A Battle to Beat the Odds : Parents of two North Hollywood brothers afflicted with a rare genetic disorder must maintain a vigil against run-of-the-mill bumps and bruises.

Daniel Gordon of North Hollywood is ready for his afternoon snack. The 6-year-old lies down on the living room floor and watches as his mother pushes up his blue T-shirt. She matter-of-factly unplugs the tube that protrudes slightly from his abdomen, inserts a syringe-like bottle and feeds him directly into his stomach.

Nearby, his 2-year-old brother toddles over to the brick fireplace. Debbie Gordon must be especially vigilant, for what would be run-of-the-mill childhood bumps and bruises for most children could be life-threatening for these youngsters.

They have a rare and difficult-to-diagnose genetic disorder called familial dysautonomia that affects Jews of Eastern European descent. Life is so perilous for these children that, until recently, the disorder was considered strictly a pediatric problem, because half of the affected children died by age 5.

Today, said Dr. Felicia Axelrod, thanks to earlier diagnosis and preventive measures, there is an 80% chance that these children will become teen-agers. The average life span is considered to be 30 years.

“But to get them to that point takes an enormous amount of effort. They are always at risk,” said Axelrod, professor of pediatrics and director of the Dysautonomia Treatment and Evaluation Center at New York University Medical Center, which has 423 registered cases worldwide. These children have a faulty autonomic nervous system, which controls such vital functions as swallowing, blood pressure, body temperature, breathing, heart rate and digestion.

There are 21 known cases in California--all but two in the Los Angeles area--and 12 in the San Fernando Valley, Axelrod said.

Symptoms can include poor sucking at birth, erratic changes in body temperature and blood pressure, vomiting, reduced weight gain, excessive sweating, smooth tongue because of a lack of taste buds, spinal curvature and poor muscle tone, frequent pneumonia and lung infections, cold hands and feet, and drooling.

One of the most noticeable symptoms is crying without tears. And because the affected children have decreased or no reaction to pain, they can hurt themselves without being aware of the injury or its seriousness.

It is estimated that one in 30 individuals of Eastern European Jewish descent is a carrier of familial dysautonomia--about the same frequency as Tay-Sachs, another genetic disorder that affects Jewish children. But unlike Tay-Sachs, in familial dysautonomia there is not yet a genetic test to determine if parents are carriers. Researchers at Massachusetts General Hospital are trying to track down that genetic marker.

When both father and mother are carriers, there is a 1 in 4 chance with each pregnancy that the baby will be affected.

“It’s hard to diagnose properly because the symptoms can be so confusing,” Axelrod said. “Some cases are very mild and can look like mild cerebral palsy, or even cystic fibrosis.”

“The earlier the diagnosis is made, the earlier the child can have appropriate intervention,” she added.

Gordon, 37, knew something was wrong with Daniel, but doctors couldn’t pinpoint the problem. As a baby, he couldn’t keep his food down, couldn’t swallow properly and couldn’t suck. As he grew older, liquids would be misdirected into his lungs, causing infections.

He didn’t cry tears. At 2 1/2, he wasn’t talking, had an unsteady gait, drooled a lot and was still vomiting frequently. He was initially diagnosed as having a mild form of cerebral palsy.

Her youngest son, Jonathan, was diagnosed with the disorder as an infant. Gordon and her husband, Greg, have another boy, Benjamin, 4, who does not have dysautonomia but has a 50% chance of carrying the gene.

Jonathan’s temperature frequently dropped to 93 degrees; he was lethargic, wouldn’t wake to eat and wasn’t nursing well. At 4 months, he started projectile vomiting. His skin was often flushed and clammy. Finally, a neurologist made the diagnosis of both children.

Today, the blond, chubby-cheeked toddler has frequent respiratory problems and gets ulcerlike stomach disorders.

In caring for babies with the disorder, “the main thing is to ensure they get good nutrition,” said Axelrod, adding that major advances in treatment came in 1978 with two surgical procedures. A gastrostomy implants a feeding tube in the stomach, and a fundoplication helps the muscle in the lower esophagus prevent food from backing up and being aspirated into the lungs. Other preventive measures include tapping or thumping on the chest to dislodge mucus and misting the air to clear bronchial passages. Sometimes infants are fed nutrients intravenously at night.

Part of Gordon’s daily routine involves taking both children to therapists--Jonathan six times a week for stimulation, physical and occupational therapies, and Daniel four times a week for physical and speech therapy.

There are other dangers to watch for.

“You have to keep them away from hot things; you have to keep checking them for injuries because they don’t feel pain. They’re not aware when they hurt themselves,” Gordon said. “You have to really watch them when they are swimming because they might not automatically take a breath.”

“You have to carefully plan outings to make sure you bring enough food,” she said, referring to the boys’ restricted diets. They cannot drink anything with protein or sugar, for example, she said. “You can’t just stop at McDonald’s if they’re hungry.”

A little bit of stress can throw an afflicted youngster into a dysautonomic crisis--blood pressure rises, the heart rate increases and they may vomit. Valium can help “normalize their fragile cardiovascular system,” Axelrod said.

Irv Ward of Tarzana, president of the Southern California FD chapter, said he believes that early detection helped his granddaughter, Casey, 4 1/2, whose illness was diagnosed as the disorder when she was 5 months old.

Despite a normal birth weight, Casey wasn’t flourishing. “She was frail, and she didn’t develop properly,” he said. “She had continuing lung problems and lots of pneumonia. We don’t how much worse she would have gotten if she was not diagnosed so early.”

Like any proud grandparent, Ward declared Casey today “a very active, very bright and beautiful child. She’s just kind of special.”