Telehealth use in Australian cystic fibrosis centers: Clinician experiences.

BACKGROUND: Telehealth has been rapidly adopted by cystic fibrosis (CF) centers and ongoing use in routine CF care is endorsed by CF consumers. However, data describing CF clinician perceptions regarding telehealth are scarce. We aimed to describe clinician experiences and attitudes towards telehealth in CF care among health professionals across Australia. METHODS: CF multidisciplinary health professionals from all CF clinics in Australia were sent an anonymous electronic survey. RESULTS: Eighty-five responses were received representing 15 of 23 (65%) centers. Most clinicians reported using telehealth for routine clinic visits, and a range of other clinical encounters (69.9%). Telehealth was widely perceived as acceptable (91.8%), and clinicians were comfortable/very comfortable (81.2%) integrating telehealth into future CF care. Despite this, 64.1% of respondents considered telehealth clinics to be much worse than face-to-face clinics and 57.5% reported quality of care was somewhat/much worse using telehealth. Home spirometry was available in 73.7% of centers, however, only 26.7% of clinics could provide spirometers for >75% eligible patients. Growth and microbiology assessments were often missed in telehealth clinics and 75.7% reported a technical issue had prevented a telehealth consultation from occurring. CONCLUSIONS: Telehealth for CF in Australia is considered feasible and acceptable by CF clinicians, although use of telehealth varies widely between centers. Concerns exist around the impact of telehealth on health outcomes, especially given core assessments are frequently omitted. Guidelines may help ensure the benefits of telehealth are realized for people with CF without compromising the standard of care.

Psychosocial needs and interventions for young children with cystic fibrosis and their families.

This review summarises the experiences of young children and their families living with CF during the first five years of life following NBS diagnosis, as well as the options of psychosocial support available to them. We present strategies embedded within routine CF care that focus on prevention, screening, and intervention for psychosocial health and wellbeing that constitute essential components of multidisciplinary care in infancy and early childhood.

Procedural anxiety among children and adolescents with cystic fibrosis and their parents.

BACKGROUND: Procedural anxiety involves acute distress around medical procedures and may lead to avoidance or resistance behaviors that interfere with effective cystic fibrosis (CF) care and health outcomes. While individuals with CF commonly endure uncomfortable and/or distressing medical procedures, procedural anxiety among children and adolescents with CF has received little research attention. This study investigated the prevalence and correlates of procedural anxiety among individuals with CF aged 6-18 and their parents. METHOD: Eighty-nine parents of children with CF completed surveys examining child procedural anxiety, anxiety, and health behaviors (including treatment adherence); and parent vicarious procedural anxiety. RESULTS: Seventy-five percent of participants rated at least one CF-related procedure as "extremely" anxiety-inducing for their child. Parental vicarious procedural anxiety was reported in 80.9% of participants. Procedural anxiety significantly correlated with child anxiety, treatment-resistive behaviors, and parent-vicarious procedural anxiety. Procedural anxiety was associated with younger age and frequency of distressing procedures, but not with forced expiratory volume in 1 s, body mass index, hospitalizations, or exposure to general anesthesia. CONCLUSION: Procedural anxiety is common among children, adolescents, and caregivers, and is associated with child anxiety and treatment resistance, emphasizing the importance of screening and interventions for procedural anxiety as part of routine CF care from early childhood. Implications for screening and intervention are discussed.

Survival 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. This population-based cohort study used prospectively collected data from 23 Australian CF centres participating in the Australian CF Data Registry (ACFDR) from 2005-2020. Period survival analysis was used to calculate median age of survival estimates for each 5-year window from 2005-2009 until 2016-2020. The overall median survival was estimated using the Kaplan-Meier method. Between 2005-2020 the ACFDR followed 4,601 people with CF, noting 516 (11.2%) deaths including 195 following lung transplantation. Out of the total sample, more than half (52.5%) were male and 395 (8.6%) had undergone lung transplantation. Two thirds of people with CF (66.1%) were diagnosed before six weeks of age or by newborn/prenatal screening. The overall median age of survival was estimated as 54.0 years (95% CI: 51.0-57.04). Estimated median survival increased from 48.9 years (95% CI: 44.7-53.5) for people with CF born in 2005-2009, to 56.3 years (95% CI: 51.2-60.4) for those born in 2016-2020. Factors independently associated with reduced survival include receiving a lung transplant, having low FEV1pp and BMI. Median survival estimates are increasing in CF in Australia. This likely reflects multiple factors, including newborn screening, improvement in diagnosis, refinements in CF management and centre-based multidisciplinary care.

