Analysis of Sickle Cell Disease

Analysis of reap hook Cell DiseaseSafiullah Barat Melissa ParkerCanada is becoming to a greater extent multicultural as unmarrieds from developing countries argon making Canada their home. As the drift of immigrants in Canada is rising, infirmitys that were uncommon are becoming more prevalent. For instance, disorders much(prenominal) as reap hook electric carrelphoneular telephoneular telephoneular phone affection are increasing in exclusively regions in Canada (Neglected Conditions, 2014). reap hook carrell illness is hereditary disorder that births unsought effects which step-downs an individuals quality of vitality. The purpose of this paper is to psycho dissect the pathophysiology of reap hook cell disease, and to re appear record based practice much(prenominal) 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 reaping hook cell disease. Further more, aspects of the disorder will be discussed in the succeeding(a) order hazard factors, clinical manifestations, diagnostic tests, complications, evidence based discourse, and diametrical takes of handicap 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 reaping hook cell disease, in order to put one across and provide competent care.Search StrategyTo help retrieve the more or less up to date, recent and peer reviewed articles, different search strategies aim been used. care for 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 textbook and published dates set from 2009 to 2014 were selected, as well as the availability of references was let in d. Boolean operators, and and or, were comm totally used furthermore, search term reaping hook cell disease and Boolean phrases including complications, treatment, diagnostic test, pediatric medicine, prevention, etiology, and Canada were combined to narrow the search.Epidemiologyreap hook 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 further nearly East China (Brown, M. 2012). A study conducted by Lanzkron et al (2013) took a look at mortality rates with individuals who had reap hook cell disease over a time frame of thirty divisions. Over the itinerary of thirty years, 16, 654 reaping hook cell-related deaths had authorise ruby-red with a mortality rate of 0.7% each year (Lanzkron et al, 2013). In Canada, one in every 2500 babies will be born(p) with sickle cell disease, and have a one in foursome chance (25%) of hav ing sickle cell disease. Furthermore, they have a one in two chance (50%) of universe a carrier for sickle cell disease ( sickle Cell Disease standstill of Canada, 2013).Etiology/Risk FactorsSickle cell disease is an inherited autosomal recessive term that causes an deviant 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 start and fecundher pass on the defective gene to their claw (Brown, M. 2012). Therefore, being a carrier of the sickle cell trail is a major(ip) 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 telephone exchange Amer ica, Middle East, Central India, southeast coast of Turkey and Mediterranean in Sicily (Pack-Mabian, A Haynes, J.r. 2009).clinical ManifestationsAlthough, each individual will flourish 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 origin cell which blocks the head for the hills of blood. As a result, clinical manifestations often show anemia, jaundice and voiceless suffering (Addis, G. 2010). Often, patients are asymptomatic except when experiencing a sickling episode (Lewis, 2014).An individual with sickle cell disease may develop anemia overdue to the complete destruction of red blood cells or haemolysis (Addis, G. 2010). Normally, red blood cells live for 120 years in our organic structure just now sickle cells usually die within ten to twenty days (Addis, G. 2010). As a result, the bodys 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, sweat and tachypnea are evident in patients with sickle cell crisis (Tortora Derrickson, 2012).In addition, the constant quantity breakdown of hemoglobin produces bilirubin, a byproduct of hemoglobin, which toiletnot be processed by the liver. As a result, the bilirubin gets stored in the blood and connective wander 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 injure as a result of vaso-occlusive crises, also cognize as sickle cell crisis. pain related to sickle cell disease accounts for ninety percent of infirmary admissions (Musumadi, L. et al. 2012). This is mainly due to episodes of sickling that prevents ox ygenated blood reaching organs, which results in ischaemia and delaying deterioration of tissue and organ function (Musumadi, L. et al., 2012).Diagnostic TestsDiagnostic tests to canvas sickle cell disease involve blood work. Often, individuals who have sickle cell disease are best diagnosed with the use of a peripheral device 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 commemorate sickle cell character between sickle cell disease. It whole works by identifying various types of hemoglobin within a blood exemplar to confirm the diagnosis of sickle cell disease (Randolph Wheelhouse, 2012).Additionally, secondary diagnostic tests could be used to diagnose complications that arise with sickle cell disease. Individuals may r equire a chest x-ray, skeletal x-ray, magnetic resonance tomography (MRI), and a Doppler ultrasound (Lewis, 2014). Skeletal x-rays are used to determine thrum and joint deformities whereas chest x-rays are used to diagnose chest transmission system (pneumonia). MRIs are used to aid in the diagnosis of a injection 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 complicationsSickle 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 infarct 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 uprises of hands and feet (Miller Meier, 2012). Vaso-occlusive pain involving the back, c hest, abdomen, or extremities carrys to occur throughout the lifespan of an