Keeping Hope Alive
08/21/2006 AT 01:00 AM EDT
08/21/2006 AT 01:00 AM EDT
"I want to go swimming!" cries Hope Moss for the umpteenth time as she watches her 8-year-old twin brothers, Trevor and TJ, splash in the family pool. Hope is a slip of a 6-year-old—just 32 inches tall and 25 lbs.—but she packs a spirited will. Worn down, Hope's dad, Tony, sets her in a circular float, only to begin his own nagging litany. "Hope!" he yells as she lunges for Trevor. "Let go of him." When TJ rests a head on Hope's float, he barks, "TJ! No! Off, please." The twins tear across the pool, and Hope chases after them. "Settle down," screams Tony, "no more racing!"
The swimming pool is one of the safest environments for Hope, but even buoyed by water, she requires supervision every moment. One of the roughly 15,000 Americans known as "children of glass," Hope has osteogenesis imperfecta (OI), an incurable but not fatal genetic disorder involving bones so brittle that they can snap at the slightest touch. In her short six years of life, she has racked up so many limb, hip, back and rib fractures that her emotionally fatigued parents lost count at 120—a tally that includes neither the arm Hope snapped in March while putting her toys away, nor the two back breaks she sustained last December when she fell on her behind while trying to walk. "The name of the game," says Tony, 40, an electrician at a paper mill, "is to get Hope as comfortable as possible so she doesn't have any more pain than she has to."
Winsome, bright and more than a tad bossy, Hope herself would rather show off her halting steps or her dolls than talk about her illness. "I don't mind it," she says in a feathery voice, then scoots off on her behind, her preferred way of navigating the family's three-bedroom house. "She's a tiny, shiny star," says Dr. Horacio Plotkin of Children's Hospital and University of Nebraska Medical Center in Omaha, who has treated Hope since she was 3 months old. "Her bones are weak, but she's amazingly strong."
Still, pain has been a problem since the day Hope was born—and probably before. After ultrasounds indicated that her femurs were getting shorter, Tony told his wife, Stacey, "We're gonna name her Hope because we're hoping she's gonna be okay." Delivered two weeks early by C-section, "she was chubby and beautiful," says Stacey, 40. "I was so relieved." Then a nurse stretched Hope's tiny limbs to take a standard measurement. "Hope screamed bloody murder," her mom recalls. X-rays found breaks in both legs and her left wrist. "They handed us literature on OI, told us most marriages end in divorce," Stacey says. "We were told, 'If she makes it through the first year, you'll be lucky.'"
Her first three months, Hope broke bones even just lying in her crib. Only Tony had hands big enough to hold her—but his careful touch wasn't failsafe. "That's an eerie feeling, to pick your child up and feel the ribs break," he says. Then, a ray of hope: They heard about a pioneering OI unit at the Shriners Hospitals for Children in Montreal. Stacey calls their first trip to Canada "life-changing." After Hope received three four-hour IV treatments of Aredia, a drug that strengthens bones, her pain eased. Doctors also offered the anxious parents a new way to look at Hope's illness. "You know she's gonna break bones," Tony says. "Their philosophy is to make life as normal as possible."
It is uncertain that Hope will grow much bigger. But buttressed by regular IV treatments and two metal rods in each leg that help her bones grow straight and minimize fractures, she is enjoying a good patch. "She's getting very playfuller," TJ helpfully explains. Able to wear only diapers until age 4 because snaps and buttons were too harsh on her spine, Hope now wears clothes in a toddler's size 2. She's finally mastering toilet training, which was disrupted four times by surgeries that left her encased in toe-to-chest casts. In June she completed kindergarten, which she attended in a wheelchair, escorted by Danni Kallerman, 24, one of two state-financed aides who together care for Hope up to 52 hours a week. "She makes friends supereasy," says Kallerman. Breaks cost Hope 73 school days, yet she stayed on top of her work, deftly switching to her left hand when her writing hand was hindered by a cast. "School is one of those areas where she's a perfectionist," says Stacey.
Hope is a happy child, given to marrying off her Barbies and exclaiming, "I love my life!" But she is growing self-conscious about her disability. Though people with OI overheat easily, Hope insisted on wearing sweaters to school to hide her back brace. "She'd sweat to death before she'd let someone see it," says Tony. Recently Hope told him, "Dad, I just want to be normal."
Hope does not yet register the financial and emotional toll her illness takes on her family. Even with health insurance and free hospital care in Montreal, her health-related expenses run so high that the Mosses declared bankruptcy in 2003. Because Hope sees doctors in Montreal, Nebraska and Minnesota, Tony and Stacey are away from home often, costing Tony wages—and both parents time with their sons. (Usually Tony's mom, Donna Vellieux, steps in.) "I don't like Mom and Dad leaving," says Trevor. The boys want to play hockey and baseball, but there's no money for league expenses and no one to carpool them. With a resigned shrug, Tony says, "Pretty much their whole life is structured around Hope."
Tony and Stacey's too. Their scorecard of child-free outings since Hope's birth stands at one movie, one wedding and no dinners. Further complicating life, Stacey was diagnosed with congestive heart failure when Hope was 14 months old, a shock that caused Tony to suffer what he describes as a "nervous breakdown." Feeling their marriage unraveling, the couple sought counseling. Today, still plagued by breathing and back problems that leave her unable to lift Hope, Stacey can only look on as Tony juggles childcare, housekeeping, cooking and shopping on top of his job. "I can't imagine how many men would stay through that," she says.
Tony deals with the stress by taking it one day at a time. "Today is today," he says. "I don't know what tomorrow will bring." Stacey worries about Hope's future. "I hope she lives long enough to have children," she says. Dr. Plotkin says Hope's life expectancy "is the same as the general population" and she will be able to bear kids despite her diminutive size. But Stacey and Tony know that prognosis could change in an instant if, say, a rib or spine break were to puncture an organ. So they seize every chance to give Hope experiences: zoo trips, fireworks displays, a tour of Niagara Falls. "There's never a minute," says Stacey, "when I'm not grateful she's with us."
For more on OI, go to www.oif.org