Time to get serious about the detection and monitoring of early lung disease in cystic fibrosis.

Structural and functional defects within the lungs of children with cystic fibrosis (CF) are detectable soon after birth and progress throughout preschool years often without overt clinical signs or symptoms. By school age, most children have structural changes such as bronchiectasis or gas trapping/hypoperfusion and lung function abnormalities that persist into later life. Despite improved survival, gains in forced expiratory volume in one second (FEV1) achieved across successive birth cohorts during childhood have plateaued, and rates of FEV1 decline in adolescence and adulthood have not slowed. This suggests that interventions aimed at preventing lung disease should be targeted to mild disease and commence in early life. Spirometry-based classifications of 'normal' (FEV1≥90% predicted) and 'mild lung disease' (FEV1 70%-89% predicted) are inappropriate, given the failure of spirometry to detect significant structural or functional abnormalities shown by more sensitive imaging and lung function techniques. The state and readiness of two imaging (CT and MRI) and two functional (multiple breath washout and oscillometry) tools for the detection and monitoring of early lung disease in children and adults with CF are discussed in this article.Prospective research programmes and technological advances in these techniques mean that well-designed interventional trials in early lung disease, particularly in young children and infants, are possible. Age appropriate, randomised controlled trials are critical to determine the safety, efficacy and best use of new therapies in young children. Regulatory bodies continue to approve medications in young children based on safety data alone and extrapolation of efficacy results from older age groups. Harnessing the complementary information from structural and functional tools, with measures of inflammation and infection, will significantly advance our understanding of early CF lung disease pathophysiology and responses to therapy. Defining clinical utility for these novel techniques will require effective collaboration across multiple disciplines to address important remaining research questions. Future impact on existing management burden for patients with CF and their family must be considered, assessed and minimised.To address the possible role of these techniques in early lung disease, a meeting of international leaders and experts in the field was convened in August 2019 at the Australiasian Cystic Fibrosis Conference. The meeting entitiled 'Shaping imaging and functional testing for early disease detection of lung disease in Cystic Fibrosis', was attended by representatives across the range of disciplines involved in modern CF care. This document summarises the proceedings, key priorities and important research questions highlighted.

Anxiety in Children with Cystic Fibrosis and Their Parents: A Systematic Review.

Individuals with cystic fibrosis (CF) are at high risk of clinically significant anxiety, which can be related to lower treatment adherence and poorer health outcomes. Additionally, up to half of the parents/caregivers of children with CF experience clinically significant anxiety. Research has focussed on CF youth aged 13 years and older, leaving anxiety among school-aged children (aged 6-12 years) largely unstudied. This review aimed to synthesize research on anxiety among children with CF and their parents, examining prevalence, risk factors, and relationships between parent and child factors. Four electronic databases were searched, and publications were included if participants were children (or parents of children) with CF with a mean age between 6 and 12 years, and a standardized anxiety measure was used. Data from fourteen studies were extracted for descriptive synthesis; however, no studies focussed exclusively on the age range of 6-12 years. Results generally indicated that anxiety is highly prevalent in both child and parent populations; anxiety was the most prevalent mental health condition among children with CF, and anxiety was higher among CF populations than control populations among both children and parents. However, there were disparities, with some papers finding low rates of anxiety, and results on the relationship between anxiety and health outcomes varying greatly. Several risk factors were identified, but few were corroborated. There is an overall deficiency of research in this area, particularly examining the relationships between parent and child anxiety, and anxiety and health outcomes. Further research on suitable screening and intervention practices is also required.

