背景:囊性纤维化(CF)是一种多系统疾病,通常需要耳鼻喉科护理。患有CF的个体通常患有慢性鼻-鼻窦炎,但也存在听力损失和发音困难。鉴于CF的这些表现,耳鼻喉科医师经常参与CF患者的护理;然而,关于鼻窦的优化管理的共识有限,耳科,和喉症状.
方法:囊性纤维化基金会召集了一个多学科的耳鼻喉科医师小组,肺病学家,听力学家,药剂师,一个社会工作者,护士协调员,呼吸治疗师,两名患有CF的成年人,以及一名患有CF的儿童的照顾者,以制定共识建议。工作组根据系统的文献审查,制定了建议声明草案,接受每份建议声明需要≥80%的共识.
结果:委员会对25项声明进行了投票。通过了11项建议治疗或干预的声明,虽然有5份声明建议反对特定治疗或干预。委员会建议在某些情况下将八项声明作为特定患者的选择,一个声明没有达成共识。
结论:这些多学科共识建议将帮助提供者驾驭与耳鼻喉科会诊相关的决策,CF-CRS的医疗和外科管理,听力,和声音在个人与CF。提倡采用协作和多学科的方法来为CF患者提供最佳护理。未来的临床研究需要利用标准化,经过验证的结果,并全面报告患者结果,调节剂疗法的效果,和遗传特征来帮助继续推进护理,降低发病率,提高CF患者的生活质量。
Cystic fibrosis (CF) is a multisystem disease that often requires otolaryngology care. Individuals with CF commonly have chronic rhinosinusitis but also present with hearing loss and dysphonia. Given these manifestations of CF, otolaryngologists are frequently involved in the care of patients with CF; however, there is limited
consensus on optimal management of sinonasal, otologic, and laryngologic symptoms.
The Cystic Fibrosis Foundation convened a multidisciplinary team of otolaryngologists, pulmonologists, audiologists, pharmacists, a social worker, a nurse coordinator, a respiratory therapist, two adults with CF, and a caregiver of a child with CF to develop
consensus recommendations. Workgroups developed draft recommendation statements based on a systematic literature review, and a ≥80%
consensus was required for acceptance of each recommendation statement.
The committee voted on 25 statements. Eleven statements were adopted recommending a treatment or intervention, while five statements were formulated recommending against a specific treatment or intervention. The committee recommended eight statements as an option for select patients in certain circumstances, and one statement did not reach
consensus.
These multidisciplinary
consensus recommendations will help providers navigate decisions related to otolaryngology consultation, medical and surgical management of CF-CRS, hearing, and voice in individuals with CF. A collaborative and multidisciplinary approach is advocated to best care for our patients with CF. Future clinical research is needed utilizing standardized, validated outcomes with comprehensive reporting of patient outcome, effects of modulator therapies, and genetic characteristics to help continue to advance care, decrease morbidity, and improve the quality of life for individuals with CF.