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Shining A Light: Actress Kimberly Williams-Paisley wants to help other families dealing with primary progressive aphasia avoid the mistakes she and her family made in caring for her mother.

Bolster, Mary; Roberts-Grey, Gina

doi: 10.1097/01.NNN.0000503486.52508.e8
Features: Kimberly Williams-Paisley

Actress Kimberly Williams-Paisley wants to help other families dealing with primary progressive aphasia avoid the mistakes her family made in caring for her mother

WEB EXTRA: For advice on preparing for the future after a diagnosis of primary progressive aphasia, visit http://bit.ly/NN-PPAFuture.

The first signs were subtle and easily explained away, or recognizable only in hindsight. One in particular happened during Kimberly Williams' wedding to country singer Brad Paisley in 2003. During the modest service, Kim's mother, Linda, was supposed to read from the Epistle of Paul to the Colossians. From the start she fumbled, mispronouncing “Colossians” and stopping and starting several times during the passage—this from a mother who was usually quick-witted and outgoing, says Kim, an actress best known for her role as the daughter in the 1991 remake of Father of the Bride and a recurring spot on the sitcom According to Jim, which ran from 2001 to 2009.

Over the next two years, Linda struggled with other symptoms. She couldn't always find the right word, and reading became more difficult. Despite these problems, she thrived initially in her new position as director of development at the Michael J. Fox Foundation, where she taught the young staff how to improve their fundraising skills. Eventually, though, her deficits were impossible to ignore. When Linda confided in Kim that she was having trouble writing checks and signing her name, Kim assured her that it was likely stress-related or that she wasn't getting enough sleep. “My reaction was, ‘It's probably nothing,'” Kim recalls. “I'd say, ‘It's probably hormones.' I never thought it was something serious that would slowly take her from us.”

Other clues emerged during a few physical examinations, when Linda couldn't remember the vice president's name, for example, or count down from 100 by sevens. A year after starting at the Michael J. Fox Foundation, Linda asked to be reassigned to a position with fewer responsibilities and lower pay, and she and her husband, Gurney, began looking for answers in earnest.

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A SOBERING DIAGNOSIS

Brain scans revealed minor abnormalities such as cerebral atrophy, a loss of neurons and a weakening of the connections between them. Linda's neurologist scheduled more extensive testing of verbal and written language comprehension over a three-month period.

When the Williams and Paisley clans gathered at Kim and Brad's home in Tennessee for Christmas in 2005, Linda and Gurney sat down with Brad, Kim, and Kim's sister, Ashley, to share some difficult news. “That's when we learned the outcome of tests we didn't even know our mom had undergone and that she had primary progressive aphasia,” says Kim. “We also learned that she would need full-time care in five to seven years.” Kim and her siblings and Brad and his family were also asked to keep Linda's diagnosis a secret. Linda wasn't ready to share it with anyone outside the family.

Linda's decision to keep her diagnosis private and her reluctance to discuss her health and her prognosis had unexpected consequences for the Williams family—consequences Kim is still grappling with. In the short term, it meant covering for Linda's sometimes inappropriate responses in conversations by passing them off as jokes and coaching her on how to behave in day-to-day situations like grocery shopping. In the long term, it meant the family agonized over what kind of care was best for their mother.

“That was a very challenging stage because my mom had such pride for so long, and we so desperately wanted to preserve and protect that. But she also had a lack of awareness of how far her disease was progressing and how it affected her actions,” says Kim.

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A RARE FORM OF DEMENTIA

Primary progressive aphasia (PPA) is a type of dementia that affects areas of the brain responsible for speech and language, so people with the disorder often have trouble finding or understanding words, says Douglas W. Scharre, MD, director of the division of cognitive neurology at the Ohio State University Wexner Medical Center. Because the disease is progressive, communication problems worsen to the point where people can't speak, read, write, or understand language, says Dr. Scharre. Cognitive impairments such as trouble with memory or the ability to reason begin to develop, and the disease eventually progresses into full-blown dementia, he says.

As was the case with Linda, PPA is most often diagnosed after communication problems become too disruptive to ignore. It's not detectable on a magnetic resonance imaging (MRI) scan or a blood test, so neurologists must conduct a thorough neurologic examination and an evaluation of verbal and written language and comprehension, says Borna Bonakdarpour, MD, FAAN, assistant professor of neurology at Northwestern University Feinberg School of Medicine in Chicago, who specializes in PPA.

Over time, people with PPA lose the ability to understand language and say even the simplest words or phrases, says Dr. Scharre. They resort to shorter and shorter sentences. Instead of saying, “I'm hungry,” for example, they might simply say, “Food,” he explains. Likewise, they have trouble understanding language, so even the simplest phrases can seem foreign. As language comprehension diminishes, other cognitive abilities that govern visual perception, driving, walking, or being able to get dressed or eat independently also begin to decline, says Dr. Scharre.

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CAREGIVER CHALLENGES

The Williams children—Kim, Ashley, and their brother, Jay—were scattered among several states, while their parents lived in New York State. So the role of caring for Linda fell to Gurney, who struggled to keep the diagnosis secret and manage on his own. It soon became too much for him, and the family started to build a network of friends and volunteers to provide emotional support, as well as to help prepare meals or run errands.

The family also turned to resources like the Alzheimer's Association (http://alz.org) for education and information. About 40 percent of PPA cases are caused by Alzheimer's disease and about 60 percent are caused by frontotemporal lobar degeneration (FTLD), says Dr. Bonakdarpour. People whose PPA is caused by FTLD may exhibit behavioral problems and troubles with executive function, which governs problem solving, reasoning, working memory, and planning, that are typically seen in frontotemporal dementia, he says.

