The changing epidemiology of pulmonary infection in children and adolescents with cystic fibrosis: an 18-year experience.

The impact of evolving treatment regimens, airway clearance strategies, and antibiotic combinations on the incidence and prevalence of respiratory infection in cystic fibrosis (CF) in children and adolescents remains unclear. The incidence, prevalence, and prescription trends from 2002 to 2019 with 18,339 airway samples were analysed. Staphylococcus aureus [- 3.86% (95% CI - 5.28-2.43)] showed the largest annual decline in incidence, followed by Haemophilus influenzae [- 3.46% (95% CI - 4.95-1.96)] and Pseudomonas aeruginosa [- 2.80%95% CI (- 4.26-1.34)]. Non-tuberculous mycobacteria and Burkholderia cepacia showed a non-significant increase in incidence. A similar pattern of change in prevalence was observed. No change in trend was observed in infants < 2 years of age. The mean age of the first isolation of S. aureus (p < 0.001), P. aeruginosa (p < 0.001), H. influenza (p < 0.001), Serratia marcescens (p = 0.006) and Aspergillus fumigatus (p = 0.02) have increased. Nebulised amikacin (+ 3.09 ± 2.24 prescription/year, p = 0.003) and colistin (+ 1.95 ± 0.3 prescriptions/year, p = 0.032) were increasingly prescribed, while tobramycin (- 8.46 ± 4.7 prescriptions/year, p < 0.001) showed a decrease in prescription. Dornase alfa and hypertonic saline nebulisation prescription increased by 16.74 ± 4.1 prescriptions/year and 24 ± 4.6 prescriptions/year (p < 0.001). There is a shift in CF among respiratory pathogens and prescriptions which reflects the evolution of cystic fibrosis treatment strategies over time.

A randomized controlled trial evaluating the effectiveness of a self-management program for adolescents with a chronic condition: a study protocol.

BACKGROUND: Self-management support is increasingly viewed as an integral part of chronic condition management in adolescence. It is well recognized that markers of chronic illness control deteriorate during adolescence. Due to the increasing prevalence of long-term chronic health conditions in childhood and improved survival rates of previously life-limiting conditions in children and adolescents, significant numbers of adolescents are having to manage their chronic condition effectively as they transition to adult health care. Therapy adherence has been identified as a major challenge for young people living with a chronic condition such as cystic fibrosis, diabetes, or asthma requiring long-term pharmacological therapy and/or lifestyle modifications. Most systematic reviews on self-management interventions address adult populations. Very few intervention studies are directed at adolescents with a chronic condition who are transitioning to adult health services. This protocol describes a prospective randomized controlled trial of a standardized self-management intervention program delivered to adolescents aged 15-18 years prior to their transfer to adult care. This study has been designed to provide evidence regarding self-management programs for adolescents and is the first study to use the Flinders Program with this important, under-researched age group. METHODS: A randomized controlled trial is used to investigate the effectiveness of a modified adolescent-friendly version of an adult self-management program. This program is directed at improving self-management in an adolescent cohort 15-18 years of age with a chronic condition being treated in a specialist pediatric hospital. Participants will be randomized to either usual care or the modified Flinders Program plus usual care. Data collection will include measures of specific illness control, unscheduled hospital admissions, and questionnaires to record self-management competencies, quality of life, self-efficacy, and outcome measures specific to the chronic condition at baseline, 3 months, 6 months, and 12 months after delivery. DISCUSSION: This study will provide a better understanding of the elements required for effective self-management programs in adolescents with a chronic condition and address some important knowledge gaps in current literature. The study will be carried out in collaboration with the Discipline of Behavioural Health at Flinders University, Adelaide, Australia, in order to inform the development of an adolescent version of the successful and validated Flinders Program™. TRIAL REGISTRATION: Australian and New Zealand Clinical Trials Registry (ACTRN12621000390886). Registered on April 8, 2021.

Transition to adult care in cystic fibrosis: The challenges and the structure.

In developed countries, it is projected that there will be a 70% increase in the number of adults living with Cystic Fibrosis (CF) between 2010 and 2025. This shift in demographics highlights the importance of high-quality transition programmes with developmentally appropriate integrated health care services as the individual moves through adolescence to adulthood. Adolescents living with CF face additional and unique challenges that may have long-term impacts on their health, quality of life and life-expectancy. CF specific issues around socially challenging symptoms, body image, reproductive health and treatment burden differentiate people with CF from their peers and require clinicians to identify and address these issues during the transition process. This review provides an overview of the health, developmental and psychosocial challenges faced by individuals with CF, their guardians and health care teams considering the fundamental components and tools that are required to build a transition programme that can be tailored to suit individual CF clinics.

