End-of-Life Care of Patients With Amyotrophic Lateral Sclerosis and Other Nonmalignant Diseases

To systematically review evidence bearing on the management of patients with amyotrophic lateral sclerosis (ALS). The authors analyzed studies from 1998 to 2007 to update the 1999 practice parameter. Topics covered in this section include slowing disease progression, nutrition, and respiratory management for patients with ALS. The authors identified 8 Class I studies, 5 Class II studies, and 43 Class III studies in ALS. Important treatments are available for patients with ALS that are underutilized. Noninvasive ventilation (NIV), percutaneous endoscopic gastrostomy (PEG), and riluzole are particularly important and have the best evidence. More studies are needed to examine the best tests of respiratory function in ALS, as well as the optimal time for starting PEG, the impact of PEG on quality of life and survival, and the effect of vitamins and supplements on ALS. Riluzole should be offered to slow disease progression (Level A). PEG should be considered to stabilize weight and to prolong survival in patients with ALS (Level B). NIV should be considered to treat respiratory insufficiency in order to lengthen survival (Level B) and to slow the decline of forced vital capacity (Level B). NIV may be considered to improve quality of life (Level C) [corrected].Early initiation of NIV may increase compliance (Level C), and insufflation/exsufflation may be considered to help clear secretions (Level C).

As the incidence and mortality rates of motor neuron diseases (MNDs) have been reported to increase steadily over the 1950s-1980s decades, we compared the results of the most recent studies (1990s decade) with the ones reported for those earlier periods. The relevant literature was retrieved on a keyword basis from online medical and official death statistics databases. Fifteen European and North American studies were analyzed, for comparison with the results reported in review papers. The 1990s incidence and mortality rates of MND average at 1.89 per 100,000/year and 1.91 per 100,000/year, respectively, thus yielding increases of 46% and 57% over the 1960s-1970s decades, respectively. This increase appears mainly due to Southern Europe countries, to female gender and to patients aged 75 years and over.Thus, the results of this analysis (i) confirm that the incidence of, and mortality from, MNDs continued to increase during the 1990s and, (ii) suggest that this increase could be partly due to increased life expectancy. Other factors might also contribute, such as better diagnosis since El Escorial criteria, and better accuracy of death certificate collection.However, a real increase in the incidence of MNDs, possibly related to environmental factors, cannot be excluded.

Most health-care professionals are trained to promote and maintain life and often have difficulty when faced with the often rapid decline and death of people with terminal illnesses such as amyotrophic lateral sclerosis (ALS). By contrast, data suggest that early and open discussion of end-of-life issues with patients and families allows time for reflection and planning, can obviate the introduction of unwanted interventions or procedures, can provide reassurance, and can alleviate fear. Patients' perspectives regarding end-of-life interventions and use of technologies might differ from those of the health professionals involved in their care, and health-care professionals should recognise this and respect the patient's autonomy. Advance care directives can preserve autonomy, but their legal validity and use varies between countries. Clinical management of the end of life should aim to maximise quality of life of both the patient and caregiver and, when possible, incorporate appropriate palliation of distressing physical, psychosocial, and existential distress. Training of health-care professionals should include the development of communication skills that help to sensitively manage the inevitability of death. The emotional burden for health-care professionals caring for people with terminal neurological disease should be recognised, with structures and procedures developed to address compassion, fatigue, and the moral and ethical challenges related to providing end-of-life care.