Cystic Fibrosis Flashcards

(75 cards)

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
What are the main active ingredients in inhaled antibiotics?
Tobramycin (in many forms e.g. Solution, powder) Aztreonam
26
What's the brand name of Tobramycin Inhaled Solution (used in chronic lung inf in CF)?
TOBI
27
What's the brand name of Tobramycin Inhalation Powder (used in chronic lung inf in CF)?
TOBI Podhaler
28
What's the brand name of Aztreonam Inhalation Solution (used in chronic lung inf in CF)?
Cayston
29
SE of Tobramycin Inhaled Solution (TOBI) used in chronic lung inf in CF?
Ototoxicity Tinnitus Voice alteration Dizziness Bronchospasm
30
How should u use the doses of Tobramycin Inhaled Solution (TOBI) used in chronic lung inf in CF?
Doses should be taken at least 6 hrs apart
31
Storing ofTobramycin Inhaled Solution (TOBI) used in chronic lung inf in CF?
Refrigerator Can be kept at room up to 28 days Store in FOIL, to protect from light
32
SE of TOBI Podhaler (used in chronic lung inf in CF)?
Same as TOBI Ototoxicity, tinnitus, voice alteration, dizziness, bronchospasm
33
How should TOBI Podhaler be used? (CF)
Use with Podhaler
34
How should the doses of TOBI Podhaler be spaced?
Doses should be taken no less than 6 hrs apart, should be taken as close to 12 hrs (same as TOBI)
35
Storage of TOBI Podhaler?
Store capsules at room temp in DRY place | Different from TOBI, refrigerator recommended as storage
36
SE of Aztreonam Lysine Inhalation Solution (Cayston) used in CF?
Allergic rxns (may be severe) Bronchospasm Fever Wheezing Cough Chest discomfort
37
How should the doses of Aztreonam (Cayston) be spaced (in CF)?
At least every 4 hours apart | Different from TOBI and TOBI Podhaler... Q 6 hrs
38
How should Aztreonam (Cayston) be used in CF?
Reconstitute with 1ml of sterile diluent. Give immediately
39
How should Aztreonam (Cayston) be stored in CF?
Recommend to refrigerate, can be kept at room temp up to 28 days Protect from light (Storage is same as TOBI)
40
List oral antibiotic used in CF
Azithromycin
41
Whats the recommended order of use, if pt is on multiple meds? (Hypertonic saline, Chest physiotherapy, Bronchodilator, inhaled antibiotics, Dornase alfa)
Bronchodilator -> Hypertonic saline -> Dornase alfa -> Chest physiotherapy -> Inhaled antiobiotics (use last)
42
List agents that promote mucus clearance in CF
Albuterol Hypertonic saline (HyperSal) Dornase alfa (Pulmozyme)
43
What's the brand name of Hypertonic Saline used to promote mucus clearance in CF?
HyperSal
44
What's the brand name of Dornase alfa used to promote mucus clearance in CF?
Pulmozyme
45
Howz Hypertonic saline (HyperSal) used?
In small ampules delivered via a nebulizer
46
How should Dornase alfa (Pulmozyme) used in CF be stored?
Store ampules in refrigerator. (Don't expose to room temp >= 24 hrs) Protect from light
47
Can Dornase alfa (Pulmozyme) be mixed with another drug in the nebulizer?
No! Don't mix with any other drug in nebulizer
48
How many tabs of multivitamin do pts with CF use daily?
At least 1-2 tabs of multivitamins with A, D, E, K
49
What's Pancrelipase? Used as a Pancreatic Enzyme Pdt (PEPs) in CF
Pancrelipase is a natural pdt harvested from porcine pancreatic glands which contains a combination of LIPASE, AMYLASE, and PROTEASE.
50
Howz Pancrelipase dosed?
Dose is individualized for each pt and is based on the LIPASE component
51
How's the dose of Pancrelipase adjusted to a pt with CF?
Once enzyme therapy is started, dose is adjusted Q 3-4 days UNTIL STOOLS ARE NORMALIZED
52
What's the max dose of Pancrelipase? Why?
> 6,000 units/kg/meal of lipase Due to colonic structure risk
53
When is Pancrelipase used?
Enzymes are given PRIOR to meals and snacks Full doses before meals 50% of mealtime dose before snacks
54
What causes a bump in Pancrelipase dose used?
Meals with high fat content req higher doses
55
Can the capsules of Pancrelipase be chewed or crush in CF?
Don't chew or crush the capsules
56
What can be done if a pt can't swallow Pancrelipase?
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
How should Pancrelipase be used wrt entire dose and liquid?
Take entire dose at the beginning of each meal or snack Take with a generous amt of liquid
58
Should u retain Pancrelipase in your mouth? Why or why not?
Don't retain in mouth before swallowing; swallow immediately Retention may cause mucosal irritation and stomatitis
59
Cam pancreatic enzyme pdts be substituted?
No! Don't substitute pancreatic enzyme pdts. This is an FDA recommendation
60
How do u store Pancrelipase pdts?
They don't require refrigeration
61
List brand names of Pancrelipase
Creon Lip-Prot-Amyl Pancreaze Pertzye Ultresa Viokace Zenpep
62
SE of Pancrelipase use?
Mucosal irritation Abdominal pain (stomach pain) Nausea Headache Neck pain
63
Are all formulations of Pancrelipase interchangeable?
No!
64
Should u mix Pancrelipase with dairy pdts such as milk or yogurt?
No! Don't mix
65
Hydration and Pancrelipase use?
Drink lots of non-caffeinated liquids every day
66
T/F? Is it important to follow the diet plan to get adequate nutrition and to keep as healthy as possible?
True
67
How u deal with missed Pancrelipase?
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
What's used to target the G1551D-CFTR Protein Defect in CF?
Ivacaftor
69
What's Ivacaftor?
Agent used to target the G1551D-CFTR Protein Defect in CF
70
How should Ivacaftor be used wrt food?
Take with high-fat containing food
71
List agents used in CF that DO require refrigeration
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
What's the usual dose of Ivacaftor (Kalydeco)?
150mg PO Q12H
73
How does CYP450 3A4 mod inhibitor or moderate-severe hepatic impairment affect Ivacaftor (Kalydeco) dosing?
Reduce daily dose - 150mg PO Daily (usually 150mg PO Q12H)
74
How does CYP34A strong inhibitors affect dosing of Ivacaftor (Kalydeco)?
150mg twice weekly (usual dose is 150mg Q12h)
75
What xteristic of cystic fibrosis?
Thick, viscous secretions