sickle cell disease essay
sickle cell disease essay
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A 38-year-old African-American woman was admitted for arthroscopic knee surgery. Her hematocrit was 25%, blood pressure was 140/94 mm Hg, and pulse was 112 beats/minute. She had a history of joint and bone pain, jaundice, and abdominal pain.
Due to the presenting symptoms (joint and bone pain, abdominal pain, and jaundice) a diagnosis of Sickle Cell Disease was considered.
Provide a brief discussion of the pathophysiology of sickle cell disease and discuss the clinical manifestations and the etiology associated with each manifestation of this disease.
Post your initial response by Wednesday at midnight. Respond to one student by Sunday at midnight. Both responses must be a minimum of 150 words, scholarly written, APA formatted, and referenced. A minimum of 2 references are required (other than your text). Refer to grading rubric for online discussion.
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Sickle Cell DiseaseSubscribe
Tallona Boddy posted Mar 16, 2021 1:37 PM
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Sickle cell disease, specifically, is an inherited blood disorder, by both parents. In the U.S., sickle cell disease is part of the newborn screening, via a blood test and can be diagnosed at any time during a person’s lifespan (Centers for Disease, 2020) sickle cell disease essay. Diagnosis of sickle cell disease is completed through blood tests, the hemoglobin electrophoresis, high performance liquid chromatography (HPLC) and deoxyribonucleic acid (DNA) test, (St. Jude Research, n.d.). A patient may present to their provider with any of the above mentioned symptoms, as well as, other symptoms such as fever, severe unexplained episodes of pain, pale skin or eyes, or signs and symptoms of a stroke. The etiology for these manifestations is again due to the shape of the sickle cell. A fever is due to a damaged spleen from the sickle cells, causing increased infections. Pale skin or eyes is due to the anemia and poor oxygenation, the sickle cells blocking and sticking to one another in smaller vessels. Signs and symptoms of a stroke are due to a potential blood clot caused by the sticky red blood cells sticky to one another and occluding a larger vessel (Mayo Clinic, 2020). Centers for Disease Control and Prevention. (14 December, 2020). Sickle cell disease. https://www.cdc.gov/ncbddd/sicklecell/index.htmlMayo Clinic. (30 January, 2020). Sickle cell anemia. https://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/symptoms-causes/syc-20355876St. Jude Children’s Research Hospital. (n.d.). Diagnostic screening for sickle cell disease. https://www.stjude.org/treatment/disease/sickle-cell-disease/diagnosing-sickle-cell.htmlless1 UnreadUnread6 ViewsViews
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View profile card for Gisselle Mustiga
Last post March 22 at 12:25 AM by Gisselle Mustiga
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Sickle cell disease affects a person’s red blood cells, typically, red blood cells are round, sickle cell red blood cells are crescent (moon) shaped and sticky. The crescent shaped red blood cells have difficulty moving through a person’s blood vessels, compared to traditional round, non-sticky red blood cells. The crescent shaped red blood cells tend to get stuck in small blood vessels and stick to one another. Due to the sticky, crescent shape of the red blood cells the patient can experience a number of symptoms sickle cell disease essay. Anemia is often present due to the sickle cells breaking easier and dying within 10-20 days, not meeting the average 120 day lifespan of a red blood cell. Pain, hand/foot swelling and vision problems are common symptoms due to the sickle cells sticky nature and crescent shape, building up and blocking blood flow in the body (Mayo Clinic, 2020).
