A home care nurse is instructing a mother of a child with cystic fibrosis about the appropriate dietary measures. The nurse tells the mother that the child needs to consume a?

Appeared in: DHS Staff Nurse - 2018 (Shift-2nd)

Explanation

Cystic fibrosis (CF) is a genetic disorder that primarily affects the exocrine glands, leading to thick, viscous secretions.
These secretions block the pancreatic ducts, causing pancreatic insufficiency. This prevents digestive enzymes from reaching the small intestine, leading to severe malabsorption of fats and proteins.
Patients with CF also have a higher metabolic rate due to the increased work of breathing and chronic respiratory infections.
To compensate for nutrient malabsorption and meet these high energy demands, a diet that is high in both calories and protein is essential for promoting growth and maintaining a healthy weight.

Option A: A low-calorie, low-fat diet is contraindicated. It would lead to severe malnutrition, weight loss, and failure to thrive in a child with CF who already has high energy needs and poor nutrient absorption.
Option B: While a high-calorie diet is correct, restricting fat is incorrect. Fat is a concentrated source of calories, and restricting it would make it very difficult to meet the child's high energy needs. Fat malabsorption is managed with pancreatic enzyme replacement therapy, not dietary restriction.
Option C: A low-calorie, low-protein diet is the opposite of what is required. It would lead to muscle wasting, impaired growth, and a weakened immune system, exacerbating the effects of the disease.

Clinical Relevance

Nursing practice connection: Use the key finding related to Nutritional management in cystic fibrosis to guide bedside assessment, documentation, and the next nursing action.
The nurse's primary role is to educate the family on the critical importance of the high-calorie, high-protein diet and the correct administration of pancreatic enzymes (with every meal and snack).
Consistent monitoring of the child's growth (weight, height, and BMI) is essential to evaluate the effectiveness of the nutritional therapy and make adjustments as needed.
Nurses should teach parents to recognize signs of malabsorption, such as steatorrhea (frequent, bulky, foul-smelling, greasy stools), abdominal distention, and poor weight gain, which may indicate a need to adjust enzyme dosage.

How to Approach the Question

First, identify the core disease in the question: Cystic Fibrosis (CF).
Recall the fundamental pathophysiology of CF related to the digestive system. CF is an exocrine gland disorder that causes thick mucus.
Connect this pathophysiology to its consequence: The thick mucus blocks pancreatic ducts, preventing digestive enzymes from reaching the intestine. This leads to malabsorption of fat and protein.
Consider the overall energy needs of a person with CF. Chronic lung disease and infections increase the body's metabolic rate and energy expenditure.
Synthesize these two points: The body is not absorbing nutrients properly AND it needs more energy than usual. Therefore, the diet must provide a surplus of nutrients to compensate.
Conclude that the diet must be high in calories (for energy) and high in protein (for growth and repair, since it's poorly absorbed).

Concept Tested & Keywords

Concept Tested: Nutritional management in cystic fibrosis
Stem keywords: cystic fibrosis, child, dietary measures, home care nurse
Lead-in keywords: appropriate
Clinical cues: Age/sex group narrows the expected diagnosis, intervention, or normal reference range.
Clinical cues: The care setting changes urgency, monitoring level, and the expected nursing action.

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E6 Text Book Of Pediatric Nursing 3rd Panchali Pal — Part 2 (pp 239-476 of 713) p. 152-154

E6 Nelson Textbook of Pediatrics(2024) — Volume 2 p. 464-466

E6 Nursing Brunner Adult Health 3SA Vol 1 Part 2 p. 62-64

Practise the full DHS Staff Nurse - 2018 (Shift-2nd)

Attempt every question from this paper in a timed mock, then review the full solution for each one.