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.

  1. 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 diseasehttps://www.cdc.gov/ncbddd/sicklecell/index.htmlMayo Clinic. (30 January, 2020). Sickle cell anemiahttps://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/symptoms-causes/syc-20355876St. Jude Children’s Research Hospital. (n.d.). Diagnostic screening for sickle cell diseasehttps://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).
    • Module 9, Eleany YaseinSubscribe
    • 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. StatPerarlshttps://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).
    • Module 9: The Hematologic System – SCDSubscribed
    • 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 Screening5(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.
    • Sickle Cell AnemiaSubscribe
    • 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 pathology14, 263–
    • complications in sickle cell disease: a concise narrative review.  Frontiers in Pediatrics8.   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 pathology14, 263–292. https://doi.org/10.1146/annurev-pathmechdis-012418-012838less1 UnreadUnread4 ViewsViews
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    • 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.
    • Sickle Cell DiseaseSubscribe
    • 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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    • 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).
    • Sickle cellSubscribe
    • 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 nursing119(6), 26–35.
      Sundd, P., Gladwin, M., & Novelli, E. (2019). Pathophysiology of Sickle Cell Disease. Annual review ofless2 UnreadUnread5 ViewsViews

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    • 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
    • Module 9Subscribe
    • 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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    • 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 Haematology102(4), 319–330. https://doi.org/10.1111/ejh.13212less1 UnreadUnread3 ViewsViews
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    • View profile card for Melissa Morgan
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    • 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 Screening5(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).
    • Dennies JonesSubscribe
    • 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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    • 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
    • Sickle CellSubscribe
    • 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). According to Huether et. al (2020) there are four major types of crises associated with this disease. A vasocclusive crises is caused by impaired blood flow through microcirculation. The plasma becomes thickened due to the increased hemolysis of RBC. This along with the changed shape increases adhesion of erythrocytes in the vessels blocking blood flow and causing pain for the individual (Sundd et. al., 2019). Sequestration occurs in children, where the liver and spleen become pooled with blood. Mortality is high if not treated in a timely manner (Huether et. al., 2020). Aplastic crisis is severe anemia that occurs due to a delay in erythropoiesis. It is usually caused by a parvovirus infection (NHLBI, 2020). Hyperhemolytic crisis can be caused by infections, medications or a reaction to a blood transfusion (Huether et. al., 2020). Huether, S. E., McCance, K. L., Brashers, V. L. (2020). Understanding Pathophysiology (7 Ed.).  Elsevier.Sundd, P., Gladwin, M. T., & Novelli, E. M. (2019). Pathophysiology of Sickle Cell Disease. Annual review of pathology14, 263–292. https://doi.org/10.1146/annurev-pathmechdis-012418-012838less1 UnreadUnread2 ViewsViews
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    • National Heart, Lung, and Blood Institute, (2020). Sickle Cell Disease. Retrieved from https://www.nhlbi.nih.gov/health-topics/sickle-cell-disease Sickle cell disease essay
    • References
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    • Sheryl Dixon posted Mar 17, 2021 10:43 PM
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    • Sickle cell diseases (SCD) is a group of inherited red blood cell disorders. Healthy red blood cells are round, and they move through small blood vessels to carry oxygen to all parts of the body. In someone who has SCD, the red blood cells become hard and sticky and look like a C-shaped farm tool called a “sickle”. The sickle cells die early, which causes a constant shortage of red blood cells. Also, when they travel through small blood vessels, they get stuck and clog the blood flow. This can cause pain and other serious problems such infection, acute chest syndrome and stroke (CDC,2020).Hemoglobin Sβ0 thalassemiaHemoglobin SCHemoglobin SEIn all types of sickle cell disease, at least one of the two abnormal genes cause a person’s body to make hemoglobin S. When a person has two hemoglobin S genes (hemoglobin SS), the disease is called sickle cell anemia. This is the most common and often most severe type of sickle cell disease. Hemoglobin SC disease and hemoglobin Sβ thalassemia are two other common types of sickle cell disease. Hemoglobin SD and hemoglobin SE are much less common(nhlibi.2020). CDC, (2020). What is Sickle cell disease. Retrieved from https://www.cdc.gov/ncbddd/sicklecell/facts.htmlNhlbi, (2020). Sickle Cell Disease. Also known as Sickle Cell Anemia, Hemoglobin S or SS disease, Sickling disorder due to hemoglobin S.  Retrieved from https://www.nhlbi.nih.gov/health-topics/sickle-cell-diseaseless1 UnreadUnread6 ViewsViews
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    • View profile card for Caroline Otto
    • Last post March 18 at 10:31 PM by Caroline Otto
    • Ihtc, (2020). Sickle Cell Disease Inheritance Patterns and Trait. Retrieved from https://www.ihtc.org/sickle-cell-causes
    •                                                                                                     References
    • As this sickle cell disease inheritance each parent has one hemoglobin A gene and one hemoglobin S gene, meaning each child of these parents has: a 25% chance of inheriting two normal genes (the child does not have sickle cell trait or disease).a 50% chance of inheriting one hemoglobin A gene and one hemoglobin S gene (the child has sickle cell trait).25% percent chance of inheriting two hemoglobin S genes (the child has sickle cell disease) (ihtc.2020).
