Intervention Decision-Making for Persons with Amyotrophic Lateral Sclerosis The Amyotrophic Lateral Sclerosis (ALS) Informatics Project is a collaborative research project among the University of Nebraska, Lincoln; the University of Nebraska Medical Center, Neurology Associates; and the Institute for Rehabilitation Science and Engineering at Madonna Rehabilitation Hospital. In conjunction with a Muscular Dystrophy Association Clinic, the speech and swallowing ... Features
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Features  |   December 01, 2004
Intervention Decision-Making for Persons with Amyotrophic Lateral Sclerosis
Author Notes
  • David R. Beukelman, is the Barkley Professor of Communication Disorders at the University of Nebraska-Lincoln and director of Research and Education of the Speech and Language Pathology Division of the Munroe-Meyer Institute of Genetics and Rehabilitation, Omaha, Nebraska. He is a senior researcher at the Institute for Rehabilitation Science and Engineering at Madonna Rehabilitation Hospital. Contact him at dbeukelman1@unl.edu.
    David R. Beukelman, is the Barkley Professor of Communication Disorders at the University of Nebraska-Lincoln and director of Research and Education of the Speech and Language Pathology Division of the Munroe-Meyer Institute of Genetics and Rehabilitation, Omaha, Nebraska. He is a senior researcher at the Institute for Rehabilitation Science and Engineering at Madonna Rehabilitation Hospital. Contact him at dbeukelman1@unl.edu.×
  • Laura J. Ball, is a faculty member of Rehabilitation Medicine in the Department of Pediatrics at the University of Nebraska Medical Center. She coordinates the AAC program at the Munroe-Meyer Institute for Genetics and Rehabilitation and at the Muscular Dystrophy Association-sponsored ALS Clinics. Contact her at lball@unmc.edu.
    Laura J. Ball, is a faculty member of Rehabilitation Medicine in the Department of Pediatrics at the University of Nebraska Medical Center. She coordinates the AAC program at the Munroe-Meyer Institute for Genetics and Rehabilitation and at the Muscular Dystrophy Association-sponsored ALS Clinics. Contact her at lball@unmc.edu.×
  • Gary L. Pattee, is a neurologist with Neurology Associates of Lincoln, Nebraska and is a clinical faculty member at the University of Nebraska Medical Center. He is a neurologist in the Muscular Dystrophy Association-sponsored ALS Clinics.
    Gary L. Pattee, is a neurologist with Neurology Associates of Lincoln, Nebraska and is a clinical faculty member at the University of Nebraska Medical Center. He is a neurologist in the Muscular Dystrophy Association-sponsored ALS Clinics.×
Article Information
Augmentative & Alternative Communication / Special Populations / Attention, Memory & Executive Functions / Features
Features   |   December 01, 2004
Intervention Decision-Making for Persons with Amyotrophic Lateral Sclerosis
The ASHA Leader, December 2004, Vol. 9, 4-5. doi:10.1044/leader.FTR2.09222004.4
The ASHA Leader, December 2004, Vol. 9, 4-5. doi:10.1044/leader.FTR2.09222004.4
The Amyotrophic Lateral Sclerosis (ALS) Informatics Project is a collaborative research project among the University of Nebraska, Lincoln; the University of Nebraska Medical Center, Neurology Associates; and the Institute for Rehabilitation Science and Engineering at Madonna Rehabilitation Hospital. In conjunction with a Muscular Dystrophy Association Clinic, the speech and swallowing performance of approximately 180 persons with amyotrophic lateral sclerosis (PALS) has been monitored at 3-month intervals from diagnosis to death.
ALS is a neurodegenerative disease resulting in mixed dysarthria (spastic/flaccid) of speech. As ALS progresses, the severity of the dysarthria worsens so that a large percentage of PALS are unable to meet their daily communication needs through speech alone and require alternative and augmentative communication (AAC) intervention (see Clinical Pathways on the AAC-RERC Web site). Changes in funding of AAC technology by Medicare and health insurance during the past three years and the elimination of the waiting period for Medicare eligibility for PALS have dramatically improved access to AAC technology (for current information on funding, visit the AAC-RERC Web site).
