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Children with Progeria are genetically predisposed to premature, progressive heart disease. Death occurs almost exclusively due to widespread heart disease, the leading cause of death in the US, and #2 worldwide. As with any person suffering from heart disease, the common events for Progeria children are strokes, high blood pressure, angina, enlarged heart, and heart failure, all conditions associated with aging.
"These children get heart disease at an incredibly accelerated pace, usually by the time they are 12, 13 or 14. In the general community, the prevalence of cardiovascular disease starts appearing in the 60s and 70s. Obviously, there's some process that's accelerated."
— Dr. Samer Najjar, head of the Human Cardiovascular Studies Unit at the National Institute on Aging
Thus there is clearly a tremendous need for research in Progeria. Because finding a cure for Progeria will not only help these children, but may provide keys for treating millions of adults with heart disease and stroke associated with the natural aging process.
Because the aging process is accelerated in children with Progeria, they offer researchers a rare opportunity to observe in just a few years what would otherwise require decades of longitudinal studies.
"A better understanding of the causes of this syndrome (Progeria) could lead to better insights into the mechanisms of both development and aging." - National Institute of Aging Associate Director Dr. Huber Warner
"It's amazing how much Progeria resembles the normal aging process. I think these proteins linked to Progeria play a very profound role in the aging process, and I think we've already seen some pretty big findings in terms of what these proteins do.
— Dr. Vilhelm Bohr, chairman of molecular gerontology at the National Institute on Aging
Children with Progeria also develop and die of atherosclerosis and arteriosclerosis (commonly referred to as heart disease). Atherosclerosis is just one of several types of "arterio"-sclerosis, which is characterized by thickening and hardening of the arteries, but the two terms are often used to mean the same thing. Some hardening of the arteries often occurs when people grow older.
Atherosclerosis involves deposits of fatty substances in the inner lining of an artery. This buildup is called plaque. Plaques can grow large enough to significantly reduce the flow of blood through an artery, or plaques become fragile and rupture to form clots that can block blood flow. If the blockage occurs to an artery that feeds into the heart, it causes a heart attack. If the blockage occurs to an artery that feeds into the brain, it causes a stroke. Atherosclerosis is not only the cause of death among children with Progeria, but also the leading cause of death in the United States and many other parts of the world in normal aging individuals. It is our hope that the discovery of the Progeria gene will have a beneficial effect not only for Progeria patients, but also for the millions of people affected by aging-related conditions, including heart attacks and stroke caused by atherosclerosis and arteriosclerosis.