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 parents of a 15-month-old boy tell the nurse that they are concerned because their son brings his spoon to his mouth but does not turn it over. What action should the nurse implement first?

Correct answer: B

Rationale: The initial action for the nurse is to question the parents about their concerns. By doing so, the nurse can gather more information to understand the situation better. This helps in determining if the child's behavior is within normal development or if further action or referrals are necessary. Choice A is incorrect as it jumps to a specialist referral without fully assessing the situation first. Choice C is also incorrect because assuming the parents need advice on proper spoon handling techniques may not be the case. Choice D is incorrect as it does not address the core concern raised by the parents.

3. A 6 year old who has asthma is demonstrating a prolonged expiratory phase and wheezing and has a 35% of personal best peak expiratory flow rate (PEFR). Based on these findings, what actions should the nurse take first?

Correct answer: A

Rationale: Administering a bronchodilator will help open the airways and improve breathing.

4. Which nursing diagnosis is a priority for a 4-year-old child diagnosed with nephrotic syndrome?

Correct answer: C

Rationale: In a child with nephrotic syndrome, fluid volume excess is a priority nursing diagnosis due to the risk of edema and related complications. This patient may experience significant fluid retention, leading to edema, hypertension, and potential respiratory distress. Monitoring and managing fluid volume excess are crucial in preventing further complications and supporting the child's health during nephrotic syndrome. The other options are not the priority in this case. Impaired urinary elimination is not typically a primary concern in nephrotic syndrome. While infection is a risk due to compromised immunity, fluid volume excess poses a more immediate threat to the child's health. Risk for impaired skin integrity may be a concern secondary to edema, but addressing fluid volume excess takes precedence.

5. 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.

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