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Objectives To describe and compare characteristics of care provided at the end of life for children with chronic complex con- ditions and neonates who died in an ICU with those who died outside an ICU. Design Substudy of a nation-wide retrospective chart review. Setting: Thirteen hospitals, including 14 pediatric and neonatal ICUs, two long-term institutions, and 10 community-based organ- izations in the three language regions of Switzerland. Patients: One hundred forty-nine children (0–18 yr) who died in the years 2011 or 2012. Causes of death were related to cardiac, neurologic, oncological, or neonatal conditions. Interventions None. Measurements and Main Results Demographic and clinical characteristics, therapeutic procedures, circumstances of death, and pat- terns of decisional processes were extracted from the medical charts. Ninety-three (62%) neonates (median age, 4 d) and children (median age, 23 mo) died in ICU, and 56 (38%) with a median age of 63 months outside ICU. Generally, ICU patients had more therapeutic and invasive procedures, compared with non-ICU patients. Changes in treatment plan in the last 4 weeks of life, such as do-not-resusci- tate orders occurred in 40% of ICU patients and 25% of non-ICU patients (p < 0.001). In the ICU, when decision to withdraw life-sus- taining treatment was made, time to death in children and newborns was 4:25 and 3:00, respectively. In institutions where it was available, involvement of specialized pediatric palliative care services was recorded in 15 ICU patients (43%) and in 18 non-ICU patients (78%) (p = 0.008). Conclusions This nation-wide study demonstrated that patients with a complex chronic condition who die in ICU, compared with those who die outside ICU, are characterized by fast changing care situations, including when to withdraw life-sustaining treatment. This highlights the importance of early effective communication and shared decision making among clinicians and families. (Pediatr Crit Care Med 2020; XX:00–00)

Pediatric palliative care (PPC) is different from palliative care (PC) for adults. However, conceptualizing PPC remains cumbersome due to the high heterogeneity of often rare diseases, the high diversity of disease trajectories, and the particular difficulty to predict the future of an individual, severely ill child. This article aims to provide an overview and critical reflection of different aspects of prognostication in children with PC needs. This includes different diseases from neurology to oncology, from the unborn baby to the young adult, new approaches in treatment, advance care planning, and, most important, communication with the affected child as well as parents.

Background Mothers and fathers are severely challenged when providing care for their terminally ill child at end of life. Caregiving needs have been studied predominantly in mothers. Differences in caregiving needs between mothers and fathers during their child’s end of life have not, however, been explored so far. This knowledge is of importance to best meet individual parental needs in paediatric end-of-life care. Methods Secondary analysis of a quantitative survey on parental needs during their child’s last 4 weeks of life, collected in the Swiss multicentre “Paediatric End-of-Life Care Needs” (PELICAN) study. Caregiving needs of mothers and fathers (parental dyad) who had lost a child due to a cardiological, neurological or oncological disease or during the neonatal period in the years 2011–2012 were retrospectively assessed using a questionnaire representing six evidence-based quality domains of paediatric palliative and end-of-life care. Results Seventy-eight parental dyads were included in this analysis. Differences between mothers and fathers were mostly found around needs to be supported as a family. In all, 28 out of 34 needs-related questionnaire items were scored higher by mothers than by fathers, indicating higher importance for that need to be met. The results indicate that these differences might relate to different caregiving roles and gender-specific coping strategies. Conclusions To best meet parental needs in paediatric end-of-life care, particular attention should be paid to both mothers and fathers and their specific caregiving roles, as differences in these roles might influence their needs in this exceptional situation. Therefore, healthcare professionals should identify how parental dyads mutually navigate care for their sick child to best meet their needs in support. Additionally, mothers and fathers should be supported in their individual coping strategies.

BACKGROUND A clinical research application must be submitted for approval by a competent ethics committee (EC) before a study can be executed. There is very limited information on how such submissions could be optimised, especially regarding research in children and adolescents, which requires particular caution and age-adapted patient information. METHODS We assessed all research applications from the University Children’s Hospital Zurich submitted to the EC of the Canton of Zurich in 2014–2015, i.e., in the first two years after Switzerland’s new Human Research Act came into effect. Moreover, we validated our findings by assessing a randomly selected sample of applications from the same hospital in 2018–2019. RESULTS We assessed a total of 86 applications from 2014–2015, originating from 29 departments and sub-specialties. The EC judged that it was not responsible for three applications and declined an assessment for another three because the studies had already been conducted. Thus, we included 80 applications in the present analysis (18 clinical trials, 52 research projects, 10 further use projects). Applicants withdrew four applications before the EC’s final decision and the EC rejected two after assessment. The EC had objections in 46 (62%) of the remaining 74 applications. Formal, including formal legal, objections (n = 503) and legal objections (n = 287) accounted for the vast majority of objections. There were also 71 ethical and 82 scientific objections. The most frequent formal and formal legal objections were incomplete or missing age-adapted patient information (49%) and incorrect templates for informed consent and signature forms (46%). A review of the 20 randomly selected applications from 2018–2019 confirmed that four out of the five most frequent deficiencies relating to informed consent were identical to those observed in the 2014–2015 applications. CONCLUSIONS Careful preparation of submission documents by the investigators and close adherence to formal and legal requirements have the potential to considerably optimise and expedite the EC review process, and thus the commencement of the clinical research. Keywords: age-adapted patient information, formal deficiencies, legal deficiencies, ethical deficiencies, scientific deficiencies

The objective of this qualitative study was to explore the perceptions and needs of families who care for a child with a life-limiting disease. Considering the heterogeneity of life-limiting diseases in childhood, three diagnostic groups were defined: (a) cancer, (b) neurological disorders, and (c) non-cancer/non-neurological conditions. Fifteen parents whose child had been treated in one of four children's hospitals and received palliative care or had died within the previous 2 years were interviewed. The main interview topics were: communication with professionals, need for support in care (at home or hospital), and bereavement support. Irrespective of the center of care, parents of children with diagnoses other than cancer reported a lack of support concerning practical issues of care and psychosocial aspects. Parents of children with cancer expressed difficulties related to coordination of care especially when care was provided at home. Bereaved parents emphasized their wish for bereavement support. Our findings demonstrate shortcomings in pediatric palliative care in Switzerland and outline basic needs of affected families including psychosocial support, coordination of care and bereavement support. Based on these findings we formulate some suggestions on how to initiate pediatric palliative care in a most efficient way and tailored to the needs of families in Switzerland.