Early disease surveillance in young children with cystic fibrosis: A qualitative analysis of parent experiences.

BACKGROUND: Sensitive measures of early lung disease are being integrated into therapeutic trials and clinical practice in cystic fibrosis (CF). The impact of early disease surveillance (EDS) using these novel and often intensive techniques on young children and their families is not well researched. METHODS: The Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF) has operated a combined clinical and research early disease surveillance program, based around annual chest CT scan, bronchoscopy and lung function from newborn screening diagnosis until age 6 years, for over two-decades. To explore parental experiences of EDS in their child, a qualitative study was conducted using audio-recorded, semi-structured interviews in n=46 mothers and n=21 fathers of children (aged 3-months to six years) attending CF centres in Perth and Melbourne, Australia. Themes were developed iteratively using thematic analysis and assessed for validity and confirmability. RESULTS: Parents' experiences were positive overall; affording a sense of control over CF, disease knowledge, and belief that EDS was in the best interests of their child. Challenges included poor understanding about EDS measures leading to anxiety and distress, self-blame surrounding adverse findings, and emotional burden of surveillance visits. Tailored information regarding the practical and psychosocial aspects of EDS were endorsed. CONCLUSION: While experiences were generally positive there is need for information and psychosocial support for parents to mitigate anxiety and develop positive coping strategies surrounding surveillance procedures and results. Managing expectations regarding risks and benefits of disease surveillance in clinical and research settings are important aspects of care.

Health-related quality-of-life in children with cystic fibrosis aged 5-years and associations with health outcomes.

BACKGROUND: The impact of early cystic fibrosis (CF) on health-related quality-of-life (HRQOL) in preschool children is poorly characterised, and data on relationships between HRQOL and health outcomes in young children with CF are limited. We aimed to characterise and compare parent-proxy and child-reported HRQOL and evaluate relationships with clinical outcomes at age 5-years. METHODS: Subjects were participating in the multi-centre Australasian Cystic Fibrosis Bronchoalveolar Lavage (ACFBAL) trial investigating BAL-directed versus standard CF therapy. Children aged 5-years and their parents rated HRQOL using the Pediatric Quality of Life Inventory (PedsQL™) and Cystic Fibrosis Questionnaire-Revised (CFQ-R) questionnaires. RESULTS: PedsQL and CFQ-R questionnaires were completed by 141 primary caregivers and 135 and 130 children, respectively. There were no differences in HRQOL between children randomised to BAL-directed versus standard CF therapy. Children with CF rated worse HRQOL than healthy children and there was poor parent-child concordance across HRQOL domains. Nutritional status, CF-CT scan score, forced expiratory volume in 1-second (FEV1), and pulmonary exacerbations correlated with HRQOL at age 5-years. FEV1 z-scores positively correlated with parent-proxy HRQOL in CFQ-R Respiratory (p = 0.018), Physical (<0.001), Emotional (p = 0.007) subscales and PedsQL Total-score (p = 0.021), Physical (p = 0.019) domains. Pulmonary exacerbation rates were inversely associated with parent-proxy CFQ-R Respiratory (p = 0.004), Physical (p = 0.022), PedsQL Total (p = 0.009) and Physical (p = 0.009) scores. CONCLUSION: Parent-reported HRQOL is a meaningful clinical endpoint to evaluate interventions in young children. Parent and child HRQOL reports provide different, complementary information. A preschool version of the CFQ-R is needed to assess relationships between HRQOL and clinical outcomes in young children.

Information needs of parents of infants diagnosed with cystic fibrosis: Results of a pilot study.

This study investigated the information needs, priorities and information-seeking behaviours of parents of infants recently diagnosed with cystic fibrosis (CF) following newborn screening, by piloting the 'Care of Cystic Fibrosis Families Survey'. The questionnaires were posted to eligible parents ( n = 66) attending CF clinics in hospitals in two Australian states; reply-paid envelopes were provided for return of the questionnaires. Twenty-six were returned (response rate 39.4%). The most common questions to which parents required answers during their initial education period related to what CF is, how it is treated and how to care for their child. Parents preferred face-to-face consultations to deliver information, and yet all reported using the Internet to search for more information at some point during the education period. Many parents provided negative feedback about being given their child's CF diagnosis via telephone. The timing, content and method of information delivery can all affect the initial education experience. We can deliver education to better suit the information needs and priorities for education of parents of infants recently diagnosed with CF. The Care of Cystic Fibrosis Families Survey was successfully piloted and recommendations for amendments have been made for use in a larger study across Australia.

