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?
"Sickle cell disease results from a dominant genetic mutation.”..
"Both parents must pass on the mutated gene for sickle cell disease to manifest.”..
"The mutation in the HBB gene leads to the production of normal hemoglobin.”..
"Sickle cell disease is inherited in an autosomal dominant manner.”..
The Correct Answer is B
Choice A rationale:
"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 rationale:
"Both parents must pass on the mutated gene for sickle cell disease to manifest.”.
This is The correct answer.
Sickle cell disease is inherited in an autosomal recessive manner, which means that it only manifests when a person inherits two copies of the mutated HBB gene, one from each parent.
If only one copy of the gene is inherited, the individual is a carrier (sickle cell trait) and does not have the disease.
Choice C rationale:
"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 rationale:
"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 Choice B.
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Naxlex Comprehensive Predictor Exams
Related Questions
Correct Answer is A
Explanation
Administering pain medication as prescribed.
Choice A rationale:
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 rationale:
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 rationale:
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 rationale:
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.
Correct Answer is D
Explanation
Choice A rationale:
"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 rationale:
"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 rationale:
"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 rationale:
"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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