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Pharmacotherapeutics > Cystic Fibrosis (Exam 2) > Flashcards

Cystic Fibrosis (Exam 2) Flashcards

1
Q

Cystic fibrosis is most common in

A

caucasian newborns

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2
Q

Cystic fibrosis results from

A

genetic mutation of CFTR

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3
Q

Most common CF mutated gene

A

F508delta

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4
Q

CF is a ____ ____ disease

A

autosomal recessive
one mutation must be present on each allele

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5
Q

Normal process of making CFTR

A

CFTR protein moves through cell surface (trafficking) then becomes a chloride channel to maintain balance of fluid in airways

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6
Q

Most common CFTR mutation class

A

Class II
Protein made but misfolded

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7
Q

Pathophys of CFTR mutation

A

Thicker mucus layer
Decreased mucus clearance

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8
Q

Which organ systems are affected in CF patients?

A

Pancreas
Lungs
Sinuses
GI and Liver
Testies

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9
Q

Most deficient enzyme in CF is _____ and ___ is used to treat

A

Lipase

PERT

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10
Q

Symptoms of pancreatic insufficiency

A

Steatorrhea
Bloating
Stools sticking to toilet
Orange ring
Weight loss

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11
Q

PERT (pancreatic enzyme replacement therapy)

A

Porcine lipase, protease, and amylase

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12
Q

May need ____ or ____ with PERT as some require higher pH

A

H2RA

PPI

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13
Q

Why are PERTs enteric-coated microspheres within capsule?

A

Withstand acidic environments
Can be crushed and added to formula

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14
Q

How should PERTs be taken?

A

Beginning of all meals/foods containing calories
Take extra with fatty meal

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15
Q

PERT Adverse Effects

A

Headache, abdominal pain, dyspepsia, neck pain, nasal congestion, lymphadenopathy

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16
Q

Types of PERTs

A

Creon
Pancreaze
Pertzye
Ultresa
Viokace
Zenpep

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17
Q

Are PERTs interchangeable?

A

NO

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18
Q

Main symptom when taking PERT

A

Fibrosing colonopathy (inflammation and colonic strictures)

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19
Q

Symptoms of fibrosing colonopathy

A

Diarrhea, ascites, abdominal pain, poor weight gain

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20
Q

Which PERT is not a delayed-release capsule?

A

Viokace - tablet

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21
Q

PERT for tube feeding

A

RELiZORB
Viokace - can crush + add

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22
Q

Vitamin A

A

vision, cell differentiation, immune function

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23
Q

Vitamin D

A

calcium metabolism, bone resorption

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24
Q

Vitamin E

A

antioxidant activity

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25
Q

Vitamin K

A

co-factor in prothrombin formation

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26
Q
A
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27
Q

Vitamin supplements in CF patient

A

Aquadeks
Vitamax

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28
Q

GI Complications

29
Q

Meds to treat CF GERD

A

First line: H2RAs or PPIs
Prokinetic Agents: Erythromycin, metoclopramide

30
Q

Meds to treat CF DIOS

A

First line: polyethylene glycol, lactulose

31
Q

Tx for CF liver disease

A

Ursodeoxycholic acid (UDCA or ursodiol)

32
Q

Ursodeoxycholic acid (UDCA or ursodiol) MOA

A

decreases cholesterol content of bile and bile stones
reduces hydrophobic bile acids
may improve bile flow in CF

33
Q

What should you monitor in ursodiol

A

liver blood test 3 months after beginning and each 6-12 months after

34
Q

Sinus issues in CF patients

A

Chronic infections
Frequent polyps in sinus cavity

35
Q

Management of sinuses in CF

A

Nasal toilet
Steroidal agents
Decongestant agents

36
Q

_____ accounts for 90% of CF fatalities.

A

Respiratory
Affects both upper and lower

37
Q

Management of CF

A

Bronchodilators
Mucolytic Agents
Anti-inflammatory agents
Antibiotics

38
Q

Bronchodilators are effective as ____-term treatment

39
Q

Beta2-Agonists

A

Albuterol
Levalbuterol

40
Q

Beta2-Agonist Side effects

A

anxiety, palpitations, tremor, insomnia

41
Q

Both beta2-agonists and anticholinergics are ____ to recommend with chronic CF

A

insufficient

42
Q

Mucolytic agents

A

Pulmozyme (Dornase alfa)
Hypertonic saline

43
Q

Mucolytic agents MOA

A

Decrease viscosity of secretions
Increase clearance of sputum

44
Q

Dornase alfa (Pulmozyme)

A

recombinant human DNA (rhDNase) that cleaves DNA
targets free extracellular DNA in CF sputum

45
Q

Hypertonic saline

A

Rehydrate CF sputum by creating hyperosmotic gradient
Increases mucociliary airway clearance

46
Q

Monitor ___ in mucolytic agents

47
Q

Dornase alfa is strongly recommended in _____ CF

48
Q

Anti-inflammatory agents

A

NSAIDs - slow rate of decline
Macrolide antibiotics - improve function + reduce exacerbations

49
Q

Chronic use of NSAIDs is recommended in ____ patients but not ____

A

pediatric

adult

50
Q

Azithromycin is NOT recommended for patients chronically infected with

A

pseudomonas aeruginosa

51
Q

Most common bacterial pathogens in CF

A

Staph. aureus
Pseudomonas aeruginosa

52
Q

Antibiotics for CF

A

Tobramycin
Aztreonam
Colistimethate
(28 days on/28 days off)

53
Q

Which antibiotic is not safe for pregnancy?

A

Tobramycin

54
Q

Which inhaled antibiotics are strongly recommended in moderate to severe lung disease?

A

Tobramycin
Azetreonam

55
Q

Order of airway clearance therapy

A

1.) Bronchodilators
2.) Mucolytics
3.) Anti-inflammatory agents
4.) Antibiotics

56
Q

Pharmacokinetic considerations for CF antibiotics

A

larger volume of distribution
enhanced total body clearance

57
Q

CFTR Modulators

A

Kalydeco
Orkambi
Symdeko
Trikafta

58
Q

Take CFTR modulators with

A

fat-containing food

59
Q

CFTR Modulator Warnings

A

AST/ALT elevation
Cataracts
DDI - CYP3A inducer/substrates

60
Q

CYP3A4 Inhibitors

A

itraconazole
clarithromycin
fluconazole

61
Q

CYP3A4 inducers

A

rifampin
phenobarbital
carbamazepine
phenytoin

62
Q

Ivacaftor (Kalydeco)

A

Potentiator
Increases CFTR open channels
G551D mutation
4+ months

63
Q

Lumacaftor/Ivacaftor (Orkambi)

A

Corrector + Potentiator
Corrects folding in F508del
2+ years with 2 copies of F508del

64
Q

Trikafta is preferred for ____ homozygotes > 6 to 11 years

65
Q

Tezacaftor/Ivacaftor (Symdeko)

A

Corrector + Potentiator
Heterozygous
6+ years with 2 copies of F508del

66
Q

Elexacaftor/Tezacaftor/Ivacaftor (Trikafta)

A

Next-generation corrector
2+ years with 1 copy of F508del

67
Q

elexacaftor and tezacaftor MOA

A

move proteins to reach cell surface

68
Q

ivacaftor MOA

A

helps CFTR protein stay open longer

69
Q

Vanzacaftor/Tezacaftor/Deutivacaftor (Alyftrek)

A

6+ years old
with fat-containing meal
one copy of F508del

Pharmacotherapeutics (16 decks)

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