**1. Introduction**

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.

Multidisciplinary Rehabilitation in Amyotrophic Lateral Sclerosis 559

Neurologist

Patient / Carer

Family

Psychiatrist Respiratory

Fig. 2. The multidisciplinary rehabilitation team in ALS (adapted from (Hardiman, 2007))

A proposed model for service interaction in caring for persons with ALS shows involvement of neurologists and palliative care teams in the acute and terminal phases of care, with a relatively smaller role for rehabilitation physicians. However rehabilitation plays a major role in long-term care and support (over years) in the more slowly progressive phase (Royal College of Physicians National Council for Palliative Care and British Society of Rehabilitation Medicine, 2008). Early rehabilitation intervention and treatment has much to contribute to improve health and quality of life prior to accumulation of disability through symptomatic and supportive therapies to enhance functional independence and community integration and reduce barriers (such as lack of knowledge about treatment, economic constraints) (Kemp, 2005). Disability management in ALS should also be planned, with deficits should be anticipated (over time) to avoid ''crisis management''. As patients deteriorate the rehabilitation and palliative care approaches can overlap, i.e. ''neuropalliative rehabilitation". Key skills in neuropalliative rehabilitation include: understanding disease progression, symptom control,

physician

MND association

ALS

Palliative Care service

Radiologist or gastroenterologist

Physiotherapist

Occupational Therapist

Dietician

MND Nurse

ALS Nurse

Rehabilitation physician

Spiritual counsellor (chaplains)

Neuropsychologist

Orthotist

Social Worker

> Speech and Language therapist

> > Psychologist

Rehabilitation is defined as ''a problem solving educational process aimed at reducing disability and increasing participation experienced by someone as a result of disease or injury'' (Wade, 1992). Although it is sometimes effective in reducing impairment, its principal focus is to reduce symptoms and limitations at the level of activity and participation, through holistic interventions, which incorporate personal and environmental factors. The multidisciplinary rehabilitation team (see Figure 2) comprises of a group of clinical professionals with expertise in ALS, directed by a physician, who work as an integrated unit to provide seamless care which is patient-centred, flexible and responsive to the evolving nature of the condition (Hardiman, 2007). The role of multidisciplinary rehabilitation in ALS is supported by a recent Cochrane review (Ng et al., 2009) which suggested some advantage for quality of life without increasing healthcare costs, reduced hospitalisation and improved disability with conflicting evidence for survival.

Fig. 1. The interaction between the various domains of the International Classification of Functioning, Disability and Health (adapted from (World Health Organization, 2001))

Rehabilitation is defined as ''a problem solving educational process aimed at reducing disability and increasing participation experienced by someone as a result of disease or injury'' (Wade, 1992). Although it is sometimes effective in reducing impairment, its principal focus is to reduce symptoms and limitations at the level of activity and participation, through holistic interventions, which incorporate personal and environmental factors. The multidisciplinary rehabilitation team (see Figure 2) comprises of a group of clinical professionals with expertise in ALS, directed by a physician, who work as an integrated unit to provide seamless care which is patient-centred, flexible and responsive to the evolving nature of the condition (Hardiman, 2007). The role of multidisciplinary rehabilitation in ALS is supported by a recent Cochrane review (Ng et al., 2009) which suggested some advantage for quality of life without increasing healthcare costs, reduced

hospitalisation and improved disability with conflicting evidence for survival.

ALS

Fig. 1. The interaction between the various domains of the International Classification of Functioning, Disability and Health (adapted from (World Health Organization, 2001))

Fig. 2. The multidisciplinary rehabilitation team in ALS (adapted from (Hardiman, 2007))

A proposed model for service interaction in caring for persons with ALS shows involvement of neurologists and palliative care teams in the acute and terminal phases of care, with a relatively smaller role for rehabilitation physicians. However rehabilitation plays a major role in long-term care and support (over years) in the more slowly progressive phase (Royal College of Physicians National Council for Palliative Care and British Society of Rehabilitation Medicine, 2008). Early rehabilitation intervention and treatment has much to contribute to improve health and quality of life prior to accumulation of disability through symptomatic and supportive therapies to enhance functional independence and community integration and reduce barriers (such as lack of knowledge about treatment, economic constraints) (Kemp, 2005). Disability management in ALS should also be planned, with deficits should be anticipated (over time) to avoid ''crisis management''. As patients deteriorate the rehabilitation and palliative care approaches can overlap, i.e. ''neuropalliative rehabilitation". Key skills in neuropalliative rehabilitation include: understanding disease progression, symptom control,

