Symptomatic palliative care for children with neurodisability

Paediatric palliative care and neurodisability are two relatively new, evolving paediatric sub-specialities that have increasing relevance in the current paediatric landscape. For many people palliative care has been synonymous with end of life care, but in paediatrics it encompasses much more and is for all children with life-threatening or life-limiting conditions, from the point of diagnosis. This breadth of focus is demonstrated well through the interface between paediatric palliative care and paediatric neurodisability. In this article we explore this unique interface through the three domains of complex symptom management, advanced care planning and end of life care. We describe the practicalities involved in all three areas and highlight the importance of early referral and the process of “dual” or “parallel” planning. We cover in more depth the specific management of the symptoms: dystonia/abnormalities of muscle tone, seizures, pain, agitation, secretions, respiratory failure, and gut failure.     

Neurodevelopmental outcomes of extremely preterm infants: theoretical and epidemiological perspectives to guide shared-care decision-making

Although births below 28 completed weeks' of gestation contribute to less than 1% of all preterm births globally, the impact of extreme prematurity (EPT) on neurodevelopmental outcomes across the life-course is disproportionately large. Higher rates and increased severity of neurodevelopmental impairments (NDIs) are reported among extremely preterm infants (EPIs). Cognitive skills, motor skills (manifesting as cerebral palsy) and vision are most commonly affected, with effects pervasive throughout school, adolescence and early adulthood. In addition, poorer academic outcomes and higher rates of psychiatric morbidity are seen among EPTs. Consistent improvements in EPI survival in recent years has not been matched with improvements in neurodevelopmental outcomes, especially for those born at less than 25 gestational weeks. However, over the last 20 years, several national and cross-national cohort studies have helped advance our understanding of extreme prematurity's developmental and life-course consequences. Here we provide an overview of the key findings from 13 multi-centre cohorts measuring neurodevelopmental outcomes and discuss the theoretical and epidemiological perspectives of NDIs in the context of extreme prematurity to guide communication with families and shared care decision-making.     

Multidisciplinary assessment at a child development centre: do we conform to recommended standards?

BACKGROUND: A proposed standard for the multidisciplinary assessment (MDA) of children with autism has been recently published by the National Autistic Society. This prompted a review of current practice at the child development centre in our local centre, to judge whether we were able to conform to the proposed national standard. The recommendation is that a child should complete a three-stage assessment process from referral to completion of assessment within 30 weeks, with set times for completion of each stage (6, 7 and 17 weeks respectively). We applied this assessment model to children with a range of neurodevelopment problems, as the process of MDA is the same, irrespective of diagnosis. METHODS: A retrospective analysis of medical and therapy records of all MDAs was carried out between April 2001 and March 2002. RESULTS: In the 12-month period studied, 52 MDAs were performed. Delays occurred, as judged by the standard, at all three stages of the assessment process. A total of 42% of children were seen within 6 weeks of initial referral, 37% within a further 7 weeks for specialist assessment, and 37% within a further 17 weeks for completion of MDA. As delays occurred at all stages, the cumulative total showed that only 19% of children completed all three stages within the recommended 30-week standard. Barriers encountered included waiting times to see professionals, parental non-attendance and prolonged assessment of complex problems. Some of these factors are outside our control, and on removing these factors the data were re-analysed. This resulted in a slight improvement to 45%, 48% and 49% for completion of stages 1, 2 and 3 respectively. The major reason for delay remained the service capacity. CONCLUSION: In our experience the standards proposed by the National Autistic Society are not practical within present resources. We suggest that a reasonable expectation is to complete all assessments, from first concern to completed MDA, should be carried out within 52 weeks with present levels of resources. Any further improvement will require additional resources to reduce waiting times and increase the capacity.     

