Search

Aim: Despite a recent increase in the use of ventilators in the home setting for children with chronic respiratory failure, there is currently no unified approach for clinical decision-making for children requiring long-term mechanical ventilation. The purpose of this study is to understand the clinician's perspective on decision-making around home ventilation for children, and how home-based care contributes to successful outcomes in this population., Methods: We recruited physicians and home ventilation nurses with at least 2 years' experience working in an Australian paediatric tertiary home ventilation service using professional networks and snowball sampling. Semi-structured interviews were conducted by two researchers between February 2019 and June 2020. Interviews were audio-recorded, transcribed, and analysed using inductive content analysis., Results: Twenty-five individuals participated (17 physicians and 8 home ventilation nurses). Participants viewed themselves as impartial medical advocates in the decision-making process, believing the decision to initiate or cease ventilation belonged to the child's family. While participants held the child's quality of life as the cornerstone of decision-making, quality of life was subjective and family specific., Conclusion: These findings provide insight into how clinicians working with children with chronic respiratory insufficiency approach the decision to introduce home-based ventilation. By understanding their role, strategies can be developed to assist them, leading to better outcomes for patients and families. Further research is needed to compare the perspectives of clinicians with the experience of ventilator-assisted children and families in Australia., (© 2023 The Authors. Journal of Paediatrics and Child Health published by John Wiley & Sons Australia, Ltd on behalf of Paediatrics and Child Health Division (The Royal Australasian College of Physicians).)

The handshake is a common greeting, parting gesture or deal sealer across the globe. Its origins may go back to pre-history, ancestorial primates using similar gestures. The handshake is cultural norm, most commonly shared between adults, which children learn as they grow up. There are many important aspects to the handshake that can promote the clinician-patient relationship, but how clinicians caring for children should use the handshake has not received attention. This paper explores the origins of the handshake and its closely allied gesture, the high-five, and considers how they can work to promote the care of children., (© 2021 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).)

Relief of suffering is an important goal of medicine and aligns with the professional maxim of 'do no harm' and the bioethical principle of non-maleficence. Capturing what individuals experience or third-parties sense in terms of suffering is difficult, made harder in the patient who is too young or not able to tell us. This paper builds on the thoughts and experience of Isaacs and Preisz who open a discussion on suffering at the end of life. The discussion is extended by Tobin who recommends the use of goals of care to try to align clinician and parent expectations of what suffering might mean at the end of life. A further paper by Brancatisano makes a comment that family resource and parental suffering might inappropriately guide parental decision-making at the end of life in cases where suffering is apparent. In my piece, I add my concerns that physicians can write their own narrative about suffering which can compete against the parent's view. Furthermore, suffering can be used as a weapon for physicians to pressure parents towards the medical view., (© 2021 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).)

Technological advances in mechanical ventilation have made home care possible for children requiring long-term ventilation. However, there are ethical and logistical challenges to transitioning home. The aim of this narrative review is to identify the experiences of the children and their families and the decision-making process to embark on home mechanical ventilation. A systematic review of the literature using Medline and OVID databases was conducted. Children <18 years requiring non-invasive ventilation or tracheostomy with ventilation or continuous positive pressure ventilation were included. The initial search yielded 1351 results, 1017 after duplications were removed and 111 after abstracts were reviewed. After applying inclusion and exclusion criteria to full text analysis, 48 references were analysed. The children and families described home ventilation as facilitating better health and functional status. However, they concurrently described social isolation, stigma, financial stress and loss of independence. The decision-making process to embark on home ventilation needs more research., (© 2021 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).)

Our culture has a rich history of fairy tales, folklore and literature. These have all served a purpose, often to entertain, but also to moralise. Authors have often included interesting characters with identifiable medical conditions or described interesting characters that lend their names to conditions we can identify today. This paper looks at the medical conditions found in fairy tales, folklore and literature., (© 2019 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).)

