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 caring for a school-age child who has cystic fibrosis.
Exhibits
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.
A. Meperidine
B. Dornase alfa
C. Acetaminophen
D. Water-soluble vitamins
E. Pancreatic lipase
The Correct Answer and Explanation is:
For a school-age child with cystic fibrosis, the nurse should expect the provider to prescribe or reconcile the following medications and supplements:
B. Dornase alfa
D. Water-soluble vitamins
E. Pancreatic lipase
Here’s an explanation of why these options are appropriate:
Dornase alfa is a medication used to manage cystic fibrosis. It is a recombinant human DNase enzyme that helps break down the thick, viscous mucus that characterizes cystic fibrosis. This helps to reduce mucus viscosity, making it easier for the child to clear the mucus from their lungs, which can alleviate respiratory symptoms such as wheezing, rhonchi, and cough.
Water-soluble vitamins are essential for children with cystic fibrosis due to the malabsorption issues caused by pancreatic insufficiency. The child’s symptoms include deficient levels of vitamins A, D, E, and K, which are fat-soluble vitamins. In cystic fibrosis, the absorption of fats and fat-soluble vitamins is impaired, necessitating supplementation. Water-soluble vitamins (like vitamin C and the B vitamins) may also be needed to ensure the child meets their nutritional requirements, as they are less likely to be stored and must be replenished regularly.
Pancreatic lipase is a pancreatic enzyme replacement therapy that is crucial for children with cystic fibrosis. These children often have pancreatic insufficiency, meaning their pancreas does not produce enough digestive enzymes to break down fats, proteins, and carbohydrates effectively. Pancreatic lipase supplements help in the digestion and absorption of nutrients, addressing issues like failure to thrive and the large, frothy, foul-smelling stools observed in the child.
Meperidine and Acetaminophen are not specific to the management of cystic fibrosis. Meperidine, an opioid pain medication, is not typically used for cystic fibrosis unless there is a specific pain management issue, and acetaminophen is a general pain reliever and antipyretic that doesn’t address the core symptoms or management needs of cystic fibrosis.
In summary, Dornase alfa, water-soluble vitamins, and pancreatic lipase are integral to the management of cystic fibrosis, targeting respiratory issues, nutritional deficiencies, and pancreatic insufficiency.