Anatomy Shared Article Research

This blog exists for the Anatomy students at Tree of Life Christian School. We will be reading various scientific articles, summarizing our research, and then commenting on others' summaries. We hope to broaden our view of the current research surrounding the human body, and to help others see how truly amazing the design of the human body is.

Friday, October 17, 2008

Progeria

http://www.sciam.com/article.cfm?id=new-hope-for-progeria-drug-for-rare-aging-disease

Progeria
Progeria is a childhood disorder that can have a two-year-old child resemble a grandparent. It afflicts one out of every four to eight million kids, and right now there are ten or so kids in the United States and fifty reported worldwide. Symptoms are hair loss, wrinkling skin, fragile bones, limited growth and stiff joints, appearing as early as age two. A few children with progeria have lived to be twenty, but about ninety percent die of heart attacks or strokes by thirteen. Progeria strikes at random and is a result of a mutation in the sperm before conception. This changes one letter of DNA and disfigures the nucleus. It also makes a toxic protein that builds up in the arteries of the heart that harden and can cause heart attacks.
But there is hope for these families. Researchers have genetically engineered mice with the progeria problem to test a promising new drug. Farnestyltransferase inhibitors or FTIs, were shown to restore the nucleus and prevent damage in younger mice. This drug also affected the older mice in that it reversed the damage. Currently, they are still testing for safety and efficiency with a Phase II trial consisting of eleven kids from sixteen different countries. Other than this fledgling drug, treatments are only a high calorie diet so as not to lose weigh and physical therapy to help with joint stiffness. Though it’s still too early to say, this drug could someday be widely accepted as the treatment for progeria.
And researchers have found that the infamous toxic protein in progeria is made by all of us, though at significantly lower levels. It’s possible that this increases with your age, causing the signs of aging.

My reaction: That is such a sad thing to imagine, that your child ages and dies before they are a teenager. People worry about fine lines and wrinkles and the signs of aging, and these kid’s lives are shorten by it. It definitely puts things in perspective. The children should be able to be like children and the aging should stop acting like children about their appearance. You have your run with your youth and your looks and when it’s over, it’s over. These kids don’t get their run. They have fragile bones and lose their hair and have wrinkles! They can’t play like other kids and people probably don’t say ‘Your look so much like your mother.’ It’d be more accurate to say they look like the grandparent or perhaps the mother in another fifty years. Age and the signs of aging are something to be earned. Hair loss should be from too many crazy styles back in the day, and laugh lines from the joys of life. Joints should be stiff from overuse and dancing all night. Birthdays should always be celebrated, whether over the hill or at the bottom- just not underneath it. To not only shorten life at the beginning, but add the elements from the end seems so cruel. And to think one letter in the DNA, one mutation can change it all. I hope the drug ends up being the answer these families have been looking for.

4 Comments:

Blogger Amanda said...

Progeria is such a sad disease! It's heartbraking to see a seven year-old child look and feel like they are seventy! It must be horrible to be trapped in an old body when you are a child who just wants to play. Seeing children in that condition makes me feel grateful that God blessed me with a working, healthy body, and ashamed when I complain about it. What those children would give to know what it feels like to not be in pain every time they move. Or not to be stared at on a daily basis. My heart brakes for them. If this medicine can do what scientist think it can, I hope it brings relief to these children and their parents.

Sunday, October 19, 2008 5:28:00 PM  
Blogger Becca said...

This is such an interesting disease and yet sad all at the same time. It's wierd to see a child who looks older than their mother or father and to have a worse body than them too. We take advantage of our lives and the things in them everyday and these kids are probably grateful to feel healthy one day. It really makes you think about your life and how it probably isn't nearly as worse as these kids. Hopefully this medicine can work and give these kids not only a longer life, but also a life they can enjoy.

Wednesday, October 22, 2008 2:32:00 PM  
Blogger hannah71190 said...

I have never heard of Progeria before, but that is probably because few people have it. It is good few people have it, because it is such a terrible disease. I hope that the medication is the miracle they need. Living life is always to short and living with the disease is almost no life at all. Not saying that their life doesn't matter, but on how short theirs are.

Wednesday, October 22, 2008 7:01:00 PM  
Blogger Adrienne Lindsey said...

I completely agree with Jamie,in that,this disease totally puts things into perspective for an apperance-obsessed society.It is so interesting how a little mutation in the sperm during conception and alter a child's life so dramatically. We often think of our bodies as pillars of strength and power that can accomplish anything. Yet this disease shows just how fragile and delicate our bodies really are.
I think that it is great that they are in the final stages of researching a drug that could help these children lead healthy lives.

Thursday, October 23, 2008 11:31:00 AM  

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