    • Hemoglobin SS
    • Hemoglobin SD
    • Hemoglobin Sβ+ thalassemia
    • People who have sickle cell disease have abnormal hemoglobin, called hemoglobin S or sickle hemoglobin, in their red blood cells. Hemoglobin is a protein in red blood cells that carries oxygen throughout the body. People who have sickle cell disease inherit two abnormal hemoglobin genes, one from each parent. The types of sickle cell disease include the following:
    • Sickle Cell DiseaseSubscribe
    • Caroline Otto posted Mar 18, 2021 10:19 PM
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    • Most of the patients I have encountered in my practice seek medical treatment for pain. They have pain in their joints, hip, or spine. This is because of the abnormal B-globin chain of Hb S polymers realigning and forming their sickle shape under periods of decreased oxygenation and dehydration, and they essentially clump in the joints causing acute pain. My patients usually report stress, a recent illness or heat exhaustion and causes. The other reason I see patients in my practice is because they will get called by their PCP and told to come to the ER because their hemoglobin is low. (They had gone to the Doctor for a check up and had labs drawn).PathophysiologyA Homozygous form is sickle cell anemia, and this type is the most severe, and it occurs when the patient has inherited two copies of Hb S (Lonergan, Cline, & Abbondanzo, 2020).The heterozygous carrier state rarely has clinical manifestations. All forms of sickle cell disease are lifelong conditions. It is most common among persons with ancestry from sub-Saharan Africa, although it may be present among individuals with ancestry from Mediterranean countries, the Arabian Peninsula, parts of India, and Spanish-speaking areas of South America, however these are less common.Clinical Manifestations and EtiologyThere are four types of acute crises that these patients experience.
      1. Vasoocclusive crisis (thrombotic crisis) sickling in the microcirculation. Vasospasm occurs due to the bunched up sickled cells that cause obstruction of the blood vessel. If not treated and reversed, infarction and thrombosis will occur. This type is very painful and may last for days and even up to weeks with the average duration of being 4-6 days. This type is unpredictable, and chronic vasoocclusion eventually results in end-stage renal disease sickle cell disease essay.
      2. Sequestration crisis: Typically, only seen in children less than five years of age. The spleen and liver are largely affected here due to large amounts of blood pooling. The risk of mortality is high and approximately half of children who experience sequestration crises will have recurrent episodes.
      3. Aplastic crisis: Profound anemia is caused by lowered erythropoiesis despite an increased need for new erythrocytes. In this condition the current life cycle is only 10-20 days, and the compensatory response in the bone marrow is compromised, and if the patient suffers a viral infection aplastic crisis develops and this crisis may last from 7 to 10 days.
      4. Hyperhemolytic crisis: this is rare, and it occurs in association with certain drugs or infections, and it may occur in the form of an acute or chronic reaction following a blood transfusion.

