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Background Likely duration of survival of children described as having cerebral palsy is of considerable interest to individuals with cerebral palsy, their families, carers, health professionals, health economists and insurers. The aim of this paper is to describe patterns of survival and mortality to the sixth decade in a geographically defined population of people with cerebral palsy stratified according to the clinical description of their impairments in early childhood. Methods Identifiers of persons born in Western Australia 1956–2011, registered with cerebral palsy on the Western Australian Register of Developmental Anomalies and surviving at least 12 months, were linked to the Australian National Death Index in December 2014. Patterns of mortality were investigated using survival analysis methods. Results Of 3185 eligible persons, 436 (13.7%) had died. Of that sample the 22% with the mildest impairment had survival patterns similar to the general population. Mortality increased with increasing severity of impairment. Of 349 (75%) with available cause of death data, 58.6% were attributed to respiratory causes, including 171 (49%) to pneumonia at a mean age of 14.6 (sd 13.4) years of which 77 (45%) were attributed to aspiration. For the most severely impaired, early childhood mortality increased in succeeding decades of birth cohorts from 1950s to 1990 with 20% dying by 4 years of age in the 1981–1990 birth cohort; it then decreased for subsequent birth cohorts, 20% mortality not being attained until 15 years of age. However by 20 years of age mortality of the most severely impaired born in the 1991–2000 birth cohort exceeded that of all other birth cohorts. Remaining life expectancies by age to 50 years have been estimated for two strata with more severe impairments. Conclusion For 22% of individuals with cerebral palsy with mild impairment survival to 58 years is similar to that of the general population. Since 1990 mortality for those with severe cerebral palsy in Western Australia has tended to shift from childhood to early adulthood.

Background Appropriate support for the health of children with an intellectual disability by parents and healthcare professionals is pivotal, given the high risk of chronic conditions. However, there is limited research that has collected important insights from parents on their learnings for supporting their child's evolving healthcare needs. Aim This study focuses on parents' experiences and learnings from managing and supporting the health of their child with intellectual disability. It aims to understand what parents wish they had known earlier, the essential knowledge and skills they needed to manage their child's health. Method A qualitative study was carried out using semi‐structured interviews with 21 parents of adolescents and young people with intellectual disability. The children had chronic health conditions that fell into six health domains, including (1) difficulties with movement and physical activity, (2) epilepsy, (3) dental care, (4) respiratory health and infection, (5) behaviour, mental health or sleep and (6) gastrointestinal health. Results Thematic analysis yielded five themes: (1) optimising mutual engagement between healthcare professionals and families; (2) planning and practising effective healthcare; (3) having the right information at the right time; (4) finding the support that was needed and (5) navigating healthcare and disability systems. Over time, parents developed specific skills and knowledge for managing their child's health effectively. Some parents expressed regret for not seeking support and information about their child's health conditions earlier. Parents described how mutual engagement between healthcare professionals and parents optimised the management of their child's healthcare. Conclusion The study found that managing the health of a child with intellectual disability is complex. The themes were consistent across health comorbidities, indicating important common experiences. The themes aligned with conceptualisations of health literacy, suggesting that improving health literacy skills can help parents better manage their children's health conditions. Patient or Public Contribution We developed the project in consultation with members of the public who have lived experience of parenting a child with intellectual disability. They commented on the study aims, interview schedule, participant recruitment and provided feedback on the analysis and discussion.

IntroductionThe most common cause of morbidity and mortality in children with severe cerebral palsy (CP) is respiratory disease. BREATHE-CP (Better REspiratory and Airway Treatment and HEalth in Cerebral Palsy) is a multidisciplinary research team who have conducted research on the risk factors associated with CP respiratory disease, a systematic review on management and a Delphi study on the development of a consensus for the prevention and management of respiratory disease in CP. These strategies have not been investigated; therefore, it is not known if implementation is feasible, if they improve patient outcomes or if they are acceptable for families.Methods and analysisMixed-method feasibility pilot randomised controlled trial with economic analysis. Twenty children with CP aged 0–12 years who are at risk of respiratory disease will be followed up for 1 year. All children will receive baseline assessments for comparison. The control group will receive usual care from their treating teams. The intervention group will receive comprehensive assessments from physiotherapy, speech pathology and respiratory medicine. An individualised investigation and treatment plan will then be made. Participants in both groups will complete fortnightly patient-reported outcome surveys to assess symptoms and health service use. Analysis will include assessments of acceptability through qualitative interviews, implementation by ability to recruit, randomise and retain, practicality including costs of intervention and hospitalisation, and explore efficacy through quality-of-life surveys and decreased health service use for respiratory-related symptoms.Ethics and disseminationEthics and governance approvals have been obtained through Child and Adolescent Health Service Human Research Ethics Committee. At completion, this study will lead to the design of the definitive protocol to test intervention efficacy that maximises recruitment, retention and adherence to interventions.Trial registration numberAustralian New Zealand Clinical Trials Registry (ACTRN12620000114943).

