GBS respiratory complications revisited.
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Biomedical subjects
Publications and source records attributed to J R Bach.
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Although the lung is almost always involved in sarcoidosis and respiratory failure is not uncommonly caused by intrinsic lung disease, chronic alveolar hypoventilation on the basis of neurosarcoidosis has not been previously described. This report describes the case of a 42-year-old woman with sarcoidosis and documented involvement of the lungs, muscle, peripheral nerves, and skin. She presented with ventilatory failure and a vital capacity of 700mL and required hospitalization for intubation and mechanical ventilation. After failing ventilator weaning attempts and requiring continuous ventilatory assistance, she was extubated to continuous use of noninvasive intermittent positive pressure ventilation (IPPV). She eventually weaned to nocturnal-only use of noninvasive IPPV. However, her PaCO2 remained elevated during daytime hours despite the fact that her vital capacity had returned to 3,600mL. She was not obese, and her oxyhemoglobin saturation (SaO2) was normal on room air. Subsequent attempts to discontinue nocturnal nasal IPPV resulted in marked nocturnal decreases in SaO2 and return of symptoms. This case illustrates successful noninvasive management of an apparently unique occurrence of central alveolar hypoventilation caused by neurosarcoidosis.
All patients with respiratory impairment have either primarily ventilatory or primarily oxygenation impairment. Patients with neuromuscular conditions fall into the former category but are all too often managed as though they had the latter with oxygen therapy, bronchodilators, chest physical therapy, intermittent positive pressure breathing, and so on. This approach can only hasten respiratory failure and management by tracheostomy-However, it has been reported that with the use of noninvasive respiratory muscle aids, respiratory morbidity and mortality can be prevented for most patients with neuromuscular disease without resort to tracheostomy or even hospitalization.
OBJECTIVE: To evaluate the use of mouth piece/nasal intermittent positive-pressure ventilation (IPPV) as an alternative to intubation or to permit extubation for patients with primarily neuromuscular ventilatory impairment and no ventilator-free breathing ability. DESIGN: A case control study. INTERVENTIONS: Using a protocol in which oxyhemoglobin desaturation was prevented or reversed by the continuous use of noninvasive IPPV and manually and mechanically assisted coughing as needed, patients with neuromuscular ventilatory failure and no ventilator-free breathing ability were managed noninvasively or extubated to continuous use of noninvasive IPPV for ventilatory support on room air. MEASUREMENTS AND MAIN RESULTS: Four of ten patients who presented in acute ventilatory failure were managed without intubation, despite becoming dependent on continuous ventilator use. The six intubated patients were extubated successfully to continuous noninvasive IPPV once normal arterial oxygen saturation levels could be maintained on room air, despite their having no ventilator-free breathing ability. CONCLUSIONS: The use of inspiratory and expiratory aids can decrease the need for intubation for patients with neuromuscular ventilatory failure in the absence of significant lung disease. It can also permit extubation, despite the need for continuous ventilatory support and, thereby, decrease the need to resort to tracheostomy.
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OBJECTIVE: To analyze the relationship between disease progression, pulmonary volumes, respiratory muscle strength (maximum inspiratory [MIP] and expiratory [MEP] pressure), and arterial blood gases for patients with Duchenne muscular dystrophy (DMD). DESIGN: An inception cohort study of pulmonary volumes, MIPs, and MEPs, correlated with age and PaCO2 levels and with each other using linear and nonlinear regression analyses. SETTING: Outpatient clinic. PATIENTS: Fifty-two consecutive DMD patients who presented for regular evaluations at a regional DMD center. RESULTS: Maximum expiratory pressures were 47.7% +/- 10.9% of normal in the 167- to 14-year-old patients and decreased linearly thereafter (MEP% = -2.7 x age +73.8; p < .001). Declines in MEP also correlated linearly with expiratory reserve volume (p < .001) and inversely with residual volume (p < .001). By contrast, MIP was 66.3% +/- 19.0% in the 357- to 14-year-old patients and then declined to 30.2% +/- 19.5% after age 14. No linear relationships were found with age but declines did correlate linearly with inspiratory reserve volume (p < .001) and total lung capacity (p < .001). PaCO2 elevations correlated best with decreases in MIP (p < .0001) and appeared when MIP was below 30cmH2O. CONCLUSIONS: Lung volume changes in DMD patients correlate with respiratory muscle weakness, and although inspiratory muscle dysfunction plays a key role in the development of chronic ventilatory insufficiency, reductions in expiratory muscle strength are the first signs of dysfunction and lead to the first episodes of respiratory failure.
