Cystic fibrosis in Adults Flashcards

1
Q

On average how long does a person with cystic fibrosis live for

A

around 37 years

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2
Q

what health problem do 80% of adults suffer from

A

recurrent pulmonary infections and pancreatic insufficiency

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3
Q

what health problem do 5% of adults with CF suffer from

A

gastrointestinal problems ONLY

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4
Q

what is Bronchiectasis

A

abnormal widening of the bronchi or their branches, causing a risk of infection.

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5
Q

what usually causes Bronchiectasis in patients with CF

A

bacteria build up causes an inflammation response leading to bronchieactasis

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6
Q

if a patient has Bronchieactasis what else are they likely to have

A

haemoptysis

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7
Q

if there is a lot of blood being coughed up due to haemoptyisis what can be done

A

a catheter can be put in and can block off any bleeding vessels

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8
Q

in CF patients what usually ends up killing them

A

recurrent pulmonary infections and pancreatic insufficiency

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9
Q

what are the symptoms of type 1 respiratory failure

A

decrease PaO2

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10
Q

what are the symptoms of type 2 respiratory failure

A

decreased PaO2

increased PaCO2

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11
Q

what are the bacterial pathogens most likely to infect a patient with CF

A

Psedomonas aeruginosa 60%

staphylococcus 40%

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12
Q

where domestically can you find pseudomonas

A

shower head “gunge” where it forms a biolayer

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13
Q

what is the natural habitat for pseudomonas

A

in the soil

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14
Q

normally what role would CFTR play in reaction to Pseudomonas entering the lungs

A

usually plays a role in endocytosis and destruction

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15
Q

why is it difficult for white blood cells to fight off pseudomonas in the lungs once they are colonised

A

as it produces a bio film to protect itself from the hosts defense’s

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16
Q

what is colonization of pseudomonas associated with

A

reduced life expectancy

rapid decline in lung function

17
Q

what can you use to treat colonization of Pseudomonas

A

oral ciprofloxacin

nebulised colomycin

18
Q

if first line of treatment fails forcolonization of Pseudomonas what is the next step

A

If fails, i.v. ceftazidime and nebulised colomycin

19
Q

what is Burkholderia cepacia

A

environmental organism that causes onion rot

20
Q

what health problems is Burkholderia cepacia associated with for CF patients

A

reduced life expectancy 16 vs 39 years

rapid decline in lung function

Some patients ‘cepacia syndrome,’ very rapid deterioration

21
Q

what is cepacia syndrome

A

immune system goes into overdrive and this is what ultimately kills the patient

22
Q

does Burkholderia cepacia

have any resistance to antibiotics

A

resistant to most antibiotics

23
Q

what are 2 other organisms that can cause respiratory infection in patients with CF

A

Stenotrophomonas maltophilia

Mycobacterium abscessus

24
Q

what is the treatment for recurrent respiratory tract infections

A

treat early and aggressively with antibiotics

25
Q

what antibiotics are used for Staph, Haemophilus, Pneumococcus

A

oral antibiotics

26
Q

what antibiotics are used forPseudomonas, Stenotrophomonas, Burkholderia

A

IV antibiotics

27
Q

what are the 2 antibiotics used to treat recurrent respiratory tract infections in CF patients

A

B-lactam + aminoglycoside

28
Q

what is Ivacaftor

A

drug that addresses the primary defect in CF by binding to the CFTR improving the transport of cl ions

29
Q

what percentage of patients with CF can be helped with ivacaftor

A

5-10% those with a certain mutation

30
Q

in patients with the mutation that will benefit from Ivacaftor, how does their health improve

A

Improves lung function 10% predicted
Weight gain
Reduces sweat chloride
Feel much better (normal)