Cystic Fibrosis: Adults

Question 1

What percentage of patients have recurrent pulmonary infections and pancreatic insufficiency?

Answer 1

80%

Question 2

Why is there persistent respiratory infections in CF?

Answer 2

Decrease in mucociliary clearance

Increase in bacterial adherence

Reduce in endocytosis of bacteria

Question 3

What is the effect of CF on bronchiectasis?

Answer 3

Causes Aggressive progressive bronchiectasis: chronic sputum production (purulent

Question 4

What is the result of decreased mucociliary clearance?

Answer 4

Increased bacterial colonisation

Question 5

What is the result of increased bacterial colonisation?

Answer 5

Inflammation

Mucus plugging

Airway ulceration

Airway damage

Question 6

What is the result of Inflammation

Airway plugging

Airway ulceration

Airway Damage?

Answer 6

Bronchiectasis?

Question 7

What is the result of bronchiectasis?

Answer 7

More

Decreased mucociliary clearance

Increased bacterial adherence

Decreased endocytosis

Question 8

Describe the onset of bronchiectasis in CF patients?

Answer 8

Aggressive and progressive - chronic sputum production

Question 9

What respiratory problems besides bronchiectasis do CF patients face?

Answer 9

Recurrent lower respiratory tract infections

Progressive airflow obstruction (survival rate related to FEV1)

Increase in exertional dyspnoea

Haemoptysis (associated with infection - possible embolisation)

Pneumothorax- poor prognosis

Question 10

Describe the onset of colonisation of pseudomonas aeruginosa

Answer 10

21% colonisation under 1 year

80% colonisation over 26 years

Question 11

Where is pseudomonas aeruginosa acquired from?

Answer 11

Environment (particularly hospitals)

Other CF patients

Question 12

How does pseudomonas aeruginosa protect itself from host defences?

Answer 12

Once colonised, it undergoes mucoid change. It forms a biofilm - rapidly acquires antibiotic resistance

Question 13

What is colonisation of pseudomonas aeruginosa associated with?

Answer 13

Reduced life expectancy 28 vs 39 years

Rapid decline in lung function

Question 14

How does the body respond to the presence of a biofilm?

Answer 14

Neutrophils and macrophages release proteinases and oxidants (free radicals) produce tissue damage

Question 15

What is treatment for first isolation of pseudomonas Aeruginosa?

Answer 15

Attempt eradication with oral ciprofloxacin & nebulised colomycin 3/12

If fails, i.v. ceftazidime & nebulised colomycin

Question 16

Where is Burkholderia cepacia acquired from?

Answer 16

Environment

Other CF patients

Question 17

What is Colonisation of Burkholderia cepacia associated with?

Answer 17

Reduced life expectancy 16 vs 39 years

Rapid decline in lung function

Some patients ‘cepacia syndrome,’ very rapid deterioration

Question 18

What is the resistance of burkholderia cepacia?

Answer 18

Innate resistance to most antibiotics

Question 19

What does Genomovar 3 indicate (a group of Burkholderia cepacia-like organisms)?

Answer 19

Contraindication for transplantations

Question 20

Which organism is usually secondary to pseudomonas?

Answer 20

Stentrophomonas maltophila

Question 21

Which bacterium is resistant to all anti-tuberculosis chemotherapy and is a contraindication for transplantation.

Answer 21

Mycobacterium abscessus

Question 22

How do you treat recurrent respiratory tract infections?

Answer 22

Early and aggressively with antibiotics

Question 23

What bacteria would you treat with oral antibiotics?

Answer 23

Staph, Haemophilus, Pneumococcus

Question 24

Which bacteria do you treat with IV antibiotics?

Answer 24

Pseudomonas, Stenotrophomonas, Burkholderia

Question 25

What is the format of antibiotic administration?

Answer 25

Two antibiotics (b-lactam + aminoglycoside)

If multiply resistant, test for synergy between antibiotic combinations

Large doses (increased volume of distribution, increased clearance)

Two week courses

Question 26

What is the function of the Ivacaftor?

Answer 26

Addresses primary defect of CTFR

CTFR potentiator, binds to CTFR and improves the transport of chloride ions

Improves lung function (10%)

Weight gain

Reduces sweat chloride

Feel much better

Question 27

What are the indications for a double lung transplant?

Answer 27

Rapidly deteriorating lung function

FEV1 < 30% predicted

Life threatening exacerbations

Estimated survival <2 years

Question 28

What is the likelihood of dying on the waiting list for a lung?

Answer 28

30-40% die on waiting list

Question 29

What is survival rate for lung transplantation?

Answer 29

70-80% at 5 years

50% at 10 years

Gradual attrition due to bronchiolitis obliterans