High- and low-level pressure support during walking in people with severe kyphoscoliosis.

To determine whether the level of pressure support (PS) provided during exercise influences endurance time in people with severe kyphoscoliosis, a double-blind randomised crossover study was performed. We hypothesised that high-level PS would be required to enhance endurance time in this population with high impedance to inflation. 13 participants with severe kyphoscoliosis performed four endurance treadmill tests in random order: unassisted; with sham PS; low-level PS of 10 cmH(2)O (PS 10); and high-level PS of 20 cmH(2)O (PS 20). Participants and assessors were blinded to the level of PS delivered during exercise. Endurance time was greater with PS 20 (median (interquartile range) 217 (168-424) s) compared with unassisted exercise (139 (111-189) s), sham PS (103 (88-155) s) and PS 10 (159 (131-206) s). In addition, isotime respiratory rate was decreased by 8 breaths x min(-1) (95% CI -11- -5 breaths x min(-1)) and isotime oxygen saturation increased by 4% (95% CI 1-7%) with PS 20 compared with unassisted exercise. People with severe kyphoscoliosis require high-level PS during walking to improve exercise performance. Investigation of high-level PS as an adjunct to exercise training or to assist in the performance of daily activities is warranted.

Obesity and the lung: 2. Obesity and sleep-disordered breathing.

As the prevalence of obesity increases in both the developed and the developing world, the respiratory consequences are often underappreciated. This review discusses the presentation, pathogenesis, diagnosis and management of the obstructive sleep apnoea, overlap and obesity hypoventilation syndromes. Patients with these conditions will commonly present to respiratory physicians, and recognition and effective treatment have important benefits in terms of patient quality of life and reduction in healthcare utilisation. Measures to curb the obesity epidemic are urgently required.

Randomised trial of CPAP vs bilevel support in the treatment of obesity hypoventilation syndrome without severe nocturnal desaturation.

BACKGROUND: Untreated, obesity hypoventilation is associated with significant use of health care resources and high mortality. It remains unclear whether continuous positive airway pressure (CPAP) or bilevel ventilatory support (BVS) should be used as initial management. The aim of this study was to determine if one form of positive pressure is superior to the other in improving daytime respiratory failure. METHODS: A prospective randomised study was performed in patients with obesity hypoventilation referred with respiratory failure. After exclusion of patients with persisting severe nocturnal hypoxaemia (Spo(2) < 80% for > 10 min) or carbon dioxide retention (> 10 mm Hg) despite optimal CPAP, the remaining patients were randomly assigned to receive either CPAP or BVS over a 3-month period. The primary outcome was change in daytime carbon dioxide level. Secondary outcome measures included daytime sleepiness, quality of life, compliance with treatment and psychomotor vigilance testing. RESULTS: Thirty-six patients were randomised to either home CPAP (n = 18) or BVS (n = 18). The two groups did not differ significantly at baseline with regard to physiological or clinical characteristics. Following 3 months of treatment, daytime carbon dioxide levels decreased in both groups (CPAP 6 (8) mm Hg; BVS 7 (7) mm Hg) with no between-group differences. There was no difference in compliance between the two treatment groups (5.8 (2.4) h/night CPAP vs 6.1 (2.1) h/night BVS). Although both groups reported an improvement in daytime sleepiness, subjective sleep quality and psychomotor vigilance performance were better with BVS. CONCLUSIONS: Both CPAP and BVS appear to be equally effective in improving daytime hypercapnia in a subgroup of patients with obesity hypoventilation syndrome without severe nocturnal hypoxaemia. TRIAL REGISTRATION NUMBER: Australian Clinical Trials Registry ACTRN01205000096651.

Non-invasive ventilation for cystic fibrosis.

