Blood Flow and Vascular Function in Cystic Fibrosis (CF-FLOW)
Cystic Fibrosis
About this trial
This is an interventional treatment trial for Cystic Fibrosis focused on measuring arterial stiffness, flow-mediated dilation, endothelial function, pulse wave velocity, inflammation, oxidative stress, pulmonary function test, Cystic Fibrosis, Lung Disease, Nitric Oxide, Exercise Capacity, CF, Muscle Function, Sildenafil, Viagra, Revatio
Eligibility Criteria
Inclusion Criteria.
- Diagnosis of CF and healthy controls
- Men and women (greater than 18 yrs. old)
- Resting oxygen saturation (room air) greater than 90%
- Forced expiratory volume (FEV1) percent predicted greater than 30%
- Patients with or without CF related diabetes
- Traditional CF-treatment medications
- Ability to perform reliable/reproducible pulmonary function tests (PFT)
- Clinically stable for 2 weeks (no exacerbations or need for antibiotic treatment within 2 weeks of testing or major change in medical status)
Exclusion Criteria.
- Children less than 17 years old
- Body mass less than 20 kg
- A diagnosis of pulmonary arterial hypertension (PAH)
- FEV1 less than 30% of predicted
- Resting oxygen saturation (SpO2) less than 90%
- Self-reported to be a smoker
- Current use of any vaso-active medications
- History of migraine headaches
- Pregnant or nursing at the time of the investigation
- A clinical diagnosis of cardiovascular disease, hypertension, or CF related diabetes
Sites / Locations
- Augusta University
Arms of the Study
Arm 1
Arm 2
Arm 3
Experimental
Experimental
Experimental
Acute Study: Sildenafil first, then Placebo
Acute Study: Placebo first, then Sildenafil
Sub-Chronic Study Sildenafil
In randomized order, on two separate days, endothelial function and exercise capacity will be determined 1 hour following a single dose of sildenafil (50 mg) or placebo.
In randomized order, on two separate days, endothelial function and exercise capacity will be determined 1 hour following a single dose of sildenafil (50 mg) or placebo.
Following the acute study, patients will be instructed to take 20 mg of sildenafil, three times a day, for 4 weeks. Endothelial function will be determined within 48 hours following the last dose.