Review Cystic Fibrosis in Children: Comprehensive Nursing Notes

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Cystic Fibrosis in Children: Comprehensive Nursing Notes

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Question 1:

A nurse is providing education to a parent of a child newly diagnosed with cystic fibrosis (CF).
The parent asks, "How is CF inherited?" Which of the following statements by the nurse is correct?

A. "CF is inherited through an X-linked dominant pattern.” B. "CF is an autosomal recessive genetic disorder.” C. "CF is inherited when a child inherits a defective CFTR gene from one parent.” D. "CF is solely caused by the deletion of phenylalanine at position 508 (F508del).”

Answer and Explanation

Choice A:

CF is not inherited through an X-linked dominant pattern. X-linked inheritance involves genes located on the X chromosome, and CF is not linked to this type of inheritance.

Choice C:

CF is not solely related to inheriting a defective CFTR gene from one parent. While inheriting a mutated CFTR gene is a factor, CF is an autosomal recessive disorder, meaning it requires mutations in both copies of the CFTR gene (one from each parent) for the disease to manifest.

Choice D:

CF is not solely caused by the deletion of phenylalanine at position 508 (F508del). While this mutation is one of the most common causes of CF, it is not the only genetic variation associated with the disease. CF can result from various mutations in the CFTR gene.

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Question 2:

A client with cystic fibrosis (CF) is concerned about how this condition affects their body.
The client asks, "Why does CF lead to the production of thick mucus in the lungs and other organs?" Which of the following responses by the nurse is accurate?

A. "The CFTR gene mutation causes excessive water transport in the body.” B. "CF results in an overproduction of a specific enzyme that thickens mucus.” C. "The CFTR gene mutation impairs chloride and water transport in cells.” D. "CF primarily affects the production of surfactant in the lungs.”

Answer and Explanation

Choice A:

The CFTR gene mutation does not cause excessive water transport in the body. In fact, CF leads to a defect in chloride and water transport in cells, which results in the production of thick mucus.

Choice B:

CF does not result in an overproduction of a specific enzyme that thickens mucus. Instead, it affects the functioning of the CFTR protein, which is responsible for maintaining the balance of chloride and water in cells.

Choice D:

CF does not primarily affect the production of surfactant in the lungs. Surfactant is a substance that helps prevent the collapse of alveoli in the lungs, and CF primarily affects the mucus and airway clearance, not surfactant production.

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Question 3:

A nurse is teaching a group of parents about managing cystic fibrosis (CF) in children.
Which of the following interventions should the nurse include in the teaching? (Select all that apply).

A. Encourage physical activity and exercise. B. Administer pancreatic enzyme replacement therapy. C. Restrict fluid intake to reduce mucus production. D. Teach airway clearance techniques. E. Promote a high-fat, high-calorie diet.

Answer and Explanation

Choice A:

Encouraging physical activity and exercise is important in managing CF. It helps improve lung function and overall health in individuals with CF.

Choice B:

Administering pancreatic enzyme replacement therapy is a crucial part of CF management. CF often affects the pancreas, leading to malabsorption, and these enzymes help digest and absorb nutrients properly.

Choice C:

Restricting fluid intake to reduce mucus production is not a recommended intervention for CF. Hydration is essential to keep mucus from becoming too thick and sticky. Dehydration can worsen mucus obstruction.

Choice D:

Teaching airway clearance techniques is essential in CF management. It helps individuals clear the thick mucus from their airways, promoting better lung function.

Choice E:

Promoting a high-fat, high-calorie diet is important for individuals with CF. CF often leads to malnutrition, and a high-calorie diet helps maintain proper nutrition and growth.

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Question 4:

A nurse is caring for a child with cystic fibrosis (CF) and is assessing the child's respiratory status.
Which of the following findings should the nurse consider as a common respiratory complication of CF?

A. Increased lung compliance. B. Decreased breath sounds. C. Normal oxygen saturation. D. Clear and thin sputum production.

