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1 :

School-age child admitted, diagnosed with cystic fibrosis at 3 months of age, has experienced failure to thrive, and has chronic obstructive pulmonary disease. The child presents with wheezing, rhonchi, paroxysmal cough, and dyspnea. The parent reports large, frothy foul- smelling stools. The child has deficient levels of vitamin A, D, E, and K.

• Barrel-shaped chest

• Clubbing of the fingers bilaterally

• Respiratory rate 40/min with wheezing and rhonchi noted bilaterally, dyspnea, and paroxysmal cough

• Temperature 3840 C (101 T F)

• Heart rate 1001min

• Respiratory rate 40/min

• Blood pressure 100/57 mm Hg

Sputum culture positive for Pseudomonas aeruginosa

• Stool analysis positive for presence of fat and enzymes

• Chest x-ray indicates obstructive emphysema

• WBC count 20,000/mm3 (5,000 to IO, OOO/mm3)


A nurse is reviewing the child's medical record. Which of the following medications should the nurse expect the provider to prescribe or reconcile from the child's home medication list? Select all that apply


Answer Options

Hint : 💨 Mucus-clearing (dornase alfa), vitamin absorption (water-soluble ADEK), and fever control (acetaminophen) = CF essentials

Correct Answers:

  • Water-soluble vitamin
  • Acetaminophen
  • Dornase alfa


Rationale :

✅ A. Water-soluble vitamin

💊 Children with cystic fibrosis have exocrine pancreatic insufficiency, leading to malabsorption of fat-soluble vitamins A, D, E, and K.

💊 As fat absorption is impaired, providers prescribe water-miscible or water-soluble forms of these vitamins to enhance absorption and avoid deficiencies.

💊 Deficiencies in these vitamins can result in vision problems (A), bone demineralization (D), neurologic symptoms (E), and coagulopathy (K).

💊 The child’s clinical profile (e.g., steatorrhea, failure to thrive, vitamin deficiencies) confirms the need for routine vitamin supplementation in a more absorbable form.

💊 This is a standard long-term management strategy in CF to support nutrition and growth.

✅ B. Acetaminophen

🌡️ The child has a temperature of 38.4°C (101.1°F), indicating fever, likely due to an acute respiratory infection, supported by WBC elevation and positive Pseudomonas culture.

🌡️ Acetaminophen is appropriate for managing fever and mild discomfort in children and is commonly used in CF exacerbations.

🌡️ It is well tolerated and avoids complications related to NSAIDs (e.g., GI upset, renal stress), especially in those with chronic conditions.

🌡️ While not curative, acetaminophen improves comfort and reduces metabolic demand during acute illness.

🌡️ It also supports better rest and pulmonary function by reducing the discomfort associated with fever.

✅ C. Dornase alfa

🫁 Dornase alfa (Pulmozyme) is a recombinant human DNase that breaks down thick, sticky DNA-based secretions in the lungs of children with CF.

🫁 It reduces mucus viscosity, improving airway clearance, oxygenation, and reducing the frequency of infections.

🫁 The child has signs of chronic obstructive pulmonary disease with dyspnea, wheezing, rhonchi, and productive cough, indicating a need for airway clearance support.

🫁 Dornase alfa is administered via nebulization, making it suitable for daily use at home or in acute care.

🫁 It is a standard pulmonary maintenance therapy for CF to minimize airway plugging and prevent exacerbations.


Distractor Analysis :

Rationale for Incorrect Options to be Available Soon

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