护理学Essay参考案例：Analysis of Sickle Cell Disease

本文是护理学专业的留学生Essay范例，题目是“Analysis of Sickle Cell Disease（镰状细胞病分析）”,随着来自发展中国家的人把加拿大当成自己的家，加拿大正变得越来越多元文化。随着加拿大移民率的上升，以前不常见的疾病变得越来越普遍。例如，在加拿大所有地区，镰状细胞病等疾病正在增加(被忽视的情况，2014年)。镰状细胞病是一种遗传性疾病，会导致不良后果，降低个人的生活质量。本文的目的是分析镰状细胞病的病理生理，并研究基于证据的实践，如管理和预防措施。首先，将简要解释所使用的搜索策略，然后详细介绍镰状细胞病的流行病学。此外，本文将按以下顺序讨论疾病的各个方面:危险因素、临床表现、诊断检查、并发症、循证治疗和不同程度的预防措施。根据rao的说法，“知识是专业实践的基础，是专业精神的一个中心方面”(护理专业精神，第28页)。显然，护士需要批判性地分析疾病，如镰状细胞病，以便应用和提供称职的护理。

Safiullah Barat & Melissa Parker萨菲乌拉·巴拉特和梅丽莎·帕克

Canada is becoming more multicultural as individuals from developing countries are making Canada their home. As the rate of immigrants in Canada is rising, diseases that were uncommon are becoming more prevalent. For instance, disorders such as sickle cell disease are increasing in all regions in Canada (Neglected Conditions, 2014). Sickle cell disease is genetic disorder that causes undesired effects which decreases an individual’s quality of life. The purpose of this paper is to analyze the pathophysiology of sickle cell disease, and to research evidence based practice such as management and prevention measures. To begin, a brief explanation of the search strategy used will be discussed, followed by details on the epidemiology of sickle cell disease. Furthermore, aspects of the disorder will be discussed in the following order: risk factors, clinical manifestations, diagnostic tests, complications, evidence based treatment, and different levels of preventive measures. According to RNAO, “knowledge provides the basis for professional practice and, is a central aspect of professionalism” (Professionalism in nursing, pg. 28). It is evident that nurses need to critically analyze disorders, such as sickle cell disease, in order to apply and provide competent care.

Search Strategy搜索策略

To help retrieve the most up to date, recent and peer reviewed articles, different search strategies have been used. Nursing databases such as ProQuest, CINHAL and MEDLINE were used to find the appropriate journal articles. After doing so, search limiters were used to narrow down the search. Peer reviewed, full text and published dates set from 2009 to 2014 were selected, as well as the availability of references was included. Boolean operators, “and” and “or”, were commonly used ; furthermore, search term “sickle cell disease” and Boolean phrases including “complications”, “treatment”, “diagnostic test”, “pediatrics”, “prevention”, “etiology”, and “Canada” were combined to narrow the search.

为了帮助检索最新的，最近的和同行评审的文章，不同的搜索策略已经被使用。使用ProQuest、CINHAL和MEDLINE等护理数据库查找相应的期刊文章。在这样做之后，搜索限制被用来缩小搜索范围。同行评议，全文和出版日期设定在2009年至2014年，并包括参考文献的可用性。布尔运算符“和”和“或”是常用的;此外，搜索词“镰状细胞病”和布尔短语(包括“并发症”、“治疗”、“诊断测试”、“儿科”、“预防”、“病因学”和“加拿大”)被合并以缩小搜索范围。

Epidemiology流行病学

Sickle cell disease is prevalent in areas where malaria is common. This includes area such as the Caribbean, Nigeria, Middle East, Mediterranean, Indian sub-continent, Greek, Turkey, India, Pakistan, Ghana, and Far East China (Brown, M. 2012). A study conducted by Lanzkron et al (2013) took a look at mortality rates with individuals who had sickle cell disease over a time frame of thirty years. Over the course of thirty years, 16, 654 sickle cell-related deaths had occurred with a mortality rate of 0.7% each year (Lanzkron et al, 2013). In Canada, one in every 2500 babies will be born with sickle cell disease, and have a one in four chance (25%) of having sickle cell disease. Furthermore, they have a one in two chance (50%) of being a carrier for sickle cell disease (Sickle Cell Disease Association of Canada, 2013).

