Rare disease robs patients of language


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

Throughout her life, words have been Mary's 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 Mary's greatest pain.

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

Mary's 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," said Boyles. "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 Mary of the ability to find and speak words.

Mary's 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," said Mary.

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," said Dr. Daniel Kaufer, Director of the Memory Disorders Program at UNC Hospitals in Chapel Hill. "And someone like Mary 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 Mary 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," said Kaufer.

So Mary 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," said Boyles 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," said Boyles.

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

That's why Mary 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 go through it."

Doctors aren't sure when Mary will stop talking, but, they think it will be in the next few years. They also point out PPA isn't memory loss, or Alzheimer's. Patients like Mary 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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