Supporting a Loved One with Primary Progressive Aphasia

When my grandmother began showing symptoms of primary progressive aphasia (PPA) — a form of frontotemporal dementia — in the mid-2010s, it wasn't something we were familiar with. Our only point of reference was Terry Jones of Monty Python who was diagnosed with PPA in 2015 and died five years later. 

It's only been over the last year or two, with Bruce Willis and Wendy Williams' diagnoses, that most people have been familiar with it, but it's been part of my family's reality for almost a decade. 

Now there's more awareness of PPA, helped in part by these high-profile cases, but it's still not as well-known as, say, Alzheimer's. This can make getting a diagnosis tricky, as well as make it more difficult for us to support those affected. 

Most people who develop PPA are middle-aged, though patients can be younger or older. According to ​​Becky Khayum, a co-chair of the National Aphasia Association's PPA task force, "Life expectancy after development of symptoms can range from two to 20 years, with the average being seven to 10 years."

There are lots of different types of aphasia, with PPA being just one. Aphasia, where someone is unable to comprehend or formulate language, can be caused by a variety of reasons such as a stroke, head trauma, brain tumors and epilepsy — not only dementia. 

Within PPA, there are three separate types, all with different causes: semantic dementia, progressive non-fluent aphasia and logopenic aphasia.

Jonathan Rohrer, M.D., professor of neurology at the UCL Queen Square Institute of Neurology in London, England, explains, "Semantic variant PPA is usually associated with an abnormal protein called TDP-43, whereas for the non-fluent variant, it is a protein called tau. The logopenic variant is usually an 'atypical' presentation of Alzheimer's disease, meaning that there are amyloid plaques and tau tangles, similar to typical (memory-onset) Alzheimer's disease. Sometimes this is called the 'language variant' of Alzheimer's disease."

Generally, PPA isn't caused by genetics. There may be environmental factors, but researchers are still trying to work out causes. In less than 5% of people with PPA, it's caused by a genetic mutation, with the most common gene affected called progranulin.

In semantic dementia, people gradually lose knowledge of the meaning of words and struggle to know what everyday objects are used for. In progressive non-fluent aphasia, speech becomes more difficult — people know what they want to say, but can make grammatical errors, miss out words, and speak more slowly. In logopenic aphasia, people can often understand language, but can struggle to find the right words and say them correctly, as well as find understanding sentences more difficult. 

When a loved one gets diagnosed with PPA, lack of awareness can make things even more difficult – it's that sense of the unknown. And there are things researchers are still trying to figure out, too. 

"Questions remain about the speed at which changes occur in PPA," says Jeanne Gallée, a researcher at the Center for Psychometric Analyses of Aging and Neurodegeneration at the Department of Medicine at the University of Washington. "There's a lack of clarity about the changes a person may experience."

At first, PPA symptoms revolve around speech and language. But it's a progressive dementia, so behavior and memory will be affected down the line. Later, your loved one may become incontinent and require help with basic life activities like getting dressed.

Nancy Treaster, who's based in Georgia and is the co-founder of The Caregiver's Journey, explains that her husband's first symptoms revolved around struggling to find the right words, sometimes substituting or skipping over them.

He didn't know he was saying the wrong words, and thought he was having a normal conversation. "As it progressed to mid-stage," she continues, "he spoke mostly jibber jabber. He didn't seem to realize he wasn't making sense — only that we didn't understand what he was saying."

It was similar for Katherine Nichols, a writer and consultant based in California. "[My husband] was well-educated and a public speaker — super dynamic and charismatic. And he started having trouble getting words out right. And then from that, he was mixing up tenses and struggling with language, just switches in very basic grammar," she explains. From language, he then developed judgment-related symptoms.

"Communication is a fundamental feature of our social experience," Gallée says. "Changing communication patterns with friends and family is challenging and can be frustrating."

She recommends giving your loved one time to hear, process and respond — without any interruptions from you. Sometimes, they might want you to fill in gaps, but it can be easy to fall into the trap of doing this too much. 

When someone with PPA is struggling to understand language, break down what you say into shorter phrases, and give them time to process each one. They might also find writing or drawing on paper, gestures and images to be useful. Show them photos on your phone for example. 

"I don't know what my husband understands and what he doesn't understand," Treaster says. She'll tell him what she's doing as she does it, or tell him what she wants him to do and does it with him: "I don't know how much of that he understands but it's worth it to show him that respect."

Whatever you do, check in with your loved one to avoid misunderstandings, and practice the techniques you use early on, so it'll become easier later.

Rohrer advises seeing a speech pathologist, and Gallée suggests that communication partner training can be useful for couples affected.

Khayum advises speaking to a social worker, as well as attorneys and financial planners. Look into a lasting power of attorney, too, so you or another trusted loved one can act on the person's behalf when they no longer have the capacity to make decisions about things like finances and welfare. 

While PPA is rare, there is support, with groups and networks for people with PPA and their caregivers and loved ones. The National Aphasia Association has bi-monthly virtual Primary Progressive Aphasia Chats, too.

It's important caregivers take time for self-care, as it's easy to get burned out and overwhelmed as your loved one's condition progresses. Khayum recommends building a support network, or "village," as soon as you're able to.

As Treaster explains, "​​You can't be a good caregiver for your loved one if you aren't good yourself, mentally and physically." She recommends scheduling self-care, be it daily breaks or time away with friends. It's just as important as taking care of your loved one, even if you might feel selfish. 

Reaching out for support can be hard. "There's shame around it, which is one of the reasons why I'm trying to talk openly," Nichols says. "It's nobody's fault. It's an illness – the more honest we can be, the more support we can find."

She wishes she'd been upfront with people and asked outright for help she needed, whether it was watching over her husband while she ran errands or picking up groceries from the store.

Make sure you're prepared for PPA to progress, too. "Educate yourself and get support so you can be prepared to prevent disaster in your family," she says. When her father was diagnosed with Lewy body dementia some years later, she was able to better prepare herself simply because she knew more. 

It can be incredibly difficult when a loved one has a degenerative brain condition. Treaster explains her husband is now in the late stages and will only say a word or two when he speaks at all: "He may say "one, one, one" when he is trying to tell you something. Just random words he can retrieve."

While it can be bleak, you might still be able to find positive in it all. As Treaster says, "Every now and then he gives me a big smile and says, "I like, like, like" and I say 'I love you too'."

Adam England 
Adam England is a freelance health, lifestyle and culture journalist based in the United Kingdom. His writing has appeared in Verywell Mind, Healthline and Well+Good, among other publications. Read More