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PHRM 865 > Exam 5 - Cystic Fibrosis > Flashcards

Exam 5 - Cystic Fibrosis Flashcards

1
Q

Autosomal (dominant or recessive) genetic disease

A

recessive

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2
Q

CF is caused by a mutation in a gene that encodes for the _____________ protein

A

cystic fibrosis transmembrane conductance regulator (CFTR)

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3
Q

what does ENaC stand for

A

epithelial sodium channel

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4
Q

Presenting Signs and Symptoms in Neonates?

A 
Meconium ileus (1st bowel movement gets stuck)
Prolonged obstructive jaundice
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5
Q

Presenting Signs and Symptoms in infants and children?

A

Cough, recurrent URI, wheezing
Failure to thrive (gain weight)
Heat intolerance

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6
Q

Presenting Signs and Symptoms in adolescentes/adults?

A

delayed sexual maturation

nasal polyposis

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7
Q

most common mutation in CF?

A

delta F508

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8
Q

pathophys/results from CFTR mutations?

A

if CFTR is wack — chloride will not get into mucus = neither will sodium – since water follows sodium - water does not get into mucus = mucus is dehydrated and hard to get out of the body/lungs

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9
Q

How to do a newborn screen?

A

get blood from their heel – its an IRT test (immunoreactive trypsinogen)

if positive for CF — still need further workup for a diagnosis

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10
Q

For diagnosis fo CF:

need at least ______ sign/symptom + evidence of ______

A

1 sign or symptom

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11
Q

what are some tests for CF?

A

sweat chloride test – gold standard
genetic testing
pancreatic function tests

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12
Q

what pancreatic function tests can be done to see if CF is happening

A

stool fat quantitation

quantitation of trypsin activity

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13
Q

how does the sweat test work?

A

they give pilocarpine and make a part of your body sweat – and measure the chloride concentration

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14
Q

what is a positive sweat test?

A

> 60 mEq/L of chloride in the sweat

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15
Q 
Ivacaftor/Kalydeco:
what class of drug is it?
A

CFTR potentiator: helps “open the gate”

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16
Q

Ivacaftor/Kalydeco:

Counseling points?

A

take with fatty food to help absorption

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17
Q

Ivacaftor/Kalydeco:

monitoring?

A

LFTs Q3months x 1 yr

eye exam - baseline and yearly

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18
Q

Ivacaftor/Kalydeco:

drug interactions?

A 
CYP3A substrate
(dont forget grapefruit!)
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19
Q

Orkambi – what are the generic drugs in it?

A

ivacaftor/lumacaftor

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20
Q

Orkambi:ivacaftor/lumacaftor:

have to have what mutation?

A

HOMOZYGOUS delta F508

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21
Q

Orkambi:ivacaftor/lumacaftor:

counseling?

A

take with fatty foods

hormonal birth control will not be effectice

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22
Q

Orkambi:ivacaftor/lumacaftor:

drug interactions?

A

hormonal birth control is ineffective
also ivacaftor = 3A substrate
lumacaftor = 3A inducer

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23
Q

Orkambi:ivacaftor/lumacaftor:

monitoring?

A

AST/ALT/Bil q3month for 1 year and then yearly

Eye exam baseline and then yearly - peds

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24
Q

Kalydeco or Orkambi has higher increase in FEV1 and why ever use the other one?

A

Kalydeco has the MUCH better FEV1 increase

use Orkambi when HOMOZYGOUS F508 — that genotype is NOT approved in kalydeco (hence why it is used)

