Respiratory Outcomes of Patients With Amyotrophic Lateral Sclerosis: An Italian Nationwide Survey

Abstract

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.

Multidisciplinary Care Improves Survival of Patients with Amyotrophic Lateral Sclerosis in the Unique Health System (SUS) in Brazil

“…Similar to other studies that showed that only [23] 6% to 30% of ALS patients use NIV [30,31], the frequency of NIV use was low in the present study (18%), which is related to economic factors and to the long delay in diagnostic time. However, strong evidence exists that shows that NIV prolongs survival in ALS patients [4,30,32].…”

The role of respiratory management of Pompe disease

“…The Respiratory approach consists of a dedicated clinic, direct fast trach hospital admission and home tele-support when necessary. 1,2 Patients are referred by general practitioners, pediatricians, neurological centres where diagnosis has been ascertained and by local associations of patients and their caregivers in a program of dedicated outpatient lung function follow-up screening. 1,2 Anthropometrics, nutritional status, Arterial blood gases (ABGs), dynamic and static lung volumes in sitting and supine position, respiratory muscle function, cardiorespiratory monitoring, polisonnigraphy, general clinical disability state by a clinical interview is usually proposed.…”

“…Nevertheless, a recent Italian survey showed that diagnosis, respiratory care, provision of information on respiratory complications and end of life decisions is still insufficient and needs to be improved. 1 In this frame, Pompe disease has a specific relevance. The low prevalence of Pompe disease, prevents established experience and increases the risk of underestimating the severity of respiratory problems, with consequent therapeutic delays.…”

Hot Topics in Noninvasive Ventilation: Report of a Working Group at the International Symposium on Sleep-Disordered Breathing in Leuven, Belgium

“…In Italy, only 10% of pulmonologists chose not to initiate longterm invasive ventilation for ALS, compared with the majority of respiratory physicians in France, Switzerland, and The Netherlands, who encouraged shared decision making and early advanced care planning and who tended to discourage long-term invasive ventilation for ALS. 33,60 Despite all doubts, long-term invasive ventilation may be indicated in the following situations: non-acceptance, intolerance, or ineffectiveness of NIV; failure to transfer to NIV following invasive ventilation; ineffective noninvasive management of airway secretions; or severe bulbar dysfunction with recurrent aspiration.…”

“…33 The ability to clear bronchopulmonary secretions is essential to prevent sputum retention and associated complications, including lower respiratory tract infection, which is the most common cause of hospital admission in these subjects. 34 The act of coughing involves 3 main components: a deep inspiration up to 85-90% of total lung capacity, glottic closure for ϳ0.2 s (which requires intact bulbar function), and effective contraction of the expiratory muscles to generate intrapleural pressures of Ͼ 190 cm H 2 O.…”