**4. References**

570 Amyotrophic Lateral Sclerosis

assistance, feeding tubes) and terminal palliative care whilst considering issues relating to quality of life, burden of therapies, their own wishes and those of their family. It is important that clinicians caring for ALS patients and their families appreciate and communicate the significance of life-threatening symptoms, monitor decision-making capacity, ensure that multiple possible end of life scenarios are anticipated and managed with all options provided (including hospice care), review advance care directives and

Medications should be available for all patients who are deteriorating and may be approaching the terminal phase, although the terminal phase may be difficult to recognise as there is usually slow deterioration until a quicker change leads to death within a few days or less (Oliver, 2007). Medications should include morphine to relieve dyspnoea and pain, midazolam to relieve distress and agitation and glycopyrronium bromide or hyoscine hydrobromide to reduce chest secretions, delivered parenterally (Oliver, 2007). Cultural and spiritual issues should also be addressed (Mitsumoto et al., 2005;Albert et al., 2007). Although many persons with ALS fear the terminal stages of ALS, with good palliative care, the later stages can be a time of fulfilment and peace for both persons with ALS and their

Bereavement in ALS occurs in both the patient and their family and continues, in families, after the death of the patient. Some families feel relieved of their caregiver burden and the burden of losses for the patient but also have feelings of guilt that they feel these emotions;

ALS is a complex and challenging condition with no cure. Current "gold-standard" management is ''multidisciplinary care''which includes neurological, rehabilitative and palliative care. As consistent with the guidelines from the American Academy of Neurology (Miller et al., 2009b) and the World Federation of Neurology (Andersen et al., 2007), multidisciplinary care should be available to all persons with ALS. Where multidisciplinary care is currently available, it should be delivered with a high level of coordination and integration, with evidence-based intervention to ensure holistic and seamless care for persons with ALS and their caregivers. Many areas in ALS are poorly understood, with research often further hindered by the logistical and ethical difficulties. Much more work is needed in the area of evidence-based interventions. At present, much of the evidence has been concentrated in areas such as respiratory and nutritional management. There is paucity of information on effective rehabilitation interventions and very little is understood with regards to the "black box of rehabilitation". For example, evidence to guide exercise prescription (such as strengthening, stretching, aerobic/endurance exercises) is much needed. The use and development of assistive technology is another area that warrants much more attention, as is a better understanding of bowel, bladder and sexuality issues. Further research is also needed into appropriate study designs; outcome measurement; the evaluation of optimal settings, type, intensity or frequency and cost-effectiveness of multidisciplinary care; and the different phases of ALS, covering the spectrum of care required for this patient population. The interface between neurological, rehabilitative and palliative components of care, and caregiver needs should be explored and developed to provide long-term support for this population. Last but not least, national and international guidelines incorporating evidence-based practice in rehabilitation should be

comprehensively consider and aggressively manage symptoms (McCluskey, 2007).

families (Oliver, 2007).

**3. Conclusion** 

hence support is vital in this area (Skyes, 2006).

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**25** 

*Italy* 

**Lateral Sclerosis** 

*Cliniche (BioNeC), University of Palermo, Palermo,* 

Daniele Lo Coco et al.\*

**Assessment and Management of Respiratory** 

*ALS Clinical Research Center, Dipartimento di Biomedicina Sperimentale e Neuroscienze* 

Amyotrophic Lateral Sclerosis (ALS) is a relatively rare neurodegenerative disorder that causes progressive dysfunction of voluntary muscle groups secondary to motor neurons death. The relentless involvement of all skeletal muscles of the body, characterized by weakness and atrophy to complete paralysis, invariably involves respiratory muscles (particularly the diaphragm) resulting in a failure to deliver adequate amounts of oxygen to, and remove carbon dioxide from blood. As a result, respiratory failure, frequently complicated by pneumonia related to respiratory muscle weakness and ineffective cough, is

Considering the natural history of ALS, only a few number of patients shows respiratory muscle dysfunction at the onset of the disease (Marti-Fabregas et al., 1995; De Carvalho et al., 1996), and the majority of patients maintains an almost normal pulmonary function for months or years. Patients thus need to be regularly and progressively evaluated to identify early signs of respiratory muscle weakness so that adequate treatment can be implemented. Indeed, in the last few years it has been repeatedly shown that non-invasive positivepressure ventilation (NIPPV), the treatment of choice for chronic hypoventilation and respiratory failure in ALS, allows a significant improvement in survival and quality of life (Heiman-Patterson & Miller, 2006). Many tests are available to objectively assess the performances of the respiratory system, and there is increasing interest toward those able to sensitively detect mild impairment. Moreover, great attention has to be put on monitoring of cough effectiveness, management of respiratory secretions and prevention of respiratory infections. For all these reasons the management of respiratory dysfunction has become a

Paolo Volanti2, Domenico De Cicco2, Antonio Spanevello3, Gianluca Battaglia2, Santino Marchese4,

*1ALS Clinical Research Center, Dipartimento di Biomedicina Sperimentale e Neuroscienze Cliniche (BioNeC),* 

Alfonsa Claudia Taiello1, Rossella Spataro1 and Vincenzo La Bella1

*2Neurorehabilitation Unit, Fondazione Salvatore Maugeri, Mistretta (ME), Italy 3Università Degli Studi dell'Insubria, Varese, Italy 4Respiratory Intensive Care Unit, Ospedale Civico ARNAS, Palermo, Italy* 

*University of Palermo, Palermo, Italy* 

the most frequent cause of death in these patients (Lo Coco et al., 2008).

**1. Introduction** 

 \*

**Dysfunction in Patients with Amyotrophic** 

