Cystic Fibrosis Flashcards
Which of the following is a true statement about dornase alfa?
a. Doses shouldn’t be mixed with any other meds in the nebulizer
b. Ampules should be stored at room temp
c. Doses should be taken with a high-fat meal
d. Capsules shouldn’t be swallowed
e. Doses are taken for up to 28 days, followed by 28 days off cycle
a
Dornase alfa (Pulmozyme) comes in ampules, which are given in a nebulizer once daily. The ampules are stored in the fridge and must be protected from light.
What is the TOBI Podhaler?
a. The nebulizer machine used with TOBI inhalation solution
b. The line used for IV administration of tobramycin
c. The device used to test for tobramycin resistance in pts with CF
d. The device used for chest physiotherapy for pts with CF
e. The inhalation device used with TOBI capsules
e
BG is a 15 y/o female with CF presenting to her pulmonologist complaining of SOB and increased albuterol use over the past few weeks She states that she has been compliant with all of her meds.
Home Meds:
- TOBI 300 mg via nebulizer Q12H
- Pulmozyme 2.5 mg via nebulizer daily
- Albuterol 2.5 mg via nebulizer Q4-6H PRN
- Hypertonic saline 4 mL via nebulizer Q12H
- Zenpep 10,000 units of lipase/kg/day PO as directed
- Lumacafotr/ivacaftor (Orkambi) 2 tablets PO Q12H
- Lansoprazole 15 mg PO daily
Which of the following is the correct way to cycle the inhaled antibiotic?
a. 1 week on followed by 1 week off
b. 3 weeks on followed by 3 weeks off
c. 1 day on followed by 1 day off
d. 4 weeks on followed by 4 weeks off
e. 3x per week
d
BG is a 15 y/o female with CF presenting to her pulmonologist complaining of SOB and increased albuterol use over the past few weeks She states that she has been compliant with all of her meds.
Home Meds:
- TOBI 300 mg via nebulizer Q12H
- Pulmozyme 2.5 mg via nebulizer daily
- Albuterol 2.5 mg via nebulizer Q4-6H PRN
- Hypertonic saline 4 mL via nebulizer Q12H
- Zenpep 10,000 units of lipase/kg/day PO as directed
- Lumacafotr/ivacaftor (Orkambi) 2 tablets PO Q12H
- Lansoprazole 15 mg PO daily
In what order should the inhaled therapies and chest physiotherapy be given?
a. TOBI
b. Chest physiotherapy
c. Hypertonic saline
d. Albuterol
e. Pulmozyme
d - c- e - b - a
BG is a 15 y/o female with CF presenting to her pulmonologist complaining of SOB and increased albuterol use over the past few weeks She states that she has been compliant with all of her meds.
Home Meds:
- TOBI 300 mg via nebulizer Q12H
- Pulmozyme 2.5 mg via nebulizer daily
- Albuterol 2.5 mg via nebulizer Q4-6H PRN
- Hypertonic saline 4 mL via nebulizer Q12H
- Zenpep 10,000 units of lipase/kg/day PO as directed
- Lumacafotr/ivacaftor (Orkambi) 2 tablets PO Q12H
- Lansoprazole 15 mg PO daily
How should BG be counseled to take her PEP?
a. Take half the dose before eating and the other half of the dose 1 hour after eating each meal
b. Take a dose at the end of each meal
c. Take a dose in the morning on an empty stomach, 30 minutes before eating
d. Take a dose at the beginning of each meal
e. Take a dose with each meal containing dairy products
d
BG is a 15 y/o female with CF presenting to her pulmonologist complaining of SOB and increased albuterol use over the past few weeks She states that she has been compliant with all of her meds.
Home Meds:
- TOBI 300 mg via nebulizer Q12H
- Pulmozyme 2.5 mg via nebulizer daily
- Albuterol 2.5 mg via nebulizer Q4-6H PRN
- Hypertonic saline 4 mL via nebulizer Q12H
- Zenpep 10,000 units of lipase/kg/day PO as directed
- Lumacafotr/ivacaftor (Orkambi) 2 tablets PO Q12H
- Lansoprazole 15 mg PO daily
Which of the following are correct counseling points for the PEP regimen?
a. Take half of a mealtime dose with snacks
b. Don’t crush or chew the capsules
c. Take with dairy to maximize absorption
d. Drink plenty of water with each dose
e. High-fat meals may require higher doses
a, b, d, e
BG is a 15 y/o female with CF presenting to her pulmonologist complaining of SOB and increased albuterol use over the past few weeks She states that she has been compliant with all of her meds.
