Management of patients with neuromuscular disorders and acute respiratory failure

Abstract

showed in term of lower mortality, treatment failure, duration of ICU in several studies. Home mechanical noninvasive ventilation and MAC may result dramatic reduction in the need for hospitalization and a prolongation of life expectancy of these patients. To avoid the progression of respiratory failure an early identification of respiratory deterioration is needed, as such as prevention of acute episodes. So, especially in the progressive diseases, is very important to monitor respiratory status with periodic lung function tests. Any respiratory tract infection in neuromuscular disease could trigger acute respiratory failure so it is mandatory take any aggressive measures to prevent and treat this complication. Some of principal preventive measures include vaccination against Pneumococcus Pn. and influenza virus, early antibiotic treatment when bacterial infection is suspected, chest physiotherapy for removal airway secretions or mechanical assisted maneuvers to increase cough efficiency. In conclusion, a proactive, preventive approach is the key for optimal management acute respiratory problems in neuromuscular disorders.KEY WORDS: acute respiratory failure; neuromuscular disorders; noninvasive ventilation; mechanically assisted coughing; intensive care unit. IntroductionAcute respiratory failure (ARF) may be a life-threatening event during the course of several neuromuscular disorders (NMD). It can be the first manifestation of respiratory involvement or most frequently a worsening episode during chronic respiratory impairment. Deterioration of lung function during NMD varies in term of severity and time course according to different diseases. However, some general issues for the management of ARF in NMD should be kept in mind by the physicians to reduce time to recovery and to minimize the risks for the patient. Neuromuscular disorders affecting respiratory functionNeuromuscular disorders are numerous and represent a group of disease involving the muscle, the nerve or the neuromuscular junction ( SummaryAcute respiratory failure (ARF) is a common cause of morbidity and mortality in most advanced neuromuscular disorders (NMD). Deterioration of lung function during NMD may have different severity and time course. During slowly progressive neuromuscular disease an acute event can precipitate the respiratory failure, while rapidly progressive NMD may present as ARF at the onset of the disease. During ARF a patient with NMD may be affected by inspiratory muscles weakness responsible for decreased ventilation, expiratory muscles weakness responsible for altered coughing effectiveness, and bulbar involvement responsible for altered swallowing and airway protection. Neuromuscular disorders may also affect central control of breathing, sleep disorder, bones and joints deformities with consequent ventilatory dysfunction. Usually ARF in NMD is prompted by precipitating factors such as upper airways infections, pneumonia, atelectasis etc. It is important to distinguish respiratory failure due to an acute event or to a ...