Review Sickle Cell Disease

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Sickle Cell Disease

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Question 1:

A nurse is explaining the etiology of sickle cell disease to a group of nursing students.
Which statement accurately describes the genetic basis of this condition?

A. "Sickle cell disease results from a dominant genetic mutation.”.. B. "Both parents must pass on the mutated gene for sickle cell disease to manifest.”.. C. "The mutation in the HBB gene leads to the production of normal hemoglobin.”.. D. "Sickle cell disease is inherited in an autosomal dominant manner.”..

Answer and Explanation

Choice A:

"Sickle cell disease results from a dominant genetic mutation.”. This statement is not accurate. Sickle cell disease is not caused by a dominant genetic mutation. It is an autosomal recessive genetic disorder, meaning that both parents must pass on the mutated gene for the disease to manifest. In this condition, a person must inherit two copies of the mutated HBB gene (one from each parent) to have sickle cell disease.

Choice B:

Choice C:

"The mutation in the HBB gene leads to the production of normal hemoglobin.”. This statement is incorrect. The mutation in the HBB gene results in the production of abnormal hemoglobin, known as hemoglobin S (HbS). This abnormal hemoglobin is responsible for the characteristic changes in red blood cells seen in sickle cell disease.

Choice D:

"Sickle cell disease is inherited in an autosomal dominant manner.”. This statement is also incorrect. Sickle cell disease is not inherited in an autosomal dominant manner. It is an autosomal recessive genetic disorder, as explained in

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Question 2:

A client with sickle cell disease asks the nurse about the effects of hemoglobin S on red blood cells.
Which statement by the nurse accurately explains the pathophysiology of this condition?

A. "Hemoglobin S causes red blood cells to become more flexible.”.. B. "Sickle-shaped cells improve blood flow in the body.”.. C. "Chronic hemolysis and anemia are not associated with sickle cell disease.”.. D. "Sickle-shaped cells can block blood vessels and impair blood flow.”..

Answer and Explanation

Choice A:

"Hemoglobin S causes red blood cells to become more flexible.”. This statement is not accurate. Hemoglobin S (HbS) actually causes red blood cells to become less flexible and take on a characteristic sickle shape. This change in shape is a key feature of sickle cell disease and can lead to various complications.

Choice B:

"Sickle-shaped cells improve blood flow in the body.”. This statement is incorrect. Sickle-shaped cells do not improve blood flow. In fact, they can impair blood flow by blocking blood vessels. The abnormal shape of these cells makes them more likely to get stuck in small blood vessels, leading to vaso-occlusive crises and other complications.

Choice C:

"Chronic hemolysis and anemia are not associated with sickle cell disease.”. This statement is also incorrect. Chronic hemolysis (the breakdown of red blood cells) is a hallmark of sickle cell disease. The abnormal shape of sickle cells makes them more fragile, leading to their premature destruction, which results in anemia.

Choice D:

"Sickle-shaped cells can block blood vessels and impair blood flow.”. This is The correct answer. Sickle-shaped red blood cells can block blood vessels, leading to vaso-occlusive crises and impaired blood flow. This is a key part of the pathophysiology of sickle cell disease.

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Question 3:

A nurse is teaching a client with sickle cell disease about self-care measures.
Select all the appropriate self-care strategies for this client:.

A. Increasing fluid intake to stay hydrated. B. Engaging in regular strenuous physical exercise. C. Avoiding extreme temperature conditions. D. Taking prescribed medications as directed. E. Smoking tobacco to alleviate pain.

Answer and Explanation

Choice A:

"Increasing fluid intake to stay hydrated.”. Staying hydrated is an important self-care strategy for individuals with sickle cell disease. Dehydration can increase the risk of vaso-occlusive crises and other complications. Adequate fluid intake helps to prevent the excessive sickling of red blood cells, which can block blood vessels.

Choice B:

"Engaging in regular strenuous physical exercise.”. Engaging in regular strenuous physical exercise is not an appropriate self-care strategy for individuals with sickle cell disease. Intense exercise can increase the risk of vaso-occlusive crises and should be done with caution. Low-impact, moderate exercise may be more suitable for these individuals.

