SICKLE CELL DISEASE 1

Transcription

1 SICKLE CELL DISEASE 1 Your topic: My topic is about sickle cell disease, I need someone to write an introduction about the disease and the effects it has on the individual and family, also have to include valid and reliable references from journals, books and reliable sites. Can't be more than 7 years old. Also need to include how much money it cost to cover patients with sickle cell disease and what is the overall view of sickle cell how do the public view it. This essay must be 2000 words Your topic's description: In this essay there are five things you will need to cover. 1- Identity the health challenge.eg sickle cell. 2-look at the psychosocial as well as economic impact on society 3- professional roles of the nurse, what are you going to do in those facing the challenge in other words health promotion strategies 4- the skills you need will make you accountable to you linking to professional issues eg accountability, consent 5- take into consideration reference to gender, culture (patients dignity), social economic status. This is a nursing course so it has to relate to nursing Your desired style of citation: Harvard Referencing Your educational level: Guaranteed First Class Number of page: 8 Words: 2000

2 SICKLE CELL DISEASE 2 Sickle Cell Disease [Name of Customer] [Name of Institute] [Name of Supervisor] Introduction

3 SICKLE CELL DISEASE 3 The sickle cell anaemia or sickle cell is considered the most frequent hereditary syndrome discovered over 100 years ago, associated with significant morbidity and decreased life expectancy. The great clinical heterogeneity of sickle cell anaemia not explained by the mutation of the beta chain of haemoglobin and erythrocyte sickling appellant. Complex mechanisms wherein the intra and extra-vascular chronic haemolysis, endothelial nitric oxide depleted, release of cytokines and adhesion molecules, activation of coagulation factors interact, leading to continuous inflammatory state and contribute to the path physiology. There are genetic and environmental modifiers that cause the clinical phenotype. Thus complications arise differently, including cerebrovascular accident (CVA), painful vaso-occlusive crises, acute chest syndrome, musculoskeletal, priapism, retinopathy, organ damage (Adams et al, 2004; DeVaun, 2011; Mabaera et al, 2008). Sickle cell patient is at risk of exacerbations reviews to change its homeostasis. Surgical procedures are a challenge which must be prevented hypoxia, hypovolemia, hypothermia and hyper. This is achieved by strict control and monitoring of vital signs, prophylactic transfusions to decrease the proportion of HbS and improve tissue oxygenation. Efficient management decreases perioperative complications. Optimize gas exchange in the lungs, assure adequate tissue perfusion, fluid and balanced return of normothermia, knowing the extent of organ involvement to prevent complications (Chou et al, 2009). Management of Sickle Cell Disease Pain is the most characteristic and debilitating complication of sickle cell anaemia, although the high variability of this syndrome, some have a life with few pictures of pain or minimal manifestations. At the other extreme are patients suffering episodes of daily pain. The

4 SICKLE CELL DISEASE 4 mechanisms responsible for this complication are poorly understood (Chou et al, 2009). These episodes may occur in patients as young as 6 months old and in unpredictable throughout life intervals. Sometimes they are managed at home without contacting the doctor. If inadequately treated, the pain of vaso-occlusive crises can cause serious consequences, including trigger acute chest (Wright & Ahmedzai, 2010) syndrome. The overall management consists of rest, hydration and analgesia. Requires frequent systematic pain assessment and continuous adjustments of relief measures, especially analgesics. Intravenous hydration should be done generously and hypotonic solutions to avoid hyper-viscosity and sickling. A variety of analgesics can be used, since intravenous acetaminophen or NSAIDs, and opioids orally or parenterally. Each has its advantages and disadvantages. The hospital management has been based on opioid analgesia controlled by the patient, rather than on-demand treatment. Morphine can be started with 0.05 mg / kg every 4 hours on request, or by continuous infusion at 0.02 mg / kg / h, and then increased or adjusted for response, not to mention side effects like nausea, constipation, pruritus and hypoventilation. It should be always available antagonist Naloxone. Nubain is an alternative to morphine (Stinson & Naser, 2003). The dose of the opioid to achieve relief varies between episodes and between patients. Evaluate the amount of analgesics applied in relation to the improvement of pain and assist the child to adjust patient-controlled doses and achieve optimal control or change the regime in cases where it can be taken over in its management. The prices vary according to the type of medication, route, frequency and opioid requirements. For pain management in Sickle cell disease following measures can be taken:

