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Children with refractory or relapsed Burkitt lymphoma (BL) or Burkitt leukemia (B-AL) have a poor chance to survive. We describe characteristics, outcome, reinduction, and transplantation approaches and evaluate risk factors among children with progression of a BL/B-AL included in Non-Hodgkin's Lymphoma-Berlin-Frankfurt-Münster studies between 1986 and 2016. Treatment recommendation was reinduction including rituximab from the early 2000s followed by blood stem cell transplantation. The 3-year survival of the 157 children was 18.5 ± 3%. Survival significantly improved from 11 ± 3% before to 27 ± 5% after 2000 (P < .001), allowing for risk factor analyses among the latter 75 patients. Survival of 14 patients with relapse after initial therapy for low-risk disease (R1/R2) was 50 ± 13% compared with 21 ± 5% for 61 patients progressing after R3/R4 therapy (P < .02). A total of 25 of 28 patients with progression during first-line therapy, 31 of 32 with progression during reinduction, 15 of 16 not reaching a complete remission (CR) before transplantation, 9 of 10 treated with rituximab front-line, and all 13 patients not receiving rituximab during reinduction died. Forty-six patients received stem cell transplantation (20 autologous, 26 allogeneic). Survival after a regimen combining rituximab with continuous-infusion chemotherapy followed by allogeneic transplantation was 67 ± 12% compared with 18 ± 5% for all other regimen and transplantations (P = .003). Patients with relapsed BL/B-AL have a poor chance to survive after current effective front-line therapies. Progression during initial or reinduction chemotherapy and initial high-risk disease are risk factors in relapse. Time-condensed continuous-infusion reinduction followed by stem cell transplantation forms the basis for testing new drugs.
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Antineoplásicos Imunológicos/uso terapêutico , Linfoma de Burkitt/terapia , Transplante de Células-Tronco Hematopoéticas , Recidiva Local de Neoplasia/terapia , Rituximab/uso terapêutico , Adolescente , Linfoma de Burkitt/epidemiologia , Criança , Progressão da Doença , Feminino , Humanos , Masculino , Recidiva Local de Neoplasia/epidemiologia , Análise de Sobrevida , Transplante Homólogo , Resultado do TratamentoRESUMO
PURPOSE: The care of older neurosurgical patients at the end life is a particularly demanding challenge. Especially, the specific needs of very old patients with glioblastoma at the end of life are at risk of being deprived of adequate care. METHODS: Based on a narrative literature review, this article aims to explore key issues of the thematic intersection of geriatric glioblastoma patients, palliative care and neurosurgery. RESULTS AND DISCUSSION: Four key issues were identified: patient-centeredness (need orientation and decision making), early palliative care, advance care planning, and multi-professionalism. Possible benefits and barriers are highlighted with regard to integrating these concepts into neurosurgery. CONCLUSIONS: Palliative care complements neurosurgical care of geriatric glioblastoma multiforme patients to optimise care for this highly vulnerable category of patients.
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Planejamento Antecipado de Cuidados , Glioblastoma , Neurocirurgia , Assistência Terminal , Idoso , Glioblastoma/cirurgia , Humanos , Procedimentos Neurocirúrgicos , Cuidados PaliativosRESUMO
OBJECTIVE: To give an overview of English and German speaking bereavement measurements for difficulties in the grieving process and complicated grief. METHOD: Systematic literature review. RESULTS: 22 measures were identified. They are categorised into general, specialized and pre-death measurements. All scales proved to have predominantly good psychometric properties. DISCUSSION: Dozens of measures of grieving have been proposed internationally. Only 3 general and 3 specialized scales are available in German language. CONCLUSION: Up to date no scale exists in German language to assess bereavement difficulties prior to death.
