Communication Support for People with ALS

Abstract

Almost all people with amyotrophic lateral sclerosis (ALS) experience a motor speech disorder, such as dysarthria, as the disease progresses. At some point, 80 to 95% of people with ALS are unable to meet their daily communication needs using natural speech. Unfortunately, once intelligibility begins to decrease, speech performance often deteriorates so rapidly that there is little time to implement an appropriate augmentative and alternative communication (AAC) intervention; therefore, appropriate timing of referral for AAC assessment and intervention continues to be a most important clinical decision-making issue. AAC acceptance and use have increased considerably during the past decade. Many people use AAC until within a few weeks of their deaths.

Full text

Hindawi Publishing Corporation
Neurology Research International
Volume 2011, Article ID 714693, 6pages
doi:10.1155/2011/714693
Review Article
Communication Support for People with ALS
David Beukelman, Susan Fager, and Amy Nordness
Institute for Rehabilitation Science and Engineering Madonna Rehabilitation Hospital and University of Nebraska,
202 Barkley Memorial Center, P.O. Box 830732, Lincoln, NE 68583-0732, USA
Correspondence should be addressed to David Beukelman, dbeukelman@unl.edu
Received 15 November 2010; Accepted 2 February 2011
Academic Editor: Peter van den Bergh
Copyright © 2011 David Beukelman et al. This is an open access article distributed under the Creative Commons Attribution
License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly
cited.
Almost all people with amyotrophic lateral sclerosis (ALS) experience a motor speech disorder, such as dysarthria, as the disease
progresses. At some point, 80 to 95% of people with ALS are unable to meet their daily communication needs using natural speech.
Unfortunately, once intelligibility begins to decrease, speech performance often deteriorates so rapidly that there is little time to
implement an appropriate augmentative and alternative communication (AAC) intervention; therefore, appropriate timing of
referral for AAC assessment and intervention continues to be a most important clinical decision-making issue. AAC acceptance
and use have increased considerably during the past decade. Many people use AAC until within a few weeks of their deaths.
1. Introduction
Almost all people with amyotrophic lateral sclerosis (ALS)
experience a motor speech disorder as the disease progresses.
Initial symptoms typically do not interfere with speech
intelligibility and may be limited to a reduction in speaking
rate, a change in phonatory (voice) quality, or imprecise
articulation. At some point in the disease progression, 80
to 95% of people with ALS are unable to meet their daily
communication needs using natural speech. In time, most
become unable to speak at all [1]. For them, communication
support involves a range of augmentative and alternative
communication (AAC) strategies involving low- and high-
technology (speech generating device) options [2]. Clinical
decision-making related to communication is quite complex
as screening, referral, assessment, acquisition of technology,
and training must occur in a timely manner, so when
residual speech is no longer effective, AAC strategies are in
place to support communication related to personal care,
medical care, social interaction, community involvement,
and perhaps employment. Although there is considerable
research on the speech characteristics of people with ALS,
it is the primary purpose of this paper to review the
published research related to communication supports for
people with ALS whose natural speech no longer meets their
communication needs.
2. Speech Characteristics
ALS involves both upper and lower motor neurons; there-
fore, it results in mixed dysarthria of the flaccid-spastic type
[3,4]. In the early stages of ALS when dysarthria is mild,
either spasticity or flaccidity is predominant. As ALS pro-
gresses and dysarthria becomes severe, profound weakness
resulting in reduced movement of the speech musculature
and severely reduced phonation become increasingly com-
mon [5,9].
Changes in speech patterns or speaking rate typically
occur before a decrease in speech intelligibility [6–9].
Initially, speaking rate gradually slows; however, speech intel-
ligibility initially remains relatively high. In time, dysarthria
becomes apparent to people with ALS and their listeners,
and then speech intelligibility decreases such that commu-
nication effectiveness is reduced at first in adverse speaking
situations, such as noisy crowds, and then in all situations.
