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For neuromuscular patients with progressive respiratory muscle weakness a new training apparatus was developed, which allows a home training of strength as well as endurance of the inspiratory muscles, especially the diaphragma. A significant positive training result could be proved in a comparative study between 2 groups of 15 Duchenne muscular dystrophy (DMD) patients each (8). By the end of the training the satisfaction of patients with the new training equipment was evaluated by means of a questionnaire. The degree of satisfaction was determined at a 10-point scale. Critical ideas were used for improvement of the newly developed training apparatus.

The Aims of Our Study Were: 1) to evaluate the long-term efficacy of nocturnal IPPV either via tracheostomy (tIPPV) or a nasal mask (nIPPV) as a means to improve alveolar ventilation in patients with chronic severe hypercapnia caused by kyphoscoliosis; and 2) to assess the effect of MV on hospitalizations and life-style. Twenty six patients with kyphoscoliosis in chronic respiratory failure were enrolled in the study. Patients were divided into two groups. The first group comprised 13 subjects who had been clinically stable for at least 1 month (arterial carbon dioxide tension (Pa,CO2) 81 +/- 1.5 kPa (60.8 +/- 10.9 mmHg), arterial oxygen tension (Pa,O2) 7.3 +/- 0.8 kPa (54.6 +/- 6.1 mmHg)). The second group comprised 13 patients who were either suffering or recovering from an episode of acute respiratory insufficiency (Pa,CO2 9.0 +/- 1.8 kPa (67.8 +/- 13.3 mmHg), Pa,O2 6.8 +/- 1.1 kPa (51.2 +/- 8.2 mmHg), breathing supplemental oxygen in seven cases). Patients in the first group were treated with nocturnal IPPV via a nasal mask, whilst those in the second received nocturnal IPPV via tracheostomy. Similar improvements in arterial blood gases (ABGs) were achieved with both methods. Despite the differences in the degree of severity at baseline, after 1 month, ABG values were: Pa,CO2 6.2 +/- 0.6 kPa (46.6 +/- 4.4 mmHg), Pa,O2 9.0 +/- 1.3 kPa (67.5 +/- 9.6 mmHg) (nIPPV patients); Pa,CO2 6.1 +/- 0.9 kPa (46.1 +/- 6.8 mmHg), Pa,O2 9.8 +/- 1.3 kPa (73.6 +/- 9.8 mmHg) (tIPPV patients). After 1 yr, this improvement was still evident. Days of hospitalization were significantly reduced in both groups during the first year of MV. We conclude that both tIPPV and nIPPV are effective in the long-term treatment of respiratory failure in patients with kyphoscoliosis. It would appear from our data that if nIPPV is initiated early in the evolution of chronic respiratory failure in patients with kyphoscoliosis it will delay the necessity to use an invasive technique; however, long-term follow-up studies and larger case series are needed to demonstrate this.

Objective: To provide baseline information on the state of pediatric pulmonary rehabilitation, including data regarding diagnosis, age, gender, race, gestational age, disposition, medications, complications, procedures, consultations, pulmonary status, and outcomes., Design: Retrospective review., Setting: Pediatric pulmonary rehabilitation unit of a pediatric rehabilitation hospital that is free-standing, but that receives most of its referrals from a tertiary care pediatric hospital., Patients: All patients (70 subjects) who had completed a course of inpatient rehabilitation over the 5-yr life of the hospital., Main Outcome Measures: Descriptive data were collected in regard to the objectives. In addition, outcome measures were developed for this study. They include measurements of developmental equivalence at admission and discharge and the changes in these scores, both actual and expected., Results: Descriptive characteristics are listed in the text. Forty-six patients had bronchopulmonary dysplasia (BPD). The success rate of weaning those with BPD who were admitted with a goal of ventilator weaning was 81% (24% for those without BPD). On admission, 36 of those with BPD required three pulmonary aids (O2, continuous positive airway pressure, ventilator, and tracheostomy); at discharge, 28 required three aids. Of those without BPD, 16 required three aids at admission and 14 did so at discharge., Conclusions: Basic descriptive data are provided. The characteristics are similar to those in previous studies, although with a larger sample size and more detail. The outcome measures described cannot be compared with a criterion standard, but do offer a starting point in this underdeveloped field and will assist in future research directions.

