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PurposeThis study assessed adherence to, feasibility of, and barriers and facilitators of implementation of an app developed to monitor and follow-up with pain in children with cancer at home.MethodsChildren (8–18 years) receiving cancer treatment (all diagnoses) or their parents (of children aged 0–7 years) used the KLIK Pain Monitor app for 3 weeks. Pain was assessed twice daily using an 11-point numeric rating scale (NRS-11) (ranging from 0 to 10). Healthcare professionals (HCP’s) from the hospital’s Pediatric Pain Service were instructed to follow-up with clinically significant pain scores (≥ 4) within 120 min (scores 4–6) or 30 min (scores 7–10). Adherence, feasibility, and implementation outcomes were assessed using questionnaires, app log data, and interviews.ResultsTwenty-seven children (M age = 7.3 years, 51.8% male) and six HCP’s participated. Sixty-three percent (N = 17) of families used the app on a daily basis during three weeks, and 18.5% (N = 5) reported pain scores twice daily during that time (family adherence). Twelve out of 27 children (44.4%) reported a clinically significant pain score at least once. In 70% (14/20) of clinically significant pain scores, HCP’s followed-up with families within the set timeframe (HCP adherence). Outcomes reveal feasibility for the majority of app functions (i.e., positive evaluation by ≥ 70% families/HCP’s), and non-feasible aspects could be resolved. Identified barriers and facilitators were used to improve future implementation efforts.ConclusionUse of the KLIK Pain Monitor app seems feasible. Future research will determine its effectiveness in reducing pain in children with cancer at home.

Background Children with life-threatening and life-limiting conditions and their families are confronted with many uncertainties, incremental losses, and difficult decisions. Attentiveness to psychosocial care, including psychological, social, and spiritual concerns, is essential during the child’s life until end-of-life and during bereavement and determines quality of life and wellbeing of child and family. However, use and integration of psychosocial care is found to be challenging. In this paper, we provide recommendations that aim to optimise psychosocial care including preloss and bereavement care in paediatric palliative care. Methods A multidisciplinary guideline development panel of 25 experts in paediatric palliative care and nine parents was established to develop recommendations on psychosocial care including preloss and bereavement care. We performed a systematic literature search to identify evidence focussing on effectivity of interventions described in systematic reviews (SRs) of randomised controlled trials (RCTs) and RCTs. From the search, we also derived evidence from SRs of observational, qualitative, or mixed-method studies on preloss and bereavement care. The GRADE methodology was used for appraising evidence. We used existing guidelines to deal with lack of evidence. Recommendations were formulated based on evidence, existing guidelines, clinical expertise, and patient and family values. Results We identified a total two SRs of RCTs and six RCTs that reported on the effect of psychological interventions, spiritual support interventions, and bereavement care intervention. The evidence showed that only some of the interventions improve certain psychosocial outcomes in children or parents. We were unable to draw conclusions on which intervention is most effective, due to the high variability in interventions and measured outcomes. The evidence from ten SRs of observational, qualitative, or mixed-method studies was used as a basis for preloss and bereavement care recommendations. In total, we formulated 48 recommendations. Discussion With the provided recommendations, we aim to optimise psychosocial care for children with life-threatening and life-limiting conditions and their families. Psychosocial care should be tailored to the child and family. We found a lack of evidence on psychosocial care including preloss and bereavement care. Although very challenging, we propose implementing strategies to improve research and thereby further optimise quality of paediatric palliative care. These strategies include the development of a standard set of outcomes and to conduct well-designed multisite RCTs.

Background In paediatric palliative care, children with life-threatening and life-limiting conditions, their families, and their health care professionals often face difficult decisions about treatment, goals of care, and delivery of care. Advance care planning and shared decision-making are strategies that can improve quality of care by discussing goals and preferences on future care. In this paper, we provide recommendations that aim to optimise advance care planning and shared decision-making in paediatric palliative care in the Netherlands. Methods A multidisciplinary guideline panel of 20 experts in paediatric palliative care and nine (bereaved) parents was established to develop recommendations on advance care planning and shared decision-making. We performed systematic literature searches to identify quantitative and qualitative evidence and used the GRADE (CERQual) methodology for appraisal of evidence. Recommendations were formulated based on quantitative and qualitative evidence, clinical expertise, and patient and family experiences. Results We identified 4 RCTs that reported on the effect of advance care planning interventions in paediatric palliative care and 33 qualitative studies on barriers and facilitators to advance care planning and shared decision-making. We formulated 28 strong recommendations in close collaboration with a multidisciplinary guideline panel that provide guidance to offer advance care planning and shared decision-making, involve children and their family, and communicate information about care and treatment. Conclusion The identified evidence and recommendations support the use of advance care planning and shared decision-making in paediatric palliative care. However, we found several knowledge gaps that should be addressed. As advance care planning and shared decision-making require specific skills and can be time-consuming, we emphasise the importance of education, adequate staffing and sufficient funding to improve integration in clinical practice. We do believe that our recommendations can be used as a starting point to develop recommendations in other countries. However, country-specific factors should be very carefully considered before applying any recommendations in other countries.

