Current status of pediatric palliative care and decision making in Japan.

Specialized pediatric palliative care (PPC) in Japan evolved primarily to care for children with cancer. As a result, the system for providing PPC to pediatric cancer patients is much more advanced than systems for non-cancer patients with life-limiting diseases. About 40% of pediatric cancer patients die at home, while most non-cancer patients die in the hospital under intensive care. In Japan, the wishes of the family tend to precede the children in decision-making for children who lack decision-making capacity. This is true even for adolescents whose medical treatment decisions may not fully reflect the wishes and preferences of the teen patients. There are efforts to empower adolescents to participate in shared decision making.

The effect of palliative care team intervention and symptom improvement using patient-reported outcomes: a multicenter prospective observational study.

PURPOSE: Hospital-based palliative care teams (HPCTs) are widespread internationally, but multicenter studies about their effectiveness, using patient-reported outcomes (PROs), are limited to Australia and a few other countries. We conducted a multicenter, prospective observational study in Japan to explore the effectiveness of the HPCTs using PROs. METHODS: Nationwide, eight hospitals participated in the study. We included newly referred patients for one month in 2021 and followed them for one month. We asked the patients to complete the Integrated Palliative Care Outcome Scale or the Edmonton Symptom Assessment System as PROs at the time of the intervention, three days later, and weekly after the intervention. RESULTS: A total of 318 participants were enrolled, of whom 86% were patients with cancer, 56% were undergoing cancer treatment, and 20% received the Best Supportive Care. After one week, the following 12 symptoms showed more than a 60% improvement from severe to moderate or less: vomiting (100%), shortness of breath (86%), nausea (83%), practical problems (80%), drowsiness (76%), pain (72%), poor sharing of feelings with family or friends (72%), weakness (71%), constipation (69%), not feeling at peace (64%), lack of information (63%), and sore or dry mouth (61%). Symptoms with improvement from severe/moderate to mild or less were vomiting (71%) and practical problems (68%). CONCLUSION: This multicenter study showed that HPCTs effectively improved symptoms in several severe conditions, as assessed by PROs. This study also demonstrated the difficulty of relieving symptoms in patients in palliative care and the need for improved care.

Current status of intensive end-of-life care in children with hematologic malignancy: a population-based study.

BACKGROUND: Adult patients with hematologic malignancies are less likely to receive palliative care and more likely to accept intensive anti-cancer treatments until end-of-life than those with solid tumors, but limited data are available regarding the quality of end-of-life care (EOLC) for children with hematologic malignancies. To improve the quality of EOLC for children with hematologic malignancies, the aims of this study were (i) to compare intensive EOLC between children with hematologic malignancies and those with solid tumors; and (ii) to describe factors associated with intensive EOLC in children with hematologic malignancies. METHODS: We retrospectively reviewed 0- to 18-year-old patients with cancer, who died in hospital between April 2012 and March 2016 in Japan using the Diagnosis Procedure Combination per-diem payment system. Indicators of intensive inpatient EOLC were defined as intensive care unit admission, cardiopulmonary resuscitation (CPR), intubation and/or mechanical ventilation, hemodialysis, or extra-corporeal membrane oxygenation in the last 30 days of life, or intravenous chemotherapy in the last 14 days. We determined factors associated with intensive EOLC using regression models. Data regarding use of blood transfusion were also obtained from the database. RESULTS: Among 1199 patients, 433 (36%) had hematological malignancies. Children with hematologic malignancies were significantly more likely than those with solid tumors to have intubation and/or mechanical ventilation (37.9% vs. 23.5%), intensive care unit admission (21.9% vs. 7.2%), CPR (14.5% vs. 7.7%), hemodialysis (13.2% vs. 3.1%) or extra-corporeal membrane oxygenation (2.5% vs. 0.4%) in their last 30 days, or intravenous chemotherapy (47.8% vs. 18.4%; all P < .01) within their last 14 days of life. Over 90% of children with hematological malignancies received a blood transfusion within the last 7 days of life. For hematological malignancies, age under 5 years was associated with CPR and ≥ 2 intensive EOLC indicators. Longer hospital stays had decreased odds of ≥ 2 intensive EOLC indicators. CONCLUSION: Children with hematologic malignancies are more likely to receive intensive EOLC compared to those with solid tumors. A younger age and shorter hospital stay might be associated with intensive EOLC in children with hematologic malignancies.

Severe Liver Disorder Following Liver Transplantation in STING-Associated Vasculopathy with Onset in Infancy.

