Kinematic upper limb analysis outperforms electromyography at grading the severity of dystonia in children with cerebral palsy.

BACKGROUND: Severity of dyskinesia in children with cerebral palsy is often assessed using observation-based clinical tools. Instrumented methods to objectively measure dyskinesia have been proposed to improve assessment accuracy and reliability. Here, we investigated the technique and movement features that were most suitable to objectively measure the severity of dystonia in children with cerebral palsy. METHODS: A prospective observational study was conducted with 12 participants with cerebral palsy with a predominant motor type of dyskinesia, spasticity, or mixed dyskinesia/spasticity who had upper limb involvement (mean age: 12.6 years, range: 6.7-18.2 years). Kinematic and electromyography data were collected bilaterally during three upper limb tasks. Spearman rank correlations of kinematic or electromyography features were calculated against dystonia severity, quantified by the Dyskinesia Impairment Scale. FINDINGS: Kinematic features were more influential compared to electromyography features at grading the severity of dystonia in children with cerebral palsy. Kinematic measures quantifying jerkiness of volitional movement during an upper limb task with a reaching component performed best (|rs| = 0.78-0.9, p < 0.001). INTERPRETATION: This study provides guidance on the types of data, features of movement, and activity protocols that instrumented methods should focus on when objectively measuring the severity of dystonia in children with cerebral palsy.

Measurement properties and feasibility of chronic pain assessment tools for use with children and young people with cerebral palsy.

PURPOSE: Chronic pain assessment tools exist for children, but may not be valid, reliable, and feasible for populations with functional, cognitive or communication limitations, for example, cerebral palsy (CP). This study aimed to (i) identify chronic pain assessment tools used with children and young people and rate their measurement properties; (ii) develop a CP specific feasibility rating tool to assess the feasibility of tools in CP; and (iii) categorise tools according to reporting method. MATERIALS AND METHODS: Assessment tools were identified by literature review. Their measurement properties were rated using the COnsensus based standards for the Selection of health Measurement INstruments. The CP specific Feasibility Rating Tool was developed and used to rate the tools. RESULTS: Fifty-seven chronic pain assessment tools were identified. Six have content validity for CP, four of these use proxy-report. Forty-two tools were considered feasible for people with CP; 24 self report and 18 observational/proxy-report. Only the Paediatric Pain Profile has content validity and feasibility for people with CP unable to self-report. CONCLUSIONS: There are few valid, reliable and feasible tools to assess chronic pain in CP. Further research is required to modify tools to enable people with cognitive limitations and/or complex communication to self-report pain.

Few of the existing chronic pain assessment tools are feasible or valid to use with all young people with cerebral palsy (CP).Modifications to self-report tools are needed to improve access for young people who have cognitive impairment or use alternative and augmentative communication.The pain assessment tool with the strongest feasibility and measurement properties for young people who cannot self-report is the Paediatric Pain Profile.Clinicians will need to consider a range of chronic pain assessment tools to assess the biopsychosocial domains important to young people with cerebral palsy.

Chronic pain in children and young people with cerebral palsy: a narrative review of challenges, advances, and future directions.

BACKGROUND: Cerebral palsy (CP), the most common physical disability of childhood, is often accompanied by a range of comorbidities including pain. Pain is highly prevalent in children and young people with CP, yet has been poorly understood, inaccurately assessed, and inadequately managed in this vulnerable population. This narrative review presents recent research advances for understanding and managing pain in children and young people with CP, focusing on chronic pain, and highlights future research directions. MAIN BODY: Pain prevalence rates in CP vary due to different methodologies of studies. Recent systematic reviews report up to 85% of children experience pain; higher in older children, females, and those with dyskinesia and greater motor impairment. Research examining the lived experience perspectives of children and their families demonstrate that even those with mild motor impairments have pain, children want to self-report pain where possible to feel heard and believed, and management approaches should be individualized. Notably, many children with cognitive and communication impairments can self-report their pain if adjustments are provided and they are given a chance. Past inadequacies of pain assessment in CP relate to a focus on pain intensity and frequency with little focus on pain interference and coping, a lack of tools appropriate for the CP population, and an assumption that many children with cognitive and/or communication limitations are unable to self-report. Recent systematic reviews have identified the most reliable and valid assessment tools for assessing chronic pain. Many were not developed for people with CP and, in their current form, are not appropriate for the spectrum of physical, communication, and cognitive limitations seen. Recently, consensus and co-design in partnership with people with lived experience and clinicians have identified tools appropriate for use in CP considering the biopsychosocial framework. Modifications to tools are underway to ensure feasibility and applicability for the spectrum of abilities seen. CONCLUSION: Recent research advances have improved our understanding of the prevalence, characteristics and lived experience of chronic pain, and refined assessment methods in children and young people with CP. However, the very limited evidence for effective and novel management of chronic pain in this population is where research should now focus.