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Pesar , HumanosAssuntos
Qualidade de Vida , Humanos , Criança , Adolescente , Prevalência , Alemanha/epidemiologia , Inquéritos e QuestionáriosRESUMO
Pediatric tumors of the central nervous system composed of oligoid tumor cells showing diffuse leptomeningeal spread without a primary mass lesion seem to represent a novel tumor entity. The terms "diffuse leptomeningeal glioneural tumor" or-preferably-"disseminated oligodendroglial-like leptomeningeal tumor of childhood" (DOGLT) were proposed. Four patients were identified with clinico-neuropathologic findings compatible with DOGLT and a mean follow-up time of 54 months was determined. Seven different biopsies obtained from the four patients were histologically evaluated. Clinical course, diagnostic measures, histopathologic and radiologic features and treatment suggestions were recorded, on the basis of which diagnostic and therapeutic algorithm was proposed. Patients with DOGLT presented with hydrocephalus as first symptom, requiring neurosurgical therapy. Open arachnoid biopsy was necessary to confirm diagnosis. The oligoid cells in a desmoplastic or focally myxoid matrix showed OLIG2-, MAP2-, S-100 and rare HuC/HuD protein-immunopositivity. IDH1 (R132H)- and CD99-immunohistochemistry was negative in all patients. None of the evaluable biopsies of three patients showed chromosome 1p/19q deletion, neither as isolated nor combined allelic loss. Chemotherapy according to the SIOP-LGG 2004 standard induction and consolidation protocol resulted in complete response and partial response, respectively, in 50 % of the patients. However, after discontinuation of chemotherapy, two patients experienced tumor progression and one of them succumbed to the disease after 19 months. Radiological criteria as well as preliminary treatment results are presented after observation of four clinical cases. Prognosis and long-term clinical courses remain to be observed.
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Neoplasias Meníngeas/diagnóstico , Neoplasias Meníngeas/terapia , Oligodendroglioma/diagnóstico , Oligodendroglioma/terapia , Criança , Pré-Escolar , Progressão da Doença , Evolução Fatal , Humanos , Lactente , Masculino , Neoplasias Meníngeas/diagnóstico por imagem , Neoplasias Meníngeas/patologia , Oligodendroglioma/diagnóstico por imagem , Oligodendroglioma/patologia , Radiografia , Resultado do TratamentoRESUMO
BACKGROUND: In Germany, numerous health care providers work in the field of bereavement care. An epidemiological study of the field immediately after the introduction of the ICD-11 diagnosis "Prolonged Grief Disorder" (PGD) is pending. METHODS: The second survey of the trend study, which is designed over a total of three measurement points at intervals of one decade each, explores whether and to what extent the field of bereavement care has evolved after the introduction of the grief-specific diagnosis PGD. For this purpose, providers of bereavement care at various organizational levels were asked to participate in an online survey. The survey was conducted from October 2020 to January 2021. RESULTS: 456 questionnaires were included in the study. Data analysis was descriptive. 80.5% of the participants have an additional grief-specific qualification, 59.4% base their work on a grief-specific concept. In view of the PGD diagnosis, only a few respondents have participated in a PGD-related advanced training. Most are afraid that the topic of grief will increasingly be subsumed in the medical-psychological-therapeutic field. An increase in bereavement research and the number and quality of continuing education is also expected. DISCUSSION: Some positive trends in German bereavement care can be identified. However, there is a need for further development in the areas "designation of the activity", "diagnostics", and "intervention". Also, there is a need for training with regard to PGD. CONCLUSION: After the introduction of PGD, the field of bereavement care in Germany turns out to be slightly different. The diagnosis is viewed skeptically.
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Luto , Cuidados Paliativos na Terminalidade da Vida , Humanos , Alemanha , Pesar , Inquéritos e QuestionáriosRESUMO
CONTEXT: Vaccine preventable diseases lead to distressful symptoms and complications among pediatric patients receiving specialized home palliative care. There was no data on the vaccination compliance. OBJECTIVES: The objective was to determine the vaccination coverage, discuss the relevance of vaccinations and provide vaccination recommendations in pediatric palliative care. METHODS: Vaccination data were compared in a multicenter cross-sectional study. Expert interviews were conducted to evaluate symptom burden. The vaccination status of patients treated by six German pediatric specialized home palliative care teams was recorded from January 2019 to December 2019. The data were compared to the national immunization schedule and the vaccination rate of a representative German pediatric cohort. Onset of missed vaccination was compared to the date of diagnosis of the life-limiting condition. A risk score was calculated to evaluate the relevance of each individual vaccinations. RESULTS: Vaccination rates of Tdpa, haemophilus influenzae type B, poliomyelitis, hepatitis B, pneumococcal disease, meningococcal diseases type C, and MMR were lower compared to healthy controls. There were no significant differences in varicella. In most cases the discontinuation of recommended immunizations occurred after diagnosis of the palliative condition. Influenza had the highest risk score and was the most frequent vaccine preventable disease in retrospective data. This paper includes a pragmatic proposal for the management of vaccination in this vulnerable population. CONCLUSION: Children and adolescents with life-limiting conditions are at increased risk of vaccine preventable diseases. Individual vaccination counselling is recommended.
