[Non-invasive Mechanical Ventilation in Acute Respiratory Failure. Clinical Practice Guidelines - on behalf of the German Society of Pneumology and Ventilatory Medicine]. / S2k-Leitlinie Nichtinvasive Beatmung als Therapie der akuten respiratorischen Insuffizienz.

The guideline update outlines the advantages as well as the limitations of NIV in the treatment of acute respiratory failure in daily clinical practice and in different indications.Non-invasive ventilation (NIV) has a high value in therapy of hypercapnic acute respiratory failure, as it significantly reduces the length of ICU stay and hospitalization as well as mortality.Patients with cardiopulmonary edema and acute respiratory failure should be treated with continuous positive airway pressure (CPAP) and oxygen in addition to necessary cardiological interventions. This should be done already prehospital and in the emergency department.In case of other forms of acute hypoxaemic respiratory failure with only mild or moderately disturbed gas exchange (PaO2/FiO2 > 150 mmHg) there is no significant advantage or disadvantage compared to high flow nasal oxygen (HFNO). In severe forms of ARDS NIV is associated with high rates of treatment failure and mortality, especially in cases with NIV-failure and delayed intubation.NIV should be used for preoxygenation before intubation. In patients at risk, NIV is recommended to reduce extubation failure. In the weaning process from invasive ventilation NIV essentially reduces the risk of reintubation in hypercapnic patients. NIV is regarded useful within palliative care for reduction of dyspnea and improving quality of life, but here in concurrence to HFNO, which is regarded as more comfortable. Meanwhile NIV is also recommended in prehospital setting, especially in hypercapnic respiratory failure and pulmonary edema.With appropriate monitoring in an intensive care unit NIV can also be successfully applied in pediatric patients with acute respiratory insufficiency.

[Aerosol Therapy in Intensive Care Unit]. / Aerosoltherapie auf Intensivstation.

Inhalation therapy is a cornerstone especially in pulmonary diseases or comorbidities, either in invasive or noninvasive mechanical ventilation. In pediatric patients, mainly in respiratory failure of the premature born child inhalation of surfactant is crucial in the therapy. Additional drugs given by inhalation are antibiotics, mucoactive substances and drugs that treat pulmonary hypertension. This article describes main deposition mechanisms of inhalation therapies and presents recommendations for correct performance of inhalation therapy in invasively as well as noninvasively ventilated patients in ICU. Also safety aspects for patients and medical staff during aerosol therapy in the Corona pandemic era are discussed.

Thick Mucus in ALS: A Mixed-Method Study on Associated Factors and Its Impact on Quality of Life of Patients and Caregivers.

In this explorative mixed-method pilot study, we set out to have a closer look at the largely under-recognized and under-investigated symptom of thick mucus in patients with ALS and its impact on patients and relatives. Thick mucus is a highly distressing symptom for both patients and caregivers. It complicates the use of non-invasive ventilation and is therefore an important prognostic factor of survival. METHODS: In our preliminary study, we used a cross-sectional design, including ten ALS patients with thick mucus who were matched to ten ALS patients without thick mucus. Lung function tests and laboratory and sputum analysis were performed and questionnaires administered in order to determine associated factors of thick mucus accumulation. In a qualitative study using semi-structured interviews, we analysed the impact of thick mucus on patients and caregivers. RESULTS: Reduced respiratory parameters as well as a higher degree of bulbar impairment were associated with the presence of thick mucus. Quality of life of patients and caregivers was strongly impaired by thick mucus accumulation. CONCLUSIONS: Thick mucus in patients with ALS has a strong impact on quality of life. Reduced cough flow and severely impaired bulbar function appear to be indicative parameters. We suggest that healthcare providers actively explore the presence of thick mucus in their patients and that it becomes included in commonly used screening tools.

[Weaning from invasive mechanical ventilation]. / Weaning von invasiver Beatmung.

Weaning from invasive mechanical ventilation is challenging for the ICU team in terms of shortening time of ventilation via endotracheal tube in order to improve the patient's prognosis by early extubation. Thereby prolonged mechanical ventilation (> 14 days), which is associated with risk of tracheotomy and prolonged weaning, shall be avoided. This article will give an overview about weaning categories, causes for weaning failure and strategies to overcome this problem. In the last part we will cover concepts in the process of prolonged weaning including discharge management with invasive mechanical ventilation.

