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NAPLEX Content > Cystic Fibrosis > Flashcards

Cystic Fibrosis Flashcards

1
Q

What organs do the xteristic thick, viscous secretion affect? Effect?

A

The thick mucus mostly affects the:

Lungs, Pancrese, Liver and Intestine

Primarily causes:

Difficulty breathing and lung infections as well as digestive complications

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2
Q

List the classic sx of cystic fibrosis (CF)?

A

Salty tasting skin

Poor growth and poor weight gain despite adequate food intake

Thick and sticky mucus production

Freq chest infection

Coughing

Shortness of breathe

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3
Q

List digestive sx associated with CF

A

Steatorrhea

Malnutrition due to poor absorption of nutrients, including fat-soluble vitamins, and a failure to thrive if not treated

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4
Q

List tx for lung problems ass with CF

A

Inhaled bronchodilators

Hypertonic saline (HyperSal)

DNAse enzyme therapy

Inhaled antibiotics

Oral Azithromycin

Transplantation

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5
Q

What’s the use of inhaled bronchodilators in CF?

A

Open the airways

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6
Q

What’s the use of Hypertonic saline (HyperSal) in CF?

A

To hydrate the airway mucus secretion

And

Facilitate mucociliary clearance

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7
Q

What’s the use of DNAse enzyme therapy in CF?

A

Used to breakdown extracellular DNA from accumulated neutrophils.

DNAse therapy is designed to THIN MUCUS and facilitate mucociliary clearance

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8
Q

What’s the use of inhaled antibiotics in CF?

A

To prevent and treat lung and sinus infections

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9
Q

What’s the use of oral Azithromycin in CF?

A

To reduce airway inflammation and disrupt P. aeruginosa biofilm formation

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10
Q

What’s the use of transplantation in CF?

A

In pts with end-stage dx

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11
Q

List tx for intestinal and nutritional problems

A

High fat and calorically-dense diet to help with nutrition

Pancreatic enzyme replacement to optimize growth and nutritional status and promote healthy bowels

Proton pump inhibitors to prevent degradation of pancreatic enzymes in stomach and for tx of GERD

Vitamin supplements, especially the fat-soluble vits A, D, E, K

Insulin for tx of CF-related diabetes mellitus

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12
Q

What’s used to help with nutrition in CF (tx for intestinal and nutritional problems)?

A

High-fat and calorically-dense diet

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13
Q

What’s used to optimize growth and nutritional status and promote healthy bowels in CF (tx for intestinal and nutritional problems)?

A

Pancreatic enzyme replacement

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14
Q

What’s used to prevent degradation of pancreatic enzymes in the stomach and for tx of GERD in CF (tx for intestinal and nutritional problems)?

A

Proton pump inhibitors

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15
Q

What vitamin supplements are essential in CF (tx for intestinal and nutritional problems)?

A

Fat-soluble vitamins A, D, E and K

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16
Q

What’s used for tx of CF-related diabetes mellitus (tx for intestinal and nutritional problems)?

A

Insulin

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17
Q

Types of CF infections?

A

Intermittent infection

Chronic infection

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18
Q

In intermittent lung infection due to CF, which organisms are most the common culprits?

A

Staphylococcus aureus and Haemophilus influenza

Followed by

P. aeruginosa

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19
Q

What xterizes acute pulmonary Exacerbations in CF?

A

Increase in cough

Sputum pdt with change in sputum color (greenish)

Shortness of breathe

Rapid decline in FEV1

Loss of appetite and weight

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20
Q

How do u treat a pt with P. aeruginosa infection in CF?

A

2 drugs given IV are recommended to provide potential synergy and prevent resistance:

Aminoglycosides
Beta lactams
Quinolones
And others that cover p. aeruginosa

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21
Q

What organism is associated with chronic lung infections in CF?

A

P. aeruginosa

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22
Q

What may be used to eradicate P. aeruginosa from the lungs?

A

Inhaled antibiotics

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23
Q

What must be given prior to using inhaled antibiotics in CF?

