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NAPLEX 2023 > 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, bicarb, and 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

high amount of chloride in CF patients

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5
Q

Classic symptoms of CF

A

salty tasting skin, poor growth and poor weight gain, coughing and SOB, frequent infections

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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

correct order critical to maximize absorption

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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 pseudomonas

A

2 IV drugs to provide potential synergy and prevent resistance
- aminoglycosides, BL, quinolones, and others that cover pseudomonas

doses larger tha normal to address altered PK in CF patients

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10
Q

What is recommended for patients with chronic pseudomonas 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 activity against pseudomonas but can be used to disrupt biofilms)

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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

decreases viscosity of mucus

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17
Q

Where should dornase alfa ampules be stored ___ and protected ____

A

in the refrigerator
and protected from light

think of keeping them in the refrigerator door

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18
Q

Which inhaled antibiotics are used to target pseudomonas

A

Tobramycin, aztreonam, azactam

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19
Q

Tobramycin brand name

A

TOBI, TOBI Podhaler (capsule for inhalation)

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20
Q

Side effects of inhaled tobramycin

A

ototoxicity, tinnitus, voice alterations, mouth & throat pain

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21
Q

Tobramycin inhaled doses must be at least ___ hrs apart

A

6

22
Q

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

A

4

remember the As look like 4

23
Q

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

A

28

24
Q

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

A

False

25
Q

TOBI podhaler must be kept

A

at room temp

26
Q

Side effects of inhaled Aztreonam and Azactam

A

allergic reactions (may be severe), bronchospasm, fever, wheezing, cough, chest discomfort

27
Q

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

A

28

28
Q

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

A

false

29
Q

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

A

Pancrelipase

30
Q

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

A

basic

31
Q

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

A

individualized
lipase

32
Q

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

A

stools are normalized

33
Q

Pancrelipase brand names

A

Creon - (remember: panCRElipase)
Zenpep - (remember PEP)
Viokace - (remember ending ACE)

34
Q

Max dose for all ages of pancrelipase

A

≤ 10,000 units/kg/day

35
Q

Pancrelipase warnings

A

fibrosing colonopathy advancing to colonic strictures, mucosal irritation

36
Q

Pancrelipase side effects

A

abdominal pain, flatulence, nausea

37
Q

What is the purpose of pancreatic enzyme replacement in CF

A

helps digest food, maintain weight and improve nutrient absorption

38
Q

T/F: PEP formulations are interchangeable

A

false

39
Q

Which PEP comes as a tablet

A

Viokace

40
Q

Viokace must be given with

A

a PPI (it is not enteric coated)

41
Q

Which PEPs come as capsules

A

Creon and Zenpep

42
Q

When should PEPs be taken

A

before or with all meals and snacks

43
Q

How much of the PEP dose should be taken with snacks

A

50% of the mealtime dose

44
Q

Ivacaftor MOA

A

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

45
Q

Lumacaftor, tezacaftor and elexacaftor MOA

A

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

46
Q

What must be performed prior to initiation of CFTR modulators

A

genotype testing

47
Q

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

A

F508del

48
Q

Ivacaftor is not approved for which mutation?

A

F508del homozygous

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

NAPLEX 2023 (88 decks)

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