Cystic Fibrosis Flashcards
1
Q
What are the signs and symptoms of cystic fibrosis?
A
- chronic daily sputum production
- pancreatic insufficiency
- recurrent respiratory infection
- recurrent pulmonary infection
- GI problems
- liver disease
- infertility
- osteoporosis
- dyspnoea
- haemoptysis
- respiratory failure
2
Q
How would you treat a respiratory infection in a person with cystic fibrosis?
A
- early and aggressively
- two antibiotics
- long course
3
Q
What does Ivacaftor do?
A
Opens calcium channels and reverses the effects of CF
4
Q
What are the disadvantages to Ivacaftor?
A
- only works for 5-10% of patients
- expensive, £180k pa
5
Q
What treatment might be used in CF to prolong survival and improve quality of life?
A
Lung transplantation