Multidisciplinary Rehabilitation in Amyotrophic Lateral Sclerosis 561

appropriate especially in those with bulbar weakness (no mouthpiece) and may be more sensitive to changes in diaphragmatic and respiratory muscle strength (Stefanutti et al., 2000;Lyall et al., 2001). It is also more reliably recorded in the later stages of ALS (Morgan et

Initial management can include chest physiotherapy and postural drainage, especially if the patient has difficulty clearing secretions from the chest (Shaw, 2003). A suction machine may also be helpful. Preventing respiratory infections is a primary goal and pneumococcal and influenza vaccines should be administered. Respiratory muscle exercise can be

Non-invasive ventilation (NIV) should be considered in respiratory dysfunction (see Figure 3) especially for nocturnal symptomatic respiratory compromise. A recent Cochrane review concluded that NIV significantly improves quality of life when tolerated and may prolong survival in those with normal to moderately impaired bulbar function especially if used for 4 hours/day (Radunovic et al., 2009). Successful use of NIV is dependent on respiratory therapists and patients working closely and patiently through the adjustment phase of NIV, especially with selection and tolerance of face masks. A small dose of anxiolytic may assist with the process in select patients. Bulbar involvement and executive dysfunction may also

Invasive ventilation should be offered when longer-term survival is the goal. Counselling is necessary with regards to benefits and burden (expense, intensive physical support with suctioning and nursing care, high caregiver burden) as many may not be able to manage invasive ventilation at home, thus requiring nursing home placement (Kaub-Wittemer et al., 2003;Miller et al., 2009a). There is evidence however that the 10-20% of persons with ALS who undergo invasive ventilation (including those administered at the time of acute respiratory failure without advance discussion) appear to have good acceptance and

Dysarthria is common as a result of bulbar involvement and is often a source of significant frustration to the persons with ALS and their families. Early changes include nasality or reduced vocal volume and changes in oral movement rates and speech rates (Yorkston et al., 1993). As weakness and spasticity of the oral and laryngeal muscles increase, imprecise consonant production, hypernasality, harsh vocal quality, slowed rate of speech and breath volumes affect intelligibility (Hillel and Miller, 1989). Speech pathologists can teach the patient to slow speech rate, exaggerate articulation and improve respiratory efficiency through phrasing (Francis et al., 1999). Palatal lift and palatal augmentation prostheses may also be of some use to reduce the hypernasal aspect of dysarthria (Esposito et al., 2000). As intelligibility in ALS worsens, Augmentative and Alternative Communication (AAC) is required. AACs can improve quality of life by optimising function and assisting with decision making (Brownlee and Palovcak, 2007). AACs range from no or low technology (gestures, communication boards with letters) to high-tech electronic communication devices that allow the user to have voice output (Brownlee and Palovcak, 2007). For example, speech-generating devices such as LightWRITERs are commonly used. These devices can be used as long as there is voluntary motor movement (including eye gaze). The specific access method depends on the abilities of the patient – for example, pointing with a body part or pointer, adapted mice or joysticks or switches and scanning technology can be used. For those who have no voluntary

instituted and may delay the onset of ventilatory failure (Schiffman, 1996).

reduce compliance (Miller et al., 2009a).

satisfactory quality of life (Vianello et al., 2010).

**2.2 Communication** 

al., 2005).

managing expectations, issues relating to communication, addressing end of life issues, legal issues (mental capacity, wills), specialist interventions (ventilation), equipment needs, counselling and support, and welfare advice (Royal College of Physicians National Council for Palliative Care and British Society of Rehabilitation Medicine, 2008).

The literature presented in this review includes all levels of evidence for multidisciplinary rehabilitation of ALS (including randomised and clinical controlled trials, case studies and expert opinion).