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Doenças Preveníveis por Vacina , Adolescente , Criança , Humanos , Lactente , Estudos Transversais , Cuidados Paliativos , Estudos Retrospectivos , VacinaçãoRESUMO
Background: The prevalence of children with life-limiting conditions is rising, and since the amendment of the social insurance code in Germany, palliative home care teams have treated an increasing number of children. These teams provide 24/7 readiness, yet some parents still call the general emergency medical service (EMS) for various reasons. EMS is exposed to complex medical problems in rare diseases. Questions arose about the experiences of EMS and whether they felt prepared for emergencies involving children treated by a palliative care team. Methods: This study used a mixed methods approach to focus on the interface between palliative care and EMS. First, open interviews were conducted, and a questionnaire was developed based on the results. The variables included demographic items and individual experiences with patients. Second, a case report of a child with respiratory insufficiency was presented to assess the spontaneous treatment intentions of EMS providers. Finally, the need, relevant topics, and duration of specific training in palliative care for EMS providers were evaluated. Results: In total, 1,005 EMS providers responded to the questionnaire. The average age was 34.5 years (±10.94SD), 74.6% were male. The average work experience was 11.8 years (±9.7), 21.4% were medical doctors. Experience with a call of a life-threatening emergency involving a child was reported by 61.5% and severe psychological distress during such a call was reported by 60.4%. The equivalent distress frequency for adult patient calls was 38.3%. (p < 0.001). After review of the case report, the EMS respondents suggested invasive treatment options and rapid transport to the hospital. Most (93.7%) respondents welcomed the consideration of special training in pediatric palliative care. This training should include basic information about palliative care, an analysis of cases involving palliatively treated children, an ethical perspective, practical recommendations, and available (24/7) local contact for further guidance and support. Conclusion: Emergencies in pediatric palliatively treated patients were more common than expected. EMS providers perceived the situations as stressful, and there is a need for specific training with practical aspects.
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Background: The prevalence of children with life-limiting conditions (LLCs) is rising. It is characteristic for these children to require 24/7 care. In emergencies, families must decide to call the emergency medical service (EMS) or a palliative care team (PCT)-if available. For EMS teams, an emergency in a child with an LLC is a rare event. Therefore, EMS providers asked for a training unit (TU) to improve their knowledge and skills in pediatric palliative care. Aim of the study: The questions were as follows: whether a TU is feasible, whether its integration into the EMS training program was accepted, and whether an improvement of knowledge can be achieved. Methods: We designed and implemented a brief TU based on findings of a previous study that included 1,005 EMS providers. The topics covered were: (1) basics in palliative home care, (2) theoretical aspects, and (3) practical aspects. After participating in the TU, the participants were given a questionnaire to re-evaluate their learning gains and self-confidence in dealing with emergencies in pediatric patients with LLC. Results: 782 (77.8%) of 1,005 participants of the previous study responded to the questionnaire. The average age was 34.9 years (±10.7 years SD), and 75.3% were male. The average work experience was 11.4 years (±9.5 years SD), and 15.2% were medical doctors. We found an increase in theoretical knowledge and enhanced self-confidence in dealing with emergencies in patients with LLC (confidence: before training: 3.3 ± 2.0 SD; after training: 5.7 ± 2.1 SD; min.: 1; max.: 10; p < 0.001). The participants changed their approaches to a fictitious case report from more invasive to less invasive treatment. Most participants wanted to communicate directly with PCTs and demanded a standard operating procedure (SOP) for treating patients with LLC. We discussed a proposal for an SOP with the participants. Conclusion: EMS providers want to be prepared for emergencies in children with LLCs. A brief TU can improve their knowledge and confidence to handle these situations adequately. This TU is the first step to improve collaboration between PCTs and EMS teams.
