The ALS Association 2014 Clinical Conference Phoenix, AZ

1. Speech Management for ALS:
Ventilator Interventions and Augmentative and
Alternative Communication (AAC) Devices
Jeannette Hoit Cassandra Faux
Department of Speech, Language, and Hearing Sciences
University of Arizona
ALS Clinical Conference
Phoenix, AZ
November, 2014

2. ALS destroys speech and
strips away the ability to communicate.

3. NNoorrmmaall SSppeeeecchh PPrroodduuccttiioonn

4. SSppeeeecchh IImmppaaiirrmmeenntt iinn AALLSS
 Soft voice
 Short phrases
 Hoarse voice
 Hypernasal voice
 Imprecise ‘tongue’ consonants
(th, t, d, k, g, s, sh, ch, j)
 Imprecise ‘lip’ consonants
(p, b, f, v)

5. TThhee MMoovvee ffrroomm SSppeeeecchh ttoo AAAACC

6. Spinal
Bulbar

7. SSppiinnaall AALLSS
Common speech symptom:
speaking dyspnea
“breathing work/effort”
“air hunger”
Respiratory muscle demands are greater for speaking than rest breathing;
higher alveolar pressures, larger lung volumes, faster inspiratory flows

8. Common speech signs:
Reduced loudness
Short phrases
Slow inspirations
SSppiinnaall AALLSS

9. TTrreeaattmmeennttss ffoorr RReessppiirraattoorryy--BBaasseedd
SSppeeeecchh PPrroobblleemmss
 Behavioral treatments
◦ Respiratory muscle training (inspiratory/expiratory)
◦ Modifications of speech breathing
◦ Speaking with ‘intent’
 Prosthetic treatments
◦ Amplification
◦ Postural supports (e.g., abdominal binder)
◦ Ventilator

10. VVeennttiillaattoorrss aanndd AALLSS:: SSuurrvviivvaall aanndd
QQOOLL
 Invasive
 Noninvasive
Survival and quality of life improve with
noninvasive ventilation for people with ALS
(Bourke et al., 2006)
Bourke et al. (2006); Radunovic et al. (2013)

11. IInnvvaassiivvee VVeennttiillaattiioonn
Hixon & Hoit (2005)

12. IInnvvaassiivvee VVeennttiillaattiioonn
Hixon & Hoit (2005)

13. IInnvvaassiivvee VVeennttiillaattiioonn
Hoit & Banzett (1997)

14. IInnvvaassiivvee VVeennttiillaattiioonn
 Ventilator adjustments
◦ Increase the time that pressure is above voicing threshold
(more continuous speech with fewer and shorter pauses)
◦ Reduce the magnitude of pressure variation
(better voice quality and less loudness variation)

15. NNoonniinnvvaassiivvee VVeennttiillaattiioonn
Facemask
Nosemas
k
Nasal pillows
Free-standing
mouthpiece

16. NNoonniinnvvaassiivvee VVeennttiillaattiioonn
Interface Advantages Disadvantages
Facemask None Part of face obscured;
Jaw encumbered;
Speech distorted and
attenuated
Nosemask Can see most of face;
Jaws/lips unencumbered
Nasal sounds distorted
Nasal Pillows Can see most of face;
Jaws/lips unencumbered
Nasal sounds distorted
Free-Standing
Mouthpiece
Can see all of face;
Jaws/lips unencumbered
Long inspiratory pauses

17. T Behavioral Trreeaattmmeennttss ttoo MMaaiinnttaaiinn SSppeeeecchh
 Compensatory strategies
◦ Overarticulate
◦ Take breaths more often
◦ Avoid speaking over noise
◦ Have listener look at you
◦ Energy conservation
 Planning ahead for AAC

18.  Recorded messages (digitizing words or phrases
spoken by the person for use in speech
generating device: SGD)
 Protocol to digitize speech samples to create a
synthesized voice based on a person’s own
speech features
 ModelTalker
 https://www.modeltalker.org/demo/
 Fatiguing/Time Consuming/After speech declines
not possible

