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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 Al 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 384 C (101.1 F)

Heart rate 100/min

Respiratory rate 40/min

Blood pressure 100/57mm 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, OOO/mm3 (57000 to 10,000/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 to apply


Answer Options

Hint : Replace what CF can't make 💊

Correct Answers:

  • Dornase alfa
  • Acetaminophen
  • Water-soluble vitamins
  • Pancreatic lipase


Rationale :

🧬 Dornase alfa (Pulmozyme):

💠 This is a recombinant human deoxyribonuclease I enzyme that cleaves extracellular DNA in the mucus of clients with cystic fibrosis (CF).

💠This DNA is released by degenerating neutrophils and contributes to the viscosity of mucus.

💠 By reducing this thickness, Dornase alfa improves airway clearance, reduces infection risk, and helps maintain pulmonary function.

🧬 Acetaminophen:

💠As an antipyretic and mild analgesic, acetaminophen is appropriate for managing fever and mild pain in pediatric clients.

💠 Children with CF are prone to recurrent infections and may present with elevated body temperature, making acetaminophen a safe and effective choice due to its minimal gastrointestinal effects.

🧬 Water-soluble vitamins (A, D, E, K):

💠These fat-soluble vitamins must be given in water-miscible forms in clients with CF due to their pancreatic insufficiency, which leads to malabsorption of fats.

💠 Deficiencies in these vitamins can result in impaired vision (Vit A), bone health issues (Vit D), coagulopathy (Vit K), and immune dysfunction (Vit E).

🧬 Pancreatic lipase (Pancrelipase):

💠A digestive enzyme replacement vital in CF, as these clients produce insufficient enzymes due to pancreatic duct blockage from thick mucus.

💠Supplementation aids in nutrient absorption, especially fats and proteins, thus preventing failure to thrive and steatorrhea (foul-smelling, greasy stools).


Distractor Analysis :

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