Respiratory outcomes of patients with amyotrophic lateral sclerosis: an Italian nationwide survey

Abstract

Background: Despite recommendations, respiratory therapies remain partially underutilized in patients with amyotrophic lateral sclerosis (ALS), and different practices are described in different countries. We surveyed attitudes and practice in Italy on ALS and other neuromuscular diseases (NMDs).

Methods: A questionnaire on ALS/NMD management was mailed to 178 pneumology units, which we classified into high-volume and low-volume centers according to the number of ALS/NMD patients followed in the last 5 years.

Results: Seventy-six pneumology units (43%) responded, and the responding units had 1,772 ALS patients and 1,490 NMD patients. Difficulty clearing respiratory secretions and disturbed sleep were the main reasons the ALS patients were referred to pneumology units. Vital capacity (measured in the sitting position) and arterial blood gases were measured regularly, while respiratory muscle function and cough ability were routinely assessed in over 85% of the pneumology units (mainly in high-volume centers), and 94% of pneumology units could provide noninvasive ventilation. Treatment of NMDs was similar to that of ALS, except for tracheostomy, which was less frequently performed in patients with NMDs. A multidisciplinary team approach to care was employed in approximately 90% of the responding pneumology units. Approximately a third of the units accessed palliative care services.

Conclusions: Combined pulmonary function evaluation, long-term noninvasive ventilation, and assisted cough have become usual practices for ALS patients. Italian practices for ALS did not significantly differ from the approach to other NMDs.

Keywords: amyotrophic lateral sclerosis; noninvasive ventilation; respiratory failure.