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Study Objectives: Ventilator users who are unable to leave the acute care setting may be transferred to a unit for chronic assisted ventilatory care (CAVC) with the goal of optimizing their level of function. In this report, we summarize the outcomes of all patients admitted to a CAVC unit between 1986 and 2001., Patients and Methods: Fifty patients (24 with neuromuscular disease [NMD], 10 with spinal cord injury [SCI], 7 with thoracic restriction [TR], 7 with COPD, and 2 with parenchymal restriction [PR]) were reviewed. Thirty-eight patients were transferred to the CAVC unit from intensive care, 5 patients were transferred from inpatient respiratory rehabilitation, 4 patients came from home, and 3 patients came from pediatric long-term care. At the time of CAVC unit admission, all patients were receiving mechanical ventilation via a tracheostomy tube., Results: Ventilator time increased gradually in patients with COPD from 16 h (SD, 5.6) to 22.9 h (SD, 3.0) per day (p < 0.05), and in patients with TR from 18.9 h (SD, 6.1) to 22.9 h (SD, 4.5) [not significant]. Five of the 10 patients with SCI were decannulated. Functional mobility, which decreased in patients with COPD and patients with TR, remained stable in NMD and PR and improved in SCI. Eighteen patients returned home or to an attendant care facility (COPD, n = 1; NMD, n = 10; SCI, n = 5; PR, n = 2); 11 patients died in the CAVC unit (COPD, n = 6; TR, n = 3; NMD, n = 1; SCI, n = 1); and 7 patients were transferred to intensive care, where they died. The average direct cost per patient per diem increased from $252 (Canadian) in 1988 to $335 in 2001., Conclusion: A CAVC unit can provide a safe environment for severely impaired, ventilator-dependent individuals, many of whom (36%) left for a more independent community-based environment. Better outcomes were seen among patients with SCI and NMD than in patients with COPD and TR.

Objective: To evaluate whether inspiratory muscle training (IMT) improves inspiratory muscle strength, respiratory capacity, fatigue, and subjective perception of physical endurance in patients with advanced multiple sclerosis (MS)., Design: Randomized controlled trial., Setting: Outpatient clinic in Sweden., Participants: Fifteen severely disabled patients with MS, randomized to a training or control group., Intervention: Seven patients trained with a Threshold inspiratory muscle trainer, twice every other day, with 3 sets of 10 loaded inspirations (40%-60% of patients' maximal inspiratory pressure [Pimax]) over a 10-week period., Main Outcome Measures: Spirometry, Pimax, maximal expiratory pressure (Pemax), clinical assessments, and questionnaires on the patients' fatigue severity and physical endurance were evaluated., Results: After training, the Pimax (P<.008) and Pemax (P<.02) increased in the training group. The improvement in Pimax after 10 weeks of training was higher than the improvement in the control group (P<.01) and was maintained 1 month after the training period ended. The training affected neither respiratory function nor the patients' symptoms., Conclusions: IMT had a beneficial effect on inspiratory muscle strength in patients with MS and is recommended as a complement to ordinary physical training.

Objective: To determine how often home mechanical ventilation (HMV) is instituted electively in children with respiratory failure from neuromuscular diseases and whether there were opportunities to discuss therapeutic options with patients/families before respiratory failure., Methods: Patients with neuromuscular disease (n = 73) requiring HMV (age, 2 months to 24 years) were studied. Whether HMV was initiated nonelectively because of acute respiratory failure or electively before acute respiratory failure, and opportunities for health care providers to discuss therapeutic options with patients/families before acute respiratory failure (hospitalization with pneumonia, clinic visits for preoperative evaluation, pulmonary function testing [PFT] and/or polysomnography [PSG]) were recorded., Results: HMV was initiated electively in 21% of patients with neuromuscular disease; 69% of the nonelective HMV group had HMV initiated after respiratory failure caused by pneumonia. In the nonelective group, opportunities for discussion of therapeutic options with the patients and families could have occurred before respiratory failure during 111 hospitalizations for pneumonia, 13 preoperative evaluations, 43 abnormal PFTs, and 24 abnormal PSGs., Conclusions: Most patients with neuromuscular disease had HMV initiated nonelectively after acute respiratory failure caused by pneumonia. Opportunities for discussing the therapeutic options with patients and families before respiratory failure were missed or ineffective.

Advances in critical care and mechanical ventilation have improved long-term survival of critically ill patients, some of whom develop the need for prolonged mechanical ventilator assistance. The rehabilitation of these individuals is aimed at restoring function, facilitating independence from mechanical ventilation, and in some cases returning them to the community. To accomplish these goals, rehabilitation programs require a multidisciplinary approach that includes physicians, respiratory therapists, nurses, physical and occupational therapists, nutrionists, speech therapists, and social services workers in a concerted effort. Chronic mechanical ventilation patients are often complicated by multiple comorbidities and by complex physiologic and psychological interactions. A careful selection and an individualized assessment are therefore paramount in identifying and achieving long-term goals. Special attention has to be paid to aggressive respiratory and nonrespiratory muscle rehabilitation, early ambulation, nutritional repletion, and psychological support. Careful evaluation and treatment by members of a multidisciplinary team may foster the patients' independence and ability to tolerate spontaneous ventilation, ultimately resulting in an improvement in their quality of life.

