Effect of Intensity of Home Noninvasive Ventilation in Individuals With Neuromuscular and Chest Wall Disorders: A Systematic Review and Meta-Analysis of Individual Participant Data

Introduction: Home noninvasive ventilation (NIV), targeting a reduction of carbon dioxide with a combination of sufficient inspiratory support and backup-rate improves outcomes in patients with chronic obstructive pulmonary disease. The aim of this systematic review with individual participant data (IPD) meta-analysis was to evaluate the effects of intensity of home NIV on respiratory outcomes in individuals with slowly progressive neuromuscular (NMD) or chest-wall disorders (CWD). Methods: Controlled, non-controlled and cohort studies indexed between January-2000 and December-2020 were sought from Medline, Embase and the Cochrane Central Register. Outcomes were diurnal PaCO2, PaO2, daily NIV usage, and interface type (PROSPERO-CRD 42021245121). NIV intensity was defined according to the Z-score of the product of pressure support (or tidal volume) and backup-rate. Results: 16 eligible studies were identified; we obtained IPD for 7 studies (176 participants: 113-NMD; 63-CWD). The reduction in PaCO2 was greater with higher baseline PaCO2. NIV intensity per se was not associated with improved PaCO2 except in individuals with CWD and the most severe baseline hypercapnia. Similar results were found for PaO2. Daily NIV usage was associated with improvement in gas exchange but not with NIV intensity. No association between NIV intensity and interface type was found. Conclusion: Following home NIV initiation in NMD or CWD patients, no relationship was observed between NIV intensity and PaCO2, except in individuals with the most severe CWD. The amount of daily NIV usage, rather than intensity, is key to improving hypoventilation in this population during the first few months after introduction of therapy. (AU)

Effect of Intensity of Home Noninvasive Ventilation in Individuals With Neuromuscular and Chest Wall Disorders: A Systematic Review and Meta-Analysis of Individual Participant Data.

INTRODUCTION: Home noninvasive ventilation (NIV), targeting a reduction of carbon dioxide with a combination of sufficient inspiratory support and backup-rate improves outcomes in patients with chronic obstructive pulmonary disease. The aim of this systematic review with individual participant data (IPD) meta-analysis was to evaluate the effects of intensity of home NIV on respiratory outcomes in individuals with slowly progressive neuromuscular (NMD) or chest-wall disorders (CWD). METHODS: Controlled, non-controlled and cohort studies indexed between January-2000 and December-2020 were sought from Medline, Embase and the Cochrane Central Register. Outcomes were diurnal PaCO2, PaO2, daily NIV usage, and interface type (PROSPERO-CRD 42021245121). NIV intensity was defined according to the Z-score of the product of pressure support (or tidal volume) and backup-rate. RESULTS: 16 eligible studies were identified; we obtained IPD for 7 studies (176 participants: 113-NMD; 63-CWD). The reduction in PaCO2 was greater with higher baseline PaCO2. NIV intensity per se was not associated with improved PaCO2 except in individuals with CWD and the most severe baseline hypercapnia. Similar results were found for PaO2. Daily NIV usage was associated with improvement in gas exchange but not with NIV intensity. No association between NIV intensity and interface type was found. CONCLUSION: Following home NIV initiation in NMD or CWD patients, no relationship was observed between NIV intensity and PaCO2, except in individuals with the most severe CWD. The amount of daily NIV usage, rather than intensity, is key to improving hypoventilation in this population during the first few months after introduction of therapy.

The role of telemonitoring in patients on home mechanical ventilation.

There is a growing number of patients being treated with long-term home mechanical ventilation (HMV). This poses a challenge for the healthcare system because in-hospital resources are decreasing. The application of digital health to assist HMV care might help. In this narrative review we discuss the evidence for using telemonitoring to assist in initiation and follow-up of patients on long-term HMV. We also give an overview of available technology and discuss which parameters can be measured and how often this should be done. To get a telemonitoring solution implemented in clinical practice is often complex; we discuss which factors contribute to that. We discuss patients' opinions regarding the use of telemonitoring in HMV. Finally, future perspectives for this rapidly growing and evolving field will be discussed.

A Randomized Trial of Initiation of Chronic Noninvasive Mechanical Ventilation at Home vs In-Hospital in Patients With Neuromuscular Disease and Thoracic Cage Disorder: The Dutch Homerun Trial.

