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Helping Them Hold On

2016; American Speech–Language–Hearing Association; Volume: 21; Issue: 10 Linguagem: Inglês

10.1044/leader.ftr1.21102016.44

1085-9586

Holly Hinshelwood, Maya L. Henry, Davida Fromm,

Dementia and Cognitive Impairment Research

You have accessThe ASHA LeaderFeature1 Oct 2016Helping Them Hold OnThrough phased treatment, speech-language pathologists can help clients with primary progressive aphasia function as normally as possible—for as long as they can. Holly Hinshelwood, MA, CF-SLP Maya Henry, andPhD, CCC-SLP Davida FrommPhD Holly Hinshelwood Google Scholar , MA, CF-SLP, Maya Henry Google Scholar , PhD, CCC-SLP and Davida Fromm Google Scholar , PhD https://doi.org/10.1044/leader.FTR1.21102016.44 SectionsAbout ToolsAdd to favorites ShareFacebookTwitterLinked In John noticed that something was amiss. For the past six months or so, the 58-year-old car salesman had been struggling to remember names for things and people, especially the names of things he didn't use often (such as "screwdriver"). "I know what I want to say, but I just can't say it," he told the neurologist he consulted for help. The neurologist referred him for further testing by a speech-language pathologist. Her evaluation showed that John's symptoms—language difficulty with no significant changes in other cognitive domains—were consistent with primary progressive aphasia (PPA). Meanwhile, the neurologist also ordered an MRI scan, which revealed degeneration on the left side of John's brain in areas that support language. He received a diagnosis consistent with the SLP's findings: logopenic variant PPA. John's diagnosis is one that many families and clinicians are unfamiliar with, despite recent improvements in diagnosis for this neurodegenerative disorder. What is PPA exactly? It's a language-based dementia, meaning a gradual deterioration in the ability to produce and comprehend language that results from the degeneration of speech and language networks in the brain. First reported in 1892, the disease wasn't formally recognized until 1982, when Marsel Mesulam described a series of patients with what he termed "primary progressive aphasia." Today, clinicians and researchers recognize three different variants: nonfluent, logopenic and semantic. As a relatively new diagnostic entity, PPA poses a unique challenge for SLPs—key players in both diagnosis and treatment of people with the disorder. Though relatively rare (PPA affects less than 200,000 in the U.S. population), the condition has been highlighted in recent news with, for example, celebrities like actress Kimberly Williams Paisley speaking out about their parents' experience with the disease. This clinical population continues to be relatively underserved, even though a growing body of research suggests that treatment can have significant and lasting benefits (see sources). The disorder may be less familiar to SLPs than aphasia secondary to a stroke. This unfamiliarity can be compounded by a sense of pessimism regarding the progressively negative prognosis of these clients. Consequently, when a patient like John is referred, many practicing SLPs have questions regarding the treatment and management of PPA, including best approaches to treatment and the unique needs of this patient population. These are questions worth asking, and clinicians can take heart: Despite the inevitable decline of these clients, there is much SLPs can do to improve, maintain, and support their communication and participation through a phased treatment approach. Unfamiliarity with primary progressive aphasia can be compounded by a sense of pessimism regarding the progressively negative prognosis of these clients. Phased treatment To date, there is significantly more research on treatment for aphasia caused by stroke than there is on PPA. However, many of the speech and language symptoms that characterize PPA are common to both patient groups. Correspondingly, most treatment approaches used in PPA are similar to those used in stroke-induced aphasia. However, clinicians also need to consider important differences when managing speech and language impairments in the context of progressive disease. In aphasia caused by stroke, clients show a static or gradually improving communication profile. But clients with PPA will eventually become mute or speak largely in jargon—using words that have some meaning to them but not to the listener. Ultimately, with the disease's progression, patients will show increasing cognitive, behavioral and motor deterioration. Given this trajectory, PPA treatment should address a client's current profile of spared and impaired abilities, while also addressing the client's inevitable decline in communication and, in later stages, other domains. Because of the evolving nature of PPA, we recommend a phased treatment approach, with recurring assessment and repeated treatment "doses" tailored to the client's current communication needs and challenges. As we follow John's case, we can see how this phased treatment approach allows his SLP to maximize his abilities while keeping future disease progression in mind. Clients with PPA will eventually become mute or speak largely in jargon—using words that have some meaning to them but not to the listener. Phase One—Restitutive treatment in mild PPA Although John knows what he intends to say, he has trouble retrieving the words he wants to use and needs to pause while speaking so he can formulate his thoughts. His friends know him as an articulate and precise speaker, but he finds himself needing to use more general terms for things (such as "car" for "convertible"). He also sometimes mispronounces words by substituting or adding