Experiences of families post treatment for childhood brain tumours during medical clinic consultations regarding health-related quality of life, unmet needs and communication barriers: A qualitative exploration.

BACKGROUND: Many studies highlight poor health-related quality of life (HRQoL) in children treated for brain tumours and their parents. However, little is known about the extent to which their informational, healthcare and communication needs regarding HRQoL are met during medical outpatient consultations. AIM: To explore the experiences of families regarding communication with physicians about HRQoL issues during consultations after treatment for childhood brain tumours. METHODS: Interviews were conducted with 18 families of children and adolescents aged 8-17 years after completion of brain tumour treatment. Participants had completed treatment within the last 5 years and were receiving regular outpatient follow-up care. Thematic analysis was undertaken using the Framework Method. RESULTS: Five main themes were identified: (i) unmet emotional and mental health needs; (ii) double protection; (iii) unmet information needs; (iv) communication barriers within consultations; and (v) finding a new normal. CONCLUSION: There was a need to improve communication between clinicians and these families, improve information provision, and overcome barriers to conversing with children within these outpatient consultations. Children and their parents should be supported to voice their current needs and concerns regarding their HRQoL. These findings will inform further development of the UK version of the 'KLIK' patient- and parent-reported outcome (PROM) portal.

Pain assessment tools in paediatric palliative care: A systematic review of psychometric properties and recommendations for clinical practice.

BACKGROUND: Assessing pain in infants, children and young people with life-limiting conditions remains a challenge due to diverse patient conditions, types of pain and often a reduced ability or inability of patients to communicate verbally. AIM: To systematically identify pain assessment tools that are currently used in paediatric palliative care and examine their psychometric properties and feasibility and make recommendations for clinical practice. DESIGN: A systematic literature review and evaluation of psychometric properties of pain assessment tools of original peer-reviewed research published from inception of data sources to April 2021. DATA SOURCES: PsycINFO via ProQuest, Web of Science Core, Medline via Ovid, EMBASE, BIOSIS and CINAHL were searched from inception to April 2021. Hand searches of reference lists of included studies and relevant reviews were performed. RESULTS: From 1168 articles identified, 201 papers were selected for full-text assessment. Thirty-four articles met the eligibility criteria and we examined the psychometric properties of 22 pain assessment tools. Overall, the Faces Pain Scale-Revised (FPS-R) had high cross-cultural validity, construct validity (hypothesis testing) and responsiveness; while the Faces, Legs, Activity, Cry and Consolability (FLACC) scale and Paediatric Pain Profile (PPP) had high internal consistency, criterion validity, reliability and responsiveness. The number of studies per psychometric property of each pain assessment tool was limited and the methodological quality of included studies was low. CONCLUSION: Balancing aspects of feasibility and psychometric properties, the FPS-R is recommended for self-assessment, and the FLACC scale/FLACC Revised and PPP are the recommended observational tools in their respective age groups.

A Systematic Review of Measures of Breakthrough Pain and Their Psychometric Properties.

CONTEXT: Breakthrough pain (BTP) is common in cancer and other conditions yet there is a lack of validated BTP measurement tools. OBJECTIVES: We aimed to identify all tools assessing or characterising BTP in patients of any age with any condition, and to critically appraise their psychometric properties. METHODS: The Cochrane Library, PROSPERO, Embase, CINAHL, Medline, PsycINFO, Web of Science, Google Scholar, ProQuest, Evidence Search and OpenGrey were searched to identify all available tools used to assess BTP. A second search identified studies that had evaluated psychometric properties of tools identified in Search 1. Databases were searched from inception to November 2020. Studies were assessed using COSMIN criteria and GRADE guidelines. RESULTS: Search 1 found 51 tools used to assess BTP. Search 2 found six tools that had a development study and/or a study evaluating a tool psychometric property. No tool had more than one study evaluating psychometric properties so a meta-analysis could not be conducted. Studies were of inadequate to very good quality. Only the Breakthrough Pain Assessment Tool (BAT) had sufficient content validity and at least low-quality evidence for sufficient internal consistency. CONCLUSION: The BAT is recommended to characterise BTP in adults with cancer; its applicability to other conditions is unknown. The remaining tools need further evaluation. Only the Breakthrough Pain Questionnaire for Children was designed for children with cancer, but no psychometric properties were evaluated. There is a need for a tool to assess and characterise BTP in children with non-cancer diagnoses and those who cannot self-report.