The results of a study by Ball et al. [10] revealed that percep-
tions of communication effectiveness for speakers with ALS
were quite similar for the speakers and their frequent listeners

2Neurology Research International
across 10 different social situations. ALS speakers and their
listeners reported a range of communication effectiveness
depending upon the adversity of specific social situations.
3. Speech Intervention
A recent review of ALS communication research [11]
concluded that, because of the pathophysiology and the
degenerative nature of ALS, speech treatment strategies
that are designed to increase strength or mobility of the
oral musculature are not recommended. People with ALS,
or those close to them, often request oral exercises to
improve strength and mobility for speech and swallowing,
as strengthening exercises seem intuitive to them as way
to increase performance. However, such exercise programs
should be discouraged, and those with ALS should be
informed that the speaking that they do each day provides a
sufficient amount of speech mechanism activity and exercise.
Speech intervention should focus on learning to conserve
energy for priority speaking tasks and to rest often to reduce
fatigue instead of increasing effort and use with speech
exercises. ALS speakers should learn to avoid adverse speak-
ing/listening situations by muting the television, inviting
people to speak with them in a quiet place rather than
in a crowded room, and using voice amplification when
speaking in noisy environments to reduce the effort required
[1,12]. As speech becomes difficult to understand, many
ALS speakers supplement their speech by identifying the first
letter of each word on an alphabet board (alphabet supple-
mentation) or by identifying the topic on a communication
board (topic supplementation). Although improvements
of speech intelligibility have been documented for these
supplementation procedures in a practice guideline article by
Hanson et al. [13], none of this research has involved ALS
speakers.
It is often difficult for speakers with ALS, their family
members and medical personnel to consider AAC strategies
when they are still using residual speech to meet daily
communication needs. However, their speaking rate should
be clinically monitored such that the referral for an AAC
intervention is initiated in a timely manner. With sufficient
education and preparation, people with ALS and their
decision-makers are ready to examine their AAC options.
However, speech deterioration can be so rapid that individ-
uals can be left with limited communication options, if they
are not prepared to act in a timely manner.
4. Timely Referral for Communication Support
Often people with ALS, their family members, and, at
times, their medical team, do not wish to consider an
AAC decision until their deteriorating speech intelligibility
limits their communication effectiveness. Unfortunately,
once intelligibility begins to decrease, speech performance
often deteriorates so rapidly that there is little time to
implement an appropriate AAC intervention. Appropriate
timing of referral for AAC assessment and intervention
continues to be a most important clinical decision-making
issue. Yorkston et al. [8] initially suggested that speaking rate
reduction precedes decreases in intelligibility in people with
ALS. Ball et al. [14,15] evaluated the speech performance
of 158 different people at 3-month intervals from diagnosis
to death. These authors reported that speaking rate is a
relatively good predictor of intelligibility deterioration for
patients with spinal, bulbar, or mixed ALS. They recommend
that ALS patients be referred for AAC assessment when
their speaking rates reach 125 words per minute on the
Speech Intelligibility Test (Sentence Subtest) [16]. The mean
speaking rate on this test for adults without disability is
190wordsperminute.Thiscomputerizedtestsupportsthe
efficient measurement of speaking rate in clinical settings, so
speaking rate information can be shared with the patient and
family immediately during clinical visits. This helps patients
and their families monitor changes over time, prepare for
an AAC evaluation, and it reinforces their understanding
of rate and intelligibility. Using the Speech Intelligibility
Test (Sentence Subtest), speaking rate can be accurately
monitored over the telephone if a patient lives at a distance
or is unable to travel due to illness weather, or support
issues [17]. It should be noted that speech intelligibility could
not be objectively assessed over the telephone, as a clinical
measure of understandability.
Nordness et al. [18] reviewed the records of nearly 300
people with ALS served by 3 different AAC centers. Each
of these centers implemented the referral guideline of 125
words per minute on the Speech Intelligibility Test (Sentence
Subtest). The authors reported that 88% of the people in the
sample received timely AAC assessments. “Of the 12% who
received “late” referrals, most (93%) were delayed because of
a late referral by their physician, travel demands, and other
interfering health conditions, while a few (7%) received a
delayed assessment because of factors related to the person
with ALS or caregivers.” Most physicians who did not refer in
a timely manner were general practitioners, neurologists not
associated with a multidisciplinary neuromuscular clinic, or
medical staffof long-term care facilities. A higher percentage
of females than males were identified as receiving late AAC
assessments.
