Cystic Fibrosis

Question 1

therapy for pancreatic enzyme replacement

Answer 1

creon, zenpep, pancreaze (contain lipase, protease, amylase)

Question 2

this class can cause nausea, abd cramps, constipation, diarrhea, greasy stools, flatulence. Fibrosing colonopathy at high doses

Answer 2

pancreatic enzyme replacement

Question 3

clinical issues with this class include inadequate enteric coating leading to denaturing and poor outcomes when switched to generic

Answer 3

pancreatic enzyme replacement

Question 4

this disease increases in CF patients as they age

Answer 4

Diabetes

Question 5

pharmacotherapy for pt with diabetes

Answer 5

insulin

Question 6

therapy for distal intestinal obstruction syndrome

Answer 6

electrolyte lavage solutions until the patient passes stool

Question 7

treatment for GERD in a CF pt

Answer 7

antacids
H2 blockers
PPI

Question 8

pt w CF has high GGT levels. How do you treat

Answer 8

Ursodeoxycholate

Question 9

this drug decreases liver inflammation and bile duct proliferation

Answer 9

Ursodeoxycholate

Question 10

As maintenance therapy, this improves ventilation and reduces the accumulation of secretions

Answer 10

ACT (airway clearance techniques)

Question 11

are oral corticosteroids a good option for AI therapy in CF

Answer 11

NO, undesirable SEs

Question 12

this anti-inflam therapy decreases rate of decline of FEV1

Answer 12

NSAIDs (ibu)

Question 13

this AI therapy slows the FEV1 decline in CF pts with pseudomonas and can improve FEV1

Answer 13

Azithromycin

Question 14

this is a chronic therapy for CF. It decreases viscosity of sputum. An aerosolized recombinant dornase alfa

Answer 14

Pulmozyme

Question 15

this drug shows improvement in FEV1 and decreased pulm exacerbation. AEs include hoarseness, voice alteration, and pharyngitis

Answer 15

Pulmozyme

Question 16

this mucolytic is not commonly used bc it is irritating and causes bronchoconstriction

Answer 16

Mucomyst (n-acetylcystine)

Question 17

this therapy allows pt to draw water into airways, increasing the ability to cough out mucous. May be associated w chronic cause and bronchospasm

Answer 17

nebulized hypertonic

Question 18

these chronic therapies may be useful in CF pts with asthma

Answer 18

inhaled B2 agonists

ICS

Question 19

major pathogen 1st year of life

Answer 19

staph aureus

Question 20

major pathogen by age 3

Answer 20

h influenzae

Question 21

major pathogen by age 5

Answer 21

pseudomonas (Colonized)

Question 22

This chronic antibiotic is nebulized tobramycin and covers for pseudomonas

Answer 22

TOBI

Question 23

this chronic ab is nebulized aztreonam and covers pseudomonas

Answer 23

Cayston

Question 24

this class is used chronically and prolongs time between acute CF exacerbations

Answer 24

chronic abx

Question 25

Ab therapy for an acute CF exacerbation

Answer 25

cover staph aureus and h influ (pseudo based on sensitivities)
generally aminoglycosides plus antipseudomonal penicillin. Dose HIGH. CF have high Vd and Cl

Question 26

Chronic therapy dietary CF

Answer 26

high calorie (120-150% RDA)
Vit A,D,E,K
may need more salt
multivitamins