PURPOSE: STING-associated vasculopathy with onset in infancy (SAVI) is a type-I interferonopathy, characterized by systemic inflammation, peripheral vascular inflammation, and pulmonary manifestations. There are three reports of SAVI patients developing liver disease, but no report of a SAVI patient requiring liver transplantation. Therefore, the relevance of liver inflammation is unclear in SAVI. We report a SAVI patient who developed severe liver disorder following liver transplantation. METHODS: SAVI was diagnosed in a 4-year-old girl based on genetic analysis by whole-exome sequencing. We demonstrated clinical features, laboratory findings, and pathological examination of her original and transplanted livers. RESULTS: At 2 months of age, she developed bronchitis showing resistance to bronchodilators and antibiotics. At 10 months of age, she developed liver dysfunction with atypical cholangitis, which required liver transplantation at 1 year of age. At 2 years of age, multiple biliary cysts developed in the transplanted liver. At 3.9 years of age, SAVI was diagnosed by whole-exome sequencing. Inflammatory cells from the liver invaded the stomach wall directly, leading to fatal gastrointestinal bleeding unexpectedly at 4.6 years of age. In pathological findings, there were no typical findings of liver abscess, vasculitis, or graft rejection, but biliary cysts and infiltration of inflammatory cells, including plasmacytes around the bile duct area, in the transplanted liver were noted, which were findings similar to those of her original liver. CONCLUSION: Although further studies to clarify the mechanisms of the various liver disorders described in SAVI patients are needed, inflammatory liver manifestations may be amplified in the context of SAVI.

Development of a Proxy Quality-of-Life Rating Scale for the End-of-Life Care of Pediatric Cancer Patients Evaluated from a Nurse's Perspective.

Background: Assessing the quality of life (QoL) of children receiving end-of-life (EoL) care through evaluations by the children and their bereaved families is challenging; presently, there is no QoL assessment measure that is appropriate for use in pediatric EoL and/or palliative care. Objective: To develop and test a proxy rating scale (the "Good Death Inventory for Pediatrics," GDI-P) for the QoL of pediatric cancer patients receiving EoL care, evaluated from the nurse's perspective, as well as a short version of the scale. Design, Setting, and Measurements: The GDI-P was developed based on previous studies. After initial testing, it was distributed to hospitals across Japan, where nurses in charge of patients with childhood cancer receiving EoL care used the scale to evaluate a patient retrospectively. To examine inter-rater reliability, we encouraged two nurses to evaluate one patient. The GDI-P was modified on the basis of the responses, and the validity and reliability were measured. Results: In total, 85 questionnaires were completed, including 32 pairs of responses from two nurses evaluating one patient. In addition, 47 retest questionnaires were returned. The final, modified GDI-P comprised eight factors with 22 items and showed high convergent and discriminant validity, scaling success rates for each item and factor, and Cronbach's α values. A short version of GDI-P was prepared, comprising eight representative items. Conclusions: The final GDI-P was confirmed to have adequate reliability and validity. The QoL scale developed in this study should provide useful outcome evaluation criteria for assessing the EoL care of pediatric cancer patients.

Number of Unused Medications at the Time of Last Admission: A Prospective Observational Study in a Single Palliative Care Unit.

BACKGROUND:: Unused medications (UM) are an important issue, with the waste associated with UM a burden to the health-care system. The aims of this study were to clarify the amount and costs of UM in patients with advanced cancer at the time of their last admission to a palliative care unit and to explore the factors contributing to the cost of UM and how patients dealt with UM. METHODS:: A prospective observational study was conducted in single palliative care unit. Unused medications were classified into 6 categories and the number and cost of UM by category calculated per patient. Patients were classified into 2 cost groups (high and low) based on the total cost of UM, and the number and cost of UM by category were compared between these 2 groups. RESULTS:: Of 194 consecutive hospitalized patients, data were analyzed for 90. The mean number and cost of UM per patient was 440 and US$301, respectively. Opioids accounted for 47% of the cost of UM. Comparing costs by UM category, the proportion of opioids (51% vs 21%; P < .0001) and oral anticancer drugs (14% vs 3%; P = .02) was higher in the high- than in the low-cost group. CONCLUSION:: Based on the results of the present study, the estimated annual waste cost of UM for patients with cancer who died in Japan was approximately US$110 million. Interventions to educate patients regarding UM and to eliminate barriers to opioid use may help reduce the cost of UM, particularly opioids and anticancer drugs.

Specialist Palliative Care Service for Children With Life-Threatening Conditions: A Nationwide Survey of Availability and Utilization.

CONTEXT: According to the International Observatory on End of Life Care, the level of pediatric palliative care in Japan is Level 2 (capacity building) and the current status of palliative care for children in Japan has not been clarified. OBJECTIVES: The objective of the study was to clarify the availability and utilization of specialist palliative care services among children with life-threatening conditions in Japan. METHODS: A questionnaire was administered to assess the availability of specialist palliative care services among children with life-threatening conditions. All 427 certified regional cancer centers having hospital-based adult palliative care teams, 15 certified children's cancer centers having pediatric palliative care teams, and 368 medical institutions having a certified palliative care unit were surveyed. RESULTS: Fifteen to twenty-one percent of adult palliative care teams and more than 90% of pediatric palliative care teams had experience providing palliative care to children with cancer. By contrast, only 2%-3% of adult palliative care teams and 15% of pediatric palliative care teams had experience providing care for the noncancer population. An estimated 12% of children with cancer in Japan used hospital-based palliative care teams in 2015. Eight children used a palliative care unit in 2015, and of those, seven (88%) had a solid tumor. An estimated 1.3% of children with cancer who died in Japan used a palliative care unit. CONCLUSION: An estimated 12% of children with cancer in Japan used hospital-based palliative care teams and an estimated 1.3% of children with cancer who died in Japan used a palliative care unit in 2015.