Choice C:

"Avoiding extreme temperature conditions.”. Avoiding extreme temperature conditions is a sensible self-care strategy for individuals with sickle cell disease. Extreme cold or heat can trigger vaso-occlusive crises and other complications. Maintaining a comfortable temperature can help prevent these issues.

Choice D:

"Taking prescribed medications as directed.”. Taking prescribed medications as directed is crucial for managing sickle cell disease. This may include medications to manage pain, prevent infections, and reduce complications. Adhering to the prescribed treatment plan is essential for improving the quality of life and preventing crises.

Choice E:

"Smoking tobacco to alleviate pain.”. Smoking tobacco is not an appropriate self-care strategy for individuals with sickle cell disease. Smoking can worsen the condition and increase the risk of complications. It is important to avoid smoking and any other activities that can harm the lungs or blood vessels in individuals with sickle cell disease.

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Question 4:

A client with sickle cell disease is experiencing a sickling crisis.
What interventions should the nurse prioritize when providing care for this client?

A. Administering pain medication as prescribed. B. Encouraging the client to engage in intense physical activity. C. Keeping the room at a very low temperature. D. Encouraging the client to smoke to relieve pain.

Answer and Explanation

Choice A:

Administering pain medication as prescribed is the highest priority when caring for a client experiencing a sickling crisis in sickle cell disease. Sickle cell crises are characterized by severe pain due to vaso-occlusion, and the nurse's primary responsibility is to alleviate this pain. Pain medication helps to relieve the suffering and improve the client's overall comfort. It is crucial to administer medication promptly to prevent further complications and distress.

Choice B:

Encouraging the client to engage in intense physical activity is not an appropriate intervention during a sickling crisis. Physical activity can exacerbate the crisis by increasing oxygen demand, which may worsen tissue hypoxia. It is essential to encourage rest and limit physical exertion to minimize the risk of complications.

Choice C:

Keeping the room at a very low temperature is not a recommended intervention for a sickle cell crisis. Cold temperatures can cause vasoconstriction, which may further worsen vaso-occlusion. Maintaining a warm and comfortable environment is more suitable to help ease the client's pain and discomfort.

Choice D:

Encouraging the client to smoke to relieve pain is not a safe or appropriate intervention. Smoking can lead to vasoconstriction and worsen the client's condition. It is essential to promote healthy behaviors and provide effective pain management rather than suggesting harmful practices like smoking.

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Question 5:

A nurse is assessing a client with sickle cell disease.
Which clinical manifestations should the nurse be particularly attentive to during the assessment?

A. Pale skin and nail beds. B. Elevation in blood pressure. C. Swelling of the lower extremities. D. Jaundice and dark urine.

Answer and Explanation

Choice A:

Pale skin and nail beds are not typically the primary clinical manifestations of sickle cell disease during a crisis. Sickle cell crises are primarily characterized by vaso-occlusive events leading to severe pain. While paleness may occur due to anemia associated with sickle cell disease, it is not the hallmark manifestation during a crisis.

Choice B:

An elevation in blood pressure is not a specific clinical manifestation of sickle cell disease. In fact, individuals with sickle cell disease may experience a drop in blood pressure during a crisis due to reduced blood flow and organ perfusion. Elevated blood pressure is more likely to be associated with other health conditions or stressors.

Choice C:

Swelling of the lower extremities is not a typical clinical manifestation of sickle cell disease during a crisis. The hallmark features of a sickling crisis include pain, jaundice, and, in some cases, dark urine, which results from the breakdown of sickled red blood cells. Swelling may occur in other conditions but is not specific to sickle cell disease.

Choice D:

Jaundice and dark urine are crucial clinical manifestations to monitor in a client with sickle cell disease during a crisis. Jaundice indicates the breakdown of red blood cells, which is a common occurrence during vaso-occlusive events. Dark urine results from the excretion of bilirubin, a byproduct of red blood cell breakdown. These manifestations provide important diagnostic information and guide treatment.

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Question 6:

A nurse is conducting an assessment of a patient with sickle cell disease who presents with severe pain in the joints and abdomen.
The nurse observes the patient wincing in pain and states, "I feel a sharp, stabbing pain in my abdomen and my joints ache terribly.”.
What is the most likely clinical manifestation of sickle cell disease that the nurse is assessing in this patient?

