When a child is diagnosed with a life threatening condition, one of the most challenging tasks facing healthcare professionals is to communicate this to the child, as well as to their parents or caregivers. Evidence-based guidelines are urgently needed for all healthcare settings, from tertiary referral centres in high income countries to resource limited environments in low and middle income countries, where rates of child mortality are high. We place this narrative review in the context of children's developing understanding of illness and death. We review the impact of communication on children's emotional, behavioural and social functioning, as well as treatment adherence, disease progression and wider family relationships. We consider the factors that influence the process of communication and the preferences of children, families and healthcare professionals about how to convey the diagnosis. Critically, the barriers and challenges to effective communication are explored. Finally, we outline principles for communicating with children, parents and caregivers, generated from a workshop of international experts. Communication with Children and Adolescents about the Diagnosis of their own Life Threatening ConditionAlan Stein (AS) conceptualized and the designed the review. Louise Dalton (LD), Lucy Hanington (LH), Kim Fredman Stein (KFS) and AS undertook the literature searches and selected the studies. AS, LD and Elizabeth Rapa (ER) gathered the information from the studies and drafted the manuscript. Myra Bluebond-Langner (MBL), Sue Ziebland (SZ), Emily Harrop (EH), Ruth Bland (RB), Brenda Kelly (BK), Tamsen Rochat (TR) and KFS provided specific input to different sections of the manuscript; Communication Expert Group commented on drafts of the manuscript and contributed to the development of the guidelines. LD and ER compiled the tables. All authors have read and approved the final version of the Review. Conflict of Interest statementsEH declares she is deputy chair of the NICE clinical guideline development committee for NG61 (End of Life Care in Infants, Children and Young People). All of the other authors declare no Conflicts of Interest Role of funding sourceThe funder of the study had no role in any aspect of the review.

BackgroundInherited epidermolysis bullosa (EB) comprises a group of rare disorders that have multi-system effects and patients present with a number of both acute and chronic pain care needs. Effects on quality of life are substantial. Pain and itching are burdensome daily problems. Experience with, and knowledge of, the best pain and itch care for these patients is minimal. Evidence-based best care practice guidelines are needed to establish a base of knowledge and practice for practitioners of many disciplines to improve the quality of life for both adult and pediatric patients with EB.MethodsThe process was begun at the request of Dystrophic Epidermolysis Bullosa Research Association International (DEBRA International), an organization dedicated to improvement of care, research and dissemination of knowledge for EB patients worldwide. An international panel of experts in pain and palliative care who have extensive experience caring for patients with EB was assembled. Literature was reviewed and systematically evaluated. For areas of care without direct evidence, clinically relevant literature was assessed, and rounds of consensus building were conducted. The process involved a face-to-face consensus meeting that involved a family representative and methodologist, as well as the panel of clinical experts. During development, EB family input was obtained and the document was reviewed by a wide variety of experts representing several disciplines related to the care of patients with EB.ResultsThe first evidence-based care guidelines for the care of pain in EB were produced. The guidelines are clinically relevant for care of patients of all subtypes and ages, and apply to practitioners of all disciplines involved in the care of patients with EB. When the evidence suggests that the diagnosis or treatment of painful conditions differs between adults and children, it will be so noted.ConclusionsEvidence-based care guidelines are a means of standardizing optimal care for EB patients, whose disease is often times horrific in its effects on quality of life, and whose care is resource-intensive and difficult. The guideline development process also highlighted areas for research in order to improve further the evidence base for future care.Electronic supplementary materialThe online version of this article (doi:10.1186/s12916-014-0178-2) contains supplementary material, which is available to authorized users.

The present study employs selective estrogen receptor (ER) agonists to determine whether 17beta-estradiol-induced neuroprotection in global ischemia is receptor mediated and, if so, which subtype of receptor (ERalpha or ERbeta) is predominantly responsible. Halothane-anesthetized female C57Bl/6J mice were ovariectomized, and osmotic minipumps containing ERbeta agonist diarylpropiolnitrile (DPN) (8 mg.kg(-1).day(-1), n = 12) or vehicle (50% DMSO in 0.9% saline) (n = 9) or ERalpha agonist propyl pyrazole triol (PPT) (2 mg.kg(-1).day(-1), n = 13) or vehicle (50% DMSO in 0.9% saline) (n = 10) were implanted subcutaneously. One week later transient global ischemia was induced by bilateral carotid artery occlusion under halothane anesthesia, and the mice were perfusion fixed 72 h later. ERbeta agonist DPN significantly reduced ischemic damage by 70% in the caudate nucleus and 55% in the CA1 region compared with vehicle controls (P < 0.05, Mann-Whitney U-statistic). In contrast, pretreatment with the ERalpha agonist PPT had no effect on the extent of neuronal damage compared with controls. The data indicate a significant estrogen receptor-mediated neuroprotection in a global cerebral ischemia model involving ERbeta.

Background Increasing numbers of children with complex health‐care needs are cared for at home by their family. The aim of this qualitative study was to explore the challenges experienced by families caring for children who need complex medical care at home. Methods We conducted a thematic analysis of eleven in‐depth interviews with parents who carry out specialist medical procedures (eg, enteral feeding, bowel washouts and tracheostomy care) for their children at home. Participants were purposely selected from an existing sample of interviews with parents whose child had abdominal surgery in the first year of life. Results We identified three overarching themes: (a) responsibilities of the parent, (b) impact on daily life and (c) the parent journey over time. Parents have substantial responsibilities, including performing medical procedures, managing emergencies (sometimes life‐threatening), co‐ordinating care and advocating for their child. Their responsibilities have an enormous impact on the family: going out of the home becomes a challenge, there are constant constraints on time, parents are sleep‐deprived and there are wider impacts on siblings. The third theme explores the parent journey over time as parents become experts and make sense of the new normal. Discussion The burden of care on families caring for children with complex medical needs is much greater than is generally understood by either multidisciplinary health‐care teams or the general public. Families need to be better prepared and supported for the responsibilities they take on and the burden of care needs to be shared by others.

The use of long-term ventilation (LTV) in children is growing in the UK and worldwide. This reflects the improvement in technology to provide LTV, the growing number of indications in which it can be successfully delivered and the acceptability of LTV to families and children. In this article, we discuss the various considerations to be made when deciding to initiate or continue LTV, describe the process that be followed, as decided by a consensus of experienced physicians, and outline the options available for resolution of conflict around LTV decision making. We recognise the uncertainty and hope provided by novel and evolving therapies for potential disease modification. This raises the question of whether LTV should be offered to allow time for a therapy to be trialled, or whether the therapy is unlikely to be effective, LTV would simply prolong suffering. We put this consensus view forward as an ethical framework for decision making in children requiring LTV.