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. When observing a distraught mother scolding her 3-year-old son for wetting his pants in the hallway of a pediatric unit, what initial action should the nurse take?

Correct answer: B

Rationale: In this situation, the nurse's initial action should be to provide disposable training pants to manage the immediate issue of wetting while also calming the mother. This approach addresses the current distressing situation and offers a practical solution to alleviate the mother's concerns.

3. A 2-year-old is admitted to the hospital with possible encephalitis, and a lumbar puncture is scheduled. Which information should the nurse provide this child concerning the procedure?

Correct answer: A

Rationale: The correct answer is A. Describing the side-lying, knees to chest position that must be assumed during the lumbar puncture procedure is essential as it helps the child understand what to expect, promotes cooperation, and reduces anxiety. This position is necessary for the procedure to be performed safely and effectively. Choice B is incorrect because mentioning loud clicking noises may increase the child's anxiety. Choice C is incorrect because there may be restrictions on activity after the procedure, depending on individual cases. Choice D is also incorrect as it provides information about fluid intake restrictions that are not directly related to the procedure itself.

4. What information should the nurse provide the parents of a 3-year-old boy with Duchenne muscular dystrophy (DMD) who are concerned about having more children?

Correct answer: A

Rationale: The correct answer is A. Duchenne muscular dystrophy is an inherited X-linked recessive disorder that primarily affects male children in the family. Since it is X-linked, sons inherit the mutation from their mothers who are carriers of the abnormal gene. Therefore, the nurse should explain to the parents that any future sons they have would have a 50% chance of inheriting the mutation and having DMD, while daughters would have a 50% chance of being carriers like the mother.

5. The healthcare provider plans to administer 10 mcg/kg of digoxin elixir as a loading dose to a child who weighs 55 pounds. Digoxin is available as an elixir of 50 mcg/ml. How many milliliters of the digoxin elixir should the healthcare provider administer to this child?

Correct answer: A

Rationale: To calculate the dose, first, convert the child's weight to kilograms by dividing 55 pounds by 2.2, which equals approximately 25 kg. Then, multiply the weight by the dose (10 mcg/kg) to get the total dose needed, which is 250 mcg. Next, divide the total dose by the concentration of the elixir (50 mcg/ml) to determine the volume needed, which is 5 ml. Therefore, the correct dose is 5 ml based on the child's weight and the concentration of the elixir.

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