Blood Flow and Vascular Function in Cystic Fibrosis
| Status: | Completed |
|---|---|
| Conditions: | Pulmonary |
| Therapuetic Areas: | Pulmonary / Respiratory Diseases |
| Healthy: | No |
| Age Range: | 18 - Any |
| Updated: | 1/11/2019 |
| Start Date: | April 2014 |
| End Date: | July 2018 |
Role of Blood Flow and Vascular Function on Exercise Capacity in Cystic Fibrosis
Cystic fibrosis (CF) has many health consequences. A reduction in the ability to perform
exercise in patients with CF is related to greater death rates, steeper decline in lung
function, and more frequent lung infections. However, the physiological mechanisms for this
reduced exercise capacity are unknown. The investigators laboratory recently published the
first evidence of systemic vascular dysfunction in patients with CF. Therefore, it is
reasonable to suspect that the blood vessels are involved with exercise intolerance in CF.
This study will look at how 1) blood flow and 2) artery function contribute to exercise
capacity in CF.
exercise in patients with CF is related to greater death rates, steeper decline in lung
function, and more frequent lung infections. However, the physiological mechanisms for this
reduced exercise capacity are unknown. The investigators laboratory recently published the
first evidence of systemic vascular dysfunction in patients with CF. Therefore, it is
reasonable to suspect that the blood vessels are involved with exercise intolerance in CF.
This study will look at how 1) blood flow and 2) artery function contribute to exercise
capacity in CF.
The most disturbing aspect of Cystic Fibrosis (CF) is the associated premature death. Low
exercise capacity predicts death in patients with CF and is also associated with a steeper
decline in lung function and more lung infections. A critical barrier to improving exercise
tolerance in patients with CF is the investigators lack of knowledge regarding the different
physiological mechanisms which contribute to their lower exercise capacity. We have
compelling data to indicate that the blood vessels may contribute to the low exercise
capacity in CF. The impact of this proof of concept investigation will test Phosphodiesterase
Type 5 inhibitors (PDE5) inhibitors as a potential therapy in CF and will explore blood flow
and endothelial function as potential mechanisms which contribute to exercise intolerance in
CF. Improvements in exercise capacity will not only contribute to a better quality of live
for patients with CF, it will also increase longevity in these patients.
exercise capacity predicts death in patients with CF and is also associated with a steeper
decline in lung function and more lung infections. A critical barrier to improving exercise
tolerance in patients with CF is the investigators lack of knowledge regarding the different
physiological mechanisms which contribute to their lower exercise capacity. We have
compelling data to indicate that the blood vessels may contribute to the low exercise
capacity in CF. The impact of this proof of concept investigation will test Phosphodiesterase
Type 5 inhibitors (PDE5) inhibitors as a potential therapy in CF and will explore blood flow
and endothelial function as potential mechanisms which contribute to exercise intolerance in
CF. Improvements in exercise capacity will not only contribute to a better quality of live
for patients with CF, it will also increase longevity in these patients.
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
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