BACKGROUND: Non-invasive ventilation (NIV) may be a means to temporarily reverse or slow the progression of respiratory failure in cystic fibrosis (CF). OBJECTIVES: To compare the effect of NIV versus no NIV in people with CF. SEARCH STRATEGY: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register comprising references identified from comprehensive electronic database searches, handsearching relevant journals and abstract books of conference proceedings. We searched the reference lists of each trial for additional publications possibly containing other trials. Most recent search: October 2006. SELECTION CRITERIA: Randomised controlled trials comparing a form of pressure preset or volume preset NIV to no NIV in people with acute or chronic respiratory failure in CF. DATA COLLECTION AND ANALYSIS: Three reviewers independently assessed trials for inclusion criteria and methodological quality, and extracted data. MAIN RESULTS: Fifteen trials were identified; seven trials met the inclusion criteria with a total of 106 participants. Six trials evaluated single treatment sessions only and one evaluated a six-week intervention. Four trials (79 participants) evaluated NIV for airway clearance compared with an alternative chest physiotherapy method and showed that airway clearance may be easier with NIV and people with CF may prefer it. We were unable to find any evidence that NIV increases sputum expectoration, but it did improve some lung function parameters.Three trials (27 participants) evaluated NIV for overnight ventilatory support. Lung function and nocturnal transcutaneous carbon dioxide were evaluated within two trials. Due to the small numbers of participants and statistical issues, there were discrepancies in the results between the RevMan and the original trial analyses. No clear differences were found between NIV compared with oxygen or room air except for exercise performance, which significantly improved with NIV compared to room air over six weeks. AUTHORS' CONCLUSIONS: Non-invasive ventilation may be a useful adjunct to other airway clearance techniques, particularly in people with CF who have difficulty expectorating sputum. Non-invasive ventilation, when used in addition to oxygen, may improve gas exchange during sleep to a greater extent than oxygen therapy alone in moderate to severe disease. These benefits of NIV have largely been demonstrated in single treatment sessions with small numbers of participants. The impact of this therapy on pulmonary exacerbations and disease progression remain unclear. There is a need for long-term randomised controlled trials which are adequately powered to determine the clinical effects of non-invasive ventilation in CF airway clearance and exercise.

Effects of short-term NIPPV in the treatment of patients with severe obstructive sleep apnea and hypercapnia.

Although nasal continuous positive airway pressure (CPAP) is effective in the treatment of most patients with obstructive sleep apnea (OSA), there is a small group of such patients in whom rapid eye movement (REM) hypoventilation and CO2 retention persist despite the use of CPAP and supplemental oxygen. In this report we describe our experience with nocturnal nasal ventilation (nocturnal nasal positive pressure ventilation [NIPPV] in such patients and its effectiveness in reversing daytime hypercapnia. Thirteen patients, aged 28 to 69 years, with severe OSA confirmed on polysomnography, failed to respond to initial CPAP therapy. All were grossly obese (body mass index [BMI] > 35 kg.ml-1) and hypercapnic (mean PaCO2, 62 mm Hg). Nocturnal nasal ventilation was commenced using a volume-cycled ventilator, which was well tolerated in all patients. After 7 to 18 days of NIPPV, significant improvements in daytime arterial blood gas values were achieved, with a rise in arterial oxygen tension from 50 +/- 2.6 (SEM) to 66 +/- 3 mm Hg (p < 0.001) and a fall in CO2 from 62 +/- 2.5 to 46 +/- 1 mm Hg (p < 0.0001). Nine of the 13 patients were able to be established on a regimen of nasal CPAP after this period, while 3 patients required a longer period (up to 3 months) before adequate nocturnal ventilation could be maintained. In one patient, the improvements in ventilatory drive achieved with NIPPV could not be maintained on CPAP, and she was transferred on to NIPPV long term. These results indicate that effective nasal ventilation leads to an overall improvement in spontaneous ventilation and blood gas values both awake and asleep. We believe this improvement is the result of improved central ventilatory drive. Short-term NIPPV provides lasting benefits allowing the majority of such patients to resume CPAP therapy. Short-term intervention with this therapy should be considered as an interim measure in patients with severe hypercapnic OSA who fail to respond to initial CPAP therapy.

Nocturnal nasal IPPV stabilizes patients with cystic fibrosis and hypercapnic respiratory failure.

Nocturnal nasal intermittent positive pressure ventilation (nIPPV) has been used successfully in the management of patients with respiratory failure due to chest wall deformity and neuromuscular disease. In order to determine if nIPPV is useful in patients with cystic fibrosis (CF) complicated by respiratory failure, we treated four hypercapnic patients for up to 18 months. All patients had failed to respond to intensive conventional therapy, including nocturnal nasal CPAP in three of the patients. Within a few days of commencing nIPPV, all reported improved length and quality of sleep. There was lessening of the degree of hypercapnia and an increase in respiratory muscle strength. After stabilization in the hospital, all patients were able to be discharged home receiving nocturnal assisted ventilation. The improvements seen in these patients have been maintained for up to 18 months. We believe nIPPV offers an effective therapeutic approach for patients with end-stage CF in hypercapnic respiratory failure and may be particularly advantageous for those awaiting heart-lung transplant.