      References:Lonergan, G. J., Cline, D. B., & Abbondanzo, S. L. (2020). Sickle cell anemia. Radiographics : a review publication of the Radiological Society of North America, Inc, 21(4), 971–994. https://doi.org/10.1148/radiographics.21.4.g01jl23971more0 UnreadUnread

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    • Sundd, P., Gladwin, M. T., & Novelli, E. M. (2019). Pathophysiology of Sickle Cell Disease. Annual review of pathology, 14, 263–292. https://doi.org/10.1146/annurev-pathmechdis-012418-012838
    • Huether, S. E., McCance, K.L. & Brashers, V.L. (2020). Understanding Pathophysiology 7th ed. Elsevier Mosby
    • General manifestations of Sickle Cell Disease include Pallor, fatigue, jaundice, and irritability in children, also pain.
    • In the US, Sickle cell anemia is most common in black people with a reported incidence of around 1:365 live births.  Interesting to note; however, its prevalence in African countries, such as Nigeria and the Democratic Republic of Congo, may be as high as 30%, and it is believed that the sickle cell trait may provide protection against lethal forms of malaria, thereby resulting in a genetic advantage for carrier who reside in regions of the world that are endemic for malaria, such as sub-Saharan Africa.
    • Sickle cell-thalassemia and sickle cell-Hb C disease are compound heterozygous forms in which the child inherits Hb S from one parent and another type of abnormal hemoglobin from the other parent. A sickle cell trait occurs when the child inherits Hb S from one parent and normal hemoglobin (Hb A) from the other.
    • This a group of autosomal recessive disorders characterized by the production of hemoglobin S within the erythrocytes. Hemoglobin S- Hb S; sickle hemoglobin; is formed because of a genetic mutation in which one amino acid (valine) replaces another (glutamic acid). When there is an increase in Oxygen demand, and dehydration, Hb S stretches and elongates giving rise to its formidable shape of the “sickle”. These sickled cells die early resulting in hemolytic anemia (Huether, page 551).
    • Incidentally, Sickle cell patients are the ones who have had the lowest Hemoglobin’s I have ever cared for. The lowest hemoglobin I ever took care of was a 4, and because they have had multiple blood transfusions, typing and cross matching takes forever in these patients because of their Antibodies.
    • Module 9 DiscussionSubscribe
    • Melissa Morgan posted Mar 17, 2021 8:19 PM
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    • Pathophysiology. Sickle cell disease (SCD) is an autosomal recessive disorder that makes the hemoglobin S sickle shaped under conditions of decreased oxygenation and dehydration (Huether, et al., 2020). The three most common sickle cell disease are sickle cell anemia, sickle cell-thalassemia disease, and sickle cell-Hb C disease sickle cell disease essay. Sickle cell anemia is the most severe out of the three and results when someone inherits two copies of Hb S, a homozygous form (Huether, et al., 2020). “Sickle cell-thalassemia and sickle cell-Hb C disease are compound heterozygous forms in which the child inherits Hb S from one parent and another type of abnormal hemoglobin from the other parent” (Huether, et al., 2020, p. 551). People who inherit Hb S from just one parent and not the other do not develop the disease and rarely show clinical manifestations (Huether, et al., 2020). Sickle cell anemia is most common in black people in the United States. According to Sundd et al., about 176,000 people die from SCD each year due to complications (2019).Aplastic crisis is presented with profound anemia that is caused by lowered erythropoiesis “despite an increased need for new erythrocytes” (Huether, et al., 2020, p. 554). This type of crisis can last 7-10 days, and usually is a result of a viral infection that has compromised the body’s ability to make new red blood cells after premature hemolysis (Huether, et al., 2020). The fourth type of crisis is hyperhemolytic crisis and may be a result of certain drugs or infections, acute or chronic reaction following a blood transfusion; this type of crisis is not usually seen in patients (Huether, et al., 2020). Many patients will initially seek medical help for a sickle cell crisis in an acute setting, however, “this initial event is the primary risk factor for potentially life-threatening complications” (Novelli & Gladwin, 2016, p. 1).Huether, S., McCance, K., and Brashers, V. (2020).  Understanding Pathophysiology (7th ed.).  ElsevierSundd, P., Gladwin, M. T., & Novelli, E. M. (2019). Pathophysiology of Sickle Cell Disease. Annual review of pathology14, 263–292. https://doi.org/10.1146/annurev-pathmechdis-012418-012838less0 UnreadUnread