OBJECTIVE: To evaluate the outcomes of an inexpensive outpatient pulmonary rehabilitation program for stable patients with chronic obstructive pulmonary disease (COPD). DESIGN: Before-and-after trial. SETTING: Private, ambulatory setting with carryover to the home. PATIENTS: Forty-six stable COPD patients, from 45 to 80 years of age. INTERVENTIONS: A pulmonary rehabilitation program consisting of education, training, group therapy, and an individualized regimen of home-based extremity and inspiratory muscle exercise with weekly ambulatory sessions over a 10-week period. MAIN OUTCOME MEASURES: Symptom-limited oxygen consumption (SLVO2), distance walked in 12 minutes (12 MW), dyspnea, endurance, forced expiratory volumes, minute ventilation (MV), changes in heart rate and blood pressure, and inspiratory muscle work tolerance (IMWT). RESULTS: There were significant increases (p < .05) in SLVO2, 12 MW, inspiratory muscle and bicycle work capacity, and forced vital capacity (FVC). The sensation of dyspnea significantly decreased during the performance of activities of daily living (ADL) (p < .05). There was no significant change in forced expiratory volume in 1 second (FEV1). The cost of the 10 outpatient sessions was $650. CONCLUSIONS: An inexpensive outpatient/home-based pulmonary rehabilitation program provided largely by a specifically trained therapist under physician supervision can significantly improve parameters associated with quality of life for patients with COPD.
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STUDY OBJECTIVE: To evaluate the effects of a new respiratory management protocol on respiratory morbidity and hospitalization rates for patients with Duchenne muscular dystrophy (DMD). DESIGN: A retrospective cohort study. METHODS: Using a protocol in which oxyhemoglobin desaturation was prevented or reversed by the use of noninvasive intermittent positive pressure ventilation (IPPV) and assisted coughing as needed, the hospitalization rates and days for 24 protocol DMD ventilator users were compared with those of 22 nonprotocol DMD tracheostomy IPPV users. RESULTS: The 22 conventionally managed patients were hospitalized a mean of 72.2+/-112 days when undergoing tracheostomy. This included a 16.1+/-5.4-day period of translaryngeal intubation. The 24 protocol patients were hospitalized a mean of 6.0+/-2.4 days (p<0.005) when beginning ventilator use. Over their next 126.2 patient-years of ventilator use, the 24 protocol patients had significantly lower rates of hospitalization (p<0.008) and hospitalization days (p<0.005) than had the tracheostomy IPPV users over a 167.2 patient-year period. This is true although 14 of the 24 protocol patients went on to require 24-h noninvasive IPPV for 4.5+/-3.6 years. Five of the 14 have yet to be hospitalized. CONCLUSION: The use of inspiratory and expiratory aids can prolong survival while significantly decreasing the pulmonary morbidity and hospitalization rates associated with conventional resort to tracheostomy IPPV.
This self-directed learning module highlights both pulmonary and nonpulmonary aspects in the rehabilitation of patients with pulmonary disease and the assessment and physical therapeutic options in the pulmonary management of patients with neurological disorders. It is part of the chapter on cardiovascular, pulmonary, and cancer rehabilitation in the Self-Directed Physiatric Education Program for practitioners and trainees in physical medicine and rehabilitation. New advances covered in this section include the use of physical medicine modalities for respiratory muscle rest and technological advances in the management of airway secretions in both pulmonary and neurological disorders. For the former, these approaches can decrease symptoms and the frequency of hospitalization and increase exercise tolerance and quality of life. The use of physical medicine modalities will be discussed as inspiratory and expiratory muscle aids. These methods, when used as alternatives to tracheal intubation and long-term tracheostomy in patients with neurological disorders, improve quality of life, minimize cost, enhance survival, and facilitate community care.
The purpose of this study was to prospectively compare parameters that might predict successful translaryngeal extubation and tracheostomy tube decannulation. Irrespective of ventilatory function, 62 extubation/decannulation attempts were made on 49 consecutive patients with primarily neuromuscular ventilatory insufficiency who satisfied criteria. Thirty-four patients required 24-h ventilatory support. Noninvasive intermittent positive pressure ventilation (IPPV) was substituted as needed for IPPV via translaryngeal or tracheostomy tubes. Successful decannulation was defined as extubation or decannulation and site closure with no consequent respiratory symptoms or blood gas deterioration for at least 2 weeks. Failure was defined by the appearance of respiratory distress and decreases in vital capacity and oxyhemoglobin saturation despite use of noninvasive IPPV and assisted coughing. The independent variables of age, extent of predecannulation ventilator use, vital capacity, and peak cough flows (PCF) were studied to determine their utility in predicting successful extubation and decannulation. Only the ability to generate PCF greater than 160 L/min predicted success, whereas inability to generate 160 L/min predicted the need to replace the tube. All 43 attempts on patients with PCF greater than 160 L/min succeeded; all 15 attempts on patients with PCF below 160 L/min failed; and of 4 patients with PCF of 160 L/min, 2 succeeded and 2 failed. We conclude that the ability to generate PCF of at least 160 L/min is necessary for the successful extubation or tracheostomy tube decannulation of patients with neuromuscular disease irrespective of ability to breathe.