Aim To develop and test the Parental PELICAN Questionnaire, an instrument to retrospectively assess parental experiences and needs during their child's end-of-life care. Background To offer appropriate care for dying children, healthcare professionals need to understand the illness experience from the family perspective. A questionnaire specific to the end-of-life experiences and needs of parents losing a child is needed to evaluate the perceived quality of paediatric end-of-life care. Design This is an instrument development study applying mixed methods based on recommendations for questionnaire design and validation. Method The Parental PELICAN Questionnaire was developed in four phases between August 2012–March 2014: phase 1: item generation; phase 2: validity testing; phase 3: translation; phase 4: pilot testing. Psychometric properties were assessed after applying the Parental PELICAN Questionnaire in a sample of 224 bereaved parents in April 2014. Validity testing covered the evidence based on tests of content, internal structure and relations to other variables. Results The Parental PELICAN Questionnaire consists of approximately 90 items in four slightly different versions accounting for particularities of the four diagnostic groups. The questionnaire's items were structured according to six quality domains described in the literature. Evidence of initial validity and reliability could be demonstrated with the involvement of healthcare professionals and bereaved parents. Conclusion The Parental PELICAN Questionnaire holds promise as a measure to assess parental experiences and needs and is applicable to a broad range of paediatric specialties and settings. Future validation is needed to evaluate its suitability in different cultures.

Background Paediatric end-of-life care is challenging and requires a high level of professional expertise. It is important that healthcare teams have a thorough understanding of paediatric subspecialties and related knowledge of disease-specific aspects of paediatric end-of-life care. The aim of this study was to comprehensively describe, explore and compare current practices in paediatric end-of-life care in four distinct diagnostic groups across healthcare settings including all relevant levels of healthcare providers in Switzerland. Methods In this nationwide retrospective chart review study, data from paediatric patients who died in the years 2011 or 2012 due to a cardiac, neurological or oncological condition, or during the neonatal period were collected in 13 hospitals, two long-term institutions and 10 community-based healthcare service providers throughout Switzerland. Results Ninety-three (62%) of the 149 reviewed patients died in intensive care units, 78 (84%) of them following withdrawal of life-sustaining treatment. Reliance on invasive medical interventions was prevalent, and the use of medication was high, with a median count of 12 different drugs during the last week of life. Patients experienced an average number of 6.42 symptoms. The prevalence of various types of symptoms differed significantly among the four diagnostic groups. Overall, our study patients stayed in the hospital for a median of six days during their last four weeks of life. Seventy-two patients (48%) stayed at home for at least one day and only half of those received community-based healthcare. Conclusions The study provides a wide-ranging overview of current end-of-life care practices in a real-life setting of different healthcare providers. The inclusion of patients with all major diagnoses leading to disease- and prematurity-related childhood deaths, as well as comparisons across the diagnostic groups, provides additional insight and understanding for healthcare professionals. The provision of specialised palliative and end-of-life care services in Switzerland, including the capacity of community healthcare services, need to be expanded to meet the specific needs of seriously ill children and their families.

End of life for children with cancer refers to the closeness of death, and consequently, care at the end of life entails the anticipation of death. Clear medical decisions and best possible symptom control should be in place. Furthermore, clinicians should be prepared to provide anticipatory guidance and to respond to questions that parents may ask including “When will my child die?”; “Will he suffer?”; “What will it be like?”; “What does she need?”; and “How can we be of help?” To assure that a child can die in the family’s preferred location, careful arrangements may need to be made. This chapter will address these end-of-life considerations for children with cancer and their families.

Aim To understand parents’ experiences and needs during a child's end-of-life care at home and to identify systemic factors that influence its provision. Background A child's end-of-life phase is an extremely difficult time for the whole family. Parents have specific needs, especially when they care for a dying child at home. Design Concurrent embedded mixed methods design. Methods This sub-study of the nationwide survey, ‘Paediatric End-of-Life Care Needs in Switzerland’ (2012-2015) included 47 children who received EOL care at home from 2011-2012. We extracted quantitative data from patients’ medical charts and obtained information via parental questionnaire and then compared parents whose child died at home or in hospital by computing generalized estimation equations. We thematically analysed interviews with parents who provided EOL care at home. Results Parents created an intimate lifeworld and a sense of normality for the child at home. They constantly balanced the family's lifeworld with the requirements and challenges posed by the outside world. This work exhausted parents. Parental ‘readiness’ and social support drove EOL care for children at home. Parents needed practical help with housekeeping and had negative experiences when dealing with insurance. In only 34.8% of cases was a child's EOL home care supported by paediatric palliative care team. Conclusion Paediatric end-of-life care at home is only feasible if parents make extraordinary efforts. If family-centred end-of-life home care is provided by a hospital-based paediatric palliative home care team, which includes paid housekeeping help and psychological support, parents needs could be better met. This article is protected by copyright. All rights reserved.

Juvenile myelomonocytic leukemia is a rare myeloproliferative disease in young children. While hematopoietic stem cell transplantation remains the only curative therapeutic option for most patients, children with juvenile myelomonocytic leukemia increasingly receive novel agents in phase I-II clinical trials as pre-transplant therapy or therapy for relapse after transplantation. However, response criteria or definitions of outcome for standardized evaluation of treatment effect in patients with juvenile myelomonocytic leukemia are currently lacking. Here we propose criteria to evaluate the response to the non-transplant therapy and definitions of remission status after hematopoietic stem cell transplantation. For the evaluation of non-transplant therapy, we defined 6 clinical variables (white blood cell count, platelet count, hematopoietic precursors and blasts in peripheral blood, bone marrow blast percentage, spleen size and extramedullary disease) and 3 genetic variables (cytogenetic, molecular and chimerism response) which serve to describe the heterogeneous picture of response to therapy in each individual case. It is hoped that these criteria will facilitate the comparison of results between clinical trials in juvenile myelomonocytic leukemia.