Background: Fully informed patients were willing to start with IPPV., Patients and Methods: Twenty-three patients with amyothropic lateral sclerosis (ALS) were treated with noninvasive intermittent mechanical ventilation (7 women, 16 men, mean age 59 years). The major symptoms were dyspnoe, heavy sleep disruption resulting in severely reduced quality of life., Results: Their symptoms improved. The blood gases normalized during spontaneous breathing. They remained stable, despite a rapid decrease in respiratory force. Thirteen patients died, 6 during an acute event, 4 during bronchopulmonary infection and 3 stopped therapy, when they were fully ventilator dependent., Conclusion: We conclude that respiratory symptoms and quality of life can be improved by home intermittent mechanical ventilation. Indication should therefore be made earlier.

In summary, advances in critical care and mechanical ventilation have resulted in an increased salvage of critically ill patients, a number of whom require long-term ventilation as a means of life support. In addition, the total number of patients receiving long-term ventilation has increased dramatically over the past couple of years, and they now are treated in many different locations throughout the health care delivery system (the intensive care unit, general medical floor, specialized weaning units, and the home). Moreover, patients who require long-term ventilation usually suffer from a complex blend of medical, physiologic, and psychological disorders that may impair tolerance of chronic ventilation. Careful evaluation and systematic treatment of psychological, medical, and physiologic disorders, coupled with optimized ventilator settings, may improve patients' tolerance and maximize their functional capacity.

Home ventilation has developed as a sufficient and safe method in many neurological diseases afflicting respiratory functions. To achieve an overview of the situation of these patients in Germany, a questionnaire was distributed by the manufacturer of the most commonly used ventilator in Germany. The patients were asked to send the form back, even anonymous, to the Department of Neurology, RWTH Aachen. 65 of 110 questionnaires were answered, most of patients with muscle dystrophy's (n = 14), Polio (n = 14) and spinal cord injuries (n = 11). Home ventilation in ALS was performed in 3 cases only. The majority of patients (n = 30) was aged between 20 and 49 years, but here were also 5 children younger than 10 years. Most patients were ventilated not more than 4 years, but there were also 5 patients ventilated for over 20 years, all of them Polio-patients. 70% of all lived at home with their relatives, who take care for them. Nevertheless, 13 were full- or part-time working.

We describe 53 patients who received ventilatory support with a rocking bed. Diagnoses included previous poliomyelitis (30), muscular dystrophy (12), motor neurone disease (4), adult-onset acid maltase deficiency (4) and a miscellaneous group (3). Patients presented with respiratory insufficiency characterized by diaphragm weakness, progressive nocturnal hypoventilation and/or acute or chronic respiratory failure. Domiciliary rocking beds were used by 43 patients for a mean of 16.0 years (range 1 month to 35 years). Most patients were able to breathe adequately by day when sitting or standing, but needed assistance by rocking bed for 6-11 h when lying down for sleep. The rocking bed was well-tolerated, and associated with both symptomatic relief and amelioration of arterial blood gas abnormalities. Seventeen of these 43 patients discontinued its use, either because of discomfort (9) or increasing respiratory insufficiency (8). The rocking bed is a valuable adjunct in the management of the respiratory insufficiency associated with neuromuscular disease.