Purpose: The aim of our study was to assess the long-term effects of specific inspiratory muscle training (IMT) in patients with neuromuscular disorders (NMDs) who have various degrees of respiratory impairment., Patients and Methods: Twenty-seven patients with NMDs (Duchenne's muscular dystrophy, 18 patients; spinal muscular atrophy, 9 patients) underwent 24 months of IMT. Patients were divided into three groups according to their vital capacity (VC) values. VC was measured as the parameter for the respiratory system involvement of the disease. Maximal inspiratory pressure (PImax) was assessed as the parameter for respiratory muscle strength, and the results of the 12-s maximum voluntary ventilation test (12sMVV) were assessed as the parameter for respiratory muscle endurance. Pulmonary and inspiratory muscle function parameters were assessed 6 months before training, at the beginning of training, and then every 3 months., Results: The PImax values improved in group A (VC, 27 to 50% predicted) from 51.45 to 87.00 cm H(2)O, in group B (VC, 51 to 70% predicted) from 59.38 to 94.4 cm H(2)O, and in group C (VC, 71 to 96% predicted) from 71.25 to 99.00 cm H(2)O. The 12sMVV values improved in group A from 52.69 to 69.50 L/min, in group B from 53.18 to 62.40 L/min, and in group C from 59.48 to 70.5 L/min. For all three groups, there was a significant improvement of PImax (p < 0.007) and 12sMVV (p < 0.015) until the 10th month when a plateau phase was reached with no decline in the following month until the end of training., Conclusion: With IMT, respiratory muscle function can be improved in the long term of up to 2 years.

The term respiratory care has more than one meaning, referring both to a subject area within clinical medicine and to a distinct health care profession. In the light of several fundamental transformations of health care during the 20th century, this article reviews the history of respiratory care in both of these contexts and offers 10 predictions for the future: (1) Less focus on raising P(aO2) as a primary goal in managing patients with acute hypoxemic respiratory failure. (2) More attention to the adequacy of tissue oxygenation in such patients, irrespective of P(aO2), and the emergence of "permissive hypoxemia," analogous to permissive hypercapnia, in managing them. (3) Smarter monitors that display information less but process it more, while interacting directly with ventilators and other devices to modify therapeutic interventions. (4) Increased use of and expertise with noninvasive ventilation, with a corresponding decrease in intubations and complications, in treating patients with acute exacerbations of COPD. (5) Increased use of triage in the intensive care unit, including earlier determination of the appropriateness of maximal supportive intervention. (6) Greater use of protocols in patient assessment and management, in all clinical settings. (7) Increased awareness of, expertise in, and resources for palliative care, with a more active and acknowledged role for respiratory therapists. (8) Accelerating progress in smoking cessation and prevention, and also in early detection and intervention in COPD, led by the respiratory care profession. (9) An increasing presence and impact of respiratory therapists as coordinators and care givers in home care. (10) A continued and enlarging role for the journal Respiratory Care in disseminating research findings, clinical practice guidelines, protocols, and practical educational materials in all areas of the field.

In the absence of severe clinical signs requiring endotracheal intubation, non-invasive mechanical ventilation is the first-line treatment in patients with chronic respiratory insufficiency. For a patient with non-invasive support, the probability to avoid intubation is 2 or 3 times higher than for a patient initially treated by oxygen therapy alone. Oxygen therapy will remain the recommended approach for staff unfamiliar with non-invasive support. In chronic obstructive pulmonary disease (COPD) ventilated patients, keeping the intrinsic positive end-expiratory pressure to a minimum level decreases the risk of barotrauma. During exacerbation, in these patients, the 3 most often prescribed medications are inhaled bonchodilators, corticosteroids, and antibiotics. In addition to long term home oxygen therapy, the preventive measures include education of patients, pulmonary rehabilitation programs, and eventually routine use of ventilatory support in a home ventilatory setting.

In France, 65,000 patients with chronic respiratory failure are managed at home. This is made possible by the collaboration between prescribing physicians and other medical and social agents in the community. It consists in various medical, technical and social interventions, all aimed at improving both survival and quality of life. Patient and family education is a key-factor to increase the success of the various therapeutic interventions that are rehabilitation, long term oxygen therapy and home mechanical ventilation. Rehabilitation programmes include optimisation of pharmacological treatments, help in smoking cessation, social and psychological support, and exercise training. National alert procedure for medical devices, and evaluation of medical devices is part of the management of respiratory failure at home. Care networks facilitate the various interventions necessary to maintain the patient with chronic respiratory failure at home.

Objective: Comparing the effects of proportional assist ventilation (PAV), pressure support ventilation (PSV), and intermittent positive pressure ventilation (IPPV) on cardiopulmonary function in patients with acute respiratory failure, in particular, evaluating the clinical significance of PAV., Methods: Ten patients with acute respiratory failure were firstly ventilated with IPPV. Elaslance (Ers) and Resistance (Rrs) were measured and calculated. Then PSV mode was adapted. Based on the parameters of IPPV, inspiratory positive airway pressure was adjusted to maintain the same tidal volume (V(T)) as that in IPPV. Finally PAV mode was used. According to the parameters of PSV, the assist ratio was adjusted to maintain the same V(T) and peak pressure peak, as those in PSV. Respiratory mechanics, arterial blood gas and hemodynamics were recorded during above three modes of ventilation., Result: Comparing with PSV and IPPV mode, PAV created a significantly lower peak, less work of breathing of patients (WoBp) and work of breathing of ventilation (WoBv) under the similar V(T); Central venous pressure (CVP) and pulmonary capillary wedge pressure (PCWP) were significantly lower in PAV mode as compared with those in IPPV; While comparing with PSV, V(T), mean blood pressure (mBP) and cardiac output (CO) were higher and mean pulmonary arterial pressure (mPAP), WoBp were lower in patients with PAV under similar peak. Among them the fall of WoBp was statistically significant., Conclusion: In patients receiving three modes of ventilation, PAV presents with lower airway pressure, less WoBp and less effect on hemodynamics as compared with those with PSV or IPPV.