Background: Life partnerships other than marriage are rarely studied in childhood cancer survivors (CCS). We aimed (1) to describe life partnership and marriage in CCS and compare them to life partnerships in siblings and the general population; and (2) to identify socio-demographic and cancer-related factors associated with life partnership and marriage. Methods: As part of the Swiss Childhood Cancer Survivor Study (SCCSS), a questionnaire was sent to all CCS (aged 20–40 years) registered in the Swiss Childhood Cancer Registry (SCCR), aged

BACKGROUND The extent to which physical performance limitations affect the ability of childhood cancer survivors to reach healthy activity levels is unknown. Therefore this study aims to describe the effect of different types of limitations on activity levels in survivors. PROCEDURE Within the Swiss Childhood Cancer Survivor Study we sent a questionnaire to all survivors (≥16 years) registered in the Swiss Childhood Cancer Registry, who survived >5 years and were diagnosed 1976-2005 aged

OBJECTIVES: We aimed to (i) evaluate psychological distress in adolescent survivors of childhood cancer and compare them to siblings and a norm population; (ii) compare the severity of distress of distressed survivors and siblings with that of psychotherapy patients; and (iii) determine risk factors for psychological distress in survivors. METHODS: We sent a questionnaire to all childhood cancer survivors aged

Purpose Despite recommendations, only a proportion of long-term childhood cancer survivors attend follow-up care. We aimed to (1) describe the follow-up attendance of young survivors aged 11–17 years; (2) describe the parental involvement in follow-up, and (3) investigate predictors of follow-up attendance and parental involvement. Methods As part of the Swiss Childhood Cancer Survivor Study, a follow-up questionnaire was sent to parents of childhood cancer survivors aged 11–17 years. We assessed follow-up attendance of the child, parents' involvement in follow-up, illness perception (Brief IPQ), and sociodemographic data. Clinical data was available from the Swiss Childhood Cancer Registry. Results Of 309 eligible parents, 189 responded (67 %; mean time since diagnosis 11.3 years, range 6.8–17.2) and 75 % (n = 141) reported that their child still attended follow-up. Of these, 83 % (n = 117) reported ≥1 visit per year and 17 % (n = 23) reported Conclusion Educating survivors and their parents on the importance and effectiveness of follow-up care might increase attendance in the longer term.

Background & aims Poor diet may increase the risk that childhood cancer survivors (CCS) will suffer from chronic disease. We compared adherence to national dietary recommendations between CCS, their siblings and the Swiss population, identified determinants of adherence, and assessed the association of adherence with cardiovascular disease (CVD) risk profiles. Methods As part of the Swiss Childhood Cancer Survivor Study (SCCSS), a questionnaire was sent to all Swiss resident CCS aged

The main objectives of this qualitative study were to describe the perceptions and needs of pediatric health care professionals (HCPs) taking care of children with palliative care needs and to develop a concept for the first Center of Competence for Pediatric Palliative Care (PPC) in Switzerland. Within two parts of the study, 76 HCPs were interviewed. The main interview topics were: (1) definition of and attitude toward PPC; (2) current provision of PPC; (3) the support needs of HCPs in the provision of PPC; and (4) the role of specialized PPC teams. HCPs expressed openness to PPC and reported distinctive needs for support in the care of these patients. The main tasks of specialized PPC teams in Switzerland would encompass the coaching of attending teams, coordination of care, symptom control, and direct support of affected families during and beyond the illness of their child. Conclusion: This study indicates the need for specialized PPC in Switzerland both inside and outside of centers providing top quality medical care (Spitzenmedizin). Specialized PPC teams could have a significant impact on the care of children and families with PPC needs. Whether hospices are an option in Switzerland remains unanswered; however, a place to meet other families with similar destinies was emphasized.

Objective: This study is the ! rst to assess posttraumatic stress disorder (PTSD) in young children with cancer and to examine individual, medical, and parent-related determinants. Methods: Forty-eight patients with cancer, ages 8‐48 months, were assessed in an average of 15 months after their diagnosis, using mothers as informants to complete the Posttraumatic Stress Disorder Semi-Structured Interview and Observational Record for Infants and Young Children. Additionally, mothers and the attending pediatric oncologist completed questionnaires on various determinants of child PTSD. Results: Nine children (18.8%) met the age-appropriate criteria for full PTSD proposed by Scheeringa et al., and 20 children (41.7%) met criteria for partial PTSD. Multivariate logistic regression analysis revealed that higher child age at diagnosis (odds ratio=1.11) and maternal PTSD severity (odds ratio=1.14) increased the risk of full or partial PTSD in children. Conclusions: This study provides evidence for a substantial prevalence of PTSD in young children with cancer and identi! es important risk factors. Copyright © 2012 John Wiley & Sons, Ltd.

Pediatric palliative care (PPC) focuses on children and adolescents with life-limiting diseases. It may be initiated at various points of the disease trajectory, if possible early enough to support living with the best possible quality of life despite a limited lifespan. From birth to adolescence, children with a broad spectrum of diseases may benefit from PPC. Since 50% of deaths in childhood occur within the first year of life, PPC is just as relevant to neonatology. Causes of death in the neonate and young infant are due to perinatal conditions such as preterm birth and congenital disorders and syndromes; in older children, external causes, such as traumatic injuries, outweigh disease-related causes of death. PPC may last from a few hours or days for neonates to many years for children with complex chronic conditions. For neonates, PPC often has the character of end-of-life (EOL) care followed by bereavement care for the family. For older children, PPC can clearly be differentiated from EOL care; its indications include progress or deterioration of disease, marked instability of the child's condition, increase in the need for technical or medical support, increase in suffering, or failure of treatment. If a child's need for palliative care is established, useless and potentially harmful treatments may be withheld and informed choices can be made about treatment, care, and the remaining life of the child. Conclusion This review aims to provide knowledge for clinicians who care for children and adolescents at risk of dying from their disease. PPC can improve the child's remaining lifetime by focusing on quality of life and goals that are defined by the child and his or her family.

Somatic mutations of the spliceosomal machinery occur frequently in adult patients with myelodysplastic syndrome (MDS). We resequenced SF3B1, U2AF35, and SRSF2 in 371 children with MDS or juvenile myelomonocytic leukemia. We found missense mutations in 2 juvenile myelomonocytic leukemia cases and in 1 child with systemic mastocytosis with MDS. In 1 juvenile myelomonocytic leukemia patient, the SRSF2 mutation that initially coexisted with an oncogenic NRAS mutation was absent at relapse, whereas the NRAS mutation persisted and a second, concomitant NRAS mutation later emerged. The patient with systemic mastocytosis and MDS carried both mutated U2AF35 and KIT in a single clone as confirmed by clonal sequencing. In the adult MDS patients sequenced for control purposes, we detected previously reported mutations in 7/30 and a novel SRSF2 deletion (c.284_307del) in 3 of 30 patients. These findings implicate that spliceosome mutations are rare in pediatric MDS and juvenile myelomonocytic leukemia and are unlikely to operate as driver mutations.

