Bach John R
Department of Physical Medicine and Rehabilitation for Rutgers New Jersey Medical School & Center for Ventilator Management Alternatives at University Hospital, Newark, NJ, USA.
Ann Rehabil Med. 2017 Aug;41(4):519-538. doi: 10.5535/arm.2017.41.4.519. Epub 2017 Aug 31.
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.
这篇综述文章描述了对呼吸肌功能障碍进行确定性无创呼吸管理，以消除诉诸气管切开术的必要性。2010年，来自18个国家22个中心的临床医生报告了1623例1型脊髓性肌萎缩症（SMA1）、杜氏肌营养不良症（DMD）和肌萎缩侧索硬化症患者使用无创通气支持（NVS），其中760例需要持续使用（CNVS）。CNVS使他们的生命得以延续超过3000患者年，而无需留置气管造口管。这些中心现已为至少74例连续无法撤机的DMD患者、超过95%依赖CNVS的SMA1患者以及数百名其他患有晚期神经肌肉疾病（NMD）的患者成功拔管，而无需进行气管切开术。两个中心报告在不进行气管切开术的情况下为258例无法撤机的患者拔管成功率达99%。患有肌病或下运动神经元疾病的患者，尽管肺活量很少或无法测量，但通过使用物理医学呼吸肌辅助装置，可通过CNVS进行长达无限期的无创管理。依赖呼吸机的患者可以拔除气管造口管。
