Can RESPiratory hospital Admissions in children with cerebral palsy be reduced? A feasibility randomised Controlled Trial pilot study protocol (RESP-ACT).

INTRODUCTION: The 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 ANALYSIS: Mixed-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 DISSEMINATION: Ethics 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 NUMBER: Australian New Zealand Clinical Trials Registry (ACTRN12620000114943).

Prevention and management of respiratory disease in young people with cerebral palsy: consensus statement.

Respiratory illness is the leading cause of mortality in children with cerebral palsy (CP). Although risk factors for developing chronic respiratory illness have been identified, comprehensive clinical care recommendations for the prevention and management of respiratory illness do not currently exist. We invited over 200 clinicians and researchers from multiple disciplines with expertise in the management of respiratory illness in children with CP to develop care recommendations using a modified Delphi method on the basis of the RAND Corporation-University of California Los Angeles Appropriateness Method. These recommendations are intended for use by the wide range of practitioners who care for individuals living with CP. They provide a framework for recognizing multifactorial primary and secondary potentially modifiable risk factors and for providing coordinated multidisciplinary care. We describe the methods used to generate the consensus recommendations, and the overall perspective on assessment, prevention, and treatment of respiratory illness in children with CP. WHAT THIS PAPER ADDS: The first consensus statement for preventing and managing respiratory disease in cerebral palsy (CP). Risk factors for respiratory disease in CP should be identified early. Individuals with CP at risk of respiratory disease require regular assessment of risk factors. Effective partnerships between multidisciplinary teams, individuals with CP, and families are essential. Treatment of respiratory disease in individuals with CP must be proactive.

La enfermedad respiratoria es la principal causa de mortalidad en niños con parálisis cerebral (PC). Aunque se han identificado los factores de riesgo para desarrollar enfermedades respiratorias crónicas, actualmente no existen recomendaciones completas de atención clínica para la prevención y el tratamiento de las enfermedades respiratorias. Invitamos a más de 200 médicos e investigadores de múltiples disciplinas con experiencia en el manejo de enfermedades respiratorias en niños con PC para desarrollar recomendaciones de atención utilizando un método Delphi modificado sobre la base del Método de adecuación RAND Corporation - Universidad de California en Los Ángeles. Estas recomendaciones están destinadas a ser utilizadas por la amplia gama de profesionales que atienden a personas que viven con PC. Proporcionan un marco para reconocer factores de riesgo multifactoriales primarios y secundarios potencialmente modificables y para proporcionar atención coordinada multidisciplinaria. Describimos los métodos utilizados para generar las recomendaciones de consenso, y la perspectiva general sobre la evaluación, prevención y tratamiento de enfermedades respiratorias de niños con PC.

Doença respiratória é a principal causa de mortalidade em crianças com paralisia cerebral (PC). Embora fatores de risco para desenvolver doença respiratória crônica tenham sido identificados, recomendações abrangentes de cuidado clínico e gerenciamento de doença respiratória não existem atualmente. Convidamos cerca de 200 clínicos e pesquisadores de múltiplas disciplinas com experiência no manejo de doença respiratória em crianças com PC para desenvolver recomendações de cuidado usando um método Delphi com base no método de Apropriação da Corporação RAND - Universidade da Califórnia. Estas recomendações são para uso de profissionais que atendem indivíduos com PC. Elas oferecem uma estrutura para reconhecer fatores de risco multifatoriais potencialmente modificáveis e prover cuidado multidisciplinar. Descrevemos métodos usados para gerar as recomendações do consenso, e a perspectiva geral de avaliação, prevenção e tratamento de doença respiratória em crianças com PC.

Interventions for management of respiratory disease in young people with cerebral palsy: A systematic review.

