CF

Question 1

What criteria is CF diagnosis based on?

Answer 1

One or more characteristic phenotypical features

• History in sibling
• Positive newborn screening test

AND

• increased sweat Cl (>60mmol/l)
• Extended genotyping - 2 CF mutations.

Question 2

Name the 4 main ways in which CF will present.

Answer 2

Meconium ileus
Intestinal malabsorption
Chest infections
Newborn screening

Question 3

What is some lifestyle advice that should be given to someone with CF?

Answer 3

Stop smoking
Annual influenza vaccination 
Avoid jacuzzis 
Clean and dry nebuliser thoroughly 
Avoid friends/ relatives with infections
Avoid stables, compost and rotting vegetation 
(Aspergillus fumigatus inhalation) 
NaCl tables in hot weather or when vigoursly exercising