respiratory disease

Question 1

CFTR is expresssed throughout the body and CF disease affects multiple systems, including major effects on the ….

Answer 1

lungs, pancreas and gastrointestinal systems

Question 2

Cystic fibrosis is a genetic mutation.
What causes cystic fibrosis?

Answer 2

CF is caused by mutations in the Cystic fibrosis transmembrane conductance regulator. CFTR is responsible for regulating chloride ions and water movements across the cell membrane.

Question 3

The mutation in the CFTR gene results in the movement of chloride and water across cell membrane being impaired –> leading to the production of thick secretions which cause the following in the LUNGS and PANCREAS

Answer 3

• Repeated chest infections, reduced lung function and cough
• exocrine ducts are blocked thereby reduced insulin production –> diabetes

Question 4

what other conditions are associated with CF

Answer 4

• infertility (98%)
• Delayed development, puberty
• bone demineralisation

Question 5

children up to the age of 3 years should receive oral antibiotic prophylaxis for …

Answer 5

staphylococcus aureus
- off label flucoxacillin

Question 6

what is the ongoing treatment for pseudomonas aeruginosa?

Answer 6

colistimethate sodium 1-2 million units BD-TDS by nebuliser

tobramycin 300mg bd by nebuliser for 28 days with a 28 day break

Question 6

what is the treatment for mild and severe infection with pseudomonas aeruginosa

Answer 6

mild - oral ciprofloxacin +/- nebulised colistimethate

severe- combination of -IV aminoglycoside e.g. tobramycin and IV beta lactam e.g. ceftazidime, tazobactam/pipracillin

Question 7

in addition to staph. aureus and pseudomonas aeruginosa, CF patients may experience frequent infections with …

Answer 7

haemophilus influenzae

Question 8

what are the signs and symptoms of infection/ exacerbation

Answer 8

increased cough, wheeze, increased sputum, change in sputum colour, haemoptysis (coughing up blood from your lungs), pyrexia (fever), decreased appetite, increased SoB, decreased exercise tolerance

Question 9

Give examples of some mucoactive agents ?

Answer 9

1st line - Dornase alfa 2500 units OD by jet neb.
- it is a genetically engineered version of a human enzyme which cleaves extracellular DNA

hypertonic sodium chloride (saline) by neb.

mannitol 400mg BD (dry powder)

Question 10

Give examples of CFTR modulators ?

Answer 10

lumacaftor/ ivacaftor

They correct the malfunctioning protein made by the CFTR gene
Different mutations causes different defects in the protein, the medication so far are only effective against specific mutations

Question 11

what are the two methods of physiotherapy in CF?

Answer 11

postural drainage - where they tip the patients body so the infected lobe is uttermost

percussion - rhythmically clapping motion upon the chest wall with cupped hands to loosen the secretions from the sides of the lungs

Question 12

what is the treatment for pancreatic problems

Answer 12

difficulty digesting fats –> more fatty foods and higher calorie intake
pancreatic insufficiency –> pancreatic enzyme replacement therapy i.e. Pancreatin tabs
GORD symptoms –> PPI
replacement enzymes required for digesting foods and are taken with every meal
if no pancreatic insufficiency –> fat soluble vitamin supplementation

Question 13

10-25% of CF adults have low bone mineral density leading to ….

Answer 13

osteoporosis