A. Impaired immune function. B. Organ damage. C. Painful vaso-occlusive crisis. D. Delayed growth and development.

Answer and Explanation

Choice A:

Impaired immune function is not the primary clinical manifestation assessed in a client with sickle cell disease experiencing severe pain in the joints and abdomen. While sickle cell disease can affect the immune system, the immediate concern in this scenario is addressing the intense pain caused by vaso-occlusion.

Choice B:

Organ damage is a potential long-term complication of sickle cell disease, but it is not the primary clinical manifestation being assessed in this patient. The description of sharp, stabbing pain in the abdomen and joint aches is indicative of a vaso-occlusive crisis, and the focus should be on pain management and addressing the crisis itself.

Choice C:

The nurse is most likely assessing a painful vaso-occlusive crisis in this patient. The description of sharp, stabbing pain and joint discomfort is consistent with the pain experienced during a vaso-occlusive crisis. These crises are a hallmark feature of sickle cell disease, characterized by the occlusion of blood vessels by sickled red blood cells, resulting in severe pain.

Choice D:

Delayed growth and development are long-term consequences of sickle cell disease, primarily seen in pediatric patients. In this scenario, the acute issue is the pain and discomfort the patient is currently experiencing, which is more indicative of a vaso-occlusive crisis.

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Question 7:

A nurse is reviewing the medical history of a child diagnosed with sickle cell disease.
The child's parent reports, "My child always looks pale and gets tired very easily.”.
Based on this information, which complication of sickle cell disease is the nurse most likely assessing in this patient?

A. Pulmonary Hypertension. B. Anemia. C. Hematuria. D. Functional Asplenia.

Answer and Explanation

Choice A:

Pulmonary Hypertension Sickle cell disease is primarily characterized by the presence of abnormal hemoglobin (HbS), leading to the formation of sickle-shaped red blood cells. While pulmonary hypertension can occur in patients with sickle cell disease, it is not the most likely complication based on the presented symptoms. The patient's paleness and easy fatigue are more indicative of anemia, which is a common complication in sickle cell disease. Anemia results from a reduced number of functional red blood cells, leading to reduced oxygen-carrying capacity and fatigue.

Choice C:

Hematuria Hematuria, the presence of blood in the urine, can occur in sickle cell disease, but it is not the most likely complication based on the child's reported symptoms of paleness and easy fatigue. Hematuria may be associated with other complications such as kidney damage, but it is not the primary concern in this case.

Choice D:

Functional Asplenia Functional asplenia refers to the impaired function of the spleen, which is a common complication in sickle cell disease. However, the symptoms described by the child's parent, including paleness and easy fatigue, are more indicative of anemia, which is a consequence of sickle cell disease.

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Question 8:

(Select all that apply).
A nurse is performing a comprehensive assessment of a patient with sickle cell disease.
Which of the following assessments are essential for monitoring signs and symptoms related to sickle cell disease? .

A. Assessment of vital signs, including temperature, heart rate, blood pressure, and respiratory rate. B. Evaluation of pain intensity and location. C. Assessment of neurologic status, including cognitive function and sensory perception. D. Evaluation of psychosocial well-being and coping mechanisms.

Answer and Explanation

Choice A:

None

Choice B:

Renal Failure Renal failure is not the most likely complication in this case. The client's sharp chest pain, aggravated by deep breaths, is more consistent with the symptoms of Acute Chest Syndrome. This syndrome is a common complication of sickle cell disease and is characterized by chest pain, fever, cough, and shortness of breath. While kidney complications can occur in sickle cell disease, they are not the primary concern based on the client's presentation.

Choice C:

Acute Chest Syndrome is a more pressing concern due to its potential to lead to severe respiratory distress.

Choice D:

Functional Asplenia Functional asplenia may contribute to the risk of infections in individuals with sickle cell disease, but it is not the primary cause of the client's current symptoms. The client's chest pain and difficulty breathing are more indicative of Acute Chest Syndrome, a serious and potentially life-threatening complication of sickle cell disease.

