Excerpted from Chapter 10 of Augmentative Communication Strategies for Adults with Acute or Chronic Medical Conditions, edited by David R. Beukelman, Ph.D., Kathryn L. Garrett, Ph.D., CCC-SLP, and Kathryn M. Yorkston, Ph.D.
Copyright © 2007 by Paul H. Brookes Publishing Co. All rights reserved. No part of this excerpt may be reproduced or reprinted without permission in writing from the publisher.
MS is the most common neurological disorder of young and middle–age adults. It is the third leading cause of disability in this age group, after trauma and arthritis. Although the relapsing–remitting pattern typically begins between the ages of 18 and 40, the progressive form of the disease begins somewhat later. The cause of MS is unknown. However, several theories exist. The three most common are an autoimmune basis, a viral basis, and a genetic predisposition (Boyden, 2000). Most researchers now believe that the etiology of MS could be a complex interaction among these three factors.
A relatively high percentage of people with MS experience some type of communication impairment, usually dysarthria, although occasional reports of aphasia have been documented (Blackwood, LaPointe, Holtzapple, Pohlman, & Graham, 1991; Devere, Trotter, & Cross, 2000; Lacour et al., 2004; Olmas–-Lau, Ginsberg, & Geller, 1977; Trinka, Unterberger, Luef, Benke, & Berger, 2001). A relatively small proportion of people with MS have such extensive unmet communication needs that they require high technology AAC (Beukelman, Kraft, & Freal, 1985; Yorkston, Klasner, et al., 2003).
What Do I Need to Know About Multiple Sclerosis to Provide AAC Services?
Characteristics of the Condition
MS is an acquired, inflammatory, demyelinating disease of the central nervous system. The disease is most frequently diagnosed in people in their mid–20s to early 30s. It is described as a progressive neurological condition; however, early phases are often characterized by a remitting–relapsing course. Most people who need AAC options have already progressed through the remitting–relapsing pattern and are in a chronic degenerative pattern called secondary progressive MS. The scattered lesions in the central nervous system produce a variety of motor, sensory, and cognitive limitations that produce impairments in these functions that must be considered in making AAC intervention decisions.
Inflammatory white matter lesions continuously appear, resolve, and recur even during periods when the clinical course is quiet. Initially, these lesions are edematous (marked by edema or swelling). Activated macrophages strip away the myelin and eventually leave the axon bare. Patches of destroyed myelin are later replaced by scar tissue, producing lesions known as plaques. Neurological dysfunction results primarily from blockage of nerve conduction through the demyelinated area.
Impact on Communication
Speech disorders have long been reported for people with MS. Dysarthria in MS often has components of mixed ataxic–spastic dysarthria. The speech characteristics of individuals with MS dysarthria are described as follows: "The most prominent speech deviations in MS are impaired control of loudness, harshness, and defective articulation. Impaired use of vocal variability for emphasis, impaired pitch control, hypernasality, inappropriate pitch level, and breathiness are observed with lesser degrees of frequency" (Darley, Aronson, & Brown, 1975, p. 242).
Although dysarthria is not universal in MS, it is relatively common. Fifty-nine percent of those studied in the Mayo Clinic group "present an overall speech performance that would be considered essentially normal in terms of its impact upon a listener" (Darley et al., 1975, p. 238). When individual speech features are tallied separately, a slightly different picture emerges. Respiratory and phonatory features occurred most frequently, with 77% of people with MS exhibiting impaired loudness control and 72% demonstrating harsh voice quality. Defective articulation occurred in 46% of the sample. When individuals with MS were grouped by severity of overall disease, the percentage reporting speech problems varied as a function of the stage of the disease. For those with no problems involving ambulation or arm and hand function, 33% reported speech changes. For those with ambulation problems only, 29% reported speech changes. For those with ambulation problems combined with weakness or lack of arm and hand control, 46% reported speech changes. Finally, for the most severe group, those with ambulation problems, weakness, and lack of arm and hand control, 67% reported speech changes.
Self–report questionnaires about communication difficulties associated with MS indicate that from 23% (Beukelman et al., 1985) to 44% (Hartelius & Svensson, 1994) of the respondents noted changes in speech. The discrepancy between these studies may be the result of differences in time post onset and disease severity and the Hartelius and Svensson sample representing more severely involved individuals. Studies in which speech-language pathologists (SLPs) rated the adequacy of speech or performed a physical examination of the speech mechanism report a higher incidence of speech problems than studies using selfreports. Hartelius, Runmarker and Anderson (2000) reported a rate of 51% in a cohort study in which individuals with MS diagnosed from 1950 and 1964 were followed for a mean of 37.5 years. Regardless of methodological differences, an important proportion of the MS population experienced changes in communication. This proportion appeared to increase over time as the disease progressed. Fortunately, only about 4% of all people with MS report that they cannot speak well enough for strangers to understand them (Beukelman et al., 1985). Published reports of AAC interventions for people with MS are very limited; however, some case reports are available (Honsinger, 1989; Porter, 1989).