Family-centred care for families living with cystic fibrosis in a rural setting: A qualitative study.

AIMS AND OBJECTIVES: To explore experiences of family-centred care among parents of children with cystic fibrosis living far from tertiary treatment centres and to understand what such distances mean to their care. BACKGROUND: Australia is a large continent. However, many families with a child with cystic fibrosis live in regional areas, often thousands of kilometres away from the primary treatment centres located in Australia's coastal capital cities. DESIGN: A qualitative, phenomenological design using a Van Manen () approach. METHODS: Individual, semi-structured interviews were conducted with parents (n = 7) of a child with cystic fibrosis who lived in regional Australia. Thematic content data analysis was used. RESULTS: The essence of the participants' experience was their seeking certainty and continuity in the changeable realm of cystic fibrosis while negotiating a collaborative approach to their child's care. Five core themes and two subthemes were identified: "Daily care: a family affair," including the subtheme "Accessing expert care"; "Family-centred care: seeking inclusion"; "Control versus collaboration: seeking mutual trust," with the subtheme "The team who grows with you"; "Future projections"; and "The CF circle." CONCLUSION: Some concerns are not unlike those of their city counterparts, but can be intensified by their sense of distance and isolation. RELEVANCE TO CLINICAL PRACTICE: Insight into this unique milieu from the parents' perspective is requisite so that care is appropriate to such a challenging environment and incorporates the whole family.

Family-centred care in cystic fibrosis: a pilot study in North Queensland, Australia.

AIMS: The aims were to: (i) examine perceptions of family-centred care of parents of children with cystic fibrosis and healthcare professionals who care for them; (ii) test design and tools in a regional population. DESIGN: Quantitative pilot study of existing questionnaire. METHODS: The methods involved were comparative, cross-sectional survey of parents of children with cystic fibrosis and health staff in North Queensland, using "Perceptions of Family Centered Care - Parent" and "Perceptions of Family Centered Care - Staff" questionnaires; and descriptive study of tools. RESULTS: Eighteen staff, 14 parents (78%, 61%); using Mann-Whitney U, showed no significant differences in scores in categories: 'support' 'respect', 'collaboration'. Comments about suitability of questionnaires varied, but were largely positive.

Induced sputum to detect lung pathogens in young children with cystic fibrosis.

INTRODUCTION: Induced sputum sampling holds promise as a method for obtaining samples representative of the lower airways in young children. Collection of induced sputum samples in young children differs from older children and adults' as pharyngeal suctioning is often required. Our aim was to determine the sensitivity and specificity of induced sputum with and without airway clearance techniques to detect lower airway pathogens in children less than age 7 with cystic fibrosis. METHODS: Microbiological culture results were compared between 61 paired induced sputum and bronchoalveolar lavage fluid samples from young children with cystic fibrosis. The first cohort received no airway clearance and the second cohort received airway clearance. Induced sputum was sampled within 7 days of bronchoscopy. RESULTS: Median age (range) of participants was 3.3 years (0.9-6.7). Sensitivity and specificity (95%CI) of induced sputum was 36.8% (16.3, 61.6), and 69% (52.9, 82.4), respectively (N = 61). In subgroup analysis, induced sputum with (N = 31) and without airway clearance (N = 30) demonstrated sensitivity of 50% (15.7, 84.3) and 27.3% (6.0, 61.0), respectively, and specificity of 60.9% (38.5, 80.3), and 78.9% (54.4, 93.9), respectively. CONCLUSION: Induced sputum is not highly sensitive or specific as routine surveillance for detection of lower airway pathogens in young children with cystic fibrosis. Pediatr Pulmonol. 2017;52:182-189. © 2016 Wiley Periodicals, Inc.