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Question 9:

A client diagnosed with sickle cell disease reports having pain in the chest and difficulty breathing.
The client states, "I have sharp chest pain, and it hurts more when I take a deep breath.”.
Which complication of sickle cell disease is the client likely experiencing?

A. Acute Chest Syndrome. B. Renal Failure. C. Cholelithiasis. D. Functional Asplenia.

Answer and Explanation

Choice A:

Assessment of vital signs, including temperature, heart rate, blood pressure, and respiratory rate Monitoring vital signs is crucial for assessing a patient's overall health and detecting any signs of deterioration. Patients with sickle cell disease can experience vaso-occlusive crises, which may result in changes in vital signs, such as an elevated heart rate and respiratory rate. Elevated temperature may indicate an infection, which is another concern in sickle cell disease.

Choice B:

Evaluation of pain intensity and location Sickle cell disease is associated with recurrent painful vaso-occlusive crises, where sickled red blood cells block blood vessels, leading to pain. Assessing the intensity and location of pain is essential to determine the severity of the crisis and to provide appropriate pain management.

Choice C:

Assessment of neurologic status, including cognitive function and sensory perception Sickle cell disease can lead to complications such as strokes, which may result in neurological deficits. Monitoring cognitive function and sensory perception is crucial to detect any neurological complications early and provide timely interventions.

Choice D:

Evaluation of psychosocial well-being and coping mechanisms Monitoring psychosocial well-being and coping mechanisms is essential for holistic care but may not directly relate to monitoring signs and symptoms of sickle cell disease. The primary focus should be on physical assessments and symptoms related to the disease itself. Now, let's provide rationales for The correct answers:

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Question 10:

A nurse is assessing a patient with sickle cell disease who complains of frequent infections and fatigue.
The patient states, "I always feel tired, and I get sick easily, especially with respiratory infections.”.
Based on this information, what aspect of sickle cell disease is the nurse likely evaluating in this patient?

A. Chronic hypoxia. B. Impaired immune function. C. Delayed growth and development. D. Vascular occlusion.

Answer and Explanation

Choice A:

Chronic hypoxia. Chronic hypoxia is not the primary concern described by the patient. While individuals with sickle cell disease can experience episodes of hypoxia, the patient's main complaints are related to fatigue and frequent infections. Chronic hypoxia could be a consequence of the disease, but it is not the aspect the nurse is likely evaluating in this case.

Choice B:

Impaired immune function. The patient's complaints of feeling tired and getting sick easily, especially with respiratory infections, suggest impaired immune function. Sickle cell disease can affect the spleen, which plays a crucial role in the immune system. Many individuals with this condition experience functional asplenia, making them more susceptible to infections, particularly encapsulated bacteria like Streptococcus pneumoniae. This compromised immune function is a significant concern for patients with sickle cell disease, and the nurse should assess and address it.

Choice C:

Delayed growth and development. Delayed growth and development are not the primary concerns mentioned by the patient in this scenario. While children with sickle cell disease can experience delayed growth and development due to chronic anemia and other factors, the patient's main complaints are related to fatigue and frequent infections, which are more indicative of impaired immune function.

Choice D:

Vascular occlusion. Vascular occlusion can be a significant issue in sickle cell disease, leading to pain and tissue damage, but it is not the aspect the nurse is likely evaluating in this patient based on the information provided. The patient's chief complaints are related to fatigue and frequent infections, suggesting that impaired immune function is the primary concern.

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Question 11:

A nurse is caring for a patient with sickle cell disease and is discussing pain management strategies.
The nurse states, "You may find relief by taking prescribed pain medications promptly and ensuring they are effective.”.
What nursing intervention is the nurse describing?

A. Administering antibiotics prophylaxis to prevent infections. B. Encouraging adequate hydration to prevent dehydration and promote blood flow. C. Assisting with activities of daily living and mobility to prevent complications and maintain independence. D. Providing emotional support and counseling to help patients cope with the chronic nature of the disease.

Answer and Explanation

Choice A:

Administering antibiotics prophylaxis to prevent infections. This option is not the intervention described by the nurse in the scenario. The nurse is discussing pain management strategies and the importance of taking prescribed pain medications promptly. Antibiotics prophylaxis is a different aspect of care for sickle cell disease that focuses on preventing infections, particularly those caused by encapsulated bacteria.

