Program Head, Respiratory Health RFA
MBChB, PhD, FRACP
Prof André Schultz is the Program Head of Respiratory Health and Head of the BREATH Team at Telethon Kids Institute. He is passionate about finding ways to improve the health trajectories of children through multiple approaches with a strong focus on lung health in First Nations children. He is also a paediatric respiratory physician and Clinical Lead for Cystic Fibrosis, Bronchiectasis and Primary Ciliary Dyskinesia at Perth Children’s Hospital which provides comprehensive, multidisciplinary care for all children and young people with cystic fibrosis in Western Australia.
He aims to optimizing health early in life to ensure the best possible health outcomes later in life. His research interests include lung health in Aboriginal children, biomarkers of lung disease, cell culture-based disease models and mental health in cystic fibrosis.
He is a committed advocate for children and families affected by rare lung disease. He is the co-founder and Chair of the multinational chILDRANZ Peer Support Team, a member of the Lung Foundation’s Young Lungs Executive Advisory Committee that raise awareness and increase support for children with rare lung diseases, and the Deputy Chair of the Steering Committee of the Australian Cystic Fibrosis Data Registry. He is also the President of the Australian Council on Smoking and Health.
He has received research funding in excess of AU$22 million (AU$3 million as CIA) and has over 92 peer reviewed publications spanning airway surface physiology to knowledge translation science. He holds a NHMRC/MRFF Investigator grant to prevent permanent lung disease in Aboriginal and Torres Strait Islander children across Australia.
-
Projects
Lung damage in children with CF occurs much earlier than previously thought, and proving this is related to the decline that occurs later will create new paradigms for prevention and treatment.
-
Publications
October 2023
The SPEC score—A quantifiable CT scoring system for primary ciliary dyskinesia
Structural lung changes seen on computed tomography scans in persons with primary ciliary dyskinesia are currently described using cystic fibrosis derived scoring systems. Recent work has shown structural changes and frequencies that are unique to PCD, indicating the need for a unique PCD-derived scoring system.
Cystic Fibrosis Published research Bacterial Respiratory Infectious Disease Group Subsite: Walyan Child and adolescent healthMarch 2023Evaluation of the implementation and clinical effects of an intervention to improve medical follow-up and health outcomes for Aboriginal children hospitalised with chest infections
Aboriginal children hospitalised with acute lower respiratory infections (ALRIs) are at-risk of developing bronchiectasis, which can progress from untreated protracted bacterial bronchitis, often evidenced by a chronic (>4 weeks) wet cough following discharge. We aimed to facilitate follow-up for Aboriginal children hospitalised with ALRIs to provide optimal management and improve their respiratory health outcomes.
Published research Aboriginal Health Subsite: Walyan BREATH Respiratory viral infectionsMarch 2023BEAT CF pulmonary exacerbations core protocol for evaluating the management of pulmonary exacerbations in people with cystic fibrosis
Cystic fibrosis (CF) is a rare, inherited, life-limiting condition predominantly affecting the lungs, for which there is no cure. The disease is characterized by recurrent pulmonary exacerbations (PEx), which are thought to drive progressive lung damage. Management of these episodes is complex and generally involves multiple interventions targeting different aspects of disease. The emergence of innovative trials and use of Bayesian statistical methods has created renewed opportunities for studying heterogeneous populations in rare diseases.
Cystic Fibrosis Published research Infectious Disease Implementation Research P4 Respiratory Health for Kids Subsite: Walyan BREATHMarch 2023Learning to make a difference for chILD: Value creation through network collaboration and team science
Addressing the recognized challenges and inequalities in providing high quality healthcare for rare diseases such as children's interstitial lung disease (chILD) requires collaboration across institutional, geographical, discipline, and system boundaries. The Children's Interstitial Lung Disease Respiratory Network of Australia and New Zealand (chILDRANZ) is an example of a clinical network that brings together multidisciplinary health professionals for collaboration, peer learning, and advocacy with the goal of improving the diagnosis and management of this group of rare and ultra-rare conditions.