Effectiveness of self-management programmes for adolescents with a chronic illness: A systematic review.

AIM: To assess what is known about the effectiveness of face-to-face self-management programmes designed specifically for adolescents (10-19 years) with a chronic illness. DESIGN: A systematic review and synthesis without meta-analysis (SWiM). DATA SOURCES: Six international web-based reference libraries were searched with a date range of 1946 to July 2020. REVIEW METHOD: The PRISMA statement and SWiM guideline were used for reporting the methods and results. The PICO format was used to develop a focused clinical question and the eligibility criteria of our review. Quality assessment of the included studies was performed using the Cochrane Effective Practice Organisation of Care criteria. RESULTS: Eight studies (four randomized controlled trials and four descriptive designs) met the inclusion criteria and were published between 2003 and 2017. Results of the review: Three studies demonstrated measures of illness control which showed initial improvements in adherence as a result of the interventions but failed to demonstrate sustained adherence over time. Booster sessions were identified as an effective strategy to improve adherence, but were often omitted. CONCLUSIONS: There is a limited body of evidence on the effectiveness of self-management programmes specifically developed for adolescents with a chronic illness, an important but under researched area. Future research lies in the development of more rigorous studies that focus on quantitative outcome measures for evaluating the effectiveness of self-management programmes to guide the development of future programmes. IMPACT: It is crucial for adolescents with a chronic illness to develop independence and the self-management skills required to effectively manage their chronic condition as they transition to adulthood. On current evidence, in planning future self-management interventions should include booster sessions. Nurses with additional training and experience have a key role in supporting adolescents with a chronic illness to develop self-management skills as they assume responsibility for their own healthcare.

Intersectionality: Social Marginalisation and Self-Reported Health Status in Young People.

BACKGROUND: The aim of this study was to measure young people's health status and explore associations between health status and belonging to one or more socio-culturally marginalised group. METHODS: part of the Access 3 project, this cross-sectional survey of young people aged 12-24 years living in New South Wales, Australia, oversampled young people from one or more of the following groups: Aboriginal and or Torres Strait Islander; living in rural and remote areas; homeless; refugee; and/or, sexuality and/or gender diverse. This paper reports on findings pertaining to health status, presence of chronic health conditions, psychological distress, and wellbeing measures. RESULTS: 1416 participants completed the survey; 897 (63.3%) belonged to at least one marginalised group; 574 (40.5%) to one, 281 (19.8%) to two and 42 (3.0%) to three or four groups. Belonging to more marginalised groups was significantly associated with having more chronic health conditions (p = 0.001), a greater likelihood of high psychological distress (p = 0.001) and of illness or injury related absence from school or work (p < 0.05). CONCLUSIONS: increasing marginalisation is associated with decreasing health status. Using an intersectional lens can to be a useful way to understand disadvantage for young people belonging to multiple marginalised groups.

Digital psychosocial assessment: An efficient and effective screening tool.

AIM: A large proportion of young people with chronic health conditions are surviving into adulthood. They face the same challenges as their healthy peers and are at increased risk of mental health problems. Psychosocial assessment is a crucial aspect of clinical care. Interviews using the internationally used and accepted HEEADSSS (home environment, education/employment, eating, peer-related activities, drugs, sexuality, suicide/depression, and safety) framework require trained clinicians, rapid interpersonal engagement, time and manual documentation. HEEADSSS-derived digital self-report surveys can be initiated by non-trained staff. This study compares the utility and information recorded using both methods. METHODS: A retrospective analysis comparing documentation from HEEADSSS guided face-to-face interview and a digital survey tool was conducted using 146 records collected by the Trapeze transition service across the two locations of the Sydney Children's Hospital Network (NSW, Australia) between 2013 and 2016. A panel of four experts used an iterative process to identify 29 data verification points, falling into seven categories. Wilcoxon signed-rank tests were used to compare category scores. RESULTS: The digital survey took an average of 15 min and showed a significantly higher rate of disclosure across all psychosocial categories, particularly in the sensitive areas of emotions, drug use, sex and safety, compared to electronic medical record documentation of interview. CONCLUSIONS: Digital survey provided a time-efficient psychosocial screening tool that was self-administered, able to be introduced by non-trained staff, had a consistent record of responses, and elicited a substantially higher disclosure rate for important areas of strength and risk that may otherwise be avoided or not recorded.