Benefits of nocturnal nasal CPAP in patients with cystic fibrosis.

Patients with cystic fibrosis (CF) often hypoventilate during sleep with marked falls in oxygen saturation (SaO2%). This occurs most commonly during REM sleep, when there is a reduction in rib cage excursion and a fall in end-expiratory lung volume (EELV). The aim of this study was to examine the effect of nocturnal nasal continuous positive airway pressure (nCPAP) on SaO2 and the respiratory disturbance index (RDI) during sleep in patients with CF and severe lung disease. Seven patients (FEV1% pred, 23 +/- 5; range, 14 to 28%) were evaluated during sleep on two nights, control and nCPAP (11 +/- 2 cm H2O; range, 8 to 16 cm H2O), with four patients breathing room air and three patients breathing supplemental oxygen on both nights. Mean awake SaO2 was 91 +/- 1% (range, 89 to 93%). All patients showed significant oxyhemoglobin desaturation and respiratory disturbance in the control study. The maximal falls in SaO2 (15 +/- 10%) were most often associated with phasic eye movements, and a decline in rib cage excursion and the sum signal (Respitrace) during REM sleep. Nasal CPAP resulted in a significant improvement in the mean minimum oxygen saturation (MMOS) during both NREM (nCPAP 91 +/- 3% vs control 88 +/- 2%, p < 0.05) and REM sleep (nCPAP 89 +/- 6% vs control 83 +/- 6%, p < 0.05). Transcutaneous CO2 measurements were not significantly different between the control and the nCPAP studies. The RDI was also significantly reduced with nCPAP especially during REM sleep (9 +/- 7 events per hour vs control 25 +/- 11 events per hour, p < 0.05). Nasal CPAP caused no change in total sleep time or sleep efficiency yet significantly reduced the RDI and improved baseline SaO2 during both NREM and REM sleep.

Predicting sleep-disordered breathing in patients with cystic fibrosis.

STUDY OBJECTIVES: To examine predictors of sleep-disordered breathing in patients with cystic fibrosis (CF) and moderate-to-severe lung disease using a comprehensive evaluation of both sleep and daytime function. DESIGN: Cross-sectional analysis of sleep studies, lung function, respiratory muscle strength, and evening and morning arterial blood gas measurements in patients with stable CF. A questionnaire addressing sleep quality was administered. Forward stepwise regression analysis was used to identify the parameters that best predict sleep-related desaturation, hypercapnia, and respiratory disturbance. SETTING: Sleep investigation unit and lung function laboratory. PATIENTS: Thirty-two patients with CF and FEV(1) < 65% predicted, in stable clinical condition. Patients were aged 27 +/- 8 years (mean +/- 1 SD) with FEV(1) of 36 +/- 10% predicted, evening PaO(2) of 68 +/- 8 mm Hg, and PaCO(2) of 43 +/- 5 mm Hg. RESULTS: Evening PaO(2) (p < 0.0001) and morning PaCO(2) (p < 0.01) were predictive of the average minimum oxyhemoglobin saturation per 30-s epoch of sleep (r(2) = 0.74; p < 0.0001). Evening PaO(2) (p < 0.001) was predictive of the rise in transcutaneous carbon dioxide (TcCO(2)) seen from non-rapid eye movement (NREM) to rapid eye movement (REM) sleep (r(2) = 0.37; p < 0.001). In addition, there was some relationship between expiratory respiratory muscle strength and the REM respiratory disturbance index (r(2) = 0.22; p < 0.01). CONCLUSION: Evening PaO(2) was found to contribute significantly to the ability to predict both sleep-related desaturation and the rise in TcCO(2) from NREM sleep to REM sleep in this subgroup of patients with CF.

Sleep and respiration: Implications for pulmonary rehabilitation.