Need and Acceptance of AAC by PALS
Most PALS experience speech loss to the extent that they require AAC technology during the course of their disease. A review of the ALS Informatics Project database, at the time this article was written, reveals that during the past four years, 96% of PALS experienced such severe communication limitations that AAC systems were recommended for them. In 1982, Sitver and Kraat reported that 80% of PALS required AAC. Perhaps the differences in our data from those reported by Sitver and Kraat reflect the fact that PALS are provided with more effective nutritional and respiratory support now than 20 years ago and their lives are being prolonged.
In a recent article (Ball, Beukelman, & Pattee, 2004), we reported on the acceptance of AAC by PALS. Fifty (n = 50) individuals for whom AAC was recommended and who died during the past three years were studied. Ninety-six percent of the PALS in this study accepted AAC technology either immediately (90%) or after some delay (6%). Only 4% of PALS (n = 2) rejected AAC technology. Both of these individuals demonstrated fronto-temporal dementia.
The results of this study are somewhat different from those reported by Mathy, Yorkston, and Gutmann (2000), referring to data of Gutmann and Gryfe (1996) who reported that 27% of PALS elected not to pursue AAC intervention. One might anticipate that improvements in AAC technology would be an important contributor to increased acceptance of AAC in recent years.
Meeting Communication Needs
Mathy, Yorkston, and Gutmann (2000) reported on AAC use by 31 PALS and Fried-Oken et al. (2004) reported on AAC use by 27 PALS. All of these individuals made extensive use of a variety of low- and high-technology AAC options. Although AAC technology may need to be adapted to accommodate changing motor capabilities of PALS, all PALS who accepted the AAC recommendation and received an AAC system used the high technology until within the last two weeks of their lives (Ball, Beukelman, & Pattee, 2004). All continued to use low-technology strategies until a few days of their deaths.
Monitoring Speech Deterioration to Predict the Need for AAC
Perhaps the most common problem experienced while providing AAC interventions to PALS is the timeliness of the referral for AAC assessment. For persons with spinal onset ALS, severity of speech deterioration may require AAC as late as three to six (or more) years after onset. For persons with bulbar onset ALS, AAC may be required within six to 12 months after onset. Finally, for persons with mixed onset ALS, the timing for AAC is quite variable.
Because the functional speech of PALS deteriorates so rapidly once they experience reductions in speech intelligibility, there is a clinical need for strategies to anticipate speech deterioration that interferes with communication. By anticipating speech deterioration, an assessment can be completed, technology recommended and purchased, and training completed before the PALS are unable to meet their communication needs through speech alone.
Through a series of research projects, Ball, Willis, Beukelman, & Pattee (2001) and Ball, Beukelman, and Pattee (2002, 2004) and Yorkston et al. (1993) demonstrated that for PALS, speaking rate tends to decrease before reductions in intelligibility of speech are observed. Among the PALS tracked by the authors using the Nebraska ALS Database, there is a tendency for sentence intelligibility, as measured by the Sentence Intelligibility Test (Yorkston, Beukelman, & Tice, 1996), to remain high during a period of initial reduction in speaking rate. The figure on p. 4 illustrates this trend for 243 separate data sessions of persons with spinal, bulbar, and mixed ALS.
For persons with bulbar onset ALS this period may be relatively brief; however, for persons with spinal ALS the gradual speaking rate reduction may be observed for an extended time. A rapid deterioration of intelligibility often occurs when speaking rate reaches approximately 45% to 65% (85-125 words per minute) of habitual rate for PALS with bulbar, spinal, or mixed type of ALS. Clinically, we monitor speaking rate during regular intervals. As speaking rate approaches 125 words per minute, we recommend an AAC assessment, even if sentence intelligibility may remain over 90%.
Monitoring Speaking Rate of Persons Who Cannot Regularly Attend Clinics
Some PALS are unable to regularly attend a clinic where their speaking rate can be monitored. In a recent clinical note (Ball et al., 2004), we described strategies to monitor speaking rate using the telephone. Speech samples produced by seven participants were audio-recorded simultaneously onsite and over a standard, analog telephone line. Results revealed that the mean speaking rates recorded over a phone line were within 0.5 words per minute of the samples recorded onsite.
These results support the use of speaking rate monitoring at a distance for PALS. In this study and in our clinical practice, speech intelligibility was not measured or monitored using telephone technology. The research team is currently studying the length of AAC technology use to determine factors that may influence ongoing use.