Background: Pulmonary oxygenation is generally temporarily impaired following cardiac surgery. We studied the factors influencing postoperative oxygenation using multivariate analysis., Methods: This study was retrospectively performed in University hospital. Fifty patients undergoing solitary coronary artery bypass grafting were included in this study. Patients were divided into two groups by mean age; older group (n=25) and younger group (n=25). Alveolar-arterial oxygen difference (A-aDO2) and respiratory index (RI) were measured at 3 hours after surgery and on postoperative day 1. Statistical analysis was performed using forward selection stepwise regression of 12 perioperative variables., Results: In all patients, A-aDO2 and RI significantly (p<0.01) increased after surgery and remained high on POD1. In stepwise regression analysis, preoperative A-aDO2 and RI were significant factors in the equations for postoperative A-aDO2 and RI in general, which was the most important factor in the older group. In the older group, preoperative A-aDO2 or RI and water balance were significant (p<0.05) factors which were responsible for half the oxygenation impairment just after surgery, and PCWP showed a significant (p<0.01) negative correlation with both A-aDO2 and RI on POD1. In the younger group, PCWP was a significant (p<0.01) factor for A-aDO2 and CVP and CI were significant for RI on POD1., Conclusions: Postoperative oxygenation was mainly influenced by the preoperative respiratory condition, especially in old patients, indicating that preoperative management by a physiotherapist may be necessary. Pleurotomy also had a negative influence. Careful intraoperative and postoperative volume control is important in old patients.

RF is a unique situation. It is specific in diagnosis and yet, at the same time, broad in concept. It can be acutely life threatening or chronic in presentation and need for intervention. Patients are cared for in ICUs, medical-surgical floors, and nursing homes as well as at home. When RF is a complicating condition of an already hospitalized patient, the road to recovery may be long and complicated. It is important that the health care team provides education and counseling so that the patient and family can cope with the changing events and the potentially long road to recovery. Outcome data from all the sites of care are currently lacking for a comparative analysis of the most effective site. All the sites of care discussed in this article are cost-effective alternatives to the ICU, but there is a lack of standards and evidence of measurable outcomes such as the quality and cost relationship. Outcome data are needed to document the cost of care and the relationship of that cost to specific outcomes such as final discharge disposition, survival, complications, and quality of life. Health care provides have a much clearer image of the care that can be provided in the multiple sites available. Unfortunately, patients and families do not have that same level of understanding. For many patients and families, regardless of what the facility is called, it is not the acute hospital, and that is where they wish to remain until discharge home. Clarification and standardization are needed regarding the terminology used to describe the various sites of care. It is important that integrated health care institutions provide education and counseling to patients and families regarding the continuum of care and the many alternatives along that path.

Introduction: In spite of the great variability in clinical signs in the mitochondrial disorders, reports of respiratory disorders in these conditions are purely anecdotal, and almost all occurred in patients with clinical data of the illness. We present a case in which respiratory insufficiency was the first sign of a mitochondrial disorder., Case Report: A 74 year old patient, with no significant clinical history, had two episodes of severe acute respiratory insufficiency without cardiopulmonary causes to justify it. The patient was investigated for neuromuscular disorders and had a muscle biopsy compatible with mitochondrial myopathy., Conclusion: The occurrence of respiratory failure in patients with mitochondrial disorders is exceptional, is not related to the degree of deterioration of the respiratory function tests and is potentially reversible.

Pulmonary rehabilitation is one of the most important components of comprehensive care for patients with significant disability due to chronic respiratory failure. Because pulmonary rehabilitation has not been popular in Japan, the long-term effectiveness of pulmonary rehabilitation has rarely been reported. We therefore examined the long-term effectiveness of an outpatient pulmonary rehabilitation program for patients with chronic respiratory failure. Our program was composed of a once-a-week introduction program for 2 months and a support program that was continued every 4 weeks as long as possible. Thirty stable patients with chronic respiratory failure were enrolled in the program; 21 patients (COPD: 15, lung complications of tuberculosis: 6) completed the 9-week introduction program and the ensuing 6-month support program. Good compliance with the home training regimen was maintained during the period. The introduction program significantly alleviated dyspnea (Fletcher's grade: 3.3 to 3.0, p < 0.01) and improved the data for activity (Spector's score: 5.3 to 5.8, p < 0.01) and 6-minute walking distance (319 to 384 m, p < 0.01). These benefits were sustained during the 6-month support program. We concluded that outpatient pulmonary rehabilitation can alleviate dyspnea and improve the activity and exercise tolerance of patients with chronic respiratory failure, and that the effectiveness of training can be well maintained with a minimal support program.

