Cardiovascular and Hematologic Disorders Essay

A 17-year-old African American female from the inner city complains of severe chest and abdominal pain. Upon examination, the attending physician performs an EKG, chest x-ray, and an abdominal and chest clinical examination and finds nothing. Cardiovascular and Hematologic Disorders Essay. Assuming she is drug seeking, he sends her home. She comes back to the ER 4 hours later and now you see the patient. She explains that she was running track this past afternoon at school and that despite being very hot (100 F) she pushed on. Afterwards, she starts feeling extensive pain in her chest and abdomen. She has jaundiced eyes, her blood pressure is 98/50, pulse is 112, T = 99.9 F, R = 28. The pain seems out of proportion to the physical findings. During a review of her medical history, she tells the provider that she has history of sickle cell anemia. At this time, it is determined that she is in a sickle cell crisis. Cardiovascular and Hematologic Disorders Essay.

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1. What is the etiology of sickle cell anemia?
2. Describe in detail the pathophysiological process of sickle cell anemia.
3. Identify hallmark signs identified from the physical exam and symptoms.
4. Describe the pathophysiology of complications of sickle cell anemia.
5. What teaching related to her diagnosis would you provide?

In addition to the textbook, utilize at least one peer-reviewed, evidence based resource to develop your post. Cardiovascular and Hematologic Disorders Essay.

1. What is the etiology of sickle cell anemia?

Sickle cell disease is “a group of disorders characterized by the presence of an abnormal form of hemoglobin—hemoglobin S (HbS)—within the erythrocytes” (McCance, Huether, Brashers, & Rote, 2013, p. 1063). It is an inherited disease from at least one of the parents. Sickle cell disease is a lifelong illness, but it can be managed. There are different types of sickle cell disease including sickle cell anemia, sickle cell-thalassemia disease, and sick cell-HbC disease. There is even the sickle cell trait where a person can be a carrier, having inherited the HB S from one parent. However, there are rarely clinical issue with those who are just carriers. Sickle cell anemia is a normocytic-normochormic anemia, or NNA, meaning that it is “characterized by erythrocytes that are relatively normal in size and hemoglobin content but insuffcient in number” (McCance, Huether, Brashers, & Rote, 2013, p. 993). There are five groups of NNAs including the hereditary hemolytic type that sickle cell anemia is because of the destruction by eryptosis, or the destruction erythrocytes through cell shrinkage, membrane blebbing, or activation of proteas (McCance, Huether, Brashers, & Rote, 2013, p. 993). Sickle cell anemia is the most common sickle cell disease and the most severe (NHBLI, 2014). It is estimated that there are between 70,000 and 100,000 Americans who suffer from sickle cell anemia with those affected most being of African ancestry. While sickle cell has a high rate of morbidity, more than 90% of diagnosed children do survive into adulthood, however, their lifespan is diminished (NHBLI, 2014). Sickle cell disease is screened in every newborn born in a hospital in the United States. This is done through a simple blood test that checks the hemoglobin in the body. Cardiovascular and Hematologic Disorders Essay.

Good job!

2. Describe in detail the pathophysiological process of sickle cell anemia.

Sickle cell disease is a genetic mutation that “results in substitution of the amino acid valine for glutamic acid at the sixth position of the beta globin chain, causing formation of hemoglobin S” (NHLBI, 2014). In a healthy body, regular hemoglobin is disc shaped and flexible, so that it can take oxygen through large and small blood vessels throughout the body. However, in a patient with sickle cell anemia, their hemoglobin is not flexible: “It can form stiff rods within the red cell, changing it into a crescent, or sickle shape Sickle-shaped cells are not flexible and can stick to vessel walls, causing a blockage that slows or stops the flow of blood” (NHBLI, 2014). This means that oxygen is not able to reach the tissue it needs to reach. When tissue is not able to receive oxygen, sudden and intense pain can occur that requires hospitalization. Cardiovascular and Hematologic Disorders Essay. The red cells also have a shorter lifespan than healthy red cells, meaning that the number of red cells in the body is lower than normal, leading to anemia.

That’s correct!

