Intractable Feeding Intolerance in Children With Severe Neurological Impairment: A Retrospective Case Review of Nine Children Known to a Pediatric Palliative Care Service.

BACKGROUND: Intractable feeding intolerance in children with severe neurological impairment (SNI) is poorly defined and understood. OBJECTIVES: (1) To describe 9 children with SNI, where intractable feeding intolerance was thought to be a contributor to their deterioration or death. (2) To consider terminology to describe the severe end of the spectrum of feeding difficulties in children with SNI. RESULTS: Mean age at death was 10.3 years (range: 5 - 15.6), and median time from palliative care referral to death was 3.1 months. Location of death was home (n = 3), hospice (n = 1), and hospital (n = 5) with 1 death in intensive care. Gastrointestinal "failure" or "dysfunction" were documented for 7 children, (median time between documentation and death was 3.9 months (range: .1 to 13.1)). All children were fed via a gastrostomy tube during their life (median age of insertion 2.5 years (range: 1.2 to 6.8 years)), and 7 via the jejunal route (median age of insertion 9.2 years (range 2.4 to 14.7 years)). Children lived a median of 9 percent of their lives after jejunal tube feeding was commenced. No child had home-based parenteral nutrition. Multiple symptom management medications were required. CONCLUSION: 'Intractable feeding intolerance' describes a clinical crossroads in a child's life where there is an opportunity to consider the appropriateness of further interventions. Further work should explore predictors of intractable feeding intolerance and the delicate balance between cause or contributor to death. The importance of clinician-family prognostic conversations and goal-concordant care both during life and in the terminal phase is highlighted.

Carer perspectives of a transition to adult care model for adolescents with an intellectual disability and/or autism spectrum disorder with mental health comorbidities.

BACKGROUND: Transition to adult care for adolescents with an intellectual disability and/or autism spectrum disorder with coexisting mental health disorders, often termed 'dual disability', is complex. It requires a family-centred approach, with collaboration among health, disability and social services and early planning. AIM: To describe carer perspectives of transition to adult care and the outcomes of a transition support intervention, Fearless, Tearless Transition, for adolescents with dual disabilities piloted at a tertiary children's hospital. METHODS: Carers of adolescents with a dual disability were invited to complete a survey at the commencement of their participation in the Fearless, Tearless Transition model, and again at the conclusion of the project. Within this intervention, carers and adolescents were encouraged to attend dedicated transition clinics and participate in a shared care general practitioner (GP) and paediatrician process. RESULTS: One hundred and fifty-one carers of adolescents with dual disabilities were included in Fearless, Tearless Transition. Of this cohort, 138 adolescents and their carers received support in a dedicated transition clinic with 99 carers completing the initial survey at the commencement of the model. Eighty-two per cent of carers reported moderate to high levels of anxiety about transitioning from paediatric to adult care with 39% feeling 'unprepared' about transition. Eighty-one per cent reported having inadequate access to respite care with 47% reporting a lack of access to services in the community and 56% expressing dissatisfaction with their GPs. One hundred and two families participated in the shared care process with 80 GPs and 33 paediatricians. Twenty-two carers completed the second survey reporting a modest but significant improvement in preparedness for transition to adult care. CONCLUSION: This study highlights the potential to improve transition outcomes for adolescents with dual disabilities and their carers through early, centralized transition planning, consistent methods of assessing adolescent and carer needs and shared care.

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.

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.

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.

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.

Development of the Fearless, Tearless Transition model of care for adolescents with an intellectual disability and/or autism spectrum disorder with mental health comorbidities.

