Primary progressive aphasia. Now what?
May 10, 2011 8:09 PM   Subscribe

My mother was recently diagnosed with primary progressive aphasia. What can we expect?

My mom, who is in her late 50s, has been suffering with some increasingly severe cognitive dysfunction for the better part of the last decade. Recently, she was diagnosed with primary progressive aphasia. This is something of a shock since we were all expecting it to be Alzheimer's. What can we expect from this? Does anyone out there have some firsthand experience with what the next several years are going to look like?

In a nutshell, mom current state sucks. She has lost her ability to function. She recognizes her kids and grandkids, but has a hard time connecting that the person we call "dad" is the same guy she calls "Bob" (and lives with- he is her primary caregiver). She can't do any the things that she has done for decades: cleaning house, laundry, driving, etc. She needs help to get dressed. She can't figure out how to change the channel on the TV, so she watches whatever channel dad leaves it on. That is the sum of her day, from what I can gather: TV. Reading is so intensely tedious that she does it as little as possible. I am not certain that she eats if dad (or someone else) isn't around. Even so, she is physically healthy and still jogs with dad most days. She doesn't talk much, and is difficult to communicate with. There is definitely no small talk. She can't follow simple requests without help every step of the way (Mom, would you please get me a glass of water? Got me a blank stare until I told her where to get a glass, and what to do next). It is really hard to not think she is pulling one over on us because sometimes the stuff she does is just so ridiculous (I know she isn't). Mom is (understandably) depressed and on a couple different anti-depressants. Losing abilites like mom has must be so isolating.

What happens next? What would make this easier for dad? Us kids provide regular respite care and house cleaning, etc, but he is a self sufficient guy of the "I can do it my damn self" type.

Finally, how did this start from mom's perspective? By the time we all figured out what was going on (for years the diagnosis was "cognitive dysfunction due to depression/anxiety") it was too late to ask her. Since this appears to be a genetic thing, how can mom's kids recognize the symptoms in ourselves or each other before it gets so bad?

Yanmd. Anon because this is not my story to tell.
posted by anonymous to Health & Fitness (5 answers total) 3 users marked this as a favorite
Aphasia is a specific medical term which means the inability to find and use words.

Primary progressive aphasia is a rare form of dementia in which the hallmark is word-finding difficulty in speech which becomes worsened over time, and extends to other difficulties with speech, reading, and writing. It typically does not involve difficulties with situational memory or recognizing people or doing daily activities, at least for the first couple of years...

Therefore, it sounds like your mother either has a rare presentation of a rare form of dementia, or has another kind of dementia along with the primary progressive aphasia part, or she had aphasia during the 'cognitive dysfunction' years you referred to and now has a much more severe case?

The story is a bit confusing but one thing I can answer for you is that in primary progressive aphasia, the initial symptom to look for would be difficulty finding words or remembering what things are called/named.
posted by treehorn+bunny at 8:42 PM on May 10, 2011

Yeah, usually the disease progression starts with aphasia, and then picks up with cognitive decline, similar to that of Alzheimer Disease (although the brain pathology is different).
posted by gaspode at 8:52 PM on May 10, 2011

I am so sorry for you and for your family. We just lost a very close family friend this year to PPA. In her case it initially presented as looking like a very-treatment resistant depression and was misdiagnosed as such for well over a year. She presented with a flat affect (emotional presentation) and was less talkative than she had been in the past. Gradually she had more specific trouble finding words, and that was when the diagnosis was made. She started to have troubles with daily activities, like your mom, but in her case seemed to recognize those close to her until the end. That is very variable from person to person and one of the hard things about the disease. She died at home this year surrounded by family and friends.

Unfortunately it is progressive, and relentlessly so and affects people much earlier than other types of dementia. You and your family will need support. Ask her treating neurologist if they can point you to a social worker who can help with the things you'll need to manage. Respite care, financial aspects, etc. Hospice was eventually a huge help to this family.

Here are some resources that may be helpful to you: National Aphasia Association, Northwestern University, PPA support group. Also, if you'd like to me-mail I'd be happy to try to help however I can.
posted by goggie at 4:16 AM on May 11, 2011

I missed a few of your questions before...

PPA is one of the "fronto-temporal" dementias, meaning that it affects the frontal lobes and temporal lobe before affecting other parts of the brain. The temporal region of the brain is involved with language, and as it is affected by PPA means that the people affected begin to first have trouble finding words to express themselves, and gradually lose the ability to understand speech easily as well. Reading is one of the functions that can be affected earlier in the disease and may explain why your mother is not interested in doing so now. The frontal lobes are involved in "executive functions" or the skills you need to accomplish a task. This can include planning, organizing, regulating your emotions, working memory and other related abilities. This is why individuals with PPA may have trouble carrying out daily tasks in that they have trouble planning and organizing the steps they need to get the job done (this is likely why she is having trouble doing things like operating the TV). The resources I listed above may give you some more specific information about this kind of thing. This can also be why individuals with PPA look "flat" or depressed, there are neurological things interfering with emotional regulation and motivation. Of course there is also depression and anxiety so it can be tough to untangle.

I've worked with a few patients with PPA, but usually a little later in the course. As far as our friend, she definitely recognized something was wrong early on, but like everyone else thought it was depression. When she was diagnosed, she was able to understand the diagnosis and information about what to expect. Her family was very involved in her care, with a large network of friends as well. Our friend was able to go out and enjoy lunches with friends and even a brief vacation with her daughters in the months after the diagnosis. She did not initiate these things, but enjoyed being part of the activity. As the disease progressed, she often preferred to spend quieter time when larger events were planned. So, for example, if there was going to be a large family function she would sometimes elect to attend an adult day care program instead. As the disease progressed, she became less aware of what was happening in terms of the disease progressing, but did not appear distressed or anxious. That can vary so it's imortant to be prepeared with a team who can help if those symptoms start to emerge.

For your dad, chronic and progressive illnesses are always enormous strains for primary caregivers. I would really encourage him and your family to work with folks who can provide support. Some of the links I provided above have links to online support groups and other resources that can help with planning and understanding what may be happening next. If there is extended family or a network of friends, starting to ask for concrete help now may be helpful (e.g., making a run to the grocery store, sitting with your mom while your dad does other things around the house, providing some respite care from time to time if you're comfortable). I would also really encourage your family to locate an adult care day center or respite provider to help with these things, as that may feel like less of a "burden" to your dad. Insurance may cover some of this and working with a social worker will help navigate some of those issues.

As far as the genetic risk, we're still figuring that all out. There is one gene that has been identified as being a cause in some cases, but I'm not sure if they recommend testing for family members. In other cases, it's not clear what the cause is. I would recommend speaking to her neurologist and asking about whether further genetic testing or counseling would be appropriate for your family. The chances are that this will not affect you or your siblings in the future, but you would want to be aware of changes simliar to what you saw with your mom...depression, apathy, language difficulties. IANAD IANYD but again, this is most likely a remote risk.

I wish the absolute best for your mom, you, and your family on this journey.
posted by goggie at 8:12 AM on May 11, 2011

If you or your family have iPhones, you can download some apps which help succeeded of aphasia communicate. Check out the app store and search for aphasia.
posted by Effigy2000 at 1:59 PM on May 12, 2011

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