BACKGROUND: The objective of this study was to describe educational achievements of childhood cancer survivors in Switzerland compared with the general population. In particular, the authors investigated educational problems during childhood, final educational achievement in adulthood, and its predictors. METHODS: Childhood cancer survivors who were aged

BACKGROUND Uncertainty about cure puts childhood cancer survivors at risk of mental distress. We asked survivors if they had been told they had been cured and investigated associated factors. PROCEDURE We used nationwide registry data and a questionnaire survey for ≥five-year survivors of childhood cancer (n = 301), followed by online focus groups with a purposive sample of Swiss pediatric oncologists (n = 17). Discussions were coded by investigators using thematic analysis. RESULTS Overall, 235 among 301 survivors (78%; 95% confidence interval, 73%-83%) reported having been told they were cured. The proportion was 89% (81%-97%) among lymphoma and 84% (77%-91%) among leukemia survivors, but only 49% (33%-65%) among central nervous system tumor survivors. Pediatric oncologists acknowledged that telling survivors they are cured may reassure them that their cancer lies behind them. However, many refrained from telling all patients. Reasons included the possibility of late effects (cure disrupted by a continued need for follow-up care) or late relapse (uncertainty of biological cure), case-by-case strategies (use of "cure" according to individual factors), and reluctance (substitution of noncommittal terms for "cure"; waiting for the patient to raise the topic). CONCLUSIONS Not all physicians tell survivors they have been cured; their choices depend on the cancer type and risk of late effects.

Purpose To evaluate the degree of psychological distress in adult childhood cancer survivors in Switzerland and to characterize survivors with significant distress. Methods Childhood cancer survivors who were age younger than 16 years when diagnosed between 1976 and 2003, had survived more than 5 years, and were currently age 20 years or older received a postal questionnaire. Psychological distress was assessed using the Brief Symptom Inventory (BSI). Raw scores were transformed into T scores according to the German norm sample, and the proportion of participants being at increased risk for psychological distress was calculated (case rule: T ≥ 63). t tests and univariable and multivariable logistic regressions were used for statistical analyses. Results One thousand seventy-six survivors (63.% of eligible survivors, 71.9% of contacted survivors) returned the questionnaire, 987 with complete data on BSI. Comparison with the norm populations showed lower T scores (T < 50) in the Global Severity Index (GSI; T = 46.2), somatization (T = 47.6), obsessive-compulsive tendencies (T = 46.9), and anxiety (T = 48.4). However, more childhood cancer survivors (especially women) had increased distress for GSI (14.4%), interpersonal sensitivity (16.5%), depression (13.4%), aggression (16.9%), and psychotic tendencies (15.6%) than the expected 10% from the norm population. Caseness was associated with female sex, being a single child, older age at study, and self-reported late effects, especially psychological problems. Conclusion Results show that childhood cancer survivors, on average, have less psychological distress than a norm population but that the proportion of survivors at risk for high psychological distress is disproportionally large. Monitoring psychological distress in childhood cancer survivors may be desirable during routine follow-up, and psychological support should be offered as needed.

Udgivelsesdato: 2009-Aug-27 Juvenile myelomonocytic leukemia is an aggressive myeloproliferative disorder characterized by malignant transformation in the hematopoietic stem cell compartment with proliferation of differentiated progeny. Seventy-five percent of patients harbor mutations in the NF1, NRAS, KRAS, or PTPN11 genes, which encode components of Ras signaling networks. Using single nucleotide polymorphism arrays, we identified a region of 11q isodisomy that contains the CBL gene in several JMML samples, and subsequently identified CBL mutations in 27 of 159 JMML samples. Thirteen of these mutations alter codon Y371. In this report, we also demonstrate that CBL and RAS/PTPN11 mutations were mutually exclusive in these patients. Moreover, the exclusivity of CBL mutations with respect to other Ras pathway-associated mutations indicates that CBL may have a role in deregulating this key pathway in JMML.

Aim. To present a protocol for a multi-phase study about the current practice of end-of-life care in paediatric settings in Switzerland. Background. In Switzerland, paediatric palliative care is usually provided by teams, who may not necessarily have specific training. There is a lack of systematic data about specific aspects of care at the end of a child’s life, such as symptom management, involvement of parents in decision-making and family-centred care and experiences and needs of parents, and perspectives of healthcare professionals. Design. This retrospective nationwide multicentre study, Paediatric End-of-LIfe CAre Needs in Switzerland (PELICAN), combines quantitative and qualitative methods of enquiry. Methods. The PELICAN study consists of three observational parts, PELICAN I describes practices of end-of-life care (defined as the last 4 weeks of life) in the hospital and home care setting of children (0-18 years) who died in the years 2011– 2012 due to a cardiac, neurological or oncological disease, or who died in the neonatal period. PELICAN II assesses the experiences and needs of parents during the end-of-life phase of their child. PELICAN III focuses on healthcare professionals and explores their perspectives concerning the provision of end-of-life care. Conclusion. This first study across Switzerland will provide comprehensive insight into the current end-of-life care in children with distinct diagnoses and the perspectives of affected parents and health professionals. The results may facilitate the development and implementation of programmes for end-of-life care in children across Switzerland, building on real experiences and needs. Trial registration: ClinicalTrials.gov Identifier: NCT01983852.

PURPOSE Childhood cancer and its treatment may affect health-related quality of life (HRQoL) in childhood cancer survivors, but population-based studies in young survivors are scarce. We aimed to: (1) compare HRQoL between young survivors and population norms and (2) find factors that influence parent-reported HRQoL in survivors. METHODS As part of the Swiss Childhood Cancer Survivor Study, a questionnaire was mailed to parents of survivors aged 8-16 years, registered in the Swiss Childhood Cancer Registry, ≥5 years after diagnosis. We used the KIDSCREEN-27 instrument to compare self- and parent-reported HRQoL between survivors (N = 425) and standardized norms in the five dimensions of physical well-being, psychological well-being, autonomy, peers and school environment (mean = 50, SD = 10). We then used multivariable linear regressions to test the influence of socio-demographic and cancer-related factors on HRQoL. RESULTS Self-reported physical well-being was comparable to norms. Other HRQoL dimensions were higher than norms, with the highest mean = 52.2 (p