Patients with chronic obstructive pulmonary disease (COPD) or those with paralytic restrictive pulmonary syndromes caused by progressive neuromuscular disease, kyphoscoliosis or traumatic quadriplegia may require frequent hospitalization because of respiratory impairment and have increased morbidity and mortality. Pulmonary rehabilitation has been shown to decrease the frequency of hospitalization, ameliorate symptoms, increase exercise tolerance, and in one study, prolong life for individuals with COPD. It is now recognized that principles of pulmonary rehabilitation can also be used to avoid hospitalization, intubation, tracheostomy and bronchoscopy while enhancing quality of life, decreasing cost, and greatly prolonging life for individuals with paralytic restrictive syndromes and global alveolar hypoventilation as well.

The lives of many individuals with severe disability and paralytic/restrictive pulmonary conditions, which progress or worsen with time, can be significantly prolonged, and the quality of life greatly enhanced, by noninvasive or conservative rehabilitation interventions. These interventions are devised to maintain or improve the individual's orthopaedic, pulmonary, and functional status. The combination of orthopaedic surgery and rehabilitation can reverse or ameliorate musculotendinous contractures and scoliosis and, thereby, maintain optimal patient comfort, function, and the possibility for nursing or personal care. Recent advances in the use of computers, environmental control systems, robotics, etc., can increase upper extremity functional activities. Non-invasive methods of airway secretion evacuation and ventilatory support can permit individuals with little or no vital capacity to avoid acute pulmonary complications, intubation and tracheostomy indefinitely.

Pulmonary rehabilitation is directed at all patients with severe chronic respiratory insufficiency of any origin. Patients with chronic obstructive pulmonary disease clearly represent the majority. In a pulmonary rehabilitation programme, the patient must gain a better understanding of his disease and of the goals of the treatment. He benefits from permanent teaching on methods of independent self-treatment and follows a regular programme of exercise training and nutritional support aimed at increasing effort capacity. The ultimate goal of the programme is to send the patient home with improved quality of life and a higher degree of independence.

The purpose of respiratory muscle training for patients with chronic respiratory failure is to improve exercise performance during daily life. Firstly, to confirm the clinical effect on respiratory muscle training, the abdominal pad method for inspiratory muscle training and abdominal pad method with expiratory resistor for both inspiratory and expiratory muscle training were simultaneously performed. Both methods were clinically useful to increase respiratory muscle power and to subjectively decrease dyspnea. Ventilatory pattern analyzed by the Konno-Mead (K-M) diagram during exercise also showed their effectiveness. Secondly, the influence of hypoxemia and hypophosphatemia, which are important factors producing respiratory muscle fatigue, was investigated in a patient with respiratory failure. (1) O2 inhalation in patients receiving home oxygen therapy was effective in terms of the endurance time and ventilatory pattern analyzed by the K-M diagram during exercise. (2) A case of hypercapnea due to hypoventilation caused by respiratory muscle fatigue developed reduced PaCO2 following correction of serum phosphate level, suggesting that hypophosphatemia is an important clinical factor producing respiratory muscle fatigue.

Patients with chronic respiratory diseases, particularly chronic respiratory failure, are seriously handicapped mentally and physically as compared with those with diseases of other medical fields. This is because their disorders relate to breathing, which is the basic physical function directly associated with sustaining life. The authors have attempted a psychosomatic approach for patients with chronic respiratory disorders. Clinical science in this field of medicine in Japan is far behind that of other advanced nations. In the Comprehensive Emphysema Inventory, it was clearly reported that in many cases, psychological stress is involved in the onset of dyspnea. In SRQ-D or MAS, moreover, decreased motivation for treatment, anxiety regarding prognosis, and conflict were notable in psychosomatic tests. Patients on home oxygen therapy (HOT) appear to have problems in family relations and in the daily-living environment, even though HOT provides both mental and physical benefits. It was also noted that holistic or comprehensive care designed for improvement of not only physical condition, but also psychosocial aspects and quality of life, is essential to achieve good results in the respiratory rehabilitation of patients affected by chronic respiratory failure.

