**2.1 Respiratory dysfunction**

Most deaths in ALS are due to respiratory failure from respiratory muscle weakness, hence the diagnosis and management of respiratory symptoms is important (Figure 3) (Miller et al., 2009a). Counselling may be initiated at the time of diagnosis especially if respiratory symptoms are present and/or forced vital capacity (FVC) is <60% of predicted. Early symptoms may be suggestive of nocturnal hypoventilation (eg. frequent arousals, morning headaches, excessive daytime sleepiness, vivid dreams) rather than overt dyspnoea (Miller et al., 2009a). It is important to discuss the options of respiratory choices, including tracheostomy and ventilatory support well before these are clinically indicated to enable advance planning or directives. It is also important to offer patients information about the terminal stages of ALS and reassure regarding terminal hypercapnoeic coma and resulting peaceful death, as many may fear ''choking to death'' (Borasio et al., 2001b).

Respiratory function should be evaluated every three months from the time of diagnosis. Whilst FVC is the most commonly used (Melo et al., 1999) and significantly predicts survival (Czaplinski et al., 2006), it can be insensitive to slight changes in muscle strength (Fitting et al., 1999). The maximal sniff nasal inspiratory force (sniff nasal pressure) may be more 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 al., 2005).

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

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

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

### **2.2 Communication**

560 Amyotrophic Lateral Sclerosis

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

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

Amyotrophic Lateral Sclerosis is a fatal disease with a challenging progressive course that results in a broad and ever-changing spectrum of care needs. Symptoms are varied (see Table 1) and need to be carefully assessed and managed. The timing of provision of appropriate care is important as whilst information needs to be provided when patients are psychologically in the right frame of mind, the options of certain interventions may be time-

Table 1. Symptoms experienced by ALS patients (adapted from (Oliver, 1996))

peaceful death, as many may fear ''choking to death'' (Borasio et al., 2001b).

Most deaths in ALS are due to respiratory failure from respiratory muscle weakness, hence the diagnosis and management of respiratory symptoms is important (Figure 3) (Miller et al., 2009a). Counselling may be initiated at the time of diagnosis especially if respiratory symptoms are present and/or forced vital capacity (FVC) is <60% of predicted. Early symptoms may be suggestive of nocturnal hypoventilation (eg. frequent arousals, morning headaches, excessive daytime sleepiness, vivid dreams) rather than overt dyspnoea (Miller et al., 2009a). It is important to discuss the options of respiratory choices, including tracheostomy and ventilatory support well before these are clinically indicated to enable advance planning or directives. It is also important to offer patients information about the terminal stages of ALS and reassure regarding terminal hypercapnoeic coma and resulting

Respiratory function should be evaluated every three months from the time of diagnosis. Whilst FVC is the most commonly used (Melo et al., 1999) and significantly predicts survival (Czaplinski et al., 2006), it can be insensitive to slight changes in muscle strength (Fitting et al., 1999). The maximal sniff nasal inspiratory force (sniff nasal pressure) may be more

Palliative Care and British Society of Rehabilitation Medicine, 2008).

expert opinion).

**2. Rehabilitation issues in ALS** 

limited as the disease continues to progress.

**2.1 Respiratory dysfunction** 

Weakness 94% Dysphagia 90% Dyspnoea 85% Pain 73% Weight loss 71% Speech issues 71% Constipation 54% Cough 48% Sleep issues 29% Emotional lability 27% Drooling 25%

> 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

Multidisciplinary Rehabilitation in Amyotrophic Lateral Sclerosis 563

A source of significant frustration for those with speech difficulties is use of the telephone. Technology is available and varies from country to country. In the United States, ''Speech to Speech" technology can be used, where trained communication assistants are used by the patient to complete phone calls. They are trained to use superior equipment to hear the caller and place the call, then repeat verbatim what the caller says so the call is completed

Dysphagia affects a third of persons with ALS at onset and the majority by late disease (Higo et al., 2004). It increases the risk of suboptimal caloric and fluid intake and can worsen weakness and fatigue (Borasio, 2001). Aspiration pneumonia (13%) is a contributor to respiratory complications and is associated with increased mortality with mean survival

Difficulties in the oral preparatory stage of swallowing (preparation of food for propulsion to the pharynx) is common (Mayberry and Atkinson, 1986). Symptoms include jaw weakness, fatigue, drooling, choking on food and slow eating. In addition, loss of upper limb function and fear of choking or depression can further impact on self-feeding abilities and oral intake (Slowie et al., 1983). A speech pathologist can evaluate the degree of dysphagia through bed-side assessments and/or further imaging (eg videofluroscopy). Mild dysphagia can be managed with specific interventions such a alteration of food consistency, upright positioning, small bolus size, soft collar for neck extensor weakness and the chintuck technique, in which the person flexes their neck to the anterior chest wall as they swallow, narrowing the inlet to the larynx and reducing the chance of food aspiration. Dieticians monitor nutritional status through body weight, percentage weight loss and body mass index. Common advice includes high calorie diets, texture modification and prescription of nutritional supplements (Rio and Cawadias, 2007). Patients may show nutritional compromise even before bulbar symptoms become significant (Slowie et al., 1983) as in addition to muscle wasting, persons with ALS at all stages of disease often do not meet their energy requirements (Kasarskis and Neville, 1996). Dehydration is also a common and important problem contributing to fatigue and thickened secretions (Francis et

As dysphagia progresses, evidence (Level B) suggests a percutaneous endoscopic gastrostomy (PEG) or equivalent (eg. radiologically inserted gastrostomy) is indicated to supplement oral intake (as long as this remains safe) for weight maintenance (Loser et al., 2005). PEGs prolong survival but there is currently little evidence regarding the impact of PEG on quality of life (Langmore et al., 2006). Timing of a PEG can be challenging. Indicators may include weight loss (5-10% of body weight loss implies nutritional risk (Francis et al., 1999)) and reduced FVC. If FVC falls below 50% of predicted (Kasarskis et al., 1999), risks of largyngeal spasm, localised infection, gastric haemorrhage, technical difficulties of PEG placement and respiratory arrest increase (Mazzini et al., 1995;Mathus-

Sialorrhoea can be a significant issue in ALS and is generally not related to increased saliva production but rather to impaired ability to swallow saliva, combined with facial weakness causing labial incompetence and neck weakness causing the head to tip forward (Francis et al., 1999). Improved positioning, use of a cervical collar and orolingual exercises may be helpful. Medications such as anticholinergics and tricyclics can also be trialled (Schiffman

successfully .

al., 1999).

Vliegen et al., 1994)).

**2.3 Swallowing and nutrition** 

time post-infection of 2 months (Sorenson et al., 2007).

motor control for communication, a recent case study using a brain-computer interface system has been reported and appears promising (Sellers et al., 2010). The emotional aspect of using an alternative form of communication however can result in significant patient resistance and acceptance as the ability to speak and use language is what distinguishes us from all other species (Pinker and Jackendoff, 2005;Brownlee and Palovcak, 2007). Hence, acceptance of an AAC may take weeks to months.

A source of significant frustration for those with speech difficulties is use of the telephone. Technology is available and varies from country to country. In the United States, ''Speech to Speech" technology can be used, where trained communication assistants are used by the patient to complete phone calls. They are trained to use superior equipment to hear the caller and place the call, then repeat verbatim what the caller says so the call is completed successfully .