Healthcare professionals' views of the use of oral morphine and transmucosal diamorphine in the management of paediatric breakthrough pain and the feasibility of a randomised controlled trial: A focus group study (DIPPER).

BACKGROUND: Oral morphine is frequently used for breakthrough pain but the oral route is not always available and absorption is slow. Transmucosal diamorphine is administered by buccal, sublingual or intranasal routes, and rapidly absorbed. AIM: To explore the perspectives of healthcare professionals in the UK caring for children with life-limiting conditions concerning the assessment and management of breakthrough pain; prescribing and administration of transmucosal diamorphine compared with oral morphine; and the feasibility of a comparative clinical trial. DESIGN/ PARTICIPANTS: Three focus groups, analysed using a Framework approach. Doctors, nurses and pharmacists (n = 28), caring for children with life-limiting illnesses receiving palliative care, participated. RESULTS: Oral morphine is frequently used for breakthrough pain across all settings; with transmucosal diamorphine largely limited to use in hospices or given by community nurses, predominantly buccally. Perceived advantages of oral morphine included confidence in its use with no requirement for specific training; disadvantages included tolerability issues, slow onset, unpredictable response and unsuitability for patients with gastrointestinal failure. Perceived advantages of transmucosal diamorphine were quick onset and easy administration; barriers included lack of licensed preparations and prescribing guidance with fears over accountability of prescribers, and potential issues with availability, preparation and palatability. Factors potentially affecting recruitment to a trial were patient suitability and onerousness for families, trial design and logistics, staff time and clinician engagement. CONCLUSIONS: There were perceived advantages to transmucosal diamorphine, but there is a need for access to a safe preparation. A clinical trial would be feasible provided barriers were overcome.

Attentional bias to somatosensory stimuli in chronic pain patients: a systematic review and meta-analysis.

ABSTRACT: This systematic review and meta-analysis aimed to evaluate the evidence pertaining to attentional bias for painful and nonpainful somatosensory stimuli in individuals with chronic pain. Eligible studies were identified through searches of Medline, PsycINFO, CINAHL, Web of Science, Scopus, and Cochrane Library databases. Search terms were words and phrases organised into 3 concept blocks: pain condition, cognitive process, and stimuli/paradigm. The search identified 29 eligible studies (reporting 32 eligible experiments), of which quantitative meta-analysis was possible for 16 studies (19 experiments). The meta-analysis found that chronic pain patients, excluding somatoform pain patients, showed significantly greater attentional bias to stimuli in the somatosensory modality than healthy controls (k = 9, g = 0.34). In addition, meta-analysis of studies that used a temporal order judgement task found that patients with unilateral chronic pain showed a spatial attentional bias away from somatosensory stimuli (k = 7, effect estimate = 22.43 ms) and visual stimuli (k = 2, effect estimate = 13.75 ms) on or near the painful body side. Most studies of attentional bias to the somatosensory modality recruited samples of patients with fibromyalgia, whereas most studies of spatial attentional bias assessed patients with complex regional pain syndrome. The extent to which these results generalise to other pain conditions is therefore unclear. We recommend future research test spatial and modality attentional biases across chronic pain conditions and examine the psychometric properties of attentional bias measurement paradigms for use with chronic pain populations. PROSPERO registration number CRD42019124510.

Systematic review: measurement properties of patient-reported outcome measures evaluated with childhood brain tumor survivors or other acquired brain injury.

