Continuous noninvasive ventilation for respiratory failure in patients with amyotrophic lateral sclerosis: current perspectives

Abstract

Respiratory failure is a recognized late complication of amyotrophic lateral sclerosis. It is related to the neurological progression of the diseases with the impairment of the respiratory musculature. Survival and quality of life of amyotrophic lateral sclerosis patients is improved by using noninvasive mechanical ventilation. The rate of long-term mechanical ventilation is different within and between countries. Cultural factors, socioeconomic conditions, and physician attitude often influence the decision to start noninvasive ventilation. Technical elements, like the choice of the correct interface, solid caregivers support, and the communication between the patient and the physician are essential for achieving therapeutic goals, especially in the case of continuous treatment.

Mesenchymal Stem Cells: A Potential Therapeutic Approach for Amyotrophic Lateral Sclerosis?

“…Usually, ALS leads to death within 3–5 years [ 1 , 2 ]. Respiratory failure is recognized as one of the main complications of ALS and one of the main causes leading to death [ 4 , 5 ]. The appearance of respiratory failure is caused by the impairment of the respiratory musculature, and it is influenced by the concomitant presence of other respiratory pathologies [ 4 , 5 ].…”

“…Respiratory failure is recognized as one of the main complications of ALS and one of the main causes leading to death [ 4 , 5 ]. The appearance of respiratory failure is caused by the impairment of the respiratory musculature, and it is influenced by the concomitant presence of other respiratory pathologies [ 4 , 5 ]. The loss of function of the phrenic nerve induces diaphragm weakness, leading as a consequence to dyspnea, orthopnea, and hypoventilation [ 4 ].…”

“…The loss of function of the phrenic nerve induces diaphragm weakness, leading as a consequence to dyspnea, orthopnea, and hypoventilation [ 4 ]. Unfortunately, respiratory symptoms are not easy to recognize and alterations of blood gas analysis become evident only in a late stage of the disease or when an acute episode of respiratory failure happens [ 5 ]. Noninvasive ventilation, considered a standard treatment for respiratory support for ALS patients, was demonstrated to improve the quality of life but also to increase the survival of patients [ 4 , 5 ].…”

Patient's treatment burden related to care coordination in the field of respiratory diseases

“…However, noninvasive ventilation may fail due to poor adherence. Although NIV should be performed for an adequate number of hours, many studies have shown suboptimal adherence in a non-negligible percentage of cases [ 54 , 55 ]. The main causes of poor adherence are: interface-related discomfort; unintentional leaks; patient–ventilator asynchrony; patient reluctance; lack of support from the family; absent or delayed follow-up by the healthcare team; absence of symptom improvement; lack of knowledge of therapeutic goals; psychological issues; and low socioeconomic status [ 56 , 57 ].…”

Intermittent abdominal pressure ventilation: feasibility and efficacy in neuromuscular disease. A case report

“…Non-invasive Ventilation Support-Settings (NVS) are often needed initially at night in patients with neuromuscular disease. However, when the condition worsens, it becomes necessary even during the day [1][2][3]. Facial masks (nasal, oronasal, nasal pillows, and mouthpieces) are the most used to convey NVS [1].…”