Spinal cord injured children have special needs because of their potential for physical, intellectual, psychological and social growth. The overriding goal for comprehensive medical rehabilitation is to provide services required by the child for maximal recovery and to compensate for lost or impaired function while permitting the fullest development of potential in all areas. Because spinal cord injuries in childhood are not common, it is imperative that rehabilitation should be comprehensively coordinated and directed by a single specialist taking overall responsibility for the chronic phase of management. Rehabilitation involves the child and his family, a hospital-based and rehabilitation centre-based team and a school. Every effort must be made to prevent medical and/or physical complications that could interfere with rehabilitation or lead to greater disability.

The purpose of this article is to present a cost analysis of in-home vs institutionalization for severely physically disabled ventilator-assisted individuals (VAIs). Following rehabilitation and adaptation to noninvasive methods of ventilatory support, 30 VAIs were maintained in the community for 12.9 +/- 1.1 years with personal care attendants organized by a home care vendor reimbursed by New York City Medicaid. The program permitted self-directed severely disabled clients, including these 30 exclusively nontracheostomized VAIs, to live in the community and direct their attendant care and personal affairs. Prior to discharge home, the 30 patients resided in the respiratory unit of a long-term care facility for a mean of 8.9 +/- 10.1 years. The unit is currently reimbursed at a mean rate of $718.80 per patient per day. The current mean total cost of maintaining these VAIs in the community is $235.13 +/- 56.73 per patient per day. The conversion to and/or maintenance on 24-h nontracheostomy ventilatory support permitted discharge to the community by allowing the VAI to be attended by trained but uncredentialed home care attendants, thus avoiding prohibitively expensive in-home nursing for tracheostomy care. This created a savings to the public of 77 percent or $176,137 per year per client. We conclude that conversion to and/or use of noninvasive methods of ventilatory aid can be a reasonable and cost-saving goal. More respiratory rehabilitation centers are needed to free up hospital beds and facilitate discharge of VAIs to the community. There is also evidence that trained attendants should be permitted to suction tracheostomized VAIs in the home.

Pulmonary function studies in 20,000 non-clinical probands throughout Austria showed abnormal findings in 20%. This is further evidence that chronic obstructive pulmonary disease (COPD) is epidemiologically similar to coronary heart disease. A staging classification of COPD has been proposed to facilitate a strategy for the prevention of the disease and to permit coordination among general practitioners, specialists and pulmonary function laboratories. This initiative aims to reduce the prevalence of advanced (stage IV) COPD. In the meantime long-term oxygen therapy and respirator therapy should be used increasingly to improve the quality of life of patients with advanced COPD.

Ventilatory insufficiency and impaired airway secretion clearance are common complications of spinal cord injury (SCI) and can lead to respiratory failure which is the leading cause of death in both the acute and chronic stages. Standard invasive management options such as intubation, tracheostomy and electrophrenic respiration have been reviewed. The review findings are consistent with our clinical experience in that these invasive options appear to entail unacceptably high morbidity and risks of mortality. A number of detailed parameters are suggested for evaluating the respiratory functioning of the individual in order to determine the most acceptable and successful noninvasive systems for both ventilatory support and evacuation of airway secretions. They are physiological substitutes for the action of the inspiratory and expiratory muscles. These techniques are described in detail. We conclude that noninvasive techniques can safely and effectively obviate the need for intubation, tracheostomy and electrophrenic pacemakers in appropriate individuals with SCI.

The author statistically analyzes 542 patients who were hospitalized in the Prolonged Respiratory Care Unit of Bethesda Lutheran Medical Center in St. Paul, Minnesota, between 1979 and 1989. A demographic description of the patients, an analysis of discharge status, and a discussion of reimbursement for care are presented. It is apparent from this analysis that: 1) there is a large number of patients who will require chronic ventilator support, 2) weaning of "unweanable" patients can occur in selected cases and can be most expeditiously accomplished in specialized units, 3) these specialized units are cost effective, and early transfer from the DRG-reimbursed ICUs would benefit not only the patient but also the reimbursing agent and referral hospital, 4) specialized, adequately funded centers for unweanable patients need to be developed, and 5) further studies and education concerning the imprudent use of ventilators must be implemented by the appropriate physician organizations.