Symptoms associated with MS vary greatly, depending on several factors including the size, age, activity, and location of the lesions. Motor symptoms, including spasticity, weakness, spasm, ataxia, and tremor, are common in MS. Sensory changes are also common and may include decrease in touch, pain or position sense. Symptoms may also include numbness, tingling, burning, shooting electrical sensations, and pain. Changes in vision may result from demyelination of the optic nerve. Like many aspects of MS, cognitive impairment in the disease varies considerably from person to person. This variability is due at least in part to the location, number, and activity of lesions. Reviews of cognition and neuropsychological testing in MS are available (Pepping & Ehde, 2005; Zakzanis, 2000). There is a general consensus that cognitive changes associated with MS are not global but rather target specific cognitive processes. The extent of cognitive impairment is not strongly correlated with the degree of neurological disability.
Fatigue in MS has been defined as a subjective lack of physical and/or mental energy that interferes with daily activities. It is often reported as the most common symptom of MS, reported by 76%–92% of people with MS (Ford, Trigwell, & Johnson, 1998). One third of people with MS indicated that fatigue was their worst symptom (Krupp, Alvarex, LaRocca, & Scheinberg, 1988). Recent assessment tools are beginning to distinguish between mental and physical fatigue (Ford et al., 1998). Fatigue is made worse by heat, stress, or physical activity. Fatigue is a critical issue because it is invisible, yet it can limit social, work, and role performance. Heat sensitivity is common in MS, and an increase in temperature often causes the appearance of new symptoms or the worsening of old ones.
Affective disorders—those that relate to emotion or feeling—are common in MS. Disorders include depression, euphoria, apathy, and irritability. Depression is a mood disorder that is associated with loss of interest or pleasure. Psychological distress, including depression, occurs frequently in individuals with MS (Ehde & Bombardier, 2005). Estimates of major depression at any given point in time ranges from 27% to 54% (Minden & Schiffer, 1990).
Issues Unique to Multiple Sclerosis
The onset of MS usually occurs during midlife, at a stage when individuals are fully engaged in employment and family and community activities. MS occurs more frequently in women than in men. Because women may be responsible for child rearing and household management as well as paid employment, limitations are particularly difficult for them. As the disease progresses, each of these areas is affected in rather unique ways depending on the particular symptom profile experienced by the individual. However, few of these changes are driven by limitations in spoken communication. For example, people with MS do not leave employment because of communication limitations but rather because of fatigue, vision, balance, cognitive, or coordination issues. Surveys of people with MS suggest that moderate or severe speech disorders do not occur in isolation; rather, they usually occur along with a complex constellation of other physical, cognitive, and psychosocial changes (Yorkston et al., 2003; Yorkston, Klasner, & Swanson, 2001). Therefore, AAC intervention almost always focuses on the need to participate in life in ways that are determined, at least in part, by the disabilities resulting from MS. As with other degenerative conditions, there is a need to plan AAC intervention with the realization that the residual capabilities of the individual continue to change. MS is unique in that these changes may occur in a variety of capabilities on which successful AAC intervention has been based, such as vision, motor control, and cognition. The changes are quite unpredictable and require regular monitoring.
Assistive Technology in Multiple Sclerosis Rehabilitation
A variety of adaptive equipment and assistive devices may be used to compensate for functional limitations in MS. Ambulation aids include canes, forearm crutches, ankle-foot orthoses, walkers, and wheelchairs. A variety of adaptive equipment and techniques are helpful in activities of daily living, such as grooming and dressing. Note that the selection of appropriate techniques depends on the underlying impairment and the individual's unique needs. For example, wrist weights that compensate for tremor might not be appropriate for individuals for whom weakness is a significant problem. A more complete description of assistive technology in MS can be found elsewhere (Blake & Bodine, 2002; Finlayson, Gugliolmello, & Liefer, 2001). As with other conditions described in this text, each assistive technology (AT) decision can affect AAC intervention choices as well.
What Is Considered During Assessment?
Because the course and pattern of symptoms in MS are so variable, a detailed interview is critical. Key topics include medical history, communication needs, anticipated changes in living situation, associated problems, and perceived extent of the problem. The clinical interview can also help to identify key decision makers. A more complete description of the assessment process can be found elsewhere (Yorkston, Miller, & Strand, 2004).
A careful medical history should focus on the course of the disease, the history of the nature and timing of exacerbations, and the course of the speech and cognitive involvement.