Medulloblastoma, the most common malignant paediatric brain tumour, is currently treated with nonspecific cytotoxic therapies including surgery, whole-brain radiation, and aggressive chemotherapy. As medulloblastoma exhibits marked intertumoural heterogeneity, with at least four distinct molecular variants, previous attempts to identify targets for therapy have been underpowered because of small samples sizes. Here we report somatic copy number aberrations (SCNAs) in 1,087 unique medulloblastomas. SCNAs are common in medulloblastoma, and are predominantly subgroup-enriched. The most common region of focal copy number gain is a tandem duplication of SNCAIP , a gene associated with Parkinson’s disease, which is exquisitely restricted to Group 4α. Recurrent translocations of PVT1 , including PVT1-MYC and PVT1-NDRG1 , that arise through chromothripsis are restricted to Group 3. Numerous targetable SCNAs, including recurrent events targeting TGF-β signalling in Group 3, and NF-κB signalling in Group 4, suggest future avenues for rational, targeted therapy. Medulloblastoma is the most common malignant brain tumour in children; having assembled over 1,000 samples the authors report that somatic copy number aberrations are common in medulloblastoma, in particular a tandem duplication of SNCAIP , a gene associated with Parkinson’s disease, which is restricted to subgroup 4α, and translocations of PVT1 , which are restricted to Group 3. The medulloblastoma genome dissected Medulloblastoma is the most common malignant brain tumour in children. Four papers published in the 2 August 2012 issue of Nature use whole-genome and other sequencing techniques to produce a detailed picture of the genetics and genomics of this condition. Notable findings include the identification of recurrent mutations in genes not previously implicated in medulloblastoma, with significant genetic differences associated with the four biologically distinct subgroups and clinical outcomes in each. Potential avenues for therapy are suggested by the identification of targetable somatic copy-number alterations, including recurrent events targeting TGFβ signalling in Group 3, and NF-κB signalling in Group 4 medulloblastomas.

Background Advance Care Planning (ACP) enables patients and relatives to define and share values, goals and preferences for future medical treatment and care. The IMplementing Pediatric Advance Care Planning Toolkit (IMPACT), developed in the Netherlands, is a method for conducting ACP in pediatric palliative care. Healthcare professionals who were trained to use IMPACT, indicated their need for ongoing support to practice ACP communication skills optimally over time. Therefore, we developed a team-based learning program aimed at teaching participants how to transfer knowledge on ACP, continue practicing ACP communication skills and reflect on ACP conversations within their own team context. The aim of this study was to evaluate the program’s transfer of knowledge as well as the professionals’ experience and team reflection on ACP. Methods A one-day IMPACT train-the-trainer course was developed and a selection of healthcare professionals (facilitators) from pediatric palliative care teams (PPCTs) from all seven Dutch university hospitals and the specialized Center for Pediatric Oncology were invited to participate. Hereafter, facilitators were asked to transfer their course-acquired knowledge to their team members (learners) by organizing two coaching-on-the-job sessions. A mixed-methods design, combining questionnaires and field notes, was used to evaluate the level of knowledge transfer and team reflection achieved. Results Eighteen healthcare professionals in the role of facilitator participated in the train-the-trainer course. In seven PPCTs one ( n  = 3) or two ( n  = 4) coaching-on-the-job session(s) took place, attended by 29 and 17 learners, respectively. In the questionnaires, 11 facilitators indicated that they had to some extent transferred acquired knowledge to their team members as intended. Sixteen out of 21 learners who participated in at least one coaching-on-the-job session, reported (somewhat) increased self-confidence for conducting ACP conversations. The reported main strength of the program was practicing with/learning from colleagues whereas dealing with workload and variation in existing ACP skills within PPCTs need more attention. Conclusions The newly developed team-based learning program resulted in intended transfer of knowledge and methodical reflection on ACP in coaching-on-the-job sessions in most participating PPCTs. Planning coaching-on-the-job sessions regarding ACP in pediatric palliative care with multiple healthcare professionals is challenging and needs more emphasis in the training.

