What is the origin and effects of Progeria?
Progeria is a very rare disorder, occuring in about 1 in 8 million births. It is a disease which causes extremely rapid aging of the body, particularly of the heart. It is usually diagnosed in early childood, and the victims rarely survive into their teens.
While progeria is a genetic disease, it is not passed down in families. It is caused by a spontaneous mutation of the LMNA gene, which codes for the protein Lamin-A. Lamin-A creates the structure of the cell nucleus. Children born with this mutation are unable to make Lamin-A correctly, so their cell nuclei become unstable, and do not maintain or repair themselves. As a result, the normal wear and tear in their bodies does not heal, and so accumulates at a very rapid rate. Every body system is affected by this; some of the more obvious symptoms are skeletal abnormalities, hair loss, thin, aged-looking skin, and very little body fat. Most progeria sufferers die of heart failure.
At this time there is no treatment that is effective for progeria. The main interventions involved psychological support for the victim and their family.