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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
Q

Symdeko – generic drugs in it?

A

tezacaftor/ivacaftor

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26
Q

Symdeko:tezacaftor/ivacaftor:

counseling?

A

take with fatty foods

two diff tabs taken: 1 in AM is combo pill; evening pill is just ivacaftor

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27
Q

Symdeko:tezacaftor/ivacaftor:

monitoring?

A

AST/ALT/Bil q3month for 1 year and then yearly
Eye exam baseline and yearly - pediatrics
Dose adjustment for liver disease

28
Q

Kalydeco, Orkambi or Symdeko:

is approved for only 12 above right now

A

symdeko

29
Q

Kalydeco, Orkambi or Symdeko:

are used in CF why?

A

“to fix the basic defect” the CFTR problem

30
Q

Kalydeco, Orkambi or Symdeko:

which one can cause chest tightness

A

Orkambi

why Symdeko is good – less chest tightness

31
Q

Kalydeco, Orkambi or Symdeko:

can cause weight gain

A

orkambi – GOOD side effect with CF pts

32
Q

CF pathophys/presentation:

common things to happen in the “head region”?

A

nasal polyps
allergy symptoms
psychological - depression

33
Q

how to treat/manage nasal polyps for CF pts?

A

sinus surgery
nasal steroids
saline nasal rinses
gentamicin nasal rinses

34
Q

how to treat/manage allergies for CF pts?

A

nasal steroids!

avoid antihistamines if possible – dont want to dry their mucus out anymore!!

35
Q

how to treat/manage depression for CF pts?

A

SSRIs (avoid any wt loss causing ones)

Mirtazipine is great because it causes appetite stimulation

36
Q

CF pts and pulmonary:

why are they at a high risk of lung problems?

A

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
Q

For all CF pts - “airway clearance” is recommended – what are the different options that can be used?

A 
manual clearance techniques (banging on the kids chest to loosen mucus)
therapy vest
flutter/acapella machine
huff coughing (?)
meta neb (?)
38
Q

drugs that can be used for maintenance lung treatment?
nebulized drugs:
anti-inflammatory:
bronchodilator:

A

nebulized drugs:dornase alfa / hypertonic saline
anti-inflamm drugs: azithromycin and ibuprofen
bronchodilator: albuterol

39
Q

MOA of dornase alfa?

A

cleaves extracellular DNA from expended neutrophils/other inflamm cells in CF mucus = reduces viscosity and promotes clearance!!

40
Q

MOA of hypertonic saline is somewhat unknown — but what is the proposed MOA?

A

NaCl in airway creates osmotic gradient = water drawn into airway/makes mucus thinner = easier to expectorate

41
Q

Dornase alfa and hypertonic saline recommended in CF pts if above age of _____

A

6 years old

42
Q

what drugs that are normally helpful lung function things should NOT be used in CF?

A

NO inhaled corticosteroids
NO leukotriene modifiers (montelukast)
NO ipratropium because it is DRYING
NO oral corticosteroids

43
Q

If CF lung exacerbation:
want to cover pathogens — if MRSA
how many drugs needed and what are the options?

A 
just 1 drug needed
bactrim
clindamycin
vanc
tetracycline
linezolid
44
Q

If CF lung exacerbation:
want to cover pathogens — if MSSA
how many drugs needed and what are the options?

A

just one drug needed
cefazolin
unasyn
a antipseudomonal beta lactam that covers MSSA is good too….

45
Q

If CF lung exacerbation:
want to cover pathogens — if pseudomonas
how many drugs needed and what are the options?

A

TWO DRUGS!!

have a beta lactam that covers pseudomonas PLUS an aminoglycoside

46
Q

what aminoglycoside is not recommended for CF/pseudomonas coverage

A

gentamicin

47
Q

Abx in CF pts can act a little different:

beta lactams are affected how and what should we do about it?

A

there is augmented/higher renal clearance – increase the dose at shortest interval

48
Q

Abx in CF pts can act a little different:

aminoglycosides are affected how and what should we do about it?

A

increase Cl and Vd (harder to penetrate the lungs!)

do an initial dose of 10 -12 mg/kg Q 24h

49
Q

Abx in CF pts can act a little different:

beta lactams should be infused how to help the PK changes?

A

infuse for LONGER; (like over 4 hours)

infusing longer = can do a higher dose with less toxicity issues

50
Q

Abx monitoring for CF pts?

A 
pulmonary function tests
O2 Sat
serum drug concentrations Q3-5 days
AG PK dose adjustments via peak/trough
SCr Q3-5
51
Q

what are some options for inhaled abx for CF pts

A

tobramycin**
aztreonam**
amikacin
colistin

**ones she talked about the most

52
Q

inhaled tobramycin can be used for what two main things in CF pts?

A

for INITIAL pseudomonas eradication - one 28 day course
OR
suppression therapy for pts with CHRONIC pseudomonas (in 28 day cycles)

53
Q

inhaled aztreonam is used when in CF pts?

A

only for SUPPRESSION/chronic pseudomonas – use in alternating months with tobramycin

54
Q

inhaled tobramycin or inhaled aztreonam:

which one is more often (TID) but shorter administration time overall?

A

aztreonam

55
Q

inhaled tobramycin or inhaled aztreonam:

which one is BID and administered for 15 - 20 minutes at a time

A

tobra

56
Q

inhaled tobramycin or inhaled aztreonam:

which one causes a cough and why?

A

tobra – because it is a powder

57
Q

benefits of nebulization?

A
  • 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
Q

what does ABPA stand for

A

allergic bronchopulmonary aspergillosis

59
Q

how to treat ABPA

A

prednisone burst then taper

and then use an antifungal that covers aspergillus (voriconazole, itraconazole, posaconazole)

60
Q

what are the possible liver issues that come CF?

A

focal biliary cirrhosis (biliary obstruction/progressive periportal fibrosis)
liver steatosis
liver failure

61
Q

pancreatic issues in CF?

A 
mucus obstructs exocrine ducts
decreased enzymes (amylase/lipase/protease)
decrease HCO3 output
62
Q

Pancreatic enzymes:

typical starting dose?

A

usually 1000 units of lipase/kg/meal

63
Q

Pancreatic enzymes:

do not exceed ______ units of lipase/kg/day

A

10,000

64
Q

Pancreatic enzymes are adjust based on what things>

A

of stools per day
fat content of stools
and growth/weight

65
Q

want to supplement what kind of vitamins?

A

fat soluble (ADEK)

66
Q

supplement want kind of minerals?

A

Ca2+
iron
SODIUM!! they loose so much in their sweat

67
Q

what are some drugs that can be used as an appetite stimulant

A

cyproheptadine
mirtazapine
dronabinal

PHRM 865 (59 decks)

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