Cystic Fibrosis & Bronciectasis

Question 1

Define cystic fibrosis (CF)

Answer 1

• multisystem, childhood dz
• genetic disorder of the exocrine glands
• fibrotic dz with obstructive features

Question 2

What does CF affect?

Answer 2

• plasma membrane Na/Cl channel of epithelial cells in lungs

Question 3

What is the result of CF’s affect?

Answer 3

• excessive, viscous, sticky, mucous plug

Question 4

What other systems are affected in CF?

Answer 4

• GI
• reproductive
• nearly all exocrine ducts

Question 5

What is the MC, lethal, autosomal recessive inheritance in caucasians?

Answer 5

• CF

Question 6

What is the affected gene in CF?

Answer 6

• CFTR

Question 7

What is the pathophysiology of CF?

Answer 7

• aberrant CFTR protein allows too much influx of Na/Cl
• causes inadequate hydration of bronchotraceal epithelium
• impairs mucocilliary fxn
• allows oportunistic bacteria

Question 8

What is the result of the pathophysiology of CF?

Answer 8

• thick, sticky mucous
• decreased mucocilliary movement
• dehydration of mucuous
• biofilm formation from mucous adhereing
• neutrophilic DNA

Question 9

What is the main cause of death in CF?

Answer 9

• respiratory failure

Question 10

What is linked to accelerated lung disease and death in CF?

Answer 10

• poor nutrition

Question 11

What are the complications of CF?

Answer 11

• cysts
• bullae
• bronciectasis
• hemoptysis
• PNA
• PAH
• cor pulmonale
• clubbing

Question 12

What are the non-pulmonary symptoms of CF?

Answer 12

• meconium ileus (intestinal blockage)
• pancreatic insufficiency
• constitutional symptoms

Question 13

What are the lung S&S of CF?

Answer 13

• persistent cough
• persistent wheezing
• excessive sputum
• barrel chest
• recurrent/severe PNA
• digital clubing

Question 14

What is the PNA bug that complicates CF?

Answer 14

• P. aerunginosa

Question 15

How does the PNA bug complicate CF?

Answer 15

• adheres to mucoid biofilm
• highly resistant to abx
• progresses to function pulm decline and bronchiectasis

Question 16

How is CF detected?

Answer 16

• universal screenings at birth: blood test to detect elevated trypsinogen
• Sweat Test

Question 17

What will the sweat test show in CF?

Answer 17

• excessive NaCl

Question 18

What is the main function of the CFTR protein?

Answer 18

• reabsorb Cl- and Na+ ions in sweat glands

Question 19

What does a loss of CFTR proteins lead to in the sweat test?

Answer 19

• decreased reabsorption and NaCl

- hypertonic sweat

Question 20

What diagnostic test are used for CF?

Answer 20

• sweat test
• if sweat test is inconclusive: nasal potential difference measurement or molecular CFTR DNA mutation test
• PFTs
• CXR

Question 21

What do the PFTs show in CF?

Answer 21

• obstructive patter progressing to restrictive

Question 22

What is the goal of tx in children with CF?

Answer 22

• delay P. aeruginosa

Question 23

What is the goal of tx in adults with CF?

Answer 23

• suppression of established P. aeruginosa

Question 24

What is the best tx approach to CF?

Answer 24

• multidiciplinary

Question 25

What are the txs for CF?

Answer 25

• chest PT

- positive expiratory pressure mask

Question 26

How is PNA treated in CF patients?

Answer 26

• need ID consult

- long term abx

Question 27

How are complications of CF prevented?

Answer 27

• clearance of viscous, lower airway secretions via inhaled DNAase or hypertonic saline
• vaccinations

Question 28

What is the definitive tx for CF?

Answer 28

• lung transplant