Three years' experience with the first pediatric hospice in Asia.

OBJECTIVE: Pediatric hospice has been the adoption of several service provision models in highly developed countries such as UK, Germany, Australia or Canada for a few decades, yet it has seldom been the case in the Asian Continent. This study aimed to evaluate the newest challenge for the children with Life-threatening illness (LTI) and described the characteristic of pediatric palliative care at the first pediatric hospice in Japan. METHODS: A retrospective review of all patients at our pediatric hospice in these three years was conducted. Of the 294 cases reviewed, 269 cases were eligible for analysis. RESULTS: We reviewed 269 patients admitted during the first three years. Most patients required intensive medical intervention. Patients were hospitalized in our pediatric hospice not only for end-of-life care (EOL), but also for respite care. Only 7% of the patients were with cancer. To support children and family to make the most of their time together, we provided a range of medical and recreational care. It is expected that the pediatric hospice will extend and establish cooperation with other hospitals or community services. CONCLUSION: Three years' experience of pediatric palliative care at the first pediatric hospice in the Asian Continent is encouraging. Further experience and improved communication with other pediatric service providers as well as their education in palliative care will enhance the recognition of the capacity of our hospice and support the needs of more children. Furthermore, we would like to introduce the idea of pediatric hospice and spread it throughout the Asian Continent in the future.

Differences between Pediatricians and Internists in Advance Care Planning for Adolescents with Cancer.

OBJECTIVE: To evaluate differences between pediatricians and internists in the practice of and barriers to advance care planning (ACP) for adolescent patients with cancer. STUDY DESIGN: A self-reported questionnaire was administered to assess the practice of ACP, advance directives, and barriers to ACP for adolescent patients with cancer. All 3392 Japanese board-certified hematologists were surveyed, and 600 hematologists (227 pediatricians, 373 internists) who take care of adolescent patients with cancer with decision-making capacity were analyzed. RESULTS: If a patient's prognosis for survival was <3 months, pediatricians were significantly less likely to discuss ACP with their patients than internists, including discussions regarding the patient's medical condition (59% vs 70%), the patient's understanding of his/her medical condition (55% vs 66%), do not attempt resuscitation orders (17% vs 24%), and ventilator treatment if the patient's condition worsened (19% vs 25%). More than 75% of hematologists (both pediatricians and internists) discussed all ACP topics with patients' families. Similarly, with regard to advance directives, pediatricians were less likely than internists to discuss cardiopulmonary resuscitation (24% vs 47%) and the use of ventilators (31% vs 51%), vasopressors (24% vs 42%), and antibiotics (21% vs 31%) with their patients. Both pediatricians and internists discussed these issues more often with patients' families than with patients, especially cardiopulmonary resuscitation (98%) as well as the use of ventilators (98%) and vasopressors (91%). CONCLUSIONS: Pediatricians were less likely than internists to discuss ACP and advance directives with patients, and both pediatricians and internists tended to discuss ACP and advance directives more often with patients' families.

[Advance Care Planning in Cancer Care].

Advance care planning (ACP) is one of the most important issues to consider in providing quality end of life care for cancer patients. ACP has been described as a process whereby a patient, in consultation with health care providers, family members, and important others, makes decisions about his or her future health care, in the event he or she becomes incapable of participating in medical treatment decisions. ACP improves rates of following end of life wishes, increases patient and family satisfaction, and reduces family stress, anxiety, and depression. This article clarifies the differences among ACP, advance directives, and living wills. Additionally, we describe, based on clinical experience, how to introduce ACP most effectively for all stages of cancer care.

How to Manage Hospital-Based Palliative Care Teams Without Full-Time Palliative Care Physicians in Designated Cancer Care Hospitals: A Qualitative Study.

OBJECTIVE: To clarify how highly active hospital palliative care teams can provide efficient and effective care regardless of the lack of full-time palliative care physicians. METHODS: Semistructured focus group interviews were conducted, and content analysis was performed. RESULTS: A total of 7 physicians and 6 nurses participated. We extracted 209 codes from the transcripts and organized them into 3 themes and 21 categories, which were classified as follows: (1) tips for managing palliative care teams efficiently and effectively (7 categories); (2) ways of acquiring specialist palliative care expertise (9 categories); and (3) ways of treating symptoms that are difficult to alleviate (5 categories). CONCLUSIONS: The findings of this study can be used as a nautical chart of hospital-based palliative care team (HPCT) without full-time PC physician. Full-time nurses who have high management and coordination abilities play a central role in resource-limited HPCTs.