Status:

COMPLETED

Safety and Efficacy of Sildenafil in Cystic Fibrosis (CF) Lung Disease

Lead Sponsor:

National Jewish Health

Collaborating Sponsors:

Cystic Fibrosis Foundation

Conditions:

Cystic Fibrosis

Eligibility:

All Genders

14+ years

Phase:

PHASE1

PHASE2

Brief Summary

The purpose of this study is to determine whether sildenafil can decrease inflammation in CF lung disease.

Detailed Description

This study is an open-label study that examines the use of sildenafil (Revatio) in clinically stable patients with mild to moderate CF lung disease. The length of participation for each subject will b...

Eligibility Criteria

Inclusion

  • Confirmed diagnosis of CF based on the following criteria:
  • Positive sweat chloride ≥60mEq/liter (by pilocarpine iontophoresis) and/or
  • Genotype with two identifiable mutations consistent with CF, and accompanied by one or more clinical features consistent with the CF phenotype
  • Male or female patients ≥ 12 years of age
  • FEV1 ≥ 50% predicted (Knudson) 31
  • Clinically stable without evidence of acute upper or lower respiratory tract infection or current pulmonary exacerbation within the 14 days prior to the screening visit
  • Ability to reproducibly perform spirometry (according to ATS criteria)
  • Ability to produce at least 1mL of sputum spontaneously, or be willing to undergo sputum induction
  • Ability to understand and sign a written informed consent or assent and comply with the requirements of the study
  • Chronic bacterial colonization (3 documented positive cultures in the prior 2 years of which at least one was obtained in the 3 months prior to randomization)

Exclusion

  • History of hypersensitivity to sildenafil
  • Use of an investigational agent within the 4-week period prior to Visit 1 (Day 0)
  • Breastfeeding, pregnant, or verbal expression of unwillingness to practice an acceptable birth control method (abstinence, hormonal or barrier methods, partner sterilization or intrauterine device) during participation in the study
  • Daily use of systemic corticosteroids and/or NSAIDs within 4 weeks of the study or as needed use within 72 hours prior to the screening visit
  • History of significant hepatic (SGOT or SGPT \> 3 times the upper limit of normal at screening, documented biliary cirrhosis, or portal hypertension), cardiovascular (history of aortic stenosis, coronary artery disease, pulmonary hypertension with right ventricular systolic pressure \>55 mmHg or life-threatening arrhythmia), neurological (history of stroke), hematologic (history of bleeding diathesis), ophthalmologic (history of retinal impairment or non-arteritic ischemic optic neuritis) or renal impairment (creatinine \>1.8 mg/dL.)
  • Inability to swallow pills
  • Previous lung transplantation
  • Use of concomitant nitrates, α-blocker, or Ca channel blocker
  • Use of concomitant medications known to be potent inhibitors of CYP3A4 (e.g. ketoconazole, itraconazole, ritonavir, clarithromycin, erythromycin, rifampin)
  • Presence of a condition or abnormality that in the opinion of the investigator would compromise the safety of the subject or the quality of the data
  • Weight less than 40 kg
  • History of sputum or throat swab culture yielding Burkholderia cepacia within 2 years of screening
  • Resting room air oxygen saturation \<93%
  • History of migraines

Key Trial Info

Start Date :

August 1 2008

Trial Type :

INTERVENTIONAL

Allocation :

ACTUAL

End Date :

December 1 2012

Estimated Enrollment :

36 Patients enrolled

Trial Details

Trial ID

NCT00659529

Start Date

August 1 2008

End Date

December 1 2012

Last Update

October 19 2020

Active Locations (1)

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1

National Jewish Health

Denver, Colorado, United States, 80206