Department of Physical Medicine and Rehabilitation, University Hospital, New Jersey Medical School, Newark, New Jersey 07103, USA.
Respir Care. 2011 Jun;56(6):744-50. doi: 10.4187/respcare.00831. Epub 2011 Feb 11.
To describe survival outcomes with noninvasive ventilation (NIV) for full ventilatory support, and a mechanically assisted cough and oximetry protocol in a series of patients with Duchenne muscular dystrophy.
We monitored end-tidal carbon dioxide (P(ETCO(2))), S(pO(2)), vital capacity, maximum insufflation capacity, and cough peak flow. Nocturnal NIV was initiated for symptomatic hypoventilation. An oximeter and mechanically assisted cough device were prescribed when the patient's maximum assisted cough peak flow fell below 300 L/min. Patients used up to continuous NIV and mechanically assisted cough to return S(pO(2)) to ≥ 95% during intercurrent respiratory infections or as otherwise needed. We recorded respiratory and cardiac hospitalizations and mortality, and quantified survival by duration of continuous NIV dependence (ie, unable to maintain oxygenation without the ventilator).
With advancing Duchenne muscular dystrophy, 101 nocturnal-only NIV users extended their NIV use throughout the daytime hours and required it continuously for 7.4 ± 6.1 years to 30.1 ± 6.1 years of age, with 56 patients still alive. Twenty-six of the 101 became continuously dependent without requiring hospitalization. Eight tracheostomized users were decannulated to NIV. Thirty-one consecutive unweanable intubated patients were extubated to NIV plus mechanically assisted cough. Of the 67 deaths (including 8 patients who died from heart failure before requiring ventilator use), 34 (52%) were probably cardiac, 14 (21%) were probably respiratory, and 19 (27%) were of unknown or other etiology.
Continuous NIV along with mechanically assisted cough and oximetry as needed can prolong life and obviate tracheotomy in patients with Duchenne muscular dystrophy. Unweanable patients can be decannulated and extubated to NIV plus mechanically assisted cough.
描述在一系列杜氏肌营养不良症患者中，使用无创通气（NIV）进行全面通气支持，以及机械辅助咳嗽和血氧测定协议的生存结果。
我们监测了呼气末二氧化碳（P（ETCO（2）））、血氧饱和度（S（pO（2）））、肺活量、最大充气容量和咳嗽峰值流量。对于有症状性通气不足的患者，开始进行夜间 NIV。当患者的最大辅助咳嗽峰值流量下降到 300 L/min 以下时，开处血氧计和机械辅助咳嗽装置。患者在并发呼吸道感染或其他需要时，使用连续 NIV 和机械辅助咳嗽，以使 S（pO（2））恢复到≥95%。我们记录了呼吸和心脏住院和死亡情况，并通过持续接受 NIV 依赖的时间（即没有呼吸机就无法维持氧合）来量化生存。
随着杜氏肌营养不良症的进展，101 名仅夜间使用 NIV 的患者将其 NIV 使用时间延长到白天，并需要持续使用 7.4±6.1 年，直到 30.1±6.1 岁，其中 56 名患者仍然存活。101 名患者中有 26 名患者成为持续依赖者，无需住院治疗。8 名气管切开术患者被拔管到 NIV。31 名连续无法脱机的插管患者被拔管到 NIV 加机械辅助咳嗽。在 67 例死亡患者中（包括 8 例因心力衰竭在需要使用呼吸机前死亡的患者），34 例（52%）可能是心脏原因，14 例（21%）可能是呼吸原因，19 例（27%）原因不明或其他原因。
持续的 NIV 加上按需使用机械辅助咳嗽和血氧测定可以延长杜氏肌营养不良症患者的生命，并避免气管切开术。无法脱机的患者可以拔管到 NIV 加机械辅助咳嗽。
