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NAPLEX 2024 > 55. Cystic Fibrosis > Flashcards

55. Cystic Fibrosis Flashcards

1
Q

T/F: Cystic fibrosis is a curable disease

A

False

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2
Q

Cystic fibrosis is caused by a mutation in the gene for the protein _____ and this mutation causes abnormal transport of _____ ion across the epithelium, leading to thick, viscous secretions

A

Cystic fibrosis transmembrane conductance regulator (CFTR)
Chloride, bicarbonate, and sodium

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3
Q

CF causes thick viscous secretions which affect ____, causing _____

A

the lungs, pancreas, liver, and intestines
causes difficulty breathing, lung infections, and digestive complications

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4
Q

The avg life expectancy of a person with CF is ___ yo with more than 75% of pts being diagnosed by ___ yo

A

35-40
2 yo

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5
Q

Dx of cystic fibrosis

A

Newborn screening first 2-3 days after baby is born
Initial screening identifies risk and then sweat chloride test (“sweat test”) is performed to confirm (looking for high salt (chloride) in sweat)

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6
Q

S/sx of CF

A

salty tasting skin, poor growth and poor weight gain (despite adequate food intake), mucus production, frequent lung infections, coughing and SOB

Patients experience obstruction of pancreatic ducts causing steatorrhea (Fatty stools) and poor absorption of nutrients (including fat-soluble vitamins)

Clubbing of the fingers may be present

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7
Q

____ and failure to thrive can result if CF is not treated

A

Malnutrition

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8
Q

For treatment of lung complications from CF, correct order when administering inhaled medications is crucial to maximize absorption and effect. What is the order?

A

1) inhaled bronchodilators (e.g. albuterol) - opens the airway
2) Hypertonic saline (e.g. HyperSal) - mobilizes mucus to improve airway clearance
3) Dornase alfa (Pulmozyme) - decreases viscosity of (thins) mucus to promote airway clearance
4) Chest physiotherapy - mobilizes mucus to improve airway clearance
5) Inhaled abx - controls airway infection

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9
Q

Inhaled therapies are the foundation of treatment in CF. The drug is delivered directly to the lungs, resulting in _____

A

minimal systemic absorption (reduces risk of toxicity)

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10
Q

The most common organisms seen early in CF disease are ___ and ___, followed by ____ in adolescents and adults

A

Staphylococcus aureus
H. influenzae
Pseudomonas aeruginosa

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11
Q

Acute pulmonary exacerbations are characterized by an increase in _____

A

cough, sputum production with change in sputum color (greenish), SOB, and a rapid decline in FEV1

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12
Q

T/F: For intermittent infections caused by Pseudomonas, monothearpy aminoglycosides is recommended

A

False - 2 IV drugs are recommended potential synergy and prevent resistance

Includes aminoglycosides, beta-lactams, quinolones, and others that cover Pseudomonas aeruginosa

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13
Q

When treating intermittent infection, IV abx doses tend to be (smaller/larger) than normal to address altered PK in pts with CF

A

larger

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14
Q

What is the difference in abx use in intermittent vs chronic infection caused by psuedomonas CF?

A

Intermittent - 2 IV drugs
Chronic - inhaled abx, 28 days on and 28 days off

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15
Q

Azithromycin does not cover pseudomonas but why is it used in cystic fibrosis?

A

Disrupts biofilm formation by bacteria which can improve lung function and decrease exacerbations

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16
Q

Dornase alfa (Pulmoyzme) storage notes

A

Store ampules in the refrigerator (do not expose to room temp ≥ 24 hrs)
Protect from light

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17
Q

T/F: Dornase alfa (Pulmozyme) cannot be mixed with any other drug in the nebulizer

A

True

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18
Q

What are inhaled abx used in CF lung infections?

A

Tobramycin (TOBI solution for inhalation, TOBI Podhaler capsule for inhalation)
Aztreonam (Cayston solution for inhalation)

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19
Q

Side effects of tobramycin (TOBI, TOBI Podhaler, Bethkis, Kitabis Pak)

A

Ototoxocity, tinnitus, voice alteration, mouth/throat pain, dizziness, bronchospasm

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20
Q

Tobramycin (TOBI, TOBI Podhaler, Bethkis, Kitabis Pak) dosing schedule

A

On + off cycle (28 days on, 28 days off)
Dosed every 12 hrs, must be at least 6 hrs apart

