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Pharmacotherapeutics > Cystic Fibrosis (Exam 2) > Flashcards

Cystic Fibrosis (Exam 2) Flashcards

(69 cards)

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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
Abdominal pain

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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

Viokace since it is not an enteric coated capsule

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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
Vitamin K
co-factor in prothrombin formation
26
27
Vitamin supplements in CF patient
Aquadeks Vitamax
28
GI Complications
GERD DIOS
29
Meds to treat CF GERD
First line: H2RAs or PPIs Prokinetic Agents: Erythromycin, metoclopramide
30
Meds to treat CF DIOS
First line: polyethylene glycol, lactulose
31
Tx for CF liver disease
Ursodeoxycholic acid (UDCA or ursodiol)
32
Ursodeoxycholic acid (UDCA or ursodiol) MOA
decreases cholesterol content of bile and bile stones reduces hydrophobic bile acids may improve bile flow in CF
33
What should you monitor in ursodiol
liver blood test 3 months after beginning and each 6-12 months after
34
Sinus issues in CF patients
Chronic infections Frequent polyps in sinus cavity
35
Management of sinuses in CF
Nasal toilet Steroidal agents Decongestant agents
36
_____ accounts for 90% of CF fatalities.
Respiratory Affects both upper and lower
37
Management of pulmonary CF
Bronchodilators Mucolytic Agents Anti-inflammatory agents Antibiotics
38
Bronchodilators are effective as ____-term treatment
short
39
Beta2-Agonists
Albuterol Levalbuterol
40
Beta2-Agonist Side effects
anxiety, palpitations, tremor, insomnia
41
Both beta2-agonists and anticholinergics are ____ to recommend with chronic CF
insufficient
42
Mucolytic agents
Pulmozyme (Dornase alfa) Hypertonic saline
43
Mucolytic agents MOA
Decrease viscosity of secretions - only dornase alpha Increase clearance of sputum - both
44
Dornase alfa (Pulmozyme)
recombinant human DNA (rhDNase) that cleaves DNA targets free extracellular DNA in CF sputum
45
Hypertonic saline
Rehydrate CF sputum by creating hyperosmotic gradient Increases mucociliary airway clearance
46
Monitor ___ in mucolytic agents
FEV1
47
Dornase alfa is strongly recommended in _____ CF
moderate
48
Anti-inflammatory agents
NSAIDs - slow rate of decline Macrolide antibiotics - improve function + reduce exacerbations
49
Chronic use of NSAIDs is recommended in ____ patients but not ____
pediatric adult
50
Azithromycin is NOT recommended for patients chronically infected with
pseudomonas aeruginosa
51
Most common bacterial pathogens in CF
Staph. aureus Pseudomonas aeruginosa
52
Antibiotics for CF
Tobramycin Aztreonam Colistimethate (28 days on/28 days off)
53
Which antibiotic is not safe for pregnancy?
Tobramycin
54
Which inhaled antibiotics are strongly recommended in moderate to severe lung disease?
Tobramycin Azetreonam
55
Order of airway clearance therapy
1.) Bronchodilators 2.) Mucolytics 3.) Anti-inflammatory agents 4.) Antibiotics
56
Pharmacokinetic considerations for CF antibiotics
larger volume of distribution enhanced total body clearance
57
CFTR Modulators
Kalydeco Orkambi Symdeko Trikafta
58
Take CFTR modulators with
fat-containing food
59
CFTR Modulator Warnings
AST/ALT elevation Cataracts DDI - CYP3A inducer/substrates
60
CYP3A4 Inhibitors
itraconazole clarithromycin fluconazole
61
CYP3A4 inducers
rifampin phenobarbital carbamazepine phenytoin
62
Ivacaftor (Kalydeco)
Potentiator Increases CFTR open channels G551D mutation 4+ months
63
Lumacaftor/Ivacaftor (Orkambi)
Corrector + Potentiator Corrects folding in F508del 2+ years with 2 copies of F508del
64
Trikafta is preferred for ____ homozygotes > 6 to 11 years
F508del
65
Tezacaftor/Ivacaftor (Symdeko)
Corrector + Potentiator Heterozygous 6+ years with 2 copies of F508del
66
Elexacaftor/Tezacaftor/Ivacaftor (Trikafta)
Next-generation corrector 2+ years with 1 copy of F508del
67
elexacaftor and tezacaftor MOA
move proteins to reach cell surface
68
ivacaftor MOA
helps CFTR protein stay open longer
69
Vanzacaftor/Tezacaftor/Deutivacaftor (Alyftrek)
6+ years old with fat-containing meal one copy of F508del

Pharmacotherapeutics (28 decks)

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