Prolonged Weaning: S2k Guideline Published by the German Respiratory Society.

Mechanical ventilation (MV) is an essential part of modern intensive care medicine. MV is performed in patients with severe respiratory failure caused by respiratory muscle insufficiency and/or lung parenchymal disease; that is, when other treatments such as medication, oxygen administration, secretion management, continuous positive airway pressure (CPAP), or nasal high-flow therapy have failed. MV is required for maintaining gas exchange and allows more time to curatively treat the underlying cause of respiratory failure. In the majority of ventilated patients, liberation or "weaning" from MV is routine, without the occurrence of any major problems. However, approximately 20% of patients require ongoing MV, despite amelioration of the conditions that precipitated the need for it in the first place. Approximately 40-50% of the time spent on MV is required to liberate the patient from the ventilator, a process called "weaning". In addition to acute respiratory failure, numerous factors can influence the duration and success rate of the weaning process; these include age, comorbidities, and conditions and complications acquired during the ICU stay. According to international consensus, "prolonged weaning" is defined as the weaning process in patients who have failed at least 3 weaning attempts, or require more than 7 days of weaning after the first spontaneous breathing trial (SBT). Given that prolonged weaning is a complex process, an interdisciplinary approach is essential for it to be successful. In specialised weaning centres, approximately 50% of patients with initial weaning failure can be liberated from MV after prolonged weaning. However, the heterogeneity of patients undergoing prolonged weaning precludes the direct comparison of individual centres. Patients with persistent weaning failure either die during the weaning process, or are discharged back to their home or to a long-term care facility with ongoing MV. Urged by the growing importance of prolonged weaning, this Sk2 Guideline was first published in 2014 as an initiative of the German Respiratory Society (DGP), in conjunction with other scientific societies involved in prolonged weaning. The emergence of new research, clinical study findings and registry data, as well as the accumulation of experience in daily practice, have made the revision of this guideline necessary. The following topics are dealt with in the present guideline: Definitions, epidemiology, weaning categories, underlying pathophysiology, prevention of prolonged weaning, treatment strategies in prolonged weaning, the weaning unit, discharge from hospital on MV, and recommendations for end-of-life decisions. Special emphasis was placed on the following themes: (1) A new classification of patient sub-groups in prolonged weaning. (2) Important aspects of pulmonary rehabilitation and neurorehabilitation in prolonged weaning. (3) Infrastructure and process organisation in the care of patients in prolonged weaning based on a continuous treatment concept. (4) Changes in therapeutic goals and communication with relatives. Aspects of paediatric weaning are addressed separately within individual chapters. The main aim of the revised guideline was to summarize both current evidence and expert-based knowledge on the topic of "prolonged weaning", and to use this information as a foundation for formulating recommendations related to "prolonged weaning", not only in acute medicine but also in the field of chronic intensive care medicine. The following professionals served as important addressees for this guideline: intensivists, pulmonary medicine specialists, anaesthesiologists, internists, cardiologists, surgeons, neurologists, paediatricians, geriatricians, palliative care clinicians, rehabilitation physicians, intensive/chronic care nurses, physiotherapists, respiratory therapists, speech therapists, medical service of health insurance, and associated ventilator manufacturers.

Position Paper for the State-of-the-Art Application of Respiratory Support in Patients with COVID-19.