5. AAC Acceptance
Ball et al. [6] reported that approximately 95% of people
with ALS in the Nebraska ALS Database become unable to
speak at some point prior to death. AAC acceptance and use
have increased considerably during the past decade. Prior
to 1996, approximately 72% of men and 74% of women
for whom AAC technology was recommended accepted and
used the technology [19]. However, in a more recent report
by Ball et al. [6], 96% of people with ALS for whom speaking
rate was monitored and AAC assessment was recommended
in a timely manner accepted and used AAC, with 6% delaying
but eventually accepting the technology. No differences were
reported for males and females. In the review by Ball et
al. [10] those who rejected AAC reported a cooccurring
functional dementia or experienced multiple severe health
issues, such as cancer, in addition to ALS.

Neurology Research International 3
AAC acceptance involves the patient with ALS as well
as family members and other caregivers. Richter et al.
[20] investigated attitudes toward AAC options by people
with ALS, caregivers, and unfamiliar listeners. The results
indicated agreement among these groups with a strong
preference for AAC use for being over difficult to understand
speech or a low-tech communication book. Fried-Oken et al.
[21] surveyed AAC caregivers. They reported very positive
attitudes toward AAC technology. Those with greater AAC
technology skills reported greater rewards associated with
caregiving. They reported increased perception of social
closeness to the individual with ALS and less difficulty in
providing care.
6. AAC Use
People with ALS use AAC technology for an extended period
of time. Mathy et al. [19] reported on 33 people with ALS
between 1988 and 1996 and found that the mean duration
of use was 14 months. More recent data from the Nebraska
ALS Database have revealed that people with ALS use their
AAC technology with an average of 24.9 months for those
with bulbar ALS and 31.1 months for those with spinal
ALS. Many people used AAC until within a few weeks of
their deaths. Because 15% of the participants in this study
continued to use their AAC technology at the time the report
was completed and were supported by invasive ventilation,
the mean duration of use reported likely underestimated the
length of use for this sample and for people with ALS in
general [1].
Due to the extended use of AAC with deteriorating
levels of physical control, it is imperative that recommended
technology has adjustable access options to meet the range
of motor capability as the disease progresses, that is, people
with ALS should be fitted with AAC technology that supports
multiple access methods such as allowing them to transition
from hand access to scanning and/or head/eye-tracking.
Many AAC devices now incorporate a variety of access
options so that the technology can continue to meet the
needs of the user despite a decline in physical capability. The
sensitivity of dynamic touch screens can be adjusted to allow
for lighter touch. The improved sensitivity of head-tracking
technology has allowed many to use this access method with
minimal head/neck movement control.
Perhaps the most significant advancement in access
technology has occurred with the widespread availability
of eye-tracking systems to allow cursor control with eye
movement to access high-technology AAC devices. As the
disease progresses, many ALS patients require the use of
eye-tracking for several reasons. First, eye-tracking is often
the least fatiguing movement for AAC access. Eye gaze is
natural, and eye muscles generally do not fatigue with use
[22,23]. Compared to other access methods such as switch-
activated scanning, eye-tracking is often reported to be the
least fatiguing access method by people with ALS [24].
Others have reported that eye-tracking technology requires
relatively little effort [25,26].Second,eyegazemaybe
the only volitional movement that the individual continues
to exhibit over time, particularly in cases where invasive
ventilation has been chosen [27].
In a follow-up investigation of 15 people with ALS, Ball
et al. [27] examined the acceptance, training, and extended
use patterns of eye-tracking technology to support commu-
nication. Ninety-three percent of the participants reported
successful implementation of the technology. For 53% of the
participants, eye-tracking technology was selected because
eye movement was the only viable access option available.
The one individual who was not able to successfully use eye-
tracking technology had difficulty with eyelid control, which
has been noted as a potential issue in ALS [28].