Aims (a) To explore parental experiences and needs during their child's end-of-life care at home; (b) to explore patient's characteristics and current provision of paediatric end-of-life care in the home care setting in Switzerland; and (c) to determine influencing system factors impacting end-of-life care at home. Background Parental experiences/needs and paediatric end-of-life care services in the home care setting are influenced by national healthcare policy, determinants of the family and the individual patient. In Switzerland, there is a lack of information about the provision of paediatric end-of-life care at home and related parent's experiences/needs. Design Sub-study of the nationwide multicenter study ‘Paediatric End-of-Life CAre Needs in Switzerland’ using a concurrent qualitative embedded mixed methods design. Methods Data will be collected from January–May 2014 through community care organizations and children's hospitals. The study includes approximately 40–50 families whose child (0–18 years) died in the years 2011–2012 due to a cardiological, neurological or oncological condition and spent at least 21 days at home during the last 4 weeks of life. Qualitative data will be collected through semi-structured interviews with parents and analysed by ‘thematic analysis’. Quantitative data about patient's characteristics will be obtained from patient's medical charts and parental experiences/needs through the parental questionnaire. Appropriate descriptive and inference statistical methods will be used for data analysis. Discussion This study will provide comprehensive basic information about parental needs and patient characteristics for the provision of paediatric end-of-life care and may promote the development of family-centred paediatric end-of-life care services at home. Study registration: The PELICAN-study is registered in the database of Clinical Trial gov. Study ID-number: NCT 01983852.

This article explores the grief process of parents following the death of a child due to a life-limiting illness, putting particular focus on dyadic coping. Participants included 46 married parents (23 couples). A mixed-methods design was used with in-depth interviews and standardized questionnaires. All parents were interviewed separately. Aspects of common dyadic coping (e.g., sharing emotions or maintaining bonds to the child) helped them work through their grief as a couple but also individually. The authors conclude that dyadic coping plays an important role in grief work and adjustment to bereavement.

Background: Paediatric palliative care is still often introduced late in the illness trajectory of children with life-limiting diseases. Translating palliative care into practice continues to be a challenge. Aim: To validate the Paediatric Palliative Screening Scale further by defining attributes that predict the need for palliative care in children between 1 and 18 years. Design: Proportional-odds logistic regression analysis was performed to investigate the relationship between the attributes of the Paediatric Palliative Screening Scale and the experts’ assessment of case vignettes with various combinations of different attribute characteristics. Estimates from regression analysis were transformed to empirical weightings of the Paediatric Palliative Screening Scale attribute characteristics. Setting/participants: Online questionnaires with case vignettes were sent to 33 paediatric palliative care experts from Europe, the United States, Canada, Australia and New Zealand. Results: The highest weightings among the five previously defined attributes were estimated life expectancy Conclusions: According to this second step of psychometric testing of the Paediatric Palliative Screening Scale, the strongest and most urgent necessity indicators for a palliative care approach are life expectancy and child/family preferences. These results are somewhat discrepant with results from the previous validation of the instrument as well as previous research findings.

Background This study compared frequency of alcohol consumption and binge drinking between young adult childhood cancer survivors and the general population in Switzerland, and assessed its socio-demographic and clinical determinants. Procedure Childhood cancer survivors aged 5 years and were currently aged 20–40 years received a postal questionnaire. Reported frequency of alcohol use and of binge drinking were compared to the Swiss Health Survey, a representative general population survey. Determinants of frequent alcohol consumption and binge drinking were assessed in a multivariable logistic regression. Results Of 1,697 eligible survivors, 1,447 could be contacted and 1,049 (73%) responded. Survivors reported more often than controls to consume alcohol frequently (OR = 1.7; 95%CI = 1.3–2.1) and to engage in binge drinking (OR = 2.9; 95%CI = 2.3–3.8). Peak frequency of binge drinking in males occurred at age 24–26 years in survivors, compared to age 18–20 in the general population. Socio-demographic factors (male gender, high educational attainment, French and Italian speaking, and migration background from Northern European countries) were most strongly associated with alcohol consumption patterns among both survivors and controls. Conclusions The high frequency of alcohol consumption found in this study is a matter of concern. Our data suggest that survivors should be better informed on the health effects of alcohol consumption during routine follow-up, and that such counseling should be included in clinical guidelines. Future research should study motives of alcohol consumption among survivors to allow development of targeted health interventions for this vulnerable group.

Atypical Spitz tumors can hardly be differentiated from spitzoid melanoma. CGH might help in the differential diagnosis. An 8 year old child with an atypical Spitz tumor (with a CGH pattern compatible with melanoma) of 8.0 mm Breslow thickness and micrometastases in two lymph node regions was seen at our department. The management and prognosis of atypical Spitz tumors is controversial, and aggressive procedural steps similar to melanoma are usually not recommended. Even performing sentinel lymph node biopsy has been questioned. After extensive interdisciplinary consultations, we did not recommend resection of both lymph node regions and chose instead to follow-up with regular whole-body MRI and adjuvant treatment with pegylated interferon. Treatment decisions for atypical Spitz tumors are a major medical and ethical challenge due to the limited available data.

To identify cytogenetic risk factors predicting outcome in children with advanced myelodysplastic syndrome, overall survival of 192 children prospectively enrolled in European Working Group of Myelodysplastic Syndrome in Childhood studies was evaluated with regard to karyotypic complexity. Structurally complex constitutes a new definition of complex karyotype characterized by more than or equal to 3 chromosomal aberrations, including at least one structural aberration. Five-year overall survival in patients with more than or equal to 3 clonal aberrations, which were not structurally complex, did not differ from that observed in patients with normal karyotype. Cox regression analysis revealed the presence of a monosomal and structurally complex karyotype to be strongly associated with poor prognosis (hazard ratio = 4.6, P < .01). Notably, a structurally complex karyotype without a monosomy was associated with a very short 2-year overall survival probability of only 14% (hazard ratio = 14.5; P < .01). The presence of a structurally complex karyotype was the strongest independent prognostic marker predicting poor outcome in children with advanced myelodysplastic syndrome.

The management of acute appendicitis in the febrile neutropenic patient after intensive chemotherapy is controversial. We report our single-center-experience of 5 children diagnosed with appendicitis during febrile neutropenia after chemotherapy for acute leukemia or lymphoma. All patients presented with an isolated appendicitis without signs of overt mucositis or more diffuse enterocolitis. The clinical diagnosis was confirmed by ultrasonography. Perforation with retrocecal abscess was present in 1 patient. Administration of combination antimicrobial regimens including meropenem resulted in complete resolution in all patients. Our observations indicate that acute appendicitis in clinically stable neutropenic cancer patients can be managed conservatively without surgery.

