BACKGROUND AND PURPOSE: Culturally diverse countries such as Switzerland face the challenge of pr... more BACKGROUND AND PURPOSE: Culturally diverse countries such as Switzerland face the challenge of providing cross-cultural competent care. Cross-cultural competent care needs an understanding of a patient's cultural context in order to provide safe and effective care. Therefore, we sought to examine cross-cultural competence of Swiss paediatric oncology care providers, and to explore their perceptions of barriers to and facilitators of cross-culturally competent care. DESIGN AND SAMPLE: We conducted a cross-sectional study. The data collection period was three weeks. Providers were recruited through collaborators at the participating paediatric oncology centres. All occupational groups who are in direct contact with patients and involved in their care were eligible (e.g., physicians, nurses, social workers, occupational therapists and physiotherapists). Surveying providers online, we captured five subscales of their cross-cultural competence and their perceptions as to how to facil...
To the Editor: According to theWorldHealthOrganization (WHO), iron deficiency (ID) affects >2 ... more To the Editor: According to theWorldHealthOrganization (WHO), iron deficiency (ID) affects >2 billion individuals worldwide, and is the leading cause of years lived with disability, with >600 million directly impacted children and adults in 2013.1,2 Prevalence in high-resource countries is estimated between 2 and 6% of preschool children and between 8 and 20% of female adolescents.3,4 Clinical manifestations of ID/iron deficiency with anemia (IDA) depend on age, the presence and severity of anemia, co-morbidities and rate of onset, and thus it ranks among the most common challenges faced by pediatricians.5 Oral iron substitution is effective in the largemajority of childrenwith ID/IDAand should be initiated when a clear-cut laboratory diagnosis is established, and differential diagnoses have been considered. Side effects of oral iron can occur but are never dangerous. In IDwithout anemia, correction of any underlying nutritional problem has priority, and family education for a healthy diet and lifestyle is needed. As the national expert group in pediatric hematology in Switzerland, we recently published consensus recommendations for iron therapy in children after extensive review of the available evidence.6,7 In addition to providing specialist care, we believe in our role to counsel the family and provide support in choosing the most physiological and less invasive option for the correction of ID in children, especially at young age. Such approach is certainlymore time consuming, butwe are convinced that it ultimately allows to build a healthy basis for the harmonious global growth of the child and his family, and foster long-term well-being. We believe that educating families and raising their health literacy cannot be substituted with the nonphysiological IV infusion of an essential nutrient, which is commonly found in foods from animal and/or vegetal sources. Thus, we oppose the IV administration of iron to children as the first-line approach to ID/IDA outside of the wellrecognized indications supported by the evidence (i.e., malabsorbtion due to inflammatory bowel disorder or coeliac disease, renal and cardiac disorders, among other), as shortand long-term benefits of IV over oral supplementation and cost-effectiveness are not yet proven. It is with interest that we read the recent paper by Ozsahin and colleagues published in this journal reporting a retrospective observational study of a cohort of 144 children and adolescents aged 18 months to 18 years with IDA/ID and inadequate or failed response to oral iron therapy, who received IV ferric carboxymaltose (FCM) at a private clinic in Switzerland from 2017 to 2019.8 As pediatric hematologists, we find ourselves at issue with some of the statements made in this publication. First, the authors claim that their study confirms the safety/efficacy of FCM in children (aged 18 months and older) and adolescents unresponsive to oral therapy in a real-world context. We disagree with the notion that a retrospective chart review study, summarizing data resulting from an off-label administration of FCM in a single center, constitutes a proper basis for this determination. It is widely recognized that to establish safety/efficacy, well-designed prospective controlled trials with predefined endpoints capturing severe adverse events (SAEs) and efficacymarkers are required. Studies on the administration of first-line IV FCM in the context of ID/IDA are underway (NCT03523117, NCT04269707), and their results should be awaited before concluding on the role of FCM in children with ID/IDA not responding to oral iron. Second, we disagree with the interpretation of the data whereby clinical improvement could be seenwith the added advantage of safety. Answering the question of clinical improvement is challenging even when endpoints are properly (i.e., prospectively) defined. As an example, a previous prospective placebo-controlled trial showed that the response of clinical symptoms (such as fatigue) after IV iron substitution in women was associated with the initial degree of iron depletion, and was difficult to distinguish from other (i.e., psycho-emotional) causes.9 Moreover, we wonder whether Ozsahin and colleagues, who reported an unusual rapid catch up growth in their cohort, provided concomitant nutritional advice in addition to the FCM infusion, as this could constitute amajor confounder for their observations. Third, while data now exist regarding the safety of IV iron in children under the specific abovementioned indications, we believe that taking any risk in this situation deserves careful consideration. In our understanding, nutritional guidance with oral iron will always constitute safer option than first-line therapy with IV iron.Wewonder whether oral iron was offered as an alternative treatment option for ID/IDA at that specific center, as the number of total children seen for this diagnosis cannot be determined from the data.We understand the authors…
The Hereditary TTP Registry is an international cohort study for patients with a confirmed or sus... more The Hereditary TTP Registry is an international cohort study for patients with a confirmed or suspected diagnosis of hereditary thrombotic thrombocytopenic purpura (hTTP) and their family members. Hereditary TTP is an ultra-rare blood disorder (prevalence of ∼1–2 cases per million), the result of autosomal-recessively inherited congenital ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) deficiency (ADAMTS13 activity <10% of the normal), and associated with yet many unanswered questions. Until December 2017, the Hereditary TTP Registry had enrolled 123 confirmed hTTP patients. Their median age at disease onset was 4.5 years (range: 0–70) and at clinical diagnosis 16.7 years (range: 0–69), a difference that highlights the existing awareness gap in recognizing hTTP. The systematic collection of clinical data of individual patients revealed their substantial baseline comorbidities, as a consequence of recurring TTP episodes in the past. Mos...
