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Background The number of children and adolescents living with life-limiting conditions and potentially in need for specialised paediatric palliative care (SPPC) is rising. Ideally, a specialised multiprofessional team responds to the complex healthcare needs of children and their families. The questions of, how SPPC is beneficial, for whom, and under what circumstances, remain largely unanswered in the current literature. This study’s overall target is to evaluate the effectiveness of a SPPC programme in Switzerland with respect to its potential to improve patient-, family-, health professional-, and healthcare-related outcomes. Methods This comparative effectiveness study applies a quasi-experimental design exploring the effectiveness of SPPC as a complex intervention at one treatment site in comparison with routine care provided in a generalised PPC environment at three comparison sites. As the key goal of palliative care, quality of life - assessed at the level of the patient-, the family- and the healthcare professional - will be the main outcome of this comparative effectiveness research. Other clinical, service, and economic outcomes will include patient symptom severity and distress, parental grief processes, healthcare resource utilisation and costs, direct and indirect health-related expenditure, place of death, and introduction of SPPC. Data will be mainly collected through questionnaire surveys and chart analysis. Discussion The need for SPPC has been demonstrated through numerous epidemiological and observational studies. However, in a healthcare environment focused on curative treatment and struggling with limited resources, the lack of evidence contributes to a lack of acceptance and financing of SPPC which is a major barrier against its sustainability. This study will contribute to current knowledge by reporting individual and child level outcomes at the family level and by collecting detailed contextual information on healthcare provision. We hope that the results of this study can help guiding the expansion and sustainability of SPPC and improve the quality of care for children with life-limiting conditions and their families internationally. Trial registration Registered prospectively on ClinicalTrials.gov on January 22, 2020. NCT04236180 Protocol version Amendment 2, March 01, 2021.

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.

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.

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.

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.

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

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.

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.

PurposeThis study investigates physical performance limitations for sports and daily activities in recently diagnosed childhood cancer survivors and siblings.MethodsThe Swiss Childhood Cancer Survivor Study sent a questionnaire to all survivors (≥ 16 years) registered in the Swiss Childhood Cancer Registry, who survived >5 years and were diagnosed 1976-2003 aged ResultsThe sample included 1038 survivors and 534 siblings. Overall, 96 survivors (9.5%) and 7 siblings (1.1%) reported a limitation in sports (Odds ratio 5.5, 95%CI 2.9-10.4, pConclusionSurvivors of childhood cancer, even those diagnosed recently and treated with modern protocols, remain at high risk for physical performance limitations. Treatment and follow-up care should include tailored interventions to mitigate these late effects in high-risk patients.

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.

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.

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