sounds, such as producing "fising" for "fishing." His wife and 30-year-old daughter are aware of his difficulty but still understand him quite easily. He retains his ability to read, write and understand. However, in his sales job, he notices he more often misspells words in emails, requiring him to spend extra time on editing. He also finds it hard to "hold on" to information he hears, such as phone numbers and product numbers. This difficulty impedes his ability to process customer and inventory information. To meet the demands of his job, he has to devote increased time and effort to communication. A crucial part of the SLP's role during the early stages of PPA is to thoroughly evaluate and document the patient's communication. This initial evaluation serves as the baseline for the progression of difficulties and helps to identify targets for treatment. In early-stage PPA, the clinician often focuses on rebuilding impaired communicative abilities via restitutive treatments. Research shows that treatment during mild stages is critical for helping clients relearn lost skills, perhaps even slowing the rate of decline by promoting maintenance of communication abilities. Accordingly, John's SLP employs a lexical retrieval training protocol: John practices using different strategies (for example, he describes the object, attempts to write the word, or says the first sound) to recall words. To make treatment more functional, the clinician incorporates words related to John's job (terms related to cars and sales), hobbies (terms relating to fishing), and home life (terms related to household chores, items around the house, and so forth). John's SLP also encourages him to explore using additional, nonverbal modalities, such as drawing or gestures, for communication. This strategy serves two purposes: John can use these modalities when he is misunderstood—he can, for example, write the word after several unsuccessful attempts to clearly say it. Introducing nonverbal modalities serves as a foundation for later stages of PPA, when John will find spoken communication more challenging. Phase Two—Toward aided communication in moderate PPA Two years after his diagnosis, John now has difficulty retrieving uncommon words, such as "compass," and common words, such as "cup." His speech contains frequent phonemic paraphasias, or sound errors in words. His language is increasingly empty, as he uses generic terms and phrases, such as "stuff" and "that thing over there." As a result, others often have a hard time understanding his message, and simple communication exchanges require multiple attempts. At work, his emails have become impossible to understand, and he is unable to effectively interact with customers and vendors. Unable to complete his job duties, John goes on disability leave. At home, he struggles to follow what others say, especially when the message is complex and the speech rate is fast. He struggles with making decisions and completing numerical calculations. Re-evaluation of John's communication reveals moderate deficits in producing and comprehending language. Neuropsychological testing reveals the emergence of additional cognitive deficits, as brain degeneration affects his memory, problem-solving and number concepts, in addition to speech and language. Given the spoken language difficulties, treatment shifts toward multimodal communication and use of augmentative and alternative communication (AAC). John's SLP creates an AAC system to supplement his current verbal output and to prepare for future decline. John and his family choose to use a communication book containing pictures paired with written labels as an AAC tool. The SLP trains John and his family to use it to ask and answer questions and initiate communication exchanges. John's wife plays a growing role in treatment, including helping to develop and maintain the communication book for John. She helps select functional phrases and words to include, such as the types of food and drinks that John would most likely request, or conversational questions he can use. The SLP trains John's wife on strategies to maximize conversational success, such as providing extra time for John to compose and express his thoughts, and simplifying her speech to aid his comprehension. The SLP also provides counseling regarding PPA, as John and his family are ready to receive additional information about the disease and John's expected progression. Despite the inevitable decline of clients with PPA, there is much SLPs can do to improve, maintain and support their functioning. Phase Three—Environmental support and communication partner training in moderate-severe to severe PPA Three years later, John's verbal communication is mostly unintelligible jargon. In addition, his language comprehension is very poor. John uses gestures and his communication book to communicate wants and needs. His family speaks to him in simple sentences, using gestures to supplement and aid comprehension. As John's condition continues to decline, he exhibits widespread cognitive difficulties. In addition to communication impairments, he has pronounced deficits in memory, attention and visuospatial skills. Behavioral changes also emerge, and John periodically suffers from delusions and hallucinations. He also develops a prominent limb apraxia, and is often unable to coordinate his movements to perform learned movements voluntarily. John becomes more dependent on his wife for daily grooming and toileting tasks and other activities of daily living. John's family finds it increasingly difficult to manage his care, so they make the difficult decision to move him into an assisted living facility. There, nursing staff can attend to his increasingly complex needs. At this