The treatment of Mycobacterium abscessus complex (MABSC) pulmonary infections is an emerging challenge in patients with cystic fibrosis (CF). Multidrug therapy for prolonged durations is required and carries the significant burden of drug-related toxicity, cost and selective pressure for multiresistant bacteria. International guidelines acknowledge that clinical and in vitro data to support treatment regimens are limited, particularly in children. As part of a collaboration between the infectious diseases and respiratory units at our institution, we have developed a modified treatment guideline that aims to balance the aims of MABSC eradication and slowing disease progression with minimising drug toxicity and resistance. The outcomes of this treatment approach will be monitored and reported. In this manuscript, we discuss the available evidence for treatment choices and present our treatment guideline for paediatric patients with CF and MABSC infection., (© 2019 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).)

There have been dramatic changes in the care of children with cystic fibrosis (CF) from 1965 to 2015. The initial improvements were the result of incremental gains in medical knowledge and better use of available therapies. Some CF-specific drugs were developed (recombinant human DNase and purified tobramycin for inhalation), but these simply delayed the progression of the lung damage. The discovery of the CF transmembrane conductance regulator gene in 1989 created an explosion of knowledge of airway biology and CF pathophysiology. Now mutation-specific therapy to potentiate mutated CF transmembrane conductance regulator is available for some patients with CF and is transforming their lives. Further advances are eagerly anticipated to benefit all patients with CF., (© 2016 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).)

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., (© 2015 The Authors. Journal of Paediatrics and Child Health © 2015 Paediatrics and Child Health Division (Royal Australasian College of Physicians).)

Aim: Mechanical in-exsufflation (MI-E) augments the weakened cough of patients with neuromuscular disease (NMD), clearing secretions and overcoming atelectasis. Little has been published on the impact of MI-E alone on rates of hospitalisation and quality of life (QOL). The aim of this study was to assess the impact of home MI-E on hospital admissions and life-style in children with NMD., Methods: A retrospective chart review was performed on children using MI-E, including data on the number of admissions to hospital, length of stay and hours of ventilation. A parental survey was used to gather information on the impact of MI-E on life-style for the child and family., Results: Ten children with NMD (seven spinal muscular atrophy, two Duchenne muscular dystrophy and one centronuclear myopathy) using MI-E at home were identified. MI-E use commenced at mean age of 8.5 years (range 1.1-16.9) with 1.4 years of use (range 0.3-3.8). MI-E pressures ranged from +/-30 to 40 cmH2 O with no complications reported. There was a significant reduction in hospital days at 6 (P = 0.036) and 12 (P = 0.028) months following commencement of home MI-E compared with the same period preceding MI-E use. The survey highlighted positive benefits of MI-E use, in particular the ability to treat many pulmonary exacerbations at home., Conclusions: Home MI-E use by children with NMD can reduce hospitalisation and benefit families by maintaining their child at home., (© 2013 The Authors. Journal of Paediatrics and Child Health © 2013 Paediatrics and Child Health Division (Royal Australasian College of Physicians).)

Aim: The Australian Immunisation Handbook (ninth edition) recommends children with cystic fibrosis (CF) receive routine scheduled immunisations plus annual influenza vaccine and an additional pneumococcal vaccine at both 12 months and 4-5 years. Adherence with these recommendations is unknown. This study aimed to determine the immunisation status of children with CF attending the Royal Children's Hospital (RCH), Melbourne., Methods: A retrospective audit of children with CF aged 6 months to 7 years (at 1 January 2008) was performed on the RCH CF outpatient clinic database. The Australian Childhood Immunisation Register (ACIR) and RCH Immunisation Service records were used to determine immunisation status., Results: Eighty-nine children with CF were identified, 52 (58%) were male, with median age of 3.6 years. Eighty-two of 89 children (92%) were up to date with routine immunisations. According to the ACIR, of the 89 children sampled, 24 (27%) were given influenza vaccine in accordance with recommendations in 2007. Eighty children were older than 12 months of age at 1 January 2008 and 17 (21%) of these children had received the additional pneumococcal vaccine at 12 months. Thirty-eight children were older than 4 years of age at 1 January 2008 and 6 (16%) received the recommended 23vPPV booster., Conclusion: Most children with CF received their routine childhood immunisations but not the additional recommended immunisations. This highlights the potential value of the ACIR to accurately record all vaccines administered and for ACIR reminder letters to include the additional vaccines for children in special-risk groups., (© 2010 The Authors. Journal of Paediatrics and Child Health © 2010 Paediatrics and Child Health Division (Royal Australasian College of Physicians).)

