Jamie and Carson’s Hope

A crusade to save lives of two children

By Sharon Boase
The Hamilton Spectator

BRANTFORD (Nov 6, 2006)

Sheldon Huelin spent all day yesterday campaigning — but his quest has got nothing to do with the Nov. 13 municipal election. The stakes are much higher for Huelin and his wife, Lori Ann Keeping, than winning political office or wielding power over people or even a whole city. This campaign is about saving the lives of their two youngsters who are doomed to die before they reach their teens without some sort of miraculous medical intervention.

Five-year-old Jamie and two-and-a-half-year-old Carson have Batten disease, a fatal, inherited nervous system disorder that causes the brain to shrink and shut down over time, eventually robbing its young victims of their speech, sight and motor abilities.

There is no known cure or prevention for it.

So Huelin, a millwright, has begun knocking on corporate doors from Burlington to Brantford looking for donors in a bid to raise $150,000 to take his children to China for a radical stem cell procedure.

“We thought, ‘Shoot, that’s pretty expensive,’” Keeping said of the experimental brain surgery.

“And I’m really nervous about it, I won’t deny it. But it’s the only thing we’ve got left to do. There’s nothing else out there for them.”

Jamie can’t speak, see or move much on her own anymore. She has to be fed through a tube and carried wherever she needs to go.

Although still sighted and mobile, Carson has lost the few words he had picked up, including “mom.”

Huelin and Keeping, a machine operator, read about the procedure in The Spectator which has tracked the progress of Travis Carter, an eight-year-old city boy who underwent the stem cell procedure in Beijing last Wednesday.

The couple has been visiting a website documenting the progress of young patients at the Beijing hospital and have spoken by telephone to a British woman whose eight-year-old daughter had the treatment.

“The pictures we’ve seen of her daughter, she looks more alert,” said Keeping.” And before, she couldn’t walk at all but since the procedure, she’s been able to walk with someone helping her.” Keeping was eight months pregnant with Carson when Jamie had a seizure just after her third birthday. At first, doctors thought she was epileptic. But when Jamie began losing words and then stopped making eye contact, the diagnosis became autism. When Jamie stopped walking and then stopped eating and drinking, she was soon diagnosed with Batten disease. Her little brother was diagnosed last September. Keeping said she and Huelin were shocked when they discovered she was pregnant with Jamie. Between working opposite shifts and coping with the grief of losing their first-born, they hadn’t had much time together. Preston Huelin, who was born with a heart defect, died on Jan. 1, 2000, age one month. Meanwhile, Huelin has an eight-year-old daughter from a previous relationship who’s been diagnosed with Lupus, a chronic disorder of the immune system. But Zackery, Keeping’s 12-year-old son from a previous relationship she is raising with Huelin, is healthy. Keeping’s Aunt Suzy and two home care workers help out with Jamie’s and Carson’s care.” I try not to dwell on it,” Keeping said. “I try not to look at my kids like they’re sick. I know they have a problem but I try not to look at them that way. I try not to treat them any different than any mother treats her children.” Donations can be made to Bank of Nova Scotia, transit number 61952-002, account number 0106089. I know they have a problem but I try not to look at them that way. I try not to treat them any different than any mother treats her children.” Donations can be made to Bank of Nova Scotia, transit number 61952-002, account number 0106089. Huelin and Keeping invite you to call them for more information at 1-866-553-0770.

Batten disease is named after the British pediatrician who first described it in 1903. It is the most common form of a group of disorders called Neuronal Ceroid Lipofuscinoses (or NCLs).

A rare disorder that has nevertheless been identified around the world, Batten disease/NCL occurs in an estimated two to four of every 100,000 births in the U.S.

Symptoms are linked to a buildup of fats and proteins in cells of the brain, eye, skin, muscle and other tissues.

Vision loss is often an early sign, as are behaviour changes, slow speech and learning, clumsiness and stumbling. It robs its victims of sight, mental function and motor skills. It also causes worsening seizures.

Children with Batten disease tend not to live beyond eight to 12 years, 20 at the outside.

No treatment is yet known to halt or reverse symptoms although seizures can be reduced with medication.