N. Mustfa

The effect of noninvasive ventilation on ALS patients and their caregivers

Background: Noninvasive ventilation (NIV) reduces mortality and improves some aspects of quality of life (QoL) in ALS. However, concerns remain that progressive disability may negate these benefits and unnecessarily burden caregivers. Methods: Thirty-nine patients requiring NIV were offered treatment. Twenty-six were established on NIV, but 13 declined or could not tolerate NIV. Fifteen patients without respiratory muscle weakness (RMW) but with similar ALS severity and age were studied in parallel. Caregivers of 21 NIV, 7 untreated, and 10 patients without RMW participated. Patients and caregivers had detailed QoL measurements for 12 months. NIV patients underwent cognitive testing before and after treatment. Results: RMW correlated with lower QoL. The median survival of untreated patients (18 days; 95% CI 11 to 25 days) was shorter than for NIV patients (298 days; 95% CI 192 to 404 days) and non-RMW patients (370 days; 95% CI 278 to 462 days; log rank test [2 df] = 81, p = 0.00001). A wide range of QoL measures improved within 1 month of starting NIV, and improvements were maintained for 12 months. QoL of non-RMW patients declined as RMW progressed. Caregivers of NIV and non-RMW patients showed similar increases in burden, but NIV patient caregivers developed a deterioration in the Short Form-36 Vitality score. No improvements were found on measures of learning and recall in the NIV patients. Conclusions: Respiratory muscle weakness has a greater impact on quality of life (QoL) than overall ALS severity. Noninvasive ventilation (NIV) improves QoL despite ALS progression. NIV has no impact on most aspects of caregiver QoL and does not significantly increase caregiver burden or stress.

Cough augmentation in amyotrophic lateral sclerosis

Cough flows and pressures were measured during cough augmentation in healthy subjects and patients with bulbar and nonbulbar amyotrophic lateral sclerosis. Manual assistance increased flow 11% in bulbar (p < 0.01) and 13% in nonbulbar (p < 0.001) patients. Mechanical insufflation-exsufflation increased flow 17% in healthy subjects (p < 0.05), 26% (p < 0.001) in bulbar, and 28% (p < 0.001) in nonbulbar patients. The greatest improvements were in patients with the weakest coughs. Patient group and level of weakness influenced the effect of augmentation.

Postprandial effects on twitch transdiaphragmatic pressure

Twitch transdiaphragmatic pressure (Pdi,tw), measured following magnetic stimulation of the phrenic nerves, is used to assess diaphragm strength, contractility and fatigue. Although the effects of posture, lung volume and potentiation on Pdi,tw are well described, it is not known whether the degree of gastric filling affects the measurement. Pdi,tw was recorded in seven healthy volunteers on two occasions with antero-lateral magnetic stimulation of the phrenic nerves. On the first occasion, the subjects had fasted for at least 8 h, whilst on the second occasion, measurements were made after each subject had eaten a substantial meal sufficient to produce a feeling of satiation. Mean postprandial unpotentiated and potentiated Pdi,tw were significantly greater than corresponding fasting Pdi,tw in all seven volunteers (29.8 versus 25.7 cmH2O and 38.9 versus 34.4 cmH2O, respectively). This was due to a significantly increased gastric pressure component (1.10 versus 0.84 and 0.94 versus 0.78, respectively), and reduced abdominal compliance (36 versus 62 mL·cmH2O−1). Twitch oesophageal pressure was preserved (15.0 versus 15.4 cmH2O). The postprandial state increases twitch transdiaphragmatic pressure, and this should be taken into account when using twitch transdiaphragmatic pressure to follow-up patients or to assess the effects of interventions on diaphragm contractility.

Diaphragm electromyograms recorded from multiple surface electrodes following magnetic stimulation

The diaphragm compound-muscle action potential (CMAPdi), elicited by unilateral magnetic stimulation (UMS) of the phrenic nerve can be recorded using surface electrodes. However, there is no consensus on the best positioning of surface electrodes and there are no data on the reproducibility of the signal. Using 36 surface electrode pairs, in five healthy subjects, the CMAPdi elicited by UMS and electrical stimulation (ES) were compared and 12 pairs were identified as providing acceptable signals. The latency and amplitude were measured for each CMAPdi, following UMS at 60–100% of maximal stimulator output, in 12 healthy subjects, on two occasions. Latencies obtained using UMS and ES ranged between 6.1–7.33 and 6.25–7.17 ms, respectively. Optimum CMAPdi were not recorded from the same electrode pair in all subjects, or for both hemidiaphragms in each subject. However, the optimal recording site for a particular individual remained unchanged on subsequent testing. When recorded from the optimal site, latencies and amplitudes of CMAPdi elicited on the two occasions were not significantly different. The current study suggests that the use of multiple chest wall electrodes can identify an optimal electrode pair, from which it is possible to obtain reproducible compound-muscle action potential signals.

