Blind Faith Luke and Rachel Watson have lost most of their sight. They eventually will lose all motor skills. Their parents are losing sleep -- do the adults tell their kids they're dying of Batten disease? By Steve McVicker

Melinda Watson is only in her mid-thirties, but she has already experienced one moment of absolute bliss. It came after the birth of her second child: Sixteen months after delivering her son, Luke, in 1994, Melinda cradled her newborn daughter, Rachel. She sensed a sort of completion. "There was a 15-minute time I was alone with my new baby that was perfection," she recently reflected. "I felt secure in the fact that…we were the perfect family." By all appearances, Melinda and Sam Watson and their children were the perfect family. The deeply religious college sweethearts married following their graduations from Texas A&M in 1989. Eight years later both were pursuing fulfilling careers, Sam as an environmental specialist with the U.S. Army Corps of Engineers and Melinda as a special-education teacher. Luke and Rachel were the perfect complements: Both were handsome with dark hair and fair complexions. Both had already developed interests: Luke loved sports, both playing them and watching. Rachel was an avid drawer; she could spend hours with a box of Crayons. But in the blink of an eye, Melinda Watson's perfect vision of her family went badly out of focus. In October 1997 a school nurse informed the Watsons that Luke had difficulty distinguishing between colors, and suggested he have a professional eye exam. An optometrist fitted Luke with prescription glasses, but his vision problems persisted. More extensive examinations revealed a serious condition: Luke wasn't simply color-blind, nor was he just going blind. Luke was dying of a little-known genetic illness most commonly called Batten disease, an especially cruel disease that most often strikes children and leads to the eventual degeneration of the brain. After blindness, the victims of Batten suffer a progressive loss of motor skills. By the time they reach their teens, these kids are bedridden invalids waiting for death. Sometimes they linger until they are 20 or 30 years old. There is no known cure. The Little Prince author Antoine de Saint-Exupéry once said that "perfection is achieved not when there is nothing more to add, but when there is nothing left to take away." The Watsons had more to lose, and it came right quickly: Seven-year-old Rachel was also diagnosed with Batten. She and Luke will die right before their parents' eyes. "I didn't think God could be so cruel," says Melinda. "But I know he has a reason. I just don't know what it is." Neither does Luke or Rachel. They don't even know they're dying.

A mischievous-looking redhead, Melinda Watson breaks into one of her increasingly infrequent smiles when she recalls how she and her husband, Sam, got together. It may not have been love at first sight, she says, but it was close. In 1985 Sam and Melinda were both 18-year-old freshmen living in dormitories at Texas A&M. Each fall at the tradition-laden university, residents of the various boys' and girls' dorms are paired to be so-called Bonfire buddies for the mother of all pep rallies prior to the school's annual Thanksgiving weekend gridiron battle with the University of Texas. The students swap gifts with their assigned partners throughout the fall semester leading up to the big game. During that time the identity of the Bonfire buddy is supposed to remain a secret, but Sam and Melinda discovered they were each other's partners and started going out right away. Although they didn't date each other exclusively, Melinda felt that special spark. Following their marriage on New Year's Eve, 1989, Sam and Melinda moved to Conroe and from there they commuted to work each day: Sam to La Porte where he worked for a small consulting firm, and Melinda to a school in Kingwood where she taught special education. After Sam landed a job with the Corps of Engineers in Galveston, the couple relocated to Humble. Then it was back to College Station while Sam finished his master's degree. After graduate school, Sam opened his own private consulting company. The couple moved to the upper-middle-class subdivision of Copperfield in northwest Harris County where the pine trees reminded Melinda of East Texas. Problems within the company led to Sam's return to the Corps of Engineers and a four-times-a-week commute to Galveston. Melinda began teaching special ed in the Cy-Fair Independent School District; it's a field that first caught her attention as a freshman at A&M where she volunteered with the Special Olympics. "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 1992 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. "We need a lot more awareness about the disease," says Johnston. "There aren't that many doctors that know what Batten disease is all about. One day we'd like to see Batten disease as recognizable as Huntington's disease or cystic fibrosis. That when you say that, people would know what you're talking about." People like Sam and Melinda Watson. In December 1998 Sam and Melinda were directed to Dr. Robert A. Lewis, a neuro-ophthalmologist at the Baylor College of Medicine.

