Cystic Fibrosis

Question 1

What is cystic fibrosis?

Answer 1

An inherited recessive genetic disease causing a genetic mutation on chromosome 7 causing problems with the CFTR protein

Question 2

What does CFTR stand for?

Answer 2

Cystic Fibrosis Transmembrane Regulating

Question 3

What does the CFTR protein control?

Answer 3

Acts as a channel protein enabling ion transport

Question 4

What three ions are compromised in CF?

Answer 4

Chloride, sodium and water

Question 5

How many variations of mutations are there of the CFTR protein?

Answer 5

1500

Question 6

What systems does cystic fibrosis affect? (7)

Answer 6

Respiratory 
Pancreatic
Biliary
Liver
Gastrointestinal 
Fertility
Sweat production

Question 7

Which ethnicity is it most prevalent?

Answer 7

Caucasian

Question 8

Name the three testing techniques for CF

Answer 8

Blood test
Sweat test
Genetic testing

Question 9

Name some common symptoms (the ones listed do not include them all, see notes for details)

Answer 9

Cough
Inflammation of lungs causing wheezing, SOB
Recurring chest infections
Large smelly stools
Malnourished/poor weight gain/stunted growth etc
Arthritis, osteoporosis, muscle atrophy etc
Urinary incontinence

Question 10

Name the three main treatments

Answer 10

Nebulisers, bronchodilators and mucolytics

Question 11

Name other common treatments (5)

Answer 11

Digestive enzymes
Diet 
Exercise
Psychological support
Vaccinations are up to date

Question 12

State three physio treatments

Answer 12

Postural drainage, breathing exercises, suction