19. VVooiiccee AAmmpplliiffiieerrss
 Speech Enhancer
◦ http//www.speechenhancer.com/
 ChatterVox
◦ http://www.chattervox.com

20. SSppeeeecchh--ttoo--TTeexxtt
 Dragon Naturally Speaking
 MyVoice X (free app for Mac and Windows that
allows you to enter and save phrases and have
them spoken)

21. FFoorrmmss ooff AAAACC
 Low Technology (communication boards)
 High Technology (dedicated/integrated/non-dedicated
speech generating devices, mobile
technology)

22.  Communication Boards
 Topic Boards
 Alphabet Boards
◦ E-tran display
 Writing
◦ White Board (App)
◦ Dry erase
◦ Notebook

23. LLooww TTeecchhnnoollooggyy AAcccceessss
 Direct Selection
◦ Head Pointers
◦ Laser pointer
 Low tech solutions
 Partner-supported systems
◦ Partner-assisted scanning

24. HHiigghh TTeecchhnnoollooggyy
 Non-Dedicated
◦Software
Etriloquist: http://www.etriloquist.com
CHIPSpeaking
 Dedicated
 Integrated
 Mobile Devices (Android, iPhone, iPad, iTouch)
◦Apps
Verbally (Intuary)
Assistive Chat (Assistive Apps)
Talk Now for Android (appszoom)


25. Free AAC Apps Available for Mobile Technology (Text-to-
Speech and Symbol-Based)
 Able AAC Free (by Able Vox)
 Pogo Boards (by Talk to Me Technologies, LLC) 
 Alexicom AAC (by Alexicom Tech LLC) 
 Say Hi AAC (by Gwendolyn Strong Foundation)
 PandaPal (AAC) (by Kim Scott, M.S., CCC-SLP) 
 Flip Writer AAC (by Navanit Arakeri)
 SayItForMeLite (by PowerTurn, Inc)
 urVoice AAC (by Cleric Software Solutions) 
 Sono Flex Lite (by Tobii Technology) 
 SaySomeMore (by Dale Hubert)
 EESpeechBasic (by Elite Express Solutions, Inc)
 CommunicationAide Free: AAC/Speech Therapy (by ObjectGraph, LLC)
 OneVoice Lite – AAC (by Legend) 
 Verbally (by Intuary) 
 EESpeech Lite – AAC (by Elite Express Solutions, Inc)
 Grid Player (by Sensory Software International)
 SoundingBrd (by AbleNet)
 VoiceSymbol AAC (by Unlimiter)
 Gabby Tabs Lite – AAC for Kids (by Tech Unlimited)
 Talkingtiles (by Mozzaz Corporation)
 SmallTalk (by Lingraphica)
◦ Has different apps for different categories of conversations (all free)

26. For-Purchase AAC Apps Available for Mobile Technology
(from $1.99 to $299.99)
 My First AAC (by Injini) - $24.99
 AAC Evaluation Genie (by Hump Software) - $11.99
 Posco AAC (by Bryan Keller) - $9.99
 Quick Talk AAC (by Digital Scribbler, Inc) - $24.99
 aacorn AAC (by aacorn) - $189.99 
 Proloque2Go (by AssistiveWare) - $219.99
 AAC Ferret (by INCLUSIVE LEARNING TECHNOLOGY) - $4.99 
 Able AAC (by AbleVox) - $24.99
 Talking Picture Board (by Scott Yang) - $1.99
 Picture AAC (by Hearty SPIN) - $29.99
 TouchChat HD – AAC with WordPower (by Silver Kite) - $299.99
 Quick Type AAC (by Digital Scribbler, Inc) - $1.99
 Choiceworks (by BBee Visual, LLC) - $6.99
 Able Jr AAC (by Ablevox) - $24.99
 SpeechHero AAC (by Andrew McKinney) - $99.99
 Avatalker AAC (by Metova, Inc) - $189.99
 Easy Speak HD – AAC (by Pocket Apps Canada, Inc) - $49.99

