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 child with cystic fibrosis is being discharged home with pancreatic enzyme replacement therapy. What information should the practical nurse reinforce with the parents?

Correct answer: C

Rationale: The correct answer is C: 'Enzymes should be taken before or with meals.' Pancreatic enzymes should be taken before or with meals to assist with digestion in children with cystic fibrosis. This timing helps maximize the effectiveness of the enzymes in breaking down nutrients from food. Giving the enzymes after meals (choice A) may not provide the necessary support for digestion. Mixing the enzymes with hot food or drinks (choice B) is not recommended as it may affect the enzymes' efficacy. Avoiding giving enzymes with any food or drink (choice D) is incorrect as enzymes need to be taken in conjunction with meals to aid in digestion.

3. The parents of a 2-month-old infant, who is being discharged after treatment for pyloric stenosis, are being educated by the healthcare provider. Which statement by the parents indicates a need for further teaching?

Correct answer: C

Rationale: The correct answer is C. Placing babies on their stomach to sleep increases the risk of sudden infant death syndrome (SIDS). The safest sleep position for infants is on their back to reduce the risk of SIDS. Teaching parents about safe sleep practices is crucial in preventing potential harm to the infant. Choices A, B, and D are all correct statements that promote the well-being of the infant. Feeding the baby in an upright position helps prevent reflux, delaying solid foods until 6 months of age is recommended for proper growth and development, and burping the baby frequently during feedings helps prevent gas buildup and colic.

4. The healthcare provider is evaluating diet teaching for a client who has nontropical sprue (celiac disease). Choosing which food indicates that the teaching has been effective?

Correct answer: A

Rationale: Creamed corn is a gluten-free food, making it a suitable option for clients with celiac disease. This choice indicates effective diet teaching as it aligns with the dietary restrictions necessary for managing the condition. Pancakes, rye crackers, and cooked oatmeal contain gluten, which is harmful to individuals with celiac disease. Therefore, they are not suitable choices and would not indicate effective teaching for a client with this condition.

5. The caregiver is caring for a 2-month-old infant with a diagnosis of bronchiolitis. Which assessment finding would be most concerning to the caregiver?

Correct answer: A

Rationale: Nasal flaring and grunting are indicative of respiratory distress, suggesting the infant is having difficulty breathing. This finding requires immediate attention as it signifies a more severe respiratory compromise compared to the other symptoms listed.

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