the parents of a 3 year old boy who has duchenne muscular dystrophy ask how can our son have this disease we are wondering if we should have any more
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Nursing Elites

HESI RN

Pediatric HESI

1. What information should the nurse provide to parents of a 3-year-old boy with Duchenne muscular dystrophy who inquire about the disease and future children?

Correct answer: A

Rationale: Duchenne muscular dystrophy is an X-linked recessive disorder caused by mutations in the DMD gene on the X chromosome. This disorder primarily affects males because they have one X chromosome, inherited from their mothers, who may be carriers of the mutated gene. Females have two X chromosomes, providing a protective effect as the normal gene on one X chromosome can compensate for the mutated gene on the other. Therefore, the nurse should explain to the parents that Duchenne muscular dystrophy is an inherited X-linked recessive disorder, which is why their son has the disease and why there is a risk of passing it on to future sons. Choice B is incorrect as it inaccurately implies that the lack of dystrophin in mothers impacts their sons' muscle groups. Choice C is incorrect as it suggests a viral infection caused the muscle damage, which is not the case with Duchenne muscular dystrophy. Choice D is incorrect as it attributes the muscle weakness to birth trauma instead of the genetic nature of the disorder.

2. The healthcare provider is preparing to administer a vaccine to a 5-year-old child. The child is visibly anxious and asks if the shot will hurt. What is the healthcare provider’s best response?

Correct answer: A

Rationale: When a child expresses fear or anxiety about receiving a vaccination, it is essential for the healthcare provider to provide honest and reassuring information. Choice A acknowledges the potential for some discomfort but also reassures the child that it will be over quickly. This response validates the child's feelings while also preparing them for the procedure. Choices B, C, and D either provide false reassurance, dismiss the child's feelings, or suggest avoidance, which are not appropriate responses in this situation.

3. A child with sickle cell anemia is being treated for a vaso-occlusive crisis. Which intervention should the practical nurse (PN) implement?

Correct answer: B

Rationale: Encouraging increased fluid intake is crucial in managing vaso-occlusive crises in patients with sickle cell anemia. Dehydration can worsen these crises, so adequate hydration is essential to prevent complications and improve outcomes. Applying cold packs to painful areas may exacerbate vaso-occlusive crises by causing vasoconstriction. Administering high doses of vitamin C is not directly indicated for vaso-occlusive crises in sickle cell anemia. Providing low-calorie meals is not the priority during a vaso-occlusive crisis; maintaining adequate nutrition is important, but hydration takes precedence in this situation.

4. When caring for a child with Kawasaki disease, which symptom is the most significant for making this diagnosis?

Correct answer: C

Rationale: Erythema of the hands and feet is a key diagnostic criterion for Kawasaki disease. This, along with other symptoms like fever and strawberry tongue, helps in making the diagnosis. While desquamation of the palms and soles, cervical lymphadenopathy, and strawberry tongue are associated with Kawasaki disease, the presence of erythema of the hands and feet is particularly significant in diagnosing this condition.

5. A 3-year-old with a congenital heart defect has had a steady decrease in heart rate, now at 76 bpm from 110 bpm four hours ago. Which additional finding should be reported immediately to a healthcare provider?

Correct answer: D

Rationale: A significant drop in heart rate and blood pressure should be reported immediately as it may indicate worsening of the congenital heart defect. The blood pressure of 70/40 is dangerously low and requires immediate attention to prevent further complications. Oxygen saturation of 94% is within an acceptable range and does not indicate immediate danger. A respiratory rate of 25 breaths/minute is slightly elevated but not a critical finding. Urine output of 20 mL/hr is low but may not be the most concerning finding in this scenario compared to the drop in blood pressure.

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