cystic dibrosis in adults

Question 1

whats the ratio of people carrying cystic fibrosis?

Answer 1

1 in 25

Question 2

if a person was a carrier for CF and they met someone thats also a carrier for CF whats the likelyhood of there child having CF?

Answer 2

1 in 4

Question 3

why has survival of CF got much better in recent years?

Answer 3

CF centres
MDT teams
Physio
Nutrition/Enzymes
Antibiotics

Question 4

what are the challenges for patients with CF?

Answer 4

Transition is a major challenge. New MDT team

Prognosis

Promise of new drugs

Possibility of lung transplant

Other conditons: Diabetes, liver disease, osteoporosis, fertility issues, haemoptysis, mental health issues – can develop anything else not related to CF

Media/Social media pressure and misinformation.

Question 5

what are common presentations for adults with cystic fibrosis?

Answer 5

infertillity
bronchiectasis
Mild resp symptoms/recurrent LRTI (40%)
Hyperinflation
Clubbing

Question 6

is CF a multisystem disease?

Answer 6

yes

Question 7

why does pulmonary infection occur?

Answer 7

decreased mucociliary clearance
increased bacterial adherence
decreased endocytosis of bacteria

Question 8

a person with CF that had a ct scan would show:

Answer 8

tramlines
signet rings
mucous plugging
consolidation

Question 9

what initiates type 2 diabetes mellitus in CF?

Answer 9

falling lung function, obesity

Question 10

do you ever see type 1 diabetes in cystic fibrosis patients?

Answer 10

no

Question 11

what are issues with cystic fibrosis when patients have diabetes?

Answer 11

Compliance with diet a problem
low sugar/high fibre diet not appropriate
OGTT /HBA1C used but not perfect
Insulin of benefit, not so much oral hypoglycaemics

Complication risk same as non CF pts but pulmonary disease gets there first

Question 12

how is osteoporosis affected with CF?

Answer 12

slower gain, faster loss of bone mass

Question 13

why does bone mass density decrease with CF patients?

Answer 13

Malnutrition and low BMI
Steroids
Delayed puberty and hypogonadism
Inflam cytokines from sepsis (we know BMD falls in septic pts)
Vit D/K defic 
Lung Tx/drugs

Question 14

whats the treatment for patients with CF that experiences pneumothorax?

Answer 14

sam treatment as other people,

drain, pleurodesis, surgery

Question 15

what gender is more likely to get pneumothorax and CF

Answer 15

both as likely as each other

Question 16

what is HAEMOPTYSIS?

Answer 16

coughing up blood that comes from the lungs or bronchial tubes

Question 17

what are risk factors of haemoptysis?

Answer 17

Severity, Exacerbations, Fungal, liver disease, Vit K def

Question 18

what are the bacterial pathogens involved with cystic fibrosis?

Answer 18

pseudomonas aeruginosa
staphylococcus aureus
BURKHOLDERIA CEPACIA

Question 19

where is pseudomonas aeroginosa acquired from?

Answer 19

Environment (particularly hospitals)
Other CF patients (epidemic strains, antibiotic resistant)
Segregation and disinfection policies

Question 20

where is BURKHOLDERIA CEPACIA acquired from?

Answer 20

Environment
Other CF patients (epidemic strains more virulent)
Segregation policies

Question 21

what is burkholderia cepacia’s colonisation associated with?

Answer 21

reduced life expectancy 16 vs 39 years
rapid decline in lung function
Do worse in pregnancy
Some patients ‘cepacia syndrome”

Question 22

does aspergillus come from environment or people?

Answer 22

environment

Question 23

how do you manage pulmonary infection?

Answer 23

Treat early and aggressively with antibiotics
Oral antibiotics (eg Staph, Haemophilus, Pneumococcus)
IV antibiotics (eg PA, Stenotrophomonas, Burkholderia)
Two antibiotics, liaise with microbiology
If multiply resistant, test for synergy between antibiotics Large doses (inc volume distribution, increased clearance)
Two week courses
Indwelling subcutaneous ports (vascuports)
Home administration
Side effects, interactions, allergies

Question 24

are nebulisers or inhaled antibiotics used instead of iv?

Answer 24

no

Question 25

why might CF patients have airway obstruction?

Answer 25

Asthma/Atopy
BHR in 40% CF pts.

2. Mechanical
   Bronchial plugging, Inflammation Bronchial wall thickening
3. Many pts (esp milder disease) - increase in FEV1 despite absence typical asthma symptoms

Question 26

what are mucolytics used for?

Answer 26

reduce viscocity of phlegm