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editIt is unclear as of 2014 if ursodeoxycholic acid is useful for those with cystic fibrosis-related liver disease.[148]
CFTR Modulators
editCFTR modulator therapies are designed to correct the function of the the cystic fibrosis transmembrane conductance regulator (CFTR) made by the CF gene. The first FDA approved CFTR modulator, ivacaftor, is a pill for people with cystic fibrosis ages 2 and older who have at least one of the following rare CF mutations: G551D, G178R, S549N, S549R, G551S, G1244E, S1251N, S1255P, G1349D and R117H (Petit & Fellner, 2014). This modulator helps sodium and fluid move into the airways, thinning the mucus so that it is easier for the individual to cough out. A second CFTR modulator is a pill that combines ivacaftor and lumacaftor and is approved for people ages 12 and older who have two copies of the F508del mutation (the most common CF mutation). Lumacaftor aids in moving the CFTR protein to the cell surface and ivacaftor increases the activity of the protein to thin the mucus build up in the lungs and other effected organs.
Pettit RS, Fellner C. CTFR Modulators for the treatment of cystic fibrosis. 2014. Pharmacy and Therapeutics 39 (7): 500-511.