How to eat, drink and speak on non-invasive ventilation.

We report our observations on six individuals with non-bulbar neuromuscular disorders using non-invasive ventilation (NIV), who were able to maintain adequate hydration and nutrition orally despite being ventilator-dependant. All had severe respiratory muscle weakness, with a vital capacity less than 500 mL and cough peak flow rate less than 250 L/min. Their median (range) age was 49 (23-64) years; they had been on NIV for 8 (2-24) years. We compared them with an age- and sex-matched normal control. Individuals with neuromuscular disorders needed to chew each mouthful of food significantly more times (median 44, range 18-120 chews) than normal controls (median 15, range 10-20 chews). They took longer to completely swallow a mouthful of food (median 37, range 24-100 s) compared to normal controls (median 14.5, range 10-21 s). Multiple swallows for each mouthful were seen in all neuromuscular individuals, but in only one normal control. Two individuals coughed after swallowing; both these subjects were clinically stable at the time of the study. The median number of NIV breaths associated with chest expansion for each mouthful was 11 (range 5-49). All subjects blocked some NIV breaths whilst eating. Before swallowing, they always waited until the expiratory phase of the NIV breath was complete; no post-swallow expiration was seen, whereas normal subjects invariably exhibited post-swallow expiration. All individuals were able to block several ventilator breaths whilst swallowing un-thickened liquids. The median (range) number of words between breaths was 5 (4-7) for the neuromuscular individuals on NIV, significantly fewer than 11 (8-13) for the matched controls. Eating, drinking and speaking are possible whilst on NIV. Use of cough-assist after eating is recommended, given the likelihood of silent aspiration.

Long-Term Survival on Noninvasive Ventilation in Adults With Thoracic Scoliosis.

BACKGROUND: Subjects with thoracic scoliosis were an important group in early studies of noninvasive ventilation (NIV). The aim of this study was to describe current rates of initiation of NIV and survival after initiation in this population. METHODS: This study included patients identified as having thoracic scoliosis and established between 1993 and 2018 on home NIV. Patients with scoliosis secondary to neuromuscular disease (other than poliomyelitis) were excluded. Survival rates were calculated for various time intervals up to 25 y. RESULTS: A total of 53 subjects with thoracic scoliosis were successfully established on NIV. [Formula: see text] levels prior to starting NIV were 55 ± 23 mm Hg. FVC was 0.5 ± 0.1 L, 18.5 ± 9% of predicted, with a Cobb angle of 101 ± 3.5 degrees. The 5-, 10-, 15-, 20-, and 25-y survival rates were 96%, 88%, 61%, 46%, and 39%, respectively. At the time of death, subjects had been on home NIV for 9.2 ± 5.1 y and were 75.5 ± 9.2 y old. There was no significant correlation between mortality and age at time of commencing home NIV, initial arterial blood gas results, FVC, or Cobb angle. There was no significant difference in survival between those with and without poliomyelitis. In 8 of 10 of the most recent years of this survey, subjects with scoliosis have been commenced on home NIV. CONCLUSIONS: Small numbers of subjects with scoliosis continued to present with respiratory failure. Once established on home NIV, around 40% survived ≥ 25 y. Long-term care will be needed for many years to come for this patient population.