AIM: First, to understand the barriers to achieving effective transition and the supports required from the perspective of parents and carers, adolescents with intellectual disability and/or autism spectrum disorder and co-existing mental health disorders (often termed 'dual disability'), and those who provide services to this group. Second, to develop an informed model of shared care to improve the transition of adolescents with dual disabilities. METHOD: Carers and a young adult with a dual disability were surveyed about their experience of transition care. Other key stakeholders including paediatricians, general practitioners, and policy makers were also interviewed. These data informed the model of care. RESULTS: Paediatricians and general practitioners reported difficulties establishing working relationships to foster smooth transitions, and carers reported lacking a regular general practitioner with adequate expertise to care for people with dual disabilities. A process of shared care between paediatricians and general practitioners was developed and initiated by a dedicated transition manager, who assisted with care coordination and service linkages. Standardized clinical assessment tools were also introduced to determine patient and carer support needs. INTERPRETATION: This study highlights the potential to improve transition outcomes for adolescents with dual disabilities and their carers through early transition planning, consistent methods of assessing patient and carer needs, and shared care. WHAT THIS PAPER ADDS: Adolescents with co-occurring disabilities require a collaborative health and disability service interface. Fearless, Tearless Transition is a new approach to transitioning adolescents with dual disabilities from paediatric to adult care. Carers of adolescents with dual disabilities require support navigating and negotiating services. Engaging general practitioners and paediatricians in shared care early during the transition process is essential.

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.

Anxiety in children and adolescents with cerebral palsy.

AIMS: To describe the prevalence of, and factors associated with, anxiety in 6-18-year-old children with cerebral palsy (CP) and determine how often clinicians screen for and manage anxiety in this group. METHODS: Using a population CP register as the sampling pool, 569 families were approached by email, and 172 (mean age of children 12 years 7 months [SD 3 years 5 months]; 96 males) participated. Parents and, where able, children completed the Screen for Child Anxiety Related Emotional Disorders (SCARED). Parents also completed the Strengths and Difficulties Questionnaire. Children's medical records were searched for previous anxiety diagnoses and treatments. RESULTS: Clinically significant anxiety was identified in 38% of children on parent reports and 46% on child reports. Girls were twice as likely to have anxiety (p = 0.02). Parent- and child-reported scores were strongly correlated (r = 0.853). Fewer parents of children with intellectual and communication impairments completed the survey. Based on the SCARED parent reports, anxiety was not identified by a clinician in 16 children (43%) with clinically significant anxiety. CONCLUSION: Anxiety symptoms are prominent among children with CP, indicating a need for routine screening. Available screening tools are unsuitable for children with more severe limitations in cognition and communication; further research is needed to address this gap.

The lived experience of chronic pain and dyskinesia in children and adolescents with cerebral palsy.

BACKGROUND: To explore the lived experience of chronic pain and dyskinesia in children and adolescents with cerebral palsy. METHODS: A convergent parallel mixed methods design was undertaken. First, a quantitative cross-sectional study of participants able to self-report their quality of life was undertaken. This study characterised pain chronicity, intensity, body locations, and quality of life. Second, semi-structured interviews were undertaken with a subset of children and adolescents experiencing chronic pain. RESULTS: Twenty-five children and adolescents took part in the cross-sectional study, 23 of whom experienced chronic pain and 13 of moderate intensity. Pain was often located in multiple bodily regions (6/21), with no trends in quality of life outcomes detected. Eight participated in semi-structured interviews, which identified three key themes including 'lives embedded with dyskinesia', 'real world challenges of chronic pain', and 'still learning strategies to manage their pain and dyskinesia'. CONCLUSIONS: A high proportion of children and adolescents with cerebral palsy and dyskinesia who were able to self-report experienced chronic pain. The physical and emotional impacts of living with chronic pain and dyskinesia existed along a spectrum, from those with lesser to greater extent of their impacts. Children and adolescents may benefit from targeted chronic pain education and management within bio-psychosocial models.

Vaccination in people with disability: a review.

People with disabilities are vulnerable to complications from vaccine-preventable diseases, and every effort should be made to ensure equitable access to immunization for this population. This paper aims to summarize the research on immunizations in people with disabilities, in order to ensure a comprehensive understanding of knowledge in this area and direct further research. The literature is weighted towards coverage data that is difficult to synthesize because of the different definitions of disability, and the variety of settings, vaccinations and age groups across the studies. In-depth qualitative data and data from a variety of health-care providers and people with disability is notably lacking. This is vital to redress in order to develop effective immunization interventions in this population.

