Tracheostomy Invasive Ventilation Research Articles

Prognosis of amyotrophic lateral sclerosis patients after tracheostomy invasive ventilation in Korea

Background: Tracheostomy invasive ventilation (TIV) is applied to a subset of amyotrophic lateral sclerosis (ALS) patients; however, its frequency and impact on prognosis vary across countries. Methods: We conducted a nationwide retrospective cohort study using Korean National Health Insurance claims data. All patients diagnosed with sporadic ALS from 2012 to 2017 were included, with the observation period until 2020. The survival time between the TIV and non-TIV groups was compared using propensity score matching analysis, and prognostic factors were assessed within the TIV group. Results: This study included 3484 ALS patients (mean [standard deviation] age, 62.4 [11.9] years, 60.4% male), among whom 1230 (35.3%) underwent TIV. After 1:1 propensity score matching, the survival duration between the two groups was not significantly different (28 vs. 25 months, p = 0.057). Cox regression indicated that older age (hazard ratios [HRs] for each decade compared to <40 years: 3.89, 3.83, 5.30, 6.78, and 8.40 [ ≥ 80 years]; p < 0.005 for all) and lower income (HR, 1.28; 95% confidence interval [CI], 1.09–1.52; p = 0.003) negatively impacted survival, while gastrostomy (HR, 0.57; 95% CI, 0.50–0.66; p < 0.001) and supportive care services (HR, 0.43; 95% CI, 0.32–0.59; p < 0.001) were associated with prolonged survival. Conclusions: TIV was administered to more than one-third of Korean ALS patients without significant survival prolongation. Older age, lower income, lack of gastrostomy, and insufficient supportive care were independent poor prognostic factors for survival, underscoring the importance of comprehensive management for ALS patients.

Characteristics and outcome of infants with bronchopulmonary dysplasia established on long-term ventilation from neonatal intensive care.

Ex-preterm infants with severe bronchopulmonary dysplasia (BPD) sometimes require long-term ventilation (LTV) to facilitate weaning from respiratory support. There are however limited data characterizing this cohort. We aim to describe the background characteristics, neonatal comorbidities, characteristics at the initiation of ventilation, and outcomes of neonatal unit graduates with BPD established on LTV. A retrospective cohort study of infants born <32 weeks gestation with BPD referred to a regional LTV service between January 2015 and December 2020. Twenty-five infants were referred during the study period. Median birth gestation was 26 + 1 weeks (24 + 0-30 + 4) and birth weight 645 g (430-1485). At 36 weeks postmenstrual age (PMA), median FiO2 was 0.45 (0.24-0.80) and one-quarterof infants remained on invasive ventilation. Twenty (80%) infants were established on noninvasive ventilation (NIV), with the smallest weighing 2085 g, and five (20%) required tracheostomy invasive ventilation (TIV). At initiation of NIV/TIV, median PMA was 41 + 1 weeks and median FiO2 0.40 (0.29-0.80). Infants established on TIV spent almost five times longer in hospital before discharge compared to those on NIV (p = 0.003). By March 2022, 18(72%) infants had discontinued ventilation, spending a median total time of 113 days (18-1792) on ventilation. Due to advances in interfaces, headgear, and ventilator technology, NIV is an attractive and practically achievable option for infants with severe BPD as small as 2 kg. Initiation and weaning should take place in a facility with the required multidisciplinary expertize.

Prognosis of Pathologically Confirmed Japanese Amyotrophic Lateral Sclerosis with Tracheostomy Invasive Ventilation, a Retrospective Institute-based Study (P2-13.007)

Prognosis of Pathologically Confirmed Japanese Amyotrophic Lateral Sclerosis with Tracheostomy Invasive Ventilation, a Retrospective Institute-based Study (P2-13.007)

Impact of tracheostomy invasive ventilation on survival in Japanese patients with multiple system atrophy

