Getting It Right in Restrictive Lung Disease

Restrictive lung disease (predominantly in patients with neuromuscular disease (NMD) and ribcage deformity) may induce chronic hypercapnic respiratory failure, which represents an absolute indication to start home NIV (HNIV). However, in the early phases of NMD, patients may present only diurnal symptoms or orthopnoea and sleep disturbances with normal diurnal gas exchange. The evaluation of respiratory function decline may predict the presence of sleep disturbances (SD) and nocturnal hypoventilation that can be respectively diagnosed with polygraphy and PCO2 transcutaneous monitoring. If nocturnal hypoventilation and/or apnoea/hypopnea syndrome are detected, HNIV should be introduced. Once HNIV has been started, adequate follow-up is mandatory. The ventilator's built-in software provides important information about patient adherence and eventual leaks to correct. Detailed data about pressure and flow curves may suggest the presence of upper airway obstruction (UAO) during NIV that may occur with or without decrease in respiratory drive. Etiology and treatment of these two different forms of UAO are different. For this reason, in some circumstances, it might be useful to perform a polygraph. PtCO2 monitoring, together with pulse-oximetry, seem to be very important tools to optimize HNIV. The role of HNIV in neuromuscular disease is to correct diurnal and nocturnal hypoventilation with the consequence of improving quality of life, symptoms, and survival.

Automated summary

Restrictive lung disease in patients with neuromuscular disease and ribcage deformity can lead to chronic hypercapnic respiratory failure, which requires the use of home non-invasive ventilation (HNIV). Early stages of neuromuscular disease may present only with daytime symptoms and sleep disturbances, while diurnal gas exchange remains normal. The decline in respiratory function can help predict the presence of sleep disturbances and nocturnal hypoventilation, which can be diagnosed using polygraphy and transcutaneous PCO2 monitoring, respectively. HNIV should be initiated if nocturnal hypoventilation or apnea/hypopnea syndrome is detected.