28. SSwwiittcchh AAcccceessss ffoorr MMoobbiillee TTeecchhnnoollooggyy
 Bluetooth (AbleNet)
 Tapio (Origin Instruments)

29.  Headmouse Extreme
◦ Origin Instruments

30. AAcccceessss TThhrroouugghh OOnn--SSccrreeeenn
KKeeyybbooaarrddss
◦ Click-N-Type (Lake Software)
◦ Dasher
◦ Origin Instruments SofType
◦ WiVik
◦ My-T-Mouse
◦ Hot Virtual Keyboard

31. EyeMax
EyeTech Digital
Systems
EyeGaze
Edge
Accent
NuEye
Tobii I-Series
Tobii EyeMobile

32.  Cursor control with eye movement
 Least fatiguing movement
 Preserved volitional movement
 Calibration
 Eyelid control
 Oculomotor Apraxia (Rambold et al., 2006; Zadikoff & Lang, 2004)
 Cataracts
 Glasses (angling the camera)
◦ Reducing glare
 Easy to train

33. MMeeddiiccaarree FFuunnddiinngg && IInntteeggrraatteedd SSyysstteemmss
 Medicare
◦ 13 month cap
◦ Hospice
◦ December 1, 2014 decision

34. PPeerriipphheerraall EEyyee TTrraacckkeerr
 PCEye Go (Tobii)
 EyePro (EyeTech Digital Systems)

35. MMoobbiillee EEyyee TTrraacckkiinngg SSyysstteemmss
Embedded Technology into goggles or glasses
EEyyeeSSppeeaakk:: BBeeyyoonndd CCoommmmuunniiccaattiioonn
bbyy LLuussoo VVuu--UUSSAA

36. TThhiinnkkTTaallkk AApppp
 Requires the use of a NeuroSky
MindWave Mobile Bluetooth-enabled
headset
 Available in the iTunes App
Store and Google Play for $9.99
 Compatible with Android 1.6+
 Compatible with iOS 7.0+ on
iPhone, iPad, and iPod touch
 Easy-to-use interface
https://itunes.apple.com/us/app/thinktalk/
id910349915?mt=8
https://itunes.apple.com/us/app/thinktalk/id910
349915?mt=8

37. NNeeuurrooSSkkyy MMiinnddWWaavvee MMoobbiillee
 Research-grade EEG
headset available
(packages starting at $99.99)
 Additional iOS and
Android apps are
available on iTunes
App Store and
Google Play,
respectively
http://store.neurosky.com/collections/mindrefle http://www.thinkgeek.com/product/eed1/
ctor-promo/products/brainwave-starter-kit

38.  Brain computer interface (BCI) technology has
generated considerable research interest for
people who are physically “locked-in” such as
those in the late stages of ALS
 To date, no investigations have reported of the
use of BCI throughout the disease progression of
ALS to determine if PALS would be able to
maintain training and functional use of the
systems.

