Exam 5 - Cystic Fibrosis Flashcards Preview

Therapeutics V Spring 2019 (P3 Spring) > Exam 5 - Cystic Fibrosis > Flashcards

Flashcards in Exam 5 - Cystic Fibrosis Deck (67):
1

Autosomal (dominant or recessive) genetic disease

recessive

2

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

cystic fibrosis transmembrane conductance regulator (CFTR)

3

what does ENaC stand for

epithelial sodium channel

4

Presenting Signs and Symptoms in Neonates?

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

5

Presenting Signs and Symptoms in infants and children?

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

6

Presenting Signs and Symptoms in adolescentes/adults?

delayed sexual maturation
nasal polyposis

7

most common mutation in CF?

delta F508

8

pathophys/results from CFTR mutations?

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

9

How to do a newborn screen?

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

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

10

For diagnosis fo CF:
need at least ______ sign/symptom + evidence of ______

1 sign or symptom

11

what are some tests for CF?

sweat chloride test -- gold standard
genetic testing
pancreatic function tests

12

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

stool fat quantitation
quantitation of trypsin activity

13

how does the sweat test work?

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

14

what is a positive sweat test?

> 60 mEq/L of chloride in the sweat

15

Ivacaftor/Kalydeco:
what class of drug is it?

CFTR potentiator: helps "open the gate"

16

Ivacaftor/Kalydeco:
Counseling points?

take with fatty food to help absorption

17

Ivacaftor/Kalydeco:
monitoring?

LFTs Q3months x 1 yr
eye exam - baseline and yearly

18

Ivacaftor/Kalydeco:
drug interactions?

CYP3A substrate
(dont forget grapefruit!)

19

Orkambi -- what are the generic drugs in it?

ivacaftor/lumacaftor

20

Orkambi:ivacaftor/lumacaftor:
have to have what mutation?

HOMOZYGOUS delta F508

21

Orkambi:ivacaftor/lumacaftor:
counseling?

take with fatty foods
hormonal birth control will not be effectice

22

Orkambi:ivacaftor/lumacaftor:
drug interactions?

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

23

Orkambi:ivacaftor/lumacaftor:
monitoring?

AST/ALT/Bil q3month for 1 year and then yearly
Eye exam baseline and then yearly - peds

24

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

Kalydeco has the MUCH better FEV1 increase

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

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