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Eleany Yasein posted Mar 17, 2021 9:09 PM
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Sickle cell disease is autosomal recessive, genetic disorder that affects multisystem in the body (Sedrak & Kondomudi, 2021). It mostly affects African Americans, about 1 in 12 African Americans carry the mutation. This disorder is caused due to a mutation on chromosome 11, there is an amino acid substitution (glutamic acid is changed to Valine) in position 6. This mutation causes the Hemoglobin molecule to change and alter the erythrocyte shape. There is adhesion of the erythrocytes and formation of heterocellular aggregation, leading to vessel occlusion and hypoxia. Moreover, Hemoglobin binds to nitric oxide and releases oxygen, which causes the erythrocytes to sickle. There is an increased HbS (sickled HbA) formation and activation of inflammatory mediators and free radicals are released, which causes reperfusion injury. Furthermore, there is an increase nitric oxide binding, neutrophil adhesiveness, platelet activation and hypercoagulability (Sedrak & Kondomudi, 2021).ReferencesSundd, P., Gladwin, T. M., & Novelli, M. E. (2018 October 17). Pathophysiology of Sickle Cell Disease. Annual Review Pathology, 14, 263-292. doi: 10.1146/annurev-pathmechdis-012418-012838more1 UnreadUnread3 ViewsViews
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View profile card for Aina Oluwo
Last post March 21 at 10:13 PM by Aina Oluwo
Sedrak, A., & Kondamudi, N. (2021, January 22). Sickle Cell Disease. StatPerarls. https://www.ncbi.nlm.nih.gov/books/NBK482384/
Patients with sickle cell disease will present with different rage of clinical manifestations depending on the body system affected and the severity (Sedrak & Kondomudi, 2021). Vaso-occlusive crisis is the most common presentation seen. Microvascular occlusion leads to ischemia, which leads to tissue damage and triggers inflammation process. This activates nociceptors and cause pain. Patient complain of pain in different part of their body, including their bones, back, chest and abdomen. Patients can also present with acute chest syndrome, which is caused due to the hypoxia and release of inflammatory mediators. Symptoms include, fever, cough, chest pain, which can lead to respiration failure (Sedrak & Kondomudi, 2021). Moreover, Sickle cell disease causes intravascular and extravascular hemolysis causing chronic anemia (Sundd et al., 2018). This causes increase in cardiac output, ventricular dilation and cause vascular injury. The endothelial dysfunction and vascular injury cause multiorgan failure. Patients can present with renal dysfunction, hypertension, cerebrovascular accidents, and pulmonary hypertension (Sundd et al., 2018).
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Gisselle Mustiga posted Mar 16, 2021 2:11 PM
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Sickle cell anemia results from mutation of the b globin gene. It should be noted that the red blood cells containing the abnormal b alleles undergo polymerization and become rigid when exposed to deoxygenated environments. These rigid red blood cells are often susceptible and liable to hemolysis and also have increased densities. Their weights affect the endothelial walls’ blood flow and integrity (Inusa et al., 2019). Hemolysis results in complex activities and events, including the consumption and dysregulation of nitric oxide. It also causes endothelial dysfunction, leading to severe complications, including stroke, priapism, leg ulceration and pulmonary hypertension. Compared to the red blood cell with a half-life of 120 days, sickle RBCs can only exist for a maximum of 20 days due to hemolysis (Huether et al. 2020). Distortion of the normal discoid RBCs into sickle cell results from the formation of long fibers of deoxyhemoglobin S.ReferencesInusa, B. P., Hsu, L. L., Kohli, N., Patel, A., Ominu-Evbota, K., Anie, K. A., & Atoyebi, W. (2019). Sickle cell disease—genetics, pathophysiology, clinical presentation and treatment. International Journal of Neonatal Screening, 5(2), 20. https://www.mdpi.com/2409-515X/5/2/20/pdf less1 UnreadUnread7 ViewsViews
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View profile card for Steven Bartos
Last post March 21 at 10:00 PM by Steven Bartos
Nickel, R. S., & Hsu, L. L. (2016). Clinical manifestations of sickle cell anaemia: infants and children. In Sickle Cell Anemia (pp. 213-229). Springer, Cham. http://ndl.ethernet.edu.et/bitstream/123456789/42788/1/pdf.237#page=223
Huether, S. E., McCance, K. L. & Brashers, V. L. (2020). Understanding Pathophysiology 7th ed. Elsevier Mosby sickle cell disease essay.
