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Flashcards in Pulmonary CF Deck (15):

Why is CF no longer a childhood disease?

Pts with CF are living longer


Describe CF Class 1

Significant mutation resulting in complete absence of CFTR channel proteins


Describe CF Class 2

Delta F508 mutation causing a defect in protein processing, the actual protein is made but does not get sent to the right site in the cell membrane


Describe CF Class 3

Defective regulation, the protein has diminished activity


Describe CF Class 4

Channel works but doesn't stay open for very long leading to lower ion flow rate


Describe CF Class 5

CFTR proteins are produced but not very many


How is the lung structurally altered in CF other than damage due to infection?

Cysts form and lead to bronchiectasis.


Where is CF treatment directed?

Against infection and structural changes mainly to prevent exacerbations of the disease. The gradual deficit of FEV-1 occurs due to exacerbations.

Exacerbations usually result from infection, chronic recruitment of the immune system damages the lung.


What is the best antibiotic used to treat moderate to severe pseudomonas infection in CF patients

INHALED tobramycin


What medication is used to break up mucous that forms in the airways?

Recombinant Human DNase
-degrades the high amount in DNA in the mucous


What medication can be used to increase airway surface hydration?

Hypertonic Saline
-after give albuterol before to dilate airways


When would inhaled steroids be used in CF patients?

Only with concurrent asthma or aspergillosis


Macrolides help CF patients by what two mechanisms?

Decreased biofilm production by pseudomonas
Suppressed inflammation


Which pathogen is a death sentence for CF patients?

Burkholderia cepacia


What are limitations to Lung Transplant for treating CF?

Doesn't address extra-pulmonary issues.

Pancreatic and bowel complicatons prevent cyclosporine absorption necessary to suppress the immune system after transplant.

Steroids complicate CF diabetes