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(Study: Vertex IIS) Does Ivacaftor Alter Wild Type CFTR-Open Probability In The Sweat Gland Secretory Coil?
Not Recruiting
Trial ID: NCT02310789
Purpose
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.
Official Title
(Study: Vertex IIS) A Study To Access the Effects of Ivacaftor on Wild Type CFTR-Open Probability (PO) In The Sweat Gland Secretory Coil
Stanford Investigator(s)
Eligibility
Inclusion Criteria:
- Healthy adults without a Cystic Fibrosis (CF) mutation
- Carriers with a known CF mutation
Exclusion Criteria:
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.
Intervention(s):
drug: Ivacaftor
drug: β-Adrenergic cocktail
drug: Pilocarpine Nitrate 5%
device: Macroduct sweat stimulator
Not Recruiting
Contact Information
Stanford University
School of Medicine
300 Pasteur Drive
Stanford,
CA
94305
Colleen Dunn, RRT, CCRC
650-736-0388