Neuromuscular disease invasive ventilation

The number of VAIs has fluctuated from decade to decade. As the need for LTMV for polio patients declined, the number of patients with spinal cord injuries or progressive neuromuscular disease (NMD) increased due to better acute care. Therefore, the number of patients administered LTMV has progressively increased, due both to advances in medical care and to the more widespread application of invasive mechanical ventilation in the acute setting. The increasing number of VAIs can be documented by comparing surveys performed in the last two decades. Regional surveys carried out in Minnesota, United States, in 1986 and 1992, documented that the prevalence of ventilatory assistance rose from 2.4/100,000 in 1986 to 4.9/100,000 in 1992 (2). Extrapolation of these data would suggest that in the United States, the potential number of VAIs rose from 5777 in 1986 to 12,279 in 1992 (4). 

Ward S, Chatwin M, Heather S, et al. Randomised controlled trial of non-invasive ventilation (NIV) for nocturnal hypoventilation in neuromuscular and chest wall disease patients with daytime normocapnia. Thorax 2005 60 1019-1024. 

Three groups of patients who require long-term ventilation (LTV) are (1) patients with neurovascular damage and cranioencephalic or cervical trauma (2) patients with neuromuscular diseases (NMD) such as amyotrophic lateral sclerosis (ALS), Duchenne muscular dystrophy, and spinal muscular atrophy and (3) patients who have not been weaned from invasive ventilation, but who are potentially weanable—such as those with chronic obstructive pulmonary disease (COPD), cardiac failure, and multiorgan failure. 

In contrast to some countries, the polio epidemics of the 1950s did not trigger the initiation of long-term ventilation (LTV) in Japan (1). The first patient to receive LTV at home was in Tokyo in 1975, when an adult with neuromuscular disease (NMD) received tracheal invasive positive pressure ventilation (TIPPV) (2). Despite the introduction of public assistance for costs associated with home mechanical ventilation (HMV), fewer than 200 patients were receiving HMV between 1990 and 1993 (Fig. 1) (2). 

Abbreviations. TIPPV, tracheal invasive positive pressure ventilation NIPPV, noninvasive positive pressure ventilation ALS, amyotrophic lateral sclerosis NMD, neuromuscular diseases LTV, long-term ventilation. 

Markstrom A, Sundell K, Lysdahl M, et al. Quality of life evaluation of patients with neuromuscular and skeletal diseases treated with non-invasive home mechanical ventilation. Chest... 

Simonds A, Ward S, Heather S, et al. Outcome of domiciliary nocturnal non-invasive mask ventilation in paediatric neuromuscular-skeletal disease. Thorax 1998 53 A10. 

---