Background Controversy exists as to what may be defined as standard of care (including markers fo... more Background Controversy exists as to what may be defined as standard of care (including markers for stratification) for patients with atypical teratoid/rhabdoid tumors (ATRTs). The European Rhabdoid Registry (EU-RHAB) recruits uniformly treated patients and offers standardized genetic and DNA methylation analyses. Methods Clinical, genetic, and treatment data of 143 patients from 13 European countries were analyzed (2009–2017). Therapy consisted of surgery, anthracycline-based induction, and either radiotherapy or high dose chemotherapy following a consensus among European experts. Fluorescence in situ hybridization, multiplex ligation-dependent probe amplification, and sequencing were employed for assessment of somatic and germline mutations in SWItch/sucrose nonfermentable related, matrix associated, actin dependent regulator of chromatin, subfamily B (SMARCB1). Molecular subgroups (ATRT-SHH, ATRT-TYR, and ATRT-MYC) were determined using DNA methylation arrays, resulting in profile...
Background: Incidence of childhood cancer increased in most countries worldwide, but the reasons ... more Background: Incidence of childhood cancer increased in most countries worldwide, but the reasons are unclear. This study investigates trends in childhood cancer incidence in Switzerland from 1985 to 2014. Methods: We extracted data on all childhood cancer cases diagnosed at ages 0-14 years in Switzerland from the Swiss Childhood Cancer Registry. We included ICCC-3 main groups I-XII and calculated age-standardised, cumulative, and age-specific incidence for different diagnostic groups. We analysed trends in annual age-standardised incidence using JoinPoint regression models. Results: Over the study period from 1985-2014, 5104 of 5486 cancer diagnoses (93%) were microscopically verified. The proportion of children treated in paediatric cancer centres increased from 84% during 1985-1994 to 93% in 1995-2004 and 98% in 2005-2014 (p<0.001). Using the 2010 European standard population, age-standardised incidence was 143 in 1985-1994, 154 in 1995-2004, and 162 per million in 2005-2014. O...
Lived experiences of childhood cancer patients and their families have been described as interrup... more Lived experiences of childhood cancer patients and their families have been described as interrupted and as a loss of normal life. Apart from symptoms due to the cancer disease, families continuously experience burden of treatment. Since coping capacities are unique to each individual, we captured variables that offer objective measures of treatment burden, with a particular focus on the disruptive effects of treatment on families' lives. Our sample was comprised by 193 children that died of cancer. Medical records were extracted retrospectively. Quantitative data were statistically analysed with respect to variables related to treatment burden. Deceased children with cancer and their families faced a significant burden of treatment. Results revealed that deceased leukaemia patients had a higher number of inpatient stays, spent more time in the hospital both during their illness and during the last month of their life, and were more likely to die in the hospital when compared to...
The goal is to present how shared decision-making in paediatric oncology occurs from the viewpoin... more The goal is to present how shared decision-making in paediatric oncology occurs from the viewpoints of parents and physicians. Eight Swiss Pediatric Oncology Group centres participated in this prospective study. The sample comprised a parent and physician of the minor patient (<18 years). Surveys were statistically analysed by comparing physicians' and parents' perspectives and by evaluating factors associated with children's actual involvement. Perspectives of ninety-one parents and twenty physicians were obtained for 151 children. Results indicate that for six aspects of information provision examined, parents' and physicians' perceptions differed. Moreover, parents felt that the children were more competent to understand diagnosis and prognosis, assessed the disease of the children as worse, and reported higher satisfaction with decision-making on the part of the children. A patient's age and gender predicted involvement. Older children and girls were m...
