Monday, September 04, 2006

Progeria

Progeria narrowly refers to Hutchinson-Gilford Progeria syndrome, but the term is also used more generally to describe any of the so-called "accelerated aging diseases". The word progeria is derived from the Greek for "prematurely old". Because the "accelerated aging" diseases display different aspects of aging, but never every aspect, they are often called segmental progerias by biogerontologists.

Hutchinson-Gilford Progeria syndrome is an extremely rare genetic condition which causes physical changes that resemble greatly accelerated aging in sufferers. The disease affects between 1 in 4 million (estimated actual) and 1 in 8 million (reported) newborns. Currently, there are approximately 40-45 known cases in the world. There is no known cure. Most people with progeria die around 13 years of age.

Symptoms generally begin appearing around 18-24 months of age. The condition is distinguished by limited growth, alopecia and a characteristic appearance with small face and jaw and pinched nose. Later the condition causes wrinkled skin, atherosclerosis and cardiovascular problems. Mental development is not affected. Individuals with the condition rarely live more than 16 years; the longest recorded life-span was 29 years. The development of symptoms is comparable to aging at a rate six to eight times faster than normal, although certain age-related conditions do not occur. Specifically, victims show no neurodegeneration or cancer predisposition.

FROM WIKIPEDIA

I was up late last night and this documentary came up on the discovery channel showing kids with a rare ageing disease and what they had to cope with such as stroke, arthritis and angina. What caught my attention wasn't so much the disease and the sadness of it all, but how full of life these kids were. They are such a great example for the rest of us. They didn't let the syndrome deter them from carrying on with life. John Tacket wanted to be a rock star and played the drums although once in a while he got angina when he over-exerted himself. He was in the school band and loved to play pool. Ashley Hegi,15, is his best friend and they got to hang out once when special progeria foundation events took place. They both attended normal schools and were popular, with lots of friends. In the documentary they have a special bond and understand what exactly what the other is experiencing. I never once saw them moan, complain, winge etc. They accepted their condition and embraced life fully. John and Ashley didn't seem to be worried about the future, only about living a good life. They really touched me in a profound way.



John passed away in 2004 at the age of 16. May his soul rest in peace.

7 comments:

Belle said...

wow thats sad....

maaaan, the diseases that are out there..!

Don Chi as Blog Marley a.k.a. El Senor Supremo said...

never knew what it was called or just forgot. i've seen stuff on the condition before and i thought they were the cutest people. shame they have to go so early becuase they are all full of life and smiles. how much we take for granted, eh?

GOD bless America and everywhere else...

Nice one kid

Life through rose-tinted glasses said...

i felt so sorry for the people while reading this its really sad and they have to die at such an early age. we just have to thank God in all situation. nice post too

LondonBuki said...

I saw a documentary about this in 1999, I still remember the kids... they were so lively... I guess they understand the value of life more than most people tend to so they are living life to the fullest!

Mona said...

mmm really sad i knew about it but not the name..ive seen a movie with someone like that in it...how's your week? xxx

Anonymous said...

Oh how terrible to hear about Ashley's friend John. I too was watching a documentary from about 10 years ago following the young life of Ashley from the time she was about 4-6 years old. It showed how fond she was of him when they would get together for their annual get togethers. I was so sad to hear about John's passing though. They sure looked sweet together in the documentary I saw. It was called "In Time's Shadow: The Hegis'"

Everyone who has posted is sure right about one thing, despite these kids' disease, they sure know how to live life to the fullest. I also found in my google search that Ashley now has a 3 year old brother after her mother married in 2002 I think. How very sweet this little girl is.

Marie said...

Just watched a tv documentary about a girl called Haley in the UK who has gone to America for treatment for this condition
There is some hope for the suffers.