blog post by Betts Peters PhD. CCC-SLP
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease affecting the motor neurons that carry signals between the brain, spinal cord, and muscles. Often this includes the neurons and muscles involved in speech and respiration, so many people with ALS experience speech or voice changes and can benefit from the use of AAC. I am a speech-language pathologist and AAC specialist, and I have worked with people with ALS for many years, first as a clinician with the ALS Association and the VA and now as a researcher. For my PhD dissertation project, I wanted to learn more about the methods people with ALS use for communication, their experiences with intervention and education related to AAC and communication, and how they participate in everyday situations involving communication.
In April through June of 2021, I conducted a nationwide online survey of people with ALS about these topics, and 216 people responded and provided information related to one or more research questions. Respondents included people living in at least 41 states, with ages ranging from 34 to 84 (mean 64.4). They had been living with ALS for up to 37 years (mean 3.9 years since diagnosis), and their scores on a self-administered version of the ALS Functional Rating Scale-Revised (ALSFRS-R, Bakker et al., 2020) indicated that their level of physical disability ranged from minimal to severe. Most reported experiencing dysarthria, with 71.3% of respondents indicating at least some detectable speech disturbance (ALSFRS-R speech rating ≤ 3), and 56.5% reduced intelligibility (ALSFRS-R speech rating ≤ 2). Some of the major findings related to communication methods, service delivery experiences, and communicative participation are summarized below.
What methods do people with ALS use for communication?
One goal of the study was to learn about how people with ALS communicate, and how they use communication-related tools and devices. Overall, 49.5% of respondents reported using some form of aided communication in face-to-face interactions. Speech was the most frequently-used method of communication for respondents with better speech function (ALSFRS-R speech ratings 4, 3, or 2). Respondents who were combining speech and non-speech methods (ALSFRS-R speech rating 1) used their speech less frequently, and reported more use of gestures, writing, and speech-generating devices (SGDs), primarily smartphones with AAC apps. Among respondents who were no longer able to speak (ALSFRS-R speech rating 0), a smaller percentage used gestures, writing, and smartphones than those at speech rating 1, while a higher percentage used purpose-built SGDs or consumer tablets with AAC software. Use of purpose-built SGDs was most common among people with ALS who used alternative access methods such as eye tracking. Among respondents who could not meet all of their communication needs using speech (ALSFRS-R speech rating 1 or 0), 84.6% used unaided methods (residual speech and/or gestures) and 90.5% used one or more SGDs, but only 52.3% used low-tech AAC methods. More respondents reported communicating via video call platforms such as Zoom or FaceTime than via telephone. This was especially true for respondents who could not meet all of their communication needs using speech (ALSFRS-R speech rating 1 or 0), who indicated that they used gestures, typing, or an SGD instead of or in addition to speech during video calls.
What kind of support do people with ALS and their families receive related to communication and AAC?
Respondents also answered questions about their experiences with AAC service delivery, i.e. intervention and education related to speech changes and strategies and devices to support communication. Most respondents reported that they had met with a speech-language pathologist to talk about these topics. However, many respondents said they did not get help from a speech-language pathologist when learning or setting up their non-speech communication methods (46.0% of respondents who used non-speech methods), or when adapting their methods due to changing needs (62.2% of respondents who required modification of their AAC methods). Fewer than half of respondents indicated that their family members or other important communication partners had received education or support related to potential communication changes associated with ALS or how to respond to those changes and support effective communication. Respondents who had met with a speech-language pathologist were significantly more likely to own an SGD, to have heard of message and voice banking, and to say that someone had talked with their family members or other important people about ALS-related speech changes and communication options.
How do speech changes and AAC use affect communicative participation for people with ALS?
Another goal was to learn more about how people with ALS rate their ease or difficulty with participation in everyday situations involving communication, and how AAC use might affect that participation. This part of the questionnaire was adapted from the Communicative Participation Item Bank (CPIB, Baylor et al., 2013), which had been used previously with people with ALS who communicate using speech (Yorkston, Baylor, & Mach, 2017; Börjesson, Hartelius, & Laakso, 2021), but not with those who use AAC. Respondents answered questions about how difficult it was for them to participate in ten different situations, such as “talking with people you know” or “getting your turn in a fast-moving conversation.” Individuals who indicated they used aided communication methods were asked to rate their difficulty with participation under two conditions: 1) using unaided communication (speech and/or gestures) only and 2) with access to all of their communication methods. Scores from eight of the items were added together to calculate a total CPIB score indicating overall ease or difficulty with communicative participation.