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Pediatric palliative home care (PPHC) provides care for children, adolescents, and young adults with life-limiting illnesses in their own homes. Home care often requires long travel times for the PPHC team, which is available to the families 24/7 during crises. The complementary use of telehealth may improve the quality of care. In this pilot study we identify the needs and concerns of patients, teams, and other stakeholders regarding the introduction of telehealth. As a first step, focus groups were conducted in three teams. For the second step, semi-structured interviews were conducted with patients and their families (n = 15). Both steps were accompanied by quantitative surveys (mixed methods approach). The qualitative data were analyzed using content analysis. A total of 11 needs were identified, which were prioritized differently. Highest priority was given to: data transmission, video consultation, access to patient records, symptom questionnaires, and communication support. The concerns identified were related to the assumption of deterioration of the status quo. Potential causes of deterioration were thought to be the negative impact on patient care, inappropriate user behavior, or a high level of technical requirements. As a conclusion, we define six recommendations for telehealth in PPHC.
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HSCT is an established treatment option for some children with life-threatening diseases, but complications remain a major cause of morbidity and mortality. This retrospective data analysis addresses the surgical issues of children with HSCT-related complications. Between 2002 and 2008, HSCT was performed in 240 children for leukemias/lymphomas (n=135), solid tumors (n=59), immunodeficiencies (n=20), lipid storage diseases (n=10), autoimmune diseases (n=9), and others (n=7). HSCT-related complications requiring surgery occurred in 24 cases (10%) and most often in the leukemias/lymphomas group (18/24 cases): HC (cystoscopic irrigation, n=7), pulmonary aspergilloses (resection, n=7), bone necroses (core decompression, n=3), GvHD bowel (colostomy/PEG, n=2), ICH (drainage, n=2), bilateral kidney abscess (nephrectomies/renal transplantation, n=1), aspergillosis of the maxillary sinus (decompression, n=1), and post-traumatic wound healing disorder (meshed skin transplantation, n=1). Survival was 50% in the group with surgery and 62% in the group without (p=0.275). Even though this difference was not statistically significant, surgical intervention should be encouraged in all cases to achieve favorable results.
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Cistite/cirurgia , Doença Enxerto-Hospedeiro/cirurgia , Transplante de Células-Tronco Hematopoéticas , Hemorragias Intracranianas/cirurgia , Micoses/cirurgia , Osteonecrose/cirurgia , Complicações Pós-Operatórias/cirurgia , Adolescente , Adulto , Criança , Pré-Escolar , Cistite/epidemiologia , Cistite/etiologia , Feminino , Doença Enxerto-Hospedeiro/epidemiologia , Doença Enxerto-Hospedeiro/etiologia , Transplante de Células-Tronco Hematopoéticas/mortalidade , Humanos , Incidência , Lactente , Recém-Nascido , Hemorragias Intracranianas/epidemiologia , Hemorragias Intracranianas/etiologia , Modelos Logísticos , Masculino , Micoses/epidemiologia , Micoses/etiologia , Razão de Chances , Osteonecrose/epidemiologia , Osteonecrose/etiologia , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida , Resultado do Tratamento , Adulto JovemRESUMO
BACKGROUND/AIM: Fatigue and asthenia are common in patients with cancer; and identifying the cause as drug toxicity versus cancer progression is difficult, particularly in clinical trials without control arms. MATERIALS AND METHODS: We carried out a systematic literature review of fatigue in placebo arms of randomized cancer trials reported in PubMed from 2000 to 2021. RESULTS: Fatigue/asthenia were reported in 100 out of 134 placebo cohorts, and the average of reported frequencies was 22.8%, with a range of 0-83%. Grade 3 or higher fatigue/asthenia was reported in 2.3% (0-17%). Fatigue/asthenia was positively correlated with nausea (R=0.683) Conclusion: For detection of drug toxicity, observations should be flagged when they are higher than the maximum reported in the placebo arm, and the assessment should be supplemented by comparing observations in early oncology trials to literature placebo arms, including both sample sizes and event numbers.