BACKGROUND: Survivors of childhood brain tumors or other acquired brain injury (ABI) are at risk of poor health-related quality of life (HRQoL); its valid and reliable assessment is essential to evaluate the effect of their illness on their lives. The aim of this review was to critically appraise psychometric properties of patient-reported outcome measures (PROMs) of HRQoL for these children, to be able to make informed decisions about the most suitable PROM for use in clinical practice. METHODS: We searched MEDLINE, EMBASE, and PsycINFO for studies evaluating measurement properties of HRQoL PROMs in children treated for brain tumors or other ABI. Methodological quality of relevant studies was evaluated using the consensus-based standards for the selection of health status measurement instruments checklist. RESULTS: Eight papers reported measurement properties of 4 questionnaires: Health Utilities Index (HUI), PedsQL Core and Brain Tumor Modules, and Child and Family Follow-up Survey (CFFS). Only the CFFS had evidence of content and structural validity. It also demonstrated good internal consistency, whereas both PedsQL modules had conflicting evidence regarding this. Conflicting evidence regarding test-retest reliability was reported for the HUI and PedsQL Core Module only. Evidence of measurement error/precision was favorable for HUI and CFFS and absent for both PedsQL modules. All 4 PROMs had some evidence of construct validity/hypothesis testing but no evidence of responsiveness to change. CONCLUSIONS: Valid and reliable assessment is essential to evaluate impact of ABI on young lives. However, measurement properties of PROMs evaluating HRQoL appropriate for this population require further evaluation, specifically construct validity, internal consistency, and responsiveness to change.

A mixed-methods systematic review and meta-analysis of barriers and facilitators to paediatric symptom management at end of life.

BACKGROUND: Symptom management for infants, children and young people at end of life is complex and challenging due to the range of conditions and differing care needs of individuals of different ages. A greater understanding of these challenges could inform the development of effective interventions. AIM: To investigate the barriers and facilitators experienced by patients, carers and healthcare professionals managing symptoms in infants, children and young people at end of life. DESIGN: A mixed-methods systematic review and meta-analysis was undertaken (PROSPERO ID: CRD42019124797). DATA SOURCES: The Cochrane Library, PROSPERO, CINAHL, MEDLINE, PsycINFO, Web of Science Core Collection, ProQuest Dissertations & Theses Database, Evidence Search and OpenGrey were electronically searched from the inception of each database for qualitative, quantitative or mixed-methods studies that included data from patients, carers or healthcare professionals referring to barriers or facilitators to paediatric end-of-life symptom management. Studies underwent data extraction, quality appraisal, narrative thematic synthesis and meta-analysis. RESULTS: A total of 64 studies were included (32 quantitative, 18 qualitative and 14 mixed-methods) of medium-low quality. Themes were generated encompassing barriers/facilitators experienced by carers (treatment efficacy, treatment side effects, healthcare professionals' attitudes, hospice care, home care, families' symptom management strategies) and healthcare professionals (medicine access, treatment efficacy, healthcare professionals' demographics, treatment side effects, specialist support, healthcare professionals' training, health services delivery, home care). Only one study included patients' views. CONCLUSION: There is a need for effective communication between healthcare professionals and families, more training for healthcare professionals, improved symptom management planning including anticipatory prescribing, and urgent attention paid to the patients' perspective.

A protocol for a systematic review and meta-analysis to identify measures of breakthrough pain and evaluate their psychometric properties.

INTRODUCTION: Breakthrough pain is common in children and adults with cancer and other conditions, including those approaching end-of-life, although it is often poorly managed, possibly partly due to a lack of validated assessment tools. This review aims to (1) identify all available instruments measuring breakthrough pain in infants, children, adolescents or adults and (2) critically appraise, compare and summarise the quality of the psychometric properties of the identified instruments using COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN) criteria. METHODS AND ANALYSIS: Two searches will be carried out between October 2019 and January 2020, one for each aim of the review. The Cochrane Library, International Prospective Register of Systematic Reviews, Embase, Cumulative Index of Nursing and Allied Health Literature, Medical Literature Analysis and Retrieval System Online (MEDLINE), PsycINFO, Web of Science Core Collection, Google Scholar, the ProQuest Dissertations & Theses Database, Evidence Search and OpenGrey databases will be searched from database inception until the date the search is conducted. Reference lists of eligible articles will be screened and authors in the field contacted. For search 1, articles will be screened by two reviewers by abstract, and full-text where necessary, to identify if a breakthrough pain assessment was used. Search 2 will then be conducted to identify studies evaluating measurement properties of these assessments. Two reviewers will screen articles from search 2 by title and abstract. All potentially relevant studies will be screened by full text by both reviewers. For search 2, data will be extracted in parallel with the quality assessment process, as recommended by COSMIN. Two reviewers will assess methodological quality using the COSMIN Risk of Bias checklist and the COSMIN updated criteria for good measurement properties. Findings will be summarised and, if possible, data will be pooled using meta-analysis. The quality of the evidence will be graded and summarised using the Grading of Recommendations, Assessment, Development and Evaluations (GRADE) guidelines. ETHICS AND DISSEMINATION: Results of this review will be submitted for publication in a peer review journal and presented at conferences. PROSPERO REGISTRATION NUMBER: CRD42019155583.