A

If pt is on bronchodilator and/or mucolytic, make sure to give these before using inhaled Antiobiotic

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24
Q

List inhaled antibiotics used in CF

A

Tobramycin inhaled solution (TOBI)

Tobramycin inhalation powder (TOBI Podhaler)

Tobramycin inhalation solution (Bethkis)

Aztreonam Lysine Inhalation Solution (Cayston)

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25
Q

What are the main active ingredients in inhaled antibiotics?

A

Tobramycin (in many forms e.g. Solution, powder)

Aztreonam

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26
Q

What’s the brand name of Tobramycin Inhaled Solution (used in chronic lung inf in CF)?

A

TOBI

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27
Q

What’s the brand name of Tobramycin Inhalation Powder (used in chronic lung inf in CF)?

A

TOBI Podhaler

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28
Q

What’s the brand name of Aztreonam Inhalation Solution (used in chronic lung inf in CF)?

A

Cayston

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29
Q

SE of Tobramycin Inhaled Solution (TOBI) used in chronic lung inf in CF?

A

Ototoxicity

Tinnitus

Voice alteration

Dizziness

Bronchospasm

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30
Q

How should u use the doses of Tobramycin Inhaled Solution (TOBI) used in chronic lung inf in CF?

A

Doses should be taken at least 6 hrs apart

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31
Q

Storing ofTobramycin Inhaled Solution (TOBI) used in chronic lung inf in CF?

A

Refrigerator

Can be kept at room up to 28 days

Store in FOIL, to protect from light

32
Q

SE of TOBI Podhaler (used in chronic lung inf in CF)?

A

Same as TOBI

Ototoxicity, tinnitus, voice alteration, dizziness, bronchospasm

33
Q

How should TOBI Podhaler be used? (CF)

A

Use with Podhaler

34
Q

How should the doses of TOBI Podhaler be spaced?

A

Doses should be taken no less than 6 hrs apart, should be taken as close to 12 hrs (same as TOBI)

35
Q

Storage of TOBI Podhaler?

A

Store capsules at room temp in DRY place

Different from TOBI, refrigerator recommended as storage

36
Q

SE of Aztreonam Lysine Inhalation Solution (Cayston) used in CF?

A

Allergic rxns (may be severe)

Bronchospasm

Fever

Wheezing

Cough

Chest discomfort

37
Q

How should the doses of Aztreonam (Cayston) be spaced (in CF)?

A

At least every 4 hours apart

Different from TOBI and TOBI Podhaler… Q 6 hrs

38
Q

How should Aztreonam (Cayston) be used in CF?

A

Reconstitute with 1ml of sterile diluent. Give immediately

39
Q

How should Aztreonam (Cayston) be stored in CF?

A

Recommend to refrigerate, can be kept at room temp up to 28 days

Protect from light

(Storage is same as TOBI)

40
Q

List oral antibiotic used in CF

A

Azithromycin

41
Q

Whats the recommended order of use, if pt is on multiple meds? (Hypertonic saline, Chest physiotherapy, Bronchodilator, inhaled antibiotics, Dornase alfa)

A

Bronchodilator -> Hypertonic saline -> Dornase alfa -> Chest physiotherapy -> Inhaled antiobiotics (use last)

42
Q

List agents that promote mucus clearance in CF

A

Albuterol

Hypertonic saline (HyperSal)

Dornase alfa (Pulmozyme)

43
Q

What’s the brand name of Hypertonic Saline used to promote mucus clearance in CF?

A

HyperSal

44
Q

What’s the brand name of Dornase alfa used to promote mucus clearance in CF?

A

Pulmozyme

45
Q

Howz Hypertonic saline (HyperSal) used?

A

In small ampules delivered via a nebulizer

46
Q

How should Dornase alfa (Pulmozyme) used in CF be stored?

A

Store ampules in refrigerator. (Don’t expose to room temp >= 24 hrs)

Protect from light

47
Q

Can Dornase alfa (Pulmozyme) be mixed with another drug in the nebulizer?

A

No!

Don’t mix with any other drug in nebulizer

48
Q

How many tabs of multivitamin do pts with CF use daily?