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Fadiga/etiologia , Neoplasias/complicações , Humanos , Ensaios Clínicos Controlados Aleatórios como AssuntoRESUMO
BACKGROUND/AIM: Adverse events (AEs) in cancer trials may be caused by the investigational agents or the underlying disease. Determining the causality is challenging, especially in early cancer drug development when a control arm is lacking. MATERIALS AND METHODS: We carried out a systematic literature review of AE frequencies in placebo arms of randomized trials for malignant solid tumors and hematologic malignancies reported in PubMed from 2016 to January 2022. RESULTS: Among 148 placebo arms, the AEs with the highest reported mean frequencies among all publications were: Fatigue (20.1%), nausea (16.3%), diarrhea (14.3%), abdominal pain (12.4%), and anemia (10.9%); AEs resulting in drug discontinuation were reported in 5.6% of placebo-treated patients and serious AEs in 18.7% of placebo patients. CONCLUSION: The data presented here may be used as a benchmark to help assess drug causality in early development cancer studies without a control arm.
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Antineoplásicos , Neoplasias , Antineoplásicos/efeitos adversos , Fadiga , Humanos , Náusea/tratamento farmacológico , Neoplasias/tratamento farmacológicoRESUMO
INTRODUCTION: In Germany, many health care providers work in bereavement care. An epidemiological study of this field of work has not yet been conducted. METHODS: In the initial survey of this three-phase trend study the situation of grief-specific health care in Germany in 2009/2010 is examined, i. e., at the time before the introduction of the new ICD-11 diagnosis of Prolonged Grief Disorder. For this purpose, bereavement care providers at different organizational levels participated in an online survey. RESULTS: 410 questionnaires were included in the study. Data analysis was descriptive. The most frequent reason for using grief-specific support services was the loss of a partner. In more than half of all cases of bereavement, people experienced a loss that was preceded by an illness and suffering. More than half of those providing bereavement care do not follow a concept of intervention. DISCUSSION: There is considerable need for further development in German bereavement care, in particular with regard to qualification and the degree of professionalization, designation of the respective interventions, diagnostics, and intervention. CONCLUSION: This three-phase trend study enables health care providers to derive bereavement care service standards that aim to treat people according to their needs. Whether the diagnosis of Prolonged Grief Disorder has led to changes in bereavement care is currently analyzed in the second survey phase of the study.
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Cuidados Paliativos na Terminalidade da Vida , Análise de Dados , Alemanha , Pessoal de Saúde , Humanos , Estudos RetrospectivosRESUMO
Introduction: Patients under palliative home care have special needs for their end-of-life support, which in general does not automatically include cardiopulmonary resuscitation. However, emergency medical services (EMS) respond to emergencies in children under palliative care that lead to cardiopulmonary resuscitation. To understand the underlying steps of decision-making, this retrospective, cross-sectional, multicenter study aimed to analyze pediatric patients under palliative home care who had been resuscitated. Methods: This study included patients from three spezialized pediatric palliative home care (SHPC) teams. The primary study parameters were the prevalence of cardiopulmonary resuscitation and the decision-making for carrying out pediatric advanced life support (PALS). Further analyses included the causes of cardiac arrest, the type of CPR (basic life support, advanced life support), the patient´s outcome, and involvement of the SHPC in the resuscitation. Descriptive statistical analysis was performed. Results: In total, 880 pediatric patients under palliative home care were included over 8.5 years, of which 17 patients were resuscitated once and two patients twice (overall, 19 events with CPR, 21.6 per 1,000 cases). In 10 of the 19 incidents (52.6%), cardiac arrest occurred suddenly without being predictable. The causes of cardiac arrest varied widely. PALS was performed in 78.9% of the cases by EMS teams. In 12 of 19 events (63.2%) resuscitation was performed on explicit wish of the parents. However, from a medical point of view, only four resuscitation attempts were reasonable. In total 7 of 17 (41.2%) patients survived cardiac arrest with a comparable quality of life. Discussion: Overall, resuscitation attempts were rare events in children under home palliative therapy, but if they occur, EMS are often the primary caregivers. Most resuscitation attempts occurred on explicit wish of the parents independently of the meaningfulness of the medical procedure. Despite the presence of a life-limiting disease, survival with a similar quality was achieved in one third of all resuscitated patients. This study indicates that EMS should be trained for advanced life support in children under home palliative therapy and SHPC should address the scenario of cardiac arrest also in early stages of palliative treatment. These results underline that advance care planning for these children is urgently needed.