Up until that point, the couple never even knew there was such a thing as a neuro-ophthalmologist, much less some sort of evil like Batten disease. As they drove to Lewis's office with their son, the couple was scared. "You never get an inkling that you'll be handed something that can't be fixed," says Melinda. "As devastated as you are -- I cried and cried on the way there -- I always thought that whatever it was, it would be fixed." At the Baylor Eye Clinic, where Lewis has his practice, the doctor put Luke through a series of tests. Lewis specializes in rare genetic eye defects and sees two or three cases of Batten, or NCL, each year. "These kids have very distinctive behavior," says Lewis. "They have a relative flat, noninteractive dynamic, so that when you talk to them, they are sweet kids, but they are very unemotional. And most five-, six- or seven-year-olds, you can kid with them. You can get them to laugh." From what he saw in Luke, Lewis was suspicious immediately. The boy's ability to read an eye chart was poor, and the doctor noted a buildup of fatty cells on the optic nerves.

To avoid alarming the Watsons unnecessarily, Lewis ordered a biopsy of the transparent layer over the white part of Luke's eyes, a sample of about one square millimeter. The sample was sent to a pathology laboratory where the cells were magnified 15,000 to 30,000 times to allow a look for accumulation of fatty tissue. He also sent off a sample of Luke's blood for a genetic screening. "There is a common alteration or rearrangement in this gene among all [affected] families in North America," says Lewis. "About 70 percent have a particular alteration of the gene where a piece of the gene is missing, deleted. And that is a very easy alteration to identify." Lewis told the Watsons that their son could have Batten disease but insisted that he wasn't sure. The couple went home to wait for the test results. And to pray. "It was like everything was suddenly in slow motion," says Sam. "We went through a month or so of just praying like crazy that it wasn't going to be it, that he was just going blind."

The news finally came on February 18, 1999. Lewis told Sam and Melinda, along with Sam's mother, that Luke was dying from Batten. Not only that, but there was a good chance Rachel had the fatal disease as well. Even before Lewis could finish speaking, Melinda began shrieking and bolted from the office. "First you get furious," she says. More than two years later the pain hasn't receded much."There have been a lot of days when I thought I was not going to make it," she admits. "That I cannot live with this every day, all day. I'm going to have to end my life. And then you realize you can't, that you've got to be here to take care of the kids. So you have to work through how you're going to do that." At first Melinda opted for denial, at least as far as Rachel was concerned. It's a time Melinda is not proud of. "I fought my husband for about four months over having her tested," she says in a hushed voice. "Even as I watched her vision go down, I knew. I just didn't want to get it confirmed yet."

That summer Sam convinced Melinda that Rachel had to be tested. In August, Rachel also was diagnosed with Batten. And as she watched her children's health deteriorate, Melinda Watson began deteriorating herself. "I was very worried about Melinda," says Ann Watson, Melinda's mother-in-law. "I thought she was having a nervous breakdown." The elder Watson's fears were shared by Melinda's co-workers at school, some of whom worried that Melinda might yet take her life. Instead, she turned to professional counseling, both spiritual and psychiatric. Sam, however, has pretty much resisted that path, preferring to deal with the overwhelming grief in his own way, like trying to balance work with spending time with his kids, time he is sure he won't get back. But, he admits, ever since getting the news, he and Melinda have experienced an almost never-ending sadness. "People ask me how I deal with it, how I make it," he says. "It's not like you make it. I wake up every morning, and it's like a new nightmare." So far, it's a nightmare the Watsons have not shared with their children. Although Luke and Rachel know they are going blind, they do not know that in a few years they won't be able to do anything for themselves. It's a future that two fairly normal children -- and Rachel and Luke do seem normal despite their declining vision -- could never imagine. The siblings are both enrolled at Lowery Elementary in the Cy-Fair district.