Aim: To describe a respiratory support programme for children at home by parents and layperson carers., Methods: Analysis of records of children with long-term mechanical respiratory support at home., Results: From 1979 to 2008 the programme managed 168 children (median age 7 years, range 3 weeks-19 years) with obstructive sleep apnoea (55, 32%), neuromuscular conditions (42, 25%), tracheo-bronchomalacia (23, 14%), kyphoscoliosis-cerebral palsy (20, 12%), acquired central hypoventilation (8, 5%), congenital central hypoventilation (7, 4%), chronic lung disease or pulmonary hypoplasia (8, 5%), traumatic quadriplegia (3, 2%) and tumour-related quadriplegia (2, 1%). One hundred and sixty-one (96%) were discharged: 73 (46%) remain in the programme; 27 (16%) transferred to adult services, 25 (15%) recovered and 36 (23%) died. Principal modes of therapy were mask continuous positive airway pressure (CPAP) 35%, mask bilevel positive airway pressure 30%, tracheostomy CPAP 20%, tracheostomy mechanical ventilation 8%, phrenic nerve pacing 3%, negative pressure chamber ventilation 2% and nasal tube CPAP 2%. Two unexpected deaths occurred at home: one from accidental tracheostomy decannulation and another unrelated to respiratory support. Average time in the programme was 3.3 years. Parents of 69 children were provided with trained carers. Successful discharge resulted from early recognition of potential to discharge, parental training, recruitment and training of carers, purchase of equipment and secure funding. Seven children were not discharged, two of whom died in the hospital and five are subject to discharge planning., Conclusion: Respiratory support of children at home by trained parents and layperson carers is safe and efficient. All modes of respiratory support may be used.

Aims: The inspired oxygen concentration (FiO(2)) is an important criterion for assessing the severity of bronchiolitis. Oxygen delivery by nasal prongs is a measure of oxygen flow, but not FiO(2). We aimed to determine whether FiO(2) of oxygen delivered by nasal prongs could be predicted from nasal flow by relating arterial oxygen concentrations achieved with prongs to those achieved via head box in children with bronchiolitis., Methods: This is a pilot study conducted at a tertiary referral paediatric hospital. We studied hospitalised children less than 24 months old requiring supplemental oxygen because of bronchiolitis, an acute viral lower respiratory tract infection. Children admitted to the intensive care unit, and those with congenital cardiac disease or recent bronchodilator use were excluded. Subjects were studied in nasal prong, then head box oxygen. Arterial oxygen concentration was measured by a transcutaneous probe (tcPO(2)). Oxygen flows by nasal prongs and FiO(2) by head box were adjusted to achieve similar tcPO(2) readings. FiO(2) values were plotted against oxygen flow rates based on matching tcPO(2)., Results: We recorded tcPO(2) across a satisfactory range of values in eight children. TcPO(2) increased with increasing FiO(2) and nasal oxygen flow, but at variable rates between subjects. FiO(2) increased with increasing nasal oxygen flow, but this relationship was highly variable., Conclusions: In this study, it was not possible to estimate FiO(2) reliably from nasal oxygen flow rates in children with bronchiolitis. Nasal prong oxygen flow rates should be used with caution when assessing the severity of bronchiolitis in children.