stage of PPA, treatment focuses on communication partner training and environmental modifications. The SLP continues to develop the communication book with John and his family, tailoring it to fit John's changing needs by including pictures of familiar people and places along with written labels. However, the SLP mostly focuses on training John's wife and family to use multimodal communication. They learn to use gestures, writing, drawing and simple phrases to convey messages to John. The SLP urges family and friends to lead communication exchanges to shift the burden from John. She instructs them to use touch, gestures, facial expression and other forms of nonverbal communication to supplement verbal exchanges. This strategy seems to lessen some of John's frustration with spoken communication and to reduce the frequency of behavioral outbursts, such as aggression toward others when not understood. As John transitions to an assisted living facility, the SLP introduces staff to the communication tools that John can still use, including his communication book. She provides a communication board that includes a daily visual schedule and pictures of common objects he uses and needs. She also trains staff on strategies for communicating with people with aphasia and dementia, as needed. This phased treatment approach allows clinicians to draw from their experience treating aphasia, while taking into account the inevitable decline associated with PPA. John's SLP used treatment approaches traditionally employed with patients with aphasia secondary to stroke, including lexical retrieval training and AAC. However, she tailored each treatment phase to John's current communication challenges, while anticipating strategies and communication tools needed in the future. Perhaps the most important lesson for clinicians is that they already possess many of the tools needed to maximize communication and quality of life for clients with PPA and their families. Health care professionals working with people with aphasia can find further information and resources at AphasiaAccess; the National Aphasia Association; and the ASHA Practice Portal. Many more aphasia-related resources are listed with the online version of this article. Choosing the Right Aphasia Treatment Software and Apps Finding apps and software that address what your clients with aphasia need can be daunting, given the dozens of programs available. The recently redesigned Aphasia Software Finder can help both you and them find the program that best fits their needs and devices. The free site was created seven years ago by the Tavistock Trust for Aphasia, which aims to help improve the quality of life for people with aphasia, their families and caregivers. Advances in technology and aphasia treatment since then prompted a redesign to make the website more user-friendly. The site includes information on aphasia-specific apps and software and also on "general" programs that are not designed specifically for people with aphasia, but can make life easier for some people who have communication difficulties. Users can search both categories by target activity, such as "activities of daily living" or "text to speech" in the general category, and "following instructions" or "talking" in the aphasia category. Information on each app or software package includes the number of exercises, platforms, customization options, price, available dialects (American and/or UK) and other characteristics. An additional information tab is designed for clinicians who want more details. For frequent visitors, a "what's new" tab shows all new and updated apps and software from the last 60 days. AphasiaBank Offers a Growing Research and Treatment Portfolio AphasiaBank is a repository of videotaped language samples, test results and demographic information from people with aphasia. The goal of the bank? To gather language data from adults with aphasia and make the information available to clinicians and researchers who are constructing methods for improving patient-oriented aphasia treatments. So far, AphasiaBank—a project led by Brian MacWhinney of Carnegie Mellon University and supported by a grant from the National Institute on Deafness and Other Communication Disorders—includes data from approximately 300 adults with aphasia and 200 adults without neurological impairment. Each participant performs the same set of language tasks and tests, so researchers can directly compare their language use. The language samples are transcribed in Codes for the Human Analysis of Transcripts (CHAT) format, which allows them to be analyzed automatically, efficiently and reliably by any of the 30-plus CLAN (Computerized Language Analysis) programs. These programs analyze grammar and tag parts of speech and morphemes for each spoken word. Some also measure rate and other aspects of speech. Brian MacWhinney describes CLAN in detail in his 2000 e-book "The Childes Project: Tools for Analyzing Talk". AphasiaBank aims to solidify the empirical database supporting the understanding of communication in aphasia, a critical step in the process of developing evidence-based treatments. More than 650 members from a variety of disciplines and countries belong to AphasiaBank. Why use AphasiaBank? The AphasiaBank website provides a range of clinical and educational resources. Membership is required to view password-protected participant data; other resources are available without membership. For example, the "Presentations and Posters" section links to conference talks and posters based on AphasiaBank data, as well as to a bibliography of published articles using AphasiaBank data or methods. Other links connect to information about aphasia, aphasia software, videos about aphasia, and