Most people have not heard about Primary Progressive Aphasia, nor do they know the term until someone in their family or a friend acquires PPA. Aphasia is defined as the loss of language from an injury to the brain due to a stroke, trauma, tumor, or infection. Primary Progressive Aphasia (PPA), on the other hand, is a progressive disorder of loss of language that is neither injury nor stroke related. It’s an impairment of language, affecting the production or comprehension of speech and the ability to read or write. It is a neurodegenerative disorder similar to Alzheimer’s, caused by a progressive degeneration of brain tissue. Unlike Alzheimer’s and other types of dementia, the brain degeneration related to PPA is specific to areas of the left hemisphere that control function related to speech in language. Often cognition remains intact, making the role of a caregiver unique and often difficult to navigate.
A Personal Story
Joanne says that she gets about 40 good consecutive minutes of talking in per day. After this window her words begin to fail her, as if she has a quota for the day. “I can tell that my speech is changing over time. It’s so exhausting to speak now. I put a lot of energy into being able to compensate for the losses. It sometimes takes a very long time to pull the right words forward through my brain.” Joanne says she’s constantly monitoring her speech, both forward and backward in time. She plans out what she’s going to say in advance, as much as possible. Then after she’s said it, she immediately looks back to see if her speech has made sense, and corrects if necessary. This forward-backward monitoring is time-consuming and wearying.
She says that as her speech deteriorates over the course of the day – and in a larger sense over time – there’s a clear pattern to it. “At first, I can choose the right words fairly well, but as I continue to talk, I realize I’m using suboptimal words. Then, if I continue speaking instead of recharging, I’ll choose a completely inadequate word, and then finally I’ll notice that I will choose absolutely the wrong word. I can see that pattern worsening over time.”
The Progression of PPA
The condition can be so severe as to make communication with the patient almost impossible. Or it can be very mild. It may affect a single aspect of language use, such as the ability to retrieve the names of objects, or the ability to put words together into sentences, or the ability to read. More commonly, however, it affects multiple aspects of communication. What is important for caregivers to keep in mind is the wide range of severity that aphasia encompasses. Some people are able to understand others with almost no problems while their speaking is most affected. Others experience the reverse scenario of interpreting speech being difficult while verbalization comes very easily. And, as PPA progresses, symptoms grow more and more difficult, eventually resulting in mutism.
Caring for Someone at Home With PPA
Caring for a loved one with Primary Progressive Aphasia typically begins at home. Upon diagnosis, and often earlier, a spouse, an adult child or even an aging parent, takes on the role of caregiver. Because the disease will progress, caregivers must plan ahead and periodically reassess how best to provide care for their loved one and meet their own needs over the course of the illness. The symptoms evolve and change over time with the only thing that is known for sure is that patients will need more assistance to maintain their quality of life.
First and foremost, a caregiver must show patience in allowing the processing time needed by those affected with aphasia when communicating. People are often compelled to “fill in the blank” for the person who is having trouble completing a thought. Also, caregivers must not be quick to reword what they are saying when the aphasic person is having trouble understanding. Often times changing the wording only complicates things further.
Unfortunately, anyone who cares for a person with a degenerative disease like PPA knows that a time will come when they are unable to provide the level of care their loved one needs. The realization that a loved one can no longer stay in the home is full of guilt and may even feel like a failure to the caregiver. The demands of providing care at home for someone with PPA are enormous and respite care should be considered. With or without in-home caregivers, a patient with PPA will come to require the 24-hour care that can be best provided in an assisted living, skilled nursing, or inpatient medical facility.
As Joanne relates, “My life is not the way I would have chosen, but I can choose what I can make of it now. Not everyone is at this exact point where I am; there may not always be strategies to intervene. But for others, there are. And earlier diagnosis means that we can have more for the future, and get the most from the time we do have.”
If you or someone you know is affected by PPA or other neurodegenerative diseases, Home Care Assist, provides a safe resource for finding compatible in-home care providers and respite workers.