Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that selectively affects motor neurons. To date, there is no cure for ALS. It has been widely demonstrated how the use of AT can increase the independence and safety of patients improving their quality of life. Interventions based on the use of AT consist of aids to support residual capacities, increased autonomy and control of oneself and one's life, increased interactivity with the surrounding environment, increased participation in family and social life, maintaining a dignified standard of living, and at the same time decreasing the workload of the caregiver. The aim of the chapter is to provide an overview of the latest empirical evidence available on the use of AT-based programs for ALS people. Empirical data have demonstrated the effectiveness and adequacy of AT interventions. In conclusion, AT-based rehabilitation programs can be useful to promote the independence and quality of life of individuals with ALS.
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Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterised by progressive muscular paralysis reflecting degeneration of motor neurones in the primary motor cortex, corticospinal tracts, brainstem and spinal cord (Wijesekera & Leigh, 2009). Upper motor neurons degeneration leads to an increased muscle stiffness (spasticity) and impaired fine movements. Lower motor neurons degeneration leads to wasting and weakness, often with prominent muscle twitching (fasciculations) and reduced or absent deep tendon reflexes (Andersen, Borasio, & Dengler, 2007). In ALS, motor neurons innervating all the voluntary muscles are affected, with the exception of eye muscles, which are seldom involved, and the bladder, which may be affected late in the illness. Involvement of lower cranial nerves, innervating the tongue and swallowing apparatus leads to difficulty with speech and in swallowing. When the disease affects this region, it is considered to be ‘bulbar’ in onset. Spinal onset disease refers to development of the first symptoms in the arms or legs (Meireles & Al-Chalabi, 2009). Therefore, ALS has a rapidly progressing that (a) typically strikes people between 40 and 60 years of age and (b) causes muscle atrophy, spasticity, dysarthria (anarthria), and respiratory problems (failure), and, eventually, death (Ahmed & Wicklund, 2011; Chio` et al., 2011). Death generally occurs 3–6 years after the diagnosis of the disease and is primarily brought about by infections and respiratory failures (Carlesi et al., 2011). Pharmacological interventions to improve this condition or slow down its progression seem to be limited to the use of anti-excitotoxic compounds such as riluzole (Carlesi et al., 2011; Cifra, Nani, & Nistri, 2011) and possibly the use of antioxidants (Bach, Gonzalez, Sharma, Swan, & Patel, 2010).
Persons with extensive neuro-motor impairments and lack of speech, following severe neurological damages, remain largely dependent on caregivers for all daily functions including leisure, occupation, and communication (Aoun, McConingley, Abernethy, & Currow, 2010). Any effort to help individuals with ASL to acquire some level of independent functioning requires the support of specific technology (Bauer & Elsaesser, 2012; Borg, Larson, & Ostergren, 2011). Behavioral interventions are considered to be critical to avoid isolation, lack of environmental engagement, and of communication activity (Brownlee & Palovcak, 2007; Gruis, Wren, & Huggins, 2011). Among behavioral interventions, assistive technology-based programs (AT) are increasingly conceived crucial to support independent leisure engagement and communication opportunities (Beukelman, Ball, & Fager, 2007). AT options are broadly recognized as essential means of support for individuals with neurodegenerative diseases, acquired brain injuries, and multiple disabilities (Mishra & Banerjea, 2020). Thus, AT-based programs are commonly planned to favourably fill the gap between behavioral/cognitive skills and environmental requests (Bertelli, et al., 2016). That is, an AT setup is conceived to build functional bridges between users, environment, and technology. Essentially, it ensures that a helpful interaction (i.e., purposeful behavior and goal-oriented) is achieved for persons with extensive motor delays (Papais, et al., 2020). That interaction is critical to enhance personal fulfilment, social image, active role, satisfaction, and improving quality of life accordingly (Troncoso-Escudero, 2020).
For example, a computer-aided program presenting brief samples of environmental and social stimuli might be used together with a microswitch device to enable passive/isolated persons with extensive neuro-motor impairment and lack of speech to manage positive leisure engagement (Lancioni, Singh, O’Reilly, Sigafoos, Buonocunto, et al., 2011). Indeed, they could trigger the microswitch through a minimal response (e.g., minimal head movement) and, through this response, choose among the aforementioned stimuli and activate those that they prefer. Computer-aided telephone technology operated via a microswitch might enable persons with minimal response repertoire and lack of speech to place phone calls to relevant partners and have a basic contact and communication with distant persons. The availability of a series of words and phrases that the persons could activate via the microswitch to ask the partners about specific topics (e.g., activities performed during the day) or answer their questions could largely improve the communication value of the call (Lancioni, Singh, O’Reilly, Sigafoos, Ricciuti, et al., 2013).