镰状细胞病在疟疾流行的地区流行。这包括加勒比地区、尼日利亚、中东、地中海、印度次大陆、希腊、土耳其、印度、巴基斯坦、加纳和中国远东地区(Brown, M. 2012)。Lanzkron等人(2013年)进行的一项研究考察了30年来镰状细胞病患者的死亡率。在30年的时间里，发生了16654例镰状细胞相关死亡，每年的死亡率为0.7% (Lanzkron et al, 2013)。在加拿大，每2500个婴儿中就有一个患有镰状细胞病，并且有四分之一的机会(25%)患有镰状细胞病。此外，他们有二分之一(50%)的机会携带镰状细胞病(加拿大镰状细胞病协会，2013年)。

Etiology/Risk Factors

Sickle cell disease is an inherited autosomal recessive condition that causes an abnormal formation of hemoglobin. Different types of sickle cell disease includes sickle cell anemia (HbSS), sickle hemoglobin-c disease (HbSC), and sickle cell thalassemia (HbS) ( Brown, M. 2012).However, the most common is sickle cell anemia (Lewis, 2014). This inheritance occurs when both the mother and father pass on the defective gene to their child (Brown, M. 2012). Therefore, being a carrier of the sickle cell trail is a major risk factor. Individuals have an increased risk of developing sickle cell disease if residing in areas where malaria is endemic. Sickle cell disease puts individuals at risk whose ancestors came from West Africa, southern Italy, northern Greece, South and Central America, Middle East, Central India, southeast coast of Turkey and Mediterranean in Sicily (Pack-Mabian, A & Haynes, J.r. 2009).

Clinical Manifestations临床表现

Although, each individual will display different signs and symptoms of sickle cell disease, they all display one similar characteristic: when exposed to factors that decrease oxygen, the hemoglobin forms into a sickle-shaped red blood cell which blocks the flow of blood. As a result, clinical manifestations often show anemia, jaundice and severe pain (Addis, G. 2010). Often, patients are asymptomatic except when experiencing a sickling episode (Lewis, 2014).

虽然每个人会表现出不同的镰状细胞病的症状和体征，但它们都有一个相似的特征:当血红蛋白暴露在降低氧气的因素中，血红蛋白会形成一个镰刀状的红细胞，阻止血液流动。因此临床表现常表现为贫血、黄疸和剧烈疼痛(Addis, G. 2010)。通常，患者无症状，除非经历镰状发作(Lewis, 2014)。

An individual with sickle cell disease may develop anemia due to the complete destruction of red blood cells or hemolysis (Addis, G. 2010). Normally, red blood cells live for 120 days in our body but sickle cells usually die within ten to twenty days (Addis, G. 2010). As a result, the body’s organs are not receiving enough oxygen. The body compensates as it increase heart rate, increase blood pressure to allow the oxygenated hemoglobin to reach the necessary organs. Furthermore, individuals will display signs and symptoms of SNS or fight or flight response. Increase pupil dilation, diaphoresis and tachypnea are evident in patients with sickle cell crisis (Tortora & Derrickson, 2012).

In addition, the constant breakdown of hemoglobin produces bilirubin, a byproduct of hemoglobin, which cannot be processed by the liver. As a result, the bilirubin gets stored in the blood and connective tissue which results in yellowing of the eyes and the skin (Addis, G. 2010).

However, the most common manifestation individuals with sickle cell disease experience are pain as a result of vaso-occlusive crises, also known as sickle cell crisis. Pain related to sickle cell disease accounts for ninety percent of hospital admissions (Musumadi, L. et al. 2012). This is mainly due to episodes of sickling that prevents oxygenated blood reaching organs, which results in ischemia and gradual deterioration of tissue and organ function (Musumadi, L. et al., 2012).

Diagnostic Tests诊断测试

Diagnostic tests to diagnose sickle cell disease involve blood work. Often, individuals who have sickle cell disease are best diagnosed with the use of a peripheral blood smear test which reveals sickle cells (Lewis, 2014). In addition, sickle hemoglobin tests involve taking red blood cells, and expose them to factors that deoxygenate the blood, and determines if there is hemolysis (Lewis, 2014). Furthermore, a test known as the hemoglobin electrophoresis helps to differentiate sickle cell trait between sickle cell disease. It works by identifying various types of hemoglobin within a blood specimen to confirm the diagnosis of sickle cell disease (Randolph & Wheelhouse, 2012).

镰状细胞病的诊断检查包括血检。通常，镰状细胞病患者最好的诊断方法是使用外周血涂片检查，发现镰状细胞(Lewis, 2014)。此外，镰状血红蛋白检测包括采集红细胞，将其暴露于使血液缺氧的因素中，并确定是否有溶血(Lewis, 2014)。此外，一种被称为血红蛋白电泳的测试有助于区分镰状细胞病和镰状细胞病的特征。它通过识别血液标本中的各种类型的血红蛋白来确认镰状细胞病的诊断(Randolph & Wheelhouse, 2012)。

Additionally, secondary diagnostic tests could be used to diagnose complications that arise with sickle cell disease. Individuals may require a chest x-ray, skeletal x-ray, magnetic resonance imaging (MRI), and a Doppler ultrasound (Lewis, 2014). Skeletal x-rays are used to determine bone and joint deformities whereas chest x-rays are used to diagnose chest infection (pneumonia). MRIs are used to aid in the diagnosis of a stroke caused by blocked blood vessels from sickled cells Likewise, a Doppler ultrasound may be used to diagnose deep vein thrombosis (DVT) (Lewis, 2014).