Reducing pain and distress related to needle procedures in children with cancer: A clinical practice guideline.

BACKGROUND: Children with cancer often undergo long treatment trajectories involving repeated needle procedures that potentially cause pain and distress. As part of a comprehensive effort to develop clinical practice guidelines (CPGs) to address pain prevention and management in children with cancer, we aimed to provide recommendations on the pharmacological and psychological management of procedure-related pain and distress. METHODS: Of the international inter-disciplinary CPG development panel (44 individuals), two working groups including 13 healthcare professionals focused on procedural pain and distress. Grading of Recommendations Assessment, Development and Evaluation methodology was used, including the use of systematic literature reviews to inform recommendations and the use of evidence to decision frameworks. At an in-person meeting in February 2018, the guideline panel discussed these frameworks and formulated recommendations which were then discussed with a patient-parent panel consisting of 4 survivors and 5 parents. RESULTS: The systematic reviews led to the inclusion of 48 randomised controlled trials (total number of participants = 2271). Quality of evidence supporting the recommendations ranged from very low to moderate. Strong recommendations were made for the use of topical anesthetics in all needle procedures, for offering deep sedation (DS)/general anesthesia (GA) to all children undergoing lumbar puncture, for the use of DS/ GA in major procedures in children of all ages, for the use of hypnosis in all needle procedures and for the use of active distraction in all needle procedures. CONCLUSION: In this CPG, an evidence-based approach to manage procedure-related pain and distress in children with cancer is presented. As children with cancer often undergo repeated needle procedures during treatment, prevention and alleviation of procedure-related pain and distress is of the utmost importance to increase quality of life in these children and their families.

Barriers and facilitators experienced by patients, carers and healthcare professionals when managing symptoms in infants, children and young people at end-of-life: a mixed methods systematic review protocol.

INTRODUCTION: This protocol describes the objective and methods of a systematic review of barriers and facilitators experienced by patients, carers and healthcare professionals when managing symptoms in infants, children and young people (ICYP) at end-of-life. METHODS AND ANALYSIS: The Cochrane Library, PROSPERO, CINAHL, MEDLINE, PsycINFO, Web of Science Core Collection, ProQuest Dissertations & Theses Database, Evidence Search and OpenGrey will be electronically searched. Reference screening of relevant articles and inquiries to researchers in the field will be undertaken. Studies will be selected if they apply qualitative, quantitative or mixed-methods designs to explore barriers and facilitators experienced by patients, carers and healthcare professionals when managing symptoms in ICYP at end-of-life.Articles will be screened by title and abstract by one reviewer with a second reviewer assessing 10% of the articles. Both reviewers will read and screen all remaining potentially relevant articles. For included articles, one reviewer will extract study characteristics and one will check this.Both reviewers will undertake independent quality assessments of included studies using established and appropriate checklists including The Critical Appraisal Skills Programme Qualitative Checklist; The evaluative criteria of credibility, transferability, dependability and confirmability; The Quality Assessment Tool for Quantitative Studies, and The Mixed Methods Appraisal Tool. Data synthesis methods will be decided after data extraction and assessment. ETHICS AND DISSEMINATION: This review will inform our understanding of symptom management in ICYP at end-of-life. The findings will be reported in a peer-reviewed journal and presented at conferences. The study raises no ethical issues. PROSPERO REGISTRATION NUMBER: CRD42019124797.

Supporting informed choice in acupuncture: effects of a new person-, evidence- and theory-based website for patients with back pain.

