Acquired Physical Disease

1. Acquired Physical Disease SLA G304 Kim Ho, PhD CCC-SLP

2. Overview • Hand back quizzes and papers • Two EBP Presentations BEFORE lecture • Lecture on Acquired Physical Diseases

3. Model of Assessment and Intervention • Communication Needs Model • Appropriate if needs are defined • Participation and communication needs • Assessment of capabilities for communication options • Assessment of external constraints • Intervention staging for progressive disease • Evaluate Intervention Outcomes

4. Amyotrophic Lateral Sclerosis • Progressive degenerative disease involving motor neurons of brain and spinal cord • Incidence 0.4 to 1.8 cases per 100,000 • Average onset age is 56 years • Early symptom is weakness, which progresses to full dependency, and may occur with spasticity • ¼ people show bulbar (brain-stem) weakness via dysarthria and dysphagia • Rapid course • Live about 2.2 years • May be able to still walk/drive but unable to speak

5. ALS Cont’d • Most show spinal involvement • Little or no dysarthria or dysphagia initially • Extraocular muscles are usually spared, as is cognitive and language functioning • 14-39% die in 5 years of onset; 10% live 10 years; And few live up to 20 years • May be some perceptual impairments

6. AAC Assessment and ALS • Communication Symptoms • 75% are unable to speak at time of death • Identify Participation patterns and Communication needs • Assess environments/domains still participating • May require a portable, durable AAC system for work/community affairs • Home-centered have more stable communication needs • May use a movable or portable system • May use systems which require extensive facilitator support

7. Assessment and ALS Cont’d • Those with Bulbar (brain-stem) symptoms are often able to direct select • Example: alphabet board, w/ mobile arm support as disease progresses • Spinal ALS usually require scanning system to type • May use lateral head movement to activate switch

8. Clinical Examples ALS • Dan: Passey Muir valve with Dragon Dictate and EZ Keys scanning when fatigued • Switch, switch site and movement may be altered with progression • hand to head site, pressure switch to P-switch • Beth: light pointer to point to an image of the human body to indicate areas of pain • Used yes/no system to select 100 words for communication board • System vital for participating in counseling and communicating with family • Mike: in nursing home; used lip reading and eye gaze to PCS because aides since ESL

9. ALS Intervention Staging • No Detectable Speech Disorder • Obvious Speech Disorder With Intelligible Speech • Reduction in Speech Intelligibility • Residual Natural Speech and AAC • Loss of Useful Speech

10. Stage One: No Detectable Speech Disorder • Confirm speech is normal; answer questions • Allow for grieving, then educational stage • Begin to discuss AAC options

11. Stage Two: Obvious Speech Disorder With Intelligible Speech • Minimize environmental interference • Reduction in speech rate to increase intelligibility • When see 50% reduction which is about 95 words per minute, begin AAC Dx and Tx • Teach to establish topics and confirm partner comprehension • Consider voice amplification for speaking in groups

12. Stage Three: Reduction in Speech Intelligibility • If rate is not slowed, encourage slower rate to facilitate intelligibility • Reduce breath group rate to conserve energy • Breakdown repair strategies: rephrase, repeat utterance • Complete AAC assessment to use AAC system to repair BD • Work with caregivers and family to assess realistic expectations, and train

13. Stage Four: Residual Natural Speech and AAC • AAC moves from secondary to primary mode of communication • May use initial letter cueing to augment speech • SGD for telephone, conversing with unfamiliar partners

14. Stage Five: Loss of Useful Speech • Total reliance on AAC system • Develop functional unaided yes/no system • Eye-pointing useful means of access • Some will require ventilator support

15. Multiple Sclerosis • Degenerative disease of the white matter of the CNS • Plaques forms which destroy the myelin sheath of the CNS (axons preserved) • Prevalence: 1 in 1,000 • Ages: 10 to 50 in 95% of cases, median onset age of 27; “disease of young people” • 3:2 female-to-male ratio

16. Multiple Sclerosis Cont’d • Life expectancy about 35 years following onset • Symptoms include: • Spasticity in extremities • Loss of bladder control • Fatigue, weakness, ataxia, and tremor • May need a wheelchair for mobility • More than ½ have cognitive impairment (deficits in conceptualization and short-term memory)