Despite the growing interest in the role of the inspiratory and accessory muscles of respiration in chronic respiratory disease, few therapists have considered the role sleep may play in the deterioration of a patient's clinical condition. A number of important physiological changes to respiration occur during sleep, which affect chest wall mechanics and gas exchange. The ensuing abnormalities caused by sleep hypoventilation and fragmentation can severely affect daytime function and wellbeing. Nocturnal nasal positive pressure ventilation (NIPPV) is now established as an effective means of preventing such changes in patients with chest wall and lung disease. Therapists involved in pulmonary rehabilitation programs must recognise the potential for nocturnal respiratory events to severely affect daytime function and understand the importance of reversing nocturnal respiratory failure in order to maximise the rehabilitation potential of patients.

An overview of nasal CPAP therapy in the management of obstructive sleep apnea.

Obstructive sleep apnea is readily reversible with nasally administered continuous positive airway pressure, although its effectiveness can be limited by poor patient compliance with therapy. With recent developments in technology, the ability to both diagnose and manage this disorder is dramatically improving. Assessment and therapeutic intervention can now be carried out not only in attended settings, such as sophisticated sleep laboratories, but in semiattended and unattended situations, including the home. What impact these advances will have on improving patient tolerance to therapy and reducing long-term clinical consequences of obstructive sleep apnea remains to be seen. However, there is no doubt that sleep-trained professionals now have many more tools at their disposal to diagnose and treat this disorder.

Bilevel ventilation during exercise in acute on chronic respiratory failure: a preliminary study.

To determine the immediate effects of bilevel non-invasive ventilation plus oxygen (NIV+O(2)) during exercise compared to exercise with O(2) alone in people recovering from acute on chronic hypercapnic respiratory failure (HRF), a randomised crossover study with repeated measures was performed. Eighteen participants performed six minute walk tests (6MWT) and 16 participants performed unsupported arm exercise (UAE) tests with NIV+O(2) and with O(2) alone in random order. Distance walked increased by a mean of 43.4m (95% CI 14.1 to 72.8, p=0.006) with NIV+O(2) compared to exercise with O(2) alone. In addition, isotime oxygen saturation increased by a mean of 5% (95% CI 2-7, p=0.001) and isotime dyspnoea was reduced [median 2 (interquartile range (IQR) 1-4) versus 4 (3-5), p=0.028] with NIV+O(2). A statistically significant increase was also observed in UAE endurance time with NIV+O(2) [median 201s (IQR 93-414) versus 157 (90-342), p=0.033], and isotime perceived exertion (arm muscle fatigue) was reduced by a mean of 1.0 on the Borg scale (95% CI -1.9 to -0.1, p=0.037) compared with O(2) alone. Non-invasive ventilation plus O(2) during walking resulted in an immediate improvement in distance walked and oxygen saturation, and a reduction in dyspnoea compared to exercise with O(2) alone in people recovering from acute on chronic HRF. The reduction of dyspnoea during walking and arm muscle fatigue during UAE observed with NIV+O(2) may allow patients to better tolerate exercise early in the recovery period.

Physiotherapy involvement in non-invasive ventilation hospital services: a British Isles survey.

The aim of this study was to assess the involvement of physiotherapists in the delivery of non-invasive ventilation (NIV) services in the British Isles. A postal questionnaire was sent to all senior physiotherapists in hospitals providing specialist respiratory medicine. The response rate was 88% (269/305). Physiotherapists were involved in managing patients using NIV in 212/233 hospitals that used NIV. The majority of physiotherapists, 97% (206/212), were involved in treating patients on NIV. Physiotherapists assessed patients for NIV in 68% (145/212) of hospitals and were involved in setting up patients on NIV in 46% (97/212) of hospitals. There were no major differences between countries, within the British Isles, in the level of involvement of Physiotherapists in the management of patients on NIV. Physiotherapists need to develop specialist skills with regard to assessment and setting up patients on NIV if they want to expand their role in the management of patients on NIV.

Effects of long-term nocturnal nasal ventilation on spontaneous breathing during sleep in neuromuscular and chest wall disorders.