Answer and Explanation

Choice A:

Increased lung compliance is not a common respiratory complication of cystic fibrosis (CF). CF is characterized by the production of thick and sticky mucus in the airways, which leads to decreased lung compliance. This thick mucus obstructs the airways, making it difficult for the child to breathe.

Choice B:

Decreased breath sounds are a common respiratory complication of CF. The thick and sticky mucus produced in CF can block the airways, leading to decreased breath sounds upon auscultation. This can result in impaired air movement and reduced ventilation in affected areas of the lungs.

Choice C:

Normal oxygen saturation is not typically a common respiratory complication of CF. CF often leads to decreased oxygen saturation due to the impaired lung function caused by mucus buildup and recurrent infections.

Choice D:

Clear and thin sputum production is not a common finding in CF. CF is associated with the production of thick and sticky mucus, leading to productive cough with thick, tenacious sputum.

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Question 5:

A parent of a child with cystic fibrosis (CF) is concerned about the child's digestive health.
The parent asks, "How does CF affect the pancreas?" Which of the following statements by the nurse is accurate?

A. "CF leads to excessive production of digestive enzymes in the pancreas.” B. "CF has no impact on the function of the pancreas.” C. "CF impairs the pancreas's ability to produce digestive enzymes.” D. "CF causes the pancreas to produce excessive insulin.”

Answer and Explanation

Choice A:

"CF leads to excessive production of digestive enzymes in the pancreas.”. This statement is not accurate. In fact, CF impairs the production of digestive enzymes in the pancreas. The thick mucus produced in CF can block the pancreatic ducts, preventing the release of enzymes needed for proper digestion.

Choice B:

"CF has no impact on the function of the pancreas.”. This statement is incorrect. CF significantly affects the function of the pancreas by obstructing the pancreatic ducts and impairing the production of digestive enzymes.

Choice C:

"CF impairs the pancreas's ability to produce digestive enzymes.”. This statement is accurate. CF causes the thick and sticky mucus to accumulate in the pancreas, obstructing the release of digestive enzymes, which are essential for the digestion of fats and proteins.

Choice D:

"CF causes the pancreas to produce excessive insulin.”. This statement is not correct. CF does not lead to excessive insulin production. Instead, it primarily affects the production of digestive enzymes and can lead to issues related to fat malabsorption.

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Question 6:

A nurse is assessing a child with cystic fibrosis.
The child presents with chronic cough, wheezing, and recurrent respiratory infections.
What should the nurse expect to find during this assessment?

A. "I have difficulty breathing when I'm playing outside.” B. "My fingers and toes look round and wide.” C. "My stools are greasy and float in the toilet.” D. "I often have trouble controlling my blood sugar.”

Answer and Explanation

Choice A:

"I have difficulty breathing when I'm playing outside.”. This statement may be relevant to a respiratory assessment in a child with CF but does not address the typical gastrointestinal symptoms associated with the condition, such as greasy stools.

Choice B:

"My fingers and toes look round and wide.”. This statement is not related to the typical symptoms of CF. The characteristic physical findings in CF are often related to respiratory and gastrointestinal issues rather than finger and toe appearance.

Choice C:

"My stools are greasy and float in the toilet.”. This statement is an accurate description of one of the common gastrointestinal symptoms of CF. CF can lead to malabsorption of fats, resulting in greasy, foul-smelling stools that float in the toilet due to their high fat content.

Choice D:

"I often have trouble controlling my blood sugar.”. This statement is not typically associated with CF. Blood sugar control issues are more commonly linked to conditions like diabetes, not CF, which primarily affects the respiratory and gastrointestinal systems.

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Question 7:

A nurse is assessing a child with cystic fibrosis who is experiencing recurrent respiratory infections.
Which part of the nursing assessment is most relevant in this case?