Choice B:

Encouraging adequate hydration to prevent dehydration and promote blood flow. While adequate hydration is essential for individuals with sickle cell disease to prevent dehydration and promote blood flow, it is not the intervention described by the nurse in this scenario. The nurse is specifically addressing the importance of taking pain medications and ensuring their effectiveness.

Choice C:

Assisting with activities of daily living and mobility to prevent complications and maintain independence. Assisting with activities of daily living and mobility is an important aspect of care for patients with sickle cell disease, but it is not the intervention mentioned by the nurse in this scenario. The nurse is emphasizing the need for effective pain management, which is crucial for patients experiencing vaso-occlusive crises.

Choice D:

Providing emotional support and counseling to help patients cope with the chronic nature of the disease. The nurse is describing the importance of providing emotional support and counseling to help patients cope with the chronic nature of sickle cell disease and the pain associated with it. This intervention is critical for addressing the emotional and psychological aspects of the disease and ensuring that patients can manage their pain effectively. It also promotes overall well-being and quality of life for individuals with this chronic condition.

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Question 12:

A client diagnosed with sickle cell disease is seeking advice on avoiding triggers for vaso-occlusive crises.
The patient asks, "What activities should I avoid to prevent these painful episodes?" What education is the nurse likely to provide?

A. "It's crucial to stay away from extreme temperatures and physical exertion to prevent vaso-occlusive crises.”.. B. "You should engage in regular physical activities to keep your body in good shape.”.. C. "Avoiding certain foods can significantly reduce the frequency of painful episodes.”.. D. "Taking hot showers or baths can help prevent vaso-occlusive crises.”..

Answer and Explanation

Choice A:

"It's crucial to stay away from extreme temperatures and physical exertion to prevent vaso-occlusive crises.”. Extreme temperatures (both hot and cold) can trigger vaso-occlusive crises in patients with sickle cell disease. Cold temperatures can cause blood vessels to constrict, leading to reduced blood flow and increased risk of blockages, while hot temperatures can lead to dehydration, which can also contribute to vaso-occlusive events. Physical exertion can increase the risk of sickling, as the cells are more likely to become misshapen and block blood vessels during periods of increased activity.

Choice B:

"You should engage in regular physical activities to keep your body in good shape.”. While physical activity is generally encouraged for overall health, patients with sickle cell disease need to be cautious about the type and intensity of exercise they engage in. Strenuous or high-impact activities can increase the risk of vaso-occlusive crises. The nurse is likely to advise the patient to engage in regular, moderate physical activity that does not overexert the body.

Choice C:

"Avoiding certain foods can significantly reduce the frequency of painful episodes.”. While a healthy diet is important for individuals with sickle cell disease, avoiding specific foods is not the primary strategy for preventing vaso-occlusive crises. The patient's question is more focused on activities and lifestyle factors that can trigger these painful episodes. Diet plays a role in overall health and can indirectly affect the frequency of crises, but it is not the primary concern in this context.

Choice D:

"Taking hot showers or baths can help prevent vaso-occlusive crises.”. Taking hot showers or baths is not a recommended strategy for preventing vaso-occlusive crises. In fact, exposure to hot water can lead to dehydration, which is a risk factor for sickle cell crises. The nurse is likely to advise the patient to use warm, not hot, water for bathing and to stay well-hydrated but not to rely on hot showers or baths as a preventive measure for vaso-occlusive events.

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Question 13:

(Select All That Apply).
A nurse is collaborating with a healthcare team to develop an individualized care plan for a patient with sickle cell disease.
Which interventions are appropriate for the nurse to include in the plan to manage the patient's condition? .

A. Administering blood transfusions to increase the number of healthy red blood cells. B. Providing genetic counseling and education for individuals and families affected by sickle cell disease. C. Educating patients about the importance of regular follow-up appointments and adherence to prescribed treatment regimens. D. Offering oxygen therapy for patients with acute chest syndrome or respiratory distress. E. Encouraging patients to engage in high-intensity physical activities to improve overall health.