Nocturnal nasal intermittent positive pressure ventilation (NIPPV) is an effective means of normalizing awake blood gases in patients with respiratory insufficiency due to neuromuscular or chest wall dysfunction. However, little attention has been paid to the effects of long-term ventilation on spontaneous breathing during sleep in such patients. The purpose of this study was to determine whether spontaneous breathing during sleep improved after long-term nasal ventilation. Fourteen patients with documented nocturnal respiratory failure, who had been treated with nocturnal NIPPV for at least 6 months, were reviewed with an all night polysomnograph on a night without ventilatory support. The severity of nocturnal desaturation both in non-rapid eye movement (NREM) and rapid eye movement (REM) sleep without nocturnal ventilation was compared to desaturation measured during the initial diagnostic study. Spontaneous daytime blood gas values (mean +/- SD) were significantly improved at follow-up compared to values obtained prior to nasal ventilation: arterial oxygen tension (Pa,O2): 7.5 +/- 1.2 to 10.2 +/- 1.3 kPa (p < 0.005); arterial carbon dioxide tension (Pa,CO2) 8.2 +/- 1.6 to 6.4 +/- 0.7 kPa (p < 0.001). Significant improvements in inspiratory muscle strength were also observed with maximal inspiratory pressure (Pl, max) increasing from a baseline value of 41 +/- 18 to 65 +/- 26% predicted measured prior to the night of ventilation withdrawal (p < 0.003). Spontaneous breathing during sleep after long-term treatment was markedly improved although still abnormal. During NREM sleep without ventilatory support, oxygen desaturation was significantly less severe compared to the initial study (arterial oxygen saturation (Sa,O2) 88 +/- 4 vs 78 +/- 8%; p < 0.001). Minimum Sa,O2 during REM sleep similarly improved from a mean value of 49 +/- 14% during the diagnostic night to 73 +/- 10% at review follow-up (p < 0.001). In 12 patients, transcutaneous carbon dioxide was measured continuously during sleep on both occasions and demonstrated significantly less CO2 retention during follow-up compared to control studies both in NREM (p < 0.003) and REM sleep states (p < 0.004). Long-term nocturnal ventilation produces improved respiratory drive both asleep and awake and improved arousal responses to abnormal blood gases.

Nasal versus full face mask for noninvasive ventilation in chronic respiratory failure.

This study was undertaken to determine the efficacy of nasal mask (NM) versus full face mask (FFM) for the delivery of noninvasive ventilation (NIV) in subjects with nocturnal hypoventilation. A total of 16 patients (11 males) were enrolled, all with nocturnal hypoventilation currently treated at home with NIV via pressure preset devices. Subjects underwent full polysomnography on three occasions; on the first night current therapy on NM was reviewed, followed by two experimental studies in randomised order using either NM or FFM. NIV settings and oxygen flow rate were the same under both conditions. Notably, 14 of the 16 subjects required the use of a chinstrap to minimise oral leak. Apnoea-hypopnoea indices were within normal limits under both conditions (1.7 +/- 3.4 NM versus 1.6 +/- 2.4 h FFM). The type of interface did not significantly affect gas exchange during sleep (minimum average arterial oxyhaemoglobin saturation total sleep time 93.4 +/- 2.1 NM versus 92.8 +/- 2.5% FFM, Delta transcutaneous carbon dioxide nonrapid eye movement sleep to rapid eye movement sleep (0.58 +/- 0.36 NM versus 0.50 +/- 0.40 kPa FFM). Sleep efficiency was significantly reduced on the FFM (78 +/- 9 NM versus 70 +/- 14% FFM), although arousal indices were comparable under both conditions (15.6 +/- 9.8 NM versus 15.8 +/- 8.8 h FFM). Full face masks appear to be as effective as nasal masks in the delivery of noninvasive ventilation to patients with nocturnal hypoventilation. However, a chinstrap was required to reduce oral leak in the majority of subjects using the nasal mask.

Low-flow oxygen and bilevel ventilatory support: effects on ventilation during sleep in cystic fibrosis.

