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Flashcards in Cystic Fibrosis Deck (17)
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1
Q

Cystic Fibrosis

A

A genetic mutation for the protein CFTR leading to progressive loss of pulmonary function

Affects the Lungs and Pancreas.

2
Q

Clinical symptoms

A

Lungs: frequent lung infections, thick mucus production

Pancreas: steatorrhea (fatty stool) malnutrition due to poor absorption of fat soluble vitamins

3
Q

TXT for lungs

A
Bronchodilator - open airways
Hypertonic Saline - Mobilize mucus
Dornase alpha - Thins mucus
chest physiotherapy - Mobilize mucus
inhaled antibiotics - Reduce airway infection and disrupt P. Ag biofilm
4
Q

Pathogens causing infections

A

Staph aureus, H. Influenzae, Pseudomonas

5
Q

Pseudomonas in CF

Acute, Chronic

A

Acute: Give two IV drugs to provide synergy and prevent resistance.

Chronic: Inhaled Antibiotics 28 days on and 28 days off.

6
Q

Hypertonic Saline

Brand, Use

A

HyperSal, PulmoSal

Use: Mucus clearance (Mobilize mucus)

7
Q

Dornase Alfa

Brand, Use and storage

A

Pulmozyme
Use: Thins Mucus

Do not mix with other drugs in nebulizer

store in refrigerator and protect from light

8
Q

Tobramycin Inhalation

Brand, use, SE, Dose timing, Storage

A

TOBI, Bethkis, Kitabis PAK
Use: Inhaled ABX in children 6 and above

SE: Ototoxicity, Tinnitus, dizziness, VOICE ALTERATIONS, bronchospasms, mouth and throat pain

Dose 6 hours apart

refrigerator reccomended but can be kept in room temp up to 28 days

9
Q

Tobramycin Inhaler powder

A

TOBI PODhaler
USE: ABX in children 6 and above

SE: SE: Ototoxicity, Tinnitus, dizziness, VOICE ALTERATIONS, bronchospasms, mouth and throat pain

Dose 6 hours apart

Do not swallow capsules store in room temp and dry place

10
Q

Aztreonam

A

Brand: Cayston (Inhalation), Azactam (IV)
Use: ABX in children 7 and above

SE: Allergic reaction, Bronchospasm, fever, wheezing, cough, chest discomfort

DOSE: at least 4 hours apart
Notes: Refrigeration recommended (kept at room temp for 28 days)
Reconstitute with 1ml of sterile diluent and give immediately

11
Q

Azithromycin

A

Zithromax

Off-label to decrease inflammation and reduce exacerbations

12
Q

Treatment of Malabsorption

5 points

A

High FAT and calorically dense diet

Pancreatic Enzyme replacement

PPI - use with Viokace to reduce GERD

Vitamin Supplement - ADEK

Insulin for CF related T2D

13
Q

Pancreatic Enzyme Products (PEP)

A

to replace pancreatic enzymes in the GI
(Lipase, amylase, and protease)

Dose is based on lipase content

Adjusted 3- 4 days until stools are normalized

14
Q
Pancrelipase
Brand (6), SE, Warning, Max dose
A

Brand: Creon, pancreaze, Pertyze, Ultresa, Viokace(non-EC must give with PPI), Zenpep

SE: Stomach Pain, Gas, Nausea

Warning: Colonic strictures (>6,000u/kg/day), mucosal irritation

Max dose: 10,000u/kg/day or <2,500u/kg/meal

15
Q
Pancrelipase 
Study Tip (4)
A

Viokace is non-EC tablet give with PPI

Formulations are not interchangeable.

ALL other are DR capsules, swallow with a full glass of water (non caffinated liquids)

Keep in OG container and protect from moisture and do not refrigerate

If capsule is difficult to swallow sprinkle on spoonful of applesause

16
Q

Ivacaftor

Brand, MOA, Use, Notes

A

Brand: Kalydeco

MOA: increase the amount of time the chloride transport channel remains open to increase chloride activity.

Use: >2 years of age with one of 23 specific mutations in the CFTR gene. Not for homozygous CFTR F508del

With high fat meals

17
Q

Lumacaftor/Ivacaftor

Brand, Use, notes

A

Brand: Orkambi (Think bi for homozygous mutation)

Use in pt >6 and above who are homozygous for the CFTR F508del mutation

With high fat meals