A

At least 1-2 tabs of multivitamins with A, D, E, K

49
Q

What’s Pancrelipase? Used as a Pancreatic Enzyme Pdt (PEPs) in CF

A

Pancrelipase is a natural pdt harvested from porcine pancreatic glands which contains a combination of LIPASE, AMYLASE, and PROTEASE.

50
Q

Howz Pancrelipase dosed?

A

Dose is individualized for each pt and is based on the LIPASE component

51
Q

How’s the dose of Pancrelipase adjusted to a pt with CF?

A

Once enzyme therapy is started, dose is adjusted Q 3-4 days UNTIL STOOLS ARE NORMALIZED

52
Q

What’s the max dose of Pancrelipase? Why?

A

> 6,000 units/kg/meal of lipase

Due to colonic structure risk

53
Q

When is Pancrelipase used?

A

Enzymes are given PRIOR to meals and snacks

Full doses before meals

50% of mealtime dose before snacks

54
Q

What causes a bump in Pancrelipase dose used?

A

Meals with high fat content req higher doses

55
Q

Can the capsules of Pancrelipase be chewed or crush in CF?

A

Don’t chew or crush the capsules

56
Q

What can be done if a pt can’t swallow Pancrelipase?

A

It can be sprinkled on soft food with low PH that doesn’t req chewing

Applesauce, gelatin, baby food etc

Don’t use with yogurt, pudding etc as they have a higher PH

57
Q

How should Pancrelipase be used wrt entire dose and liquid?

A

Take entire dose at the beginning of each meal or snack

Take with a generous amt of liquid

58
Q

Should u retain Pancrelipase in your mouth? Why or why not?

A

Don’t retain in mouth before swallowing; swallow immediately

Retention may cause mucosal irritation and stomatitis

59
Q

Cam pancreatic enzyme pdts be substituted?

A

No!

Don’t substitute pancreatic enzyme pdts. This is an FDA recommendation

60
Q

How do u store Pancrelipase pdts?

A

They don’t require refrigeration

61
Q

List brand names of Pancrelipase

A

Creon

Lip-Prot-Amyl

Pancreaze

Pertzye

Ultresa

Viokace

Zenpep

62
Q

SE of Pancrelipase use?

A

Mucosal irritation

Abdominal pain (stomach pain)

Nausea

Headache

Neck pain

63
Q

Are all formulations of Pancrelipase interchangeable?

A

No!

64
Q

Should u mix Pancrelipase with dairy pdts such as milk or yogurt?

A

No! Don’t mix

65
Q

Hydration and Pancrelipase use?

A

Drink lots of non-caffeinated liquids every day

66
Q

T/F? Is it important to follow the diet plan to get adequate nutrition and to keep as healthy as possible?

A

True

67
Q

How u deal with missed Pancrelipase?

A

Give as soon as u remember

But, if it’s close to time for the next dose, skip it and go back to usual schedule

68
Q

What’s used to target the G1551D-CFTR Protein Defect in CF?

A

Ivacaftor

69
Q

What’s Ivacaftor?

A

Agent used to target the G1551D-CFTR Protein Defect in CF

70
Q

How should Ivacaftor be used wrt food?

A

Take with high-fat containing food

71
Q

List agents used in CF that DO require refrigeration

A

TOBI (can be kept at room temp for 28 days only)

Aztreonam (Cayston) (can be kept at room temp for 28 days only)

Dornase alfa (Pulmozyme) (can be kept at room temp for 24 hrs only)

72
Q

What’s the usual dose of Ivacaftor (Kalydeco)?

A

150mg PO Q12H

73
Q

How does CYP450 3A4 mod inhibitor or moderate-severe hepatic impairment affect Ivacaftor (Kalydeco) dosing?

A

Reduce daily dose - 150mg PO Daily (usually 150mg PO Q12H)

74
Q

How does CYP34A strong inhibitors affect dosing of Ivacaftor (Kalydeco)?

A

150mg twice weekly (usual dose is 150mg Q12h)

75
Q

What xteristic of cystic fibrosis?

A

Thick, viscous secretions

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