Progeria is a rare, fatal, genetically determined disease of childhood characterized by dramatic, premature ageing that occurs at about seven to ten times the normal rate. Because of this accelerated ageing, a child of ten years will have similar respiratory, cardiovascular, and arthritic conditions that a 70-year-old would have. Progeria is the most radical of the ageing illnesses.
Its name is derived from Greek and means “prematurely old”. The condition is estimated to affect one in 8 million newborns worldwide with a total reported incidence of just over 100 in the century since it’s been identified. There are currently around 90 known cases worldwide of Progeria. In the past 15 years, children with Progeria have been reported all over the world, including in Algeria, Argentina, Australia, Austria, Canada, China, Cuba, England, France, Germany, Israel, Italy, Mexico, the Netherlands, Poland, Puerto Rico, South Africa, South America, South Korea, Switzerland, Turkey, the US, Venezuela, Vietnam, and Yugoslavia.
The most severe form of the disease is Hutchinson-Gilford Progeria Syndrome, recognizing the efforts of Dr. Jonathan Hutchinson, who first described the disease in 1886, and Dr. Hastings Gilford who did the same in 1904.
As newborns, children with Progeria usually appear normal. However, within a year, their growth rate slows and they soon are much shorter and weigh much less than others their age. While possessing normal intelligence, affected children develop a distinctive appearance characterized by baldness, aged-looking skin, a pinched nose, and a small face and jaw relative to head size. They also often suffer from symptoms typically seen in much older people: stiffness of joints, baldness, hip dislocations and severe, progressive cardiovascular disease. Death occurs on average at age 13, usually from heart attack or stroke. Most children with Progeria don’t live beyond their early teenage years, though one or two have lived to their early 20s.
Children with Progeria are genetically predisposed to premature, progressive heart disease. Death occurs almost exclusively due to widespread heart disease, one of the leading causes of death worldwide. As with any person suffering from heart disease, the common events for Progeria children are high blood pressure, strokes, angina (chest pain due to poor blood flow to the heart itself), enlarged heart, and heart failure, all conditions associated with ageing. Thus, there is clearly a tremendous need for research in Progeria. 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 ageing process.