Effects of Mouthpiece Noninvasive Ventilation on Speech in Men with Muscular Dystrophy: A Pilot Study.

Purpose The use of noninvasive ventilation (NIV) is on the rise as an alternative to tracheostomy for individuals with neuromuscular disorders with life-prolonging and quality-of-life benefits. This pilot study was designed to determine if mouthpiece NIV (M-NIV) alters speech in individuals with muscular dystrophy (MD). Method Eight men (23-44 years), seven with Duchenne MD and one with Becker MD, who used daytime M-NIV, were asked to sustain phonation, count, and read under three conditions: (a) (no instructions), (b) (cued to use M-NIV with all speaking breaths), and (c) (as tolerated). Breath group and inspiratory durations, syllables/breath group, and relative sound pressure level were determined from audio and video recordings. Results Uncued condition: Participants used the ventilator for all inspirations that preceded sustained phonation and counting. During reading, four participants used M-NIV for all inspirations, one never used it, and three used it for some (19%-41%) inspirations. With- versus Without-M-NIV conditions: Breath group duration was significantly longer across all tasks, syllables per breath group were significantly greater during reading, and inspiratory pause duration during reading was significantly longer with M-NIV than without. Sound pressure level was significantly higher during the first second of sustained phonation with M-NIV (though not for counting and reading). Two participants were unable to complete the reading task audibly without using their M-NIV. Conclusions Speech may be better with M-NIV than without because it is possible to produce longer breath groups and some people with severe respiratory muscle weakness may not be able to speak at all without ventilator-supplied air. Nevertheless, the longer inspiratory pauses that accompany M-NIV may interrupt the flow of speech. Future research is needed to determine the most effective way to use M-NIV for speaking and whether training participants in its use can bring even greater speech benefits

Factor Analysis of the Pediatric Symptom Checklist With a Chronically Ill Pediatric Population

The psychometric properties and factor structure of a widely used screening measure for behavioral and emotional dysfunction, the Pediatric Symptom Checklist (PSC), was extended to a population of chronically ill children. Parents of 404 children ranging from 6 to 17 years of age and diagnosed with either insulin-dependent diabetes mellitus (IDDM) or sickle cell disease (SCD) completed the PSC while waiting for a routine medical appointment. The measure\u27s internal consistency was found to be high, Cronbach\u27s alpha = .89, and test-retest reliability across 4 months was observed to be acceptable, r = .77. A principal components analysis with an oblique (promax) rotation yielded a four-factor solution with factors that included items representative of internalizing, externalizing, attention, and chronic illness-related problems, respectively. Cronbach alpha estimates ranged from .78 to .83 for the first three factors but was lower for the chronic illness-related problems factor (Cronbach\u27s alpha = .60). A three-factor solution and reliability estimates were recomputed without the chronic illness items that yielded the same reliability estimates for each of the three factors and for the full scale. The three-factor solution was also found to be similar to a published factor structure obtained with a primary care sample, rc = .90-.91. The findings lend support to extending the PSC\u27s clinical utility to tertiary care pediatric settings. Further research is recommended with a broader range of chronic illness groups to increase generalizability

Experiences of Speaking With Noninvasive Positive Pressure Ventilation: A Qualitative Investigation

Purpose The aim of this study was to describe experiences of speaking with 2 forms of noninvasive positive pressure ventilation (NPPV)—mouthpiece NPPV (M-NPPV) and nasal bilevel positive airway pressure (BPAP)—in people with neuromuscular disorders who depend on NPPV for survival. Method Twelve participants (ages 22−68 years; 10 men, 2 women) with neuromuscular disorders (9 Duchenne muscular dystrophy, 1 Becker muscular dystrophy, 1 postpolio syndrome, and 1 spinal cord injury) took part in semistructured interviews about their speech. All subjects used M-NPPV during the day, and all but 1 used BPAP at night for their ventilation needs. Interviews were audio-recorded, transcribed, and verified. A qualitative descriptive phenomenological approach was used to code and develop themes. Results Three major themes emerged from the interview data: (a) M-NPPV aids speaking (by increasing loudness, utterance duration, clarity, and speaking endurance), (b) M-NPPV interferes with the flow of speaking (due to the need to pause to take a breath, problems with mouthpiece placement, and difficulty in using speech recognition software), and (c) nasal BPAP interferes with speaking (by causing abnormal nasal resonance, muffled speech, mask discomfort, and difficulty in coordinating speaking with ventilator-delivered inspirations). Conclusion These qualitative data from chronic NPPV users suggest that both M-NPPV and nasal BPAP may interfere with speaking but that speech is usually better and speaking is usually easier with M-NPPV. These findings can be explained primarily by the nature of the 2 ventilator delivery systems and their interfaces