Trial Search Results
(Study: Vertex IIS) Does Ivacaftor Alter Wild Type CFTR-Open Probability In The Sweat Gland Secretory Coil?
Clinical studies of lumacaftor + ivacaftor (combo therapy) produced better FEV1 (forced expiratory volume in 1 second) improvements than ivacaftor alone, without further improvement in sweat chloride results.
To help understand why sweat chloride was unresponsive, the investigators will use a newly developed sweat secretion test that provides accurate, in vivo readout of CFTR (cystic fibrosis transmembrane conductance regulator) function in the sweat gland secretory coil.
The investigators devised a protocol to determine if short courses of ivacaftor (3.5 days) will produce significant increases in WT (Wild-Type, i.e. normal) CFTR open probability by measuring CFTR-dependent sweating (C-sweat) in subjects with WT CFTR.
Stanford is currently not accepting patients for this trial.
Richard Barry Moss
- Drug: Ivacaftor
- Drug: β-Adrenergic cocktail
- Drug: Pilocarpine Nitrate 5%
- Device: Macroduct sweat stimulator
- Healthy adults without a Cystic Fibrosis (CF) mutation
- Carriers with a known CF mutation
1. Documented liver disease
2. Participants should not be taking:
- medicines that are strong CYP3A (Cytochrome P450, family 3, subfamily A)
inducers, such as:
- the antibiotics rifampin and rifabutin;
- seizure medications (phenobarbital, carbamazepine, or phenytoin); and
- the herbal supplement St. John's Wort, substantially decreases exposure of
ivacaftor and may diminish effectiveness.
Ages Eligible for Study
18 Years - N/A
Genders Eligible for Study