Published research Early Childhood Development Subsite: Walyan BREATH Rare diseasesDecember 2022Change in health outcomes for First Nations children with chronic wet cough: rationale and study protocol for a multi-centre implementation science study
In children, chronic wet cough may be a sign of underlying lung disease, including protracted bacterial bronchitis (PBB) and bronchiectasis. Chronic (> 4 weeks in duration) wet cough (without indicators pointing to alternative causes) that responds to antibiotic treatment is diagnostic of PBB. Timely recognition and management of PBB can prevent disease progression to irreversible bronchiectasis with lifelong consequences. However, detection and management require timely health-seeking by carers and effective management by clinicians.
Published research Academic Biostatistics Aboriginal Health Infectious Diseases Subsite: Walyan BREATHNovember 2022Survival of people with cystic fibrosis in Australia
Survival statistics, estimated using data from national cystic fibrosis (CF) registries, inform the CF community and monitor disease progression. This study aimed to estimate survival among people with CF in Australia and to identify factors associated with survival.
Cystic Fibrosis Published research Subsite: Walyan BREATHSeptember 2022Monitoring disease progression in childhood bronchiectasis
Bronchiectasis (not related to cystic fibrosis) is a chronic lung disease caused by a range of etiologies but characterized by abnormal airway dilatation, recurrent respiratory symptoms, impaired quality of life and reduced life expectancy.
Published research Children's Lung Health P4 Respiratory Health for Kids Subsite: Walyan BREATH Immune systemSeptember 2022Prevalence of chronic respiratory diseases in Aboriginal children: A whole population study
The burden of bronchiectasis is disproportionately high in Aboriginal adults, with early mortality. Bronchiectasis precursors, that is, protracted bacterial bronchitis and chronic suppurative lung disease, often commence in early childhood.
Aboriginal Health & Wellbeing Academic Biostatistics Aboriginal Health Children's Lung Health Subsite: Walyan BREATH Respiratory viral infectionsSeptember 2022Spring-infusors: How a simple and small solution can create king-sized complexity
The aims of the study were to investigate family and hospital staff views about the use of spring-infusor devices for administration of intravenous antibiotic medications, to examine if the device is acceptable and feasible and to map a process for implementation.
Cystic Fibrosis Published research Subsite: Walyan BREATHSeptember 2022Protocol for establishing a core outcome set for evaluation in studies of pulmonary exacerbations in people with cystic fibrosis
Pulmonary exacerbations are associated with increased morbidity and mortality in people with cystic fibrosis (CF). There is no consensus about which outcomes should be evaluated in studies of pulmonary exacerbations or how these outcomes should be measured.
Cystic Fibrosis Published research Infectious Disease Implementation Research Infectious Diseases Epidemiology Subsite: Wesfarmers Centre of Vaccines and Infectious Diseases Subsite: Walyan BREATHSeptember 2022The role of exome sequencing in childhood interstitial or diffuse lung disease
Children’s interstitial and diffuse lung disease (chILD) is a complex heterogeneous group of lung disorders. Gene panel approaches have a reported diagnostic yield of ~ 12%. No data currently exist using trio exome sequencing as the standard diagnostic modality.
Published research Subsite: Walyan BREATHJune 2022Lung abscess: 14 years of experience in a tertiary paediatric hospital
Lung abscess is a rare condition in paediatrics with a paucity of literature. Intravenous antibiotics is the main therapy; however interventional radiological approaches have led to the use of percutaneous drainage. Surgery is reserved for the management of complications.
Published research Aboriginal Health P4 Respiratory Health for Kids BREATHMay 2022Reducing exacerbations in children and adults with primary ciliary dyskinesia using erdosteine and/or azithromycin therapy (REPEAT trial): study protocol for a multicentre, double-blind, double-dummy, 2×2 partial factorial, randomised controlled trial
Primary ciliary dyskinesia (PCD) is a rare, progressive, inherited ciliopathic disorder, which is incurable and frequently complicated by the development of bronchiectasis. There are few randomised controlled trials (RCTs) involving children and adults with PCD and thus evidence of efficacy for interventions are usually extrapolated from people with cystic fibrosis.