Steroids on the neonatal unit

Corticosteroids are a potent class of drugs that, since their discovery, have become a cornerstone of therapy in many areas of medical practice. Their range of anti-inflammatory and immune modulating actions make them an attractive option, however their use has to be balanced against a significant side effect profile which is well known in children and adults but has not been so extensively studied in preterm infants. Currently antenatal steroid therapy in expectant preterm deliveries has been shown to reduce neonatal morbidity and mortality and is recommended as standard therapy. Postnatal steroids on the neonatal unit have mainly been used in the treatment of hypotension and in the prevention/treatment of chronic lung disease. Despite evidence confirming that postnatal treatment with dexamethasone reduced the incidence of chronic lung disease major concerns about long-term neurological outcomes persist, resulting in a need for careful balancing of the risks and benefits. Systematic reviews and meta-analyses have looked at the evidence to clarify the most effective steroid; timing of administration; dose and route to further guide their safe and effective use. This article aims to provide a summary of the currently available evidence regarding the risks and benefits of steroid use in the neonatal intensive care unit and offer guidance for paediatricians.     

Management of drooling in children: a survey of UK paediatricians' clinical practice

Background Drooling is common in children with disordered oral‐motor control. There is little evidence about the comparative effectiveness of different interventions used to reduce the impact of drooling. Anecdotal reports suggest clinicians' management of drooling varies widely. The aims of this survey were to establish which drooling interventions are currently used, how their effectiveness is monitored and how frequently adverse effects are reported. Methods 151 UK paediatricians completed a questionnaire about their management of drooling. Results Paediatricians saw one new child with problematic drooling and three follow‐up children per month. The most common prescribing pattern was hyoscine first line (84.7%) followed by glycopyrronium bromide second line. The reported rate of adverse effects of medications was lower than expected (median 10% for hyoscine). Very few paediatricians used standardized methods of measuring the medication's effectiveness or adverse effects. Conclusion Paediatricians regularly see small numbers of children with problematic drooling. Their clinical management of drooling varies; this is most likely because of a lack of evidence about the most effective approach. Comparative trials of interventions and the development of evidence‐based clinical guidelines would improve the management of children's drooling.     

(Unpacking) father involvement in the context of childhood neurodisability research: a scoping review

Background: In the past thirty years, theoretical and empirical scholarship on father involvement has emerged and firmly established itself. Efforts to define, measure, and explore outcomes related to father involvement in the context of childhood neurodisability are evident but less well established. The purpose of this study was to systematically map empirical studies on father involvement in the context of childhood neurodisability in order to delineate the current state of research and to highlight profitable directions for future research.

Methods: A rigorous scoping review method was used to select and analyze empirical studies published between the years 1988 and 2016 in order to systematically map research findings about fathers’ affective, behavioral, and cognitive involvement.

Results: Fifty-four (n?=?54) studies (quantitative n?=?47 and qualitative n?=?7) met inclusion criteria associated with three levels of review. Four main trends emerged: (a) paternal “stress” is a main concept of interest; (b) comparison of mothers and fathers on affective and cognitive involvement; (c) lack of a focus on fathers’ behavioral involvement, and (d) the absence of research designs that allow for examination of fathers’ unique perspectives.

Conclusions: Fathers are generally underrepresented in research in the context of childhood neurodisability. While there is a lack of depth in this area of research, granular analyses revealed important and unique differences about fathers’ parenting experiences. Recommendations for research and practice are provided.

• Implications for rehabilitation

• Fathers are underrepresented in the parenting in childhood neurodisability literature.

• Fathers who report feeling competent in parenting and connected to their child also report less parenting distress and more satisfaction in their couple relationship and family environment.

• Rehabilitation and allied health professionals should include fathers in parenting/family assessments.

• Manifestation of distress may differ among family members. Rehabilitation and allied health professionals should offer individualized care that is attuned to the needs of all family members.

     

Case series: Use of Glyceryl Trinitrate patches to improve peripheral circulation in children with severe neurodisability

Aim

Evaluation of topical Glyceryl trinitrate as a therapeutic option to improve peripheral circulation in 16 children with severe complex neurodisability, poor capillary return and signs of vascular insufficiency, including discomfort.