This past year Luke was pulled from all language arts classes. He now practices Braille; he even recently put on a Braille demonstration for the other students in his class. The rest of the time, Rachel and Luke attend regular classes with their own full-time aide, provided by the school district, to assist them. Melinda admits the district has a good program for visually impaired children, but she says her kids feel isolated. When she asks them who they had lunch with, they have no idea. And she concedes that social situations are becoming more difficult, especially for Rachel. While Luke now uses a cane, Rachel refuses. "There's a point coming where she's going to have to quit relying on her eyes to get around," says Melinda. "She's going to have to start using a cane next year in school. And she's so humiliated about the thought of doing that. She just can't stand it. She says she doesn't need it." Perhaps to prove her point, a few weeks ago, after being dropped off at home by friends following Sunday-evening church services, Rachel told her mother that she would race her to the garage. "And dumb me," says Melinda, "I said okay. And Rachel took off running and ran smack into the back of my van [Melinda slaps her hands together for effect] with her face. It flipped her backwards, and I caught her. She just never even saw it. She's also run into a tree. She's at a very difficult time right now. She's not as coordinated as Luke." Luke displays a similar desire to maintain a normal life.

Despite his lack of vision, he relentlessly pesters his parents about playing baseball this summer -- organized baseball. He did play T-ball last summer. After hitting the ball, Luke would run toward the voice of his father, who would be waiting at first base. "The hardest thing…," says Sam, his voice trailing off momentarily. "Luke came up to me the other day -- of course we love to play baseball and play together all the time -- and said, 'Daddy, I've decided when I grow up I want to be a professional baseball player.' It just totally breaks your heart." It's during those moments that Sam tends to agree with Melinda -- that, at least for now, it's best that their children not know the whole truth. What good, the parents wonder, would come from spoiling such sweet dreams with the horror of reality? Medical ethics experts tend to agree. "I doubt that one answer fits all," says Dr. Raymond Lawrence, chaplain of Columbia Presbyterian Hospital in New York City.

While Lawrence places a high value on full disclosure in medical situations, he emphasizes that each child should be evaluated on his or her ability to deal with devastating information. However, he says, keeping important secrets from children can also backfire. "There is always the possibility that a child will find out about his condition through another source," says Lawrence, a former Houstonian who worked at the Texas Medical Center. "That's what you call a political risk. There is always the risk of a ghost in the room, and then secrets get disclosed when it is least advantageous to the person." Despite those risks, Lewis, who first diagnosed Luke and Rachel, believes the Watsons have made the right decision. "I totally support the parents' decision for these children," the doctor says. "There is no other reasonable or ethical decision." That position is also endorsed by BDSRA president Johnston, who says he did not tell his own daughter that she was dying. "My daughter did not want to know," he says. "She knew that things were going bad, but she didn't want to know what was going on. So we never told her. Eventually as their cognitive powers are affected, it becomes a moot question. They reach a point when they wouldn't understand if you told them anyway." The medical community may side with Sam and Melinda, but not everyone in the Watson family does.

JW Farm is located down a dirt and gravel road south of U.S. Highway 290, just east of Chappell Hill in Washington County. In the spring, the area is one of the best bluebonnet viewing spots within driving distance of Houston. Sam Watson's parents, John and Ann Watson, bought the farm a few years after they retired. The Watsons' home, where they live full-time,also serves as the semiregular Sunday lunch gathering spot for the Watsonsand all their children and grandchildren.On a recent Sunday afternoon, a sizable portion of the Watson clan converges on JW Farm for a Mexican buffet and raspberry cake. There are discussions of politics and literature, and Sam's older sisters tease their little brother as if they were all still kids themselves. The usually gregarious Melinda is somewhat withdrawn -- that is, until a bittersweet moment after lunch when she holds a sister-in-law's new baby in her arms, and gently rocks and coos to it.

