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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.

THE BALTIMORE SUN

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.

Strikingly attractive at 17, Keishell said she now wants to obtain her high school equivalency degree, study music and get in talent shows.

She wants to become a rapper.

Her mother accepted the responsibility without complaint but with a sadness that's impossible to hide.

Keishell and her 15-year-old sister, Martine, were entering an age of self-sufficiency, and Paylor thought the time would soon arrive when she could slip out to a movie, get together with friends or go bowling on a whim.

She has pushed those thoughts aside.

"Once you have a child like this, you don't have a life no more," she said. "I can't go anyplace.

"Parents at Kennedy Krieger said, 'You haven't seen nothing yet. You won't have a life.' "

Paylor quit her secretarial job several months ago when she realized she couldn't possibly juggle her work and child care responsibilities.

Now, she lives off a patchwork of public assistance payments. Medicaid pays Jasmine's health care bills, which can surpass $53,000 a month when she stays at home.

A recent two-month hospital bill came to $180,000.

Paylor mines a deep reserve of inner strength and resourcefulness to get things done.

When she discovered she needed a new electrical outlet to support Jasmine's medical equipment, she asked the Park Sausage company to donate a few hours of an electrician's time. The job was gladly done.

Now, she is trying to figure out how to air-condition a room that has no windows.

Jasmine lies in a plastic shell that resembles the portable carriers sold in baby stores.

It is molded to the peculiar contours of her body and rests on a slight incline, elevating her head so that secretions drain away from her lungs. A fleece under-blanket protects her from bed sores.

Balloons and music

On a recent morning, she lay happily in her crib, surrounded by Mylar balloons, stuffed animals and cassettes of nursery rhymes put to music. A tape player boomed one of her favorites.

Nurse Maisonette sang along: "Oh where, oh where has my little dog gone ?"

The nurse hovered above Jasmine, shaking rattles and toys to the music. Jasmine's eyes followed the action, darting left and right, up and down. Her right foot bobbed in rhythm.

Everyone who works with Jasmine takes her attentiveness as a sign of intelligence.

"One thing in her favor is that she doesn't look so sickly when you look her in the face," said Dr. George Capone, a physician at Kennedy Krieger.

"She's very alert, very with it. It may mean that she'll be motivated to do things."

But no one denies that it will take enormous effort to keep her stimulated and well.

Jasmine takes antibiotics, painkillers and drugs for her stomach, kidneys, heart and lungs -- nine medications in all.

Her feeding tube delivers formula 20 hours a day and must be kept clean to prevent infection.

And because she doesn't eat by mouth, her nurses work at keeping her taste buds active by stimulating them with a sweetened cloth.

The major challenge is to keep her lungs healthy. Doctors say there may be no way to put a stop to her respiratory infections, and they flatly admit that one could kill her at any time.

As a precaution, she is hooked to a monitor that will sound an alarm if she stops breathing.

High fever scare

Recently, Jasmine scared everyone when she spiked a 105-degree fever, the result of a lung infection that she might have acquired from a family member who had a simple cold or flu.

She was rushed back to the hospital for yet another week of treatment.

Another challenge, of course, is protecting her bones.

Changing and bathing Jasmine is a feat, accomplished by tilting her this way and that with cradled arms.

L To lift an arm or leg by itself is to risk certain fracture.

"I rock her every now and then, but only for about 20 minutes," said Paylor, who gingerly lifts her onto a bed of pillows resting on her lap.

L "The least thing can fracture her, and it's nobody's fault."

This points to a central dilemma.

"Someone who's this severe, fractures can occur with normal cautious handling," said Shapiro, the Hopkins bone specialist.

"The other side of the coin is that you can't immobilize people like this. The worst thing for even normal bone is not to stress it. You must be subjected to normal stress and strain."

Bones strengthen in response to the forces that are part of

routine exercise, he said.

Jasmine's therapists use simple strategies, such as encouraging her to grab toys that are placed easily within reach. And with her body positioned at a slight incline, her spine benefits from the normal force of gravity.

Some children can gain bone strength artificially -- with metal rods that are surgically implanted into the long bones of the skeleton.

This is probably out of the question for Jasmine. Her bones are so fragile, her body so poorly aligned and her head so wobbly that nobody seriously considers the possibility that she will stand, much less walk.

"She's not going to walk because she's going to have very skinny bones that will never support her," said Shapiro.

"There's no architecture that would be able to support her weight."

An immediate goal is getting Jasmine into a more upright position.

A technician recently fitted her for a more supportive seat that is a grid of interlocking balls, each hinged to the next and adjusted to hug her body.

To give her mobility, the seat can be adapted to a wheelchair that is likely to resemble a motorized stroller with platforms to hold oxygen equipment, intravenous poles and toys.

"The idea is to get her up and be a baby," said Atkins, the physical therapist. "To get out of the house so she can be more functional as part of the family, part of the community.

"You'd like her from the very beginning to be everything she can be. That includes having some control over her environment, enjoying toys."

Little hope in medicine

For now, doctors cannot promise a medical treatment that will greatly transform Jasmine's life.

Experimental treatments offer some hope but are preliminary at best. Growth hormones and bone marrow transplants have been tried with little gain, said Shapiro.

He gets visibly upset when talking about the transplants, saying they needlessly subject children to harrowing side effects.

He is somewhat more hopeful about a class of drugs given to adults with osteoporosis to slow their bone deterioration.

The drugs, called bisphosphonates, have been tried experimentally on adults who have osteogenesis imperfecta, with some evidence of improvement.

For now, anyway, Jasmine's grandmother does not count on miracles.

"You pray every day that she'll be a teen-ager," said Paylor. "But last time she went to the hospital, it was really bad.

"Each time she goes there, it's really hard to imagine."

Pub Date: 4/02/97

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