5 SICKLE CELL DISEASE 5 Parenterally l/m2/day Hydration with 0.45 % solution alternating with 5% glucose solution; Analgesics - Ketoprofen: 5mg/kg/dosis every 8 hours; Dipyrone : 0.03 ml / kg / dose (parenterally); Morphine mg / kg / dose, slow intravenous every 4 hours, depending on the patient's condition and continuous monitoring of vital signs. Dilute 1 ampoule of 10mg in 10cc of saline solution to a concentration of 1mg/cc; If the pain improves, this can be managed on an outpatient basis with: Ibuprofen 10mg/kg/dosage every 6-8 hours; Acetaminophen : 15mg/kg/dosage every 4-6 hours; Oral hydration: 1,500 l/m2/day; If the painful crisis does not improve, the patient should be hospitalized For management of Management of splenic sequestration Immediate treatment in children with splenic sequestration is correct hypovolemia with leucorreducido globular concentrate transfusion. It is important to educate the mother, father and / or representatives to palpate the spleen and signs / symptoms of alert in order to make early splenic sequestration diagnosis; Consider joining UTI signs of cardiovascular compromise; Transfuse 5-10ml/Kg leukoreduced RBCs, given that there is a large volume of blood trapped in the spleen to be released and further increase the Hb / Hct; Control vital signs every 2 hours until the patient is stabilized, then every 4 hours

6 SICKLE CELL DISEASE 6 Functional asplenia begins during the first year of age, leading to an increased susceptibility to severe infections by encapsulated bacteria, such as pneumococcus, meningococcus and Haemophilus influenza. Infections and bacteremia are the major cause of morbidity and mortality in sickle cell children, being the justification for universal neonatal screening. Penicillin prophylaxis should be started at two months of age and treated aggressively each febrile episode with parenteral antibiotics and close monitoring. The vaccination is essential, with emphasis on vaccine: Haemophilus type B, 13V Pneumococcal, Meningococcal and 23-valent. Hospitalize all patients younger than 1 year, or any age with risk criteria : signs of systemic toxicity, or evidence of other complications (severe pain, aplastic crises, splenic sequestration, acute chest syndrome, hemiplegia, priapism) or after sepsis, fever 40 C, WBC> /mm3 or < 5000/mm3, splenectomized. Impact of Sickle Cell Disease In addition to the need for treatment and the fear of complications, sickle cell anaemia usually generates a feeling of being different, misfit and bad. Patients and their families may have post- traumatic stress syndrome, not necessarily related to the severity of their illness. The social, psychological support and therapeutic education should be done in all cases. Stress is a risk factor for the signs and symptoms of ER appear (stress as causal) factor, or (following stress) fester. Many people affected explained that stressful situations are usually related to the appearance of specific symptoms or worsening of these conditions (external stressors). In many cases, the concern of the patient before the onset of symptoms and the efforts to prevent them or conceal (internal stressors) and the responses of excessive attention or rejection of these

7 SICKLE CELL DISEASE 7 symptoms generated in the social and family environment (external stressors), often become a source of distress and even greater stress than the disease and increase the likelihood that the symptoms are manifested. The interaction of internal and external stressors, increases the vulnerability of the affected often forming a discouraging vicious circle. The hospitalization for pain crises represented the majority of the costs and the costs projected for the calculated differential between hydroxyurea and placebo, annual average U.S. $ 12,160 (95% CI: U.S. $ 49,440 to U.S. $ 414,880) for the year hydroxyurea and U.S. $ 17,290 (95 %: U.S. $ 13,010 to U.S. $ 21,570) for placebo. Prevention Strategies The cerebrovascular accident (CVA) is ischemic one of the most devastating complications in children affected by sickle cell anemia, can cause neurological damage and permanent sequelae. It occurs with an incidence of about 10 %, mainly in the HbSS and HbSβ such genotypes, with a peak at 4 years of age and high probability of recurrence (> 90 %). In addition, we describe to 35 % incidence of silent infarcts affecting neuro-cognitive function significantly (Verduzco & Nathan, 2009). In adolescents and young adults is no alteration in cerebral perfusion and neuro-cognitive impairment, with radiological evidence (MRI) cortical infarcts, atrophy and white matter lesions. It is thought that part of the progressive organ damage that occurs in this disease. Hemorrhagic strokes are more often at this stage. The path physiology differs between children and adults, is complex and not yet clearly elucidated. The most important variables are severe anemia, intravascular hemolytic and decreasing the bioavailability of nitric oxide, causing vasomotor changes in large and small cerebral arteries. Progressive atherosclerotic vascular disease develops with increased prothrombotic factors and endothelial

8 SICKLE CELL DISEASE 8 adhesion molecules and increased white blood cells and platelets that help in neurological pathology. Strategies to prevent and treat neurological complications have been extensively studied. The use of transcranial Doppler ultrasound as a tool for universal screening in all children with sickle cell anemia has been proven useful in the primary prevention of stroke. It helps us to identify individuals with a high probability to have a stroke, so as to establish a chronic transfusion regimen, decreasing significantly (> 90 %) the chance of this complication. It is important to know the neurological complications for early detection through the transcranial Doppler ultrasound or imaging studies or clinical setting, to establish prophylactic and / or therapeutic measures that can help these children to have a better quality of life and Directions adolescents become healthy and productive adults (Hankins et al, 2008). Early diagnosis through neonatal screening programs, early initiation of prophylaxis against infections, vaccination and parental education, are tools that have shown a decrease in mortality of children under 5 affected (Quinn et al, 2010). It is necessary for comprehensive care including genetic counselling, paediatricians, nurse practitioners, nutrition education, pain management, dentistry, by subspecialists in different areas to provide preventive clinical practice and improve the quality of life. The GP should be the paediatrician or haematologist (Kavanagh et al, 2011)). Folic acid 5mg daily; In patients with surgical splenectomy or with a history of invasive pneumococcal disease, consider continuing the long-term penicillin. If they are allergic to penicillin, erythromycin use (Thompson, 2011);