Early Lung Disease in Infants and Preschool Children with Cystic Fibrosis. What Have We Learned and What Should We Do about It?

The past decade has seen significant advances in understanding of the pathogenesis and progression of lung disease in cystic fibrosis (CF). Pulmonary inflammation, infection, and structural lung damage manifest very early in life and are prevalent among preschool children and infants, often in the absence of symptoms or signs. Early childhood represents a pivotal period amenable to intervention strategies that could delay or prevent the onset of lung damage and alter the longer-term clinical trajectory for individuals with CF. This review summarizes what we have learned about early lung disease in children with CF and discusses the implications for future clinical practice and research.

Interstitial lung disease in infancy: A general approach.

Childhood Interstitial lung disease (chILD) is an umbrella term used to define a broad range of rare, diffuse pulmonary disorders with altered interstitial structure that leads to abnormal gas exchange. Presentation of chILD in infancy can be difficult to differentiate from other common causes of diffuse lung disease. This article aimed at paediatricians provides an overview of interstitial lung disease presenting in infancy and includes key clinical features, a suggested approach to investigation and a summary of management. An overview of three clinical cases has been included to demonstrate the diagnostic approach, characteristic investigation findings and varied clinical outcomes.

Parental Experience of Information and Education Processes Following Diagnosis of Their Infant With Cystic Fibrosis Via Newborn Screening.

UNLABELLED: Following diagnosis with cystic fibrosis (CF), initial education powerfully influences parental adjustment and engagement with care teams. This study explored the education needs of ten parents following their infant's diagnosis with CF via newborn screening. DESIGN AND METHODS: Phenomenological study using van Manen's approach, with ten participant parents of children 1-8 years with CF. RESULTS: Parents recounted varying degrees of coping with information they acknowledged as overwhelming and difficult. For some it was too much too soon, while others sought such clarity to put CF into context. CONCLUSIONS: Participants delivered insight into their engagement with their education about CF. Their recommendations for appropriate context, content, format and timing of delivery enable development of education that is accurate and relevant.

Psychosocial characteristics and predictors of health-care use in families of young children with cystic fibrosis in Western Australia.

OBJECTIVE: Early childhood psychosocial experiences determine future health and health-care use. Identifying psychosocial predictors in cystic fibrosis may inform intervention strategies that can reduce health-care utilization. DESIGN: The study was designed as a prospective cohort study. SETTING: The study was set in the only cystic fibrosis clinic in Western Australia. PATIENTS: The patients were children up to 6 years diagnosed with cystic fibrosis in Western Australia between 2005 and 2011. MAIN OUTCOME MEASURES: Psychosocial data collected for each year of life were compared with Australian population data and analysed as predictors of annual hospital, emergency and outpatient visits. RESULTS: Compared with the Australian population, cystic fibrosis families demonstrated lower socio-economic status and labour supply (P < 0.001), increased residential mobility (P < 0.001) and trends towards increased rates of parental separation (P = 0.066). Marital discord and maternal and child psychological stress significantly predicted increased hospital admissions, emergency and outpatient visits. CONCLUSIONS: Social gradients may exist for families of young children with cystic fibrosis in Western Australia with potential implications for child health. Family psychological and relationship stress predicted increased child cystic fibrosis-related health-care use.

Protocol for a study of the psychosocial determinants of health in early childhood among children with cystic fibrosis.

AIMS: To investigate the causal associations between family relationships, family functioning, social circumstances and health outcomes in young children with cystic fibrosis. BACKGROUND: The anticipated health gains for patients with cystic fibrosis, promised by early diagnosis through newborn screening, have yet to be fully realized, despite advances in cystic fibrosis health care with aggressive management in multidisciplinary clinics and the development of specific medications. Adverse psychosocial functioning may underpin the current lack of progress as it is well recognized that compromised early parent-child attachment relationship experiences and adverse social circumstances have negative impacts on lifelong health status and health resource use, even in healthy children. DESIGN: A cross-sectional (initial) and longitudinal (progressive), multicentre study of children aged 3 months-6 years with cystic fibrosis, who have been diagnosed by newborn screening. METHODS: Questionnaire and observational measures of parent psychosocial functioning, parenting and parent-child attachment and social markers; and including clinical outcomes of regular health surveillance with clinical, lung imaging (computerized tomography) and bronchoalveolar lavage for airway microbiology and inflammation. CONCLUSION: This will be the first study to investigate the causal effect of psychosocial functioning, parenting and attachment on physical health outcome measures in children with cystic fibrosis.