BACKGROUND: There is an increasing demand for home mechanical ventilation (HMV) in patients with chronic respiratory insufficiency. At present, noninvasive ventilation is exclusively initiated in a clinical setting at all four centers for HMV in the Netherlands. In addition to its high societal costs and patient discomfort, commencing HMV is often delayed because of a lack of hospital bed capacity. RESEARCH QUESTION: Is HMV initiation at home, using a telemonitoring approach, noninferior to in-hospital initiation in a nationwide study? STUDY DESIGN AND METHODS: We conducted a nationwide, randomized controlled noninferiority trial, in which every HMV center recruited 24 patients (home [n = 12] vs hospital [n = 12]) with a neuromuscular disease or thoracic cage disorder, all with an indication to start HMV. Change in arterial CO2 (Paco2) over a 6-month period was considered the primary outcome, and quality of life and costs were assessed as secondary outcomes. RESULTS: A total of 96 patients were randomized, most of them diagnosed with neuromuscular disease. We found a significant improvement in Paco2 within both groups (home: from 6.1 to 5.6 kPa [P < .01]; hospital: from 6.3 to 5.6 kPa [P < .01]), with no significant differences between groups. Health-related quality of life showed significant improvement on various subscales; however, no significant differences were observed between the home and hospital groups. From a societal perspective, a cost reduction of more than €3,200 ($3,793) per patient was evident in the home group. INTERPRETATION: This nationwide, multicenter study shows that HMV initiation at home is noninferior to hospital initiation, as it shows the same improvement in gas exchange and health-related quality of life. In fact, from a patient's perspective, it might even be a more attractive approach. In addition, starting at home saves over €3,200 ($3,793) per patient over a 6-month period. TRIAL REGISTRY: ClinicalTrials.gov; No.: NCT03203577; URL: www.clinicaltrials.gov.

Home initiation of chronic non-invasive ventilation in COPD patients with chronic hypercapnic respiratory failure: a randomised controlled trial.

INTRODUCTION: Chronic non-invasive ventilation (NIV) has become evidence-based care for stable hypercapnic COPD patients. While the number of patients increases, home initiation of NIV would greatly alleviate the healthcare burden. We hypothesise that home initiation of NIV with the use of telemedicine in stable hypercapnic COPD is non-inferior to in-hospital NIV initiation. METHODS: Sixty-seven stable hypercapnic COPD patients were randomised to initiation of NIV in the hospital or at home using telemedicine. Primary outcome was daytime arterial carbon dioxide pressure (PaCO2) reduction after 6 months NIV, with a non-inferiority margin of 0.4 kPa. Secondary outcomes were health-related quality of life (HRQoL) and costs. RESULTS: Home NIV initiation was non-inferior to in-hospital initiation (adjusted mean difference in PaCO2 change home vs in-hospital: 0.04 kPa (95% CI -0.31 to 0.38 kPa), with both groups showing a PaCO2 reduction at 6 months compared with baseline (home: from 7.3±0.9 to 6.4±0.8 kPa (p<0.001) and in-hospital: from 7.4±1.0 to 6.4±0.6 kPa (p<0.001)). In both groups, HRQoL improved without a difference in change between groups (Clinical COPD Questionnaire total score-adjusted mean difference 0.0 (95% CI -0.4 to 0.5)). Furthermore, home NIV initiation was significantly cheaper (home: median €3768 (IQR €3546-€4163) vs in-hospital: median €8537 (IQR €7540-€9175); p<0.001). DISCUSSION: This is the first study showing that home initiation of chronic NIV in stable hypercapnic COPD patients, with the use of telemedicine, is non-inferior to in-hospital initiation, safe and reduces costs by over 50%. TRIAL REGISTRATION NUMBER: NCT02652559.

[Diaphragm pacemaker: alternative for long-term ventilatory support; 5 years later]. / Diafragmapacemaker: alternatief voor chronische beademing.

Currently, more than 3000 patients in the Netherlands receive long-term ventilatory support. In the majority of patients, long-term ventilatory support leads to increased survival without any complications. Diaphragm pacing with an external pacemaker (diaphragm pacing system, DPS) seems an attractive alternative for long-term ventilatory support by mask or tracheostomy. Scientific research has since shown that DPS is effective in patients with high cervical paraplegia. In addition, patients with congenital central hypoventilation syndrome are also eligible for DPS. Patients with diaphragm paralysis are a new group of patients who may be eligible for DPS. Two European studies have shown that DPS should not be used in patients with amyotrophic lateral sclerosis. In our experience, patients are no longer completely dependent on a ventilator or may even be able to discontinue using one if the procedure was successful. In the Netherlands, as far as we know, the technique is only used at the University Medical Center Groningen.

[Diaphragm pacemaker: alternative for chronic ventilatory support]. / Diafragmapacemaker: alternatief voor chronische beademing.

Currently, more than 2200 patients in the Netherlands receive chronic ventilatory support. In the majority of patients this leads to increased survival without any complications. Nevertheless, in case of ventilatory support via a mask, problems such as skin irritation, leakage and claustrophobia can occur. In case of tracheostomy, it can lead to increased pulmonary secretion. Diaphragm pacing with an external pacemaker might be an attractive alternative to prevent these symptoms as it can replace ventilatory support by mask or tracheostomy. Current indications are patients with spinal cord injury or with congenital central hypoventilation syndrome who are chronically respiratory insufficient. In our experience, patients can be completely or partially weaned from mechanical ventilation when using the diaphragm pacer. In the Netherlands, the technique is only performed at the University Medical Center Groningen.

[Home mechanical ventilation in the Netherlands]. / Chronische beademing in Nederland.

Over the last 20 years the number of Dutch patients on home mechanical ventilation has increased from 200 to 2000. Home mechanical ventilation is a cost-effective treatment which significantly improves the quality of life of patients. In 2011 83% of patients on home mechanical ventilation in the Netherlands is living at home. In the future further growth can be expected in the number of patients with obesity hypoventilation syndrome and a potential new group of patients with COPD. Strict conditions are necessary to ensure safety in the complex care that home mechanical ventilation entails.