Cystic Fibrosis

Question 1

Malnutrition is common in CF, how to manage it

Answer 1

Management

• High calorie diet
• Vit supplement: ADEK
• Pancreatic enzymes

Question 2

Tobi

• Dose/frequency
• Indication

Answer 2

Tobromycin

• 300mg Neb BID 28d on and 28d off
• CF Chronic pseudo colonization

Question 3

Dornase alpha

• MOA
• Indication
• Dose
  If also on Tobi, give which one first
• Age
• Neb use:

Answer 3

Pulmonzyme

• MOA: mucolytic
• Indication: CF
• Dose: 1 amp by INH QD. Do not mix w/ other nebulizer
  If also use w/ Tobi, use dornase alpha first.
• Age: > 6 y.o
• Neb: Pari LC jet Nebulizer

Question 4

Creon

• Contain
• Time of administration
• Counseling

Answer 4

Pancreatic enzymes

• Contain: amylase, lipase, proteinase
• Taken just b/4 meals and snacks

Counseling

• Avoid Ca carbonate and Mg: effect coating of beads
• Do not chew/crush
• Take w/ liquid and swallow whole
• May mix in small amt of soft food
• Avoid mix w/ milk base foods
• do not mix enzymes into foods ahead of time
• Keep enzymes at RT
• Do not fridge
• Do NOT accept generic
• Enzymes are needed with all meals and snacks
• Some foods and drinks do not require enzymes b/c they contain only simple carbo that digest easily.

Question 5

Pancrease

Answer 5

Pancreatic enzyme

Question 6

Pancrecarb

Answer 6

Pancreatic enzyme

Question 7

Ultrase

Answer 7

Pancreatic enzyme

Question 8

Viokase

Answer 8

Pancreatic enzyme

Question 9

Cayston

• Form:
• Indication
• Frequency
• Storage

Answer 9

Aztreonam

• Form:
• Indication: CF chronic pseudo colonization
• Frequency: 750 mg INH TID 28 days on and 28 days off
• Store in fridge.
  RT ok for 28d.
  Protect from light

Question 10

Kalydeco

• Indication
• Age

Answer 10

Ivacaftor

• CF pt with G551D mutation
• Age: > 6 y.o

Question 11

Hypertonic saline

• Indication
• SEs

Answer 11

• CF

- ADEs: cough, sore throat, chest pain, tissue irritation