Cystic Fibrosis

Question 1

Cystic Fibrosis

Answer 1

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

Affects the Lungs and Pancreas.

Question 2

Clinical symptoms

Answer 2

Lungs: frequent lung infections, thick mucus production

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

Question 3

TXT for lungs

Answer 3

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

Question 4

Pathogens causing infections

Answer 4

Staph aureus, H. Influenzae, Pseudomonas

Question 5

Pseudomonas in CF

Acute, Chronic

Answer 5

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

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

Question 6

Hypertonic Saline

Brand, Use

Answer 6

HyperSal, PulmoSal

Use: Mucus clearance (Mobilize mucus)

Question 7

Dornase Alfa

Brand, Use and storage

Answer 7

Pulmozyme
Use: Thins Mucus

Do not mix with other drugs in nebulizer

store in refrigerator and protect from light

Question 8

Tobramycin Inhalation

Brand, use, SE, Dose timing, Storage

Answer 8

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

Question 9

Tobramycin Inhaler powder

Answer 9

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

Question 10

Aztreonam

Answer 10

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

Question 11

Azithromycin

Answer 11

Zithromax

Off-label to decrease inflammation and reduce exacerbations

Question 12

Treatment of Malabsorption

5 points

Answer 12

High FAT and calorically dense diet

Pancreatic Enzyme replacement

PPI - use with Viokace to reduce GERD

Vitamin Supplement - ADEK

Insulin for CF related T2D

Question 13

Pancreatic Enzyme Products (PEP)

Answer 13

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

Question 14

Pancrelipase
Brand (6), SE, Warning, Max dose

Answer 14

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

Question 15

Pancrelipase 
Study Tip (4)

Answer 15

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

Question 16

Ivacaftor

Brand, MOA, Use, Notes

Answer 16

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

Question 17

Lumacaftor/Ivacaftor

Brand, Use, notes

Answer 17

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