BackgroundRespiratory disease is a leading cause of hospitalisations in children with cerebral palsy (CP). Over 40% of individuals with CP are born preterm; however, the relationship between prematurity, CP and respiratory disease is unknown.ObjectivesTo establish the impact of gestational age (GA) and bronchopulmonary dysplasia (BPD) on respiratory hospitalisations in children with CP.Study designA 30-year (1986–2015) retrospective data linkage cohort study in children with CP.ResultsA total of 1915 individuals with CP (mean age 15 years and 5 months, 57% male) recorded 5566 respiratory admissions. Higher rates of respiratory admissions were seen in those born preterm (<37 weeks). Preterm infants with CP and BPD had 4.4 (incidence rate ratio 95% CI 3.3 to 6.0) times more respiratory-related admissions (after controlling for age, CP severity, epilepsy, speech impairment and gastrostomy) compared with individuals with CP born at term. Preterm infants with CP and BPD had respiratory-related hospitalisations at a younger age (median: 2 years 4 months vs term birth 20 years, p<0.001).ConclusionPreterm birth and BPD are risk factors for earlier and higher rates of respiratory-related admissions in individuals with CP. Perinatal and neonatal events should be considered when assessing respiratory health in CP.

Background: Achondroplasia, the most prevalent skeletal dysplasia, stems from a functional mutation in the fibroblast growth factor receptor 3 gene, leading to growth impairment. This condition presents multifaceted medical, functional and psychosocial challenges throughout childhood, adolescence and adulthood. Current management strategies aim to minimise medical complications, optimise functional capabilities and provide comprehensive supportive care. Vosoritide (trade name: VOXZOGO®, BioMarin Pharmaceuticals) is the first disease-modifying pharmaceutical treatment approved for the management of patients with achondroplasia and became available in Australia in May 2023. Methods: Standardised clinical guidelines for its optimal use are not yet widely available. To address this gap, a multidisciplinary Australian Vosoritide Working Group, comprising 12 experts with experience in achondroplasia management from across Australia, developed recommendations to guide the use of vosoritide in clinical practice. Results: The recommendations, which are expert opinions of the Australian Vosoritide Working Group, aim to (i) standardise the use of vosoritide across Australia, (ii) support the safe clinical rollout of vosoritide and (iii) support universal access. Conclusions: These recommendations have been developed for healthcare professionals and institutions that are engaged in using vosoritide in the management of achondroplasia and will be revised using a formal framework for clinical guideline development once more evidence is available.

ObjectivesTo report on knowledge translation strategies and outcomes from the implementation of the early detection guidelines for cerebral palsy (CP) in a state-wide tertiary early intervention (EI) service and investigate the impact of social determinants on clinical services.DesignRetrospective longitudinal cohort study.SettingThe Western Australia tertiary paediatric EI service.ParticipantsEI clinicians, consumers and children using the EI service.Outcome measuresKnowledge translation strategies including consumer perspectives, clinician training and Communities of Practice (CoP) guided implementation. We measured changes in referral number and age, delivery of early detection and intervention following the implementation of the guidelines. Exposure to adverse childhood experiences (ACEs), appointment non-attendance (DNA) rates, remoteness and socioeconomic quintiles were used to measure social determinants of health using negative binomial (Incidence Rate Ratios, IRR) and logistic regression (Odds Ratios, ORs).ResultsTen consumers participated in Focus Groups, 100 clinicians were trained and 22 clinicians established a monthly CoP. Referrals increased fourfold to 511 children. Corrected gestational age at referral decreased from a median of 16.1 to 5.1 months (p<0.001) and at first appointment from 18.8 to 6.8 months (p<0.001). Children living in social disadvantage had the highest DNA risk (quintile 1 vs 5: IRR 2.2, 95% CI 1.1 to 4.6, p=0.037). Children exposed to ACEs had higher odds of living in social disadvantage (quintile 1 vs 5, OR=3.8, 95% CI 1.4 to 10.0, p=0.007). No significant association was found between remoteness and DNA rate or ACE score.ConclusionsImplementation strategies reduced referral age and improved the delivery of early detection assessments. Further investigation of the association between social disadvantage, DNA risk and ACE score is required in the development of a state-wide early detection network.