Examining tools for assessing the impact of chronic pain on emotional functioning in children and young people with cerebral palsy: stakeholder preference and recommendations for modification.

PURPOSE: To firstly identify tools for assessing the impact of chronic pain on emotional functioning in children and young people with cerebral palsy (CP), and secondly identify suggestions to improve their relevance, comprehensiveness, comprehensibility and feasibility for the CP population. Improving assessment of the impact of pain on emotional functioning can enhance quality of life by improving access to interventions for pain-related physical disability, anxiety and depression. METHODS: Ethics approval was granted through the Women's and Children's Health Network Human Research Ethics Committee (2022/HRE00154). A mixed methods study with people with lived experience and clinicians, and guided by the Consensus-based Standards for Measurement Instruments (COSMIN), was undertaken. An online survey identified the highest rated tools for validation and/or modification for young people with CP and chronic pain. Focus groups and interviews investigated content validity and feasibility of the tools identified as highest rated. RESULTS: The Fear of Pain Questionnaire for Children-SF (FOPQ-C-SF) and Modified Brief Pain Inventory (mBPI) were the highest rated for pain coping and multidimensional assessment (respectively) from the online survey (n = 61) of eight tools presented. Focus group and interview data (n = 30), including 58 unique modification suggestions, were coded to six categories: accessibility, comprehensibility, feasibility, relevance, presentation and comprehensiveness. CONCLUSION: Potential modifications have been identified to improve the appropriateness and feasibility of the FOPQ-C-SF and mBPI for children and young people with CP. Future research should implement and test these modifications, prioritising the involvement of people with lived experience to ensure their needs are met alongside clinicians.

Up to 75% of children and young people with cerebral palsy report chronic pain, which is much higher than those without cerebral palsy. Assessing how pain impacts emotional functioning, and how each individual copes with pain, is of particular importance due to known links between emotional functioning and long term pain outcomes. Reliable assessment of how pain impacts emotional functioning may also help to identify those who would benefit from psychological treatments. Although pain questionnaires are available, many are not suitable for children and young people with cerebral palsy with different communication, cognitive and movement abilities. This study had two aims: (1) to work out which of the currently available tools that assess how pain impacts emotional functioning are considered best for people with cerebral palsy, and (2) to identify potential modifications to these tools. The two most relevant and easy to understand questionnaires selected for modification were the Fear of Pain Questionnaire for Children and the modified Brief Pain Inventory. A number of modifications were identified, including improving how relevant the questions were to people with cerebral palsy, improving accessibility for people with complex communication needs or cognitive impairment and improving how easy to understand the questions and answer options are. These modifications can now be implemented to make it easier for people with cerebral palsy to use the pain assessments. They should then be tested in people with cerebral palsy with different communication, cognitive and movement abilities.

Comprehensive care programmes for children with medical complexity.

