Department of Pediatrics, Boston Medical Center, Boston, Massachusetts, USA.
Department of Pediatrics, Boston University School of Medicine, Boston, Massachusetts, USA.
Pediatr Blood Cancer. 2021 Aug;68(8):e29136. doi: 10.1002/pbc.29136. Epub 2021 Jun 8.
Acute chest syndrome (ACS) is a leading cause of morbidity and mortality among children with sickle cell disease (SCD). Preventing hypoxemia by optimizing lung aeration during sleep remains a challenge.
To explore safety, feasibility, and tolerability of noninvasive, bi-level positive airway pressure ventilation (BiPAP) as preventative, supportive care for hospitalized, medically stable children with SCD on a general pediatric inpatient unit.
Retrospective chart review of patients ≤22 years of age with SCD admitted to the general pediatric inpatient unit from February 1, 2017 to March 1, 2020 for whom BiPAP was recommended as supportive care. Hospitalizations were excluded if patients were admitted to the pediatric intensive care unit (PICU), required BiPAP for respiratory failure, or used BiPAP at home for obstructive sleep apnea.
Twenty-three patients had 53 hospitalizations in which BiPAP was recommended. Fifty-two (98%) hospitalizations included acute SCD pain. Indications for BiPAP included prior ACS (94%), chest or back pain (79%), and/or oxygen desaturation (66%). On 17 occasions, patients already had mild to moderate ACS but were stable when BiPAP was recommended. BiPAP was used successfully during 75% of hospitalizations for a median of two nights. There were no adverse effects associated with BiPAP. PICU transfer for respiratory support occurred during three hospitalizations. In 26 hospitalizations of children at risk for ACS who tolerated BiPAP, 23 (88%) did not develop ACS.
BiPAP is safe, feasible, and well tolerated as supportive care for hospitalized children with SCD. Next steps include an intervention trial to further assess the efficacy of BiPAP on ACS prevention.
急性胸部综合征（ACS）是镰状细胞病（SCD）儿童发病率和死亡率的主要原因。通过优化睡眠期间的肺通气来预防低氧血症仍然是一个挑战。
探索无创双水平气道正压通气（BiPAP）作为预防性支持治疗在普通儿科住院病房中患有 SCD 的住院、病情稳定的儿童的安全性、可行性和耐受性。
回顾性分析 2017 年 2 月 1 日至 2020 年 3 月 1 日期间因 BiPAP 作为支持性治疗而被推荐在普通儿科住院病房住院的≤22 岁 SCD 患者的病历。排除患者因呼吸衰竭入住儿科重症监护病房（PICU）、需要 BiPAP 或在家中使用 BiPAP 治疗阻塞性睡眠呼吸暂停的住院病例。
23 例患者共 53 次住院，其中推荐使用 BiPAP。52 次（98%）住院治疗包括急性 SCD 疼痛。BiPAP 的适应证包括既往 ACS（94%）、胸痛或背痛（79%）和/或氧饱和度降低（66%）。在推荐使用 BiPAP 时，有 17 例患者已经出现轻度至中度 ACS，但病情稳定。BiPAP 在 75%的住院治疗中成功使用，中位数为两个晚上。BiPAP 无不良反应。因呼吸支持需要转入 PICU 的情况发生在 3 次住院期间。在 26 次有 ACS 风险且能耐受 BiPAP 的儿童住院治疗中，23 例（88%）未发生 ACS。
BiPAP 作为 SCD 住院儿童的支持性治疗是安全、可行且耐受性良好的。下一步包括干预试验，以进一步评估 BiPAP 在预防 ACS 方面的疗效。