Noninvasive mechanical ventilation is provided without establishing an endotracheal airway. In acute respiratory failure due to chronic obstructive lung disease, noninvasive positive-pressure ventilation delivered through a nasal or face mask may eliminate the need for intubation and related complications, reduce the duration of hospitalisation and improve survival compared to assisted ventilation through an endotracheal tube. In chronic respiratory failure related to neuromuscular diseases and thoracic deformities, the advent of noninvasive intermittent longterm ventilation is well established. However, it is not clear, whether addition of noninvasive ventilation to standard treatment with medication and longterm oxygen improves symptoms and survival in chronic respiratory failure due to chronic obstructive lung disease. During introduction of noninvasive ventilation, patients must be monitored closely. Patient selection and information, meticulous optimization of the ventilator settings and delivery system, education and experience of the personnel have an important impact on the success of this novel way of providing mechanical ventilatory support.

Respiratory muscle weakness, predominantly of the expiratory muscles, is characteristic of individuals with advanced multiple sclerosis and can result in difficulty in clearing secretions and repeated episodes of pneumonia. This pilot study evaluated the effectiveness of music therapy in strengthening respiratory muscles through an emphasis on diaphragmatic breathing and coordination of breath and speech. Twenty patients were randomly assigned to one of two groups: one that received music therapy or one that attended music appreciation sessions. Participants' inspiratory and expiratory muscle strength was measured by testing mouth pressure before and after the intervention. The experimental group showed some improvement in terms of expiratory muscle strength, in contrast to the control group, which showed deterioration. The results were not statistically significant, however. Patients in both groups exhibited considerable weakness in their expiratory muscles, and results for 79% of the participants were below 30% of the predicted values. Variability, a major confounding factor that resulted in reduced statistical power, led the investigators to suggest an intercenter collaboration to amass sufficient numbers of patients for a future study. Early manifestation of respiratory muscle weakness warrants inclusion of respiratory muscle testing in examination protocols and early intervention efforts.

Intermittent prone positioning (PP) is a promising therapy of patients with severe respiratory failure. Evaluations of patient outcomes can not, to dare, be found in the literature. This study was conducted to investigate the effects of intermittent PP on patients with posttraumatic respiratory failure (PaO2/FiO2 < 280 mmHg) in comparison with conventional therapy in suspine position. The collected data is part of our prospective polytrauma study. 136 polytraumized patients (mean ISS 23.4) were included and evaluated. 77 patients had a severe chest trauma with an AIS > or = 3. Of these, 47 patients developed a respiratory failure. 19 of these patients were treated conventionally in suspine position, 28 patients were intermittently turned prone. Having similar AIS (3.6 vs. 33), the PP-patients had a significantly severer trauma (ISS 35.8 vs. 24.5). Though the injury severity of the PP-patients was much higher, the time of ventilation (32 vs. 31 d) and the ICU stay (39 vs. 36 d) was similar to the patients treated in suspine position. The mortility of PP-patients was 0%, of suspine positioned patients 26%. After the first PP the PaO2/FiO2 ratio increased with an average of 82 mmHg (26-151 mmHg). The FiO2 was reduced from 0.45 (0.35-1.0) to 0.26 (0.21-0.35). Beside the beneficial effect of PP on the oxygenation we have, for the first time evidence that PP improves the outcome of patients with posttraumatic respiratory failure.

Respiratory failure plays a major role in morbidity and mortality after high cervical spine injury. Initially the patients are threatened by acute loss of motor function of the respiratory muscles and the development of neurogenic pulmonary edema. In the further course alterations in the mechanical properties of the respiratory system, changes in lung function, pulmonary infections and airway problems are the main reasons for life threatening respiratory failure. Early surgical stabilization, careful weaning from mechanical ventilation, meticulous search and treatment of infections, aggressive posturing and mobilization and support of impaired mechanical functions by physiotherapy, muscle training, noninvasive respiratory support and in some cases electrophrenic pacing increase survival and independency of quadriplegic patients.

Objective: Pulmonary rehabilitation has been shown to be of benefit to clinically stable patients with chronic obstructive pulmonary disease (COPD). This study examined the effect of pulmonary rehabilitation on some physiologic variables in COPD patients recovering from an episode of acute respiratory failure., Design: A prospective, randomized study., Setting: A respiratory intensive care unit (RICU)., Patients: Eighty COPD patients recovering from an episode of acute respiratory failure were randomized in a 3:1 fashion to receive stepwise pulmonary rehabilitation (group A, n=60 patients) or standard medical therapy (group B, n=20 patients)., Main Outcome Measures: Improvements in exercise tolerance, sense of breathlessness, respiratory muscle function, and pulmonary function test values were measured, respectively, by exercise capacity (6-minute walking distance [6MWD]), dyspnea score (Visual Analog Scale [VAS]), maximal inspiratory pressure (MIP), forced expiratory volume in 1 second (FEV1), and forced vital capacity (FVC)., Interventions: Group A received pulmonary rehabilitation that consisted of passive mobilization (step I), early deambulation (step II), respiratory and lower skeletal muscle training (step III), and if the patients were able, complete lower extremity training on a treadmill (step IV). Group B received standard medical therapy plus a basic deambulation program., Results: Sixty-one of 80 patients were mechanically ventilated at admission to the unit and most of them were bedridden. Twelve of the 60 group A patients and 4 of the 20 group B patients died during their RICU stay, and 9 patients required invasive mechanical ventilation at home after their discharge. The total length of RICU stay was 38+/-14 days for patients in group A versus 33.2+/-11 days for those in group B. Most patients from both groups regained the ability to walk, either unaided or aided. At discharge, 6 MWD results were significantly improved (p < .001) in Group A only. MIP improved in Group A only (p < .05), while VAS scores improved in both groups, but the improvement was more marked in group A (p < .001) than in group B (p < .05)., Conclusions: COPD patients who were admitted to a RICU in critical condition after an episode of acute respiratory failure and who, in most cases, required mechanical ventilation benefited from comprehensive early pulmonary rehabilitation, compared with patients who received standard medical therapy and progressive ambulation.