Most babies with cystic fibrosis (CF) are born to parents who did not know they were carriers until their baby was diagnosed with CF, usually by newborn screening. It is only after the birth of their first child with CF that couples are offered genetic counselling and reproductive choices. Most use this information for prenatal testing of subsequent pregnancies. With the high uptake of first trimester screening for Down syndrome (80% in Victoria) most couples have had screening during the CF affected pregnancy. Yet screening for CF carrier status is available, costs are similar to that for Down syndrome screening and CF carrier screening only ever needs to be done once. Waiting for couples to have a baby with CF before they are identified as carriers denies them choice. A national policy on CF carrier screening in Australia, and determination to equitably fund such a programme, is required.

Aim: Once-daily tobramycin in patients with cystic fibrosis (CF) is a more convenient dosing regimen than thrice daily dosing. There are limited data on the pharmacokinetic (PK) profile for once-daily tobramycin in patients with CF. The aim of this study was to define the PK parameters for once-daily tobramycin in children with CF and develop an algorithm for therapeutic drug monitoring dosing., Methods: CF patients admitted to hospital were commenced on once-daily intravenous tobramycin (12 mg/kg/day) and ticarcillin/clavulinic acid. Serum tobramycin levels were taken at 30 min, 2-4 h and 12 h post dose. Data points for the PK model included: age, sex, weight, tobramycin dose, time of tobramycin doses and levels, tobramycin levels. WinNonMix was used to obtain the PK parameters., Results: Forty-four children with 86 admissions who were aged 9 months-20 years were included. A one-compartment intravenous infusion model with first order elimination kinetics produced the best model. Population parameters were: volume of distribution (V(d)) = 0.267 L/kg (95% confidence interval (CL) 0.260-0.272), clearance (CL) 0.103 L/kg/h (95% CI 0.098-0.107) and half-life (t(1/2)) 1.82 (95% CI 1.77-1.88) h. Once the population model was established post hoc analysis was used to calculate individual subject predictions. Plots of individual prediction curves agreed well with observed values., Conclusion: This study has established an algorithm for routine monitoring of once-daily tobramycin in children with CF. Satisfactory serum levels of tobramycin were obtained with a dose of 12 mg/kg/day and a regimen algorithm that uses only one measurement to monitor the plasma concentration is suggested.

Aim: To investigate parental attitudes to cystic fibrosis (CF) carrier detection of their infant by newborn screening (NBS)., Methods: Data were collected from a postal questionnaire sent to parents of infants identified as CF carriers by NBS in 1996-1997 (inclusive) and 2001 in Victoria, Australia (n = 66)., Results: Almost all parents remembered their child being identified as a CF carrier (97%: 1996/1997; 100%: 2001); yet the majority were unaware at the time that NBS could detect carriers (70%: 1996/1997; 49%: 2001). More parents in the later cohort reported having carrier testing compared with the earlier cohort (85% and 53% respectively) but recall was more uncertain in the earlier cohort when validated against health records. Cascade testing was not utilised frequently by other family members in either cohort. Residual risk of being a carrier if testing was negative was not well understood by parents. Some parents (28%: 1996/1997; 18%: 2001) had residual anxiety about the current health of their carrier child and their future reproductive decision making. Most parents were satisfied with the information provided to them at the time of the sweat test. Few differences were seen between the cohorts., Conclusion: Although the NBS process for CF in Victoria is working efficiently for the majority of families whose infant is identified as a carrier there are areas that can be improved. We recommend that greater attention should be given to informing parents that a consequence of NBS is CF carrier detection and strategies to improve utilisation of cascade testing should be developed.

A 2-week-old female infant was transferred from a regional hospital for mechanical ventilation after developing severe respiratory distress. Stridor had been present since the age of 1 week and was complicated by coryzal illness. Mechanical ventilation was difficult with marked inspiratory and expiratory flow obstruction recorded by the ventilator. Echocardiogram showed a normal heart. Flexible bronchoscopy revealed mid-tracheal extrinsic compression (unchanged with positive end-expiratory pressure) and advancement of the endotracheal tube by 2 cm completely corrected the flow obstruction. Repeat echocardiogram showed a double aortic arch. This case report emphasizes the importance of clinical history, examination findings and interpretation of the ventilator waveforms in the differential diagnosis of a difficult-to-ventilate infant with bronchiolitis.