Background Juvenile myelomonocytic leukemia (JMML) is a rare myeloproliferative disease of infancy. Allogeneic hematopoietic stem cell transplantation (HSCT) is currently the only curative treatment modality, while the role of anti-leukemic therapy prior to HSCT is uncertain. A comparative evaluation of the efficacy of different clinical protocols and great variety of anti-neoplastic drugs applied pre-HSCT is hampered by the lack of uniform criteria of response. Classification schemas applied in other forms of leukemia are of little value, because in JMML therapy may result in divergent responses in solid organs compared to peripheral blood (PB). Procedure We therefore defined separate response criteria for white blood count (WBC), platelet count, liver size, and spleen size. We then retrospectively evaluated the efficacy of 129 treatment courses other than HSCT administered to 63 children with JMML. Treatment consisted of intensive therapy according to AML-type chemotherapy, maintenance-type combination therapy, and single agent therapy. To account for the variability observed in the natural course of disease, we also evaluated 32 episodes of “no therapy.” Results Best responses within 3 months of initiation of therapy were highly variable for the four response criteria. In contrast to platelet count and liver size, there was a significant correlation between WBC or spleen size and therapy. Response rates for WBC and spleen size were best for purine analogs, etoposide, and cytarabine as single agents or for maintenance-type combination therapy. Conclusion To rigorously test future therapeutic strategies in this rare disease an international consensus on the definition of response criteria will be helpful. Pediatr Blood Cancer 2007;49:629–633. © 2006 Wiley-Liss, Inc.

Background The introduction of paediatric palliative care and referral to specialised teams still occurs late in the illness trajectory of children with life-limiting diseases. The aim of this ongoing multipart study was to develop a screening instrument for paediatricians that would improve the timely identification of children who could benefit from a palliative care approach. Methods We used a qualitative study approach with semi-structured interviews (Part 1) and a focus group discussion (Part 2) to define the domains and items of the screening instrument. Seven international paediatric palliative care experts from the UK, France, USA, and Canada took part in face-to-face interviews, and eleven paediatric health professionals from the University Children’s Hospital, Zurich, participated in a subsequent focus group discussion. Results This preliminary phase of development and validation of the instrument revealed five domains relevant to identifying children with life-limiting diseases, who could benefit from palliative care: 1) trajectory of disease and impact on daily activities of the child; 2) expected outcome of disease-directed treatment and burden of treatment; 3) symptom and problem burden; 4) preferences of patient, parents or healthcare professional; and 5) estimated life expectancy. Where palliative care seems to be necessary, it would be introduced in a stepwise or graduated manner. Conclusions This study is a preliminary report of the development of an instrument to facilitate timely introduction of palliative care in the illness trajectory of a severely ill child. The instrument demonstrated early validity and was evaluated as being a valuable approach towards effective paediatric palliative care.

The definition of palliative care is the cornerstone of a medical subspecialty that plays a particular role for all who need it, for all who practice it, and increasingly for those who try to understand it. The difficulties around the definition and terminology arise from problems in separating it from other concepts such as supportive care, constructs such as “palliative care is only about dying”, or, in children, the rather vague use of terms like life-threatening and life-limiting diseases. These weaknesses have been recognized and important steps have been taken. This review discusses current definitions as well as efforts to overcome their weaknesses and make the term palliative care—for both children and adults—more intelligible.

Disclosure: No relevant conflicts of interest to declare. To study the clinical significance of recurrent chromosome aberrations in childhood MDS, cytogenetic data of 394 consecutive children with refractory cytopenia (RC) (N=215), RAEB (N=141) and RAEB-T (N=38) analyzed in the regional cytogenetic reference centers and registered in the prospective study EWOG-MDS 98 between 1998 and 2005 were evaluated. At diagnosis, a karyotype could be defined in 279/394 patients (pts) (71%). No karyotype was obtained in 16% of pts with RC compared to 8% pts with RAEB and RAEB-t (p

RC is one of the most common variants of childhood MDS, characterized by clonal ineffective hematopoiesis and sustained peripheral cytopenia. Allogeneic HSCT represents a widely employed, curative treatment for children with RC. We analyzed the outcome of 48 children (33 males, 15 females; median age at transplantation 11 years, range 2.9–19) affected by RC, given allogeneic HSCT after a myeloablative preparative regimen and reported to the European Working Group on Childhood MDS (EWOG-MDS). Karyotype analysis was available in all patients, but 4. Clonal abnormalities were detected in 17 patients, involving chromosome 7 in 13 children. Bone marrow cellularity was reduced in 34, normal in 8 and increased in the remaining 6 patients. Nineteen patients were transplanted from an HLA-identical sibling, while the remaining 29 were given HSCT from an unrelated donor (UD). Bone marrow, cord blood and peripheral blood stem cells were employed in 38, 1 and 9 patients, respectively. Busulfan (BU) and cyclophosphamide (CY) were used as preparation to the allograft in 12 patients, while 36 children were given a regimen including BU, CY and melphalan (L-PAM). Cyclosporine-A (Cs-A) alone was mainly used as graft-versus-host disease (GvHD) prophylaxis in children transplanted from a relative, while the majority of patients transplanted from an UD were given the combination of Cs-A, short-term methotrexate and anti-thymocyte globulin. All patients engrafted, the median time to neutrophil and platelet recovery being 16 days (range, 9–79) and 26 days (range, 10–170), respectively. Two patients had secondary graft failure at 74 and 93 days after HSCT, respectively; one of them was successfully re-transplanted. The cumulative probability of developing grade II-IV acute GVHD was 37% (95% CI, 26–54%). Thirteen out of the 41 patients at risk developed chronic GVHD, which was limited in 8 patients and extensive in 5. The cumulative incidence of chronic GVHD was 35% (95% CI, 23–55%). With a median follow-up of 3.5 years (range 4 months-7.8 years), 39 patients are alive with sustained donor engraftment (1 after a second transplant), 9 patients having died for either transplant-related complications (8 patients) or disease recurrence (1 child). The 5-year probability of disease-free survival (DFS) is 77% (95% CI, 65–89%). In patients transplanted from an HLA-identical sibling and from an UD the DFS probability at 5 years is 78% (95% CI, 59–97%), and 76% (95% CI, 60–91%), respectively (P=n.s). The 5-year probability of DFS was 61% (95% CI, 38–83%) and 86% (95% CI, 74–99%) for children who did or did not experience grade II-IV acute GvHD (P=0.04). The outcome of patients prepared with either BU/CY or BU/CY/L-PAM was 92% (95% CI, 76–100%) and 71% (95% CI, 56–86%), respectively (P=n.s). These data indicate that a myeloablative allogeneic HSCT is able to cure a large proportion of children with RC, especially if GvHD is successfully prevented. Transplantation-related mortality represents the main cause of treatment failure, while relapse is rarely observed. The addition of L-PAM does not offer any advantage in comparison to BU/CY. Outcome of children with RC given allogeneic HSCT from either a relative or an UD is similar.