individual with sickle-cell disease (Pack-Mabien, 2009). Furthermore, both pediatrics and adults get wind 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 unwholesomeness and mortality in patients with sickle cell disease (Miller Meier, 2012). Additionally, sickling episodes can modify the pulmonary system in all affected age groups and causes penetrating chest syndrome, a disorder that includes pulmonary complications such as pneumonia, fat embolism, systemic infection, pulmonary infarction, and if not treated, can lead to re spiratory also-ran (Miller Andrew, 2012). Unfortunately, children and adults are also at risk for stroke due to intellectual 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).TreatmentsThe 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 subacute pain (Addis, 2010). Another treatment includes RBC blood transfusion and is required as an requirement 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 Ca nada (Canadian tie-up of Sickle cell). Hydroxyurea increases the level of fetal hemoglobin that results in an overall decrease in circulating sickle cells (metalworker et al., 2011). A clinical study conducted by Smith et al (2011), has proven a decrease in pain durability 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 recover 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 MeasuresPrimary Preventive MeasuresAlthough genetic risk factors are non-modifiable, there is a preventive measure for sickle cell disease genetic direction. Genetic counselling should be promote in patients with the sickle cell trait especially when planning to have a child. This preventive measure helps patients und erstand 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 naughty 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 taproom measures in the secondary level focuses on diagnostic tests and screening for sickle cell disease. neonate screening is the earliest way to detect whether the child has sickle cell disease in time to prevent real complications from occurring (Newborn Screening Ontario, 2013). According to the Newborn Screening Ontario (2013), immature screening can prevent infection and sepsis, growth delay, painful sickle crisis, tissue ischemia and organ damage.Secondary preventive measures also involve previously verbali ze diagnostic testing for complications such MRI for stroke, the use of x-rays for chest infections, and so on 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 sagacity 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 maintain of arousal component (Assessment and Management of Pain, 2013).Tertiary Preventive MeasuresTertiary preventive measures involve treatments that aid in regaining patients useable 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 p ain 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). shutdownIn 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 dis ease 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 data to apply to everyday practice.ReferencesAddis, G. (2010). Sickle cell disease, part 1 reason the cond ition. British ledger Of School nurse, 5(5), 231-234.Brown, M. (2012). Managing the acutely ill adult with sickle cell disease.British Journal Of Nursing,21(2), 90-96.Lanzkron, S., Carroll, C., Haywood Jr., C. (2013). Mortality Rates and Age at finish from Sickle Cell Disease U.S., 1979-2005. Public Health Reports, 128(2), 110-116.Lewis, S. M. (2010). Medical-surgical nursing in Canada assessment and management of clinical problems (2nd Canadian ed.). Toronto Mosby Elsevier Canada.Memish, Z., Saeedi, M. (2011). Six-year outcome of the national premarital screening and genetic counseling program for sickle cell disease and beta-thalassemia in Saudi Arabia. Annals Of Saudi Medicine, 31(3), 229-235. doi10.4103/0256-4947.81527Miller, Andrew C., and Mark T. Gladwin. (2012) pulmonic Complications of Sickle Cell Disease.American Journal of Respiratory and Critical Care Medicine185.11 (2012) 1154-65..Miller, J. L., Meier, E. (2012). Sickle Cell Disease in Children. Drugs, 72(7), 895- 906. doi10.2165/11632890-000000000-00000Musumadi, L., Westerdale, N., Appleby, H. (2012). An overview of the effects of sickle cell disease in adolescents. Nursing Standard, 26(26), 35-40Neglected conditions. (2014). Canadian Medical Association.Journal, 186(6), 452-453.Newborn Screening Ontario. (2013). Newborn screening manual a scout for newborn care providers. Retrieved from http//www.newbornscreening.on.ca/data/1/rec_docs/795_CHO0093-NSM-Web.pdfPack-Mabien, A., Haynes, J. r. (2009). A primary care providers guide to preventive and acute care management of adults and children with sickle cell disease. Journal Of The American Academy Of Nurse Practitioners, 21(5), 250-257. doi10.1111/j.1745-7599.2009.00401.xRandolph, T. R., Wheelhouse, J. (2012). Novel test system (sickle confirm) to differentiate sickle cell anemia from sickle cell trait for potential use in developing countries. Clinical Laboratory Science, 25(1), 26-34.Registered Nurses Association of Ontario. (2013). Nur sing Best Practice Guidelines Assessment and Management of Pain. Retrieved from http//rnao.ca/Registered Nurses Association of Ontario. (2007). Nursing Best Practice Guidelines Professionalism in Nursing. Retrieved from http//rnao.caSickle Cell Disease Association (2013). Reterieved from http//www.sicklecelldisease.ca/Smith, W. R., Ballas, S. K., McCarthy, W. F., Bauserman, R. L., Swerdlow, P. S., Steinberg, M. H., Waclawiw, M. A. (2011). The Association Between Hydroxyurea Treatment and Pain Intensity, Analgesic Use, and Utilization in Ambulatory Sickle Cell Anemia Patients. Pain Medicine, 12(5), 697-705. doi10.1111/j.1526-4637.2011.01096.xTortora, G. J., Derrickson, B. (2012). autonomic nervous system Neurotransmitters and Receptors. Anatomy Physiology Princples of Anatomy. Danvers, MA JOHN WILEY.