Clinical and research priorities for children and young people with bronchiectasis: an international roadmap

Abstract

The global burden of children and young people (CYP) with bronchiectasis is being recognised increasingly. They experience a poor quality-of-life and recurrent respiratory exacerbations requiring additional treatment, including hospitalisation. However, there are no published data on patient-driven clinical needs and/or research priorities for paediatric bronchiectasis.Parent/patient-driven views are required to understand the clinical needs and research priorities to inform changes that benefit CYP with bronchiectasis and reduce their disease burden. The European Lung Foundation and the European Respiratory Society Task Force for paediatric bronchiectasis created an international roadmap of clinical and research priorities to guide, and as an extension of, the clinical practice guideline.This roadmap was based on two global web-based surveys. The first survey (10 languages) was completed by 225 respondents (parents of CYP with bronchiectasis and adults with bronchiectasis diagnosed in childhood) from 21 countries. The parents/patients’ survey encompassed both clinical and research priorities. The second survey, completed by 258 health practitioners from 54 countries, was limited to research priorities.The two highest clinical needs expressed by parents/patients were: having an action management plan for flare-ups/exacerbations and access to physiotherapists. The two highest health practitioners’ research priorities related to eradication of airway pathogens and optimal airway clearance techniques. Based on both surveys, the top 10 research priorities were derived and unanimous consensus statements were formulated from these priorities.This document addresses parents/patients’ clinical and research priorities from both the parents/patients and clinicians’ perspectives and will help guide research and clinical efforts to improve the lives of people with bronchiectasis.

Burden of Care for Children with Bronchiectasis from Parents/Carers Perspective

“…The ERS and ELF have highlighted the need for an action management plan for respiratory exacerbations amongst the highest clinical needs for parents of children with bronchiectasis [7]. Another top clinical priority of this taskforce document was "good communication between healthcare professionals and each person with bronchiectasis" [7]. As over three quarters of parents had never had a written management plan from their doctor our research further supports this need in another cohort of parents of children with bronchiectasis.…”

“…To date there are no studies evaluating an action management plan in children with bronchiectasis [26]. The ERS and ELF have highlighted the need for an action management plan for respiratory exacerbations amongst the highest clinical needs for parents of children with bronchiectasis [7]. Another top clinical priority of this taskforce document was "good communication between healthcare professionals and each person with bronchiectasis" [7].…”

“…To do so, we need to hear and heed the voices from the patients and parents of children, and it is increasingly appreciated that the knowledge they provide is very valuable in both the clinical and research contexts [6]. Further, the importance of the patient and caregivers' perspective has been identified as imperative in guiding future research [7,8]. Yet there is a relative paucity of data on general concerns/worries of parents/carers (hereafter referred to as parents) of children with bronchiectasis or their current knowledge base, especially relating to respiratory exacerbations.…”

Management of children and adolescents with bronchiectasis: summary of the ERS clinical practice guideline

“…In an international survey undertaken by the European Lung Foundation (ELF), the top clinical needs of parents of children with bronchiectasis included standard treatments afforded to other chronic lung diseases, such as having access to paediatric chest physiotherapy services and being taught ACT and how to use the equipment at home [ 15 ].…”

“…Diagnosing bronchiectasis at an early stage was a top priority for parents of children/adolescents with bronchiectasis and also for adults who had bronchiectasis as a child [ 15 ]. The positive impact of diagnosing bronchiectasis promptly, as described in the CPG's narrative evidence [ 4 ], included improving QoL [ 16 ] and lung function in many cohorts from various aetiologies (including primary immunodeficiency disorders [ 17 ]).…”