3. Identify hallmark signs identified from the physical exam and symptoms.

A person with sickle cell disease or anemia had that issue from birth. However, most symptoms do not occur until an infant is around 6 months old, but diagnosis can occur much later. The biggest symptom are random episodes of pain. These are called pain crises or vaso-occlusive. When the sickle cells get stuck or cannot deliver the oxygen needed, the pain the patient feels can be “without warning and are described sharp, intense, stabbing, or throbbing” (NHBLI, 2014). Pain can occur anywhere but seems to be most common in the lower back, legs, arms, abdomen, and chest. Other than pain crises, patients can feel chronic pain all the time, not just off and on like in a pain crisis. Other symptoms can include painful swelling of the hands and feet, extreme fatigue or irritability, as well as the yellowing of the skin or eyes known as jaundice (NHBLI, 2014). As the National Heart, Lung, and Blood Institute notes the symptoms can be different with each patient and “most of the signs and symptoms of SCD are related to complications of the disease” (2014). Cardiovascular and Hematologic Disorders Essay.

That was very thorough – good work!

4. Describe the pathophysiology of complications of sickle cell anemia.

• Stroke: A stroke can occur when the blood flow to the brain is blocked. When this happens, the cells in the brain can be damaged or die. The sickled cells can block the blood vessels to the brain, meaning that it is not getting the blood or oxygen that it needs. Some symptoms of a clinical stroke can include “weakness of an arm or leg on one side of the body, loss of balance, and severe headache” (NHLBI, 2014). Depending on the kind of hemoglobin, like hemoglobin SS or hemoglobin SC, might raise the patient’s risk for stroke.
• Infections: Due to the spleen’s importance in filtering out germs and bacteria, it is an important organ in fighting infections. However, sickle cell anemia can damage organs in the body due to lack of oxygen and hemoglobin. The spleen is very susceptible to this meaning that those who suffer from sickle cell anemia are very prone to infections. Even small things in a healthy person like the flu can be bad enough in a patient with sickle cell to kill them (McCance, Huether, Brashers, & Rote, 2013, p. 1066). Cardiovascular and Hematologic Disorders Essay.
• Acute Chest Syndrome: Sickled cells can even affect the lungs. When the cells get stuck and are not delivered to the lungs, it can cause tissue damage meaning the lungs will not be able to exchange oxygen properly anymore. The text says that “Sickled RBCs attach to the endothelium of the injured, underventilated, and inflamed lung and, as they fail to be reoxygenated, will eventually undergo additional inflammation and lung infarction” (McCance, Huether, Brashers, & Rote, 2013, p. 1066).

Other complications generally start due to the destruction of the organs because of lack of oxygen and hemoglobin. These can include eye problems and heart complications including heart disease and hypertension (NHLBI, 2014).

You hit all of the major ones – way to go!

5. What teaching related to her diagnosis would you provide?

I would first explain to the patient that she did the right thing by coming to the ER while she was experiencing a pain crisis. I would then apologize profusely for the fact that she was turned away to begin with. It can be difficult for healthcare providers to know those who really need help and those who are just trying to take advantage of the system. Once we got the patient’s pain under control using things like NSAIDs, I would explain to her that an infusion might be best to get her hemoglobin count back up. Once she was stable and no longer in pain, I would explain to her the many complications that come with sickle cell anemia and giver her tips and tricks on how to avid things like infections, heart disease, and making sure that she doesn’t over exert herself like she did today. I would also point her toward mental health counseling as a diagnosis like sickle cell anemia can take quite a mental toll on the patient. Cardiovascular and Hematologic Disorders Essay. Counseling could help her come to terms with her diagnosis and also possibly help with any chronic pain through meditation and other things like that.

References

McCance, K. L., Huether, S. E., Brashers, V. L., & Rote, N. S. (2013). Pathophysiology: The

biologic basis for disease in adults and children (7th ed.). St. Louis, MO: Mosby.

NHLBI. (2014). Evidence-based management of Sickle Cell Disease: Expert panel report.

National Heart, Lung, and Blood Institute. Retrieved from https://www.nhlbi.nih.gov/

sites/default/files/media/docs/sickle-cell-disease-report%20020816_0.pdf. Cardiovascular and Hematologic Disorders Essay.