Answer and Explanation

Choice A:

Administering blood transfusions to increase the number of healthy red blood cells. This choice is not appropriate for the management of sickle cell disease because blood transfusions are typically reserved for specific situations, such as severe anemia, acute complications, or in patients with frequent vaso-occlusive crises. It is not a routine intervention for all patients with sickle cell disease.

Choice B:

Providing genetic counseling and education for individuals and families affected by sickle cell disease. Genetic counseling and education are essential for patients and families affected by sickle cell disease. This helps individuals understand the inheritance pattern, make informed family planning decisions, and manage the emotional and psychological aspects of living with a genetic disorder. Education can also include information on preventive measures.

Choice C:

Educating patients about the importance of regular follow-up appointments and adherence to prescribed treatment regimens. Regular follow-up appointments and adherence to prescribed treatment regimens are crucial in managing sickle cell disease. Patients need ongoing monitoring and support to detect complications early, manage pain, and optimize their overall health. Non-adherence can lead to exacerbations and complications.

Choice D:

Offering oxygen therapy for patients with acute chest syndrome or respiratory distress. Oxygen therapy is vital for patients with sickle cell disease, especially when they experience acute chest syndrome or respiratory distress. This intervention helps improve oxygenation, alleviate respiratory distress, and prevent further complications. Adequate oxygenation is essential to minimize tissue damage and support the body during these acute events.

Choice E:

Encouraging patients to engage in high-intensity physical activities to improve overall health. Encouraging high-intensity physical activities is not appropriate for patients with sickle cell disease, as it can trigger vaso-occlusive crises and increase the risk of complications. Patients with sickle cell disease are typically advised to engage in moderate, low-impact physical activities to maintain their health without exacerbating their condition.

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Question 14:

A client with sickle cell disease is experiencing acute chest syndrome and is having difficulty breathing.
The nurse decides to initiate oxygen therapy.
What is the rationale behind this intervention?

A. To increase the number of healthy red blood cells in the patient's body. B. To reduce the frequency of pain crises and complications. C. To improve oxygenation and alleviate respiratory distress. D. To prevent infections and enhance the immune system response.

Answer and Explanation

Choice A:

To increase the number of healthy red blood cells in the patient's body. The primary goal of oxygen therapy in acute chest syndrome is not to increase the number of healthy red blood cells but to provide immediate relief by improving oxygenation. Increasing healthy red blood cell production would take time and is not a suitable acute intervention.

Choice B:

To reduce the frequency of pain crises and complications. Oxygen therapy is not primarily aimed at reducing the frequency of pain crises. It is used to address acute respiratory distress and improve oxygenation, which is essential in acute chest syndrome.

Choice D:

To prevent infections and enhance the immune system response. Oxygen therapy is not administered to prevent infections or enhance the immune system response. Its main purpose is to address respiratory distress and hypoxia in patients with acute chest syndrome.

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Question 15:

A nurse is providing psychosocial support and counseling to a patient with sickle cell disease. The patient expresses feelings of anxiety and depression related to the chronic nature of the disease.
What is the primary goal of the nurse's intervention in this situation?

A. To encourage the patient to avoid stressful situations to minimize anxiety. B. To promote self-care strategies, such as stress management and pain management techniques. C. To address the emotional and psychological impact of the disease and provide support and coping strategies. D. To administer prescribed antidepressant medications to alleviate symptoms of depression.

Answer and Explanation

Choice A:

To encourage the patient to avoid stressful situations to minimize anxiety.
Avoiding stressful situations is not a comprehensive solution for managing anxiety and depression related to a chronic disease.
While stress management is important, the primary goal should be to address the emotional and psychological impact of the disease and provide support and coping strategies.

Choice B:

To promote self-care strategies, such as stress management and pain management techniques.
Promoting self-care strategies is a valuable component of addressing anxiety and depression, but the primary goal is to address the emotional and psychological impact of the disease.
This can include providing emotional support, counseling, and coping strategies beyond stress and pain management.

Choice D:

To administer prescribed antidepressant medications to alleviate symptoms of depression.
While administering antidepressant medications may be a treatment option, it is not the primary goal of the nurse's intervention.
The primary goal is to address the emotional and psychological impact and provide support and coping strategies.
Medications may be part of a broader treatment plan, but they are not the primary focus of nursing care.

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Sickle Cell Disease

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