Luke and Rachel settle into a pallet of quilts under some shade trees in back of the main house. As three goofy-eyed dogs lick their faces, one of their cousins -- 11-year-old Anna McFarland, a cute, skinny kid with shoulder-length blond hair -- sits in a wooden chair at the foot of the pallet with an open book in her lap. When a visitor asks her what she is reading to her cousins, Anna gets up and pulls the stranger aside, out of earshot of Luke and Rachel. "I'm not reading anything," she says with a smile. "I'm just making it up." As Anna goes back to entertaining her cousins, their grandmother takes a seat at one of the glass-top tables next to the swimming pool. Ann Watson is still a striking woman. She is also a woman who doesn't hesitate to speak her mind. Over iced tea she laments that she and her husband can't step in and make Luke and Rachel's problems disappear. "As a grandmother, you feel like you ought to be able to fix it all," she says. She also feels inadequate when trying to answer Luke and Rachel's questions. For example, she says, Rachel recently asked, "Why does God want me to be blind?" Like her daughter-in-law, Ann believes that "God knows what he's doing. I would just like an explanation. And I truly believe that one day I will understand, and I'll say, 'Oh, that's why.' " But unlike Melinda, Ann Watson believes that Luke and Rachel deserve an explanation -- not just about why they are going blind, but full disclosure about their medical condition.

As a former nurse, Ann believes the truth is always in the best interest of the patient. She suspects that Luke and Rachel may be able to handle the truth better than their parents think. "But I have to respect Melinda's wishes," she says. Ann's comments are the only indication of tension within the family over Luke and Rachel's condition. It is an undercurrent of tension that Sam confirms. "That's something we're struggling with," he admits. "I don't even know how to deal with thinking about it. They know there's something going on. But at least right now, I don't think we'll tell them, unless they ask." Sam acknowledges that the situation has been devastating not just to him and Melinda but to their entire family as well. "I know that all my family members love Luke and Rachel dearly," says Sam. "And it's been so hard watching them go through it, too. Everybody's suffering through this thing. It's just one more thing to deal with. Not that it's a burden. We're blessed that they do love them so much. It's just a part I hadn't thought about before. Everybody else is suffering along with us, and they suffer in their own ways." At the same time, Sam and Melinda have been amazed by the kindness of strangers. It's as if people who don't know the Watsons were better equipped -- perhaps better insulated -- to share the family's pain. For example, the Watsons are members of Foundry United Methodist Church, where Astros manager Larry Dierker and his wife, Judy, also worship. According to Sam, when the Dierkers learned of Luke and Rachel's illness, they didn't hesitate to help.

This past spring the Watsons went to Disney World, a trip arranged for Rachel through the Make A Wish Foundation; the year before, the organization had provided the same trip for Luke. Before leaving for Florida, the Watsons inquired through their church if Luke could meet a player at the Astros' spring training camp in Kissimmee. Instead, Dierker arranged for tickets to a game, a tour of the clubhouse, and introductions to several members of the team. "Burt Hooten's wife came and met us at the gate, took us in the complex," says Sam. "[Dierker] came out and talked to us before and after the game. Moises Alou stopped on his way out of the dugout and shook Luke's hand. Signed a cap for him. Tony Eusebio did the same thing. So did Luke's hero Jose Lima. Luke can't see them, but he's in awe of them. "I've just been floored by so many people going the extra mile to help out. They care so much about our situation. It's definitely been eye-opening." In the days and years to come, the Watsons will need those new friends, as well as their family, more than ever. As their children's health continues to decline, the Watsons' financial and emotional pain will only increase.