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25
Symdeko -- generic drugs in it?
tezacaftor/ivacaftor
26
Symdeko:tezacaftor/ivacaftor: | counseling?
take with fatty foods | two diff tabs taken: 1 in AM is combo pill; evening pill is just ivacaftor
27
Symdeko:tezacaftor/ivacaftor: | monitoring?
AST/ALT/Bil q3month for 1 year and then yearly Eye exam baseline and yearly - pediatrics Dose adjustment for liver disease
28
Kalydeco, Orkambi or Symdeko: | is approved for only 12 above right now
symdeko
29
Kalydeco, Orkambi or Symdeko: | are used in CF why?
"to fix the basic defect" the CFTR problem
30
Kalydeco, Orkambi or Symdeko: | which one can cause chest tightness
Orkambi | why Symdeko is good -- less chest tightness
31
Kalydeco, Orkambi or Symdeko: | can cause weight gain
orkambi -- GOOD side effect with CF pts
32
CF pathophys/presentation: | common things to happen in the "head region"?
nasal polyps allergy symptoms psychological - depression
33
how to treat/manage nasal polyps for CF pts?
sinus surgery nasal steroids saline nasal rinses gentamicin nasal rinses
34
how to treat/manage allergies for CF pts?
nasal steroids! avoid antihistamines if possible -- dont want to dry their mucus out anymore!!
35
how to treat/manage depression for CF pts?
SSRIs (avoid any wt loss causing ones) | Mirtazipine is great because it causes appetite stimulation
36
CF pts and pulmonary: | why are they at a high risk of lung problems?
the dehydrated mucus that doesn't get cleared easily creates a wonderful environment for bacteria to grow this cycle of infection/inflammation/tissue damage -- will overall lead to decrease lung function
37
For all CF pts - "airway clearance" is recommended -- what are the different options that can be used?
``` manual clearance techniques (banging on the kids chest to loosen mucus) therapy vest flutter/acapella machine huff coughing (?) meta neb (?) ```
38
drugs that can be used for maintenance lung treatment? nebulized drugs: anti-inflammatory: bronchodilator:
nebulized drugs:dornase alfa / hypertonic saline anti-inflamm drugs: azithromycin and ibuprofen bronchodilator: albuterol
39
MOA of dornase alfa?
cleaves extracellular DNA from expended neutrophils/other inflamm cells in CF mucus = reduces viscosity and promotes clearance!!
40
MOA of hypertonic saline is somewhat unknown --- but what is the proposed MOA?
NaCl in airway creates osmotic gradient = water drawn into airway/makes mucus thinner = easier to expectorate
41
Dornase alfa and hypertonic saline recommended in CF pts if above age of _____
6 years old
42
what drugs that are normally helpful lung function things should NOT be used in CF?
NO inhaled corticosteroids NO leukotriene modifiers (montelukast) NO ipratropium because it is DRYING NO oral corticosteroids
43
If CF lung exacerbation: want to cover pathogens --- if MRSA how many drugs needed and what are the options?
``` just 1 drug needed bactrim clindamycin vanc tetracycline linezolid ```
44
If CF lung exacerbation: want to cover pathogens --- if MSSA how many drugs needed and what are the options?
just one drug needed cefazolin unasyn a antipseudomonal beta lactam that covers MSSA is good too....
45
If CF lung exacerbation: want to cover pathogens --- if pseudomonas how many drugs needed and what are the options?
TWO DRUGS!! | have a beta lactam that covers pseudomonas PLUS an aminoglycoside
46
what aminoglycoside is not recommended for CF/pseudomonas coverage
gentamicin
47
Abx in CF pts can act a little different: | beta lactams are affected how and what should we do about it?
there is augmented/higher renal clearance -- increase the dose at shortest interval
48
Abx in CF pts can act a little different: | aminoglycosides are affected how and what should we do about it?
increase Cl and Vd (harder to penetrate the lungs!) | do an initial dose of 10 -12 mg/kg Q 24h
49
Abx in CF pts can act a little different: | beta lactams should be infused how to help the PK changes?
infuse for LONGER; (like over 4 hours) | infusing longer = can do a higher dose with less toxicity issues
50
Abx monitoring for CF pts?
``` pulmonary function tests O2 Sat serum drug concentrations Q3-5 days AG PK dose adjustments via peak/trough SCr Q3-5 ```
51
what are some options for inhaled abx for CF pts
tobramycin** aztreonam** amikacin colistin **ones she talked about the most
52
inhaled tobramycin can be used for what two main things in CF pts?
for INITIAL pseudomonas eradication - one 28 day course OR suppression therapy for pts with CHRONIC pseudomonas (in 28 day cycles)
53
inhaled aztreonam is used when in CF pts?
only for SUPPRESSION/chronic pseudomonas -- use in alternating months with tobramycin
54
inhaled tobramycin or inhaled aztreonam: | which one is more often (TID) but shorter administration time overall?
aztreonam
55
inhaled tobramycin or inhaled aztreonam: | which one is BID and administered for 15 - 20 minutes at a time
tobra
56
inhaled tobramycin or inhaled aztreonam: | which one causes a cough and why?
tobra -- because it is a powder
57
benefits of nebulization?
- delivery of the drug to the site of infection - reduced systemic exposure - decrease risk of systemic side effects - route allows for chronic admin of antipseudomonal abx without IV - ability to get high concentrations of the drug to the site
58
what does ABPA stand for
allergic bronchopulmonary aspergillosis
59
how to treat ABPA
prednisone burst then taper | and then use an antifungal that covers aspergillus (voriconazole, itraconazole, posaconazole)
60
what are the possible liver issues that come CF?
focal biliary cirrhosis (biliary obstruction/progressive periportal fibrosis) liver steatosis liver failure
61
pancreatic issues in CF?
``` mucus obstructs exocrine ducts decreased enzymes (amylase/lipase/protease) decrease HCO3 output ```
62
Pancreatic enzymes: | typical starting dose?
usually 1000 units of lipase/kg/meal
63
Pancreatic enzymes: | do not exceed ______ units of lipase/kg/day
10,000
64
Pancreatic enzymes are adjust based on what things>
of stools per day fat content of stools and growth/weight
65
want to supplement what kind of vitamins?
fat soluble (ADEK)
66
supplement want kind of minerals?
Ca2+ iron SODIUM!! they loose so much in their sweat
67
what are some drugs that can be used as an appetite stimulant
cyproheptadine mirtazapine dronabinal

PHRM 865 (59 decks)

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