TABLE 2

Expanded quality standards of care statements

Quality standard statement 1
 Quality statementChildren and adolescents suspected of bronchiectasis have this confirmed by a chest CT scan.
 Quality measure• Structure: evidence of health services ensuring that chest CT scans are interpreted using paediatric criteria with HCPs having access to the full CT scan interpretation.
• Process: proportion of children and adolescents with a clinical diagnosis of bronchiectasis confirmed by multi-detector CT (MDCT) with a high-resolution CT (HRCT) scan using paediatric criteria.
• Numerator: the number of children and adolescents with a clinical diagnosis of bronchiectasis confirmed by MDCT with a HRCT scan and interpreted using paediatric criteria.
• Denominator: the total number of children and adolescents clinically diagnosed with bronchiectasis.
 Description of what the quality statement means for each audience• Health service providers/commissioners ensure systems are in place to record the result of a chest CT scan that is interpreted using paediatric criteria, and with access to the CT scan report being available to HCPs when required.
• HCPs ensure that a MDCT with a HRCT scan is performed and interpreted using paediatric criteria, and that the result recorded where there is a clinical diagnosis of bronchiectasis.
• Health service providers/commissioners ensure that appropriate CT scan services are available to allow the diagnosis of bronchiectasis to be confirmed.
• Children and adolescents with a clinical diagnosis of bronchiectasis have their diagnosis confirmed by MDCT with HRCT scans, using paediatric criteria.
 Relevant existing indicatorsNone identified.
 Other possible national data sourcesNone identified.
 Source referencesERS guideline for managing children and adolescents with bronchiectasis [12].
 Rationale• Children and adolescents with a clinical diagnosis of bronchiectasis should have the diagnosis confirmed by appropriate chest CT scans as there are other causes of chronic wet or productive cough in this age group.
• A normal chest radiograph is very insensitive and an abnormal chest radiograph is often inaccurate.
• A MDCT scan with HRCT images is more sensitive at detecting bronchiectasis than conventional HRCT alone.
• Using paediatric criteria is important as the broncho-arterial ratio (BAR) is significantly lower in children than in adults. Also, BAR correlates with age and increases as bronchiectasis becomes more severe (from cylindrical to varicose to cystic). Detecting bronchiectasis earlier requires using a paediatric BAR diagnostic threshold to identify abnormal airways and in so doing allows earlier treatment.
Quality standard statement 2
 Quality statementChildren and adolescents with bronchiectasis are taught appropriate ACTs by a respiratory physiotherapist.
 Quality measure• Structure: evidence of local arrangements to ensure that all children and adolescents with bronchiectasis are reviewed by a paediatric respiratory physiotherapist.
• Process: proportion of children and adolescents with bronchiectasis who received education on ACTs by a paediatric respiratory physiotherapist.
• Numerator: the number of children and adolescents with a diagnosis of bronchiectasis who received education on ACTs by a paediatric respiratory physiotherapist.
• Denominator: the total number of children and adolescents diagnosed with bronchiectasis.
 Description of what the quality statement means for each audience• Health service providers/commissioners ensure systems are in place to allow children and adolescents with bronchiectasis to see a paediatric respiratory physiotherapist.
• HCPs ensure that all children and adolescents with a diagnosis of bronchiectasis are: 1) referred to a paediatric respiratory physiotherapist to be taught ACTs, 2) have the appropriate equipment where relevant, and 3) advised of the frequency of ACTs during stable and exacerbation states.
• Health service providers/commissioners ensure that access to paediatric respiratory physiotherapists is available.
• Children and adolescents with a diagnosis of bronchiectasis are: 1) are taught appropriate ACTs by a paediatric respiratory physiotherapist, 2) have the necessary equipment, and 3) advised of the frequency and duration with which these ACTs should be undertaken.
 Relevant existing indicatorsNone identified.
 Other possible national data sourcesNone identified.
 Source referencesERS guideline for managing children and adolescents with bronchiectasis [12].
 Rationale• Children and adolescents with a diagnosis of bronchiectasis and their parents/guardians should receive specific education on ACTs.
• Individualised ACTs that are development- and age-appropriate are best taught by a paediatric-trained chest physiotherapist with their frequency also individualised.
• As children and adolescents mature, techniques may need to be changed and thus, the ACT type and frequency is best reviewed at least biannually by physiotherapists with expertise in paediatric respiratory care.
