Management of patients with amyotrophic lateral sclerosis

Standard recommendations for the clinical management of patient with ALS have been edited in recent years. These documents emphasise the importance of patient’s autonomy. Aim of study: To measure how these different recommendations can be applied in the context of a general hospital without a specific ALS clinic. Methods: Review of medical records of 21 pa-tients with an ALS diagnosis treated by the Uni-versity Hospitals Geneva who died from 1996– 2002. Results: Patients suffered from distressing symptoms during their last hospitalisation. Artifi-cial nutrition was given to 5 patients. Six patients had non invasive ventilation (NIV). Written ad-vance directives were only available in 2 cases. Dis-cussions about theses issues were also conducted late in the evolution of the disease. Conclusion: Some discrepancies between our daily practice and the existing recommendations exist, particularly regarding the key issues of arti-ficial nutrition and ventilatory support. Key words: amyotrophic lateral sclerosis; palliative care; advance care planning Amyotrophic lateral sclerosis is the most com-mon degenerative motor neuron disorder occur-ring in adult life. It’s prevalence has been estimated at 6–8/105, with an incidence of 2/105 per year [1]. To date, no curative treatment exists for ALS and symptomatic care must be provided ab initio [2–4]. Recommendations for the clinical manage-ment of patients with ALS and their families have been reviewed in recent years by a subcommittee of the American Academy of Neurology (AAN ALS practice parameters) [5]. The aim of the study was to better characterise the end-of-life of patients with ALS and to mea-sure how these different recommendations could be applied in the absence of a specific ALS clinic