Multidisciplinary Rehabilitation in Amyotrophic Lateral Sclerosis

Abstract

Amyotrophic Lateral Sclerosis (ALS) is the most common chronic neurodegenerative disorder of the motor system in adults. It is a relatively rare disease with a reported population incidence of between 1.5 and 2.5 per 100,000 per year worldwide and a gender ratio of 3:2 men: women. Amyotrophic Lateral Sclerosis is characterized by the loss of motor neurons in the cortex, brain stem, and spinal cord, manifested by upper and lower motor neuron signs and symptoms affecting bulbar, limb, and respiratory muscles. Death usually results from respiratory failure and follows on average two to four years after onset, but some may survive for a decade or more. Amyotrophic Lateral Sclerosis is a devastating condition with unknown aetiology and no current cure. The symptoms in ALS are diverse and challenging and include weakness, spasticity, limitations in mobility and activities of daily living, communication deficits and dysphagia, and in those with bulbar involvement, respiratory compromise, fatigue and sleep disorders, pain and psychosocial distress. The International Classification of Functioning, Disability and Health (ICF) (World Health Organization, 2001), defines a common language for describing the impact of disease at different levels: impairment (body structure and function), limitation in activity and participation (see Figure 1). Within this framework ALS related impairments (weakness, spasticity), can limit ‘‘activity” or function (decreased mobility, self-care, pain) and ‘‘participation” (driving, employment, family, social reintegration). ‘‘Contextual factors’‘, such as environmental (extrinsic) and personal factors (intrinsic) interact with all the other constructs to shape the impact of ALS on patients and their families. The impact of ALS upon patients, their caregivers (often family members) and on society is substantial, often beginning long before the actual diagnosis is made, and increasing with increasing disability and the need for medical equipment and assisted care (Klein and Forshew, 1996). Given the broad spectrum of needs, current management spans from diagnosis (acute neurological needs) through to symptomatic and supportive rehabilitation and palliative care. The interface between neurology, rehabilitation and palliative care is of utmost importance to ensure co-ordinated care for persons with ALS rather than duplicating services (Royal College of Physicians National Council for Palliative Care and British Society of Rehabilitation Medicine, 2008). It should be noted however that the focus of this chapter is on the rehabilitation phases, hence discussion of acute neurological and palliative care aspects are limited.