Break, heartbreak Bones: A Baltimore grandmother tries to care for an infant afflicted with a disease that causes her bones to fracture at the slightest pressure.

April 02, 1997|By Jonathan Bor | Jonathan Bor,SUN STAFF

X-rays tell the story of her life: healed fractures across her arms, legs, ribs and neck, fractures so numerous that the question "How many?" seems cruel and absurd.

At 9 months, Jasmine Pass might not have a major bone that hasn't broken at least once.

Look at Jasmine and you see eager, intelligent eyes -- eyes that follow everything that moves.

Look again and you see a child spending life in a molded shell, a child who can break at any moment, a child who is handled like china.

"She'll have fractures her whole life," said her 38-year-old grandmother, Ellen Paylor, who cares for her in a narrow Baltimore rowhouse on North Lakewood Avenue.

"This is the period of time to tell whether she's going to make it or not. This changes you all the way around."

In an era when scientists can clone sheep and slow the course of AIDS, they are powerless to reverse Jasmine's condition, the "soft-bone disease" known as osteogenesis imperfecta.

The genetic defect made her bones so fragile that they started cracking in the womb, the softest and safest place of all.

Six weeks ago, Jasmine came home from the Kennedy Krieger Institute Inc., the specialty hospital where she spent many months getting used to feeding tubes, respiratory equipment, seating devices and the other equipment that sustain her.

Her grandmother and nurses are proud that she seems to have gone without breaking anything since she's been home.

One cannot be certain because pain medication can mask a fracture -- and because, even in the best times, small bones can crack and heal in an endless cycle.

Protecting Jasmine is a daily challenge.

And no matter how carefully one lifts, turns or cushions her, there are hazards that come from the simple business of living.

She can literally sneeze and break a rib.

"She can break her arms herself just by lifting them," said Anna Maisonette, the energetic woman who was Jasmine's day nurse until a fall down the stairs sidelined her.

"She can lift them like she's trying to get to me, and there's nothing I can do to prevent it."

There are an estimated 15,000 to 20,000 people in the United States with osteogenesis imperfecta. It varies greatly in severity, with some children so mildly affected they can play sports.

Many use walkers and wheelchairs but go to mainstream schools, drive cars and find employment. Others live lives of great dependency.

Few, however, are as disabled as Jasmine.

"She is probably the most severe that lives," said Eileen Atkins, Jasmine's physical therapist at Kennedy Krieger.

Any worse, she explained, and the child is unlikely to make it past birth.

The disorder is caused by an inherited defect of collagen, long intertwined proteins that form the supporting matrix for bone.

Collagen normally functions like steel reinforcing rods in a concrete structure.

The faulty gene, however, produces fibers that are poorly organized -- relaxed rather than tightly bound.

"You don't end up with very structured bone," said Dr. Jay Shapiro, a nationally regarded authority on bone diseases at the Johns Hopkins School of Medicine.

"It's of much looser texture, depending on the severity."

Many people don't grow beyond 3 or 4 feet. Their arms and legs bow inward, the result of muscles pulling on soft bones.

The soft-bone effect is seen even in the forehead, which is often wide and flat. This is the effect of gravity pulling on a developing skull.

Jasmine, who has all these characteristics in the extreme, was born June 18 at Mercy Hospital.

Her mother is Keishell Pass, Ellen Paylor's 16-year-old daughter. Doctors performed a Caesarean section because the baby was in a difficult feet-first position, but her skeletal condition wasn't known until doctors delivered her and found extensive fractures.

She was transferred to the Johns Hopkins Hospital and, later, to Kennedy Krieger.

Back to the hospital

Doctors discharged her four times, but each time she developed life-threatening lung infections after inhaling formula that had backed up from her stomach.

The problem stems from the physics of digesting food in a nearly horizontal position, and her misshapen torso is of no help in keeping food down.

The first time Jasmine came home, Paylor realized she would have to raise the child.

Keishell made that clear.

"She said she couldn't take care of her with the disease she had," Paylor said. "Either I take her or she'd be put in an institution."

"Even if she was healthy," Keishell said recently, "it would be hard for me.

"Having a child who has a disease and needs a lot of attention, at my age it's like backing me up. She has to have oxygen, feeding tubes. Her bones break easy."

Ordinarily, the soft-bone disease occurs when a child inherits a single dominant gene from an afflicted parent. But tests show that neither Keishell nor the baby's father, who is not helping to raise her, carries the culprit gene.

This could mean that the genetic flaw started in one parent's sperm or egg -- an accident of nature -- and didn't enter the family history until Jasmine was conceived.

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