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PHRM 865 > CF > Flashcards

CF Flashcards

1
Q

CF incidence

A

1 in 3,000

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2
Q

CF inheritance pattern

A

autosomal recessive
CFTR mutation
on chromosome 7

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3
Q

most common mutation in CF

A

F508del

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4
Q

mechanism of CF

A

CFTR channels absent or reduced
CFTR not properly transporting Cl

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5
Q

screening in newborns for CF

A

IRT test to see if genes present

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6
Q

diagnosis of CF

A

symptom and presence of CFTR dysfunct
sweat chloride test >60
also genetic and pancreatic testing

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7
Q

class I mutations

A

X
CFTR not there

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8
Q

class II mutations

A

F508del
little to no CFTR, the CFTR there does not properly transport Cl

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9
Q

class III mutations

A

G551D
normal number of CFTR, Cl not transported properly

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10
Q

class IV mutations

A

R
normal number of CFTR, some Cl transported properly

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11
Q

class V mutations

A

G to A
reduced number of CFTR but it transports properly

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12
Q

CFTR modulator drugs

A

Kalydeco (ivacaftor)
Orkambi (ivacaftor, lumacaftor)
Symdeko (ivacaftor, tezacaftor)
Trikafta (ivacaftor, tezacaftor, elaxacaftor)
Alyftrek (vanzacaftor, texacaftor, deutivacaftor)

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13
Q

Kalydeco (ivacaftor) age range

A

> 1 month

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14
Q

Kalydeco (ivacaftor) mechanism

A

potentiator, helps open door so Cl can flow through
(class III, IV)

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15
Q

Kalydeco (ivacaftor) counseling

A

take w fatty foods
interaction w grapefruit jucie and 3A4s

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16
Q

Kalydeco (ivacaftor) approved for what

A

responsive mutations

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17
Q

Kalydeco (ivacaftor) monitoring considerations

A

LFTs every 3 months for 1 year then yearly
eye exam baseline and yearly (peds)
dose adjust for hepatic impairment

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18
Q

Orkambi (ivacaftor/lumacaftor) age

A

> 1 month

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19
Q

Orkambi (ivacaftor/lumacaftor) indication

A

F508del homozygous mutation

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20
Q

Orkambi (ivacaftor/lumacaftor) counseling

A

take with fatty foods
birth control drug interaction
lumacaftor 3A4 inducer

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21
Q

Orkambi (ivacaftor/lumacaftor) side effect

A

chest tightness and SOB

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22
Q

Orkambi (ivacaftor/lumacaftor) monitoring

A

AST/ALT/bili every 3 months for 1 year then yearly
eye exam baseline and yearly
dose adjust hepatic impairment

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23
Q

Symdeko (ivacaftor/tezacaftor) age

A

> 6 years

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24
Q

Symdeko (ivacaftor/tezacaftor) counseling

A

take with fatty foods
iva CYP3A4

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25
Symdeko (ivacaftor/tezacaftor) approved for what
F508del homozygous or responsive mutation
26
Symdeko (ivacaftor/tezacaftor) monitoring
AST/ALT/bili every 3 months for 1 year then yearly eye exam baseline then yearly dose adjust liver disease
27
Trikafta (elexacaftor/tezacaftor/ivacaftor) age
> 2 years
28
Trikafta (elexacaftor/tezacaftor/ivacaftor) counseling
take w fatty foods
29
Trikafta (elexacaftor/tezacaftor/ivacaftor) monitoring
eye exam baseline and yearly AST/ALT/bili/ALK phos every month for 6 months then every 3 months for 12 months then yearly
30
Trikafta (elexacaftor/tezacaftor/ivacaftor) approved for who
one F508del mutation or responsive
31
if pt misses orange tablet by more than 6 hours what should we tell them
take organge tablet and skip blue evening tablet
32
Alyftrek (vnzacaftor/tezacaftor/deutivacaftor) administration
once daily
33
Alyftrek (vnzacaftor/tezacaftor/deutivacaftor) counseling
take w fatty foods
34
Alyftrek (vnzacaftor/tezacaftor/deutivacaftor) monitoring
AST/ALT/bili/Alk phos every month for 6 months then every 3 months for 1 year then yearly eye exam anually and basseline
35
Alyftrek (vnzacaftor/tezacaftor/deutivacaftor) approved for what
one F508del or responsive
36
airway clearance recommended for who
everyone (ex. vest)
37
maintenance lung treatments
dornase alfa hypertonic saline inhaled mannitol
38
doranse alfa MOA
cleaves extracellular DNA reducing viscosity and promoting clearnace nebulize solution
39
dornase alfa age limit
> 6 years recommended for everyone
40
hypertonic saline MOA
draws water into airway so reduced mucus thickness nebulized solution
41
hypertonic saline age and recommendation
> 6 years recommended for everyone
42
hypertonic saline consideration
could give albuterol before
43
inhaled mannitol consideration
could give albuterol before dry powder inhaler 18 +
44
anti-inflammatory agents given
azithromycin ibuprofen
45
who would we give azithromycin to in CF
chronic pseudomonas or for anti-inflam effects
46
ibuprofen consideration in CF
high dose dont use w aminoglycosides check levels of ibuprofen
47
which therapies not recommended in CF?
inhaled corticosteroids leukotrine modifiers (montelukast) oral corticossteroids
48
pathogens causing pneumonia in CF
staph aureus pseudomonas h. flu Klebsiella e. coli stenotrophomonas Burkholder aspergillus achromobacter
48
albuterol use in CF
before hypertonic saline to open up airways
49
MRSA agents used in CF
TMP-SMX vancomycin linezolid tetracycline clindamycin
50
MSSA agents used in CF
cefazolin Unasyn
51
pseudomonas agents used in CF
pip/tazo cefepime ciprofloxacin carbapenem WITH aminoglyocoside like tobramycin
52
altered kinetics of beta lactams in CF
increased clearance need higher doses and continuous/ more frequent infusion
53
altered kinetics of aminoglycosides in CF
increased cl and VD need higher doses
54
what do we do if pt has non-symptomatic chronic pseudomonas
inhaled tobramycin 28 days then culture again, if its still there 28 days on 28 days off
55
what do we do in chronic pseudomonas for pts who cant tolerate tobramycin
aztreonam inhaled nebulizer
56
recommended lipase per meal
500-2500 units lipase/kg/meal
57
startin dose of lipase per kg per meal
1,000 units/kg/meal
58
max lipase per day
10,000 units/kg/day
59
units for pancreatic enzymes
units of lipase per capsule
60
which vitamins should we monitor in CF
ADEK (fat soluble)
61

PHRM 865 (33 decks)

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