Derivation of the top 10 research priorities#
| Consensus priorities (parent/patient and health practitioners) and confirmed with two parent advisory groups | Parent/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 bronchiectasis | Identifying what makes some patients’ bronchiectasis get worse | How best to prevent development of bronchiectasis? |
| Finding ways to prevent bronchiectasis | ||
| Identifying the underlying aetiologies of bronchiectasis | Identifying the cause(s) of bronchiectasis | What 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 referrals | Discovering 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 exacerbations | Identifying triggers for an exacerbation | What are the most important factors at preventing acute exacerbations? |
| Exploring the link between getting a cold (for example rhinovirus) and having an exacerbation | What 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 techniques | Finding new physiotherapy/airway clearance techniques | What 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 established | Using 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 bronchiectasis | Testing new techniques for managing bronchiectasis in real world environments, such as at home and community | What 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 outcomes | Having regular lung function testing to help notice changes or increased risk of an exacerbation | What 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 outcomes | To know how bronchiectasis affects other body parts/organs in addition to the lung | Do 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 outcomes | Identifying what makes some patients’ bronchiectasis get worse | What 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 bronchiectasis | What 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.