• During acute exacerbations of bronchiectasis, children and adolescents should receive ACTs more frequently.
Quality standard statement 3
 Quality statementChildren and adolescents with bronchiectasis with an acute exacerbation are prescribed at least 14 days of antibiotics.
 Quality measure• Structure: evidence of local arrangements to ensure that children and adolescents with bronchiectasis with an acute exacerbation are prescribed at least 14 days of antibiotics.
• Process: proportion of children and adolescents with an acute exacerbation of bronchiectasis who are prescribed at least 14 days of antibiotics.
• Numerator: the number of children and adolescents with a diagnosis of bronchiectasis and an acute exacerbation who are prescribed at least 14 days of antibiotics.
• Denominator: the total number of children and adolescents diagnosed with an acute exacerbation of bronchiectasis.
 Description of what the quality statement means for each audience• Health service providers/commissioners ensure systems are in place where children and adolescents with an acute exacerbation of bronchiectasis are prescribed at least 14 days of antibiotics.
• HCPs ensure that all children and adolescents with an acute exacerbation of bronchiectasis are prescribed least 14 days of antibiotics. The empiric antibiotic of choice is amoxicillin-clavulanate, but the type of antibiotics chosen should be based upon the patient's airway cultures (e.g. those with Pseudomonas aeruginosa require different treatment regimens to those without this organism) and history of antibiotic tolerance and hypersensitivity reactions.
• Health service providers/commissioners ensure that there is access to appropriate prescriptions of antibiotics.
• Children and adolescents with an acute exacerbation of bronchiectasis are prescribed at least 14 days of antibiotics, using amoxicillin-clavulanate unless their airway culture results suggest an alternative should be prescribed or when penicillins are contraindicated.
 Relevant existing indicatorsNone identified.
 Other possible national data sourcesNone identified.
 Source referencesERS guideline for managing children and adolescents with bronchiectasis [12].
 Rationale• Patients should have access to appropriate antibiotics for the recommended duration of treatment.
• In children and adolescents with an acute exacerbation of bronchiectasis, using amoxicillin-clavulanate significantly increases the resolution of exacerbations after 14 days of treatment and also shortens their duration (c.f. placebo).
• Antibiotic treatments for acute exacerbations of bronchiectasis are considered standard of care and this practice is supported by a high-quality randomised controlled trial.
• Recurrent respiratory exacerbations are common and associated with an impaired quality of life, poorer long-term clinical outcomes and substantial costs to the family and healthcare systems.
Quality standard statement 4
 Quality statementChildren and adolescents with bronchiectasis who have >1 hospitalised or ≥3 non-hospitalised exacerbations in the previous 12 months are offered at least a 6-month trial of macrolide antibiotics and the response assessed.
 Quality measure• Structure: evidence of local arrangements to ensure that children and adolescents with bronchiectasis who have >1 hospitalised or ≥3 non-hospitalised exacerbations in the previous 12 months are offered long-term macrolide antibiotics and the response assessed.
• Process: proportion of children and adolescents with bronchiectasis who have >1 hospitalised or ≥3 non-hospitalised exacerbations in the previous 12 months that are offered long-term macrolide antibiotics and their response assessed.
• Numerator: the number of children and adolescents with bronchiectasis who have >1 hospitalised or ≥3 non-hospitalised exacerbations in the previous 12 months offered long-term macrolide antibiotics and their response assessed.
• Denominator: the total number of children and adolescents with bronchiectasis who have >1 hospitalised or ≥3 non-hospitalised exacerbations in the previous 12 months.
 Description of what the quality statement means for each audience• Health service providers/commissioners ensure systems are in place where children and adolescents with bronchiectasis are offered long-term macrolide antibiotics when appropriate. Systems should also support the monitoring of adherence.
• HCPs ensure that all children and adolescents with bronchiectasis who have >1 hospitalised or ≥3 non-hospitalised exacerbations in the previous 12 months are offered long-term macrolide antibiotics and its beneficial effects (or otherwise) recorded. When used, a course should be for at least 6 months with regular reassessment to determine whether the antibiotic continues to provide a clinical benefit. Also, strategies to ensure adherence to the macrolide regimen should be in place, as ≥70% adherence improves effectiveness and reduces antibiotic resistance.
• Health service providers/commissioners ensure that children and adolescents with bronchiectasis have access to appropriate prescriptions of macrolide antibiotics.
• Children and adolescents with bronchiectasis who have >1 hospitalised or ≥3 non-hospitalised exacerbations in the previous 12 months are offered long-term macrolide antibiotics and the response assessed.