The first clinical manifestation of sickle cell anemia is the vaso-occlusive crisis which occurs due to obstruction of microcirculation resulting in ischemic injuries and pain to the organs. This is associated with increased emergency department visits and hospitalization among patients with SCD. The pain can affect any body part, including soft tissues, joints, abdomen and bones (Nickel & Hsu, 2016). The other manifestation associated with the pain is infarctions and auto-splenectomy that affects the spleen resulting in failures and life-threatening infections. Papillary necrosis is a renal manifestation that results in isosthenuria. The other manifestation is the aplastic crisis which is caused by infection with Parvovirus B-19, causing fifth disease, mild rash, and fever. Splenic sequestration is characterized by rapid enlargement of the spleen and increased reticulocyte counts. Sickle cell disease can also cause progressive injury to the liver with significant fibrosis, often cirrhosis, and decreased liver function by adulthood. Asymptomatic patients commonly have hepatomegaly and elevated liver enzyme levels. Other manifestations include acute chest syndrome and hand-foot syndrome.
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Lois Chappell posted Mar 21, 2021 9:39 PM
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Sickle cell disease an autosomal recessive genetic disorder belonging to a is a group of inherited red blood cell disorders affecting millions of people worldwide (Sundd, Gladwin,& Novelli, 2019). Sickle cell anemia is the most common form of the disease, and accounts for 70% of African Americans who have sickle cell disease (Sundd, Gladwin, & Novelli, 2019). In sickle cell disease, red blood cells that are normally round become hard and sticky and form a “c” shape, causing them to get stuck during normal blood flow. Along with endothelial interactions, systemic inflammation, oxidant stress, and activation of the coagulation system, these cellular alterations can cause the predominant symptoms of sickle cell disease including stroke, acute chest syndrome and infection (Centers for Disease Control, 2021). Center for Disease Control and Prevention (2021). Sickle Cell Disease. Retrieved from Field, J., Vichinsky, E., Tyrnauer, J. (2021). Acute chest syndrome in adults with sickle cell disease. Retrieved from Ochocinski, D., Dalal, M., Black, L. Vandy, C., Lew, J., Sullivan, K., and Kissoon, N. (2020). Life-threatening infectioushttps://doi.org/10.3389/fped.2020.00038.292. https://doi.org/10.1146/annurev-pathmechdis-012418-012838 sickle cell disease essay. less0 UnreadUnread
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Sundd, P., Gladwin, M. T., & Novelli, E. M. (2019). Pathophysiology of Sickle Cell Disease. Annual review of pathology, 14, 263–
complications in sickle cell disease: a concise narrative review. Frontiers in Pediatrics, 8. Retrieved from
https://www.uptodate.com/contents/acute-chest-syndrome-in-adults-with-sickle-cell-disease.
https://www.cdc.gov/ncbddd/sicklecell/index.html.
In sickle cell disease, strokes can occur as a result of red blood cells getting stuck in a vessel, reducing blood flow to the brain (Centers for Disease Control, 2021). Acute chest syndrome can be caused by infection, asthma, or hypoventilation or as the result of fat emboli, bone marrow necrosis or both (Up to Date, 2021). Infection in sickle cell disease is due to impaired splenic clearance of bacteria (Ochocinski, Dalal, Black, Vandy, Lew, Sullivan, and Kissoon, 2020).
