CF - L5 Flashcards
1
Q
What are the main goals of treatment?
A
maintaining lung function
adequate growth
managing complications
2
Q
What are 5 airway clearance techniques?
A
active cycle of breathing techniques (ACBT) autogenic drainage (AD) assisted AD positive expiratory pressure (PEP) activity & exercise
3
Q
What are 3 respiratory therapies?
A
antimicrobials
mucolytics (Pulmozyme) - recombinant human DN-1
hypertonic saline
anti-inflammatory agents
4
Q
What are the features of a CF patients diet?
A
high fat high protein
fat-soluble vitamins
salt replacement
5
Q
What is gene therapy?
A
introducing a normal copy of CFTR into the conducting airways
but the lung is an efficient barrier
6
Q
What are some vectors?
A
viral
non viral
7
Q
What does Ivacaftor do?
A
protein rescue therapy for the class III mutation G551D works well, but small % and expensive
8
Q
What does Lumacaftor do?
A
protein rescue therapy for the class II mutation F508del
9
Q
What do potentiators do?
A
Activate protein in Class III mutations