Objective: Protein-energy malnutrition (PEM) constitutes an important complication in children submitted to intensive care treatment for respiratory failure (RF). This complication is not usually evaluated in assessment protocols. Nutrition assessment in a Pediatric Intensive Care Unit (PICU) was performed in children with respiratory failure., Patients and Methods: A prospective study involving 65 children (1-158 months of age) with RF at admission to the PICU was carried out. The patients studied were divided into four groups according to their etiology: Group I (n = 29) included those with acute bronchial pathology; Group II (n = 17) those with pneumonia: Group III (n = 11) those in the post-operative period following cardiac surgery; and Group IV (n = 8) those patients with multiple-organ dysfunction. All patients in Groups I, II, and III had chronic diseases. Anthropometrical and nutritional evaluation included weight, height and body mass index (w/h2)., Results: The weight for age was low (59%). Height deficit was observed in a significantly lower percentage of the patients (13.1%). The body mass index showed values in relationship to the 50th percentile that varied between 83.4 +/- 23.1% and 97.1 +/- 4.7%. In 68.9% of the patients the criteria of global malnutrition were met according to the classification of McLaren and Read., Conclusions: The data confirm that PEM is frequent in patients with RF in the PICU and emphasize the usefulness of anthropometrical evaluation as an important, simple and non-invasive method of nutritional evaluation. Early and intensive nutritional intervention can reduce or prevent the vicious circle of malnutrition in RF patients hospitalized in the PICU.

Since 1981 long-term oxygen therapy (LTOT) has become an important procedure for the rehabilitation of patients with chronic respiratory insufficiency in Switzerland too. As a result of long-term clinical experience as well as technical progress, there is, however, increasing need for an updated reassessment of guidelines and standardized management respectively: the indications for LTOT-so far restricted to patients with COPD-are extended to patients with pulmonary diseases other than COPD associated with chronic hypoxemia (pO2 < 7.3 kPa) provided there is enough evidence of clinical benefit. Another important aspect of LTOT focuses on the appropriate application of different oxygen sources. Oxygen concentrators are still accepted to be the most economic domiciliary source. For short-term outdoor activities, small portable cylinders in combination with an oxygen conserving system should be used. However, in regular mobile oxygen therapy liquid oxygen is the best choice, generally consisting of a domiciliary reservoir and portable canisters. Delivery of oxygen through a transtracheal catheter is superior to nasal cannula because of lower oxygen consumption, decreased breathing work and improved compliance respectively. To organize and follow-up LTOT in Switzerland, a very efficient concept was introduced many years ago which is characterized by economical cooperation between physicians, insurances, technical suppliers and social providers. Today more than 3000 patients-corresponding to 38/100,000 inhabitants-benefit from LTOT. Future perspectives should target an earlier start to continuous as well as mobile LTOT, in order to improve rehabilitation in still active conditions instead of merely palliating the terminal stages.

This study investigated the impact of deep diaphragmatic breathing (DB) on blood gases, breathing pattern, pulmonary mechanics and dyspnoea in severe hypercapnic chronic obstructive pulmonary disease (COPD) patients recovering from an acute exacerbation. Transcutaneous partial pressure of carbon dioxide (Ptc,CO2) and oxygen (Ptc,O2) and arterial oxygen saturation (Sa,O2), were continuously monitored in 25 COPD patients with chronic hypercapnia, during natural breathing and DB. In eight of these patients, breathing pattern and minute ventilation (V'E) were also assessed by means of a respiratory inductance plethysmography. In five tracheostomized patients, breathing pattern and mechanics were assessed by means of a pneumotachograph/pressure transducer connected to an oesophageal balloon. Subjective rating of dyspnoea was performed by means of a visual analogue scale. In comparison to natural breathing deep DB was associated with a significant increase in Ptc,O2 and a significant decrease in Ptc,CO2, with a significant increase in tidal volume and a significant reduction in respiratory rate resulting in increased V'E. During DB, dyspnoea worsened significantly and inspiratory muscle effort increased, as demonstrated by an increase in oesophageal pressure swings, pressure-time product and work of breathing. We conclude that in severe chronic obstructive pulmonary disease patients with chronic hypercapnia, deep diaphragmatic breathing is associated with improvement of blood gases at the expense of a greater inspiratory muscle loading.

This study was conducted to investigate the effects of intermittent prone positioning of patients with post-traumatic respiratory failure (paO2/FiO2 < 280 mm Hg) in comparison with conventional therapy in a supine position. Although the severity of injury of the prone-positioned patients was much higher (ISS 35.8 vs 24.5), the ventilation time (32 vs 31 days) and ICU stay (39 vs 36 days) were similar to patients treated in the supine position. Besides the beneficial effect of prone positioning on oxygenation, for the first time we have evidence that prone positioning improves the outcome of patients with post-traumatic respiratory failure.