OBJECTIVES: To test whether a newly developed person-, theory- and evidence-based website about acupuncture helps patients make informed decisions about whether or not to use acupuncture for back pain. METHODS: A randomised online study compared a newly developed 'enhanced website' to a 'standard website'. The enhanced website provided evidence-based information in a person-based manner and targeted psychological constructs. The standard website was based on a widely used patient information leaflet. In total, 350 adults with recent self-reported back pain were recruited from general practices in South West England. The two primary outcomes were knowledge change and making an informed choice about using acupuncture. Secondary outcomes were beliefs about and willingness to have acupuncture. RESULTS: Participants who viewed the enhanced acupuncture website had a significantly greater increase in knowledge about acupuncture (M = 1.1, standard deviation (SD) = 1.7) than participants who viewed the standard website (M = 0.2, SD = 1.1; F(1, 315) = 37.93, p < 0.001, η2 = .107). Participants who viewed the enhanced acupuncture website were also 3.3 times more likely to make an informed choice about using acupuncture than those who viewed the standard website (χ2(1) = 23.46, p < 0.001). There were no significant effects on treatment beliefs or willingness to have acupuncture. CONCLUSION: The enhanced website improved patients' knowledge and ability to make an informed choice about acupuncture, but did not optimise treatment beliefs or change willingness to have acupuncture. The enhanced website could be used to support informed decision-making among primary care patients and members of the general public considering using acupuncture for back pain.

On-line preparatory information for children and their families undergoing dental extractions under general anesthesia: A phase III randomized controlled trial.

BACKGROUND: Family-centered interactive on-line games are increasingly popular in healthcare, but their effectiveness for preoperative preparation needs further research. www.scottga.org is the new on-line version of a proven nonweb-based game for children and parents/caregivers. AIMS: The aim of this study was to evaluate if www.scottga.org improved children's anxiety and families' satisfaction compared with controls. METHODS: In this phase III double-blind randomized controlled trial, children/parents/caregivers received (i) www.scottga.org, (ii) standard care, or (iii) a placebo hand-washing game. The intervention and placebo games were available online for home usage and provided again on the ward before surgery. All children were accompanied by parent/caregivers at induction and observed and scored using validated measures. Stratified randomization and generalized linear models were used. An intention-to-treat approach was adopted. RESULTS: Overall, 52/176 children had baseline "psychological disturbance." Children's anxiety increased preinduction, but there were no differences between groups (Facial Image Scale: video-standard OR = 1.08, P = .82, 95% CI [0.56, 2.1]; video-placebo OR = 0.9, P = .77 95% CI [0.46, 1.8]). There were no differences in induction behavior (visual analog scale: video mean = 3.5; standard care mean = 3.5; placebo mean = 3.7: video-standard OR = 2.0, P = .42, 95% CI [-0.6, 1.3]; video-placebo OR = 1.53, P = .65, 95% CI [-0.8, 1.1]) or induction anxiety (modified Yale Preoperative Anxiety Scale: video-standard OR 1.02, P = .97, 95% CI [0.61, 2.6]; video-placebo OR 1.38, P = .49, 95% CI [0.87, 3.81]). Families favored the intervention regarding the "child handling the visit better" (Treatment Evaluation Inventory: video-standard OR = 12; 95% CI 4.7-32; P < .001; video-placebo OR = 8.2; 95% CI 3-22; P < .001) and "improving the child's ability to cope" (Treatment Evaluation Inventory: video-standard OR = 21; 95% CI 8-56; P < .001 and video-placebo OR = 13; 95% CI 5-34; P < .001). CONCLUSION: Families believed that a video-game preparation helped their child's perioperative anxiety, but there were no objective measures of behavioral improvement associated with this intervention.

Persistent postsurgical pain in children and young people: prediction, prevention, and management.

Ensuring optimum preoperative and postoperative pain management should always be a priority in children.

Assessing knowledge about acupuncture: A survey of people with back pain in the UK.

