cystic fibrosis- adults

Question 1

what are some of the problems associated with CF?

Answer 1

recurrent pulmonary infections
pancreas insufficiency
gastrointestinal problems
diabetes 
liver disease
osteoporosis
fertility issues

Question 2

why do sufferers of CF suffer from persistent respiratory infections?

Answer 2

decreased mucocilliary clearance
increased bacterial adherence
decreased endocytosis of bacteria

Question 3

what does the bacterial infection caused by CF cause?

Answer 3

inflammation
mucus plugging
airway ulceration
airway damage

Question 4

what can CF ultimately lead to with recurrent respiratory infections?

Answer 4

bronchiectasis

Question 5

what sort of sputum is produced with bronchiectasis?

Answer 5

purulent

Question 6

what sort of respiratory failure will a sufferer of CF go into first?

Answer 6

type 1 (then into type 2)

Question 7

why may haemoptosis be caused by CF?

Answer 7

it is associated with infection and bronchiactisis

Question 8

what are some respiratory complications of CF?

Answer 8

bronchiectasis
recurrent LRTIs
progressive airflow obstruction
respiratory failure
haemoptysis
pneumothorax

Question 9

what are some of the bacteria that commonly infect CF patients?

Answer 9

pseudomonas aeruginosa
staphylococcus aureus
haemophilus influenzae
stenotrophomonas maltophilia
burkholderia cepacia
mycobacterium abscessus

Question 10

what does pseudomonas aeruginosa form on infection?

Answer 10

a biofilm

Question 11

what is the purpose of a biofilm?

Answer 11

protects the bacteria from host defences and antibiotics

Question 12

why is pseudomonas aeruginosa difficult to treat?

Answer 12

it produces a biofilm and rapidly acquires multiple antibiotic resistance

Question 13

what do you treat the first isolation of pseudomonas aeruginosa with?

Answer 13

oral ciprofloxacin and nebulised colomycin

Question 14

what is the second treatment for pseudomonas aeruginosa if first treatment fails?

Answer 14

ceftazidime and nebulised colomycin

Question 15

what does a colonisation of pseudomonas aeruginosa do to the prognosis for a CF patient?

Answer 15

reduced life expectancy

rapid decline in lung function

Question 16

what does a colonisation of burkholdena cepacia do to the prognosis of a CF patient?

Answer 16

reduces life expectancy

rapid decline in lung function

Question 17

what is cepacia syndrome?

Answer 17

patient has a very large immune response to burkholdena cepacia infection causes very rapid deterioration

Question 18

does mycobacterium abscessus affect the ability of the patient to have a lung transplant?

Answer 18

yes it is a complete contraindication

Question 19

what are the two antibiotics given to CF patients for recurrent respiratory tract infections?

Answer 19

B-lactam and aminoglycoside (together)

Question 20

what is the name of the new drug with the possibility to cure CF in some patients?

Answer 20

ivacaftor

Question 21

what are the indications for lung transplantation in CF patients?

Answer 21

• Rapidly deteriorating lung function
• FEV1 < 30% predicted
• Life threatening exacerbations
• Estimated survival <2 years