[Guideline for Long-Term Oxygen Therapy - S2k-Guideline Published by the German Respiratory Society]. / Leitlinie zur Langzeit-Sauerstofftherapie.

Long-term oxygen therapy is of great importance both for reducing mortality and for improving performance in patients with chronic lung diseases. The prerequisites for Long-term oxygen therapy are adequate diagnostics and clearly defined indication. A causal distinction into chronic hypoxaemic and hypercapnic respiratory failure is reasonable, from which the differential indication for non-invasive ventilation results.The revised guideline covers the diagnostics and indication of chronic lung and heart diseases, the role of oxygen in terminal illness and gives a detailed description of available oxygen devices. The guideline is intended to help avoid undersupply, oversupply and false prescriptions. Furthermore, the chapter "Postacute Oxygen Therapy" discusses the procedure, relevant in everyday life, but not yet clearly defined, for prescribing oxygen therapy for the home at the end of an inpatient stay. Another important point, the correct prescription of mobile oxygen systems, is also presented in the guideline. This document is a revised version of the guideline for longterm oxygen therapy and replaces the version of 2008.

[Guidelines for Non-Invasive and Invasive Home Mechanical Ventilation for Treatment of Chronic Respiratory Failure - Update 2017]. / S2k-Leitlinie: Nichtinvasive und invasive Beatmung als Therapie der chronischen respiratorischen Insuffizienz ­ Revision 2017.

Today, invasive and non-invasive home mechanical ventilation have become a well-established treatment option. Consequently, in 2010 the German Society of Pneumology and Mechanical Ventilation (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.The currently updated guidelines are valid for the next three years, following their first online publication on the home page of the Association of the Scientific Medical Societies in German (AWMF) in the beginning of July 2017. A subsequent revision of the guidelines remains the aim for the future.

[Non-invasive and invasive mechanical ventilation for treatment of chronic respiratory failure. S2-Guidelines published by the German Medical Association of Pneumology and Ventilatory Support]. / Nichtinvasive und invasive Beatmung als Therapie der chronischen respiratorischen Insuffizienz. S2-Leitlinie herausgegeben von der Deutschen Gesellschaft für Pneumologie und Beatmungsmedizin e. V.

The field of mechanical ventilation is highly important in pulmonary medicine. The German Medical Association of Pneumology and Ventilatory Support ["Deutsche Gesellschaft für Pneumologie und Beatmungsmedizin e. V. (DGP)"] therefore has formulated these guidelines for home mechanical non-invasive and invasive ventilation. Non-invasive home mechanical ventilation can be administered using various facial masks; invasive home mechanical ventilation is performed via a tracheostomy. Home mechanical ventilation is widely and increasingly accepted as a treatment option for chronic ventilatory failure which most often occurs in COPD, restrictive lung diseases, obesity-hypoventilation syndrome and neuromuscular disorders. Essential for the initiation of home mechanical ventilation are the presence of symptoms of ventilatory failure and the detection of hypoventilation, most importantly hypercapnia. These guidelines comprise general indication criteria along with disease-specific criteria summarised by treatment algorithms. In addition, the management of bronchial secretions and care of paediatric patients are addressed. Home mechanical ventilation must be organised around a specialised respiratory care centre with expertise in patient selection, the initiation and the control of home mechanical ventilation. In this regard, the guidelines provide detailed information about technical requirements (equipment), control and settings of mechanical ventilation as well as organisation of patient care. A key requirement for home mechanical ventilation is the qualification of specialised home-care services, which is addressed in detail. Independent living and the quality of respiratory care are of highest priority in patients receiving home mechanical ventilation, since home mechanical ventilation can interfere with the integrity of a patient and often marks a life-sustaining therapy. Home mechanical ventilation has been shown to improve health-related quality of life of patients with chronic ventilatory failure. Long-term survival is improved in most patient groups, even though the long-term prognosis is often severely limited. For this reason, ethical issues regarding patient education, communication with ventilated patients at the end of life, living will, testament and medical care during the dying process are discussed.

[Palliative care in respiratory medicine]. / Palliativmedizin in der Pneumologie.

Palliative care should be part of respiratory medicine for two reasons: first, many respiratory diseases--besides thoracic tumours--need palliative care in the late stages of the disease. Second, dyspnoea is a common symptom in advanced, primary extrapulmonary diseases and the knowledge of respiratory specialists can be beneficial in the treatment of this symptom. In this paper we describe frequent symptoms of advanced pulmonary diseases and their treatment. Moreover, we focus on the structure of palliative care in Germany.

