Locked inside

Kara-Lynn Hodges is an 11-year-old girl with a sweet smile.

Her mother, Marie Sullivan, is a tiny woman with big determination.

Kara-Lynn has Rett Syndrome, a neurological disorder that mostly affects girls. Considered the most severe form of autism, the condition robs girls of their ability to speak, walk, crawl and use their hands.

Article Photos

Marie Sullivan is shown with her daughter, Kara-Lynn Hodges, who has Rett Syndrome, a neurological disorder that mostly affects girls and robs tem of their ability to speak, walk, crawl and use their hands.

On a hot day this past summer, Kara-Lynn is watching cartoons from her bed with a fan blowing cool air over her.

Her dad, Jeremy Hodges, speaks to her in a cheerful tone and she smiles and claps, opening her arms wide and bringing her hands together. The clapping is something she does a lot. She also moves her fingers in a pen-rolling motion.

Sullivan and Hodges would like to see their daughter out of bed, working on sitting and standing. But the family is having trouble getting the funds approved for a wheelchair that Kara-Lynn can use.

Sullivan said Kara-Lynn seemed like a normal baby.

"She could say, 'Mama, Dada, kaka, Nana,'" Sullivan remembered.

But then even those words are gone.

"They lose things that they gain - it takes things from them," Sullivan said. "She could wake up tomorrow and forget how to swallow."

Scoliosis can be a symptom of Rett, and by last year, Kara-Lynn's back was at a 143-degree curve.

"You had to pick her up in a cradle-like fashion, not under her arms like normal," her mother said.

After her surgery last year at the Shriners Hospital in Philadelphia, Kara-Lynn went from 3-foot-9-inches tall to 4-foot, 5 inches.

Sullivan has learned as much as she can to help her daughter.

"I got the book," she said, pulling a thick volume from under the coffee table in the living room of their small, upstairs apartment. She's read it cover to cover, and she's found even more information online from other parents through sites like YouTube and Facebook.

Kathy Kenngott of Akron has a 17-year-old daughter, Sarah, who has Rett Syndrome. Kenngott is the regional representative for the International Rett Syndrome Foundation, which is based in Cincinnati.

There are about 20 families in the northeast Ohio vicinity - Youngstown, Akron, Cleveland, Garrettsville - that are affected by Rett, she said.

The condition manifests itself in different ways.

"It depends on what mutation you have and the severity of that mutation," Kenngott said.

Some Rett children can walk, some cannot. Some will have no purposeful hand use. Some are communicative with their eyes, while others appear blank.

Kenngott said Sarah has never walked, and she also had surgery for scoliosis. At the time, they had just received a new chair that was then changed to suit her daughter "because they are so expensive."

Another common symptom of Rett Syndrome is seizures. When Kara-Lynn was going out of the home for therapy, Sullivan said her daughter was having 20 to 25 seizures each week because of the excitement and stress.

Sullivan has learned to do all the physical therapy herself at home, and now Kara is down to one or two seizures a week.

Since the surgery in Philadelphia, Sullivan has been trying to work with Medicaid to get a wheelchair. The girl has been evaluated by a certified physical therapist, so Chesapeake Rehab Equipment in Pittsburgh has the specifications.

When Kara-Lynn sits in her current regular wheelchair, her ankles appear bruised because of lack of circulation. She can spend about 15 minutes that way. A new chair would prevent the circulation problems, provide a spring for her rocking motions and grow with her. In addition, a standing table would allow her to stand every day, putting weight on her legs.

"If I had the proper tools, I could do more therapy with her," Sullivan said. "I don't understand why she can't have it."

Sullivan said there is a 16-year-old girl in a different country who also is in need of a chair.

"Her mom has been carrying her for 16 years," she said. "It's so sad, because not even a lot of doctors know a lot about Rett Syndrome, and all us parents are trying so very hard to help our girls and get their stories out."

Kenngott said she's been to school and parent meetings and listened to parents talk about their children's disabilities - or in many cases, one disability.

"I think, 'I wish that was her only problem,'" she said. "The girls with Rett are just locked away. They are the classic multi-handicapped child."

According to Girl Power 2 Cure, a nonprofit organization, Rett Syndrome is as prevalent as cystic fibrosis, ALS and Huntington's, with another little girl born with it every 90 minutes. That makes 1 in every 10,000 births.

That group believes Rett Syndrome can be the first reversible neurological disorder. The cause of Rett was discovered in 1999. A gene called MECP2 produces a protein in the brain that controls thousands of other genes. Girls who develop Rett Syndrome have a mutation on this gene and cannot produce enough protein to function normally. Recent research, however, has shown the syndrome to be reversible in mice.

GP2C also offers a site for families called rettgirl.org, where there are stories, blogs and ideas shared between parents.

So as Kara-Lynn seems trapped inside her body, and as her parents hope the paperwork to get the chair is right this time, families of Rett girls and Rett girls themselves support each other in their quest to communicate and be healed.

Since that hot summer day, Sullivan said her daughter has learned to recognize about 15 sight words, such as "on," "off," "chair" and "dog." A teacher has been coming to their home. Sullivan said Kara has been "pretty smiley."

She's hoping when the chair comes Kara-Lynn will be able to start school.

"To tell the truth, I am going everywhere I can and still no chair," she wrote in a recent email. "I am just crushed as a mom. I feel a drunk that falls and now needs a chair would get one faster than my Kara - sad, but it's true."

mrobbins@tribtoday.com