Review Sickle Cell Disease

Pathophysiology of the Hematologic System > Pathophysiology

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Sickle Cell Disease

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Question 1:

A nurse is caring for a patient with sickle cell disease (SCD)
The patient asks, "Why do my red blood cells become sickle-shaped?" Which response by the nurse is most accurate?

A. "Your red blood cells become sickle-shaped due to a genetic mutation that produces abnormal hemoglobin S (HbS)” B. "Sickle-shaped red blood cells occur because of an autoimmune reaction in your body.” C. "The sickle shape of your red blood cells is caused by a viral infection.” D. "Your diet and lifestyle choices contribute to the sickle shape of your red blood cells.”

Answer and Explanation

Choice A:

Sickle cell disease (SCD) is a genetic disorder caused by a mutation in the HBB gene, leading to the production of abnormal hemoglobin called hemoglobin S (HbS) This abnormal hemoglobin causes red blood cells to become sickle-shaped when exposed to low oxygen levels, which is a hallmark of SCD. The nurse's response accurately reflects the genetic basis of this condition.

Choice B:

This choice is incorrect. Sickle-shaped red blood cells in SCD are not the result of an autoimmune reaction.

Choice C:

This choice is incorrect. Sickle-shaped red blood cells are not caused by a viral infection.

Choice D:

This choice is incorrect. Diet and lifestyle choices do not contribute to the sickle shape of red blood cells in SCD.

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Question 2:

A client with sickle cell disease (SCD) presents with jaundice and gallstones.
The client asks the nurse for an explanation.
Which response by the nurse is correct?

A. "Jaundice and gallstones are common complications of SCD due to hemolysis, the destruction of your abnormal red blood cells.” B. "Jaundice and gallstones in SCD are caused by excessive iron intake in your diet.” C. "These symptoms occur because of an allergic reaction to certain foods.” D. "Jaundice and gallstones result from an overproduction of normal red blood cells.”

Answer and Explanation

Choice A:

Jaundice and gallstones are common complications of sickle cell disease (SCD) because of hemolysis, which is the destruction of abnormal red blood cells. The breakdown of these cells releases bilirubin, leading to jaundice, and can also result in the formation of gallstones.

Choice B:

This choice is incorrect. Jaundice and gallstones in SCD are not caused by excessive iron intake in the diet.

Choice C:

This choice is incorrect. Allergic reactions to foods do not lead to jaundice and gallstones in SCD.

Choice D:

This choice is incorrect. Jaundice and gallstones in SCD do not result from an overproduction of normal red blood cells.

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Question 3:

A nurse is educating a group of patients with sickle cell disease (SCD) about complications.
Select all that apply:

A. Increased risk of infection. B. Hypoxia and ischemia. C. Blood clot formation. D. Reduced risk of stroke. E. Reduced adhesion of RBCs to the endothelium.

Answer and Explanation

Choice A:

Patients with sickle cell disease (SCD) have an increased risk of infection due to the compromised immune function associated with the disease. SCD can lead to functional asplenia, making individuals more susceptible to infections, particularly those caused by encapsulated bacteria.

Choice B:

Hypoxia (lack of oxygen) and ischemia (reduced blood flow) are common complications of SCD. The sickle-shaped red blood cells can obstruct blood vessels, leading to reduced oxygen delivery to tissues (hypoxia) and tissue damage due to impaired blood flow (ischemia)

Choice C:

Blood clot formation is a known complication of SCD. The altered shape of sickle cells can lead to the blockage of blood vessels, causing painful vaso-occlusive crises and increasing the risk of clot formation.

Choice D:

This choice is incorrect. Reduced risk of stroke is not associated with SCD. In fact, individuals with SCD may have an increased risk of stroke due to the potential for vasculopathy and clot formation.

Choice E:

Reduced adhesion of red blood cells (RBCs) to the endothelium is not a typical feature of SCD. In fact, the adhesion of sickle cells to the endothelium is one of the pathophysiological mechanisms leading to vaso-occlusive events in SCD.

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Question 4:

A client with sickle cell disease (SCD) is experiencing a severe episode of pain.
The nurse recognizes this as a:

A. Sickle cell crisis. B. Hemolysis event. C. Jaundice episode. D. Inflammatory reaction.

Answer and Explanation

Choice A:

A severe episode of pain in a client with sickle cell disease (SCD) is indicative of a sickle cell crisis. Sickle cell crisis is a common complication of SCD, characterized by sudden, severe pain due to the formation of sickle-shaped red blood cells that obstruct blood flow in small blood vessels. This vaso-occlusion leads to tissue ischemia and pain in various parts of the body. It is a hallmark symptom of SCD and requires prompt management, typically with pain medications and hydration.