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    • Novelli, E. M., & Gladwin, M. T. (2016). Crises in Sickle Cell Disease. Chest149(4), 1082–1093. https://doi.org/10.1016/j.chest.2015.12.016
    • References
    •  Clinical manifestations. Signs and symptoms of sickle cell disease can vary, because some patients have mild symptoms and others suffer from repeated vasoocclusive crises (Huether, et al., 2020). Clinical manifestations include pallor, fatigue, jaundice, and irritability (Huether, et al., 2020). This disease has four types of acute crises. Vasoocclusive crisis (thrombotic crisis) begins in the microcirculation and obstructs blood flow, which causes a vasospasm through the vessels (Huether, et al., 2020). This type of crisis is extremely painful with an average duration of 4-6 days, can last for weeks, and include complications like stroke, and end-stage renal disease (Huether, et al., 2020). Thrombosis and infarction of local tissue will occur if blood flow is not restored. The frequency of this crisis is variable and unpredictable. In Sequestration crisis is usually seen in children less than 5 years of age. The spleen and liver become saturated with blood, and because the spleen can hold one-fifth of the body’s blood supply at one time, the mortality rate is high if the patient is not treated (Huether, et al., 2020) sickle cell disease essay. About 50% of children will have recurrent episodes.
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    • Candice Russell posted Mar 16, 2021 9:14 PM
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    •             “Sickle Cell Disease (SCD) is a group of autosomal recessive disorders characterized by the production of hemoglobin S within erythrocytes” (Linder and McCance, 2020, p. 551). About 100,000 people in the United States and millions worldwide are affected by this inherited disorder. There are many forms of SCD, the most prevalent are sickle cell anemia, sickle cell-thalassemia, and sickle cell Hb C disease. Allali et al. (2020) stated “Although the genetic and molecular bases have long been characterized, the pathophysiology remains incompletely elucidated and therapeutic options are limited” (para. 1). In documenting the pathophysiology of SCD, evidence continues to point to innate immune cells playing a significant role in promoting inflammation, adhesion, and pain in SCD (Allali et al., 2020, para. 1) Sundd et al. (2019) stated the pathophysiology of SCD “is considered to be a vicious cycle of four major processes” (para. 1). Sundd et al. (2019) described this cycle as (a) hemoglobin S polymerization, (b) impaired biorheology and increased adhesion-mediated vaso-occlusion, (c) hemolysis-mediated endothelial dysfunction, and (d) concerted activation of sterile inflammation (Toll-like receptor 4– and inflammasome-dependent innate immune pathways. The promotion of acute and chronic pain and end-stage-organ injury/failure in SCD are produced by the coordination of molecular, cellular, and biophysical processes (Sundd et al., 2019, para. 1). Linder and McCance (2020) detailed the pathophysiology of SCD, stating when oxygenated appropriately, soluble Hb S causes no issues. When the reduction of oxygen tension takes place, polymerization of abnormal beta-globin chain in Hb S produce abnormal fluid polymers (Linder and McCance, 2020, p. 553). Sickle shaped red blood cells (RBCs) form due to the flawed hemoglobin (Hb). Normal RBCs are smooth, disk-shaped, and flexible, moving through blood vessels with ease. Sickle shaped RBCs are stiff and sticky, making it difficult to move through blood vessels.            