Rare disease makes girl unable to feel pain

MSNBC ^ | Nov 1 2004 | AP

[FourtySeven]

PATTERSON, Ga. - Ashlyn Blocker’s parents and kindergarten teachers all describe her the same way: fearless. So they nervously watch her plunge full-tilt into a childhood deprived of natural alarms.

--snip--

Ashlyn is among a tiny number of people in the world known to have congenital insensitivity to pain with anhidrosis, or CIPA — a rare genetic disorder that makes her unable to feel pain.

The untreatable disease also makes Ashlyn incapable of sensing extreme temperatures — hot or cold — disabling her body’s ability to cool itself by sweating. Otherwise, her senses are normal.

That’s because the genetic mutation that causes CIPA only disrupts the development of the small nerve fibers that carry sensations of pain, heat and cold to the brain.

“There are all kinds of different nerve cells that help us feel different sensations,” says Dr. Felicia Axelrod, a professor of pediatrics and neurology at New York University School of Medicine. “You can have one sense removed, just like you can lose your hearing but still smell things.”

Specialists such as Axelrod don’t know how many people suffer from CIPA. As director of a treatment center that specializes in CIPA and related disorders, Axelrod has 35 patients with the disease on file. Only 17 of them are from the United States. Japan has the world’s only association for CIPA patients. It has 67 members.

[FourtySeven]

One picture shows Ashlyn in her Christmas dress, hair neatly coifed, with a swollen lip, missing teeth, puffy eye and athletic tape wrapped around her hands to protect them. She smiles like a little boxer who won a prize bout.

Her first serious injury came at age 3, when she laid her hand on a hot pressure washer in the back yard. Ashlyn’s mother found her staring at her red, blistered palm.

“That was a real reality check for me. At that point I realized we’re not going to be able to stop all the bad stuff,” Tara Blocker says. “She needs a normal life, with limitations.”

So when Ashlyn goes to her kindergarten class at Patterson Elementary School, she gets daily check-ups with school nurse Beth Cloud after recess. Cloud and Ashlyn’s mother discussed having her wear a helmet on the playground, but decided it would look too odd.

And when teacher’s aide Sue Price puts ice in Ashlyn’s chili at lunch, her dozen classmates get ice in theirs too.

Infections with no outward symptoms also concern them. They heard of a case where a child with CIPA had appendicitis that went untreated until her appendix burst.

“It’s a lot to take in. It opens your eyes to things you wouldn’t normally think about,” says Tara Blocker. “If she sees blood, she knows to stop. There’s only so much you can tell a 5-year-old.”

She looks like quite the little trooper, God Bless her.

I do wonder though why her school puts ice in the chili of her classmates as well? Is there any logical reason behind that, other than the group think that permeates our public school system nowadays?

[jdm]

bttt

[Tax-chick]

Is there any logical reason behind that, other than the group think that permeates our public school system nowadays?

No, not really, although the little kids probably think the ice makes school lunch chili taste better!

[js1138]

I wonder what the life expectancy for these kids is?

Many years ago there was a TV show called Science Fiction Theater. They had an episode in which a schoolboy with this condition was thought by neighbors to be an alien.

[Izzy Dunne]

YIKES! Never heard of this.

Your first reaction might be that to feel no pain would be a good thing. Not so.
Pain protects you by warning you that what is happening is damaging...

[laotzu]

Wouldn't this make her capable of impressive acts of strength?

[jperspective]

When she grows up she could join the X-men.

[duffthor]

Perhaps....but it also makes her capable of getting squished because she can't feel her limits.

[ArrogantBustard]

I do wonder though why her school puts ice in the chili of her classmates as well?

It's the self-esteem groupthink nonsense. In the real world, the hot chili business would be a good opportunity to teach the child that she is, in fact, different from other folks. She needs to learn to be careful in ways other folks don't. But this valuable survival lesson is lost to political correctness.

[The Grammarian]

I think it does, although at the expense of more wear and tear on her muscles, since she would be pushing them further than most anyone else.

[Galena Nevada]

There's going to be a whole class of kids who grow up thinking that ice normally goes in chili. I can see it now... One of these kids makes chili for a frat party his first year at college and puts ice in it.

[dandelion]

I know, because I used to teach Pre-Kindergarten...

If Ashley has it, they ALL will want it. Just try it with your little kids at home and see. If the one child likes it, and gets it, then they just HAVE to have it!

[anniegetyourgun]

Why can't us ladies get this disease on a temporary basis - from the first twing of labor to 48 hours after delivery?

[Mount Athos]

As politically correct groupthink nonsense goes though, it sure is harmless.

[The_Republican]

I wish I could borrow her disease for tomorrow night ONLY and maybe for part of Wednesday....

[najida]

I thought the same thing...

Anytime you are around a group of kids, if you do something with one, you have to do it for all.

That is the way kids work.

[John O]

Genetic lepresy. This little one is in for a short ugly life if she doesn't learn to protect herself. God help her

[mlmr]

I imagine that the chili is at a hot tempature and they cool it down so she will not blister her mouth. She cannot feel pain. Why they dont have one of the lunch ladies cool a bowl down beforehand for her is beyond me.

[ArrogantBustard]

I agree regarding putting ice in her chili ... putting ice in the rest of the kids' chili is the feel-good nonsense.

[boris]

"Genetic lepresy."

Almost as bad as leprosy.