Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

NAPLEX 21 > Cystic Fibrosis > Flashcards

Cystic Fibrosis Flashcards

1
Q

an incurable, hereditary disease caused by a mutation in the gene for the protein cystic fibrosis transmembrane conduct regulator (CFTR)

A

CF

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What does the CFTR mutation cause…

abnormal transport of ____,____and____ ions across the epithelium.. which causes?

A

abnormal transport of chloride, bicarbonate, and sodium ions across the epithelium, leading to think, viscous secretions affecting the lungs, pancreas, liver and intestines

–> difficulty breathing, lung infections, digestive complications

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

When are CF patients normally diagnosed? what age?

A

by age 2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

If the initial screening identifies a risk of CF, then what test is performed?

A

a sweat chloride test, “sweat test”

confirms diagnosis - HIGH sodium in CF sweat

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What are the classic symptoms of CF?

A

salty tasting skin, poor growth, and poor weight gain, coughing, SOB.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Patients experience obstruction of pancreatic ducts causing…

A

steatorrhea (fatty stools).

Malnutrition can result if CF is not treated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

In CF, what is the first intervention (opens the airways)

A

inhaled bronchodilators (albuterol)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

In CF, what is the second intervention (mobilizes mucus to improve airway clearance)

A

hypertonic saline (HyperSal)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

In CF, what is the third intervention (decreases viscosity of mucus to promote airway clearance)

A

Dornase alfa (pulmozyme)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

In CF, what is the fourth intervention (mobilizes mucus to improve airway clearance)

A

chest physiotherapy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

In CF, what is the fifth and final intervention (controls airway infection)

A

inhaled antibiotics (targets p. aeruginosa)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

The correct order of inhaled medication for CF is critical for…

A

to maximize absorption and effect

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Do inhaled therapies have large systemic absorption?

A

no! minimal, small risk of toxicity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Impaired mucus clearance causes bacterial colonization and lung infections.

The most common organisms seen EARLY in the disease are:

A

staphylococcus aureus

H. influenzae

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Which organism is commonly found in adolescents and adults?

A

Pseudomonas aeruginosa

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is characterized as an increase in cough, sputum production with change in sputum color (green), SOB and a rapid decline in FEV1

A

acute pulmonary exacerbations

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

How are infections caused by p aeruginosa treated?

A

2 IV antibiotics to provide synergy and prevent resistance, doses tend to be LARGER than normal

options: AG, beta lactase, quinolone and others that covers pseudomonas

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

For chronic infections, what is the recommended treatment for patients with pseudomonas lung infections?

A

inhaled antibiotics to reduce the bacterial burden; treatment is cycled with 28 days on, 28 days off

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

For chronic infections when a patient is receiving inhaled antibiotics, when should their bronchodilator/mucolytic be administered?

A

BEFORE antibiotics

20
Q

Does azithromycin have direct bactericidal activity against pseudomonas?

A

no! but it disrupts biofilm formation by the bacteria which can improve lung function and decrease exacerbations

21
Q

List airway clearance therapies (3)

A

1) bronchodilator (albuterol)
2) Hypertonic saline (HyperSal, PulmoSal) - nebulizer
3) Dornase alfa (Pulmozyme)

22
Q

This drug works by degrading extracellular DNA in the lungs to decrease viscosity of thin mucus

A

Dornase alfa/ Pulmozyme

23
Q

Which drug should NOT be mixed with any other drug in the nebulizer?

A

dornase alfa/ Pulmozyme

24
Q

Which drug has to have the ampules stored in the fridge and need to be protected from light?

A

dornase alfa/ pulmozyme

25
Q

List the inhaled antibiotics that target pseudomonas

A

1) tobramycin (TOBI, TOBI Podhaler)
2) aztreonam (Cayston)
3) azactam (IV) - acute

26
Q

This drug’s side effect profile:

ototoxicity, tinnitus, voice alteration, mouth and throat pain

A

tobramycin INH

28 on, 28 off

27
Q

tobramycin - this formulation recommends refrigeration, but can be kept at room temp for up to 28 days, but can’t be mixed with any other drug in the nebulizer

A

TOBI, bethkis, kitabis

28
Q

tobramycin - this formulation has the capsules stored at room temp

A

TOBI Podhaler

q12 hours

29
Q

These drug’s side effect profile: allergic reactions may be severe, bronchospasm, fever, wheezing, cough, chest discomfort

refrigeration recommended! do not mix with any other drug in the nebulizer

A

Aztreonam

azactam

28 on, 28 off

q8 hours

30
Q

This oral drug is used off-label to decrease inflammation and decease exacerbations

A

azithromycin (Zithromax) PO

31
Q

what do pancreatic enzyme products (PEPs) break down?

A

fat

starches

protein

32
Q

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

A

pancrelipase

33
Q

PEPs are formulated to dissolve in the more BASIC pH where?

A

duodenum

34
Q

How are PEPs dosed?

A

individualized for each patient based on the LIPASE component

dose is adjusted every 3-4 days until stools are normalized

35
Q

Brand: Creon, Viokace, Zenpep

A

generic: pancrelipase

36
Q

What is the max dose of Creon?

A

<10,000unit/kg/day

37
Q

This drug has a warning for colonic strictures (rare, but higher risk once passed the max dose), can also cause mucosal irritation

side effects include abdominal pain, flatulance, nausea

A

Creon

38
Q

Is creon interchangeable?

A

no!

39
Q

Which PEP is a tablet? the only one; what must it be taken with?

(all others are capsules - want to be taken with acidic food, not dairy)

A

Viokace - it is non-enteric coached and MUST be given with a PPI

40
Q

do you refrigerate PEPs?

A

no! but protect from moisture

Zenpep is not dispense in original container

41
Q

This drug works by increasing the time the CFTR channels open, which enhances chloride transport activity

A

Ivacaftor (CFTR modulator)

> 6 months

substrate of CYP3A4…D/I!!

42
Q

These drugs help correct the CFTR folding defect, which increases the amount of CFTR delivered to the cell surface

A

Lumacaftor, tezacaftor, elexavaftor (CFTR modulators)

43
Q

Because each CFTR modulator is approved for very specific mutations, what must be performed prior to initiation?

A

genotype testing

44
Q

What is the most common mutation in the CFTR gene?

A

homozygous F508del mutation

Orkambi>2y, Symdeko >6y, Trikafta >12y approved

45
Q

What type of meal do you take CFTR modulators with?

A

high fat

46
Q

What vitamin supplements are required for CF?

A

fat-soluble! ADEK for cellular function

47
Q

Many patients with CF will eventually require…

A

insulin.. CF-related DM

NAPLEX 21 (60 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions