Self-Supporting Nasopharyngeal Airway (ssNPA) Treating Upper Airway Obstruction in Hypotonia

对于持续性阻塞性睡眠呼吸暂停(OSA)，迫切需要安全有效的治疗方案 低张上呼吸道梗阻患者(华奥)。华奥包含脑部等病症 瘫痪、缺氧性脑病、综合征性张力异常、21三体或唐氏综合征，以及神经肌肉疾病，通常具有类似的多部位上呼吸道塌陷模式。OSA的特点是睡眠期间反复出现部分或完全上呼吸道阻塞，并伴有相关的觉醒和/或氧气减饱和。与儿童阻塞性睡眠呼吸暂停综合征的发病率1-5%相比，患有低张状态、脑性瘫痪和21-三体/唐氏综合征的儿童阻塞性睡眠呼吸暂停的发生率要高得多。在接受多导睡眠监测(PSG)以检查睡眠呼吸紊乱症状的低张患者中，25%的患者有中度OSA，56.3%的患者有重度OSA。因此，患者不仅更有可能患有阻塞性睡眠呼吸暂停，而且可能会更加严重。尽管在大多数这些患者中可以看到明显的上呼吸道塌陷，但文献中关于这些情况下阻塞性睡眠呼吸暂停的患病率和严重程度以及治疗效果的数据很少。 尤其是关于多节段梗阻，而且与治疗效果的持续时间有关。目前 现有的治疗方案，从姑息治疗到气管切开术，往往不能完全满足 这些病人。我们的多学科团队已经开发出一种非常有效的非手术鼻咽 在低张患者中表现出良好耐受性的呼吸道支架。 在华奥治疗阻塞性睡眠呼吸暂停综合征是一个极其困难的挑战，部分原因是弥漫性的多层次 阻塞，缺乏肌肉的呼吸道张力。这就产生了许多外科手术入路，除了 气管切开术，很可能失败。尽管腺体扁桃体切除术等手术方式很常见，但大多数儿童 与华奥术后有残留性阻塞性睡眠呼吸暂停综合征，由于残存的解剖和生理异常。 这通常需要尝试用正压(PAP)治疗或考虑 气管切开术。然而，在典型的发育中的儿童中，遵守PAP的比率可能很低，甚至 有低张状态者较低。此外，在呕吐过程中无法摘下口罩可能会造成 有窒息的风险。气管切开术伴随着发病率和死亡率的相关风险， 护理需求、家庭负担、护理需求以及数十万美元的医疗费用。 我们的多学科团队，由耳鼻喉科-头颈外科、睡眠医学和工程学-领导， 设计、开发并成功试行了一种自助式鼻咽通道(SsNPA)装置 这绕过了具有挑战性的、弥漫的、多水平的上呼吸道阻塞。该设备已经展示了最初的 安全性和初步疗效在试验组患者中，重度阻塞性睡眠呼吸暂停。赠款的启动阶段(R61) 将测试该设备的增强版本的可接受性和耐受性。关键是，插入、坚持和 将为R33阶段的准备工作优化合规方案。R33阶段将评估一种动力 观察PSG和生活质量结果的队列。

There is a critical need for safe and effective treatment options for persistent obstructive sleep apnea (OSA) in patients with Hypotonic Upper Airway Obstruction (HUAO). HUAO encompass conditions such as cerebral palsy, hypoxic encephalopathy, syndromic tone anomalies, Trisomy 21 or Down Syndrome, and neuromuscular disorders, and typically share a similar pattern of multisite upper airway collapse. OSA is characterized by recurrent episodes of partial or complete upper airway obstruction during sleep with associated arousals and/ or oxygen desaturations. In contrast to the incidence of OSA among children which is between 1-5%, there is a significantly higher prevalence of OSA among children with hypotonic conditions, cerebral palsy, and Trisomy 21/Down Syndrome. Of hypotonic patients undergoing polysomnography (PSG) for symptoms of sleep disordered breathing, 25% had moderate OSA, and 56.3% had severe OSA. Thus, not only are patients more likely to have OSA, but it is likely to be much more severe. Despite the obvious upper airway collapse seen in most of these patients, there is a paucity of data in literature, both on prevalence and severity of OSA in these conditions, but also on treatment efficacy particularly with regards to multi-level obstruction and moreover, with duration of treatment efficacy. Currently available treatment options, ranging from palliative care to tracheostomy, often fail to fully meet the needs of these patients. Our multidisciplinary team has developed a dramatically effective non-surgical nasopharyngeal airway stent that has demonstrated good tolerability in hypotonic patients. Treatment of OSA in HUAO presents an extremely difficult challenge, in part due to diffuse multilevel obstruction with lack of muscular airway tone. This renders many surgical approaches, other than tracheostomy, likely to fail. Although surgical options such as adenotonsillectomy are common, most children with HUAO have residual OSA after the procedure, due to residual anatomic and physiological abnormalities. This often necessitates attempted treatment with Positive Airway Pressure (PAP) or consideration for tracheostomy. However, the rate of adherence with PAP in typically developing children can be low, and even lower in those with hypotonic conditions. Moreover, inability to remove a mask during an emesis can pose a suffocation risk. Tracheostomy carries associated risks for morbidity and mortality, a substantial addition of care needs, family burden, nursing demands, and hundreds of thousands of dollars of medical costs. Our multidisciplinary team, led by Otolaryngology – Head and Neck Surgery, Sleep Medicine, and Engineering, has designed, developed, and successfully piloted a self-supported nasopharyngeal airway (ssNPA) device that bypasses challenging, diffuse, multilevel upper airway obstruction. The device has demonstrated initial safety and preliminary efficacy in a pilot group of patients, severe OSA. The Start-up phase (R61) of the grant will test an enhanced version of the device for acceptability and tolerability. Critically, insertion, adherence, and compliance protocols will be optimized for preparation of the R33 phase. The R33 phase will assess a powered cohort looking at PSG and quality of life outcomes.