BACKGROUND: Respiratory disease is a leading cause of hospitalizations and deaths in young people with cerebral palsy (CP). It is insidious and multifactorial. Clinical management can be complex. This systematic review describes effects of interventions for the prevention and management of respiratory disease in young people with CP. METHODS: Nine databases (Cochrane, CINAHL, Embase, EMCare, MEDLINE, PEDro, OpenGrey, ScienceDirect, and SpeechBITE) were searched. Eligibility criteria were as follows: the population included at least 50% individuals with CP aged under 26 years, the intervention was for chronic respiratory illness, the outcomes were any measurable indicators of respiratory health or morbidity; the study design could be any original study reporting effects of an intervention on measurable outcomes, and the study was published January 1998 or later. The American Academy for Cerebral Palsy and Developmental Medicine methodology guided study appraisal and synthesis. The review was registered with PROSPERO (reference number CRD42018086314). RESULTS: The search yielded 3,347 papers; 37 papers (reporting 34 studies) of these met the eligibility criteria. They included 582 participants with CP (ranging from 1 to 77 across studies) with ages from 5 months to 25 years. Interventions were diverse and included: airway clearance techniques, exercise, positioning, mealtime management, salivary management, upper airway interventions, antibiotics, gastro-intestinal interventions, and spinal surgery. There were no interventions aimed at prevention of respiratory disease in this population. Research designs were classified as Levels 2 (n = 3), 3 (n = 2), 4 (n = 25), and 5 (n = 4). CONCLUSIONS: Evidence for most respiratory interventions for young people with CP is absent or weak. No controlled trials demonstrated significant effects on respiratory morbidity, owing to their scarcity, weak designs, and inadequate power. There is an urgent need for well-designed prospective controlled studies investigating prevention and management of respiratory disease in young people with CP.

Predicting respiratory hospital admissions in young people with cerebral palsy.

OBJECTIVE: To determine the early predictors of respiratory hospital admissions in young people with cerebral palsy (CP). DESIGN: A 3-year prospective cohort study using linked data. PATIENTS: Children and young people with CP, aged 1 to 26 years. MAIN OUTCOME MEASURES: Self-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. RESULTS: 482 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. CONCLUSIONS: Most 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.

Factors Associated with Respiratory Illness in Children and Young Adults with Cerebral Palsy.

OBJECTIVE: To describe associations between respiratory illness and its potential predictors in children and young adults with cerebral palsy (CP). STUDY DESIGN: Cross-sectional survey of self- and caregiver-reported respiratory symptoms for individuals aged up to 26 years with CP. Respiratory illness was indicated by 2 outcomes: (1) ≥1 respiratory hospitalizations in the past year; and (2) ≥2 courses of antibiotics for respiratory symptoms in the past year. ORs were calculated using univariate and multivariate logistic regression. RESULTS: There were 551 participants, aged 1-26 years, distributed across all gross motor function classification scale (GMFCS) levels. In univariate analyses, factors significantly associated with respiratory hospitalizations were weekly respiratory symptoms (OR 2.31, 95% CI 1.78-3.00), respiratory symptoms during meals (OR 3.23, 95% CI 1.50-5.80), gastroesophageal reflux (OR 3.01, 95% CI 1.71-5.31), coughing or choking on saliva (OR 4.36, 95% CI 2.38-8.01), current asthma (OR 3.56, 95% CI 1.97-6.42), age (0-3 years) (OR 3.24, 95% CI 1.19-8.80, compared with 13-17 years), seizures (OR 3.45, 95% CI 1.96-6.08), and scoliosis (OR 2.14, 95% CI 1.16-3.97). Nonambulatory individuals (GMFCS IV-V) were at significantly increased risk of hospitalizations only if they had food modifications and/or nasogastric or gastrostomy tube feeds (OR 5.36, 95% CI 2.89-9.96, compared with GMFCS I-III with no food modifications and no tube). All factors, except seizures and scoliosis, were significantly associated with multiple courses of antibiotics in univariate analyses. CONCLUSIONS: Oromotor dysfunction is strongly associated with respiratory illness in patients with CP.