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 healthcare provider is developing the plan of care for a hospitalized child with von Willebrand disease. What priority nursing intervention should be included in this child's plan of care?

Correct answer: C

Rationale: Children with von Willebrand disease have a deficiency in a clotting protein, putting them at risk of bleeding episodes. The priority nursing intervention for a child with von Willebrand disease is to guard against bleeding injuries to prevent excessive bleeding or hemorrhage. Choices A, B, and D are not the priority interventions for von Willebrand disease. While reducing exposure to infection is important for any hospitalized child, it is not the priority for von Willebrand disease. Eliminating contact with cold objects is more relevant for conditions like Raynaud's disease. Reducing contact with other children is not a specific priority related to managing von Willebrand disease.

3. The heart rate for a 3-year-old with a congenital heart defect has steadily decreased over the last few hours; it is now 76 bpm, whereas the previous reading 4 hours ago was 110 bpm. Which additional finding should be reported immediately to a healthcare provider?

Correct answer: D

Rationale: A significant drop in heart rate and blood pressure should be reported immediately as it may indicate worsening of the congenital heart defect. A decrease in blood pressure to 70/40 is a critical finding that suggests potential cardiovascular compromise and requires urgent attention to prevent further deterioration. Oxygen saturation, respiratory rate, and urine output are important parameters, but in this scenario, the alarming decrease in blood pressure is a more critical finding that necessitates immediate reporting to the healthcare provider.

4. A 2-year-old child with heart failure (HF) is admitted for replacement of a graft for coarctation of the aorta. Prior to administering the next dose of digoxin (Lanoxin), the nurse obtains an apical heart rate of 128 bpm. What action should the nurse take?

Correct answer: B

Rationale: Administering the scheduled dose is appropriate in this scenario. The nurse obtained an apical heart rate of 128 bpm, which is within the expected range for a 2-year-old child. Therefore, there is no immediate concern to withhold the scheduled dose of digoxin. Determining the pulse deficit is not necessary as the heart rate is appropriate. Calculating the safe dose range is not needed as the current dose is within the therapeutic range. Reviewing the serum digoxin level may be indicated later for monitoring but is not urgent based on the heart rate assessment. Administering the scheduled dose of digoxin is the correct action at this time.

5. 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.

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