The figure below illustrates the relationship between ALSFRS-R speech rating (from 4, no speech change, to 0, unable to speak) and total CPIB score (higher scores indicate easier communicative participation), as well as the results of nonparametric nonlinear kernel-based regression analysis of that relationship. CPIB scores for participants who do not use aided communication are represented by purple markers, while the “unaided only” and “all methods” scores for those who do use aided communication are represented by lighter and darker green markers, respectively. In general, respondents with better speech function reported easier participation in communication situations. Respondents who used aided methods had significantly higher CPIB scores (indicating easier communicative participation) under the “all methods” condition than the “unaided communication only” condition. Respondents had more and more difficulty with communicative participation with lower levels of speech function, up to the point of needing to use non-speech methods in addition to speech (ALSFRS-R speech rating 1). However, as long as they had access to all of their communication methods, some respondents who reached the point of being unable to speak (ALSFRS-R Speech rating 0) actually found it easier to participate in many situations than those who were still using both speech and non-speech methods; this is represented by the “ski jump” look of the regression line for the “all methods” condition. It is possible that communicative participation is easier for these respondents because they had been using their non-speech methods longer. They may also have changed their expectations for communication, or have a different understanding of what makes communication “difficult.”
Limitations
Although survey respondents were diverse in some ways (e.g. age, time since diagnosis, speech and physical function, and state of residence), people with ALS from racial and ethnic minority groups appear to be underrepresented. The sample also included a larger proportion of individuals with postgraduate degrees than the general population. Most respondents were frequent computer users before their ALS diagnosis, and all had internet access and an email address (or the assistance of a family member or other support person with those resources). Individuals with less access to or familiarity with technology may have been less likely to participate, and may have had different responses. PALS with more severe physical impairments may also be underrepresented due to difficulties with computer control or fatigue. Considering these characteristics of the survey sample, generalizability of the results may be limited.
Conclusion
Results highlight the diverse experiences of individuals living with ALS and related changes in communication needs and strategies. People with ALS use a variety of communication methods, with different methods being more commonly used at different levels of speech impairment. Aided communication methods do appear to support communicative participation for people with ALS, especially those with more severe speech impairments. There is room for improvement in AAC intervention for people with ALS, which should involve early referral and ongoing treatment, education and support for communication partners, and intervention that addresses the varied and changing communication needs of each individual. Detailed results from this study are described in the author’s dissertation (Peters, 2022), and will be published in peer-reviewed journals.
References
Bakker, L. A., Schröder, C. D., Tan, H. H., Vugts, S. M., van Eijk, R. P., van Es, M. A., … & van den Berg, L. H. (2020). Development and assessment of the inter-rater and intra-rater reproducibility of a self-administration version of the ALSFRS-R. Journal of Neurology, Neurosurgery & Psychiatry, 91(1), 75-81.
Baylor, C., Yorkston, K., Eadie, T., Kim, J., Chung, H., & Amtmanna, D. (2013). The Communicative Participation Item Bank (CPIB): Item Bank Calibration and Development of a Disorder-Generic Short Form. Journal of Speech, Language, and Hearing Research, 56, 1190-1208.
Börjesson, M. S., Hartelius, L., & Laakso, K. (2021). Communicative participation in people with amyotrophic lateral sclerosis. Folia Phoniatrica et Logopaedica, 73(2), 101-108.
Peters, B. (2022). Augmentative and alternative communication use, service delivery experiences, and communicative participation for people with amyotrophic lateral sclerosis (Dissertation No. 29212757) [Doctoral dissertation, Portland State University]. ProQuest Dissertations Publishing.Yorkston, K., Baylor, C., & Mach, H. (2017). Factors associated with communicative participation in amyotrophic lateral sclerosis. Journal of Speech, Language, and Hearing Research, 60(6S), 1791-1797.
About the Author
Betts Peters PhD. CCC-SLP, is a researcher, speech-language pathologist, and assistive technology specialist, and conducts brain-computer interface (BCI) research as part of the REKNEW lab at Oregon Health & Science University (OHSU) and the Consortium for Accessible Multimodal Brain-Body Interfaces (CAMBI). She also works with the OHSU Neurology and Neurosurgery departments to provide clinical care for patients undergoing awake craniotomy procedures. Her research focuses on improving the assistive technologies and related systems available to individuals with severe speech and physical impairments. She previously worked as an assistive technology specialist for The ALS Association Oregon & SW Washington Chapter and the Portland VA Medical Center.
Thank you for this very important article.
I especially think the sentence “There is room for improvement in AAC intervention for people with ALS, which should involve early referral and ongoing treatment, education and support for communication partners, and intervention that addresses the varied and changing communication needs of each individual” should be heard by everyone. Early and ongoing access to information, meaningful supports and appropriate technologies is needed. How can we do a better job increasing awareness and advocating for all individuals who ALS?
Last year, my 68-year-old partner was diagnosed with Lou Gehrig’s disease also known as ALS. Speaking and swallowing were two of his challenges. His collapse was swift and catastrophic, and neither the riluzole nor the medical staff did much to aid him. He would not have survived if our primary care physician hadn’t given him attentive care and attention, as the hospital center didn’t provide any psychological support. His fall was abrupt and catastrophic. His hands and legs gave way to weakness in his arms. This year our family physician suggested using vine health centre . co m ALS/MND treatment, which my husband has been receiving for a few months now. I’m delighted to say that the treatment greatly reduced and reversed his symptoms of ALS, he no longer requires a feeding tube, sleeps soundly, works out frequently, and is now very active. In the hopes that it could be useful, I thought I would relate my husband’s tale; in the end, you have to do what suits you the best.