Progeria – Before Their Time

Now this has to be one of the saddest fucking things I have ever seen. Progeria, derived from a Greek word meaning “prematurely old”, is a crippling and fatal disease that literally causes children and teenagers to die of old age.

There are a few kinds of Progeria but the most common is Hutchinson-Gilford Progeria Syndrome named after the first two men to encounter and describe it; first in England in 1886 by Dr. Jonathan Hutchinson and again in 1897 by Dr. Hastings Gilford.

Today, the cause of the disease has been linked to a mutation of gene LMNA which produces the Lamin A protein. This protein is responsible for the stability of cell nuclei. Our cells wear out and die over time (aging), but this deficient protein causes cells to wear out and die abnormally fast, before they can be replaced. Hence the rapid aging of the body affected.

This condition cannot be detected in utero. Progerians are born completely normal but will begin to show signs of the disease within two years. They develop conditions we normally associate with old people; loss of body fat and hair, stiff joints, arthritis, hip dislocation, atherosclerosis, diminished size, senility/dementia (Alzheimer’s) as brain cells deteriorate and suffer heart disease and stroke. The average life expectancy of these kids is about fourteen years old although some have made it to twenty. Most of those afflicted with Progeria however, will die of massive heart attack or stroke before they are even old enough to drive.

There is an annual convention devoted to raising awareness and gives Progeria children a chance to meet others like themselves. One of the more famous Progerians is Sam Berns (shown in the first two images) who was given his own reality show on HBO which raised world wide awareness of the disease. He was a very smart young man and a lego prodigy, able to build complex and elaborate structures from lego blocks. If you noticed I’m speaking about him in the past tense it is because, yes, he died from heart related issue just this past January at the ripe old age of seventeen.

As is the case with Harlequin Ichthyosis and Cherubism, only one parent needs to carry a defective gene in order to bring it out in their offspring. They can possess this defect without showing any sign of it themselves. There is no cure and very little in the way of treatment at this moment although the real nameless, faceless heroes of this world are working trying to find a cure or at the very least make this condition more chronic than fatal. But please, expecting parents, do not fear. This is a very rare condition. As of May 2014, there are 114 Progerians currently diagnosed. Compare that to the eight billion people walking the planet and I’d say that your odds of having healthy offspring are pretty good. And for those of you who already have children, I suggest you give your little ones a big hug and thank whatever god you may believe in for their health and happiness.