Objective justification

This systematic review comprises of an objective appraisal of the evidence in regard to the management of respiratory insufficiency for patients with motor neurone disease (MND), in particular amyotrophic lateral sclerosis (ALS). The primary aim is to establish evidence-based and patient and carer centred guidelines, with secondary aims of generating new research questions to be addressed in future studies and identifying areas where further research is needed. Respiration requires the normal function of three muscle groups: the muscles of inspiration, the muscles involved in expiration and the muscles that control the upper airway. As motor neurone disease progresses in a patient, a weakness or impairment of the inspiratory and expiratory muscles will develop. Inspiratory muscle weakness has been identified as the primary determinant of ventilatory failure and respiratory symptoms (Polkey et al, 1998). Expiratory muscle strength does not appear to affect ventilation directly but does result in the loss of cough or inability to clear secretions from the lungs, thus leading to a higher risk of atelectasis and lower respiratory tract infections (Sherman & Paz, 1994). The weakening of respiratory muscles is a significant indicator of survival and a progressive worsening of respiratory muscle weakness can lead to respiratory insufficiency and a diminished quality of life (Bourke et al, 2001). Such morbidity includes difficulties in breathing, morning headaches, weak cough, aspiration, breathing disturbances during sleep, chronic nocturnal hypoventilation, and chest infections. Most patients with MND/ALS will develop symptoms of respiratory insufficiency during the course of their disease and respiratory failure is frequently an urgent medical problem either as a first symptom or later in the course of the disease (Bromberg et al, 1996). Respiratory failure is also responsible for the majority of deaths from MND/ALS (Borasio, Gelinas & Yanagisawa, 1998). Since systems of respiratory insufficiency compromise the quality of life of MND/ALS patients, clinical management is necessary. In practice, the clinical management of MND/ALS patients in Europe tends to be largely based on clinical experience and on 'expert opinions' (Borasio et al, 2001). Surveys on standards of care for the management of respiratory failure in MND/ALS patients report varying levels of provision of care in western countries. Borasio et al (2000) in their survey on standards of clinical management of palliative care for MND/ALS patients in Europe found that 43% of centres regularly checked respiratory function using FVC, 90% routinely discussed respiratory issues. Bourke et al (2002) also found a …