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. A mother brings her 3-month-old infant to the clinic because the baby does not sleep through the night. Which finding is most significant in planning care for this family?

Correct answer: D

Rationale: Severe skin breakdown in the diaper area is a significant finding indicating a potential health issue that needs immediate attention. It may be a sign of a skin condition, such as a diaper rash, which can cause discomfort and pain for the infant. Addressing this concern promptly is crucial to prevent further complications and ensure the baby's well-being. The other choices may also be important in assessing the overall situation of the family, but in terms of immediate care for the infant, the severe skin breakdown takes priority.

3. The nurse is measuring the frontal occipital circumference (FOC) of a 3-month-old infant and notes that the FOC has increased by 5 inches since birth, and the child's head appears large in relation to body size. Which action is most important for the nurse to take next?

Correct answer: B

Rationale: Palpating the anterior fontanel for tension and bulging is crucial in assessing for increased intracranial pressure. In this scenario, the infant's large head size and rapid increase in the frontal occipital circumference raise concerns for potential issues such as hydrocephalus. Measuring the head-to-toe length (Choice A) is not the priority when assessing for increased intracranial pressure. Observing for sunken eyes (Choice C) is more indicative of dehydration rather than increased intracranial pressure. While plotting the measurement on the infant's growth chart (Choice D) is important for tracking growth, it does not address the immediate concern of assessing for increased intracranial pressure.

4. What is the best response for a two-year-old boy who begins to cry when the mother starts to leave?

Correct answer: D

Rationale: The best response for a two-year-old boy who begins to cry when the mother starts to leave is to wave bye-bye to mommy. This action helps the child understand that the separation is temporary and gives him a sense of closure. Choice A is the correct answer. Choice B is incorrect as it generalizes the behavior of two-year-olds. Choice C may invalidate the child's feelings by telling him to 'be a big boy' instead of acknowledging his emotions and providing comfort.

5. A child with acute lymphocytic leukemia (ALL) who is receiving chemotherapy via a subclavian IV infusion has an oral temperature of 103 degrees. In assessing the IV site, the nurse determines that there are no signs of infection at the site. Which intervention is the most important for the nurse to implement?

Correct answer: A

Rationale: Obtaining a specimen for blood cultures is crucial in this situation as it helps identify the source of infection, if present, and guide appropriate treatment. This is important in a child with leukemia receiving chemotherapy to prevent potential complications and ensure timely intervention. Assessing the CBC may provide overall information on the child's condition but may not specifically identify a potential infection. Monitoring the oral temperature is important but obtaining blood cultures takes precedence in this scenario. Administering acetaminophen can help reduce fever but does not address the need to identify a possible infection source.

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