This article traces the development of mechanical ventilatory support methods from the use of body ventilators to tracheal cannulation to the use of noninvasive ventilatory support and airway secretion management alternatives. Although it has been known that tracheostomy tubes could be used for ventilatory support and airway secretion management since 1869, body ventilators continued to be the main methods of long-term ventilatory support in the United States, with tracheostomy performed only for patients with severe bulbar muscle dysfunction, until the late 1950s. Recent technological developments, however, have created renewed interest in noninvasive alternatives.
Most patients with primarily ventilation impairment can use noninvasive alternatives to tracheostomy for long-term ventilatory support and airway secretion management. The most important and versatile method of noninvasive ventilatory support is mouthpiece intermittent positive pressure ventilation. Mouthpiece intermittent positive pressure ventilation also can be used via a Lipseal for nocturnal support. The intermittent abdominal pressure ventilator is an option for daytime aid, and nasal intermittent positive pressure ventilation is usually preferred for nocturnal support. Manually assisted coughing and mechanical insufflation-exsufflation can be critical for airway secretion elimination.
Post-poliomyelitis respiratory impairment is extremely common and entails considerable risk of morbidity and mortality. Respiratory muscle weakness is the primary etiological factor but post-poliomyelitis individuals (PPIs) also have a high incidence of scoliosis, obesity, sleep disordered breathing, and bulbar muscle dysfunction, all of which can add to the risk. One hundred forty-five PPIs were managed by noninvasive alternatives to intermittent positive pressure ventilation (IPPV) via an indwelling tracheostomy. When properly managed in this manner, acute respiratory failure requiring hospitalization, tracheal intubation, and bronchoscopies were avoided. Timely introduction of mouthpiece IPPV, nasal IPPV, manually and mechanically assisted coughing, and noninvasive blood gas monitoring in the home were the principal techniques used for optimizing quality of life and for avoiding complications.
The purpose of this study was to determine which pulmonary function variables best predicted the potential for prolonging survival of individuals with amyotrophic lateral sclerosis (ALS) by the use of physical medicine respiratory muscle aid alternatives to tracheostomy for ventilatory support and airway suctioning. The records of 27 such ALS ventilator users with less than 15 minutes of ventilator-free breathing time for a mean +/- standard deviation of 23.7 +/- 20.3 months (range, 1 to 65) were reviewed. All patients underwent measurements of vital capacity (VC), maximum insufflation capacity (MIC), MIC VC difference, forced expiratory volumes, and peak cough expiratory flows (PCEF) every 1 to 6 months, depending on rate of disease progression, until requiring 24-hour ventilatory support. The ability to generate assisted PCEF in excess of 3L/sec and the ability to hold an insufflation deeper than the VC were associated with the capacity to prolong survival by methods other than tracheostomy, whereas the extent of decrease in VC and autonomous breathing ability were not. Because the PCEF and MIC VC difference correlate with bulbar muscle function, it can be concluded that the ability to use 24-hour ventilatory support by noninvasive means is a function of residual bulbar muscle strength and is independent of VC or the extent of need for ventilatory support. Properly equipped and trained, some ALS patients can use noninvasive respiratory muscle aids to delay or eliminate the need for tracheostomy.
Ten individuals with spinal muscular atrophy (SMA) and chronic ventilatory insufficiency were trained in the use of assisted coughing techniques and received intermittent positive pressure ventilation (IPPV) via oral and/or nasal interfaces for a mean of 5.3 (range = 1 to 17) years. During this time they had significantly fewer respiratory complications than before introduction of noninvasive respiratory muscle aids despite the fact that 6 of the 10 went on to require more than 20 hours per day of ventilator use with less than 2 hours of ventilator-free breathing time (VFBT). All except two noninvasive IPPV users had vital capacities (VCs) less than 13% of predicted normal. They could, however, communicate verbally and take nutrition by mouth. All of the patients remained in the community. Five patients were gainfully employed and four were in school. We conclude that noninvasive respiratory muscle aids including noninvasive IPPV and assisted coughing techniques are effective and practical alternatives to tracheostomy for SMA patients with ventilatory failure but functional bulbar musculature.