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Patients with amyotrophic lateral sclerosis (ALS) often fear of dying from suffocation. This fear is also common in relatives and caregivers. Research has, however, shown that ALS patients seldom die from suffocation. More than 90% of all ALS patients die peacefully. Death is mostly preceded by a peracute decrease in consciousness due to hypercapnia caused by alveolar hypoventilation. Mechanical ventilation, especially at night, can reduce the symptoms caused by hypoventilation. However, little by little, the effectiveness of ventilation may decrease to such an extent that it is no longer useful or desirable. Termination of long-standing ventilation requires careful preparation. Intensive guidance of the relevant medical, practical and ethical aspects are necessary. Particularly in the pre-terminal and terminal phases, support given to an ALS patient requires a pro-active attitude on the part of the treating physician. To this end, physicians may seek advice from the Dutch ALS Center, a palliative care consultation team, a hospice physician or a center for home mechanical ventilation.

Our objective was to estimate the economic burden of patients with amyotrophic lateral sclerosis (ALS) and to examine the effect of treatment in a multidisciplinary ALS treatment centre versus general care on costs and to describe differences in costs according to clinical characteristics. In a cross-sectional study 208 patients with ALS and their caregivers were interviewed and were asked to fill in a cost diary for six months. Patients were divided in groups according to type of treatment they received and according to clinical characteristics (site of onset, severity). The direct healthcare and non-healthcare costs were calculated according to Dutch guidelines for cost analysis in healthcare research, standardized for the year 2003. Ninety-eight patients were included when they had filled in the cost diary for at least three months and were affected for three years or less. Mean monthly costs were 1336 euros for the group receiving multidisciplinary care and 1271 euros for those receiving general care. This study shows that the costs of multidisciplinary ALS care were practically identical to the costs of general care. Earlier study showed that patients receiving multidisciplinary care had a better quality of life; therefore, the present study encourages the formation of multidisciplinary teams of professionals specialized in ALS care to further improve standards of care and QoL of patients suffering from ALS.

Symptoms of nocturnal hypoventilation may negatively influence the quality of life (QoL) of ALS patients long before respiratory failure ensues. Non-invasive mechanical ventilation (NIV) is considered a treatment option for nocturnal hypoventilation. The primary objective of NIV is improving quality of life (QoL). It may also prolong life by several months. A systematic review of the literature was performed to analyse what is known of the effect of NIV on survival, QoL and other outcome measures. A computerized literature search was performed to identify controlled clinical trials and observational studies of treatment of ALS-associated nocturnal hypoventilation from 1985 until May 2005. Twelve studies fulfilled the inclusion criteria. Four studies were retrospective, seven prospective and in one study randomization was used. All studies reported beneficial effects of NIV on all outcome measures. In seven studies NIV was associated with prolonged survival in patients tolerant for NIV, and five studies reported an improved QoL. In conclusion, studies on the use of NIV in ALS differ in study design and endpoint definitions. All studies suggest a beneficial effect on QoL and other outcome measures (Evidence level Class II-III). Well-designed randomized controlled trials comparing the effect on QoL and survival have not been performed.

Background: In the Netherlands, rehabilitation medicine plays an important role in the symptomatic and palliative treatment of ALS patients. Detailed information about the actual care of ALS patients in the Netherlands and about the attitude of consultants in rehabilitation medicine towards the management of this disease was lacking., Objective: To obtain detailed information about the rehabilitation care for patients with ALS in the Netherlands., Methods: We have performed a survey among all consultants in rehabilitation medicine in the Netherlands, using a questionnaire about the organisation of care and the care management of ALS patients., Results: Two hundred eighty one questionnaires were gathered with a response rate of 98%. There were 14 specialised ALS centres spread throughout the country, except in the northwest and southwest. Most consultants worked with an ALS multidisciplinary team and most patients were treated in an outpatient rehabilitation clinic. Follow up visits were performed in most cases 5-6 times per year. The majority of the patients were followed up until death. The Dutch protocol for rehabilitative management in ALS was used in 89% of all treated ALS patients. Follow up and care management was not different in the specialised centres compared with the non-specialised centres., Conclusion: In conclusion, this study indicated that the actual care for ALS patients was reasonably well organised in the Netherlands, based on the results and reactions of the consultants in rehabilitation medicine.