People with MS differ in terms of communication needs and the environments in which they communicate. Initially, some are employed in jobs that demand public contact and superior speech. Later, they might be in a much more sheltered environment, where their function is not limited by mildly distorted speech. Therefore, a thorough review of communication needs is critical. During this interview, the person with MS might be asked to "think about how you use your speech everyday at work, in your community, at social activities and with family life." Rather than involving a standard series of questions, the interviews often take the form of openended questions such as "Try to remember situations that you found difficult, and then tell me about the things that made them difficult." The needs assessment forms described in Chapters 3, 5, and 9 are quite useful for people with MS. These forms can be found on the accompanying CD-ROM.
Anticipated Living Changes
As MS progresses, living arrangements change from the family home to assisted living to long–term care. Within each setting, the use of care personnel can also change. Some people choose to remain in the family home and bring extensive support services into that setting, while others choose an assisted living setting. Often, a change in living arrangements is associated with changing communication support needs. While family members are very familiar with the person and his or her speech and life routines, unfamiliar caregivers in the home, assisted living, or long–term care facility might well require additional communication support. At times, caregivers do not speak English as a first language and might have difficulty understanding dysarthric speech.
Depending on the course of the condition, people with MS may experience a variety of problems that affect communication. For example, presence and pattern of fatigue, a common symptom of MS, affects the presence and severity of speech symptoms (Yorkston et al., 2001). An account of cognitive impairments is needed because these difficulties can influence communication problems and interfere with work. Finally, the presence of mood disorders such as depression, euphoria, or emotional lability should be explored. Other symptoms also have implications for the selection of AAC approaches. For example, motor symptoms such as intention tremor may affect use of keyboards, and visual problems may affect the ability to use various AAC displays.
Awareness of the Problem
Some people with MS and mild dysarthria complain of many restrictions in participation. Others complain that communication takes extra effort. On the other hand, some people with severe dysarthria report no functional limitations. Information should be obtained about how people with MS judge the severity of their disorder and how it limits their ability to take part in valued activities as they carry out their daily activities. Issues related to effort should also be explored.
Identification of Decision Makers
Given the early age of onset, people with MS can be expected to live many years with increasing levels of disability. Thus, a range of decision makers is often involved in the life choices that are made with and for people with MS. For example, early in the disease, children might be too young to actively influence decisions, but as time passes, one or more children might become very important decision makers. People with MS, in consultation with others, often make decisions about living arrangements, communication options, and care needs. It is important that AAC interventionists are aware of influential decision makers and their roles in different areas of care and support. A more complete discussion of the decision–making process can be found in Chapter 12.
What AAC Intervention Options Are Available?
Because different options are appropriate for different people with MS, the following describes intervention during the early, middle, and late phases. These phases are defined according to the severity of communication problems.
In the early phases of MS, speech remains functional, but technology might be required to compensate for visual problems that affect reading or motor problems such as intention tremor that affects keyboard access. For some, visual limitations interfere with computer operations, and they need help to obtain large monitors and to learn to enhance the size of images and fonts on their computer applications. Typically, font enlargement is not difficult on widely commercially available wordprocessing and spreadsheet programs. However, some experience difficulty with customized, specialty software applications. For others, keyboard control becomes difficult as motor discoordination or tremor interferes with conventional typing. Arm and hand supports may be necessary to inhibit nonproductive movement patterns. Other high–tech solutions for intention tremor are also being studied (Feys et al., 2001)
Johnson and Johnson (2005) presented a series of four cases of people with MS in subacute residential facilities. Their ages ranged from 33 to 54 years, and all experienced limited use of their arms and legs, with some vision changes, fatigue, and, in one case, speech involvement (primarily a weak voice). All had worked before retirement due to MS, but none had access to computers or the Internet. Although none had severe speech problems, the goal of the AT intervention was to reduce social isolation by enabling the individuals to communicate by e–mail with friends and family, to read books and other materials online, to conduct research on MS and other topics, and to communicate with other people with MS. In all cases, computer access was enhanced with relatively inexpensive positioning strategies and software. Participants reported improved quality of life and independence. The authors advocate for residential facilities to provide computer access and related AT as part of their basic services.
Memory and organizational impairments can occur throughout MS. People with MS routinely use low-tech memory boards and calendars to compensate for these problems. In addition, personal digital assistant (PDA) technology is increasingly used as a highly portable, conventional appearing compensatory strategy. Blake and Bodine (2002) describe the initial use of these technologies for people with cognitive impairments. Examples of memory aids can also be founded in Chapter 8.
