Peole with ALS present the greatest challenge regarding communication issues. Approximately 75% of all people diagnosed with ALS will need some form of communication assistance. (1) While progression of speech disturbance varies in each person with ALS, most people will experience a severe communication disorder during the last few months of life. In a retrospective study of 100 hospice patients with ALS, 28% were anarthric (unable to speak) and 47% were severely dysarthric (slurred speech) at the time of their deaths. Only 25% could speak understandably during the terminal stage of the illness.(2)
Following is a guide to help you better understand the needs, as well as options for communicating.
Individuals with ALS will often present with dysarthria which is defined by Hedge as “ a group of motor speech disorders resulting from disturbed muscular control of the speech mechanism due to damage of the peripheral or central nervous system; oral communication problems due to weakness, incoordination, or paralysis of speech musculature; physiologic characteristics include abnormal or disturbed strength, speed, range, steadiness, tone, and accuracy of muscle movements; communication characteristics include disturbed pitch, loudness, voice quality, resonance, respiratory support for speech, prosody and articulation.” The act of speaking is an extremely fine motor activity. Therefore, when the muscles that allow us to articulate are weakened, uncoordinated, or rigid speech can sound slurred. ALS patients present with spastic, flaccid or a mixed spastic-flaccid dysarthria. (3)
1. Use of voice amplification
1. No Technology
a. Establish a yes/no/maybe system. Always have a “maybe” because the way the question is phrased may be incorrect or needs clarification.
2. Low Tech
a. Writing via use of pen and paper, whiteboard, MagnaDoodles, etc.
b. Letterboard or Picture Board, which is alphabet board or personal patient picture board (depending on patient’s cognitive status). These boards can be used with the patient pointing to the appropriate letter or if the patient does not have hand function, the listener using “partner assisted scanning”. The speaker does not need hand function to utilize a letterboard. (16), (17), (18), (19), (20)
c. Chart based encoding
d. Laser Pointer in combination with a letterboard
3. High Tech
a. Use of Smartphones: Combining voice output apps (example Speak It, Small for Aphasia, Locabulary, Proloquo2Go) that are available for the iPhone, Blackberry, and Droid phones. This access is only available for those that have hand and finger function. (26), (27)
b. Personal Computers with voice output software
c. Speech Generating Devices (SGD)
d. Electronic Eye Gaze
D. Locked In Syndrome
a. Two different variations that may affect a person with ALS
i. Locked-in syndrome (LIS), also know as ventral pontine syndrome, refers to severl motor impairment in which an individual is conscious but is quadriplegic. Voluntary movement is limited to eye movements or perhaps eye blinks. (35)
1. Those with LIS may be able to use a letter or picture board via partner assisted scanning, use a communication device with a switch, or use an electronic eye gaze system
ii. Total Locked-in syndrome
1. Total locked-in syndrome is characterized by tetraplegia, anarthria and paralysis of eye motility (36)
a. There are no commercially available devices that will assist a person with TLS with communication
1. Saunders C, Walsh T, Smith M. Hospice Care in the Motor Neuron Diseases. London: Edward Arnold, 1981.
2. Saunders C, Walsh T, Smith M. Hospice Care in the Motor Neuron Diseases. London: Edward Arnold, 1981.
3. Brownlee A, Palovcak M, The Role of Augmentative Communication Devices in the Medical Management of ALS. Journal of NeuroRehabilitation, Volume 22, Number 2, 2007, 445-447.
4. Luterman D, Counseling Person with Communication Disorders and Their Families, Fifth Edition, Austin Texas, pro-ed Publishing, 51
5. Gray D, Quatrano L, Lieberman M, Designing and Using Assistive Technology-The Human Perspective, Baltimore, MD: Paul H. Brooks 1998, 100.
6. Luterman D, Counseling Person with Communication Disorders and Their Families, Fifth Edition, Austin Texas, pro-ed Publishing, 52
7. Brownlee A, Palovcak M, The Role of Augmentative Communication Devices in the Medical Management of ALS. Journal of NeuroRehabilitation, Volume 22, Number 2, 2007, 449.
8, 9.and 10. Luterman D, Counseling Person with Communication Disorders and Their Families, Fifth Edition, Austin Texas, pro-ed Publishing, 52
11. Brownlee A, Palovcak M, The Role of Augmentative Communication Devices in the Medical Management of ALS. Journal of NeuroRehabilitation, Volume 22, Number 2, 2007, 449.
12. Cook A, Hussey S. Augmentative and alternative communication systems. Assistive Technologies, Principles and Practice. Second Edition ed. St. Louis: Mosby, 2002, 285-286, 287.
13. Beukelman D, Mirenda P. Introduction. In: Augmentative and Alternative Communication, Management of Severe Communication Disorders in Children and Adults. Second ed. Baltimore, MD: Paul H. Brooks, 1998, 4-5.