The communicative functions served by eye-tracking
devicesinBallandcolleagues’[27] investigation were
extensive. All of the participants (100%) used their eye-
tracking device to support face-to-face communication.
Other functions included group communication (43%),
phone (71%), email (79%), and internet (86%). Six of
the participants (43%) also reported using the eye-tracking
technology to support other computer-based functions
(e.g., word processing, vocation-related software programs).
Others have also reported a wide range of communicative
functions served by AAC for people with ALS [21,29–31]
including word processing, providing accounting services, or
consulting over the phone or Internet.
7. Communication and Life Expectancy
Life expectancy of patients with ALS varies depending on
a number of factors. Those who experience initial spinal
symptoms survive approximately five times longer than those
with initial bulbar (brainstem) symptoms. Life expectancy
is longer for those who opt for noninvasive and invasive
ventilation than for those who do not [32]. According to a
database review [33], the decision to use invasive ventilation
extends the length of AAC use overall, as well as the duration
of time during which AAC technology must be controlled
with minimal or no limb or head movement. Adequate
nutrition at the time of diagnosis and artificial nutrition,
such as a percutaneous endoscopic gastrostomy (PEG), as
the disease progresses improves the quality of life and may
extend the length of life somewhat [32,34,35]. It potentially
could have an impact on AAC use, in that people with
ALS who use artificial nutrition spend less time eating, have
more energy, and have more time to participate in the social
activities of their choice. Often, such participation in social
situations increases the need and opportunity for AAC use.
8. AAC Training and Support
Training and support are an essential component of AAC
service delivery for people with ALS. The significant changes
in movement capabilities require that service providers not
only be proactive in their AAC technology recommen-
dations by providing technology options that can meet
the changing physical needs over time, but also provide
adequate training and support to ensure that the people with
ALS and their caregivers can successfully implement these

4Neurology Research International
access strategies over time. Reports of low AAC use often
accompany descriptions of minimal training or follow-up
[36]. New advances in AAC technology (e.g., eye-tracking)
may require a greater amount of training and intervention
than other access options. Ball and colleagues [27]found
that implementation of eye-tracking systems often required
trouble-shooting in the form of physical or environmental
compensations for successful use of the technology. For
example, the use of glasses often required adjustments to the
LED camera angle to separate the glint on the pupil from the
glare on the glasses. Others required environmental lighting
changes (e.g., changing incandescent bulbs to fluorescent
lighting, dimming lights, and closing shades). The mean
length of instruction provided for these people was 5 hours
(range of 2–20 hours) with a mean troubleshooting time of
2.27 hours (range of 0–10 hours).
While AAC specialists are professionals who provide the
AAC intervention services such as assessment and initial
instruction, AAC facilitators for people with ALS tend to
be family members who typically provide ongoing support
including instruction of new communication partners and
caregivers, programming new messages into the AAC device,
maintaining the AAC system, and interacting with the
technology manufacturer if necessary [37]. Ball et al. [38]
surveyed 68 people with ALS who used AAC technology.
All identified a primary AAC facilitator. Ninety-six percent
of the AAC facilitators were family members, most with
nontechnical backgrounds. In response to a survey, these
primary facilitators preferred hands-on, detailed step-by-
step instruction. They reported receiving slightly over 2
hours of instruction and reported that amount of training
as appropriate.
9. Future Research Directions
Although not documented with published research findings,
AAC service delivery models for people with ALS differ
considerably. The Nebraska Database was collected from
a highly integrated intervention system in which a speech
language pathologist with considerable AAC expertise is
a regular staffmember in three regional clinics that also
includes a neurologist, physical therapist, occupational ther-
apist, registered dietitian, respiratory therapist, and social
worker. This AAC interventionist provides routine speech
screening and education with families. When the speaking
rate threshold of 125 words per minute on the Speech
Intelligibility Test (Sentence Subtest) is reached, ALS patients
are referred to one of three AAC specialty programs for
assessment, implementation, and follow-up. The acceptance
and use data for each patient are reported back to the
coordinating AAC specialist involved in the AAC clinics.