technology use in aphasia treatment. The "Grand Rounds" link provides video samples and descriptions of classic aphasia types. It also provides guidance for discussing the language samples and poses questions about possible approaches to treatment. Through the "Browsable Database," users can view video samples in the AphasiaBank media collection, along with their corresponding transcripts. Users can search the browsable database on a variety of variables, such as aphasia type, aphasia severity, language task and aphasic errors. Educators can find resources developed by other educators for classroom activities, including transcribing, error coding, morphosyntactic analyses, acoustic analyses, case studies, assessment and treatment planning. Educators can also supervise their students' use of the database for independent research projects, theses and dissertations. Finally, and perhaps most important, users can create CHAT transcripts from their own patients' language samples and then tap CLAN programs to monitor changes in their clients' discourse and compare their clients with others in the AphasiaBank database. How can you contribute? To become a member of AphasiaBank, send an email request to Brian MacWhinney ([email protected]). Include contact information, professional affiliation and a brief explanation of why you want to join. Membership allows access to password-protected AphasiaBank data and links. Members are also invited to join the AphasiaBank Google group, a forum for discussing relevant topics. ASHA members are also urged to consider sharing their own data, such as group treatment videos and transcribed language samples with accompanying audio or video. Several presentations at the 2015 Research Symposium at the ASHA Convention focused on research into primary progressive aphasia, including: Advances in Diagnosis, Prognosis and Treatment; Clinical Features, Neuroimaging Correlates, and Underlying Pathology; and Connected Speech and Syntactic Impairment. Primary Progressive Aphasia Treatment Resources The Association for Frontotemporal Deterioration (AFTD) National Aphasia Association Dr. Lawrence Albert Memorial Webinar Series for Speech Language Pathologists Treating PPA Augmentative and Alternative Communication (AAC) for Persons with PPA Fresh Look at ACC and Dementia with Melanie Fried Oken ASHA Practice Portal: Aphasia Aphasia Research and Treatment Lab (ARTLab) at the University of Texas at Austin Aphasia Access (alliance of life-participation providers) Sources Croot K., Nickels L., Laurence F., & Manning M. (2009). Impairment- and activity/participation-directed interventions in progressive language impairment: Clinical and theoretical issues.Aphasiology, 23(2), 125–160. CrossrefGoogle Scholar Farrajota L., Maruta C., Maroco J., Martins I. P., Guerreiro M., & de Mendonça A. (2012). Speech therapy in primary progressive aphasia: A pilot study.Dementia and Geriatric Cognitive Disorders Extra, 2(1), 321–331. CrossrefGoogle Scholar Fried-Oken M., Mooney A., & Peters B. (2015). Supporting communication for patients with neurodegenerative disease.NeuroRehabilitation, 37(1), 69–87. CrossrefGoogle Scholar Fried-Oken M. (2008). Augmentative and alternative communication treatment for persons with primary progressive aphasia.Perspectives on Augmentative and Alternative Communication, 17, 99–104. ASHAWireGoogle Scholar Gorno-Tempini M. L., Brambati S. M., Ginex V., Ogar J., Dronkers N. F., Marcone A., … Miller B. L. (2008). The logopenic/phonological variant of primary progressive aphasia.Neurology, 71(16), 1227–1234. CrossrefGoogle Scholar Gorno-Tempini M. L., Hillis A. E., Weintraub S., Kertesz A., Mendez M., Cappa S. F., … Grossman M. (2011). Classification of primary progressive aphasia and its variants.Neurology, 76, 1006–1014. CrossrefGoogle Scholar Henry M. L., Rising K., DeMarco A. T., Miller B. L., Gorno-Tempini M. L., & Beeson P. M. (2013). Examining the value of lexical retrieval treatment in primary progressive aphasia: Two positive cases.Brain and Language, 127, 145–156. CrossrefGoogle Scholar Jokel R., Graham N. L., Rochon E., & Leonard C. (2014). Word retrieval therapies in primary progressive aphasia.Aphasiology, 28(8–9), 1038–1068. CrossrefGoogle Scholar Mesulam M. (1982). Slowly progressive aphasia without generalized dementia.Annuals of Neurology. 11(6), 592–598. CrossrefGoogle Scholar Taylor C., Kingma R. M., Croot K., & Nickels L. (2009). Speech pathology services for primary progressive aphasia: Exploring an emerging area of practice.Aphasiology, 23(2), 161–174. CrossrefGoogle Scholar Author Notes Holly Hinshelwood, MA, CF-SLP, is a clinical fellow at Wake Forest Baptist Medical Center in Winston Salem, North Carolina, where she works with adult patients with swallowing and communication disorders in the acute care setting. [email protected] Maya Henry, PhD, CCC-SLP, is an assistant professor in the Department of Communication Sciences and Disorders at the University of Texas, Austin. She also is the director of the Aphasia Research and Treatment Lab (ARTLab), which investigates the nature and treatment of aphasia caused by stroke and neurodegenerative disease. She is an affiliate of ASHA Special Interest Group 2, Neurophysiology and Neurogenic Speech and Language Disorders. [email protected] —Davida Fromm, PhD, is a research faculty member in the Department of Psychology at Carnegie Mellon University. [email protected] Advertising Disclaimer | Advertise With Us Advertising Disclaimer | Advertise With Us Additional Resources FiguresSourcesRelatedDetailsCited byAmerican Journal of Speech-Language Pathology32:2 (391-410)9 Mar 2023"Please Don't Assess Him to Destruction": The R.A.I.S.E. 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