Course of the disease and complications病程及并发症

Sickle cell disease causes a wide range of complications that begin at infancy and worsen with age when not controlled. Complications develop when sickling episodes causes vaso-occlusion which leads infarction of body tissues and organs (Lewis et al., 2010), and with increasing age, causes end-organ complications (Miller & Meier, 2012). At age 2, children begin to experience dactylitis, pain in small bones of hands and feet (Miller & Meier, 2012). Vaso-occlusive pain involving the back, chest, abdomen, or extremities continues to occur throughout the lifespan of an individual with sickle-cell disease (Pack-Mabien, 2009). Furthermore, both pediatrics and adults encounter the difficulty of anemia as it leads to other complications such as bone marrow suppression, renal insufficiency, and splenic or hepatic sequestration (Pack-Mabien, 2009). The infarction of the spleen can begin in infancy and causes another major complication: infection. The dysfunction of the spleen and its inability to phagocytize foreign objects can cause major infection in both children and adults “and is the leading cause of morbidity and mortality in patients with sickle cell disease” (Miller & Meier, 2012). Additionally, sickling episodes can affect the pulmonary system in all affected age groups and causes acute chest syndrome, a disorder that includes pulmonary complications such as pneumonia, fat embolism, systemic infection, pulmonary infarction, and if not treated, can lead to respiratory failure (Miller & Andrew, 2012). Unfortunately, children and adults are also at risk for stroke due to cerebral infarction (Miller & Meier, 2012). Other complications include gallstones, kidney failure, priapism or involuntary erection, delayed sexual development, delayed growth, bone necrosis, and leg ulcers (Brown, M. 2012).

镰状细胞病引起各种并发症，这些并发症始于婴儿时期，如果不加以控制，随年龄增长而恶化。当镰状镰状发作导致血管阻塞导致机体组织和器官梗死时，就会出现并发症(Lewis等，2010年)，并且随着年龄的增长，会导致末端器官并发症(Miller & Meier, 2012年)。在2岁时，儿童开始经历指关节炎，手和脚的小骨头疼痛(Miller & Meier, 2012)。涉及背部、胸部、腹部或四肢的血管闭塞性疼痛在镰状细胞病患者的整个生命周期中继续发生(Pack-Mabien, 2009)。此外，儿科和成人都面临贫血的困难，因为它会导致其他并发症，如骨髓抑制、肾功能不全和脾或肝隔离(Pack-Mabien, 2009)。婴儿期可发生脾梗塞，并引起另一主要并发症:感染。脾脏功能障碍及其无法吞噬异物，可引起儿童和成人的重大感染，“是镰状细胞病患者发病和死亡的主要原因”(Miller & Meier, 2012)。此外，镰状镰刀发作可影响所有受影响年龄组的肺部系统，并导致急性胸部综合征，这种疾病包括肺部并发症，如肺炎、脂肪栓塞、全身感染、肺梗死，如果不治疗，可导致呼吸衰竭(Miller & Andrew, 2012)。不幸的是，由于脑梗死，儿童和成人也有中风的风险(Miller & Meier, 2012)。其他并发症包括胆结石、肾衰竭、阴茎勃起或不自主勃起、性发育迟缓、生长迟缓、骨坏死和腿部溃疡(Brown, M. 2012)。

Treatments

The treatment of sickle cell disease involves the reduction of symptoms and complications. Treatment includes pain management involving opioid or non-opioid analgesics, anti-inflammatory drugs, and NSAIDS (Addis, 2010). Also, cognitive behavioural therapy and non-pharmacological approaches can benefit patients with chronic or acute pain (Addis, 2010). Another treatment includes RBC transfusion and is required “as an emergency measure or to prevent short or long-term complications.” (Addis, 2010). Furthermore, a medication called hydroxyurea is a major advancement in the management of sickle cell disease and is available in Canada (Canadian Association of Sickle cell…). Hydroxyurea increases the level of fetal hemoglobin that results in an overall decrease in circulating sickle cells (Smith et al., 2011). A clinical study conducted by Smith et al (2011), has proven a decrease in pain intensity in patients undergoing hydroxyurea therapy, as well as a decrease in analgesic use and a significantly lower reduction in crisis and mortality. Moreover, multiple studies have shown bone marrow transplant as a potential cure of sickle cell disease; however, further studies are needed for this treatment to be recommended as a standard treatment for sickle cell disease (Thompson, 2012).

