LEBANON, Ohio - A wheelchair-accessible van will not extend the lives of Layla and Angel Martin of Lebanon. The best that their parents can expect is that the prize in an Internet contest will ease the difficult days ahead for the little girls.
Layla is 3, Angel is 2. By a brutal roll of the genetic dice, they suffer from an exceptionally rare neurological disorder, and they probably will not see five candles on a birthday cake. Even crueler is the possibility that their baby brother also has the disease.
“Sometimes,” says the children’s mother, Christina Martin, “I ask myself, ‘Why would God give me these children if He is only going to take them away from me?’ It’s a hard thing to understand.”
Feet that "curled up like candy canes"
Christina Shirkey and Bradley Martin dated in high school then went their separate ways, married other people, and each had a daughter by the time they reunited and married. Christina dreamed of nursing school. Bradley worked for Cardinal Landscape, Tree Service and Lawn Care. In April 2011, Layla came along, “a fine, normal baby,” Christina said, who in her first year quickly mastered walking and talking. (Below: A photograph of Layla before she got sick hangs in the Martin home in Lebanon)
But one morning, when Layla stood up, her toes rolled under; her feet had “curled up like candy canes.”
The development was alarming, but doctors at first decided that Layla would grow out of it. Then Layla stopped walking. Her eyes crossed. She woke screaming in pain or gasping for air. Some mornings, she couldn’t be woken at all. Finally, a magnetic-resonance imaging test delivered a shocking result: Layla’s brain was shrinking. What the MRI couldn’t explain was why.
The search for an answer meant repeated trips to hospitals in Cincinnati and Cleveland. Doctors were universally mystified. Layla got double pneumonia and barely survived. She now has a feeding tube.
Angel came along in March 2012 and, to her parents’ horror, precisely tracked Layla’s path.
“I had a feeling,” Christina said. “I didn’t want to accept it. It didn’t seem possible.”
But Angel’s brain, too, was shrinking. The family's calendar filled with trips to specialists. In the midst of the medical whirl, in October 2013, Christina had a third child, a boy named Bradley Jr.
A diagnosis, finally
In March, genetic tests at last yielded a diagnosis: infantile neuroaxonal dystrophy, a disease so rare that Layla and Angel apparently are the only Ohioans with the condition. The doctors suggested sending the girls’ records to the one place in the United States studying the genetic disorder, in Portland, Ore. Christina and Bradley agreed.
For still-unknown reasons, a gene can cause the buildup of iron in the brain and cause a host of illnesses, including Alzheimer’s disease and multiple sclerosis. An international consortium of institutions is studying infantile neuroaxonal dystrophyn, and the leading U.S. facility is Oregon Health and Sciences University.
For years, infantile neuroaxonal dystrophy was nearly impossible to diagnose without risky, painful procedures such as a nerve biopsy. In 2006, researchers found the recessive gene that causes the disorder. For the disease to develop, both parents must carry that gene then pass it to their child.
But diagnosis brings more bad news: The disease worsens over time. Babies lose milestones they once had and ultimately go blind and become bedridden. Life expectancy is five to 10 years.
Support for families is the best medicine
Christina Martin sits in her Lebanon home with her daughters Layla, Angel and Brianna, 13, who is the toddlers' half-sister. Brianna helps to care for her younger sisters who suffer from a rare genetic disorder.
Genetic counselor Allison Gregory works with Dr. Susan Hayflick in the department of molecular and medical genetics to study the condition. The National Institutes of Health classifies it as “ultra-rare,” with the incidence about one in a million. Gregory says the global registry of cases, about 20 years old, lists a few hundred families.
Medical advances allow sick children to live longer.
“You don’t die from INAD,” Gregory said. “You die from being immobile, with fluid in your lungs, from aspirational pneumonia, from poor nutrition from the feeding tube. But we’ve gotten much better at managing these secondary issues that can really be life-threatening. And parents are more assertive in taking care of their kids.”
Gregory said new research suggests other genes may come into play, raising the prospect of gene therapy. Studies of mice at Washington University in St. Louis could yield treatment options. But all that progress is years away.
“What parents of these children need is support from the community,” Gregory said. “They could use extra support driving kids around, with therapies, with medical bills piling up. We have families raising funds for research, which is constantly amazing to me. That’s one of the nice things – we’re a small community, but we all band together. We have a common goal.”
It takes a community
The Martins attend Village Community Church in South Lebanon, and the congregation has pulled together to help the family.
Christina finds comfort there, but she often wonders how she and Bradley will manage with two sick children--and perhaps a third. Bradley Jr. recently had blood tests to learn whether he has infantile neuroaxonal dystrophy. The family must wait four to six anxious weeks for an answer.
“I don’t know if there’s any real comfort when you’re faced with what she’s facing,” said Kevin Peyton, the church’s pastor. “It’s still a mother losing a child. What I hope Bradley and Christina experience here is a pure community, despite the circumstances they’re facing in this present reality. It’s the relationships that we take with us. There’s a little bit of heaven in that for them, I hope, that there are people who really care about them.”
Winter is a tough season for landscapers, and during the past hard winter, Bradley Martin was out of work for months. Peyton said Bradley mentioned that if he had more tools, he could pick up jobs on evenings and weekends.
“So I reached out to some business guys in the community, and I told them I’d put in the first $100 to get Bradley some rakes and shovels and a wheelbarrow. In about 48 hours, we raised $2,000.”
The money not only bought new tools, but a used truck.
“I told Bradley, ‘The guys want to remain anonymous. They’re silent partners. Just know they love you,’” Peyton said.
Hoping for a win
In a small duplex in Lebanon, Christina Martin soothed Layla and Angel as they howled through nights of pain. An easy day is only one doctor’s appointment for either Layla or Angel; some days have four appointments. The Martins have a used minivan, but getting a toddler’s wheelchair aboard exhausts Christina.
Through friends, the Martins learned of an Internet contest for “local heroes” run by the National Mobility Equipment Dealers Association, a nonprofit group that donates American-made wheelchair-accessible vans as prizes. The contest is in its third year. Voting on the website began March 11 and ends May 9. The top 10 percent of vote-getters then go before a panel that will choose four winners, to be announced in late May.
The church community has been turning out the vote; Christina’s mother, the deli manager at the Kroger on Mason-Montgomery Road, rallied the vote among store workers.
Christina wrote the entry nominating Layla as her local hero before she and Bradley learned of the diagnosis, when they had two sick little girls and no answers. The entry celebrates the few precious moments when Layla still does things children do:
“No matter what, I know things will be okay, because if my daughter can smile and move forward being so happy with herself, I know I can, too.”
Connect with WCPO contributor Anne Saker on Twitter: @apsaker.