Cystic Fibrosis Flashcards
What is cystic fibrosis?
autosomal recesssive genetic disorder effecting the cycstic fibrosis transmembrane conductance reguator (CFTR)
What is the CTFR gene used for?
Production of channel proteins on all epithelial cells - commonly in respiratory, gastrointestinal and hepatobilary tracts
Protein becomes folded and so channel is absent
No transmission of Chloride ions out of the cell int the secretions
Chloride required to increase concentration gradient and cause water to move into the secretions
What is the respiratory presentation of CF?
Thick and sticky mucus - due to lack of water
Reduced sol layer of MCE - due to lack of water
Recurrent infections - due to mucus –> inflammation of airways –> oedema and increased mucus –> elastase from neutrophils causes destruction and dilation of bronchial wall –> bronchiectasis
What are the physiotherapy interventions for CF?
Main aim is to clear secretions to minimise chances of infection - risk is +++
treatment of bronchiectasis