Preventive Measures

Primary Preventive Measures

Although genetic risk factors are non-modifiable, there is a preventive measure for sickle cell disease: genetic counselling. Genetic counselling should be encouraged in patients with the sickle cell trait especially when planning to have a child. This preventive measure helps patients understand and adapt to the implications of genetic contributions to the disease and offers “counselling to promote informed choices and adaptation to the risk or condition” (Lewis, 2014. p. 787). A 6-year study in Saudi Arabia, a country of high prevalence of sickle cell disease, showed a significant decrease in the genetic disease through the use a genetic counselling program as it decreased the number of at-risk marriages (Memish, 2011).

Secondary Preventive Measures二级预防措施

Prevention measures in the secondary level focuses on diagnostic tests and screening for sickle cell disease. Newborn screening is the earliest way to detect whether the child has sickle cell disease in time to prevent serious complications from occurring (Newborn Screening Ontario, 2013). According to the Newborn Screening Ontario (2013), newborn screening can prevent “infection and sepsis, growth delay, painful sickle crisis, tissue ischemia and organ damage.”

二级预防措施侧重于镰状细胞病的诊断检测和筛查。新生儿筛查是及时发现儿童是否患有镰状细胞病以防止发生严重并发症的最早方法(新生儿筛查安大略省，2013年)。根据安大略省新生儿筛查(2013年)，新生儿筛查可以预防“感染和败血症、生长迟缓、痛苦的镰状危机、组织缺血和器官损伤”。

Secondary preventive measures also involve previously stated diagnostic testing for complications such MRI for stroke, the use of x-rays for chest infections, etc. Also, pain is a major complication in both adults and pediatrics and should be diagnosed; however, it is challenging to detect pain in unresponsive clients such as neonates. Registered Nurses Association of Ontario recommends the use of a validated pain assessment tool for neonates called Neonatal Infant Pain Scale (NIPS) due its evidence of reliability and validity. This tool includes components such as facial express, cry, breathing patterns, arms, legs, and state of arousal component (Assessment and Management of Pain, 2013).

Tertiary Preventive Measures

Tertiary preventive measures involve treatments that aid in regaining patient’s functional ability and the elimination of the disease. For individuals with sickle cell disease, preventive measures at this level include medical interventions to prevent and control symptoms and complications. For example, interventions are pain management, hydroxyurea, and transfusion therapies as mentioned earlier. Furthermore, penicillin can be given to children starting at 2 months of age, and vaccinations against pneumococcal infections, flu, meningitis, and hepatitis are important to prevent infections and early death (Addis, 2010).

Conclusion结论

In conclusion, sickle cell disease is one that affects the quality of life of affected individuals. The analysis focused on different aspects of sickle cell disease such as the epidemiology in which prevalence is high in areas where Malaria is predominant. Also, due to increasing immigration, the incidence of the disease is increasing in Canada. Furthermore, known risk factors have been identified such as genetic and environmental factors. In addition, the main clinical symptoms with sickle cell disease include pain as well as symptoms of anemia. Furthermore, a variety of tests which include blood work are the determinants of the presence of sickle cell disease. Sickle cell disease potentiates of a variety of complications, most commonly vaso-occlusive pain, acute chest syndrome, anemia and other major organ complications. Unfortunately, treatment only aids in controlling the complications and does not provide a cure for the disease. It is evident that the need for further research in stem cell transplant as a potential cure is highly needed. Lastly, genetic counseling, neonatal screening, diagnostic tests, and current evidence based treatment such as pain management, hydroxyuria and transfusion therapy, are all preventive measures of symptoms and complications of the disease. The findings in this analysis are significant for nurses to apply when having encountered a patient with sickle cell disease. Certainly, it is important for nurses to acknowledge the increasing diversity in Canada and to continue competency by constantly seeking new pertinent information to apply to everyday practice.

总之，镰状细胞病是一种影响患者生活质量的疾病。分析侧重于镰状细胞病的不同方面，例如在疟疾占主导地位的地区流行率很高的流行病学。此外，由于移民的增加，加拿大的发病率也在增加。此外，已知的风险因素已被确定，如遗传和环境因素。此外，镰状细胞病的主要临床症状包括疼痛和贫血症状。此外，包括血检在内的各种检查是镰状细胞病存在的决定因素。镰状细胞病可加重多种并发症，最常见的是血管闭塞性疼痛、急性胸综合征、贫血和其他主要器官并发症。不幸的是，治疗只能帮助控制并发症，而不能治愈这种疾病。显然，需要进一步研究干细胞移植作为潜在的治疗是非常必要的。最后，遗传咨询、新生儿筛查、诊断检测和目前基于证据的治疗，如疼痛管理、羟基尿和输血治疗，都是预防该病症状和并发症的措施。这项分析的结果对于护士在遇到镰状细胞病患者时应用具有重要意义。当然，对护士来说，认识到加拿大日益增加的多样性，并通过不断寻求新的相关信息来应用于日常实践，从而继续胜任工作是很重要的。

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