ALS References
Ball, L., Beukelman, D., & Pattee, G. (2002). Timing of speech deterioration in people with amyotrophic lateral sclerosis. Journal of Medical Speech-Language Pathology, 231–235.
Ball, L., Beukelman, D., & Pattee, G. (2002). Timing of speech deterioration in people with amyotrophic lateral sclerosis. Journal of Medical Speech-Language Pathology, 231–235.×
Ball, L., Beukelman, D., & Pattee, G. (2004). Augmentative and alternative communication acceptance by persons with amyotrophic lateral sclerosis. Augmentative and Alternative Communication, 20, 113–123. [Article]
Ball, L., Beukelman, D., & Pattee, G. (2004). Augmentative and alternative communication acceptance by persons with amyotrophic lateral sclerosis. Augmentative and Alternative Communication, 20, 113–123. [Article] ×
Ball, L., Beukelman, D., Ullman, C., Maassen, M., & Pattee, G. (2004-submitted). Monitoring speaking rate at a distance for persons with amyotrophic lateral sclerosis, Journal of Medical Speech-Language Pathology.
Ball, L., Beukelman, D., Ullman, C., Maassen, M., & Pattee, G. (2004-submitted). Monitoring speaking rate at a distance for persons with amyotrophic lateral sclerosis, Journal of Medical Speech-Language Pathology.×
Ball, L., Willis, A., Beukelman, D., & Pattee, G. (2001). A protocol for identification of early bulbar signs in amyotrophic lateral sclerosis. Journal of the Neurological Sciences, 191, 43–53. [Article] [PubMed]
Ball, L., Willis, A., Beukelman, D., & Pattee, G. (2001). A protocol for identification of early bulbar signs in amyotrophic lateral sclerosis. Journal of the Neurological Sciences, 191, 43–53. [Article] [PubMed]×
Fried-Oken, M., Fox, L., Rau, M., Tullman, J., Baker, G., Hindal, M., Wile, N., & Lou, J. (2004-submitted). Purposes of AAC device use for persons with ALS as reported by caregivers.
Fried-Oken, M., Fox, L., Rau, M., Tullman, J., Baker, G., Hindal, M., Wile, N., & Lou, J. (2004-submitted). Purposes of AAC device use for persons with ALS as reported by caregivers.×
Mathy, P., Yorkston, K., & Gutmann, M. (2000). AAC for individuals with amyotrophic lateral sclerosis. In Beukelman, D., Yorkston, K., & Reichle, J., (Eds.). Augmentative and alternative communication for adults with acquired neurologic disorders. Baltimore: Paul H. Brookes.
Mathy, P., Yorkston, K., & Gutmann, M. (2000). AAC for individuals with amyotrophic lateral sclerosis. In Beukelman, D., Yorkston, K., & Reichle, J., (Eds.). Augmentative and alternative communication for adults with acquired neurologic disorders. Baltimore: Paul H. Brookes.×
Sitver, M. & Kraat, A. (1982). Augmentative communication for the person with amyotrophic lateral sclerosis (ALS). ASHA, 24, 783.
Sitver, M. & Kraat, A. (1982). Augmentative communication for the person with amyotrophic lateral sclerosis (ALS). ASHA, 24, 783.×
Yorkston, K., Beukelman, D., & Tice, R. (1996). Sentence Intelligibility Test. Lincoln, NE: Institute for Rehabilitation Science and Engineering at Madonna Rehabilitation Hospital.
Yorkston, K., Beukelman, D., & Tice, R. (1996). Sentence Intelligibility Test. Lincoln, NE: Institute for Rehabilitation Science and Engineering at Madonna Rehabilitation Hospital.×
Yorkston, K., Strand, E., Miller, R., Hillel, A., & Smith, K. (1993). Speech deterioration in amyotrophic lateral sclerosis: Implications for the timing of intervention. Journal of Medical Speech-Language Pathology, 1, 35–46.
Yorkston, K., Strand, E., Miller, R., Hillel, A., & Smith, K. (1993). Speech deterioration in amyotrophic lateral sclerosis: Implications for the timing of intervention. Journal of Medical Speech-Language Pathology, 1, 35–46.×
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December 2004
Volume 9, Issue 22