[Weaning from mechanical ventilation. A survey of the situation in pneumologic respiratory facilities in Germany]. / Entwöhnung von der Beatmungstherapie. Eine Erhebung zur Situation pneumologischer Beatmungszentren in Deutschland.

BACKGROUND AND OBJECTIVE: The prevalence of difficult or prolonged weaning from mechanical ventilation is increasing because of a growing number of elderly patients with multiple diseases and pulmonary problems requiring mechanical ventilation. Intensive care units (ICU) are inclined to refer to specialized unit those patients who are difficult to wean. A nationwide survey of German facilities was conducted and this article reports the current state of weaning centers staffed by chest physicians. PATIENTS AND METHODS: 38 centers participated in the survey, which was divided into 10 items, covering characteristics of the hospital, weaning strategies, patients and outcomes during 2006. The survey included 2718 patients in whom weaning was difficult or prolonged. Almost three quarters of patients were transferred to one of the weaning centers from the ICU of another hospital. RESULTS: The weaning success rate was 66.4%. In 31,9 % of patients home mechanical ventilation was started after they had been weaned. The overall hospital mortality rate was 20.8%. There were major differences between individual centres concerning the number of patients, organization of the weaning unit and weaning strategies. CONCLUSIONS: Weaning was successful in two thirds of patients who had been on prolonged mechanical ventilation and had then been transferred to weaning facilities staffed by chest physicians. These centres effectively improved the quality of care of patients on prolonged mechanical ventilation by avoiding long-term invasive ventilation and sparing cost-intensive ICU resources. The problems that still exist may be overcome by a network of weaning facilities.

[Mechanical ventilation in chronic ventilatory insufficiency]. / Beatmung bei chronisch ventilatorischer Insuffizienz.

Mechanical ventilation has become an important treatment option in chronic ventilatory failure. There are different diseases which lead to ventilatory failure and to home mechanical ventilation (HMV). A primary loss of in- and expiratory muscle strength is the reason for respiratory deterioration in neuromuscular disease. In most of these diseases ventilatory failure develops because of the progressive character of muscular damage. Initially, ventilatory failure can be found during night-time. In the case of hypercapnia at daytime, life expectancy is strongly reduced, especially in amyotrophic lateral sclerosis and Duchenne muscular dystrophy. HMV leads to a prolongation of life and to an increase in quality of life, if bulbar involvement is not severe. Impressive clinical improvements under HMV have been found in restrictive disorders of the rib cage like kyphoscoliosis or posttuberculosis sequelae, with an increase of quality of life, walking distance and a decrease in pulmonary hypertension. Only few data are published about long-term results of HMV in Obesity Hypoventilation. In terms of retrospective analyses of clinical data HMV seems to improve survival in this population. Some patients only need CPAP treatment, but most patients have to be treated with ventilatory support. The application of HMV in patients with chronic ventilatory failure due to chronic obstructive pulmonary disease (COPD) is growing, but there are controversial results in randomised clinical trials. Analysis of these data suggest better results of HMV in patients with severe hypercapnia, with the application of higher effective ventilatory pressure and a ventilator mode with a significant reduction in the work of breathing. Under such conditions HMV leads to a reduction of hypercapnia, an improvement in sleep quality, walking distance and quality of life, but until now there is no evidence in reduction of mortality in COPD.

[Respiratory therapist: introduction of a new profession]. / Respiratory Therapist -- Atmungstherapeut. Einführung eines neuen Berufsbildes.

Respiratory therapists are healthcare professionals taking care of patients with pulmonary disorders. They are an allied health specialty, practicing under medical direction. The professionalism of nurses and therapists must grow up to act successfully in new fields of medicine, where evidence-based independent action is necessary. Specialized therapists can help us coordinate separated processes (diagnoses, therapy and nursing). The profession "Respiratory Therapist" was created in the United States 50 years ago. We intend to introduce this profession also in Germany. We follow many other countries who have already taken this step. We hope that we can reach yet a higher quality of patient care.

[Noninvasive mechanical ventilation in patients with stable severe COPD]. / Die nichtinvasive Beatmung bei Patienten mit stabiler, schwergradiger chronisch-obstruktiver Bronchitis und Lungenemphysem (COPD).