Cystic Fibrosis Published research P4 Respiratory Health for Kids BREATHApril 2022A pilot study of disease related education and psychotherapeutic support for unresolved grief in parents of children with CF
Diagnosis of chronic disease in a child can result in unresolved grief (UG) in parents. This study aimed to evaluate the efficacy of psychological insight-oriented therapy (IOT) as a treatment for UG compared to disease related education in parents of children with cystic fibrosis. Sequence of delivery, first IOT then disease related education (or vice versa) was also examined, to let all participants experience both interventions.
Cystic Fibrosis Published research Academic Biostatistics P4 Respiratory Health for Kids Subsite: Walyan BREATHMarch 2022Implementation of a strategy to facilitate effective medical follow-up for Australian First Nations children hospitalised with lower respiratory tract infections: study protocol
First Nations children hospitalised with acute lower respiratory infections (ALRIs) are at increased risk of future bronchiectasis (up to 15-19%) within 24-months post-hospitalisation. An identified predictive factor is persistent wet cough a month after hospitalisation and this is likely related to protracted bacterial bronchitis which can progress to bronchiectasis, if untreated.
Published research Academic Biostatistics Aboriginal Health P4 Respiratory Health for Kids Wet Cough BREATH Respiratory viral infectionsSeptember 2021Acute haemoptysis, fever and abdominal pain in an adolescent from northern Australia
Published research Infectious Disease Implementation Research Infectious Diseases Epidemiology P4 Respiratory Health for Kids Subsite: Wesfarmers Centre of Vaccines and Infectious Diseases Healthy Skin and ARF Prevention BREATHFebruary 2022Primary Nasal Epithelial Cells as a Surrogate Cell Culture Model for Type-II Alveolar Cells to Study ABCA-3 Deficiency
ATP Binding Cassette Subfamily A Member 3 (ABCA-3) is a lipid transporter protein highly expressed in type-II alveolar (AT-II) cells. Mutations in ABCA3 can result in severe respiratory disease in infants and children. To study ABCA-3 deficiency in vitro, primary AT-II cells would be the cell culture of choice although sample accessibility is limited. Our aim was to investigate the suitability of primary nasal epithelial cells, as a surrogate culture model for AT-II cells, to study ABCA-3 deficiency.
Published research Airway Epithelial Research Subsite: Walyan BREATH Translational GeneticsFebruary 2022Genomic testing for children with interstitial and diffuse lung disease (chILD): parent satisfaction, understanding and health-related quality of life
Research is needed to determine best practice for genomic testing in the context of child interstitial or diffuse lung disease. We explored parent's and child's health-related quality of life, parents' perceived understanding of a genomic testing study, satisfaction with information and the study and decisional regret to undertake genomic testing.
Published research Bacterial Respiratory Infectious Disease GroupJanuary 2022Preferred health outcome states following treatment for pulmonary exacerbations of cystic fibrosis
Treatment for pulmonary exacerbations of cystic fibrosis (CF) can produce a range of positive and negative outcomes. Understanding which of these outcomes are achievable and desirable to people affected by disease is critical to agreeing to goals of therapy and determining endpoints for trials.
Cystic Fibrosis Published research Infectious Diseases Epidemiology BREATHNovember 2021Fissure adjacent partial lobe atelectasis in primary ciliary dyskinesia
Establishing the underlying cause in a child with chronic suppurative lung disease (CSLD) allows for targeted treatment and screening for associated complications. One cause of CSLD is primary ciliary dyskinesia (PCD). Testing for PCD requires specialist expertise which is not widely available.
Published research BREATHOctober 2021Novel method to select meaningful outcomes for evaluation in clinical trials
A standardised framework for selecting outcomes for evaluation in trials has been proposed by the Core Outcome Measures in Effectiveness Trials working group. However, this method does not specify how to ensure that the outcomes that are selected are causally related to the disease and the health intervention being studied. Causal network diagrams may help researchers identify outcomes that are both clinically meaningful and likely to be causally dependent on the intervention, and endpoints that are, in turn, causally dependent on those outcomes.
