I wish I had this ALS guide for speech-language pathologists (SLPs) five years ago! ALS may be a rare disease, but I almost always have at least one person on my caseload.
I’ve learned a lot, and I’m happy to share. I know I have more to learn, so please share what you do as well!
Read on to learn the basics of ALS, find useful resources, and see how I approach assessment and treatment.
Free DIRECT downloads: Communication Strategies (patient handout) and the ALS Clinical Checklist (cheat sheet). (Email subscribers, access in the Free Subscription Library.)
Outline:
- Learn about ALS.
- Understand best practices.
- The emerging role for exercise.
- The SLP’s ongoing role in ALS management.
- Swallow assessment.
- Dysphagia intervention.
- Communication assessment.
- AAC and communication intervention.
- Cognitive-communication assessment.
- Treatment for cognitive-communication impairment.
- Walk in with a plan.
- Related Eat, Speak, & Think posts.
- Share your experience!
Learn about ALS
Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease, is a devastating progressive disease. ALS affects both upper motor neurons (UMN) in the brain and lower motor neurons (LMN) in the spinal cord.
Motor neurons die and muscles weaken
According to the ALS Association, “amyotrophic” literally means “no muscle nourishment”. As the motor neurons in the central and peripheral nervous system die, muscles weaken.
As the disease progresses, a person loses the ability to talk, swallow, move, and breathe. Cognitive impairment affects 20% – 50% of people with ALS.
Rare, and more often seen among military veterans
ALS is “the most common adult-onset motor neuron disease“. People are usually diagnosed between 40 and 70 years of age. For some reason, military veterans are twice as likely to be diagnosed with ALS than the general public. My youngest patient was 26 years old.
Even so, ALS is a rare disease. There are roughly 25,000 people in the United States affected by ALS, with 5,000 people being diagnosed every year.
ALS can advance rapidly or slowly
ALS begins with symptoms affecting either the arms and legs (limb-onset ALS) or speech and swallowing (bulbar-onset ALS). People with bulbar onset ALS often experience a faster progression of the disease and a shorter life expectancy.
Prognosis is poor
Prognosis is poor, with the median survival roughly three years from onset of symptoms and one year from diagnosis. Most patients die from respiratory failure between two and five years after the onset of symptoms.
Sometimes, ALS can lead to death from respiratory complications very quickly. One person I worked with passed away very shortly after diagnosis and after only one week on my caseload.
On the other hand, people with ALS can live a long time. Although a rare case, Stephen Hawking lived for more than 50 years with ALS.
One person I’ve worked with off and on over the past four years is pretty stable. When I first met him four years ago, he was non-verbal, wasn’t eating or drinking by mouth but had a feeding tube, and was wheelchair bound. At that time, I set him up with a speech-generating device (SGD) accessed through eye-gaze.
This year, I went back to teach partner-assisted scanning to him and his family as a back-up communication method. He didn’t look like he had changed at all. He was still smiling, making jokes, and communicating through the SGD via eye-gaze.
Regardless of how long a person may have, we do our best to provide our patients and their families with the skills and knowledge they need to help improve their daily experience of life.
The SLP may be the first to suspect ALS
If the disease begins by affecting the bulbar region of the brain, then early symptoms will involve speech and/or swallowing. It’s quite possible that the SLP may be the first person to suspect a neurological disorder in these cases.
Just one more reason for a thorough history and cranial nerve exam! Cranial nerve exam refreshers are easily found via online search. You can also check out this open-access Otolaryngology paper “Cranial nerve examination for neurogenic dysphagia patients.”
Understand best practices
It’s important to keep current with research and guidelines regarding the management of ALS.
Just as we wouldn’t want to be treated by a physician who practices the same way today as they did 10 years ago, we also shouldn’t be practicing the same way year in and year out.
Having said this, no one can possibly keep up with every advance in every area of our profession. This is why it’s so important to know where to look for guidance and support. For instance, we can connect with more experienced colleagues and access resources from ASHA and other organizations.
Connect with more experienced colleagues
- Your area’s ALS clinic or movement disorders clinic.
- The local chapter of the ALS Association.