 Relevant existing indicatorsNone identified.
 Other possible national data sourcesNone identified.
 Source referencesERS guideline for managing children and adolescents with bronchiectasis [12].
 Rationale• Recurrent respiratory exacerbations are common and associated with an impaired quality of life, poorer long-term clinical outcomes and substantial costs to the family and healthcare systems.
• In children and adolescents with bronchiectasis, using regular macrolide antibiotics (azithromycin) halves the number of children and adolescents experiencing any exacerbations.
• The efficacy of macrolides at reducing exacerbations is consistent across studies. The importance and impact of exacerbations upon children and families were crucial considerations for the recommendation of using macrolides but only in those who have had >1 hospitalised or ≥3 non-hospitalised exacerbations in the previous 12 months.
• Children and adolescents receiving longer treatment courses (>24 months) should continue to be evaluated for risk versus benefit. This suggestion is in the context of lacking trial data for long-term azithromycin beyond this timepoint and the need for caution because of increasing antibiotic resistance amongst bacterial pathogens within patients and the community. Thus, their response to treatment should continue to be assessed.
• Furthermore, this approach should only be considered once ACTs have been optimised, comorbidities addressed and adherence with treatments confirmed.
Quality standard statement 5
 Quality statementChildren and adolescents with bronchiectasis have the minimum panel of diagnostic tests undertaken.
 Quality measure• Structure: evidence of local arrangements to ensure that children and adolescents with bronchiectasis have the minimum panel of diagnostic tests undertaken.
• Process: proportion of children and adolescents with bronchiectasis who have had the minimum panel of diagnostic tests undertaken.
• Numerator: the number of children and adolescents with bronchiectasis who have had the minimum panel of diagnostic tests undertaken.
• Denominator: the total number of children and adolescents diagnosed with bronchiectasis.
 Description of what the quality statement means for each audience• Health service providers/commissioners ensure systems are in place to record the results of the required tests.
• HCPs ensure that all children and adolescents with bronchiectasis have the minimum set of tests undertaken, i.e. 1) chest CT scan (to diagnose bronchiectasis), 2) sweat test, 3) lung function tests (in children and adolescents who can perform spirometry), 4) full blood count, 5) immunological tests (total IgG, IgA, IgM, IgE, specific antibodies to vaccine antigens) and 6) lower airway bacteriology. In settings where tuberculosis or HIV have a high prevalence and/or there is a history of close contact with tuberculosis, assessment for tuberculosis infection/disease or HIV, respectively, is also undertaken as part of the minimum panel of tests.
• Health service providers/commissioners ensure that services to undertake the recommended minimum panel of tests are available.
• Children and adolescents with bronchiectasis have possible underlying causes excluded and additional tests that help direct treatment.
 Relevant existing indicatorsNone identified.
 Other possible national data sourcesNone identified.
 Source referencesERS guideline for managing children and adolescents with bronchiectasis [12].
 Rationale• Identifying the aetiology has management implications (e.g. specific treatment for immunodeficiency or genetic causes for future family planning). Although lung function and respiratory cultures do not identify the cause, these tests help assess severity and guide antibiotic choices, respectively, thus optimising treatment.
• Determination of a standard set of investigations will help screen for major causes of bronchiectasis that are common or critical, e.g. immunodeficiency, infection or cystic fibrosis. Identifying underlying causes can have a positive early impact upon treatment.
Quality standard statement 6
 Quality statementChildren and adolescents with bronchiectasis receive specialist paediatric respiratory physician care.
 Quality measure• Structure: evidence of local arrangements to ensure that children and adolescents with bronchiectasis receive specialist paediatric respiratory physician care.
• Process: proportion of children and adolescents with bronchiectasis that receive specialist paediatric respiratory physician care.
• Numerator: the number of children and adolescents with bronchiectasis that receive specialist paediatric respiratory physician care.
• Denominator: the total number of children and adolescents diagnosed with bronchiectasis.
 Description of what the quality statement means for each audience• Health service providers/commissioners ensure systems are in place where children and adolescents with bronchiectasis receive specialist paediatric respiratory physician care.