Against the background of the pandemic caused by infection with the SARS-CoV-2 virus, the German Respiratory Society has appointed experts to develop therapy strategies for COVID-19 patients with acute respiratory failure (ARF). Here we present key position statements including observations about the pathophysiology of (ARF). In terms of the pathophysiology of pulmonary infection with SARS-CoV-2, COVID-19 can be divided into 3 phases. Pulmonary damage in advanced COVID-19 often differs from the known changes in acute respiratory distress syndrome (ARDS). Two types (type L and type H) are differentiated, corresponding to early- and late-stage lung damage. This differentiation should be taken into consideration in the respiratory support of ARF. The assessment of the extent of ARF should be based on arterial or capillary blood gas analysis under room air conditions, and it needs to include the calculation of oxygen supply (measured from the variables of oxygen saturation, hemoglobin level, the corrected values of Hüfner's factor, and cardiac output). Aerosols can cause transmission of infectious, virus-laden particles. Open systems or vented systems can increase the release of respirable particles. Procedures in which the invasive ventilation system must be opened and endotracheal intubation carried out are associated with an increased risk of infection. Personal protective equipment (PPE) should have top priority because fear of contagion should not be a primary reason for intubation. Based on the current knowledge, inhalation therapy, nasal high-flow therapy (NHF), continuous positive airway pressure (CPAP), or noninvasive ventilation (NIV) can be performed without an increased risk of infection to staff if PPE is provided. A significant proportion of patients with ARF present with relevant hypoxemia, which often cannot be fully corrected, even with a high inspired oxygen fraction (FiO2) under NHF. In this situation, the oxygen therapy can be escalated to CPAP or NIV when the criteria for endotracheal intubation are not met. In ARF, NIV should be carried out in an intensive care unit or a comparable setting by experienced staff. Under CPAP/NIV, a patient can deteriorate rapidly. For this reason, continuous monitoring and readiness for intubation are to be ensured at all times. If the ARF progresses under CPAP/NIV, intubation should be implemented without delay in patients who do not have a "do not intubate" order.

Prolonged Weaning from Mechanical Ventilation.

BACKGROUND: To accommodate the increasing number of patients requiring prolonged weaning from mechanical ventilation, specialized weaning centers have been established for patients in whom weaning on the intensive care unit (ICU) was unsuccessful. METHODS: This study aimed to determine both the outcome of treatment and the factors associated with prolonged weaning in patients who were transferred from the ICU to specialized weaning centers in Germany during the period 2011 to 2015, based on a nationwide registry covering all specialized weaning centers currently going through the process of accreditation by the German Respiratory Society. RESULTS: Of 11 424 patients, 7346 (64.3%) were successfully weaned, of whom 2236 were switched to long-term non-invasive ventilation; 1658 (14.5%) died in the weaning unit; and 2420 (21.2%) could not be weaned. The duration of weaning decreased significantly from 22 to 18 days between 2011 and 2015 (p <0.0001). Multivariate analysis revealed that the factor most strongly associated with in-hospital mortality was advanced age (odds ratio [OR] 11.07, 95% confidence interval [6.51; 18.82], p <0.0001). The need to continue with invasive ventilation was most strongly associated with the duration mechanical ventilation prior to transfer from the ICU (OR 4.73 [3.25; 6.89]), followed by a low body mass index (OR 0.38 [0.26; 0.58]), pre-existing neuromuscular disorders (OR 2.98 [1.88; 4.73]), and advanced age (OR 2.96 [1.87; 4.69]) (each p <0.0001). CONCLUSION: Weaning duration has decreased over time, but prolonged weaning is still unsuccessful in one third of patients.Overall, the results warrant the establishment of specialized weaning centers. Variables associated with death and weaningfailure can be integrated into ICU decision-making processes.

Nasal high-flow versus noninvasive ventilation in patients with chronic hypercapnic COPD.

Background: Despite the encouraging results of noninvasive ventilation (NIV) in chronic hypercapnic COPD patients, it is also evident that some patients do not tolerate NIV or do not benefit from it. We conducted a study in which COPD patients with stable, chronic hypercapnia were treated with NIV and nasal high-flow (NHF) to compare effectiveness. Methods: In a multi-centered, randomized, controlled, cross-over design, patients received 6 weeks of NHF ventilation followed by 6 weeks of NIV ventilation or vice-versa (TIBICO) between 2011 and 2016. COPD patients with stable daytime hypercapnia (pCO2≥50 mmHg) were recruited from 13 German centers. The primary endpoint was pCO2 changes from baseline blood gas, lung function, quality of life (QoL), the 6 min walking test, and duration of device use were secondary endpoints. Results: A total of 102 patients (mean±SD) age 65.3±9.3 years, 61% females, body mass index 23.1±4.8 kg/m2, 90% GOLD D, pCO2 56.5±5.4 mmHg were randomized. PCO2 levels decreased by 4.7% (n=94; full analysis set; 95% CI 1.8-7.5, P=0.002) using NHF and 7.1% (95% CI 4.1-10.1, P<0.001) from baseline using NIV (indistinguishable to intention-to-treat analysis). The difference of pCO2 changes between the two devices was -1.4 mmHg (95% CI -3.1-0.4, P=0.12). Both devices had positive impact on blood gases and respiratory scores (St. George's Respiratory Questionnaire, Severe Respiratory Insufficiency Questionnaire). Conclusions: NHF may constitute an alternative to NIV in COPD patients with stable chronic hypercapnia, eg, those not tolerating or rejecting NIV with respect to pCO2 reduction and improvement in QoL.