Splenic Sequestration Crisis: Reduction of oxygen tension due to decreased blood flow through the spleen and increased polymerization in Hb S, resulting in narrowing capillaries in the splenic vascular bed. Entrapment of affected blood cells and RBC polymerization leads to a cycle of hypoxia and reduced blood flow causing enlargement of the spleen (Inusa et al., 2019, para. 6.5). Pooling of blood within the liver and spleen results in shock and circulatory failure sickle cell disease essay. A rapid increase in spleen size can cause abdominal distension, sudden weakness, polydipsia, tachycardia, and tachypnea. Increased risk of mortality if this crisis is left untreated. Death can occur in 1-2 hours due to circulatory failure (Araujo, 2009, as cited by Inusa et al., 2019, para. 6.5)Hyperhemolytic Crisis: Certain drugs and infections may induce this unusual crisis (Linder and McCance, 2020, p. 555). Rapid reduction in Hb levels is a rare and dangerous complication of sickle cell disease, which can quickly lead to organ failure and death (Srinivasan & Gourishankar, 2019, para. 1). This crisis can be acute or chronic, usually preceded by a blood transfusion.Sickle cell anemia, the most severe form of SCD, occurs in 1 out of 365 African American people. Some symptoms associated with sickle cell anemia include episodic pain, swelling of hands and feet, joint and bone pain, neuropathic pain, jaundice, pallor, dizziness, fatigue, and abdominal pain. With this patient’s reported hx of symptoms, race, along with a decreased hematocrit level, it is appropriate to consider sickle cell anemia. This patient’s presentation of tachycardia may or may not be involved in sickle cell anemia, however, it is common for SCD patients to experience tachycardia. Allali, S., Maciel, T. T., Hermine, O., & de Montalembert, M. (2020). Innate immune cells, major protagonists of sickle cell disease pathophysiology. Haematologica105(2), 273–283. https://doi.org/10.3324/haematol.2019.229989   less0 UnreadUnread
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    • Sundd, P., Gladwin, M. T., & Novelli, E. M. (2019). Pathophysiology of Sickle Cell Disease. Annual review of pathology14, 263–292. https://doi.org/10.1146/annurev-pathmechdis-012418-012838
    • Srinivasan, A., & Gourishankar, A. (2019). A Differential Approach to an Uncommon Case of Acute Anemia in a Child With Sickle Cell Disease. Global pediatric health6, 2333794X19848674. https://doi.org/10.1177/2333794X19848674
    • Huether, S. E., McCance, K. L., & Brashers, V. L. (2020). Understanding pathophysiology / Sue E. Huether, Kathryn L. McCance ; section editor, Valentina L. Brashers (7th ed.). Elsevier.
    • Reference:
    • Clinical manifestations can vary in SCD, ranging from mild symptoms to repeated vasoocclusive crises.  The most common signs of SCD are associated with anemia and pain. Tiredness, fatigue, bone, and abdominal pain are present. Pain can be acute or chronic, damage to many parts of the body can occur. There is an increased risk of infections, which can be life-threatening, due to a damaged spleen. Damage to the lungs, known as acute chest syndrome, can occur due to infection or sickled cells. Other complications of SCD include stroke and priapism in males. Many other complications are noted in SCD, as it can affect many body systems and structures. SCD is present at birth, signs, and symptoms are usually not present until 6 months after birth sickle cell disease essay.
    • Aplastic Crisis: In SCD erythrocyte lifespan is 10 to 20 days, leading to a significant reduction in Hb concentration. “Profound anemia is caused by lowered erythropoiesis despite increased need for new erythrocytes” (Linder and McCance, 2020, p. 554). Normal compensation of prematurely hemolyzed cells is compromised in SCD, occurring after a viral infection. “The most common cause of acquired bone marrow failure in SCD and other haemolytic disorders is caused by Parvovirus B19” (Minhas et al., 2017, as cited by Inusa et al., 2019, para. 6.3) Aplastic crisis typically last about 1 week.
    •             Vaso-Occlusive Crisis (Pain): Pain is considered a cardinal feature of SCD. Intense pain may be manifested in early infancy to adulthood. Pain accounts for most hospitalizations and has a significant impact on quality of life for individuals with SCD. Microcirculation of sickled RBCs obstructs blood flow through the vessels. Microvascular occlusion initiates the stimulation of nociceptive nerve fibers, causing pain. “The microcirculation is obstructed by sRBCs, thereby restricting the flow of blood” to the organ and this results in (i) ischaemia, (ii) oedema, (iii) pain, (iv) necrosis, and (v) organ damage” (De Montalembert, 2002, as cited by Inusa et al., 2019, para. 6.1). Pain is characterized as unpredictable, episodic, and spontaneous, it’s “described as one of the most excruciating forms of pain that affect human beings” (Inusa et al., 2019, para. 6.1).