Choice B:

Hemolysis event is not the correct choice in this context. Hemolysis refers to the premature destruction of red blood cells, which can occur in SCD but does not directly correlate with the severe pain experienced during a sickle cell crisis.

Choice C:

Jaundice episode is not the correct choice in this context either. Jaundice, characterized by yellowing of the skin and eyes, can occur in individuals with SCD due to the breakdown of hemoglobin from ruptured red blood cells, but it does not specifically address the severe pain described in the question.

Choice D:

Inflammatory reaction is not the correct choice for this scenario. While inflammation can play a role in the pathophysiology of SCD, it does not directly describe the acute and severe pain experienced during a sickle cell crisis.

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Question 5:

A nurse is performing a nursing assessment of a patient with sickle cell disease (SCD)
Which of the following is a priority problem to identify in this patient?

A. Skin rash. B. Increased energy levels. C. Pain and discomfort. D. Improved lung function.

Answer and Explanation

Choice A:

Skin rash is not the priority problem to identify in a patient with sickle cell disease (SCD) While skin manifestations can occur in SCD, such as leg ulcers, they are not typically the most critical issue for these patients. Pain and discomfort related to sickle cell crisis, vaso-occlusion, and tissue ischemia take precedence as a priority.

Choice B:

Increased energy levels are not a priority problem in a patient with SCD. In fact, patients with SCD often experience fatigue and weakness, especially during sickle cell crises. Identifying and managing pain and discomfort are more crucial for the patient's well-being.

Choice C:

Pain and discomfort are the priority problems to identify in a patient with SCD. Sickle cell crises can lead to excruciating pain in various parts of the body, and prompt management of this pain is essential to improve the patient's quality of life and prevent complications.

Choice D:

Improved lung function is not the priority problem to identify in a patient with SCD. While lung complications can occur in SCD (e.g., acute chest syndrome), addressing pain and discomfort is more urgent and essential for the patient's immediate well-being. .

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Question 6:

A nurse is assessing a patient with sickle cell disease (SCD)
The patient reports pain in various areas, including the chest and joints.
What other clinical manifestations of SCD should the nurse be vigilant for?

A. "I've been experiencing fever and chills recently.” B. "I've noticed a yellowing of my skin and eyes.” C. "I've been having trouble breathing and feeling weak.” D. "I've had painful erections and impotence.”

Answer and Explanation

Choice A:

"I've been experiencing fever and chills recently." Fever and chills can be associated with various illnesses and infections but are not specific clinical manifestations of sickle cell disease (SCD) The primary concern in SCD is vaso-occlusion, anemia, and pain, which should be monitored closely.

Choice B:

"I've noticed a yellowing of my skin and eyes." Yellowing of the skin and eyes (jaundice) can occur in SCD due to the breakdown of hemoglobin, but it is not directly related to the pain in the chest and joints described in the question.

Choice C:

"I've been having trouble breathing and feeling weak." While respiratory symptoms and weakness can occur in SCD, they are not the primary clinical manifestations associated with pain in the chest and joints. Painful erections and impotence are more directly related to SCD complications, such as priapism, which is a medical emergency and requires prompt attention.

Choice D:

"I've had painful erections and impotence." Painful erections and impotence are potential complications of sickle cell disease (SCD), particularly due to priapism, a condition where blood becomes trapped in the penis. This can lead to severe pain and, if not treated promptly, permanent erectile dysfunction. Therefore, the nurse should be vigilant for these clinical manifestations to address them promptly.

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Question 7:

A client with SCD presents to the healthcare facility with symptoms of fatigue, weakness, and dizziness.
The nurse is concerned about potential complications.
Which additional clinical manifestations should the nurse assess for?

A. "I've been having vision problems lately.” B. "I've had delayed growth in my child.” C. "I had a stroke a few years ago.” D. "I'm experiencing chest pain, fever, and cough.”

Answer and Explanation

Choice A:

"I've been having vision problems lately." Rationale: Vision problems are not directly associated with complications of sickle cell disease (SCD) SCD primarily affects the blood and vascular system, leading to symptoms such as anemia, pain crises, and organ damage. Vision problems may be caused by other underlying conditions, but they are not a typical manifestation of SCD complications.

