A nurse is caring for a school-age child who has cystic fibrosis.
History and Physical
Vital Signs
Laboratory Results
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.
A Meperidine
B Dornase alfa
C Acetaminophen
D Water-soluble vitamins
E Pancreatic lipase
The Correct Answer and Explanation is:
In the care of a school-age child with cystic fibrosis (CF), the nurse should expect the provider to prescribe or reconcile the following medications from the child’s home medication list:
B. Dornase alfa
D. Water-soluble vitamins
E. Pancreatic lipase
Here’s why each of these medications is relevant for a child with CF:
Dornase alfa
Dornase alfa is an enzyme therapy used to manage CF. It is a recombinant human DNase enzyme that helps to break down the thick, sticky mucus in the lungs by cleaving extracellular DNA that accumulates in the mucus. This action reduces the viscosity of the mucus, making it easier for the patient to clear it from their airways. This medication is particularly crucial for patients with CF who have persistent lung infections and mucus buildup, leading to wheezing, rhonchi, and chronic obstructive pulmonary disease (COPD).
Water-Soluble Vitamins
CF patients often have malabsorption issues due to pancreatic insufficiency, which can lead to deficiencies in fat-soluble vitamins (A, D, E, and K). However, the clinical context and potential dietary management would still require supplementation of water-soluble vitamins (e.g., vitamin C and the B vitamins) as part of a comprehensive approach to addressing deficiencies and supporting overall health. In CF, a multivitamin supplement that includes both fat-soluble and water-soluble vitamins is often prescribed to address the broad spectrum of deficiencies.
Pancreatic Lipase
CF affects the pancreas, impairing its ability to produce digestive enzymes necessary for proper digestion and absorption of nutrients. Pancreatic lipase, a component of pancreatic enzyme replacement therapy (PERT), helps to digest fats, proteins, and carbohydrates. It is essential for preventing malnutrition and failure to thrive, which are common in CF due to the inability to properly digest and absorb nutrients from food. The enzyme replacement helps in breaking down dietary fats and proteins, which addresses the child’s reported large, frothy, foul-smelling stools—a sign of steatorrhea due to undigested fats.
Medications Not Included
A. Meperidine
Meperidine is an opioid analgesic and is not typically used in the management of cystic fibrosis. It is used for pain relief and is not related to the treatment of CF symptoms or complications.
C. Acetaminophen
While acetaminophen may be used for pain relief or fever reduction, it is not specific to CF management. Its role is supportive rather than directly addressing the core issues related to CF such as mucus clearance or pancreatic enzyme replacement.
In summary, Dornase alfa, water-soluble vitamins, and pancreatic lipase are all integral to the management of CF. They address respiratory symptoms, nutritional deficiencies, and digestive issues, respectively.