BACKGROUND: Children with medical complexity (CMC) represent a small, but growing, proportion of all children. Regardless of their underlying diagnosis, by definition, all CMC have similar functional limitations and high healthcare needs. It has been suggested that improving aspects of healthcare delivery for CMC improves health- and quality of life-related outcomes for children and their families and reduces healthcare-related expenditure. As a result, dedicated comprehensive care programmes have been established at many hospitals to meet the needs of CMC; however, it is unclear if such programmes are effective. OBJECTIVES: Our main objective was to assess the effectiveness of comprehensive care programmes that aim to improve care coordination and other aspects of health care for CMC and to assess whether the effectiveness of such programmes differs according to the programme setting and structure. We aimed to assess their effectiveness in relation to child and parent health, functioning, and quality of life, quality of care, number of healthcare encounters, unmet healthcare needs, and total healthcare-related costs. SEARCH METHODS: We searched CENTRAL, MEDLINE, Embase, and CINAHL in May 2023. We also searched reference lists, trial registries, and the grey literature. SELECTION CRITERIA: Randomised and non-randomised trials, controlled before-after studies, and interrupted time series studies were included. Studies that compared enrolment in a comprehensive care programme with non-enrolment in such a programme/treatment as usual were included. Participants were children that met the criteria for the definition of CMC, which is: having (i) a chronic condition, (ii) functional limitations, (iii) increased health and other service needs, and (iv) increased healthcare costs. Studies that included the following types of outcomes were included: health; quality of care; utilisation, coverage and access; resource use and costs; equity; and adverse outcomes. DATA COLLECTION AND ANALYSIS: Two review authors independently extracted data, assessed the risk of bias in each included study, and evaluated the certainty of evidence according to GRADE criteria. Where possible, data were represented in forest plots and pooled. We were unable to undertake a meta-analysis for comparisons and outcomes, so we used a structured synthesis approach. MAIN RESULTS: We included four studies with a total of 912 CMC as participants. All included studies were randomised controlled trials conducted in hospitals in the USA or Canada. Participants varied across the included studies; however, all four studies included children with complex and chronic illness and high healthcare needs. While the primary aim of the intervention was similar across all four studies, the components of the interventions differed: in the four studies, the intervention involved some element of care coordination; in two of the studies, it involved the child receiving care from a multidisciplinary team, while in one study, the intervention was primarily centred on access to an advanced practice nurse care coordinator and another study involved nurse a practitioner-paediatrician dyad partnering with families. The risk of bias in the four studies varied across domains, with issues primarily relating to the lack of blinding of participants, personnel, and outcome assessors, inadequate allocation concealment, and incomplete outcome data. Comprehensive care for CMC compared to usual care may make little to no difference to child health, functioning, and quality of life at 12 or 24 months (three studies with 404 participants) and we assessed the evidence for the outcomes in this category (child health-related quality of life and functional status) as being of low certainty. For CMC, comprehensive care probably makes little or no difference to parent health, functioning, and quality of life compared to usual care at 12 months (one study with 117 participants) and we assessed the evidence for this outcome as being of moderate certainty. Comprehensive care for CMC compared to usual care may slightly improve child and family satisfaction with, and perceptions of, care and service delivery at 12 months (three studies with 453 participants); however, we assessed the evidence for these outcomes as being of low certainty. For CMC, comprehensive care probably makes little or no difference to the number of healthcare encounters (emergency department visits) and the number of hospitalised days (hospital admissions) compared to usual care at 12 months (three studies with 668 participants), and we assessed the evidence for these outcomes as being of moderate certainty. Three of the included studies (668 participants) reported cost outcomes and had conflicting results, with one study reporting significantly lower healthcare costs at 12 months in the intervention group compared to the control group, one reporting no differences between groups, and the other study reporting a greater increase in total healthcare costs in the intervention group compared to the control group. Overall, comprehensive care may make little or no difference to overall healthcare costs in CMC; however, the methods used to measure total healthcare costs varied across studies and the certainty of the evidence relating to this outcome is low. No studies assessed the costs to the family. AUTHORS' CONCLUSIONS: The findings of this review should be treated with caution due to the limited amount and quality of the published research that was available to be included. Overall, the certainty of the evidence for the effectiveness of comprehensive care for CMC ranged from low to moderate across outcomes and there is currently insufficient evidence on which to draw strong conclusions. There is a need for more high-quality randomised trials with consistency of the target population and intervention components, methods of reporting outcomes, and follow-up periods, as well as full cost analyses, taking into account both costs to the family and costs to the healthcare system.

Pharmacological and neurosurgical management of cerebral palsy and dystonia: Clinical practice guideline update.

Dystonia, typically characterized by slow repetitive involuntary movements, stiff abnormal postures, and hypertonia, is common among individuals with cerebral palsy (CP). Dystonia can interfere with activities and have considerable impact on motor function, pain/comfort, and ease of caregiving. Although pharmacological and neurosurgical approaches are used clinically in individuals with CP and dystonia that is causing interference, evidence to support these options is limited. This clinical practice guideline update comprises 10 evidence-based recommendations on the use of pharmacological and neurosurgical interventions for individuals with CP and dystonia causing interference, developed by an international expert panel following the Grading of Recommendations, Assessment, Development and Evaluations (GRADE) approach. The recommendations are intended to help inform clinicians in their use of these management options for individuals with CP and dystonia, and to guide a shared decision-making process in selecting a management approach that is aligned with the individual's and the family's values and preferences.

Chronic pain interference assessment tools for children and adults who are unable to self-report: A systematic review of psychometric properties.

AIM: To identify and evaluate psychometric properties of assessment tools for assessing pain interference in children, adolescents, and adults with chronic pain and the inability to self-report. METHOD: The protocol was registered with PROSPERO (CRD42022310102). A search was run in MEDLINE, Embase, and PsycInfo (29th March 2022) to identify articles reporting psychometric properties of pain interference assessment tools for children, adolescents, and adults with chronic pain and the inability to objectively self-report pain. Retrieved studies were reviewed by two authors (MGS, LCF) and study quality was assessed using COSMIN. RESULTS: Psychometric properties of 10 pain interference tools were assessed from 33 studies. The Paediatric Pain Profile (PPP) had low-quality evidence for content validity and internal consistency with children and adolescents who are unable to self-report. No tools for adults had evidence for content validity and internal consistency. No tool had evidence for all nine psychometric properties. INTERPRETATION: The PPP is recommended for pain interference assessment in children and adolescents with chronic pain and the inability to self-report. Few tools are available for adults. Three tools for children (Patient-Reported Outcome Measurement Information System Pediatric Proxy Pain Interference Scale; Bath Adolescent Pain Questionnaire for Parents; modified Brief Pain Inventory-Proxy [mBPI]) and three tools for adults (Doloplus-2; Patient-Reported Outcome Measurement Information System Pain Interference Scale-proxy; Brief Pain Inventory-proxy) are promising but require further investigation.

Physiotherapy via telehealth for acute respiratory exacerbations in paediatric cystic fibrosis.

INTRODUCTION: The demand for Hospital in the Home has increased, especially as an avenue for treatment of respiratory exacerbations. However, a limiting factor of Hospital in the Home efficiency is excess travel. Telehealth can potentially increase in-home access to specialist care such as physiotherapy. This study examined clinical outcomes achieved with a hybrid telehealth model and assessed safety and efficiency. METHOD: This study was an observational benchmarking study of Hospital in the Home physiotherapy episodes of care during respiratory exacerbations between January 2017-June 2019. The participants were young people aged 8-18 years, with cystic fibrosis receiving intravenous antibiotics and bi-daily physiotherapy. The intervention was physiotherapy via either a hybrid model (1× telehealth, 1× face-to-face session) or standard care (2× face-to-face sessions). The outcomes were frequency of return to at least 95% of baseline percentage predicted forced expiratory volume in the first second (ppFEV1), ppFEV1 change, adverse events, travel time and distance saved. RESULTS: There were 82 episodes of Hospital in the Home; 41 hybrid and 41 standard care. Return to at least 95% of baseline was achieved in 49% of the hybrid group and 32% of standard care. Median ppFEV1 change was +6% for the hybrid group and +2% standard care. There were no adverse events. Estimated travel time and distance saved was 16,520 min and 12,301.2 km. CONCLUSION: Preliminary information supports a hybrid telehealth physiotherapy model as an alternative to standard care for young people with cystic fibrosis during an exacerbation. Safety of telehealth in conjunction with home visits favoured its use to improve efficiency and capacity without added risk.

Development of a Clinical Framework for the Assessment of Dyskinesia and Function in the Upper Limb in Children with Cerebral Palsy.

OBJECTIVE: Dyskinesia in cerebral palsy (CP) is a complex movement disorder that can significantly impact upper limb function. Despite a range of available tools, there is no consensus on best practice assessment of upper limb function in children with CP and dyskinesia. This study aimed to develop a clinical framework for the assessment of the impact of dyskinesia on upper limb function in children with CP. DESIGN: Modified Delphi study using expert consensus. METHODS: An expert panel of six highly experienced Australian therapists ranked assessment tools sourced from existing evidence-based literature using a five-point Likert scale. Tools rated as important for use "most" or "all" of the time, by 80% of respondents were accepted into the framework following two survey rounds and a third stage discussion. RESULTS: Of 21 tools, 12 were included in the framework under five categories: (i) screening for dyskinesia; (ii) measuring the severity of dyskinesia; (iii) Classifying upper limb functional ability; (iv) measuring upper limb functional ability; and (v) measuring upper limb movement. CONCLUSIONS: The framework for assessing the impact of dyskinesia on upper limb function in CP aims to guide clinicians to improve assessment consistency and facilitate individualized goal-directed management. Further studies with a larger number of expert clinicians and researchers will further strengthen the utility of the framework.

Pain coping tools for children and young adults with a neurodevelopmental disability: A systematic review of measurement properties.

AIM: To systematically identify and evaluate the measurement properties of patient-reported outcome measures (PROMs) and observer-reported outcome measures (parent proxy report) of pain coping tools that have been used with children and young adults (aged 0-24 years) with a neurodevelopmental disability. METHOD: A two-stage search using MEDLINE, Embase, CINAHL, Web of Science, and PsycInfo was conducted. Search 1 in August 2021 identified pain coping tools used in neurodevelopmental disability and search 2 in September 2021 located additional studies evaluating the measurement properties of these tools. Methodological quality was assessed using the COnsensus-based Standards for the Selection of Health Measurement INstruments (COSMIN) guidelines (PROSPERO protocol registration no. CRD42021273031). RESULTS: Sixteen studies identified seven pain coping tools, all PROMs and observer-reported outcome measures (parent proxy report) versions. The measurement properties of the seven tools were appraised in 44 studies. No tool had high-quality evidence for any measurement property or evidence for all nine measurement properties as outlined by COSMIN. Only one tool had content validity for individuals with neurodevelopmental disability: the Cerebral Palsy Quality of Life tool. INTERPRETATION: Pain coping assessment tools with self-report and parent proxy versions are available; however, measurement invariance has not been tested in young adults with a neurodevelopmental disability. This is an area for future research.

Perioperative Care of Children with Severe Neurological Impairment and Neuromuscular Scoliosis-A Practical Pathway to Optimize Peri-Operative Health and Guide Decision Making.

Neuromuscular scoliosis is a common feature in children with severe neurological impairment (SNI), including those with severe cerebral palsy. Surgical correction of scoliosis is the mainstay of treatment. This group of patients also have associated medical complexity. The complication rates post-surgery are high, although, for many, they are worth the risk. There are currently no published practice guidelines or care pathways for children with SNI who are undergoing scoliosis corrective surgery. In response to the high uptake of this surgery, coupled with the expected complication rates, our hospital established a perioperative clinic. The purpose of this paper is to describe our perioperative approach. This clinic has developed into a service beyond perioperative care and, with the collaborative meeting, enables shared decision-making to identify the right candidate for surgery. The process involves surgical expertise, understanding the family and child at the centre, and optimisation of medical care pre- and post-surgery. In this paper, we describe the process in a step-by-step manner. We provide clinical vignettes, as well as the proformas that we use, and we highlight the benefits of the team-based process.

Task-specific training for bicycle-riding goals in ambulant children with cerebral palsy: a randomized controlled trial.

AIM: To determine whether a task-specific physiotherapist-led training approach is more effective than a non-specific parent-led home programme for attaining bicycle-riding goals in ambulant children with cerebral palsy (CP). METHOD: Sixty-two ambulant children with CP aged 6 to 15 years (33 males, 29 females, mean age 9y 6mo) with bicycle-riding goals participated in this multi-centre, assessor-blind, parallel-group, superiority randomized controlled trial. Children in the task-specific group participated in a physiotherapist-led, group-based, intensive training programme. Children in the parent-led home group were provided with a practice schedule, generic written information, and telephone support. Both programmes involved a 1-week training period. The primary outcome was goal attainment at 1 week after training measured using the Goal Attainment Scale. Secondary outcomes included bicycle skills, participation in bicycle riding, functional skills, self-perception, physical activity, and health-related quality of life at 1 week and 3 months after training. RESULTS: Children in the task-specific training group had greater odds of goal attainment than those in the parent-led home programme at 1 week after intervention (odds ratio [OR] 10.4, 95% confidence interval [CI] 2.8-38.6), with evidence for superiority retained at 3 months (OR 4.0, 95% CI 1.3-12.5). INTERPRETATION: The task-specific physiotherapist-led training approach was more effective for attaining bicycle-riding goals than a non-specific parent-led home programme in ambulant children with CP.

Caregiver perspectives of managing chronic pain in children and adolescents with dyskinetic and mixed dyskinetic/spastic CP with communication limitations.