    • A. KaurSubscribe
    • Amandeep Kaur posted Mar 15, 2021 2:13 PM
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    • Sickle cell disease is a chronic disorder of red blood cells (Vacca Jr.& Blank, 2017). It causes an abnormal production of hemoglobin, which is responsible for carrying oxygen. Due to the inability of the hemoglobin to carry oxygen pH becomes acidic. The RBS will also lose its normal round, pliable, biconcave shape. Intracellular changes cause the RBC to become deformed, rigid, and crescent-shaped. The RBC begins to adhere to different areas vessels of the body and can accumulate, causing increased blood viscosity. Manifestations include anemia due to cells easily breaking apart and dying (Sickle cell anemia, 2020). Individuals can also have episodes of pain, this is due to blocked blood flow through the tiny vessels of the chest, abdomen, and joints. Other symptoms include swelling of hands and feet, frequent infections, delayed growth or puberty, and issues with vision. These symptoms are consistent with the patient’s symptoms. This disease is an inherited disease, specifically, it is autosomal recessive (Sundd et al., 2019). This means both parents would need to at least be carriers of the disease for the children to have it. It is more prevelent among individuals of African American descent sickle cell disease essay.  References Sickle cell anemia. (2020, January 30). Retrieved March 15, 2021, from https://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/symptoms-causes/syc-20355876Sundd, P., Gladwin, M. T., & Novelli, E. M. (2019). Pathophysiology of Sickle Cell Disease. Annual review of pathology14, 263–292.less1 UnreadUnread5 ViewsViews
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  • Last post March 16 at 10:59 AM by Cassie Fritzinger
  • Vacca Jr., V. M., & Blank, L. (2017). Sickle cell disease: Where are we now? Nursing47(4), 26–35.
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  • Cassie Fritzinger posted Mar 12, 2021 3:11 PM
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  •       Sickle Cell Disease (SCD) is an inherited disease which needs to be passed down from both parents. If there is only one genetic mutation the patient is considered to have ‘sickle cell trait’ and is generally healthy. The person with both genetic mutations will display the sickle cell phenotype. The incidence of SCD is approximately 100,000 Americans have SCD, and it is more often seen in Blank/African Americans with 1 in every 365 births having SCD and 1 in every 13 having the trait (NIH, 2020).      Symptoms often seen in patients with SCD include jaundice or sclera icterus from a significant amount of red cell hemolysis. The patient will also most likely experience fatigue from the significant anemia associated with SCD. SCD patients may also display painful swelling of their hands and feet from blood flow being blocked inside small bones in the hands and feet (NIH, 2020).  Triggers which can exacerbate a sickle cell crisis include extremes of temperature (or rapid change in temperature) dehydration, stress, & venous stasis such as that when hospitalized or bedbound (Espinoza, 2020).      Sickle cell disease patient’s can be often complex to treat. They will often require higher doses of pain medications than the non-SCD patient. Sickle cell pain crisis often last an average of 4-6 days or longer without proper treatment. For our patient of discussion, she was hospitalized for an arthroscopic knee surgery, she is already starting low with the hematocrit percentage of 25%, her blood pressure may be elevated due to pain or other physiologic reasons such as compensation for low blood volume. Further, her heart rate is also high noting the same cause potential. Abdominal pain would make one suspicious for concerns in the liver/spleen. While our discussion does not say if we are treating this patient pre-operatively or post-operatively, it would be important to optimize the patient’s pre/post op oxygenation, hydration, normothermia, positioning, and of course pre/postop anxiolytic use to decrease the chances of further crisis developing from these triggers sickle cell disease essay.
    • Increase ambient temperature in the OR maintain thermoregulation with warm forced air blankets over the body wherever possible.
    • 500ml bolus of Lactated Ringers followed by 8ml/kg/hr maintenance
    • Padding with warm blankets to decrease venous stasis and loss of thermoregulation through conduction.