Air leaking through the mouth has been reported in kyphoscoliotic patients receiving nasal ventilation via volume-limited ventilators. This study accessed the frequency of occurrence and effect on sleep quality of air leaking through the mouth during nocturnal nasal ventilation in patients with chest wall and neuromuscular disease using pressure-limited ventilation. Overnight and daytime polysomnography was performed in six stable experienced users of nocturnal nasal noninvasive positive-pressure ventilation (NPPV) who had chronic respiratory failure due to neuromuscular disease or chest wall deformity. All patients used the BiPAP S/T-D ventilatory support system (Respironics, Inc., Murrysville, PA). Measures included sleep scoring, leak quantitation, diaphragm and submental electromyograms (EMGs), and tidal and leak volumes. All patients had air leaking through the mouth for the majority of sleep. Sleep quality was diminished because of poor sleep efficiency and reduced percentages of slow-wave and rapid eye movement (REM) sleep. Air leaking through the mouth was associated with frequent arousals during stages 1 and 2 and REM sleep that contributed to sleep fragmentation, but arousals were infrequent during slow-wave sleep. Despite prevalent leaking, oxygenation was well maintained in all but one patient. Patients used a-combination of passive and active mechanisms to control air leaking. Although nasal ventilation improves nocturnal hypoventilation and symptoms in patients with restrictive thoracic disorders, air leaking through the mouth is very common during use. The leaking is associated with frequent arousals during lighter stages of sleep that interfere with progression to deeper stages, compromising sleep quality. Portable pressure-limited ventilators compensate for leaks, maintaining ventilation and oxygenation, but further studies are needed to determine which interfaces and ventilator techniques best control air leaking and optimize sleep quality.

The purpose of the lung is intrapulmonary gas exchange. The circulatory system delivers the respiratory gases to the tissue. The ventilatory pump however is responsible for the circulation of air between the lungs and the ambient atmosphere. Due to better diffusing capabilities, hypercapnia always is a result of pump failure and little dependent on the lung. Ventilatory failure, either compensated with an increased demand on the muscles or decompensated with an additional increase in pCO2, should be separated from lung failure where primarily oxygen exchange is involved. Decompensated hypercapnic ventilatory failure is then the indication for intermittent mechanical ventilation. The pCO2, either arterial or transcutaneously registered together with the noninvasive evaluation of the mouth occlusion pressures during tidal breathing and during a maximal inspiratory effort, define well the severity of ventilatory failure. In acute on chronic ventilatory failure, noninvasive mechanical ventilation in three randomised and controlled studies resulted in a better survival compared to intubation. To fulfil certain weaning criteria is no longer required in difficult to wean patients, as a transfer from invasive to noninvasive mechanical ventilation can be performed if only cooperativity is preserved together with a minimal capacity of spontaneous breathing. Weaning will thereafter occur by progressive relief from intermittent noninvasive ventilation. 2300 difficult to wean patients in Germany should profit from this approach. Chronic ventilatory failure as a result of neuromuscular disease or scoliosis of the thoracic spine are the classical indications. COPD and myasthenia gravis are under discussion as indications for intermittent mechanical ventilation with an increasing tendency to ventilate. Epidemiological data however can only be roughly estimated due to the heterogeneity of indication and selection of the patients.

Background: Nocturnal mechanical ventilation (NMV) can reduce symptoms of hypoventilation and improve gas exchange in patients with chronic respiratory failure but there is no data concerning the impact of nocturnal mechanical ventilation on daily activity. We prospectively measured daily activity as judged by total steps per day., Patients and Methods: Thirty stable patients (17 men, mean age 55.2 years, pCO2: 62.3 +/- 9.0 mmHg) with chronic respiratory failure., Diagnosis: Kyphoscoliosis (n = 11), COLD (n = 7), neuromuscular diseases (n = 6), post-tbc sequelae (n = 6). Applying a pedometer over a 7 day period before and 3 months after initiating nocturnal mechanical ventilation the steps per day were counted. We also obtained arterial blood gases and applied the St. George's Respiratory Questionnaire (scoring range from 0 = complete health to 100 = worst possible)., Results and Conclusions: The daily activity in patients with hypercapnic respiratory failure as judged by the total amount of steps per day increased by 120% after 3 months of nocturnal mechanical ventilation (from 1606.9 +/- 1341.3 to 3535 +/- 1813.8 steps per day, p < 0.0001). This was associated with a significant improvement in daytime blood gases and quality of life (QoL) as judged by questionnaire (total score: from 61.3 +/- 14.9 to 48.3 +/- 18.7, p = 0.0006). However no correlation between steps per day and QoL was found.

Background: The purpose of this study is to assess quality of life in patients with chronic respiratory failure who require home mechanical ventilation (HMV)., Patients and Methods: Patients with COPD (n = 20), scoliosis (n = 20), neuromuscular diseases (n = 20) and others (n = 7) were examined. A specific questionnaire containing forms for the patient, the physician and for the relatives was developed according to the formulation of the question (Fragebogen zur chronischen Heimbeatmung = FCH, Interview zur chronischen Heimbeatmung = ICH). In addition to that the Hospital Anxiety and Depression Scale (HAD) and the Asthma Quality of Life Questionnaire were used. Arterial blood gas tensions, pulmonary function and inspiratory mouth occlusion pressure were studied., Results: Quality of life in patients with scoliosis and neuromuscular diseases is improved during HMV compared to patients with COPD who especially are impaired with regard to psychological and functional conditions. Correlations with physiological parameters and with the compliance could not be observed., Conclusion: Despite progression of the disease HMV enhances quality of life in patients with scoliosis and neuromuscular diseases. The outcome in COPD is less evident. Therefore the indication must be considered more critically.