Epidemiology of paediatric chronic fatigue syndrome in Australia.

OBJECTIVE: To estimate the paediatrician-diagnosed incidence of chronic fatigue syndrome (CFS) in Australia, and describe demographic and clinical features, as well as approaches to diagnosis and management. METHODS: The Australian Paediatric Surveillance Unit facilitates monthly national surveillance of uncommon conditions seen by paediatricians. Data from young people aged <18 years diagnosed with CFS were collected. Incidence was estimated based on new cases reported from April 2015 to April 2016. RESULTS: A total of 164 cases of newly diagnosed CFS in young people aged 4-17 years were identified for inclusion. The estimated national incidence for children aged 4-9 years was 0.25 per 100 000 per annum. In children aged 10-17 years, the estimated incidence of paediatrician-diagnosed cases for Victoria (17.48 per 100 000) was substantially greater than other Australian states (range 1.31-5.51 per 100 000). Most cases were female and Caucasian, most commonly presenting after an infectious illness with symptoms gradual in onset. The majority were diagnosed at least 13 months after symptom onset. Symptoms, associations, investigations and management strategies were highly variable. CONCLUSIONS: Current findings suggest that, consistent with other countries, the Australian incidence of CFS in children aged <10 years is very low. In contrast, the national incidence of CFS in older children and adolescents (aged 10-17 years) is more unclear, with marked variability between geographical regions apparent. This may be due to variation in service accessibility and clinician understanding of CFS. Accordingly, national initiatives to improve equity of care for children with CFS may be required.

Implementing a new adolescent epilepsy service: Improving patient experience and readiness for transition.

AIM: To implement and appraise a new model of care in terms of: patient experience, knowledge of epilepsy, readiness for transition and emotional and behavioural support in a new purpose-built facility for adolescents and young adults. METHODS: The new model of care included: upskilling of neurology staff in adolescent engagement and provision of group education sessions on epilepsy and mental health (MH), along with MH support, in a new purpose-built adolescent facility. Parameters examined pre- and post-attendance at the new clinic included: adolescent experience of service delivery, transition readiness, emotional and behavioural well-being, epilepsy knowledge and medication adherence. RESULTS: A total of 45 adolescents (mean age 15.7 years) attended the new epilepsy clinic between February 2017 and December 2017. Adolescents felt significantly better informed following education in relation to epilepsy and driving, alcohol/street drugs and birth control/pregnancy. There was no significant improvement in self-reported medication adherence, transition readiness or mental well-being at follow-up. While MH education was ranked highly in terms of importance by adolescents and parents at baseline, attendance at MH education and engagement with MH support was low. CONCLUSIONS: This paper documents what is important to young people with epilepsy regarding service delivery. The new adolescent service was well received. Based on feedback from adolescents and parents relating to the service, and the suboptimal uptake of MH supports, the model of care has been revised to reduce attendance burden on families and improve patient experience.

Smoking Cessation in Adolescents: targeted approaches that work.

Smoking Cessation in adolescents can be considered in a developmental context to enable the clinician to individualise the appropriate assessment and management of the young person they are seeing whether it is in a primary or tertiary care setting. Adolescence is a time of rapid neurocognitive and hormonal change with these factors affected by personality and behavioural factors as well as family, cultural and psychosocial context. Adolescents are uniquely vulnerable to smoking initiation and nicotine addiction throughout these years. Increased awareness of the risks of smoking and using opportunities to assess and intervene regarding smoking cessation are integral to clinical practice for all clinicians seeing young people. This review will discuss the demographics of adolescent smoking, risk factors, assessing smoking and nicotine addiction, the importance of brief interventions, the evidence base for appropriate interventions, particularly in high risk groups and will emphasise innovative training for health professionals in adolescent smoking cessation.

Family functioning as a protective factor in treating adolescents with complex medico-psychosocial presentations.

PURPOSE: Studies of adolescents with somatic symptoms with no identified organic cause make a clear connection between the health and wellbeing of the adolescent and the functioning of the family. There has been little systematic examination of the role of family functioning in treatment. We aim to determine whether there was an association between family functioning and treatment outcome in this 12 month prospective study. METHODS: Fifty adolescents aged between 12 and 17 years (M=14.4, SD=1.32) and their parents were recruited. All adolescents presented with one or more physical symptoms as their primary complaint. Adolescent functioning and family relationships were measured at recruitment, 4 months into treatment and 12 months later. Generalised estimating equations (GEE) analyses were conducted to examine the relationships between the variables of interest. RESULTS: Results suggested significant improvements in adolescent outcomes between baseline and 4 months (all p<0.01), which were sustained to 12 months (all p<0.01). Adolescents from healthier families at initial presentation had significantly improved psychosocial functioning over the course of the study (all p<0.001). CONCLUSION: Our findings highlight the importance of taking a family approach to the treatment of adolescents with medically unexplained symptoms.

