The ALS Health Profile Study: quality of life of amyotrophic lateral sclerosis patients and carers in Europe

The measurement of functioning and well-being from the perspective of the p atient has in recent years become central to the assess; ment of health and the evaluation of treatment regimes. The past decade has seen an enormous growth in the application of measures designed to assess quality of life in a vast array of medical specialties. However, the use of such measures in neurology has been relatively limited, and this has certainly been the case in amyotrophic lateral sclerosis (ALS), The European ALS Health Profile St udy is a longitudinal survey of patients diagnosed with ALS or other motor neurone diseases in which patients are asked to complete questionnaires con cerning their subjective health status. Data from clinical assessments are also collected. It is intended that the information collected will provide more systematic and detailed evidence of the impact of the disease from the perspective of the patient. This contribution documents results from basel ine assessment obtained from data supplied by clinicians, carers and patien ts themselves. Three outcome measures are assessed in this paper: the SF-36 , a generic measure of well being and functioning, the ALS Functional Ratin g Scale and the Carer Strain Index. The evidence presented here suggests th at these measures provide a meaningful and valid picture of the impact of t he disease, The data indicate that ALS has substantial adverse effects both upon the functioning and well being of patients and carers, as well as an association between the emotional health status of patients and carers, and between the physical health status of patients and carers.