We measured ventilation in all sleep stages in patients with cystic fibrosis (CF) and moderate to severe lung disease, and compared the effects of low-flow oxygen (LFO2) and bilevel ventilatory support (BVS) on ventilation and gas exchange during sleep. Thirteen subjects, age 26 +/- 5.9 yr (mean +/- 1 SD), body mass index (BMI) 20 +/- 3 kg/m2, FEV1 32 +/- 11% predicted, underwent three sleep studies breathing, in random order, room air (RA), LFO2, and BVS +/- O2 with recording of oxyhemoglobin saturation (SpO2) (%) and transcutaneous carbon dioxide (TcCO2) (mm Hg). During RA and LFO2 studies, patients wore a nasal mask with a baseline continuous positive airway pressure (CPAP) of 4 to 5 cm H2O. Minute ventilation (V I) was measured using a pneumotachograph in the circuit and was not different between wake and non-rapid eye movement (NREM) sleep on any night. However, V I was reduced on the RA and LFO2 nights from awake to rapid eye movement (REM) (p < 0.01) and from NREM to REM (p < 0.01). On the BVS night there was no significant difference in V I between NREM and REM sleep. Both BVS and LFO2 improved nocturnal SpO2, especially during REM sleep (p < 0.05). The rise in TcCO2 seen with REM sleep with both RA and LFO2 was attenuated with BVS (p < 0.05). We conclude that BVS leads to improvements in alveolar ventilation during sleep in this patient group.

Noninvasive pressure preset ventilation for the treatment of Cheyne-Stokes respiration during sleep.

Cheyne-Stokes respiration (CSR) during sleep is common in patients with congestive heart failure (CHF). This pattern of breathing fragments sleep, leading to daytime symptoms of sleepiness and fatigue. It was hypothesized that by controlling CSR with noninvasive pressure preset ventilation (NPPV), there would be a decrease in sleep fragmentation and an improvement in sleep quality. Nine patients (eight males, one female; mean +/- SD 65 +/- 11 yrs) with symptomatic CSR diagnosed on overnight polysomnography (apnoea/hypopnoea index (AHI) 49 +/- 10 x h(-1), minimum arterial oxygen saturation (Sa,O2, 77 +/- 7%) and CHF (left ventricular ejection fraction 25 +/- 8%) were studied. After a period of acclimatization to NPPV (variable positive airway pressure (VPAP) II ST, Sydney, NSW, Australia and bilevel positive airway pressure (BiPAP), Murraysville, PA, USA), sleep studies were repeated on therapy. NPPV almost completely abolished CSR in all patients with a reduction in AHI from 49 +/- 10 to 6 +/- 5 x h(-1) (p<0.001). Residual respiratory events were primarily due to upper airway obstruction at sleep on-set. Arousal index was markedly decreased from 42 +/- 6 to 17 +/- 7 x h(-1) (p <0.001). Sleep architecture showed a trend toward improvement with a reduction in stage 1 and 2 (79 +/- 7% during the diagnostic night versus 72 +/- 10% during NPPV, (p=0.057)), whilst sleep efficiency, slow-wave sleep (SWS), and rapid eye movement (REM) were not altered. Controlling Cheyne-Stokes respiration with noninvasive pressure preset ventilation resulted in reduced arousal and improved sleep quality in the patients with congestive heart failure. Noninvasive pressure preset ventilation should be considered a potential therapy for Cheyne-Stokes respiration in congestive heart failure in those patients who do not respond or fail to tolerate nasal continuous positive airway pressure therapy.

Breathing during sleep in patients with nocturnal desaturation.

The mechanisms leading to hypoxemia during sleep in patients with respiratory failure remain poorly understood, with few studies providing a measure of minute ventilation (V I) during sleep. The aim of this study was to measure ventilation during sleep in patients with nocturnal desaturation secondary to different respiratory diseases. The 26 patients studied had diagnoses of chronic obstructive pulmonary disease (COPD) (n = 9), cystic fibrosis (CF) (n = 2), neuromusculoskeletal disease (n = 4), and obesity hypoventilation syndrome (OHS) (n = 11). Also reported are the results for seven normal subjects and seven patients with effectively treated obstructive sleep apnea (OSA) without desaturation during sleep. Ventilation was measured with a pneumotachograph attached to a nasal mask. In the treated patients with OSA and in the normal subjects, only minor alterations in V I were observed during sleep. In contrast, mean V I for the group with nocturnal desaturation decreased by 21% during non-rapid-eye-movement (NREM) sleep and by 39% during rapid-eye-movement (REM) sleep as compared with wakefulness. This reduction was due mainly to a decrease in tidal volume (V T). Hypoventilation was most pronounced during REM sleep, irrespective of the underlying disease. These data indicate that hypoventilation may be the major factor leading to hypoxia during sleep, and that reversal of hypoventilation during sleep should be a major therapeutic strategy for these patients.