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 9-week-old infant is scheduled for cleft lip repair. Which information is most important for the nurse to convey to the surgeon before transporting the infant to the surgical suite?

Correct answer: A

Rationale: The correct answer is A because a low red blood cell count may indicate anemia, which could pose risks during surgery. Anemia can affect oxygen delivery to tissues, impacting wound healing and overall surgical outcomes. The other options, such as white blood cell count, weight gain, and urine specific gravity, are less critical for immediate surgical considerations. White blood cell count is more related to infection risk postoperatively rather than immediate surgical risk. Weight gain reflects good overall growth but does not impact the immediate surgical situation. Urine specific gravity is more indicative of hydration status rather than immediate surgical risk.

3. What action should the nurse implement after the infusion is complete for a 16-year-old with acute myelocytic leukemia receiving chemotherapy via an implanted medication port at the outpatient oncology clinic?

Correct answer: C

Rationale: After completing the chemotherapy infusion via the implanted medication port, the nurse should flush the mediport with saline and heparin solution. This action helps prevent clot formation in the port, ensuring its patency for future use and reducing the risk of complications associated with catheter occlusion. Administering Zofran (Choice A) is used for managing chemotherapy-induced nausea and vomiting, not for post-infusion care. Obtaining blood samples for RBCs, WBCs, and platelets (Choice B) is important for monitoring the patient's blood count but is not the immediate post-infusion priority. Initiating an infusion of normal saline (Choice D) is not necessary after completing the chemotherapy infusion.

4. A 15-year-old adolescent with anorexia nervosa is admitted to the hospital for severe weight loss. The nurse notes that the client has dry skin, brittle hair, and is severely underweight. What is the nurse’s priority intervention?

Correct answer: C

Rationale: In this scenario, the priority intervention for the nurse is to initiate a structured eating plan. Anorexia nervosa is a serious eating disorder characterized by severe food restriction, which can lead to malnutrition and severe weight loss. By starting a structured eating plan, the nurse can ensure the client receives the necessary nutrition to begin the process of weight restoration and recovery. Monitoring vital signs is essential, but without addressing the nutrition deficiency, vital signs may not improve significantly. Establishing a therapeutic relationship is crucial for long-term care but may not address the immediate risk of malnutrition. Providing education about healthy eating habits is important but may not be effective initially due to the severity of the client's condition.

5. A 9-year-old child is brought to the clinic with complaints of fatigue, pallor, and shortness of breath. The nurse notes that the child has a history of iron-deficiency anemia. What is the nurse’s priority action?

Correct answer: A

Rationale: In a child with a history of iron-deficiency anemia presenting with symptoms of fatigue, pallor, and shortness of breath, the priority action for the nurse is to administer iron supplements as prescribed. Iron supplementation is essential to treat iron-deficiency anemia and improve the child's symptoms promptly. Monitoring hemoglobin levels is important but administering iron supplements takes precedence to address the underlying cause. Educating parents about dietary iron sources is valuable for prevention but not the immediate priority. Notifying the healthcare provider may be necessary but should not delay the initiation of treatment with iron supplements.

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