Creutzfeldt-Jacob Disease

Couple learn to live, laugh as death nears Brain disease leaves Wilson man with only 6 more months of life.
By Julie Bisbee
Staff Writer
THE OKLAHOMAN
Sunday, July 6, 2008
WILSON — After 55 years of marriage, Clara and Don Sparks are learning to do things differently.
They have no choice. A fatal disease apparently is attacking Don Sparks' brain, making it difficult for Sparks, 75, to walk or control his movements.
This man, who spent 50 years of his life working in the oil fields of southern Oklahoma, now relies on his son to carry him to the car and needs help standing up.
These are some of the symptoms of Creutzfeldt-Jakob disease or CJD, the brain disorder that doctors believe Sparks is battling. Doctors can only make the diagnosis based on his symptoms — poor balance, forgetfulness and involuntarily muscle twitches. The only way to be sure of the diagnosis is to test a patient's brain tissue after death. Doctors have told Sparks he has six months to live.
Facing death and his dwindling physical capabilities, Sparks is surprisingly upbeat. He jokes with neighbors, asking them to help him pick out a casket. Most things tickle him, and his chuckle is infectious.
"You can laugh or you can cry," Sparks said. "I like laughing best."
How it began
Clara Sparks, 72, remembers clearly the night her husband's brain began turning on itself. Don was working as a security guard at a nearby casino and called because he couldn't remember how to turn on the computer at work — a task he had done daily since he began working there.
"I knew something was wrong. We have a computer here and he messes with it all the time," she said. "I just told him to get one of the girls to show him how. I knew then that something was getting bad."
Years of hard labor in the oil fields had tangled up the nerves in Don Sparks' back. First his doctors thought that maybe a pinched nerve was causing him to have memory lapses.
The Sparkses went to numerous doctors searching for treatment or an answer.
Finally, they ended up at neurologist's office in Temple, Texas. A doctor looked at an MRI image of Don Sparks' brain and saw a shadow. Then as he asked Sparks to walk down the hall, watching his unsteady stride, the doctor knew, Clara Sparks said.
"He could tell just by the way he was walking what was wrong with him," Clara Sparks said. "He said he didn't want to tell us what it was, but he knew he'd have to sooner or later."
About the disease
Creutzfeldt-Jakob disease is often compared to bovine spongiform encephalopathy, or "mad cow disease." The diseases are similar in how they affect the brain. Eventually, the protein mutation in the brain tissue will eat small holes in the brain tissue, causing damage to the central nervous system.
In cattle, a tell-tale sign of BSE is stumbling and poor balance in the livestock.
The two diseases are related, and a small percentage of Creutzfeldt-Jakob cases can be linked to consuming contaminated beef. However, no cases of Creutzfeldt-Jakob in the United States can be attributed to eating contaminated meat, said Kristy Bradley, state epidemiologist with the Oklahoma Health Department.
In fact, the cause of most cases of Creutzfeldt-Jakob is a mystery. Some cases can be traced through the lineage of family genetics. Other cases appear spontaneously, however, and quickly take hold of a person's life.
Experts say the disease typically hits people over 55. About one out of every 1 million people will be diagnosed with the disease. In Oklahoma, roughly 3.5 million people live in the state, so the state can expect to see an average of three cases of Creutzfeldt-Jakobper each year, Bradley said.
"We've had family clusters of this illness in Oklahoma," Bradley said. "It can be a genetic mutation passed from family member to family member."
Having an autopsy after a person's death is the only way to confirm Creutzfeldt-Jakob disease, Bradley said.
"That final step really needs to be taken to confirm and arrive at the diagnosis," Bradley said. "It's very difficult for families to watch their loved ones slowly deteriorate. But there's an additional reason to encourage families to do the testing. If it's the inherited form of CJD, that could have health implications of other family members."
A long life together
Don and Clara Sparks married young. Don Sparks was dashing and kept rough boys from harassing Clara, who worked at a cafe in Depew. After a few months of dating, Don Sparks left eastern Oklahoma for Marine Corps boot camp.
"I was so mad at him," Clara Sparks said. "He joined and didn't say a thing to me."
But she waited for him to come back, pouring coffee and brushing off the advances of other boys who asked her for dates in Sparks' absence.
Soon after he returned from training at Camp Pendleton, Clara and Don married.
"She talked me into that," Don Sparks said jokingly.
The Sparkses share the same birthday. When they married on a June day, Clara was 15, Don was 19.
"I just always kind of thought we were made for each other," Clara Sparks said.
Staring down death
The couple have three sons and five grandchildren. They've operated a business together and now stare down death together.
In March, doctors told Don Sparks he had six months to live. He's not in pain, and the only physical signs that Sparks' brain is betraying his body is the limited use of his legs and the involuntarily movements of his hands from time to time.
Clara Sparks' sons have told her to be tough and strong, but tears spill down her cheeks as she contemplates life without her husband.
"Used to be, there wasn't nothing he couldn't do or figure out, now he can't remember how to fix stuff," Clara Sparks said. "It's just like being alone without your helper. He's been my helper for 55 years and now he can't help me."
DID YOU KNOW?
How the disease works
Creutzfeldt-Jakob disease affects about one person in 1 million. There are three different types of the disease, in which proteins in the brain grow abnormally and attack the tissue of the brain. Some of the early signs of the disease are balance problems or general confusion.
"The proteins are misfolding and the message is not being delivered," said Florence Kranitz, president of the Ohio-based CJD Foundation. "It could start out very subtly and it then it starts to have a rapid system decline."
Eventually the brain becomes so deteriorated that a person's body forgets how to chew, swallow or breathe.
"They most often sink into a coma," Kranitz said.
Creutzfeldt-Jakob Disease in Oklahoma
In 2003, CJD became a disease that health officials are required to track. In Oklahoma, a total of eight cases have been reported since 2003. The state reports incidents to the Centers for Disease Control and Prevention.
Cases reported by year
2003: 1
2004: 1
2005: 4
2006: 1
2007: 1
Source: Oklahoma State Health Department