Purpose of ReviewCerebral palsy is the most common physical disability of childhood, but the rate is falling, and severity is lessening. We conducted a systematic overview of best available evidence (2012–2019), appraising evidence using GRADE and the Evidence Alert Traffic Light System and then aggregated the new findings with our previous 2013 findings. This article summarizes the best available evidence interventions for preventing and managing cerebral palsy in 2019.Recent FindingsEffective prevention strategies include antenatal corticosteroids, magnesium sulfate, caffeine, and neonatal hypothermia. Effective allied health interventions include acceptance and commitment therapy, action observations, bimanual training, casting, constraint-induced movement therapy, environmental enrichment, fitness training, goal-directed training, hippotherapy, home programs, literacy interventions, mobility training, oral sensorimotor, oral sensorimotor plus electrical stimulation, pressure care, stepping stones triple P, strength training, task-specific training, treadmill training, partial body weight support treadmill training, and weight-bearing. Effective medical and surgical interventions include anti-convulsants, bisphosphonates, botulinum toxin, botulinum toxin plus occupational therapy, botulinum toxin plus casting, diazepam, dentistry, hip surveillance, intrathecal baclofen, scoliosis correction, selective dorsal rhizotomy, and umbilical cord blood cell therapy.SummaryWe have provided guidance about what works and what does not to inform decision-making, and highlighted areas for more research.

ObjectiveTo determine the early predictors of respiratory hospital admissions in young people with cerebral palsy (CP).DesignA 3-year prospective cohort study using linked data.PatientsChildren and young people with CP, aged 1 to 26 years.Main outcome measuresSelf-reported and carer-reported respiratory symptoms were linked to respiratory hospital admissions (as defined by the International Statistical Classification of Diseases and Related Health Problems 10th Revision codes) during the following 3 years.Results482 participants (including 289 males) were recruited. They were aged 1 to 26 years (mean 10 years, 10 months; SD 5 years, 11 months) at the commencement of the study, and represented all Gross Motor Function Classification Scale (GMFCS) levels. During the 3-year period, 55 (11.4%) participants had a total of 186 respiratory hospital admissions, and spent a total of 1475 days in hospital. Statistically significant risk factors for subsequent respiratory hospital admissions over 3 years in univariate analyses were GMFCS level V, at least one respiratory hospital admission in the year preceding the survey, oropharyngeal dysphagia, seizures, frequent respiratory symptoms, gastro-oesophageal reflux disease, at least two courses of antibiotics in the year preceding the survey, mealtime respiratory symptoms and nightly snoring.ConclusionsMost risk factors for respiratory hospital admissions are potentially modifiable. Early identification of oropharyngeal dysphagia and the management of seizures may help prevent serious respiratory illness. One respiratory hospital admission should trigger further evaluation and management to prevent subsequent respiratory illness.

Abstract Young people with intellectual disability experience higher risks for chronic health conditions than their peers. Improving their health literacy could be a strengths-based approach for reducing these risks. However, there is limited research that explores the perspectives of young people with intellectual disability on their health literacy. Given the limited research centring on the voice of young people with intellectual disability, this study examined their health literacy from their own perspectives. We had two aims: (i) explore the health literacy skills and needs of young people with intellectual disability and (ii) apply an adaptive interviewing approach and describe insights about its effectiveness. The adaptive interviewing method included visual materials and communication partners as needed. Sixteen young people with intellectual disability aged between 12 and 22 years who communicated with words or nonverbally were interviewed online. Data was coded using directed content analysis following the framework of the Health Literacy Questionnaire. The findings showed variation in the participants’ health literacy skills regarding relationships with healthcare professionals, managing health, and understanding health information. The results suggest that the health literacy skills of young people with intellectual disability are diverse, with different support needs. Targeted health promotion strategies are needed to meet these diverse needs. The adaptive interviewing was successful in capturing young people’s perspectives. With further refinements, including use of in-person and repeat interviews, this approach can be further utilized to enhance the accessibility of research.