Multidisciplinary care in amyotrophic lateral sclerosis: a 4-year longitudinal observational study

BACKGROUND: Multidisciplinary management (MDM) of amyotrophic lateral sclerosis (ALS) has been associated with increased survival and improved health-related quality of life (HRQL). AIMS OF THE STUDY: The aims of this study were (1) to present the specifics and structure of MDM of ALS in our area and the roles of the specialists involved, and (2) to provide the results of MDM concerning aspects such as survival, modalities and timing of ventilator and nutritional support, and HRQL. METHODS: During a four-year period, systematic data collection on all ALS patients included in our MDM structure was performed at three-month intervals. The data covered neurologic assessment, respiratory function, nutritional status, ENT and phoniatric evaluation, palliative care, HRQL, existing social support, and need for occupational and physical therapy. RESULTS: 68 patients were included (50% women, 19% with bulbar onset). Mean (SD) age at the time of first symptoms was 66.5 (12.3) years. Median (Q1-Q3) time elapsed from onset of symptoms to diagnosis was 11 (7-18) months. Median survival was 50 (34-95) months from first symptoms and 35 (18-70) months from diagnosis. Riluzole was prescribed in 74% of cases. Noninvasive ventilation was implemented in 28 (41%) and percutaneous gastrostomy in 14 (21%) patients. CONCLUSIONS: ALS patients and neurologists in private practice adhered to multidisciplinary management of ALS in our area. Implementation of noninvasive ventilation and percutaneous endoscopic gastrostomy could be performed electively in most cases and emergency procedures were seldom required. Decisions on noninvasive ventilation, percutaneous endoscopic gastrostomy, optimal symptom control and advanced care planning were shared among different specialists.