Doing good while grappling with illness

Jeremy Hawkins celebrated his 9th birthday last week with presents, cake and ice cream as his parents snapped hundreds of pictures of him and his 12-year-old brother, Brandon.

In many ways, it was a typical birthday. But then again, it wasn't. The boys' parents, Chris and Wendy Hawkins, know the timeline they're working with. They don't have many years left.

Brandon and Jeremy have Batten Disease, a disease that causes mental impairment, seizures, progressive loss of sight and motor skills and eventually death.

On days like Mother's Day, Wendy feels mixed emotions.

"It's a difficult day for me," she said. "But I celebrate the fact that I have them both with me."

They're creating memories.

Sometimes, those memories are as simple as dancing together to music from "Alvin and the Chipmunks."

"I cry every day," Wendy said. "But the tears I cry are sometimes tears of joy from watching them create wonderful memories."

The Concord family will host the fourth annual OurBoys 5K, an event to raise money for the Batten Disease Support and Research Association, on May 8 Saturday at Harris Road Middle School. They've raised about $30,000 in the last two years for Batten Disease research.

It's brutal, and it's rare. Batten Disease is a childhood genetic disorder that occurs in about two to four children for every 100,000 births in the United States, according to the BDSRA.

When Brandon started school, his parents noticed he was struggling. A vision screening revealed that his vision was impaired, and doctors diagnosed him with cone-rod dystrophy, a disease affecting the retina. Not long after that diagnosis, Brandon had a massive seizure, sending doctors scurrying to find the cause.

The answer came in 2006 with the results of a DNA test: Batten Disease.

Both Chris and Wendy are carriers of the defective gene that causes the disease, which they had never heard of until Brandon's diagnosis. The disease is linked to a buildup of lipopigments, which are made up of fats and proteins, in the body's cells. The disease kills neurons, the cells found in the brain, retina and the central nervous system.

The Hawkins' children had a one in four chance of inheriting the gene, and the chances didn't work in their favor.

Doctors also tested Jeremy, then a 6-year-old who showed no symptoms. He tested positive too.

Now Brandon is mostly blind, only able to distinguish light and some shading. Jeremy has slightly more vision, and both are on medication to control their seizures.

Behavioral problems accompany Batten Disease, and the Hawkins are starting to notice those, as well.

They're also beginning to see short-term memory problems, particularly word retrieval. Brandon can remember who gave him a particular toy for his birthday three years ago, but he recently forgot the word for the spout on a garden house. A nozzle, his dad reminded him.

Eventually, the boys will lose speech and motor skills. They'll have to use feeding tubes. The life expectancy is grim. Children with Batten Disease are expected to live only into their late teens or early 20s.

The Hawkins know children who've died from the disease at age 15. But they also know of one person who lived into the early 30s.

It's hope that keeps them going.

Chris is coping by taking action. He recently took a 70-day, paid volunteer leave of absence from Wachovia, where he works as a phone specialist, to work for BDSRA.

Wendy got involved, too. She's been busy putting out the word about Batten Disease, contacting popular morning shows and reaching out to celebrities who might serve as a spokesman for the disease.

It's been an emotionally draining journey.

Knowing the inevitable outcome is different than having a child die in an unexpected accident, Chris said.

"To know 14 or 20 years in advance and see it happen before your eyes. ... As a parent, I can't imagine anything worse you could do to me than make me sit and watch them deteriorate."

The Hawkins know a family from Chicago who recently lost two of their three children with Batten Disease within 10 days of each other.

"I've learned never to take anything for granted," Chris said. "We live on a carpe diem philosophy."

The boys don't dwell on the disease, either.

Brandon, a seventh-grader at Harris Road Middle School, loves going to school. His favorite subject is science. Jeremy, a third grader at W.R. Odell Elementary School, plays soccer. They have individual assistants at school, and they sometimes use canes.

One day after school recently, Jeremy loudly sang the theme song from an Indiana Jones movie he had been watching upstairs before he came sliding down the steps, smiling as he got to the floor.

In their home's driveway, Brandon - already 5 feet 11 inches tall at 12 years old - played basketball, using a ball with a bell inside so the boys can hear its movement. His father positioned Brandon in front of the basket, and Brandon shot and missed.

"That one's on you," Brandon said to his dad. "You moved me!"

Jeremy is a Boy Scout, and Brandon is a video game guru who can play the games by sound alone..

Typical boys, their parents say.

"We appreciate what we've been blessed with even though it's not ideal," Chris said. "We want other families to really appreciate what they've been blessed with."