This review article describes definitive noninvasive respiratory management of respiratory muscle dysfunction to eliminate need to resort to tracheotomy. In 2010 clinicians from 22 centers in 18 countries reported 1,623 spinal muscular atrophy type 1 (SMA1), Duchenne muscular dystrophy (DMD), and amyotrophic lateral sclerosis users of noninvasive ventilatory support (NVS) of whom 760 required it continuously (CNVS). The CNVS sustained their lives by over 3,000 patient-years without resort to indwelling tracheostomy tubes. These centers have now extubated at least 74 consecutive ventilator unweanable patients with DMD, over 95% of CNVS-dependent patients with SMA1, and hundreds of others with advanced neuromuscular disorders (NMDs) without resort to tracheotomy. Two centers reported a 99% success rate at extubating 258 ventilator unweanable patients without resort to tracheotomy. Patients with myopathic or lower motor neuron disorders can be managed noninvasively by up to CNVS, indefinitely, despite having little or no measurable vital capacity, with the use of physical medicine respiratory muscle aids. Ventilator-dependent patients can be decannulated of their tracheostomy tubes.
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Method: Thirty-one patients with DMD were investigated. The vital capacity (VC), maximum insufflation capacity (MIC), maximal inspiratory (MIP), and expiratory pressure (MEP) were measured. Unassisted peak cough flow (UPCF) and assisted PCF at three different conditions were evaluated.
Results: The mean value of MICs (1,873⁑644 cc) was higher than that of VCs (1,509⁑640 cc). MIP and MEP were 48.8⁑21.4% and 29.5⁑19.5% of predicted normal value respectively. MIP was correlated with UPCFs as well as MEP. All of three assisted cough methods showed significantly higher value than unassisted method (p＜0.01). The manual assisted PCFs at MIC significantly exceeded those produced by manual assisted or PCFs at MIC. The positive correlation between the MIC-VC difference and PCF at MIC-UPCF difference was seen (p＜0.01).
Conclusion: Inspiratory muscle strength and the preservation of pulmonary compliance is important for the development of effective cough as well as expiratory muscle power. Thus, the clinical implication of the inspiratory phase in assisting a cough should be emphasized. (J Korean Acad Rehab Med 2003; 27: 43-48)
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