9 SICKLE CELL DISEASE 9 Avoid cold, dehydration and infection, as these promote sickling; Vaccination Schedule according to age, with emphasis on pneumococcal polysaccharide and conjugate vaccine, Haemophilus, hepatitis B, meningococcal; Take adequate amount of water or liquid daily (8-10 glasses / day); Do not smoke or drink alcohol; One must perform physical activity as tolerated, maintaining abundant moisture and avoid making physical activity under the sun in hours around noon. Do not exceed your limits and do extreme powers; Educate the patient and / or their parents in relation to their illness, complications, prevention, palpation of the spleen, danger if fever or greater pallor (Abo-Zenah et al, 2009) Nursing Accountability The role of nurses is highly important in management of all the diseases. They must have a responsible and ethical character which is accountable for all of their actions. Patients and their family members trust nurses and often have a relationship with nurses which play a pivotal role in the overall well being of the patients. For this relationship of trust it is necessary that the role of nurses should be transparent. Trust and respect are essential for achieving advances in health care. Based on shared global principles, the framework consensus places the patient as the highest priority, encourages research and innovation ethics ensures independence and ethical behaviour and promotes transparency and accountability. The nursing ethics and accountability should be based on the following principles: Position the patient as the highest priority;

10 SICKLE CELL DISEASE 10 Encourage research and innovation ethics; Ensure the independence and ethical behaviour; Promote transparency and accountability Scientific advances and progress in health depend largely on regular processes of information sharing and interaction between all partners. While individual codes of practice governing the activities, this broad consensus framework applies horizontally much of the healthcare community, and including multiple interactions involving patients, nurses, pharmacists, medical and healthcare industry. Essential to the agreed framework is transparency and accountability in nursing. By adhering to the above mentioned principles, nurses are committed to making open, transparent and informed decisions, which results a resounding responsibility for their actions. The provision of health care requires multiple interactions between patients, health professionals, pharmaceutical industry and other stakeholders. While the developed and developing economies struggling to address pressing health challenges in a complex healthcare environment and rapidly evolving, collaboration and trust between all partners is crucial. Health care requires certainty and confidence in what health professionals can do for their patients. Providing health care is a team challenge today, so that cooperation must be transparent, accountable and professional. However, health care and ethics are not enough. The consensus for ethical collaboration between patients, healthcare professionals and the pharmaceutical industry framework is available.

11 SICKLE CELL DISEASE 11 References Abo-Zenah, H., Moharram, M. & El Nahas, A.M. (2009). Cardiorenal risk prevalence in sickle cell hemoglobinopathy. Nephron Clin Pract, 112 (2):c98-c106. Adams, R.J., Brambilla, D.J., Granger, S., Gallagher, D., Vichinsky, E. & Abboud, M.R. (2004). Stroke and conversion to high risk in children screened with transcranial Doppler ultrasound during the STOP study. Blood, 103(10): Chou, R., Fanciullo, G.J., Fine, P.G., Adler, J.A., Balantyne, J.C. & Davies, P. (2009). Clinical guidelines for the use in chronic non-cancer pain. J Pain,10(2): DeVaun, M. (2011). Secondary prevention of overt strokes in sickle cell disease: therapeutic strategies and efficacy. Hematology Am Soc Hematol Educ Program, 2011: Hankins, J.S., Fortner, G.L., McCarville, M.B., Smeltzer, M.P., Wang, W.C. & Li, C. S. (2008). The natural history of conditional transcranial Doppler flow velocities in children with sickle cell anemia. Br J Haematol, 142(1): Kavanagh, P.L., Sprinz, P.G., Vinci, S.R., Bauchner, H. & Wang, C. J. (2011). Management of children with sickle cell disease: a comprehensive review of the literature. Pediatrics, 128 (6): Mabaera, R., West, R.J. & Conine, S.J. (2008). A cell stress signaling model of fetal hemoglobin induction: what does not kill red blood cells may make them stronger. Exp Hematol, 36: Quinn, C.T., Rogers, Z.R., McCavit, T.L. & Buchanan, G.R. (2010). Improved survival of Children and adolescents with sickle cell disease. Blood, 115(17): Stinson, J. & Naser, B. (2003). Pain management in children with sickle cell disease. Paediatr Drugs, 5(4): Thompson, A. A. (2011). Primary prophylaxis in sickle cell disease: is it feasible? Is it effective? Hematology Am Soc Hematol Educ Program 2011Rev, Verduzco, L.A. & Nathan, D.G. (2009). Sickle cell disease and stroke. Blood, 114(25): Wright, J. & Ahmedzai, S. (2010). The management of painful crisis in sickle cell disease. Curr

12 SICKLE CELL DISEASE 12 Opin Supp Palliat Care; 4(2):