Home Meds:
- TOBI 300 mg via nebulizer Q12H
- Pulmozyme 2.5 mg via nebulizer daily
- Albuterol 2.5 mg via nebulizer Q4-6H PRN
- Hypertonic saline 4 mL via nebulizer Q12H
- Zenpep 10,000 units of lipase/kg/day PO as directed
- Lumacafotr/ivacaftor (Orkambi) 2 tablets PO Q12H
- Lansoprazole 15 mg PO daily
BG is admitted to the hospital for IV antibiotics. The pharmacist realizes that the hospital doesn’t have Zenpep on formulary and would like to substitute with Creon. What should the pharmacist do?
a. Don’t substitute, per the FDA. Inpatients don’t require enzymes during tx for acute conditions.
b. Don’t substitute. The med can be omitted as long as the pt’s hospital admission is < 7 days.
c. If the pt can’t receive her usual pancreatic enzyme product, she should be maintained on a liquid diet only.
d. Substitute at the prescriber’s discretion to the most equivalent dose of Creon.
e. Substitute Zenpep with Creon; 1 unit of lipase from Zenpep = 2 units of lipase from Creon.
d
BG is a 15 y/o female with CF presenting to her pulmonologist complaining of SOB and increased albuterol use over the past few weeks She states that she has been compliant with all of her meds.
Home Meds:
- TOBI 300 mg via nebulizer Q12H
- Pulmozyme 2.5 mg via nebulizer daily
- Albuterol 2.5 mg via nebulizer Q4-6H PRN
- Hypertonic saline 4 mL via nebulizer Q12H
- Zenpep 10,000 units of lipase/kg/day PO as directed
- Lumacafotr/ivacaftor (Orkambi) 2 tablets PO Q12H
- Lansoprazole 15 mg PO daily
Which of BG’s home meds required genotype testing prior to use?
a. Pulmozyme
b. TOBI
c. Lumacaftor/ivacaftor
d. Albuterol
e. Lansoprazole
c
Which of the following signs and symptoms are expected in a pt with untreated cystic fibrosis?
a. Coughing and SOB
b. Epistaxis
c. Poor weight gain and growth despite adequate food intake
d. Steatorrhea
e. Sweet-tasting skin
a, c, d
To prevent Viokace tablets from breaking down in the stomach, they must be:
a. Crushed prior to administration
b. Mixed with dairy products
c. Consumed with a low-fat meal
d. Taken with a proton pump inhibitor
e. Taken with a caffeinated beverage
d
Viokace is a non-enteric coated tablet. To prevent destruction in the stomach, it must be taken with a PPI.
Which med is used to open the airway in CF before administering other meds?
a. Acyclovir
b. Fluticasone
c. Albuterol
d. Hydralazine
e. Montelukast
c
Which of the following are correct statements concerning pancreatic enzymes (pancrelipase products)?
a. The enzymes work in the gut to breakdown fat, starches, and protein for better absorption
b. The enzymes should be stored in a dry location
c. The enzymes must be stored in the fridge
d. The enzymes should be taken with foods that have a high pH, such as dairy products
e. The products contain lipase, amylase, and protease
a, b, e
Pancreatic enzyme products (PEPs) are formulated to dissolve in the:
b. Basic pH of the stomach
b. Acidic pH of the duodenum
c. Acidic pH of the duodenum
d. Acidic pH of the mouth
e. Basic pH of the duodenum
e
A pediatric nurse is treating a pt with CF. The pt needs to be taken to radiology for a lung CT this afternoon. She would like to give the next dose of Cayston as early as possible to accommodate the scheduled imaging. What is the earliest time that she can give the next dose?
Cayston 75 mg via nebulizer TID:
Scheduled Dose - Administration Yesterday - Administration Today:
0800 - 0830 - 0800
1400 - 1400
2000 - 2030
a. 1000
b. 1100
c. 1200
d. 1400
e. 1600
c
TID dosing of aztreonam should be scheduled as close to Q8H as possible. The doses must be at least 4 hours apart to provide adequate drug concentrations throughout the day.
Which of the following pts is most likely to benefit from Zenpep?
a. A pt with excess sputum production d/t COPD
b. A pt with pseudomonal UTI
c. A pt who has had a colonscopy
d. A pt with fibromyalgia
e. A pt who has had a pancreatectomy
e