39.  http://www.youtube.com/watch?v=NIG47YgndP8

40.  Memory Books (App: Scrapbook by Album tArt LLC)
 Major Events
 Visual Scenes
Phukan et al., 2011

42.  Insurance & Medicare
 ALS Association
 AzTAP
◦ SW Human Development
◦ TACT
 MDA
 VA
 Out-of-pocket purchase

43. The ability to communicate
is key to maintaining
quality of life
in people with ALS.

44. RReeffeerreenncceess
 Ball, L. J., Beukelman, D. R., & Pattee, G. L. (2004). Acceptance of augmentative and alternative communication technology by persons with amyotrophic lateral
sclerosis. Augmentative and Alternative Communication, 20(2), 113-122. http://dx.doi.org/10.1080/0743461042000216596
 Ball, L. J., Nordness, A. S., Fager, S. K., Kersch, K., Mohr, B., Pattee, G. L., & Beukelman, D. R. (2010). Eye-gaze access to AAC technology for people with
amyotrophic lateral sclerosis. Journal of Medical Speech-Language Pathology, 18(2), 11-23.
 Baxter, S., Enderby, P., Evans, P., & Judge, S. (2012). Barriers and facilitators to the use of high-technology augmentative and alternative communication devices: a
systematic review and qualitative synthesis. 47(2), 115-129. http://dx.doi.org/10.1111/j.1460-6984.2011.00090.x
 Beukelman, D. R., Fager, S., Ball L., & Dietz, A. (2007). AAC for adults with acquired neurological conditions: A review. Augmentative and Alternative
Communication, 23(3), 230-242. http://dx.doi.org/10.1080/07434610701553668
 Bourke, S., Tomlinson, M., Williams, T., Bullock, R., Shaw, P., & Gibson, G. (2006). Effects of non-invasive ventilation on survival and quality of life in patients with
amyotrophic lateral sclerosis: A randomised controlled trial. Lancet Neurology, 5, 140-147.
 Doyle, M., & Phillips, B. (2001). Trends in augmentative and alternative communication use by individuals with amyotrophic lateral sclerosis. Augmentative and
Alternative Communication, 20(2), 113-122. http://dx.doi.org/10.1080/0743461042000216596
 Fried-Oken, M., Fox, L., Rau, M. T., Tullman, J., Baker, G., Hindal, M., Wile, N., Lou, J. (2006). Purposes of AAC device use for persons with ALS as reported by
caregivers. Augmentative and Alternative Communication, 22(3), 209-221. http://dx.doi.org/10.1080/07434610600650276
 Hess, D. (2006). Noninvasive ventilation in neuromuscular disease: Equipment and application. Respiratory Care, 51(8), 896-912.
 Hixon, J., & Hoit, J. (2005). Evaluation and Management of Speech Breathing Disorders. San Diego: Plural Publishing.
 Hoit, J., Banzett, R., Lohmeier, H., Hixon, T., & Brown, R. (2003). Clinical ventilator adjustments that improve speech. Chest, 124, 1512-1521.
 Kübler, A., Nijboer, F., Mellinger, J., Vaughan, T. M., Pawelzik, H., Schalk, G., . . . Wolpaw, J. R. (2005). Patients with ALS can use sensorimotor rhythms to operate a
brain-computer interface. Neurology, 64, 1775-1777. http://dx.doi.org/10.1212/01.WNL.0000158616.43002.6D
 Light, J. & McNaughton, D. (2014). Communicative competence for individuals who require augmentative and alternative communication: A new definition for a new
era of communication? Augmentative and Alternative Communication, 30(1), 1-18. http://dx.doi.org/10.3109/07434618.2014.885080
 McNaughton, D., Light, J., & Groszyk. (2001). “Don’t give up”: Employment experiences of individuals with amyotrophic lateral sclerosis who use augmentative and
alternative communication. Augmentative and Alternative Communication, 17(3), 179-195. http://dx.doi.org/10.1080/aac.17.3.179.195
 McNaughton, D., & Light, J. (2013). The iPad and mobile technology revolution: Benefits and challenges for individuals who require augmentative and alternative
communication. Augmentative and Alternative Communication, 29(2), 107-116. http://dx.doi.org/10.3109/07434618.2013.784930
 Prigent, H., Garguilo, M., Pascal, S., Pouplin, S., Bouteille, J., Lejaille, M., Orlikowski, D., & Lofaso, R. (2010). Speech effects of a speaking valve versus external
PEEP in tracheostomized ventilator-dependent neuromuscular patients. Intensive Care Medicine, 36, 1681-1687.
 Radunovic, A., Annanne, D., & Rafiq, M. (2013). Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease (Review). The Cochrane Collaboration.
John Wiley & Sons, Ltd.
 Wendt, O. (2003). Outcomes of low and high technology AAC intervention strategies for AAC patients [PDF document]. Paper presented at the annual conference of
the Center On Disabilities Technology and Persons With Disabilities, Northridge, CA. Retrieved from
http://www.edst.purdue.edu/aac/ALS%20and%20AAC%20CSUN%202003%20handout.pdf
 Wilkinson, K. M., & Mitchell, T. (2014). Eye tracking research to answer questions about augmentative and alternative communication and intervention. Augmentative
and Alternative Communication, 30(2), 106-119. http://dx.doi.org/10.3109/07434618.2014.904435