Noninvasive positive pressure ventilation in patients with stable chronic obstructive pulmonary disease. The role of non-invasive positive pressure ventilation (NIPPV) is well documented in patients with restrictive thoracic diseases like kyphoscoliosis, tuberculosis sequelae or neuromuscular disease. There is also a good evidence for the use of NIPPV in acute respiratory failure in patients with an exacerbation of COPD. The application of NIPPV in patients with chronic respiratory failure is growing, but there is less evidence than in restrictive disorders. NIPPV can unload the respiratory muscles in patients with chronic hypercapnic COPD and so alleviates fatigue of the respiratory pump, but improvement in the maximal inspiratory pressure (Pi (max)) is small or even absent. An improvement of sleep quality has also postulated, there was an increase in total sleep time and sleep effectiveness when using higher inspiratory pressure. An increase of the walking distance was shown in short term studies, only. In most studies, there was an increase in quality of life as a main topic. Mortality was unchanged in the two long-term randomised controlled studies. Current data suggest a possible role of NIPPV in patients with severe hypercapnia. A high effective inspiratory pressure and a ventilator mode with a significant reduction in the work of breathing should be choosen. NIPPV should be started in hospital, a close reassessment must be performed. Patients who accepted NIPPV in the first weeks had a good compliance for long-term use.

[Intermittent assisted ventilation in neuromuscular diseases: course and quality of life]. / Intermittierende Selbstbeatmung bei neuromuskulären Erkrankungen: Verlauf und Lebensqualität.

Intermittent non-invasive home ventilation is expected to improve the quality of life, but progression of underlying neuromuscular diseases may lead to a deterioration. We observed after 82-1085 days of such home mechanical ventilation (7 patients with muscular dystrophy (age [mean/std.) 33 +/- 15 years), 8 patients with amyotrophic lateral sclerosis (ALS. age 60 +/- 8 years) a nonsignificant decrease of vital capacity (1.6 +/- 0.4 rp. 1.2 +/- 0.4 l) and an improvement of hypercapnia (49.3 +/- 8.5 rp. 43 +/- 18.5 mmHg). Quality of life (SF-36, Medical Outcomes Trust, Boston, USA) increased significantly only for mental health in patients with ALS (55 +/- 13 rp. 64 +/- 17%). Despite progression of the underlying disease the quality of life remained stable under home mechanical ventilation and mental health improved.

[Use of home mechanical ventilation in patients with high grade chronic obstructive lung disease (COPD)]. / Einleitung einer Heimbeatmungstherapie bei Patienten mit schwergradiger chronisch-obstruktiver Atemwegserkrankung (COPD).

UNLABELLED: Nasal intermittent positive pressure ventilation (NIPPV) ist well established in the treatment of chronic hypercapnic ventilatory failure in patients with scoliosis or neuromuscular diseases. It has been introduced in acute respiratory failure in patients with COPD. The role of NIPPV in the long term treatment in severe stable hypercapnic COPD patients has not been well established (Thorax 1996; 51: 455-7). PATIENTS AND METHODS: We analysed the results of blood gases and lung function in all stable chronic hypercapnic COPD patients (PaCO2 59 +/- 6 mmHg), who underwent a trial of NIPPV from 11/95 to 1/98 (n = 25; 12 f/13 m; mean age 62 +/- 10 years). Patients with acute respiratory failure or an additional obstructive sleep apnoea syndrome were excluded. NIPPV was performed over an individual hand-molded nasal mask in the assisted/controlled mode (4 volume-, 21 pressure-cycled). At the time of discharge (25 +/- 15 days after the initiation of NIPPV) patients were able to apply the ventilator during night for al least 6 h. At the time PaCO2 during NIPPV was 43 +/- 6 mmHg. RESULTS: 5 patients failed to continue NIPPV for long-term treatment, so it was discontinued after a period of 6 weeks. 20 patients (80%) continued NIPPV for 13 +/- 8 months (range 1-27 months), 2 patients died during NIPPV (after 1 and 13 months). NIPPV had no significant influence on lung function (FEV1 predicted 28 +/- 13 vs. 30 +/- 11%; intrathoracic lung volume 6.9 +/- 2.5 vs. 6.2 +/- 1.7 l) or respiratory muscle strength (Pimax 4.2 +/- 0.9 vs. 4.5 +/- 1.6 kPa). However, we observed a significant improvement in PaCO2 during spontaneous breathing (59 +/- 6 vs. 48 +/- 8 mmHg; p < 0.001) and in case of base excess (7.4 +/- 4.1 vs. 3.4 +/- 2.4 mmol/l; p < 0.003). CONCLUSION: NIPPV can improve hypercapnic ventilatory failure in a subgroup of severe stable COPD, provided patients are motivated and home mechanical ventilation is adequately performed.

[Acceptance and long-term results of home mechanical ventilation in various thoracic diseases]. / Akzeptanzraten und Langzeitergebnisse der Heimbeatmung bei verschiedenen Erkrankungen.

BACKGROUND: Home mechanical ventilation (HMV) is an important therapy for patients with respiratory insufficiency on the basis of neuromuscular diseases (NMD), chest wall deformities (CWD) and chronic obstructive pulmonary disease (COPD). PATIENTS AND METHODS: We retrospectively analyzed the long-term results of all 144 patients (CWD = 47, COPD = 54, NMD = 43) who underwent a trial of non-invasive HMV from March 1990 to September 1997. RESULTS: Twenty-eight patients did not accept the HMV (19%), 7 with CWD (15%), 17 with COPD (32%) and 4 with NMD (9%). Thirty-nine of 113 patients, who accepted HMV, completed nasal ventilation for a minimum of 1 year. For all 3 groups the hypercapnia improved significantly (CWD 58 +/- 6 to 48 +/- 4 mm Hg, p < 0.001, COPD 61 +/- 7 to 46 +/- 6 mm Hg, p < 0.001, NMD 53 +/- 8 to 42 +/- 6 mm Hg, p < 0.001). CONCLUSION: HMV improves the hypercapnic ventilatory failure independent of the underlying disease. The rate of acceptance is lower in patients with COPD in comparison to CWD and NMD.

[Quality of life of various patient groups during home mechanical ventilation]. / Anderung der Lebensqualität unter intermittierender Selbstbeatmung bei unterschiedlichen Patienten-gruppen.

BACKGROUND: During home mechanical ventilation quality of life depends on improvement of ventilation and progress of the underlying disease. PATIENTS AND METHODS: Patients with chronic obstructive pulmonary disease (COPD; n = 11), neuromuscular diseases (NMD; n = 8) and scoliosis (n = 8) answered before and after 306 +/- 232 (64 to 910) days home mechanical ventilation a standardized and validated questionnaire (SF 36, Medical Outcomes Trust, Boston, USA). RESULTS: For all patients together physical functioning (16 +/- 22 to 24 +/- 26%), general health (33 +/- 15 to 41 +/- 20%) and vitality (25 +/- 16 to 45 +/- 22%) improved significantly (p < 0.05). For COPD vitality (22 +/- 15 to 46 +/- 22%), for patients with NMD mental health (62 +/- 9 to 70 +/- 10%) and for patients with scoliosis vitality (35 +/- 15 to 59 +/- 22%) and mental health (61 +/- 11 to 74 +/- 4%) improved. CONCLUSION: Home mechanical ventilation improves quality of life, but the improvement depends on the underlying disease.

[The etiology of chronic hypercapnia]. / Die Ursache der chronischen Hyperkapnie.