A subgroup of people with MS have many characteristics in common with people with spinal cord injury. They are quadriplegic and are cognitively intact. Their oral articulation is not affected, but respiratory weakness combined with MSassociated fatigue prevents them from participating in many communication situations. These individuals may benefit from portable speech amplification devices (see Chapter 9). A more complete discussion of AAC issues in spinal cord injury can be found in Chapter 4, which reviews computer access including selected switches and mounting options.
During this phase, speech requires compensatory assistance. Typically, middlephase interventions focus on the supplementation of natural speech. As dysarthria worsens, effective communication interaction may be affected in adverse situations such as in noisy environments or with people who are hearing impaired.
Alphabet board supplementation is a strategy in which speakers point to the first letter of each word as they say it. This approach has several effects, including slowing speech, marking spoken word boundaries, and providing the listener with the initial letter of each word. People who use this strategy typically experience difficulty making themselves understood and are willing to sacrifice speech rate, naturalness, and eye contact with the listener to improve intelligibility. They also have sufficient hand function to point to letters on a board. Those who use alphabet supplementation often require considerable support and encouragement from their listeners, as many speakers find this procedure to be somewhat difficult to accept because it require the adoption of an atypical communication style. Frequently, communication partners must be introduced to the alphabet supplementation strategy with an explanation of its benefits and a demonstration. Given the visual and motor limitations of individuals with MS, the size and visual boldness of the alphabet display might need to be adjusted. Fortunately, these boards can easily be produced with conventional computer equipment, printed, and laminated.
A systematic review of the literature (Hanson, Yorkston, & Beukelman, 2004) has documented the impact of alphabet supplementation for speakers of a range of neurological conditions. The treatment effect is quite consistent in the midrange of sentence intelligibility (about a gain of 25%). However, the impact for speakers in the profound range is more variable, in that it can be limited or extensive. Therefore, the impact for each person should be evaluated as part of the intervention. This can be done by having a person speak with and without alphabet supplementation and comparing the speech intelligibility under both conditions.
People with MS and severe or profound dysarthria typically experience involvement of multiple neural systems. Natural speech might no longer be functional in all situations. Providing AAC services to these people is often challenging. Hand function for writing or keyboard access is likely to be limited by spasticity, ataxia, or intention tremor. Direct selection access to AAC systems might be limited even with aids such as keyguards. A protocol for motor screening can be found in Chapter 5 (see Worksheet 5.8 on the accompanying CD–ROM). Visual problems might limit strategies that require reading. The ability to learn new AAC approaches might be limited by cognitive and behavioral problems. Examples of memory aids and interest albums along with tools for assessing wants, needs, and safety can be found in Chapter 8. A form for gathering biographical information (Form 8.8) can also be found on the accompanying CD–ROM.
Motor deficits in MS are often complicated by severe, variable visual problems. The first step in developing an intervention plan is careful assessment. Honsinger (1989) proposed the following questions to ask during the visual examination of individuals with severe dysarthria:
- What is the status of visual acuity?
- Are there problems with peripheral vision?
- Are field cuts or field neglect present?
- What is the person's response to motion?
- What are the person's tracking skills?
- What are the effects of color and position of visual information?
In developing visual displays, the use of bold print and proper positioning of material in the visual field can be helpful. A protocol for visual screening can be found in Chapter 5 (see Worksheet 5.7 on the accompanying CD–ROM). When visual problems are severe, auditory scanning systems may be used, as described in Chapter 3. (See Clinical Tool 3.10 on the accompanying CD–ROM.)
Issues of vocabulary and message selection are critical for people with latephase MS. Inclusion of whole messages is desirable because of the slow communication rate. These people tend to live in sheltered environments and often interact only with familiar people. Lists of core vocabulary and messages for adults are available (see Resources at the end of this chapter). In addition to traditional categories such as people, physical feelings or needs, activities, and food/drink, people in latephase MS may also need topics such as religious/spiritual terms, emotions, and financial terms.
Case Report 1
Martha is a 35–year–old woman with MS that affected her speech, limb control, walking, and vision. Initially, she came to our center for assistance in communicating under adverse conditions and in accessing her computer to support writing and bookkeeping activities. As time passed, she transitioned from her home into a long–term care facility. In this setting, she interacted with many individuals (other residents as well as care staff) who were unfamiliar with her speech, so she came to rely more and more on her communication partners to meet her communication needs. Following an AAC assessment, Martha was given a trial with a System 2000 AAC device. To meet her visual needs, the size of the print on the screen was enlarged, and the color patterns were optimized for her. She accessed the system through scanning because her intention tremor eliminated the possibility of direct selection. The features of this device allowed her to control wordprocessing, spreadsheet, and Internet software. As the MS progressed, Martha was able to access her AAC device only in the wheelchair. When in bed, she required dependent scanning support from her communication partners.