During recent years home ventilation has been shown to be useful for a growing number of patients with neuromuscular diseases and respiratory insufficiency caused by weakness of the respiratory muscles. Despite its technical simplicity, home ventilation leads to a number of social, ethical, medical and infrastructural problems. This consensus paper discusses the special situation of patients with neuromuscular diseases in home ventilation and describes the current thinking in the following topics: (1) definition of home ventilation; (2) aims of home ventilation; (3) indications and preconditions of home ventilation; (4) techniques and forms of home ventilatory support; (5) preparation for and practical questions of home ventilation; (6) stopping home ventilation.

Respiratory rehabilitation is defined as a medical practice including a multidisciplinary medical program fitting each individual. Personalized retraining by means of exercises, is the master part of it, its aim is to improve the physical fitness in specialised institution then to maintain it when he becomes an out patient. In both cases, this retraining complies with strict rules concerning the mode of exercises (imposed power--duration of sessions--weekly frequency--progressiveness of overloading ...). This codification rests mainly on the recommendations of the American College of Sports Medicine. The choice of intensity at the beginning of the stay will be determined either by the maximal reserve of cardiac frequency or by the ventilatory threshold. This training has to involve extensive muscular mass and must not neglect the upper limbs. Ventilatory physiotherapy also plays an important part. The other components of rehabilitation concern optimisation of bronchodilator treatment, cessation of smoking, health education, physical education and relaxation, appraisal of nutritional status, assessment of therapeutic programs, of the quality of life and a long-term program for reinforcement of acquisitions. The therapeutic programs improve ventilatory performance, maximal oxygen intake, maximal tolerated power and quality of life. An adaptation of the St. George's Respiratory Questionnaire to patients hosted at the TOKI EDER Medical Center points out that the quality of life of patients with chronic respiratory failure is improved very highly significantly by this rehabilitation.

Introduction: Patients suffering from the most frequent muscle disorders Duchenne muscular dystrophy (DMD) and spinal muscular atrophies (SMA), who ceased walking respectively are confined from the outset to the wheel-chair, are developing commonly a progressive scoliosis (collapsing spine) due to an increasing muscle weakness. Basing on the pelvic obliquity these scolioses are leading first of all to problems in sitting as well as difficulties in trunk and head control. Along with the increasing weakness of respiratory muscles these phenomena entail a restrictive respiratory insufficiency., Conservative Treatment: An effective conservative treatment is not available for these scolioses. The use of a corset, however, can only be taken into consideration as a compromise, either for very young patients or those who refused an operation respectively who have reached an inoperable stage. The exclusive use of so-called "anatomic sitting supports" in the wheel-chair in order to treat or prevent a progressive scoliosis in DMD or SMA is absolutely to be rejected. They should only be applied for very young patients with SMA type II as a transitional solution until a corset or better an surgical stabilisation of the spine will be effected, or as a palliative measure in late stages., Surgical Treatment: Only the early as possible performed surgical stabilisation of the spine using adequate instrumentation (Luque, CD or modifications), enabling an early mobilization without corset or cast, is the most effective treatment of these scoliosis. Patients with DMD or SMA type III should be stabilized after loss of walking ability and definitive confinement to wheel-chair, if the curve is more than 20 degrees-30 degrees Cobb and progressive and forced vital capacity (FVC) is > 35%. The instrumentation should be applied between D3 or D4 and sacrum. The bony fusion mass should include the lumbar and lumbosacral region. The unfused instrumentation with the telescope-rod after Naumann is a good solution for patients with SMA type II and progressive curves already in the early childhood from ca. 5 years of age. First of all surgical spinal stabilisation improves the sitting comfort. Over and above this the improved cosmetic appearance should not be underestimated for the psychological condition of these patients. Additionally it is proved, that surgical stabilisation of the spine prolongs the life expectancy of patients with DMD. Furthermore stabilization of lung function can be achieved for both DMD and SMA patients in comparison to the natural history of these diseases. Altogether a decisive improvement of quality of life can be reached for all these patients.

100 patients with chronic obstructive bronchitis were examined and treated in the therapeutic department of rehabilitation center. Combined treatment including low-energy laser radiation produced good results. Laser therapy has improved bronchial permeability, sensitivity of bronchial beta 2-receptors to sympathomimetics. As a result, their intake was reduced or discontinued. Laser radiation combined well with other non-pharmacological modalities.

To provide evidence for the potentialities of normobaric hypoxic training (NHT), oxygen cascades from the lung to tissues and responses to hypoxic and hypercapnic stimuli were analyzed in chronic bronchitis patients with Stages 1, 2, and respiratory failure. Since at Stages 2 and 3, compensatory responses are substantially decreased, there is arterial, and occasionally, venous hypoxemia, NHT is contraindicated in these patients. Compensatory responses are preserved only during early stages 0 and 1 respiratory failure, showing it expedient to employ NHT. NHT regimens (as many as 20 sessions) used in these patients improved the patients' condition and physical activity, normalized ventilation function. However, the concurrent diseases exacerbated in some cases. Medical monitoring is needed when the NHT regimen is used.

