Brainscape's Knowledge GenomeTM

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


See full index

Cystic Fibrosis > Cystic Fibrosis > Flashcards

Cystic Fibrosis Flashcards

1
Q

Pathophysiology of Cystic Fibrosis?

A

A mutation on the gene for the protein cystic fibrosis transmembrane conductance regulator (CFTR).
This mutation causes an abnormal transport of chloride, bicarbonate and sodium ions across the epithelium causing thick, viscous secretions in the lungs, pancreas, liver and intestines.

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

Symptoms of Cystic Fibrosis?

A
  • Difficulty breathing
  • Lung infections
  • Digestive complications
  • Salty tasting skin
  • Poor growth
  • Poor weight gain
  • Steatorrhea (fatty stool)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

At what age is Cystic Fibrosis diagnosed?

A

70% by the age of 2

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

How is Cystic Fibrosis diagnosed?

A

Newborn screening (NBS)

If NBS is positive, then sweat test is preformed to confirm diagnosis, by measuring the chloride in the sweat. CF pt have high chloride in the sweat.

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

When treating lung complications in Cystic Fibrosis patients with airway clearance therapy and inhaled antibiotic therapy, what is the preferred sequence of treatment administration?

A

Airway clearance therapy

  • 1st: Bronchodilator (Albuterol)
  • 2nd: Hypertonic saline (via nebulizer to match pH level of airway)
  • 3rd: Dornase alfa

then

  • 4th: Chest physiotherapy

then

  • 5th: Inhaled antibiotic therapy

Note: PO meds for CF patient can be given at anytime

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

What is the primary treatment goal in Cystic Fibrosis patients?

A
  • Preventing/treating lung infections
  • Maintaining adequate nutrition
  • Optimize quality of life
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Which bacterias are most commonly known to cause lung infections in Cystic Fibrosis patients?

A
  • Staph aureus
  • H. Influenzae
  • Pseudomonas aeruginosa

(in that order)

Note: Lung infection cause by Pseudomonas are associated with a more rapid decline in pulmonary function

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

Symptoms of acute pulmonary exacerbation in Cystic Fibrosis patients?

A
  • Increased cough
  • Increased sputum production with change in color to greenish
  • Shortness of breath
  • Rapid decline in FEV1
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

In Cystic Fibrosis patients with an acute pulmonary exacerbation due to infection, what is the duration of antibiotic treatment?

A

2 - 4 weeks

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

In Cystic Fibrosis patients with a lung infection caused by Pseudomonas, what is recommended?

A

Large dose of 2 IV antibiotics which cover Pseudomonas

Usually:

  • Aminoglycosides
  • Beta-lactams
  • Quinolones
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

True of False: In Cystic Fibrosis patients, lung infection starts out intermittently at first but eventually becomes chronic.

A

True

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

What 2 treatment methods are recommended for Cystic Fibrosis patients with chronic lung infection caused by Pseudomonas?

A

1) Inhaled antibiotics cycled with 28 days on therapy , followed by 28 days off
2) 6 month trial of Azithromycin

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

Since Azithromycin has no Pseudomonas coverage, why is it still considered, and in some cases recommended, in Cystic Fibrosis patients with chronic lung infection caused by Pseudomonas?

A

It can disrupt the biofilm formation by the bacteria which can improve lung function and decrease exacerbations

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

Dornase alfa MOA

A

Works by degrading extracellular DNA in the lungs to decrease mucus viscosity

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

Dornase alfa clinical pearls (3)

A
  • Do NOT mix with any other drug in the nebulizer
  • Protect form light
  • Store ampules in the refrigerator
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What are the 2 inhaled antibiotics available for Cystic Fibrosis patients with chronic lung infection?

A
  • Tobramycin
  • Aztreonam

(both have Pseudomonas coverage)

17
Q

Other than the Lungs, what other organ is effected by Cystic Fibrosis? What is the pathophysiology?

A

Gastrointestinal tract:

The thick mucus obstructs enzyme flow resulting in the lack of pancreatic enzymes such as lipase, amylase, and protease from reaching the gastrointestinal tract and causes malabsorption.

Tell-tell sign of this: Steatorrhea (fatty stool)

18
Q

What PO drugs are used in the treatment of Cystic Fibrosis patients?

(other than Azithromycin)

A

Pancreatic Enzyme Products (PEP)
Pancrelipase: pancreatic enzyme (lipase, amylase, and protease) replacement

  • Pancrelipase (Creon, Viokace, Zenpep)

Dose adjusted until stool is normal

19
Q

Clinical pearls wrt Pancreatic Enzyme Products (PEP)

A
  • Viokace is the only PEP that is a tablet
  • PEP formulations are not interchangeable
  • PEP capsules should not be crush or chewed, but can be sprinkled on a spoonful of soft food such as applesauce
  • Capsules must be swallowed immediately after placing in the mouth. Don’t let sit in the mouth
  • Protect PEP from light
20
Q

MOA of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Modulators

A

Increases the time the CFTR channels remain open and thereby increases the amount of CFTR delivered to the cell surface

21
Q

What are the agents in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Modulators class

A
  • Kalydeco
  • Orkambi
  • Symdeko
22
Q

Which Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Modulators agent is approved for the homozygous F508del mutation

A
  • Orkambi

- Symdeko

23
Q

What are some supplements and recommendation in Cystic Fibrosis patients?

A
  • Vitamin A, D, E, K supplements
  • High fat and calorically-dense diet
  • Most CF patient will develop CF-related diabetes mellitus so they will need insulin replacement.
24
Q

Inhaled antibiotic Tobramycin (TOBI Podhaler) clinical pearl

A
  • 1 dose consists of 4 capsules inhaled one at a time.
  • Take doses as close to 12 hours but no less than 6 hours apart.
  • Keep at room temp in a dry please

Cystic Fibrosis (1 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions