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The Dana-Farber Cancer Institute and Children's Hospital Boston are currently conducting a research study in patients who have multi-vessel (greater than two vessels) pulmonary vein stenosis. The purpose of this study is to determine the effectiveness of using two standard biologic agents that target myofibroblasts in pulmonary veins and control the abnormal growth of these cells to treat patients with this condition.
The study involves treatment with Avastin® and Gleevec® or Gleevec® alone based on disease association with congenital heart defect or lung disease, or occurring in isolation. These two medications have been used safely in children to treat childhood tumors. Multi-vessel intraluminal pulmonary vein stenosis is not thought to be a tumor, but it does share a common feature with tumors: the unwanted growth of cells.
One medicine is given daily by mouth; while the other is given through intravenous route once every two weeks. They have a low side-effect profile and have been well tolerated in infants, children and adults. The goal of treatment is to stabilize the process to improve life expectancy and the overall quality of life. The ongoing trial involves treatment for one year, and can be coordinated close to a patient's home area.
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