Choice B:

"I've had delayed growth in my child." Rationale: Delayed growth in a child could be associated with SCD, as it may be a result of chronic anemia and inadequate oxygen delivery to tissues. However, it is not a direct clinical manifestation of complications. Other more common complications, such as pain crises, acute chest syndrome, or organ damage, should be assessed first to determine the extent of the disease's impact on the patient's health.

Choice C:

"I had a stroke a few years ago." Rationale: This statement is significant because stroke is a known complication of sickle cell disease. SCD can lead to the occlusion of blood vessels, including those in the brain, resulting in stroke. Therefore, the nurse should assess for any neurological deficits and gather more information about the stroke episode to assess its severity and potential impact on the patient's current condition.

Choice D:

"I'm experiencing chest pain, fever, and cough." Rationale: Chest pain, fever, and cough are indicative of acute chest syndrome (ACS), which is a severe complication of SCD. ACS can lead to respiratory distress and is considered a medical emergency. The presence of these symptoms warrants immediate assessment and intervention, making choice D the correct answer.

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Question 8:

(Select all that apply):
A nurse is performing a comprehensive assessment of a patient with SCD.
Which aspects of the nursing assessment should the nurse prioritize?

A. Gathering information about the patient's personal and family history of SCD or trait. B. Assessing the location, intensity, and duration of pain. C. Measuring the patient's temperature, pulse, and blood pressure. D. Inspecting for signs of anemia and jaundice during the physical examination. E. Conducting genetic testing to confirm the diagnosis of SCD.

Answer and Explanation

Choice A:

Gathering information about the patient's personal and family history of SCD or trait. Rationale: Understanding the patient's personal and family history of SCD or the sickle cell trait is crucial in assessing the risk and potential complications associated with the disease. It helps identify genetic factors, family support, and the likelihood of the patient experiencing complications related to SCD.

Choice B:

Assessing the location, intensity, and duration of pain. Rationale: Pain is a hallmark symptom of SCD, and assessing the location, intensity, and duration of pain is essential in managing and monitoring the patient's condition. This information helps healthcare providers determine the severity of the pain crisis, make treatment decisions, and assess treatment effectiveness.

Choice C:

Measuring the patient's temperature, pulse, and blood pressure. Rationale: While vital signs are important components of a nursing assessment, they are not specific to sickle cell disease. Monitoring vital signs is a routine practice in healthcare but may not provide specific information about the disease's complications or progression.

Choice D:

Inspecting for signs of anemia and jaundice during the physical examination. Rationale: Anemia and jaundice are common clinical manifestations of SCD. Anemia results from the destruction of sickled red blood cells, while jaundice occurs due to the breakdown of these cells and the release of bilirubin. Assessing for signs of anemia and jaundice, such as pallor and yellowing of the skin and sclera, is essential in monitoring the patient's overall health.

Choice E:

Conducting genetic testing to confirm the diagnosis of SCD. Rationale: Genetic testing is essential for diagnosing sickle cell disease, but it is typically performed before the patient is confirmed to have SCD. Once a diagnosis is established, genetic testing may not be necessary for routine assessment. It is crucial in the initial diagnostic phase but is not a part of ongoing nursing assessment.

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Question 9:

A client with suspected SCD is undergoing diagnostic evaluation.
Which laboratory test is essential for confirming the diagnosis and identifying the presence of abnormal hemoglobins?

A. Complete blood count (CBC) B. Peripheral blood smear. C. Solubility test or sickling test. D. Imaging studies, such as a chest x-ray.

Answer and Explanation

Choice A:

A is not the essential test for confirming the diagnosis.

Choice B:

Peripheral blood smear. Rationale: A peripheral blood smear can be useful in assessing the morphology of red blood cells, but it may not be specific enough to confirm the presence of abnormal hemoglobins or sickle cell disease definitively. It can provide supportive evidence but is not the primary diagnostic test.

Choice C:

Solubility test or sickling test. Rationale: The solubility test or sickling test is essential for confirming the diagnosis of sickle cell disease and identifying the presence of abnormal hemoglobins, specifically hemoglobin S (HbS) This test is the gold standard for diagnosing SCD, making choice C the correct answer.

