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 should the surgical correction of hypospadias in a newborn infant typically be done?

Correct answer: C

Rationale: Surgical repairs for hypospadias are typically recommended to be performed before the child is potty trained. This timing helps in avoiding complications, ensures better outcomes, and makes the surgical process smoother. Early correction also minimizes the psychological impact on the child regarding genital differences and can improve long-term psychological well-being. Choices A, B, and D are incorrect because repairing hypospadias at one month to prevent bladder infection, after sexual maturity to form a proper urethra, or delaying the repair until school age to reduce castration fears are not the standard recommendations. The optimal timing for surgical correction is before the child is potty trained to achieve the best results and psychological outcomes.

3. The practical nurse is providing care for a toddler who has just returned from surgery for a tonsillectomy. Which intervention is a priority in the immediate postoperative period?

Correct answer: C

Rationale: Monitoring for frequent swallowing is a priority intervention in the immediate postoperative period after a tonsillectomy. Frequent swallowing may indicate bleeding from the surgical site, which requires immediate attention to prevent complications such as hemorrhage. Offering clear fluids frequently may not be appropriate immediately after surgery. Encouraging coughing and deep breathing may increase the risk of bleeding. Applying a warm compress to the throat area is not recommended as it can increase blood flow to the surgical site, potentially causing bleeding.

4. What information should the practical nurse ensure the family understands about caring for a child with a tracheostomy?

Correct answer: A

Rationale: The correct answer is A: Cardiopulmonary resuscitation. It is essential for families to be educated in cardiopulmonary resuscitation (CPR) to manage emergencies involving patients with tracheostomies. Maintaining a clear airway is crucial for the child's safety and well-being. Choice B, hygiene practices, although important, is not as critical as CPR in managing a tracheostomy. Choice C, the proper technique for tracheostomy suctioning, is also crucial but does not take precedence over CPR in emergency situations. Choice D, application of powder around the stoma, is not a standard practice and may not be necessary for tracheostomy care.

5. After observing a mother giving her 11-month-old ferrous sulfate followed by two ounces of orange juice, what should the nurse do next?

Correct answer: D

Rationale: Providing positive feedback is essential in reinforcing correct behaviors. By praising the mother for properly administering the ferrous sulfate to her 11-month-old, the nurse can encourage her to continue following the correct procedure. This positive reinforcement can boost the mother's confidence and adherence to the recommended administration method, ultimately benefiting the infant's health.

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