Faces of Rare Disease: Batten Disease

Faces of Rare Disease: Batten Disease

A four-year-old Bell City boy has just been diagnosed with a deadly disease that is causing him to go backwards in development. Speaking, chewing, swallowing, seeing and walking were once thoughtless tasks for Coy Fruge, but now he is rapidly losing each ability.

Teri and Jeremy Fruge's dream of starting a family together became reality four years ago when beautiful baby boy, Coy, was born. "I always wanted to be a mom," said Teri, "and he always wanted to be a dad."

The first year of life for Coy was marked by the typical milestones: rolling over, crawling, cooing and smiling. "It was pretty normal," said Jeremy, "he was fine and showed no signs of anything the first year."

At fifteen months, Coy's development seemed to shift into reverse - stumping these parents and the doctors they turned to. "Coy stopped talking and he went backwards," said Teri.

"He kind of quit talking and wasn't really doing the things he had been doing before," said Jeremy.

Teri was a teacher at the time and knew the importance of keeping Coy's development on track before getting into a school setting.

Coy underwent intensive speech therapy, but even after two years there were no improvements. "After he started being delayed with speech, he began to drool and Coy had never drooled," said Teri, "that really concerned me and then he started choking on his food."

Then, the scariest changes could be seen just by looking into Coy's eyes: dilated and glazed over.  It was not long until seizures started and the Fruges were at Texas Children's Hospital for genetic testing last fall.  "The last panel they did, the one that found Batten's," said Teri, "they tested 40 genes and they found it."

That one defective gene keeps Coy's body from being able to break down fats and proteins or "lipopigments." That builds up in the body's tissue, depositing in the brain, eyes, skin and muscle.  "His body will deteriorate slowly," said Jeremy, "his muscles, his cognitive ability and finally to take his life."

The prognosis is not good. "A child with Batten's is not expected to live past 12 and the average age is 10," said Teri, "it's always terminal, there's no cure, there's no treatment."

This disease, offering little hope, is autosomal recessive - meaning a child has to inherit two copies of the defective gene, one from each parent. Two-year-old Gage appears to be unaffected, but Coy's diagnosis is confirmed. "Getting the diagnosis was horrible, but having to tell our parents that they were going to lose their grandchild was much worse," said Teri.

"You're pretty shook up and worried and scared...just helpless," said Jeremy.

As Coy's brain dies, his body does too.  "You lose all muscle tone, which Coy's already losing, and they stop walking and start crawling, stop crawling and they're bedridden," said Teri.

Coy is also losing his sight and will eventually become blind.  There are days that he cannot balance enough to walk.  Falls happen a lot and Coy is used to his protective helmet.

But even with a parent's arms holding him up, Coy can still enjoy being a big brother and a spring of joy in his family.  "My hope is that while we have him here, he is blissfully happy and he only knows love and that he can remain with us as fully as possible," said Teri.

Coy is on several medications to minimize his seizures and also does physical and occupational therapies.

Batten Disease affects about two out of every 100,000 people.

Insurance does not cover a lot of the treatment, including travel back and forth to his doctors in Houston. You can help off-set the medical expenses at this Saturday's benefit for Coy Fruge at Wilkerson Arena located at 2400 Highway 384 in Bell City.

It is called "Cowboys for Coy" and is cowboy-themed with a ranch rodeo, break away roping, kids activities, food and music by Geno Delafosse.  There will also be live and silent auction.  Click here for the event details.  Everything begins at 9:00 A.M. and ends after the ranch rodeo!

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