Choice D:

Imaging studies, such as a chest x-ray. Rationale: Imaging studies like chest x-rays are not used as primary diagnostic tools for sickle cell disease. They may be employed to assess complications such as acute chest syndrome or other respiratory issues associated with SCD, but they do not confirm the diagnosis or identify abnormal hemoglobins.

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Question 10:

A nurse is reviewing the diagnostic evaluation process for a patient with SCD.
What is the primary purpose of hemoglobin electrophoresis in the diagnostic workup?

A. To measure the number and size of red blood cells. B. To identify the presence and amount of abnormal hemoglobins. C. To expose a blood sample to a deoxygenating agent. D. To analyze the DNA of a blood sample.

Answer and Explanation

Choice A:

Hemoglobin electrophoresis is not primarily used to measure the number and size of red blood cells. It focuses on the types of hemoglobin present in the blood, not their quantity or size.

Choice B:

The primary purpose of hemoglobin electrophoresis in the diagnostic workup for sickle cell disease (SCD) is to identify the presence and amount of abnormal hemoglobins. This test helps diagnose and differentiate various types of hemoglobinopathies, including SCD. Abnormal hemoglobins like hemoglobin S (HbS) are characteristic of SCD.

Choice C:

Hemoglobin electrophoresis does not expose a blood sample to a deoxygenating agent. Instead, it separates hemoglobin molecules based on their electrical charge, which is useful for identifying abnormal hemoglobins associated with SCD.

Choice D:

Hemoglobin electrophoresis does not analyze the DNA of a blood sample. It primarily focuses on the characterization of hemoglobin types and their proportions within the blood.

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Question 11:

A nurse is caring for a patient with sickle cell disease (SCD)
The patient is experiencing severe pain and requests pain relief.
Which nursing intervention is appropriate for managing the patient's pain?

A. Administering nonsteroidal anti-inflammatory drugs (NSAIDs) as prescribed. B. Providing emotional support and distraction techniques. C. Suggesting stem cell transplantation to cure the disease. D. Encouraging the patient to avoid triggers such as cold and stress.

Answer and Explanation

Choice A:

Administering nonsteroidal anti-inflammatory drugs (NSAIDs) may be part of the pain management plan for SCD, but it is not the most appropriate immediate intervention for severe pain. NSAIDs can be used for mild to moderate pain, but severe pain in SCD often requires stronger analgesics.

Choice B:

Providing emotional support and distraction techniques is appropriate for managing the patient's pain. SCD pain crises can be excruciating, and emotional support, along with distraction techniques, can help the patient cope with the pain. These interventions can be used alongside pain medications.

Choice C:

Suggesting stem cell transplantation to cure the disease is not an appropriate immediate intervention for managing severe pain. Stem cell transplantation is a complex and long-term treatment option for SCD, and it does not provide immediate relief from pain.

Choice D:

Encouraging the patient to avoid triggers such as cold and stress is important for preventing pain crises in SCD, but it is not the most appropriate immediate intervention for managing severe pain during a crisis. Pain relief measures should be prioritized to alleviate the patient's suffering.

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Question 12:

A client with SCD is receiving oxygen therapy through a nasal cannula.
What is the purpose of oxygen therapy in the management of SCD?

A. To provide relief from severe pain. B. To prevent tissue hypoxia. C. To administer analgesics. D. To induce relaxation.

Answer and Explanation

Choice A:

Oxygen therapy in the management of SCD is primarily aimed at preventing tissue hypoxia, not providing relief from severe pain. SCD patients may experience pain due to tissue ischemia caused by the sickling of red blood cells, and oxygen can help prevent this by increasing the oxygen-carrying capacity of the blood.

Choice B:

The primary purpose of oxygen therapy in the management of SCD is to prevent tissue hypoxia. SCD patients are at risk of vaso-occlusive crises and tissue damage due to reduced oxygen delivery. Supplemental oxygen helps maintain adequate tissue oxygenation and reduces the risk of complications.

Choice C:

Oxygen therapy is not administered for the purpose of directly administering analgesics. Analgesics are typically administered separately to manage pain in SCD patients.

Choice D:

Oxygen therapy is not used to induce relaxation. Its main goal is to improve oxygen saturation and prevent tissue hypoxia in SCD patients.

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Question 13:

(Select all that apply):
A nurse is educating a patient with sickle cell disease (SCD) and their family about the treatment and management of the condition.
Which interventions should the nurse include in the education and counseling plan? (Select three)

A. Medication adherence. B. Avoidance of cold and altitude. C. Blood transfusion as a first-line treatment. D. Stem cell transplantation for all SCD patients. E. Providing emotional support and coping strategies.