- Your local VA Medical Center.
- State AT program.
- ASHA’s Special Interest Groups:
- AAC SGD vendors such as Tobii Dynavox, LC Technologies, and PRC.
- Clinical Facebook groups focusing on dysphagia or AAC.
ASHA resources
We also have access to a wealth of research and guidelines online from ASHA and other sources. For instance:
- ALS Evidence Map.
- Journal of Speech, Language, and Hearing Research.
- American Journal of Speech-Language Pathology.
- Dysarthria practice portal.
- Resonance practice portal.
- Dysphagia practice portal.
- Summary of “Provisional Best Practices Guidelines for the Evaluation of Bulbar Dysfunction in Amytrophic Lateral Sclerosis”.
Other resources
Clinical Measures of Bulbar Dysfunction in ALS (Yunusova et al, 2019) offers helpful information for assessment. You’ll find:
- Cranial nerve exam findings consistent with UMN and LMN degeneration.
- Dysarthria findings associated with UMN and LMN degeneration.
- Information about different assessment tools.
Other online helpful resources include:
- Quality Measures for the management of ALS (American Academy of Neurology, re-affirmed in 2015).
- AAC-RERC provides clinical pathways for communication and swallowing in ALS.
- ALS Association research news – includes blog, newsletter, and free webinars.
The emerging role of exercise in ALS
Emily Plowman, PhD, CCC-SLP, and colleagues are studying how exercise may be neuro-protective for people with ALS. Research has shown some benefit of exercise for the limbs in ALS. Dr. Plowman and her team are researching expiratory-muscle strength training (EMST) to see if people can improve cough, swallowing, and quality of life.
I’ll provide a brief synopsis of Dr. Plowman’s two studies below.
2015 study participants
In 2015, Dr. Plowman and colleagues published a study of 25 people with ALS who participated in a 5-week EMST exercise program (15 completed the study). People were excluded if they had cognitive impairment, trach or vent, a diaphragmatic pacer, or an active respiratory disease.
The EMST exercise protocol
The handheld EMST exercise device was set to 50% of each person’s Maximum Expiratory Pressure (MEP). The participant completed the exercise program five days a week for five weeks. Each exercise sessions consisted of five sets of five breaths through the device. A therapist recalibrated the device each week to maintain an exercise load of 50% of MEP.
Results were promising
Of the 19 participants who actually began the EMST exercise program, four dropped out (one moved to another state). Of the 15 who completed the study, none reported pain, fatigue, discomfort, or other adverse events. The people who dropped out tended to be more severely affected by ALS. It’s unknown if they dropped out due to difficulty tolerating the exercise or weekly travel to the clinic or some other reason.
Overall, participants demonstrated “immediate gains in expiratory force and greater hyoid displacement during swallowing.” Theoretically, these gains should improve cough effectiveness and airway protection.
Follow-up double-blind, randomized, controlled EMST study
Dr. Plowman and colleagues conducted a larger double-blind, randomized, controlled trial of 48 people with ALS. Participants had early-stage ALS and completed the same EMST exercise program described above. Half completed the exercise program and half completed a sham exercise program.
Participants did not have to travel to the clinic every week; instead a research therapist conducted the first session each week in their home. The research therapist was not blinded to treatment group.
The participants tolerated the exercise program well. Only one participant from each group withdrew from the study. One due to a new diagnosis of cancer, and the other did not wish to participate any longer.
The people in the active treatment group demonstrated significantly higher MEP at the end of the study compared to the sham treatment group.
The people in the active treatment group maintained their DIGEST scores, while the sham treatment group worsened.
Moderate EMST appears beneficial in early-stage ALS
Based on this research, I discuss EMST with my patients who have mild symptoms. If they’re interested, I contact their neurologist for verbal approval before providing my patient with purchasing information.
I’ve had perhaps two or three people with ALS go ahead with EMST over the past three years. All of their physicians agreed with the moderate exercise. None of them completed the entire protocol as described above, but all of them felt it helped their breathing.
The SLP’s ongoing role in ALS management
SLPs can and should play a large role in helping people with ALS and their families to improve and maintain participation in their lives as best as possible for as long as possible.
