By Chris Lane
By Jeff Balke
By Aaron Reiss
By Angelica Leicht
By Dianna Wray
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By Camilo Smith
By Craig Malisow
"After that, I changed my major the next month," she says. "It's just what I love to do. Special ed's my thing."
Melinda Watson never realized she would have to bring her work home.
There were no early signs of trouble in 1993 when Sam and Melinda Watson decided to begin a family. Their first child, Luke, was born that September. Seven months later Melinda was expecting again.
"We were poor but happy," says Melinda in a sort of half-laugh and half-lament. "I wasn't real excited to be pregnant that soon, but when Rachel was born I was happy, and it was good. I was staying home. I was taking care of the babies."
In his first six years Luke developed into a sports fanatic. Every day at breakfast, Sam read the sports page aloud to his son. Whatever season it was, Luke played the appropriate sport, either by himself, with friends or with his dad. If there was a sporting event on television, Luke would watch it, regardless of whether he knew anything about either team involved. Luke would always choose a team to root for, and if that team lost, he'd cry.
It was Luke's TV habits that gave the Watsons their first indication that something might be wrong with his vision. The couple noticed that Luke was sitting closer and closer to the screen. Then there was the incident at Sam's parents' farm near Brenham in November 1998. As Melinda and Luke walked across a field, the child turned and called for his mother, who was not more than 20 feet in front of him. "And I said, 'I'm right here,' " remembers Melinda. "And he said, 'Where? Where are you, Mommy?' And my heart just We didn't know what was wrong with him at the time. But we knew there was something."
Before that, the only other hint of trouble had come about a month earlier. A nurse at Luke's preschool told the Watsons that their boy could not distinguish between colors. A trip to the eye doctor resulted in prescription glasses for Luke. "I felt so guilty," says Melinda. "Because by the time we got him to the doctor, and [the doctor] really started seeing what Luke couldn't see, he was probably at 20-400. How I did not know that that child could not see is beyond me."
That November, Sam and Melinda took Luke to another eye specialist. "Suddenly we went from worrying that Luke was going to get kidded for wearing eyeglasses to worrying if he was going blind," says Sam.
The Watsons would soon be praying that their son was just going blind.
According to the National Institutes of Health, Batten disease is named for the British pediatrician who first described it in 1903. It strikes two to four out of every 100,000 children born in the United States but is somewhat more prevalent in northern Europe. Although uncommon itself, Batten is the most common form of a group of disorders called neuronal ceroid lipofuscinoses, or NCLs. The various forms of the disease are tied to the age of the child when first diagnosed. For example, babies suffer from infantile Batten; two- to four-year-olds can have late infantile Batten; and children diagnosed in the five- to eight-year range are afflicted with juvenile Batten. (There is also an even rarer form of adult NCL, which usually surfaces in an individual before the age of 40. The symptoms are milder, and it does not result in blindness.)
Victims of juvenile Batten are usually first diagnosed with a progressive loss of sight. The diagnosis is tantamount to a death sentence: The disease is always fatal. Most children die by their late teens or early twenties, but some have been known to live a few years longer. Batten, however, is not contagious. Instead, it is transmitted genetically from the parents to the child. For the disease to be passed on, both parents must carry the defective gene. There is only a one in 800,000 chance that both parents will carry the defective gene, but if they do, the odds are one in four that their children will be afflicted with Batten. The disorder often strikes more than one child in a family.
The disease is linked to the accumulation of substances called lipopigments, made up of fats and proteins, in the cells of the brain and eyes. Researchers believe that as these lipopigments build up, the diseased cells are unable to rid themselves of them, resulting in a degeneration of the optic nerve, and then the brain.
"One of the things that makes it so difficult for parents, brothers and sisters and other family members is when the children lose their speech," says Lance Johnston, president of the Batten Disease Support and Research Association. "Because when they lose their speech, they can't tell us what's going on, how they feel or if something hurts, or if they need something. So we have to get pretty good at reading body language and sounds."
BDSRA has a membership of about 800 families, and Johnston says that at least 1,900 children worldwide suffer from the disease. Johnston lost a daughter to juvenile Batten, which, along with late juvenile Batten, is the most common form of the disease. His daughter Lorena was diagnosed when she was six years old. She died at the age of 22.