Sodium valproat (VPA) is a commonly used antiepileptic drug in children. It is known to cause a variety of different haematological side effects, including thrombocytopenia, a decrease in plasma fibrinogen, von Willebrand factor (VWF)- and factor XIII, impaired platelet aggregation with ADP and Collagen and prolonged PFA-100 closure times. The mechanisms responsible for such a variety of haematological abnormalities are still poorly understood and controversy exists about their clinical relevance and the risk for significant bleeding. We report on 4 children with severe bleeding complications during VPA treatment that had been followed at our institution. A first child with a cerebral malformation and no history of bleeding developed a subdural haematoma during VPA treatment without trauma. Additional studies revealed decreased von Willebrand factor levels that persisted with episodes of severe epistaxis also after VPA was stopped. A second infant suffered from herpes encephalitis with generalised seizures and died from an intracranial bleeding a day after a VPA loading dose was administred. Laboratory studies revealed prolonged PFA100 closure times. A third child with a therapeutic VPA serum-levels developed asymptomatic mild thrombocytopenia (around 100 G/l); unexpectedly in this child a tonsillectomy was complicated with sever diffuse haemorrhage that required red cell transfusion and intensive care treatment. Finally, a forth child who had been on VPA therapy for years causing prolonged PFA100 closure times, developed a large spinal epidural haematoma during epidural anaesthesia three days after urological surgery. This child had received VWF containing factor concentrate preoperatively only. For the bleeding additional VWF containing factor concentrate was given, the catheter was removed and the child recovered completely. Paediatricians, neurologists and anesthesists should be aware of the haemostatic side effects of VPA that can lead to significant bleeding. Before starting VPA therapy, patients should be evaluated for the presence of a congenital bleeding disorder. Preoperatively for any surgical intervention and in case of the occurrence of bleeding symptoms the patients have to be further evaluated for a VPA-induced disorder of the plasmatic haemostasis or an impaired platelet function. Haemostatic side effects of VPA can occur at therapeutic VPA serum levels and are found at any time during the course of VPA treatment. VPA has to be used cautiously in severely ill children and in intensive care patients and the risk of bleeding of any surgical or anesthesiological procedure has to be evaluated. Treatment options for VPA induced haemostatic abnormalities include plasma derived factor concentrates that contain VWF and the use of antifibrinolytic agents. Further studies have to assess the feasibility of standard treatment with the vasopressin analog DDAVP for this group of patients taking into account their high risk for seizures.

MDS in children is a rare disorder characterized by dysplasia and defined genetic abnormalities. In most patients (pts) MDS arises without known predisposing conditions (primary MDS). Here, we report the results of 55 males and 30 females with advanced primary MDS enrolled in the prospective EWOG-MDS trial 97. Data were analysed according to the most advanced FAB-type prior to SCT: 32 pts were classified as RAEB, 40 as RAEB-t and 13 as myelodysplasia-related AML (MDR-AML). Median age at diagnosis was 9.5 yrs (0.1–17.6) and median time from diagnosis of advanced MDS to SCT 4 mo (0.5–31). Cytogenetics revealed monosomy 7 in 32 pts, trisomy 8 in 7, a complex karyotype in 9 and other abnormalities in 9; karyotype was normal in 26 pts and unknown in 2. 31 pts had received AML-like therapy prior to SCT. All pts were given an unmanipulated graft after condititioning with busulfan 16 mg/kg, cyclophosphamide 120 mg/kg and melphalan 140 mg/m. Source of stem cells was bone marrow in 56 pts, peripheral blood in 25, cord blood in 2 and unknown in 2. 36 pts were transplanted from an HLA-identical relative (MFD), 49 pts from an HLA-identical or 1-antigen disparate unrelated donor (UD). GVHD prophylaxis consisted of CSA alone for MFD, whereas recipients of a UD graft generally received CSA, methotrexate and anti-lymphocyte globulin. Two pts suffered graft failure. The cumulative incidence of grade II-IV acute GVHD and chronic GVHD was 40% (SE 5%) and 25% (SE 5%), respectively. 18 pts suffered transplant-related mortality (TRM), the cumulative incidence of TRM in pts grafted from a MFD or UD being 14 and 25%, respectively (p=n.s.). Presence of acute GVHD II-IV (p

are drawn from the lumen that is not used for infusion. Cyclosporin is given intravenously or orally. Drug concentrations are measured by whole blood mono immunoassay. We have used silicone and polyurethane CVCs. We present the data on cyclosporin blood concentrations of six patients with allogeneic stem-cell transplantation and two patients with severe aplastic anaemia (table). With silicone CVCs, cyclosporin concentrations drawn through an intermittently blocked CVC (patients 1, 2, 3, 4, 5) always reflected the administered dose. The cyclosporin concentrations obtained through an intermittently blocked silicone CVC differed from concentrations simultaneously obtained by venepuncture by 19% to 0% (median 4%). With a continously perfused polyurethane CVC, the cyclosporin concentrations were 2–35% (median 15%) higher than those obtained by venepuncture (patient 8). In blood samples drawn though a blocked polyurethane CVC, however, cyclosporin concentrations were 127% to 1579% (median 281%) higher than those simultaneously obtained by venepuncture (patients 6, 7, 8). We show spuriously high cyclosporin concentrations drawn by polyurethane CVCs. Properties of the polyurethane are probable causes for the discrepancies in cyclosporin A concentrations drawn via CVC and venepuncture. Without a rinsing effect by continuous perfusion, the measured cyclosporin concentrations are spuriously high and cannot be used for adjusting the dose. Universitats-Kinderklinik, Medizinische Universitatsklinik, 79106 Freiburg, Germany (U Duffner) Spuriously raised cyclosporin concentrations drawn through polyurethane central venous catheter