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The loss of a loved one can have serious health implications. In Germany, however, bereavement care services often provide support regardless of risk or need. A structural framework within which these services are provided systematically and which enables the establishment of qualitative standards throughout Germany has not yet been proposed. A British stepped care model for professionalized bereavement support is actually being discussed internationally. In this paper the British model is adapted to the German context in order to improve nationwide bereavement care services.
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Luto , Cuidados Paliativos na Terminalidade da Vida , Alemanha , Humanos , Apoio SocialRESUMO
Context: Children with life-limiting diseases suffer from gastrointestinal (GI) symptoms. Since the introduction of specialized palliative home care (SPHC) in Germany, it is possible to care for these children at home. In phase 1 of care the aim is to stabilize the patient. In phase 2, terminal support is provided. Objectives: Analysis were performed of the differences between these phases. The causes and modalities/outcome of treatment were evaluated. Methods: A retrospective study was performed from 2014 to 2020. All home visits were analyzed with regard to the abovementioned symptoms, their causes, treatment and results. Results: In total, 149 children were included (45.9% female, mean age 8.17 ± 7.67 years), and 126 patients were evaluated. GI symptoms were common in both phases. Vomiting was more common in phase 2 (59.3 vs. 27.1%; p < 0.001). After therapy, the proportion of asymptomatic children in phase 1 increased from 40.1 to 75.7%; (p < 0.001). Constipation was present in 52.3% (phase 1) and 54.1% (phase 2). After treatment, the proportion of asymptomatic patients increased from 47.3 to 75.7% in phase 1 (p < 0.001), and grade 3 constipation was reduced from 33.9 to 15% in phase 2 (p < 0.05). Conclusion: Painful GI symptoms occur in both palliative care phases but are more common in phase 2. The severity and frequency can usually be controlled at home. The study limitations were the retrospective design and small number of patients, but the study had a representative population, good data quality and a unique perspective on the reality of outpatient pediatric palliative care in Germany.
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Introduction: Specialized palliative home care (SPHC) enables children and adolescents with life-limiting illnesses and complex needs to receive care at home. In addition to controlling symptoms and stabilizing the psychosocial situation, crisis anticipation is a component of SPHC. Since the establishment of the reporting SPHC team, parents have called for additional help from emergency medical services (EMS) in emergency situations with unexpected frequency. Children with life limiting diseases could undergo invasive procedures and unhelpful treatments with uncertain consequences. The questions arose as to which factors led to the involvement of the EMS in a palliative situation, what therapy was performed and what outcome could be reached. Methods: Records of the pediatric SPHC patients and EMS call-outs in these children of the reporting SPHC-team in the central region of Hesse, Germany (population: 1.1 million) were retrospectively analyzed from 01.11.2014 to 01.05.2021. The causes of the call-outs, the existence of an emergency agreement, the National Advisory Committee for Aeronautics (NACA) score, EMS therapy and outcome were examined. Patient data included age, palliative-justifying diagnosis, duration and intensity of care, place of death and median overall survival (MOS) and palliative SHPC treatment. Results: In total, 172 patients were analyzed during the study period. There were 27 EMS calls for a total of 20 patients/families (= EMS group). Palliative illness or a complication was the most frequent cause of call-outs. The patients in the EMS group were significantly less likely to have a DNR order, required more home visits and telephone calls and were under SPHC care for longer. There was a significantly higher proportion of crisis interventions at home visits. The children in the EMS group died less often from the underlying disease. Of the remaining 152 patients (= non-EMS group), a significantly higher proportion had a European home country. Conclusions: Despite the introduction of the SPHC, parents still call the EMS. Good cooperation and joint training should be sought to prepare all those involved for future call-outs.