Relation between corset use and lung function postural variation in spinal cord injury.

Corsets are widely used to improve trunk stability in patients with spinal cord injury (SCI) and can improve respiratory function. The aim of the present study was to identify predictors of respiratory benefits from wearing a corset in SCI patients. In a prospective observational study, respiratory function was tested in the supine and upright seated position with and without a corset in 36 SCI patients who regularly used a corset. SCI patients who no longer used a corset were matched to users on sex, injury level and severity. Vital capacity (VC) did not differ between users and nonusers in the supine position. In users, contrary to nonusers, VC increased significantly in the supine position compared to the seated position (increase of 0.43+/-0.39 versus -0.05+/-0.32 L in nonusers; p<0.0001). Corset use was associated with a significant VC increase in the upright position (2.13+/-0.71 L without versus 2.41+/-0.69 L with the corset; p<0.001). The VC increase with the corset in the upright position correlated significantly with the VC increase induced by being supine, compared to sitting without the corset. The VC increase induced in SCI patients by lying supine may predict the effects of wearing a corset. The long-term effects of corset use should be evaluated.

Measuring inspiratory muscle strength in neuromuscular disease: one test or two?

Inspiratory muscle strength monitoring is crucial in patients with neuromuscular disorders. The sniff nasal inspiratory pressure (SNIP) and maximal inspiratory pressure (P(I,max)) are usually measured. The present study investigated whether the test yielding the best value at baseline continued to yield the best value during follow-up. The present study included 25 patients with Duchenne muscular dystrophy (DMD) and 61 with myotonic muscular dystrophy (MMD). SNIP and P(I,max) were measured at baseline and then annually. At baseline, SNIP was lower than P(I,max) in 20 (80%) DMD patients and 32 (52%) MMD patients. During follow-up in DMD patients, changes in the best method always occurred from SNIP to P(I,max). In MMD patients, when SNIP was better than P(I,max) at baseline, SNIP was usually (88%) better during follow-up, whereas a better P(I,max) than SNIP at baseline was frequently (50%) followed by a shift to SNIP. Maximal inspiratory pressure may be sufficient for monitoring inspiratory muscle function in Duchenne muscular dystrophy adults. In myotonic muscular dystrophy, the marked variability in the test yielding the best value at baseline indicates a need for performance of both tests at baseline. However, when sniff nasal inspiratory pressure measurement yields the best value at baseline, using sniff nasal inspiratory pressure alone during follow-up may be appropriate.

Characteristics of tracheostomy phonation valves.

Phonation valves are commonly used devices that allow the restoration of speech in tracheostomised patients. However, their use should not compromise the physiological benefit of tracheostomy. Six commercialised phonation valves were studied in a dynamic set-up simulating a respiratory frequency of 20 breaths.min(-1), a tidal volume of 0.5 L and a peak flow rate of 0.5 L.s(-1). Resistance and additional work of breathing (WOB) were calculated. In 10 tracheostomised patients, evaluations using no phonation valve (baseline), and the most and one of the least resistive valves were carried out. Respiratory patterns and gas exchanges were recorded. Inspiratory difficulty was evaluated using the modified Borg scale. Valves displayed a wide array of resistance ranging 1.3-5.9 cmH2O.L(-1).s(-1). Additional WOB varied with a ratio of 4.4 between the best and the worst valve. While the different clinical conditions did not modify respiratory patterns and gas exchanges, a significant effect on the Borg scale rating was observed using ANOVA and post hoc analysis of baseline versus worst valve and one of the best valves versus worst valve. In conclusion, the variety of aerodynamic characteristics of phonation valves should be considered when choosing the device, according to the underlying condition of the patients benefiting from their use.

Sniff nasal inspiratory pressure: what is the optimal number of sniffs?

Sniff nasal inspiratory pressure (SNIP) measurement is a volitional noninvasive assessment of inspiratory muscle strength. A maximum of 10 sniffs is generally used. The purpose of the present study was to investigate whether the maximum SNIP improved after the tenth sniff. In total, 20 healthy volunteers and 305 patients with various neuromuscular and lung diseases were encouraged to perform 40 and 20 sniffs, respectively. The best SNIP among the first 10 sniffs was lower than the best SNIP among the next 10 sniffs in the healthy volunteers and patients. The SNIP improvement after the twentieth sniff was marginal. In conclusion, a learning effect persists after the tenth sniff. The current authors suggest using 10 additional sniffs when the best result of the first 10 sniffs is slightly below normal, or when sniff nasal inspiratory pressure is used to monitor a progressive decline in inspiratory muscle strength.

