Care Partner Training: Resources and Guidance for Managing Primary Progressive Aphasia

Dementia is not only a disease that affects older people. Patients as young as 15 have been diagnosed with Frontal Temporal Degeneration (FTD), although it more typically affects people in their late 40s, 50s and 60s.

Will Reiter, education program manager for the Association of Frontal Temporal Degeneration, described FTD as “a complex cluster of disorders” in a recent program presented by Insight Memory Care in collaboration with AARP Virginia.

FTD is a “younger-onset type of dementia,” Reiter said. Alzheimer's disease, on the other hand, typically has an onset in patients over age 65.

Another key difference between FTD and Alzheimer’s are the areas of the brain affected, with Alzheimer’s affecting the hippocampus—located deep in the brain-- causing memory deterioration. Degeneration of the frontal lobes, which affect reasoning, impulse control, and executive function, is what results in an FTD diagnosis.

“FTD disorders are not well understood in the general community,” said Reiter. There are about 60,000 FTD patients in the United States, but the exact number can be hard to determine, depending on when a patient is diagnosed.

Unlike Alzheimer’s, which can often be genetic, about 60% of FTD patients have no genetic causation. Reiter said 40% have a history of neurogenerative disease—which are chronic conditions that damage and destroy parts of the nervous system, particularly the brain--and 20% are believed to have a genetic predisposition.

Symptoms of FTD include communication issues, especially speaking and understanding language. Behavioral problems and personality changes are also common. Memory issues typically are not an issue, especially early in the diagnosis.

“Usually, changes in a person’s neurodegenerative condition detected by family result in bringing the person in for diagnosis,” said Reiter.

Aphasia is a descriptive term for impairment with language or communication. It can be caused by a variety of factors, including stroke, head injury, or brain tumor.

Primary Progressive Aphasia (PPA) is part of the spectrum associated with Frontal Temporal Degeneration.

“Primary Progressive Aphasia is problems with communications brought out by the neurogenerative degeneration process that takes place over time,” Reiter said.

Since FTD is a degeneration of the frontal lobes, which affect speech and verbal memory, PPA is often seen in FTD patients, although some forms can also be present with Alzheimer’s patients.

A diagnosis of PPA helps create a plan of care, said Reiter. There are three primary types of PPA.

• In the nonfluent/agrammatic type, the patient tends to speak like they are sending a telegram, as opposed to complete sentences. For example, said Reiter, a patient may look at a picture of a family walking by a lake and describe it as “man woman children walking lake.”  Articulation becomes more difficult, and the patient may have trouble swallowing. They may also have trouble with movement, especially on the right side of the body.
  
• With semantic PPA, the person has difficulty recalling names of objects and has comprehension issues. Over time, they lose the knowledge of what words mean and what objects are. They experience a decline in empathy or awareness of other people’s emotions.  
  
• Logopenic PPA is difficulty finding the right words when speaking and difficulty repeating phrases, and often includes memory issues. This type is more likely to be found in Alzheimer’s patients rather than those with FTD. 

Reiter strongly recommends the use of a speech language pathologist (SLP) to develop a personalized plan of care and address issues between the patient and others. “It’s surprising how often PPA patients don’t get referred” to a SLP, said Reiter.

In the early stages of PPA, the focus is on creating an environment where there is better communication and understanding. A SLP can help the patient retrain, learn, and grow by maximizing communication through tools.

Care partners can help by reducing background noise and distractions, using face-to-face communication as much as possible, speaking slowly and allowing time for a response, and asking for clarification.

“Patience is key,” said Reiter.

During the middle stage of PPA, a speech language pathologist can help develop scripts to help conversations on items of interest or favorite activities.

Communication becomes more challenging during the middle phase. Reiter recommends asking closed questions, such as “Do you want pizza or pasta for dinner,” as opposed to asking what they want to eat.

He also recommends letting the patient take the lead on communicating and using affirming statements like “take your time, I can wait.”

Communication becomes much more limited during the late stage of PPA. Care partners can use gestures, writing single words, drawing, or a picture board to help communicate. A pathologist can help if the patient has trouble swallowing. Palliative care can help with the patient’s comfort and quality of life.

If the care partner sees new behavioral or movement challenges with the patient at any stage, Reiter advises the care partner to seek a neurological or neuropsychological assessment.

Although there is currently more research than ever on FTD, there are no Federal Drug Administration approved medications for symptoms or disease modifying treatments, said Reiter.

The Association for Frontal Temporal Degeneration offers research, awareness, support, education, and advocacy for those affected by FTD and their care partners.

AFTD holds an annual conference, both in person and virtually. Information and recordings from previous conference, as well as many helpful resources, is available on the AFTD website.