End
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


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 : 🫁 CF needs enzymes, mucus thinners, and fat-soluble vitamins

Correct Answers:

  • Water-soluble vitamins
  • Dornase alfa
  • Pancreatic Apase


Rationale :

A. Water-soluble vitamins (Fat-soluble vitamins A, D, E, K in water-miscible form)

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

🫁 These vitamins are essential for vision (A), bone health (D), neurological function (E), and clotting (K)—all of which are at risk due to fat malabsorption.

🫁 The vitamins should be given in a water-miscible form, which allows for absorption without the need for pancreatic enzymes.

🫁 This supplementation is lifelong and essential for growth, immune function, and overall development in children with CF.

B. Dornase alfa

🫁 Dornase alfa (Pulmozyme) is a recombinant human deoxyribonuclease that breaks down DNA in thick pulmonary secretions, improving airway clearance.

🫁 It is a standard part of respiratory management in CF to reduce the viscosity of sputum and prevent recurrent infections and airway obstruction.

🫁 Administered via nebulizer, it should be given daily or twice daily, depending on severity.

🫁 Dornase alfa improves pulmonary function, reduces exacerbations, and enhances quality of life in CF patients with chronic obstructive lung disease.

D. Pancreatic lipase (Pancreatic enzyme replacement therapy – PERT)

🫁 Children with CF need pancreatic enzyme replacement therapy (PERT) because their pancreatic ducts are blocked, preventing normal secretion of digestive enzymes.

🫁 Pancreatic lipase, amylase, and protease are replaced to allow for digestion and absorption of nutrients, especially fats and proteins.

🫁 This therapy helps reduce steatorrhea (foul-smelling, frothy stools), promotes weight gain, and improves nutritional status.

🫁 Pancreatic enzymes must be taken with every meal and snack, and doses are based on body weight and stool consistency.


Distractor Analysis :

Rationale for Incorrect Options to be Available Soon

QLexNursing Calculator Click & Drag ×
AC
(
)
CE
π
e
φ
τ
Inv
sin-1
cos-1
tan-1
log
ln
!
deg
√
x²
x³
^
7
8
9
÷
4
5
6
×
1
2
3
-
0
.
exp
+
Rnd
Ans
=