COPD Advanced Patient Management.

COPD is the third leading cause of death in the United States, with current rates of both morbidity and mortality persisting and contributing significantly to long-term disability. More than 11 million Americans are diagnosed with COPD, with an additional 13 million people estimated to be living with undiagnosed disease. For patients diagnosed with COPD, the turning point will be hospitalization. It is important, therefore, that new treatment techniques that manage the signs and symptoms of the COPD and impact the prevalence and severity of exacerbations, hospital admissions, quality of life, and activities of daily living, and innovative clinical management strategies that optimize hospital discharge planning, all show promise in improving outcomes for patients with COPD. In particular, readmissions following COPD hospitalization are associated with high morbidity, mortality, and costs of care, and therefore hospital readmissions are receiving close scrutiny as an opportunity to improve patient care. To this end, programs to assess the presence and severity of dyspnea, and secretion burden and clearance, through implementation of a telemedicine program, use of noninvasive ventilation or supplemental oxygen, and development of a comprehensive self-management program have all been found to be variously effective as elements of a posthospitalization treatment plan. In this series of multi-media presentations and roundtable discussions published in CHEST (available at http://journal.cme.chestnet.org/copd-advanced-patient), leading international faculties discuss some of these specific interventions in detail to provide clinicians with possible solutions to the challenges of managing their patients with advanced COPD.

German National Guideline for Treating Chronic Respiratory Failure with Invasive and Non-Invasive Ventilation: Revised Edition 2017 - Part 1.

Today, invasive and non-invasive home mechanical ventilation have become a well-established treatment option. Consequently, in 2010, the German Respiratory Society (Deutsche Gesellschaft für Pneumologie und Beatmungsmedizin, DGP) has leadingly published the Guidelines on "Non-Invasive and Invasive Mechanical Ventilation for Treatment of Chronic Respiratory Failure." However, continuing technical evolutions, new scientific insights, and health care developments require an extensive revision of the Guidelines. For this reason, the updated Guidelines are now published. Thereby, the existing chapters, namely technical issues, organizational structures in Germany, qualification criteria, disease-specific recommendations including special features in pediatrics as well as ethical aspects and palliative care, have been updated according to the current literature and the health care developments in Germany. New chapters added to the Guidelines include the topics of home mechanical ventilation in paraplegic patients and in those with failure of prolonged weaning. In the current Guidelines, different societies as well as professional and expert associations have been involved when compared to the 2010 Guidelines. Importantly, disease-specific aspects are now covered by the German Interdisciplinary Society of Home Mechanical Ventilation (DIGAB). In addition, societies and associations directly involved in the care of patients receiving home mechanical ventilation have been included in the current process. Importantly, associations responsible for decisions on costs in the health care system and patient organizations have now been involved.

German National Guideline for Treating Chronic Respiratory Failure with Invasive and Non-Invasive Ventilation - Revised Edition 2017: Part 2.

Today, invasive and non-invasive home mechanical ventilation have become a well-established treatment option. Consequently, in 2010, the German Respiratory Society (DGP) has leadingly published the guidelines on "Non-Invasive and Invasive Mechanical Ventilation for Treatment of Chronic Respiratory Failure." However, continuing technical evolutions, new scientific insights, and health care developments require an extensive revision of the guidelines. For this reason, the updated guidelines are now published. Thereby, the existing chapters, namely technical issues, organizational structures in Germany, qualification criteria, disease-specific recommendations including special features in pediatrics as well as ethical aspects and palliative care, have been updated according to the current literature and the health care developments in Germany. New chapters added to the guidelines include the topics of home mechanical ventilation in paraplegic patients and in those with failure of prolonged weaning. In the current guidelines, different societies as well as professional and expert associations have been involved when compared to the 2010 guidelines. Importantly, disease-specific aspects are now covered by the German Interdisciplinary Society of Home Mechanical Ventilation (DIGAB). In addition, societies and associations directly involved in the care of patients receiving home mechanical ventilation have been included in the current process. Importantly, associations responsible for decisions on costs in the health care system and patient organizations have now been involved.