A. "Monitoring the child's blood sugar levels.” B. "Assessing the child's dental hygiene practices.” C. "Evaluating the child's lung sounds and respiratory rate.” D. "Observing for signs of gastrointestinal obstructions.”

Answer and Explanation

Choice A:

Monitoring the child's blood sugar levels is not relevant to a child with cystic fibrosis experiencing recurrent respiratory infections. Cystic fibrosis primarily affects the respiratory and digestive systems, and blood sugar levels are not a significant concern in this context.

Choice B:

Assessing the child's dental hygiene practices is important for overall health, but it is not the most relevant assessment in the case of a child with cystic fibrosis and recurrent respiratory infections. The primary focus should be on the respiratory system.

Choice D:

Observing for signs of gastrointestinal obstructions is an essential part of caring for a child with cystic fibrosis, but in the context of recurrent respiratory infections, evaluating lung sounds and respiratory rate is the most critical assessment. Gastrointestinal issues are important but secondary to the respiratory concerns in this case.

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Question 8:

A nurse is caring for a child with cystic fibrosis.
The nurse is responsible for providing nutritional support as part of the treatment plan.
What is an appropriate nursing intervention in this context?

A. "You should avoid high-calorie foods to prevent weight gain.” B. "Let's discuss ways to incorporate a high-protein diet into your child's meals.” C. "We won't worry about the diet; it doesn't impact cystic fibrosis.” D. "Your child should limit food intake to avoid digestive issues.”

Answer and Explanation

Choice A:

Avoiding high-calorie foods to prevent weight gain is not an appropriate nursing intervention for a child with cystic fibrosis. These children often have difficulty maintaining their weight due to malabsorption, and they may need a high-calorie diet to ensure proper growth and development.

Choice C:

Disregarding the diet's impact on cystic fibrosis is not a valid approach. Nutrition plays a crucial role in managing the condition, and dietary choices can significantly impact a child's health and well-being. The statement is inaccurate and should not be followed.

Choice D:

Limiting food intake to avoid digestive issues is not a suitable approach for a child with cystic fibrosis. While digestive issues can be a concern, it is essential to provide adequate nutrition to support growth and development. Restricting food intake can lead to malnutrition in these patients.

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Question 9:

A nurse is educating the family of a child with cystic fibrosis about infection prevention strategies.
What should the nurse recommend for effective infection control?

A. "Encourage frequent handwashing to reduce the risk of infections.” B. "It's unnecessary to worry about hand hygiene in this case.” C. "Have your child interact closely with friends and classmates to build immunity.” D. "Avoid vaccinations, as they can exacerbate cystic fibrosis symptoms.”

Answer and Explanation

Choice B:

Stating that hand hygiene is unnecessary in this case is incorrect. Hand hygiene is crucial in infection control, especially for individuals with cystic fibrosis who may be more susceptible to infections due to compromised lung function. Encouraging frequent handwashing is a key preventive measure.

Choice C:

Having a child interact closely with friends and classmates to build immunity is not a recommended strategy for infection control in a child with cystic fibrosis. Close contact with others can increase the risk of exposure to respiratory infections, which should be minimized.

Choice D:

Avoiding vaccinations is not advisable, as vaccinations are an essential part of preventing various infectious diseases. While certain vaccinations may need to be discussed with the healthcare provider to account for the child's individual health status, it is generally not recommended to avoid vaccinations as they can help prevent illness.

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Question 10:

Select all the nursing interventions appropriate for managing cystic fibrosis in children:.

A. Administer respiratory treatments, including chest physiotherapy. B. Encourage a low-calorie, low-protein diet. C. Emphasize the importance of hand hygiene. D. Provide psychological counseling and support. E. Avoid exercise and physical activity.

Answer and Explanation

Choice A:

Administering respiratory treatments, including chest physiotherapy, is essential in managing cystic fibrosis in children. Cystic fibrosis often results in thick, sticky mucus in the lungs, making it difficult to clear airway secretions. Chest physiotherapy helps loosen and mobilize these secretions, improving lung function and reducing the risk of respiratory infections.

