21/02/24 (afternoon)

Question 1

Features of cystic fibrosis?

Answer 1

• Meconium ileus, neonatal jaundice (less commonly)
• Recurrent chest infections
• Malabsorption: steatorrhoea, failure to thrive
• Other features: liver disease

Question 2

What important advice should be given to CF patients to reduce the chance of chest infections?

Answer 2

• Reduce contact with other CF patientss
• Reduces transmission of Pseudomonas and Bulkholderia infections

Question 3

What is the standard management of CF?

Answer 3

• Regular (twice delay) chest physio and postural drainage
• High calorie, high fat diet
• Minimise contact with other CF patients
• Vitamin and pancreatic enzyme supplementation
• Lung transplant (can’t be done if infected with Bulkholderia)

Question 4

What can be given to CF patients who have homozygous F508 mutation in the CFTR gene?

Answer 4

Lumacaftor/Ivacaftor