    • Preoxygenate and avoid a traumatic intubation to decrease any chance of hypoxic episode
    • Administer Dexamethasone and Cefazolin for inflammation and decreased risk of infection/illness post operatively
    • Administer anxiolytic medication as appropriate to help the patient relax prior to surgery
    • Maintain adequate pain control

    Post op treatments would be similar to ensure the patient maintains adequate hydration, pain control, thermoregulation, and limits venous stasis. In 2019 the FDA approved Voxelotor a medication for SCD which prevents red blood cells from binding and changing to sickle shape. They will also decrease the destruction of blood cells decreasing the risk of anemia and improving overall blood flow to end organs. An IV medication Crizanlizumab-tmca can also be given with similar results (NIH, 2020). The common treatment of Hydroxyurea has shown to reduce and prevent sick cell complications by inhibiting DNA synthesis as well as providing an anti-inflammatory effect through decreased leukocyte production.           Espinoza, C. (2020). Perioperative Management for Sickle Cell Disease: Efficacy of Recommendations. International Student Journal of Nurse Anesthesia, 19(3), 46–49.NIH (2020). Sickle Cell Disease. National Heart, Lung, and Blood Institute. Retrieved March 12, 2021 from https://www.nhlbi.nih.gov/health-topics/sickle-cell-disease

  • Zazzman, S. M., Zamora, F. J., & Roberts, J. D. (2019). Inpatient pain management in sickle cell disease. American Society of Health System Pharmacists. 76 (23) 1965-1971. 10.1093/ajhp/zxz228
  • Heuther, S. E., McCance, K. L., & Brashers, V. L. (2020). Understanding Pathophysiology (7th ed.). Elsevier.
  • References
  •       Inpatient management for the patient with SCD includes adequate pain management up to and including intravenous or parenteral patient-controlled analgesia (PCA). However it is important to transition the patient to oral products to ensure adequate pain control once discharged from the hospital. Zassman, Zamora, & Roberts (2019), provides a tier approach to transitioning the patient to oral agents. While there are multifactorial considerations in choosing agents to transition, it is important to do an appropriate 24-hour capture of the parenteral medication which is effective for patient pain control sickle cell disease essay. Next is to choose a short-acting oral agent (something they can be discharged home on) and convert the parenteral 24 total dose into a equivalent 24 hour oral medication dose. It would also be important to consider decreasing this dose by 25%-50% for incomplete cross-tolerance. This 24 hour dose should then be divided appropriately throughout the day dosing (Zassman, Zamora, & Roberts 2019).
  • Espinoza (2020), recommends the following Pre/Intraoperative treatments
  •       Significant complications which can occur in SCD include sequestration crisis, aplastic crisis, and hyperhemolytic crisis.  Sequestration crisis (typically seen in children) is when the patient’s blood gets stuck within the liver & spleen. Because a large volume percentage of the patient’s blood can be held within the spleen there is not enough circulating blood placing the patient at significant risk of mortality if not recognized and treated early (Heuther, McCance, & Brasher, 2020). Aplastic crisis generally caused from parvovirus B19 (fifth disease) can cause bone marrow suppression of blood production leading to worsening/severe anemia (NIH, 2020). Hyperhemolytic crisis while rare is a condition of SCD when there is a significant amount of hemoglobin lysis/loss which can be caused from certain medications, infections and occasionally blood transfusion reactions. This creates a significant anemia which can lead to death from decreased organ perfusion (Heuther, McCance, & Brasher, 2020).
  •       Every state within the District of Columbia, United States and US territories require prenatal and newborn screening for SCD. For this reason parents are often aware of their child having SCD at a very young age and can manage their condition better (NIH, 2020). Our patient in discussion would most likely have known she had SCD by the age of 38, unless she was lucky enough to never have any sickle cell crisis and was not previously evaluated by a medical professional who may have suspected SCD. A sickle cell crisis is also known as a vasoocclusive crisis. This generally begins when sickle cell shaped red blood cell enter the host microcirculation subsequently causing vasospasm and blockage of the vessel. When this is not reversed it will lead to ischemia or infarction of the local tissue. The red blood cells take this sickle shape in SCD secondary to a genetic mutation when amino acids are altered. Under decreased oxygen and dehydration conditions the cell stretches and elongates assuming the sickle cell shape (Heuther, McCance, & Brasher, 2020) sickle cell disease essay.