The uptake of adolescent vaccinations through the School Immunisation Program in specialist schools in Victoria, Australia.

BACKGROUND: As part of the National Immunisation Program (NIP) students in Australia receive adolescent immunisations through the School Immunisation Program at 12 to 13 years. For children with disabilities attending specialist schools, no vaccine uptake data is collected at this time point. We aimed to determine uptake of diphtheria-tetanus-pertussis (dTpa) and Human Papillomavirus (HPV) immunisations amongst young people with disabilities in specialist schools in Victoria. METHODS: A prospective cohort study was conducted in Victoria, Australia. Data was collected on immunisation days in the 2017 school year from specialist schools in Victoria. The school immunisation coordinator entered data online for eligible students for receipt of dTpa and HPV on each school immunisation day. Demographic data, motor and intellectual function of students and reasons for non-receipt of dTpa and HPV vaccine were recorded. Data were analysed using descriptive statistics. RESULTS: Of 73 eligible specialist schools in Victoria, 28 (38%) participated. dTpa was received by 63% (237/374) of participating students and HPV dose 1 (HPV1) was received by 66% (76/114) females and 67% (174/260) male students respectively. Three doses of HPV were received by only 41% (100/241) of students. The main reasons for missed immunisation were absence from school, lack of consent and inability to immunise due to the student's behaviour and/or anxiety. CONCLUSION: This is the first study in Australia to report that uptake of adolescent immunisations in specialist schools for young people with a disability is significantly lower than in mainstream settings. Comparative data during the same time period for students in mainstream schools demonstrated higher uptake, at 89% for dTpa and 75% for three doses of HPV. These data highlight the inequity of receipt of school-based immunisations for this group of adolescents, the barriers to which could be more thoroughly explored through qualitative inquiry from a socio-ecological perspective.

Prevalence and characteristics of pain in children and young adults with cerebral palsy: a systematic review.

AIM: The primary aim of this review is to evaluate the evidence for pain prevalence in children and young adults with cerebral palsy. Secondary aims are to identify pain characteristics and types of pain measurement used in this population. METHOD: Ovid MEDLINE, Embase, CINAHL Plus, and PubMed were searched in October 2016 and updated in November 2017. Two authors independently screened studies according to Preferred Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Pain outcomes were categorized within a biopsychosocial pain framework, with pain prevalence extracted for all recall periods and measurement types. RESULTS: One hundred and six publications from 57 studies met inclusion criteria. Pain prevalence varied widely from 14 per cent to 76 per cent and was higher in females, older age groups, and those classified within Gross Motor Function Classification System level V. Pain was most frequent in the lower limbs, back, and abdomen and associated with reduced quality of life or health status. The influence of pain on psychological functioning, interference, and participation was inconclusive. INTERPRETATION: Variation exists in reported pain prevalence because of sampling bias, inconsistent measurement, varying recall periods, and use of different participant age ranges. WHAT THIS PAPER ADDS: Pain prevalence varies from 14 per cent to 76 per cent in children and young adults with cerebral palsy. Pain is more prevalent in females, older age groups, and children in Gross Motor Function Classification System level V.

Intrathecal baclofen therapy: benefits and complications.

BACKGROUND: Spasticity and dystonia in children with cerebral palsy has been treated with intrathecal baclofen therapy (ITB) at the Royal Children's Hospital, Melbourne, Australia (RCH) since 1999. METHODS: The records of children having received or still receiving ITB during the period September 1999 until August 2005 were studied to evaluate complications and side effects. Parents answered a questionnaire focused on the health and functional impact in the children. RESULTS: There were 18 first insertions of pumps, 6 removals, and 4 reinsertions. The longest treatment was 5 years and 11 months and was still ongoing. Seventeen complications occurred in 14 out of 18 children. Despite the high complication rate and the lack of significant functional improvement, 11 out of 12 parents agreed that ITB was beneficial. CONCLUSION: ITB treatment at RCH over the years has resulted in some complications, mostly occurring shortly after pump insertion. For the majority of children there are substantial benefits.