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To report the latest long-term outcome of amyotrophic lateral sclerosis (ALS) and to analyze the predictors of prognosis.
Subjects who were diagnosed with ALS between January 2005 and December 2009 at a single institute were followed up until death or up to December 2014. Data regarding age, sex, date of onset, date of diagnosis, presence of bulbar symptoms on onset, date of initiation of non-invasive ventilation (NIV), and the date of tracheostomy were collected. Survival was assessed using Kaplan-Meier curves and multivariate analyses of the risk of death were performed using the Cox proportional hazards model.
Among 212 suspicious subjects, definite ALS was diagnosed in 182 subjects. The survival rate at 3 and 5 years from onset was 61.5% and 40.1%, respectively, and the survival rate at 3 and 5 years post-diagnosis was 49.5% and 24.2%, respectively. Further, 134 patients (134/182, 73.6%) were initiated on NIV, and among them, 90 patients (90/182, 49.5%) underwent tracheostomy. Male gender and onset age of ≥65 years were independent predictors of adverse survival.
The analysis of long term survival in ALS showed excellent outcomes considering the overall poor prognosis of this disease.
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To compare the respiratory muscle strength between patients with stable and acutely exacerbated (AE) chronic obstructive pulmonary disease (COPD) at various stages.
A retrospective medical record review was conducted on patients with COPD from March 2014 to May 2016. Patients were subdivided into COPD stages 1–4 according to the Global Initiative for Chronic Obstructive Lung Disease guidelines: mild, moderate, severe, and very severe. A rehabilitation physician reviewed their medical records and initial assessment, including spirometry, maximum inspiratory pressure (MIP), maximum expiratory pressure (MEP), COPD Assessment Test, and modified Medical Research Council scale. We then compared the initial parameters in patients with a stable condition and those at AE status.
The AE group (n=94) had significantly lower MIP (AE, 55.93±20.57; stable, 67.88±24.96; p=0.006) and MIP% (AE, 82.82±27.92; stable, 96.64±30.46; p=0.015) than the stable patient group (n=36). MIP, but not MEP, was proportional to disease severity in patients with AE and stable COPD.
The strength of the inspiratory muscles may better reflect severity of disease when compared to that of expiratory muscles.
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To report successful cases of extubation from invasive mechanical ventilation at our institution using pulmonary rehabilitation consisting of noninvasive ventilation (NIV) in neuromuscular patients with experience of reintubation.
Patients who experienced extubation failure via the conventional weaning strategy but afterwards had extubation success via NIV were studied retrospectively. Continuous end-tidal CO2 (ETCO2) and pulse oxyhemoglobin saturation (SpO2) monitoring were performed. Extubation success was defined as a state not requiring invasive mechanical ventilation via endotracheal tube or tracheotomy during a period of at least 5 days.
A total of 18 patients with ventilatory failure who initially experienced extubation failure were finally placed under part-time NIV after extubation. No patient had any serious or long-term adverse effect from NIV, and all patients left the hospital alive.
NIV may promote successful weaning in neuromuscular patients with experience of reintubation.
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To reveal the significance of continuous transcutaneous carbon dioxide (CO2) level monitoring through reviewing cases which showed a discrepancy in CO2 levels between arterial blood gas analysis (ABGA) and continuous transcutaneous blood gas monitoring.
Medical record review was conducted retrospectively of patients with neuromuscular diseases who had started home mechanical ventilation between June 2008 and May 2010. The 89 patients underwent ABGA at the 1st hospital day, and changes to their CO2 level were continuously monitored overnight with a transcutaneous blood gas analysis device. The number of patients who initially appeared to show normal PaCO2 through ABGA, yet displayed hypercapnea through overnight continuous monitoring, was counted.
36 patients (40.45%) presented inconsistent CO2 level results between ABGA and continuous overnight monitoring. The mean CO2 level of the 36 patients using ABGA was 37.23±5.11 mmHg. However, the maximum and mean CO2 levels from the continuous monitoring device were 52.25±6.87 mmHg and 46.16±6.08 mmHg, respectively. From the total monitoring period (357.28±150.12 minutes), CO2 retention over 45 mmHg was detected in 198.97 minutes (55.69%).
Although ABGA only reflects ventilatory status at the puncturing moment, ABGA results are commonly used to monitor ventilatory status in most clinical settings. In order to decide the starting point of home mechanical ventilation in neuromuscular patients, continuous overnight monitoring should be considered to assess latent CO2 retention.
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