NR 507 Week 3 Discussion Part One What is the etiology of sickle cell anemia?

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NR 507 Week 3 Discussion Part One What is the etiology of sickle cell anemia?

NR 507 Week 3 Discussion Part One What is the etiology of sickle cell anemia

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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. 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.

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?

A Sample Answer For the Assignment: NR 507 Week 3 Discussion Part One What is the etiology of sickle cell anemia?

Title: NR 507 Week 3 Discussion Part One What is the etiology of sickle cell anemia?

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

 

• 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.

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. 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.

 

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).

 

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).
• 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).

 

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. 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 NR 507 Week 3 Discussion Part One What is the etiology of sickle cell anemia?

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

Jul 23, 2018Jul 23 at 11:24pm

Manage Discussion Entry

Hello Nakisha,

I enjoyed reading your post. Although sickle cell disease is usually found at birth with a blood test during routine newborn screening tests and with a second blood test to confirm the diagnosis, I found it to be pretty interesting that sickle cell disease can possibly be diagnosed before a baby is born with a test on the amniotic fluid or with a sample of tissue from the placenta (Kingwell, 2016). On the other hand it is good to know that stem cell transplant is a known cure for sickle cell disease. I am aware that transplants are complex and risky, but it seems that for now, it is an option only for some patients. One day, it’s hoped that doctors can stop the disease by changing or replacing the abnormal gene that causes it. But even without a cure, kids with sickle cell disease can lead fairly normal lives if they follow their treatment plan and that’s a great thing!

Reference NR 507 Week 3 Discussion Part One What is the etiology of sickle cell anemia?

Kingwell, K. (2016). Sickle cell solutions in sight. Nature Reviews. Drug Discovery, 15(9), 593-4. doi:10.1038/nrd.2016.177

 

Jul 24, 2018Jul 24 at 1:17am

Manage Discussion Entry

 

Hello Nakisha,

I enjoyed reading your post. Although sickle cell disease is usually found at birth with a blood test during routine newborn screening tests and with a second blood test to confirm the diagnosis, I found it to be pretty interesting that sickle cell disease can possibly be diagnosed before a baby is born with a test on the amniotic fluid or with a sample of tissue from the placenta (Kingwell, 2016). On the other hand it is good to know that stem cell transplant is a known cure for sickle cell disease. I am aware that transplants are complex and risky, but it seems that for now, it is an option only for some patients. One day, it’s hoped that doctors can stop the disease by changing or replacing the abnormal gene that causes it. But even without a cure, kids with sickle cell disease can lead fairly normal lives if they follow their treatment plan and that’s a great thing!

Reference NR 507 Week 3 Discussion Part One What is the etiology of sickle cell anemia?

Kingwell, K. (2016). Sickle cell solutions in sight. Nature Reviews. Drug Discovery, 15(9), 593-4. doi:10.1038/nrd.2016.177

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NR 507 Week 3 Discussion Part One What is the etiology of sickle cell anemia? Grading Rubric Guidelines

