Cystic Fibrosis

Question 1

Cystic fibrosis definition

Answer 1

genetic disorder effecting the lungs mostly

Question 2

Estimated survival of CF patient born in 2000s is …… years

Answer 2

> 40

Question 3

CF in adults

Answer 3

recurrent pulmonary infections and pancreatic insufficiency

or just

recurrent pulmonary infections

Question 4

Respiratory abnormalities in CF

Answer 4

less mucociliary clearance

increased bacterial adherence

reduced endocytosis of bacteria

Question 5

symptoms of cystic fibrosis

Answer 5

aggressive progressive bronchiectasis (chronic sputum production)

recurrent lower respiratory tract infections

progressive airflow obstruction (increasing dyspnoea)

respiratory failure

Question 6

Common symptoms of CF

Answer 6

Haemoptysis

Pneumothorax (common in older males, poor prognosis)

Question 7

Common bacteria in CF

Answer 7

pseudomonas aeruginosa

Question 8

CTFR usually plays a role in ……… of pseudomonas aeruginosa

Answer 8

endocytosis and destruction

Question 9

pseudomonas aeruginosa is usually acquired from

Answer 9

environment

other CF patients

Question 10

pseudomonas aeruginosa once colonised undergoes ………. and is therefore protected from host defences and has……

Answer 10

mucoid change

acquired multiple antibiotic resistance

Question 11

colonisation of pseudomonas aeruginosa is associated with

Answer 11

reduced lung function

reduced life expectancy

Question 12

Burkholderia Cepacia

Answer 12

envrionmental organsim

also reduces lung function and life expectancy

innate resistance to most antibiotics

Question 13

Stenotrophomonas maltophila

Answer 13

increasingly frequent colonisation

multiple antibiotic resistance

Question 14

Oral antibiotics for

Answer 14

staph

haemophilus

pneumococcus

Question 15

IV antibiotics for

Answer 15

pseudomonas

stenotrophomonas

Burkholderia

Question 16

Double antibiotics to recurrent respiratory infections in CF such as

Answer 16

beta lactase and aminoglycoside

Question 17

Ivacaftor

Answer 17

first new class of drug addressing the primary defect in CF

Question 18

Ivacaftor function

Answer 18

CFTR potentiator

binds to CFTR to improve the transport of chloride ions

Question 19

Ivacaftor only given to ….

Answer 19

G551D patients (Celtic mutation)

5-15% of patients

Question 20

CF does affect fertility in men

Answer 20

> 95% males are infertile

blocked or absent vas deferens

Question 21

In order for CF patients to qualify for a lung transplant they must be

Answer 21

FEV1 <30%

weight loss ++

hypoxia at rest and exercise

hypercapnia

sepsis

worsening quality of life