Specialised ALS care and Quality of Life

Amyotrophic lateral sclerosis (ALS) is a devastating neuromuscular disorder characterized by degeneration of lower and upper motor neurons. The incidence ranges from 1.5 to 2.0 per 100,000 population per year with an overall male predominance. The course of the disease is relentlessly progressive, but the rate of deterioration varies from patient to patient. Approximately 50% of the patients die within 3 years after onset of the disease. About 20% of the patients survive diagnosis more than five years. Patients usually die from respiratory failure. Because there is no cure for ALS, the best treatment we can offer patients is optimal supportive care. In recent years, multidisciplinary ALS clinics emerged that exclusively provide symptomatic and palliative care to patients with ALS. The concentration of larger numbers of patients in these ALS clinics leads to an accumulation of resources and clinical expertise that facilitates the management of this relatively rare, progressive disease. Although multidisciplinary ALS care prolongs survival of patients with ALS, the effect on quality of life (QoL) is not known. QoL in patients with ALS is determined by several factors which include psychological and existential factors, relationships and other support factors, religion and spirituality, in addition to strength and physical function. Identifying the determinants of QoL may be helpful when assessing the needs for care of patients with ALS, although the effect of standard of care on QoL is largely unknown.