49. Cystic Fibrosis (CF) Flashcards Preview

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Flashcards in 49. Cystic Fibrosis (CF) Deck (24):
1

Aaron is receiving dornase alfa therapy for cystic fibrosis. Choose the correct statements concerning dornase alfa: (SelectALL that apply.)

A. It reduces mucous viscosity and improves airflow in the lung.
B. The brand name is Pulmozyme.
C. The ampules are stored at room temperature.
D. The ampules must be protected from light.
E. Do not mix with any other drug in the nebulizer.

A, B, D, E. Dornase alfa (Pulmozyme) comes in 2.5 mg ampules which are given in a nebulizer once daily for 6 months. The ampules are stored in the refrigerator and must be protected from light. Do not mix this medication with any other drug in the nebulizer.

2

Rachel has cystic fibrosis and is currently taking pancreatic enzyme products. Which of the following are FDA approved pancreatic enzyme formulations? (Select ALL that apply.)

A. Lip-Prot-Amyl
B. Creon
C. Zenpep
D. Pulmozyme
E. Desogen

A, B, C. Pulmozyme is the brand name for dornase alfa, which helps with mucus clearance.

3

Cystic fibrosis (CF) is a condition in which mucus production primarily negatively affects which of the following organs?

A. Kidney and liver
B. Lungs and liver
C. Pancreas and liver
D. Pancreas and lungs
E. Kidney and lungs

D.

4

Edgar takes Protonix QAM as part of his cystic fibrosis treatment regimen, which includes Creon, Albuterol, HyperSal andTobi. Select the likely reason for the use of this medication by Edgar:

A. To reduce the risk of C. difficile infections and to help treat H. pylori, if present.
B. To increase bone density and reduce the risk of osteoporosis.
C. To open the airways.
D. To reduce mucus secretion viscosity.
E. To prevent the breakdown of the pancreatic enzymes in his stomach and, if he has symptoms of GERD, to help treat the GERD.

E. The PEP Viokace requires a PPI, but other CF patients using other PEPs may be using a PPI for reflux symptoms.

5

Lung infections in patient with cystic fibrosis are common. Choose the correct statements. (Select ALL that apply.)

A. Staphylococcus aureus and Haemophilus influenzae cause infections commonly in younger patients, followed byP. aeruginosa in adolescents and adults.
B. Acute lung infections are treated with 2-4 weeks of antibiotics, methods to increase the clearance of mucus from the lungs, and nutritional support.
C. In children the most common organisms are Klebsiella pneumoniae, followed by Haemophilus influenzae in adults.
D. In older patients gram positive coverage is the most essential.
E. Good gram positive coverage is obtained by using drugs such as tobramycin and amikacin.

A, B. In older patients the most prevalent organism is P. aeruginosa, which is a gram negative organism. Tobramycin and amikacin provide good gram negative coverage (unless they are used synergistically for gram positive coverage; in CF, they are used to cover primarily P. aeruginosa, which is a gram negative organism).

6

Which of the following statements regarding pancreatic enzymes are correct? (Select ALL that apply.)

A. The dose is adjusted until the stool is normalized (normal consistency).
B. They contain a combination of lipase, amylase, and protease.
C. The products are formulated to dissolve in a more basic pH.
D. Do not use doses >500 units/kg/meal of lipase due to colonic stricture risk.
E. They are dosed based on the amylase component.

A, B, C. Pancreatic enzymes are formulated to dissolve in the more basic pH of the duodenum. The dose is individualized for each patient. They are dosed based on the lipase content. Do not use doses > 6,000 units/kg/meal of lipase due to colonic stricture risk.

7

What is the rationale for using Cayston in cystic fibrosis?

A. To hydrate the airway mucus secretions and facilitate mucus clearance.
B. To target the CFTR mutation.
C. To open the airways prior to administering other medications.
D. To breakdown extracellular DNA from accumulated neutrophils and help increase mucus clearance.
E. To treat active infection or to eradicate colonized organisms.

A.

Cayston (aztreonam): lysine inhalation solution

8

Tom is picking up a prescription for pancreatic enzymes. How should the pharmacist counsel the patient regarding administration of pancreatic enzymes? (Select ALL that apply.)

A. Take half of the dose before eating and the other half of the dose 1 hour after eating each meal.
B. Take this medication at bedtime with a full glass of water.
C. Take this medication in the morning on an empty stomach, 30 minutes before eating.
D. Take at the beginning of meals.
E. Take 1/2 your normal dose with snacks.

D, E. Pancreatic enzymes should be given at meals and half-doses are given with snacks. The entire dose for that meal or snack should be taken at the beginning. The enzymes are needed in the gut to breakdown and absorb food and nutrients.