21
Q

Tobramycin (TOBI, TOBI Podhaler, Bethkis, Kitabis Pak) storage notes

A

TOBI, Bethkis, Kitabis: refrigeration recommended (can be kept at room temp up to 28 days); protect from light; do not mix with any other drug in nebulizer
TOBI Podhlaer: room temp in dry place; use with Podhaler device; do NOT swallow capsules

22
Q

T/F: Tobramycin Podhaler capsules can be PO or nebulized in a special Podhaler device

A

Flase - do NOT swallow capsules, nebulizer only

23
Q

Inhaled Aztreonam (Cayston) side effects

A

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

24
Q

Inhaled Aztreonam (Cayston) dosing schedule

A

On + off cycle (28 days on, 28 days off)
Dosed every 8 hrs but must be at least 4 hrs apart

25
Q

Inhaled Aztreonam (Cayston) storage notes

A

Refrigeration recommended, can be kept at room temp up to 28 days
Protect from light
Use with Altera nebulizer system

26
Q

Why do most CF patients supplement their diet with pancreatic enzyme products (PEPs)?

A

Thick mucus in CF obstructs pancreatic enzyme flow, resulting in a lack of these enzymes reaching the GI tract and subsequent malabsorption (causes smelly greasy/oily stools)

PEPs help break down fat, starches, and protein

27
Q

Pancrelipase is a natural produce harvested from ____ which contains a combination of _____

A

Porcine pancreatic glands
Lipase, amylase, and protease

28
Q

PEPs are formulated to dissolve in the more basic pH of ___

A

duodenum

29
Q

PEP is individualized for each pt and based on the ___ component

A

Lipase

30
Q

PEP dosing is adjusted every ___ until stools are normalized

A

3-4 days

31
Q

Max dosing of pancrelipase

A

Lipase ≤2500 units/kg/meal or ≤10,000 units/kg/day

Note: Doses >6,000 units/kg/meal are a/w colonic stricture

32
Q

What are some examples of pancrelipase products

A

Creon
Viokace
Zenpep

33
Q

Warnings pancrelipase

A

Fibrosing colonopathy advancing to colonic strictures (rare, higher risk with doses >10,000 lipase units/kg/day), mucosal irritation, hyperuricemia

34
Q

Side effects pancrelipase

A

Abdominal pain, flatulence, nausea, HA, neck pain

35
Q

Most PEP formulation is capsules. Which PEP formulation is the only PEP that is a tablet (non-enteric coated) and requires PPI?

A

Viokace

36
Q

T/F: PEP formulations are not interchangeable

A

True

37
Q

PEP administration timing

A

before or with all meals and snacks
High fat meals may require higher doses
Use 50% of mealtime dose with snacks

38
Q

Can PEP capsules be opened to be sprinkled on soft food?

A

DR capsules with enteric coated microspheres/microtablets may be opened and sprinkled on soft, acidic foods (pH≤4.5) like applesauce
Avoid foods with high pH such as dairy

Note: do not retain capsule contents in mouth, swallow immediately with water to avoid mucosal irritation and stomatitis

39
Q

PEP should be dispensed in original container except ___

A

Zenpep and some Creon strengths

40
Q

The most common mutation in CFTR gene is ____

A

homozygous F508del mutation (2 copies of the same allele)

41
Q

Which CFTR modulators are approved for homozygous F508del mutation+additional responsive mutations?

A

Lumacaftor/ivacaftor (Orkambi)
Tezacaftor/ivacaftor (Symdeko)

42
Q

Which CFTR modulators are approved for heterozygous F508del mutation+additional responsive mutations??

A

Elexacaftor/tezacaftor/ivacaftor (Triakfta)

43
Q

Which CFTR modulators are NOT approved for homozygous F508del mutation but approved for use in other responsive mutations?

A

Ivacaftor (Kalydeco)

44
Q

Ivacaftor MOA

A

Increasing the time the CFTR channels remain open = enhances chloride transport activity

45
Q

Lumacaftor, tezacaftor, elexacaftor MOA

A

Help correct CFTR folding defect, which increases the amount of CFTR dilvered to the cell surface

46
Q

What type of diet is recommended to help with nutrition, normal weight and growth, increased energy needs and to prolong survival?

A

High fat and calorically dense diet

47
Q

What vitamin supplements are required for CF patients?

A

Fat-solute vitamins A, D, E, and K

48
Q

T/F: many pts with CF will eventually require metformin for treatment of CF-related DM

A

False - insulin

NAPLEX 2024 (74 decks)

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