As the disease progresses and abilities decline, most patients benefit from skilled intervention to make adjustments. It’s likely that our patients will be off and on caseload as time passes.
People can only absorb so much information at a time. And many people need some time to process and adjust to what’s happening to them before they’re open to learning about what the future may hold for them.
There’s no predicting exactly how the disease will affect their swallowing, communication, and cognition. So we can’t give advice and education in one session or one episode of care and expect that to suffice.
Swallowing and communication are basic activities that also play huge roles in people’s identity and pleasure in life. The skill of the SLP is to provide education and training as needs and goals change in order to help our patients have the best day possible.
Swallow assessment
Check out ASHA’s adult dysphagia assessment summary on the practice portal. For your assessments, you may consider, for example:
- Medical history and interview.
- ALS Severity Scale (Hillel et al; PDFs available via online search).
- EAT-10.
- Functional Oral Intake Scale.
- Oral mechanism exam / cranial nerve exam.
- Yale 3 ounce water test.
- Clinical swallow evaluation.
- Review or request instrumental swallow study.
One important piece of information to ask about is whether your patient has decided to get a feeding tube. Their physician almost certainly will have already discussed this with them.
Dysphagia intervention
Read ASHA’s statement regarding treatment considerations related to progressive disorders and end-of-life issues.
- Swallow strategies.
- Food modification.
- Liquid modification.
- Energy conservation.
- High-calorie choices.
- The importance of oral care for reducing aspiration pneumonia risk.
- If symptoms are mild, ask if patient is interested in an EMST program.
Communication assessment
Check out ASHA’s summaries of adult dysarthria assessment and voice assessment. To assess communication, you may choose, for example:
- Interview.
- Oral mechanism exam / cranial nerve exam.
- Assess voice.
- Assess speech.
- Observations from interview.
- ALS Severity Scale (Hillel et al; PDFs available via online search ).
- Frenchay Dysarthria Assessment – Second Edition.
- Grandfather or Rainbow passage (easily found on search).
- Assess language.
- Observations from interview.
- Picture narrative subtest of the BDAE-3 (Cookie Theft).
- Formal assessment if suspect problems.
AAC and communication intervention
Communication strategies
It’s surprising how difficult it can be for people to realize they have communication habits that may have worked fine in the past, but cause problems now. For instance, many people try to have a conversation between rooms with the TV on. They get frustrated when the other person doesn’t hear them, thinking they’re not paying attention.
Here are the communication strategies that I teach:
- Gain the other person’s attention before talking.
- Talk face-to-face in good lighting.
- Turn off background noise.
- Check for comprehension.
- If the other person doesn’t understand something, try one of these:
- Say it again a little louder.
- Point to the first letter on an alphabet board and say it again.
- Write it down.
- Point to it, if it’s visible.
- Make a gesture to show what it is.
- Say it another way.
- Take a break.
- Talk about important things when you feel rested.
Tip for clinicians, family, and friends
If you don’t understand something, don’t simply say “What?”. This forces the person to repeat the entire sentence. Instead, repeat what you understood and ask them to fill in the blank(s).
For instance:
- Person with ALS: Can you pick up some mshwrk?
- Spouse: Can I pick up some what?
- Person with ALS: Milk.
Voice amplification
Only a few of my patients have chosen to use voice amplification. They all used the ChatterVox. One device was provided as a free loan from the ALS Association. Another was purchased by the VA. And the third one was a free loan from a local non-profit agency.
If these sources aren’t available to your patient and funding assistance is required, Team Gleason accepts applications for personal assistance.
Low-tech/mid-tech AAC
From my experience, people want to communicate the easiest way possible. I’ve had patients who’ve gotten an SGD early, before they really had to rely on it.
I always strongly encourage people to start using it, so that they learn how to use it more efficiently and can make adjustments while they still have another way to communicate.
But without fail, outside of my sessions, every single one of them put it on the shelf until they really had to use it.
So I always provide or recommend low-tech and mid-tech AAC options.
Low-tech materials examples:
- Pain scale and body outline.