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Hilary Szpara posted Mar 17, 2021 12:09 PM
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Sickle cell disease is a broad term that describes a group of conditions which arise from the inheritance of hemoglobin S (HbS) (Houwing et al., 2019). There are different variations of the gene mutation and people can also have the sickle cell trait or be a sickle cell carrier (Houwing et al., 2019). Sickle cell disease is an inherited disease that is autosomal recessive and is caused by single mutation in the B-globin gene (Houwing et al., 2019). There are four major pathobiological processes which drive the disease; including vaso-occlusion, hemolysis-mediated endothelial dysfunction, sterile inflammation, and hemoglobin S polymerization (Sundd et al., 2019). With hemoglobin S polymerization, the hemoglobin S polymers grow rapidly and form long fibers which increase cellular rigidity and distort the cell membrane, leading to the sickling of the cell, dehydration, energetic failure and stress, and premature hemolysis (Sundd et al., 2019) sickle cell disease essay. Vaso-occlusion leading to ischemia is responsible for the vaso-occlusive crises. Chronic hemolysis and sickle shape increases the plasma viscosity and contributes to the impaired flow of blood through capillaries (Sundd et al., 2019). Endothelial dysfunction occurs due to the chronic hemolysis, which results in anemia. The anemia exerts extra stress on the cardiovascular system by increasing cardiac output, ventricular chamber dilation, and ventricular wall stress (Sundd et al., 2019). Vaso-occlusion contribute to the ischemia-reperfusion injury, along with the release of erythrocyte damage-associated molecular patterns (eDAMPs), which are products of hemolysis, contribute to the process of sterile inflammation (Sundd et al., 2019). There are also a variety of complications that can arise due to sickle cell disease, including increased risk of infection, cerebrovascular accidents, acute chest syndrome, acute kidney injury, hepatobiliary complications, splenic complications, priapism, as well as a long list of chronic complications (Houwing et al., 2019). Sundd, P., Gladwin, M. T., & Novelli, E. M. (2019). Pathophysiology of Sickle Cell Disease. Annual review of pathology, 14, 263–292. https://doi.org/10.1146/annurev-pathmechdis-012418-012838less1 UnreadUnread4 ViewsViews
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View profile card for Candice Russell
Last post March 21 at 8:16 PM by Candice Russell
Houwing, M. E., De Pagter, P. J., Van Beers, E. J., Biemond, B. J., Rettenbacher, E., Rijneveld, A. W., Schols, E. M., Philipsen, J. N. J., Tamminga, R. Y. J., Fijn van Draat, K., Nur, E., Cnossen, M. H. (2019). Sickle cell disease: Clinical presentation and management of a global health challenge. Blood Reviews, 37.
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Jennifer Bryant posted Mar 16, 2021 3:54 PM
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Sickle cell disease is a collection of inherited blood disorders that affects approximately 100,000 people in the United States and millions worldwide. Sickle cell disease is caused by the inheritance of abnormal beta-globin alleles carrying the sickle mutation on the HBB gene (Ware et al., 2017).Acute splenic sequestration crisis results from the trapping of red blood cells in the spleen of a person with sickle cell disease. The pooling and destruction of blood cells within the spleen results in decreased circulating blood volume, potentially leading to hypovolemic shock, cardiovascular collapse, and even death (Esterson et al, 2021). Hyperhemolytic crisis is a rare and dangerous complication of sickle cell disease where the hemoglobin level drops rapidly. This can quickly lead to organ failure and death (Srinivasan & Gourishankar, 2019).Darbari, DS, Sheehan, VA, Ballas, SK. (2020) The vaso‐occlusive pain crisis in sickle cell246. https://doi-org.wilkes.idm.oclc.org/10.1111/ejh.13430sectional imaging appearance of an uncommon diagnosis, Clinical Imaging 69 369-373Minhas, P. S., Virdi, J. K., & Patel, R. (2017). Double whammy- acute splenic sequestrationCommunity Hospital Internal Medicine Perspectives, 7(3), 194-195.Srinivasan, A & Gourishankar A. (2019) “A Differential Approach to an Uncommon Case ofhttps://doi.org/10.1177/2333794X19848674.(2018). Vaso-occlusive crisis in sickle cell disease: Current paradigm on paindoi:http://dx.doi.org.wilkes.idm.oclc.org/10.2147/JPR.S185582disease sickle cell disease essay. The Lancet, 390(10091), 311-323.less2 UnreadUnread5 ViewsViews
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View profile card for Dennies Jones
Last post March 21 at 7:33 PM by Dennies Jones
doi:http://dx.doi.org.wilkes.idm.oclc.org/10.1016/S0140-6736(17)30193-9
Ware, R. E., de Montalembert, M., Tshilolo, L., & Abboud, M. R. (2017). Sickle cell
management. Journal of Pain Research, 11, 3141-3150.