PURPOSE: Caregivers provide unique insights into managing chronic pain in children and adolescents with dyskinetic and mixed dyskinetic/spastic cerebral palsy with communication limitations. This study explored the personal challenges caregivers face in supporting their child's everyday pain management, including barriers and facilitators to effective chronic pain management. METHODS: Semi-structured interviews were undertaken with ten caregivers (all mothers) of children with either dyskinetic or mixed dyskinetic/spastic cerebral palsy. All children had chronic pain (> 3 months), were aged from 5 to 15 years, had significant functional limitations, and had either limited or no capacity to self-report their pain. Interpretative phenomenological analysis was used to explore caregivers' subjective experiences of managing their child's chronic pain within family, school, and healthcare contexts. RESULTS: Five superordinate themes emerged: 1. the continual challenge of problem solving pain and dyskinesia; 2. the pursuit of a solution; 3. unfulfilled preferences within pain management; 4. all-encompassing effects on families; and 5. the ongoing impacts of pain and dyskinesia with age. CONCLUSION: There is a need for structured pain education and resources targeted towards caregivers and support workers that account for the complex overlay of dyskinesia. There is a further need to ensure caregiver preferences for non-pharmacological pain treatments are met within family-centred care models.

Establishing consensus for the assessment of chronic pain in children and young people with cerebral palsy: a Delphi study.

PURPOSE: Inconsistent and inadequate pain assessment practices in cerebral palsy (CP) have resulted from a lack of standardisation of pain assessment, limited use of appropriate tools and failure to integrate disability and biopsychosocial models. To assist with improving consistency, this study aimed to establish consensus from key stakeholders regarding domains considered essential for measuring chronic pain in children and young people with CP. METHOD: A modified electronic Delphi study was conducted on 83 stakeholders, including clinicians, researchers, people with CP and parents of children with CP. Participants rated 18 domains sourced from existing literature as either "core", "recommended", "exploratory" or "not required". RESULTS: After two rounds of surveys, 12 domains were considered core: pain location, pain frequency, pain intensity, changeable factors, impact on emotional wellbeing, impact on participation, pain communication, influence on quality of life, physical impacts, sleep, pain duration and pain expression. CONCLUSION: These domains reflect the complexity of pain in a heterogeneous population where medical comorbidities are common and communication and intellectual limitations impact significantly on the ability of many to self-report. The domains will be utilised to build a framework of pain assessment specific to children and young people with CP guided by the biopsychosocial model.Implications for rehabilitationChronic pain is under-identified and poorly assessed in the cerebral palsy (CP) population.The perspectives of clinicians, researchers and consumers are vital for developing a framework for chronic pain assessment in CP.Consensus of key stakeholders found 12 domains considered essential to incorporate into a chronic pain assessment model in CP.

Barriers and Facilitators to Seeking Sleep Solutions for Children With Cerebral Palsy: A Qualitative Study.

Background: Published evidence to date suggests that sleep problems are common in children with cerebral palsy (CP). This qualitative study is a follow up to a previously published quantitative phase on the experience and impact of sleep problems in this population. Aims: The aim of this study was to explore the experience and impact of sleep disturbance and seeking of sleep solutions for parents of school aged children with CP. Materials and Methods: Semi-structured 19 qualitative interviews were conducted with parents of children with CP aged 6-12 years. Interview data were transcribed verbatim and the thematic analysis techniques by Braun and Clarke was used to identify themes. Results: Thematic analysis identified 7 themes: (1) My Child Doesn't Fit into the Box, (2) A Mother's Ears are Always On, (3) Sleep Disturbance is like Water Torture, (4) Sleep is One of Many Spot Fires, I Put it on the Backburner, (5) Luck, Money or Jumping Up and Down, (6) There is Never One Silver Bullet and (7) Help: The Earlier the Better. The key finding was that parents of children with CP often described their child's needs being distinct from what is provided by systems and services. Conclusion: Parents face significant challenges sourcing effective sleep solutions for their child with CP. Sleep is often not a priority for either the parent or the clinician as other health problems take precedence. Parents reflected that early sleep intervention for their child was or would have been helpful. The barriers and facilitators to sleep care identified in this study should be used to inform clinical change in care for children with CP. Sleep needs to be prioritized in healthcare for children.

