NAPLEX Cystic Fibrosis Questions with Correct Answers (Grade
A+)
Question 1: What causes cystic fibrosis
Answer: mutation of the CFTR gene causing abnormal Na, bicarb, and Cl transport
Question 2: What organs does cystic fibrosis affect?
Answer: lungs, pancreas, liver, intestines
Question 3: How is cystic fibrosis diagnosed?
Answer: all newborns screened, then Cl sweat test to confirm
Question 4: s/sx of cystic fibrosis
Answer: salty skin, poor growth/weight gain, thick mucus, lung infections, cough, SOB, steatorrhea,
malnutrition, failure to thrive
Question 5: What is the order of inhaled medications for CF?
Answer: 1) albuterol 2) hypertonic saline 3) dornase alfa 4) physiotherapy 5) antibiotics
Question 6: What are the common causes of infection in CF?
Answer: Staph aureus and H. flu in young children, pseudomonas in adolescents and adults
Question 7: How is a CF pulmonary exacerbation caused by pseudomonas treated?
Answer: double PSA coverage with IV antibiotics, often larger doses than general population
Question 8: How is a chronic PSA infection in CF treated?
Answer: cycled inhaled antibiotics - 28d on/28d off (aztreonam, tobramycin); can try azithromycin if still
worsening
Question 9: Why is azithromycin used in chronic pseudomonal infections in CF?
Answer: does not directly inhibit PSA, but disrupts biofilm formation
Question 10: HyperSal, PulmoSal
Answer: inhaled hypertonic saline
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, Question 11: Pulmozyme
Answer: dornase alfa
Question 12: dornase alfa
Answer: Pulmozyme
Question 13: TOBI, TOBI Pedhaler, Bethkis, Kitabis Pak
Answer: tobramycin for inhalation
Question 14: tobramycin for inhalation
Answer: TOBI, TOBI Podhaler, Bethkis, Kitabis Pak
Question 15: Cayston
Answer: aztreonam inhalation
Question 16: aztreonam inhalation
Answer: Cayston
Question 17: How is inhaled hypertonic saline delivered?
Answer: via nebulizer
Question 18: dornase alfa MOA
Answer: degrades extracellular DNA to decrease viscosity of mucus in the lungs
Question 19: dornase alfa contraindications
Answer: hypersensitivity to Chinese Hamster Ovary (CHO) products
Question 20: dornase alfa ADE
Answer: chest pain, fever, rash, rhinitis, laryngitis, voice alteration
Question 21: How is dornase alfa stored?
Answer: in the fridge, protect from light
Page 2
A+)
Question 1: What causes cystic fibrosis
Answer: mutation of the CFTR gene causing abnormal Na, bicarb, and Cl transport
Question 2: What organs does cystic fibrosis affect?
Answer: lungs, pancreas, liver, intestines
Question 3: How is cystic fibrosis diagnosed?
Answer: all newborns screened, then Cl sweat test to confirm
Question 4: s/sx of cystic fibrosis
Answer: salty skin, poor growth/weight gain, thick mucus, lung infections, cough, SOB, steatorrhea,
malnutrition, failure to thrive
Question 5: What is the order of inhaled medications for CF?
Answer: 1) albuterol 2) hypertonic saline 3) dornase alfa 4) physiotherapy 5) antibiotics
Question 6: What are the common causes of infection in CF?
Answer: Staph aureus and H. flu in young children, pseudomonas in adolescents and adults
Question 7: How is a CF pulmonary exacerbation caused by pseudomonas treated?
Answer: double PSA coverage with IV antibiotics, often larger doses than general population
Question 8: How is a chronic PSA infection in CF treated?
Answer: cycled inhaled antibiotics - 28d on/28d off (aztreonam, tobramycin); can try azithromycin if still
worsening
Question 9: Why is azithromycin used in chronic pseudomonal infections in CF?
Answer: does not directly inhibit PSA, but disrupts biofilm formation
Question 10: HyperSal, PulmoSal
Answer: inhaled hypertonic saline
Page 1
, Question 11: Pulmozyme
Answer: dornase alfa
Question 12: dornase alfa
Answer: Pulmozyme
Question 13: TOBI, TOBI Pedhaler, Bethkis, Kitabis Pak
Answer: tobramycin for inhalation
Question 14: tobramycin for inhalation
Answer: TOBI, TOBI Podhaler, Bethkis, Kitabis Pak
Question 15: Cayston
Answer: aztreonam inhalation
Question 16: aztreonam inhalation
Answer: Cayston
Question 17: How is inhaled hypertonic saline delivered?
Answer: via nebulizer
Question 18: dornase alfa MOA
Answer: degrades extracellular DNA to decrease viscosity of mucus in the lungs
Question 19: dornase alfa contraindications
Answer: hypersensitivity to Chinese Hamster Ovary (CHO) products
Question 20: dornase alfa ADE
Answer: chest pain, fever, rash, rhinitis, laryngitis, voice alteration
Question 21: How is dornase alfa stored?
Answer: in the fridge, protect from light
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