BACKGROUND AND PURPOSE: Culturally diverse countries such as Switzerland face the challenge of pr... more BACKGROUND AND PURPOSE: Culturally diverse countries such as Switzerland face the challenge of providing cross-cultural competent care. Cross-cultural competent care needs an understanding of a patient's cultural context in order to provide safe and effective care. Therefore, we sought to examine cross-cultural competence of Swiss paediatric oncology care providers, and to explore their perceptions of barriers to and facilitators of cross-culturally competent care. DESIGN AND SAMPLE: We conducted a cross-sectional study. The data collection period was three weeks. Providers were recruited through collaborators at the participating paediatric oncology centres. All occupational groups who are in direct contact with patients and involved in their care were eligible (e.g., physicians, nurses, social workers, occupational therapists and physiotherapists). Surveying providers online, we captured five subscales of their cross-cultural competence and their perceptions as to how to facil...
To the Editor: According to theWorldHealthOrganization (WHO), iron deficiency (ID) affects >2 ... more To the Editor: According to theWorldHealthOrganization (WHO), iron deficiency (ID) affects >2 billion individuals worldwide, and is the leading cause of years lived with disability, with >600 million directly impacted children and adults in 2013.1,2 Prevalence in high-resource countries is estimated between 2 and 6% of preschool children and between 8 and 20% of female adolescents.3,4 Clinical manifestations of ID/iron deficiency with anemia (IDA) depend on age, the presence and severity of anemia, co-morbidities and rate of onset, and thus it ranks among the most common challenges faced by pediatricians.5 Oral iron substitution is effective in the largemajority of childrenwith ID/IDAand should be initiated when a clear-cut laboratory diagnosis is established, and differential diagnoses have been considered. Side effects of oral iron can occur but are never dangerous. In IDwithout anemia, correction of any underlying nutritional problem has priority, and family education for a healthy diet and lifestyle is needed. As the national expert group in pediatric hematology in Switzerland, we recently published consensus recommendations for iron therapy in children after extensive review of the available evidence.6,7 In addition to providing specialist care, we believe in our role to counsel the family and provide support in choosing the most physiological and less invasive option for the correction of ID in children, especially at young age. Such approach is certainlymore time consuming, butwe are convinced that it ultimately allows to build a healthy basis for the harmonious global growth of the child and his family, and foster long-term well-being. We believe that educating families and raising their health literacy cannot be substituted with the nonphysiological IV infusion of an essential nutrient, which is commonly found in foods from animal and/or vegetal sources. Thus, we oppose the IV administration of iron to children as the first-line approach to ID/IDA outside of the wellrecognized indications supported by the evidence (i.e., malabsorbtion due to inflammatory bowel disorder or coeliac disease, renal and cardiac disorders, among other), as shortand long-term benefits of IV over oral supplementation and cost-effectiveness are not yet proven. It is with interest that we read the recent paper by Ozsahin and colleagues published in this journal reporting a retrospective observational study of a cohort of 144 children and adolescents aged 18 months to 18 years with IDA/ID and inadequate or failed response to oral iron therapy, who received IV ferric carboxymaltose (FCM) at a private clinic in Switzerland from 2017 to 2019.8 As pediatric hematologists, we find ourselves at issue with some of the statements made in this publication. First, the authors claim that their study confirms the safety/efficacy of FCM in children (aged 18 months and older) and adolescents unresponsive to oral therapy in a real-world context. We disagree with the notion that a retrospective chart review study, summarizing data resulting from an off-label administration of FCM in a single center, constitutes a proper basis for this determination. It is widely recognized that to establish safety/efficacy, well-designed prospective controlled trials with predefined endpoints capturing severe adverse events (SAEs) and efficacymarkers are required. Studies on the administration of first-line IV FCM in the context of ID/IDA are underway (NCT03523117, NCT04269707), and their results should be awaited before concluding on the role of FCM in children with ID/IDA not responding to oral iron. Second, we disagree with the interpretation of the data whereby clinical improvement could be seenwith the added advantage of safety. Answering the question of clinical improvement is challenging even when endpoints are properly (i.e., prospectively) defined. As an example, a previous prospective placebo-controlled trial showed that the response of clinical symptoms (such as fatigue) after IV iron substitution in women was associated with the initial degree of iron depletion, and was difficult to distinguish from other (i.e., psycho-emotional) causes.9 Moreover, we wonder whether Ozsahin and colleagues, who reported an unusual rapid catch up growth in their cohort, provided concomitant nutritional advice in addition to the FCM infusion, as this could constitute amajor confounder for their observations. Third, while data now exist regarding the safety of IV iron in children under the specific abovementioned indications, we believe that taking any risk in this situation deserves careful consideration. In our understanding, nutritional guidance with oral iron will always constitute safer option than first-line therapy with IV iron.Wewonder whether oral iron was offered as an alternative treatment option for ID/IDA at that specific center, as the number of total children seen for this diagnosis cannot be determined from the data.We understand the authors…
The Hereditary TTP Registry is an international cohort study for patients with a confirmed or sus... more The Hereditary TTP Registry is an international cohort study for patients with a confirmed or suspected diagnosis of hereditary thrombotic thrombocytopenic purpura (hTTP) and their family members. Hereditary TTP is an ultra-rare blood disorder (prevalence of ∼1–2 cases per million), the result of autosomal-recessively inherited congenital ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) deficiency (ADAMTS13 activity <10% of the normal), and associated with yet many unanswered questions. Until December 2017, the Hereditary TTP Registry had enrolled 123 confirmed hTTP patients. Their median age at disease onset was 4.5 years (range: 0–70) and at clinical diagnosis 16.7 years (range: 0–69), a difference that highlights the existing awareness gap in recognizing hTTP. The systematic collection of clinical data of individual patients revealed their substantial baseline comorbidities, as a consequence of recurring TTP episodes in the past. Mos...