The art of breathing exercises can be traced to the late 1800s. In the past 10 years, the increased demand for treatment for respiratory muscle failure of dysfunction has resulted in numerous studies evaluating methods of treatment or training. This article provides an overview of research and practice, focused on treating the medical patient experiencing dyspnea or loss of respiratory muscle strength and endurance.

This paper examines the impact changing ventilation technology and the ideologies of rehabilitation medicine on the long term adaptations of people with post-polio respiratory disability. Multiple in-depth interviews with 10 respirator-dependent people indicates that cultural ideologies learned during rehabilitation continue to effect the adoption of technology and daily management of respiratory insufficiency. Adaptive strategies through which benefits of technology are maximized and the social costs minimized, have transformed rehabilitation ideologies and moderated the late, aging-related effects of poliomyelitis.

For about twenty years, the method for prolonged artificial respiration was an imitation of spontaneous breathing pattern. New pathophysiological informations about acute respiratory failure showed that a technique which is widely different in frequency, cycle, and tidal volume is more suitable for the fast and safe restauration of spontaneous breathing. Especially the determination of pulmonary shunt flow (QS/Qt), and of the ratio of dead space to tidal volume (VD/VT) have completed our knowledge. In the management of patients with prolonged artificial ventilation, the calculation of shunt flow allows an evaluation of therapeutic procedures. This was the reason to present a nomogram, which simplifies the determination of pulmonary shunt and to discuss an easy, non-invasive method to at least estimate its amount.

Two respiratory physiotherapy methods were evaluated by analyzing changes in the transcutaneous partial pressure of CO2 (tcPCO2) in 15 patients with respiratory insufficiency. All were receiving continuous oxygen therapy and had CO2 retention. The physiotherapy methods compared were thoracic compression (TC) and positive expiratory pressure (PEP). The mean decrease (+/- SD) in tcPCO2 was the same with both methods, 0.6 +/- 0.4 kPa, but there were interindividual differences. This study indicates that both TC and PEP effectively decrease tcPCO2, although only temporarily in many cases. Continuous tcPCO2 monitoring is a useful clinical method for determining and teaching optimal respiratory therapy.

During a two-year period we admitted 13 respirator-dependent quadriplegic patients to the spinal cord injury service at Wood VAMC for weaning from a mechanical ventilator, and rehabilitation. The patients were sent to the Spinal Cord Injury Center because initial weaning attempts from the respirator at other medical centers were unsuccessful. We successfully weaned them from the respirator, and at the time of discharge, only two patients required an indwelling tracheostomy tube for suction. The time required for weaning off the respirator varied from two days to 14 months. Most of the patients were discharged and many of them could independently perform the activities of daily living. We conclude that are four main factors which influence the successful weaning of dependent quadriplegics from the mechanical ventilator: alleviation of patient's anxiety and depression; family support; close working relationship between staff; prevention of complications such as pneumonia and urinary tract infections.

The application of skeletal muscle training principles to the respiratory muscles is a relatively new field. Strength and endurance training of the respiratory muscles can be achieved in normal humans and in patients with neuromuscular and chronic obstructive pulmonary diseases. Careful monitoring is required throughout a training program to ensure that the respiratory muscles are, in fact, being trained. This is a promising area, although further research is necessary to determine the indications for respiratory muscle training. Furthermore, it is still necessary to determine the optimal mode of training.

The authors described an optical technique enabling patients with chronic respiratory insuffiency, at first to get used to assisted ventilation, then to control it, to obtain an active ventilation operated by machine corresponding to a particular shape of the pressure curve and to the lesser haemodynamic repercussion.

A pneumobelt with a positive pressure respirator was used for sleep for three patients with chronic respiratory insufficiency secondary to syringomyelia, poliomyelitis, and Friedreich's ataxia respectively. This device was found to be effective in improving and maintaining their daily functioning in the community or institute.