Longitudinal assessment of noninvasive positive pressure ventilation adjustments in ALS patients.
ABSTRACT The absence of data guiding optimal titration of noninvasive positive pressure ventilation (NIPPV) over time in ALS patients may contribute to the under-prescribing of NIPPV. We conducted a retrospective, single-center, chart review assessment of NIPPV pressure settings used for symptomatic treatment of ALS patients to determine NIPPV adjustments, and to compare survival between those who were tolerant and intolerant to NIPPV. All subjects were started on nocturnal NIPPV at 8 and 3 cm H2O inspiratory and expiratory pressure, respectively. Of the 18 tolerant subjects identified, 4 (22%) had no NIPPV pressure changes before death; 8 (44%), 1 change; 4 (22%), 2 changes; 1 (6%), 3 changes; and 1 (6%), 5 changes. Most pressure changes occurred during the first year of NIPPV initiation. The maximum pressure needed for comfort by any patient in this study was 19/5 cm H2O, while 4 (22%) found the original 8/3 cm H2O settings to be sufficient until death. Subjects in the tolerant group had better survival, when adjusting for age and site of symptom onset (bulbar versus limb), with a hazard ratio of 0.23 [95% confidence interval: 0.10, 0.54]. The current data suggest that ALS patients who are tolerant to NIPPV typically need at least one upward change in pressure settings. Tolerance to relatively low NIPPV inspiratory pressures is associated with improved survival.
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ABSTRACT: Aim: The purpose of this study was to describe the status of ventilator use, respiratory problems, and caregiver well-being relating to patients with amyotrophic lateral sclerosis (ALS) using a home-based ventilator as well as to examine the relationship among ventilator use, respiratory problems, and caregiver well-being. Methods: Patients with ALS (n = 141) registered in the Severe-Rare Disease Center of the Korean Center for Disease Control and their caregivers (n = 83) were surveyed from August 2008 to April 2009. Trained research assistants visited patient homes; collected data using questionnaires; and then performed analyses with descriptive statistics, χ test, and t test as well as partial correlation analysis using SPSS WIN 18.0. Results: Thirty-two patients used noninvasive ventilation (NIV), and 109 used tracheostomy and mechanical ventilation (TMV). One hundred of the TMV patients used the ventilator 24 hours per day. The ventilator circuit exchange cycle was 0.96 times per month for NIV patients and 1.17 times per month for TMV patients (t = -4.91, p < .001). However, NIV patients had a higher level of tidal volume than TMV patients (t = 3.34, p = .001). Approximately 22% of NIV patients and 24% of TMV patients used one or more physiotherapies for airway clearance. There was a significant relationship between hypoventilation symptoms and caregiver burden (r = .31, p = .006). Hypoventilation symptoms were positively related to physiotherapy (r = .24, p = .042), and physiotherapy was positively related to caregiver burden (r = .24, p = .043). Conclusion: On the basis of the findings of this study, care management for patients with ALS with a home-based ventilator as well as their caregiver's well-being was relatively inappropriate. We recommend that community-based support programs and burden relief programs be considered as managerial interventions.Journal of Neuroscience Nursing 10/2014; 46(5):E25-E32. · 0.91 Impact Factor
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ABSTRACT: SUMMARY In the last three decades, improvements in respiratory management are responsible for increasing survival and improving quality of life for amyotrophic lateral sclerosis (ALS) patients. Nowadays, ALS patients with respiratory involvement are offered a support treatment other than the traditional respiratory palliative care. Knowledge about available respiratory support potentialities is essential for appropriate, customized and effective treatment of ALS, which should probably be started sooner than the conventional approach. There is evidence supporting that respiratory support has a larger impact than riluzole on survival. Noninvasive ventilation is essential in the treatment of ALS patients with respiratory involvement. In this article methods to determine respiratory failure in ALS, mechanical invasive and noninvasive ventilation, telemetry, diaphragm pacing, cough aids and respiratory exercise are reviewed, after a brief overlook of respiratory insufficiency in ALS.Neurodegenerative disease management. 02/2014; 4(1):83-102.
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ABSTRACT: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease in which most patients die of respiratory failure. Although volume-targeted non-invasive bilevel positive airway pressure (BPAP) ventilation has been studied in patients with chronic respiratory failure of various etiologies, its use in ALS has not been reported. We present the case of a 66-year-old woman with ALS and respiratory failure treated with volume-targeted BPAP ventilation for 15 weeks. Weekly data downloads showed that disease progression was associated with increased respiratory muscle weakness, decreased spontaneous breathing, and increased use of non-invasive positive pressure ventilation, whereas tidal volume and minute ventilation remained relatively constant.Jornal brasileiro de pneumologia: publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia 07/2014; 40(4):443-447.