IntroductionTracheostomy invasive ventilation (TIV) is therapeutic intervention to prolong survival. However, few reports have addressed TIV in multiple system atrophy (MSA). This study sought to evaluate the impact of TIV on survival in MSA patients. MethodsThis retrospective cohort study examined medical records of probable or definite MSA for patients in Hyogo-Chuo National Hospital from January 2000 to September 2021 to investigate overall survival and cause of death in those with tracheostomy and TIV. ResultsThe study enrolled 12 definite and 127 probable MSA patients. Mean age at onset was 61.3 ± 9.8 years, and median survival time was 9.0 years. Tracheostomy was performed in 53 patients, 21 of whom were ventilated. Mean time from onset to tracheostomy and TIV was 7.0 ± 3.0 and 8.4 ± 4.4 years, respectively. After propensity score matching, tracheostomy showed a significant prolongation of median survival compared with no tracheostomy (10.1 vs. 7.5 years, p = 0.001) and TIV significantly prolonged survival compared with tracheostomy alone (17.8 vs. 9.2 years, p = 0.023). On Cox regression analysis, the hazard ratio for tracheostomy was 0.35 (95% confidence interval [CI] 0.17–0.68, p = 0.002) and TIV was 0.22 (95% CI 0.07–0.89, p = 0.032). In MSA with TIV, sudden death was significantly lower compared with tracheostomy alone, and infection was the most common cause of death. ConclusionResults showed that TIV prolonged survival and reduced sudden death compared with tracheostomy alone in MSA, although sudden death can never be completely prevented.

Factors associated with assisted ventilation use in amyotrophic lateral sclerosis: a nationwide population-based study in Korea

Few studies have investigated the factors associated with assisted ventilation use in amyotrophic lateral sclerosis (ALS) in western countries with a relatively small number of participants. This study aimed to evaluate the factors associated with assisted ventilation use using a large nationwide cohort covering the entire Korean population. We selected patients with primary or secondary diagnoses of ALS (ICD-10 code: G12.21) and a registration code for ALS (V123) in the rare intractable disease registration program. Covariates included in the analyses were age, sex, socioeconomic status and medical condition. Factors associated with non-invasive ventilation (NIV) and tracheostomy invasive ventilation (TIV) were evaluated. Logistic regression analyses were performed using odds ratios and 95% confidence intervals. In total, 3057 patients with ALS were enrolled. During the 6-year follow-up period, 1228 (40%) patients started using assisted ventilation: 956 with NIV and 272 with TIV. There was no significant difference in the assisted ventilation use according to sex, whereas different patterns of discrepancies were noted between the sexes: Females living in non-metropolitan areas showed decreased use of assisted ventilation, whereas high income levels showed a positive relationship with assisted ventilation use only in males. Patients aged ≥ 70 years showed decreased use of NIV. NIV use was more affected by socioeconomic status than TIV, whereas TIV showed a significant relationship with medical conditions such as nasogastric tube insertion and gastrostomy. We found that various factors, including age, socioeconomic status, and medical condition, were related with assisted ventilation use. Understanding the pattern of assisted ventilation use would help set optimal management strategies in patients with ALS.

Changing medical care for amyotrophic lateral sclerosis patients and cause of death - review of muscular dystrophy wards (1999-2013)

We analyzed the records of inpatients with amyotrophic lateral sclerosis (ALS) treated at 27 specialized institutions for muscular dystrophy in Japan from 1999 to 2013 registered in a database on October 1 of each year. The total number of ALS inpatients in 1999 was 29, then that showed rapid increases in 2006 and 2007, and reached 164 in 2013. Age regardless of year was predominantly greater than 50 years. In 1999, the respirator dependent rate was 68.9% and then increased to 92.7% in 2013, while the oral nutritional supply rate was 41.4% in 1999 and decreased to 10.4% in 2013. The number of deaths from 2000 to 2013 was 118. Cause of death was respiratory failure in 26 of 30 patients who maintained voluntary respiration at the time of death and in 5 of 6 with non-invasive ventilation. On the other hand, the main cause of death in patients with tracheostomy invasive ventilation was respiratory infection, which was noted in 26 of 82, while other causes varied. It is expected that the number of ALS patients admitted to specialized institutions with muscular dystrophy wards will continue to increase.

Non-invasive and tracheostomy invasive ventilation in amyotrophic lateral sclerosis: Utilization and survival rates in a cohort study over 12 years in Germany.

