Felipe Montiel Almudena, Álvarez Fernández Antonio, Culebras Amigo Mario, Blasi Francesco, Gramegna Andrea, Elborn Stuart, Horsley Alexander, Madge Susan, Stolz Daiana, Tabin Nathalie, Pannetier Carine, Polverino Eva
Department of Respiratory Medicine (Adult Cystic Fibrosis Unit), Vall d'Hebron University Hospital, Vall d'Hebron Research Institute (VHIR), Universitat Autònoma de Barcelona, Barcelona, Spain.
CIBER de Enfermedades Respiratorias, Instituto de Salud Carlos III, Madrid, Spain.
ERJ Open Res. 2024 May 13;10(3). doi: 10.1183/23120541.00065-2024. eCollection 2024 May.
Significant progress in the field of cystic fibrosis (CF) has substantially extended the life expectancy of patients with CF (pwCF). Consequently, the population of adult pwCF has outnumbered paediatric patients in most developed countries. Ageing is a new factor that can contribute to disease complexity and can require adaptation of CF units. Therefore, the necessity for standardised, specialised and multidisciplinary care is imperative. Concerns arise regarding the adequacy of current healthcare, therapeutic and educational offerings.
To address these concerns, a multinational survey was conducted to assess the current state of care in specialised multidisciplinary adult and paediatric CF units and identify areas for improvement. Responses were collected from 44 centres providing regular care to CF patients.
The survey unveiled considerable disparities in the availability of critical resources, including diagnostic access, supplementary testing, treatment modalities, transplant and transition programmes, and healthcare professionals' training.
This study underscores the urgent need to standardise care across these centres in order to minimise disparities in terms of available resources and training with a particular emphasis on adult pwCF who are becoming more numerous and showing different needs with ageing. The changing landscape of CF in adulthood will require constant monitoring to ensure proper adaptation of the current model of care.
囊性纤维化(CF)领域的重大进展显著延长了CF患者(pwCF)的预期寿命。因此,在大多数发达国家,成年pwCF患者的数量已超过儿童患者。老龄化是一个新因素,它会导致疾病复杂性增加,可能需要CF治疗单元进行调整。因此,标准化、专业化和多学科护理的必要性势在必行。人们对当前的医疗保健、治疗和教育服务的充分性产生了担忧。
为了解决这些担忧,开展了一项跨国调查,以评估专门的多学科成人和儿童CF治疗单元的当前护理状况,并确定需要改进的领域。从44个为CF患者提供常规护理的中心收集了反馈意见。
调查揭示了关键资源的可用性存在相当大的差异,包括诊断途径、补充检测、治疗方式、移植和过渡计划以及医疗保健专业人员的培训。
本研究强调迫切需要在这些中心之间实现护理标准化,以尽量减少可用资源和培训方面的差异,尤其要关注数量日益增多且随着年龄增长表现出不同需求的成年pwCF患者。成年期CF情况的不断变化需要持续监测,以确保当前护理模式得到适当调整。