Abstract
We examined the data from the clinical research forms of sporadic amyotrophic lateral sclerosis (ALS) patients with mechanical ventilation, who had received the subsidy for the medical costs of the specified intractable disease treatment research program, in Tokyo, Japan. Comparing the implements of respiratory/dietary treatments between 2006 and 2012, the number of patients with ventilation, especially non–invasive ventilation (NIV) had increased in 2012 (55 in 2012 vs 5 in 2006). The duration from the onset to the beginning of ventilation in 2012 was shorter than that in 2006 (2.6 vs 3.7 years), and the installation term of tracheostomy with invasive ventilation (TIV) was longer (63.7 vs 42.7 months). The number of the patients who had percutaneous endoscopic gastrostomy (PEG) increased (257 in 2012 vs 130 in 2006), and patients receiving home care also increased especially in NIV patients (41 vs 0), but only 43.9% of them had doctor's visits to home.
When limited to the patients within 6 years from the onset, the onset ages were higher than those of all patients in both 2006 and 2016 (67.3 vs 59.5 in 2006, 68.9 vs 59.2 in 2012). More than 60% introduced either NIV or TIV within 2 years after onset, and more than 70% underwent ventilation before PEG.
This research indicates that it is necessary for TIV patients with progressing disability to receive and maintain the appropriate care environment for a long time, and for NIV patients to receive home medical care including emergency treatment and palliation. Moreover, since there are older patients with rapid disease progression, establishing the care support system at the beginning is obviously important.
