THE EMERGENCE OF SYMPTOMS OF MILD PPA, AGE 66

The first time Ben M. notices a change in his speech is when his “mouth doesn’t cooperate” when he attempts to produce long words. In the earliest stages of PPA, pre-diagnosis, Ben has difficulty articulating more complex words (e.g. “presentation”). As these symptoms become more prominent, he seeks help from a neurologist, who diagnoses him with anxiety and prescribes anti-anxiety medication. Ben and his family notice an overall decline in his functioning and he decides to discontinue the medication. About a year later, his neurologist orders additional testing by an SLP, who suggests that Ben’s symptoms are consistent with progressive aphasia. The neurologist orders an MRI scan that shows degeneration on the left side of Ben’s brain, in areas that support speech and language. At this time, he receives a diagnosis of nonfluent/agrammatic variant primary progressive aphasia.

BEN’S COMMUNICATION

Although Ben knows exactly what he intends to say, he has difficulty producing longer words clearly and accurately. His wife and daughter are aware of this difficulty; however, they aren’t terribly concerned with the subtle changes in his communication because they have no trouble understanding him. Ben’s ability to read, write and understand are completely intact. However, he has noticed that he needs to spend a little extra time editing his emails, as some connecting words (e.g. “the,” “and”) are occasionally missing. He is still working as a business manager and has been able to meet the demands of his position by devoting increased time and effort to work-related communication.

Over the course of the next year, Ben begins to experience difficulty with “finding words,” and pauses frequently in the middle of sentences to formulate what he wishes to say. At this time, his wife and daughter understand that there is a broader problem with his communication. Ben begins to make deliberate modifications to his speech and language during conversation. For example, he chooses to say words that occur frequently and are shorter in length (e.g., “car” instead of “convertible”). Ben occasionally corrects words that are mispronounced, and sometimes requires several attempts to produce a word correctly. He begins to feel increasingly frustrated and anxious about his speech.

THE ROLES OF THE SLP AND THE CAREGIVER/ COMMUNICATION PARTNER IN TREATMENT

A primary role of Ben’s SLP in the early stage of PPA is to thoroughly evaluate his speech and language abilities. This evaluation will serve as a baseline for the progression of his communication difficulties and will help to identify goals for treatment. Ben’s evaluation confirms that his most prominent deficit in The primary goal at this stage is to improve his intelligibility when producing long words that are related to his job… communication is with the motor aspects of articulating words, with additional difficulties in grammar and word retrieval. The SLP works with Ben and his family to generate goals for treatment. The primary goal at this stage is to improve his intelligibility when producing long (multisyllabic) words that are related to his job. He is instructed to slow his speech rate and to produce words in a syllable-by-syllable fashion.

Another goal involves training Ben to use word-finding strategies, such as describing objects he can’t name or attempting to write part or all of a word he can’t retrieve. Lastly, Ben begins to explore the use of additional (nonverbal) modalities for communication, including writing, which he can use when his speech is misunderstood. Ben’s SLP suggests that he carry a card identifying and explaining his condition (see Online Resources, page 4) but he declines, as he is still insistent upon using spoken communication.

Ben’s wife begins to observe the speech-language treatment sessions that the SLP provides twice per week. The SLP and Mrs. M. create a home program to work on word-finding and articulation. In this way, Mrs. M. and the clinician work as a team to make the most of Ben’s limited insurance coverage for treatment.

PROGRESSION TO MODERATE PPA, AGE 68

BEN’S COMMUNICATION

Two years into his diagnosis, Ben’s speech production is more laborious and communication partners find him difficult to understand. Where previously, his difficulty in pronunciation was restricted to long words, now he has difficulty with initiating speech regardless of the word length. He uses only simple sentences and frequently omits grammatical words (such as “the”) from his speech. As a result, his wife and daughter often have a hard time understanding his message, and simple communication exchanges require multiple attempts.

At work, Ben’s superiors have noticed that his emails are very difficult to understand, due to missing and misspelled words. He is also unable to successfully give oral presentations, and he is experiencing increasing anxiety regarding his communication. Ben applies for and goes on disability leave, as he has become increasingly unable to complete the duties of his position. He begins to have some difficulty understanding what people are saying to him, especially when the message is complex, and he may ask his wife or daughter to repeat themselves. In addition to his communication problems, Ben begins having trouble coordinating movements with his right arm and hand.