Uwaezuoke, S. N., Ayuk, A. C., Ndu, I. K., Eneh, C. I., Mbanefo, N. R., & Ezenwosu, O. U.
Acute Anemia in a Child With Sickle Cell Disease.” Global Pediatric Health
doi:http://dx.doi.org.wilkes.idm.oclc.org/10.1080/20009666.2017.1340729
crisis in patient with aplastic crisis due to acute parvovirus infection. Journal of
https://doi.org/10.1016/j.clinimag.2020.10.016.
Esterson, Y.B., Sheth, S., Kawamoto, S. (2021) Splenic sequestration in the adult: cross
disease: Definition, pathophysiology, and management. Eur J Haematol. 105: 237-
References
Transient aplastic crisis usually occurs in older children or adults with sickle cell disease commonly due to acute human parvovirus B19 infection and is characterized by reticulocytopenia and mild neutropenia/thrombocytopenia (Minhas et al, 2017).
Vaso-occlusive crisis or acute painful crisis constitutes the major morbidity in sickle cell disease (Uwaezuoke et al, 2018). Sickled erythrocytes cause vaso-occlusion together with many other cellular and plasma factors and abnormal endothelial interactions, leading to a broad range of acute and chronic clinical complications caused by repeated ischemia and inflammation (Ware et al., 2017).
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Aina Oluwo posted Mar 17, 2021 10:23 PM
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According to literature, Sickle cell disease (SCD) is an autosomal recessive disorder of a gene mutation that affects about 1 in 500 African American children and 1 in 36,000 Hispanic American children. (Borhade & Kondamudi, 2021). SCD based on its pathology results in symptoms ranging from anemia to sickle cell crisis (SCC) with often manifests as ”acute pain crisis’’ and will often require hospitalization. The term “sickle cell crisis” is used to describe several acute conditions such as the vaso-occlusive crisis (acute painful crisis), aplastic crisis, splenic sequestration crisis, hyper hemolytic crisis, hepatic crisis, dactylitis, and acute chest syndrome. (Borhade & Kondamudi, 2021) sickle cell disease essay.In our case study, our 38 y/o patient presented with low hematocrit and associated joint and bone pain as well as findings consistent with jaundice and abdominal pain. The diagnosis of sickle cell disease was considered based on this presentation however it is essential to note that a diagnosis of sickle cell disease cannot be made by the findings of low hematocrit only but rather a combination of all her symptoms as well as genotype assessment. Individuals will have at least one sickle hemoglobin (HbS) allele, which is an abnormal allele for the β-globin gene (Adams-graves & Bronte-Jordan, 2016). The disease can be misdiagnosed or incorrectly self-reported by patients and this is where the providing physician must assess the patient’s presenting physical symptoms as well as a complete detailed history of past medical presentations and subsequently obtain genotype testing for sickle cell disease confirmation. Normal red blood cells are round and flexible and move easily through blood vessels however in sickle cell disease, abnormal hemoglobin causes red blood cells to become sickle shaped and rigid and this allows the cells to easily become lodged in smaller blood vessels, depriving tissues of oxygen and triggering painful episodes (Tanabe et al 2019). Once these cells begin to occlude vessels, this triggers painful events leading to hemolysis, anemia in varying organs and most notably, the spleen. Splenic injury greatly increases the risk of death from infection at a young age sickle cell disease essay.The pathophysiology of sickle cell disease is characterized by a single amino acid substitution in the β-globin chain leads to polymerization of mutant hemoglobin S, impairing erythrocyte rheology and survival. Clinically, erythrocyte abnormalities in SCD manifest in hemolytic anemia and cycles of microvascular vaso-occlusion leading to end-organ ischemia-reperfusion injury and infarction. (Sundd et al 2019). With continued progression of the disease the risk for multiorgan injury as well as severe clinical presentations are often seen and an interplay between multiple disciplines are essential in combating and providing appropriate medical management to limit recurrence of acute crisis and frequent ED visits and hospitalizations.
