cystic fibrosis Flashcards

1
Q

CF is

A

an incurable heredetery disease from mutation in the gene for cystic fibrosis transmembrane conductance regulator- abnormal secretion of chloride, bicarb and sodium affects lungs pancrease liver and intestines

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2
Q

symptoms of CF

A

sallty tasting skin, poor growth adn poor weight gain
steatorrhea

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3
Q

tx

A

lung complications treated with local methods- inhalations
order is important
chronic lung infection treated 28 days on and 28 days off - azithromycin indirectly

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4
Q

order of tx

A

inhaled bronchodilators(albuterol)–>
hypertonic saline (hypersal)–>
Dornase Alfa (pulmozyme) –>
chest physiotherapy —>
inhaled abx

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5
Q

what airway clearance therapies are used

A

brochodilators
hypertonic saline
dornase alfa

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6
Q

ornase alfa

A

ampules stored in the refrigerator
protect from light
do not mix with any other drug

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7
Q

abx inhaled for psudomonas

A

tobramycin (TOBI)
aztreonam

oral azithromycin

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8
Q

what abx in Cystic can cause otoxicity

A

tobramycin

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9
Q

which medication is 28days on 28days off

A

azetreonam

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10
Q

pancrelipase contains

A

lipase protease amylase

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11
Q

Creon

A

pan-CRE-lipase

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12
Q

zenpep

A

contains pancreatic enzyme product PEP

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13
Q

ViokACE

A

ase-ace
only tablet and should be used with PPI

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14
Q

AE of pancrelipase

A

abdominal pain, flatulence nausea

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15
Q

notes on Pancrelipase

A

do not refrigerate
dispense in original container
protect from moisture
do not crush or chew the tabs only DR can be mixed with soft food

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16
Q

CFTR modulator

A

ivacaftor
and combination
MOA- increaseing the time CFTR channel remains open
F508del mutation cannot be used
take with high fat containing food

17
Q

diet

A

vit supplements ADEK
high fat diet