MOR103, a human monoclonal antibody to granulocyte-macrophage colony-stimulating factor, in the treatment of patients with moderate rheumatoid arthritis: results of a phase Ib/IIa randomised, double-blind, placebo-controlled, dose-escalation trial.

OBJECTIVES: To determine the safety, tolerability and signs of efficacy of MOR103, a human monoclonal antibody to granulocyte-macrophage colony-stimulating factor (GM-CSF), in patients with rheumatoid arthritis (RA). METHODS: Patients with active, moderate RA were enrolled in a randomised, multicentre, double-blind, placebo-controlled, dose-escalation trial of intravenous MOR103 (0.3, 1.0 or 1.5 mg/kg) once a week for 4 weeks, with follow-up to 16 weeks. The primary outcome was safety. RESULTS: Of the 96 randomised and treated subjects, 85 completed the trial (n=27, 24, 22 and 23 for pooled placebo and MOR103 0.3, 1.0 and 1.5 mg/kg, respectively). Treatment emergent adverse events (AEs) in the MOR103 groups were mild or moderate in intensity and generally reported at frequencies similar to those in the placebo group. The most common AE was nasopharyngitis. In two cases, AEs were classified as serious because of hospitalisation: paronychia in a placebo subject and pleurisy in a MOR103 0.3 mg/kg subject. Both patients recovered fully. In exploratory efficacy analyses, subjects in the MOR103 1.0 and 1.5 mg/kg groups showed significant improvements in Disease Activity Score-28 scores and joint counts and significantly higher European League Against Rheumatism response rates than subjects receiving placebo. MOR103 1.0 mg/kg was associated with the largest reductions in disease activity parameters. CONCLUSIONS: MOR103 was well tolerated and showed preliminary evidence of efficacy in patients with active RA. The data support further investigation of this monoclonal antibody to GM-CSF in RA patients and potentially in those with other immune-mediated inflammatory diseases. TRIAL REGISTRATION NUMBER: NCT01023256.

Non-invasive positive pressure ventilation for the treatment of severe stable chronic obstructive pulmonary disease: a prospective, multicentre, randomised, controlled clinical trial.

BACKGROUND: Evidence is weak for the ability of long-term non-invasive positive pressure ventilation (NPPV) to improve survival in patients with stable hypercapnic chronic obstructive pulmonary disease (COPD). Previous prospective studies did not target a reduction in hypercapnia when adjusting ventilator settings. This study investigated the effect of long-term NPPV, targeted to markedly reduce hypercapnia, on survival in patients with advanced, stable hypercapnic COPD. METHODS: This investigator-initiated, prospective, multicentre, randomised, controlled clinical trial enrolled patients with stable GOLD stage IV COPD and a partial carbon dioxide pressure (PaCO2) of 7 kPa (51.9 mm Hg) or higher and pH higher than 7.35. NPPV was targeted to reduce baseline PaCO2 by at least 20% or to achieve PaCO2 values lower than 6.5 kPa (48.1 mm Hg). Patients were randomly assigned (in a 1:1 ratio) via a computer-generated randomisation sequence with a block size of four, to continue optimised standard treatment (control group) or to receive additional NPPV for at least 12 months (intervention group). The primary outcome was 1-year all-cause mortality. Analysis was by intention to treat. The intervention was unblinded, but outcome assessment was blinded to treatment assignment. This study is registered with ClinicalTrials.gov, number NCT00710541. FINDINGS: Patients were recruited from 36 respiratory units in Germany and Austria, starting on Oct 29, 2004, and terminated with a record of the vital status on July 31, 2011. 195 patients were randomly assigned to the NPPV group (n=102) or to the control group (n=93). All patients from the control group and the NPPV group were included in the primary analysis. 1-year mortality was 12% (12 of 102 patients) in the intervention group and 33% (31 of 93 patients) in the control group; hazard ratio 0.24 (95% CI 0.11-0.49; p=0.0004). 14 (14%) patients reported facial skin rash, which could be managed by changing the type of the mask. No other intervention-related adverse events were reported. INTERPRETATION: The addition of long-term NPPV to standard treatment improves survival of patients with hypercapnic, stable COPD when NPPV is targeted to greatly reduce hypercapnia. FUNDING: German Lung Foundation; ResMed, Germany; Tyco Healthcare, Germany; and Weinmann, Germany.