The aim of this study was to investigate utilization rates, treatment pathways and survival prognosis in patients with amyotrophic lateral sclerosis (ALS) undergoing non-invasive (NIV) and tracheostomy invasive ventilation (TIV) in a real-world setting. A prospective cohort study using a single-centre register of 2702 ALS patients (2007 to 2019) was conducted. Utilization of NIV/TIV and survival data were analysed in three cohorts: (i) non-NIV; (ii) NIV (NIV without subsequent TIV); and (iii) TIV (including TIV preceded by NIV). A total of 1720 patients with available data were identified, 72.0% of whom (n=1238) did not receive ventilation therapy. NIV was performed in 20.8% of patients (n=358). TIV was performed in 9.5% of patients (n=164), encompassing both primary TIV (7.2%, n=124) and TIV with preceding NIV (2.3%, n=40). TIV was more often utilized without previous NIV (25.7% vs. 8.3% of all ventilated patients), demonstrating that primary TIV was the prevailing pathway for invasive ventilation. The median (range) survival was significantly longer in the NIV cohort (40.8[37.2-44.3] months) and the TIV cohort (82.1[68.7-95.6] months) as compared to the non-NIV cohort (33.6[31.6-35.7] months). Although NIV represents the standard of care, its utilization rate was low. TIV was mainly started without preceding NIV, suggesting that TIV may not be confined to NIV treatment escalation. However, TIV was pursued in a minority of patients who had previously undergone NIV. The survival benefit observed in the patients with NIV was equal to that reported in a controlled pivotal trial, but the prognosis with TIV is highly variable. The determinants of utilization of NIV/TIV and of survival (bulbar syndrome, availability of ventilation-related home nursing, cultural factors) warrant further investigation.

Prognosis of amyotrophic lateral sclerosis patients undergoing tracheostomy invasive ventilation therapy in Japan

ObjectiveThe aim of this study is to describe and clarify the factors affecting the prognosis of Japanese patients with amyotrophic lateral sclerosis (ALS) undergoing tracheostomy invasive ventilation (TIV) therapy.MethodsWe conducted...

The cause of death in pathologically confirmed amyotrophic lateral sclerosis with invasive tracheostomy ventilation, a retrospective institute-based study

We performed retrospective institute-based prognosis study to clarify the cause of death and the clinical course in pathologically confirmed amyotrophic lateral sclerosis (ALS) patients with tracheostomy invasive ventilation (TIV). To elucidate the clinical characteristics of pathologically confirmed ALS, we retrospectively reviewed medical records of 96 consecutive autopsied patients between 2008 and 2016. Pathological diagnosis was confirmed when the following two histopathological features were observed. First of them was systematic both upper and lower motor neuronal degeneration, and second was the existence of TDP-43 inclusion body pathology and/or Bunina bodies. We identified 11 patients of pathologically diagnosed ALS with TIV (ALS-TIV) in this period. Female to male ratio was 4 to 7. Average age of death was 71.1 y.o. and median survival time was 123 months. Age of onset ranged from 43 to 82 years old, and age of death ranged from 56 to 90 years old. According to initial symptom, 5 cases were upper limb onset, 3 cases were bulbar onset, 1 case was respiratory onset, and a case was lower limb onset. All ALS-TIV patients had bronchopneumonia at their autopsied time. Respiratory insufficiency was the main cause of death in 6 of these patients. Sepsis was another main cause of death in 4 patients. There were no pulmonary embolism in this series. From this retrospective study, TIV could markedly improve prognosis of pathologically proved ALS patients. Sepsis and respiratory failures were the main cause of death in ALS-TIV.

C-Reactive Protein ( CRP ) is Significantly Higher in Amyotrophic Lateral Sclerosis (ALS) Patients on Non-Invasive Ventilation (NIV) and Tracheostomy-Invasive Ventilation (TIV) Compared with ALS Patients at Intake Clinic Evaluation and Decreases Following Riluzole Administration – Is CRP Potentially a Biomarker for Treatment Responsiveness? (P4.447)

C-Reactive Protein ( CRP ) is Significantly Higher in Amyotrophic Lateral Sclerosis (ALS) Patients on Non-Invasive Ventilation (NIV) and Tracheostomy-Invasive Ventilation (TIV) Compared with ALS Patients at Intake Clinic Evaluation and Decreases Following Riluzole Administration – Is CRP Potentially a Biomarker for Treatment Responsiveness? (P4.447)

Automatic intratracheal suctioning system improved airway clearance of amyotrophic lateral sclerosis patients with tracheostomy-invasive ventilation

Background: We provide long-term medical care for neuromuscular disease patients as a neuromuscular-disease center hospital in Hokuriku area of Japan. We have also developed multidisciplinary care team for amyotrophic lateral sclerosis (ALS). Partly as a result of our support, about 25% of our ALS patients choose tracheostomy-invasive ventilation (TIV). So, management of ALS-TIV respiratory condition is the important part of our care. To reduce respiratory complications, our respiratory care support team (RST) began to apply automatic intratracheal suctioning system (AISS) since 2012.