Choice B:

Encouraging a low-calorie, low-protein diet is not an appropriate intervention for managing cystic fibrosis in children. Children with cystic fibrosis typically have increased nutritional needs due to malabsorption of nutrients, and a low-calorie, low-protein diet could exacerbate their nutritional deficiencies and growth issues.

Choice C:

Emphasizing the importance of hand hygiene is a crucial nursing intervention for managing cystic fibrosis in children. Cystic fibrosis patients are more susceptible to respiratory infections, and good hand hygiene helps prevent the spread of infections, which is especially important in a healthcare setting.

Choice D:

Providing psychological counseling and support is essential for children with cystic fibrosis. The chronic nature of the disease, frequent hospitalizations, and daily treatments can take a toll on a child's mental health. Psychological support helps them cope with the emotional challenges and stress associated with their condition.

Choice E:

Avoiding exercise and physical activity is not an appropriate intervention for managing cystic fibrosis in children. Physical activity is beneficial for maintaining lung function and overall health in individuals with cystic fibrosis. It helps improve airway clearance and strengthen respiratory muscles.

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Question 11:

A nurse is discussing treatment options with a child who has cystic fibrosis.
What pharmacological interventions might be part of the treatment plan for this child?

A. "You'll need to take bronchodilators as prescribed.” B. "Antibiotics are unnecessary for cystic fibrosis.” C. "Mucolytics can worsen your condition, so avoid them.” D. "Skip the medications; they won't help.”

Answer and Explanation

Choice A:

Bronchodilators are commonly prescribed to children with cystic fibrosis to help open airways, making it easier for them to breathe. These medications assist in relieving airway constriction and improving respiratory function.

Choice B:

Stating that "antibiotics are unnecessary for cystic fibrosis" is incorrect. Antibiotics are often required for cystic fibrosis patients to treat and prevent respiratory infections, which are common due to the thick mucus buildup in the airways.

Choice C:

Mucolytics are medications that help thin and mobilize mucus in the airways. Advising the child to "avoid mucolytics" is not appropriate, as these drugs can be beneficial in managing cystic fibrosis by facilitating mucus clearance and reducing the risk of lung infections.

Choice D:

Telling the child to "skip the medications; they won't help" is incorrect and potentially dangerous advice. Medications prescribed for cystic fibrosis are essential for managing the disease, and skipping them can lead to worsened symptoms and complications.

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Question 12:

A nurse is providing psychosocial support to a child with cystic fibrosis.
What is an essential aspect of this support?

A. "Isolation is the best way to manage cystic fibrosis.” B. "Avoid discussing the condition with your child; it may cause anxiety.” C. "Offer emotional support and resources for coping with the challenges.” D. "Minimize contact with friends and social activities to prevent illness.”

Answer and Explanation

Choice A:

Suggesting that "isolation is the best way to manage cystic fibrosis" is not appropriate. Isolation can lead to feelings of loneliness and depression in children with cystic fibrosis. Social support and interaction are important for their emotional well-being.

Choice B:

Advising the child to "avoid discussing the condition" is not a suitable approach. Open and honest communication about the disease can help the child understand and cope with their condition better. Avoiding discussions may lead to increased anxiety and uncertainty.

Choice C:

"Offering emotional support and resources for coping with the challenges" is a vital aspect of psychosocial support for children with cystic fibrosis. Providing emotional support and connecting the child and their family with resources such as support groups and counseling can help them navigate the emotional and psychological challenges that come with the disease.

Choice D:

"Minimizing contact with friends and social activities" is not a recommended approach for managing cystic fibrosis. While infection control is essential, complete isolation from social activities can lead to social and emotional isolation. It's important to strike a balance between precautions and maintaining a healthy social life.

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Cystic Fibrosis in Children: Comprehensive Nursing Notes

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