IPAH is a rare lung disorder in which the blood pressure in the pulmonary artery rises far above normal levels. In response to these pressure rises, the wall of the pulmonary artery thickens, causing the heart to work harder and eventually fail. What triggers this thickening is not known in a lot of cases. Although there is no known cure for the disease, treatments are available. Many patients with pulmonary hypertension, however, continue to worsen and some eventually require a heart-lung transplant.
The CMREF research initiative is designed to support a network of multidisciplinary, collaborative transplant and research centers to study the origins of IPAH. A coordinating center will be responsible for the design, maintenance and analysis of the IPAH database.
"This field has traditionally been individual institutions performing their own research, with little direct communication between groups," said Peter Jones, PhD, director of the Penn/CMREF Center. "The idea of networking and pooling our resources is going to get us to better treatments and hopefully a cure for this disease much faster than working individually."
Penn's IPAH Center will have specific tasks to perform, including, acquiring control and IPAH tissues, cells and fluids from patients, and using these samples to identify new markers using state-of-the art cellular and molecular biology approaches ranging from proteomics to imaging.
"What this center is really focused on is idiopathic hypertension, hypertension with no known cause," added Jones. "We want to discover new molecular and genetic markers for this disease, then feed our results to other institutes within the network that are doing additional types of research on idiopathic hypertension. On an annual basis we will meet to share and discuss our findings then continue to move forward."
Darren Taichman, MD, PhD, Associate Director of the Pulmonary and Vascular Disease Program at Penn Presbyterian Medical Center, part of the University of Pennsylvania Health System, is working with Jones and is leading the effort to collect information and samples to be used in the study. "We have been consenting patients to collect samples of their blood, then we will get some follow up information like medicines they are taking and if there is any family history of hypertension," explained Taichman. "This will help characterize the samples we pass to Dr. Jones. Once this is done the patients' role is complete, but the information they provide allows us to analyze the laboratory findings in the context of how well a patient does with treatment"
Over the last several years numerous hypertension drugs have entered the market. From a clinical standpoint, finding which drug is best for a given patient is one of the study's primary goals. "The problem right now is we don't know if one drug is better than the other for a chosen individual," concluded Taichman. " Since we are talking about a disease that can progress at a rapid pace you would love to know ahead of time that a certain drug will be most beneficial to a certain patient, versus any of the other different drugs."
Jones adds, "It would be great if we could use the information we find to develop new diagnostic markers or targeted therapies for IPAH treatments and discover the repercussions for other diseases, including certain forms of cancer and atherosclerosis, that share certain characteristics of IPAH. Time will tell."
Jones and Taichman's research will be based at Penn's multi-disciplinary Institute for Medicine and Engineering, the Hospital of the University of Pennsylvania's Department of Pathology and Laboratory Medicine, and the Pulmonary, and the Allergy and Critical Care Section of Presbyterian Medical Center (PMC).
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