BACKGROUND: The ventilatory and the pressure response to CO2 in patients with advanced thoracic disorders are critically dependent on the mechanics of the lung and the respiratory muscles. Changes in drive, therefore, can not be directly assessed with that method. However during changes as a result of intermittent mechanical ventilation, changes in drive can be assessed, if lung and muscle mechanics remain unaffected. In addition, to study changes in ventilatory drive independently in patients successfully treated by intermittent mechanical ventilation, we determined the recruitment threshold, pCO2RT, of the unloaded ventilatory pump to CO2. PATIENTS: 16 patients with various disorders (4 COPD, 4 COPD and sleep apnoea, 7 scoliosis, 1 fibrothorax) were studied, 14 during nasal IPPV and 2 during mechanical ventilation via tracheostomy. RESULTS: After they had been successfully adapted to the ventilator, they were entered into the study. The apnoea threshold in all cases had already been reached during the adaptation period. pCO2AT was determined 32 +/- 5 mm Hg. While the patients were passively ventilated, the inspiratory CO2 was increased every 5 minutes, resulting in a stepwise increase in arterial pCO2 by 3 mm Hg. The recruitment threshold pCO2RT was then defined as the lowest pCO2, which resulted in a deformation of the inspiratory pressure curve by the patients own inspiratory efforts. pCO2RT was reproducible within trials and in different trials with a standard error of 1.2 mm Hg. It was found 6 +/- 4 mm Hg above the pCO2 during spontaneous breathing (p < 0.01) in all patients. pCO2RT decreased from 58 +/- 10 to 47 +/- 4 mm Hg during intermittent IPPV and so did the threshold during CO2 rebreathing, while spontaneous pCO2 decreased from 53 +/- 12 to 42 +/- 5 mm Hg. The slope, reflecting drive was decreased to 0.28 compared to normals but remained unchanged 0.32 (n. s.) during the study. Lung function did not change. A highly significant increase in the indices of maximal inspiratory force was observed (p < 0.002) and as a result a decrease in the inspiratory demand (p < 0.008). CONCLUSION: Intermittent IPPV does efficiently suppress phasic respiratory drive via thoracic afferent inhibition and therefore effectively unloads the ventilatory pump. The CO2 threshold is increased in patients with hypercapnic ventilatory failure, probably to minimise the load to the ventilatory muscles. With the increase in inspiratory capacity the pCO2 threshold can be restored to normal by intermittent noninvasive or invasive IPPV.

[Problems in adjustment to negative pressure ventilation]. / Probleme bei Einstellung auf Negativdruckbeatmung.

We tried to establish a nasal intermittent positive pressure ventilation for a 54-year old patient with post-polio kyphoscoliosis. Due to intractable rhinitis the patient stopped the treatment. A negative pressure ventilation via a cuirass exhibited an inverse ventilation: during the inspiratory cycle of the ventilator the diaphragm was elevated and the patient was forced to exhale, afterwards he needs to inhale by himself. The ventilatory support is now done via a combined nasal-mouth mask and intermittent positive pressure ventilation.

[Prospective study of the quality of life in intermittent self-ventilation]. / Eine prospektive Untersuchung der Lebensqualität unter intermittierender Selbstbeatmung.

PATIENTS AND METHOD: We assessed quality of life for 17 patients (age 14 to 74 years) before and during intermittent (nightly) nasal home mechanical ventilation with a standardized questionnaire (SF 36, Medical Outcomes Trust, Boston, USA). Underlying diseases were amyotrophic lateral sclerosis, bronchiectasis, kyphoscoliosis, pulmonary emphysema, muscular dystrophy and sequelae of tuberculosis. Blood gas and lung function data were collected during every examination. RESULTS: We observed statistically significant increases for items of general health, mental health, vitality and capillary oxygen partial pressure. CONCLUSION: The SF 36 allows to assess quality of life for patients under intermittent mechanical ventilation at home.

[Effects of intermittent self-ventilation on ventilatory drive and respiratory pump function]. / Effekte der intermittierenden Selbstbeatmung auf Atemantrieb und Atempumpfunktion.

BACKGROUND: A chronic hypercapnic ventilatory failure appears in patients with restrictive chest wall disorders, chronic obstructive pulmonary disease (COPD) or obstructive sleep apnea (OSA), but it can also appear in patients with a disorder of the central respiratory drive. PATIENTS AND METHOD: We studied the lung function, the respiratory muscle function and the PCO2 recruitment threshold (pCO2RT) during nasal intermittent positive pressure ventilation (IPPV) in 16 chronic hypercapnic patients (scoliosis = 8, COPD = 4, OSA = 4). RESULTS: The pCO2RT decreased from 61 +/- 6 mm Hg to 48 +/- 4 mm Hg (p < 0.0001) during intermittent IPPV, while spontaneous pCO2 decreased from 55 +/- 6 mm Hg to 42 +/- 5 mm Hg (p < 0.0001). The load of the respiratory pump decreased (P0.1/P0.1 max:0.27 +/- 0.18 before, 0.15 +/- 0.08 after intermittent IPPV; p < 0.04). CONCLUSION: We conclude, that the pCO2RT can be normalized by intermittent nasal IPPV as well as the pCO2 under spontaneous breathing. The load of the respiratory pump decreases due to an increase of the inspiratory muscle strength.