Effectiveness of automatic intratracheal suctioning system for amyotrophic lateral sclerosis patients with tracheostomy-invasive ventilation (P4.131)

Effectiveness of automatic intratracheal suctioning system for amyotrophic lateral sclerosis patients with tracheostomy-invasive ventilation (P4.131)

Ventilatory Support and Management of Secretions in Amyotrophic Lateral Sclerosis

The term amyotrophic lateral sclerosis (ALS) comprises a group of motor neuron diseases which are characterized by rapid disease progression and poor prognosis which is mostly due to severe respiratory muscle weakness and its sequelae. Since causative treatment options are limited it is crucial to offer comprehensive symptomatic therapies to affect patients. Symptoms of respiratory muscle weakness, sleep-disordered breathing and, subsequently, chronic hypercapnic respiratory failure are known to severely affect health-related quality of life and social functioning of patients with ALS. This review article delineates the clinical presentation of respiratory muscle weakness, diagnostic procedures to assess diaphragmatic function, and practical aspects of both mechanical ventilation and cough assistance, respectively. Various technical and electrophysiological methods allow for detection of diaphragmatic weakness and nocturnal hypoventilation. These include spiro-manometric tests of respiratory muscle strength, cardiorespiratory polygraphy and polysomnography, transcutaneous capnography, and blood gas analysis. Once the diagnosis of respiratory muscle weakness is established, non-invasive ventilation, tracheostomy-invasive ventilation (if the patient agrees to it), and management of secretions all become increasingly important in the course of the disease.

Clinicopathological characteristics of patients with amyotrophic lateral sclerosis resulting in a totally locked-in state (communication Stage V).

In the present study, we performed a comprehensive analysis to clarify the clinicopathological characteristics of patients with amyotrophic lateral sclerosis (ALS) that had progressed to result in a totally locked-in state (communication Stage V), in which all voluntary movements are lost and communication is impossible. In 11 patients, six had phosphorylated TAR DNA-binding protein 43 (pTDP-43)-immunoreactive (ir) neuronal cytoplasmic inclusions (NCI), two had fused in sarcoma (FUS)-ir NCI, and three had copper/zinc superoxide dismutase (SOD1)-ir NCI. The time from ALS onset to the need for tracheostomy invasive ventilation was less than 24 months in ten patients. Regardless of accumulated protein, all the patients showed common lesions in the pallido–nigro–luysian system, brainstem reticular formation, and cerebellar efferent system, in addition to motor neurons. In patients with pTDP-43-ir NCI, patients with NCI in the hippocampal dentate granule neurons (DG) showed a neuronal loss in the cerebral cortex, and patients without NCI in DG showed a preserved cerebral cortex. By contrast, in patients with FUS-ir NCI, patients with NCI in DG showed a preserved cerebral cortex and patients without NCI in DG showed marked cerebral degeneration. The cerebral cortex of patients with SOD1-ir NCI was preserved. Together, these findings suggest that lesions of the cerebrum are probably not necessary for progression to Stage V. In conclusion, patients with ALS that had progressed to result in communication Stage V showed rapidly-progressed symptoms, and their common lesions could cause the manifestations of communication Stage V.

Macroglossia in advanced amyotrophic lateral sclerosis.

An enlarged tongue (macroglossia) has been reported in advanced-stage patients with amyotrophic lateral sclerosis (ALS). In this study we examined the prevalence of macroglossia and analyzed clinical correlations in 65 ALS patients on tracheostomy-invasive ventilation (TIV). Macroglossia was found in 22 patients (33.8%). Compared with those without macroglossia, patients with macroglossia had a younger age of onset, longer duration of disease and TIV use, lower ALS Functional Rating Scale score, higher body mass index, lower energy intake, more severe communication impairment, and lower oral function. Logistic multivariate analysis showed that body mass index (BMI; P = 0.007) and communication impairment (P = 0.029) were significantly correlated with macroglossia. The duration of TIV use was at the cut-off level of significance (P = 0.05). Macroglossia may be the result of overfeeding and replacement by fat during long-term TIV use in patients with advanced ALS. Muscle Nerve, 2016 Muscle Nerve 54: 386-390, 2016.

Tracheostomy and invasive mechanical ventilation in amyotrophic lateral sclerosis: decision-making factors and survival analysis.

