Derivation of the top 10 research priorities#

Consensus priorities (parent/patient and health practitioners) and confirmed with two parent advisory groupsParent/patient survey (verbatim from survey questions)Health practitioner survey (verbatim from survey questions)
Theme: Understanding mechanisms and biology of bronchiectasis
 Identifying risk and protective factors for  bronchiectasisIdentifying what makes some patients’ bronchiectasis get worseHow best to prevent development of bronchiectasis?
Finding ways to prevent bronchiectasis
 Identifying the underlying aetiologies of  bronchiectasisIdentifying the cause(s) of bronchiectasisWhat are the baseline investigations to identify underlying aetiologies of bronchiectasis?
Theme: Diagnosis
 Discovering ways to diagnose  bronchiectasis earlier, including ways  to increase health practitioner  awareness and to facilitate earlier  referralsDiscovering ways to diagnose bronchiectasis earlier, such as by local doctors
Theme: Improving knowledge and treatment of exacerbations
 Identifying triggers/prevention factors  and optimal antibiotic treatment for  acute exacerbationsIdentifying triggers for an exacerbationWhat are the most important factors at preventing acute exacerbations?
Exploring the link between getting a cold (for example rhinovirus) and having an exacerbationWhat is the optimal antibiotic therapy (dosage, how many antibiotics, type, oral versus intravenous versus inhaled/nebulised and length of therapy) for an exacerbation of bronchiectasis?
Using vaccines to prevent exacerbations
Theme: Finding new ways to improve treatment
 Finding new and optimal airway  clearance techniquesFinding new physiotherapy/airway clearance techniquesWhat are the optimal and most cost-effective airway clearance techniques?
When should airway clearance techniques be started in patients with bronchiectasis, and how often should it be done during the stable state and for exacerbations?
 Defining optimal antibiotic therapy for  eradicating specific pathogens (e.g.  Pseudomonas aeruginosa) and for  suppressing bacteria once chronic  infection is establishedUsing longer-term antibiotic therapy when a person's condition is stable+When and how (antibiotic, dose, regimen, route (intravenous, oral or inhaled/nebulised) and duration) should P. aeruginosa be eradicated, and do patient outcomes improve afterwards?
When and how (antibiotic, dose, regimen, route (intravenous, oral or inhaled/nebulised) and duration) should pathogens other than P. aeruginosa be eradicated, and do patient outcomes improve afterwards?
What are the indications of oral versus inhaled/nebulised long-term suppressive antibiotic treatment?
What is the best antibiotic, dose, regimen and duration for long-term oral antibiotic therapy in patients with bronchiectasis (according to the presence or absence of P. aeruginosa or other pathogens)?
 Finding new medications and/or  techniques for managing  bronchiectasisTesting new techniques for managing bronchiectasis in real world environments, such as at home and communityWhat is the role of different mucoactive agents (e.g. inhaled hypertonic or isotonic saline, mannitol, oral erdosteine or N-acetyl cysteine)?
Finding new medicines to treat bronchiectasis
Theme: Improving monitoring and how to identify predictors of disease progression
 Identifying lung function tests/indices  that predict outcomesHaving regular lung function testing to help notice changes or increased risk of an exacerbationWhat are the best and most pragmatic functional tests (such as carbon monoxide diffusing capacity, 6-min walk test, lung clearance index, endurance shuttle walk, incremental exercise tests or accelerometers) as markers for severity of the disease, outcomes and end-points for the clinic?
 Understanding the relationship between  causes and comorbidities of  bronchiectasis with clinical outcomesTo know how bronchiectasis affects other body parts/organs in addition to the lungDo different aetiologies and/or comorbidities of bronchiectasis predetermine microbiological characteristics, and affect severity, patients’ quality of life and disease progression?
To understand the relationship between bronchiectasis and other medical conditions e.g. asthma, “acid” reflux
 Identifying factors associated with worse  bronchiectasis outcomesIdentifying what makes some patients’ bronchiectasis get worseWhat are the risk factors and causes of rapid progression of lung disease and poor outcomes (e.g. hospitalisation, lung transplantation and mortality)?
Identifying people at increased risk of poor outcomes or needing urgent treatment for their bronchiectasisWhat are the risk or protective factors for lung function decline in patients with bronchiectasis?
  • #: the list is not in order of priority, i.e. all are considered equal. : refers to parent's/patient's experience of delayed referral due to lack of awareness of the symptoms of bronchiectasis and dismissing children's chronic wet cough; we do not expect primary care doctors to undertake computed tomography scans in young children. +: refers to identifying when and in whom long-term antibiotics should be used to induce clinical stability.