Performance Category	10	9	8	4	0
Scholarliness Demonstrates achievement of scholarly inquiry for professional and academic decisions.	Provides relevant evidence of scholarly inquiry clearly stating how the evidence informed or changed professional or academic decisions Evaluates literature resources to develop a comprehensive analysis or synthesis. Uses valid, relevant, and reliable outside sources to contribute to the threaded discussion	Provides relevant evidence of scholarly inquiry but does not clearly state how the evidence informed or changed professional or academic decisions. Evaluates information from source(s) to develop a coherent analysis or synthesis. Uses some valid, relevant, reliable outside sources to contribute to the threaded discussion.	Discusses using scholarly inquiry but does not state how scholarly inquiry informed or changed professional or academic decisions. Information is taken from source(s) with some interpretation/evaluation, but not enough to develop a coherent analysis or synthesis. Little valid, relevant, or reliable outside sources are used to contribute to the threaded discussion. Demonstrates little or no understanding of the topic.	Discusses using scholarly inquiry but does not state how scholarly inquiry informed or changed professional or academic decisions. Information is taken from source(s) without any interpretation/evaluation. The posting uses information that is not valid, relevant, or reliable	No evidence of the use of scholarly inquiry to inform or change professional or academic decisions. Information is not valid, relevant, or reliable
Performance Category	10	9	8	4	0
Application of Course Knowledge – Demonstrate the ability to analyze, synthesize, and/or apply principles and concepts learned in the course lesson and outside readings and relate them to real-life professional situations	Posts make direct reference to concepts discussed in the lesson or drawn from relevant outside sources; Applies concepts to personal experience in the professional setting and or relevant application to real life.	Posts make direct reference to concepts discussed in the lesson or drawn from relevant outside sources. Applies concepts to personal experience in their professional setting and or relevant application to real life Interactions with classmates are relevant to the discussion topic but do not make direct reference to lesson content	Posts are generally on topic but do not build knowledge by incorporating concepts and principles from the lesson. Does not attempt to apply lesson concepts to personal experience in their professional setting and or relevant application to real life Does not demonstrate a solid understanding of the principles and concepts presented in the lesson	Posts do not adequately address the question posed either by the discussion prompt or the instructor’s launch post. Posts are superficial and do not reflect an understanding of the lesson content Does not attempt to apply lesson concepts to personal experience in their professional setting and or relevant application to real life	Posts are not related to the topics provided by the discussion prompt or by the instructor; attempts by the instructor to redirect the student are ignored No discussion of lesson concepts to personal experience in the professional setting and or relevant application to real life
Performance Category	5	4	3	2	0
Interactive Dialogue Replies to each graded thread topic posted by the course instructor, by Wednesday, 11:59 p.m. MT, of each week, and posts a minimum of two times in each graded thread, on separate days. (5 points possible per graded thread)	Exceeds minimum post requirements Replies to each graded thread topic posted by the course instructor, by Wednesday, 11:59 p.m. MT, of each week, and posts three or more times in each graded thread, over three separate days. Replies to a post posed by faculty and to a peer Summarizes what was learned from the lesson, readings, and other student posts for the week.	Replies to each graded thread topic posted by the course instructor, by Wednesday, 11:59 p.m. MT, of each week, and posts a minimum of two times in each graded thread, on separate days Replies to a question posed by a peer Summarizes what was learned from the lesson, readings, and other student posts for the week.	Meets expectations of 2 posts on 2 different days. The main post is not made by the Wednesday deadline Does not reply to a question posed by a peer or faculty	Has only one post for the week Discussion posts contain few, if any, new ideas or applications; often are a rehashing or summary of other students’ comments	Does not post to the thread No connections are made to the topic
	Minus 1 Point	Minus 2 Point	Minus 3 Point	Minus 4 Point	Minus 5 Point
Grammar, Syntax, APA Note: if there are only a few errors in these criteria, please note this for the student in as an area for improvement. If the student does not make the needed corrections in upcoming weeks, then points should be deducted. Points deducted for improper grammar, syntax and APA style of writing. The source of information is the APA Manual 6th Edition	2-3 errors in APA format. Written responses have 2-3 grammatical, spelling, and punctuation errors. Writing style is generally clear, focused, and facilitates communication.	4-5 errors in APA format. Writing responses have 4-5 grammatical, spelling and punctuation errors. Writing style is somewhat focused.	6-7 errors in APA format. Writing responses have 6-7 grammatical, spelling and punctuation errors. Writing style is slightly focused making discussion difficult to understand.	8-10 errors in APA format. Writing responses have 8-10 grammatical, spelling and punctuation errors. Writing style is not focused, making discussion difficult to understand.	Post contains greater than 10 errors in APA format. Written responses have more than 10 grammatical, spelling and punctuation errors. Writing style does not facilitate communication. The student continues to make repeated mistakes in any of the above areas after written correction by the instructor
	0 points lost				-5 points lost
Total Participation Requirements per discussion thread	The student answers the threaded discussion question or topic on one day and posts a second response on another day.				The student does not meet the minimum requirement of two postings on two different days
Early Participation Requirement per discussion thread	The student must provide a substantive answer to the graded discussion question(s) or topic(s), posted by the course instructor (not a response to a peer), by Wednesday, 11:59 p.m. MT of each week.				The student does not meet the requirement of a substantive response to the stated question or topic by Wednesday at 11:59 pm MT.

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