Adams-Graves, P., & Bronte-Jordan, L. (2016). Recent treatment guidelines for managing adult patientsof Hematology, 9(6), 541. https://doi.org/10.1080/17474086.2016.1180242https://www.ncbi.nlm.nih.gov/books/NBK526064/Complications of Sickle Cell Disease. The American journal of nursing, 119(6), 26–35.
Sundd, P., Gladwin, M., & Novelli, E. (2019). Pathophysiology of Sickle Cell Disease. Annual review ofless2 UnreadUnread5 ViewsViews
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View profile card for Jazmin Jerez-Rivera
Last post March 20 at 11:34 AM by Jazmin Jerez-Rivera
pathology, 14, 263–292. https://doi.org/10.1146/annurev-pathmechdis-012418-012838
https://doi.org/10.1097/01.NAJ.0000559779.40570.2c
Tanabe, P., Spratling, R., Smith, D., Grissom, P., & Hulihan, M. (2019). CE: Understanding the
Borhade, M., & Kondamudi, N. (2021). Sickle cell Crisis. Statpearls.
with sickle cell disease: Challenges in access to care, social issues, and adherence. Expert Review
References
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Steven Bartos posted Mar 17, 2021 8:07 PM
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Sickle cell disease (SCD) is a group of autosomal recessive disorders where a mutation in the B-globin gene produces mutated hemoglobin molecules that causes the erythrocytes to form a sickle shape (Gladwin, 2016). The sickling is a result of decreased oxygenation and pH, and dehydration sickle cell disease essay. Acute infections, temperature changes, and high altitude can increase sickling, as this affects oxygenation. Sickle cells’ irregular shape block the blood vessels, causing a thickening of the blood and decreased circulation, resulting in vascular injury and dysfunction of the spleen, bones, and liver. SCD has a higher prevalence among black people and African countries (Huether et al., 2017). The disease can manifest in the form of various disorders, with the most common being to the cardiovascular, hepatobiliary, renal, and musculoskeletal joint systems.The hepatobiliary system is defined as liver dysfunction and hyperbilirubinemia as a result of intrahepatic sickling, subsequently causing ischemia, pooling of blood in the liver and spleen, and cholestasis. Pancreatitis, peptic ulcer disease, as well acute and chronic liver failure are common manifestations. Abdominal pain and jaundice are common symptoms related to these disorders (Shah, et al., 2017).Musculoskeletal issues are common in patients with SCD, with its etiology stemming from the vaso-occlusion of postcapillary venules, resulting in bone infarction and inflammation. Steroid induced vaso-occlusive disease may also present itself when patients are being treated for rheumatic conditions as well, as this type of steroid therapy can cause painful vaso-occlusive episodes. Osteomyelitis, intramedullary marrow hyperplasia, osteonecrosis, restricted bone growth, inflammatory arthritis, and gout are also common musculoskeletal manifestations of SCD (Hughes et al., 2016).Airy, M., & Garabed, E. (2017). The kidney in sickle hemoglobinopathies. Clinical Nephrology, 87(2), 55 – 68. http://dx.doi.org.wilkes.idm.oclc.org/10.5414/CN108991Hughes, M., Akram, Q., Rees, D.C., & Jones, A.K.P. (2016). Haemoglobinopathies and the rheumatologist. Rheumatology, 55(12), 2109 – 2118. https://doi.org/10.1093/rheumatology/kew042less2 UnreadUnread3 ViewsViews
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View profile card for Tallona Boddy
Last post March 20 at 12:43 AM by Tallona Boddy
Shah, R., Taborda, C., & Chawla, S. (2017). Acute and chronic hepatobiliary manifestations of sickle cell disease: A review. World Journal of Gastrointestinal Pathophysiology, 8(3), 108 – 116. https://dx.doi.org/10.4291%2Fwjgp.v8.i3.108
Gladwin, M.T. (2016). Cardiovascular complications and risk of death in sickle-cell disease. The Lancet, 387(10037), 2565 – 2574. http://dx.doi.org.wilkes.idm.oclc.org/10.1136/bcr-2020-236743
References
Kidney problems develops as a result of the sickling process obstructing the glomerulus, tubule, and medulla of the kidney. Albuminuria and the inability to concentrate urine are warning signs of renal failure due to SCD. Chronic kidney disease accounts for approximately 14 – 18% of mortality in SCD (Airy & Eknoyan, 2017) sickle cell disease essay.