Cystic Fibrosis Published research Infectious Diseases Epidemiology Subsite: Wesfarmers Centre of Vaccines and Infectious Diseases BREATHOctober 2021Duration of amoxicillin-clavulanate for protracted bacterial bronchitis in children (DACS): a multi-centre, double blind, randomised controlled trial
Protracted bacterial bronchitis (PBB) is a leading cause of chronic wet cough in children. The current standard treatment in European and American guidelines is 2 weeks of antibiotics, but the optimal duration of therapy is unknown. We describe the first randomised controlled trial to assess the duration of antibiotic treatment in children with chronic wet cough and suspected PBB.
Published research Infectious Diseases Wet Cough BREATHSeptember 2021Conducting decolonizing research and practice with Australian First Nations to close the health gap
The purpose of this paper is to highlight a perspective for decolonizing research with Australian First Nations and provide a framework for successful and sustained knowledge translation by drawing on the recent work conducted by a research group, in five remote communities in North-Western Australia.
Published research Aboriginal Health Subsite: Walyan BREATHOctober 2021Respiratory follow-up to improve outcomes for Aboriginal children: twelve key steps
Among Aboriginal children, the burden of acute respiratory tract infections (ALRIs) with consequent bronchiectasis post-hospitalisation is high. Clinical practice guidelines recommend medical follow-up one-month following discharge, which provides an opportunity to screen and manage persistent symptoms and may prevent bronchiectasis.
Published research Aboriginal Health Infectious Diseases P4 Respiratory Health for Kids BREATHAugust 2021Frequency of protracted bacterial bronchitis and management pre-respiratory referral
To determine the frequency of protracted bacterial bronchitis (PBB) in children referred to tertiary care with chronic cough and describe management prior to referral. A retrospective cohort study of all new patients with a history of ≥4 weeks of cough seen at the only tertiary paediatric outpatient respiratory service in Western Australia.
Published research Wet Cough BREATHJuly 2021Variation in treatment preferences of pulmonary exacerbations among Australian and New Zealand cystic fibrosis physicians
Despite advances in cystic fibrosis (CF) management and survival, the optimal treatment of pulmonary exacerbations remains unclear. Understanding the variability in treatment approaches among physicians might help prioritise clinical uncertainties to address through clinical trials.
Cystic Fibrosis Published research Subsite: Wesfarmers Centre of Vaccines and Infectious Diseases BREATHJuly 2021Paediatric headbox as aerosol and droplet barrier
High-flow nasal oxygen (HFNO) is frequently used in hospitals, producing droplets and aerosols that could transmit SARS-CoV-2. Our aim was to determine if a headbox could reduce droplet and aerosol transmission from patients requiring HFNO.
Published research BREATH COVID-19June 2021Does machine learning have a role in the prediction of asthma in children?
Asthma is the most common chronic lung disease in childhood. There has been a significant worldwide effort to develop tools/methods to identify children's risk for asthma as early as possible for preventative and early management strategies. Unfortunately, most childhood asthma prediction tools using conventional statistical models have modest accuracy, sensitivity, and positive predictive value.
Asthma Published research Children's Lung Health Subsite: Walyan BREATHMay 2021The measurement properties of tests and tools used in cystic fibrosis studies: a systematic review
There is no consensus on how best to measure responses to interventions among children and adults with cystic fibrosis (CF). We have systematically reviewed and summarised the characteristics and measurement properties of tests and tools that have been used to capture outcomes in studies among people with CF, including their reliability, validity and responsiveness. This review is intended to guide researchers when selecting tests or tools for measuring treatment effects in CF trials. A consensus set of these tests and tools could improve consistency in how outcomes are captured and thereby facilitate comparisons and synthesis of evidence across studies.
