CF4 Flashcards
what is the one drug for potential CF liver disease?
ursodiol
what is the effect of ursodiol in CF patients
normalizes liver enzymes; not been shown to decrease progression to liver failure
when should ursodiol be started
- consistently increased liver enzymes (greater than 1.5 x ULN)
- biliary sludging on imaging
- hepatomegaly
- other hepatic abnormalities
what are the aggressive nutritional support plans for CF patients
- 130-150% of recommended calories for age
- 200% of recommended protein
- high fat/ high protein diet
what are BMI goals for CF patients
- kids greater than 50th percentile
2. adults greater than 22 for women; greater than 23 for men
what is the preferred source of vitamin D for CF patients
cholecalciferol (D3); not ergocalciferol (D2)
what is goal level of 25 (OH)D level in CF patients
greater than 30
what is pancreatic enzyme supplements dosing based on
units of lipase
what is used for pancreatic “enzyme boosting” in CF patients
H2 blocker or PPI to neutralize stomach acid; pancreatic enzymes are acid labile
(max enzyme dose before adding these drugs)
what is the initial dose of lipase for children less than 4 years old
1000 units of lipase/ kg/ meal
what is the initial dose of lipase for people 4 years old or older
500 units of lipase/ kg/ meal
enzyme doses should be titrated to what?
to desired effects on symptoms and lab values:
- adequate fat and protein absorption
- no oily stool
- vitamin levels at goal
- meeting nutritional goals
when should enxyme supplements be taken
with all meals and snacks
what is the upper limit of units of lipase
2500 units of lipase/ kg/ meal
or
10,000 units of lipase/kg/ day
why are there upper limits on units of lipase
risk of fibrosing colonopathy
what is range of appropriate lipase dose for children less than 4
500-2500 units/ kg/ meal
what is range of appropriate lipase dose for adults
1000-2500 units/ kg/ meal
pseudomonas treatment usually includes what?
aminoglycoside plus a cell wall agent
what is the only oral agent for pseudomonas treatment of pulmonary exacerbations
ciprofloxacin
what are the common cell wall agents used in conjunction with tobramycin for treating pseudomonas in pulmonary exacerbations
- Ticarcillin/ Clav or Zosyn
- ceftazidime or cefepime
- meropenem
- aztreonam
what treatment is most common for pulmonary exacerbations with MRSA
Bactrim
vancomycin
doxycycline
linezolid
what is most common for methicillin susceptible staph in pulmonary exacerbations
nafcillin
what is the acute therapy endpoint when treating a pulmonary exacerbation
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
