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 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.

3. A 6-year-old boy with bronchial asthma takes the beta-adrenergic agonist albuterol (Proventil). The child’s mother tells the nurse that she uses this medication to open her son’s airway when he is having trouble breathing. What is the nurse’s best response?

Correct answer: C

Rationale: The correct response is to assure the mother that she is using the medication correctly. Albuterol is a beta-adrenergic agonist that helps open the airways during an asthma attack. By reassuring the mother, the nurse reinforces the correct usage of the medication, which is crucial in managing the child's asthma symptoms effectively. Option A is incorrect because immediate evaluation may not be necessary if the child's symptoms are being managed effectively with albuterol. Option B is incorrect as chronic bronchitis is not typically associated with the overuse of albuterol. Option D is incorrect as albuterol primarily acts as a bronchodilator and does not directly reduce airway inflammation.

4. A toddler with a chronic illness that requires frequent hospitalization is likely to experience which psychosocial developmental challenge?

Correct answer: B

Rationale: Toddlers with chronic illnesses requiring frequent hospitalization may experience interference with the development of autonomy. This is because the need for constant medical care can restrict their independence and ability to explore and make choices, which are essential aspects of achieving autonomy according to Erikson's stages of psychosocial development. Choices A, C, and D are incorrect. Fixation with feelings of inadequacy (Choice A) is more commonly associated with Erikson's stage of industry vs. inferiority in middle childhood. Distortion of differentiation of self from parent (Choice C) is related to the separation-individuation phase of Mahler's theory of object relations, typically occurring in infancy. Delayed language, fine-motor, and self-care skills (Choice D) may be impacted by chronic illness but are not the primary psychosocial developmental challenge faced by toddlers in this context.

5. The healthcare provider is caring for a 3-year-old child who is hospitalized with dehydration. The child is now receiving IV fluids and has started to produce urine. What is the best indicator that the child’s dehydration is improving?

Correct answer: A

Rationale: An increase in urine output is a reliable indicator that the child's hydration status is improving. Adequate urine output signifies that the kidneys are functioning properly and that the body is effectively eliminating waste and excess fluids, indicating improved hydration levels. The other options are not as direct indicators of hydration status. Skin turgor and weight changes can be influenced by various factors, and stable vital signs do not specifically reflect hydration status.

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