The Hindu : International : The pain of living without pain

Online edition of India's National Newspaper
Tuesday, Nov 02, 2004

International

International

The pain of living without pain

PATTERSON, NOV. 1. Ashlyn Blocker's parents and kindergarten teachers describe her the same way: fearless. So they nervously watch her plunge full-tilt into a childhood deprived of natural alarms.

In the school cafeteria, teachers put ice in 5-year-old Ashlyn's chilli. If her lunch is scalding hot, she will gulp it down anyway. On the playground, a teacher's aide watches Ashlyn from within 4.5 metres , keeping her off the jungle gym and following her when she runs. If she takes a hard fall, Ashlyn will not cry.

Ashlyn is among a minuscule 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. "Some people would say that's a good thing. But no, it's not," says Tara Blocker, Ashlyn's mother. "Pain's there for a reason. It lets your body know something's wrong and it needs to be fixed. I'd give anything for her to feel pain."

Extreme temperatures

The untreatable disease 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.

Ashlyn can feel the texture of nickels and dimes that she sorts into piles on her bedroom floor, the weight of the pink backpack she takes to school and the embrace of a hug. She has cravings for her favourite after-school snack of pickles and strawberry milk. That is because the genetic mutation that causes CIPA only disrupts the development of the small nerve fibres 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 Felicia Axelrod, a Professor of paediatrics and neurology at the 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 Dr. Axelrod do not know how many suffer from CIPA. As the director of a treatment centre that specialises in CIPA and related disorders, she 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 with 67 members.

Here in Patterson, a rural town of 800 people in southeast Georgia, John and Ms. Blocker had no idea the disorder existed before they took Ashlyn to the doctor for a bloodshot, swollen left eye when she was eight months old. The doctor put drops in Ashlyn's eye to stain any particles that might be irritating it. The infant smiled and bounced in her mother's lap while the dye revealed a massive scratch across her cornea. "They put the dye in her eye and I remember the look of puzzlement on all their faces," Ms. Blocker says. "She was not fazed by it by any means."

Tests by a geneticist led to the diagnosis. To have the disorder, Ashlyn had to inherit two copies of the mutated gene — one from each parent.

Ashlyn's father, a telephone technician, and mother, who holds a degree in physical education, were largely on their own in learning to cope with their daughter's strange indifference to injury.

Many things could not be anticipated. Ashlyn's baby teeth posed problems. She would chew her lips bloody in her sleep, bite through her tongue while eating, and once even stuck a finger in her mouth and stripped flesh from it.

Family photos show a series of self-inflicted injuries. One picture shows Ashlyn in her Christmas dress, hair neatly coiffed, 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 was at age three, when she laid her hand on a hot pressure washer in the backyard. Ashlyn's mother found her staring at her red, blistered palm. "That was a real reality check for me. At that point I realised we're not going to be able to stop all the bad stuff," Ms. Blocker says.

Daily check-ups

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

Infections with no outward symptoms also concern them. They heard of a child with CIPA who 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 Ms. Blocker. "If she sees blood, she knows to stop. There's only so much you can tell a 5-year-old."

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