Invasive and/or non-invasive mechanical ventilation are most important options of respiratory management in amyotrophic lateral sclerosis. We evaluated the frequency, clinical characteristics, decision-making factors about ventilation and survival analysis of 190 people with amyotrophic lateral sclerosis patients from 1990 until 2013. Thirty-one percentage of patients underwent tracheostomy invasive ventilation with the rate increasing more than the past 20 years. The ratio of tracheostomy invasive ventilation in patients >65 years old was significantly increased after 2000 (25%) as compared to before (10%). After 2010, the standard use of non-invasive ventilation showed a tendency to reduce the frequency of tracheostomy invasive ventilation. Mechanical ventilation prolonged median survival (75 months in tracheostomy invasive ventilation, 43 months in non-invasive ventilation vs natural course, 32 months). The life-extending effects by tracheostomy invasive ventilation were longer in younger patients ≤65 years old at the time of ventilation support than in older patients. Presence of partners and care at home were associated with better survival. Following factors related to the decision to perform tracheostomy invasive ventilation: patients ≤65 years old: greater use of non-invasive ventilation: presence of a spouse: faster tracheostomy: higher progression rate; and preserved motor functions. No patients who underwent tracheostomy invasive ventilation died from a decision to withdraw mechanical ventilation. The present study provides factors related to decision-making process and survival after tracheostomy and help clinicians and family members to expand the knowledge about ventilation.

気管切開下陽圧換気導入後の筋萎縮性側索硬化症患者の安静時唾液分泌量

Patients with amyotrophic lateral sclerosis (ALS) often suffer from salivation problems such as drooling and dry mouth. We examined resting salivation rate cross-sectionally in 66 advanced ALS patients with tracheostomy invasive ventilation using a cotton roll method, and investigated clinical factors associated with salivation rate. Resting salivation rate in the patients was well preserved (median value 0.6 g/min), and was significantly more increased in patients with impairment of jaw movement (P = 0.007) or mouth opening (P = 0.003) than in patients with less impairment, and in patients with the mouth being constantly open ≥ 10 mm in rostrocaudal length than in patients with < 10 mm. These data indicate that salivation rate was increased with progression of dysfunction of voluntary jaw movement. Appropriate oral care is required in advanced ALS patients to maintain their oral hygiene and to avoid penetration of saliva into the airway.

Predictors of impaired communication in amyotrophic lateral sclerosis patients with tracheostomy-invasive ventilation

Predictors of communication impairment in patients with amyotrophic lateral sclerosis (ALS) using tracheostomy-invasive ventilation (TIV) were investigated. Seventy-six ALS patients using TIV were enrolled and classified into three subgroups of communication ability: patients who could communicate with communication devices (Stage I), patients who had difficulty with communication (Stage II, III, or IV), and patients who could not communicate by any means (Stage V). Predictors of communication impairment were analysed by the Cox proportional hazard model.Results demonstrated that there were no significant differences in disease duration between subgroups. Within 24 months after disease onset, patients who needed TIV and tube feeding, developed oculomotor impairment or became totally quadriplegic and progressed from Stage I to II and V significantly earlier. Multivariate analyses revealed that within 24 months from onset, the need for TIV and progression to total quadriplegia were significant events in patients who progressed to Stage II, whereas the development of oculomotor limitation was significant in patients who progressed to Stage V. In conclusion, TIV, impaired oculomotor movement and total quadriplegia are predictors of severe communication impairment. Rapid disease progression might indicate future communication impairment after the use of TIV. We highly recommend early detection of impaired communication and identification of the best methods of communication.

Occurrence of Cholelithiasis and Cholecystitis in Amyotrophic Lateral Sclerosis Patients with Long-Term Tracheostomy Invasive Ventilation

We clarified the prevalence of cholelithiasis/cholecystitis in long-term tracheostomy invasive ventilation (TIV) patients in our hospital. We investigated the occurrence of cholelithiasis/cholecystitis in twenty-seven TIV patients over the past eight years. There were ten patients with cholelithiasis/cholecystitis. The underlying disease was amyotrophic lateral sclerosis (ALS), except for 1 patient. Mean age was 56.2 ± 4.7 years and the average period to the attack of cholelithiasis/cholecystitis from TIV was 57.6 ± 15.1 months. Two patients of acute cholecystitis had acalculous cholecystitis. Five of the ten patients developed a severe state of cholecystitis and needed aggressive therapy. The prevalence of gallstones, symptom prevalence of asymptomatic cholelithiasis, the percentage of acalculous cholecystitis of acute cholecystitis in TIV patients was higher than in the general population. Therefore, it may be possible that TIV is a risk for cholelithiasis/cholecystitis. Some of the ALS patients with long-term TIV develop severe cholelithiasis/cholecystitis, and those facts should be considered in our daily clinical practice.