- Alphabet board and number board.
- Printed list of frequent requests and statements.
Mid-tech AAC includes AAC apps, such as:
PrAACtical AAC is a great source of information for finding appropriate AAC apps. For instance, you can learn how to match the features of the device to the needs and abilities of your patient. And here’s a list of 133 free and lite versions of AAC apps.
Partner-assisted scanning
Partner-assisted scanning is one of the best strategies I’ve learned to help people communicate better. In my experience, this is very easy to teach patients and their families and friends. It’s an easy way to communicate, and everyone I’ve taught it to has used it regularly.
Download John Costello’s free materials, including the AEIOU alphabet board, pain board, Yes-No board, and a tabbed flip-book template.
Message banking and/or voice banking
I discuss message banking and voice banking with all of my patients who have ALS. I explain the pros and cons of each, and how I can assist with the entire process.
You can read more in my message banking tutorial and my voice banking tutorial. I’ve also written about the Voice Keeper app, an easy way to do voice banking.
Eye-gaze access for SGD
I always discuss the likelihood that a person may have to rely on eye-gaze selection with an SGD to communicate. Most of my patients are open to trying it out. So I arrange for an SGD vendor representative to meet me in my patient’s home to trial a device.
If my patient is open to going ahead with the process of getting an SGD, I’ll trial at least one other device before choosing one. You can read more about how to do AAC evaluations in the home.
Sometimes my patient isn’t ready to look into communication devices. In this case, I make sure they understand that they can ask for another speech therapy evaluation at any point. I also make sure they understand that getting a device can easily take a month or two, so I recommend starting the process when they notice changes to their ability to communicate.
Cognitive-communication assessment
Given that 20% – 50% of people who have ALS also have a cognitive impairment, it’s helpful to assess cognitive-communication skills. It’s also required for the AAC evaluation report to request funding from insurers.
You can read ASHA’s technical report on assessing and treating cognitive-communication disorders in adults. I base my choice of evaluation tool on the presenting complaints, if any. If my patient and their family deny any concerns or problems, then I administer something like the Neuro-QoL Cognitive Function Short Form and the Short Blessed Test.
An SLP from a multidisciplinary clinic just told me about the following two cognitive-behavioral screening tools. I plan to try them out.
- ALS Cognitive Behavioral Screen (ALS-CBS). (Developed by Susan Woolley, PhD.)
- Edinburgh Cognitive and Behavioural ALS Screen (ECAS).
Treatment for cognitive-communication impairment
I develop treatment goals in partnership with my patient and their family. So far, I haven’t worked with anyone who had ALS who was concerned about their cognitive skills.
I’ve had a few patients who had a mild impairment for temporal orientation and/or short term memory. Even so, they had no trouble learning how to use the AAC methods and devices.
However, many people with ALS do experience troublesome cognitive changes.
Walk in with a plan
When I meet with a new patient who has ALS, I walk in with a plan which may cover the entire episode of care:
- Listen to their concerns, problems, and goals.
- Discuss how I can help them.
- Assess swallowing.
- Recommend an instrumental swallow test.
- Teach swallow recommendations.
- Discuss EMST if appropriate (and get MD approval).
- Assess communication and cognitive-communication skills.
- Teach communication strategies.
- Address cognition if it’s a concern.
- Teach low/mid-tech AAC and partner-assisted scanning.
- Discuss and facilitate message banking and/or voice banking.
- Set up eye-gaze SGD trials.
- Teach the use of the eye-gaze SGD after it’s obtained.
- Be sure my patient and family have a back-up form of communication (ex. AAC boards + partner-assisted scanning).
I only address what they’re interested in, but I do make sure that they know what I can offer, which I leave in writing as well. I make sure they understand that they can ask their doctor for another ST consult whenever they’d like.
Related Eat, Speak, & Think posts
- How to do AAC evaluations in the home.
- Message banking tutorial.
- Voice banking tutorial: Acapela and ModelTalker.
- Easy voice banking with The Voice Keeper.
- Take the guesswork out of thickening liquids with the IDDSI flow test.
- A practical guide to pureed food at home.
Share your experience!