17. Classes of MS 1. Relapsing and remitting • 70%; virtual full recovery in between relapse 2. Chronic progressive • More common for older adults at onset of MS • Motor and neurological signs and symptoms gradually worsen 3. Combined relapsing/remitting with chronic progression • Gradual deterioration of capabilities over time with periods of remission

18. Classes of MS Cont’d 4. Benign: • 20% of cases • Relatively normal life span with little or no progression of disease 5. Malignant • 5-10% of young people show rapid and extensive progression in cognitive, cerebellar, and pyramidal systems and death

19. AAC Assessment for MS Identify Participation patterns and Communication needs • Dysarthria is common, but not universal • Many individuals with MS are not aware of the severity of their speech impairment • Most do not require AAC systems • Beukelman et al. (1985) surveyed 656 respondents with MS and found only 4% used AAC • Since later onset, typically finished education, have begun career

20. AAC Assessment for MS Cont’d • If have AAC needs, likely no longer working due to severity of impairments and medical needs • May live in residential or nursing homes due to level of care • Communication needs tend to be conversational and writing

21. One evening . . . I had gone to the bathroom for a shower . . All was well as I entered the bathroom and showered. Then I began to wheel myself to the bedroom after I had finished. I tried to say something to my wife as I neared the door, but the words would not come out and all I could manage was a babbling as I tried to express myself. My wife said to me, “What did you do, flush your voice down the drain?” Now this is not a real funny line. However, under those circumstances, it sounded hilarious. We both burst into laughter . . . My voice control did not return for a few days. . .(but it) did return. (A man with MS, in Michael, 1981, p. 27).

22. Assess Specific Capabilities MS • Visual problems are common • Loss of central vision from optic neuritis, in 16-30% • Impacts AAC services and access to print • Assess short-term memory skills, try to build AAC system on old skills • Motor assessment • Intention tremor (occurs during or exaggerated by voluntary movement) limits switch placement • May attach switch to hand/body part so can move whole switch and still access with finger volitionally

23. Intervention Staging for MS • Use similar intervention staging to plan AAC intervention with MS • See text, p. 443-444

24. Guillain-Barre Syndrome (GBS) • Usually temporary condition • Acute onset, progressive destruction and regeneration of myelin sheath of peripheral nerve axons • Paralysis progresses bilaterally from lower to upper extremities • Maximal paralysis occurring at 1-3 weeks post-onset • Myelin sheath regenerates; nerve and muscular strength slowly returns

25. GBS Cont’d • About 85% individuals experience full recovery • Incidence of 1.7 per 100,000 people • 1/3 people require intubation on ventilator

26. Intervention Staging for GBS • Deterioration Phase • Loss of Speech • Prolonged Speechlessness • Spontaneous Recovery of Speech • Long-Term Residual Motor Speech Disorder

27. Stage One: Deterioration Phase • Dx usually before speech is impaired, but will occur quickly • Medical staff should monitor communication impairment • Educate individual and family on course and estimated loss of speech

28. Stage Two: Loss of Speech • Symptoms stabilize • If unable to speak due to respirator, provide reliable unaided yes/no system then an eye-pointing or eye-linking technique • May develop communication boards to support communication partners in: • visual and/or auditory scanning, yes/no questions, and eye-pointing procedures • Include social messages, health needs, alphabet and numbers for novel messages

29. Stage Three: Prolonged Speechlessness • May require weeks/months of AAC • Typically continues use of low-tech strategies • High-tech options may be discussed to allow for greater independence • Access usually limited to scanning, with eyelid or head movement • Spelling or alphabetically encoded messages

30. Stage Four: Spontaneous Recovery of Speech • Transition may take several weeks or months • May still require ventilator • oral-type electrolarynx • control sound with a head switch • May use AAC system to set topic, then natural speech • AAC system to repair breakdowns

31. Stage Five: Long-Term Residual Motor Speech Disorder • 15% have residual weakness after recovery and dysarthria • Maximize intelligibility and effectiveness of residual speech