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
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
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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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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).
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 Screening, 5(2), 20. https://www.mdpi.com/2409-
515X/5/2/20/pdf less1 UnreadUnread7 ViewsViews
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