Progeria Disease Pictures, Details as seen in Paa MovieBy Jayita, Gaea News Network
Saturday, December 5, 2009
Have you seen the trailer of the movie “Paa” ? The Bollywood movie starring Amitabh Bachchan and his son Abhishek Bachchan. Amitabh will appear in a phenomenal look to play the role of a 13 year old boy, affected by Progeria.
Progeria, also known as Hutchinson-Gilford Progeria syndrome (HGPS) is a severe genetic disorder that cause accelerated aging. It causes children to age almost eight times faster than they are supposed to.
It was discovered in England in 1886 and was named after Jonathan Hutchinson, who first discovered it and Hastings Gilford, who was the first to refer to the disease as Progeria. The name Progeria is derived from Greek and means “prematurely old”.
Progeria is commonly referred to as an “early aging disease“. It’s a very rare disease and affect only 1 to 2 children in 8 million.
Progeria is associated with a short lifespan. Those born with progeria typically live about thirteen years, although many have been known to live into their late teens and early twenties and rare individuals may even reach their forties.
Unlike, premature aging syndrome, dyskeratosis congenita (DKC), progeria is not inheritable. It is not seen in siblings of affected children, although — in very rare circumstances — more than one child in a family can have the condition, research done on progeria reveals.
What causes progeria?
Due to the extreme rarity of Progeria, little research has been done until recent years. In April 2003, researchers isolated the genetic mutation that causes progeria. So, the gene has been found. Ninety percent of children with progeria have a mutation on the gene that encodes the protein lamin A. Progeria usually occurs without cause.
- The earliest symptoms include failure to thrive and a localized scleroderma-like skin condition.
- Growth failure as the child ages past infancy.
- Narrow, wrinkled face and jaw(micrognathia)
- small, fragile bodies, like those of elderly people
- Large head for size of face (macrocephaly)
- Limited range of motion
- Later, the condition causes atherosclerosis, and cardiovascular problems.
Still, as of right now, there is no known cure or treatment for progeria. However, there are treatments for the symptoms such as medicine and exercise.
- Most treatment focuses on reducing complications such as cardiovascular disease with heart bypass surgery or low-dose aspirin.
- High-calorie diet may be proved beneficial for progeria affected children.
- In some cases “Growth hormone” treatment has been attempted.
- A type of anticancer drug, farnesyltransferase inhibitors has been proposed, although it has limited scope.
A Phase II clinical trial using the FTI Lonafarnib began in May 2007. This development brings hope to all the families affected by Progeria.
In India, two brothers, Ikramul Khan and Ali Hussain are suffering from progeria. They look like aged people, despite being in their early twenties. Three of their other siblings were also progeria affected and died at early age of 17, 24 and 13 respectively. .
Their last wish is to meet Bollywood megastar Amitabh Bachchan and thank him for creating awareness about the disease.
Doctors have already told that they have no chance of living beyond their early twenties. So we hope Mr. Bachchan would difinitely turn up to fulfill their last dream.