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. What is the best response for the nurse when a 2-year-old boy begins to cry as the mother starts to leave?

Correct answer: D

Rationale: The best response for the nurse in this situation is to help the child understand that the separation is temporary. Waving bye-bye to mommy can be reassuring to the child and make the separation process easier. It acknowledges the child's feelings while providing a positive and comforting interaction. Choice A may distract the child temporarily but doesn't address the underlying issue of separation anxiety. Choice B is inaccurate as children may continue to cry even after the parent leaves. Choice C diminishes the child's emotions and doesn't offer a supportive approach.

3. A child admitted with diabetic ketoacidosis is demonstrating Kussmaul respirations. The nurse determines that the increased respiratory rate is a compensatory mechanism for which acid-base alteration?

Correct answer: D

Rationale: Kussmaul respirations are deep, rapid breathing patterns observed in metabolic acidosis, such as diabetic ketoacidosis. In this condition, the body tries to compensate for the acidic environment by increasing the respiratory rate to eliminate excess carbon dioxide (CO2) and decrease the acid levels, thereby helping to correct the acid-base imbalance. Therefore, the correct answer is metabolic acidosis.

4. A school-age child with a history of type 1 diabetes mellitus is brought to the emergency department with confusion and rapid breathing. The practical nurse (PN) suspects diabetic ketoacidosis (DKA). Which initial intervention should the PN anticipate?

Correct answer: C

Rationale: Intravenous fluids are typically the initial intervention in diabetic ketoacidosis (DKA) to treat dehydration and stabilize the patient's condition. The fluid replacement helps correct electrolyte imbalances and improve perfusion, which are crucial in managing DKA. Insulin therapy follows after fluid resuscitation to address the underlying cause of DKA, which is the lack of insulin leading to increased ketone production. Administering subcutaneous insulin (Choice A) would be premature without first addressing the dehydration and electrolyte imbalances. Giving oral glucose tablets (Choice B) is contraindicated in DKA as the patient already has high blood glucose levels. Administering oxygen therapy (Choice D) may be necessary based on the patient's condition, but addressing dehydration with intravenous fluids is the priority intervention in DKA.

5. The parents of a 5-year-old child, recently diagnosed with celiac disease, are being educated by the healthcare provider. Which statement by the parents indicates a need for further teaching?

Correct answer: D

Rationale: The correct answer is D. Children with celiac disease must strictly adhere to a gluten-free diet. Even small amounts of gluten can cause harm by triggering an immune response that damages the intestines. It is crucial for parents to understand that allowing their child to have small amounts of gluten occasionally is not safe and can lead to complications. Therefore, further teaching is needed to emphasize the importance of complete avoidance of gluten-containing foods for a child with celiac disease. Choices A, B, and C demonstrate understanding of the need to avoid gluten-containing foods and hidden sources of gluten, which are essential in managing celiac disease. Choice D is incorrect as it suggests a lax approach to the child's diet, which can be harmful in the case of celiac disease.

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