Although most of their medical expenses are covered by the insurance plan Sam has through the Corps of Engineers, that soon will change, say BDSRA officials. Children with Batten disease often fall through the cracks in the health care system. "If you go in and talk to the different services like social security, Medicaid and a lot of the other services, you'll find that there is no description in any of their books about Batten disease, but there are descriptions of cystic fibrosis, cerebral palsy, the more common childhood disorders," says BDSRA president Johnston. "When the child progresses to where he or she needs full-time care, the only thing the insurance companies will pay for are durable medical goods like wheelchairs, and in-home skilled nursing. But most of the children usually end up with a feeding tube. They have to have breathing treatments and things like that that insurance companies define as custodial care, and they don't cover that. They don't cover diapers, feeding formulas and such things. So we either have to turn to Medicaid or Medicaid waiver programs or some other type of state waiver program. Or foot the bill ourselves." In the few years that it will take for Rachel and Luke to lose all ability to care for themselves, Sam and Melinda Watson will be facing a double set of bills covered by neither insurance nor federal or state programs. But when the Watsons talk about money, they don't dwell on their lack of it or the looming avalanche of medical expenses. Instead, they seem more concerned about the abysmal amount of funding that trickles into Batten disease research each year.

According to the BDSRA, less than $3.5 million was spent on researching the disease last year. "When you stop to figure the cost, it's in the government's interest to put more into research and find a treatment for it," Johnston says. "We need awareness and we need funding." They also need a cure, but none is yet on the horizon. In recent clinical trials scientists made some headway in treating infantile Batten with a drug known as Cystagon. They are also looking into gene therapy and enzyme replacement in the battle against late infantile Batten. But so far little progress has been made in the area of juvenile Batten. In the meantime, about all that Sam and Melinda Watson can do is pray. Pray and enjoy the time they have left with their children. In a small way, they even feel a bit lucky, and maybe even a little blessed. "We've been given the gift of knowing how much time we have left with them," says Sam. "A lot of our friends are going through divorces, and they never see their kids. We can make the best of our time. We don't miss out when an opportunity comes." Sometimes Sam thinks about the future, about the life he and Melinda will share after Luke and Rachel are gone. He doesn't believe they will risk having more children, but he admits, "The thought has crossed my mind." He's fairly sure it has crossed Melinda's mind, too. "I see Melinda when she holds my sister's baby," he says, "and she feels like she'd like to" have another one. "But I've had a vasectomy, so I've kind of passed that point." Reminded that vasectomies can be reversed, Sam laughs out loud. "Yeah, I've seen those signs," he says. "But my experience was so bad, I don't think I want to go back." He then quickly turns serious again. "But I don't think I would ever want to [have more children]. I think it would kind of take away from [Luke and Rachel] for some reason."

Earlier this spring, on one of the hottest days so far this year, Luke guides Melinda and Rachel through several hallways and doors to the enclosed swimming pool at The Lighthouse of Houston complex on West Dallas. The Lighthouse is a nonprofit organization dedicated to assisting the blind and visually impaired. Luke has only been to the Lighthouse pool once, but he remembers his way through the maze. He stops just before each door but has difficulty finding the handles. Inside the steamy pool room, swimming instructors put the children through a series of aquatic drills. As her kids go through their paces, Melinda relaxes on a bench and announces that she has resigned from her teaching position. There's a chance she might go back part-time, but she's not sure. Her reasons are hard to refute: Quality moments such as these will soon fade away; Melinda wants to spend all her time with Rachel and Luke. People say she should get a job at her children's school. But Melinda says it would be too hard on her -- too painful to see her kids around healthy children. Luke and Rachel's swimming lessons are private.

Melinda had hoped for group lessons to provide her kids with some interaction with other blind children. However, the group lessons also include special-education boys and girls. Melinda balks at having Luke and Rachel interact with slow children at this point; they will be special-ed kids soon enough, she says. At the end of the 30-minute session, Melinda wraps her children in a couple of beach towels. As they dry off, Luke and Rachel talk about their favorite foods: He likes pizza; she prefers hamburgers. This summer they will travel to Alabama with their parents to visit one of Rachel's friends. Melinda has told her kids that the reporter is here to write a story about blind children. When asked what is the most difficult thing about losing their vision, Rachel refuses to discuss her situation, other than to say it makes her "mad." Luke, on the other hand, insists he is "happy" to lose his sight. Of course, he also plans to be a major-league baseball player.