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Progeria Facts

Progeria Facts
Progeria is a genetic disorder that is very rare, characterized by rapid aging, affecting only 1 in 8 million babies. Progeria refers to Hutchinson-Gilford progeria syndrome (HGPS). Jonathan Hutchinson first described the disorder in 1886 and then in 1897 it was again described by Hastings Gilford, leading to the name Hutchinson-Gilford progeria syndrome. Symptoms of progeria usually appear within the first few months after a baby is born, including a failure to thrive and a skin condition. Once past infancy there is full body hair loss, limited growth, and distinct facial features are evident, which include a small face, pinched nose, and a recessed and shallow jaw. Health problems progress to include wrinkled skin, organ failure, and fragile bones. There is no cure and most of those children affected do not live past their early twenties, with the average lifespan being only 14 years.
Interesting Progeria Facts:
Progeria has been identified in 43 countries around the world, but little funding has been raised to study it because of the disorder's rarity.
Only approximately 100 children around the world have been identified as having the disorder at any given time.
The rapid aging of progeria causes a child in their teenage years to appear as if they are 80 to 90 years old.
Progeria does not affect a child's mind. Children with this disorder are the same as other children in respect to intelligence, and learning and wanting to be part of the world around them.
Progeria is not hereditary. It is caused by a spontaneous mutation of a gene called LMNA.
Progeria is 100% fatal, but new research is hoping to develop drugs that will slow the progression of progeria.
A child born with progeria will begin to age approximately 7 to 10 times as fast as normal once they reach the age of one.
Atherosclerosis is one of the biggest risks a child with progeria will face. This condition involves the hardening of arteries and by the time a child with progeria reaches their teen years it can be a life-threatening disease.
The Progeria Research Foundation was established by Dr. Leslie Gorson and Dr. Scott Berns in 1999. They were parents of a child with progeria, and their work has led to the first drug therapy for progeria and the discovery of the gene responsible for the disorder's development.
In the past a diagnosis of progeria could only be done by the presence of physical symptoms. Today it is possible to perform a genetic test if it is suspected, which allows for earlier treatment to help slow the aging process.
Drugs used to treat progeria are usually those used to treat the complications of aging, such as cholesterol-lowering drugs and growth hormone treatment, as well as some anti-cancer medications.
Despite the appearance of a child with progeria - appearing much older than they actually are - they will still behave as a child because the disease does not age their mind - just their body.
Progeria has been the subject of several movies such as Paa, The Hunger, and Life According to Sam (based on Sam Berns - the son of the doctors who founded the Progeria Research Foundation).


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