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a - naplex > Chapter 55: Cystic Fibrosis > Flashcards

Chapter 55: Cystic Fibrosis Flashcards

1
Q

What mutation causes cystic fibrosis

A

cystic fibrosis transmembrane conductance regulator (CFTR)

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2
Q

The mutation for CF causes

A

abnormal transport of chloride, bicarband sodium ions across the epithelium

leading to thick, viscous secretions

which affects the lungs, pancreas, liver and intestines

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3
Q

Most patients are diagnosed with CF by what age

A

2 years

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4
Q

What is used to determine a CF diagnosis

A

Sweat test

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5
Q

Classic symptoms of CF

A

salty tasting skin

poor growth

poor weight gain

coughing and SOB

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6
Q

Patients with CF experience obstruction of pancreatic ducts, causing _____ and poor absorption of nutrients, including fat-soluble vitamins

A

steatorrhea (fatty stools)

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7
Q

What is the correct order to administer medications for CF

A
  1. inhaled bronchodilators (e.g., albuterol) - opens airways
  2. Hypertonic saline (e.g., HyperSal) - mobilizes mucus
  3. Dornase alfa (Pulmozyme) - thins mucus to promote airway clearance
  4. Chest physiotherapy - mobilizes mucus to improve airway clearance
  5. Inhaled antibiotics - controls airway infection
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8
Q

Most common organisms seen early in CF

A

Staph aureus & H. influenzae,

followed by PSA in adolescents and adults

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9
Q

Treatment for intermittent infections in CF caused by PSA

A

2 IV drugs to provide potential synergy and prevent resistance

aminoglycosides
BL
quinolones

and others that cover PSA

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10
Q

What is recommended for patients with chronic PSA lung infections to reduce the bacterial burden

A

Inhaled antibiotics

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11
Q

How are inhaled antibiotics used in chronic PSA lung infections for CF

A

cycled with 28 days on therapy and 28 days off

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12
Q

Which antibiotic can be considered for patients with chronic infection who are worsening on conventional treatment to decrease inflammation and exacerbations

A

Azithromycin (has no direct bactericidal activity against PSA)

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13
Q

How is hypertonic saline administered

A

via nebulizer

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14
Q

Dornase alfa brand name

A

Pulmozyme

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15
Q

T/F: dornase alfa can be mixed with other drugs in a nebulizer

A

false

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16
Q

Which medication thins mucus

A

dornase alfa

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17
Q

Where should dornase alfa ampules be stored

A

in the refrigerator

think of keeping them in the refrigerator door

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18
Q

Dornase alfa must be protected from

A

light

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19
Q

Which inhaled antibiotics are used to target PSA

A

tobramycin

aztreonam

azactam

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20
Q

Tobramycin brand name

A

TOBI

TOBI Podhaler (capsule for inhalation)

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21
Q

Side effects of inhaled tobramycin

A

ototoxicity

tinnitus

voice alterations

mouth & throat pain

22
Q

Tobramycin inhaled doses must be at least ___ hrs apart

A

6

23
Q

Aztreonam and Azactam inhaled doses must be at least ___ hrs apart

A

4

remember the As look like 4

24
Q

TOBI, Bethkis, and Kitabis are recommended to be kept refrigerated, but can be kept at room temp up to __ days

A

28

25
Q

T/F: TOBI, Bethkis, and Kitabis can be mixed with other meds in the nebulizer

A

False

26
Q

Tobipodhaler must be kept

A

at room temp

27
Q

Side effects of inhaled Aztreonam and Azactam

A

allergic reactions (may be severe) bronchospasm

fever

wheezing

cough

chest discomfort

28
Q

Aztreonam and Azactam are recommended to be kept refrigerated, but can be kept at room temp up to __ days

A

28

29
Q

T/F: Aztreonam and Azactam can be mixed with other meds in the nebulizer

A

false

30
Q

____ is a natural product harvested from porcine pancreatic glands which contains a combination of lipase, amylase and protease

A

Pancrelipase

31
Q

Pancreatic enzyme products (PEPs) are formulated to dissolve in the more (acidic/basic) pH of the duodenum

A

basic

32
Q

The dose of pancrelipase is individualized for each patient and is based on the ____ component

A

lipase

33
Q

Once PEP therapy is started, the dose is adjusted every 3-4 days until

A

stools are normalized

34
Q

pacrelipase brand names

A

Creon - (remember: panCRElipase)

Zenpep - (remember PEP)

Viokace - (remember ending ACE)

35
Q

Max dose for all ages of pancrelipase

A

< / = 10,000 units/kg/day

36
Q

Pacrelipase warnings

A

fibrosing colonopathy

advancing to colonic strictures

mucosal irritation

37
Q

Pacrelipase side effects

A

abdominal pain
flatulence
nausea

38
Q

What is the purpose of pancreatic enzyme replacement in CF

A

helps digest food
maintain weight
improves nutrient absorption

39
Q

T/F: PEP formulations are not interchageable

A

True

40
Q

Which PEP comes as a tablet

A

Viokace

41
Q

Viokace must be given with

A

a PPI (it is not enteric coated)

42
Q

Which PEPs come as capsules

A

Creon

Zenpep

43
Q

When should PEPs be taken

A

before or with all meals and snacks

44
Q

How much of the PEP dose should be taken with snacks

A

50% of the mealtime dose

45
Q

Ivacaftor MOA

A

increases the time the CFTR channels remain open, which enhances chloride transport activity

46
Q

Lumacaftor, tezacaftor and elexacaftor MOA

A

help correct the CFTR folding deficit, which increases the amount of CFTR delivered to the cell surface

47
Q

What must be performed prior to initiation of CFTR modulators

A

genotype testing

48
Q

The most common mutation in the CFTR gene is a homozygous ____ mutation

A

F508 del

49
Q

Which drugs are approved for use in the homozygous F508del mutation

A

Lumacaftor/ivacaftor
tezacaftor/ivacaftor
elexacaftor/tezacaftor/ivacaftor

50
Q

How should CFTR modulators be taken

A

with high-fat containing food

a - naplex (83 decks)

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