a 6 month old infant is diagnosed with cystic fibrosis what explanation should the nurse provide to the parents about this condition
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HESI Pediatrics Quizlet

1. What explanation should the nurse provide to the parents of a 6-month-old infant diagnosed with cystic fibrosis?

Correct answer: A

Rationale: The correct answer is A. Cystic fibrosis is a genetic disorder that primarily affects the respiratory and digestive systems. It results in the production of thick, sticky mucus that can clog the lungs and obstruct the pancreas. This explanation is crucial for parents to understand the impact of the condition on their child's health. Choice B is incorrect because cystic fibrosis is not an autoimmune disorder. Choice C is partially correct in that cystic fibrosis is a genetic disorder, but it requires a comprehensive management approach beyond just medication. Choice D is incorrect as cystic fibrosis is not caused by prenatal exposure to toxins but rather by inheriting specific genetic mutations.

2. The caregiver is caring for an infant with osteogenesis imperfecta and is providing instruction on how to reduce the risk of injury. Which response from the caregiver indicates a need for further teaching?

Correct answer: B

Rationale: Lifting the baby by supporting the head and neck can cause fractures in infants with osteogenesis imperfecta. Caregivers should avoid lifting infants in this manner due to the risk of injury. Choices A, C, and D demonstrate correct understanding of how to prevent injuries in infants with osteogenesis imperfecta by avoiding excessive force on the arms or legs, preventing awkward positions, and lifting the legs in a safer manner to change diapers.

3. A healthcare provider is assessing a child with suspected Kawasaki disease. What clinical manifestation is the healthcare provider likely to observe?

Correct answer: B

Rationale: Peeling skin on the hands and feet is a characteristic clinical manifestation of Kawasaki disease, known as desquamation. This occurs during the convalescent phase of the illness. While Kawasaki disease is associated with a high fever, which is a common early sign, and can also present with other symptoms like conjunctivitis, mucous membrane changes, and lymphadenopathy, the peeling skin on the hands and feet is a classic feature that distinguishes Kawasaki disease from other conditions. Generalized rash is not a specific hallmark of Kawasaki disease, and low-grade fever is not typically associated with this condition. Therefore, the correct answer is B, peeling skin on the hands and feet, which is a key feature of Kawasaki disease.

4. A 13-year-old girl tells the nurse at the pediatric clinic that she took a pregnancy test and it was positive. She adds that her grandfather, with whom she, her younger sisters, and her mother live, has repeatedly molested her for the past 3 years. When the nurse asks the girl if she has told this to anyone, she replies, 'Yes, but my mother doesn’t believe me.' Legally, who should the nurse notify?

Correct answer: C

Rationale: In this scenario, the nurse should notify Child Protective Services for immediate intervention. The girl disclosed ongoing sexual abuse by her grandfather, which is a serious concern requiring immediate protection and intervention by the appropriate authorities. Child Protective Services are trained to handle cases of child abuse and neglect, ensuring the safety and well-being of the child. While notifying the police about a possible sex crime is crucial, Child Protective Services should be the first point of contact in cases of suspected child abuse due to their specialized role. Confirming the pregnancy through a healthcare provider is not the priority at this moment, as ensuring the safety of the child is paramount. Informing the girl's mother about the positive test result is not appropriate given the lack of belief in the abuse disclosure and the potential risk to the child's safety.

5. The parents of a newborn with phenylketonuria (PKU) need help and support in adhering to specific dietary restrictions. They ask the nurse, “How long will our child have to be on this diet?” How should the nurse respond?

Correct answer: D

Rationale: The correct answer is D: “This is a lifelong problem, and it is recommended that dietary restrictions must be continued.” PKU requires a lifelong adherence to a specific diet low in phenylalanine to prevent cognitive and developmental issues. Phenylalanine buildup can lead to irreversible damage, making it crucial for individuals with PKU to maintain dietary restrictions throughout their lives. Choices A, B, and C are incorrect because they do not address the lifelong nature of dietary restrictions necessary for PKU management.

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