This report describes the design and use of a wheeled walker that can accommodate a ventilator and oxygen tanks. It is constructed of aluminum tubing. The front of the walker has receptacles to support a Mark 7 Bird Ventilator and oxygen tanks. The back end of the walker has a bench seat that is lifted to allow entry into the walker. The seat provides rigidity and stability to the frame of the walker. The walker has been used in the Medical Intensive Care Unit of the Albany Medical Center to facilitate early ambulation of patients who are ventilator dependent or who require a portable source of oxygen to begin ambulation training.

Background: In patients with hypercapnic respiratory failure due to restrictive lung diseases home mechanical ventilation leads to improvement of daytime blood gases and symptoms. In this paper we regard the possible pitfalls and complications associated with intermittent mechanical ventilation in patients with kyphoscoliosis., Patients and Methods: From 1990 to 1994 we treated 30 patients suffering from severe kyphoscoliosis with home mechanical ventilation., Results and Conclusions: 1. As our cases show in the prehospital phase before initiating home mechanical ventilation there is still room for the outpatient pneumologists to improve their knowledge and awareness of symptoms and treatment of chronic respiratory failure. 2. In the subsequent inpatient phase and specialised center must be aware and experienced in regarding possible problems inherent with home mechanical ventilation. 3. After discharge during chronic home mechanical ventilation a good cooperation between the center, patient, relatives, general services and general pulmolgists is necessary. The centers should offer training courses for the caring persons and general pneumologists.

Background: Proportional assist ventilation (PAV), a new mode for assist ventilation, allows the patient not only to trigger the ventilator but enables him to keep his breathing pattern. The basis of PAV is a positive feed back between patient and ventilator., Patients and Method: PAV was applied in 6 patients, who were under long-term ventilation and who were stable. The ventilator was adjusted to compensate for the endotracheal tube, different parts between 90 and 40% of resistance and elastance were assisted. Airway pressure, flow and tidal volume were measured. PAV was compared with pressure support ventilation (PSV)., Result: No difference in blood gases were found in PAV as compared to PSV. Airway pressure were lower in PAV than in PSV (10.4 +/- 3.3 cm H2O vs. 18.8 +/- 5.9 cm H2O in PSV). Breathing frequency was higher (22.4 +/- 8.1 vs. 15.8 +/- 5.9 in PSV)., Conclusion: The higher breathing frequencies in PAV were associated with the underlying diseases and resulted in a better synchronisation with the respiratory center output.

Introduction: Noninvasive intermittent ventilation is usually performed in patients with severe ventilatory pump disorder. From 1988 to 3/1995 we treated 163 patients with the aim of home mechanical ventilation (HMV)., Patients and Results: In March 1993 115 of these 163 patients practiced HMV, 22 had already died and 26 had rejected or broken off ventilation therapy. The 115 patients were classified in three main diagnostic groups: Scoliosis or chest wall disease (n = 76), COPD (n = 11) and neuromuscular disease (n = 28). The mean pCO2 at rest of all patients before ventilation therapy was 56 (+/- 12) Torr and fell to 46 (+/- 5) Torr in the course of therapy. The maximum statical inspiration pressure PImax rose from average 3, 8 (+/- 2, 3) to 4, 9 (+/- 2, 0) kPa. There was a probability of surviving two years after onset of ventilation therapy of 85% in the scoliosis group, of 60% in the neuromuscular group and of 30% in the COPD group., Conclusions: According to results of others home ventilation therapy was very successful in patients with chest wall disease. In some patients with neuromuscular disorder quality of life could be improved and life prolonged. Only half of the COPD patients could be treated successfully, whereas the other half had no benefit from noninvasive ventilation therapy.

Background: There are only small experiences with mechanical ventilation via nasal mask in childhood., Patients and Methods: Eleven patients using NIPPV (9 patients aged 4 to 18 years and 2 patients with cystic fibrosis aged 20 and 25 years)., Results: NIPPV was effective in all 11 patients. Seven patients needed supplemental oxygen. Theophyllin, Almitrin and Salbutamol could support the nasal ventilation in special conditions., Conclusion: Intermittent ventilation via nasal mask is a noninvasive and effective treatment of chronic respiratory failure in childhood. Monitoring with continuous pulse-oximetry is necessary.

To evaluate the effects of pulmonary rehabilitation in patients with chronic respiratory failure, 43 patients with stable chronic respiratory failure were enrolled in a pulmonary rehabilitation program. The program included education, instruction in diaphragmatic breathing, respiratory muscle training, and exercise training (walking and riding a stationary bicycle). Patients were divided into 2 groups by the lowest SpO2 during a 12-minute-walk test done before rehabilitation: lowest SpO2 < 90% (Group A, 32 patients) and lowest SpO2 > or = 90% (Group B, 11 patients). In group A, SpO2 during exercise training was maintained over 90% by O2 inhalation, by load reduction, or by maintaining a slower walking speed. During 2 months of rehabilitation, the distance walked in 12 minutes and the maximum distance walked with an SpO2 > or = 90% significantly increased in both groups, but PaO2, VC, and FEV1 increased only in group A. In group A, the patients with chronic obstructive pulmonary disease had significant increases in PaO2, VC, and FEV1, but those with old tuberculosis sequelae had significant increases in PaO2 only. Pulmonary rehabilitation in patients with chronic respiratory failure may not only increase exercise tolerance but may also improve arterial oxygenation and pulmonary function.