THE ROLES OF THE SLP AND THE CAREGIVER/ COMMUNICATION PARTNER IN TREATMENT

Ben’s SLP implements a script training treatment, to facilitate f luent conversation about topics that he would like to discuss in his daily life. The speech pathologist works with Ben and his family to derive a set of scripts that will be functional for him, and he provides a majority of the content for the scripts. They are to include: a self-disclosure script regarding his PPA diagnosis, a script about his family and a script about football.

Ben practices his scripts with a video recording at home and works on using them in conversation during sessions with the clinician. He is gaining mastery of his scripts, and he and his wife report that he is using sentences and phrases from them in the community and with his family. He is encouraged to continue to use writing to supplement speech, however, this is becoming more challenging due to weakness in his right arm and hand. The SLP again suggests that Ben carry a card explaining his condition. After a routine traffic stop where Ben struggles to explain his speech difficulties, he agrees. The SLP …they also discuss strategies to maximize conversational success, such as providing Ben with increased t ime to formulate and express his thoughts… suggests that a communication book may be useful when verbal communication is especially taxing. Ben declines, as he remains determined to focus on spoken communication.

Mrs. M. plays an integral role in developing topics and content for each script. She and the SLP also discuss ways that she may encourage Ben to use nonverbal communication strategies, such as gestures and pointing to pictures. They also discuss strategies to maximize conversational success, such as providing Ben with increased time to formulate and express his thoughts, and simplifying rather than repeating information when he has difficulty comprehending.

MODERATELY-SEVERE PPA AND THE EMERGENCE OF NON-LANGUAGE SYMPTOMS, AGE 69

BEN’S COMMUNICATION

Ben is now communicating verbally through the use of single words, with occasional two-word utterances. These are often not understood by his wife and daughter, due to increasingly distorted speech. Phrases and words from his scripts do continue to be largely intelligible, and he relies on these practiced words to communicate verbally. Ben is using many gestures to communicate his wants and needs. In addition, he uses the writing of single words or phrases to contextualize any verbal or nonverbal (e.g. gestures, drawing pictures) communication.

He has less anxiety regarding his communication, as he, his wife and daughter speak to him in simple sentences. They also check for comprehension often.... becomes more proficient with and accepting of using modalities other than speech. Ben has difficulty understanding some messages, and his wife and daughter speak to him in simple sentences. They also check for comprehension often, and summarize the main points of their communication exchanges.

Ben continues to have increasing weakness and rigidity in his right hand and arm, and has difficulty coordinating his movements. It is recommended that the family consult with a doctor to discuss ways to minimize Ben’s risk of falling due to limb weakness and incoordination (see the Partners in FTD Care Summer 2015 issue, which includes information on falls prevention.)

THE ROLES OF THE SLP AND THE CAREGIVER/ COMMUNICATION PARTNER IN TREATMENT

As spoken communication becomes increasingly challenging, the SLP focuses on creating an augmentative and alternative communication (AAC) system. A low-tech communication book is chosen by Ben and his family. This book contains short phrases and questions paired with symbols (e.g., “May I have a drink?” with pictured choices of beverages). In addition, the book contains pictures of familiar people and places with written labels. Once the book has been completed, the clinician works with Ben on using it to ask and answer questions and to initiate communication exchanges. Ben then practices using the book with his family and friends.

The SLP also focuses on providing training for multi-modality communication for Ben and his family. This involves using gestures, writing and drawing pictures to convey his message dynamically to conversation partners. The goal of therapy at this stage is to foster communication through any modality, rather than to focus solely on verbal communication. Mrs. M. is trained to model multi-modality communication by using a variety of verbal and nonverbal communication strategies with Ben. Family and friends are encouraged to take an active role in communication exchanges in an attempt to increasingly shift the burden away from Ben. They work to simplify their messages and to give him plenty of time to communicate.

ADDITIONAL SYMPTOMS AND A TRANSITION IN LIVING, AGE 70

Ben begins to have greater difficulty coordinating his limbs, especially when walking. His short-term memory and cognition have shown some decline, and his motivation to communicate has diminished. Although his wife has modified the home environment to reduce his fall risk, Ben has fallen on several occasions.

In addition, Ben begins to cough when drinking liquids. He develops pneumonia and is taken to the hospital for treatment. He is diagnosed with dysphagia, a swallowing disorder (see the Partners in FTD Care Summer 2015 issue), subsequent to an evaluation with an SLP. Upon discharge, Ben returns home, where Mrs. M. prepares modified meals for him daily (e.g., thickened liquids and pureed solids).

