Department of Neurology, Radboud University Medical Center, Nijmegen, The Netherlands.
Department of Rehabilitation, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands.
J Neuromuscul Dis. 2018;5(4):431-438. doi: 10.3233/JND-180302. Epub 2018 Aug 27.
Non-invasive ventilation (NIV) is an established treatment for respiratory failure in patients with amyotrophic lateral sclerosis (ALS). Several studies have shown room for improvement with regard to respiratory care for ALS patients, including latency of referral. These studies focused on the time period starting at the moment of referral to a home ventilation service (HVS) onwards. In the current study we performed a nationwide survey to gain insight in the trajectory before referral. We questioned the assessment of respiratory impairment by ALS physicians/care teams, including criteria for referral to an HVS.
We requested 40 ALS care teams in the Netherlands to fill in an online questionnaire on respiratory management in ALS patients.
Thirty-two ALS care teams (80%) responded. Forced vital capacity was the most frequently used test at each outpatient visit (72%) and often served as a criterion (78%) for referral to an HVS. Other respiratory function measurements that were performed less often included peak cough flow (50%), maximum inspiratory/expiratory pressure (31% /28%) and sniff nasal inspiratory pressure (13%). Morning headache was the most frequently questioned complaint (94%), followed by daytime sleepiness (91%). Dyspnoea and orthopnoea were reported by 38% and 59% as important complaints. Out of all patients under the care of the ALS care teams, the mean estimated proportion of patients that was referred to an HVS was 69% (range 20-100%). When physicians refrained from referral, the most often cited reasons were patient's decision to withhold NIV (94%) and cognitive impairment (50%). Sixteen percent of the respondents stated bulbar impairment as a reason to refrain from referral.
Despite findings in previous studies on the superiority of SNIP and PCF as compared to FVC, our study shows that a majority of ALS care teams still prefers to use FVC for the assessment of respiratory dysfunction and for the timing of referral to an HVS. Another finding is that bulbar impairment is not an obstacle for referral for NIV.
无创通气（NIV）是治疗肌萎缩侧索硬化症（ALS）患者呼吸衰竭的一种既定治疗方法。多项研究表明，ALS 患者的呼吸护理仍有改进的空间，包括转诊的潜伏期。这些研究主要集中在从转诊到家庭通气服务（HVS）开始的时间段。在本研究中，我们进行了一项全国性调查，以深入了解转诊前的轨迹。我们询问了 ALS 医生/护理团队对呼吸功能障碍的评估，包括转诊到 HVS 的标准。
我们要求荷兰的 40 个 ALS 护理团队填写一份关于 ALS 患者呼吸管理的在线问卷。
32 个 ALS 护理团队（80%）做出了回应。用力肺活量是每次门诊就诊时最常使用的测试（72%），并且经常作为转诊到 HVS 的标准（78%）。其他较少进行的呼吸功能测量包括峰流速（50%）、最大吸气/呼气压力（31%/28%）和嗅探鼻吸气压力（13%）。晨发性头痛是最常被问到的症状（94%），其次是日间嗜睡（91%）。呼吸困难和端坐呼吸分别有 38%和 59%的患者报告为重要症状。在 ALS 护理团队护理下的所有患者中，平均估计转诊到 HVS 的患者比例为 69%（范围为 20%-100%）。当医生不转诊时，最常提到的原因是患者决定拒绝 NIV（94%）和认知障碍（50%）。6%的受访者表示球部损害是拒绝转诊的原因。
尽管之前的研究表明 SNIP 和 PCF 优于 FVC，但我们的研究表明，大多数 ALS 护理团队仍然更喜欢使用 FVC 来评估呼吸功能障碍，并确定转诊到 HVS 的时机。另一个发现是，球部损害并不是进行 NIV 转诊的障碍。
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