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The photographs below were taken during the annual gathering of progeria children's families in June of 2001. These people are invited to assemble from countries all over the world. In 2001, it was held at the Adam's Mark Hotel in Philadelphia, PA -- and unfortunately the gathering coincided with the "Beach Party" District Rally, limiting the number of GoldWings that showed up to give the kids a ride. But there were more than enough of us to keep the kids riding for a couple of hours, and the kids (and their folks) enjoyed it immensely. GWRRA Chapters from NJ and PA supported the gathering by showing these children their motorcycles, their vests with all the pins, and driving them around on the bikes, a treat the likes of which those kids could not get enough. Unfortunately, the Saturday we met with the children was rainy and we had to confine our riding to the inside of the hotel's parking garage. But that just made the horns seem even louder when the kids got to toot them. And toot them they did! My ears haven't stopped ringing! |
Click on "thumbnail" photos for larger pictures.
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From About.Com: Progeria Syndromes - Give appearance of premature aging
Many people know progeria from seeing children who look very old on TV talk shows and documentaries. Those children have the more rare form of progeria; there is another more common type that affects adolescents and adults.
Hutchinson-Gilford progeria
This syndrome occurs in about 1 in 8 million children. At birth, a child with Hutchinson-Gilford Progeria appears normal. The syndrome begins to show around 6-12 months of age, when the baby fails to gain weight and skin changes occur. Over time, the child begins to look like an elderly person. Some of the characteristic symptoms are:
About 97% of the children affected are Caucasian, but all children with Hutchinson-Gilford Progeria have a similar appearance, regardless of racial/ethnic background. Children with the syndrome survive to an average age of 13 years old, with most succumbing to heart disease.
Werner Syndrome
Werner syndrome is a less well-known but more common form of progeria, occurring in about 1 in 1 million individuals. Typically, Werner syndrome is identified when an adolescent fails to have a normal growth spurt. Over time, the young person begins to look elderly. Some of the characteristic symptoms are:
Striking difference between the person's appearance and his/her real age
Head and face: gray hair and/or balding, wrinkling of the face, cataracts, sunken cheeks and small jaw, a high-pitched voice
Werner syndrome appears to occur more often in people of Japanese and Sardinian heritage. People with Werner syndrome survive to an average age of 46 years old, with most succumbing to heart disease or cancer.
Causes
In 1992, researchers pegged Chromosome 8 as being the locus of Werner syndrome;
in 2003 the responsible gene was identified. Researchers hope that they will be
able to discover what roles this gene plays in the body and thereby figure out
the mechanisms behind the syndrome.
The gene responsible for Hutchinson-Gilford syndrome has not been identified, so researchers do not have a grasp yet of what causes the syndrome. They suspect a gene defect (mutation) occurs accidentally in a human embryo, and the child then develops progeria.
May not be premature aging
In both syndromes, research suggests that although the affected person may look
like he or she is growing old prematurely, there are a number of conditions that
develop that are very different from those of elderly people. For example,
people with Werner syndrome develop osteoporosis, as in aging, but their form of
the bone disease is unusually severe in the long bones of the legs. Yet
researchers are hopeful that both syndromes will give some clues about the
genetic factors involved in the aging process.
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