Haemodynamic changes in pulmonary hypertension in patients with interstitial lung disease treated with PDE-5 inhibitors.

BACKGROUND AND OBJECTIVE: Interstitial lung diseases (ILD) are often associated with pulmonary hypertension (PH). This study aimed to evaluate the therapeutic benefit of phosphodiesterase-5 (PDE-5) inhibitors in pulmonary hypertension secondary to ILD. METHODS: Patients with ILD and PH were treated with sildenafil or tadalafil. Right heart catheterization was performed before and after a minimum of 3-month treatment. In addition, lung function, 6-min walk distance (6MWD) and plasma brain natriuretic peptide (BNP) concentration were assessed. RESULTS: Ten ILD patients (three female, mean age 64.4 ± 9.0 years, six with idiopathic pulmonary fibrosis (IPF), four with hypersensitivity pneumonitis, (HP)) with significant precapillary PH (mean pulmonary artery pressure (PAPm) ≥ 25 mmHg, pulmonary vascular resistance (PVR) > 280 dyn*s*cm(-5) ; pulmonary artery wedge pressure (PAWPm) ≤ 15 mmHg) were treated with either sildenafil (n = 5) or tadalafil (n = 5). Pulmonary haemodynamics were severely impaired at baseline (PAPm 42.9 ± 5.4 mmHg; cardiac index (CI) 2.7 ± 0.6 L/min/m2; PVR 519 ± 131 dyn × sec × cm(-5)). After mean follow-up of 6.9 ± 5.8 months an increase in CI (2.9 ± 0.7 L/min/m2 , P = 0.04) and a decrease in PVR (403 ± 190 dyn × sec × cm(-5) , P = 0.03) were observed. 6MWD and BNP did not change significantly. CONCLUSIONS: Our data suggest that treatment with PDE-5 inhibitors improves pulmonary haemodynamic patients with PH secondary to ILD.

Invasive home mechanical ventilation, mainly focused on neuromuscular disorders.

