Rare disease robs patients of language

February 27, 2008 10:17:15 PM PST
Mary Boyles is a retired english professor who must live with the reality of knowing she is suffering from a disease that is eventually going to make her mute.

"If I could just take off a leg or arm or both, that would be much better for me," Boyles said.

Throughout her life, words have been Boyles' love. She built her career around words as a retired UNC Pembroke professor with a PhD in American Literature. It took doctors almost four years to diagnose, but, in 2002, words became her greatest pain.

"It's just not, not," Boyles pauses to find the words. "It's not fair for me."

Her diagnosis is primary progressive aphasia, or PPA, a rare neurological disorder that's slowly robbing her of her language function. She also believes the same disease robbed her mother of the ability to speak.

"She stopped talking," Boyles said. "She just stopped one day. And, I know what it's going to do. That I can't talk but I can listen."

PPA is stripping Boyles of the ability to find and speak words.

Her husband, Bob explains the difficulty of his wife living with PPA saying, "It's probably the most devastating thing that could have happened to her. Because, starting from an early age, her success was built around her verbal ability."

"I just love words," Boyles said.

At this point, writing is easier than speaking for Mary. So, she journals her struggles.

PPA is a rare progressive disorder of language function. Doctors don't know what causes it, but brain imaging studies of patients, reveals shrinkage in the section of the brain devoted to language.

"Unlike most people with Alzheimer's disease who have very limited awareness there's a problem, individuals who have PPA are usually exquisitely aware they do have this problem with language," Dr. Daniel Kaufer, director of the Memory Disorders Program at UNC Hospitals in Chapel Hill said. "And someone like Boyles who has achieved such a high level of language skill, it particularly strikes her at the core of her intellectual strengths."

Dr. Kaufer treats Boyles and is trying to slow the effects of the disease by encouraging her to engage other parts of her brain.

"Like artistic activity [and] social activities," Kaufer said.

So Boyles has taken up drumming, meditating and drawing as therapy.

"They're trying to get that [side of my brain] really up because the problem is over here," Boyles said, pointing to the front of her head.

She says her anger at the situation actually improves her ability to find words.

"The madding, madder, madder I get, the better that I do it," Boyles said.

Knowing one day her speech will most likely disappear all together isn't an easy reality to deal with, but, Boyles' husband says they're planning for it. "We're working on different methods to enable her to communicate when the language does go," he said.

That's why Boyles is telling her story now; before she becomes mute. She's helping spread the word about this rare disease using her words now while she still can. Boyles offers this advice for others, "you can get through it."

Doctors are not sure when she will stop talking, but, they think it will be in the next few years. They also point out PPA is not memory loss, or Alzheimer's.

Patients like Boyles with PPA are perfectly healthy-able to see, hear, think and understand what is happening. But, they just can't get the words from the brain to the mouth. There is no cure for PPA.


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