Family fights nervous system disorder
Drug trial could slow disease, but funds are needed.
Tuesday, April 22, 2008
FAIRFIELD —Fairfield grandparents Dennis and Sharon Depoi love their grandchildren but are heartbroken to think they only have a short time with two of them before they are expected to die.
Grandchildren Courtney Johnson, 10, and Noah Johnson, 7, of Georgetown were diagnosed last year with Batten Disease, an inherited disorder of the nervous system.
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Get latest headlines via RSS feedsThey are only expected to live into their late teens or early 20s, unless the Depois and other families facing Batten Disease can get a clinical trial started. Batten Disease Support and Research Association officials are looking to raise $500,000 for that.
Currently, there is no hope to reverse the effects of the disorder, which robs children of their eyesight and mental capabilities as the brain reduces in size, according to the National Institute of Neurological Disorders and Stroke. But a clinical trial can test a drug used on children in need of organ transplants to see if it can slow or halt the process, Dennis Depoi said.
Noah has developed blindness, but it hasn't affected the straight-A student's mind, Dennis Depoi said. Courtney has suffered the vision loss and mental problems, Dennis Depoi said.
"Courtney still does go to school, but they don't grade her because she can't remember what four and four is sometimes," he said. He said she behaves like a child with dementia.
"She'll be in her own house and say, 'Where am I at?' or she'll look at us and say, 'Who are you?' " Dennis Depoi said.
The siblings' older brother, Jacob, 14, does not have any signs of the disease.
Public awareness
Courtney and Noah are two of about 2,000 children worldwide and 10 in Ohio known to have the disease, said Lance Johnston, executive director for the Batten Disease Support and Research Association.
Batten Disease is the common name for neuronal ceroid lipofuscinoses, or NCLs, Johnston said. The Johnsons have the juvenile version of NCL.
"We really don't know how many there are in the United States, much less in the rest of the world," Johnston said.
About 75 families worldwide, and about 50 to 60 families in the United States, become part of the BDSRA each year. The association grew from 60 families in 1987 to 1,200 families, which include 500 children with all forms of Batten Disease.
