Fragile Hopes

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 14 months, Jasmine Pass might not have a major bone that hasn’t broken at least once.

Look at her 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 Jasmine in a narrow Baltimore row house.

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.

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

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.

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 that 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.

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.

Many people don’t grow beyond 3 or 4 feet tall. 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, 1996. Her mother is Keishell Pass, Paylor’s 17-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.

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.

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.

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.

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

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.

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 Jasmine onto a bed of pillows resting on her lap.

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.

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,” Shapiro said.

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, Shapiro said.

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 teenager,” Paylor said. “But last time she went to the hospital, it was really bad.

“Each time she goes there, it’s really hard to imagine.”