Patients with amyotrophic lateral sclerosis survived longer using noninvasive ventilation for their respiratory failure, according to a study published in Muscle and Nerve.
Researchers in this retrospective study analyzed 197 patients with sporadic amyotrophic lateral sclerosis from baseline until they had a tracheostomy with invasive ventilation or death occurred, either without treatment, while on noninvasive ventilation, or after transition to tracheostomy invasive ventilation. Investigators collected data on initial symptoms, timing of percutaneous endoscopic gastrostomy, the Revised Amyotrophic Lateral Sclerosis Functional Rating Scale, disease progression, blood analysis, and lung function.
Results showed that noninvasive ventilation had a median survival time of 43 months compared with 32 months without treatment (P <.001) and 78 months with tracheostomy invasive ventilation (P =.001). Disease progression rate at time of diagnosis, percentage of predicted forced vital capacity at noninvasive ventilation initiation, and bulbar symptoms at noninvasive ventilation initiation were independent factors on post-noninvasive ventilation survival. Thirty-four percent of patients transitioned from noninvasive ventilation to tracheostomy invasive ventilation. Overall, women and patients who lived longer with noninvasive ventilation were less likely to transition to tracheostomy invasive ventilation.
Future studies need to increase sample size and diversity, include patients and family quality of life assessments, and devise a mechanism for determining ideal timing for use of noninvasive ventilation in patients with bulbar dysfunction.
In conclusion, noninvasive ventilation is an effective option for patients with amyotrophic lateral sclerosis with respiratory failure. The findings on survival and transitioning to tracheostomy invasive ventilation from noninvasive ventilation will aid clinicians and family members in the decision-making process.
Hirose T, Kimura F, Tani H, et al. Clinical characteristics of long-term survival with noninvasive ventilation and factors affecting the transition to invasive ventilation in amyotrophic lateral sclerosis [published online April 20, 2018]. Muscle Nerve. doi:10.1002/mus.26149