Myelodysplastic syndrome (MDS) represents a heterogeneous group of hematopoietic stem cell disorders. Although the precise pathogenesis of MDS remains to be clarified, cytogenetic abnormalities appear to be involved in the disease mechanism and are considered an important factor in predicting clinical outcome. Here, we report the outcome of conventional cytogenetic analysis performed at diagnosis in 300 children with MDS who were enrolled in the prospective study EWOG-MDS 98. The patients were divided into 3 clinical groups: primary refractory cytopenia (RC) (n=142), primary advanced MDS (aMDS) (n=94), and MDS secondary to chemo- or radiation therapy, bone marrow failure disorders or familial disease (sMDS) (n=64). A full cytogenetic result was obtained in 263 cases (87.7%) including 116 (44.1%) with an abnormal clone. Comparing the 3 diagnostic subgroups, there was a statistically significant difference in the frequency of abnormalities observed. In RC, only 25 of 116 patients with successful analysis had a detectable clone that showed monosomy 7 as sole aberration in the majority of cases (52%). In addition, this subgroup also showed the greatest rate of failure to obtain a karyotypic result. In contrast, 42 (65.6%) of the sMDS cases were cytogenetically abnormal with a broad spectrum of aberrations including 16 patients (25%) with a complex karyotype (≥4 abr.). The presence of karyotypic evolution was also more frequently observed in this group. The overall karyotypic results showed monosomy 7 to be present in 50% of all abnormal cases, a figure that greatly exceeded the prevalence of the other frequent non-random aberrations of −5/del(5q), +8, del(7q), and +21, each of which occurred in 5–10% of abnormal cases. Despite the small numbers of these latter cases, clear trends of karyotypic events occurring in a particular clinical subgroup were demonstrated in the form of +8 with aMDS and −5/del(5q) with sMDS. The clinical and cytogenetic subgroups were further analyzed for correlations with gender and age. Whereas no clinical group showed any correlation between gender and karyotype, in RC and aMDS it was shown that patients with monosomy 7 were significantly younger than those with another karyotypic result (median 3.2 vs. 10.4 yrs and 5.2 vs. 11.0 yrs, respectively). The results demonstrate that while a general karyotypic picture can be defined in childhood MDS, future studies need to clearly differentiate between individual clinical subgroups.

Treatment-related myelodysplastic syndrome (tMDS) is a serious complication of therapy of childhood cancer. Here we report on 59 patients (pts) (35 males, 24 females) with tMDS enrolled in the prospective study EWOG-MDS 98. Prior malignancy was ALL in 21 pts, AML in 5, lymphoma in 6, CNS tumors in 12, and other solid tumors in 15. Median age at diagnosis of tMDS was 11.2 yrs (range 1.2 – 23.2), and median time between first malignancy and tMDS 3.3 yrs (range 0.5 – 11.1). Disease was classified as refractory cytopenia in 15 pts, RAEB in 30 and RAEB-T in 14. Of the 47 pts with cytogenetic analysis at diagnosis, 77% had an abnormal karyotype, including 38% that were complex. Monosomy 7 with or without other abnormalities was detected in 38%. Progression of disease was noted in 26 of 52 evaluable pts at a median time of 2.5 mo (range 0.3 – 16) from diagnosis; the cumulative incidence of progression being 74%. After a median follow-up time of 20 mo (range 3–69) 28 pts are alive with an estimated 5-year overall survival of 32% (95% CI 20 – 51). No child survived beyond 15 mo from diagnosis without receiving allogeneic hematopoietic stem cell transplantation (HSCT). HSCT was performed in 45 pts. Source of stem cells was bone marrow in 24 pts, peripheral blood in 20 and cord blood in 1. The donor was an HLA-identical relative (MFD) in 18 cases, while 26 pts were transplanted from an HLA-matched or 1-antigen/allele disparate unrelated donor (UD) and 1 pt from a 2-antigen disparate parent. Preparative regimen included busulfan 16 mg/kg, cyclophosphamide 120 mg/kg and melphalan 140 mg/m2 in 32 pts. Prophylaxis of GVHD generally consisted of cyclosporine-A for MFD, combined with methotrexate and ALG for UD. Two patients had graft failure. The cumulative incidence of grade II–IV acute GVHD and chronic GVHD were 32% (95% CI 28 – 37) and 24% (95% CI 19 – 28), respectively. Twelve pts suffered transplant-related mortality (TRM), the cumulative incidence of TRM in pts transplanted from a MFD or UD being 12% and 39%, respectively (P=0.058). Prior therapy with platinum compounds was an independent predictor of an increased risk of TRM. Fifteen pts relapsed at a median time of 9 mo (range 2 – 29) after HSCT, the 5-year cumulative incidence of relapse being 44% (95% CI 21 – 66). A WBC > 4 G/L at diagnosis predicted a higher risk of relapse. Twenty-one of the 45 pts transplanted are alive and 19 are disease-free. The estimated 5-year EFS after HSCT was 27% (95% CI 15 – 47). Prior therapy with platinum compounds, a WBC > 4 G/L at diagnosis, and a female donor predicted inferior EFS, while HSCT from an UD versus MFD resulted in similar outcome (5-year EFS, 21% and 39%, respectively). AML-type therapy prior to HSCT did not improve survival. Innovative stragegies for improving the outcome of these patients are warranted.

Background Since siblings of pediatric cancer patients are at risk for emotional, behavioral, and social problems, there is considerable interest in development of early psychological interventions. This paper aimed at evaluating the effectiveness of a two-session psychological intervention for siblings of newly diagnosed pediatric cancer patients. Methods Thirty siblings age 6-17 years were randomly assigned to an intervention group or an active control group with standard psychosocial care. The manualized intervention provided to siblings in the first 2 months after the cancer diagnosis of the ill child included medical information, promotion of coping skills, and a psychoeducational booklet for parents. At 4 to 6 weeks, 4 months, and 7 months after the diagnosis, all siblings and their parents completed measures (from standardized instruments) of social support, quality of life, medical knowledge, posttraumatic stress symptoms, and anxiety. Results At follow-up siblings in the intervention group showed better psychological well-being, had better medical knowledge, and reported receiving social support from more people. However, the intervention had no effects on posttraumatic stress symptoms and anxiety. Conclusions The results of this pilot trial suggest that a two-session sibling intervention can improve siblings' adjustment, particularly psychological well-being, in the early stage after a cancer diagnosis. Trial Registration ClinicalTrials.gov NCT00296907