Background Provision of paediatric palliative care for children with life-threatening or life-limiting conditions and their families is often complex. Guidelines can support professionals to deliver high quality care. Stakeholders expressed the need to update the first Dutch paediatric palliative care guideline with new scientific literature and new topics. This paper provides an overview of the methodology that is used for the revision of the Dutch paediatric palliative care guideline and a brief presentation of the identified evidence. Methods The revised paediatric palliative care guideline was developed with a multidisciplinary guideline panel of 72 experts in paediatric palliative care and nine (bereaved) parents of children with life-threatening or life-limiting conditions. The guideline covered multiple topics related to (refractory) symptom treatment, advance care planning and shared-decision making, organisation of care, psychosocial care, and loss and bereavement. We established six main working groups that formulated 38 clinical questions for which we identified evidence by updating two existing systematic literature searches. The GRADE (CERQual) methodology was used for appraisal of evidence. Furthermore, we searched for additional literature such as existing guidelines and textbooks to deal with lack of evidence. Results The two systematic literature searches yielded a total of 29 RCTs or systematic reviews of RCTs on paediatric palliative care interventions and 22 qualitative studies on barriers and facilitators of advance care planning and shared decision-making. We identified evidence for 14 out of 38 clinical questions. Furthermore, we were able to select additional literature (29 guidelines, two textbooks, and 10 systematic reviews) to deal with lack of evidence. Conclusions The revised Dutch paediatric palliative care guideline addresses many topics. However, there is limited evidence to base recommendations upon. Our methodology will combine the existing evidence in scientific literature, additional literature, expert knowledge, and perspectives of patients and their families to provide recommendations.

Background Children with life-threatening and life-limiting conditions can experience high levels of suffering due to multiple distressing symptoms that result in poor quality of life and increase risk of long-term distress in their family members. High quality symptom treatment is needed for all these children and their families, even more so at the end-of-life. In this paper, we provide evidence-based recommendations for symptom treatment in paediatric palliative patients to optimize care. Methods A multidisciplinary panel of 56 experts in paediatric palliative care and nine (bereaved) parents was established to develop recommendations on symptom treatment in paediatric palliative care including anxiety and depression, delirium, dyspnoea, haematological symptoms, coughing, skin complaints, nausea and vomiting, neurological symptoms, pain, death rattle, fatigue, paediatric palliative sedation and forgoing hydration and nutrition. Recommendations were based on evidence from a systematic literature search, additional literature sources (such as guidelines), clinical expertise, and patient and family values. We used the GRADE methodology for appraisal of evidence. Parents were included in the guideline panel to ensure the representation of patient and family values. Results We included a total of 18 studies that reported on the effects of specific (non) pharmacological interventions to treat symptoms in paediatric palliative care. A few of these interventions showed significant improvement in symptom relief. This evidence could only (partly) answer eight out of 27 clinical questions. We included 29 guidelines and two textbooks as additional literature to deal with lack of evidence. In total, we formulated 221 recommendations on symptom treatment in paediatric palliative care based on evidence, additional literature, clinical expertise, and patient and family values. Conclusion Even though available evidence on symptom-related paediatric palliative care interventions has increased, there still is a paucity of evidence in paediatric palliative care. We urge for international multidisciplinary multi-institutional collaboration to perform high-quality research and contribute to the optimization of symptom relief in palliative care for all children worldwide.

Background: Childhood brain tumor survivors (CBTS) are at risk for hypothalamic-pituitary (HP) dysfunction, mainly caused by radiation exposure or tumor involvement of the HP-region. The risk for HP dysfunction (HPD) may vary between different age groups due to maturation of the brain and differences in oncologic treatment protocols. The aim of this study was to determine the prevalence and risk factors of HPD in infant (IBTS) and toddler brain tumor survivors (TBTS) compared to older childhood brain tumor survivors (OCBTS). Patients and Methods: A retrospective analysis in a nationwide cohort of CBTS was performed. Prevalence and risk factors for HPD were compared between IBTS (aged 0-1 years at diagnosis), TBTS (aged 1-3 years at diagnosis) and OCBTS (aged >3-18 years at diagnosis). Results: In 718 included CBTS, with a median follow-up time of 7.9 years, overall no differences in percentage of HPD were found between the three age groups. Treatment with radiotherapy (RT) (OR 15.41; 95%CI 8.33 to 28.48), suprasellar tumor location (OR 46.62; 95%CI 19.64 to 110.66) and younger age (OR 1.09; 95%CI 1.02 to 1.15) were associated with HP dysfunction. Because IBTS were significantly less often treated with RT, subanalyses were performed for all CBTS not treated with radiation (n=459). In non-irradiated CBTS, IBTS and TBTS were significantly more frequently diagnosed with TSH-, ACTH- and ADH deficiency, compared to ECBTS. IBTS and TBTS showed significantly more weight gain (p<0.0001) and smaller height SDS (p=0.001) during follow-up. Conclusion: Infant and toddler brain tumor survivors seem to be more vulnerable to develop HP dysfunction than when compared to older children. These results emphasize the importance of special infant and toddlers brain tumor treatment protocols and endocrine surveillance in children treated for a brain tumor at young age.