Cardiovascular issues develop as a result of repeated vaso-occlusive episodes, which lead to cardiac ischemia. However, chronic hemolytic anemia, whereby red blood cells die faster than they are made, is the major cause of cardiovascular morbidity and mortality in patients with SCD. Chronic red blood cell transfusions and associated secondary iron overload is another factor that causes heart damage. Pulmonary hypertension, chronic thromboembolic pulmonary hypertension, systolic and diastolic heart dysfunction, and ischemic heart disease are found in patients with sickle cell. An elevated pulse and high blood pressure may be typical vital signs given these manifestations (Gladwin, 2016) sickle cell disease essay.
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Joanne Hogan posted Mar 16, 2021 11:07 AM
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Sickle cell is one of the most common life threatening inherited disorders in the world, most commonly affecting people of African, Arab, and Indian ancestry (Inusa et al., 2019). The gene frequency is more prominent in Western Africa with the likelihood being 25-30% (Inusa et al., 2019).Clinical manifestation of sickle cell can be mild but hemolytic anemia generally causes jaundice, pallor, and fatigue (Huether et al., 2020). Vasospasm and vasoocclusion cause severe pain that may last days to weeks (Huether et al., 2020). Leg ulceration, priapism, stroke, and pulmonary hypertension can occur due to hemolysis and tissue ischemia (Inusa et al., 2019). In children under the age of 5, sequestering occurs and large amounts of blood pools in the liver and spleen which can cause death if not noticed (Huether et al., 2020). Aplastic crisis can occur due to the rapid cell cycle of the red blood cells in sickle cell patients and hyperhemolytic crisis may occur due to certain infections or drugs (Huther et al., 2020). Infection is more common in sickle cell patients and can trigger a sickle cell crisis (Inusa et al., 2019). Huether, S. E., McCance, K. L., & Brashers, V. L. (2020). Understanding pathophysiology (7th ed.). Elsevier.Piccin, A., Murphy, C., Eakins, E., Rondinelli, M., Daves, M., Vecchiato, C., Wolf, D., Mc Mahon, C., & Smith, O. P. (2019). Insight into the complex pathophysiology of sickle cell anaemia and possible treatment. European Journal of Haematology, 102(4), 319–330. https://doi.org/10.1111/ejh.13212less1 UnreadUnread3 ViewsViews
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View profile card for Melissa Morgan
Last post March 19 at 5:02 PM by Melissa Morgan
Inusa, B., Hsu, L., Kohli, N., Patel, A., Ominu-Evbota, K., Anie, K., & Atoyebi, W. (2019). Sickle cell disease—genetics, pathophysiology, clinical presentation and treatment. International Journal of Neonatal Screening, 5(2), 20. https://doi.org/10.3390/ijns5020020
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References
Sickle cell anemia is an autosomal recessive disease and is caused by abnormal hemoglobin production due to a mutation in the inherited β -globin gene (Piccin et al., 2019). In this mutation, the hydrophobic amino acid valine is substituted with the hydrophilic glutamic amino acid in the β- globin chain (Inusa et al., 2019). This mutation results in hemoglobin called hemoglobin- S that is poorly soluble when deoxygenated and highly unstable (Piccin et al., 2019). Red blood cells are dehydrated which causes low potassium levels and altering of the cell membrane, giving red blood cells their sickle shape (Piccin et al., 2019). Sickling causes clogged vessels and increases blood viscosity (Huether et al., 2020). The sickling of cells is usually not permanent, and cells can regain their normal shape after they are reoxygenated and rehydrated (Huether et al., 2020).