Cystic Fibrosis Published research Infectious Diseases Epidemiology Subsite: Wesfarmers Centre of Vaccines and Infectious Diseases Subsite: Walyan BREATHApril 2021Surfactant protein disorders in childhood interstitial lung disease
Surfactant, which was first identified in the 1920s, is pivotal to lower the surface tension in alveoli of the lungs and helps to lower the work of breathing and prevents atelectasis. Surfactant proteins, such as surfactant protein B and surfactant protein C, contribute to function and stability of surfactant film.
Published research Early Childhood Development BREATHJanuary 2021Recognition and Management of Protracted Bacterial Bronchitis in Australian Aboriginal Children: A Knowledge Translation Approach
Chronic wet cough in children is the hallmark symptom of protracted bacterial bronchitis (PBB) and if left untreated can lead to bronchiectasis, which is prevalent in Indigenous populations. Underrecognition of chronic wet cough by parents and clinicians and underdiagnosis of PBB by clinicians are known.
Aboriginal Health Subsite: Wesfarmers Centre of Vaccines and Infectious Diseases Wet Cough Subsite: WalyanDecember 2020Assessment of different techniques for the administration of inhaled salbutamol in children breathing spontaneously via tracheal tubes, supraglottic airway devices, and tracheostomies
Perioperative respiratory adverse events account for a third of all perioperative cardiac arrests, with bronchospasm and laryngospasm being most common. Standard treatment for bronchospasm is administration of inhaled salbutamol, via pressurized metered dose inhaler. There is little evidence on the best method of attaching the pressurized metered dose inhaler to the artificial airway during general anesthesia. The aim of this study is to investigate the best method to deliver aerosolized salbutamol via pressurized metered dose inhaler to the lungs of an anesthetized child.
Published research P4 Respiratory Health for Kids Perioperative MedicineDecember 2020BAL Inflammatory Markers Can Predict Pulmonary Exacerbations in Children With Cystic Fibrosis
Pulmonary exacerbations in cystic fibrosis are characterized by airway inflammation and may cause irreversible lung damage. Early identification of such exacerbations may facilitate early initiation of treatment, thereby potentially reducing long-term morbidity. Research question: Is it possible to predict pulmonary exacerbations in children with cystic fibrosis, using inflammatory markers obtained from BAL fluid?
Cystic Fibrosis Published research Early Childhood Development P4 Respiratory Health for Kids Subsite: Cystic Fibrosis Subsite: WalyanNovember 2020Outcomes and endpoints reported in studies of pulmonary exacerbations in people with cystic fibrosis: A systematic review
There is no consensus about which outcomes should be evaluated in studies of pulmonary exacerbations in people with cystic fibrosis (CF). Outcomes used for evaluation should be meaningful; that is, they should capture how people feel, function or survive and be acknowledged as important to people with CF, or should be reliable surrogates of those outcomes. We aimed to summarise the outcomes and corresponding endpoints which have been reported in studies of pulmonary exacerbations, and to identify those which are most likely to be meaningful.
Cystic Fibrosis Published research P4 Respiratory Health for Kids Subsite: Wesfarmers Centre of Vaccines and Infectious DiseasesDecember 2020Assessment of different techniques for the administration of inhaled salbutamol in children breathing spontaneously via tracheal tubes, supraglottic airway devices, and tracheostomies
Perioperative respiratory adverse events account for a third of all perioperative cardiac arrests, with bronchospasm and laryngospasm being most common. Standard treatment for bronchospasm is administration of inhaled salbutamol, via pressurized metered dose inhaler. There is little evidence on the best method of attaching the pressurized metered dose inhaler to the artificial airway during general anesthesia. The aim of this study is to investigate the best method to deliver aerosolized salbutamol via pressurized metered dose inhaler to the lungs of an anesthetized child.