9

Dominic is a newly diagnosed cystic fibrosis patient. The family is being counseled on the use of chest percussion, his inhaled bronchodilators, and his inhaled antibiotics. What is the proper order in which he should receive each of these therapies?

A. Inhaled bronchodilators, followed by chest percussion, followed by inhaled antibiotics
B. Inhaled antibiotics, followed by chest percussion, followed by inhaled bronchodilators
C. Chest percussion, followed by inhaled antibiotics, followed by inhaled bronchodilators
D. Chest percussion, followed by inhaled bronchodilators, followed by inhaled antibiotics
E. The order in which these are done does not matter

A. It is important to counsel patients on the order of chest percussion in relation to their medications. Bronchodilators are given first to open the airways. Chest percussion is done next to loosen and cough up mucus from the opened airways. Inhaled antibiotics are given last, once the mucus has been cleared to allow for better penetration into the lungs.

10

Ernesto has cystic fibrosis and is admitted for a pseudomonal infection. Which of the following is an appropriate therapy?

A. High dose tobramycin monotherapy
B. High dose cefepime monotherapy
C. Standard doses of tobramycin + cefepime
D. High doses of tobramycin + cefepime
E. Standard doses of vancomycin +/- an aminoglycoside

D. With an active infection 2 drugs (IV) are used that cover the organism, in larger doses than normally used.

11

The pharmacist receives a prescription for Cayston 75 mg TID x 28 days. Which of the following is an appropriate generic substitution for Cayston?

A. Acetylcysteine
B. Aztreonam inhalation
C. Tobramycin inhalation
D. Albuterol inhalation
E. Arformoterol inhalation

B.

12

A patient with cystic fibrosis is currently on tobramycin (TOBI) inhalation solution. The patient also has mild to moderate asthma. Which of the following statements regarding tobramycin inhaled solution is correct? (Select ALL that apply.)

A. The tobramycin can be used with the same nebulizer the patient uses for her asthma medications.
B. Tobramycin by inhalation is used to treat patients colonized with Streptococcus to reduce infection incidence.
C. Ototoxicity and voice alterations are possible complications of therapy.
D. 28 days of therapy are followed by 28 days off therapy.
E. The ampules (liquid-filled capsules) used in the nebulizer are stored in the refrigerator (the ones in use can be kept at room temperature) and should not be exposed to light.

C, D, E. Tobramycin inhaled solution (TOBI) is indicated for cystic fibrosis patients 6 years and older who are colonized withPseudomonas to reduce infections and hospitalizations. Tobramycin inhaled solution should only be used with the PARI LC Plus nebulizer and this nebulizer should not be used with any other medications.

13

Which of the following are correct statements concerning pancreatic enzymes? (Select ALL that apply.)

A. Meals with high fat content require higher doses.
B. The microsphere-contents can be sprinkled on soft food with a low pH that does not require chewing, such as applesauce or baby food or gelatin (using a small amount).
C. Do not crush or chew.
D. Limit fluid intake until after meals.
E. Retention in the mouth before swallowing may cause mucosal irritation and stomatitis.

A, B, C, E. Take with generous amounts of liquid.

14

Which of the following are the mainstay medications used in patients with cystic fibrosis? Select the most complete answer.

A. Inhaled bronchodilators + inhaled antibiotics
B. Inhaled antibiotics+ enzyme replacement therapy
C. Inhaled bronchodilators + inhaled antibiotics + enzyme replacement therapy + hypertonic saline
D. Inhaled bronchodilators + hypertonic saline
E. Inhaled bronchodilators + inhaled antibiotics + enzyme replacement therapy

C.

15

Edgar is a cystic fibrosis patient who has been recently admitted. He had been using Creon. However, the hospital only has the Zenpep available. What should the pharmacist do?

A. Do not substitute, per the FDA. Inpatients do not require enzymes during treatment for acute conditions.
B. Do not substitute. It is not important to restart this medication while in the hospital as long as the stay is kept to within a few weeks.
C. If the patient cannot use the usual pancreatic enzyme product they should be given a liquid diet only.
D. Substitute at the prescribers discretion to the most equivalent dose of Zenpep. It may take 1-2 weeks for patients to adjust to the new product.
E. Substitute Creon with Zenpep at a 1:2 dosing conversion because they are interchangeable, at this dosing ratio.

D. When switching a patient to another pancreatic enzyme product, consider starting with a similar amount of lipase enzyme, then adjust the dose based on the patient's response. Per the FDA, do not switch around but if required due to drug shortage or similar it will take the patient time to accommodate. They are not interchangeable.

16

What is the rationale for using Pulmozyme in cystic fibrosis?

A. To hydrate the airway mucus secretions and facilitate mucus clearance.
B. To target the CFTR mutation.
C. To open the airways prior to administering other medications.
D. To breakdown extracellular DNA from accumulated neutrophils and help increase mucus clearance.
E. To treat active infection or to eradicate colonized organisms.