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Dennies Jones posted Mar 17, 2021 9:37 PM
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with a hematocrit of 25%, elevated blood pressure and pulse, BP 140/94, and P 112. The patient has a history of joint and bone pain, jaundice, and abdominal pain. Due to symptoms, she was diagnosed with sickle cell disease. According to Harding et al. (2020),”Sickle cell disease (SCD) is a group of inherited, autosomal recessive disorders characterized by an abnormal form of hemoglobin in the RBC. Because this is a genetic disorder, SCD is usually found during routine neonatal screening” (p. 33838). According to (Bender M.A 2021), “The clinical manifestation of Sickle cell disease is characterized by intermittent vaso-occlusive events and chronic hemolytic anemia. Vaso-occlusive events result in tissue ischemia leading to acute and chronic pain and organ damage that can affect any organ system, including the bones, spleen, liver, brain, lungs, kidneys, and joints. Dactylitis (pain and/or swelling of the hands or feet) is often the earliest manifestation of SCD” (p. 10). Baba, I., Lewis, H., Neeraj, K., Patel, A., Ominu-Evbota, K., Kofi, A., & Wale, A. (2019). Sickle Cell Disease—Genetics, Pathophysiology, Clinical Presentation and Treatment. Neonatal Screening. doi:doi:10.3390/ijns5020020 less1 UnreadUnread4 ViewsViews
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Last post March 19 at 1:25 AM by Sheryl Dixon
Harding, Mariann M., Kwong, Jeffrey, Roberts, Dottie, Hagler, Debra, Reinisch, Courtney. Lewis’s Medical-Surgical Nursing E-Book (Kindle Locations 33838-33840). Elsevier Health Sciences. Kindle Edition.
Bender, M. A. (2021). Sickle Cell Disease. In M. P. Adam (Eds.) et. al., GeneReviews®. University of Washington, Seattle.
Reference:
SCD is one of the most common inherited disorders. It predominantly affects people of color. Early screening is essential for the survival of newborns. Among the common causes of death is the absence of early diagnosis followed by education and preventive therapies such as penicillin prophylaxis and chronic surveillance include infections, severe anemia, and multi-organ failure (Baba et al., 2019).
Baba et al. (2019) explained the pathophysiology of sickle cell as “red blood cells (RBCs) that contain HbS or HbS in combination with other abnormal β alleles when exposed to deoxygenated environment undergo polymerization and become rigid. The rigid RBCs are liable to hemolysis. Due to increased density, it may affect blood flow and endothelial vessel wall integrity” (p.5)—the dense, rigid RBCs lead to vaso-occlusion tissue ischemia, infarction as well as hemolysis.
A 38-year-old African American was admitted for knee surgery. The patient presented
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Jazmin Jerez-Rivera posted Mar 17, 2021 9:23 PM
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Sickle cell disease (SCD) is an autosomal recessive disorder which produces hemoglobin S inside erythrocytes. The mutation occurs when glutamic acid is substituted with valine causing a mutation in the hemoglobin tetramer HbS (Sundd et. al., 2019). Erythrocytes gain their sickle shape under conditions of low oxygen and dehydration in the body. Protein strands form inside the red blood cell (RBC) causing it to stretch taking on a crescent shape (NHLBI, 2020). Clinical manifestations vary depending on the severity of the disease. Early symptoms include jaundice, fatigue and dactylitis (painful swelling of hands and feet) (NHLBI, 2020). A