Resting energy expenditure in Duchenne patients using home mechanical ventilation.

Nutritional status is both important and difficult to assess in patients with Duchenne muscular dystrophy (DMD), particularly in those requiring mechanical ventilation (MV). The current authors evaluated body composition (bio-impedancemetry), resting energy expenditure (REE; indirect calorimetry) and energy intake in 20 adult patients with DMD using home MV (nocturnal: n = 13; continuous: n = 7) and 12 age-matched healthy controls. The patients were smaller in height than the controls and had a lower body weight. Most of the reduction in body mass index was accounted for by a reduction in fat free mass (FFM). REE (kJ) was significantly reduced in the patients (4559+/-853 kJ x 24 h(-1) versus 7407+/-1312 kJ x 24 h(-1)), but the difference disappeared after correction for FFM. REE and FFM were correlated in both the controls and patients, but less strongly in the latter, the lower strength of the association being due to the patients using continuous MV (REE and FFM uncorrelated). The food intake of the patients was 1.2+/-0.4 greater than their REE. This study shows that patients with advanced forms of Duchenne muscular dystrophy have balanced energy intakes and resting energy expenditure.

Limitations of sniff nasal pressure in patients with severe neuromuscular weakness.

BACKGROUND: Inspiratory muscle strength in patients with neuromuscular disorders can be assessed using sniff inspiratory nasal pressure (Pn(sn)) and maximum inspiratory mouth pressure (PI(max)). However, the relative merits of Pn(sn) against PI(max) are not known in patients with severe neuromuscular disease. OBJECTIVE: To investigate whether severity of disease modifies the relation between Pn(sn) and PI(max). METHODS: Vital capacity (VC), Pn(sn), and PI(max) were measured in 258 patients with neuromuscular disorders. RESULTS: Data were analysed from 241 patients, 17 being unable to perform PI(max) or Pn(sn) manoeuvres. The correlation between Pn(sn) and PI(max) was +0.94 (p<0.0001), with a mean (SD) difference between Pn(sn) and PI(max) of -4.8 (21.2) cm H(2)O (the limits of agreement were 37.6 and -47.2 cm H(2)O). VC (% predicted) was positively correlated with Pn(sn)/PI(max) (r = +0.86; p<0.0001), with a lower Pn(sn)/PI(max) value in patients with a VC <40% of predicted than in those with a VC >40% (0.80 (0.35) v 1.04 (0.41); p<0.0001). CONCLUSIONS: PI(max) is greater than Pn(sn) in patients with a severe restrictive ventilatory defect caused by neuromuscular disease. Pn(sn) may not accurately reflect inspiratory muscle strength in such patients and it is thus advisable to use both tests.

Supine fall in lung volumes in the assessment of diaphragmatic weakness in neuromuscular disorders.

OBJECTIVE: To determine whether diaphragmatic function can be determined by noninvasive respiratory indices in neuromuscular disease. DESIGN: Vital capacity (VC) and mouth pressure generated during a maximal static inspiratory effort (Pi max) were measured with patients in both sitting and supine positions. SETTING: Rehabilitation hospital. PATIENTS: Twenty-four patients with generalized neuromuscular disease. MAIN OUTCOME MEASURES: Changes in indices from sitting to supine position were compared with invasive diaphragmatic function indices consisting of transdiaphragmatic pressures during maximal sniff (Pdi sniff) and the ratio of gastric pressure (Pga) increases over transdiaphragmatic pressure (DeltaPga/DeltaPdi) during quiet breathing. RESULTS: The fall in VC in the supine position was greater in the 15 patients who had spontaneous paradoxical diaphragmatic motion (DeltaPga/DeltaPdi < 0) than in the 9 patients who did not. Specificity and sensitivity of a greater than 25% supine fall in VC for the diagnosis of diaphragmatic weakness (DeltaPga/DeltaPdi < 0 and/or Pdi sniff < 30cmH2O) were 90% and 79%, respectively. Stepwise multiple regression analysis of Pdi sniff showed that both the supine fall in VC and Pi max were associated with diaphragmatic weakness (R(2) =.66; p <.0001). These factors contributed 52% and 14% of the Pdi sniff variance, respectively. CONCLUSIONS: Simple VC measurement in the sitting and supine positions may be helpful in detecting severe or predominant diaphragmatic weakness.