OBJECTIVES: Despite the prevalence of acupuncture treatment in the UK, and the increasing evidence of safety and effectiveness, the information presented to patients by practitioners frequently contains inaccuracies. As knowledge of treatment affects both patient decision-making and treatment outcomes, this study aimed to establish what is known about acupuncture in a sample of people who had, and had not, previously experienced acupuncture. DESIGN: A 15-item questionnaire was constructed to assess knowledge of acupuncture. SETTING: Online survey of people with a history of back pain. RESULTS: 202 participants completed the questionnaire. 66.8% of the sample was female and 33.2% male, with a mean age of 35 years (range 18-74 years). 87.6% had back pain in the past six months, 44.1% currently. 21.8% had previously received acupuncture, and 69.8% had previously read or heard information about acupuncture. On average participants answered 11.03 of 15 questions about acupuncture correctly (SD=2.64). Items relating to common concerns about acupuncture, acupuncture efficacy, and types of acupuncture were correctly answered by ≥80% of participants. Participants possessed less knowledge of accessibility, Government legislation, and methods of administration. CONCLUSIONS: The study identified key gaps in knowledge about acupuncture among patients. In particular, many participants were unaware that acupuncture is available from the UK National Health Service and that acupuncturists are not subject to statutory regulation in the UK. These knowledge gaps should be addressed in order to increase people's understanding of and access to acupuncture.

Treatment of Female Sexual Pain Disorders: A Systematic Review.

Sexual pain disorders affect women's sexual and reproductive health and are poorly understood. Although many treatments have been evaluated, there is no one "gold standard" treatment. The aim of this systematic review was to investigate what treatments for female sexual pain have been evaluated in clinical studies and their effectiveness. The search strategy resulted in 65 papers included in this review. The articles were divided into the following categories: medical treatments; surgical treatments; physical therapies; psychological therapies; comparative treatment studies; and miscellaneous and combined treatments. Topical and systemic medical treatments have generally been found to lead to improvements in, but not complete relief of, pain, and side effects are quite common. Surgical procedures have demonstrated very high success rates, although there has been variability in complete relief of pain after surgery, which suggests less invasive treatments should be considered first. Physical therapies and psychological therapies have been shown to be promising treatments, supporting a biopsychosocial approach to sexual pain disorders. Although most of the interventions described have been reported as effective, many women still experience pain. A multidisciplinary team with active patient involvement may be needed to optimize treatment outcome.

Screening for cognitive deficits in 8 to 14-year old children with cerebellar tumors using self-report measures of executive and behavioral functioning and health-related quality of life.

BACKGROUND: We aimed to identify a brief screening measure for detection of cognitive deficit in children treated for cerebellar tumors that would be useful in clinical practice. METHODS: A sample of 72 children, aged 8-14 years, and within 3 years post diagnosis for standard-risk medulloblastoma (n = 37) or low-grade cerebellar astrocytoma (n = 35) and 38 children in a nontumor group were assessed using teacher-, parent-, and child-report of the Behavior Rating Inventory of Executive Function (BRIEF), Strengths and Difficulties Questionnaire (SDQ), and Pediatric Quality of Life Inventory (PedsQL). The accuracy of these scores as a screen for a full-scale Intelligence Quotient (FSIQ) < 80 on the Wechsler Intelligence Scale for Children (WISC-IV UK) was assessed using their receiver operating characteristic (ROC) curves. RESULTS: The questionnaires with the highest areas under the ROC curves were the child- and parent-report PedsQL, the teacher-report BRIEF, and the SDQ. At optimal cutoff scores, their sensitivities (95% CIs) to cases of FSIQ < 80 were 84 (60-96)%, 65 (41-84)%, 79 (54-93)%, and 84 (60-96)%, and their specificities (95% CIs) were 79 (68-86)%, 87 (77-93)%, 77 (66-86)%, and 71 (64-84)% respectively. All cases of FSIQ < 80 screened positive on either teacher-report SDQ or self-report PedsQL. CONCLUSIONS: The PedsQL child- and parent-report and the teacher-report BRIEF and SDQ have moderately good accuracy for discriminating between children with and without a FSIQ < 80. The PedsQL could be used in a clinical setting, and the BRIEF and SDQ in an educational setting, to screen for cases with FSIQ < 80 in children treated for brain tumors.

Pain care for patients with epidermolysis bullosa: best care practice guidelines.

BACKGROUND: Inherited 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. METHODS: The 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. RESULTS: The 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. CONCLUSIONS: Evidence-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.

Child-related characteristics predicting subsequent health-related quality of life in 8- to 14-year-old children with and without cerebellar tumors: a prospective longitudinal study.