Published research P4 Respiratory Health for Kids Perioperative MedicineApril 2020We won't find what we don't look for: Identifying barriers and enablers of chronic wet cough in Aboriginal children
Key barriers to effective management of chronic wet cough are limited training in chronic wet cough management combined with competing complexities
Published research Children's Lung Health Wet CoughMarch 2020Developing a smartphone application to support social connectedness and wellbeing in young people with cystic fibrosis
This study developed and tested a highly usable, and moderately acceptable, smartphone app to improve the psychosocial health of young people living with CF
Cystic Fibrosis Published research Human Development and Community WellbeingDecember 2019Prevalence of chronic wet cough and protracted bacterial bronchitis in aboriginal children
Strategies to address reasons for and treatment of chronic wet cough and protracted bacterial bronchitis in young Aboriginal children in remote north Western Australia are required
Published research Children's Lung Health Wet CoughNovember 2019An introduction to clinical trial design
This manuscript will give a brief overview of clinical trial design including the strengths and limitations of various approaches
Published research Infectious Disease Implementation ResearchSeptember 2019Efficacy of oral amoxicillin-clavulanate or azithromycin for non-severe respiratory exacerbations in children with bronchiectasis (BEST-1)
Amoxicillin-clavulanate treatment is beneficial in terms of resolution of non-severe exacerbations of bronchiectasis in children
Published researchSeptember 2019Changing Prevalence of Lower Airway Infections in Young Children with Cystic Fibrosis
Aspergillus species and P. aeruginosa are commonly present in the lower airways from infancy
Cystic Fibrosis Published research Academic Biostatistics P4 Respiratory Health for KidsAugust 2019Discrete choice experiment to evaluate preferences of patients with cystic fibrosis among alternative treatment-related health outcomes: A protocol
The aim of this study is to identify and quantify the preferences of patients with cystic fibrosis regarding treatment outcomes
Cystic Fibrosis Published research Infectious Disease Implementation ResearchJuly 2019Chronic wet cough in Aboriginal children: It's not just a cough
Results highlight the need for a culturally appropriate information and education of the importance of chronic wet cough in children
Published research Wet CoughJune 2019Identifying pediatric lung disease: A comparison of forced oscillation technique outcomes
These findings suggest the utility of specific FOT outcomes is dependent on the respiratory disease being assessed
Asthma Cystic Fibrosis Published research Children's Lung HealthApril 2019Research Note: Adaptive trials
This Research Note has explored the strengths, risks and potential complexity of adaptive trials
Published research Infectious Disease Implementation ResearchApril 2019CF derived scoring systems do not fully describe the range of structural changes seen on CT scans in PCD
Structural changes identified on CT scans in primary ciliary dyskinesia are not identical to those previously described in cystic fibrosis patients
Cystic Fibrosis Published research P4 Respiratory Health for KidsMarch 2019Trial refresh: A case for an adaptive platform trial for pulmonary exacerbations of cystic fibrosis
This review will discuss the necessary steps required for a Bayesian adaptive platform trial to optimize treatment of pulmonary exacerbations of cystic fibrosis
Cystic Fibrosis Published research Infectious Disease Implementation ResearchSeptember 2018Side effects of medications used to treat childhood interstitial lung disease
Interstitial lung disease in children comprises a range of different rare diseases
Published research P4 Respiratory Health for KidsAugust 2018CrossTalk opposing view: Mucosal acidification does not drive early progressive lung disease in cystic fibrosis
Whether airway mucosal acidification drives early progressive lung disease is controversial
Cystic Fibrosis Published research P4 Respiratory Health for KidsJanuary 2018Humidified high-flow nasal cannula oxygen for bronchiolitis: Should we go with the flow?
Current NICE 2015 guidelines for bronchiolitis state that the use of HFNC is becoming widespread without demonstration of additional efficacy.
Published researchNovember 2017Current options in aerosolised drug therapy for children receiving respiratory support
In this review, we explore current knowledge and provide guidance as to when and how the inhaled route may be of value when treating patients whose tracheas are intubated
Published research Airway Epithelial ResearchNovember 2017Airway surface liquid pH is not acidic in children with cystic fibrosis
Modulation of ASL pH has been proposed as a therapy for CF. However, evidence that ASL pH is reduced in CF is limited and conflicting.
Cystic Fibrosis Published research Airway Epithelial Research P4 Respiratory Health for Kids -
Education & Qualifications
-
Active Collaborations