A pilot feasibility study of gabapentin for managing pain in children with dystonic cerebral palsy.

BACKGROUND: Gabapentin is often used to manage pain in children with dystonic cerebral palsy, however the evidence for its effectiveness in this population is limited. The primary objective of this feasibility pilot study was to assess the factors which might impact on a future randomised controlled trial including the ability to recruit and retain participants, assess adherence/compliance to the prescribed intervention, and ability to complete all outcome assessments. The secondary objective was to gather preliminary evidence for the effectiveness of gabapentin at reducing pain, improving comfort and reducing dystonia in children with dystonic cerebral palsy. METHODS: This open label pilot study recruited children aged 5-18 years with dystonic cerebral palsy and accompanying pain affecting daily activities from four centres around Australia. Children were prescribed gabapentin for 12 weeks and were assessed at baseline, 6 weeks and 12 weeks. The primary outcome was feasibility of the protocol. Secondary outcomes were pain behaviour, pain intensity, care and comfort, individualised goal setting and dystonia severity. RESULTS: Thirteen children (mean age 10.4 years (SD 2.4yrs), 9 females) were recruited from 71 screened over 15 months. Two children withdrew while eight children experienced side effects. There were issues with adherence to medication dosage regimens and data collection. Improvements were seen in pain behaviour, comfort and pain related goals at 12 weeks. Dystonia was not significantly changed. CONCLUSIONS: Whilst gabapentin has potential to improve pain and comfort in children with dystonic CP, the feasibility of implementing a definitive randomised controlled trial is low. Alternative trials designs are required to further examine the effectiveness of gabapentin in this heterogeneous population. TRIAL REGISTRATION: The trial was registered with the Australian Clinical Trial Registry ( ACTRN12616000366459 ) on 22/03/2016 and the Therapeutic Goods Administration (CT-2016-CTN-00500-1) on 22/06/2016.

The Dyskinetic Cerebral Palsy Functional Impact Scale: development and validation of a new tool.

AIM: To outline the development and examine the content and construct validity of a new tool, the Dyskinetic Cerebral Palsy Functional Impact Scale (D-FIS), which measures the impact of dyskinesia on everyday activities in children with cerebral palsy (CP). METHOD: D-FIS content was informed by a systematic review of dyskinesia outcome measures, in collaboration with children with dyskinetic CP, parents, caregivers, and expert clinicians. The D-FIS uses parent proxy to rate impact of dyskinesia on everyday activities. Construct validity was determined by examining internal consistency; known groups validity with the Gross Motor Function Classification System (GMFCS), Manual Ability Classification System (MACS), Communication Function Classification System (CFCS), and Eating and Drinking Ability Classification System (EDACS); and convergent validity with the Barry-Albright Dystonia Scale (BADS). RESULTS: Fifty-seven parents of children (29 males, 28 females, mean [SD] age 11y 8mo [4y 4mo], range 2y 6mo-18y) completed the D-FIS. Correlation between D-FIS and GMFCS was r=0.86 (95% confidence interval [CI]: 0.77-0.91, p<0.001); MACS r=0.84 (95% CI: 0.73-0.90, p<0.001); CFCS r=0.80 (95% CI: 0.67-0.88, p<0.001); and EDACS r=0.78 (95% CI: 0.66-0.87). Correlation between D-FIS and BADS was r=0.77 (95% CI: 0.64-0.86, p<0.001). Cronbach's alpha was 0.96. INTERPRETATION: The D-FIS demonstrates good construct validity and high internal consistency. The D-FIS will be useful for identifying priorities for intervention. It adds to the measurement tool kit for children with dyskinetic CP by addressing functional impact of dyskinetic movements and postures. What this paper adds The Dyskinetic Cerebral Palsy Functional Impact Scale (D-FIS) assesses the perceived impact of dyskinesia on daily activities in children with cerebral palsy (CP). The D-FIS demonstrates good construct validity and high internal consistency. The D-FIS is a clinically feasible, family-centred tool that fills a current gap in the dyskinetic CP assessment toolkit.

National surveillance of oral medication prescription for children with dystonic cerebral palsy.