This process typically provides a gradual familiarization
with AAC, which reflects a process reported to increase
adaptation to or acceptance of other supports, such as
assisted ventilation [39]. On the other hand, the Murphy
[36] article documents AAC acceptance and usage associated
with a much less integrated service delivery system. The
authors suggest that the organization of the service delivery
system may have impacted the AAC acceptance and use data
reported in this study. Research is needed to investigate the
impact of AAC service delivery strategies on intervention
effectiveness.
The impact of cognitive function on AAC acceptance and
useneedstobeinvestigatedsystematically.Balletal. [1]
note that while the prevalence of cognitive impairments in
people with ALS is more common than previously thought,
these impairments seem to influence AAC acceptance and
use in a relatively small percentage of those with ALS. As
was reported earlier in this paper, a limited number of
AAC patients with severe frontotemporal dementia rejected
AAC intervention. A recent research summary has reported
that between 10–75% of ALS patients experience cognitive
impairment and between 15–41% experience a fronto-
temporal dementia (FTD) as measured by neuropsycholog-
ical testing, although its effect on management of ALS is
unknown [40]. In preparation for this paper the authors
reviewed the Nebraska Database for the ALS patients served
in the last 30 months (N=87). According to the
multidisciplinary clinical screen, 77.0% did not demonstrate
cognitive impairments, 18.4% demonstrated a mild cognitive
impairment, and 4.6% demonstrated a fronto-temporal
dementia (FTD). Of the patients with FTD, two were not
capable of using AAC, one was able to write and gesture,
and one was still able to use some speech. Of the patients
with a mild cognitive impairment, 62.5% (N=10) were
able to communicate with AAC, 25% (N=4) did not yet
require AAC, and 12.5% (N=2) rejected AAC. Anecdotally,
the authors supported numerous patients with ALS whose
cognitive limitations were of concern to the ALS clinic team,
but who accepted and used high- and low-technology AAC
strategies successfully to meet their communication needs.
Research is needed to objectively document AAC acceptance
and use related primarily to cognitive impairment. Further
research is also needed to clarify the level of cognitive
impairment that tends to interfere with AAC intervention.
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. BCI
research includes invasive (implantable electrodes on or in
the neocortex) and noninvasive means (including electroen-
cephalography (EEG), magnetoencephalography (MEG),
fMRI, and the less expensive near-infrared spectography
(NIRS)). Non-invasive methods have been utilized more
extensively than invasive methods for people with disabilities
(such as those with ALS) [41–43]. While those with ALS
and other conditions who are in a “locked-in” physical state
have motivated research in this area, very few systems have
been successful with this population. It has been postulated
that some forms of cognitive impairment and changes in
EEG signatures in late stage ALS may contribute to the
lack of success using BCI technology as the technology was
introduced after the participants had become “locked-in”
[41,44]. The most successful application for communication
has occurred in people at the beginning stages of the disease
[45–47]. To date, no investigations have reported of the
use of BCI throughout the disease progression of ALS to
determine if these people would be able to maintain training
and functional of the systems.

Neurology Research International 5
Use of AAC interventions, including speech generating
devices, is recognized as the standard of care (practice) for
people with speech-related functional losses associated with
ALS. Considerable research has documented the need for
AAC support, as well as the acceptance, use, and effectiveness
of AAC strategies for people with this medical diagnosis. As
in other fields, additional research is needed to develop new
intervention strategies and to document their effectiveness.
Acknowledgments
The preparation of this paper was supported in part
by The Rehabilitation Engineering Research Center on
Communication Enhancement (AAC-RERC) funded under
Grant no.H133E080011 from the National Institute on
Disability and Rehabilitation Research (NIDRR) in the U.S.
Department of Education’s Office of Special Education
and Rehabilitative Services (OSERS), the Barkley Trust, the
Munroe-Meyer Institute of Genetics and Rehabilitation, and
Madonna Rehabilitation Hospital. The authors report no
conflicts of interest. The authors alone are responsible for the
content and writing of the paper.
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