When left alone, Ben frequently does not follow the modified diet. He develops pneumonia a second time and is again hospitalized. The level of care that he requires upon discharge is more than Mrs. M. and her daughter can manage. Together, they decide to move Ben into an assisted living facility, where nursing staff can help to manage Ben’s increasingly complex medical needs.

TRANSITIONAL SUPPORT PROVIDED BY SLP AND CAREGIVER/PRIMARY COMMUNICATION PARTNER

Ben’s SLP and Mrs. M. introduce the staff at the assisted living facility to the various communication tools that he is still able to utilize. For example, facility staff are oriented to Ben’s communication book and a new communication “wallet,” which he wears on a lanyard. The wallet contains functional pictures and words that he is likely to need during daily routines (e.g., pictures of foods, selfcare items and daily activities), as well as words that will be useful as fall prevention and dysphagia management practices are introduced.

The SLP also creates a communication board, which contains symbols for common objects in his room so that he is able to nonverbally communicate his wants and needs to the staff. The staff is familiarized with words and phrases that Ben is likely to use, including his practiced scripts, in order to provide them with a context for his communicative attempts. When possible, the staff uses words and ideas from his scripts when communicating with him, given that he appears to have somewhat preserved comprehension for this content.

Recordings of Ben’s scripts in his own voice (from his early days in speech therapy) have also been programmed into his iPad. He uses this vocal “bank” of scripts to communicate with the staff and other residents. He seems to enjoy being able to “speak” using his own voice to introduce these familiar topics. Occasionally, Ben feels frustrated by his limited communication and increasing physical needs and becomes aggressive toward staff or other residents. In these instances, staff are instructed to shift the interaction toward the use of nonverbal communication, when possible, and to emphasize feelings rather than words during interactions. Ben’s SLP provides an in-service to the staff, focusing on strategies for communicating with individuals with aphasia. These include: using a welcoming but adult tone of voice; simplifying the message (to single words and short phrases); allowing additional time; and using multi-modality communication. With these simple guidelines, caregivers in the facility are better equipped to communicate not only with Ben, but with other individuals in their care who have difficulty with communication as well.

Questions for Discussion
(Use for staff in-service training or in resident-specific situations.)

What communication strategies did the speech-language pathologist target with Ben as his aphasia progressed?

Mild PPA:

• The SLP worked with Ben to decrease his speech errors, focusing on a set of functional words that he used frequently when at work. He was instructed to speak slowly and produce these words syllable by syllable.
• The SLP also worked with him on strategies for finding words during instances of naming difficulty. Strategies included describing the thing he couldn’t name, and attempting to write part or all of the word.
• Ben was encouraged to use nonverbal modalities for communication when his speech was difficult for others to understand.

Moderate PPA

• The SLP implemented a script training treatment for topics that Ben reported he would like to discuss in his daily life.
• Ben was encouraged to continue to utilize writing and nonverbal modalities to supplement his verbal communication.

More Advanced PPA

• The strategies used in this stage focused on multimodality communication (gestures, writing and drawing). Ben’s family was instructed in these techniques as well, in order to “normalize” their use during conversation.
• The SLP worked with Ben and his family to develop a communication book containing words paired with pictures, in order to promote successful nonverbal communication.

What signs and symptoms indicated that a change in living situation was necessary?

Ben began to fall frequently and had increasing difficulty walking. In addition, he was hospitalized due to his trouble with swallowing, and his diet was subsequently modified. He did not consistently adhere to his dietary modifications and, as a result, he was again hospitalized. Ben’s wife and daughter decided that he would be safer and more comfortable in an assisted living facility.

How can communication partners (e.g., family members) and facility staff be trained to promote successful communication for individuals with primary progressive aphasia?

Communication strategies that can be implemented by communication partners and facility staff include:

• Using simple, short phrases with gestures or drawings to provide redundancy for the message.
• Providing increased time for the individual with PPA to convey their message.
• Modeling and promoting the use of multi-modality communication (e.g. drawing, gesturing, writing).
• Using a communication book or communication board to provide a context for comprehension and expression.

In Ben’s case, incorporating words and phrases from script training and encouraging him to use recorded videos of scripts to communicate about important topics were both productive.