BACKGROUND: We identified child-related determinants of health-related quality of life (HRQoL) in children aged 8-14 years who were treated for 2 common types of pediatric brain tumors. METHODS: Questionnaire measures of HRQoL and psychometric assessments were completed by 110 children on 3 occasions over 24 months. Of these 110, 72 were within 3 years of diagnosis of a cerebellar tumor (37 standard-risk medulloblastoma, 35 low-grade cerebellar astrocytoma), and 38 were in a nontumor group. HRQoL, executive function, health status, and behavioral difficulties were also assessed by parents and teachers as appropriate. Regression modeling was used to relate HRQoL z scores to age, sex, socioeconomic status, and 5 domains of functioning: Cognition, Emotion, Social, Motor and Sensory, and Behavior. RESULTS: HRQoL z scores were significantly lower after astrocytoma than those in the nontumor group and significantly lower again in the medulloblastoma group, both by self-report and by parent-report. In regression modeling, significant child-related predictors of poorer HRQoL z scores by self-report were poorer cognitive and emotional function (both z scores) and greater age (years) at enrollment (B = 0.038, 0.098, 0.136, respectively). By parent-report, poorer cognitive, emotional and motor or sensory function (z score) were predictive of lower subsequent HRQoL of the child (B = 0.043, 0.112, 0.019, respectively), while age at enrollment was not. CONCLUSIONS: Early screening of cognitive and emotional function in this age group, which are potentially amenable to change, could identify those at risk of poor HRQoL and provide a rational basis for interventions to improve HRQoL.

Unstimulated cortisol secretory activity in everyday life and its relationship with fatigue and chronic fatigue syndrome: a systematic review and subset meta-analysis.

The hypothalamic-pituitary-adrenal (HPA) axis is a psychoneuroendocrine regulator of the stress response and immune system, and dysfunctions have been associated with outcomes in several physical health conditions. Its end product, cortisol, is relevant to fatigue due to its role in energy metabolism. The systematic review examined the relationship between different markers of unstimulated salivary cortisol activity in everyday life in chronic fatigue syndrome (CFS) and fatigue assessed in other clinical and general populations. Search terms for the review related to salivary cortisol assessments, everyday life contexts, and fatigue. All eligible studies (n=19) were reviewed narratively in terms of associations between fatigue and assessed cortisol markers, including the cortisol awakening response (CAR), circadian profile (CP) output, and diurnal cortisol slope (DCS). Subset meta-analyses were conducted of case-control CFS studies examining group differences in three cortisol outcomes: CAR output; CAR increase; and CP output. Meta-analyses revealed an attenuation of the CAR increase within CFS compared to controls (d=-.34) but no statistically significant differences between groups for other markers. In the narrative review, total cortisol output (CAR or CP) was rarely associated with fatigue in any population; CAR increase and DCS were most relevant. Outcomes reflecting within-day change in cortisol levels (CAR increase; DCS) may be the most relevant to fatigue experience, and future research in this area should report at least one such marker. Results should be considered with caution due to heterogeneity in one meta-analysis and the small number of studies.

Cancer pain: part 2: physical, interventional and complimentary therapies; management in the community; acute, treatment-related and complex cancer pain: a perspective from the British Pain Society endorsed by the UK Association of Palliative Medicine and the Royal College of General Practitioners.

OBJECTIVE: This discussion document about the management of cancer pain is written from the pain specialists' perspective in order to provoke thought and interest in a multimodal approach to the management of cancer pain, not just towards the end of life, but pain at diagnosis, as a consequence of cancer therapies, and in cancer survivors. It relates the science of pain to the clinical setting and explains the role of psychological, physical, interventional and complementary therapies in cancer pain. METHODS: This document has been produced by a consensus group of relevant healthcare professionals in the United Kingdom and patients' representatives making reference to the current body of evidence relating to cancer pain. In the second of two parts, physical, invasive and complementary cancer pain therapies; treatment in the community; acute, treatment-related and complex cancer pain are considered. CONCLUSIONS: It is recognized that the World Health Organization (WHO) analgesic ladder, whilst providing relief of cancer pain towards the end of life for many sufferers world-wide, may have limitations in the context of longer survival and increasing disease complexity. To complement this, it is suggested that a more comprehensive model of managing cancer pain is needed that is mechanism-based and multimodal, using combination therapies including interventions where appropriate, tailored to the needs of an individual, with the aim to optimize pain relief with minimization of adverse effects.