Background Controversy exists as to what may be defined as standard of care (including markers fo... more Background Controversy exists as to what may be defined as standard of care (including markers for stratification) for patients with atypical teratoid/rhabdoid tumors (ATRTs). The European Rhabdoid Registry (EU-RHAB) recruits uniformly treated patients and offers standardized genetic and DNA methylation analyses. Methods Clinical, genetic, and treatment data of 143 patients from 13 European countries were analyzed (2009–2017). Therapy consisted of surgery, anthracycline-based induction, and either radiotherapy or high dose chemotherapy following a consensus among European experts. Fluorescence in situ hybridization, multiplex ligation-dependent probe amplification, and sequencing were employed for assessment of somatic and germline mutations in SWItch/sucrose nonfermentable related, matrix associated, actin dependent regulator of chromatin, subfamily B (SMARCB1). Molecular subgroups (ATRT-SHH, ATRT-TYR, and ATRT-MYC) were determined using DNA methylation arrays, resulting in profile...
Background: Incidence of childhood cancer increased in most countries worldwide, but the reasons ... more Background: Incidence of childhood cancer increased in most countries worldwide, but the reasons are unclear. This study investigates trends in childhood cancer incidence in Switzerland from 1985 to 2014. Methods: We extracted data on all childhood cancer cases diagnosed at ages 0-14 years in Switzerland from the Swiss Childhood Cancer Registry. We included ICCC-3 main groups I-XII and calculated age-standardised, cumulative, and age-specific incidence for different diagnostic groups. We analysed trends in annual age-standardised incidence using JoinPoint regression models. Results: Over the study period from 1985-2014, 5104 of 5486 cancer diagnoses (93%) were microscopically verified. The proportion of children treated in paediatric cancer centres increased from 84% during 1985-1994 to 93% in 1995-2004 and 98% in 2005-2014 (p<0.001). Using the 2010 European standard population, age-standardised incidence was 143 in 1985-1994, 154 in 1995-2004, and 162 per million in 2005-2014. O...
Lived experiences of childhood cancer patients and their families have been described as interrup... more Lived experiences of childhood cancer patients and their families have been described as interrupted and as a loss of normal life. Apart from symptoms due to the cancer disease, families continuously experience burden of treatment. Since coping capacities are unique to each individual, we captured variables that offer objective measures of treatment burden, with a particular focus on the disruptive effects of treatment on families' lives. Our sample was comprised by 193 children that died of cancer. Medical records were extracted retrospectively. Quantitative data were statistically analysed with respect to variables related to treatment burden. Deceased children with cancer and their families faced a significant burden of treatment. Results revealed that deceased leukaemia patients had a higher number of inpatient stays, spent more time in the hospital both during their illness and during the last month of their life, and were more likely to die in the hospital when compared to...
The goal is to present how shared decision-making in paediatric oncology occurs from the viewpoin... more The goal is to present how shared decision-making in paediatric oncology occurs from the viewpoints of parents and physicians. Eight Swiss Pediatric Oncology Group centres participated in this prospective study. The sample comprised a parent and physician of the minor patient (<18 years). Surveys were statistically analysed by comparing physicians' and parents' perspectives and by evaluating factors associated with children's actual involvement. Perspectives of ninety-one parents and twenty physicians were obtained for 151 children. Results indicate that for six aspects of information provision examined, parents' and physicians' perceptions differed. Moreover, parents felt that the children were more competent to understand diagnosis and prognosis, assessed the disease of the children as worse, and reported higher satisfaction with decision-making on the part of the children. A patient's age and gender predicted involvement. Older children and girls were m...
Uploads
Papers by Heinz Hengartner