How is paediatric chronic fatigue syndrome/myalgic encephalomyelitis diagnosed and managed by paediatricians? An Australian Paediatric Research Network Study.

AIM: The diagnosis and management of paediatric chronic fatigue syndrome/myalgic encepnalomyelitis (CFS/ME) represent ongoing challenges for paediatricians. A better understanding of current approaches at a national level is important in informing where research and education could improve treatment outcomes. We aimed to examine current diagnosis and management practices for CFS/ME by Australian paediatricians. METHOD: An online survey was sent to members of the Australian Paediatric Research Network. The primary outcomes of interest included diagnostic criteria used, medical investigations and management practices in paediatric CFS/ME. RESULTS: One hundred seventy-eight (41%) of 430 eligible paediatricians responded, with 70 of the 178 (39%) reporting that they diagnose and manage CFS/ME as part of their practice. Medical investigations used for diagnosis were variable. Conditions that more than half of the paediatricians reported as commonly co-occurring (i.e. present in >50% of cases) included somatisation disorders, anxiety, depression and fibromyalgia. There was wide variation in behavioural and pharmacological management strategies but most paediatricians commonly engaged a school teacher, physiotherapist and/or psychologist as part of their management. CONCLUSION: The diagnostic and management practices of paediatricians for CFS/ME within Australia vary widely. This likely reflects a paucity of paediatric-specific guidelines, together with limited evidence to guide best practice and limited training in this area. There is a need for guidance and education for the diagnosis and management of paediatric CFS/ME in Australia.

Addressing adolescent substance use in a paediatric health-care setting.

AIM: The aim of this study is to review the operation of a specialist adolescent drug and alcohol consultation liaison service in a tertiary paediatric hospital. METHOD: A retrospective review of patient records was conducted to identify patient characteristics and assess service utilisation. RESULTS: Two hundred adolescents were referred over 4 years. Most presented during mid-adolescence (14-16 years). Alcohol, cannabis and nicotine were the most frequently reported substances, and almost half of referrals involved polysubstance use. Mental health diagnoses and behavioural problems were commonly reported. Almost two-thirds (63.5%) attended an appointment for drug and alcohol assessment and intervention (n = 92) or were referred to appropriate services (n = 35). Adolescents more likely to engage and attend an appointment with the specialist adolescent addiction medicine service included those with amphetamine use, polysubstance use, chronic illness, any mental health diagnosis and mood disorder. Indigenous Australians and those with a history of aggression were more difficult to engage. CONCLUSIONS: Adolescents present to paediatric health settings with drug- and alcohol-related issues, including associated harms. These comprise, but are not limited to, physical and sexual assault, family conflict, mood and behavioural concerns (including psychosis), and forensic issues. Early intervention aims to reduce long-term risks such as dependence in adulthood. Specialist adolescent drug and alcohol services may assist in identifying and engaging these high-risk and often complex young people in developmentally appropriate treatment.

Adolescent chronic fatigue syndrome and somatoform disorders: a prospective clinical study.

AIM: To examine and compare the presenting characteristics and the change in the physical and psychosocial functioning of adolescents with chronic fatigue syndrome (CFS) or somatoform disorders who have received an adaptable multidisciplinary intervention over a 12-month period. METHODS: Fifty adolescents presenting to the Complex Adolescent Clinic at The Children's Hospital at Westmead, Sydney, Australia were assessed. Their physical and psychosocial functioning was rated by the adolescents and their parents using the Child Health Questionnaire. Participants were assessed at baseline, 4 months and 12 months after initiating treatment. Analyses examined whether diagnosis and/or illness precipitants were related to treatment outcome. RESULTS: Adolescents with both CFS and somatoform disorders demonstrated improvement in physical and psychosocial functioning over the first 4 months of treatment, sustained at 12-month follow-up. A diagnosis of CFS was associated with poorer physical functioning over time and a trend towards a longer illness time course compared with somatoform disorder. Adjustment for a physical precipitant reduced the association between diagnosis and physical functioning. Those who had a physical precipitant to their illness had significantly poorer physical functioning over time than those who did not, regardless of diagnostic category. Diagnosis and physical precipitant were not associated with psychosocial functioning. CONCLUSIONS: Improvement in adolescent physical and psychosocial functioning over time suggests that a multidisciplinary treatment model may be effective for varied complex medico-psychosocial presentations, irrespective of diagnosis and illness precipitant. Illness precipitant may have a greater influence on treatment outcome than diagnostic category.

