpatient visits for continual assessment and management of sickle cell disease; and the educational needs for home care of the disease. Discharge planning includes incorporation of the requirements related to the particular event that caused hospitalization and also the general needs of the disease (Bowden & Greenberg, 2009). Education of the patient and the family is a significant aspect of discharge planning and includes the topics for specific situations. For instance, according to Beyer and Simmons, 2004, “severe, recurrent, and unpredictable pain is a prominent feature of sickle cell disease (SCD), is the most distressing symptom of SCD”. Thus education of the patient must involve pain relief measures, which are not solely reliant of analgesics, but incorporates non-pharmacological pain relief measures at home (Beyer & Simmons, 2004). Ambulatory care planning involves the outpatient visits, which should be a minimum of twice yearly. The purpose of these out patient visits is to evaluate the physical and psych-social needs of the patient and the family (Bowden & Greenberg, 2009). Discharge planning for home care tries to make the life of the individual as near normal as possible. For instance, normal life for a child means attending school and taking part in activities like games. However, contact sports have to be avoided. Activities that involve severe exertion like long distance running must be avoided, as it can lead to dehydration. The patient has to consume a lot of water during summer to avoid dehydration. In essence dehydration, vigorous exercise, cold temperatures, and stress can trigger acute events and so must be avoided or minimised ((Pillitteri, 2009). 5. Consumer Groups and Their Utility The utility of social and consumer groups as support for patients with sickle cell disease and their families has to be evaluated from two perspectives. The first is their utility for the patients themselves. Telfair and Gardener, 1999, investigating the benefits received by adolescent African-Americans with sickle cell disease found that such social and consumer groups gave social and psychological services that provided the benefit of psychosocial well being to these patients. The social and consumer groups that aim to promote the well-being of patients with sickle cell disease promote knowledge on sickle cell disease and the use of folic acid to reduce the impact of the illness (Hunter-Grant, 2006). Support and consumer groups have proven to be useful for the family members of patients with sickle cell disease. While family members sought material and emotional support from within the family groupings, social and consumer groups are looked at as the means of improving awareness and knowledge on sickle cell disease. Furthermore, being a part of social and consumer groups assists the family members to reduce the stress experienced (Hill, 2003). Recommendation On diagnosis of sickle cell disease, the individual and the family must be provided with adequate knowledge, so that they are fully aware of the implications of the disease and what needs to be done to reduce the likelihood of acute episodes of the disease, making it easier on the patient and the family members to cope with disease. In addition, the patient and the family members must be made to realize the importance of out patient visits and the recognition of the onset of an acute episode for prompt attention. The patient and the family members must be advised to draw additional support from social and consumer groups for improving their state of mind and reducing the stress experienced as a result of the disease. Literary References Beyer. J. E. & Simmons, L. E. (2004). Home Treatment of Pain for Children and Adolescents With Sickle Cell Disease. Pain Management Nursing, 5(3), Retrieved March 22, 2010, from Medscape Today Web Site: http://www.medscape.com/viewarticle/490484 Bowden, V. R. & Greenberg, C. S. (2009). Children and Their Families: The Continuum of Care. Second Edition. Philadelphia: Lippincott, Williams & Wilkins. Creary, M., Williamson, D. & Kulkarni, R. (2007). Sickle Cell Disease: Current Activities, Public Health Implications and Future Directions. Journal of Women’s Health, 16(5), 575-582. Gustafson, K. E., Bonner, M. J., Hardy, K. K. & Thompson, Jr., R. J. (2006). Biopsychosocial and Developmental Issues in Sickle Cell Disease. In Robert, T. Brown (Ed.), Comprehensive Handbook of Childhood Cancer and Sickle Cell Disease: A Biopsychosocial Approach (pp.431-448), Oxford: Oxford University Press. Hunter-Grant, C. (2006). Sickle Cell Disease: Whats Going On? Insights For Women. In Catherine Fisher Collins (Ed.) African Americans Health and Social Issues. Second Edition (pp. 82-91), Westport, CT: Greenwood Publishing Group, Inc. Hill, S. A. (2003). Managing Sickle Cell Disease in Low-Income Families. Philadelphia, PA: Temple University Press. Nestor, W.E., Anderson, G.D., Roberts Jr., E.C., Pearsall, N.N., Nester, T.M. & Hurley, D. (2004). MICROBIOLOGY: A HUMAN PERSPECTIVE, Fourth Edition. Boston: McGraw Hill Higher Education. Pillitteri, A. (2009). Maternal and Child Health Nursing: Care of the Childbearing and Childrearing Family. Philadelphia: Lippincott, Williams & Wilkins. Radcliffe, J., Barakat, L. P. & Boyd, R. C. (2006). Family Systems Issues in Pediatric Sickle Cell Disease. In Robert, T. Brown (Ed.), Comprehensive Handbook of Childhood Cancer and Sickle Cell Disease: A Biopsychosocial Approach (pp.496-513), Oxford: Oxford University Press. Tanya, R. A. (2003). Sickle Cell Disease: Health Promotion and Maintenance and the Role of Primary Care Nurse Practitioners. Journal of the American Academy of Nurse Practitioners, 15(9), 389-397. Tashiro, C. J. (2005). The Meaning of Race in Health Care and Research - Part 1: The Impact of History. Pediatric Nursing, 31(3), 208-210. Telfair, J. & Gardener, M. M. (1999). African American Adolescents with Sickle Cell Disease: Support Groups and Psychological Well-Being. Journal of Black Psychology, 25(3), 378-390. Read More