What is Progeria?
Hutchinson-Gilford Progeria Syndrome “Progeria” or “HGPS” is a rare, deadly genetic condition characterized by an appearance of accelerated aging in children. Its name is derived from Greek means “prematurely old.” While there are different forms of Progeria, the original type is HGPS. Other Progeroid syndromes include Werner’s syndrome, also known as “adult progeria” which does not have an onset until the late teen years, with a lifespan into the 40’s and 50’s. 90% of children with Progeria have a mutation on the gene that encodes Lamin A, a protein that holds the nucleus of the cell together. It is believed that the defective Lamin A protein makes the nucleus unstable. This instability seems to lead to the process of premature aging among Progeria patients. Progeria appears to occur without cause – it is not seen in siblings of affected children. In extremely rare cases more than one child in the same family may have the condition. Also Progeria is hereditary.
What are the characteristics of Progeria?
In Progeria patients, the cell nucleus has dramatically aberrant morphology. Children with Progeria are born looking healthy. When they are about 10 to 24 months old, features of accelerated aging start to appear. Signs of Progeria may include:
- Growth failure
- Loss of body fat
- Loss of hair
- Skin starts to look aged
- Stiffness in the joints
- Hip dislocation
- Cardio and heart disease
Although they may come from varying ethnic backgrounds, children with Progeria have a surprisingly similar appearance. Progeria patients generally die between the ages of 8 and 21 – with the average age being only 13.
How does HGPS Affect Us?
I have no relationship with anybody diagnosed with HGPS, and although this does not effect me and maybe even some people in this classroom. It affects the people surrounding killing them at an early age. And without an personal relationship with many people I feel that HGPS is unappreciated.