Cystic fibrosis (PRSC)

Question 1

Background

Answer 1

Genetic disorder affects:
Lungs
Pancreas
Liver
Intestines
Reproductive organs

Question 2

Symptoms

Answer 2

Cough, wheezing, SOB, Greasy stools, constipation, bloating, salty tasting skin, growth issues

Complications
Pulmonary lung disease
- Can weaken immune system so get recurrent infections

Produces thick sputum

Hard to digest and absorb food and nutrients due to lack of pancreas function.

Liver disease

Osteoporosis

Question 3

Treatment

Answer 3

Aims
Prevent & Manage lung function, loosening & removing thick sticky mucus from lungs, preventing/Treating intestinal blockage, Providing sufficient nutrition/Hydration.

NOTE - Lung function predicts life expectancy in CF = Better lungs = Longer life.

Treatment
1st line - Dornase alfa (via nebuliser)
ALT Mannitol dry powder
Abx for lung infections.