It started with double vision. That’s when Ellen Horton knew something was wrong with her husband, Raymond. The couple was en route back to Shelby from a car show in Pigeon Forge, Tenn., in September. She described his driving as erratic.
“He was all over the road. He would run off the road and cross the center lines,” she said. “I found out (that) to him, the line in the roads were curved because of his eyes.”
“He couldn’t hold a sandwich. He forgot where his mouth was to feed him,” Horton remembered. “He was fine in Pigeon Forge. He saw the ceiling fan continuously turning, but the fan was off.”
Reading became impossible for Raymond. The avid reader had read the Bible more than once. Horton said all Raymond wanted was his eyesight back. Tears sat on the rim of her eyelids. What Raymond is believed to have battled affects less than 400 people each year, according to the Centers for Disease Control and Prevention.
It would be December before Horton discovered what plagued her husband. Raymond saw Dr. Kevin Klein, head neurologist of Klein Neurology and Sleep, in December. Klein used an EEG and MRI to gain a closer look inside Raymond’s brain.
“He was unsteady when he walked. He had recent issues with memory,” Klein recalled.
The answers lied in the MRI scan, which shows detailed images of organs and tissues. Parts of Raymond’s brain cortex appeared white on the MRI scan. Klein said the discoloration, or abnormal diffusions, were similar to what doctors see in stroke patients. Raymond never had a stroke.
“Most people who have what he did don’t lose their vision first, but he did,” Klein said.
Klein diagnosed Raymond with Creutzfeldt-Jakob disease. The rare, degenerative brain disease — not unlike mad cow disease — is marked by abnormal proteins called prions that can develop in humans and animals, the CDC reports.
It was previously reported two Cleveland County deaths with links to CJD, one in December and another in January. Both deaths were labeled “probable cases” of CJD.
CJD can come in various forms, including classic/sporadic, which develops naturally in humans. It can lay dormant for many years. Symptoms usually surface about age 65 in those affected, according to the National Library of Medicine’s website. The disease is contagious.
Horton found relief in her husband’s diagnosis. But Raymond’s condition worsened. He was admitted to Cleveland County Hospice in mid-December. Nurses bathed and shaved him. Members from the N.C. Foothills Marine Corps League Detachment 1164 visited their former commandant while he was in Hospice. Horton recalled a moment where she laid her head on her ailing husband.
“I told him I loved him,” she said. “His mouth moved and I read, ‘I love you, too.’”
She sat calmly on her living room couch. A white tissue caught the falling tears from her eyelids.
“He went through three months of hell,” Horton said.
Raymond died Dec. 28. He was 66 years old.
All forms of CJD are fatal. There is no known cure.
Klein diagnosed two other patients in Cleveland County with sporadic CJD in 2012. Both were between 60 and 70 years old. Both passed away shortly after.
“That’s a high number,” Klein said. “It’s unknown where sporadic CJD comes from.”
CJD can mirror other diseases such as dementia, Alzheimer’s and some later-staged cancers. Symptoms include difficulty speaking and swallowing, memory loss, blurred vision and jerky movements, according to the National Library of Medicine’s website. Raymond displayed all of them.
About 85 percent of CJD cases in the nation are sporadic, the CDC reports. Since 2011, The N.C. Department of Health and Human Services reported 17 confirmed cases of CJD. Nine were labeled probable. Most with sporadic CJD live up to seven months before succumbing to the disease, the MayoClinic reports.
CJD can only be confirmed through a brain biopsy or a cerebrospinal fluid test, according to the National Institute of Neurological Disorders and Stroke’s website. All three of Klein’s patients are called “probable cases” of CJD because neither test was performed. Any tests to confirm CJD must be arranged by the hospital handling the case, it was previously reported.
Horton said, because CJD was new to her, she didn’t realize a brain biopsy could be done. Later, Horton requested a test on Raymond’s spinal fluids from Wake Forest Baptist Medical Center in Winston-Salem. The results are pending.
Horton believes Raymond suffered from CJD after a doctor from Wake Forest Baptist agreed with Klein’s diagnosis.
“I didn’t want to put his body through anymore,” Horton said about opting out of a brain biopsy.
Horton described her husband as an “all-around kind of guy.” Those who found a father figure in Raymond called him “paw-paw” or “Daddy Raymond.” She doesn’t know if Raymond knew he had CJD.
“He never asked about it or mentioned it,” she said. “I still wonder if I’m going to end up with it.”
Raymond’s case was the first time Horton heard of CJD. It could be the same for many others. She hopes sharing Raymond’s story not only leads to closure, but awareness.
“We don’t know what we’re walking around with in our bodies. We don’t,” she said. “So little is known about it.”
Horton clutched a framed picture of Raymond smiling. He wore a Marines cap. Horton said, she and Raymond lived to help others. She wants his story to do the same.