Answer and Explanation

Choice A:

Medication adherence is essential in the treatment and management of sickle cell disease (SCD) Patients with SCD often require medications such as hydroxyurea to reduce the frequency of painful crises and other complications. Non-adherence to medications can lead to worsened outcomes and increased morbidity. Educating the patient and their family about the importance of taking medications as prescribed is crucial.

Choice B:

Avoidance of cold and altitude is important for patients with SCD because exposure to cold temperatures and high altitudes can trigger vaso-occlusive crises. Cold can cause red blood cells to sickle more easily, leading to pain and tissue damage. Altitude can result in lower oxygen levels in the blood, exacerbating the risk of sickling. Educating the patient and their family about these environmental factors and strategies to minimize exposure is essential for SCD management.

Choice C:

Blood transfusion is not a first-line treatment for all SCD patients. It is typically reserved for specific indications, such as severe anemia or acute complications like stroke. Stem cell transplantation (

Choice D:

Stem cell transplantation is not recommended as a treatment for all SCD patients. It is considered a high-risk procedure with potential complications, and its suitability depends on factors such as patient age, disease severity, and the availability of a suitable donor. Therefore, it is not included as a standard intervention in the education and counseling plan for all SCD patients.

Choice E:

Providing emotional support and coping strategies is a crucial component of managing SCD. Patients with SCD often experience chronic pain, frequent hospitalizations, and a reduced quality of life. Emotional support can help them cope with the physical and emotional challenges associated with the disease. Teaching patients and their families how to manage stress, anxiety, and depression can improve their overall well-being.

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Question 14:

A nurse is assessing a patient with SCD who has just received a blood transfusion.
What should the nurse monitor for during and after the transfusion to ensure the patient's safety?

A. Signs of transfusion reaction such as fever, chills, rash, or dyspnea. B. The patient's response to music therapy. C. The patient's fluid intake and output. D. The patient's emotional state.

Answer and Explanation

Choice A:

Monitoring for signs of transfusion reaction is a critical nursing responsibility during and after a blood transfusion. Transfusion reactions, such as fever, chills, rash, or dyspnea, can occur due to various factors, including compatibility issues or bacterial contamination of the blood product. Detecting these signs promptly allows for immediate intervention, including stopping the transfusion and providing appropriate treatment. This ensures the patient's safety and well-being during the transfusion process.

Choice B:

Monitoring the patient's response to music therapy is not a standard nursing assessment during or after a blood transfusion. While music therapy can have benefits in managing pain and anxiety, it is not directly related to the safety of the transfusion process.

Choice C:

Monitoring the patient's fluid intake and output is essential in many clinical situations, but it is not specifically related to the safety of a blood transfusion. This parameter is more relevant in assessing the patient's hydration status and renal function.

Choice D:

Monitoring the patient's emotional state is important for overall patient care, but it is not a primary concern during and immediately after a blood transfusion. The focus during this time should be on detecting any adverse reactions or complications related to the transfusion itself.

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Question 15:

A client with SCD has an elevated serum ferritin level.
What is the significance of an elevated serum ferritin level in a patient with SCD?

A. It indicates adequate iron stores. B. It suggests iron deficiency anemia. C. It may signal iron overload. D. It reflects normal hemoglobin levels.

Answer and Explanation

Choice A:

An elevated serum ferritin level does not indicate adequate iron stores. In fact, it suggests the opposite, as it implies an excess of stored iron in the body.

Choice B:

An elevated serum ferritin level is not indicative of iron deficiency anemia. Iron deficiency anemia is characterized by low serum ferritin levels, as ferritin stores are depleted in this condition.

Choice C:

An elevated serum ferritin level in a patient with sickle cell disease (SCD) may signal iron overload. Iron overload is a potential complication of chronic blood transfusions, which are often required in SCD to treat anemia and prevent complications. Excessive iron accumulation can lead to organ damage, particularly in the liver, heart, and endocrine glands. Monitoring and managing iron levels, including serum ferritin, are essential in SCD patients who receive regular transfusions.

Choice D:

An elevated serum ferritin level does not reflect normal hemoglobin levels. Ferritin is a marker of iron storage and does not directly indicate the hemoglobin level, which measures the oxygen-carrying capacity of red blood cells. .

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Sickle Cell Disease

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