AIM: Oral medications are often first-line medical management for children with cerebral palsy who have generalised dystonia; however, evidence for their effectiveness is limited and dosing practices are inconsistent. As a first step to improve consistency, this study aimed to examine current clinical practice of expert doctors for prescribing medications for children with dystonic cerebral palsy including prescribing patterns and combinations of medications used. METHODS: This was a prospective surveillance study of medical doctors working in major Australian centres who manage children with cerebral palsy. Each week over a continuous 6-month period, doctors completed a custom developed online survey for children seen that week with dystonic cerebral palsy for whom they prescribed a new medication to treat dystonia. RESULTS: Twenty-five doctors consented to participate, 16 of whom prescribed new medications for dystonia in children with cerebral palsy over the study period. There were 77 children who were prescribed new medications. Baclofen and gabapentin were prescribed most, followed by levodopa, trihexyphenidyl and diazepam. The most common combinations of medications were baclofen and diazepam or baclofen and gabapentin. Dosage regimens were variable, particularly for trihexyphenidyl and diazepam. CONCLUSION: Inconsistencies in dosing regimens remain for oral medication prescription by Australian doctors when managing dystonia in cerebral palsy. Future studies using the consensus of expert clinicians will be conducted to develop guidelines in an area where the evidence for individual medications is extremely limited.

Clinician Perspectives of Chronic Pain Management in Children and Adolescents with Cerebral Palsy and Dyskinesia.

AIMS: To explore perspectives of clinicians from interdisciplinary teams on the barriers and facilitators to chronic pain management for children and adolescents with cerebral palsy and dyskinesia. METHODS: Interdisciplinary focus groups (n = 2) were conducted at two Australian tertiary pediatric hospitals. Twenty-five experienced clinicians took part, including ten physiotherapists, six pediatricians, four rehabilitation physicians, four occupational therapists, and one speech and language therapist. An external moderator conducted the focus groups and data were analyzed using inductive thematic analysis. RESULTS: Four key themes emerged: "balancing the intersection of pain and dyskinesia," "difficulty communicating between so many providers," "uncertainty surrounding chronic pain education," and "differing priorities." Key barriers were identified including a lack of access to some interdisciplinary team members and formalized guidance for health professionals regarding chronic pain education. CONCLUSION: Key issues were reported to impact the delivery of coordinated inter-disciplinary chronic pain management at the tertiary level for children and adolescents with cerebral palsy and dyskinesia. In the absence of strong evidence, a strategy for implementing effective chronic pain management for children and adolescents with cerebral palsy and dyskinesia and gaining clinician consensus regarding the best practice management are recommended.

Pain in children with dyskinetic and mixed dyskinetic/spastic cerebral palsy.

AIM: To evaluate pain prevalence and characteristics in children and adolescents with predominant dyskinetic and mixed (dyskinetic/spastic) cerebral palsy (CP) motor types. METHOD: Seventy-five participants with a diagnosis of CP and confirmed dyskinetic or mixed (dyskinetic/spastic) motor type took part in a multisite cross-sectional study. The primary outcome was carer-reported pain prevalence (preceding 2wks) measured using the Health Utilities Index-3. Secondary outcomes were chronicity, intensity, body locations, quality of life, and activity impact. RESULTS: Mean participant age was 10 years 11 months (SD 4y 2mo, range 5-18y). There were 44 males and 31 females and 37 (49%) had predominant dyskinetic CP. Pain was prevalent in 85% and it was chronic in 77% of participants. Fifty-two per cent experienced moderate-to-high carer-reported pain intensity, which was significantly associated with predominant dyskinetic motor types (p=0.008). Pain occurred at multiple body locations (5 out of 21), with significantly increased numbers of locations at higher Gross Motor Function Classification System levels (p=0.02). Face, jaw, and temple pain was significantly associated with predominant dyskinetic motor types (p=0.005). Poorer carer proxy-reported quality of life was detected in those with chronic pain compared to those without (p=0.03); however, chronic pain did not affect quality of life for self-reporting participants. INTERPRETATION: Pain was highly prevalent in children and adolescents with predominant dyskinetic and mixed (dyskinetic/spastic) motor types, highlighting a population in need of lifespan pain management. WHAT THIS PAPER ADDS: Chronic pain prevalence in children and adolescents with predominant dyskinetic and mixed (dyskinetic/spastic) motor types is high. Pain occurs across multiple body locations in predominant dyskinetic and mixed (dyskinetic/spastic) motor types. Less recognized locations of pain include the face, jaw, and temple for predominant dyskinetic motor types.