Evaluation of the effectiveness of a home-based inspiratory muscle training programme in patients with chronic obstructive pulmonary disease using multiple inspiratory muscle tests

Abstract Purpose: To evaluate the effectiveness of a home-based inspiratory muscle training (IMT) programme using multiple inspiratory muscle tests. Method: Sixty-eight patients (37 M) with moderate to severe chronic obstructive pulmonary disease (COPD) (Mean [SD], FEV1 36.1 [13.6]% pred.; FEV1/FVC 35.7 [11.2]%) were randomised into an experimental or control group and trained with a threshold loading device at intensity >30% maximum inspiratory pressure (PImax) or <15% PImax, respectively, for 7 weeks. Thirty-nine patients (23 M) completed the study. The following measures were assessed pre- and post-IMT: PImax, sniff inspiratory nasal pressure (SNIP), diaphragm contractility (Pdi,tw), incremental shuttle walk test (ISWT), respiratory muscle endurance (RME), chronic respiratory disease questionnaire (CRDQ), the hospital anxiety and depression scale (HADS) and the SF-36. Between-group changes were assessed using one-way analysis of variance (ANOVA). Results: PImax and perception of well-being improved significantly post-IMT [p = 0.04 and <0.05 in four domains, respectively]. This was not reflected in SNIP [p = 0.7], Pdi,tw [p = 0.8], RME [p = 0.9] or ISWT [p = 0.5]. Conclusions: A seven-week, community-based IMT programme, with realistic use of health-care resources, improves PImax and perception of well-being but a different design may be required for improvement in other measures. Multiple tests provide a more comprehensive evaluation of changes in muscle function post-IMT. Implications for Rehabilitation A seven-week, home-based inspiratory muscle training programme improves maximal inspiratory pressure and perception of well-being in patients with moderate to severe COPD but not sniff nasal inspiratory pressure or diaphragm contractility, respiratory muscle endurance and exercise capacity. Multiple tests are recommended for a more comprehensive assessment of changes in muscle function following inspiratory muscle training programmes. Therapists need to explore different community-based inspiratory muscle training regimes for COPD patients and identify the optimal exercise protocol that is likely to lead to improvements in diaphragm contractility and exercise capacity.

Sniff Nasal Inspiratory Pressure in Patients with Moderate-to-Severe Chronic Obstructive Pulmonary Disease: Learning Effect and Short-Term Between-Session Repeatability

Background: Sniff nasal inspiratory pressure (SNIP) is a non-invasive measure of inspiratory muscle function often used as an outcome measure in clinical studies. An initial period of familiarisation with the test is recommended to minimise the learning effect. The repeatability of SNIP in patients with chronic obstructive pulmonary disease (COPD) is currently unknown. Objectives: The aim of this study was to assess the between-session repeatability of SNIP over a 3-week period in moderate-to-severe COPD patients and compare it with that of maximal inspiratory (PImax) and expiratory pressure (PEmax). Methods: Twenty-one patients (13 males) with a mean forced expiratory volume in 1 s (FEV1) of 38% of predicted (SD: 15) and FEV1/forced vital capacity of 34.3% (SD: 10.4) performed SNIP and PImax and PEmax manoeuvres on 3 different sessions (S1, S2 and S3) 3-7 days apart. SNIP was performed at functional residual capacity (FRC), and PImax was performed at FRC and at residual volume (RV) to explore volume-dependent differences in the learning effect between sessions and PEmax from total lung capacity. Results: The intra-class correlation coefficient (ICC) for SNIP was the highest of the three measures: S1-S3 ICC (95% CI) SNIP: 0.96 (0.88-0.94); PImax at FRC 0.82 (0.63-0.92); PImax at RV: 0.89 (0.78-0.95), and PEmax: 0.96 (0.92-0.98), and had the lowest mean change between sessions [mean S2 - S1: 2.1(p = 0.4) and S3 - S2: -0.3 (p = 0.9)]. Conclusions: SNIP is repeatable over a period of 3 weeks in medically stable, moderate-to-severe COPD patients. In our study, 2 sessions were adequate to learn how to perform the test.