D.

17

What is the rationale for using hypertonic saline (HyperSal) in cystic fibrosis?

A. To hydrate the airway mucus secretions and facilitate mucus clearance.
B. To target the CFTR mutation.
C. To open the airways prior to administering other medications.
D. To breakdown extracellular DNA from accumulated neutrophils and help increase mucus clearance.
E. To treat active infection or to eradicate colonized organisms.

A.

18

Which of the following are correct statements concerning pancreatic enzymes (pancrelipase products)? (Select ALL that apply.)

A. They help the person's gut break down fat, starches and protein so they can be absorbed.
B. If infants spit out the medication, immediately follow with liquid until they are swallowed.
C. They must be kept refrigerated.
D. Pancreaze and Creon can be substituted for each other (AB rated); the others are not interchangeable.
E. Pancrelipase products contain lipase, amylase, and protease.

A, B, E. None of the FDA-approved PEP's are interchangeable. They do not require refrigeration. They have short expiration dates (check the date prior to dispensing).

19

What is the rationale for using nebulized medications in cystic fibrosis? (Select ALL that apply.)

A. To improve eradication of pancreatic-based organisms.
B. To prevent or treat lung infections.
C. To be able to use less expensive antibiotic regimens.
D. To reduce systemic toxicity.
E. To reduce the incidence of acute exacerbations, and to increase pulmonary function.

B, D, E. The medicines go into the cup in the nebulizer that is attached to the air compressor. The compressor blows air through the cup and makes a mist. Patients breathe the mist in through a mouthpiece or mask.

20

Which of the following statements concerning the pathophysiology of cystic fibrosis are correct? (Select ALL that apply.)

A. Cystic fibrosis is an acquired disorder that occurs primarily in children of lower income households who have been exposed to lead in paint or from airborne pollutants.
B. Cystic fibrosis causes abnormal movement of chloride, bicarbonate, and sodium ions across the epithelium, leading to thick, viscous mucus secretions. The thick mucus mostly affects the lungs, pancreas, liver and intestine, primarily causing difficulty breathing and lung infection as well as digestive complications.
C. The name cystic fibrosis refers to the characteristic scarring (fibrosis) and cyst formation that occurs within the pancreas.
D. Cystic fibrosis is caused by a mutation in the gene for the protein cystic fibrosis transmembrane conductance regulator (CFTR).
E. Ivacaftor is a new drug that is used to decrease mucus secretions by decreasing mucus viscosity.

B, C, D. Ivacaftor works by targeting the mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene.

21

Which of the following statements concerning the new drug Ivacaftor are correct? (Select ALL that apply.)

A. Ivacaftor works by increasing the time CFTR channels remain open.
B. The brand name is Kalydeco.
C. It should be taken on an empty stomach at least one hour between meals.
D. It should be taken with high fat containing food.
E. The majority of patients will benefit from this new drug, however it is very expensive.

A, B, D. Ivacaftor (Kalydeco) works well, but only for select CFTR mutations, which does not include the majority of CF patients. Pharmacogenetics testing needs to be performed to determine if the patient is a good candidate for ivacaftor. Other agents are progressing through clinical trials that will be able to help a larger number of patients.

22

What is the rationale for using albuterol in cystic fibrosis?

A. To hydrate the airway mucus secretions and facilitate mucus clearance.
B. To target the CFTR mutation.
C. To open the airways prior to administering other medications.
D. To breakdown extracellular DNA from accumulated neutrophils and help increase mucus clearance.
E. To treat active infection or to eradicate colonized organisms.

C.

23

A pharmacist receives a prescription for Creon. Which of the following statements regarding Creon is correct?

A. The pharmacist can fill the prescription with Pancreaze since he is out of stock on Creon.
B. The pharmacist should counsel the patient to store the medication in the refrigerator.
C. The pharmacist should counsel the patient to chew the capsules for better absorption.
D. The pharmacist should warn the patient of the risk of blood clots, a side effect of this medication
E. Creon is enteric-coated and designed to dissolve in a more basic pH than the gut fluid.

E. Do not substitute pancreatic enzyme products. They do not require refrigeration. Instruct the patient not to chew or crush the capsules; they are swallowed whole. The capsules can opened the and contents can be sprinkled on soft food with a low pH, such as applesauce or pureed bananas or pears.

24

Patients with cystic fibrosis are a risk of the following vitamin deficiencies:

A. Vitamins C and E
B. Folic acid, vitamin C and riboflavin
C. Folic acid and biotin
D. Thiamine, riboflavin and pyridoxine
E. Vitamins A, D, E, and K

E. Patient with cystic fibrosis have trouble absorbing proteins and fats, including the fat-soluble vitamins A, D, E and K.

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