Cystic Fibrosis

Question 1

What is Cystic Fiborsis?

Answer 1

• incurable disease that is caused by a mutation in cystic fibrosis transmembrane conductance regulator (CFTR)

Question 2

What does the mutation in CFTR do within the body?

Answer 2

• Causes abnormal levels of Chlorde, Bicarb, Sodium = thick mucus secretion
• Affects breathing, increased risk of lung infections, and digestive complications

Question 3

What is way that Cystic Fibrosis is diagnosed?

Answer 3

• Sweat Test - looking for “salty” sweat

Question 4

What are some of the Classic Symptoms of cystic fibrosis?

Answer 4

• salty tasting skin, poor growth, poor weight gain
• Mucus production, frequent lung infections, coughing, SOB
• Fatty stools

Question 5

What is the proper order that one with cystic fibrosis should taken their medications?

Answer 5

• 1st: Inhaled Bronchodilators (opens airways)
• 2nd: Hypertonic Saline (moves mucus)
• 3rd: Dornase Alfa (thins mucus)
• 4th: Chest Physiotherapy (moves mucus)
• 5th: Inhaled Antibiotics (controls infections)

Increases the antibiotics ability to get into the lungs

Question 6

What are some of the bugs that are seems within intermittent infection of Cystic Fibrosis?

Answer 6

• S. Aureus or H. Flu (EARLY)
• Pseudomonas (more in kids and adults)

IV ANTIBIOTICS is best

Question 7

What are some of the bugs seen in Chronic infections for Cystic Fibrosis?

Answer 7

• Pseudomonas (USE INHALED ANTIBIOTICS)

28 days on then 28 days off

Question 8

What are some of the airway clearance therapies (Inhaled) that are used for Cystic Fibrosis?

Answer 8

• Albuterol
• Hypertonic Saline
• Dornase Alfa (Pulmozyme)

Question 9

What are some of the side effects for Dornase Alfa?

Answer 9

• Chest pain, fever, rash, rhinitis, voice change

Question 10

What are some addtional notes for Dornase Alfa?

Answer 10

• Store in fridge
• Protect from light
• DO NOT mix with other drugs

Question 11

What are some of the inhaled antibiotics that are used for Cystic Fibrosis?

Answer 11

• Tobramycin (TOBI, TOBI Podhaler)
• Aztreonam

target Pseudo

Question 12

What are some of the side effects for Tobramycin used for Cystic fibrosis?

Answer 12

• Ototoxicity, tinnitus, voice alterations, mouth/throat irritation

Question 13

What are some of the additional notes for tobramycin used for cystis fibrosis?

Answer 13

• 28 days on then 28 days off
• 6 hours apart
• TOBI: refrigerate & DO NOT mix with other drugs
• TOBI Podhaler: Room temp & DO NOT swallow caps

Question 14

What are some of the side effects for Aztreonam used for Cystic fibrosis?

Answer 14

• Allergic reactions, bronchospams, fever, wheezing, cough chest discomfort

Question 15

What are some addtional notes for Atreonam used for cystic fibrosis?

Answer 15

• 28 days on then 28 days off (could do alternate to tobra?)
• 4 hours apart
• Refrigerate & DO NOT mix with other drugs

Question 16

What are some of the Oral antibiotics used for cystric fibrosis?

Answer 16

• Azithormycin (decrease inflammation and exacerbations)

Question 17

What are some of the CFTR Modulators that are used for Cystic Fibrosis?

Answer 17

• Ivacaftor (Kalydeco)
• Lumacaftor/Ivacaftor (Orkambi)
• Tezacaftor/Ivacaftor (Symdeko)
• Elexacaftor/Tezacaftor/Ivacaftor (Trikafta)

Question 18

What is the way that Ivacaftor works in the body for cystic fibrosis?

Answer 18

• Increases the time that the CFTR channel is open –> enchance Cl movement

Question 19

What is the way that Lumacaftor, Tezacaftor, Elexacaftor work within the body for cystic fibrosis?

Answer 19

• corrects CFTR folding –> increase the amount of CFTR that is delivered to the cell surface

Question 20

What is the most common mutation that the CFTR Modulators are used for?

Answer 20

• F508del Mutations