Do you do anything differently than what I’ve described above? I’m sure at least some of you must! I know there’s always more to learn, so please share your experience working with people who have ALS.
Free DIRECT downloads: Communication Strategies (patient handout) and the ALS Clinical Checklist (cheat sheet). (Email subscribers, access in the Free Subscription Library.)
Featured image by Ricardo IV Tamayo on Unsplash.
Lisa earned her M.A. in Speech-Language Pathology from the University of Maryland, College Park and her M.A. in Linguistics from the University of California, San Diego.
She participated in research studies with the National Institute on Deafness and other Communication Disorders (NIDCD) and the University of Maryland in the areas of aphasia, Parkinson’s Disease, epilepsy, and fluency disorders.
Lisa has been working as a medical speech-language pathologist since 2008. She has a strong passion for evidence-based assessment and therapy, having earned five ASHA Awards for Professional Participation in Continuing Education.
She launched EatSpeakThink.com in June 2018 to help other clinicians be more successful working in home health, as well as to provide strategies and resources to people living with problems eating, speaking, or thinking.
Hello,
I am looking for research to either support or disprove the use of reading aloud in helping with ALS and speech longevity. I am trying to see if there is any scientific merit to the all-to-common ALS phrase “the more you use the faster you lose.” Do you know of any recent research or researchers I could maybe talk to about this
Best Regards
Sarah
EverythingALS
Hi Sarah,
Great question! I’m not aware of any research that looked into reading aloud as an exercise to maintain speech in people who have ALS. As far as the saying “the more you use, the faster you lose” goes, the research I mentioned in the article above indicates that does not seem to be true. For instance, moderate exercise for respiratory muscles in people who have are in the earlier stages of ALS improved or maintained cough and swallowing. Other research has shown that exercise for the limbs is neuroprotective (check out the research to find out how intensive the exercise was and for what stage). If you belong to ASHA’s SIG2, that would probably be a great place for your question. I hope that’s helpful.
I’m an SLP and have a client , for the first time, with ALS. How do you determine mild, moderate and severe?
Hi Adelaide, great question. If you’re asking how to determine if the ALS overall is mild, moderate, or severe, there is the ALS Severity Score (Hillel).
This paper was brilliant for me, as a lay person researching for a speech and language trainee therapist in Morocco. Thank you so much for taking the time and putting in so much effort into the potential of so much current research to follow-up.
Well done, you are a shining star!
Florence Longhorn
Thank you so much for your generous feedback, Flo!
Lisa, I just want to say that your site is beyond helpful -it is perfect for aiding the clinician in their profession to give each patient their very best treatment – as I was reading through you site, I came across you mentioning some “sticky colored dots” that you can put on a TV remote for a patient. I am trying to locate those. Can you direct me back to that link?
I currently have an ALS patient- although I have practiced for 32 yrs- I am not experienced with ALS- your site fully set my up for success.
Again, no words for how helpful your site is. Thank you for all the time you have put into it.
Hi Julie, my apologies for such a delayed response. You can find the sticky dots by searching “tactile sticky dots for low vision”, such as this product on Amazon: https://amzn.to/4bRw2re (That is an affiliate link, EST may earn a small commission with no extra charge to you.)
Thanks so much for your kind words, and again, my deepest apologies for such a delayed response!
Thank you so much for the helpful information, Lisa!
I having been searching for hours but have been unable to find the ALS-CBS. I wonder if others are having the same experience.
Thanks for letting me know the link to the ALS-CBS no longer works. I just spent 30 minutes looking for it, with no luck. I did find the copyright holder and will email Dr Woolley momentarily to see where we can access it. https://www.sutterhealth.org/research/investigators/susan-woolley
Dr Woolley’s email address on that bio doesn’t work. I found her on Linked In, but I can’t message her without jumping through a lot of hoops (already jumped through multiple hoops, but I’m drawing the line at uploading my drivers license since I don’t use Linked In). If you have a Linked In account, you could message her and ask where you can find the ALS-CBS. I did send a message to the HR department at her current place of employment (if her Linked In bio is up to date). If you discover where to find the tool, please let us know!