INTRODUCTION AND BACKGROUND: Invasive home mechanical ventilation is used for patients with chronic respiratory insufficiency. This elaborate and technology-dependent ventilation is carried out via an artificial airway (tracheal cannula) to the trachea. Exact numbers about the incidence of home mechanical ventilation are not available. Patients with neuromuscular diseases represent a large portion of it. RESEARCH QUESTIONS: Specific research questions are formulated and answered concerning the dimensions of medicine/nursing, economics, social, ethical and legal aspects. Beyond the technical aspect of the invasive home, mechanical ventilation, medical questions also deal with the patient's symptoms and clinical signs as well as the frequency of complications. Economic questions pertain to the composition of costs and the differences to other ways of homecare concerning costs and quality of care. Questions regarding social aspects consider the health-related quality of life of patients and caregivers. Additionally, the ethical aspects connected to the decision of home mechanical ventilation are viewed. Finally, legal aspects of financing invasive home mechanical ventilation are discussed. METHODS: Based on a systematic literature search in 2008 in a total of 31 relevant databases current literature is viewed and selected by means of fixed criteria. Randomized controlled studies, systematic reviews and HTA reports (health technology assessment), clinical studies with patient numbers above ten, health-economic evaluations, primary studies with particular cost analyses and quality-of-life studies related to the research questions are included in the analysis. RESULTS AND DISCUSSION: Invasive mechanical ventilation may improve symptoms of hypoventilation, as the analysis of the literature shows. An increase in life expectancy is likely, but for ethical reasons it is not confirmed by premium-quality studies. Complications (e. g. pneumonia) are rare. Mobile home ventilators are available for the implementation of the ventilation. Their technical performance however, differs regrettably. Studies comparing the economic aspects of ventilation in a hospital to outpatient ventilation, describe home ventilation as a more cost-effective alternative to in-patient care in an intensive care unit, however, more expensive in comparison to a noninvasive (via mask) ventilation. Higher expenses arise due to the necessary equipment and the high expenditure of time for the partial 24-hour care of the affected patients through highly qualified personnel. However, none of the studies applies to the German provisionary conditions. The calculated costs strongly depend on national medical fees and wages of caregivers, which barely allows a transmission of the results. The results of quality-of-life studies are mostly qualitative. The patient's quality of life using mechanical ventilation is predominantly considered well. Caregivers of ventilated patients report positive as well as negative ratings. Regarding the ethical questions, it was researched which aspects of ventilation implementation will have to be considered. From a legal point of view the financing of home ventilation, especially invasive mechanical ventilation, requiring specialised technical nursing is regulated in the code of social law (Sozialgesetzbuch V). The absorption of costs is distributed to different insurance carriers, who often, due to cost pressures within the health care system, insurance carriers, who consider others and not themselves as responsible. Therefore in practice, the necessity to enforce a claim of cost absorption often arises in order to exercise the basic right of free choice of location. CONCLUSION: Positive effects of the invasive mechanical ventilation (overall survival and symptomatic) are highly probable based on the analysed literature, although with a low level of evidence. An establishment of a home ventilation registry and health care research to ascertain valid data to improve outpatient structures is necessary. Gathering specific German data is needed to adequately depict the national concepts of provision and reimbursement. A differentiation of the cost structure according to the type of chosen outpatient care is currently not possible. There is no existing literature concerning the difference of life quality depending on the chosen outpatient care (homecare, assisted living, or in a nursing home specialised in invasive home ventilation). Further research is required. For a so called participative decision - made by the patient after intense counselling - an early and honest patient education pro respectively contra invasive mechanical ventilation is needed. Besides the long term survival, the quality of life and individual, social and religious aspects have also to be considered.

When should home mechanical ventilation be started in patients with different neuromuscular disorders?

BACKGROUND AND OBJECTIVES: Current international consensus guidelines identify a number of indicators for the establishment of home mechanical ventilation (HMV) for patients with neuromuscular diseases but do not address the possible clinical differences between each of the underlying disorders. This study assessed the differences in the physiological parameters of patients with neuromuscular disease commenced on HMV for the treatment of symptomatic chronic hypercapnic respiratory failure. METHODS: Patients commenced on HMV for the treatment of symptomatic chronic hypercapnic respiratory failure over a 9-year period were studied. Physiological parameters at the time of referral for HMV, impact of HMV and survival were analysed. RESULTS: The study recruited 66 patients with neuromuscular disease. Thirty-one patients had rapidly progressive disease: amyotrophic lateral sclerosis (ALS, n = 19), Duchenne muscular dystrophy (DMD, n = 12) and 35 patients had slowly progressive disease. Mean FVC at HMV onset was 40.3 +/- 17.5% predicted in all patients, but was >50% predicted in eight patients (12%). ALS patients were more hypercapnic (P = 0.03) and more hypoxaemic (P < 0.001), but had better FEV(1) at HMV onset, compared with DMD patients (P = 0.005). Maximal inspiratory mouth occlusion pressure (PImax) was 3.0 +/- 1.6 kPa in all patients, but values were lower compared with international consensus guidelines (5.88 kPa). Median survival in DMD, slowly progressive diseases and ALS was 132, 82 and 16 months, respectively (P < 0.001). CONCLUSIONS: Blood gases and lung function parameters vary substantially between patients with differing underlying neuromuscular disorders when commenced on HMV for the treatment of symptomatic chronic hypercapnic respiratory failure. In contrast, PImax is equally reduced in all patients and more severely reduced compared with consensus guidelines. The specific underlying neuromuscular disease has a major impact on outcome. Specific selection criteria are needed for the use of HMV in the different diseases that comprise neuromuscular disorders.