14. Kazandjian M. Communication intervention. First ed. San Diego, CA: Singular, 1997, 15-16; 17; 18; 19; 20; 21; 22.
15. Beukelman D, Garrett K, Yorkson K, Augmentative Communication Strategies for Adults with Acute or Chronic Medical Conditions, First ed., Paul H. Brooks, Baltimore, MD, 2007, 299-300.
16. Examples of letterboards
17. The National ALS Association offers a free kit of letterboards to any interested hospice. Contact Alisa Brownlee at email@example.com to receive a kit.
18. Pre-made letterboards are available from AliMed at http://www.alimed.com/Alimed/product/The-Critical-Communicator,14192,339.html. These boards come in a variety of languages including English, French, Spanish, German, Italian, Arabic, Korean, Greek, Chinese, Hindi, Polish, Indonesian, Farsi, Hebrew, Turkish, Romanian, Vietnamese, Hungarian, Hmong/Laotian, Russian, Japanese, Tagalog/Filipino.
19. You Tube Videos on Use of Letterboards
2. Partner assisted scanning with a letterboard: http://www.youtube.com/watch?v=pLb6-Oi3uR0
20. Communication vocabulary to make your own communication board: http://www.temple.edu/instituteondisabilities/aacvocabulary/HEALTH_FULL.shtml
21. Cook A, Hussey S. Augmentative and alternative communication systems. Assistive Technologies, Principles and Practice. Second Edition ed. St. Louis: Mosby, 2002, 487.
22.Example of an encoding chart:
- I am in pain
- I need to be repositioned
- I need a drink
- I need my glasses
- I need a tissue
- I need my medication
- I am cold
- I am hot
23. Safe Laser Pointer by Zygo: (www.zygo-usa.com)
24. Laser pointer mounted to a headset
25. Megabee (www. http://megabee.net)
26. iPhones, iPads, and iTouches for Communication
27. Apps for iPhones/iTouches/iPads
a. Speak It! Turns anything a user types into speech. Good voice synthesizer with very clear speech. $1.99 through iTunes.
b. Small Talk for Aphasia. Designed for people with aphasia, Small Talk provides a vocabulary of pictures and videos that talk in a natural human voice. Fee on iTunes.
c. Speak! Text to Speech for iPhone and iTouch with Historic Quotes. Type anything into the communication box and tap Say It, and the iPhone will repeat what the user has typed. Free on iTunes.
d. iSpeech. Type anything into the communication box and tap Say It, and the iPhone will repeat what the user has typed. Free on iTunes.
e. Talk to Me. Does the same as above but reads words as the user types them. $1.99 on iTunes.
f. Locabulary . Allows the user to communicate words, phrases, and sentences. It is a small, compact alternative to a larger AAC devices. Free on iTunes.
g. Proloquo2Go: AAC in Your Pocket: Proloquo2Go™ is a new product from AssistiveWare that provides a full-featured communication solution for people who have difficulty speaking. It brings natural sounding text-to-speech voices, up-to-date symbols, powerful automatic conjugations, a default vocabulary of over 7000 items, full expandability and extreme ease of use to the iPhone and iPod touch. $189.00 on iTunes.
28. Free and Low Cost Software available on the Internet.
|Cost||Name of Software||Website||Non-English Available?||PC or Mac|
29. HeadMouse Systems
a. Origin Instruments (http://www.orin.com/access/headmouse) $995.00
b. Madentec (http://madentec.com/products/tracker-pro.php) $995.00
c. Camera Mouse (www.cameramouse.com) FREE. (requires user have a webcam—about $20.00)
30. You Tube Video showing use of headmouse:
31. Speech Generating Device Manufacturers
h. Dynavox (www.dynavoxtech.com)
i. Prentke Romich (http://www.prentrom.com)
j. Tobii/ATI (http://tobiiati.com)
k. Words+ (www.words-plus.com)
32. Electronic Eye Gaze Units
33. Manufacturers of Electronic Eye Gaze Units:
a. Dynavox (www.dynavoxtech.com)
b. LC Technologies (http://www.eyegaze.com)
c. Prentke Romich (http://www.prentrom.com
d. Tobii/ATI (http://tobiiati.com)
e. Quick Glance (http://www.eyetechds.com)
34. You Tube Video for Electronic Eye Gaze Devices
35. Beukelman D, Garrett K, Yorkson K, Augmentative Communication Strategies for Adults with Acute or Chronic Medical Conditions, First ed., Paul H. Brooks, Baltimore, MD, 2007, 63
36. Schnakers C, Perrin F, Schabus M, Hustinx R, Majerus S, Moonen G; Boly M, Vanhaudenhuyse A, Bruno M, Laureys S, Detecting consciousness in a total locked-in syndrome: An active event-related paradigm, Neurocase: The Neural Basis of Cognition, 2009, Volume 15, 1355-4794,