Progeria, officially Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic disorder that can be devastating to young families. The condition causes rapid aging in children, and currently there is no cure or treatment for Progeria.
Progeria baffles doctors and scientists because of the remarkable way it manifests. Children who have Progeria appear normal at birth, but by the age of 12 months old, it becomes obvious to parents that something strange is going on with their child.
The symptoms of Progeria resemble normal human aging, but this aging occurs in young children, who exhibit the signs of being elderly, even before they have become old enough to attend Kindergarten.
Symptoms of Progeria include a lack or failure of normal growth, wrinkles and a shrunken face, dry, scaly skin, no eyebrows or eyelashes, short stature, small jaw, open “soft spot” on the head, scleroderma (hardening of connective tissue in the skin), insulin resistance, delayed, absent or misshapen teeth and atherosclerosis (hardening) of blood vessels.
It is this last symptom or sign that can threaten the child’s life. As vessels continue to narrow, these children can become vulnerable to cardiac arrest or stroke. Unfortunately, Progeria is associated with a shorten lifespan. The average child with Progeria only lives into his or her teens, although there have been some cases of Progeria patients reaching their early 20s.
Scientist are making some progress toward finding a cure or treatment options for Progeria. They now know that 90 percent of Progeria patients have a mutation on the gene that encodes the protein lamin A. While there is strong evidence that there is a genetic component to this condition, is usually occurs without clear cause. Even with one child diagnosed with Progeria, other siblings of that child usually are not affected with Progeria. It is rare to find the condition in more than one child in a single family.
There is a support group for families affected by Progeria. Visit www.progeriaresearch.org.