CF4

Question 1

what is the one drug for potential CF liver disease?

Answer 1

ursodiol

Question 2

what is the effect of ursodiol in CF patients

Answer 2

normalizes liver enzymes; not been shown to decrease progression to liver failure

Question 3

when should ursodiol be started

Answer 3

1. consistently increased liver enzymes (greater than 1.5 x ULN)
2. biliary sludging on imaging
3. hepatomegaly
4. other hepatic abnormalities

Question 4

what are the aggressive nutritional support plans for CF patients

Answer 4

1. 130-150% of recommended calories for age
2. 200% of recommended protein
3. high fat/ high protein diet

Question 5

what are BMI goals for CF patients

Answer 5

1. kids greater than 50th percentile

2. adults greater than 22 for women; greater than 23 for men

Question 6

what is the preferred source of vitamin D for CF patients

Answer 6

cholecalciferol (D3); not ergocalciferol (D2)

Question 7

what is goal level of 25 (OH)D level in CF patients

Answer 7

greater than 30

Question 8

what is pancreatic enzyme supplements dosing based on

Answer 8

units of lipase

Question 9

what is used for pancreatic “enzyme boosting” in CF patients

Answer 9

H2 blocker or PPI to neutralize stomach acid; pancreatic enzymes are acid labile
(max enzyme dose before adding these drugs)

Question 10

what is the initial dose of lipase for children less than 4 years old

Answer 10

1000 units of lipase/ kg/ meal

Question 11

what is the initial dose of lipase for people 4 years old or older

Answer 11

500 units of lipase/ kg/ meal

Question 12

enzyme doses should be titrated to what?

Answer 12

to desired effects on symptoms and lab values:

1. adequate fat and protein absorption
2. no oily stool
3. vitamin levels at goal
4. meeting nutritional goals

Question 13

when should enxyme supplements be taken

Answer 13

with all meals and snacks

Question 14

what is the upper limit of units of lipase

Answer 14

2500 units of lipase/ kg/ meal
or
10,000 units of lipase/kg/ day

Question 15

why are there upper limits on units of lipase

Answer 15

risk of fibrosing colonopathy

Question 16

what is range of appropriate lipase dose for children less than 4

Answer 16

500-2500 units/ kg/ meal

Question 17

what is range of appropriate lipase dose for adults

Answer 17

1000-2500 units/ kg/ meal

Question 18

pseudomonas treatment usually includes what?

Answer 18

aminoglycoside plus a cell wall agent

Question 19

what is the only oral agent for pseudomonas treatment of pulmonary exacerbations

Answer 19

ciprofloxacin

Question 20

what are the common cell wall agents used in conjunction with tobramycin for treating pseudomonas in pulmonary exacerbations

Answer 20

1. Ticarcillin/ Clav or Zosyn
2. ceftazidime or cefepime
3. meropenem
4. aztreonam

Question 21

what treatment is most common for pulmonary exacerbations with MRSA

Answer 21

Bactrim
vancomycin
doxycycline
linezolid

Question 22

what is most common for methicillin susceptible staph in pulmonary exacerbations

Answer 22

nafcillin

Question 23

what is the acute therapy endpoint when treating a pulmonary exacerbation

Answer 23

pulmonary function tests back to baseline or plateau
(also look at symptoms such as weight loss or fatigue)
x rays and traditional markers of infection aren’t useful