Adolescent and young adult medicine is a special and specific area of medical practice.

Adolescent and young adult medicine is a concept that has gained traction in the last decade or so. The medical literature has come primarily from oncology. Advances in neuroscience that document continuing brain development into the third decade, and research that shows risk behaviours associated with adolescence both remain and may increase in the third decade, have been two of the drivers in the conversation around linking these two age groups together as a medical practice group. A third driver of importance is transition care in chronic illness, where older adolescents and young adults continue to have difficulties making effective linkages with adult care. The case for specific training in adolescent and young adult medicine, including the developmental concepts behind it, the benefits of the delineation and the particular challenges in the Australian health-care system, are discussed. On balance, there is a strong case for managing the health issues of adolescents and young adults together. This scenario does not fit easily with the age demarcations that are in place in acute care facilities. However, this is less the case in community services and can work in focused private practice. Such a situation suggests that both paediatric and adult physicians might be interested in adolescent and young adult medicine training and practice.

Transition of adolescents with cystic fibrosis from paediatric to adult care.

Transition to adult health care for adolescents with complex chronic illness such as cystic fibrosis (CF) is a challenge for the health care system with increasing numbers of young people requiring lifelong adult health care due to improvements in early diagnosis, treatment and survival. The changing face of CF and current guidelines for transition to adult care will be reviewed with a discussion and case study illustrating effective transition. Key features include (i) early preparation, planning and facilitating self-management skills; (ii) engaging all involved by a coordinated approach, including young people with CF, their families and the paediatric and adult teams; (iii) detailed communication, including comprehensive written referral report and documentation of prior complications of CF; (iv) feedback between the paediatric and the adult health care teams; and (v) ongoing audit of the transition process. The barriers to effective transition will be examined in the context of challenges faced by the paediatric centres, the development and resourcing of appropriate adult services, difficulties for the young person and their family as well as integrating the health care system overall. While acknowledging the development and evaluation of models of care for transition services with CF, continued evaluation of transition services can provide an evidence base to ensure effective systems with allocation of resources, inform training of health professionals and meet the needs of young people with CF and other chronic illnesses as they navigate the health care system.

Contraception, communication and counseling for sexuality and reproductive health in adolescents and young adults with CF.

With survival now into the fourth decade and rapid growth of the adolescent and adult population of people with cystic fibrosis CF sexual and reproductive health issues are integral to the management of adolescents and adults with CF. Education and counseling for sexual health related issues must be included in the daily routine of CF care. With advances in genetic counseling, contraception, assisted reproductive technology and collaborative management adolescents and young adults with CF realizing their sexual and reproductive potentials safely and realistically can be possible .

Diagnosis and management of asthma in adolescents.

OBJECTIVES: In this review we explore some of the issues surrounding the diagnosis and misdiagnosis of asthma in adolescents and suggest a management approach which might facilitate the provision of optimal treatment in order to minimise morbidity from asthma in this vulnerable and often difficult-to-manage age group. RESULTS: We highlight important diagnostic traps which occur in the adolescent age group, including the misdiagnosis of asthma in young people presenting with exercise-related symptoms or cough, and stress the importance of considering alternative diagnoses, including vocal cord dysfunction. We explore how adolescence impacts on asthma management and emphasise the importance of an understanding of normal adolescent development and an awareness of high-risk indicators in developing a strategy to optimally manage a young person with asthma. We also illustrate how psychosocial functioning may impact on both perceived asthma severity and quality of life as well as interfere with optimal asthma control both directly and by increasing non-adherence. A suggested management strategy for adolescents is provided which emphasises a supportive approach to help facilitate optimal control of asthma by involving the young person in management decisions about their asthma. Specific mention is made about smoking cessation assistance and the interrelationship between physical activity, obesity and asthma. Finally we discuss the importance of an appropriate transition process to prepare the young person for transfer from child-centred care to adult-centred care. CONCLUSION: Many different factors is of outmost importance when diagnosing adolescents with shortness of breath; furthermore, management of asthma need a supportive approach in both paediatric and adult setting.