• HCPs ensure that all children and adolescents with bronchiectasis receive specialist paediatric respiratory physician care where the following occurs:
o Nutrition is optimised, including vitamin D status.
o Exercise is encouraged on an ongoing basis.
o Children and adolescents are fully immunised according to their national immunisation programmes, including pneumococcal and seasonal influenza vaccines if these are not part of this programme.
o Factors important for reversibility and/or prevention of bronchiectasis are identified early. These include treatment of inhaled foreign bodies, preventing early and severe pneumonia, preventing recurrent PBB, treating primary immunodeficiency disorders causing bronchiectasis, promoting breastfeeding, and avoiding tobacco smoke, vaping and other airborne pollutants.
• Health service providers/commissioners ensure that access to specialist paediatric respiratory physician care is available.
• Children and adolescents with bronchiectasis are referred appropriately.
 Relevant existing indicatorsNone identified.
 Other possible national data sourcesNone identified.
 Source referencesERS guideline for managing children and adolescents with bronchiectasis [12].
 Rationale• Children and adolescents with bronchiectasis should receive specialist paediatric respiratory physician care so their treatment is optimised.
• It has been shown that the current standard of care in specialist settings where children and adolescents with bronchiectasis receive optimised management improves lung function post-diagnosis.
• In children and adolescents with bronchiectasis, the desirable effects of routine immunisation, exercise and physical activity, good nutrition and avoiding air pollutants are not disputed, but their magnitude of effect is uncertain.
• In some children and adolescents, their bronchiectasis is reversible and/or preventable. Factors important for reversibility and/or prevention of bronchiectasis include early identification and treatment of inhaled foreign bodies, preventing early and severe pneumonia, preventing recurrent PBB, treating primary immunodeficiency disorders causing bronchiectasis, promoting breastfeeding and immunisation, and avoiding tobacco smoke, vaping and other inhaled pollutants.
Quality standard statement 7
 Quality statementChildren and adolescents with bronchiectasis are reviewed at least 6-monthly by an MDT.
 Quality measure• Structure: evidence of local arrangements to ensure that children and adolescents with bronchiectasis are reviewed at least 6-monthly by an MDT; the MDT should include respiratory nurses and specialist paediatric physiotherapists as well as easy access to dieticians, psychologists, pharmacists, and other medical specialists, e.g. immunologists and radiologists with respiratory expertise.
• Process: proportion of children and adolescents with bronchiectasis that are reviewed at least 6-monthly by an MDT.
• Numerator: the number of children and adolescents with bronchiectasis that are reviewed at least 6-monthly by an MDT.
• Denominator: the total number of children and adolescents diagnosed with bronchiectasis.
 Description of what the quality statement means for each audience• Health service providers/commissioners ensure systems are in place where children and adolescents with bronchiectasis are reviewed at least 6-monthly by an MDT.
• HCPs ensure that all children and adolescents with bronchiectasis are reviewed at least 6-monthly by an MDT to:
o Monitor their general wellbeing and respiratory status, including lung function when age appropriate;
o Detect any complications; and
o Ensure they receive psychological support and education on equipment use and care.
• Health service providers/commissioners ensure access to regular multidisciplinary care and increase accessibility of children and adolescents to centres practising current standards of care.
• Children and adolescents with bronchiectasis are referred and reviewed appropriately.
 Relevant existing indicatorsNone identified.
 Other possible national data sourcesNone identified.
 Source referencesERS guideline for managing children and adolescents with bronchiectasis [12].
 Rationale• The positive effects of psychological support and teaching appropriate equipment use and care for children and adolescents with chronic illness are highly desirable, but there are no data on type, duration or intensity of support or how to assist with maintaining equipment.
• The current standard of care in specialist settings with optimised management includes multidisciplinary care. This leads to improved lung function post-diagnosis in children and adolescents with bronchiectasis.

The Quality Standards contained in this document are based upon the European Respiratory Society (ERS) guideline for managing children and adolescents with bronchiectasis published in 2021 [12] with an accompanying version that highlights these recommendations with case studies [13]. This document is to be used in healthcare services that treat children and adolescents with clinically significant bronchiectasis (patients with the clinical syndrome of chronic wet or productive cough with recurrent exacerbations, and radiographic confirmation of bronchiectasis). This document aims to improve the standards of care for children and adolescents with bronchiectasis. Its purpose is to provide health services, including planners and administrators, and patients with a guide to the minimum standards of care that children and adolescents with bronchiectasis should expect, together with measurable markers of good practice. CT: computed tomography; HCP: healthcare professional; ACTs: airway clearance techniques; PBB: protracted bacterial bronchitis; MDT: multidisciplinary team.