More and more, Kim wished her family had dealt with Linda's condition more aggressively and had had frank conversations from the beginning, before it was too late. “I regret not asking my mother about her long-term care preferences and wishes when she had the chance to convey them,” she says. It would have been helpful to know if and when her mother wanted to move to a long-term care facility and to what extent she wanted her husband and children to be her primary caregivers, she says.

Because they didn't know, the family shouldered the burden for far too long. As she writes in her memoir about the family's experience, Where the Light Gets In (Crown Archetype, 2016), her mother became aggressive and hostile toward Gurney. She also insisted on continuing to drive, even after several accidents. As Linda's condition worsened, she was becoming increasingly difficult to manage. She required care beyond what her family could provide. But driven by guilt, indecision, and uncertainty, the family, particularly Gurney, stumbled on.

It was a situation Kim thought could have been avoided if the family had had a conversation with Linda before the disease robbed her of insight and judgment. “It's important for families to emphasize and vocalize the need for members to not try to do all the caregiving on their own. It's important for caregivers to reach out and ask for help,” she says.

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EDUCATING OTHERS

A diagnosis of PPA can be an opportunity to inform others, says Dr. Bonakdarpour. “At some point, it's important for families to educate those around them,” he says. “In return, they may get some help from friends and family.” Every awkward encounter is a chance to destigmatize the disease. When someone acts inappropriately in a public place, the situation may escalate if others don't realize the person has PPA, says Dr. Bonakdarpour. “[The general public] needs education. If they knew about it, they would behave differently.” That's why Kim's book is so important, he says. “This is the first time someone is writing about PPA. Now the public will know more about this disease.”

Like the Williams family, many relatives of people with PPA feel guilty or frustrated, says Dr. Bonakdarpour. “You're always dealing with guilt. We constantly reassure family members when they are doing a great job. Other people may judge families for putting their loved one into an assisted living facility or for carrying on with their lives instead of devoting all their time to caring for their relative, but this is a very difficult disease.”

Other people may avoid families who are dealing with PPA, he adds. “They simply don't know how to interact due to lack of knowledge or awareness about the disease.” He also notes that when someone with PPA has a supportive family like Linda's, he or she generally fares better. “I think the Williams family have done a wonderful job,” he says, based on Kim's memoir.

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FINDING A NEW MOM

For the first few years of her mother's illness, Kim yearned for the fun, charming, vocal person her mother had once been—a yearning that prevented her from engaging fully with the mom she now had. As Linda's disease progressed and the family moved her to an assisted living facility, Kim worked hard to let go of the “old mom,” she says. Through much soul-searching, she concluded that her mother was offering her an opportunity to love unconditionally and, as she wrote in her memoir, “to practice being comfortable with what is uncomfortable. To grieve and also embrace what is broken. To know that some days I can receive who my mother is now and some days I struggle with it.”

A song by Leonard Cohen, which became a comforting mantra and informed the title of her book, summed up her new perspective: “Ring the bells that still can ring/Forget your perfect offering/There is a crack in everything/That's how the light gets in.”

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LIFE GOES ON

Kim thought her book would end with the death of her mother, but Linda is still alive. She sleeps a lot and speaks little, but she still reveals glimpses of her old sense of humor, still perks up when family members visit. And she's still inspiring Kim, teaching her new lessons and reminding her to embrace life fully and completely.

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Advice for Families

Dealing with a family member who has primary progressive aphasia is challenging, but certain tactics can help.

A diagnosis of primary progressive aphasia (PPA) may seem hopeless and can cause patients and families to despair, but experts say much can be done to soften the journey and improve everyone's quality of life. “The focus in the beginning is to improve the patient's cognitive reserve and ability to concentrate,” says Borna Bonakdarpour, MD, FAAN, assistant professor of neurology at Northwestern University Feinberg School of Medicine, who has been researching PPA since 1998.

Initially, Dr. Bonakdarpour connects families with a speech pathologist and a team of social workers. He also spends a great deal of time educating families about the disease and directing them to resources such as the Alzheimer's Association (http://alz.org) and the Association for Frontotemporal Degeneration (http://theaftd.org), since about 60 percent of PPA patients are ultimately diagnosed with frontotemporal dementia. He also identifies and treats underlying conditions such as depression and anxiety and teaches patients how to communicate their anger when the disease gets worse.

To help patients cope with aphasia, Dr. Bonakdarpour recommends music therapy and other expressive arts. “Music therapy is good for people with aphasia. They can participate on varying levels. In fact, aphasia patients often can sing much better than they can speak.” The goal is to focus on abilities the patient still has to make him or her feel better. “If people have or develop a passion for painting, I encourage them to do more of it,” he says. “It's a helpful channel for expression.”

Dr. Bonakdarpour also encourages patients and their families to consider participating in clinical trials. With more research, physicians may be able to stop the disease before it starts, he says. Until that time, Dr. Bonakdarpour urges families to speak out about the disease. “If more people are educated about PPA and react helpfully, there is no benefit to hiding it,” he says.

He believes books such as Where the Light Gets In (Crown Archetype, 2016), actress Kimberly Williams-Paisley's memoir of her family's experience with her mother's PPA, are vital. “People can learn from her story,” he says. “It's a great resource for people to see how things are and how families deal with them, even if imperfectly. We are all imperfect.”

© 2016 American Academy of Neurology