Inflammatory responses to individual microorganisms in the lungs of children with cystic fibrosis.

BACKGROUND: We hypothesized that the inflammatory response in the lungs of children with cystic fibrosis (CF) would vary with the type of infecting organism, being greatest with Pseudomonas aeruginosa and Staphylococcus aureus. METHODS: A microbiological surveillance program based on annual bronchoalveolar lavage (BAL) collected fluid for culture and assessment of inflammation was conducted. Primary analyses compared inflammation in samples that grew a single organism with uninfected samples in cross-sectional and longitudinal analyses. RESULTS: Results were available for 653 samples from 215 children with CF aged 24 days to 7 years. A single agent was associated with pulmonary infection (≥10(5) cfu/mL) in 67 BAL samples, with P. aeruginosa (n = 25), S. aureus (n = 17), and Aspergillus species (n = 19) being the most common. These microorganisms were associated with increased levels of inflammation, with P. aeruginosa being the most proinflammatory. Mixed oral flora (MOF) alone was isolated from 165 BAL samples from 112 patients, with 97 of these samples having a bacterial density ≥10(5) cfu/mL, and was associated with increased pulmonary inflammation (P < .001). For patients with current, but not past, infections there was an association with a greater inflammatory response, compared with those who were never infected (P < .05). However, previous infection with S. aureus was associated with a greater inflammatory response in subsequent BAL. CONCLUSIONS: Pulmonary infection with P. aeruginosa, S. aureus, or Aspergillus species and growth of MOF was associated with significant inflammatory responses in young children with CF. Our data support the use of specific surveillance and eradication programs for these organisms. The inflammatory response to MOF requires additional investigation.

Value of serology in predicting Pseudomonas aeruginosa infection in young children with cystic fibrosis.

BACKGROUND: Early detection of Pseudomonas aeruginosa is essential for successful eradication. The accuracy of serum antibodies against specific and multiple P aeruginosa antigens at predicting lower airway infection in young children with cystic fibrosis (CF) was investigated. METHODS: A commercial P aeruginosa multiple antigen (MAg) ELISA and an in-house exotoxin A (ExoA) ELISA were compared in two populations: a discovery population of 76 children (0.1-7.1 years) undergoing annual bronchoalveolar lavage (BAL)-based microbiological surveillance and a test population of 55 children (0.1-5.6 years) participating in the Australasian CF Bronchoalveolar Lavage Trial. RESULTS: In the discovery population, P aeruginosa was cultured from BAL fluid (≥10(5) colony-forming units (cfu)/ml) in 15/76 (19.7%) children (median age 1.88 years). Positive MAg and ExoA serological results were found in 38 (50.0%) and 30 (39.5%) children, respectively. Positive (PPV) and negative (NPV) predictive values for serology at diagnosing P aeruginosa infection (≥10(5) cfu/ml) were 0.14 and 0.99 respectively (MAg assay) and 0.11 and 0.98 (ExoA assay). In the test population, P aeruginosa was cultured from BAL fluid (≥10(5) cfu/ml) in 16/55 (29.1%) children (median age 1.86 years) and from oropharyngeal swabs in 32/36 (88.9%). Positive MAg and ExoA serology was detected in 19 (34.5%) and 33 (60.0%) children, respectively. The PPV and NPV of serology were 0.26 and 0.94 respectively (MAg assay) and 0.19 and 0.98 (ExoA assay) and were marginally higher for oropharyngeal cultures. CONCLUSIONS: Measuring serum antibody responses against P aeruginosa is of limited value for detecting early P aeruginosa infection in young children with CF.